What are the latest developments in tetralogy of Fallot treatment. How does tetralogy of Fallot affect patients long-term. What is the prognosis for adults with repaired tetralogy of Fallot. How are ventricular arrhythmias managed in tetralogy of Fallot patients. What genetic factors contribute to tetralogy of Fallot risk. How does tetralogy of Fallot impact quality of life in adulthood. What are the current mortality rates for tetralogy of Fallot patients.

Understanding Tetralogy of Fallot: A Complex Congenital Heart Defect

Tetralogy of Fallot (TOF) is a congenital heart defect characterized by four anatomical abnormalities. These include ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy, and an overriding aorta. This complex cardiac condition requires early intervention and lifelong management to ensure optimal outcomes for patients.

The anatomical complexities of TOF can vary between patients, influencing treatment approaches and long-term prognosis. Understanding the nuances of this condition is crucial for healthcare providers and patients alike.

Key Anatomical Features of Tetralogy of Fallot

• Ventricular septal defect (VSD)
• Pulmonary stenosis
• Right ventricular hypertrophy
• Overriding aorta

These anatomical abnormalities work in concert to create a complex circulatory issue that requires careful management and often surgical intervention.

Current Treatment Approaches for Tetralogy of Fallot

Treatment for TOF has evolved significantly over the years, with surgical repair remaining the cornerstone of management. Early intervention is crucial to prevent complications and improve long-term outcomes.

Surgical Repair Techniques

Surgical repair of TOF typically involves closing the ventricular septal defect and relieving the obstruction to blood flow from the right ventricle to the pulmonary arteries. This can be achieved through various techniques, including:

• Transatrial-transpulmonary repair
• Right ventriculotomy approach
• Staged repair (initial palliative shunt followed by complete repair)

The choice of surgical technique depends on the patient’s specific anatomy, age, and overall health status. Advancements in surgical techniques have significantly improved outcomes for TOF patients.

Catheter-Based Interventions

In addition to surgical repair, catheter-based interventions play an important role in managing TOF patients, particularly in addressing residual or recurrent issues post-repair. These may include:

• Pulmonary valve replacement
• Balloon dilation of pulmonary arteries
• Stenting of pulmonary arteries

These minimally invasive procedures can help improve pulmonary blood flow and right ventricular function, potentially delaying the need for additional open-heart surgeries.

Long-Term Outcomes and Prognosis for Tetralogy of Fallot Patients

The prognosis for patients with TOF has improved dramatically over the past few decades. However, long-term follow-up studies reveal that these patients face several challenges as they transition into adulthood.

Survival Rates and Life Expectancy

Recent studies have shown encouraging survival rates for TOF patients. A study by Dennis et al. (2017) found that adults with repaired TOF had low mortality rates up to middle age. However, morbidity remains a significant concern, with many patients experiencing complications that impact their quality of life.

The standardized mortality ratio for adult congenital heart disease patients, including those with TOF, has shown improvement over time. Wu et al. (2018) reported a decreasing trend in mortality rates from 2000 to 2014, indicating advancements in management strategies.

Common Long-Term Complications

Despite improved survival rates, TOF patients often face several long-term complications:

• Pulmonary regurgitation
• Right ventricular dysfunction
• Arrhythmias
• Exercise intolerance
• Heart failure

These complications underscore the importance of lifelong cardiac monitoring and management for TOF patients.

Managing Ventricular Arrhythmias in Tetralogy of Fallot

Ventricular arrhythmias pose a significant risk for sudden cardiac death in TOF patients. Effective management of these arrhythmias is crucial for improving long-term outcomes.

Risk Stratification

Identifying patients at high risk for ventricular arrhythmias is essential. Diller et al. (2012) found that left ventricular longitudinal function could predict life-threatening ventricular arrhythmia and death in adults with repaired TOF. This highlights the importance of comprehensive cardiac imaging and functional assessment in risk stratification.

Treatment Strategies

Management of ventricular arrhythmias in TOF patients may include:

• Antiarrhythmic medications
• Catheter ablation
• Implantable cardioverter-defibrillators (ICDs)
• Pulmonary valve replacement (to address underlying hemodynamic issues)

The choice of treatment depends on the patient’s specific arrhythmia pattern, hemodynamic status, and overall risk profile.

Genetic Factors Contributing to Tetralogy of Fallot

Understanding the genetic underpinnings of TOF is crucial for risk assessment and potential targeted therapies. Several genetic variants have been associated with an increased risk of TOF.

MTHFR Gene Variants

The methylenetetrahydrofolate reductase (MTHFR) gene has been implicated in TOF risk. Marinho et al. (2009) found that the C677T variant of the MTHFR gene may act as a susceptibility gene for TOF. This association highlights the potential role of folate metabolism in the development of congenital heart defects.

VEGF Gene Polymorphisms

Li et al. (2015) reported that vascular endothelial growth factor (VEGF) gene polymorphisms are associated with TOF risk. This finding suggests that genetic variations affecting vascular development may contribute to the complex etiology of TOF.

Comprehensive Genetic Analysis

Rauch et al. (2010) conducted a comprehensive genotype-phenotype analysis in 230 TOF patients, revealing a complex genetic landscape. Their findings underscore the importance of thorough genetic evaluation in TOF patients, which may inform prognosis and guide management strategies.

Quality of Life Considerations for Adults with Tetralogy of Fallot

As survival rates for TOF patients continue to improve, there is an increasing focus on quality of life outcomes in adulthood. Understanding the challenges faced by adult TOF survivors is crucial for providing comprehensive care.

Exercise Capacity and Functional Status

Exercise intolerance is a common issue among adult TOF patients. Dluzniewska et al. (2018) investigated the relationship between ventricular function, volumes, and exercise capacity in adults with repaired TOF. Their findings highlight the complex interplay between cardiac function and physical performance in these patients.

Psychosocial Aspects

Living with a chronic cardiac condition can have significant psychosocial impacts. Adult TOF survivors may face challenges related to:

• Employment and career planning
• Family planning and pregnancy
• Mental health and anxiety related to their cardiac condition
• Social integration and relationships

Addressing these psychosocial aspects is an essential component of comprehensive care for adult TOF patients.

Emerging Trends and Future Directions in Tetralogy of Fallot Management

The field of TOF management continues to evolve, with several promising areas of research and development on the horizon.

Advanced Imaging Techniques

Cardiac MRI and MR angiography have become invaluable tools in the assessment of complex TOF cases. Boechat et al. (2005) highlighted the role of these advanced imaging modalities in evaluating TOF patients, particularly those with pulmonary atresia. Continued refinement of imaging techniques promises to improve diagnostic accuracy and guide treatment decisions.

Novel Biomarkers

Bhardwaj et al. (2017) explored the use of basic arterial blood gas biomarkers as predictors of mortality in TOF patients. The development of reliable biomarkers could enhance risk stratification and guide personalized treatment approaches.

Pharmacological Interventions

While surgical and catheter-based interventions remain the mainstay of TOF treatment, there is ongoing research into pharmacological therapies. Gabriele et al. (2018) discussed the potential role of pharmacological therapy in preventing sudden death in surgically treated TOF patients. This area of research may lead to new strategies for managing long-term complications.

Tissue Engineering and Regenerative Medicine

Advances in tissue engineering and regenerative medicine hold promise for developing novel treatment approaches for TOF. These may include:

• Bioengineered heart valves
• Cell-based therapies for myocardial regeneration
• 3D-printed cardiac patches for septal defect repair

While still in early stages, these innovative approaches could revolutionize TOF treatment in the future.

In conclusion, the management of tetralogy of Fallot has seen remarkable progress over the past decades, with significant improvements in survival rates and quality of life for patients. However, challenges remain, particularly in managing long-term complications and optimizing outcomes for adult survivors. Ongoing research in genetics, imaging, and novel therapeutic approaches offers hope for continued advancements in TOF care. As our understanding of this complex condition deepens, we can anticipate further refinements in treatment strategies and improved long-term outcomes for individuals living with tetralogy of Fallot.

Tetralogy of Fallot (TOF) in Adults: Practice Essentials, Background, Anatomy

1. Refaat MM, Ballout J, Mansour M. Ablation of atrial fibrillation in patients with congenital heart disease. Arrhythm Electrophysiol Rev. 2017 Dec. 6(4):191-4. [QxMD MEDLINE Link]. [Full Text].

2. Maury P, Sacher F, Rollin A, et al. Ventricular arrhythmias and sudden death in tetralogy of Fallot. Arch Cardiovasc Dis. 2017 May. 110(5):354-62. [QxMD MEDLINE Link]. [Full Text].

3. Fraser CD, Bacha EA, Comas J, Sano S, Sarris GE, Tsang VT. Tetralogy of Fallot. Semin Thorac Cardiovasc Surg. 2015 Summer. 27(2):189-204. [QxMD MEDLINE Link].

4. Bhardwaj V, Kapoor PM, Irpachi K, Ladha S, Chowdhury UK. Basic arterial blood gas biomarkers as a predictor of mortality in tetralogy of Fallot patients. Ann Card Anaesth. 2017 Jan-Mar. 20 (1):67-71. [QxMD MEDLINE Link].

5. Boechat MI, Ratib O, Williams PL, Gomes AS, Child JS, Allada V. Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 2005 Nov-Dec. 25(6):1535-46. [QxMD MEDLINE Link].

6. Gabriele B, Maurizio B, Marco B. Does pharmacological therapy still play a role in preventing sudden death in surgically treated tetralogy of Fallot?. Mini Rev Med Chem. 2018. 18(6):490-4. [QxMD MEDLINE Link].

7. Faccini A, Micheletti A, Negura DG, et al. Heart failure in grown-up congenital heart disease. Minerva Cardioangiol. 2018 Jun. 66(3):329-36. [QxMD MEDLINE Link].

8. Diaz-Frias J, Guillaume M. Tetralogy of Fallot. StatPearls [Internet]. 2021 Jan. [QxMD MEDLINE Link]. [Full Text].

9. Anderson RH, Weinberg PM. The clinical anatomy of tetralogy of Fallot. Cardiol Young. 2005 Feb. 15 suppl 1:38-47. [QxMD MEDLINE Link].

10. Sarris GE. Tetralogy surgery – back to Baltimore 70 years later: Melbourne Heritage and Group tribute to Juan Comas. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan. 20:84-8. [QxMD MEDLINE Link].

11. Karl TR, Stocker C. Tetralogy of fallot and its variants. Pediatr Crit Care Med. 2016 Aug. 17(8 suppl 1):S330-6. [QxMD MEDLINE Link].

12. Marinho C, Alho I, Guerra A, Rego C, Areias J, Bicho M. The methylenetetrahydrofolate reductase gene variant (C677T) as a susceptibility gene for tetralogy of Fallot. Rev Port Cardiol. 2009 Jul-Aug. 28(7-8):809-12. [QxMD MEDLINE Link].

13. Lee CN, Su YN, Cheng WF, et al. Association of the C677T methylenetetrahydrofolate reductase mutation with congenital heart diseases. Acta Obstet Gynecol Scand. 2005 Dec. 84(12):1134-40. [QxMD MEDLINE Link].

14. Li X, Liu CL, Li XX, Li QC, Ma LM, Liu GL. VEGF gene polymorphisms are associated with risk of tetralogy of Fallot. Med Sci Monit. 2015 Nov 12. 21:3474-82. [QxMD MEDLINE Link].

15. Anabtawi A, Mondragon J, Dodendorf D, Laskey WK. Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood. Open Heart. 2017. 4(2):e000690. [QxMD MEDLINE Link]. [Full Text].

16. Wu MH, Lu CW, Chen HC, Kao FY, Huang SK. Adult congenital heart disease in a nationwide population 2000-2014: epidemiological trends, arrhythmia, and standardized mortality ratio. J Am Heart Assoc. 2018 Feb 8. 7(4):[QxMD MEDLINE Link]. [Full Text].

17. Dennis M, Moore B, Kotchetkova I, Pressley L, Cordina R, Celermajer DS. Adults with repaired tetralogy: low mortality but high morbidity up to middle age. Open Heart. 2017. 4(1):e000564. [QxMD MEDLINE Link]. [Full Text].

18. Rauch R, Hofbeck M, Zweier C, et al. Comprehensive genotype-phenotype analysis in 230 patients with tetralogy of Fallot. J Med Genet. 2010 May. 47(5):321-31. [QxMD MEDLINE Link].

19. Pillutla P, Shetty KD, Foster E. Mortality associated with adult congenital heart disease: trends in the US population from 1979 to 2005. Am Heart J. 2009 Nov. 158(5):874-9. [QxMD MEDLINE Link].

20. Diller GP, Kempny A, Liodakis E, et al. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of fallot. Circulation. 2012 May 22. 125(20):2440-6. [QxMD MEDLINE Link].

21. Chiu SN, Wu MH, Su MJ, et al. Coexisting mutations/polymorphisms of the long QT syndrome genes in patients with repaired tetralogy of Fallot are associated with the risks of life-threatening events. Hum Genet. 2012 Aug. 131(8):1295-304. [QxMD MEDLINE Link].

22. Dluzniewska N, Podolec P, Miszalski-Jamka T, et al. Effect of ventricular function and volumes on exercise capacity in adults with repaired tetralogy of Fallot. Indian Heart J. 2018 Jan – Feb. 70(1):87-92. [QxMD MEDLINE Link]. [Full Text].

23. Vehmeijer JT, Koyak Z, Bokma JP, et al. Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts?. Europace. 2018 Jun 1. 20(FI1):f122-8. [QxMD MEDLINE Link].

24. Gaydos SS, Varga-Szemes A, Judd RN, Suranyi P, Gregg D. Imaging in adult congenital heart disease. J Thorac Imaging. 2017 Jul. 32(4):205-16. [QxMD MEDLINE Link].

25. Bokma JP, de Wilde KC, Vliegen HW, et al. Value of cardiovascular magnetic resonance imaging in noninvasive risk stratification in tetralogy of Fallot. JAMA Cardiol. 2017 Jun 1. 2(6):678-83. [QxMD MEDLINE Link]. [Full Text].

26. Scalone G, Gomez-Monterrosas O, Fiszer R, Szkutnik M, Galeczka M, Bialkowski J. Combined strategy of Waterston shunt percutaneous occlusion and medical treatment with sildenafil for management of pulmonary hypertension in an adult patient with corrected tetralogy of Fallot. Postepy Kardiol Interwencyjnej. 2017. 13(3):277-8. [QxMD MEDLINE Link]. [Full Text].

27. com”>McRae ME, Coleman B, Atz TW, Kelechi TJ. Patient outcomes after transcatheter and surgical pulmonary valve replacement for pulmonary regurgitation in patients with repaired tetralogy of Fallot: A quasi-meta-analysis. Eur J Cardiovasc Nurs. 2017 Aug. 16(6):539-53. [QxMD MEDLINE Link].

28. Bhagra CJ, Hickey EJ, Van De Bruaene A, Roche SL, Horlick EM, Wald RM. Pulmonary valve procedures late after repair of tetralogy of Fallot: current perspectives and contemporary approaches to management. Can J Cardiol. 2017 Sep. 33(9):1138-49. [QxMD MEDLINE Link].

29. Pragt H, van Melle JP, Javadikasgari H, et al. Mechanical valves in the pulmonary position: an international retrospective analysis. J Thorac Cardiovasc Surg. 2017 Oct. 154(4):1371-8.e1. [QxMD MEDLINE Link]. [Full Text].

30. Therrien J, Marx GR, Gatzoulis MA. Late problems in tetralogy of Fallot–recognition, management, and prevention. Cardiol Clin. 2002 Aug. 20(3):395-404. [QxMD MEDLINE Link].

31. Ezzat VA, Ryan MJ, O’Leary J, et al. Radiofrequency ablation of atrial tachyarrhythmias in adults with tetralogy of Fallot – predictors of success and outcome. Cardiol Young. 2017 Mar. 27(2):284-93. [QxMD MEDLINE Link].

32. Davidson J, Tong S, Hancock H, Hauck A, da Cruz E, Kaufman J. Prospective validation of the vasoactive-inotropic score and correlation to short-term outcomes in neonates and infants after cardiothoracic surgery. Intensive Care Med. 2012 Jul. 38(7):1184-90. [QxMD MEDLINE Link].

33. Menting ME, van den Bosch AE, McGhie JS, et al. Assessment of ventricular function in adults with repaired Tetralogy of Fallot using myocardial deformation imaging. Eur Heart J Cardiovasc Imaging. 2015 Dec. 16(12):1347-57. [QxMD MEDLINE Link].

34. Lindsey CW, Parks WJ, Kogon BE, Sallee D 3rd, Mahle WT. Pulmonary valve replacement after tetralogy of Fallot repair in preadolescent patients. Ann Thorac Surg. 2010 Jan. 89(1):147-51. [QxMD MEDLINE Link].

35. Bokma JP, Geva T, Sleeper LA, et al. A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart. 2018 May. 104(9):738-44. [QxMD MEDLINE Link].

36. Heng EL, Gatzoulis MA, Uebing A, et al. Immediate and midterm cardiac remodeling after surgical pulmonary Vvalve replacement in adults with repaired tetralogy of Fallot: a prospective cardiovascular magnetic resonance and clinical study. Circulation. 2017 Oct 31. 136(18):1703-13. [QxMD MEDLINE Link]. [Full Text].

37. Mitropoulos FM, Kanakis MA, Ntellos C, et al. Pulmonary valve replacement in patients with corrected tetralogy of Fallot. J Cardiovasc Thorac Res. 2017. 9(2):71-7. [QxMD MEDLINE Link]. [Full Text].

38. Caruana M, Grech V. A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta. Congenit Heart Dis. 2017 May. 12(3):301-8. [QxMD MEDLINE Link].

39. Kanter KR, Kogon BE, Kirshbom PM, Carlock PR. Symptomatic neonatal tetralogy of Fallot: repair or shunt?. Ann Thorac Surg. 2010 Mar. 89(3):858-63. [QxMD MEDLINE Link].

40. Tsang FH, Li X, Cheung YF, Chau KT, Cheng LC. Pulmonary valve replacement after surgical repair of tetralogy of Fallot. Hong Kong Med J. 2010 Feb. 16(1):26-30. [QxMD MEDLINE Link].

41. Alkashkari W, Alsubei A, Hijazi ZM. Transcatheter pulmonary valve replacement: current state of art. Curr Cardiol Rep. 2018 Mar 15. 20(4):27. [QxMD MEDLINE Link].

42. Jones MI, Qureshi SA. Recent advances in transcatheter management of pulmonary regurgitation after surgical repair of tetralogy of Fallot. F1000Res. 2018. 7:[QxMD MEDLINE Link]. [Full Text].

43. Plessis J, Hascoet S, Baruteau A, et al. Edwards SAPIEN transcatheter pulmonary valve implantation: results from a French registry. JACC Cardiovasc Interv. 2018 Oct 8. 11(19):1909-16. [QxMD MEDLINE Link]. [Full Text].

44. [Guideline] Cotts TB. 2018 AHA/ACC guideline for adults with congenital heart disease. American College of Cardiology. Available at https://www.acc.org/latest-in-cardiology/ten-points-to-remember/2018/08/13/16/26/2018-aha-acc-guideline-for-the-management-of-achd. August 16, 2018; Accessed: October 25, 2018.

45. Senzaki H, Ishido H, Iwamoto Y, et al. Sedation of hypercyanotic spells in a neonate with tetralogy of Fallot using dexmedetomidine. J Pediatr (Rio J). 2008 Jul-Aug. 84(4):377-80. [QxMD MEDLINE Link].

46. Rodan L, McCrindle BW, Manlhiot C, et al. Stroke recurrence in children with congenital heart disease. Ann Neurol. 2012 Jul. 72(1):103-11. [QxMD MEDLINE Link].

47. Tanel RE. ECGs in the ED. Pediatr Emerg Care. 2007 Jun. 23(6):428-9. [QxMD MEDLINE Link].

48. Witte KK, Pepper CB, Cowan JC, Thomson JD, English KM, Blackburn ME. Implantable cardioverter-defibrillator therapy in adult patients with tetralogy of Fallot. Europace. 2008 Aug. 10(8):926-30. [QxMD MEDLINE Link].

49. webmd.com”>Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008 Jan 22. 117(3):363-70. [QxMD MEDLINE Link].

50. Chrysostomou C, Sanchez De Toledo J, Avolio T, et al. Dexmedetomidine use in a pediatric cardiac intensive care unit: can we use it in infants after cardiac surgery?. Pediatr Crit Care Med. 2009 Nov. 10(6):654-60. [QxMD MEDLINE Link].

51. Shepard SM, Tejman-Yarden S, Khanna S, Davis CK, Batra AS. Dexmedetomidine-related atrial standstill and loss of capture in a pediatric patient after congenital heart surgery. Crit Care Med. 2011 Jan. 39(1):187-9. [QxMD MEDLINE Link].

52. Al Habib HF, Jacobs JP, Mavroudis C, et al. Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg. 2010 Sep. 90(3):813-9; discussion 819-20. [QxMD MEDLINE Link].

53. Gustafson RA, Murray GF, Warden HE, Hill RC, Rozar GE Jr. Early primary repair of tetralogy of Fallot. Ann Thorac Surg. 1988 Mar. 45(3):235-41. [QxMD MEDLINE Link].

54. Park CS, Lee JR, Lim HG, Kim WH, Kim YJ. The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg. 2010 Sep. 38(3):311-7. [QxMD MEDLINE Link].

55. Robinson JD, Rathod RH, Brown DW, et al. The evolving role of intraoperative balloon pulmonary valvuloplasty in valve-sparing repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2011 Dec. 142(6):1367-73. [QxMD MEDLINE Link].

56. Pacifico AD, Kirklin JK, Colvin EV, McConnell ME, Kirklin JW. Transatrial-transpulmonary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg. 1990 Jan. 2(1):76-82. [QxMD MEDLINE Link].

57. Yao Q, Hu XH, Shen QL, et al. Differential effect of the ratio of right ventricular volume to left ventricular volume in children with repaired tetralogy of Fallot. Cardiology. 2016. 133(3):135-40. [QxMD MEDLINE Link].

58. Aboulhosn J, Child JS. Management after childhood repair of tetralogy of fallot. Curr Treat Options Cardiovasc Med. 2006 Dec. 8(6):474-83. [QxMD MEDLINE Link].

59. Devore GR, Polanko B. Tomographic ultrasound imaging of the fetal heart: a new technique for identifying normal and abnormal cardiac anatomy. J Ultrasound Med. 2005 Dec. 24(12):1685-96. [QxMD MEDLINE Link].

60. Duro RP, Moura C, Leite-Moreira A. Anatomophysiologic basis of tetralogy of Fallot and its clinical implications. Rev Port Cardiol. 2010 Apr. 29(4):591-630. [QxMD MEDLINE Link].

61. Fox D, Devendra GP, Hart SA, Krasuski RA. When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot. Cleve Clin J Med. 2010 Nov. 77(11):821-8. [QxMD MEDLINE Link].

62. He GW. Current strategy of repair of tetralogy of Fallot in children and adults: emphasis on a new technique to create a monocusp-patch for reconstruction of the right ventricular outflow tract. J Card Surg. 2008 Nov-Dec. 23(6):592-9. [QxMD MEDLINE Link].

63. Horer J, Friebe J, Schreiber C, et al. Correction of tetralogy of Fallot and of pulmonary atresia with ventricular septal defect in adults. Ann Thorac Surg. 2005 Dec. 80(6):2285-91. [QxMD MEDLINE Link].

64. Hovels-Gurich HH, Konrad K, Skorzenski D, et al. Long-term behavior and quality of life after corrective cardiac surgery in infancy for tetralogy of Fallot or ventricular septal defect. Pediatr Cardiol. 2007 Sep-Oct. 28 (5):346-54. [QxMD MEDLINE Link].

65. Kantorova A, Zbieranek K, Sauer H, Lilje C, Haun C, Hraska V. Primary early correction of tetralogy of Fallot irrespective of age. Cardiol Young. 2008 Apr. 18(2):153-7. [QxMD MEDLINE Link].

66. Laidman J. Fluconazole linked to increased risk for rare birth defect. Medscape Medical News from WebMD. August 28, 2013. Available at http://www.medscape.com/viewarticle/810149. Accessed: September 5, 2013.

67. Miatton M, De Wolf D, Francois K, Thiery E, Vingerhoets G. Intellectual, neuropsychological, and behavioral functioning in children with tetralogy of Fallot. J Thorac Cardiovasc Surg. 2007 Feb. 133(2):449-55. [QxMD MEDLINE Link]. [Full Text].

68. Molgaard-Nielsen D, Pasternak B, Hviid A. Use of oral fluconazole during pregnancy and the risk of birth defects. N Engl J Med. 2013 Aug 29. 369(9):830-9. [QxMD MEDLINE Link]. [Full Text].

69. Patel CR, Agamanolis DP, Stewart JW. Prenatal diagnosis of tetralogy of Fallot with obstructed supracardiac totally anomalous pulmonary venous connection. Cardiol Young. 2005 Dec. 15(6):656-9. [QxMD MEDLINE Link].

70. Redington AN. Determinants and assessment of pulmonary regurgitation in tetralogy of Fallot: practice and pitfalls. Cardiol Clin. 2006 Nov. 24(4):631-9, vii. [QxMD MEDLINE Link].

71. Sakamoto T, Nagase Y, Hasegawa H, Shin’oka T, Tomimatsu H, Kurosawa H. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg. 2005 Dec. 130(6):1717-8. [QxMD MEDLINE Link]. [Full Text].

72. Srivastava S, Salem Y, Chatterjee S, et al. Echocardiographic myocardial deformation evaluation of right ventricular function in comparison with CMRI in repaired tetralogy of Fallot: a cross-sectional and longitudinal validation study. Echocardiography. 2013 Feb. 30(2):196-202. [QxMD MEDLINE Link].

73. Starr JP. Tetralogy of Fallot: yesterday and today. World J Surg. 2010 Apr. 34(4):658-68. [QxMD MEDLINE Link].

74. Aguayo-Gomez A, Arteaga-Vazquez J, Svyryd Y, et al. Identification of copy number variations in isolated tetralogy of Fallot. Pediatr Cardiol. 2015 Dec. 36(8):1642-6. [QxMD MEDLINE Link].

75. Shahanavaz S, Qureshi AM, Levi DS, et al. Transcatheter pulmonary valve replacement with the Melody valve in small diameter expandable right ventricular outflow tract conduits. JACC Cardiovasc Interv. 2018 Mar 26. 11(6):554-64. [QxMD MEDLINE Link]. [Full Text].

76. Naidu P, Grigg L, Zentner D. Mortality in adults with congenital heart disease. Int J Cardiol. 2017 Oct 15. 245:125-30. [QxMD MEDLINE Link].

77. Apostolopoulou SC, Vagenakis G, Rammos S. Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature. Cardiol Young. 2017 Nov. 2(9):1861-4. [QxMD MEDLINE Link].

78. [Guideline] Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 2. 73(12):e81-e192. [QxMD MEDLINE Link]. [Full Text].

79. Kwak JG, Kim WH, Kim ER, Lim JH, Min J. One-year follow-up after tetralogy of Fallot total repair preserving pulmonary valve and avoiding right ventriculotomy. Circ J. 2018 Nov 24. 82(12):3064-8. [QxMD MEDLINE Link].

Prediction of prognosis in patients with tetralogy of Fallot based on deep learning imaging analysis

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Congenital heart disease

Original research

Prediction of prognosis in patients with tetralogy of Fallot based on deep learning imaging analysis

1. http://orcid.org/0000-0003-3050-5248Gerhard Paul Diller1,
2. http://orcid.org/0000-0002-2836-6333Stefan Orwat1,
3. Julius Vahle1,
4. Ulrike M M Bauer2,3,
5. Aleksandra Urban3,
6. Samir Sarikouch5,
7. Felix Berger5,6,
8. Philipp Beerbaum7,
9. Helmut Baumgartner1
10. German Competence Network for Congenital Heart Defects Investigators

1. ¹
   Department of Cardiology III – Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany
   
2. ²
   Competence Network for Congenital Heart Defects, DZHK (German Centre for Cardiovascular Research), Berlin, Germany
   
3. ³
   National Register for Congenital Heart Defects, DZHK (German Centre for Cardiovascular Research), Berlin, Germany
   
4. ⁴
   Department of Heart- Thoracic- Transplantation- and Vascular Surgery, Hannover Medical School, Hannover, Germany
   
5. ⁵
   Department of Congenital Heart Disease – Pediatric Cardiology, German Heart Institute Berlin, Augustenburger Platz 1, Berlin, Germany
   
6. ⁶
   DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, Augustenburger Platz 1, Berlin, Germany
   
7. ⁷
   Department of Pediatric Cardiology and Pediatric Intensive Care, Hannover Medical School, Hannover Medical School, Hannover, Germany
   
1. Correspondence to
   Professor Gerhard Paul Diller, Adult Congenital and Valvular Heart Disease Center, Department of Cardiology and Angiology, University Hospital Muenster, Muenster 48149, Germany; Gerhard. Diller{at}ukmuenster.de

Abstract

Objective To assess the utility of machine learning algorithms for automatically estimating prognosis in patients with repaired tetralogy of Fallot (ToF) using cardiac magnetic resonance (CMR).

Methods We included 372 patients with ToF who had undergone CMR imaging as part of a nationwide prospective study. Cine loops were retrieved and subjected to automatic deep learning (DL)-based image analysis, trained on independent, local CMR data, to derive measures of cardiac dimensions and function. This information was combined with established clinical parameters and ECG markers of prognosis.

Results Over a median follow-up period of 10 years, 23 patients experienced an endpoint of death/aborted cardiac arrest or documented ventricular tachycardia (defined as >3 documented consecutive ventricular beats). On univariate Cox analysis, various DL parameters, including right atrial median area (HR 1. 11/cm², p=0.003) and right ventricular long-axis strain (HR 0.80/%, p=0.009) emerged as significant predictors of outcome. DL parameters were related to adverse outcome independently of left and right ventricular ejection fraction and peak oxygen uptake (p<0.05 for all). A composite score of enlarged right atrial area and depressed right ventricular longitudinal function identified a ToF subgroup at significantly increased risk of adverse outcome (HR 2.1/unit, p=0.007).

Conclusions We present data on the utility of machine learning algorithms trained on external imaging datasets to automatically estimate prognosis in patients with ToF. Due to the automated analysis process these two-dimensional-based algorithms may serve as surrogates for labour-intensive manually attained imaging parameters in patients with ToF.

• congenital heart disease
• tetralogy of Fallot
• cardiac magnetic resonance (CMR) imaging
• advanced cardiac imaging

http://dx. doi.org/10.1136/heartjnl-2019-315962

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• congenital heart disease
• tetralogy of Fallot
• cardiac magnetic resonance (CMR) imaging
• advanced cardiac imaging

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Footnotes

• GPD and SO contributed equally.

• Collaborators German Competence Network for Congenital Heart Defects Investigators: Gunter Kerst, Jaime F. Vazquez-Jimenez, Dimitrios Gkalpakiotis, Andrea Schedifka, Gernot Buheitel, Joachim Streble, Kai Thorsten Laser, Eugen Sandica, Burkhard Trusen, Felix Berger, Oliver Miera, Stanislav Ovroutski, Björn Peters, Katharina Schmitt, Stephan Schubert, Joachim Photiadis, Felix Berger, Bernd Opgen-Rhein, Katja Weiss, Christoph Berns, Carl-Christian Blumenthal-Barby, Thomas Boeckel, Guido Haverkämper, Andreas Kästner, Heike Koch, Christian Köpcke, Frank Streichan, Jens Timme, Birgit Franzbach, Gabriela Senft, Frank Beyer, Klaus Winter, Johannes Breuer, Bahman Esmailzadeh, Martin Schneider, Boulos Asfour, Jens Bahlmann, Eberhard Griese, Trong Phi Lê, Joachim Hebe, Jan-Hendrik Nürnberg, Annette Magsaam, Ronald Müller, Ludger Potthoff, Renate Voigt, Tim Krüger, Hubert Gerleve, Ulrich Kleideiter, Dirk Schneider-Kulla, Jürgen Krülls-Münch, Elmo Feil, Thomas Menke, Martin Lehn, Antje Heilmann, Helge Tomczak, Otto N. Krogmann, Gleb Tarusinov, Michael Scheid, Ertan Mayatepek, Frank Pillekamp, Artur Lichtenberg, Christiane Terpeluk, Bruno Kolterer, Sven Dittrich, Ulrike Gundlach, Robert Cesnjevar, Ulrich Neudorf, Geert Morf, Anoosh Esmaeili, Stephan Backhoff, Brigitte Stiller, Friedhelm Beyersdorf, Johannes Kroll, Nicole Häffner, Jannos Siaplaouras, Antje Masri-Zada, Christian Jux, Andreas Böning, Hakan Akintürk, Thomas Paul, Matthias Sigler, Theodor Tirilomis, Gabriele Schürer, Johannes Hartmann, Ralph Grabitz, Uta Liebaug, Claudius Rotzsch, Rainer Kozlik-Feldmann, Jörg Sachweh, Arlindo Riso, Stefan Renz, Andreas Schemm, Bernd Friedrich, Otmar Schlobohm, Philipp Beerbaum, Dietmar Böthig, Alexander Horke, Johann Bauersachs, Mechthild Westhoff-Bleck, Matthias Gorenflo, Matthias Karck, Tsvetomir Loukanov, Hermann Schrüfer, Martin Wilken, Hashim Abdul-Khaliq, Tanja Rädle-Hurst, Axel Rentzsch, Hans-Joachim Schäfers, Hagen Reichert, Thomas Kriebel, Arnulf Boysen, Anselm Uebing, Joachim Thomas Cremer, Jens Scheewe, Regina Buchholz-Berdau, Peter Möller, Wolfgang Ram, Konrad Brockmeier, Gerardus B. W. E. Bennink, Alex Gillor, Tim Niehues, Peter Terhoeven, Steffen Leidig, Ingo Dähnert, Peter Kinzel, Martin Kostelka, Liane Kändler, Martin Bethge, Stefan Köster, Christoph Schröder, Jens Karstedt, Uwe Seitz, Christoph Kampmann, Christian-Friedrich Vahl, Frank Stahl, Mojtaba Abedini, Joachim Müller-Scholden, Peter Ewert, Alfred Hager, Harald Kaemmerer, Nicole Nagdyman, Jörg Schoetzau, Oktay Tutarel, Rüdiger Lange, Jürgen Hörer, Nikolaus A. Haas, Lale Rosenthal, Michael Hauser, Alexander Roithmaier, Hans-Gerd Kehl, Edward Malec, Helmut Baumgartner, Gerhard Diller, Roswitha Bahle, Gerald Hofner, Stefan Zink, Roland Reif, Helmut Singer, Christoph Parlasca, Matthias W. Freund, Michael Schumacher, Oliver Dewald, Christine Darrelmann, Reinald Motz, Olaf Willmann, Norbert Schmiedl, Peter Quick, Dirk Hillebrand, Stephan Michele Eiselt, Torsten Nekarda, Michael Eberhard, Georg Baier, Frank Uhlemann, Ioannis Tzanavaros, Alexander Beyer, Gudrun Binz, Steffen Hess, Thomas Teufel, Ronald-Peter Handke, Michael Hofbeck, Renate Kaulitz, Ludger Sieverding, Christian Schlensak, Christian Apitz, Michael Kaestner, Christoph Kupferschmid, Jürgen Holtvogt, Carl-Friedrich Wippermann, Andreas Heusch, Johannes Wirbelauer, Wolfgang Brosi.

• Contributors GPD, SO and JV collected the data and performed the deep learning-based analysis. UMMB and AU collected the clinical data and reviewed the manuscript. SS, FB, PB and HB were involved in the study design and critical review of the manuscript. GPD wrote the manuscript and performed the statistical analysis.

• Funding This study was supported by a research grant from the EMAH Stiftung Karla Voellm, Krefeld, Germany, and by the German Competence Network for Congenital Heart Defects (funded by the German Federal Ministry of Education and Research, BMBF, FKZ 01G10210, 01GI0601 until 2014 and the DZHK, German Centre for Cardiovascular Research, as of 2015).

• Competing interests None declared.

• Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.

• Patient consent for publication Not required.

• Ethics approval The study was registered with the National Registry for Congenital Heart Disease, and approval of the study protocol was obtained from the appropriate ethics committee. Patients were enrolled in the national registry as part of recruitment and gave appropriate informed consent before inclusion.

• Provenance and peer review Not commissioned; externally peer reviewed.

• Data availability statement Data are available upon reasonable request. Data is available for academic institutions from the German National Registry for Congenital Heart Disease subject to submission of a reasearch protocol and approval by the study board of the registry.

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Fallot tetrad surgery abroad

Dr. Vadim Berezhnoy

General practitioner, Medical expert, Head of the department of medical assistance.

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Operation of tetralogy of Fallot in children
8 2. How is the operation for tetralogy of Fallot in children
3. Complications after pi surgery blue heart disease in children
4. Treatment prognosis for tetralogy of Fallot
5. Leading clinic for the treatment of tetralogy of Fallot abroad

Reading time – 12 minutes

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Tetralogy of Fallot is a congenital heart disease that combines four anatomical anomalies – ventricular septal defect, aortic displacement, stenosis and hypertrophy of the right ventricle. The disease occurs in every 3rd child out of 10,000 newborns. Girls and boys get sick with the same frequency. This disease leads to the fact that the level of oxygen saturation in the blood gradually decreases. When this indicator reaches below 94%, disturbances in the functioning of the whole organism appear – immunity decreases, the work of the brain and the overall development of the body slow down, the skin becomes cyanotic.

Children with this diagnosis do not gain weight, grow poorly and cannot feed themselves. To cure this pathology requires a complex operation. How and where is the surgical treatment of Fallot’s tetrad abroad – find out from our article.

Treatment of tetralogy of Fallot

To determine the treatment tactics for tetralogy of Fallot , the patient’s medical history, the severity of cardiac disorders and the patient’s age are taken into account. Surgical intervention is considered the basis of the fight against the disease. As a rule, the operation is resorted to in the first 4-6 months of a child’s life.

Earlier surgery is indicated for severe defects in cardiac structures and a critically low level of oxygen saturation in the blood. Such patients immediately after birth undergo infusion of prostaglandin . This keeps the ductus arteriosus open and increases the level of oxygen in the blood. However, such therapy only postpones the operation for a while.

Newborn patients with this pathology can be operated on in several stages. First, cardiac surgeons perform an operation for the primary correction of defects – shunting . This makes it possible to ensure normal blood flow.

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A shunt is a small plastic tube sewn between the aorta and the pulmonary artery. It will be removed after the main operation is performed to eliminate the combined heart disease. Bypass surgery is also performed as a maintenance procedure for inoperable cases of tetralogy of Fallot.

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Among all heart defects that require treatment in early childhood, Fallot’s tetrad accounts for 10%. Without surgical treatment more than 90% of patients do not survive to 12 years of age.

How is the operation for tetralogy of Fallot in children?

The intervention is performed on the open heart using the cardiopulmonary bypass system . Its average duration is about 4 hours.

During surgery, doctors replace the pulmonary valve and widen the passage to the pulmonary artery. They apply patch to a ventricular septal defect. This is necessary to close the opening between the two lower chambers of the heart. Such actions improve blood flow to the lungs and other organs.

Most babies can lead active and healthy lives after surgery. However, they will need regular visits to a cardiologist .

Monitoring of the patient’s condition is necessary for the timely detection of any abnormalities and other diseases that may develop as the child grows up.

Complications after surgery for blue heart disease in children

In the postoperative period in some patients the functions of the heart muscle decrease . For this reason, in children after the intervention, there is a weakening of the function of the right ventricle, and in rare cases, a violation of the work of the heart muscle of the left ventricle. To eliminate this condition, drugs are prescribed that help the heart pump blood better, as well as medicines to control blood pressure.

Abroad the development of postoperative complications occurs only in 3% of cases. Early side effects of surgery to correct complex heart disease include recanalization of the ventricular septal defect and stenosis of the right ventricular outflow canal.

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Some long-term problems may include obstruction between the right pumping chamber and the pulmonary arteries. Children with an operated tetralogy of Fallot have a higher risk of heart rhythm disturbances – arrhythmias. Some patients experience dizziness or fainting.

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Children with tetralogy of Fallot are at increased risk of endocarditis, inflammation of the inner lining of the heart. Within 20 years after surgery, about 10% of patients need a second intervention.

Prognosis for the treatment of tetralogy of Fallot

A timely operation to correct cyanotic heart disease allows 85% of patients to recover completely and return to a full life. After undergoing surgery, children develop normally – they study, play sports, work and create families.

Leading Clinics for the Treatment of Tetralogy of Fallot

Foreign patients choose large departments and centers of cardiac surgery in Turkey, Korea, Lithuania, Germany, Spain and Israel for the prompt treatment of this pathology. Many patients prefer Turkish and Lithuanian hospitals. Practice here international level cardiac surgeons who successfully perform open heart surgery. Also, these countries provide the best value for money medical services. The private clinic Kardiolita and hospitals of the Liv Hospital network are especially popular.

Kardiolita Private Clinic

Read about the clinic →

The hospital performs all types of cardiac surgery of varying levels of complexity. Cardiac surgeons with 40 years of experience, who have more than 5,000 successful heart surgeries, practice here.

Cardiac surgery centers in Spain and Germany stand out among European countries in the treatment of complex heart defects in newborns and young children. Innovative surgical technologies are available here, which make it possible to carry out complex surgical interventions with minimal risks to the health of patients.

German Heart Center Berlin

Read about clinic →

According to the independent rating of the German magazine FOCUS, the clinic is one of the 3 best cardiology centers in Germany. Heart diseases in children are treated at the Department of Pediatric Cardiology and Congenital Heart Diseases. Every year, more than 600 operations are performed here to correct congenital cardiopathologies. Treatment of tetralogy of Fallot is carried out in full compliance with international protocols. The success of operations for combined heart defects exceeds 97%.

Teknon University Hospital

Read about the clinic →

The Teknon Institute of Cardiovascular Health is one of the most promising and high-tech institutions for the fight against cardiological diseases in Spain. A hybrid operating room operates here, where operations are successfully performed on a beating heart for young children. The hospital offers advanced diagnostic methods – catheter-free cardiac imaging TC-MD CT, 3D cardiac ultrasound, cardiac magnetic resonance imaging and PET-CT.

Many patients from the CIS countries turn to Korean and Israeli clinics for the treatment of blue heart disease in newborns. It has the necessary equipment and qualified personnel to successfully combat this pathology. Israeli cardiac surgeons are considered among the best in the world in the treatment of combined heart defects in children. High success rates for operations with such pathologies are shown by the Hadassah Medical Center and the Sung Chong Hyang Hospital.

Hadassah Clinic

Read about the clinic →

On the basis of the Hadassah Clinic there is a Center for Pediatric Cardiology and Cardiac Surgery. Minimally invasive heart surgeries are actively used here, which makes it possible to eliminate even the most severe cardiopathologies without complications.

Sung Chung Hyang Hospital

Read about the clinic →

According to the Ministry of Health of South Korea, the Heart Center of Sung Chung Hyang Hospital is one of the best in the country for the diagnosis and treatment of cardiac diseases, in particular heart defects in children.

Abstract

Tetralogy of Fallot is a blue heart disease that combines four anatomical anomalies – ventricular septal defect, aortic displacement, stenosis and hypertrophy of the right ventricle. This disease leads to the fact that the level of oxygen saturation of the blood gradually decreases. When this figure reaches below 94%, there are serious violations in the work of the whole organism.

To determine the tactics of treatment of Fallot’s tetrad, the patient’s medical history, the severity of cardiac disorders and the patient’s age are taken into account. Surgical intervention is considered the basis of the fight against the disease.

Foreign patients choose major departments and centers of cardiac surgery in Turkey, Korea, Lithuania, Germany, Spain and Israel to deal with this pathology promptly.

A timely operation to correct cyanotic heart disease allows 85% of patients to fully recover and return to a full life. After undergoing surgery, children develop normally – they study, play sports, work and create families. Only 10% of patients need reoperation within 20 years.

To learn more about the treatment of Fallot’s tetralogy abroad, please contact the medical coordinators of the international MediGlobus platform. Our experts will help you choose the best solution for your medical problem and organize your trip.