063 A Case of Chronic Thromboembolic Pulmonary Hypertension in a Heart Transplant Patient, Successfully Treated With Pulmonary Endarterectomy

Abstract

We present a case of chronic thromboembolic pulmonary hypertension (CTEPH) 15 years post heart transplantation, requiring pulmonary endarterectomy, with subsequent improvement of pulmonary hypertension (PH) and symptoms. The patient was a 71 year old male presenting with dyspnoea, who previously underwent heart transplantation for idiopathic dilated cardiomyopathy. His co-morbidities included a pulmonary embolism (PE) after traumatic pulmonary contusion ten years prior, mild renal impairment, type 2 diabetes, hypertension and obstructive sleep apnoea. Medications included warfarin, cyclosporin, everolimus, mycophenolate mofetil and prednisolone. Transthoracic echocardiogram demonstrated elevated pulmonary pressures. On right heart catheter (RHC); pulmonary artery systolic pressure (PASP) was 66 mmHg, pulmonary artery diastolic pressure (PADP) was 28 mmHg, and mean pulmonary artery pressure (mPAP) was 45 mmHg. The mean right atrial pressure (RAP) was 9 mmHg, mean pulmonary capillary wedge pressure (MPCWP) was 8 mmHg and pulmonary vascular resistance (PVR) was 562 dynes/sec/cm5. V/Q and CTPA demonstrated bilateral chronic PE, confirmed on formal pulmonary angiography. The patient’s six-minute walk test (6MWT) was 317m (50% predicted). Riociguat was commenced and warfarin continued, and he underwent a pulmonary endarterectomy. The patient’s symptoms improved and his 6MWT was 397 m (63% predicted). RHC 11 months later showed a PASP of 47 mmHg, PADP of 23 mmHg, mPAP of 33 mmHg, RAP of 10 mmHg and PVR of 240 dynes/sec/cm5; demonstrating improvement. This demonstrates the first described case of CTEPH post-heart transplant. As a treatable cause of PH, the importance of a high index of suspicion for CTEPH is clear.