ardiac resynchronization therapy (CRT) has become the standard of care for the treatment of heart failure in adults with decreased ventricular function and conduction delay who remain symptomatic despite optimal medical therapy. Although there are no prospective and randomized trial data, a retrospective series show that CRT is similarly effective for managing dyssynchronyassociated heart failure in children. [1][2][3] The heterogeneity of anatomical and functional substrates in which CRT shows efficacy calls for further studies defining the usefulness of CRT in children. 4 Children with systolic dysfunction secondary to cardiomyopathy have mechanical dyssynchrony, unrelated to electrical dyssynchrony, and are potential candidates for CRT. 5 Left ventricular non-compaction (LVNC) is a unique form of cardiomyopathy, characterized by numerous prominent left ventricular (LV) trabeculations and deep intertrabecular recesses. 6,7 In patients with LVNC, mechanical dyssynchrony between non-compacted and compacted myocardium contribute to global LV dysfunction. 8 Tissue Doppler velocities are significantly reduced in patients with LVNC, and help identify children with LVNC who are at risk of adverse clinical outcomes, including death, and need cardiac transplantation. 9 Successful CRT in adults with isolated LVNC has been reported recently, 10 however, the efficacy of CRT for pediatric patients with LVNC has not been reported. The use of CRT in children has gained a great deal of attention, especially in Japan where there is little chance of heart transplantation.Here we report the successful use of CRT in a 3-year-old girl with intractable heart failure caused by isolated LVNC with narrow QRS complex.
Case ReportThe patient was diagnosed with heart failure caused by isolated LVNC at 3 months of age. LVNC was diagnosed by echocardiographic criteria including: (1) presence of prominent or numerous LV trabeculations, predominantly in the distal portion (apex) of the left ventricle; (2) a 2-layered structure of the myocardium with an increased non-compacted to compacted ratio (>1.4); and (3) Cardiac resynchronization therapy (CRT) is a new method of treatment for refractory heart failure. However, for children, its indication, efficacy, and long-term prognosis remain unclear. This study describes the use of CRT for a 3-year-old girl with intractable heart failure caused by isolated left ventricular non-compaction (LVNC) with narrow QRS complex. Echocardiography showed diffuse hypokinetic left ventricular (LV) wall motion (ejection fraction =29.3%) with dyssynchrony between the apex, posterior and lateral walls, where numerous prominent trabeculations existed, and severe mitral regurgitation. Biventricular resynchronization using epicardial pacing leads was performed under general anesthesia. Pacing sites for optimal synchronization in the ventricular walls where chosen using tissue Doppler imaging, and AV delay was adjusted to achieve maximal systolic blood pressure and maximal cardiac output. Over a follow-up period of 2...
