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Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators

Part of: Functionally Univentricular Heart

Published online by Cambridge University Press:  22 March 2021

Kanta Kishi Open the ORCID record for Kanta Kishi [Opens in a new window] ,
Hiroshi Katayama ,
Shintaro Nemoto ,
Noriyasu Ozaki ,
Yutaka Odanaka ,
Atsuko Ashida ,
Hayato Konishi  and
Akira Ashida
Kanta Kishi*
Affiliation:
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan
Hiroshi Katayama
Affiliation:
Department of Pediatrics, Takatsuki Red Cross Hospital, Takatsuki, Osaka, Japan
Shintaro Nemoto
Affiliation:
Department of Pediatric Thoracic and Cardiovascular Surgery, Osaka Medical College, Takatsuki, Osaka, Japan
Noriyasu Ozaki
Affiliation:
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan
Yutaka Odanaka
Affiliation:
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan
Atsuko Ashida
Affiliation:
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan
Hayato Konishi
Affiliation:
Department of Pediatric Thoracic and Cardiovascular Surgery, Osaka Medical College, Takatsuki, Osaka, Japan
Akira Ashida
Affiliation:
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan
*
Author for correspondence: K. Kishi, MD, PhD, Department of Pediatrics, Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka569-8686, Japan. Tel: +81 72 683 1221; Fax: +81 72 684 5798. E-mail: [email protected]
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Abstract

Background:

Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery.

Methods:

We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital.

Results:

We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure.

Conclusion:

Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

Keywords

CHDscoronary arterial fistulaepulmonary atresia with intact ventricular septumsingle-ventricle physiologypulmonary vasodilatorsheart failure
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 11 , November 2021 , pp. 1823 - 1828
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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