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Long-Term Outcomes After an Individualized Strategy in Patients with Pulmonary Atresia and Intact Ventricular Septum

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Abstract

This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were − 3.30 (− 15.15 to 1.83), − 0.70 (− 4.65 to 2.33), and − 1.51 (− 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.

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Acknowledgements

We would like to thank the Editage (www.editage.com) for English language editing.

Funding

This research received no grant from any funding agency in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, 1-8-1, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka, 806-8501, Japan

    Jun Muneuchi, Mamie Watanabe, Yuichiro Sugitani, Hirohito Doi, Takashi Furuta, Masaru Kobayashi & Hiroki Ezaki

  2. Department of Cardiovascular Surgery, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan

    Yoshie Ochiai

Authors
  1. Jun Muneuchi
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  2. Mamie Watanabe
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  3. Yuichiro Sugitani
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  4. Hirohito Doi
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  5. Takashi Furuta
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  6. Masaru Kobayashi
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  7. Hiroki Ezaki
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  8. Yoshie Ochiai
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Contributions

JM designed and conducted this study and described the first draft. YS, HD, TF, MK, and HE collected catheterization and echocardiographic data. YS also contributed to data analysis. MW and YO critically reviewed this manuscript.

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Correspondence to Jun Muneuchi.

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Conflicts of interest

The authors have no conflict of interest to declare.

Ethical Approval

This retrospectively single-institutional cohort study was approved by the Institutional Ethics Committee of Kyushu Hospital, Japan Community Healthcare Organization (approval number 695).

Consent to Participate

In all subjects, their parents or guardians provided informed consent for the procedures, but informed consent for this cohort study was waived owing to the retrospective nature.

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All authors approved this publication.

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Muneuchi, J., Watanabe, M., Sugitani, Y. et al. Long-Term Outcomes After an Individualized Strategy in Patients with Pulmonary Atresia and Intact Ventricular Septum. Pediatr Cardiol 43, 435–442 (2022). https://doi.org/10.1007/s00246-021-02740-9

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  • DOI: https://doi.org/10.1007/s00246-021-02740-9

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