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Case report
Anesthetic management of a schoolboy with uncorrected truncus arteriosus type I, and severe pulmonary hypertension undergoing repair of congenital dislocation of the knee. Case report
Manejo anestésico de un escolar con tronco arterioso tipo I no corregido e hipertensión pulmonar severa sometido a reparación de luxación congénita de rodilla. Reporte de caso
Luis Alfonso Díaz-Fosadoa,
Corresponding author
mdlalfonsodiaz@yahoo.com.mx

Corresponding author at: Departamento Anestesiología, Quirófanos 2° Piso, Instituto Nacional de Pediatría, Insurgentes Sur 3700, Letra C, Delegación Coyoacán, México Distrito Federal CP 04530, Mexico.
, Lina Sarmientob,c, Tamara Velazquez-Martínezd
a Professor of the Pediatric Anesthesiology Program, Pediatric Anesthetist, National Institute of Pediatrics, Mexico D.F., Mexico
b Pediatric Anesthetist, National Institute of Pediatrics, Mexico D.F., Mexico
c Anesthetist, San Ignacio University Hospital, Bogota D. C., Colombia
d Third Year Resident of Anesthesiology, Hospital Regional B Veracruz Alta Especialidad ISSSTE, Veracruz, Mexico
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital heart disease continues to be one of the primary anomalies at birth&#46; About 30&#37; of these patients may require some type of non-cardiac surgery during their first year of life and it is estimated that up to 80&#37; grow up to become adults in the United States&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">1</span></a> Unfortunately in our environment there is a large number of children with congenital heart disease&#46; Late diagnosis and lack of treatment result in severe complications that significantly increase perioperative morbidity and mortality&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Truncus arteriosus represents only 1&#46;2&#8211;3&#37; of the complex congenital heart diseases&#46; If uncorrected&#44; less than 20&#37; of these patients will survive their first year of life&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">2</span></a> The development of pulmonary hypertension is a serious complication of this type of condition and is characterized by a mean pulmonary artery pressure above 25<span class="elsevierStyleHsp" style=""></span>mmHg at rest and above 30<span class="elsevierStyleHsp" style=""></span>mmHg during exercise&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">3</span></a> Our objective is to discuss a case report of a schoolboy diagnosed with uncorrected truncus arteriosus and severe pulmonary hypertension undergoing an elective orthopedic procedure&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Case presentation</span><p id="par0015" class="elsevierStylePara elsevierViewall">9-year old male patient with a diagnosis of uncorrected truncus arteriosus type I&#44; and severe pulmonary hypertension that presents with severe right knee pain and gate limitation&#46; The MRI shows intact cruciate ligaments and discoid lateral meniscus&#46; The patient was evaluated at the service of Pediatric Orthopedics and diagnosed with congenital right knee dislocation and was programmed for surgical repair&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The pre-anesthesia evaluation reported uneventful family history with a diagnosis of velocardiofacial syndrome and mild psychomotor retardation&#46; The findings of the physical evaluation indicated a cooperative patient&#44; functional class NYHA II&#44; generalized cyanosis&#44; precordial fremitus&#44; multifocal systolic murmur&#44; pulse oximetry 87&#37;&#44; and body weight of 22<span class="elsevierStyleHsp" style=""></span>kg&#46; The transthoracic echocardiographic examination confirmed the cardiology diagnosis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Image 1</a>&#41;&#46; The patient has a history of anesthetic sedation for cardiac catheterization showing that the pulmonary pressure equaled the systemic blood pressure&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The orthopedic surgery was done under regional anesthesia&#46; The patient was admitted to the OR with a patent 20G peripheral catheter&#46; The initial monitoring indicated a BP of 99&#47;62<span class="elsevierStyleHsp" style=""></span>mmHg&#44; HR 113<span class="elsevierStyleHsp" style=""></span>lpm&#44; room air SpO<span class="elsevierStyleInf">2</span> 88&#37;&#44; Temperature 36&#46;8<span class="elsevierStyleHsp" style=""></span>&#176;C&#46; Intravenous sedation with fentanyl 50<span class="elsevierStyleHsp" style=""></span>mcg&#44; lidocaine 20<span class="elsevierStyleHsp" style=""></span>mg&#44; propofol 45<span class="elsevierStyleHsp" style=""></span>mg and invasive monitoring with right radial arterial line was later on performed&#46; During the procedure the patient maintained spontaneous ventilation and received supplemental oxygen through a nasal cannula&#46; A propofol infusion between 90 and 120<span class="elsevierStyleHsp" style=""></span>mcg&#47;kg&#47;min was used for maintenance of anesthesia&#46; Regional anesthesia was delivered through a posterior sciatic block and lumbar plexus block using neurostimulator guidance with Ropivacaine and Lidocaine with no complications &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Image 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">The hemodynamic parameters were stable during the procedure with arterial blood pressure measurements between 68 and 80<span class="elsevierStyleHsp" style=""></span>mmHg&#44; HR 96&#8211;105<span class="elsevierStyleHsp" style=""></span>lpm&#44; SpO<span class="elsevierStyleInf">2</span> 82&#8211;90&#37;&#46; Bleeding was minimal&#46; At the end of the procedure the patient was admitted to the pediatric ICU with Aldrete score of 9&#44; 2 in Ramsay&#39;s scale&#44; 3 in Bromage&#39;s scale&#44; 0 pain in the visual analog scale&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The truncus arteriosus is characterized by the presence of a single arterial vessel emerging from the base of the heart and irrigates the coronary bed&#44; the pulmonary circulation&#44; the systemic circulation&#44; and usually presents interventricular communication&#46; From the embryological perspective this defect is the result of a failed division of the common truncus between the pulmonary artery and the aorta&#46; A deletion of the 22q11 chromosome has been identified in up to 35&#37; of the patients&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">7</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Collett and Edwards described 4 types of truncus arteriosus&#44; based on the origin of the pulmonary artery&#46; Type I &#40;48&#8211;68&#37;&#41; is defined as the presence of a small pulmonary trunk arising from the arterial trunk with branching of the right and left pulmonary branches&#46; In type II &#40;29&#8211;48&#37;&#41;&#44; the trunk of the pulmonary artery is missing but the pulmonary branches emerge close to one another from the arterial trunk&#46; Type III &#40;6&#8211;10&#37;&#41; is also characterized by the absence of the trunk of the pulmonary artery but the right and left branches emerge distant from one another&#46; Type IV is no longer considered an anomaly of the truncus arteriosus and is now recognized as a form of pulmonary atresia with ventricular septal defect&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">8</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">As a result of the changes in cardiopulmonary circulation&#44; patients with truncus arteriosus present with a left to right shunt that increases the pulmonary blood flow&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">9</span></a> The pulmonary blood vessels chronically exposed to an increased blood flow&#44; develop an obstructive vascular pathology leading to increased pulmonary vascular resistance and hence increased pulmonary pressure&#46; When this pressure equals or exceeds the systemic blood pressure&#44; there will be reversal of the shunt and causes severe hypoxia&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">10</span></a> Patients with uncorrected congenital heart disease and left to right shunt will develop Eisenmenger&#39;s disease or Eisenmenger&#39;s complex characterized by pulmonary hypertension&#44; right ventricular atrophy&#44; impaired pulmonary vessels&#44; reversal of right to left shunt&#44; and hypoxemia refractory to oxygen therapy&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">2</span></a> When the pulmonary vascular resistance exceeds 8 Wood units&#44; or if Eisenmenger&#39;s syndrome has been diagnosed&#44; corrective surgery is contraindicated&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">11</span></a> Eventually patients usually die from heart failure&#46;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">12</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Carmosino et al&#46; showed that children with pulmonary hypertension have a significantly higher risk of developing perioperative complications&#44; including cardiac arrest&#44; pulmonary hypertension crisis&#44; and death when undergoing procedures under sedation or under general anesthesia&#46; Most complications were more frequent in those patients with pulmonary pressure equal or higher than the systemic blood pressure&#44; and there was no association either with age or etiology&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">3</span></a> Unless a previous genetic study is available&#44; any blood products that need to be administered to patients with truncus arteriosus shall undergo prior radiation&#44; because of the strong association with Di George syndrome and T-lymphocytes deficiency&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">7</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The anesthetic considerations include a broad range of variables to be considered&#46; The presence and the severity of cardiac failure may require preoperative management with inotropic agents&#46; The principal goal during the induction of anesthesia will be maintaining a balance between the pulmonary vascular resistance &#40;PVR&#41; and the systemic vascular resistance &#40;SVR&#41;&#46; Hyperventilation or using high oxygen concentrations leads to a reduced PVR&#44; contributing to a considerable increase of the pulmonary blood flow&#44; lower diastolic blood pressure&#44; and even results in systemic hypoperfusion&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">7</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">There is no consensus about the safest anesthesia in patients with severe pulmonary hypertension or in patients with Eisenmenger&#39;s syndrome&#46; In a literature review&#44; Martin et al&#46; claim that using regional anesthesia has no advantage over the use of general anesthesia&#46; However&#44; the best predictor of mortality is the type of surgical intervention&#44; since patients undergoing major surgery experienced up to 24&#37; mortality as compared to 5&#37; in patients undergoing minor surgery&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">13</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The anesthetic management of this patient was planned based on the cardiovascular pathophysiology of the truncus arteriosus with severe pulmonary hypertension&#46; The pulmonary and systemic blood flows arise from a common vessel and consequently the blood flow circulation and distribution may be affected when manipulating the vascular resistance&#46; The patient&#39;s pulmonary pressure was similar to the systemic blood pressure&#44; so our priority was to avoid any changes in the pulmonary and systemic vascular resistance since such changes could favor the imbalance between both types of circulation&#46; We decided to keep the patient under spontaneous ventilation since endotracheal intubation and the use of mechanical ventilation could increase the PVR&#46; CO<span class="elsevierStyleInf">2</span> was monitored via an arterial line and was kept between 38 and 43<span class="elsevierStyleHsp" style=""></span>mmHg&#44; since both hypocapnia and hypercapnia change the pulmonary vessels resistance&#46; FiO<span class="elsevierStyleInf">2</span> was maintained between 21 and 25&#37; in order to avoid a drop in PVR&#46; We used propofol to maintain sedation because its pharmacological characteristics make it predictable&#59; at the usual doses propofol has no negative inotropic effect and has a short half-life&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">One of the major challenges during the procedure was to select an anesthetic technique that provided stronger hemodynamic stability&#46; We decided to use a lumbar plexus block&#44; in addition to the posterior sciatic nerve block and this provided adequate anesthesia with no impact on systemic vascular resistance&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">In conclusion&#44; the management of anesthesia in patients with congenital heart disease must be individualized and should take into account the complexity of the non-cardiac surgical procedure&#44; the level of cardiovascular involvement resulting from the congenital heart disease&#44; and the hemodynamic impact on the various organs&#46; When there is uncorrected truncus arteriosus&#44; the primary goal of anesthesia shall be to maintain a balance between the pulmonary vascular resistance and the systemic vascular resistance&#44; and consider regional anesthesia with peripheral nerve block as an alternative for managing these patients&#46; Moreover&#44; these procedures should be done by a multidisciplinary team with experience in these conditions and at a hospital with the adequate technology available for diagnosis and perioperative management&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Ethical disclosure</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Protection of persons and animals</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors declare that this research did not involve any experiments in human beings or animals&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Confidentiality of data</span><p id="par0085" class="elsevierStylePara elsevierViewall">The authors affirm that they have followed the protocols of their institution on the publication of patient information&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Right to privacy and informed consent</span><p id="par0090" class="elsevierStylePara elsevierViewall">The authors obtained the informed consent of all patients and&#47;or subjects herein mentioned&#46; These documents are under custody of the corresponding author&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Funding</span><p id="par0095" class="elsevierStylePara elsevierViewall">Authors&#8217; personal funds&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">No conflicts of interest to disclose&#46; This article was written by the authors and is proprietary&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The presence of truncus arteriosus represents just 1&#46;2&#8211;3&#37; of all complex congenital heart pathologies and if not corrected&#44; less than 20&#37; survive beyond one year of life&#46; When the disease progresses patients usually develop severe pulmonary hypertension and may even develop into Eisenmenger&#39;s syndrome&#46; The paper discusses a case of a schoolboy with a diagnosis of uncorrected truncus arteriosus type I&#44; and severe pulmonary arterial hypertension undergoing non-cardiac surgery&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case discussion</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">This is a 9-year old schoolboy with complex heart disease and similar pulmonary and systemic blood pressures&#44; undergoing elective orthopedic surgery under regional anesthesia&#44; with lumbar plexus block and posterior sciatic block&#46; This anesthetic approach provided adequate anesthesia with hemodynamic stability and no impact on vascular resistance&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conclusion</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The choice of the anesthetic technique should be a planned decision based on the cardiovascular pathophysiology of the truncus arteriosus&#44; the level of pulmonary hypertension&#44; and the surgical procedure to be performed&#46; Patients with severe pulmonary hypertension are at increased risk of developing suprasystemic pulmonary pressures with considerable hemodynamic involvement&#46; Consequently&#44; the anesthetic technique chosen shall provide adequate anesthesia and ensure the least hemodynamic impact&#46; Whenever possible&#44; it is important to consider the peripheral nerve block as the first line approach for orthopedic surgery&#46;</p></span>"
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        "resumen" => "<span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Introducci&#243;n</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La presencia de tronco arterioso representa &#250;nicamente del 1&#46;2 al 3&#37; de las cardiopat&#237;as cong&#233;nitas complejas y de no ser corregida&#44; menos del 20&#37; sobreviven despu&#233;s del a&#241;o de vida&#46; Si la patolog&#237;a progresa usualmente desarrollan hipertensi&#243;n arterial pulmonar severa y pueden manifestarse incluso como un S&#237;ndrome de Eisenmenger&#46; Se presenta un caso de un escolar con diagn&#243;stico de tronco arterioso tipo I no corregido e hipertensi&#243;n arterial pulmonar severa llevado a cirug&#237;a no cardiaca&#46;</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Presentaci&#243;n del caso</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Escolar de 9 a&#241;os de edad con cardiopat&#237;a compleja y presi&#243;n arterial pulmonar similar a la presi&#243;n arterial sist&#233;mica sometido a cirug&#237;a ortop&#233;dica electiva bajo anestesia regional con bloqueo de plexo lumbar y bloqueo ci&#225;tico posterior&#46; Esta t&#233;cnica anest&#233;sica nos proporcion&#243; una adecuada anestesia con estabilidad hemodin&#225;mica sin repercusi&#243;n en las resistencias vasculares&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusi&#243;n</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">La elecci&#243;n de la t&#233;cnica anest&#233;sica debe ser planeada en base a la fisiopatolog&#237;a cardiovascular del tronco arterioso&#44; del grado de hipertensi&#243;n pulmonar y del procedimiento quir&#250;rgico a realizarse&#46; Los pacientes con hipertensi&#243;n pulmonar severa tienen mayor riesgo de manifestar presiones pulmonares suprasist&#233;micas con compromiso hemodin&#225;mico importante&#44; por lo que la t&#233;cnica anest&#233;sica elegida ser&#225; aquella que produzca una adecuada anestesia y menor repercusi&#243;n hemodin&#225;mica&#46; Es importante considerar de ser posible&#44; el bloqueo de nervios perif&#233;ricos como primera elecci&#243;n en cirug&#237;a ortop&#233;dica&#46;</p></span>"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Superficial markings on the patient&#58; sciatic nerve block using the Winnie<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">5</span></a> modified Labat<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">4</span></a> technique and lumbar plexus block with the Capdevilla<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">6</span></a> technique&#46;</p>"
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                      "titulo" => "Anaesthetic considerations in children with congenital heart disease undergoing non-cardiac surgery"
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                      "titulo" => "Anaesthetic management for hip arthroplasty in a 46-yr-old patient with uncorrected truncus arteriosus type IV"
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                      "titulo" => "Perioperative complications in children with pulmonary hypertension undergoing noncardiac surgery or cardiac catheterization"
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                    0 => array:2 [
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                        "fecha" => "1923"
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos