Mechanical Circulatory Support for Patients With Adult Congenital Heart Disease

Abstract

Advances in surgical and medical care of children born with heart defects have led to the emergence of a unique subgroup of young adults known as adults with congenital heart disease (ACHD). Heart failure (HF) is the leading cause of mortality and morbidity in this subset. Management of HF is challenging in these patients owing to inherent structural variations with their associated physiological consequences. Heart transplantation is of limited utility in this group either because of donor shortage or associated comorbidities that make these patients ineligible for transplantation. Mechanical circulatory support (MCS) devices have evolved as an alternative treatment modality in supporting the failing myocardium of this population, but are often used less frequently than in those with a structurally normal heart because of the unique anatomical and physiological variations. These variations create a need to gather adequate knowledge on how best to support the hearts of ACHD patients in order to reduce mortality and morbidity. This review presents clinical experience with MCS in ACHD patients.

Changes in waitlist and posttransplant outcomes in patients with adult congenital heart disease after the new heart transplant allocation system

“…However, they are often used less frequently in ACHD patients compared to those with structurally normal hearts due to their unique anatomical and physiological variations. 21 A larger portion of delisted patients are delisted due to decompensation in the post-policy change era (40.9% vs. 28.5%), and higher PVR is an independent predictor of delisting. Preoperative end-organ dysfunction is a risk factor for post-transplant mortality for ACHD recipients.…”

“…Mechanical circulatory support devices (MCS) have evolved as alternative treatment modalities to support the failing myocardium in ACHD patients. However, they are often used less frequently in ACHD patients compared to those with structurally normal hearts due to their unique anatomical and physiological variations 21 . A larger portion of delisted patients are delisted due to decompensation in the post‐policy change era (40.9% vs. 28.5%), and higher PVR is an independent predictor of delisting.…”

Cardiogenic shock in adults with congenital heart disease: Insights from the FRENSHOCK registry

“…ACHD patients with heart failure frequently present with more advanced disease than adults without CHD (non-ACHD) [5], but they seem less likely to be treated with advanced cardiac therapies, such as mechanical circulatory support or heart transplantation, than non-ACHD patients [6,7]. Management of heart failure is very challenging in these patients because of inherent structural variations, with their associated physiological consequences [8,9].Recent consensuses from the European Society of Cardiology and the American Heart Association have been published to help clinicians with the management of chronic heart failure in ACHD patients [10,11]. However, few data concern the management of acute heart failure, and even fewer pertain to cardiogenic shock (CS).…”

Cardiogenic shock in adults with congenital heart disease: Insights from the FRENSHOCK registry

“…ACHD patients with heart failure frequently present with more advanced disease than adults without CHD (non-ACHD) [5], but they seem less likely to be treated with advanced cardiac therapies, such as mechanical circulatory support or heart transplantation, than non-ACHD patients [6,7]. Management of heart failure is very challenging in these patients because of inherent structural variations, with their associated physiological consequences [8,9].Recent consensuses from the European Society of Cardiology and the American Heart Association have been published to help clinicians with the management of chronic heart failure in ACHD patients [10,11]. However, few data concern the management of acute heart failure, and even fewer pertain to cardiogenic shock (CS).…”