Severe Pulmonary Hypertension in the Setting of Complex Structural Heart Disease

Abstract

Pulmonary hypertension (pHTN) complicating structural heart disease leads to distinct diagnostic and management dilemmas. We present a case of severe pulmonary hypertension in the setting of perimembranous VSD and severe AS. 76 yo female with restrictive VSD and severe AS presented with heart failure. Cardiac findings included RV heave and AS murmur. TTE revealed normal EF with severe pHTN, severe RV dysfunction, and severe AS. Her perimembranous septum was aneurysmal, with systolic jet velocity of 4.5 m/s. There was no CAD. RHC demonstrated PAP of 96/38/57 mmHg, PVR of 7.2 WU, PCWP 23 mmHg, CI of 3.04, and Qp/Qs of 1.1. The PCWP and PVR indicated pre- and post-capillary pHTN due to longstanding AS. She was deemed inoperable and was high risk for TAVR given aortic annular continuity with the VSD and ∼25% annular deficiency. There was concern that her elevated LVEDP was masking Eisenmenger physiology, and that relieving the AS may precipitate hypoxia. Balloon aortic valvuloplasty (BAV) was performed as bridge to decision. This enabled assessment of the integrity of the aortic annulus, the pulmonary pressure response to LV unloading, and development of right to left shunting after a decrease in LVEDP. Successful BAV was performed, and the patient was discharged. Following improvement in both PA pressures and her symptoms, a TAVR was offered. A self-expanding valve was selected to minimize risk of annular rupture. After successful deployment, her PAP were 78/42/54 and PCWP 23. She was diuresed and discharged. Our case highlights important points in management of such complex patients with pHTN. Careful evaluation of hemodynamics is paramount in guiding decision making. The preserved PAPi with minimal shunting suggested the VSD was not significantly contributing to the severe pHTN. Additionally, BAV plays an important role as a bridge to determine potential response to TAVR. Although patients with WHO II pHTN can develop an irreversible pre-capillary component, such assessment can only be made following durable reduction in LVEDP. Lastly, interdisciplinary patient care is essential. Successful management of our patient required input from a multidisciplinary team consisting of pulmonology, structural cardiology, critical care cardiology and heart failure.