The PE that never was: A rare case of combined PVOD and pulmonary vein thrombosis.
Albujoq K, Awdish R, and Hegab S. The PE that never was: A rare case of combined PVOD and pulmonary vein thrombosis. Am J Respir Crit Care Med 2017; 195.
Am J Respir Crit Care Med
Pulmonary Veno-Occlusive Disease is a subset of group 1 pulmonary hypertension characterized by diffuse occlusion of the pulmonary veins resulting in torturous dilation of the pulmonary capillaries. Despite early identification, the natural progression of disease results in significant mortality. We describe a case of a patient with a diagnosis of PVOD and pulmonary vein thrombosis. Case Presentation: The patient is a healthy 23 yo male who initially presented in December 2015 with a 3 month history of shortness of breath. CT PE suggested pneumonia and likely PE. Despite therapy, he rapidly deteriorated. Further workup with echocardiogram revealed a PAP 70mmHg. The patient was transferred to our facility for further pulmonary hypertension workup. Autoimmune studies were negative. V/Q scan was high probability. CT showed septal thickening, ground glass opacities, and hilar lymphadenopathy concerning for PVOD. RHC revealed PAP 73/37/46 with PCWP 5, PVR 6.38, and CO/CI 6.42/2.69. Pulmonary angiogram showed multiple cut-offs throughout the entire right lung and left upper lobe during the venous phase not consistent with CTEPH, but concerning for extensive pulmonary vein thrombosis. Bronchoscopy with biopsy was negative for sarcoidosis or malignancy. Transplant evaluation was initiated. Hypercoaguability workup for pulmonary vein thrombosis was negative. He was stabilized and discharged with supplemental oxygen and anticoagulation. He returned within one month with dyspnea at rest. He was admitted and listed for lung trasplant with a LAS of 49.21. A trial of low dose IV epoprostenol was unsuccessful. The patient had progressive RV failure with increasing oxygen demands and was initiated on VA ECMO to avoid intubation and the risk of progression of RV dysfunction with positive pressure mechanical ventilation. After 2 days of VA ECMO, the patient had b/l lung transplant. Post transplant echocardiogram showed normal RV size and mildly reduced RV systolic function. Pathology of the explanted lungs were consistent with PVOD. Genetic testing was negative. The patient continues to rehab successfully. Discussion: Our patient had PVOD and evidence of pulmonary vein thrombosis of unclear etiology. It is suspected that his initial imaging suggesting PE was misread and was actually the pulmonary vein thrombosis. PVOD may be implicated in up to 10% of group 1 PHTN, and is often misdiagnosed. Although rare, PVOD is a rapidly progressive diagnosis resulting in mortality prior to effective treatment with lung transplant. It is imperative to refer patients with PVOD to a tertiary center with transplant capabilities for diagnosis and treatment.