Ventricular Assist Devices in Patients with Systemic Right Ventricular Failure due to Congenitally Corrected Transposition of the Great Arteries

Abstract

Congenitally corrected transposition of the great arteries is increasingly recognized as an adult congenital heart problem. Although early atrial switch surgical repairs resulted in improved overall survival, these patients are further predisposed to systemic ventricular failure in their fourth and fifth decades of life. Early diagnosis and management of systemic ventricular dysfunction are often challenging. Ventricular assist devices may become a mainstay endstage treatment option for these patients as a destination therapy or a bridge to heart transplantation.

Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care

“…Although the experience of subaortic VAD in patients with a 2V-RV is still evolving, survival is comparable with or exceeds that of VAD in acquired heart disease and is effective for similar indications (bridge-to-transplant, bridgeto-decision, bridge-to-candidacy). 57,58 Patients with 2V-RV circulations are in particular at risk of developing pulmonary hypertension. 44 As with patients with acquired heart disease, reducing subaortic RV end diastolic pressure with a VAD is effective at reducing PA pressures in some patients to levels acceptable for heart transplant, with those with persistent elevation of pulmonary vascular resistance continued on VAD as destination therapy or considered for combined heart-lung transplant.…”

“…There is a low threshold to replace a regurgitant aortic valve, because aortic regurgitation increases after VAD insertion reducing the effectiveness of the VAD due to the resultant circular shunt. 58,60 Similarly, an accurate assessment of the subpulmonary LV function is vital. Exacerbation of 2V-RV, biventricular circulation with subaortic right ventricle; ACHD, adult congenital heart disease; ACHD-HF, heart failure in adult congenital heart disease; BNP, brain-type natriuretic peptide; CI, confidence interval; FALD, Fontan-associated liver disease; HF, heart failure; HLA, human leucocyte antigen; HR, hazard ratio; NT-ProBNP, N-type pro hormone B-typr naturetic peptide; NYHA, New York Heart Association; PLE, protein losing enteropathy; VAD, ventricular assist device.…”