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.
Similar content being viewed by others
References
Rao PS. Consensus on timing of intervention for common congenital heart diseases: Part I - Acyanotic heart defects (Editorial). Indian J Pediatr. 2012; doi:10.1007/s12098-012-0833-6.
Fyler DC. Nadas’ Pediatric Cardiology. Philadelphia, PA: Hanley & Belfus, Inc.; 1992.
Fallot E. Contribution a l’anatomie pathologique de la maladie bleu (cyanose cardiaque). Marseille Med. 1888;25:418–25.
Alapati S, Rao PS. Tetralogy of Fallot in the neonate. Neonatology Today. 2011;6:1–10.
Blalock A, Taussig HB. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA. 1945;128:189–94.
de Leval MR, McKay R, Jones M, Stark J, Macartney FJ. Modified Blalock-Taussig shunt: Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts. J Thorac Cardiovasc Surg. 1981;81:112–9.
Rao PS, Brais M. Balloon pulmonary valvuloplasty for congenital cyanotic heart defects. Am Heart J. 1988;115:1105–10.
Rao PS. Transcatheter management of cyanotic congenital heart defects: A review. Clin Cardiol. 1992;15:483–96.
Rao PS, Wilson AD, Thapar MK, Brais M. Balloon pulmonary valvuloplasty in the management of cyanotic congenital heart defects. Cathet Cardiovasc Diagn. 1992;25:16–24.
Rao PS. Pulmonary valve in cyanotic heart defects with pulmonary oligemia. In: Sievert H, Qureshi SA, Wilson N, Hijazi Z, eds. Percutaneous interventions in congenital heart disease. Oxford: Informa Health Care; 2007. pp. 197–200.
Kohli V, Azad S, Sachdev MS, Joshi R, Ebeid MR. Balloon dilation of the pulmonary valve in premature infants with tetralogy of Fallot. Pediatr Cardiol. 2008;29:946–9.
Laudito A, Bandisode VM, Lucas JF, Radtke WA, Adamson WT, Bradley SM. Right ventricular outflow tract stent as a bridge to surgery in a premature infant with tetralogy of Fallot. Ann Thorac Surg. 2006;81:744–6.
Dohlen G, Chaturvedi RR, Benson LN, et al. Stenting of the right ventricular outflow tract in the symptomatic infant with tetralogy of Fallot. Heart. 2009;95:142–7.
Morgan GJ. Echocardiographic guidance for neonatal right ventricular outflow tract stent implantation. Congenit Heart Dis. 2012; doi:10.1111/j.1747-0803.2011.00623.x.
Gibbs JL, Rothman MT, Rees M, Parsons JM, Blackburn ME, Ruiz CE. Stenting of the arterial duct: A new approach to palliation for pulmonary atresia. Br Heart J. 1992;67:240–5.
Siblini G, Rao PS, Singh GK, Tinker K, Balfour IC. Transcatheter management of neonates with pulmonary atresia and intact ventricular septum. Cathet Cardiovasc Diagn. 1997;42:395–402.
Gibbs JL, Orhan U, Blackburn MEC, Wren C, Hamilton JR, Watterson KG. Fate of stented arterial duct. Circulation. 1999;99:2621–5.
Alwi M, Choo KK, Latiff HA, Kandavello G, Samion H, Mulyadi MD. Initial results and medium-term follow-up of stent implantation of patent ductus arteriosus in duct-dependent pulmonary circulation. J Am Coll Cardiol. 2004;44:438–45.
Rao PS. Role of interventional cardiology in the treatment of neonates: Part III. Congenital Cardiol Today. 2008;6:1–10.
Kuhn MA, Mulla NF, Dyar D, Cephus C, Larsen RL. Valve perforation and balloon pulmonary valvuloplasty in an infant with tetralogy of Fallot and pulmonary atresia. Cathet Cardiovasc Diagn. 1997;40:403–6.
Rome JJ, Mayer JE, Castaneda AR, Lock JE. Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries. Circulation. 1993;88:1691–8.
Puga FJ, Leoni FE, Julsrud PR, Mair DD. Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries: Experience with preliminary unifocalization procedures in 38 patients. J Thorac Cardiovasc Surg. 1989;98:1018–28.
Sawatari K, Imai Y, Kurosawa H, Isomatsu Y, Momma K. Staged operation for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries: New technique for complete unifocalization. J Thorac Cardiovasc Surg. 1989;98:738–50.
Yagihara T, Yamamoto F, Nishigaki K, et al. Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 1996;112:392–402.
Castaneda AR, Jonas RA, Mayer JE, et al. Tetralogy of Fallot. In: Castaneda AR, Jonas RA, Mayer JE, eds. Cardiac Surgery of the Neonate and Infant. Philadelphia: WB Saunders; 1994. pp. 215.
Nakata S, Imai Y, Takanashi Y, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg. 1984;88:610–9.
Powell AJ, Lock JE, Keane JF, Perry SB. Prolongation of RV-PA conduit life span by percutaneous stent implantation: Intermediate-term results. Circulation. 1995;92:3282–8.
Arensman FW, Francis PD, Helmsworth JA, et al. Early medical and surgical intervention for tetralogy of Fallot with absence of pulmonic valve. J Thorac Cardiovasc Surg. 1982;84:430–6.
Heinemann MK, Hanley FL. Preoperative management of neonatal tetralogy of Fallot with absent pulmonary valve syndrome. Ann Thorac Surg. 1993;55:172–4.
Rao PS, Lawrie GM. Absent pulmonary valve syndrome: Surgical correction with pulmonary arterioplasty. Br Heart J. 1983;50:586–9.
Ilbawi MN, Idriss FS, Muster AJ, Wessel HU, Paul MH, Deleon SY. Tetralogy of Fallot with absent pulmonary valve: Should valve insertion be a part of the intracardiac repair? J Thorac Cardiovasc Surg. 1981;81:906–15.
Rao PS. Transposition of the great arteries in the neonate. Neonatology Today. 2010;5:1–8.
Liebman J, Cullum L, Belloc NB. Natural history of transposition of the great arteries, Anatomy and birth and death characteristics. Circulation. 1969;40:237–62.
Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy. JAMA. 1966;196:991–2.
Rao PS. Role of interventional cardiology in neonates: Part I. Non-surgical atrial septostomy. Neonatology Today. 2007;2:9–14.
Shumacker Jr HB. A new operation for transposition of the great vessels. Surgery. 1961;50:773–7.
Senning A. Surgical correction of transposition of the great vessels. Surgery. 1959;45:966–80.
Mustard WT, Keith JD, Trusler GA, Fowler R, Kidd L. The surgical management of transposition of the great vessels. J Thorac Cardiovasc Surg. 1964;48:953–8.
Mustard WT. Successful two-stage correction of transposition of the great vessels. Surgery. 1964;55:469–72.
Jatene AD, Fontes VF, Paulista PP, et al. Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg. 1976;72:364–70.
Bisoi AK, Sharma P, Chauhan S, et al. Primary arterial switch operation in children presenting late with d-transposition of great arteries and intact ventricular septum. When is it too late for a primary arterial switch operation? Eur J Cardiothorac Surg. 2010;38:707–13.
Yacoub M, Bernhard A, Lange P, et al. Clinical and hemodynamic results of the two-stage anatomic correction of simple transposition of the great arteries. Circulation. 1980;62:I190–6.
Jonas RA, Giglia TM, Sanders SP, et al. Rapid, two-stage arterial switch for transposition of the great arteries and intact ventricular septum beyond the neonatal period. Circulation. 1989;80:I203–8.
Yacoub MH, Radley-Smith R, Hilton CJ. Anatomical correction of complete transposition of the great arteries and ventricular septal defect in infancy. Br Med J. 1976;1:1112–4.
Rastelli GC, Wallace RB, Ongley PA. Complete repair of transposition of the great arteries with pulmonary stenosis. A review and report of a case corrected by using a new surgical technique. Circulation. 1969;39:83–95.
Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transposition of the great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg. 1969;58:545–52.
Kawashima Y, Fujita T, Miyamoto T, Manabe H. Intraventricular rerouting of blood for the correction of Taussig-Bing malformation. J Thorac Cardiovasc Surg. 1971;62:825–9.
Lecompte Y, Zannini L, Hazan E, et al. Anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg. 1981;82:629–31.
Nikaidoh H. Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg. 1984;88:365–72.
Rao PS. Terminology: Tricuspid atresia or univentricular heart? In: Rao PS, ed. Tricuspid Atresia. Mount Kisco, NY: Futura Publishing Co; 1982. pp. 3–6.
Rao PS. Demographic features of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura Publishing Co; 1992. pp. 23–9.
Rao PS. A unified classification for tricuspid atresia. Am Heart J. 1980;99:799–804.
Rao PS. Classification of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura Publishing Co; 1992. pp. 59.
Rao PS, Sissman NJ. Spontaneous closure of physiologically advantageous ventricular septal defects. Circulation. 1971;43:83–90.
Rao PS, Linde LM, Liebman J, Perrin E. Functional closure of physiologically advantageous ventricular septal defects: Observations in three cases with tricuspid atresia. Am J Dis Child. 1974;127:36–40.
Rao PS. Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. Br Heart J. 1977;39:276–88.
Rao PS. Further observations on the spontaneous closure of physiologically advantageous ventricular septal defects in tricuspid atresia: Surgical implications. Ann Thorac Surg. 1983;35:121–31.
Rao PS. Natural history of ventricular septal defects in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura Publishing Co; 1992. pp. 261.
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240–8.
Kreutzer G, Bono H, Galindez E, et al. Una operacion para la correccion de la atresiatricuspidea. Argentina: Ninth Argentinean Congress of Cardiology, Buenos Aires; 1971.
Annecchino FP, Fontan F, Chauve A, Quaegebeur J. Palliative reconstruction of the right ventricular outflow tract in tricuspid atresia: A report of 5 patients. Ann Thorac Surg. 1980;29:317–21.
McCredie RM, Lee CL, Swinburn MJ, Lee CL, Warner G. Balloon dilatation pulmonary valvuloplasty in pulmonary stenosis. Aust New Zealand J Med. 1986;16:20–3.
Rao PS, Covitz W, Chopra PS. Principles of palliative management of patients with tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura Publishing Co; 1992. pp. 297.
Rao PS. Tricuspid Atresia. eMedicine from WebMD. Updated February 09, 2009. Available at: http://www.emedicine.com/ped/topic2550.htm
Bonnet D, Sidi D, Vouhé PR. Absorbable pulmonary artery banding in tricuspid atresia. Ann Thorac Surg. 2001;71:360–1.
Rao PS. Absorbable pulmonary artery band in tricuspid atresia (Editorial). Ann Thorac Surg. 2001;71:361–2.
Dick M, Fyler DC, Nadas AS. Tricuspid atresia: Clinical course in 101 patients. Am J Cardiol. 1975;36:327–37.
Rao PS. Cardiac catheterization in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. Mount Kisco, NY: Futura Publishing Co; 1982. pp. 153–78.
Park SC, Neches WH, Zuberbuhler JR, et al. Clinical use of blade atrial septostomy. Circulation. 1978;58:600–6.
Rao PS. Transcatheter blade atrial septostomy. Cathet Cardiovasc Diagn. 1984;10:335–42.
Salim M, Muster AJ, Paul MH, Benson DW Jr. Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. J Am Coll Cardiol. 1989;14:750–5.
de Leval MR, Kilner P, Gewilling M, Bull C. Total cavopulmonary connection: A logical alternative to atriopulmonary connection for complex Fontan operation. J Thorac Cardiovasc Surg. 1988;96:682–95.
Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure: Modification of the Fontan operation for patients with increased risk. Circulation. 1990;82:1681–91.
Laks H, Pearl JM, Haas GS, et al. Partial Fontan: Advantages of an adjustable interatrial communication. Ann Thorac Surg. 1991;52:1084–94.
Rao PS, Chandar JS, Sideris EB. Role of inverted buttoned device in transcatheter occlusion of atrial septal defect or patent foramen ovale with right-to-left shunting associated with complex congenital cardiac anomalies. Am J Cardiol. 1997;80:914–21.
Lev M, Saphir O. Truncus arteriosus communispersistens. J Pediatr. 1942;20:74–84.
Ebert PA, Turley K, Stanger P. Surgical treatment of truncus arteriosus in the first 6 months of life. Ann Surg. 1984;200:451–6.
Bando K, Turrentine MW, Ensing GJ, et al. Surgical treatment of total anomalous pulmonary venous connection. Circulation. 1996;94:II12–6.
Noonan JA, Nadas AS. The hypoplastic left heart syndrome. Pediat Clinics North Am. 1958;5:1029–39.
Rao PS, Striepe V, Merrill WH. Hypoplastic left heart syndrome. In: Kambam J, ed. Cardiac Anesthesia for Infants and Children. St. Louis, MO: Mosby-Year Book; 1994. pp. 296–309.
Rao PS, Turner DR, Forbes TJ. Hypoplastic Left Heart Syndrome. eMedicine from WebMD. Updated September 22, 2009. Available at: http://emedicine.medscape.com/article/890196-overview
Norwood WI Jr. Hypoplastic left heart syndrome. Ann Thorac Surg. 1991;52:688–95.
Norwood WI, Lang P, Castaneda AR, Campbell DN. Experience with operation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1981;82:511–9.
Bailey L, Concepcion W, Shattuk H, Huang L. Method of heart transplantation for treatment of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1986;92:1–9.
Sano S, Ishino K, Kawada M, et al. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:504–9.
Galantowicz M, Cheatham JP. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome [published correction appears in PediatrCardiol 2005; 26: 307]. Pediatr Cardiol. 2005;26:190–9.
Pizarro C, Murdison, KA. Off pump palliation for hypoplastic left heart syndrome: Surgical approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annual. 2005:66–71.
Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. 1983;308:23–6.
Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol. 1980;45:87–91.
Sano S, Ishino K, Kado H, et al. Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome: A multi-institutional study. Ann Thorac Surg. 2004;78:1951–7. discussion 1957–8.
Ghanayem NS, Jaquiss RDB, Cava JR, et al. Right ventricle-to-pulmonary artery conduit versus Blalock-Taussig shunt: A hemodynamic comparison. Ann Thorac Surg. 2006;82:1603–9.
Ohye RG, Sleeper LA, Mahony L, et al. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010;362:1980–92.
Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: Lessons learned from 115 consecutive patients. Circulation. 2002;106:I82–9.
Rao PS. Comprehensive management of pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 1985;40:409–13.
Rao PS. Pulmonary atresia with intact ventricular septum. Curr Treat Options Cardiovasc Med. 2002;4:321–36.
Alwi M, Choo KK, Radzi NA, Samion H, Pau KK, Hew CC. Concomitant stenting of the patent ductus arteriosus and radiofrequency valvotomy in pulmonary atresia with intact ventricular septum and intermediate right ventricle: Early in-hospital and medium-term outcomes. J Thorac Cardiovasc Surg. 2011;141:1355–61. doi:10.1016/j.jtcvs.2010.08.085.
Buck SH, Rao PS, Merrill W, Kambam J. Double-outlet right ventricle. In: Kambam J, ed. Cardiac Anesthesia for Infants and Children. St. Louis, MO: Mosby-Year Book; 1993. pp. 312–39.
Sharma SK, Rao PS. Interrupted aortic arch. The encyclopedic reference of molecular mechanisms of disease. Berlin: Springer; 2008.
Celoria GC, Patton RB. Congenital absence of the aortic of the aortic arch. Am Heart J. 1959;58:407–13.
Rao PS. Tricuspid valve abnormalities other than tricuspid atresia. In: Long WA, ed. Fetal and neonatal cardiology. Philadelphia, PA: W.B. Saunders; 1990. pp. 541–50.
Rao PS, Kambam J. Ebstein’s malformation of the tricuspid valve, Chapter 29. In: Kambam J, ed. Cardiac anesthesia for infants and children. St. Louis, MO: Mosby-Year Book; 1994. pp. 320–32.
Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein’s anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg. 1991;101:1082–7.
Saxena A. National consensus meeting on “Management of congenital heart diseases in India” held on 26th august 2007 at the All India Institute of Medical Sciences, New Delhi, India, supported by The Cardiological Society of India. Indian Heart J. 2007;59:515–21.
Working Group on Management of Congenital Heart Diseases in India. Consensus on timing of intervention for common congenital heart disease. Indian Pediatr. 2008;45:117–26.
Acknowledgements
The author of this editorial and the editors of this Journal thank Dr. Vikas Kohli of Indraprastha Apollo Hospital and Delhi Child Heart Center, New Delhi, India, Dr. Bharat Dalvi of Glenmark Cardiac Centre, Matunga (E), Mumbai, India and Dr. I.B. Vijayalakshmi of Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, India for their review and constructive criticism of this paper.
Conflict of Interest
None.
Role of Funding Source
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Rao, P.S. Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part II - Cyanotic Heart Defects. Indian J Pediatr 80, 663–674 (2013). https://doi.org/10.1007/s12098-013-1039-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-013-1039-2
Keywords
- Cyanotic congenital heart defects
- Tetralogy of fallot
- Transposition of the great arteries
- Tricuspid atresia
- Truncus arteriosus
- Total anomalous pulmonary venous connection
- Double-inlet left ventricle
- Hypoplastic left heart syndrome
- Pulmonary atresia
- Double-outlet right ventricle
- Interrupted aortic arch
- Ebstein’s anomaly of the tricuspid valve
- Indications for intervention
- Palliative surgery
- Corrective surgery
- Transcatheter therapy