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Congenital Non-Confluent Pulmonary Artery Sourced from Bilateral Arterial Ducts: A Rare Anomaly

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Abstract

This study aimed to identify the influence of the non-confluent pulmonary artery originating from the bilateral arterial ducts (AD) on the outcomes of the Fontan circulation. We retrospectively reviewed the records of nine patients with bilateral AD and a non-confluent pulmonary artery in a single ventricle at our institution between 1993 and 2023. Three patients showed maintained AD or underwent a systemic–pulmonary shunt for stenotic AD, followed by the Glenn procedure. Four patients underwent systemic–pulmonary shunt with angioplasty as the first palliation, followed by the Glenn procedure. Two patients underwent systemic–pulmonary shunt or AD stenting as the first palliation and systemic–pulmonary shunt with angioplasty as the second palliation. There were no cases of interstage mortality. Pulmonary arteries grew and achieved a good balance (pre-Fontan pulmonary artery index [PAI], 164 ± 27 mm2/m2; right/left PAI ratio, 1.06 ± 0.23). All patients underwent the Fontan procedure (median, 3.5 years; range, 2.3–6.4 years) and were followed up for 7.8 years (range, 0.1–16.4 years) after the procedure. One patient required hospitalization for heart failure at 1.8 years, and three patients required catheter intervention for pulmonary stenosis within 2 months after the Fontan procedure. Non-confluent pulmonary arteries originating from the bilateral AD do not preclude Fontan completion and good Fontan outcomes by restoring balance to pulmonary blood flow. Due to the limited sample size in this study, additional research is imperative to delve deeper into our findings and enhance understanding of the most effective surgical approach for this disease.

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Funding

No funds, grants, or other support was received.

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Authors and Affiliations

  1. Department of Cardiovascular Surgery, Osaka Women’s and Children’s Hospital, 840 Murodocho, Izumi, Osaka, 594-1101, Japan

    Koji Miwa, Shigemitsu Iwai, Tomomitsu Kanaya & Shota Kawai

Authors
  1. Koji Miwa
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  2. Shigemitsu Iwai
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  3. Tomomitsu Kanaya
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  4. Shota Kawai
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Contributions

Conceptualization: KM and SI. Methodology: SI. Review and editing: TK and SK.

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Correspondence to Koji Miwa.

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The authors have no competing interests to declare that are relevant to the content of this article.

Ethical Approval

The study was approved by the institutional review board of Osaka Women’s and Children’s Hospital (approval number: 1566; approval date: September 1, 2022) and was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

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The requirement for written informed consent was waived owing to the retrospective design of the study.

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Miwa, K., Iwai, S., Kanaya, T. et al. Congenital Non-Confluent Pulmonary Artery Sourced from Bilateral Arterial Ducts: A Rare Anomaly. Pediatr Cardiol 44, 1438–1446 (2023). https://doi.org/10.1007/s00246-023-03230-w

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