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Biventricular repair for pulmonary atresia with intact ventricular septum associated with sinusoidal communications
Motoyuki Hisagi1, Yasuyuki Suzuki, Shisei Nakayama
1Department of Cardiovascular Surgery, Tokyo Metropolitan Hachioji Children's Hospital, 4-33-13 Daimachi, Hachioji, Tokyo 193-0931, Japan.
Insights
Pulmonary atresia with intact ventricular septum (PA-IVS) can be challenging due to severe right ventricular hypoplasia. This case demonstrates successful biventricular repair by ligating fistulas, showing it
Area of Science:
- Congenital heart disease
- Pediatric cardiology
- Cardiac surgery
Background:
- Pulmonary atresia with intact ventricular septum (PA-IVS) presents complex surgical challenges.
- Treatment decisions are often guided by the degree of right ventricular hypoplasia and coronary artery anomalies.
- Biventricular repair is typically considered unsuitable for severe right ventricular hypoplasia or sinusoidal communications.
Observation:
- A 17-day-old infant presented with PA-IVS, severe right ventricular hypoplasia, and large sinusoidal communications.
- The patient underwent surgical intervention including biventricular repair.
- Coronary artery and right ventricular fistulas were ligated, alongside right ventricular outflow tract reconstruction.
Findings:
- Successful biventricular repair was achieved in a patient with severe right ventricular hypoplasia and sinusoidal communications.
- Ligation of fistulas was crucial for decompressing the right ventricle.
- The patient experienced a positive outcome with good health post-surgery.
Implications:
- Sinusoidal communications do not necessarily preclude biventricular repair in PA-IVS.
- Effective management of right ventricular fistulas can enable successful biventricular repair without inducing ventricular dysfunction.
- This case expands the criteria for potential biventricular repair in complex PA-IVS cases.
Abstract:
Pulmonary atresia with intact ventricular septum (PA-IVS) is very difficult to treat due to the variety of right ventricular hypoplasia and coronary artery anomalies. Biventricular repair is viewed as possible in patients with mild right ventricular hypoplasia but not in those with severe right ventricular hypoplasia or with sinusoidal communication. We report a case of a 17-day-old boy with PA-IVS and severe right ventricular hypoplasia and large sinusoidal communications. We successfully conducted biventricular repair, ligated coronary artery and right ventricular fistulas, and implemented right ventricular outflow tract reconstruction. He now enjoys good health and attends elementary school. Sinusoidal communications are not always a limiting factor in biventricular repair for PA-IVS if the right ventricle can be decompressed without inviting ventricular dysfunction by ligating fistulas.