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Current Pediatrics Reports

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01.06.2019 | Cardiology (W Zuckerman and E Silver, Section Editors)

Modern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation

verfasst von: M. Abigail Simmons, Robert W. Elder

Erschienen in: Current Pediatrics Reports | Ausgabe 2/2019

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Abstract

Purpose of Review

The majority of individuals who have undergone Fontan palliation are surviving into adulthood, yet complications are common. This review will focus on late complications—cardiac and extra-cardiac—which may present insidiously.

Recent Findings

Recent meta-analysis of patients after Fontan operation suggests that over 80% will survive 20 years beyond surgery. This group is at risk for structural complications, arrhythmias, vascular complications, and heart failure. Not all forms of Fontan failure are the same, and categorization into separate entities such as failure with preserved versus reduced function may help guide therapies. Pulmonary vasodilators in particular may be useful to improve hemodynamics. Novel therapies aimed at lymphatic complications are emerging and promising. Late hepatic complications including ascites and liver cancer may be seen, and scoring systems may identify patients at higher risk.

Summary

Individuals who have undergone a Fontan surgery face challenges as they age which include both cardiac and non-cardiac systems.

•• Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102:1081–6 This paper details the current understanding of Fontan physiology including optimal and suboptimal hemodynamic conditions. CrossRefPubMedPubMedCentral

•• Poh CL, d’Udekem Y. Life after surviving fontan surgery: a meta-analysis of the incidence and predictors of late death. Heart Lung Circ. 2018;27(5):552–9 One of the largest series detailing the long-term outcomes in Fontan patients. CrossRefPubMed

•• Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2018;S0735-1097(18):36845–1 The guidelines detail the anatomic and physiologic classification of congenital heart disease, general guidelines for the care of adult congenital heart disease, and lesion-specific guidance.

Deal BJ, Jacobs ML. Management of the failing Fontan circulation. Heart. 2012;98(14):1098–104.CrossRefPubMedPubMedCentral

McElhinney D, Reddy V, Hanley F, Moore P. Systemic venous collateral channels causing desaturation after bidirectional cavopulmonary anastomosis: evaluation and management. J Am Coll Cardiol. 1997;30(3):817–24.CrossRefPubMed

Lluri G, Levi DS, Aboulhosn J. Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation. Int J Cardiol. 2015;189:159–63.CrossRefPubMed

Hoffman J. Normal and abnormal pulmonary arteriovenous shunting: occurrence and mechanisms. Cardiol Young. 2013;23(5):629–41.CrossRefPubMed

Sundareswaran KS, de Zelicourt D, Sharma S, Kanter KR, Spray TL, Sotiropoulos F, et al. Correction of pulmonary artery malformation using image-based surgical planning. JACC Cardiovasc Imaging. 2009;2(8):1024–30.CrossRefPubMedPubMedCentral

Triedman JK, Bridges ND, Mayer JE, Lock JE. Prevalence and risk factors for aortopulmonary collateral vessels after Fontan and bidirectional Glenn procedures. JACC. 1993;22(1):207–15.CrossRefPubMed

10.

Grosse-Wortmann L, Al-Otay A, Yoo S-J. Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion quantification with MRI. Circulation. 2(3):219–25.

11.

Wang R-P, Liang C-H, Huang M-P, Liu H, Deng Q-P, Yang M-F. Assessment of aortopulmonary collateral flow and pulmonary vascular growth using a 3.0 T magnetic resonance imaging system in patients who underwent bidirectional Glenn shunting. Eur J Cardiothorac Surg. 2012;41(6):e146–53.CrossRefPubMed

12.

Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, et al. 40-year follow-up after the Fontan operation long-term outcomes of 1,052 patients. JACC. 2015;66(15):1700–10.CrossRefPubMed

13.

Lasa J, Glatz AC, Daga A, Shah M. Prevalence of arrhythmias late after the Fontan operation. Am J Cardiol. 2014;113(7):1184–8.CrossRefPubMed

14.

Pundi KN, Johnson JN, Dearani JA, Li Z, Driscoll DJ, et al. Sudden cardiac death and late arrhythmias after the Fontan operation. Congenit Heart Dis. 2017;12(1):17–23.CrossRefPubMed

15.

Fontan F, Kirkin JW, Fernandez G, Costa F, Naftel DC, Tritto F, et al. Outcome after a perfect Fontan. Circulation. 1990;81(5):1520–36.CrossRefPubMed

16.

d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Redefining expectations of long-term survival after the Fontan procedure. Circulation. 2014;130(11 suppl 1):S32–8.CrossRefPubMed

17.

•• Hebson C, Book W, Elder RW, Ford R, Jokhadar M, Kanter K, et al. Frontiers in Fontan failure: a summary of conference proceedings. Congenit Heart Dis. 2017;12(1):6–16 This document introduced the use of a standardized classification system for Fontan failure including the clinical findings associated with different phenotypes of Fontan failure. CrossRefPubMed

18.

•• Book WM, Geradin J, Saraf A, Valente AM, Rodriguez F. Clinical phenotypes of Fontan failure: implications for management. Congenit Heart Dis. 2016;11:296–308 A document summarizing phenotype-specific management strategies for Fontan failure. CrossRefPubMed

19.

Wilson TG, Iyengar AJ, d’Udekem Y. The use and misuse of ACE inhibitors in patients with single ventricle physiology. Heart Lung Circ. 2016;25(3):229–36.CrossRefPubMed

20.

Kouatli A, Garcia J, Zellers TM, Weinstein E, Mahony LM. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation. 1997;96(5):1507–12.CrossRefPubMed

21.

Hebert A, Mikkelsen U, Thilen U, Idorn L, Jensen A, Nagy E, et al. Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study. Circulation. 2014;130(23):2021–30.CrossRefPubMed

22.

Wang W, Hu X, Liao W, Rutahoile WH, Malenka DJ, Zeng X, et al. The efficacy and safety of pulmonary vasodilators in patients with Fontan circulation: a meta-analysis of randomized controlled trials. Pulmonary Circulation. 2019;9(1).

23.

Van der Ven J, Van den Bosch E, Bogers AJCC, Helbing WA. State of the art of the Fontan strategy for treatment of univentricular heart disease. F1000Research. 2018;935:1–14.

24.

Feldt R, Driscoll D, Offord K, Cha RH, Perrault J, Schaff HV, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996;112(3):672–80.CrossRefPubMed

25.

Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. J Thorac Cardiovasc Surg. 1998;115(5):1063–73.CrossRefPubMed

26.

Schumacher KR, Stringer KA, Donohue JE, Yu S, Shaver A, Caruthers RL, et al. Fontan-associated protein-losing enteropathy and plastic bronchitis. J Pediatr. 2015;166(4):970–7.CrossRefPubMedPubMedCentral

27.

Dori Y, Keller MS, Fogel MA, Rome JJ, Whitehead KK, Harris MA, et al. MRI of lymphatic abnormalities after functional single-ventricle palliation surgery. Am J Roentgenol. 2014;203(2):426–31.CrossRef

28.

• Dori Y, Keller MS, Rome JJ, Gillespie MJ, Glatz AC, Dodds K, et al. Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease. Circulation. 2016;133(12):1160–70 The authors report on the success of a novel percutaneous intervention for plastic bronchitis. CrossRefPubMed

29.

John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol. 2014;64(1):54–62.CrossRefPubMed

30.

• Egbe AC, Connolly HM, Niaz T, Yogeswaran V, Taggart NW, Qureshi MY, et al. Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation. Am Heart J. 2017;183:10–7 Extensive retrospective review of thromboembolic complications in 387 adults with a Fontan procedure. CrossRefPubMed

31.

Coon PD, Rychik J, Novello RT, Ro PS, Gaynor JW, Spray TL. Thrombus formation after the Fontan operation. Ann Thorac Surg. 2001;71(6):1990–4.CrossRefPubMed

32.

Dennis M, Zannino D, du Plessis K, Bullock A, Disney P, Radford DJ, et al. Clinical outcomes in adolescents and adults after the Fontan procedure. J Am Coll Cardiol. 2018;71(9):1009–17.CrossRefPubMed

33.

Alsaied T, Alsidawi S, Allen CC, Faircloth J, Palumbo JS, Veldtman GR. Strategies for thromboprophylaxis in Fontan circulation: a meta-analysis. Heart. 2015;101(21):1731–7.CrossRefPubMed

34.

• Georgekutty J, Kazerouninia A, Want Y, Ermis P, Parekh D, Franklin W, et al. Novel oral anticoagulant use in adult Fontan patients: a single center experience. Congenital Heart Dis. 2018;1–7. This study reports the outcomes of novel anticoagulant use in Fontan patients for the prevention of thromboembolic complications.

35.

Yang H, Bouma BJ, Mulder BJ. Vitamin antagonist anticoagulants for prevention in adult congenital heart disease investigators NK. Is initiating NOACs for atrial arrhythmias safe in adults with congenital heart disease? Cardiovasc Drugs Ther. 2017;31(4):413–7.CrossRefPubMedPubMedCentral

36.

• Goldberg DJ, Surrey LF, Glatz AC, Dodds K, O’Byrne ML, Lin HC, et al. Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: a liver biopsy and hemodynamic study. J Am Heart Assoc. 2017;6(5):e004809-9 This study details the universal presence of hepatic fibrosis following Fontan palliation and correlates this fibrosis with clinical and hemodynamic outcomes. CrossRef

37.

•• Daniels CJ, Bradley EA, Landzberg MJ, Aboulhosn J, Beekman RH, Book W, et al. Fontan-associated liver disease proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol. 2017;70(25):3173–94 This document provides the first set of suggested guidelines for the screening and management of Fontan-associated liver disease. CrossRefPubMed

38.

Lemmer A, VanWagner LB, Ganger D. Assessment of advanced liver fibrosis and the risk for hepatic decompensation in patients with congestive hepatopathy. Hepatology. 2018;68(4):1633–41.CrossRefPubMed

39.

Wu FM, Earing MG, Aboulhosn JA, Johncilla ME, Singh MN, Odze RD, et al. Predictive value of biomarkers of hepatic fibrosis in adult Fontan patients. J Heart Lung Transplant. 2017;36(2):211–9.CrossRefPubMed

40.

Elder RW, McCabe NM, Hebson C, Veledar E, Romero R, Ford RM, et al. Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol. 2013;168(4):3764–9.CrossRefPubMedPubMedCentral

41.

Assenza GE, Graham DA, Landzberg MJ, Valente AM, Singh MN, Bashir A, et al. MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery. Heart. 2013;99:491–6.CrossRefPubMed

42.

Egbe AC, Poterucha JT, Warnes CA, Connolly HM, Baskar S, Ginde S, et al. Hepatocellular carcinoma after Fontan operation. Circulation. 2018;138(7):746–8.CrossRefPubMed

43.

Liptzin DR, Maria MV, Younoszai A, Narkewicz MR, Kelly SL, Wolfe KR, et al. Pulmonary screening in subjects after the Fontan procedure. J Pediatr. 2018;199:140–3.CrossRefPubMed

44.

Opotowsky AR, Landzberg MJ, Earing MG, Wu FM, Triedman JK, Casey A, et al. Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity. Am J Physiol Heart Circ Physiol. 2014;307(1):H110–7.CrossRefPubMedPubMedCentral

45.

Fredriksen P, Therrien J, Veldtman G, Warsi M, Liu P, Siu S, et al. Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart. 2001;85(3):295–9.CrossRefPubMedPubMedCentral

46.

Matthews I, Fredriksen P, Bjørnstad PG, Thaulow E, Gronn M. Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity. Cardiol Young. 2006;16(3):261–7.CrossRefPubMed

47.

Nakhleh N, Francis R, Giese RA, Tian X, Li Y, Zariwala MA, et al. High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy. Circulation. 2012;125(18):2232–42.CrossRefPubMedPubMedCentral

48.

Ali L, Pingitore A, Piaggi P, Brucini F, Passera M, Marotta M, et al. Respiratory training late after Fontan intervention: impact on cardiorespiratory performance. Pediatr Cardiol. 2018;39(4):695–704.CrossRef

49.

Hedlund ER, Ljungberg H, Söderström L, Lundell B, Sjöberg G. Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training. Cardiol Young. 2018;28(9):1115–22.CrossRefPubMed

50.

Marino B, Lipkin P, Newburger J, Peacock G, Gerdes M, Gaynor J, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012;126(9):1143–72.CrossRefPubMed

51.

Bellinger DC, Watson CG, Rivkin MJ, Robertson RL, Roberts AE, Stopp C, et al. Neuropsychological status and structural brain imaging in adolescents with single ventricle who underwent the Fontan procedure. J Am Heart Assoc. 2015;4(12):e002302.CrossRefPubMedPubMedCentral

52.

Luyckx K, Rassart J, Goossens E, Apers S, Oris L, Moons P. Development and persistence of depressive symptoms in adolescents with CHD. Cardiol Young. 2015;26(6):1115–22.CrossRef

53.

Kovacs AH, Saidi AS, Kuhl EA, Sears SF, Silversides C, Harrison JL, et al. Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol. 2009;137(2):158–64.CrossRefPubMed

54.

DeMaso DR, Calderon J, Taylor GA, Holland JE, Stopp C, White MT, et al. Psychiatric disorders in adolescents with single ventricle congenital heart disease. Pediatrics. 2017;139(3):e20162241.CrossRefPubMedPubMedCentral

55.

McCrindle BW, Williams RV, Mitchell PD, Hsu DT, Paridon SM, Atz AM, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation. 2006;113(8):1123–9.CrossRefPubMed

56.

Gurvitz M, Valente A, Broberg C, Cook S, Stout K, Kay J, et al. Prevalence and predictors of gaps in care among adult congenital heart disease patients HEART-ACHD (The Health, Education, and Access Research Trial). J Am Coll Cardiol. 2013;61(21):2180–4.CrossRefPubMedPubMedCentral

57.

Yeung E, Kay J, Roosevelt GE, Brandon M, Yetman AT. Lapse of care as a predictor for morbidity in adults with congenital heart disease. Int J Cardiol. 2008;125(1):62–5.CrossRefPubMed

58.

Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM, Connolly HM, et al. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues. Circulation. 2011;123(13):1454–85.CrossRefPubMed

Titel: Modern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation
verfasst von: M. Abigail Simmons
Robert W. Elder
Publikationsdatum: 01.06.2019
Verlag: Springer US
Erschienen in: Current Pediatrics Reports / Ausgabe 2/2019
Elektronische ISSN: 2167-4841
DOI: https://doi.org/10.1007/s40124-019-00192-7

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Modern Day Care of Patients With Single Ventricle Heart Disease: Late Complications of Fontan Palliation