Biventricular pacing in an infant with noncompaction of the ventricular myocardium, congenital AV block, and prolonged QT interval.
We describe the case of a newborn with isolated noncompaction of the ventricular myocardium, congenital atrioventricular block, and prolonged QT, who developed a dilated cardiomyopathy after 4 months of right ventricular permanent pacing. The implanted system was upgraded to biventricular pacing. Resynchronization therapy was associated with normalized septal motion and shortening of the interventricular delay and, within 2 months after initiation, resulted in markedly improved left ventricular ejection fraction and reduced ventricular volume.
Drago
Fabrizio
F
Pediatric Cardiology Department, Bambino Gesù Pediatric Hospital, Piazza Sant' Onofrio, 4 00165, Rome, Italy. drago@opbg.net
Stefano Silvetti
Massimo
M
Annichiarico
Marco
M
Michielon
Guido
G
Brancaccio
Gianluca
G
Zanoni
Stefano
S
Valsecchi
Sergio
S
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000628:therapy; D054537:Atrioventricular Block / Q000151:congenital / Q000175:diagnosis / Q000628:therapy*; D002304:Cardiac Pacing, Artificial / Q000379:methods*; D015150:Echocardiography, Doppler / Q000379:methods; D004562:Electrocardiography; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000175:diagnosis* / Q000628:therapy*; D008133:Long QT Syndrome / Q000151:congenital / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D010138:Pacemaker, Artificial; D018570:Risk Assessment; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D020257:Ventricular Remodeling / Q000502:physiology