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[Outcome of surgery for interrupted aortic arch combined cardiac anomaly]
Qi-bin Yu1, Xiang-dong Shen, Shou-jun Li
1Pediatric Center, Fuwai Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100037, China.
Insights
Surgical repair of interrupted aortic arch (IAA) with cardiac anomalies is feasible. One-stage median sternotomy offers a viable approach for most patients, demonstrating good long-term outcomes.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Congenital Heart Defects
Context:
- Interrupted aortic arch (IAA) is a rare congenital heart defect requiring complex surgical intervention.
- Associated intracardiac anomalies and patent ductus arteriosus (PDA) are common in IAA patients.
- Surgical strategies vary based on the presence and type of associated anomalies.
Purpose:
- To review the surgical experience and outcomes for patients with interrupted aortic arch and associated cardiac anomalies.
- To evaluate the effectiveness of different surgical techniques, including one-stage repair via median sternotomy.
- To assess the safety and efficacy of intraoperative management strategies like selective cerebral perfusion and hypothermia.
Summary:
- A retrospective review of 36 patients (35 children, 1 adult) with IAA and associated anomalies treated between 1997 and 2008.
- 33 patients had PDA and intracardiac abnormalities; 3 had isolated IAA or PDA.
- Surgical techniques included conduit connection, direct anastomosis, and patch augmentation. One-stage median sternotomy was performed in 31 patients, utilizing selective cerebral perfusion or hypothermic circulatory arrest.
- Hospital mortality was 5 (13.9%), with causes including pulmonary infection and pulmonary hypertension crisis.
- Thirty-one survivors were followed for 3 months to 5 years with no late deaths or reoperations.
Impact:
- One-stage repair through median sternotomy, employing selective cerebral perfusion or deep hypothermia with low flow, is a successful approach for the majority of IAA cases with associated anomalies.
- The study highlights the feasibility and positive long-term results of this surgical strategy.
- This experience provides valuable insights for managing complex congenital aortic arch abnormalities.
Objective:
To retrospectively review the experience in repair of interrupted aortic arch (IAA) and associated cardiac anomaly.
Methods:
From January 1997 to January 2008, 36 patients with interrupted aortic arch and associated cardiac anomaly underwent surgical treatment. There were 22 male and 14 female. Mean age of the 35 children patients was 2.8 years, with a range from 2 months to 7 years. There was a 31 years old adult patient. Types of interrupted aortic arch include 30 cases of type A and 6 cases of type B. In all 36 patients, 33 cases had patent ductus arteriosus (PDA) and intracardiac abnormality, including 28 cases of simple anomaly as ventricular septal defect and 5 cases of complex anomaly, two cases were single IAA arch without PDA and other cardiac defect, one case had no intracardiac anomaly but PDA. For 33 patients with PDA and intracardiac anomaly, median sternotomy was used to simultaneously repair interrupted aortic arch and intracardiac defect in 31 cases, left thoracotomy and median sternotomy were applied to repair IAA and intracardiac anomaly respectively in one case, one patient had palliative repair. For three patients without intracardiac anomaly, left thoracotomy was applied in two cases, median sternotomy and abdominotomy were used in one adult patient. Techniques of operation for interrupted aortic arch include 16 cases of conduit connection, 9 cases of direct anastomosis, 9 cases of direct anastomosis with patch augmentation, 1 case of subclavian flap aortoplasty. In all 31 cases of one-stage operation through median sternotomy, selective cerebral perfusion was used in 17 patients, deep hypothermia and low flow were applied in 8 cases, deep hypothermia circulatory arrest was performed in 6 patients.
Results:
There were 5 hospital deaths. Three cases died of pulmonary infection, 1 case died of of pulmonary hypertension crisis, and another case died of postoperative low cardiac output, which was misdiagnosed before operation. Seven cases had other main postoperative complications. Thirty-one survivors were followed up from 3 months to 5 years, there was no late death and reoperation.
Conclusion:
One-stage repair through median sternotomy using selective cerebral perfusion or deep hypothermia and low flow can be applied to most of the cases with associated cardiac anomaly.
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