Abstract
We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.
Highlights
We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation
In NYHA class II, TTE showed a slight improvement in left ventricular function, while maintaining right chamber dilation and pulmonary hypertension (PASP 67 mmHg)
This case illustrates the complexity of management of patients with congenital CAVB
Summary
Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy: New Light for an Old Disease. We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. A 7-year-old boy was sent, in 1997, to a pediatric cardiology outpatient clinic, due to a slow heart rate and exercise intolerance His EKG showed sinus rhythm, 44 bpm, complete atrioventricular block (CAVB), and left bundle branch block (Figure 1). At age 11, for worsening of exercise tolerance, a VVIR pacemaker was implanted (Figure 2) He was completely asymptomatic, and by 19 years old the TTE showed a severe LVSD, with slight increase of LV diameter and mild right ventricular systolic dysfunction; he started lisinopril and bisoprolol. In NYHA class II, TTE showed a slight improvement in left ventricular function, while maintaining right chamber dilation and pulmonary hypertension (PASP 67 mmHg)
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