Cardiac resynchronization therapy in a case with single ventricle and concommitant noncompaction cardiomyopathy.
Deniz Elçik1, Mustafa Fehmi Bireciklioğlu1, Ali Doğan1
1Department of Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
Summary
This case report details the first successful cardiac resynchronization therapy with a defibrillator (CRT-D) implantation in a patient with a single ventricle, myocardial noncompaction, and complete atrioventricular block. The procedure effectively reduced QRS duration and improved heart failure symptoms.
Area of Science:
- Cardiology
- Electrophysiology
- Congenital Heart Disease
Background:
- Complete atrioventricular (AV) block and single ventricle physiology present complex management challenges.
- Myocardial noncompaction is a rare congenital cardiomyopathy.
- Cardiac resynchronization therapy with a defibrillator (CRT-D) is indicated for heart failure with reduced ejection fraction and ventricular dyssynchrony.
Observation:
- A 32-year-old male presented with syncope, complete AV block, and a wide QRS complex (218 ms) with a heart rate of 40 bpm.
- Echocardiography revealed a single ventricle with AV valves opening into it and evidence of myocardial noncompaction.
- The patient had New York Heart Association class III heart failure.
Findings:
- CRT-D implantation was successfully performed.
- Post-implantation, the QRS duration decreased from 218 ms to 183 ms.
- This represents the first reported case of CRT-D in a patient with this specific combination of conditions.
Implications:
- CRT-D can be a viable therapeutic option for selected patients with complex congenital heart disease and heart failure.
- This case expands the understanding of CRT-D application in rare cardiac conditions.
- Further research is warranted to evaluate long-term outcomes of CRT-D in patients with single ventricle and myocardial noncompaction.
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