title: Consensus on timing of intervention for common congenital heart diseases: part II - cyanotic heart defects.
abstract: The purpose of this review/editorial is to discuss how and when to treat the most common cyanotic congenital heart defects (CHDs); the discussion of acyanotic heart defects was presented in a previous editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. While some patients with acyanotic CHD may not require surgical or transcatheter intervention because of spontaneous resolution of the defect or mildness of the defect, the majority of cyanotic CHD will require intervention, mostly surgical. Total surgical correction is the treatment of choice for tetralogy of Fallot patients although some patients may need to be palliated initially by performing a modified Blalock-Taussig shunt. For transposition of the great arteries, arterial switch (Jatene) procedure is the treatment of choice, although Rastelli procedure is required for patients who have associated ventricular septal defect (VSD) and pulmonary stenosis (PS). Some of these babies may require Prostaglandin E1 infusion and/or balloon atrial septostomy prior to corrective surgery. In tricuspid atresia patients, most babies require palliation at presentation either with a modified Blalock-Taussig shunt or pulmonary artery banding followed later by staged Fontan (bidirectional Glenn followed later by extracardiac conduit Fontan conversion usually with fenestration). Truncus arteriosus babies are treated by closure of VSD along with right ventricle to pulmonary artery conduit; palliative banding of the pulmonary artery is no longer recommended. Total anomalous pulmonary venous connection babies require anastomosis of the common pulmonary vein with the left atrium at presentation. Other defects should also be addressed by staged correction or complete repair depending upon the anatomy/physiology. Feasibility, safety and effectiveness of treatment of cyanotic CHD with currently available medical, transcatheter and surgical methods are well established and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.
authors: Department of Pediatrics, Division of Pediatric Cardiology, The University of Texas-Houston Medical School/Children's Memorial Hermann Hospital, 6410 Fannin Street, UTPB Suite # 425, Houston, TX 77030, USA. p.syamasundar.rao@uth.tmc.edu
authors: P Syamasundar
authors: PS
authors: Rao
mesh_terms: D006348:Cardiac Surgical Procedures; D003097:Collateral Circulation; D003490:Cyanosis / Q000601:surgery; D004310:Double Outlet Right Ventricle / Q000601:surgery; D004437:Ebstein Anomaly / Q000601:surgery; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D018633:Pulmonary Atresia / Q000601:surgery; D013771:Tetralogy of Fallot / Q000601:surgery; D014188:Transposition of Great Vessels / Q000453:epidemiology / Q000601:surgery; D018785:Tricuspid Atresia / Q000453:epidemiology / Q000601:surgery; D014338:Truncus Arteriosus / Q000601:surgery
references: Br Heart J. 1983 Dec;50(6):586-9
references: 6652001
references: Ann Thorac Surg. 1993 Jan;55(1):172-4
references: 8417672
references: J Thorac Cardiovasc Surg. 1971 Nov;62(5):825-9
references: 5117247
references: Thorax. 1971 May;26(3):240-8
references: 5089489
references: J Thorac Cardiovasc Surg. 1981 Oct;82(4):629-31
references: 7278356
references: Surgery. 1961 Nov;50:773-7
references: 14039248
references: Am J Cardiol. 1997 Oct 1;80(7):914-21
references: 9382008
references: J Thorac Cardiovasc Surg. 2011 Jun;141(6):1355-61
references: 21227471
references: Heart. 2009 Feb;95(2):142-7
references: 18332061
references: Indian J Pediatr. 2013 Jan;80(1):32-8
references: 22752706
references: J Thorac Cardiovasc Surg. 1989 Nov;98(5 Pt 1):738-50
references: 2811411
references: Am Heart J. 1959 Sep;58:407-13
references: 13808756
references: Circulation. 1969 Jan;39(1):83-95
references: 5782810
references: J Thorac Cardiovasc Surg. 1981 Jun;81(6):906-15
references: 7230858
references: J Thorac Cardiovasc Surg. 1986 Jul;92(1):1-5
references: 3523049
references: J Thorac Cardiovasc Surg. 1982 Sep;84(3):430-6
references: 6213821
references: Circulation. 1978 Oct;58(4):600-6
references: 688569
references: Ann Thorac Surg. 2006 Feb;81(2):744-6
references: 16427896
references: J Thorac Cardiovasc Surg. 2003 Aug;126(2):504-9; discussion 509-10
references: 12928651
references: J Thorac Cardiovasc Surg. 1981 Oct;82(4):511-9
references: 6168869
references: Pediatr Cardiol. 2008 Sep;29(5):946-9
references: 18663512
references: Circulation. 2002 Sep 24;106(12 Suppl 1):I82-9
references: 12354714
references: Ann Thorac Surg. 2004 Dec;78(6):1951-7; discussion 1957-8
references: 15561007
references: Ann Thorac Surg. 1991 Nov;52(5):1084-94; discussion 1094-5
references: 1953128
references: Br Heart J. 1977 Mar;39(3):276-88
references: 849388
references: Ann Thorac Surg. 1985 Oct;40(4):409-13
references: 3901947
references: Ann Thorac Surg. 2001 Jan;71(1):360-1; discussion 361-2
references: 11216785
references: Aust N Z J Med. 1986 Feb;16(1):20-3
references: 3458441
references: JAMA. 1966 Jun 13;196(11):991-2
references: 4160716
references: J Thorac Cardiovasc Surg. 1969 Oct;58(4):545-52
references: 5387997
references: Circulation. 1999 May 25;99(20):2621-5
references: 10338453
references: J Thorac Cardiovasc Surg. 1964 Dec;48:953-8
references: 14239984
references: Ann Surg. 1984 Oct;200(4):451-6
references: 6237622
references: Cathet Cardiovasc Diagn. 1997 Dec;42(4):395-402
references: 9408620
references: Am J Cardiol. 1980 Jan;45(1):87-91
references: 6153137
references: Circulation. 1971 Jan;43(1):83-90
references: 5540854
references: N Engl J Med. 1983 Jan 6;308(1):23-6
references: 6847920
references: Am J Cardiol. 1975 Sep;36(3):327-37
references: 52296
references: Surgery. 1959 Jun;45(6):966-80
references: 13659340
references: Am Heart J. 1988 May;115(5):1105-10
references: 2452561
references: Am J Dis Child. 1974 Jan;127(1):36-40
references: 4809792
references: Eur J Cardiothorac Surg. 2010 Dec;38(6):707-13
references: 20663683
references: Circulation. 1996 Nov 1;94(9 Suppl):II12-6
references: 8901712
references: J Thorac Cardiovasc Surg. 1989 Dec;98(6):1018-28; discussion 1028-9
references: 2586116
references: Circulation. 1969 Aug;40(2):237-62
references: 4240356
references: Ann Thorac Surg. 1980 Apr;29(4):317-21
references: 7362323
references: Circulation. 1980 Aug;62(2 Pt 2):I190-6
references: 7397994
references: Clin Cardiol. 1992 Jul;15(7):483-96
references: 1386794
references: J Thorac Cardiovasc Surg. 1991 Jun;101(6):1082-7
references: 2038202
references: Curr Treat Options Cardiovasc Med. 2002 Aug;4(4):321-336
references: 12093389
references: J Thorac Cardiovasc Surg. 1976 Sep;72(3):364-70
references: 957754
references: Am Heart J. 1980 Jun;99(6):799-804
references: 6990738
references: Cathet Cardiovasc Diagn. 1984;10(4):335-42
references: 6207930
references: Br Med J. 1976 May 8;1(6018):1112-4
references: 1268585
references: Congenit Heart Dis. 2013 Nov-Dec;8(6):585-8
references: 22309144
references: Circulation. 1993 Oct;88(4 Pt 1):1691-8
references: 8403314
references: Ann Thorac Surg. 1991 Sep;52(3):688-95
references: 1898174
references: Ann Thorac Surg. 1983 Feb;35(2):121-31
references: 6186206
references: Pediatr Cardiol. 2005 Mar-Apr;26(2):190-9
references: 15906023
references: Cathet Cardiovasc Diagn. 1997 Apr;40(4):403-6; discussion 407
references: 9096946
references: J Thorac Cardiovasc Surg. 1984 Oct;88(4):610-9
references: 6482493
references: J Thorac Cardiovasc Surg. 1996 Aug;112(2):392-402
references: 8751508
references: J Thorac Cardiovasc Surg. 1988 Nov;96(5):682-95
references: 3184963
references: Indian Heart J. 2007 Nov-Dec;59(6):515-21
references: 19151472
references: JAMA. 1984 Apr 27;251(16):2123-38
references: 6368878
references: Pediatr Clin North Am. 1958 Nov;5(4):1029-56
references: 13600906
references: Circulation. 1995 Dec 1;92(11):3282-8
references: 7586315
references: J Thorac Cardiovasc Surg. 1981 Jan;81(1):112-9
references: 6450303
references: Ann Thorac Surg. 2006 Nov;82(5):1603-9; discussion 1609-10
references: 17062213
references: Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005;:66-71
references: 15818360
references: J Thorac Cardiovasc Surg. 1984 Sep;88(3):365-72
references: 6471887
references: Indian Pediatr. 2008 Feb;45(2):117-26
references: 18310790
references: N Engl J Med. 2010 May 27;362(21):1980-92
references: 20505177
references: J Am Coll Cardiol. 2004 Jul 21;44(2):438-45
references: 15261945
references: Cathet Cardiovasc Diagn. 1992 Jan;25(1):16-24
references: 1555222
references: J Am Coll Cardiol. 1989 Sep;14(3):750-5
references: 2527902
references: Circulation. 1990 Nov;82(5):1681-9
references: 2225370
references: Br Heart J. 1992 Mar;67(3):240-5
references: 1372815
references: Circulation. 1989 Sep;80(3 Pt 1):I203-8
references: 2766528
references: Surgery. 1964 Mar;55:469-72
references: 14133108