16 Years of Cardiac Resynchronization Pacing Among Congenital Heart Disease Patients: Direct Contractility (dP/dt-max) Screening When the Guidelines Do Not Apply

Objectives The purpose of this study was to use direct cardiac resynchronization therapy (CRT)-paced contractility (dP/dt-max) response as a pre-implantation evaluation among patients with congenital heart disease (CHD) and follow clinical parameters and contractility indexes after CRT implantation. Background Patients with CHD often develop early heart failure with few therapeutic options, leading to heart transplantation (HT). Unfortunately, guidelines for CRT do not apply, and function evaluations by cardiac ultrasound are often inaccurate among CHD anatomies. Therefore, which CHD patients would benefit from CRT remains an enigma. Methods From 1999 to 2015, 103 CHD patients with New York Heart Association (NYHA) functional class II to IV were listed for HT; 40 patients on optimal medical therapy were referred for paced contractility response cardiac catheterization before CRT consideration. If dP/dt-max improved ≥15% from baseline, these “responders” were given the option of CRT with continued follow-up after implantation. Results Of 40 patients studied, 26 (65%) (age 22 ± 8.2 years; 9 of 26 [35%] single or systemic right ventricle; 17 of 26 [65%] with pacemakers) met criteria for possible hemodynamic benefit and underwent CRT implantation. All 26 patients improved in NYHA functional classification: 5 of 26 patients (19%) were later relisted for HT (4 to 144 months, mean 55 months) after CRT implantation, whereas 21 of 26 (81%) continued with improved NYHA functional class (12 to 112 months, mean 44 months) later. A repeat dP/dt-max study following long-term CRT showed stable function or continued contractility improvement. Conclusions Heart failure is common among CHD patients, and therapies are limited. CRT guidelines do not address clinical and anatomic issues of CHD. Short-term paced contractility response testing identifies those CHD patients who are likely to respond to CRT regardless of anatomy.