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[Left cor triatriatum. Clinical and echocardiographic polymorphism]
M A Nunes1, A Macedo, M P Magalhães
1Serviços de Cardiologia Pediátrica e Cirurgia Cardio-Torácica, Hospital de Santa Marta, Lisboa.
Insights
Cor triatriatum, a rare congenital heart defect, presents challenges in diagnosis and treatment. Early detection through echocardiography is crucial for improving surgical outcomes in affected children.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Cor triatriatum is a rare congenital heart anomaly characterized by a membrane dividing the left atrium.
- It can occur in isolation or with other cardiac defects, complicating diagnosis and management.
- Early diagnosis and intervention are critical for improving patient outcomes.
Purpose of the Study:
- To describe four pediatric cases of cor triatriatum.
- To highlight the diagnostic utility of echocardiography.
- To discuss the impact of associated anomalies and early diagnosis on surgical outcomes.
Main Methods:
- Case series review of four pediatric patients with cor triatriatum.
- Detailed clinical and investigational data analysis.
- Emphasis on echocardiographic findings.
Main Results:
- Four cases of cor triatriatum in infants aged 4 days to 12 months were analyzed.
- Presentations included isolated disease and associations with ventricular septal defect, anomalous pulmonary venous connection, and persistent left superior vena cava.
- One patient underwent successful surgical correction; two died pre-operatively, and one awaits surgery.
Conclusions:
- Isolated cor triatriatum can mimic primary lung disease, delaying diagnosis.
- Associated cardiac anomalies can lead to earlier diagnosis due to prompt referral.
- Surgical success and prognosis are significantly influenced by the presence of associated anomalies and the timeliness of diagnosis.
Abstract:
Four cases of cor triatriatum in children aged from 4 days to 12 months are described. In one case isolated classical form of the disease occurred, and in the other it was associated respectively to ventricular septal defect, anomalous pulmonary venous connexion and persistence of left superior venae cava. One child had successful surgical correction, two died before surgery could be attempted and one waits operation. Relevant clinical and investigational data are described and the role of echocardiography as an excellent diagnostic tool is stressed. We conclude that isolated form of cor triatriatum can simulate primary lung disease and when associated to other cardiac anomalies it has an earlier diagnosis due to early referral. Surgical results and prognosis depend not only on associated anomalies but also on early diagnosis.