{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T12:54:35Z","timestamp":1792414475806,"version":"4.4.0"},"reference-count":49,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2018,1,30]]},"abstract":"<jats:p>The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload. Management of the SRV remains an ongoing challenge because SRV dysfunction has implications on short- and long-term outcomes for all patients irrespective of underlying cardiac morphology. SRV dysfunction can be subclinical, underscoring the need for tertiary follow-up and timely management of target hemodynamic lesions. Catheter interventions and surgery have an established role in selected patients. Cardiac resynchronization therapy is increasingly used, whereas pharmacological therapy is largely empirical. Mechanical assist device and heart transplantation remain options in end-stage heart failure when other management strategies have been exhausted. The present report focuses on the SRV with its pathological subtypes, pathophysiology, clinical features, current management strategies, and long-term sequelae. Although our article touches on issues applicable to neonates and children, its main focus is on adults with SRV.<\/jats:p>","DOI":"10.1161\/circulationaha.117.031544","type":"journal-article","created":{"date-parts":[[2018,1,29]],"date-time":"2018-01-29T14:11:07Z","timestamp":1517235067000},"page":"508-518","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":157,"title":["Systemic Right Ventricle in Adults With Congenital Heart Disease"],"prefix":"10.1161","volume":"137","author":[{"given":"Margarita","family":"Brida","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (M.B., G.-P.D., M.A.G.)"},{"name":"Department of Cardiovascular Medicine, Division of Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Germany (M.B., G.-P.D.)"},{"name":"Department of Cardiovascular Medicine, Division of Adult Congenital Heart Disease, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Croatia (M.B.)"},{"name":"National Heart and Lung Institute, Imperial College, London, United Kingdom (M.B., G.-P.D., M.A.G.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard-Paul","family":"Diller","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (M.B., G.-P.D., M.A.G.)"},{"name":"Department of Cardiovascular Medicine, Division of Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Germany (M.B., G.-P.D.)"},{"name":"National Heart and Lung Institute, Imperial College, London, United Kingdom (M.B., G.-P.D., M.A.G.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Gatzoulis","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (M.B., G.-P.D., M.A.G.)"},{"name":"National Heart and Lung Institute, Imperial College, London, United Kingdom (M.B., G.-P.D., M.A.G.)"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02083294"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.carpath.2016.09.003"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.592352"},{"key":"e_1_3_3_5_2","first-page":"966","article-title":"Surgical correction of transposition of the great vessels.","volume":"45","author":"Senning A","year":"1959","unstructured":"Senning A. 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