DIAGNOSIS OF PULMONARY VENO-OCCLUSIVE DISEASE BY CRYOBIOPSY

Abstract

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/22/2019 3:45 PM - 4:45 PM INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) and pulmonary veno-occlusive disease (PVOD) are rare disorders that are characterized by vascular proliferation of alveolar capillaries and pulmonary venules, respectively. Proliferation leads to vessel obstruction, elevated pulmonary vascular resistance and right heart failure. Given their identical clinical course, similar pathologic changes, and shared genetic mutation, they are now considered the same disease. Definitive diagnosis is made histologically. Patients with severe right heart failure are high-risk candidates for surgical lung biopsy, making pathologic confirmation prior to autopsy or explant is rare. Clinical recognition is crucial due to the dismal prognosis and risk for clinical worsening with vasodilators. CASE PRESENTATION: A 76-year-old woman presented to the emergency room with dyspnea. She was previously active until a few weeks prior when she developed shortness of breath, a 5-pound weight gain and new leg edema. She completed treatment for breast cancer several years prior. Her oxygen saturation was 93% breathing ambient air, and her chest X-ray showed vascular congestion. She was admitted for suspected heart failure exacerbation. An echocardiogram showed normal left ventricular systolic function, normal right ventricular systolic function, with an elevated right ventricular systolic pressure at 73 mmHg. A right heart catheterization showed a mean pulmonary artery pressure of 32mmHg, a pulmonary artery occlusion pressure of 5mmHg, with a pulmonary vascular resistance of 4.7 woods units, consistent with pulmonary arterial hypertension. A CT of the chest demonstrated intralobular septal thickening and ground glass opacities, multiple peripheral nodules, and bilateral mediastinal and hilar lymphadenopathy. An endobronchial ultrasound-guided biopsy was planned for her lymph nodes. Because of her pulmonary edema in the setting of pre-capillary pulmonary hypertension in the absence of left heart disease, PVOD was suspected and a cryobiopsy of the lung was also performed. No significant bleeding was noted. Pathology results of her lymph nodes were negative for malignancy, and the cryobiopsy of her lung parenchyma demonstrated septal capillary proliferation consistent with PCH. DISCUSSION: Cryobiopsy a less-invasive alternative to surgical lung biopsy. A larger lung sample with more intact architecture is obtained as compared to traditional transbronchial biopsy. Cryobiopsy has gained attention for diagnosis of interstitial lung disease in patients who are too high-risk to undergo surgery. Our case illustrates the first known case of PCH/PVOD diagnosed by cryobiopsy, and was achieved without significant bleeding. CONCLUSIONS: Diagnosis of patients with suspected PCH/PVOD is crucial to guide management, but they are often unable to undergo surgical lung biopsy for diagnosis. Cryobiopsy may be a safer alternative. Reference #1: Montani D, Lau EM, Dorfmüller P, Girerd B, Jaïs X, Savale L, Perros F, Nossent E, Garcia G, Parent F, Fadel E, Soubrier F, Sitbon O, Simonneau G, Humbert M. Pulmonary Veno-occlusive disease. European Respiratory Journal, May 2016, 47 (5) 1518-1534 DISCLOSURES: No relevant relationships by Isabel Bazan, source=Web Response No relevant relationships by Inderjit Singh, source=Web Response