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Congenital heart disease (CHD) patients (pts) often develop early heart failure (HF) with few therapeutic options leading to transplant (HT). Unfortunately, guidelines for cardiac resynchronization therapy (CRT) don't apply and function evaluations by cardiac ultrasound are often inaccurate among CHD anatomies. Therefore, which CHD pts would benefit from CRT remains an enigma. The purpose of this study was to use acute CRT paced-contractility (dP/dt-max) response (PCR) as a pre-implant evaluation among CHD pts and to follow clinical parameters post implant .
Forty CHD pts ( NYHA II-IV HF) on optimal medical therapy were referred for PCR cardiac catheterization prior to CRT consideration. If dP/dt-max improved ≥15% from baseline, these “responders” were given the option of CRT with continued follow-up after implant.
Of 40 pts studied, 26 (65%) (mean (SD) age 22±8.2y; 9/26 (35%) single or systemic “right” ventricle; 17/26 (65%) with pacemakers) met criteria for possible hemodynamic benefit and underwent CRT implant while 14 (35%) (mean age 29±13y) did not and continued on standard HF management. Following CRT, all 26 pts improved in NYHA classification: 5/26 pts (19%) were later re-listed for HT (4-144 months, mean 46±35) while 21/26 (81%) continued off the HT list 12-112 (mean 35) months later. A repeat study following chronic CRT showed continued improved contractility.
HF is common among CHD pts and therapies are limited. CRT guidelines do not address clinical and anatomical issues of CHD. Acute PCR testing identifies those CHD pts likely to respond to CRT regardless of anatomy.