From the Guidelines
Pulmonary atresia with intact ventricular septum is repaired through a staged surgical approach tailored to the patient's specific anatomy, with initial management involving establishment of adequate pulmonary blood flow through a prosthetic shunt, followed by right ventricular outflow tract reconstruction or a single-ventricle pathway, as determined by right ventricular size and other factors 1. The goal of treatment is to establish a stable and efficient circulation, while minimizing the risk of complications and promoting optimal quality of life.
- Key considerations in the repair of pulmonary atresia with intact ventricular septum include:
- Establishment of adequate pulmonary blood flow, often through a modified Blalock-Taussig shunt or PDA stent
- Right ventricular outflow tract reconstruction with a transannular patch or pulmonary valve replacement, for patients with adequate right ventricular size
- Closure of any atrial communications
- Single-ventricle pathway, involving a Glenn procedure and subsequent Fontan procedure, for patients with severely hypoplastic right ventricles
- The choice of repair strategy depends on various factors, including right ventricular size, tricuspid valve dimensions, and coronary circulation, as some patients have right ventricle-dependent coronary circulation that affects surgical planning 1.
- Prostaglandin E1 infusion is crucial in the immediate neonatal period to maintain ductal patency until surgical intervention, typically starting at a dose of 0.05-0.1 mcg/kg/min.
- Ongoing cardiac follow-up and monitoring are essential for patients with pulmonary atresia with intact ventricular septum, to detect potential complications such as atrial arrhythmias, restrictive RV physiology, and ventricular fibrosis 1.
From the Research
Pulmonary Atresia with Intact Ventricular Septum Repair
There are no research papers to assist in answering this question directly, as the provided studies focus on atrial septal defect (ASD) closure. However, one study mentions pulmonary atresia with intact ventricular septum in the context of a patient's medical history:
- A study published in 2017 2 mentions that two patients had a history of pulmonary atresia and intact ventricular septum, for which they underwent percutaneous radiofrequency perforation and balloon angioplasty.
Atrial Septal Defect Closure
The provided studies discuss various aspects of ASD closure, including:
- Transcatheter closure of ASD using the Cocoon septal occluder 3
- Long-term complications after transcatheter ASD closure 4
- Comparison of surgical and Amplatzer device closure of ASD 5
- Surgical closure of ASD 6
- A technique for ASD closure using a venous-arterial circuit from the right internal jugular vein to the femoral artery 2