Rapid Reversal of Pulmonary Hypertension in Failing Mustard Physiology After Continuous Flow Systemic Ventricular Assist Device

Systemic right ventricular failure is a well-known sequela in the decades following atrial switch for D-transposition of the great arteries. As patients who underwent these procedures are now entering adulthood, the disease burden can only be expected to grow. Cardiac transplantation is a well-accepted therapy for systemic RV failure, but secondary pulmonary hypertension may often be prohibitive. We present a case of severe systemic RV failure and pulmonary hypertension, with subsequent complete normalization of pulmonary pressures following ventricular assist device implantation. Our patient is a 34 year old male with D-TGA who underwent Mustard operation at 14 months of age. Surveillance monitoring showed stable pulmonary pressures (Table 1), but slowly decreasing RVEF and increasing RVEDV. In late June 2014, the patient presented in acute heart failure. Catheterization on July 3, 2014 (Table 1), revealed significantly increased PA pressure and PVR (>9 Woods units), as well as decreased CI, confirming cardiogenic shock. Due to the patient’s inotrope dependence, and prohibitive elevation in PVR, he underwent implantation of a HeartWare HVAD for systemic RV support. He tolerated the procedure very well. VAD flows were titrated with TEE monitoring. His post-operative course was complicated by hemothorax in his left pleural space requiring evacuation on POD# 13. He was successfully discharged on POD# 22. He has not had any unplanned hospital readmissions and is now able to walk more than one mile daily without symptoms. Repeat catheterization in November 2014 showed complete normalization of his pulmonary pressure, PVR, and CI (Table 1). He continues to do well and is now undergoing evaluation for cardiac transplantation. There are few case reports that describe VAD implantation for RV failure in a patient with prior history of atrial switch. To our knowledge, this is the first report of complete normalization of pulmonary hemodynamics following VAD implantation into a systemic RV in less than 4 months. Ventricular assist devices may be a safe and viable option to bridge patients with systemic RV failure and secondary pulmonary hypertension to cardiac transplantation. Additionally, VADs may serve this group of patients who are not immediately able to be transplanted due to donor supply shortage or hemodynamic instability. In the future, this can possibly be expanded to failing systemic right ventricles in patient with other types of complex congenital heart disease.Table 1DateMPAP (mm Hg)PVR (wood's units)RVEDP (mm Hg)PCWP (mm Hg)Cl (L/min/m2)2/9/2012363.618232.195/24/2012342.3214232.3910/29/2013353.0120232.487/2/2014529.5821351.6711/3/2014272.58NR182.97 Open table in a new tab