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Sinus node dysfunction as the first manifestation of left ventricular noncompaction with multiple cardiac
Baris Gungor1, Ahmet T Alper, Ahmet Celebi
1Department of Cardiology, Siyami Ersek Thoracic and Cardiovascular Surgery Center, Training and Research Hospital, Istanbul, Turkey.
Insights
Left ventricular noncompaction (LVNC), a rare cardiomyopathy, often presents late. This case highlights a 23-year-old female with LVNC, coexistent cardiac defects, and sinus node dysfunction presenting with dizziness.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Left ventricular noncompaction (LVNC) is a rare, genetically diverse cardiomyopathy.
- Symptoms like dyspnea and arrhythmias often lead to late diagnosis in adulthood.
- Symptomatic sinus node dysfunction is an uncommon presentation of LVNC.
Purpose of the Study:
- To report a rare case of adult-onset LVNC.
- To highlight unusual coexistent cardiac defects in an adult LVNC patient.
- To emphasize atypical initial presentation of LVNC.
Main Methods:
- Case report of a 23-year-old female patient.
- Review of clinical presentation, echocardiography, and cardiac history.
- Analysis of coexistent cardiac anomalies and sinus node function.
Main Results:
- The patient presented with dizziness due to persistent sinus node dysfunction.
- Diagnosed with LVNC, patent ductus arteriosus, and bicuspid aortic valve.
- Demonstrated a rare combination of isolated LVNC with congenital heart defects in adulthood.
Conclusions:
- LVNC can present atypically in adulthood with sinus node dysfunction and congenital defects.
- Early diagnosis and management are crucial for patients with LVNC.
- This case expands the understanding of LVNC's varied clinical manifestations.
Abstract:
Left ventricular noncompaction (LVNC) is a genetically heterogenous form of cardiomyopathy which may remain undiagnosed till adulthood due to the late presentation of typical symptoms such as dyspnea, congestion, ventricular arrhythmias and thromboembolism. Symptomatic bradycardia secondary to persistent sinus node dysfunction is very rare. Coexistent cardiac defects are common in children however in adults the disease is usually in isolated form. Here, we present a case of twenty-three year-old female LVNC patient with patent ductus arteriosus, bicuspid aortic valve and persistent sinus node dysfunction who presented with dizziness as the first manifestation of the disease.
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