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A perforation procedure for pulmonary atresia with intact ventricular septum

Egyptian experience and adaptations

Perforationsverfahren bei Pulmonalatresie und intaktem Ventrikelseptum

Ägyptische Erfahrungen und Anpassungen

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Pulmonary atresia with intact ventricular septum (PA-IVS) is an uncommon disorder with significant morphological heterogeneity. The use of percutaneous radiofrequency (RF)-assisted perforation of the atretic valve and subsequent balloon dilation provides a relatively easy but expensive procedure that is expected to establish ante-grade flow through the pulmonary valve in most patients.


The aim of the study was to attempt a cost reduction by using catheters and wires readily available in our catheter laboratory.


A total of 50 patients presenting with PA-IVS to Cairo University Children’s Hospital (CUCH) were taken to the catheterization laboratory for radiofrequency perforation using the Baylis RFP-100 generator (Baylis Medical, Montréal, Canada) or the stiff end of a coronary wire. A hybrid approach was used in selected cases.


The overall success rate for valve perforation was 92% (46 cases), 80% of which had successful primary perforation (40 cases). Success correlated with both tricuspid valve (TV) annulus and pulmonary valve (PV) annulus Z-scores, with P values of 0.2 and 0.5, respectively.


The management of PA-IVS is complex. This is a disease that necessitates a dedicated team and working collaboration between the cardiologists and cardiac surgeons. Cost limitation is essential in developing countries and innovative ideas to reduce costs are essential, especially if comparable success can be expected.



Eine Pulmonalatresie bei intaktem ventrikulärem Septum (PA-IVS) ist eine ungewöhnliche Herzfehlbildung mit hoher morphologischer Heterogenität. Die von einer Ballondilatation gefolgte perkutane Hochfrequenzperforation der atretischen Pulmonalklappe stellt eine relativ einfache, aber teure Prozedur dar, von der erwartet wird, dass sie bei den meisten Patienten zur Etablierung eines antegraden pulmonalen Blutflusses führt.


Ziel der Studie war der Versuch einer Kosteneinsparung durch Verwendung von Kathetern und Drähten, die in unserem Haus bereits zur Verfügung stehen.


Insgesamt 50 Patienten, die in CUCH (Cairo University Children‘s Hospital) mit PA-IVS vorgestellt wurden, wurden der Hochfrequenzperforation unter Nutzung des Baylis RFP-100-Generators (Fa. Baylis Medical, Montreal, Kanada) oder einer Klappeneröffnung unter Nutzung des starren Endes eines Koronardrahtes zugeführt. In bestimmten Fällen wurde ein kombiniertes Vorgehen gewählt.


Die Klappeneröffnung war in 92 % (46 Fälle) der behandelten Kinder erfolgreich, bei 80 % von ihnen bereits beim ersten Eröffnungsversuch. Der Erfolg korrelierte sowohl mit den Trikuspidalklappen- als auch den Pulmonalklappenanulus-Z-Scores mit p-Werten von 0,2 bzw. 0,5.


Das Management bei PA-IVS ist komplex. Diese Fehlbildung erfordert ein engagiertes Team und eine Zusammenarbeit von Kardiologen und Herzchirurgen. Kostenbegrenzung ist in Entwicklungsländern von essenzieller Bedeutung, und innovative Ideen zur Kostensenkung sind wichtig, insbesondere, wenn damit vergleichbare Erfolge erwartet werden können.

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  1. Freedom RM (1989) Etiology and Incidence. In: Freedom RM (ed) Pulmonary atresia with intact ventricular septum. Futura, Mount Kisco, pp 1–8

    Google Scholar 

  2. Guleserian KJ, Armsby LB, Thiagarajan RR et al (2006) Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg 81:2250–2257

    Article  Google Scholar 

  3. Parsons JM, Rees MR, Gibbs JL (1991) Percutaneous laser valvotomy with balloon dilatation of the pulmonary valve as primary treatment for pulmonary atresia. Br Heart J 66:36–38

    Article  CAS  Google Scholar 

  4. Latson LA (1991) Nonsurgical treatment of a neonate with pulmonary atresia and intact ventricular septum by transcatheter puncture and balloon dilatation of the atretic valve membrane. Am J Cardiol 68:277–279

    Article  CAS  Google Scholar 

  5. Qureshi SA, Rosenthal E, Tynan M et al (1991) Transcatheter laser-assisted balloon pulmonary valve dilation in pulmonic valve atresia. Am J Cardiol 67:428–431

    Article  CAS  Google Scholar 

  6. Justo RN, Nykaneu DG, William WG et al (1997) Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn. Cathet Cardiovasc Diagn 40:408–413

    Article  CAS  Google Scholar 

  7. Camino M, Brugada J, Mortera C et al (2001) Valvulotomí pulmonar percutánea mediante radiofrecuencia en la atresia pulmonar con septo interventricular íntegro. Rev Esp Cardiol 54:243–246

    Article  CAS  Google Scholar 

  8. Hijazi ZM, Patel H, Cao QL et al (1998) Transcatheter retrograde radio-frequency perforation of the pulmonic valve in pulmonary atresia with intact ventricular septum, using a 2 French catheter. Cathet Cardiovasc Diagn 45:151–154

    Article  CAS  Google Scholar 

  9. Alwi M, Budi RR, Mood MC et al (2013) Pulmonary atresia with intact septum: the use of conquest pro coronary guidewire for perforation of atretic valve and subsequent interventions. Cardiol Young 23(2):197–202

    Article  Google Scholar 

  10. Benson LN, Nykanen D, Collison A (2002) Radiofrequency perforation in the treatment of congenital heart disease. Catheter Cardiovasc Interv 56:72–82

    Article  Google Scholar 

  11. Li S, Chen W, Zhang Y, Zhang H et al (2011) Hybrid therapy for pulmonary atresia with intact ventricular septum. Ann Thorac Surg 91(5):1467–1471

    Article  Google Scholar 

  12. Kothari SS, Sharma SK, Naik N (2004) Radiofrequency perforation for pulmonary atresia and intact ventricular septum. Indian Heart J 56:50–53

    PubMed  Google Scholar 

  13. Lopes A, Parames F, Martins JDF et al (2011) Percutaneous treatment of pulmonary atresia with intact ventricular septum: Pulmonary valve perforation using radiofrequency energy and arterial duct stenting. Rev Port Cardiol 30(01):83–93

    PubMed  Google Scholar 

  14. Daubeney PEF, Wang D, Delany DJ et al (2005) Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg 130(1071):e1–e9

    Google Scholar 

  15. Schmidt KG, Cloez JL, Silverman NH (1992) Changes of right ventricular size and function in neonates after valvotomy for pulmonary atresia or critical pulmonary stenosis and intact ventricular septum. J Am Coll Cardiol 19(5):1032–1037

    Article  CAS  Google Scholar 

  16. Lightfoot NE, Coles JG, Dasmahapatra HK et al (1989) Analysis of survival in patients with pulmonary atresia and intact ventricular septum treated surgically. Int J Cardiol 24(2):159–164

    Article  CAS  Google Scholar 

  17. Wang JK, Wu MH, Chang CI et al (1999) Outcomes of transcatheter valvotomy in patients with pulmonary atresia and intact ventricular septum. Am J Cardiol 84:1055–1060

    Article  CAS  Google Scholar 

  18. Lee ML, Tsao LY, Chiu HY et al (2009) Outcomes in neonates with pulmonary atresia and intact ventricular septum underwent pulmonary valvulotomy and valvuloplasty using a flexible 2‑french radiofrequency catheter. Yonsei Med J 50(2):245–251

    Article  Google Scholar 

  19. Alwi M, Geetha K, Bilkis AA et al (2000) Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt. J Am Coll Cardiol 35:468–476

    Article  CAS  Google Scholar 

  20. Chubb H, Pesonen E, Sivasubramanian S et al (2012) Long-term outcome following catheter valvotomy for pulmonary atresia with intact ventricular septum. J Am Coll Cardiol 59(16):1468–1476

    Article  Google Scholar 

  21. Agnoletti G, Piechaud JF, Bonhoeffer P et al (2003) Perforation of the atretic pulmonary valve. Long-term follow-up. J Am Coll Cardiol 41(8):1399–1403

    Article  Google Scholar 

  22. Gibbs JL, Blackburn ME, Uzun O et al (1997) Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years’ experience. Heart 77:225–228

    Article  CAS  Google Scholar 

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The authors would like to thank all members of the pediatric Cath Lab team at the CUCH, as they all shared in the management of patients. We would also like to extend our gratitude to the nurses that participated in preparing the cases and tolerated the long procedures.


This research received no specific grant from any funding agency or from commercial and not-for-profit sectors.

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Correspondence to W. A. Attia.

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S.A. El Saiedi, W.A. Attia, O.M. Abd El-Aziz, W.N. Lotfy, A.M. Abd El-Rahim, H. Hassanein and S. Qureshi declare that they have no competing interests.

The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation, and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the institutional committees in Cairo University Hospitals.

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El Saiedi, S.A., Attia, W.A., Abd El-Aziz, O.M. et al. A perforation procedure for pulmonary atresia with intact ventricular septum. Herz 43, 633–641 (2018).

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