Surgical Management of Congenital Heart Defects with Pulmonary Atresia

Pulmonary atresia encompasses a spectrum of rare congenital heart defects characterised by complete obstruction of the right ventricular outflow tract. Anatomical variations range from intact native pulmonary arteries to complex networks of major aortopulmonary collateral arteries supplying the lungs. Surgical management aims to establish reliable pulmonary blood flow, relieve right ventricular pressure overload, and achieve complete intracardiac repair where feasible. Strategies include staged repair, involving initial palliation with systemic-to-pulmonary shunts or right ventricle–pulmonary artery conduits, followed by unifocalization of collateral vessels and ventricular septal defect closure. In selected centres, single-stage unifocalization and correction are performed in infancy to reduce cumulative operative risk. Preoperative imaging with echocardiography, computed tomography and magnetic resonance angiography is essential for anatomical delineation and surgical planning. Long-term outcomes rely on timely conduit replacement, management of residual gradients and regurgitation, and a multidisciplinary approach to minimise right ventricular dysfunction. Advances in homograft durability, three-dimensional surgical planning and tailored rehabilitation strategies have improved survival and functional capacity globally, yet intercentre variability in protocols underscores the need for continued innovation and shared best practice.

Research from Nature Portfolio

No recent Nature Portfolio content available.

Surgical Management of Congenital Heart Defects with Pulmonary Atresia publication trend

The graph below shows the total number of articles in surgical management of congenital heart defects with pulmonary atresia across all publications each year (not limited to Nature Index journals).

Technical terms

Pulmonary atresia: Complete absence of a functional pulmonary valve or outflow tract.

Ventricular septal defect (VSD): A hole in the septum separating the right and left ventricles.

Unifocalization: Surgical unification of multiple collateral arteries into a single pulmonary artery confluence.

Major aortopulmonary collateral arteries (MAPCAs): Aberrant vessels that supply pulmonary blood flow from the systemic circulation.

Right ventricle–pulmonary artery (RV-PA) conduit: Tube graft connecting the right ventricle to the pulmonary arteries to establish blood flow.

References

1. Pulmonary flow-study can predict in-hospital prognosis of unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries. Heliyon (2024).
2. Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia - usefulness of MR angiography in clinical routine. Journal of Cardiovascular Magnetic Resonance (2010).
3. Management of ventricular septal defect with pulmonary atresia and major aorto pulmonary collateral arteries: Challenges and controversies. Annals of Pediatric Cardiology (2010).
4. Complete Preoperative Evaluation of Pulmonary Atresia with Ventricular Septal Defect with Multi-Detector Computed Tomography. PLOS ONE (2016).
5. 3D Virtual Reality Imaging of Major Aortopulmonary Collateral Arteries: A Novel Diagnostic Modality. World Journal for Pediatric and Congenital Heart Surgery (2021).
6. Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of Pulmonary Atresia by Dual-Source Computed Tomography. Congenital Heart Disease (2023).
7. Staged correction of pulmonary atresia, ventricular septal defect, and collateral arteries. Journal of Cardiac Surgery (2022).
8. Homograft durability after correction of pulmonary atresia and ventricular septal defect with or without systemic pulmonary collateral arteries. JTCVS Open (2021).