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Pulmonary atresia with intact ventricular septum — Clinical Guidelines

Overview

Pulmonary atresia with intact ventricular septum (PA-IVS) is a congenital heart defect characterized by the absence of pulmonary valve and blood flow to the lungs, necessitating interventions to establish adequate pulmonary circulation and right ventricular decompression. 1 3

Diagnosis

Echocardiography is essential for initial diagnosis, assessing anatomy, and guiding intervention planning. 1 3 10

Fetal echocardiography can identify coronary arterial abnormalities prenatally. 10

Cardiac MRI and 4D flow MRI may be used to evaluate right heart function and diastolic energy loss. 2 6

Tissue Doppler echocardiography helps assess right and left ventricular function post-repair. 9

Management

Initial Intervention:

- Catheter-based interventions, including balloon valvuloplasty and creation of a systemic-pulmonary shunt, are increasingly favored in neonates. 1 4 7 - Surgical approaches, such as transventricular pulmonary valvotomy and central aortopulmonary shunt, remain viable options. 15

Right Ventricular Decompression: Early decompression is crucial to promote right heart growth and development. 13

Management of Coronary Anomalies: Careful assessment and management of RV-dependent coronary circulation, including fistulas and stenoses, to prevent ischemia. 14 16

Special Populations

Pediatrics: Early intervention strategies significantly impact long-term outcomes, with catheter-based techniques showing promise in reducing morbidity. 1 3 7

Adults: Monitoring for diastolic energy loss and right heart growth is critical, as these factors correlate with major cardiac events. 2 6

Key Recommendations

Early Right Ventricular Decompression is essential for promoting right heart growth and improving long-term outcomes in neonates with PA-IVS. (Evidence: Strong 13)

Catheter-Based Interventions should be considered as a first-line approach for initial management in neonates due to favorable short-term outcomes and reduced morbidity compared to surgery. (Evidence: Moderate 1 4 7)

Comprehensive Assessment of Coronary Anomalies is necessary before and after interventions to prevent ischemia and ensure optimal cardiac function. (Evidence: Moderate 14 16)

References

1 Manhem S, Odermarsky M, Wåhlander H, Ekman-Joelsson BM. Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review. Pediatric cardiology 2025. link 2 Shiina Y, Inai K, Itatani K, Shimada E, Nagao M. Increased Diastolic Energy Loss Associated with Cardiac Events in Adults with Pulmonary Atresia Suffering from Intact Ventricular Septum. Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine 2025. link 3 Zou M, Dong S, Liu S, Du C, Sun Y, Dong J et al.. Influencing factors of prognosis in children with pulmonary atresia with intact ventricle septum after transthoracic balloon dilation of pulmonary valve and construction of a nomograph prediction model. Biotechnology & genetic engineering reviews 2024. link 4 Cheung EW, Mastropietro CW, Flores S, Amula V, Radman M, Kwiatkowski D et al.. Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study. The Annals of thoracic surgery 2023. link 5 Prakoso R, Putra BE, Kurniawati Y, Siagian SN, Lelya O, Sembiring AA et al.. A retrograde approach for transcatheter valvotomy procedure in infants with pulmonary atresia intact ventricular septum (PA-IVS): retrograde versus antegrade approach. Cardiology in the young 2023. link 6 Sugitani Y, Muneuchi J, Watanabe M, Matsuoka R, Doi H, Ezaki H et al.. Late Adverse Events in Patients With Pulmonary Atresia With Intact Ventricular Septum After Valvuloplasty. The Annals of thoracic surgery 2022. link 7 Lawley C, Hockey K, Yeo LL, Liava'a M, Roberts P. Increasing Use of Neonatal Catheter Intervention for Pulmonary Atresia With Intact Ventricular Septum: Management Trends From a Single Centre. Heart, lung & circulation 2022. link 8 Hwang MS, Taylor GP, Freedom RM. Decreased left ventricular coronary artery density in pulmonary atresia and intact ventricular septum. Cardiology 2008. link 9 Mi YP, Cheung YF. Assessment of right and left ventricular function by tissue Doppler echocardiography in patients after biventricular repair of pulmonary atresia with intact ventricular septum. International journal of cardiology 2006. link 10 Sandor GG, Cook AC, Sharland GK, Ho SY, Potts JE, Anderson RH. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life. Cardiology in the young 2002. link 11 Akagi T, Benson LN, Williams WG, Trusler GA, Freedom RM. Ventriculo-coronary arterial connections in pulmonary atresia with intact ventricular septum, and their influences on ventricular performance and clinical course. The American journal of cardiology 1993. link90356-h) 12 Gentles TL, Colan SD, Giglia TM, Mandell VS, Mayer JE, Sanders SP. Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies. Circulation 1993. link 13 Giglia TM, Jenkins KJ, Matitiau A, Mandell VS, Sanders SP, Mayer JE et al.. Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum. Circulation 1993. link 14 Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992. link 15 Shaddy RE, Sturtevant JE, Judd VE, McGough EC. Right ventricular growth after transventricular pulmonary valvotomy and central aortopulmonary shunt for pulmonary atresia and intact ventricular septum. Circulation 1990. link 16 Hausdorf G, Grävinghoff L, Keck EW. Effects of persisting myocardial sinusoids on left ventricular performance in pulmonary atresia with intact ventricular septum. European heart journal 1987. link

Pulmonary atresia with intact ventricular septum