Management of Pulmonary Atresia With Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac malformation characterised by complete obstruction of the right ventricular outflow tract and absence of a ventricular septal defect. Spectrum of disease extends from atresia of the pulmonary valve with variable right ventricular hypoplasia to more complex forms with ventriculo-coronary arterial communications. Management aims to establish reliable antegrade pulmonary blood flow, promote growth of the right ventricular inflow and outflow tracts, and achieve biventricular circulation whenever feasible. Initial stabilisation often relies on maintenance of ductal patency by prostaglandin infusion, followed by definitive therapy which may be percutaneous, surgical or a hybrid of both approaches. Catheter-based interventions such as perforation of the atretic valve and balloon valvuloplasty have been increasingly used in neonates, offering less invasive relief of right ventricular outflow obstruction. Surgical techniques range from one-and-a-half ventricular repair and modified right ventricular overhauling procedures to systematic palliation with Blalock-Taussig shunts and eventual Fontan circulation in cases unsuitable for two-ventricle repair. Decision-making is guided by detailed echocardiographic assessment, tricuspid valve z-scores, right ventricular morphology and the presence or absence of coronary connections. Long-term follow-up focuses on functional capacity, arrhythmic risk and right ventricular growth. Global registries and multicentre studies underscore variation in practice and highlight the importance of individualised, multidisciplinary strategies to optimise outcomes in this highly heterogeneous condition.

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Management of Pulmonary Atresia With Intact Ventricular Septum publication trend

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

Technical terms

Pulmonary atresia with intact ventricular septum (PAIVS): Complete absence of the pulmonary valve orifice in the presence of an intact interventricular septum.

Biventricular repair: Surgical or interventional strategy aimed at preserving or restoring function of both ventricles in series with the pulmonary and systemic circulations.

Tricuspid valve z-score: Standardised measurement of tricuspid annular dimension adjusted for body size, used to assess right heart development.

Right ventricular outflow tract (RVOT): The portion of the right ventricle leading to the pulmonary artery, critical in interventions to relieve obstruction.

Ventriculo-coronary arterial communication (VCAC): Abnormal channels linking the right ventricular cavity and the coronary arterial circulation, affecting management choices.

References

1. One and a half ventricular repair as an alternative for hypoplastic right ventricle. Brazilian Journal of Cardiovascular Surgery (2010).
2. Echocardiographic Parameters for Risk Prediction in Borderline Right Ventricle: Review with Special Emphasis on Pulmonary Atresia with Intact Ventricular Septum and Critical Pulmonary Stenosis. Journal of Clinical Medicine (2023).
3. Is there a transcatheter solution for a sick neonate with hypoplastic right heart syndrome?: Pulmonary valve perforation in a neonate with hypoplastic right ventricle with pulmonary atresia, restrictive VSD—a case report. The Egyptian Heart Journal (2020).
4. Percutaneous Balloon Pulmonary Valvuloplasty of Critical Pulmonary Stenosis and severe pulmonary stenosis in Neonates and Early Infancy: A Challenge in the Cyanotic. Journal of Cardiovascular and Thoracic Research (2021).
5. Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum. Congenital Heart Disease (2021).
6. Role of Surgery on Growth of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum: Mid-Term Results of Modified Right-Ventricular Overhauling Procedure. Congenital Heart Disease (2023).
7. Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review. Pediatric Cardiology (2024).