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Congenital complete atrioventricular block and dilated cardiomyopathy: new light for an old disease
Mariana Paiva1, Vania Ribeiro1, Raquel Garcia1
1Cardiology Department, Centro Hospitalar São João, 4200-319 Porto, Portugal.
Insights
A patient with congenital heart block developed severe heart muscle dysfunction after pacemaker implantation. Cardiac resynchronization therapy improved his condition, demonstrating its effectiveness in complex cardiac cases.
Area of Science:
- Cardiology
- Electrophysiology
- Heart Failure
Background:
- Complete congenital atrioventricular block (CAVB) can necessitate long-term pacing.
- Pacemaker implantation, particularly VVI-R pacing, can be associated with the development of cardiomyopathy.
- Biventricular dysfunction presents a significant clinical challenge, often refractory to medical management.
Purpose of the Study:
- To report a case of VVI-R pacemaker-induced cardiomyopathy in a patient with CAVB.
- To evaluate the efficacy of cardiac resynchronization therapy (CRT) in managing severe biventricular dysfunction secondary to pacing in CAVB.
- To highlight the potential benefits of CRT in improving clinical and electrocardiographic parameters in such complex cases.
Main Methods:
- Case presentation of a patient with CAVB and subsequent dilated cardiomyopathy.
- Review of medical history including VVI-R pacemaker implantation and medical therapy.
- Application of cardiac resynchronization therapy (CRT) and assessment of outcomes.
Main Results:
- The patient developed severe biventricular dysfunction ten years post-pacemaker implantation.
- Optimal medical therapy was insufficient to alleviate symptoms.
- Following CRT, significant clinical and electrocardiographic improvement was observed within one month.
Conclusions:
- Cardiac resynchronization therapy can be a valuable treatment option for pacing-induced cardiomyopathy in patients with congenital heart block.
- CRT may reverse or improve severe biventricular dysfunction in selected cases.
- This case underscores the importance of considering advanced pacing strategies in patients with complex congenital cardiac conditions.
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.
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