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Denovo Pulmonary Arterial Hypertension After Liver Transplantation in Patient with History of Portopulmonary Hypertension
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A3696 - Denovo Pulmonary Arterial Hypertension After Liver Transplantation in Patient with History of Portopulmonary Hypertension
Author Block: D. Kay1, A. Karki1, J. Cope2, H. Alnuaimat1, A. Ataya1; 1Pulmonary and Critical Care, University of Florida, Gainesville, FL, United States, 2University of Florida, Gainesville, FL, United States.
Introduction: Porto-pulmonary hypertension (PoPH) is a rare pulmonary complication of advanced portal hypertension. It is associated with increased mortality and also poses challenges in management. The occurrence of pulmonary arterial hypertension (PAH) in a patient with a history of portopulmonary hypertension that resolved after liver transplant has rarely been described in literature. We present a case of a patient with resolution of PoPH after liver transplant and who developed denovo PAH 16 years later.
Case: A 59-year-old female with a history of liver cavernous hemangiomas underwent orthotopic liver transplant 16 years ago. Prior to her liver transplant, she was found to have very elevated pulmonary pressures on echocardiogram and was treated with inhaled and intravenous Flolan. After transplant, she was asymptomatic until 16 years later when she presented to us with symptoms of shortness of breath with more than normal activity (WHO functional class 2). Pulmonary function testing showed normal spirometry with an isolated reduced DLCO (55%). A six minute walk test showed desaturations to 88% on exertion and an echocardiogram revealed enlarged right chambers with preserved ejection fraction of 55%. Negative ventilation-perfusion scan. On right heart catheterization, she was found to have significantly elevated pulmonary pressures, with a mean pulmonary arterial pressure of 62mmHg, PCWP 15, PVR 11.8, CO/CI 4.0/2.2, and no evidence of elevated hepatic pressure gradient. She was diagnosed with PAH and started on treatment with furosemide, Tadalafil and Macitentan, with stable oxygen requirements and symptoms on followup.
Discussion: The occurrence of pulmonary hypertension post liver transplant is usually the result of persistent porto-pulmonary hypertension. When this is the case, the patients have persistence of symptoms or recurrence of symptoms shortly after the transplant. Our case, however, is unique as the patient was asymptomatic with resolution of echocardiogram findings posttransplant as well as symptoms for many years before the new onset of shortness of breath. This points towards a new diagnosis of pulmonary arterial hypertension. To our knowledge, this is only the second case of denovo pulmonary arterial hypertension post liver transplant and the pathophysiologic mechanism is not very clear. However, the patient responded well to the therapy for pulmonary hypertension.
Author Block: D. Kay1, A. Karki1, J. Cope2, H. Alnuaimat1, A. Ataya1; 1Pulmonary and Critical Care, University of Florida, Gainesville, FL, United States, 2University of Florida, Gainesville, FL, United States.
Introduction: Porto-pulmonary hypertension (PoPH) is a rare pulmonary complication of advanced portal hypertension. It is associated with increased mortality and also poses challenges in management. The occurrence of pulmonary arterial hypertension (PAH) in a patient with a history of portopulmonary hypertension that resolved after liver transplant has rarely been described in literature. We present a case of a patient with resolution of PoPH after liver transplant and who developed denovo PAH 16 years later.
Case: A 59-year-old female with a history of liver cavernous hemangiomas underwent orthotopic liver transplant 16 years ago. Prior to her liver transplant, she was found to have very elevated pulmonary pressures on echocardiogram and was treated with inhaled and intravenous Flolan. After transplant, she was asymptomatic until 16 years later when she presented to us with symptoms of shortness of breath with more than normal activity (WHO functional class 2). Pulmonary function testing showed normal spirometry with an isolated reduced DLCO (55%). A six minute walk test showed desaturations to 88% on exertion and an echocardiogram revealed enlarged right chambers with preserved ejection fraction of 55%. Negative ventilation-perfusion scan. On right heart catheterization, she was found to have significantly elevated pulmonary pressures, with a mean pulmonary arterial pressure of 62mmHg, PCWP 15, PVR 11.8, CO/CI 4.0/2.2, and no evidence of elevated hepatic pressure gradient. She was diagnosed with PAH and started on treatment with furosemide, Tadalafil and Macitentan, with stable oxygen requirements and symptoms on followup.
Discussion: The occurrence of pulmonary hypertension post liver transplant is usually the result of persistent porto-pulmonary hypertension. When this is the case, the patients have persistence of symptoms or recurrence of symptoms shortly after the transplant. Our case, however, is unique as the patient was asymptomatic with resolution of echocardiogram findings posttransplant as well as symptoms for many years before the new onset of shortness of breath. This points towards a new diagnosis of pulmonary arterial hypertension. To our knowledge, this is only the second case of denovo pulmonary arterial hypertension post liver transplant and the pathophysiologic mechanism is not very clear. However, the patient responded well to the therapy for pulmonary hypertension.
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