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Clinical implications of isolated double aortic arch and its complex with intracardiac anomalies
Insights
This study analyzed six pediatric patients with double aortic arch (DAA), finding that surgical division of the smaller left aortic arch improved outcomes for those with complex congenital heart disease. Early intervention is key for managing DAA complications.
Area of Science:
- Cardiology
- Pediatric Surgery
- Medical Genetics
Background:
- Double aortic arch (DAA) is a rare congenital vascular anomaly.
- DAA can present with various intracardiac anomalies, significantly impacting clinical presentation and management.
- Early diagnosis and surgical intervention are crucial for improving patient outcomes.
Purpose of the Study:
- To describe the clinical characteristics, management, and surgical outcomes of pediatric patients with double aortic arch (DAA).
- To investigate the association between DAA and intracardiac anomalies.
- To evaluate the efficacy of surgical division of the smaller left aortic arch in complex DAA cases.
Main Methods:
- Retrospective case series of six patients diagnosed with DAA between July 1, 1989, and June 30, 1996.
- Clinical data including age, sex, associated anomalies, presenting symptoms, diagnostic imaging (barium esophagogram, echocardiography), surgical procedures, and outcomes were reviewed.
- Patients were categorized into isolated DAA and complex DAA with intracardiac anomalies.
Main Results:
- Six patients (4 male, 2 female) aged 16 days to 6.5 years were identified.
- Two patients had isolated DAA, and four had complex DAA with intracardiac anomalies including Tetralogy of Fallot (TOF), tricuspid atresia, and ventricular septal defect (VSD).
- Four patients (two isolated, two complex) survived surgical division of the smaller left aortic arch; management varied based on complexity, with two complex cases managed conservatively.
Conclusions:
- Surgical division of the smaller left aortic arch is a viable treatment for symptomatic double aortic arch (DAA), even in complex cases.
- The presence of intracardiac anomalies significantly influences the clinical presentation and management strategy for DAA.
- Multidisciplinary management is essential for optimizing outcomes in pediatric patients with DAA and associated cardiac defects.
Abstract:
From July 1, 1989 to June 30, 1996, a total of six patients, four male and two female, with double aortic arch (DAA) were registered. They aged 16 days to 6.5 years. There were two patients with isolated DAA, and four complicated with intracardiac anomalies. All six patients presented respiratory distress and feeding problem early in life. However, cyanosis, owing to decreased pulmonary blood flow, predominated the clinical picture in each patient having DAA intricated with tetralogy of Fallot (TOF) and pulmonary stenosis, TOF with absent pulmonary valve syndrome, and type II-A tricuspid atresia. DiGeorge syndrome was documented in one patient with TOF and pulmonary stenosis. Expiratory wheezing was remarkable in the patient with TOF and absent pulmonary valve syndrome. Mild cyanosis and heart failure was noted in one patient with ventricular septal defect (VSD), and bicuspid pulmonary valve with mild valvular stenosis. Barium esophagogram showed indentations on esophagus in five patients. Echocardiography was yielding in three infants. Four patients, two isolated and two complex DAA, survived the surgical division of the smaller left aortic arch. One-stage and two-stage operation was performed in patients having DAA intricated with VSD and TOF, respectively. The other two patients with complex DAA remained in close observation owing to the tolerance of symptoms related to intracardiac anomalies.