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Improved results with selective management in pulmonary atresia with intact ventricular septum
M Jahangiri1, D Zurakowski, D Bichell
1Department of Cardiac Surgery, Children's Hospital, Boston, MA 02115, USA.
Insights
Selective surgical strategies for pulmonary atresia with intact ventricular septum can improve outcomes. Careful patient stratification, considering right ventricle-dependent coronary circulation, is key to achieving excellent survival rates.
Area of Science:
- Congenital heart disease
- Pediatric cardiology
- Cardiac surgery
Background:
- Pulmonary atresia with intact ventricular septum (PA/IVS) historically has poor late outcomes.
- A tailored surgical approach is crucial for improving survival in these complex cases.
Purpose of the Study:
- To evaluate the late outcomes of a selective surgical strategy for PA/IVS.
- To determine the impact of right ventricle-dependent coronary circulation (RVDC) on surgical decision-making and outcomes.
Main Methods:
- Retrospective chart review of 47 patients undergoing surgery between 1991 and 1998.
- Stratification based on RVDC and right ventricular growth.
- Surgical interventions included systemic-pulmonary artery shunts, bidirectional Glenn shunts, Fontan procedures, and biventricular repairs.
Main Results:
- Patients with RVDC (34%) had smaller tricuspid valves and underwent initial shunting with one death; 9 of 14 proceeded to Fontan procedure with no deaths.
- Patients without RVDC (66%) had varied initial procedures, with 10 achieving 2-ventricle repair, 6 a 1.5-ventricle repair, and 8 a Fontan procedure.
- Overall survival was excellent at 98% at 1, 5, and 7 years, with only one early death.
Conclusions:
- A well-stratified surgical approach can lead to excellent survival in PA/IVS.
- This success may be achieved through a combination of single-ventricle palliation and biventricular repair strategies.
- The presence of RVDC significantly influences the surgical pathway and long-term management.
Objective:
Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle.
Methods:
A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998.
Results:
Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years.
Conclusions:
If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.