Gated Myocardial Perfusion Scintigraphy in Children with Sickle Cell Anemia: Correlation with Echocardiography | Revista Española de Medicina Nuclear e Imagen Molecular

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Although the rate of cardiac death was significantly related to severity of scan abnormalities ( P=0.004), however, the same association was not found between the severity of MPI abnormality and the rate of myocardial infarction ( P=0.551).**Summed stress scores < 4 were considered normal; 4 to 8, mildly abnormal; 9 to 13, moderately abnormal; and > 13, severely abnormal." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "S.M. Modarres Mosalla, N. Rezaei, H. Tavakoli, A. Gholamrezanezhad" "autores" => array:4 [ 0 => array:2 [ "Iniciales" => "S.M." "apellidos" => "Modarres Mosalla" ] 1 => array:2 [ "Iniciales" => "N." "apellidos" => "Rezaei" ] 2 => array:2 [ "Iniciales" => "H." "apellidos" => "Tavakoli" ] 3 => array:2 [ "Iniciales" => "A." "apellidos" => "Gholamrezanezhad" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X2253654X11349372?idApp=UINPBA00004N" "url" => "/2253654X/0000003000000006/v0_201308051355/X2253654X11349372/v0_201308051356/en/main.assets" ] "itemAnterior" => array:16 [ "pii" => "X2253654X11349356" "issn" => "2253654X" "estado" => "S300" "fechaPublicacion" => "2011-11-01" "documento" => "article" "crossmark" => 0 "subdocumento" => "fla" "cita" => "Rev Esp Med Nucl Imagen Mol. 2011;30:351-3" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 2600 "formatos" => array:3 [ "EPUB" => 9 "HTML" => 1978 "PDF" => 613 ] ] "es" => array:12 [ "idiomaDefecto" => true "titulo" => "Adaptación del módulo de 18FDG para la preparación de una disolución inyectable de [ 18F] fluoruro sódico de conformidad con la Farmacopea Americana (USP 32) y Europea (PhEur 6)" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "351" "paginaFinal" => "353" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Adaptation of the 18FDG module for the preparation of a sodium fluoride [ 18F] injection solution in agreement with the United States (USP 32) and European Pharmacopeia (PhEur 6)" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig1" "etiqueta" => "Figura 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "125v30n06-90034935fig1.jpg" "Alto" => 558 "Ancho" => 971 "Tamanyo" => 149688 ] ] "descripcion" => array:1 [ "es" => "Rendimiento final de la síntesis expresado en tanto por ciento (%R) en función del procedimiento de acondicionamiento de la columna (a: K 2CO 3 0.5M 10 ml y etanol 10 ml; b: K 2CO 3 0.5M 10 ml y agua para inyección 10 ml; c: etanol 10 ml y agua para inyección 10 ml)." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "T. Martínez, B. Cordero, S. Medín, A. Sánchez Salmón" "autores" => array:4 [ 0 => array:2 [ "Iniciales" => "T." "apellidos" => "Martínez" ] 1 => array:2 [ "Iniciales" => "B." "apellidos" => "Cordero" ] 2 => array:2 [ "Iniciales" => "S." "apellidos" => "Medín" ] 3 => array:2 [ "Iniciales" => "A." "apellidos" => "Sánchez Salmón" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S1578200X11000210" "doi" => "10.1016/j.remngl.2011.02.004" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578200X11000210?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X2253654X11349356?idApp=UINPBA00004N" "url" => "/2253654X/0000003000000006/v0_201308051355/X2253654X11349356/v0_201308051355/es/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "titulo" => "Gated Myocardial Perfusion Scintigraphy in Children with Sickle Cell Anemia: Correlation with Echocardiography" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "354" "paginaFinal" => "359" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "O. Hallioglu, E. Ceylan Gunay, S. Unal, A. Erdogan, S. Balci, D. Citirik" "autores" => array:6 [ 0 => array:3 [ "Iniciales" => "O." "apellidos" => "Hallioglu" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] 1 => array:4 [ "Iniciales" => "E." "apellidos" => "Ceylan Gunay" "email" => array:1 [ 0 => "ecgunay@yahoo.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "affb" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor1" ] ] ] 2 => array:3 [ "Iniciales" => "S." "apellidos" => "Unal" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "c" "identificador" => "affc" ] ] ] 3 => array:3 [ "Iniciales" => "A." "apellidos" => "Erdogan" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "affb" ] ] ] 4 => array:3 [ "Iniciales" => "S." "apellidos" => "Balci" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] 5 => array:3 [ "Iniciales" => "D." "apellidos" => "Citirik" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Mersin University, Faculty of Medicine, Department of Pediatric Cardiology, Mersin, Turkey" "etiqueta" => "a" "identificador" => "affa" ] 1 => array:3 [ "entidad" => "Mersin University, Faculty of Medicine, Department of Nuclear Medicine, Mersin, Turkey" "etiqueta" => "b" "identificador" => "affb" ] 2 => array:3 [ "entidad" => "Mersin University, Faculty of Medicine, Department of Pediatric Hematology, Mersin, Turkey" "etiqueta" => "c" "identificador" => "affc" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor1" "etiqueta" => "*" "correspondencia" => "Corresponding author. ecgunay@yahoo.com" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Gated-SPECT de Perfusión Miocárdica en Niños con Anemia Falciforme: correlación con ecocardiografía" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig1" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "125v30n06-90034936fig1.jpg" "Alto" => 2700 "Ancho" => 1834 "Tamanyo" => 1497471 ] ] "descripcion" => array:1 [ "en" => "A) Myocardial perfusion SPECT images and B) QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion." ] ] ] "textoCompleto" => "<a name="sec0005" class="elsevierStyleCrossRefs"></a>IntroductionSickle cell anemia (SCA) is a chronic and congenital hematological disease which causes crucial destroys in many organs. Although cardiac problems do not seem to be frequently observed in children with SCA, even myocardial ischemia has been reported in literature.<a href="#bib1" class="elsevierStyleCrossRefs">1</a>, <a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib3" class="elsevierStyleCrossRefs">3</a> Myocardial infarction and fibrosis were detected in 9.7% of postmortem examinations of adults and children with SCA.<a href="#bib4" class="elsevierStyleCrossRefs">4</a> Chronic anemia and iron overload are considered to be the possible causes of cardiac defects in SCA, although the exact reason is generally unknown. In recent literature, it has been suggested that cardiac abnormalities in children with SCA assumed to be related to disordered breathing, low asleep and waking oxygen saturations associated with left ventricle (LV) abnormalities.<a href="#bib5" class="elsevierStyleCrossRefs">5</a>Finding out the myocardial problems in early stages is critical to prevent cardiac problems that might possibly develop later. Treadmill and echocardiographic examinations are not sufficient to detect myocardial ischemia in SCA patients.<a href="#bib6" class="elsevierStyleCrossRefs">6</a> In the field of nuclear cardiology, Tc-99m methoxy isobutyl isonitrile (MIBI) myocardial perfusion scintigraphy is a frequently and efficiently used method in order to demonstrate myocardial perfusion defects.<a href="#bib7" class="elsevierStyleCrossRefs">7</a> By using gated data, perfusion and the left ventricular function can be evaluated at the same time.<a href="#bib8" class="elsevierStyleCrossRefs">8</a> The aim of this prospective study is to evaluate myocardial perfusion and left ventricle functions with G-MPS in children with SCA and to compare the results with echocardiographic parameters.<a name="sec0010" class="elsevierStyleCrossRefs"></a>Methods<a name="sec0015" class="elsevierStyleCrossRefs"></a>PatientsIn this study, we evaluated 43 consecutive children with SCA with a median age 12 years (ranged between 6 to 18 years). Fifteen of the patients were females; 28 were males. Children with diagnoses of homozygous SCA from the Pediatric Hematology Unit who were consulted to Pediatric Cardiology Unit and conducted to nuclear medicine department were evaluated prospectively between February 2007 and April 2009. Detailed physical examination, median levels of ferritin, pain crisis, history of splenectomy and hydroxyurea treatment as well as frequencies of transfusion, cerebrovascular accident, acute chest syndrome during the last year were recorded. The patients with the history of cerebrovascular accident and recurrent acute chest syndrome have been transfused every 3-4 weeks. Electrocardiography (ECG), echocardiography and Gated myocardial perfusion scintigraphy (G-MPS) were performed to all of the patients.The exclusion criteria were not to have sickle-cell crisis during the last 3-months period, hemoglobin SC, hemoglobin S-thalassemia and the existence of other cardiac problems or chronic diseases. In accordance with the ethical standards laid down in the Declaration of Helsinki, the permission from the local ethics committee as well as the informed consent forms were obtained for this study (Local Ethical Committee B.30.2.MEÜ.0.20.05.04/347). Control cases were not included in the study because of the radiation effect.<a name="sec0020" class="elsevierStyleCrossRefs"></a>Echocardiographic evaluationA complete two-dimensional echocardiography was performed on each subject using an ultrasound imaging system with a size-appropriate transducer. Standard M-mode and Doppler echocardiography were performed in the supine and left lateral decubitus positions using a Vivid I echocardiography machine (GE Medical Systems Ultrasound, Tirat Hacarmel, Israel). Standard parasternal, apical, subxiphoid, and suprasternal views were used. The left ventricular end systolic and end diastolic dimensions (LVESD and LVEDD) were measured directly using M-mode echocardiography according to the recommendations of the American Society of Echocardiography.<a href="#bib9" class="elsevierStyleCrossRefs">9</a> Then; shortening fraction (SF) which is a quantitative measure of systolic function has also been calculated. As for the calculation of LVEDD z score, taking the literature into consideration, above+2 SD values were accepted as a dilated ventricle.<a href="#bib10" class="elsevierStyleCrossRefs">10</a> On mitral flow, we measured maximum velocity of E (early flow velocity) and A (late flow velocity) waves and calculated E/A ratio. The myocardial performance index (MPI) of LV was calculated as the ratio of the summation of isovolumic contraction and relaxation time to the ejection time.<a href="#bib11" class="elsevierStyleCrossRefs">11</a> Higher MPI values have indicated worsening of combined systolic/diastolic function.<a name="sec0025" class="elsevierStyleCrossRefs"></a>Gated myocardial perfusion scintigraphyAll patients underwent stress G-MPS. After 3 hours of fasting, a dose of Tc-99m MIBI scaled on the basis of the body surface area (mean 12 mCi, ranged between 8-15 mCi) was injected after the pharmacological stress testing by using dypridamole (140 μg/kg/min for 4 min). Thirty minutes after intravenous injection of Tc-99m MIBI, G-MPS imaging was acquired by a 90o configuration dual detector gamma camera (e-cam, Siemens Medical Systems, Germany) equipped with low-energy high-resolution collimators. The acquisition protocol of single photon emission computed tomography (SPECT) included; 64 projections with a 1.45 zooming factor (25 s/projection, 32 per detector, matrix size 64×64) over a 180° anterior arc divided into 8 frames per cardiac cycle. G-MPS images were reconstructed using back projection algorithm and a Butterworth filter with a cut-off 0.45 cycles per pixel and order 5. The rest study was omitted if the stress myocardial perfusion was interpreted as normal. None of the patients required sedation.The images were evaluated by two nuclear medicine physicians visually as well as semi-quantitatively by the software programs, quantitative perfusion single photon emission computed tomography (QPS) and quantitative gated single-photon emission computed tomography (QGS) (Cedars-Sinai Medical Centre, Los Angeles, California USA). LV end-diastolic volume (LV-EDV), end-systolic volume (LV-ESV), ejection fraction (EF) parameters were also calculated by QGS. Index values of cardiac volumes (LV-EDVI and LV-ESVI) were derived as ml/m2 by normalizing LV-EDV and LV-ESV according to body surface area (BSA) of the patients. In G-MPS, LV-EDVI over 75 ml/m2 was accepted as dilatation depending on the LV-EDV reference values in relation to BSA.<a href="#bib12" class="elsevierStyleCrossRefs">12</a>The perfusion, motion, and thickening scores were expressed as summed stress score (SSS), summed motion score (SMS), and summed thickening score (STS). The scores were obtained as a sum of all LV regions in a 20-segment model, on a 5-point scale for each segment between 0 and 5 for SSS (0: normal perfusion; 1: mildly reduced perfusion; 2: moderately reduced perfusion; 3: severely reduced perfusion; 4: absent uptake). A likewise scoring system was used for SMS (0: normal; 1: mild hypokinesis; 2: moderate hypokinesis; 3: severe hypokinesis; 4: akinesis; 5: dyskinesis) and STS (0: normal; 1: mildly impaired; 2: moderately impaired; 3: severely impaired; 4: absent thickening), as well. The LV EF was calculated using QGS program and was compared with the echocardiographic EF values. Lung-to-heart ratio (L/H), a sign of ventricular function deterioration, was also calculated by using 5×5 pixel size regions of interests over the lung and heart on anterior projection image. The L/H was derived by measuring and dividing the counts in the left upper lung by the counts over the myocardium.<a href="#bib13" class="elsevierStyleCrossRefs">13</a> The patients’ radionuclide imaging results were compared to the clinical data and echocardiography.<a name="sec0030" class="elsevierStyleCrossRefs"></a>Statistical analysisAll quantitative data were expressed as median and the interquartile range (IQR) due not to a normal distribution. The correlations between the findings were calculated with Spearman's correlation coefficient (SCC). The SCC values were accepted as: 0-0.25= none or very weak correlation, 0.25-0.5= weak-to-moderate correlation, 0.50-0.75= good correlation, and 0.75-1 = very good correlation. Patients were divided into two groups according to the presence of left ventricular dilatation detected by both G-MPS and echocardiography. SPSS 16.0 software (SPSS Inc., Chicago, Illinois) was used for the statistical analyses. Mann Whitney U test and X2 test were applied in order to compare groups. P<0.05 was accepted as significant.<a name="sec0035" class="elsevierStyleCrossRefs"></a>ResultsThe total numbers of the patients in the studied group were 43 and 65.2% of them were males. Twelve patients were under iron chelation therapy and median ferritin level was 2,110 ng/ml. Nine of the patients were under chronic transfusion program and all of them were having iron chelation therapy with deferasirox. LV dilatation has been detected in 5 of the 6 patients who had frequent transfusion (over 10 times a year). All of the patients were having hydroxyurea treatment except seven of them. Six of the patients had right axis deviation and no arrhythmia or ST-T changes were observed in the electrocardiography of the patients. Detailed demographic data and clinical findings of the patients were mentioned in <a href="#t0005" class="elsevierStyleCrossRefs">Table 1</a>.Table 1. Demographic data and clinical findings of the patients with sickle cell anemia.<a name="t0005" class="elsevierStyleCrossRefs"></a><table><tr align="left"><td>Age (year)</td><td>11.2±3.2 (range: 6-18 years)</td></tr><tr align="left"><td>Gender</td><td>15 Female/28 male</td></tr><tr align="left"><td>Hydroxurea therapy</td><td>36 (83%)</td></tr><tr align="left"><td colspan="2"> </td></tr><tr align="left"><td colspan="2">History of painful crises in the last one year</td></tr><tr align="left"><td>No</td><td>16 (37.2%)</td></tr><tr align="left"><td>1-4</td><td>16 (37.2%)</td></tr><tr align="left"><td>5-9</td><td>9 (21%)</td></tr><tr align="left"><td>Over of 10</td><td>2 (4.6%)</td></tr><tr align="left"><td colspan="2"> </td></tr><tr align="left"><td colspan="2">Frequency of the transfusion in the last one year</td></tr><tr align="left"><td>0 (none)</td><td>22 (51.1%)</td></tr><tr align="left"><td>1-4</td><td>10 (23.3%)</td></tr><tr align="left"><td>5-9</td><td>5 (11.6%)</td></tr><tr align="left"><td>Over of 10</td><td>6 (14%)</td></tr><tr align="left"><td colspan="2"> </td></tr><tr align="left"><td>History of splenectomia</td><td>15 (34.8%)</td></tr><tr align="left"><td>History of acute chest syndrome</td><td>16 (37.2%)</td></tr><tr align="left"><td>History of cerebrovascular accident</td><td>8 (18.6%)</td></tr><tr align="left"><td>History of chelation therapy</td><td>12 (27.9%)</td></tr><tr align="left"><td>Ferritin levels of the patients taking chelation therapy</td><td>2,110 (range: 1,093-3,334)</td></tr></table>Myocardial perfusion defects were not observed by G-MPS in any of the patients (<a href="#f0005" class="elsevierStyleCrossRefs">Figure 1</a>). However, LV dilatation in 15 patients with additional septal or anterolateral hypokynesis in 2 of them was observed. A total of 15 patients (34.8%) had LV dilatation detected both by echocardiography and G-MPS. L/H ratio was within normal limits in all of the patients. SSS, SMS and L/H ratio was not statistically different from each other in patients with and without LV dilatation. However there was significant difference between G-MPS EF, echocardiographical LVEDD and LVESD and EF, SF and LV-MPI values. In patients with LV dilatation, echocardiographical LVEDD, LVESD and LV-MPI values were high whereas EF and SF values were lower significantly. Mitral E, A and E/A ratio were similar in two groups (<a href="#t0010" class="elsevierStyleCrossRefs">Table 2</a>).<a name="f0005" class="elsevierStyleCrossRefs"></a><img src="125v30n06-90034936fig1.jpg" alt="A) Myocardial perfusion SPECT images and B) QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion."></img>Figure 1. A) Myocardial perfusion SPECT images and B) QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion.Table 2. Comparison of the patients with and without LV dilatation according to the parameters of echocardiography and G-MPS.<a name="t0010" class="elsevierStyleCrossRefs"></a><table><tr align="left"><td> </td><td>With LV dilatation/motion disorder (n=15) Median (IQR)</td><td>Without LV dilatation/motion disorder (n=28) Median (IQR)</td><td>P value</td></tr><tr align="left"><td>Age (year)</td><td>13 (4.5)</td><td>10 (3.8)</td><td>NS</td></tr><tr align="left"><td>BSA (m2)</td><td>1.06 (0.29)</td><td>1.05 (0.26)</td><td>NS</td></tr><tr align="left"><td>LVEDD (mm)</td><td>45 (9)</td><td>41 (4.5)</td><td>P<0.01*</td></tr><tr align="left"><td>LVESD (mm)</td><td>29 (5.5)</td><td>24 (4)</td><td>P<0.01*</td></tr><tr align="left"><td>IVSd (mm)</td><td>8 (2.5)</td><td>8.5 (1.3)</td><td>NS</td></tr><tr align="left"><td>IVSs (mm)</td><td>11 (3)</td><td>10 (3)</td><td>NS</td></tr><tr align="left"><td>LVPWd (mm)</td><td>8 (2)</td><td>8 (2)</td><td>NS</td></tr><tr align="left"><td>LVPWs (mm)</td><td>12 (2)</td><td>12 (2.3)</td><td>NS</td></tr><tr align="left"><td>ECHO-EF (%)</td><td>67 (7)</td><td>71 (5.5)</td><td>P<0.05*</td></tr><tr align="left"><td>SF (%)</td><td>37 (5)</td><td>40 (5.3)</td><td>P<0.05*</td></tr><tr align="left"><td>LA volume (cm2)</td><td>10.4 (5.5)</td><td>9.7 (3.2)</td><td>NS</td></tr><tr align="left"><td>Mitral E</td><td>0.98 (0.13)</td><td>0.97 (0.25)</td><td>NS</td></tr><tr align="left"><td>Mitral A</td><td>0.49 (0.20)</td><td>0.57 (0.21)</td><td>NS</td></tr><tr align="left"><td>Mitral E/A</td><td>1.93 (0.67)</td><td>1.65 (0.37)</td><td>NS</td></tr><tr align="left"><td>LV-MPI</td><td>0.529 (0.171)</td><td>0.428 (0.168)</td><td>P<0.05*</td></tr><tr align="left"><td>LV-EDV</td><td>110 (43.5)</td><td>67.5 (54.8)</td><td>P<0.01*</td></tr><tr align="left"><td>LV-ESV</td><td>37 (25.5)</td><td>23.5 (14.5)</td><td>P<0.01*</td></tr><tr align="left"><td>LV-EDVI</td><td>90 (27)</td><td>65 (14.3)</td><td>P<0.01*</td></tr><tr align="left"><td>LV-ESVI</td><td>36 (14.5)</td><td>20.5 (5)</td><td>P<0.01*</td></tr><tr align="left"><td>G-MPS EF</td><td>61 (10)</td><td>67 (10.3)</td><td>P<0.01*</td></tr><tr align="left"><td>SSS</td><td>2 (4)</td><td>2 (2)</td><td>NS</td></tr><tr align="left"><td>SMS</td><td>2 (6.5)</td><td>1 (3)</td><td>NS</td></tr><tr align="left"><td>STS</td><td>3 (8)</td><td>0 (1)</td><td>P<0.01*</td></tr><tr align="left"><td>L/H</td><td>0.37 (0.04)</td><td>0.36 (0.04)</td><td>NS</td></tr></table>* Statistically significant. A: late flow velocity; BSA: body surface area; E: early flow velocity; EDV: end diastolic volume; EDVI: end diastolic volume index (EDV/m2); EF: ejection fraction; ESV: end systolic volume; ESVI: end systolic volume index (ESV/m2), G-MPS EF: gated myocardial perfusion scintigraphy ejection fraction; IVSd: interventricular septum diastolic diameter; IVSs: interventricular septum systolic diameter; L/H: lung to heart ratio; LA: left atrium; LV-MPI: left ventricle myocardial performance index; LVEDD: left ventricle end-diastolic diameter; LVESD: left ventricle end-systolic diameter; LVPWd: left ventricle posterior wall diastolic diameter; LVPWs: left ventricle posterior wall systolic diameter; NS: not significant; SF: shortening fraction; SMS: summed motion score; SSS: summed stress score; STS: summed thickening score. The calculated median (IQR) values of EF in G-MPS and corresponding echocardiography (ECHO-EF) were 65 (12), and 70 (7), respectively. The difference between EF values calculated by G-MPS and echocardiography was statistically significant (p<0.05). There was no meaningful (or very weak) relation between G-MPS EF and ECHO-EF (SCC: 0.217). A negative correlation was obtained between G-MPS EF and LVEDD as well as LVESD (SCC: –0.381, –0.374 respectively). There was a good correlation between LV ESV-EDV and LVEDD-LVESD parameters (<a href="#t0015" class="elsevierStyleCrossRefs">Table 3</a>).Table 3. Correlation between Gated EF, ESV, EDV and LVESD, LVDD, ECHO-EF.<a name="t0015" class="elsevierStyleCrossRefs"></a><table><tr align="left"><td> </td><td>LVESD</td><td>LVEDD</td><td>ECHO-EF</td></tr><tr align="left"><td>Gated EF</td><td>–0.374</td><td>–0.381</td><td>0.177</td></tr><tr align="left"><td> </td><td>P=0.014*</td><td>P=0.012*</td><td>P=0.256</td></tr><tr align="left"><td>ESV</td><td>0.636</td><td>0.683</td><td>–0.252</td></tr><tr align="left"><td> </td><td>P<0.001*</td><td>P<0.001*</td><td>P=0.103</td></tr><tr align="left"><td>EDV</td><td>0.633</td><td>0.699</td><td>–0.197</td></tr><tr align="left"><td> </td><td>P<0.001*</td><td>P<0.001*</td><td>P=0.206</td></tr></table>* Statistically significant. ECHO-EF: echocardiographical ejection fraction; EDV: end-diastolic volume; EF: ejection fraction; ESV: end-systolic volume; LVEDD: left ventricle end-diastolic diameter; LVESD: left ventricle end-systolic diameter. On the basis of the clinical findings, statistically significant relations between LV dilatation/motion disorder and frequent transfusion (over 10 times/year) have been detected (p<0.05). There were no meaningful relations between the other clinical parameters and LV dilatation/motion disorder.<a name="sec0040" class="elsevierStyleCrossRefs"></a>DiscussionMyocardial perfusion scintigraphy in childhood has been used for many purposes such as myocarditis, congenital AV block, dilated cardiomyopathy and systemic lupus erythematosus.<a href="#bib8" class="elsevierStyleCrossRefs">8</a>, <a href="#bib14" class="elsevierStyleCrossRefs">14</a>, <a href="#bib15" class="elsevierStyleCrossRefs">15</a>, <a href="#bib16" class="elsevierStyleCrossRefs">16</a> Although myocardial perfusion scintigraphy in patients with SCA has been studied before, there is limited data on gated-SPECT studies in children with SCA. In this study, no perfusion defect has been detected in none of the patients. In the literature, apparently there are three studies published by a French study group on sickle cell disease which were established to assess myocardial perfusion using Thallium-201 SPECT in children.<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib17" class="elsevierStyleCrossRefs">17</a>, <a href="#bib18" class="elsevierStyleCrossRefs">18</a> In these studies differing from ours, significant perfusion defects in SCA patients were observed.<a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib16" class="elsevierStyleCrossRefs">16</a>, <a href="#bib17" class="elsevierStyleCrossRefs">17</a> For instance, in one of the studies concerning 23 patients, an impairment of myocardial perfusion in 61% (14 of 23) of the patients has been found, existing myocardial ischemia in 39% of the cases.<a href="#bib19" class="elsevierStyleCrossRefs">19</a> On the other hand, Montalambert et al have studied the patients with chest pain, heart failure, abnormal electrocardiogram which might possibly be the cause of increased rate of perfusion defects.<a href="#bib18" class="elsevierStyleCrossRefs">18</a> In that study myocardial perfusion defects were detected in all of the patients with cardiac symptoms such as heart failure, ventricular arrhythmias or angina. In our study, 43 children with SCA between the ages of 6 and 18 were the subjects who were consulted to nuclear medicine department in order to detect any myocardial perfusion impairment, but none of them showed any perfusion defects. Most of the patients had nonspecific chest pain which may not be directly related to cardiac disorders, but may frequently be seen in SCA. Aessopos et al have found out myocardial ischemia during treadmill exercise test within 27% of 30 patients with sickle beta thalassemia who had no cardiac symptoms.<a href="#bib19" class="elsevierStyleCrossRefs">19</a> Coronary angiograms of these patients were normal. Thus, they emphasize that the sickling process, activated by exercise, could be the common underlying mechanism.<a href="#bib19" class="elsevierStyleCrossRefs">19</a> No occlusion of the major coronary arteries was found in postmortem examinations of patients with SCA which strongly suggests that microcirculation is involved.<a href="#bib20" class="elsevierStyleCrossRefs">20</a>, <a href="#bib21" class="elsevierStyleCrossRefs">21</a> Coronary autopsies found out the thrombosis of the microcirculation.<a href="#bib20" class="elsevierStyleCrossRefs">20</a> Consequently, it can probably be thought that, if the children with SCA have no cardiac symptoms, coronary involvement and myocardial ischemia may not be an expected finding except exercise.On the other hand, cardiac enlargement is a well known observation in children with SCA.<a href="#bib21" class="elsevierStyleCrossRefs">21</a> In these patients, cardiomyopathies are described from echocardiographic data and exhibit LV dilatation and normal contraction. Such cardiomyopathies are not specific and are usually free of cardiac symptoms.<a href="#bib22" class="elsevierStyleCrossRefs">22</a> Lester et al suggest that the major cardiac abnormalities in children are related to the volume overload effects of chronic anemia in children and there is no evidence for a distinct “sickle cell cardiomyopathy” or cardiac dysfunction. They found 60% of cases with LV dilatation in 64 children with SCA.<a href="#bib22" class="elsevierStyleCrossRefs">22</a> LV dilatation in our cases were present in only 34.8% of them and none of the patients have had cardiomyopathy. Apart from these, in our study, we detected only one statistically significant relation among the clinical data which was between LV dilatation/motion disorder and frequent transfusions. It is known that, especially in frequently transfused patients, cardiac toxicity may develop because of iron overload.A clinical improvement of SCA has been reported using hydroxyurea treatment. The beneficial effects of hydroxyurea therapy on the prognosis of the patients with SCA is commonly known.<a href="#bib23" class="elsevierStyleCrossRefs">23</a> It may be related to both an increase of fetal hemoglobin synthesis responsible for an inhibition of hemoglobin S polymerization, and a decrease of highly adhesive reticulocytes.<a href="#bib24" class="elsevierStyleCrossRefs">24</a> It is also believed that hydroxyurea can improve hypoperfusion.<a href="#bib22" class="elsevierStyleCrossRefs">22</a> Since all of our patients except 7 of them use hydroxyurea, the effects of the medicine cannot be considered.Montalambert et al have emphasized that the age of the patients with SCA who had perfusion defects were older and they had vasooclusive crisis more frequently.<a href="#bib18" class="elsevierStyleCrossRefs">18</a> In our study, although the ages of the patients with LV dilatation/motion disorder were older than the others, there was no statistically significant difference. However, since the p value was very close to 0.05 (p=0.052), in case of increasing the number of patients, it is assumed that statistical difference may occur.All of the ECHO-EF and G-MPS EF values of the patients were within normal range in our study. Nevertheless, there was no meaningful relation between G-MPS EF and ECHO-EF. Since ECHO-EF values get deteriorated in the late period, it may not be useful to take EF values into consideration on the follow-up of patients with no cardiac symptoms. G-MPS is reported to be correlated to echocardiography on the measurement of LV EF and volume in adults,<a href="#bib25" class="elsevierStyleCrossRefs">25</a> whereas there is limited data on childhood. The role of G-MPS, as an alternative measurement, has yet to be determined for children. Kıratlı and Bozkurt demonstrated poor agreement between gated myocardial perfusion scintigraphy and echocardiography, and found G-MPS less suitable for assessing left ventricular volumes and ejection fraction in children.<a href="#bib26" class="elsevierStyleCrossRefs">26</a> The left ventricular cavity in children is much smaller than adults, which may result in worse resolution, thus creating difficulties for automatic programs. On the other hand, in a study by Nakajima et al, LV volume and EF as assessed by gated SPECT in pediatric studies were in agreement with echocardiographic measurements. The quantitative reliability was enhanced by SPECT filters with a high cut-off frequency, a high system resolution and appropriate zooming.<a href="#bib27" class="elsevierStyleCrossRefs">27</a> In our study, scintigraphically obtained LV-EDV and LV-ESV values as well as echocardiographically obtained LVEDD and LVESD parameters were correlated with each other. However, EF values in echocardiography and G-MPS were not correlated to each other. The reason of this is probably preferring the M-Mode echocardiography which has been widely used in children instead of using Modified Simpson Method. As for the EF calculation, while LV dimensions are used in M-Mode echocardiography process, LV volumes are used in Modified Simpson Method. In another study in the literature which is parallel to ours, G-MPS EF values calculated by QPS were found out less than EF values obtained by M-Mode echocardiography.<a href="#bib26" class="elsevierStyleCrossRefs">26</a> If we had used Modified Simpson Method instead of M-Mode during EF calculations of echocardiography, the results would have been different.According to our knowledge, normal values for cardiac volumes calculated by G-MPS for children are not determined in literature. However it has been suggested that variations in reference values of cardiac volumes would be reduced by normalization to body surface area (BSA) calculated from the subject's height and weight.<a href="#bib28" class="elsevierStyleCrossRefs">28</a> For that reason, we have preferred to calculate index values (LV-EDVI) by dividing LV-EDV to BSA for normalization. In our study, LV volume over 75 ml/m2 in G-MPS was accepted as dilatation depending on the reference values in a study by Sharir et al.<a href="#bib12" class="elsevierStyleCrossRefs">12</a> LV dilatation assumed by LV-EDVI in G-MPS was correlated to LV dilatation detected in echocardiography. However the benefits that obtained by G-MPS was not sufficient to put that test as a high priority examination in SCA.MPI is an acceptable and simple index which consists of Doppler-derived systolic and diastolic time intervals to generate a combined index of global ventricular function that is independent of geometric assumptions. Higher MPI values have been found to correlate with worsening myocardial systolic and diastolic function.<a href="#bib29" class="elsevierStyleCrossRefs">29</a> Among a number of studies, it has been reported that LV-MPI values increased in children with SCA.<a href="#bib30" class="elsevierStyleCrossRefs">30</a>, <a href="#bib31" class="elsevierStyleCrossRefs">31</a> In this study, parallel to those, LV-MPI values were significantly higher in the patients who had LV dilatation/motion disorder which is supposed to depend on volume overload.One of the limitations of our study was the relatively low number of the patients since SCA is not a frequently seen hematological disease. Another point is that the normal reference values of quantitative evaluation by G-MPS have not been clearly determined in childhood in litrature. That is the reason that we preferred to use index values in order to define LV dilatation in G-MPS. But it should also be kept in mind that index normalization may be in limited value if the BSA of the patient examined is very low.<a href="#bib28" class="elsevierStyleCrossRefs">28</a> Comparing our results with a control group would be more reliable, however control cases were not included in our study because of the radiation effects.In conclusion, myocardial perfusion defect in SCA is not a frequently observed finding in childhood period. Due to the fact that, it may not be considerable to perform scintigraphy to the children who have no serious cardiac symptoms such as ECG abnormality, angina and heart failure. In order to detect LV dilatation, although scintigraphic process adjusted to BSA seems to be correlated to echocardiography, due to the radiation effect it should not be considered to have a priority. Despite of the fact that, EF values of the children with SCA are not deteriorated in early stages, it can be useful to have the measurements of LVDD and LV-MPI in follow-up to detect the cardiac problems that might possibly develop later. In addition, in patients with sickle cell anemia who takes frequent transfusion, it should be kept in mind that LV dilatation/motion disorders may develop.<a name="sec0045" class="elsevierStyleCrossRefs"></a>Conflicts of interestThe authors have no conflicts of interest to declare.Received 10 November 2010 Accepted 1 February 2011 Corresponding author. ecgunay@yahoo.com" "pdfFichero" => "125v30n06a90034936pdf001.pdf" "tienePdf" => true "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec241017" "palabras" => array:5 [ 0 => "Myocardial perfusion" 1 => "scintigraphy" 2 => "echocardiography" 3 => "sickle cell anemia" 4 => "children" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec241018" "palabras" => array:5 [ 0 => "Perfusión miocárdica" 1 => "gammagrafía" 2 => "ecocardiografía" 3 => "anemia de células falciformes" 4 => "niños" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:1 [ "resumen" => "Objectives The heart is one of the organs affected by sickle cell anemia (SCA). This prospective study has aimed to evaluate myocardial perfusion and left ventricle (LV) function in children with SCA by gated myocardial perfusion scintigraphy (G-MPS) and to compare the results with echocardiographic parameters.Methods Forty-three patients with SCA were evaluated by G-MPS and echocardiography. Myocardial perfusion and motion with thickening function were analyzed both visually and quantitatively. End-diastolic (EDV), end-systolic volumes (ESV), ejection fraction (EF), lung-to-heart (L/H) ratio were also calculated.Results None of the patients showed stress perfusion impairment in G-MPS. LV dilatation in 15 patients was observed both by G-MPS and echocardiography. EF values were within normal limits. Correlation between EF values calculated by two methods was not statistically significant. However, LV dilatation detected by both methods and EDV-ESV values in G-MPS were correlated to end-diastolic and end-systolic diameters calculated in echocardiography (p<0.05). M-Mode echocardiography revealed higher myocardial performance index (LV-MPI) in patients with LV dilatation. There was also a significant relationship between LV dilatation and frequent blood transfusions (>5/years) and acute chest syndrome (p<0.05).Conclusion Myocardial perfusion impairment in children with SCA is not frequently observed. Thus, performing the scintigraphy only in patients with cardiac symptoms should be considered. Since EF values of the children with SCA are not deteriorated in early stages, LV-MPI and LV dilatation should be considered as a significant parameter other than EF or perfusion data." ] "es" => array:1 [ "resumen" => "Objetivo El corazón es uno de los órganos afectados por la anemia de células falciforme (SCA). El objetivo de este estudio prospectivo es evaluar la perfusión miocárdica y la función del ventrículo izquierdo (LV) en niños con SCA por la gammagrafía de perfusión miocárdica (G-MPS) y comparar los resultados con los hallazgos ecocardiográficos.Métodos Se evaluaron cuarenta y tres pacientes con SCA mediante estudios de G-MPS y ecocardiografía. La perfusión miocárdica y la función ventricular con la valoración de la motilidad y del engrosamiento se analizaron tanto de forma visual como cuantitativa. Se calcularon el EDV, ESV, EF, y el índice pulmón /corazón (L/H).Resultados Ninguno de los pacientes presentó deterioro de la perfusión post-estrés en el G-MPS. Se observó dilatación del LV en 15 pacientes, que también estuvo presente en el ecocardio. Los valores de EF se encontraban dentro de los límites normales. La correlación entre los valores de EF, mediante ambos métodos no fueron estadísticamente significativas. Sin embargo la dilatación del ventrículo izquierdo detectados por ambos métodos y los valores de EDV-ESV en la G-MPS se correlacionaron con los diámetros sistólico y diastólico final calculados por la ecocardiografía (p<0,05). La ecocardiografía en modo-M mostró mayor índice de rendimiento miocárdico (LV-MPI) en pacientes con dilatación del LV. También hubo una relación significativa entre la dilatación del ventrículo izquierdo y la frecuencia de transfusiones sanguíneas (> 5/año) y el síndrome torácico agudo (p <0,05).Conclusión El deterioro de la perfusión miocárdica en los niños con SCA no se observa con frecuencia. Debe considerarse el realizar la gammagrafía sólo en pacientes con síntomas cardíacos. Puesto que los valores de EF de los niños con SCA no se deterioran en las primeras etapas, el LV-MPI y la dilatación del ventrículo izquierdo deberían considerarse como un parámetro significativo." ] ] "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "125v30n06-90034936fig1.jpg" "Alto" => 2700 "Ancho" => 1834 "Tamanyo" => 1497471 ] ] "descripcion" => array:1 [ "en" => "A) Myocardial perfusion SPECT images and B) QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion." ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:31 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Myocardial infarction in sickle cell disease." 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Gated Myocardial Perfusion Scintigraphy in Children with Sickle Cell Anemia: Correlation with Echocardiography

Gated-SPECT de Perfusión Miocárdica en Niños con Anemia Falciforme: correlación con ecocardiografía

O.. Hallioglua, E.. Ceylan Gunayb,

Corresponding author

ecgunay@yahoo.com

Corresponding author. ecgunay@yahoo.com

, S.. Unalc, A.. Erdoganb, S.. Balcia, D.. Citirika

a Mersin University, Faculty of Medicine, Department of Pediatric Cardiology, Mersin, Turkey

b Mersin University, Faculty of Medicine, Department of Nuclear Medicine, Mersin, Turkey

c Mersin University, Faculty of Medicine, Department of Pediatric Hematology, Mersin, Turkey

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    "textoCompleto" => "<a name="sec0005" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Introduction</span><p class="elsevierStylePara">Sickle cell anemia &#40;SCA&#41; is a chronic and congenital hematological disease which causes crucial destroys in many organs&#46; Although cardiac problems do not seem to be frequently observed in children with SCA&#44; even myocardial ischemia has been reported in literature&#46;<a href="&#35;bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a> Myocardial infarction and fibrosis were detected in 9&#46;7&#37; of postmortem examinations of adults and children with SCA&#46;<a href="&#35;bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a> Chronic anemia and iron overload are considered to be the possible causes of cardiac defects in SCA&#44; although the exact reason is generally unknown&#46; In recent literature&#44; it has been suggested that cardiac abnormalities in children with SCA assumed to be related to disordered breathing&#44; low asleep and waking oxygen saturations associated with left ventricle &#40;LV&#41; abnormalities&#46;<a href="&#35;bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a></p><p class="elsevierStylePara">Finding out the myocardial problems in early stages is critical to prevent cardiac problems that might possibly develop later&#46; Treadmill and echocardiographic examinations are not sufficient to detect myocardial ischemia in SCA patients&#46;<a href="&#35;bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a> In the field of nuclear cardiology&#44; Tc-99m methoxy isobutyl isonitrile &#40;MIBI&#41; myocardial perfusion scintigraphy is a frequently and efficiently used method in order to demonstrate myocardial perfusion defects&#46;<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> By using gated data&#44; perfusion and the left ventricular function can be evaluated at the same time&#46;<a href="&#35;bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">8</span></a> The aim of this prospective study is to evaluate myocardial perfusion and left ventricle functions with G-MPS in children with SCA and to compare the results with echocardiographic parameters&#46;</p><a name="sec0010" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Methods</span><a name="sec0015" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Patients</span><p class="elsevierStylePara">In this study&#44; we evaluated 43 consecutive children with SCA with a median age 12 years &#40;ranged between 6 to 18 years&#41;&#46; Fifteen of the patients were females&#59; 28 were males&#46; Children with diagnoses of homozygous SCA from the Pediatric Hematology Unit who were consulted to Pediatric Cardiology Unit and conducted to nuclear medicine department were evaluated prospectively between February 2007 and April 2009&#46; Detailed physical examination&#44; median levels of ferritin&#44; pain crisis&#44; history of splenectomy and hydroxyurea treatment as well as frequencies of transfusion&#44; cerebrovascular accident&#44; acute chest syndrome during the last year were recorded&#46; The patients with the history of cerebrovascular accident and recurrent acute chest syndrome have been transfused every 3-4 weeks&#46; Electrocardiography &#40;ECG&#41;&#44; echocardiography and Gated myocardial perfusion scintigraphy &#40;G-MPS&#41; were performed to all of the patients&#46;</p><p class="elsevierStylePara">The exclusion criteria were not to have sickle-cell crisis during the last 3-months period&#44; hemoglobin SC&#44; hemoglobin S-thalassemia and the existence of other cardiac problems or chronic diseases&#46; In accordance with the ethical standards laid down in the Declaration of Helsinki&#44; the permission from the local ethics committee as well as the informed consent forms were obtained for this study &#40;Local Ethical Committee B&#46;30&#46;2&#46;ME&#220;&#46;0&#46;20&#46;05&#46;04&#47;347&#41;&#46; Control cases were not included in the study because of the radiation effect&#46;</p><a name="sec0020" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Echocardiographic evaluation</span><p class="elsevierStylePara">A complete two-dimensional echocardiography was performed on each subject using an ultrasound imaging system with a size-appropriate transducer&#46; Standard M-mode and Doppler echocardiography were performed in the supine and left lateral decubitus positions using a Vivid I echocardiography machine &#40;GE Medical Systems Ultrasound&#44; Tirat Hacarmel&#44; Israel&#41;&#46; Standard parasternal&#44; apical&#44; subxiphoid&#44; and suprasternal views were used&#46; The left ventricular end systolic and end diastolic dimensions &#40;LVESD and LVEDD&#41; were measured directly using M-mode echocardiography according to the recommendations of the American Society of Echocardiography&#46;<a href="&#35;bib9" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">9</span></a> Then&#59; shortening fraction &#40;SF&#41; which is a quantitative measure of systolic function has also been calculated&#46; As for the calculation of LVEDD <span class="elsevierStyleItalic">z</span> score&#44; taking the literature into consideration&#44; above&#43;2 SD values were accepted as a dilated ventricle&#46;<a href="&#35;bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">10</span></a> On mitral flow&#44; we measured maximum velocity of E &#40;early flow velocity&#41; and A &#40;late flow velocity&#41; waves and calculated E&#47;A ratio&#46; The myocardial performance index &#40;MPI&#41; of LV was calculated as the ratio of the summation of isovolumic contraction and relaxation time to the ejection time&#46;<a href="&#35;bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">11</span></a> Higher MPI values have indicated worsening of combined systolic&#47;diastolic function&#46;</p><a name="sec0025" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Gated myocardial perfusion scintigraphy</span><p class="elsevierStylePara">All patients underwent stress G-MPS&#46; After 3 hours of fasting&#44; a dose of Tc-99m MIBI scaled on the basis of the body surface area &#40;mean 12 mCi&#44; ranged between 8-15 mCi&#41; was injected after the pharmacological stress testing by using dypridamole &#40;140 &#956;g&#47;kg&#47;min for 4 min&#41;&#46; Thirty minutes after intravenous injection of Tc-99m MIBI&#44; G-MPS imaging was acquired by a 90<span class="elsevierStyleSup">o</span> configuration dual detector gamma camera &#40;e-cam&#44; Siemens Medical Systems&#44; Germany&#41; equipped with low-energy high-resolution collimators&#46; The acquisition protocol of single photon emission computed tomography &#40;SPECT&#41; included&#59; 64 projections with a 1&#46;45 zooming factor &#40;25 s&#47;projection&#44; 32 per detector&#44; matrix size 64&#215;64&#41; over a 180&#176; anterior arc divided into 8 frames per cardiac cycle&#46; G-MPS images were reconstructed using back projection algorithm and a Butterworth filter with a cut-off 0&#46;45 cycles per pixel and order 5&#46; The rest study was omitted if the stress myocardial perfusion was interpreted as normal&#46; None of the patients required sedation&#46;</p><p class="elsevierStylePara">The images were evaluated by two nuclear medicine physicians visually as well as semi-quantitatively by the software programs&#44; quantitative perfusion single photon emission computed tomography &#40;QPS&#41; and quantitative gated single-photon emission computed tomography &#40;QGS&#41; &#40;Cedars-Sinai Medical Centre&#44; Los Angeles&#44; California USA&#41;&#46; LV end-diastolic volume &#40;LV-EDV&#41;&#44; end-systolic volume &#40;LV-ESV&#41;&#44; ejection fraction &#40;EF&#41; parameters were also calculated by QGS&#46; Index values of cardiac volumes &#40;LV-EDVI and LV-ESVI&#41; were derived as ml&#47;m<span class="elsevierStyleSup">2</span> by normalizing LV-EDV and LV-ESV according to body surface area &#40;BSA&#41; of the patients&#46; In G-MPS&#44; LV-EDVI over 75 ml&#47;m<span class="elsevierStyleSup">2</span> was accepted as dilatation depending on the LV-EDV reference values in relation to BSA&#46;<a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a></p><p class="elsevierStylePara">The perfusion&#44; motion&#44; and thickening scores were expressed as summed stress score &#40;SSS&#41;&#44; summed motion score &#40;SMS&#41;&#44; and summed thickening score &#40;STS&#41;&#46; The scores were obtained as a sum of all LV regions in a 20-segment model&#44; on a 5-point scale for each segment between 0 and 5 for SSS &#40;0&#58; normal perfusion&#59; 1&#58; mildly reduced perfusion&#59; 2&#58; moderately reduced perfusion&#59; 3&#58; severely reduced perfusion&#59; 4&#58; absent uptake&#41;&#46; A likewise scoring system was used for SMS &#40;0&#58; normal&#59; 1&#58; mild hypokinesis&#59; 2&#58; moderate hypokinesis&#59; 3&#58; severe hypokinesis&#59; 4&#58; akinesis&#59; 5&#58; dyskinesis&#41; and STS &#40;0&#58; normal&#59; 1&#58; mildly impaired&#59; 2&#58; moderately impaired&#59; 3&#58; severely impaired&#59; 4&#58; absent thickening&#41;&#44; as well&#46; The LV EF was calculated using QGS program and was compared with the echocardiographic EF values&#46; Lung-to-heart ratio &#40;L&#47;H&#41;&#44; a sign of ventricular function deterioration&#44; was also calculated by using 5&#215;5 pixel size regions of interests over the lung and heart on anterior projection image&#46; The L&#47;H was derived by measuring and dividing the counts in the left upper lung by the counts over the myocardium&#46;<a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a> The patients&#8217; radionuclide imaging results were compared to the clinical data and echocardiography&#46;</p><a name="sec0030" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Statistical analysis</span><p class="elsevierStylePara">All quantitative data were expressed as median and the interquartile range &#40;IQR&#41; due not to a normal distribution&#46; The correlations between the findings were calculated with Spearman&#39;s correlation coefficient &#40;SCC&#41;&#46; The SCC values were accepted as&#58; 0-0&#46;25&#61; none or very weak correlation&#44; 0&#46;25-0&#46;5&#61; weak-to-moderate correlation&#44; 0&#46;50-0&#46;75&#61; good correlation&#44; and 0&#46;75-1 &#61; very good correlation&#46; Patients were divided into two groups according to the presence of left ventricular dilatation detected by both G-MPS and echocardiography&#46; SPSS 16&#46;0 software &#40;SPSS Inc&#46;&#44; Chicago&#44; Illinois&#41; was used for the statistical analyses&#46; Mann Whitney U test and X2 test were applied in order to compare groups&#46; P&#60;0&#46;05 was accepted as significant&#46;</p><a name="sec0035" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Results</span><p class="elsevierStylePara">The total numbers of the patients in the studied group were 43 and 65&#46;2&#37; of them were males&#46; Twelve patients were under iron chelation therapy and median ferritin level was 2&#44;110 ng&#47;ml&#46; Nine of the patients were under chronic transfusion program and all of them were having iron chelation therapy with deferasirox&#46; LV dilatation has been detected in 5 of the 6 patients who had frequent transfusion &#40;over 10 times a year&#41;&#46; All of the patients were having hydroxyurea treatment except seven of them&#46; Six of the patients had right axis deviation and no arrhythmia or ST-T changes were observed in the electrocardiography of the patients&#46; Detailed demographic data and clinical findings of the patients were mentioned in <a href="&#35;t0005" class="elsevierStyleCrossRefs">Table 1</a>&#46;</p><p class="elsevierStylePara">Table 1&#46; Demographic data and clinical findings of the patients with sickle cell anemia&#46;</p><a name="t0005" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"></p><table><tr align="left"><td>Age &#40;year&#41;</td><td>11&#46;2&#177;3&#46;2 &#40;range&#58; 6-18 years&#41;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Gender</span></td><td>15 Female&#47;28 male</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Hydroxurea therapy</span></td><td>36 &#40;83&#37;&#41;</td></tr><tr align="left"><td colspan="2">&#160;</td></tr><tr align="left"><td colspan="2"><span class="elsevierStyleItalic">History of painful crises in the last one year</span></td></tr><tr align="left"><td>No</td><td>16 &#40;37&#46;2&#37;&#41;</td></tr><tr align="left"><td>1-4</td><td>16 &#40;37&#46;2&#37;&#41;</td></tr><tr align="left"><td>5-9</td><td>9 &#40;21&#37;&#41;</td></tr><tr align="left"><td>Over of 10</td><td>2 &#40;4&#46;6&#37;&#41;</td></tr><tr align="left"><td colspan="2">&#160;</td></tr><tr align="left"><td colspan="2"><span class="elsevierStyleItalic">Frequency of the transfusion in the last one year</span></td></tr><tr align="left"><td>0 &#40;none&#41;</td><td>22 &#40;51&#46;1&#37;&#41;</td></tr><tr align="left"><td>1-4</td><td>10 &#40;23&#46;3&#37;&#41;</td></tr><tr align="left"><td>5-9</td><td>5 &#40;11&#46;6&#37;&#41;</td></tr><tr align="left"><td>Over of 10</td><td>6 &#40;14&#37;&#41;</td></tr><tr align="left"><td colspan="2">&#160;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">History of splenectomia</span></td><td>15 &#40;34&#46;8&#37;&#41;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">History of acute chest syndrome</span></td><td>16 &#40;37&#46;2&#37;&#41;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">History of cerebrovascular accident</span></td><td>8 &#40;18&#46;6&#37;&#41;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">History of chelation therapy</span></td><td>12 &#40;27&#46;9&#37;&#41;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Ferritin levels of the patients taking chelation therapy</span></td><td>2&#44;110 &#40;range&#58; 1&#44;093-3&#44;334&#41;</td></tr></table><p class="elsevierStylePara">Myocardial perfusion defects were not observed by G-MPS in any of the patients &#40;<a href="&#35;f0005" class="elsevierStyleCrossRefs">Figure 1</a>&#41;&#46; However&#44; LV dilatation in 15 patients with additional septal or anterolateral hypokynesis in 2 of them was observed&#46; A total of 15 patients &#40;34&#46;8&#37;&#41; had LV dilatation detected both by echocardiography and G-MPS&#46; L&#47;H ratio was within normal limits in all of the patients&#46; SSS&#44; SMS and L&#47;H ratio was not statistically different from each other in patients with and without LV dilatation&#46; However there was significant difference between G-MPS EF&#44; echocardiographical LVEDD and LVESD and EF&#44; SF and LV-MPI values&#46; In patients with LV dilatation&#44; echocardiographical LVEDD&#44; LVESD and LV-MPI values were high whereas EF and SF values were lower significantly&#46; Mitral E&#44; A and E&#47;A ratio were similar in two groups &#40;<a href="&#35;t0010" class="elsevierStyleCrossRefs">Table 2</a>&#41;&#46;</p><a name="f0005" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"><img src="125v30n06-90034936fig1.jpg" alt="A&#41; Myocardial perfusion SPECT images and B&#41; QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion&#46;"></img></p><p class="elsevierStylePara">Figure 1&#46; A&#41; Myocardial perfusion SPECT images and B&#41; QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion&#46;</p><p class="elsevierStylePara">Table 2&#46; Comparison of the patients with and without LV dilatation according to the parameters of echocardiography and G-MPS&#46;</p><a name="t0010" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"></p><table><tr align="left"><td>&#160;</td><td>With LV dilatation&#47;motion disorder &#40;n&#61;15&#41; Median &#40;IQR&#41;</td><td>Without LV dilatation&#47;motion disorder &#40;n&#61;28&#41; Median &#40;IQR&#41;</td><td>P value</td></tr><tr align="left"><td>Age &#40;year&#41;</td><td>13 &#40;4&#46;5&#41;</td><td>10 &#40;3&#46;8&#41;</td><td>NS</td></tr><tr align="left"><td>BSA &#40;m<span class="elsevierStyleSup">2</span>&#41;</td><td>1&#46;06 &#40;0&#46;29&#41;</td><td>1&#46;05 &#40;0&#46;26&#41;</td><td>NS</td></tr><tr align="left"><td>LVEDD &#40;mm&#41;</td><td>45 &#40;9&#41;</td><td>41 &#40;4&#46;5&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>LVESD &#40;mm&#41;</td><td>29 &#40;5&#46;5&#41;</td><td>24 &#40;4&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>IVSd &#40;mm&#41;</td><td>8 &#40;2&#46;5&#41;</td><td>8&#46;5 &#40;1&#46;3&#41;</td><td>NS</td></tr><tr align="left"><td>IVSs &#40;mm&#41;</td><td>11 &#40;3&#41;</td><td>10 &#40;3&#41;</td><td>NS</td></tr><tr align="left"><td>LVPWd &#40;mm&#41;</td><td>8 &#40;2&#41;</td><td>8 &#40;2&#41;</td><td>NS</td></tr><tr align="left"><td>LVPWs &#40;mm&#41;</td><td>12 &#40;2&#41;</td><td>12 &#40;2&#46;3&#41;</td><td>NS</td></tr><tr align="left"><td>ECHO-EF &#40;&#37;&#41;</td><td>67 &#40;7&#41;</td><td>71 &#40;5&#46;5&#41;</td><td>P&#60;0&#46;05&#42;</td></tr><tr align="left"><td>SF &#40;&#37;&#41;</td><td>37 &#40;5&#41;</td><td>40 &#40;5&#46;3&#41;</td><td>P&#60;0&#46;05&#42;</td></tr><tr align="left"><td>LA volume &#40;cm<span class="elsevierStyleSup">2</span>&#41;</td><td>10&#46;4 &#40;5&#46;5&#41;</td><td>9&#46;7 &#40;3&#46;2&#41;</td><td>NS</td></tr><tr align="left"><td>Mitral E</td><td>0&#46;98 &#40;0&#46;13&#41;</td><td>0&#46;97 &#40;0&#46;25&#41;</td><td>NS</td></tr><tr align="left"><td>Mitral A</td><td>0&#46;49 &#40;0&#46;20&#41;</td><td>0&#46;57 &#40;0&#46;21&#41;</td><td>NS</td></tr><tr align="left"><td>Mitral E&#47;A</td><td>1&#46;93 &#40;0&#46;67&#41;</td><td>1&#46;65 &#40;0&#46;37&#41;</td><td>NS</td></tr><tr align="left"><td>LV-MPI</td><td>0&#46;529 &#40;0&#46;171&#41;</td><td>0&#46;428 &#40;0&#46;168&#41;</td><td>P&#60;0&#46;05&#42;</td></tr><tr align="left"><td>LV-EDV</td><td>110 &#40;43&#46;5&#41;</td><td>67&#46;5 &#40;54&#46;8&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>LV-ESV</td><td>37 &#40;25&#46;5&#41;</td><td>23&#46;5 &#40;14&#46;5&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>LV-EDVI</td><td>90 &#40;27&#41;</td><td>65 &#40;14&#46;3&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>LV-ESVI</td><td>36 &#40;14&#46;5&#41;</td><td>20&#46;5 &#40;5&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>G-MPS EF</td><td>61 &#40;10&#41;</td><td>67 &#40;10&#46;3&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>SSS</td><td>2 &#40;4&#41;</td><td>2 &#40;2&#41;</td><td>NS</td></tr><tr align="left"><td>SMS</td><td>2 &#40;6&#46;5&#41;</td><td>1 &#40;3&#41;</td><td>NS</td></tr><tr align="left"><td>STS</td><td>3 &#40;8&#41;</td><td>0 &#40;1&#41;</td><td>P&#60;0&#46;01&#42;</td></tr><tr align="left"><td>L&#47;H</td><td>0&#46;37 &#40;0&#46;04&#41;</td><td>0&#46;36 &#40;0&#46;04&#41;</td><td>NS</td></tr></table><p class="elsevierStylePara">&#42; Statistically significant&#46; A&#58; late flow velocity&#59; BSA&#58; body surface area&#59; E&#58; early flow velocity&#59; EDV&#58; end diastolic volume&#59; EDVI&#58; end diastolic volume index &#40;EDV&#47;m<span class="elsevierStyleSup">2</span>&#41;&#59; EF&#58; ejection fraction&#59; ESV&#58; end systolic volume&#59; ESVI&#58; end systolic volume index &#40;ESV&#47;m<span class="elsevierStyleSup">2</span>&#41;&#44; G-MPS EF&#58; gated myocardial perfusion scintigraphy ejection fraction&#59; IVSd&#58; interventricular septum diastolic diameter&#59; IVSs&#58; interventricular septum systolic diameter&#59; L&#47;H&#58; lung to heart ratio&#59; LA&#58; left atrium&#59; LV-MPI&#58; left ventricle myocardial performance index&#59; LVEDD&#58; left ventricle end-diastolic diameter&#59; LVESD&#58; left ventricle end-systolic diameter&#59; LVPWd&#58; left ventricle posterior wall diastolic diameter&#59; LVPWs&#58; left ventricle posterior wall systolic diameter&#59; NS&#58; not significant&#59; SF&#58; shortening fraction&#59; SMS&#58; summed motion score&#59; SSS&#58; summed stress score&#59; STS&#58; summed thickening score&#46;<br></br></p><p class="elsevierStylePara">The calculated median &#40;IQR&#41; values of EF in G-MPS and corresponding echocardiography &#40;ECHO-EF&#41; were 65 &#40;12&#41;&#44; and 70 &#40;7&#41;&#44; respectively&#46; The difference between EF values calculated by G-MPS and echocardiography was statistically significant &#40;p&#60;0&#46;05&#41;&#46; There was no meaningful &#40;or very weak&#41; relation between G-MPS EF and ECHO-EF &#40;SCC&#58; 0&#46;217&#41;&#46; A negative correlation was obtained between G-MPS EF and LVEDD as well as LVESD &#40;SCC&#58; &#8211;0&#46;381&#44; &#8211;0&#46;374 respectively&#41;&#46; There was a good correlation between LV ESV-EDV and LVEDD-LVESD parameters &#40;<a href="&#35;t0015" class="elsevierStyleCrossRefs">Table 3</a>&#41;&#46;</p><p class="elsevierStylePara">Table 3&#46; Correlation between Gated EF&#44; ESV&#44; EDV and LVESD&#44; LVDD&#44; ECHO-EF&#46;</p><a name="t0015" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"></p><table><tr align="left"><td>&#160;</td><td>LVESD</td><td>LVEDD</td><td>ECHO-EF</td></tr><tr align="left"><td>Gated EF</td><td>&#8211;0&#46;374</td><td>&#8211;0&#46;381</td><td>0&#46;177</td></tr><tr align="left"><td>&#160;</td><td>P&#61;0&#46;014&#42;</td><td>P&#61;0&#46;012&#42;</td><td>P&#61;0&#46;256</td></tr><tr align="left"><td>ESV</td><td>0&#46;636</td><td>0&#46;683</td><td>&#8211;0&#46;252</td></tr><tr align="left"><td>&#160;</td><td>P&#60;0&#46;001&#42;</td><td>P&#60;0&#46;001&#42;</td><td>P&#61;0&#46;103</td></tr><tr align="left"><td>EDV</td><td>0&#46;633</td><td>0&#46;699</td><td>&#8211;0&#46;197</td></tr><tr align="left"><td>&#160;</td><td>P&#60;0&#46;001&#42;</td><td>P&#60;0&#46;001&#42;</td><td>P&#61;0&#46;206</td></tr></table><p class="elsevierStylePara">&#42; Statistically significant&#46; ECHO-EF&#58; echocardiographical ejection fraction&#59; EDV&#58; end-diastolic volume&#59; EF&#58; ejection fraction&#59; ESV&#58; end-systolic volume&#59; LVEDD&#58; left ventricle end-diastolic diameter&#59; LVESD&#58; left ventricle end-systolic diameter&#46;<br></br></p><p class="elsevierStylePara">On the basis of the clinical findings&#44; statistically significant relations between LV dilatation&#47;motion disorder and frequent transfusion &#40;over 10 times&#47;year&#41; have been detected &#40;p&#60;0&#46;05&#41;&#46; There were no meaningful relations between the other clinical parameters and LV dilatation&#47;motion disorder&#46;</p><a name="sec0040" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Discussion</span><p class="elsevierStylePara">Myocardial perfusion scintigraphy in childhood has been used for many purposes such as myocarditis&#44; congenital AV block&#44; dilated cardiomyopathy and systemic lupus erythematosus&#46;<a href="&#35;bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">8</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib14" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">14</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib16" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">16</span></a> Although myocardial perfusion scintigraphy in patients with SCA has been studied before&#44; there is limited data on gated-SPECT studies in children with SCA&#46; In this study&#44; no perfusion defect has been detected in none of the patients&#46; In the literature&#44; apparently there are three studies published by a French study group on sickle cell disease which were established to assess myocardial perfusion using Thallium-201 SPECT in children&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib17" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">17</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib18" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">18</span></a> In these studies differing from ours&#44; significant perfusion defects in SCA patients were observed&#46;<a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib16" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">16</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib17" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">17</span></a> For instance&#44; in one of the studies concerning 23 patients&#44; an impairment of myocardial perfusion in 61&#37; &#40;14 of 23&#41; of the patients has been found&#44; existing myocardial ischemia in 39&#37; of the cases&#46;<a href="&#35;bib19" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">19</span></a> On the other hand&#44; Montalambert et al have studied the patients with chest pain&#44; heart failure&#44; abnormal electrocardiogram which might possibly be the cause of increased rate of perfusion defects&#46;<a href="&#35;bib18" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">18</span></a> In that study myocardial perfusion defects were detected in all of the patients with cardiac symptoms such as heart failure&#44; ventricular arrhythmias or angina&#46; In our study&#44; 43 children with SCA between the ages of 6 and 18 were the subjects who were consulted to nuclear medicine department in order to detect any myocardial perfusion impairment&#44; but none of them showed any perfusion defects&#46; Most of the patients had nonspecific chest pain which may not be directly related to cardiac disorders&#44; but may frequently be seen in SCA&#46; Aessopos et al have found out myocardial ischemia during treadmill exercise test within 27&#37; of 30 patients with sickle beta thalassemia who had no cardiac symptoms&#46;<a href="&#35;bib19" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">19</span></a> Coronary angiograms of these patients were normal&#46; Thus&#44; they emphasize that the sickling process&#44; activated by exercise&#44; could be the common underlying mechanism&#46;<a href="&#35;bib19" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">19</span></a> No occlusion of the major coronary arteries was found in postmortem examinations of patients with SCA which strongly suggests that microcirculation is involved&#46;<a href="&#35;bib20" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">20</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib21" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">21</span></a> Coronary autopsies found out the thrombosis of the microcirculation&#46;<a href="&#35;bib20" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">20</span></a> Consequently&#44; it can probably be thought that&#44; if the children with SCA have no cardiac symptoms&#44; coronary involvement and myocardial ischemia may not be an expected finding except exercise&#46;</p><p class="elsevierStylePara">On the other hand&#44; cardiac enlargement is a well known observation in children with SCA&#46;<a href="&#35;bib21" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">21</span></a> In these patients&#44; cardiomyopathies are described from echocardiographic data and exhibit LV dilatation and normal contraction&#46; Such cardiomyopathies are not specific and are usually free of cardiac symptoms&#46;<a href="&#35;bib22" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">22</span></a> Lester et al suggest that the major cardiac abnormalities in children are related to the volume overload effects of chronic anemia in children and there is no evidence for a distinct &#8220;sickle cell cardiomyopathy&#8221; or cardiac dysfunction&#46; They found 60&#37; of cases with LV dilatation in 64 children with SCA&#46;<a href="&#35;bib22" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">22</span></a> LV dilatation in our cases were present in only 34&#46;8&#37; of them and none of the patients have had cardiomyopathy&#46; Apart from these&#44; in our study&#44; we detected only one statistically significant relation among the clinical data which was between LV dilatation&#47;motion disorder and frequent transfusions&#46; It is known that&#44; especially in frequently transfused patients&#44; cardiac toxicity may develop because of iron overload&#46;</p><p class="elsevierStylePara">A clinical improvement of SCA has been reported using hydroxyurea treatment&#46; The beneficial effects of hydroxyurea therapy on the prognosis of the patients with SCA is commonly known&#46;<a href="&#35;bib23" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">23</span></a> It may be related to both an increase of fetal hemoglobin synthesis responsible for an inhibition of hemoglobin S polymerization&#44; and a decrease of highly adhesive reticulocytes&#46;<a href="&#35;bib24" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">24</span></a> It is also believed that hydroxyurea can improve hypoperfusion&#46;<a href="&#35;bib22" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">22</span></a> Since all of our patients except 7 of them use hydroxyurea&#44; the effects of the medicine cannot be considered&#46;</p><p class="elsevierStylePara">Montalambert et al have emphasized that the age of the patients with SCA who had perfusion defects were older and they had vasooclusive crisis more frequently&#46;<a href="&#35;bib18" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">18</span></a> In our study&#44; although the ages of the patients with LV dilatation&#47;motion disorder were older than the others&#44; there was no statistically significant difference&#46; However&#44; since the p value was very close to 0&#46;05 &#40;p&#61;0&#46;052&#41;&#44; in case of increasing the number of patients&#44; it is assumed that statistical difference may occur&#46;</p><p class="elsevierStylePara">All of the ECHO-EF and G-MPS EF values of the patients were within normal range in our study&#46; Nevertheless&#44; there was no meaningful relation between G-MPS EF and ECHO-EF&#46; Since ECHO-EF values get deteriorated in the late period&#44; it may not be useful to take EF values into consideration on the follow-up of patients with no cardiac symptoms&#46; G-MPS is reported to be correlated to echocardiography on the measurement of LV EF and volume in adults&#44;<a href="&#35;bib25" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">25</span></a> whereas there is limited data on childhood&#46; The role of G-MPS&#44; as an alternative measurement&#44; has yet to be determined for children&#46; K&#305;ratl&#305; and Bozkurt demonstrated poor agreement between gated myocardial perfusion scintigraphy and echocardiography&#44; and found G-MPS less suitable for assessing left ventricular volumes and ejection fraction in children&#46;<a href="&#35;bib26" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">26</span></a> The left ventricular cavity in children is much smaller than adults&#44; which may result in worse resolution&#44; thus creating difficulties for automatic programs&#46; On the other hand&#44; in a study by Nakajima et al&#44; LV volume and EF as assessed by gated SPECT in pediatric studies were in agreement with echocardiographic measurements&#46; The quantitative reliability was enhanced by SPECT filters with a high cut-off frequency&#44; a high system resolution and appropriate zooming&#46;<a href="&#35;bib27" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">27</span></a> In our study&#44; scintigraphically obtained LV-EDV and LV-ESV values as well as echocardiographically obtained LVEDD and LVESD parameters were correlated with each other&#46; However&#44; EF values in echocardiography and G-MPS were not correlated to each other&#46; The reason of this is probably preferring the M-Mode echocardiography which has been widely used in children instead of using Modified Simpson Method&#46; As for the EF calculation&#44; while LV dimensions are used in M-Mode echocardiography process&#44; LV volumes are used in Modified Simpson Method&#46; In another study in the literature which is parallel to ours&#44; G-MPS EF values calculated by QPS were found out less than EF values obtained by M-Mode echocardiography&#46;<a href="&#35;bib26" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">26</span></a> If we had used Modified Simpson Method instead of M-Mode during EF calculations of echocardiography&#44; the results would have been different&#46;</p><p class="elsevierStylePara">According to our knowledge&#44; normal values for cardiac volumes calculated by G-MPS for children are not determined in literature&#46; However it has been suggested that variations in reference values of cardiac volumes would be reduced by normalization to body surface area &#40;BSA&#41; calculated from the subject&#39;s height and weight&#46;<a href="&#35;bib28" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">28</span></a> For that reason&#44; we have preferred to calculate index values &#40;LV-EDVI&#41; by dividing LV-EDV to BSA for normalization&#46; In our study&#44; LV volume over 75 ml&#47;m<span class="elsevierStyleSup">2</span> in G-MPS was accepted as dilatation depending on the reference values in a study by Sharir et al&#46;<a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a> LV dilatation assumed by LV-EDVI in G-MPS was correlated to LV dilatation detected in echocardiography&#46; However the benefits that obtained by G-MPS was not sufficient to put that test as a high priority examination in SCA&#46;</p><p class="elsevierStylePara">MPI is an acceptable and simple index which consists of Doppler-derived systolic and diastolic time intervals to generate a combined index of global ventricular function that is independent of geometric assumptions&#46; Higher MPI values have been found to correlate with worsening myocardial systolic and diastolic function&#46;<a href="&#35;bib29" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">29</span></a> Among a number of studies&#44; it has been reported that LV-MPI values increased in children with SCA&#46;<a href="&#35;bib30" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">30</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib31" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">31</span></a> In this study&#44; parallel to those&#44; LV-MPI values were significantly higher in the patients who had LV dilatation&#47;motion disorder which is supposed to depend on volume overload&#46;</p><p class="elsevierStylePara">One of the limitations of our study was the relatively low number of the patients since SCA is not a frequently seen hematological disease&#46; Another point is that the normal reference values of quantitative evaluation by G-MPS have not been clearly determined in childhood in litrature&#46; That is the reason that we preferred to use index values in order to define LV dilatation in G-MPS&#46; But it should also be kept in mind that index normalization may be in limited value if the BSA of the patient examined is very low&#46;<a href="&#35;bib28" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">28</span></a> Comparing our results with a control group would be more reliable&#44; however control cases were not included in our study because of the radiation effects&#46;</p><p class="elsevierStylePara">In conclusion&#44; myocardial perfusion defect in SCA is not a frequently observed finding in childhood period&#46; Due to the fact that&#44; it may not be considerable to perform scintigraphy to the children who have no serious cardiac symptoms such as ECG abnormality&#44; angina and heart failure&#46; In order to detect LV dilatation&#44; although scintigraphic process adjusted to BSA seems to be correlated to echocardiography&#44; due to the radiation effect it should not be considered to have a priority&#46; Despite of the fact that&#44; EF values of the children with SCA are not deteriorated in early stages&#44; it can be useful to have the measurements of LVDD and LV-MPI in follow-up to detect the cardiac problems that might possibly develop later&#46; In addition&#44; in patients with sickle cell anemia who takes frequent transfusion&#44; it should be kept in mind that LV dilatation&#47;motion disorders may develop&#46;</p><a name="sec0045" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Conflicts of interest</span><p class="elsevierStylePara">The authors have no conflicts of interest to declare&#46;</p><p class="elsevierStylePara">Received 10 November 2010 <br></br>Accepted 1 February 2011 </p><p class="elsevierStylePara">Corresponding author&#46; ecgunay&#64;yahoo&#46;com</p>"
    "pdfFichero" => "125v30n06a90034936pdf001.pdf"
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    "PalabrasClave" => array:2 [
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        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec241017"
          "palabras" => array:5 [
            0 => "Myocardial perfusion"
            1 => "scintigraphy"
            2 => "echocardiography"
            3 => "sickle cell anemia"
            4 => "children"
          ]
        ]
      ]
      "es" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec241018"
          "palabras" => array:5 [
            0 => "Perfusi&#243;n mioc&#225;rdica"
            1 => "gammagraf&#237;a"
            2 => "ecocardiograf&#237;a"
            3 => "anemia de c&#233;lulas falciformes"
            4 => "ni&#241;os"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Objectives</span><br/><p class="elsevierStylePara">The heart is one of the organs affected by sickle cell anemia &#40;SCA&#41;&#46; This prospective study has aimed to evaluate myocardial perfusion and left ventricle &#40;LV&#41; function in children with SCA by gated myocardial perfusion scintigraphy &#40;G-MPS&#41; and to compare the results with echocardiographic parameters&#46;</p><span class="elsevierStyleSectionTitle">Methods</span><br/><p class="elsevierStylePara">Forty-three patients with SCA were evaluated by G-MPS and echocardiography&#46; Myocardial perfusion and motion with thickening function were analyzed both visually and quantitatively&#46; End-diastolic &#40;EDV&#41;&#44; end-systolic volumes &#40;ESV&#41;&#44; ejection fraction &#40;EF&#41;&#44; lung-to-heart &#40;L&#47;H&#41; ratio were also calculated&#46;</p><span class="elsevierStyleSectionTitle">Results</span><br/><p class="elsevierStylePara">None of the patients showed stress perfusion impairment in G-MPS&#46; LV dilatation in 15 patients was observed both by G-MPS and echocardiography&#46; EF values were within normal limits&#46; Correlation between EF values calculated by two methods was not statistically significant&#46; However&#44; LV dilatation detected by both methods and EDV-ESV values in G-MPS were correlated to end-diastolic and end-systolic diameters calculated in echocardiography &#40;p&#60;0&#46;05&#41;&#46; M-Mode echocardiography revealed higher myocardial performance index &#40;LV-MPI&#41; in patients with LV dilatation&#46; There was also a significant relationship between LV dilatation and frequent blood transfusions &#40;&#62;5&#47;years&#41; and acute chest syndrome &#40;p&#60;0&#46;05&#41;&#46;</p><span class="elsevierStyleSectionTitle">Conclusion</span><br/><p class="elsevierStylePara">Myocardial perfusion impairment in children with SCA is not frequently observed&#46; Thus&#44; performing the scintigraphy only in patients with cardiac symptoms should be considered&#46; Since EF values of the children with SCA are not deteriorated in early stages&#44; LV-MPI and LV dilatation should be considered as a significant parameter other than EF or perfusion data&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Objetivo</span><br/><p class="elsevierStylePara">El <span class="elsevierStyleBold">c</span>oraz&#243;n es uno de los &#243;rganos afectados por la anemia de c&#233;lulas falciforme &#40;SCA&#41;&#46; El objetivo de este estudio prospectivo es evaluar la perfusi&#243;n mioc&#225;rdica y la funci&#243;n del ventr&#237;culo izquierdo &#40;LV&#41; en ni&#241;os con SCA por la gammagraf&#237;a de perfusi&#243;n mioc&#225;rdica &#40;G-MPS&#41; y comparar los resultados con los hallazgos ecocardiogr&#225;ficos&#46;</p><span class="elsevierStyleSectionTitle">M&#233;todos</span><br/><p class="elsevierStylePara">Se evaluaron cuarenta y tres pacientes con SCA mediante estudios de G-MPS y ecocardiograf&#237;a&#46; La perfusi&#243;n mioc&#225;rdica y la funci&#243;n ventricular con la valoraci&#243;n de la motilidad y del engrosamiento se analizaron tanto de forma visual como cuantitativa&#46; Se calcularon el EDV&#44; ESV&#44; EF&#44; y el &#237;ndice pulm&#243;n &#47;coraz&#243;n &#40;L&#47;H&#41;&#46;</p><span class="elsevierStyleSectionTitle">Resultados</span><br/><p class="elsevierStylePara">Ninguno de los pacientes present&#243; deterioro de la perfusi&#243;n post-estr&#233;s en el G-MPS&#46; Se observ&#243; dilataci&#243;n del LV en 15 pacientes&#44; que tambi&#233;n estuvo presente en el ecocardio&#46; Los valores de EF se encontraban dentro de los l&#237;mites normales&#46; La correlaci&#243;n entre los valores de EF&#44; mediante ambos m&#233;todos no fueron estad&#237;sticamente significativas&#46; Sin embargo la dilataci&#243;n del ventr&#237;culo izquierdo detectados por ambos m&#233;todos y los valores de EDV-ESV en la G-MPS se correlacionaron con los di&#225;metros sist&#243;lico y diast&#243;lico final calculados por la ecocardiograf&#237;a &#40;p&#60;0&#44;05&#41;&#46; La ecocardiograf&#237;a en modo-M mostr&#243; mayor &#237;ndice de rendimiento mioc&#225;rdico &#40;LV-MPI&#41; en pacientes con dilataci&#243;n del LV&#46; Tambi&#233;n hubo una relaci&#243;n significativa entre la dilataci&#243;n del ventr&#237;culo izquierdo y la frecuencia de transfusiones sangu&#237;neas &#40;&#62; 5&#47;a&#241;o&#41; y el s&#237;ndrome tor&#225;cico agudo &#40;p &#60;0&#44;05&#41;&#46;</p><span class="elsevierStyleSectionTitle">Conclusi&#243;n</span><br/><p class="elsevierStylePara">El deterioro de la perfusi&#243;n mioc&#225;rdica en los ni&#241;os con SCA no se observa con frecuencia&#46; Debe considerarse el realizar la gammagraf&#237;a s&#243;lo en pacientes con s&#237;ntomas card&#237;acos&#46; Puesto que los valores de EF de los ni&#241;os con SCA no se deterioran en las primeras etapas&#44; el LV-MPI y la dilataci&#243;n del ventr&#237;culo izquierdo deber&#237;an considerarse como un par&#225;metro significativo&#46;</p>"
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          "en" => "A&#41; Myocardial perfusion SPECT images and B&#41; QGS analysis of a 18-year-old male patient with sickle cell anemia with left ventricular dilatation and normal myocardial perfusion&#46;"
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Article information

ISSN: 2253654X
Original language: English

The statistics are updated each day

Year/Month	Html	Pdf	Total

2024 October	0	2	2
2024 September	0	6	6
2024 August	0	6	6
2024 July	0	8	8
2024 June	0	4	4
2024 May	0	3	3
2024 April	0	8	8
2024 March	0	8	8
2024 February	0	2	2
2024 January	0	14	14
2023 December	0	15	15
2023 November	0	3	3
2023 October	0	8	8
2023 September	0	4	4
2023 August	0	7	7
2023 July	0	9	9
2023 June	0	2	2
2023 May	0	2	2
2023 April	0	7	7
2023 March	0	1	1
2023 February	0	1	1
2023 January	0	2	2
2022 December	0	6	6
2022 November	0	5	5
2022 October	0	1	1
2022 September	0	1	1
2022 August	0	7	7
2022 July	0	8	8
2022 June	0	6	6
2022 May	0	3	3
2022 April	0	8	8
2022 March	0	5	5
2022 February	0	4	4
2022 January	0	3	3
2021 December	0	2	2
2021 November	0	8	8
2021 October	0	14	14
2021 September	0	2	2
2021 August	0	4	4
2021 July	0	4	4
2021 June	0	10	10
2021 May	0	4	4
2021 March	0	5	5
2021 February	0	1	1
2020 November	0	7	7
2020 August	0	1	1
2020 July	0	3	3
2020 May	0	3	3
2020 April	0	2	2
2020 February	0	1	1
2020 January	0	3	3
2019 December	0	4	4
2019 November	0	3	3
2019 October	0	1	1
2019 September	0	1	1
2019 August	0	4	4
2019 July	0	4	4
2019 June	0	6	6
2019 May	0	38	38
2019 April	0	12	12
2019 February	0	3	3
2019 January	0	2	2
2018 December	0	15	15
2018 November	0	6	6
2018 October	0	2	2
2018 February	5	4	9
2018 January	7	0	7
2017 December	8	0	8
2017 November	2	1	3
2017 October	8	1	9
2017 September	9	1	10
2017 August	6	6	12
2017 July	10	1	11
2017 June	13	2	15
2017 May	15	1	16
2017 April	11	8	19
2017 March	11	8	19
2017 February	20	2	22
2017 January	6	0	6
2016 December	11	1	12
2016 November	12	1	13
2016 October	16	2	18
2016 September	7	0	7
2016 August	6	0	6
2016 July	7	1	8
2016 June	17	2	19
2016 May	15	4	19
2016 April	6	2	8
2016 March	10	7	17
2016 February	10	7	17
2016 January	8	12	20
2015 December	20	6	26
2015 November	17	6	23
2015 October	23	6	29
2015 September	16	3	19
2015 August	17	3	20
2015 July	12	2	14
2015 June	5	0	5
2015 May	22	8	30
2015 April	32	11	43
2015 March	11	4	15
2015 February	10	0	10
2015 January	21	5	26
2014 December	29	5	34
2014 November	5	1	6
2014 October	15	3	18
2014 September	7	0	7
2014 August	6	1	7
2014 July	7	2	9
2014 June	5	0	5
2014 May	7	1	8
2014 April	6	2	8
2014 March	3	3	6
2014 February	7	0	7
2014 January	10	4	14
2013 December	13	2	15
2013 November	26	3	29
2013 October	12	1	13
2013 September	15	0	15
2013 August	11	2	13
2013 July	8	0	8
2013 June	1	0	1
2013 May	3	0	3
2011 November	589	0	589

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