{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:30:00Z","timestamp":1787776200376,"version":"build-2784847793"},"reference-count":10,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Partial cavopulmonary shunt is an established procedure for patients with abnormal right ventricular (RV) physiology. Late failure of the Glenn shunt is usually due to further progression of the primary disease process. However, there may be surprises, as in our patient. We report an 8-year-old child who was diagnosed with tricuspid valve (TV) dysplasia with borderline RV hypoplasia. He underwent TV repair with a right cavopulmonary shunt. He presented with features of heart failure after 6 years of the surgery. An echocardiographic evaluation suggested a restrictive filling pattern of the RV with preserved size and function. Computed tomography and cardiac magnetic resonance imaging revealed a normal pericardial thickness with no evidence of myocardial involvement. Despite the contradicting clinical and imaging features, endomyocardial biopsy confirmed a diagnosis of desmin cardiomyopathy. Restrictive cardiomyopathy may remain an elusive diagnosis despite extensive imaging. In our case, the altered loading conditions due to a cavopulmonary shunt further masked pathophysiological hemodynamic changes.<\/jats:p>","DOI":"10.4103\/apc.apc_78_24","type":"journal-article","created":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T05:00:11Z","timestamp":1727845211000},"page":"207-210","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Heart failure following cavopulmonary shunt \u2013 An unusual story of desmin cardiomyopathy"],"prefix":"10.4103","volume":"17","author":[{"given":"Lamk","family":"Kadiyani","sequence":"first","affiliation":[{"name":"Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sivasubramanian","family":"Ramakrishnan","sequence":"additional","affiliation":[{"name":"Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sudheer Kumar","family":"Arava","sequence":"additional","affiliation":[{"name":"Department of Pathology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandeep","family":"Seth","sequence":"additional","affiliation":[{"name":"Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2024,10,1]]},"reference":[{"key":"R1-20241204","doi-asserted-by":"crossref","first-page":"4679","DOI":"10.1093\/eurheartj\/ehac543","article-title":"Restrictive cardiomyopathy: Definition and diagnosis","volume":"43","author":"Rapezzi","year":"2022","journal-title":"Eur Heart J"},{"key":"R2-20241204","doi-asserted-by":"crossref","first-page":"128","DOI":"10.4103\/2395-5414.191522","article-title":"Desmin related cardiomyopathy presenting as restrictive cardiomyopathy: A case report with review of literature","volume":"2","author":"Kumari","year":"2016","journal-title":"J Pract Cardiovasc Sci"},{"key":"R3-20241204","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1111\/jocs.14372","article-title":"Early degeneration of two different tricuspid bioprostheses in restrictive cardiomyopathy","volume":"35","author":"Carino","year":"2020","journal-title":"J Card Surg"},{"key":"R4-20241204","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1161\/01.CIR.35.5.913","article-title":"Radiological findings in endomyocardial fibrosis","volume":"35","author":"Cockshott","year":"1967","journal-title":"Circulation"},{"key":"R5-20241204","doi-asserted-by":"crossref","first-page":"G19","DOI":"10.1530\/ERP-19-0051","article-title":"Education Committee of the British Society of Echocardiography. Echocardiographic assessment of the right heart in adults: a practical guideline from the British Society of Echocardiography","volume":"7","author":"Zaidi","year":"2020","journal-title":"Echo Res Pract"},{"key":"R6-20241204","first-page":"105","article-title":"Cardiac MRI in restrictive cardiomyopathy","volume":"67","author":"Gupta","year":"2012"},{"key":"R7-20241204","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/1532-429X-13-15","article-title":"The relative atrial volume ratio and late gadolinium enhancement provide additive information to differentiate constrictive pericarditis from restrictive cardiomyopathy","volume":"13","author":"Cheng","year":"2011","journal-title":"J Cardiovasc Magn Reson"},{"key":"R8-20241204","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1161\/CIRCIMAGING.113.001613","article-title":"Echocardiographic diagnosis of constrictive pericarditis: Mayo clinic criteria","volume":"7","author":"Welch","year":"2014","journal-title":"Circ Cardiovasc Imaging"},{"key":"R9-20241204","doi-asserted-by":"crossref","first-page":"1852","DOI":"10.1161\/01.CIR.0000087606.18453.FD","article-title":"Constrictive pericarditis in 26 patients with histologically normal pericardial thickness","volume":"108","author":"Talreja","year":"2003","journal-title":"Circulation"},{"key":"R10-20241204","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/j.hlc.2014.10.003","article-title":"Tissue Doppler imaging in echocardiography: Value and limitations","volume":"24","author":"Kadappu","year":"2015","journal-title":"Heart Lung Circ"}],"container-title":["Annals of Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_78_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:03:07Z","timestamp":1787774587000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_78_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5]]},"references-count":10,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024]]}},"URL":"https:\/\/doi.org\/10.4103\/apc.apc_78_24","relation":{},"ISSN":["0974-2069","0974-5149"],"issn-type":[{"value":"0974-2069","type":"print"},{"value":"0974-5149","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5]]},"assertion":[{"value":"2024-04-22","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-20","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}