Ebstein's anomaly with coarctation of the aorta. An unusual association.
Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solitus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a DeVega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.
Ebaid
M
M
Instituto do Coração do Hospital das Clínicas, FMUSP, Brazil.
Azeka
E
E
Ikari
N M
NM
Sosa
E A
EA
Marcial
M B
MB
Atik
E
E
D000293:Adolescent; D001017:Aortic Coarctation / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000150:complications* / Q000175:diagnosis; D004562:Electrocardiography; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D014261:Tricuspid Valve / Q000601:surgery