Abstract
Introduction Cardiac resynchronization (CRT) therapy with biventricular (biV) or multi-site ventricular pacing has been shown to improve cardiac function and outcomes in adult patients with cardiomyopathy. However, little is known about the benefits of this therapy in infants with complex congenital heart disease. We present 4 inotrope-dependent heart failure patients being considered for heart transplant, 1 year or younger, from our institution who avoided heart transplantation following the initiation of CRT. Case Report Patient 1 has neonatal lupus and dilated cardiomyopathy with severely decreased ejection fraction (EF). Following a cardiac arrest at 2 months, she was unable to wean off ECMO and underwent CRT pacemaker (PM) placement. She weaned off ECMO in 48 hours and inotropes after 15 days. Her EF normalized 7 months after CRT upgrade. QRS improved from 126 to 108 milliseconds (ms) Patient 2 has double inlet left ventricle (DILV) with heart block requiring a dual chamber PM. He then developed dyssynchronous function that prevented him from second stage repair, so he was upgraded to a CRT pacing system at 7 months. 4 days later, his echocardiogram (echo) showed improved synchrony, and he was able to have his surgery at 12 months. QRS improved from 158 to 132 ms. Patient 3 has DILV and congenital heart block who had a single chamber PM placed. Following a surgical procedure, she suffered hemodynamic compromise and remained on ECMO. Her PM was converted to a dual chamber system and her EF normalized in 7 days; however, 5 weeks later she had severely diminished EF. After multiple attempts to wean inotropes, she was upgraded to a CRT system at 17 months. Her EF significantly improved in 10 days, and she was off inotropes 21 days later. QRS improved from 180 to 130 ms. Patient 4 developed heart block after cardiac fibroma resection and had a dual chamber PM placed. Despite this, she remained on inotropes and was upgraded to a biV CRT system at 3 months. Her EF never normalized, but she weaned off inotropes after 64 days. QRS improved from 252 to 148 ms. Unfortunately, she suffered a fatal seizure at 2 years. Summary In this series of infants with severely depressed ventricular function, cardiac resynchronization improved ejection fraction and prevented the need for heart transplantation. Following CRT initiation, all patients were weaned off inotropic support and discharged without heart transplantation.
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