{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T07:20:41Z","timestamp":1783581641327,"version":"3.55.0"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T00:00:00Z","timestamp":1783555200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T00:00:00Z","timestamp":1783555200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Deutsches Herzzentrum M\u00fcnchen"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Cardiol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Pulmonary Atresia with Intact Ventricular Septum (PA\/IVS) associated with multiple large, coronary artery fistula represents a rare and complex congenital heart defect. The presence of Right Ventricular Dependent Coronary Circulation (RVDCC) critically influences management and prognosis. We report the case of a newborn with PA\/IVS, extensive coronary fistulae involving both the right and left coronary arteries. A staged, stepwise approach was undertaken to exclude RVDCC, followed by transcatheter closure of the coronary fistulae and ballon perforation of the pulmonary valve. This sequential approach facilitated gradual right ventricular decompression and the establishment of antegrade pulmonary blood flow while minimizing the risk of coronary ischemia. Definitive biventricular circulation was subsequently achieved with placement of a right ventricle-to-pulmonary artery (RV\u2013PA) conduit, resulting in right ventricular pressures decreasing to approximately onehalfsystemic levels.<\/jats:p>\n                  <jats:p>This case demonstrates the feasibility and effectiveness of a staged, multidisciplinary hybrid approach in carefully selected patients with PA\/IVS and complex coronary artery fistulae. It underscores the importance of comprehensive coronary assessment and individualized management in optimizing outcomes in this challenging congenital cardiac anomaly.<\/jats:p>","DOI":"10.1007\/s00246-026-04358-1","type":"journal-article","created":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T06:54:09Z","timestamp":1783580049000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Percutaneous Closure of Multiple Large Coronary Fistulae and Right Ventricular Outflow Tract Opening to Establish Biventricular Circulation in a Child with Pulmonary Atresia and Intact Ventricular Septum \u2013 A Case Report"],"prefix":"10.1007","author":[{"given":"Julia","family":"Danner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Ewert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katarzyna","family":"Gendera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,9]]},"reference":[{"issue":"5","key":"4358_CR1","doi-asserted-by":"publisher","first-page":"1516","DOI":"10.1161\/01.cir.86.5.1516","volume":"86","author":"TM Giglia","year":"1992","unstructured":"Giglia TM, Mandell VS, Connor AR, Mayer JE Jr., Lock JE (1992) Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. 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J Thorac Cardiovasc Surg 164(5):1277\u20131288. https:\/\/doi.org\/10.1016\/j.jtcvs.2021.11.104","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"2","key":"4358_CR5","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1007\/s00246-021-02740-9","volume":"43","author":"J Muneuchi","year":"2022","unstructured":"Muneuchi J, Watanabe M, Sugitani Y, Doi H, Furuta T, Kobayashi M, Ezaki H, Ochiai Y (2022) Long-term outcomes after an individualized strategy in patients with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 43(2):435\u2013442. https:\/\/doi.org\/10.1007\/s00246-021-02740-9","journal-title":"Pediatr Cardiol"},{"issue":"6","key":"4358_CR6","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1055\/s-0030-1250101","volume":"58","author":"J Cleuziou","year":"2010","unstructured":"Cleuziou J, Schreiber C, Eicken A, H\u00f6rer J, Busch R, Holper K, Lange R (2010) Predictors for biventricular repair in pulmonary atresia with intact ventricular septum. 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