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Health Dictionary

Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Congenital heart disease surgery

Introduction

Surgery

Surgery

Some defects require surgical procedures to repair as much as possible to restore circulation back to normal. In some cases, multiple surgeries are needed to be performed to help balance the circulation. Interventional cardiology now offers patients minimally invasive alternatives to surgery. Device closures can now be treated with a standard transcatheter procedure using a closure device mounted on a balloon catheter.

2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1]

2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1]

Noncardiac Surgery (DO NOT EDIT)[1]

Class I
“1. Basic preoperative assessment for ACHD patients should include systemic arterial oximetry, an ECG, chest x-ray, TTE, and blood tests for full blood count and coagulation screen. (Level of Evidence: C)”
“2. It is recommended that when possible, the preoperative evaluation and surgery for ACHD patients be performed in a regional center specializing in congenital cardiology, with experienced surgeons and cardiac anesthesiologists. (Level of Evidence: C)”
“3. Certain high-risk patient populations should be managed at centers for the care of ACHD patients under all circumstances, unless the operative intervention is an absolute emergency. High-risk categories include patients with the following:”
“a. Prior Fontan procedure. (Level of Evidence: C)”
“b. Severe pulmonary arterial hypertension (PAH). (Level of Evidence: C)”
“c. Cyanotic CHD. (Level of Evidence: C)”
“d. Complex CHD with residua such as heart failure, valve disease, or the need for anticoagulation. (Level of Evidence: C)”
“e. Patients with CHD and malignant arrhythmias. (Level of Evidence: C)”
“4. Consultation with ACHD experts regarding the assessment of risk is recommended for patients with CHD who will undergo noncardiac surgery. (Level of Evidence: C)”
“5. Consultation with a cardiac anesthesiologist is recommended for moderate- and high-risk patients. (Level of Evidence: C)”

Heart and Heart/Lung Transplantation (DO NOT EDIT)[1]

Class I
“1. Patients with CHD and heart failure who may require heart transplantation should be evaluated and managed in tertiary care centers with medical and surgical personnel with experience and expertise in the management of both CHD and heart transplantation. (Level of Evidence: C)”
“2. Patients with CHD and heart or respiratory failure who may require lung or heart/lung transplantation should be evaluated and managed in tertiary care centers with medical and surgical personnel with experience and expertise in the management of CHD and lung or heart/lung transplantation. (Level of Evidence: C)”
2012 ACC/AHA/HRS Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities (DO NOT EDIT)[2][3]

2012 ACC/AHA/HRS Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities (DO NOT EDIT)[2][3]

Permanent Pacing in Children, Adolescents, and Patients With Congenital Heart Disease (DO NOT EDIT)[3]

Class I
“1. Permanent pacemaker implantation is indicated for advanced second- or third-degree AV block associated with symptomatic bradycardia, ventricular dysfunction, or low cardiac output. (Level of Evidence: C)”
“2. Permanent pacemaker implantation is indicated for SND with correlation of symptoms during age-inappropriate bradycardia. The definition of bradycardia varies with the patient’s age and expected heart rate. (Level of Evidence: B)[4][5][6][7]”
“3. Permanent pacemaker implantation is indicated for postoperative advanced second- or third-degree AV block that is not expected to resolve or that persists at least 7 days after cardiac surgery. (Level of Evidence: B)[8][9]”
“4. Permanent pacemaker implantation is indicated for congenital third-degree AV block with a wide QRS escape rhythm, complex ventricular ectopy, or ventricular dysfunction. (Level of Evidence: B)[10][11][12]”
“5. Permanent pacemaker implantation is indicated for congenital third-degree AV block in the infant with a ventricular rate less than 55 bpm or with congenital heart disease and a ventricular rate less than 70 bpm. (Level of Evidence: C)[13][14]”
Class III (Harm)
“1. Permanent pacemaker implantation is not indicated for transient postoperative AV block with return of normal AV conduction in the otherwise asymptomatic patient. (Level of Evidence: B)[15][16]”
“2. Permanent pacemaker implantation is not indicated for asymptomatic bifascicular block with or without first-degree AV block after surgery for congenital heart disease in the absence of prior transient complete AV block. (Level of Evidence: C)”
“3. Permanent pacemaker implantation is not indicated for asymptomatic type I second-degree AV block. (Level of Evidence: C)”
“4. Permanent pacemaker implantation is not indicated for asymptomatic sinus bradycardia with the longest relative risk interval less than 3 seconds and a minimum heart rate more than 40 bpm. (Level of Evidence: C)”
Class IIa
“1. Permanent pacemaker implantation is reasonable for patients with congenital heart disease and sinus bradycardia for the prevention of recurrent episodes of intra-atrial reentrant tachycardia; SND may be intrinsic or secondary to antiarrhythmic treatment. (Level of Evidence: C)[17][18][19]”
“2. Permanent pacemaker implantation is reasonable for congenital third-degree AV block beyond the first year of life with an average heart rate less than 50 bpm, abrupt pauses in ventricular rate that are 2 or 3 times the basic cycle length, or associated with symptoms due to chronotropic incompetence. (Level of Evidence: B)[20][21]”
“3. Permanent pacemaker implantation is reasonable for sinus bradycardia with complex congenital heart disease with a resting heart rate less than 40 bpm or pauses in ventricular rate longer than 3 seconds. (Level of Evidence: C)”
“4. Permanent pacemaker implantation is reasonable for patients with congenital heart disease and impaired hemodynamics due to sinus bradycardia or loss of AV synchrony. (Level of Evidence: C)[22]”
“5. Permanent pacemaker implantation is reasonable for unexplained syncope in the patient with prior congenital heart surgery complicated by transient complete heart block with residual fascicular block after a careful evaluation to exclude other causes of syncope. (Level of Evidence: B)[12][23][24][25]”
Class IIb
“1. Permanent pacemaker implantation may be considered for transient postoperative third-degree AV block that reverts to sinus rhythm with residual bifascicular block. (Level of Evidence: C)[15]”
“2. Permanent pacemaker implantation may be considered for congenital third-degree AV block in asymptomatic children or adolescents with an acceptable rate, a narrow QRS complex, and normal ventricular function. (Level of Evidence: B)[10][21]”
“3. Permanent pacemaker implantation may be considered for asymptomatic sinus bradycardia after biventricular repair of congenital heart disease with a resting heart rate less than 40 bpm or pauses in ventricular rate longer than 3 seconds. (Level of Evidence: C)”
2006 ACC/AHA/ESC Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT)[26]

2006 ACC/AHA/ESC Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT)[26]

Congenital Heart Disease (DO NOT EDIT)[26]

Class I
“1. ICD implantation is indicated in patients with congenital heart disease who are survivors of cardiac arrest after evaluation to define the cause of the event and exclude any reversible causes. ICD implantation is indicated in patients who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: B)”
“2. Patients with congenital heart disease and spontaneous sustained VT should undergo invasive hemodynamic and EP evaluation. Recommended therapy includes catheter ablation or surgical resection to eliminate VT. If that is not successful, ICD implantation is recommended. (Level of Evidence: C)”
Class III (Harm)
“1. Prophylactic antiarrhythmic therapy is not indicated for asymptomatic patients with congenital heart disease and isolated PVCs. (Level of Evidence: C)”
Class IIa
“1. Invasive hemodynamic and EP evaluation is reasonable in patients with congenital heart disease and unexplained syncope and impaired ventricular function. In the absence of a defined and reversible cause, ICD implantation is reasonable in patients who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: B)”
Class IIb
“1. EP testing may be considered for patients with congenital heart disease and ventricular couplets or NSVT to determine the risk of a sustained ventricular arrhythmia. (Level of Evidence: C)”
ICD in pediatric patients with congenital heart disease

ICD in pediatric patients with congenital heart disease

2012 ACCF/AHA/HRS Focused Update Incorporated Into the ACCF/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities[1]

Implantable Cardioverter-Defibrillators in Pediatric Patients and Patients With Congenital Heart Disease [2]

Class I
“1. ICD implantation is indicated in the survivor of cardiac arrest after evaluation to define the cause of the event and to exclude any reversible causes.[3][4][5][6] (Level of Evidence: B)”
“2. ICD implantation is indicated for patients with symptomatic sustained VT in association with congenital heart disease who have undergone hemodynamic and electrophysiological evaluation. Catheter ablation or surgical repair may offer possible alternatives in carefully selected patients.[7] (Level of Evidence: C)”
Class III (No Benefit)

“1. All Class III recommendations found in Section 3, “Indications for Implantable Cardioverter-Defibrillator Therapy,” apply to pediatric patients and patients with congenital heart disease, and ICD implantation is not indicated in these patient populations. (Level of Evidence: C)”

Class IIa

“1. ICD implantation is reasonable for patients with congenital heart disease with recurrent syncope of undetermined origin in the presence of either ventricular dysfunction or inducible ventricular arrhythmias at electrophysiological study.[8][9] (Level of Evidence: B)”

Class IIb

“1. ICD implantation may be considered for patients with recurrent syncope associated with complex congenital heart disease and advanced systemic ventricular dysfunction when thorough invasive and noninvasive investigations have failed to define a cause.[10][11] (Level of Evidence: C)”

  1. ↑ Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Tracy CM, Epstein AE, Darbar D, DiMarco JP, Dunbar SB, Estes NA, Ferguson TB, Hammill SC, Karasik PE, Link MS, Marine JE, Schoenfeld MH, Shanker AJ, Silka MJ, Stevenson LW, Stevenson WG, Varosy PD (2013). “2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society”. J. Am. Coll. Cardiol. 61 (3): e6–75. doi:10.1016/j.jacc.2012.11.007. PMID 23265327.
  2. ↑ Epstein AE, DiMarco JP, Ellenbogen KA; et al. (2008). “ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons”. Circulation. 117 (21): e350–408. PMID 18483207. Text “doi:10.1161/CIRCUALTIONAHA.108.189742 ” ignored (help); Unknown parameter |month= ignored (help)
  3. ↑ Silka MJ, Kron J, Dunnigan A, Dick M (1993). “Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients. The Pediatric Electrophysiology Society”. Circulation. 87 (3): 800–7. PMID 8443901.
  4. ↑ Hamilton RM, Dorian P, Gow RM, Williams WG (1996). “Five-year experience with implantable defibrillators in children”. Am J Cardiol. 77 (7): 524–6. PMID 8629596.
  5. ↑ Alexander ME, Cecchin F, Walsh EP, Triedman JK, Bevilacqua LM, Berul CI (2004). “Implications of implantable cardioverter defibrillator therapy in congenital heart disease and pediatrics”. J Cardiovasc Electrophysiol. 15 (1): 72–6. doi:10.1046/j.1540-8167.2004.03388.x. PMID 15028076.
  6. ↑ Choi GR, Porter CB, Ackerman MJ (2004). “Sudden cardiac death and channelopathies: a review of implantable defibrillator therapy”. Pediatr Clin North Am. 51 (5): 1289–303. doi:10.1016/j.pcl.2004.04.015. PMID 15331285.
  7. ↑ Karamlou T, Silber I, Lao R, McCrindle BW, Harris L, Downar E; et al. (2006). “Outcomes after late reoperation in patients with repaired tetralogy of Fallot: the impact of arrhythmia and arrhythmia surgery”. Ann Thorac Surg. 81 (5): 1786–93, discussion 1793. doi:10.1016/j.athoracsur.2005.12.039. PMID 16631673.
  8. ↑ Mushlin AI, Hall WJ, Zwanziger J, Gajary E, Andrews M, Marron R; et al. (1998). “The cost-effectiveness of automatic implantable cardiac defibrillators: results from MADIT. Multicenter Automatic Defibrillator Implantation Trial”. Circulation. 97 (21): 2129–35. PMID 9626173.
  9. ↑ Khairy P, Landzberg MJ, Gatzoulis MA, Lucron H, Lambert J, Marçon F; et al. (2004). “Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study”. Circulation. 109 (16): 1994–2000. doi:10.1161/01.CIR.0000126495.11040.BD. PMID 15051640.
  10. ↑ Kammeraad JA, van Deurzen CH, Sreeram N, Bink-Boelkens MT, Ottenkamp J, Helbing WA; et al. (2004). “Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries”. J Am Coll Cardiol. 44 (5): 1095–102. doi:10.1016/j.jacc.2004.05.073. PMID 15337224.
  11. ↑ Dubin AM, Berul CI, Bevilacqua LM, Collins KK, Etheridge SP, Fenrich AL; et al. (2003). “The use of implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation”. J Card Fail. 9 (5): 375–9. PMID 14583898.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dima Nimri, M.D. [2]

References

References

  1. ↑ 1.0 1.1 1.2 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons”. J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
  2. ↑ Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Tracy CM, Epstein AE, Darbar D, DiMarco JP, Dunbar SB, Estes NA, Ferguson TB, Hammill SC, Karasik PE, Link MS, Marine JE, Schoenfeld MH, Shanker AJ, Silka MJ, Stevenson LW, Stevenson WG, Varosy PD (2013). “2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society”. J. Am. Coll. Cardiol. 61 (3): e6–75. doi:10.1016/j.jacc.2012.11.007. PMID 23265327.
  3. ↑ 3.0 3.1 Epstein AE, Dimarco JP, Ellenbogen KA, Estes NA, Freedman RA, Gettes LS; et al. (2008). “ACC/AHA/HRS 2008 guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: executive summary”. Heart Rhythm. 5 (6): 934–55. doi:10.1016/j.hrthm.2008.04.015. PMID 18534377.
  4. ↑ Kay R, Estioko M, Wiener I (1982). “Primary sick sinus syndrome as an indication for chronic pacemaker therapy in young adults: incidence, clinical features, and long-term evaluation”. Am Heart J. 103 (3): 338–42. PMID 6461235.
  5. ↑ Ector H, Rolies L, De Geest H (1983). “Dynamic electrocardiography and ventricular pauses of 3 seconds and more: etiology and therapeutic implications”. Pacing Clin Electrophysiol. 6 (3 Pt 1): 548–51. PMID 6191291.
  6. ↑ Kelly AM, Porter CJ, McGoon MD, Espinosa RE, Osborn MJ, Hayes DL (2001). “Breath-holding spells associated with significant bradycardia: successful treatment with permanent pacemaker implantation”. Pediatrics. 108 (3): 698–702. PMID 11533339.
  7. ↑ Beder SD, Gillette PC, Garson A, Porter CB, McNamara DG (1983). “Symptomatic sick sinus syndrome in children and adolescents as the only manifestation of cardiac abnormality or associated with unoperated congenital heart disease”. Am J Cardiol. 51 (7): 1133–6. PMID 6837459.
  8. ↑ Strasberg B, Amat-Y-Leon F, Dhingra RC, Palileo E, Swiryn S, Bauernfeind R; et al. (1981). “Natural history of chronic second-degree atrioventricular nodal block”. Circulation. 63 (5): 1043–9. PMID 7471363.
  9. ↑ LILLEHEI CW, SELLERS RD, BONNABEAU RC, ELIOT RS (1963). “CHRONIC POSTSURGICAL COMPLETE HEART BLOCK. WITH PARTICULAR REFERENCE TO PROGNOSIS, MANAGEMENT, AND A NEW P-WAVE PACEMAKER”. J Thorac Cardiovasc Surg. 46: 436–56. PMID 14074450.
  10. ↑ 10.0 10.1 Michaëlsson M, Jonzon A, Riesenfeld T (1995). “Isolated congenital complete atrioventricular block in adult life. A prospective study”. Circulation. 92 (3): 442–9. PMID 7634461.
  11. ↑ Moak JP, Barron KS, Hougen TJ, Wiles HB, Balaji S, Sreeram N; et al. (2001). “Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela”. J Am Coll Cardiol. 37 (1): 238–42. PMID 11153745.
  12. ↑ 12.0 12.1 Villain E, Coastedoat-Chalumeau N, Marijon E, Boudjemline Y, Piette JC, Bonnet D (2006). “Presentation and prognosis of complete atrioventricular block in childhood, according to maternal antibody status”. J Am Coll Cardiol. 48 (8): 1682–7. doi:10.1016/j.jacc.2006.07.034. PMID 17045907.
  13. ↑ Jaeggi ET, Hamilton RM, Silverman ED, Zamora SA, Hornberger LK (2002). “Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution’s experience of 30 years”. J Am Coll Cardiol. 39 (1): 130–7. PMID 11755298.
  14. ↑ Pinsky WW, Gillette PC, Garson A, McNamara DG (1982). “Diagnosis, management, and long-term results of patients with congenital complete atrioventricular block”. Pediatrics. 69 (6): 728–33. PMID 7079038.
  15. ↑ 15.0 15.1 Krongrad E (1978). “Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects”. Circulation. 57 (5): 867–70. PMID 346255.
  16. ↑ Weindling SN, Saul JP, Gamble WJ, Mayer JE, Wessel D, Walsh EP (1998). “Duration of complete atrioventricular block after congenital heart disease surgery”. Am J Cardiol. 82 (4): 525–7. PMID 9723647.
  17. ↑ Silka MJ, Manwill JR, Kron J, McAnulty JH (1990). “Bradycardia-mediated tachyarrhythmias in congenital heart disease and responses to chronic pacing at physiologic rates”. Am J Cardiol. 65 (7): 488–93. PMID 2305688.
  18. ↑ Stephenson EA, Casavant D, Tuzi J, Alexander ME, Law I, Serwer G; et al. (2003). “Efficacy of atrial antitachycardia pacing using the Medtronic AT500 pacemaker in patients with congenital heart disease”. Am J Cardiol. 92 (7): 871–6. PMID 14516898.
  19. ↑ Pfammatter JP, Paul T, Lehmann C, Kallfelz HC (1995). “Efficacy and proarrhythmia of oral sotalol in pediatric patients”. J Am Coll Cardiol. 26 (4): 1002–7. doi:10.1016/0735-1097(95)00268-3. PMID 7560592.
  20. ↑ Dewey RC, Capeless MA, Levy AM (1987). “Use of ambulatory electrocardiographic monitoring to identify high-risk patients with congenital complete heart block”. N Engl J Med. 316 (14): 835–9. doi:10.1056/NEJM198704023161403. PMID 3821827.
  21. ↑ 21.0 21.1 Sholler GF, Walsh EP (1989). “Congenital complete heart block in patients without anatomic cardiac defects”. Am Heart J. 118 (6): 1193–8. PMID 2480059.
  22. ↑ Cohen MI, Rhodes LA, Wernovsky G, Gaynor JW, Spray TL, Rychik J (2001). “Atrial pacing: an alternative treatment for protein-losing enteropathy after the Fontan operation”. J Thorac Cardiovasc Surg. 121 (3): 582–3. doi:10.1067/mtc.2001.110681. PMID 11241095.
  23. ↑ Villain E, Ouarda F, Beyler C, Sidi D, Abid F (2003). “[Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiopathy]”. Arch Mal Coeur Vaiss. 96 (5): 495–8. PMID 12838840.
  24. ↑ Gross GJ, Chiu CC, Hamilton RM, Kirsh JA, Stephenson EA (2006). “Natural history of postoperative heart block in congenital heart disease: implications for pacing intervention”. Heart Rhythm. 3 (5): 601–4. doi:10.1016/j.hrthm.2006.01.023. PMID 16648069.
  25. ↑ Banks MA, Jenson J, Kugler JD (2001). “Late development of atrioventricular block after congenital heart surgery in down syndrome”. Am J Cardiol. 88 (1): A7, 86–9. PMID 11423068.
  26. ↑ 26.0 26.1 Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). “ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society”. Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Priyamvada Singh, MBBS [2] Kristin Feeney, B.S. [3]