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.

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