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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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Abstract

Background

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.

Objectives

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

Methods

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.

Results

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.

Conclusion

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.

Zusammenfassung

Hintergrund

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

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

Methoden

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.

Ergebnisse

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.

Schlussfolgerung

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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Acknowledgements

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.

Funding

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

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Authors and Affiliations

  1. Department of Pediatrics, Pediatric Cardiology division, Cairo University, Ali Ibrahim St., Al-Sayeda Zainab, Cairo, Egypt

    S. A. El Saiedi, W. A. Attia, O. M. Abd El-Aziz & W. N. Lotfy

  2. Department of Anesthesia, Cairo University, Cairo, Egypt

    A. M. Abd El-Rahim

  3. Department of Cardiothoracic Surgery, Cairo University, Cairo, Egypt

    H. Hassanein

  4. Pediatric Cardiology Department, Evelina London Children Hospital, London, UK

    S. Qureshi

Authors
  1. S. A. El Saiedi
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  2. W. A. Attia
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  3. O. M. Abd El-Aziz
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  4. W. N. Lotfy
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  5. A. M. Abd El-Rahim
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  6. H. Hassanein
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  7. S. Qureshi
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Corresponding author

Correspondence to W. A. Attia.

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Conflict of interest

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). https://doi.org/10.1007/s00059-017-4606-x

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  • DOI: https://doi.org/10.1007/s00059-017-4606-x

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