SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: The accurate etiology of pulmonary arterial hypertension (PAH) can be challenging and misleading on the basis of clinical grounds alone. Here we present a unique case of severe PAH with unexpected findings on autopsy.
CASE PRESENTATION: A 73 year-old man known to have history of heavy exposure to asbestos. He had been followed for interstitial lung disease (ILD), asbestosis with plaques and pulmonary hypertension. Patient was admitted with progressive worsening dyspnea (NYHA functional Class IV). His past medical history included coronary artery disease, hypertension, atrial fibrillation on warfarin and sleep apnea (OSA). His exam was consistent with ILD and right sided heart failure. Patient has systolic murmur which increases during inspiration, bibasilar inspiratory crackles and lower extremity edema. His chest x-ray showed bilateral interstitial infiltrates with evidence of pleural plaques. Recent right heart catheterization showed severe pulmonary arterial hypertension (table 1). Pulmonary function test was consistent with a restrictive pattern. His DLCO declined from 56% in 2010 to 36% in 2013. Patient had recent 6 minute walk test and was only able to walk 22% of his predicted distance. Limited autopsy of the heart and lungs ruled out pulmonary embolism, acute myocardial infarction or significant asbestosis, however, it showed profound pulmonary capillary hemangiomatosis (Figure 1).
DISCUSSION: Pulmonary capillary hemangiomatosis (PCH) is rare cause of pulmonary arterial hypertension was first reported by Wagenvoort et al in 1978 . Less than 100 cases are reported to date. In this case, the interstitial lung disease is secondary to the proliferating capillary hemangiomatosis with hemosiderosis and fibrosis from chronic recurrent bleeding. The asbestosis contribution was minimal based on the trichrome staining which showed only mild interstitial fibrosis.
CONCLUSIONS: PCH can present at any age. When pulmonary arterial hypertension is out of proportion to the mild restrictive pathophysiology, we should consider etiologies other than ILD or OSA. The precise diagnosis of PCH is very important because the treatment with pulmonary vasodilators such as prostacyclin can cause fatal pulmonary edema .
Reference #1: Wagenvoort CA, Beetstra A, Spijker J. Capillary haemangiomatosis of the lungs. Histopathology. 1978; 2:401Y406.
Reference #2: Dufour B, Maitre S, Humbert M, et al. High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease. AJR Am J Roentgenol. 1998; 171:1321Y1324.
DISCLOSURE: The following authors have nothing to disclose: Abdulmonam Ali, Sri krishna Chaitanya Arudra, Sarthi Shah, Vasuki Anandan, Ragheb Assaly
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