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Acute Onset of Severe Shortness of Breath in a 12-Year-Old Asthmatic FREE TO VIEW

Angel Brown, MD; Keith Wille, MD
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UAB, Birmingham, AL

Chest. 2013;144(4_MeetingAbstracts):897A. doi:10.1378/chest.1703309
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SESSION TITLE: Miscellaneous Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary veno-occlusive disease (PVOD) is a rarely encountered cause of pulmonary arterial hypertension (PAH). It is rapidly progressive, difficult to recognize and carries a poor prognosis.

CASE PRESENTATION: A 12 year-old female diagnosed with asthma presented with acute onset of dyspnea at rest, unresponsive to nebulized albuterol. She reported fatigue, weight loss, and palpitations during the preceding 6 months. Her surgical, family and social histories were unremarkable. Home medications included inhaled fluticasone/salmeterol, albuterol HFA and cetirizine. Physical exam revealed HR 110, room air SpO2 in the 70s and SpO2 92% on 4 liters nasal cannula, with inspiratory and expiratory wheezes. Radiography showed a large pulmonary trunk, enlarged heart and a right pleural effusion. CT angiography was negative for pulmonary emboli but did show diffuse ground-glass opacities and mediastinal and hilar lymphadenopathy. Laboratory data was normal. Her TLC was 76% and DLCO was 54% of predicted. ECHO showed a hyperdynamic left ventricle with an EF of 90%, PAP(diastolic) 30mmHg and right ventricular pressure 140mmHg. Right heart catheterization revealed PAP 73/58 and pulmonary artery occlusion pressure (PCWP) of 11mmHg. She was initially treated with supplemental oxygen, epoprostenol, milrinone, methylprednisolone and nitric oxide for symptom relief. She underwent a diagnostic open lung biopsy. The wedge biopsy revealed multifocal pulmonary and venular intimal fibrosis and occlusions with moderate medial hypertrophy of pulmonary arteries and focal muscularization of lymphatic vessels. Her condition deteriorated rapidly. Less than four months after the biopsy she received bilateral lobar lung transplants.

DISCUSSION: PVOD is a rare post-capillary cause of pulmonary hypertension. It is characterized by occlusion or narrowing of pulmonary veins and venules. Historically, PVOD was first described in 1934 in a patient who died of right-sided heart failure. The incidence is 0.2 cases per million annually. The etiology remains unknown. Diagnosis is based upon PAH, pulmonary edema and normalPCWP. Although surgical lung biopsy is definitive, it is not necessary to make the diagnosis in the right clinical setting. Medical management is directed towards reduction of pulmonary hypertension. While epoprostenol therapy has sometimes improved hemodynamics, there is a risk of fulminant pulmonary edema and/or death. There is no consensus on therapy, but lung transplantation is considered curative.

CONCLUSIONS: PVOD is a rare cause of pulmonary hypertension. Causes of this disorder in isolation are not well understood. Without treatment, prognosis is poor, with death at 1-2 years following diagnosis. Treatment is controversial but lung transplant is considered curative. Early referral for transplant is essential.

Reference #1: Celli BR, Karlinsky JB, eds. Textbook of Pulmonary Diseases. Philadelphia, Pa: Lippincott-Raven; 1998:1273-95.

DISCLOSURE: The following authors have nothing to disclose: Angel Brown, Keith Wille

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