cardiovascular-agents and Hypoplastic-Left-Heart-Syndrome

Hypoplastic left heart syndrome (HLHS) is the most common functional single ventricle congenital cardiac defect. This syndrome is characterised by a functional single right ventricle and systemic outflow obstruction. The systemic and pulmonary circulations compete for cardiac output with a resultant precarious balance among systemic, pulmonary and coronary blood flows. A once fatal diagnosis, advances in operative and perioperative care have resulted in a dramatic improvement in survival. The preoperative management of neonates with HLHS is based predominately on clinical experience and extrapolated data from the postoperative literature. Management focuses on maintaining patency of the systemic outflow, balancing the pulmonary and systemic blood flows, and preserving the function of a single right ventricle to maximise oxygen delivery to the tissues. This paper reviews the available therapies for the preoperative management of HLHS.

Topics: Cardiovascular Agents; Disease Management; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Preoperative Care

Newborns with hypoplastic left heart (HLH) are usually palliated with the Norwood procedure or a hybrid stage I procedure. Hybrid is our preferred approach. Given the critical relationship between stage I, interstage, and comprehensive stage II or advanced biventricular repair, we hypothesized that appropriate drug treatment is a significant therapeutic cornerstone, especially for the management of the high-risk interstage.. We report a single-center observational study addressing the cardiovascular effects of, in particular, oral β-blockers and the additional use of angiotensin-converting enzyme (ACE) and mineralocorticoid inhibitors.. In total, 51 newborns-30 with HLH syndrome (HLHS) and 21 with HLH complex (HLHC)-with a median bodyweight of 3.0 kg (range 1.9-4.4; nine with bodyweight ≤ 2500 g) underwent an uneventful "Giessen hybrid approach" using a newly approved duct stent. All patients were discharged home with a single, double or triple therapy consisting of ß-blockers, ACE and mineralocorticoid inhibitors; 90% of the patients received bisoprolol, 10% received propranolol, 72% received lisinopril, and 78% received spironolactone. Resting heart rate decreased from 138 bpm (range 112-172; n = 51) at admission to 123 bpm (range 99-139; n = 51) at discharge and 110 bpm before stage II/biventricular repair/heart transplantation (range 90-140; n = 37) accompanied by favorable bodyweight gain. No side effects were evident.. In view of drug risk/benefit profiles, as well as the variable morphology and hemodynamics, the highly selective β1-adrenoceptor blocker bisoprolol is our preferred drug for treatment of HLHS/HLHC in the interstage. We avoid using ACE inhibitor monotherapy and exclude potential risks for coronary and cerebral perfusion pressure beforehand.

Topics: Cardiovascular Agents; Female; Heart Transplantation; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Male; Norwood Procedures; Retrospective Studies; Treatment Outcome