A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation.
Fontan circulation
Fontan-associated liver disease
Multidisciplinary care
Single ventricle
Journal
Pediatric cardiology
ISSN: 1432-1971
Titre abrégé: Pediatr Cardiol
Pays: United States
ID NLM: 8003849
Informations de publication
Date de publication:
Aug 2022
Aug 2022
Historique:
received:
22
01
2022
accepted:
28
04
2022
pubmed:
24
5
2022
medline:
22
7
2022
entrez:
23
5
2022
Statut:
ppublish
Résumé
Today, it is anticipated most individuals diagnosed with single-ventricle malformation will survive surgical reconstruction through a successful Fontan operation. As greater numbers of patients survive, so has the recognition that individuals with Fontan circulation face a variety of challenges. The goal of a normal quality and duration of life will not be reached by all. The hurdles fall into a variety of domains. From a cardiovascular perspective, the Fontan circulation is fundamentally flawed by its inherent nature of creating a state of chronically elevated venous pressure and congestion, accompanied by a relatively low cardiac output. Ventricular dysfunction, atrioventricular valve regurgitation, and arrhythmia may directly impact cardiac performance and can progress with time. Problems are not limited to the cardiovascular system. Fontan circulatory physiology impacts a multitude of biological processes and health parameters outside the heart. The lymphatic circulation is under strain manifesting as variable degrees of protein-rich lymph loss and immune system dysregulation. Organ system dysfunction develops through altered perfusion profiles. Liver fibrosis is ubiquitous, and a process of systemic fibrogenesis in response to circulatory stressors may affect other organs as well. Somatic growth and development can be delayed. Behavioral and mental health problems are common, presenting as clinically important levels of anxiety and depression. Most striking is the high variability in prevalence and magnitude of these complications within the population, indicating the likelihood of additional factors enhancing or mitigating their emergence. We propose that optimal care for the individual with single ventricle and a Fontan circulation is ideally offered in a comprehensive multidisciplinary manner, with attention to elements that are beyond cardiac management alone. In this report, we share the concepts, our experiences, and perspectives on development of a clinic model-the "Fontan rehabilitation, wellness and resilience development" or FORWARD program. We provide insights into the mechanics of our multidisciplinary model of care and the benefits offered serving our growing population of individuals with a Fontan circulation and their families.
Identifiants
pubmed: 35604474
doi: 10.1007/s00246-022-02930-z
pii: 10.1007/s00246-022-02930-z
pmc: PMC9125546
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
1175-1192Commentaires et corrections
Type : ErratumIn
Informations de copyright
© 2022. The Author(s).
Références
Liu MY, Zielonka B, Snarr BS, Zhang X, Gaynor JW, Rychik J (2018) Longitudinal assessment of outcome from prenatal diagnosis through Fontan operation for over 500 fetuses with single ventricle-type congenital heart disease: the Philadelphia Fetus-to-Fontan cohort study. J Am Heart Assoc 7:e009145
pubmed: 30371305
pmcid: 6404885
doi: 10.1161/JAHA.118.009145
Downing TE, Allen KY, Glatz AC et al (2017) Long-term survival after the Fontan operation: twenty years of experience at a single center. J Thorac Cardiovasc Surg 154:243-253 e2
pubmed: 28341469
doi: 10.1016/j.jtcvs.2017.01.056
Rychik J, Atz AM, Celermajer DS et al (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. https://doi.org/10.1161/CIR.0000000000000696
doi: 10.1161/CIR.0000000000000696
pubmed: 31256636
Meadows AT (2006) Pediatric cancer survivorship: research and clinical care. J Clin Oncol 24:5160–5165
pubmed: 17093280
doi: 10.1200/JCO.2006.07.3114
Rychik J, Veldtman G, Rand E et al (2012) The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium. Pediatr Cardiol 33:1001–1012
pubmed: 22534759
pmcid: 3442163
doi: 10.1007/s00246-012-0315-7
Daniels CJ, Bradley EA, Landzberg MJ et al (2017) Fontan-associated liver disease: proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol 70:3173–3194
pubmed: 29268929
doi: 10.1016/j.jacc.2017.10.045
Emamaullee J, Zaidi AN, Schiano T et al (2020) Fontan-associated liver disease: screening, management, and transplant considerations. Circulation 142:591–604
pubmed: 32776846
pmcid: 7422927
doi: 10.1161/CIRCULATIONAHA.120.045597
Goldberg DJ, Surrey LF, Glatz AC et al (2017) 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. https://doi.org/10.1161/JAHA.116.004809
doi: 10.1161/JAHA.116.004809
pubmed: 28947562
pmcid: 5634289
Nii M, Inuzuka R, Inai K et al (2021) Incidence and expected probability of liver cirrhosis and hepatocellular carcinoma after Fontan operation. Circulation 144:2043–2045
pubmed: 34928702
doi: 10.1161/CIRCULATIONAHA.121.056870
Lemmer A, VanWagner L, Gasanova Z, Helmke S, Everson GT, Ganger D (2019) Assessing hepatic impairment in Fontan-associated liver disease using the HepQuant SHUNT test. Congenit Heart Dis 14:978–986
pubmed: 31369200
pmcid: 7102438
doi: 10.1111/chd.12831
Dillman JR, Trout AT, Alsaied T, Gupta A, Lubert AM (2020) Imaging of Fontan-associated liver disease. Pediatr Radiol 50:1528–1541
pubmed: 32809067
doi: 10.1007/s00247-020-04776-0
Wells ML, Hough DM, Fidler JL, Kamath PS, Poterucha JT, Venkatesh SK (2017) Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma. Abdom Radiol (NY) 42:2623–2631
doi: 10.1007/s00261-017-1181-9
Egbe AC, Miranda WR, Veldtman GR, Graham RP, Kamath PS (2020) Hepatic venous pressure gradient in Fontan physiology has limited diagnostic and prognostic significance. CJC Open 2:360–364
pubmed: 32995721
pmcid: 7499375
doi: 10.1016/j.cjco.2020.04.011
Surrey LF, Russo P, Rychik J et al (2016) Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation. Hum Pathol 57:106–115
pubmed: 27476041
doi: 10.1016/j.humpath.2016.07.006
Srinivasan A, Guzman AK, Rand EB et al (2019) Percutaneous liver biopsy in Fontan patients. Pediatr Radiol 49:342–350
pubmed: 30506328
doi: 10.1007/s00247-018-4311-9
Ndrepepa G, Kastrati A (2016) Gamma-glutamyl transferase and cardiovascular disease. Ann Transl Med 4:481
pubmed: 28149843
pmcid: 5233492
doi: 10.21037/atm.2016.12.27
Martinez-Quintana E, Pardo-Maiza J, Deniz-Alvarado B, Riano-Ruiz M, Gonzalez-Martin JM, Rodriguez-Gonzalez F (2021) Gamma-glutamyl transferase and cardiovascular events in patients with congenital heart disease. Eur J Clin Investig. https://doi.org/10.1111/eci.13720
doi: 10.1111/eci.13720
Mancilla EE, Zielonka B, Roizen JD et al (2021) Growth in children with a Fontan circulation. J Pediatr 235:149-155 e2
pubmed: 33887332
doi: 10.1016/j.jpeds.2021.04.019
Menon SC, Al-Dulaimi R, McCrindle BW et al (2018) Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: a multicenter cross-sectional study. Congenit Heart Dis 13:463–469
pubmed: 29521004
doi: 10.1111/chd.12597
Matsumura S, Yana A, Kuwata S et al (2020) Prevalence of short stature and growth hormone deficiency and factors associated with short stature after Fontan surgery. Circ Rep 2:243–248
pubmed: 33693236
pmcid: 7921366
doi: 10.1253/circrep.CR-20-0009
Avitabile CM, Goldberg DJ, Zemel BS et al (2015) Deficits in bone density and structure in children and young adults following Fontan palliation. Bone 77:12–16
pubmed: 25882907
pmcid: 4447577
doi: 10.1016/j.bone.2015.04.012
Vaikunth SS, Leonard MB, Whitehead KK et al (2021) Deficits in the functional muscle-bone unit in youths with Fontan physiology. J Pediatr 238:202–207
pubmed: 34214589
doi: 10.1016/j.jpeds.2021.06.068
Thacker D, Patel A, Dodds K, Goldberg DJ, Semeao E, Rychik J (2010) Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation. Ann Thorac Surg 89:837–842
pubmed: 20172140
doi: 10.1016/j.athoracsur.2009.09.063
Grattan MJ, McCrindle BW (2010) Recurrent exacerbations of protein-losing enteropathy after initiation of growth hormone therapy in a Fontan patient controlled with spironolactone. Congenit Heart Dis 5:165–167
pubmed: 20412490
doi: 10.1111/j.1747-0803.2009.00320.x
Tomasulo CE, Chen JM, Smith CL, Maeda K, Rome JJ, Dori Y (2020) Lymphatic disorders and management in patients with congenital heart disease. Ann Thorac Surg. https://doi.org/10.1016/j.athoracsur.2020.10.058
doi: 10.1016/j.athoracsur.2020.10.058
pubmed: 33373590
Morsheimer MM, Rychik J, Forbes L et al (2016) Risk factors and clinical significance of lymphopenia in survivors of the fontan procedure for single-ventricle congenital cardiac disease. J Allergy Clin Immunol Pract 4:491–496
pubmed: 26897303
doi: 10.1016/j.jaip.2015.11.034
Magdo HS, Stillwell TL, Greenhawt MJ et al (2015) Immune abnormalities in Fontan protein-losing enteropathy: a case-control study. J Pediatr 167:331–337
pubmed: 26009017
doi: 10.1016/j.jpeds.2015.04.061
Gonzalez VJ, Kimbro RT, Cutitta KE et al (2021) Mental health disorders in children with congenital heart disease. Pediatrics. https://doi.org/10.1542/peds.2020-1693
doi: 10.1542/peds.2020-1693
pubmed: 34794410
pmcid: 8600488
DeMaso DR, Calderon J, Taylor GA et al (2017) Psychiatric disorders in adolescents with single ventricle congenital heart disease. Pediatrics. https://doi.org/10.1542/peds.2016-2241
doi: 10.1542/peds.2016-2241
pubmed: 28148729
pmcid: 5330395
Gaynor JW, Ittenbach RF, Gerdes M et al (2014) Neurodevelopmental outcomes in preschool survivors of the Fontan procedure. J Thorac Cardiovasc Surg 147:1276–1282 (discussion 1282–1283 e5)
pubmed: 24521968
pmcid: 5662937
doi: 10.1016/j.jtcvs.2013.12.019
Ward WL, Smith A, Munns C, Bai S (2021) The process of integrating psychology into medical clinics: pediatric psychology as an example. Clin Child Psychol Psychiatry 26:323–341
pubmed: 33353382
doi: 10.1177/1359104520982323
Asarnow JR, Rozenman M, Wiblin J, Zeltzer L (2015) Integrated medical-behavioral care compared with usual primary care for child and adolescent behavioral health: a meta-analysis. JAMA Pediatr 169:929–937
pubmed: 26259143
doi: 10.1001/jamapediatrics.2015.1141
McGrady ME, Hommel KA (2016) Targeting health behaviors to reduce health care costs in pediatric psychology: descriptive review and recommendations. J Pediatr Psychol 41:835–848
pubmed: 26359311
doi: 10.1093/jpepsy/jsv083
Marino BS, Tomlinson RS, Wernovsky G et al (2010) Validation of the pediatric cardiac quality of life inventory. Pediatrics 126:498–508
pubmed: 20805147
doi: 10.1542/peds.2009-2973
Burkhart K, Asogwa K, Muzaffar N, Gabriel M (2020) pediatric integrated care models: a systematic review. Clin Pediatr (Phila) 59:148–153
doi: 10.1177/0009922819890004
Weinreb SJ, Dodds KM, Burstein DS et al (2020) End-organ function and exercise performance in patients with Fontan circulation: what characterizes the high performers? J Am Heart Assoc 9:e016850
pubmed: 33317366
pmcid: 7955385
doi: 10.1161/JAHA.120.016850
Tay EL, Peset A, Papaphylactou M et al (2011) Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol 151:307–312
pubmed: 20580108
doi: 10.1016/j.ijcard.2010.05.066
Rychik J, Dodds KM, Goldberg D et al (2020) Protein losing enteropathy after Fontan operation: glimpses of clarity through the lifting fog. World J Pediatr Congenit Heart Surg 11:92–96
pubmed: 31835975
doi: 10.1177/2150135119890555
Paridon SM, Mitchell PD, Colan SD et al (2008) A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J Am Coll Cardiol 52:99–107
pubmed: 18598887
doi: 10.1016/j.jacc.2008.02.081
Goldberg DJ, Zak V, McCrindle BW et al (2021) Exercise capacity and predictors of performance after Fontan: results from the pediatric heart network Fontan 3 study. Pediatr Cardiol 42:158–168
pubmed: 32975603
doi: 10.1007/s00246-020-02465-1
Avitabile CM, Leonard MB, Zemel BS et al (2014) Lean mass deficits, vitamin D status and exercise capacity in children and young adults after Fontan palliation. Heart 100:1702–1707
pubmed: 24973081
doi: 10.1136/heartjnl-2014-305723
Cordina RL, O’Meagher S, Karmali A et al (2013) Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology. Int J Cardiol 168:780–788
pubmed: 23154055
doi: 10.1016/j.ijcard.2012.10.012
Avitabile C, Goldmuntz E, Rychik J (2021) Exercise is medicine in the Fontan circulation. Int J Cardiol 343:50–52
pubmed: 34537310
doi: 10.1016/j.ijcard.2021.09.019
Houlihan TH, Lopez S, Dodds K et al (2021) Living-related donor kidney transplant in a patient with single ventricle and Fontan circulation. World J Pediatr Congenit Heart Surg 12:673–675
pubmed: 33899567
doi: 10.1177/2150135120978959
Dori Y, Keller MS, Rome JJ et al (2016) Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease. Circulation 133:1160–1170
pubmed: 26864093
doi: 10.1161/CIRCULATIONAHA.115.019710
Itkin M, Piccoli DA, Nadolski G et al (2017) Protein-losing enteropathy in patients with congenital heart disease. J Am Coll Cardiol 69:2929–2937
pubmed: 28619193
doi: 10.1016/j.jacc.2017.04.023
Alsaied T, Allen KY, Anderson JB et al (2020) The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States. Cardiol Young 30:1070–1075
pubmed: 32635947
doi: 10.1017/S1047951120001869