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Clinical note
Lung ventilation/perfusion scintigraphy in pulmonary capillary hemangiomatosis: A pattern to consider
Gammagrafía pulmonar de ventilación/perfusión en la hemangiomatosis capilar pulmonar: un patrón a considerar
S. Carmonaa,
Corresponding author
smmc24@yahoo.com

Corresponding author.
, M.J. Loureirob, J. Santosa, A. Oliveirac, R. Camachod, A.I. Santosa
a Servicio de Medicina Nuclear, Hospital Garcia de Orta, Almada, Portugal
b Servicio de Cardiologia, Hospital Garcia de Orta, Almada, Portugal
c Servicio de Anatomia Patológica, Hospital Garcia de Orta, Almada, Portugal
d Servicio de Radiologia, Hospital Garcia de Orta, Almada, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Pulmonary capillary hemangiomatosis &#40;PCH&#41; is a rare disease&#44; first recognized in 1978 by Wagenvoort and colleagues and still of unknown aetiology&#46; It is characterized by small capillaries proliferation&#44; behaving like a very-low-grade vascular tumour leading to pulmonary arterial hypertension &#40;PAH&#41;&#44;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;4</span></a> although some authors defend this is not a neoplastic process&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Since it is clinically unspecific and undistinguishable from other more frequent forms of PAH&#44; it is frequently misdiagnosed as idiopathic pulmonary arterial hypertension &#40;IPAH&#41; or as pulmonary veno-occlusive disease &#40;PVOD&#41;&#46; Although PVOD differs pathologically from PCH&#44; being characterized by small pulmonary veins and venules occlusion&#44; these two entities are very similar on diagnostic and therapeutic features&#46; PCH &#40;or PVOD&#41; diagnosis&#44; which typically requires a lung biopsy&#44;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a> is essential for selecting a correct treatment&#44; since it is distinct from the therapeutic approaches for other forms of PAH&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Lung ventilation&#8211;perfusion scintigraphy &#40;V&#47;Q scan&#41; is a simple and important exam on the evaluation of chronic pulmonary hypertension&#44; permitting to differentiate embolic&#44; from non-embolic aetiologies&#44; with a reported 90&#8211;100&#37; sensitivity and 94&#8211;100&#37; specificity in 3 studies&#46; False positive results are typically described in cases of vascular occlusion&#44; other than embolic&#44; as arterial sarcoma&#44; vasculitis&#44; extrinsic vascular compression and PVOD&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Although there are few published cases of V&#47;Q scan in PCH&#44; different patterns have been described&#44; from normal scans to ventilation and&#47;or perfusion defects&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;4&#44;7&#8211;10</span></a> Some authors report the existence of enhanced perfusion to the hemangiomatous tissue and argue the existence of a characteristic V&#47;Q scan pattern in PCH&#44;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9&#44;10</span></a> but false positive results for pulmonary embolism or parenchyma disease have also been reported in this entity&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case report</span><p id="par0025" class="elsevierStylePara elsevierViewall">A 47-year-old man was referred to our institution complaining of a 2 years progressive chronic cough&#44; accompanied by exertional syncope for the latter 2 months&#44; without other relevant symptoms&#46; Electrocardiogram revealed sinus rhythm &#40;75 beats&#47;min&#41;&#44; frontal plane QRS right axis deviation &#40;96&#176;&#41;&#44; P pulmonale &#40;RA dilatation&#41; and incomplete right bundle branch block &#40;QRS &#8211; 100<span class="elsevierStyleHsp" style=""></span>ms&#41;&#46; A transthoracic echocardiography showed a slight left ventricle hypertrophy with preserved function&#44; paradoxical ventricular septum movement&#44; left auricle and ventricle dilatation&#44; right ventricle hypertrophy with systolic function commitment and PAH &#40;systolic pulmonary arterial pressure<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>90&#8211;95<span class="elsevierStyleHsp" style=""></span>mmHg&#41; with slight tricuspid insufficiency&#46; Chest radiograph demonstrated cardiac and pulmonary arteries enlargement and vascular attenuation on the periphery&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">On a first impression&#44; the V&#47;Q scan was evaluated as a diffuse homogeneous decreased perfusion primary in the upper lobes&#44; with preserved ventilation&#46; After careful research&#44; the perfusion study was considered as augmented perfusion on the bases of both lungs &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Thoracic CT demonstrated a micronodular centrilobular pattern with ground glass opacities&#44; more prominent on the lower two-thirds of lungs&#44; mediastinal lymphadenopathy and enlargement of the central and segmental pulmonary arteries &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The thoracic CT pattern was considered suspected of PCH or eventually hypersensitivity pneumonitis&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Pulmonary function tests demonstrated reduced alveolocapillary diffusion&#46; Right cardiac catheterization disclosed a right atrial pressure of 8<span class="elsevierStyleHsp" style=""></span>mmHg&#44; pulmonary artery pressure of 101&#47;43<span class="elsevierStyleHsp" style=""></span>mmHg &#40;mean&#58; 63<span class="elsevierStyleHsp" style=""></span>mmHg&#41;&#44; pulmonary capillary wedge pressure of 8<span class="elsevierStyleHsp" style=""></span>mmHg&#44; cardiac index of 1&#46;49<span class="elsevierStyleHsp" style=""></span>L&#47;min&#47;m<span class="elsevierStyleSup">2</span>&#44; pulmonary vascular resistance of 1692 dynes<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>s&#47;m<span class="elsevierStyleSup">2</span> and trans-pulmonary pressure gradient of 55<span class="elsevierStyleHsp" style=""></span>mmHg&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Lung biopsy revealed pulmonary focus of alveolar wall thickening&#44; due to capillary proliferation&#44; expanding around vessel walls and bronchioles&#44; hyperplasia of tunica media of small arteries and arterioles&#44; venules dilatation extending to subpleural space and intra-alveolar haemorrhage &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; These findings were consistent with PCH&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0050" class="elsevierStylePara elsevierViewall">PCH is a rare and specific cause of PAH&#44; characterized by diffuse capillary proliferation&#44; in a vaguely lobular and patchy distribution&#44; through the pulmonary interstitium and alveolar walls&#44; also surrounding and invading small pulmonary vessels&#44; small bronchi and the pleura&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;7&#44;9</span></a> Its aetiology remains uncertain&#44; although a myriad of associated conditions as systemic lupus erythematosus&#44; scleroderma&#44; Takayasu arteritis&#44; Kartagener syndrome or hypertrophic cardiomyopathy have been reported&#46; It is recognized to occur in congenital&#44; familial and sporadic forms&#44; this last one being the most frequent&#46; The congenital form is usually associated with other development abnormalities and the familiar form is very rare&#44; with an autosomal recessive inherence documented only in one family&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;7&#44;10</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">A correct diagnosis is of extreme importance&#44; since in addition to the fact that PCH has a specific treatment&#44; distinct from other forms of PAH&#46; PCH patients can have their state aggravated if a standard and not specific IPAH therapeutic is established&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The new guidelines for the diagnosis and treatment of PAH&#44; from the European Society of Cardiology and the European Respiratory Society&#44; re-enforce the specificity of PCH and classify both PCH and PVOD on a separate subgroup within PAH&#44; for its specific pathological&#44; clinical and therapeutic issues&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Although the precise prevalence of PCH and PVOD is not known&#44; they are thought to account for 5&#8211;10&#37; of the forms of PAH initially considered idiopathic&#44; with PCH being less prevalent than PVOD&#46; We have found 63 published cases until September 2011&#46; This research was based on MEDLINE database&#44; using the key word pulmonary capillary hemangiomatosis&#59; additional case reports were identified from the references of selected articles&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">Patients with PCH usually present with unspecific symptoms of progressive dyspnoea and fatigue&#44; and are frequently misdiagnosed as IPAH&#46; They may also have chronic cough&#44; chest pain&#44; syncope&#44; digital clubbing&#44; haemoptysis and haemorrhagic pleural effusion&#46; These last two&#44; when present&#44; should raise suspicion of the possibility of PCH&#46; As the disease progresses right sided heart failure and its symptoms appear and a median survival of 3 years from the initial presentation is expected&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;4&#44;9</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Electrocardiography typically demonstrates right axis deviation and right ventricular hypertrophy&#46; Echocardiogram reveals PAH and excludes structural lesions and left ventricular dysfunction&#46; Haemodynamic parameters present two distinct features&#58; elevated pulmonary arterial pressure and normal or low pulmonary capillary wedge pressure&#46; Chest radiograph demonstrates PAH &#40;enlarged central pulmonary arteries and right sided prominence of the heart&#41;&#44; and diffuse or bibasilar reticulonodular or micronodular areas of opacity&#59; mediastinal lymphadenopathy may be reported&#46; Chest CT shows pulmonary arterial enlargement and widespread centrilobular nodules&#44; mixed with lobular ground-glass opacities&#59; sporadically&#44; septal thickening&#44; lymphadenopathy&#44; pleural effusion&#44; enlargement of right heart chambers and pericardial effusion are seen&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;4&#44;10</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">As advocated by this case&#44; presently it is accepted that combining clinical suspicion and radiological findings&#44; namely a high resolution CT&#44; the diagnosis of PCH or PVOD can be established with almost certainty&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a> This is an important feature since lung biopsies must be made with extreme care in these patients&#44; especially in people with elevated PAH&#44; due to the elevated risk of complications&#46; More difficult is to differentiate PCH and PVOD&#44; usually only provided by pathological examination&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Although few cases of V&#47;Q scan on PCH have been published&#44; this exam is usually considered of no use in the diagnosis of PCH since several patterns have been described&#44; such as diffuse augmented perfusion on the bases&#44; perfusion defects with preserved ventilation&#44; matched ventilation and perfusion defects&#44; ventilation defects with normal perfusion and even normal exams&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;4&#44;7&#8211;10</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Augmented perfusion on the pulmonary bases is described by some authors as a typical pattern of PCH&#44; since&#44; in this condition&#44; these are the regions of lung most affected by proliferating capillaries&#44; as documented in our case&#46; These authors defend that this pattern can help to distinguish PCH from other forms of PAH&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">In perfusion mismatched defects&#44; two different patterns were described&#58; diffuse defects in the upper lobes or focal defects&#46; The former&#44; when we look retrospectively&#44; can in fact correspond to the pattern of augmented perfusion on the bases that gives the erroneous perception of decreased perfusion on upper lobes&#44; as in our case&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> The focal defects mimic thromboembolism and can be attributed to the development of in situ thrombosis&#44; as it has been reported in some autopsies&#44; or small vessels compression by proliferating capillaries&#44; that has also been described in PCH&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;7&#44;8</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Perhaps the normal&#44; augmented perfusion on bases and mismatched perfusion defects represent different phases of the disease or even different physiopathologic expressions of PCH&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">Ventilation defects with normal perfusion or perfusion and ventilation matched defects have been also described in this pathology&#44; and although in some patients no concomitant disease was found in the lungs&#44; perhaps this pattern was caused by other condition than PCH&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;4&#44;9&#44;10</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">Although the role of V&#47;Q scan on PCH seems to be limited&#44; among all the V&#47;Q scan patterns reported&#44; the authors consider the one of augmented perfusion on the lung bases emerge as a relevant one&#46; In fact this pattern seems to help on the diagnosis of PCH&#44; as supported by our case and other authors&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> but can be misinterpreted as decreased perfusion on the upper lobes&#44; leading to erroneous diagnostic hypotheses&#46; Therefore&#44; the authors conclude that awareness of this pattern is important&#44; especially considering that&#44; although PCH is a rare condition&#44; the elevated number of V&#47;Q scans performed on PAH patients&#44; increases the possibility of coming across it&#46;</p></span></span>"
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          "identificador" => "xpalclavsec106896"
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          "identificador" => "xres119615"
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          "titulo" => "Introduction"
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    "fechaRecibido" => "2012-01-12"
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          "clase" => "keyword"
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            0 => "Pulmonary hypertension"
            1 => "Pulmonary capillary hemangiomatosis"
            2 => "Ventilation&#47;perfusion lung scintigraphy"
            3 => "Thoracic CT"
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            0 => "Hipertensi&#243;n pulmonar"
            1 => "Hemangiomatosis capilar pulmonar"
            2 => "Gammagraf&#237;a pulmonar de ventilaci&#243;n&#47;perfusi&#243;n"
            3 => "TC tor&#225;cica"
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      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pulmonary capillary hemangiomatosis is a rare disease&#44; characterized by small capillaries proliferation&#44; leading to pulmonary hypertension&#46; The authors report a case of pulmonary capillary hemangiomatosis&#44; and discuss its diagnostic difficulties&#46; Special attention is presented to ventilation&#47;perfusion scintigraphy&#44; given both its importance to the evaluation of pulmonary hypertension&#44; and its referred limited usefulness in pulmonary capillary hemangiomatosis&#46; The few published cases of ventilation&#47;perfusion scintigraphy on this entity have showed different patterns&#44; which are discussed&#46; This case presents a pattern with augmented perfusion on lung bases and normal ventilation&#44; which has been described by other authors as typical for pulmonary capillary hemangiomatosis&#46; The authors consider important to retain this pattern&#44; when evaluating pulmonary hypertensive patients&#44; given not only its possible ability to help on pulmonary capillary hemangiomatosis diagnosis&#44; but also mainly its risk of misinterpretation as a decreased perfusion on upper lung lobes&#44; leading to erroneous diagnostic hypotheses&#46;</p>"
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La hemangiomatosis capilar pulmonar constituye una enfermedad rara&#44; caracterizada por la proliferaci&#243;n de peque&#241;os capilares que originan una hipertensi&#243;n pulmonar&#46; Los autores presentan un caso de hemangiomatosis capilar pulmonar&#44; debatiendo sus dificultades diagn&#243;sticas&#46; Se presta una atenci&#243;n especial a la gammagraf&#237;a de ventilaci&#243;n&#47;perfusi&#243;n&#44; dada su importancia para la evaluaci&#243;n de la hipertensi&#243;n pulmonar y su utilidad limitada en la hemangiomatosis capilar pulmonar&#46; Se discuten los pocos casos publicados de gammagraf&#237;a de ventilaci&#243;n&#47;perfusi&#243;n en esta entidad cr&#243;nica&#44; que han mostrado diferentes patrones&#46; Este caso presenta un patr&#243;n con incremento de perfusi&#243;n en las bases pulmonares y ventilaci&#243;n pulmonar normal&#44; que ha sido descrito por otros autores como t&#237;pico en la hemangiomatosis capilar pulmonar&#46; Los autores consideran importante la identificaci&#243;n de este patr&#243;n al evaluar a los pacientes con hipertensi&#243;n pulmonar&#44; dado que podr&#237;a suponer una ayuda&#44; no s&#243;lo para el diagn&#243;stico de la hemangiomatosis capilar pulmonar&#44; sino tambi&#233;n por el riesgo de interpretar mal el descenso de perfusi&#243;n en los l&#243;bulos pulmonares superiores&#44; que puede conducir a unas hip&#243;tesis diagn&#243;sticas err&#243;neas&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Lung ventilation &#40;upper row&#41; and perfusion &#40;lower row&#41; scintigraphy show homogeneous ventilation in both lungs and augmented diffuse perfusion in both lung bases&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Thoracic CT scan shows &#40;A&#41; a micronodular centrilobular pattern with ground glass opacities&#44; feature more prominent on the lower two-thirds of both lungs &#40;lung window&#41;&#59; &#40;B&#41; enlargement of the central and segmental pulmonary arteries &#40;mediastinal window&#41; and &#40;C&#41; mediastinal lymphadenopathy &#40;mediastinal window&#41;&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Histology of resected lung tissue&#46; &#40;A&#41; On lower magnification&#44; a patchy alveolar wall thickening with extensive capillary proliferation&#44; and intra-alveolar haemorrhage can be seen &#40;hematoxilin&#8211;eosin stain&#41;&#46; &#40;B&#41; On higher magnification&#44; proliferating capillary channels within the septa&#44; surrounding pulmonary vessels and muscular hyperplasia on small arteries are better demonstrated with this vascular endothelial marker &#40;CD31 immunohistochemical stain&#41;&#46;</p>"
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ISSN: 22538089
Original language: English
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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