Long-Term Survivor With Pulmonary Veno-Occlusive Disease
2012; Lippincott Williams & Wilkins; Volume: 125; Issue: 12 Linguagem: Inglês
10.1161/circulationaha.111.088450
ISSN1524-4539
AutoresKensuke Matsushita, Masahiko Kanna, Takuya Yazawa, Satoru Shimizu, Manabu Nitta, Tetsu Takamizawa, Kentaro Arakawa, Hideto Yano, Masanori Nishikawa, Hideo Himeno,
Tópico(s)Mechanical Circulatory Support Devices
ResumoHomeCirculationVol. 125, No. 12Long-Term Survivor With Pulmonary Veno-Occlusive Disease Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessBrief ReportPDF/EPUBLong-Term Survivor With Pulmonary Veno-Occlusive Disease Kensuke Matsushita, MD, Masahiko Kanna, MD, Takuya Yazawa, MD, Satoru Shimizu, MD, Manabu Nitta, MD, Tetsu Takamizawa, MD, Kentaro Arakawa, MD, Hideto Yano, MD, Masanori Nishikawa, MD and Hideo Himeno, MD Kensuke MatsushitaKensuke Matsushita From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Masahiko KannaMasahiko Kanna From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Takuya YazawaTakuya Yazawa From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Satoru ShimizuSatoru Shimizu From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Manabu NittaManabu Nitta From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Tetsu TakamizawaTetsu Takamizawa From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Kentaro ArakawaKentaro Arakawa From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Hideto YanoHideto Yano From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. , Masanori NishikawaMasanori Nishikawa From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. and Hideo HimenoHideo Himeno From the Division of Cardiology, Fujisawa City Hospital, Kanagawa (K.M., S.S., M.N., T.T., K.A., H.Y., M.N., H.H.); Division of Cardiology, National Hospital Organization Sagamihara National Hospital, Kanagawa (M.K.); and Department of Pathology, Kyorin University School of Medicine, Tokyo (T.Y.), Japan. Originally published27 Mar 2012https://doi.org/10.1161/CIRCULATIONAHA.111.088450Circulation. 2012;125:e503–e506A 51-year-old woman was admitted to our hospital because of shortness of breath. Her mean pulmonary artery pressure was 52 mm Hg, and idiopathic pulmonary artery hypertension was diagnosed. She received beraprost sodium, imidapril, warfarin, and home-based oxygen therapy. At 67 years of age, dyspnea on effort worsened to New York Heart Association functional class III. The plasma level of brain natriuretic peptide was 57 pg/mL. Transthoracic echocardiography showed an enlarged right ventricle, severe tricuspid regurgitation, and a large pericardial effusion. At 68 years of age, she was urgently admitted to our hospital because of severe dyspnea at rest. A chest x-ray showed bilateral pulmonary artery enlargement (Figure 1). Computed tomography of the chest revealed that both main bronchi were compressed by the pulmonary artery aneurysms, with no mural thrombus in the pulmonary arteries (Figure 2). The diameters of the right and left main pulmonary arteries were 69.8 and 55.5 mm, respectively. Bronchoscopy during mechanical ventilation confirmed nearly complete extrinsic compression of the left and right main stem bronchi without positive airway pressure (Figure 3). On hospital day 3, continuous administration of epoprostenol was begun. She received continuous positive airway pressure by mechanical ventilation. Because the pulmonary hypertension had been worsening, treatment with imatinib (50 mg/d) was started on hospital day 69. However, the patient died of recurrent obstructive pneumonia 12 months after admission, and autopsy was conducted.Download figureDownload PowerPointFigure 1. A decubitus anteroposterior chest x-ray showing cardiomegaly, a dilated main pulmonary artery, and pleural effusion.Download figureDownload PowerPointFigure 2. A computed tomographic scan showing an enlarged pulmonary artery, gradually compressing both bronchi. A, The scan taken in 1992. B, The scan taken in 2001. C, The scan taken in 2009.Download figureDownload PowerPointFigure 3. Bronchoscopy during mechanical ventilation shows the respiratory tract well opened with high positive end-expiratory pressure (PEEP; top). On the other hand, without positive airway pressure, the left and right main stem bronchi were almost completely compressed (bottom).Histopathologically, pulmonary arteries/arterioles revealed dilatation, and alveolar walls were thickened with increased capillaries, deposition of hemosiderin, and loose fibrosis (Figure 4a and 4b). However, severe concentric medial hypertrophy, intimal fibrous thickening, and plexiform lesion, which are characteristic findings of idiopathic pulmonary artery hypertension, were not found in the pulmonary arteries/arterioles (Figure 4a and 4b). Normal pulmonary veins in the thickened interlobular septa mostly disappeared, and capillaries increased instead (Figure 4c and 4d).Download figureDownload PowerPointFigure 4. Autopsy lung revealed dilatation of pulmonary arteries (A and B; arrows). Alveolar walls were thickened with increased capillaries and hemosiderosis (C). In the interlobular septa, capillaries increased instead of pulmonary veins (C and D; arrows). BR indicates bronchus; PA, pulmonary artery; HE, hematoxylin and eosin stain; and EVG, elastica van Gieson stain.The pulmonary veins might have been destroyed during the prolonged disease course. Enlarged bronchial veins (Figure 5) probably served as collateral vessels of the occluded pulmonary veins. On reviewing the computed tomography scans obtained at the patient's initial admission, we found ground-glass opacities and smooth thickened intralobular septa, which are characteristic features of pulmonary veno-occlusive disease (PVOD; Figure 6).Download figureDownload PowerPointFigure 5. Enlarged bronchial veins served as collateral pathways (A and B). BR indicates bronchus; BV, bronchial vein; HE, hematoxylin and eosin stain; and EVG, elastica van Gieson stain.Download figureDownload PowerPointFigure 6. An axial computed tomographic scan showing ground-glass opacities, smooth thickened intralobular septa, and enlarged pulmonary veins (arrows).DiscussionPVOD is a rare cause of pulmonary hypertension. The origin of PVOD is unknown. It might represent an idiopathic process or a common pathological response to various injurious stimuli.1The symptoms of PVOD resemble those of pulmonary hypertension resulting from other causes and include progressive dyspnea on exertion, nonproductive cough, and edema of the lower extremities. However, medical treatment of primary pulmonary hypertension with prostacyclin can be fatal in patients with PVOD, and an early diagnosis is essential before therapy. PVOD is difficult to diagnose on routine examinations (eg, blood tests, chest radiography, ECG, echocardiography, Swan-Ganz catheterization). High-resolution computed tomography is essential for the diagnosis of PVOD because a lung biopsy can worsen respiratory status.2 The presence of ground-glass opacities (particularly with a centrilobular distribution), septal lines, and adenopathy are indicative of PVOD.3 In patients with PVOD, the interlobular septal veins are occluded; the capillary network is secondarily dilated, forming multiple vascular loops; the lymphatic channels are enlarged; and the interlobular septum is edematous.2 The only curative therapy is lung transplantation.In our patient, PVOD was definitively diagnosed at autopsy, although characteristic computed tomography findings had already developed (Figure 6). She survived for >15 years after initial admission, despite the poor prognosis of PVOD. To the best of our knowledge, this patient is the longest PVOD survivor.Prolonged survival might be attributed to several factors. First, the patient received early treatment with drugs such as warfarin and an angiotensin-converting enzyme inhibitor and showed no evidence of thrombi in the pulmonary arteries, which can excessively increase pulmonary artery pressure. Second, rich collateral vessels surrounded the alveoli (Figure 5) might have decreased the pulmonary artery pressure, acting like pulmonary veins in a healthy lung. Third, the dilated pulmonary artery might have contributed to slower disease progression, as reported previously.4ConclusionWe described a long-term survivor with PVOD who had a giant pulmonary artery aneurysm. Early medical intervention may improve outcomes in patients with PVOD.DisclosuresNone.FootnotesCorrespondence to Kensuke Matsushita, MD, Division of Cardiology, Fujisawa City Hospital, Kanagawa, Japan, 2-6-1, Fujisawa, Kanagawa, 251-8511, Japan. E-mail [email protected]co.jpReferences1. Barboza CE, Jardim CV, Hovnanian AL, Dias BA, Souza R. Pulmonary veno-occlusive disease: diagnostic and therapeutic alternatives. J Bras Pneumol. 2008; 34:749–752.CrossrefMedlineGoogle Scholar2. Rabiller A, Jaïs X, Hamid A, Resten A, Parent F, Haque R, Capron F, Sitbon O, Simonneau G, Humbert M. Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J. 2006; 27:108–113.CrossrefMedlineGoogle Scholar3. Resten A, Maitre S, Humbert M, Rabiller A, Sitbon O, Capron F, Simonneau G, Musset D. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 2004; 183:65–70.CrossrefMedlineGoogle Scholar4. Petrovic V, Ryerson CJ, Levy RD. Long-term survival in idiopathic pulmonary arterial hypertension associated with massive pulmonary artery dilation. Can Respir J. 2011; 18:e50–e51.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Gupta M, Agrawal A, Iakovou A, Cohen S, Shah R and Talwar A (2020) Pulmonary artery aneurysm: a review, Pulmonary Circulation, 10.1177/2045894020908780, 10:1, (1-10), Online publication date: 1-Jan-2020. Luo Q, Jin Q, Zhao Z, Zhao Q, Yu X, Yan L, Zhang Y, Xiong C and Liu Z (2019) Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?, BMC Pulmonary Medicine, 10.1186/s12890-019-1031-3, 19:1, Online publication date: 1-Dec-2019. Dai Z and Matsui Y (2014) Pulmonary Veno-Occlusive Disease: An 80-Year-Old Mystery, Respiration, 10.1159/000359973, 88:2, (148-157), . Weerakkody Y (2012) Pulmonary veno-occlusive disease Radiopaedia.org, 10.53347/rID-16679 March 27, 2012Vol 125, Issue 12 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.088450PMID: 22451612 Originally publishedMarch 27, 2012 PDF download Advertisement SubjectsComputerized Tomography (CT)Pulmonary BiologyPulmonary Hypertension
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