Tetralogy of fallot natural history complications and prognosis: Difference between revisions
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{{Tetralogy of fallot}} | {{Tetralogy of fallot}} | ||
{{CMG}}; '''Associate Editors-In-Chief:''' [[Priyamvada Singh| Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; | {{CMG}}; '''Associate Editors-In-Chief:''' {{Fs}}, [[Priyamvada Singh| Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu] | ||
==Overview== | ==Overview== | ||
The prognosis of patients with repaired tetralogy of | The [[prognosis]] of [[patients]] with repaired tetralogy of Fallot has improved over the years and these patients now have the potential to lead normal lives. As these [[patients]] grow, there may be leakage of the repaired [[pulmonic valve]]. There may be a persistent risk of [[sudden cardiac death]]. | ||
==Natural History== | ==Natural History== | ||
* If left untreated, the natural history of tetralogy of | * If left untreated, the natural history of tetralogy of Fallot results in progressive [[right ventricular hypertrophy]] and [[Right ventricle|right ventricular]] dilatation due to the increased [[resistance]] to [[Blood flow|flow]] into the [[Pulmonary circulation|pulmonary circuit]]. | ||
* The [[dilated cardiomyopathy]] progresses to [[right heart failure]], usually with accompanying [[left heart failure]]. | * The [[dilated cardiomyopathy]] progresses to [[right heart failure]], usually with accompanying [[left heart failure]]. | ||
==Complications== | ==Complications== | ||
Common [[complications]] of tetralogy of fallot include:<ref name="CheungKonstantinov2005">{{cite journal|last1=Cheung|first1=Michael M.H.|last2=Konstantinov|first2=Igor E.|last3=Redington|first3=Andrew N.|title=Late Complications of Repair of Tetralogy of Fallot and Indications for Pulmonary Valve Replacement|journal=Seminars in Thoracic and Cardiovascular Surgery|volume=17|issue=2|year=2005|pages=155–159|issn=10430679|doi=10.1053/j.semtcvs.2005.02.006}}</ref><ref name="GreggFoster2007">{{cite journal|last1=Gregg|first1=David|last2=Foster|first2=Elyse|title=Pulmonary insufficiency is the nexus of late complications in tetralogy of Fallot|journal=Current Cardiology Reports|volume=9|issue=4|year=2007|pages=315–322|issn=1523-3782|doi=10.1007/BF02938380}}</ref> | |||
* [[Delayed growth and development]] | * [[Delayed growth and development]] | ||
* Irregular heart rhythms ([[arrhythmias]]) | * Irregular heart rhythms ([[arrhythmias]]) | ||
| Line 17: | Line 19: | ||
* [[Stroke]] and [[embolic]] complications such as a [[brain abscess]] | * [[Stroke]] and [[embolic]] complications such as a [[brain abscess]] | ||
* [[Pulmonary embolism]] | * [[Pulmonary embolism]] | ||
* [[Sudden cardiac death]]: The worse the right ventricle dysfunction, the worse the risk for [[ventricular tachycardia]] and sudden death. | * [[Sudden cardiac death]]: The worse the [[right ventricle]] dysfunction, the worse the risk for [[ventricular tachycardia]] and [[sudden death]]. | ||
* [[Bacterial endocarditis]] | * [[Bacterial endocarditis]] | ||
* Reoperation for [[pulmonary stenosis]] | * Reoperation for [[pulmonary stenosis]] | ||
==Prognosis== | ==Prognosis== | ||
===Unrepaired Tetralogy of Fallot | ===Unrepaired Tetralogy of Fallot=== | ||
The survival for unrepaired tetralogy of | The [[Survival rate|survival]] for unrepaired tetralogy of Fallot is as follows:<ref name="pmid685856">{{cite journal |vauthors=Bertranou EG, Blackstone EH, Hazelrig JB, Turner ME, Kirklin JW |title=Life expectancy without surgery in tetralogy of Fallot |journal=Am. J. Cardiol. |volume=42 |issue=3 |pages=458–66 |date=September 1978 |pmid=685856 |doi=10.1016/0002-9149(78)90941-4 |url=}}</ref> | ||
:*75% at one year | :*75% at one year | ||
:*70% at two years | :*70% at two years | ||
| Line 35: | Line 38: | ||
===Repaired Tetralogy of Fallot=== | ===Repaired Tetralogy of Fallot=== | ||
* Patients with repaired tetralogy of | *[[Patient|Patients]] with repaired tetralogy of Fallot now have the potential to lead normal lives with continued excellent cardiac function. | ||
* Current techniques for total surgical repair greatly improve the hemodynamic function of the heart with tetralogy of | *Most survivors are in [[NYHA]] Class I heart failure. | ||
* Ninety percent of patients with total repair as [[infant]]s develop a progressively leaky pulmonary valve ([[pulmonary regurgitation|pulmonic insufficiency]] as the [[heart]] grows to its adult size. Patients also may have some degree of residual right ventricular outflow obstruction and damage to the conduction system of the [[heart]] from surgical corrections, causing conduction abnormalities on the [[EKG]] and/or [[arrhythmia]]s. | *Some [[patients]] have more pronounced [[Symptom|symptoms]] with exertion.<ref name="pmid20346688">{{cite journal |vauthors=Park CS, Lee JR, Lim HG, Kim WH, Kim YJ |title=The long-term result of total repair for tetralogy of Fallot |journal=Eur J Cardiothorac Surg |volume=38 |issue=3 |pages=311–7 |date=September 2010 |pmid=20346688 |doi=10.1016/j.ejcts.2010.02.030 |url=}}</ref><ref name="pmid21354809">{{cite journal |vauthors=Lindberg HL, Saatvedt K, Seem E, Hoel T, Birkeland S |title=Single-center 50 years' experience with surgical management of tetralogy of Fallot |journal=Eur J Cardiothorac Surg |volume=40 |issue=3 |pages=538–42 |date=September 2011 |pmid=21354809 |doi=10.1016/j.ejcts.2010.12.065 |url=}}</ref> | ||
* Tetralogy of | * Current techniques for total surgical repair greatly improve the [[Hemodynamics|hemodynamic]] function of the [[heart]] with tetralogy of Fallot but do not provide a lifetime correction of the defect. | ||
* Tetralogy of | * Ninety percent of patients with total repair as [[infant]]s develop a progressively leaky [[pulmonary valve]] ([[pulmonary regurgitation|pulmonic insufficiency]] as the [[heart]] grows to its adult size. [[Patients]] also may have some degree of residual [[right ventricular]] outflow obstruction and damage to the conduction system of the [[heart]] from [[Surgery operation|surgical]] corrections, causing conduction abnormalities on the [[EKG]] and/or [[arrhythmia]]s. | ||
* Tetralogy of Fallot patients are at risk for development of [[heart failure]]. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to assess and monitor these [[risks]] and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary. | |||
* Tetralogy of Fallot [[Patient|patients]] are at risk for [[sudden cardiac death]] with a 1% to 5% lifetime incidence. [[Risk factors]] for [[sudden cardiac death]] include: | |||
:*Older age at repair | :*Older age at repair | ||
:*Male sex | :*[[Male]] sex | ||
:*Advanced NY heart association class | :*Advanced NY heart association class | ||
:*Repair via atriotomy | :*Repair via atriotomy | ||
:*Two major electrocardiographic risk factors include [[complete heart block]] beyond the third post operative day and [[QRS]] duration > 18 milliseconds, and rapid development of [[QRS]] prolongation in the first 6 months after repair. | :*Two major [[electrocardiographic]] [[risk factors]] include [[complete heart block]] beyond the third post operative day and [[QRS]] duration > 18 milliseconds, and rapid development of [[QRS]] prolongation in the first 6 months after repair. | ||
*There is limited data regarding the benefit of [[automatic implantable cardiac defibrillator]] ([[AICD]]) implantation in these patients. | *There is limited data regarding the benefit of [[automatic implantable cardiac defibrillator]] ([[AICD]]) implantation in these patients. | ||
* [[Antibiotic]] [[prophylaxis]] is indicated during [[dental]] treatment in order to prevent [[infective endocarditis]]. | * [[Antibiotic]] [[prophylaxis]] is indicated during [[dental]] treatment in order to prevent [[infective endocarditis]]. | ||
Latest revision as of 17:54, 14 April 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Fahimeh Shojaei, M.D., Priyamvada Singh, M.B.B.S. [2]; Kristin Feeney, B.S. [3]
Overview
The prognosis of patients with repaired tetralogy of Fallot has improved over the years and these patients now have the potential to lead normal lives. As these patients grow, there may be leakage of the repaired pulmonic valve. There may be a persistent risk of sudden cardiac death.
Natural History
- If left untreated, the natural history of tetralogy of Fallot results in progressive right ventricular hypertrophy and right ventricular dilatation due to the increased resistance to flow into the pulmonary circuit.
- The dilated cardiomyopathy progresses to right heart failure, usually with accompanying left heart failure.
Complications
Common complications of tetralogy of fallot include:[1][2]
- Delayed growth and development
- Irregular heart rhythms (arrhythmias)
- Seizures as a result of hypoxia
- Stroke and embolic complications such as a brain abscess
- Pulmonary embolism
- Sudden cardiac death: The worse the right ventricle dysfunction, the worse the risk for ventricular tachycardia and sudden death.
- Bacterial endocarditis
- Reoperation for pulmonary stenosis
Prognosis
Unrepaired Tetralogy of Fallot
The survival for unrepaired tetralogy of Fallot is as follows:[3]
- 75% at one year
- 70% at two years
- 60% at four years
- 50% at six years
- 30% at ten years
- 10% at twenty years
- 5% at forty years
- If pulmonary atresia is present as well, survival is even poorer with only 50% of patients surviving to one year, and only 8% of patients surviving to 10 years.
Repaired Tetralogy of Fallot
- Patients with repaired tetralogy of Fallot now have the potential to lead normal lives with continued excellent cardiac function.
- Most survivors are in NYHA Class I heart failure.
- Some patients have more pronounced symptoms with exertion.[4][5]
- Current techniques for total surgical repair greatly improve the hemodynamic function of the heart with tetralogy of Fallot but do not provide a lifetime correction of the defect.
- Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary valve (pulmonic insufficiency as the heart grows to its adult size. Patients also may have some degree of residual right ventricular outflow obstruction and damage to the conduction system of the heart from surgical corrections, causing conduction abnormalities on the EKG and/or arrhythmias.
- Tetralogy of Fallot patients are at risk for development of heart failure. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to assess and monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary.
- Tetralogy of Fallot patients are at risk for sudden cardiac death with a 1% to 5% lifetime incidence. Risk factors for sudden cardiac death include:
- Older age at repair
- Male sex
- Advanced NY heart association class
- Repair via atriotomy
- Two major electrocardiographic risk factors include complete heart block beyond the third post operative day and QRS duration > 18 milliseconds, and rapid development of QRS prolongation in the first 6 months after repair.
- There is limited data regarding the benefit of automatic implantable cardiac defibrillator (AICD) implantation in these patients.
- Antibiotic prophylaxis is indicated during dental treatment in order to prevent infective endocarditis.
References
- ↑ Cheung, Michael M.H.; Konstantinov, Igor E.; Redington, Andrew N. (2005). "Late Complications of Repair of Tetralogy of Fallot and Indications for Pulmonary Valve Replacement". Seminars in Thoracic and Cardiovascular Surgery. 17 (2): 155–159. doi:10.1053/j.semtcvs.2005.02.006. ISSN 1043-0679.
- ↑ Gregg, David; Foster, Elyse (2007). "Pulmonary insufficiency is the nexus of late complications in tetralogy of Fallot". Current Cardiology Reports. 9 (4): 315–322. doi:10.1007/BF02938380. ISSN 1523-3782.
- ↑ Bertranou EG, Blackstone EH, Hazelrig JB, Turner ME, Kirklin JW (September 1978). "Life expectancy without surgery in tetralogy of Fallot". Am. J. Cardiol. 42 (3): 458–66. doi:10.1016/0002-9149(78)90941-4. PMID 685856.
- ↑ Park CS, Lee JR, Lim HG, Kim WH, Kim YJ (September 2010). "The long-term result of total repair for tetralogy of Fallot". Eur J Cardiothorac Surg. 38 (3): 311–7. doi:10.1016/j.ejcts.2010.02.030. PMID 20346688.
- ↑ Lindberg HL, Saatvedt K, Seem E, Hoel T, Birkeland S (September 2011). "Single-center 50 years' experience with surgical management of tetralogy of Fallot". Eur J Cardiothorac Surg. 40 (3): 538–42. doi:10.1016/j.ejcts.2010.12.065. PMID 21354809.