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Giant right atrium in a child with dilated cardiomyopathy: A case report
Benzhen Wang1, Guangsong Shan1, Zhen Bing1
1Heart Center, Qingdao Women and Children's Hospital, Qingdao University, Qingdao, China.
Insights
A rare case of dilated cardiomyopathy (DCM) presented with a giant atrium in an infant. This finding expands the clinical spectrum of pediatric DCM and highlights the need for careful follow-up.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Genetics
- Congenital Heart Disease
Background:
- Dilated cardiomyopathy (DCM) is a significant cause of heart failure in children.
- Giant atrium as the initial presentation of DCM is exceptionally rare in pediatric cases.
- Early identification and management are crucial for improving outcomes in pediatric heart failure.
Observation:
- A male infant presented with a markedly enlarged right atrium as the primary clinical manifestation.
- Surgical reduction of the enlarged right atrium was performed due to clinical deterioration and risks of arrhythmia and thrombosis.
- Midterm follow-up revealed the development of DCM and recurrent right atrial enlargement.
Findings:
- The patient was ultimately diagnosed with familial dilated cardiomyopathy, supported by echocardiographic findings in the mother.
- This case represents a novel presentation of DCM, characterized by a giant atrium preceding or concurrent with cardiomyopathy.
- The progression of the condition underscores the complex and variable nature of inherited cardiomyopathies.
Implications:
- This case broadens the known clinical spectrum of dilated cardiomyopathy in children.
- It emphasizes the importance of thorough cardiac evaluation and long-term monitoring for infants with idiopathic right atrial dilatation.
- Highlights the potential genetic underpinnings and familial transmission patterns in pediatric DCM.
Abstract:
Dilated cardiomyopathy (DCM) is one of the leading causes of heart failure in children with diverse clinical characteristics. To date, DCM with a giant atrium as the first manifestation is rare and has not been reported in previous literature. We report a case of a male infant born with a significantly enlarged right atrium. Due to worsened clinical symptoms and the risk of arrhythmias and thrombosis, we performed the surgical reduction of the right atrium. Unfortunately, DCM and a progressive re-enlargement of the right atrium appeared during midterm follow-up. The mother's echocardiogram also suggested DCM, and the patient was eventually considered for a diagnosis of familial DCM. This case may expand the clinical spectrum of DCM and reminds us of the importance of good follow-up of children with idiopathic dilatation of the right atrium.
