Cardiovascular Drug Therapy during Interstage After Hybrid Approach: A Single-Center Experience in 51 Newborns with Hypoplastic Left Heart

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Fig. 1

Summary of the follow-up surgeries after hybrid stage I. BVR biventricular repair, ccTGA congenital corrected transposition of the great arteries, HLHC hypoplastic left heart complex, HLHS hypoplastic left heart syndrome, HTX heart transplantation, LV left ventricle, TCPC total cavo-pulmonary connection

Fig. 2

Body weight data from patients with hypoplastic left heart syndrome at admission and discharge, and weight gain before comprehensive stage II

Fig. 3

Echocardiographic four-chamber view showing the volume-loaded RV in hypoplastic left heart syndrome before (a) and unloaded and hypertrophied after comprehensive stage II (b). Based on this well-known phenomenon following comprehensive stage II, we hypothesized that a bisoprolol–lisinopril–spironolactone treatment strategy might be beneficial to reduce the severity of a diastolic dysfunctional RV. hLV hypoplastic left ventricle, LA left artery, LV left ventricle, RA right artery, RV right ventricle