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The diagnosis of cor triatriatum sinistrum in children: a continuing dilemma
A C Tahernia1, K W Ashcraft, P J Tutuska
1Department of Pediatric Cardiology, Stormont-Vail Health Care, Topeka, Kan, USA.
Insights
Cor triatriatum (CT), a rare congenital heart defect, can be challenging to diagnose in children. Echocardiography plays a crucial role in identifying CT, with surgical membrane excision offering excellent long-term outcomes.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Cor triatriatum (CT) is an uncommon congenital cardiac anomaly characterized by a variable degree of obstruction within the left atrium.
- Diagnosis can be challenging, particularly in asymptomatic or mildly symptomatic pediatric patients.
- Understanding the spectrum of clinical presentations and diagnostic modalities is essential for timely intervention.
Observation:
- The study reviewed three pediatric cases of Cor triatriatum, including two asymptomatic males and one female with exertional dyspnea.
- Clinical, radiographic, and electrocardiographic findings were often nonspecific, highlighting diagnostic challenges.
- Echocardiography proved pivotal in accurately diagnosing CT and associated intracardiac anomalies, such as atrial septal defects and patent foramen ovale.
Findings:
- Two male patients presented with classical CT; one had a history of atrial septal defect repair and pulmonary hypertension.
- The female patient exhibited CT with an accessory chamber communicating with the right atrium and a patent foramen ovale, indicating an atrial level shunt.
- Surgical excision of the intra-atrial membrane via a transseptal approach resulted in excellent outcomes with no recurrence during 2-4 years of follow-up.
Implications:
- This case series underscores the diagnostic difficulties of Cor triatriatum in children and emphasizes the indispensable role of echocardiography.
- Prompt diagnosis and surgical correction of the fibromuscular membrane are crucial for preventing long-term complications.
- Successful surgical intervention offers excellent long-term prognosis for pediatric patients with Cor triatriatum.
Abstract:
Cor triatriatum (CT) is a rare congenital cardiac anomaly. The salient clinical, roentgenographic, electrocardiographic, echocardiographic, and hemodynamic findings are presented in two asymptomatic children and one with nonspecific dyspnea on exertion. Two male children had a classical form of cor triatriatum with normal physical and inconsequential roentgenographic and electrocardiographic findings. One of the male patients had surgery for a large atrial septal defect ostium secundum (ASD 2 degrees) and pulmonary hypertension in infancy. The female patient had CT with a communicating accessory chamber to right atrium and a rare patent foramen ovale. Her clinical findings confirmed an atrial level shunt. All patients had excision of the fibromuscular membrane from the right and left atrial transseptal approach with excellent results and with no recurrence during 2 to 4 year follow-up. We report the dilemma encountered in the clinical diagnosis of CT in children and the pivotal role played by echocardiography in the diagnosis of this anomaly.