Pulmonary Vascular Disease |

Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension FREE TO VIEW

Glenda Euceda, MD; Michael Denson, MD; Steven Sieber, MD; David Oelberg, MD
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Danbury Hospital, Danbuy, CT

Chest. 2015;148(4_MeetingAbstracts):989A. doi:10.1378/chest.2267442
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SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH), mimicking pulmonary veno-occlusive disease. We report a case of PCH in a renal transplant recipient with rapidly progressive right heart failure.

CASE PRESENTATION: A 66 year old nonsmoking Caucasian female, presented with worsening ankle edema and exertional dyspnea over two months. She was a renal transplant recipient on tacrolimus and mycophenolate mofetil, who was using nocturnal CPAP for obstructive sleep apnea. An echocardiogram showed right ventricular dysfunction and an RVSP of 72 mmHg. Her symptoms progressed despite diuretics and supplemental oxygen. She presented to the ER, hypotensive, hypoxic, with pulmonary edema on chest radiograph. She was intubated and given dobutamine with norepinephrine. Pulmonary arterial catheterization confirmed severe PH with reduced cardiac output. Within 24 hours, she developed multi-organ failure and died. A post-mortem exam revealed PCH, characterized by patchy expansion of the alveolar septa, peribronchiolar and perivenular interstitium by a proliferation of capillaries and intraalveolar hemosiderin-laden macrophages.

DISCUSSION: PCH is a rare cause of PH involving proliferation of capillaries within the alveolar septa as well as bronchial and venous walls, pleura, and regional lymph nodes, rendering the histopathology of a low-grade capillary neoplasm. Described by Wagenvoort et al in 1978, it occurs in patients 6 to 71 years of age, but most are in their third and fourth decades.2 It typically causes slowly progressive dyspnea and right heart failure, with a hemodynamic profile mimicking pulmonary veno-occlusive disease.1 The prognosis is poor with a median survival of three years.3 Treatment with diuretics, ACE inhibitors, corticosteroids, and warfarin have been unsuccessful, and the use of prostaglandins has been associated with worsened hypoxia, edema and death. However, case reports suggest that interferon alpha 2a may lead to clinical improvement and histologic regression by inhibiting endothelial cell proliferation. Only bilateral lung or heart/lung transplant currently offer a long-term survival advantage.2

CONCLUSIONS: PCH is a low-grade tumor-like disorder of pulmonary capillaries characterized by a locally infiltrative growth leading to severe PH, and often fatal right heart failure. This case illustrates that it can be rapidly progressive. Interferon alfa 2a may be beneficial, but only lung transplantation offers the potential to avoid a fatal outcome.

Reference #1: Eltorky, M et al (1994), PCH. A Clinicopathologic Review. Ann Thorac Surg (57), pp. 772-6

Reference #2: Yi, E. S. (2004), Tumors of the Pulmonary Vasculature. Cardiology Clinics, (22), pp. 431-440

Reference #3: El-Gabaly, M et al (2007). PCH Imaging findings and Literature Update. J Comput Assist Tomogr, (31), pp.608-610

DISCLOSURE: The following authors have nothing to disclose: Glenda Euceda, Michael Denson, Steven Sieber, David Oelberg

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