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Congenital malformation of the right heart

MedGen UID:
868809
•Concept ID:
C4023219
•
Congenital Abnormality
HPO: HP:0011723

Definition

Defect or defects of the morphogenesis of the right heart identifiable at birth. [from HPO]

Professional guidelines

PubMed

Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria.
Corrado D, Perazzolo Marra M, Zorzi A, Beffagna G, Cipriani A, Lazzari M, Migliore F, Pilichou K, Rampazzo A, Rigato I, Rizzo S, Thiene G, Anastasakis A, Asimaki A, Bucciarelli-Ducci C, Haugaa KH, Marchlinski FE, Mazzanti A, McKenna WJ, Pantazis A, Pelliccia A, Schmied C, Sharma S, Wichter T, Bauce B, Basso C
Int J Cardiol 2020 Nov 15;319:106-114. Epub 2020 Jun 16 doi: 10.1016/j.ijcard.2020.06.005. PMID: 32561223
Evaluation and Management of Right-Sided Heart Failure: A Scientific Statement From the American Heart Association.
Konstam MA, Kiernan MS, Bernstein D, Bozkurt B, Jacob M, Kapur NK, Kociol RD, Lewis EF, Mehra MR, Pagani FD, Raval AN, Ward C; American Heart Association Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; and Council on Cardiovascular Surgery and Anesthesia
Circulation 2018 May 15;137(20):e578-e622. Epub 2018 Apr 12 doi: 10.1161/CIR.0000000000000560. PMID: 29650544
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M; ESC Scientific Document Group
Eur Heart J 2016 Jan 1;37(1):67-119. Epub 2015 Aug 29 doi: 10.1093/eurheartj/ehv317. PMID: 26320113

Recent clinical studies

Etiology

Fetal cardiac interventions: Where do we stand?
Friedman KG, Tworetzky W
Arch Cardiovasc Dis 2020 Feb;113(2):121-128. Epub 2020 Feb 26 doi: 10.1016/j.acvd.2019.06.007. PMID: 32113817
Assessment and management of heart failure in the systemic right ventricle.
Carazo M, Andrade L, Kim Y, Wilson W, Wu FM
Heart Fail Rev 2020 Jul;25(4):609-621. doi: 10.1007/s10741-020-09914-2. PMID: 31970551
The Forgotten Ventricle? The Left Ventricle in Right-Sided Congenital Heart Disease.
Tretter JT, Redington AN
Circ Cardiovasc Imaging 2018 Mar;11(3):e007410. doi: 10.1161/CIRCIMAGING.117.007410. PMID: 29855426
Origins and consequences of congenital heart defects affecting the right ventricle.
Woudstra OI, Ahuja S, Bokma JP, Bouma BJ, Mulder BJM, Christoffels VM
Cardiovasc Res 2017 Oct 1;113(12):1509-1520. doi: 10.1093/cvr/cvx155. PMID: 28957538
Patent foramen ovale.
Homma S, Messé SR, Rundek T, Sun YP, Franke J, Davidson K, Sievert H, Sacco RL, Di Tullio MR
Nat Rev Dis Primers 2016 Jan 21;2:15086. doi: 10.1038/nrdp.2015.86. PMID: 27188965

Diagnosis

Ebstein Anomaly in the Adult Patient.
Fuchs MM, Connolly HM
Cardiol Clin 2020 Aug;38(3):353-363. Epub 2020 Jun 6 doi: 10.1016/j.ccl.2020.04.004. PMID: 32622490
Assessment and management of heart failure in the systemic right ventricle.
Carazo M, Andrade L, Kim Y, Wilson W, Wu FM
Heart Fail Rev 2020 Jul;25(4):609-621. doi: 10.1007/s10741-020-09914-2. PMID: 31970551
Cardiac imaging in Ebstein anomaly.
Qureshi MY, O'Leary PW, Connolly HM
Trends Cardiovasc Med 2018 Aug;28(6):403-409. Epub 2018 Jan 12 doi: 10.1016/j.tcm.2018.01.002. PMID: 29409687
Patent foramen ovale.
Homma S, Messé SR, Rundek T, Sun YP, Franke J, Davidson K, Sievert H, Sacco RL, Di Tullio MR
Nat Rev Dis Primers 2016 Jan 21;2:15086. doi: 10.1038/nrdp.2015.86. PMID: 27188965
Aorto-ventricular tunnel.
McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908Free PMC Article

Therapy

Fetal cardiac interventions: Where do we stand?
Friedman KG, Tworetzky W
Arch Cardiovasc Dis 2020 Feb;113(2):121-128. Epub 2020 Feb 26 doi: 10.1016/j.acvd.2019.06.007. PMID: 32113817
Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.
Garcia AM, Beatty JT, Nakano SJ
Am J Physiol Heart Circ Physiol 2020 Apr 1;318(4):H947-H965. Epub 2020 Feb 28 doi: 10.1152/ajpheart.00518.2019. PMID: 32108525Free PMC Article
Ebstein's Anomaly.
Holst KA, Connolly HM, Dearani JA
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):138-144. doi: 10.14797/mdcj-15-2-138. PMID: 31384377Free PMC Article
Contemporary management and outcomes in congenitally corrected transposition of the great arteries.
Kutty S, Danford DA, Diller GP, Tutarel O
Heart 2018 Jul;104(14):1148-1155. Epub 2018 Jan 11 doi: 10.1136/heartjnl-2016-311032. PMID: 29326110
Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions.
Ohye RG, Schranz D, D'Udekem Y
Circulation 2016 Oct 25;134(17):1265-1279. doi: 10.1161/CIRCULATIONAHA.116.022816. PMID: 27777296Free PMC Article

Prognosis

Ebstein Anomaly in the Adult Patient.
Fuchs MM, Connolly HM
Cardiol Clin 2020 Aug;38(3):353-363. Epub 2020 Jun 6 doi: 10.1016/j.ccl.2020.04.004. PMID: 32622490
Cardiac imaging in Ebstein anomaly.
Qureshi MY, O'Leary PW, Connolly HM
Trends Cardiovasc Med 2018 Aug;28(6):403-409. Epub 2018 Jan 12 doi: 10.1016/j.tcm.2018.01.002. PMID: 29409687
Cat eye syndrome.
Sharma D, Murki S, Pratap T, Vasikarla M
BMJ Case Rep 2014 May 19;2014 doi: 10.1136/bcr-2014-203923. PMID: 24842361Free PMC Article
Echocardiographic evaluation of the single right ventricle in congenital heart disease: results of new techniques.
Cua CL, Feltes TF
Circ J 2012;76(1):22-31. Epub 2011 Dec 3 doi: 10.1253/circj.cj-11-1267. PMID: 22139360
The hemi-Fontan operation.
Jacobs ML, Pourmoghadam KK
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:90-7. doi: 10.1053/pcsu.2003.50007. PMID: 12740775

Clinical prediction guides

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):e383-e414. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.06.019. PMID: 34304896
Ebstein's Anomaly.
Holst KA, Connolly HM, Dearani JA
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):138-144. doi: 10.14797/mdcj-15-2-138. PMID: 31384377Free PMC Article
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
Cardiac imaging in Ebstein anomaly.
Qureshi MY, O'Leary PW, Connolly HM
Trends Cardiovasc Med 2018 Aug;28(6):403-409. Epub 2018 Jan 12 doi: 10.1016/j.tcm.2018.01.002. PMID: 29409687
Aorto-ventricular tunnel.
McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908Free PMC Article

Recent systematic reviews

Pulmonary Valve Replacement in Tetralogy of Fallot: An Updated Meta-Analysis.
Van den Eynde J, Sá MPBO, Vervoort D, Roever L, Meyns B, Budts W, Gewillig M, Ruhparwar A, Zhigalov K, Weymann A
Ann Thorac Surg 2022 Mar;113(3):1036-1046. Epub 2020 Dec 27 doi: 10.1016/j.athoracsur.2020.11.040. PMID: 33378694
Pulse oximetry screening for critical congenital heart defects.
Plana MN, Zamora J, Suresh G, Fernandez-Pineda L, Thangaratinam S, Ewer AK
Cochrane Database Syst Rev 2018 Mar 1;3(3):CD011912. doi: 10.1002/14651858.CD011912.pub2. PMID: 29494750Free PMC Article
Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis.
Buca DIP, Khalil A, Rizzo G, Familiari A, Di Giovanni S, Liberati M, Murgano D, Ricciardulli A, Fanfani F, Scambia G, D'Antonio F
Ultrasound Obstet Gynecol 2018 Mar;51(3):323-330. doi: 10.1002/uog.17546. PMID: 28603940
Risk Factors for Coarctation of the Aorta on Prenatal Ultrasound: A Systematic Review and Meta-Analysis.
Familiari A, Morlando M, Khalil A, Sonesson SE, Scala C, Rizzo G, Del Sordo G, Vassallo C, Elena Flacco M, Manzoli L, Lanzone A, Scambia G, Acharya G, D'Antonio F
Circulation 2017 Feb 21;135(8):772-785. Epub 2016 Dec 29 doi: 10.1161/CIRCULATIONAHA.116.024068. PMID: 28034902
Coronary-Pulmonary Artery Fistulas: A Systematic Review.
Verdini D, Vargas D, Kuo A, Ghoshhajra B, Kim P, Murillo H, Kirsch J, Lane M, Restrepo C
J Thorac Imaging 2016 Nov;31(6):380-390. doi: 10.1097/RTI.0000000000000232. PMID: 27768631

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