{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:36:35Z","timestamp":1786649795947,"version":"build-2736575974"},"reference-count":7,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,1]]},"abstract":"\n Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly, commonly associated with tetralogy of Fallot (TOF). Non-TOF variant, APVS with intact ventricular septum (IVS) and patent ductus arteriosus (PDA) are infrequently reported. While airway compression is a well-known complication, hemodynamic compromise with circular shunting is extremely rare. A full-term neonate with prenatally diagnosed APVS and IVS presented with severe pulmonary stenosis, pulmonary regurgitation (PR), tricuspid regurgitation (TR), and a large PDA. On the 3\n rd<\/jats:sup>\n day of life, the infant developed respiratory distress and systemic hypoperfusion. Echocardiography revealed a circular shunt comprising severe PR and TR, right-to-left shunting at the atrial level, and left-to-right ductal flow. Emergent PDA ligation resulted in rapid hemodynamic recovery. The infant remained well at the 8-month follow-up. This appears to be the first reported case of circular shunting in APVS and intact IVS, emphasizing the need for vigilance and timely surgical intervention.\n <\/jats:p>","DOI":"10.4103\/apc.apc_192_25","type":"journal-article","created":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T07:00:29Z","timestamp":1776236429000},"page":"92-95","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Rare case of absent pulmonary valve syndrome with circular shunt"],"prefix":"10.4103","volume":"19","author":[{"given":"Ardra","family":"Prakash","sequence":"first","affiliation":[{"name":"Department of Paediatric Cardiology, Narayana Institute of Cardiac Sciences, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sweta","family":"Mohanty","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Narayana Institute of Cardiac Sciences, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prakash","family":"Ramachandra","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Narayana Institute of Cardiac Sciences, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,4,15]]},"reference":[{"key":"R1-20260420","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1002\/uog.1729","article-title":"Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus","volume":"24","author":"Volpe","year":"2004","journal-title":"Ultrasound Obstet Gynecol"},{"key":"R2-20260420","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1002\/uog.2807","article-title":"Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve","volume":"28","author":"Galindo","year":"2006","journal-title":"Ultrasound Obstet Gynecol"},{"key":"R3-20260420","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1016\/S0002-9149(02)03238-1","article-title":"Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome","volume":"91","author":"Razavi","year":"2003","journal-title":"Am J Cardiol"},{"key":"R4-20260420","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1002\/pd.5094","article-title":"Absent pulmonary valve syndrome \u2013 Diagnosis, associations, and outcome in 71 prenatally diagnosed cases","volume":"37","author":"Axt-Fliedner","year":"2017","journal-title":"Prenat Diagn"},{"key":"R5-20260420","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1016\/j.mjafi.2013.07.002","article-title":"Absent pulmonary valve syndrome \u2013 Antenatal diagnosis","volume":"70","author":"Grewal","year":"2014","journal-title":"Med J Armed Forces India"},{"key":"R6-20260420","first-page":"71","article-title":"Circular shunt physiology: Recognition and treatment","volume":"8","author":"Tabbutt","year":"1998","journal-title":"Prog Pediatr Cardiol"},{"key":"R7-20260420","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1002\/ccr3.1337","article-title":"Emergency transcatheter closure of a stented PDA in a patient with pulmonary atresia and intact ventricular septum: Be ready for the unexpected!","volume":"6","author":"Faccini","year":"2018","journal-title":"Clin Case Rep"}],"container-title":["Annals of Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_192_25","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T18:58:27Z","timestamp":1786647507000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_192_25"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":7,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/apc.apc_192_25","relation":{},"ISSN":["0974-2069","0974-5149"],"issn-type":[{"value":"0974-2069","type":"print"},{"value":"0974-5149","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"2025-07-22","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-06","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}