Skip to main content
Erschienen in: Pediatric Cardiology 1/2019

29.08.2018 | Original Article

Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey

verfasst von: Michael V. Di Maria, David W. Brown, Frank Cetta, Salil Ginde, David Goldberg, Shaji C. Menon, Heather M. Phelps, Jack Rychik, Kurt R. Schumacher, Philip Thrush, Gruschen Veldtman, Gail Wright, Adel K. Younoszai

Erschienen in: Pediatric Cardiology | Ausgabe 1/2019

Einloggen, um Zugang zu erhalten

Abstract

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500–2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3–4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.
Literatur
1.
Zurück zum Zitat Tweddell JS, Nersesian M, Mussatto KA, Nugent M, Simpson P, Mitchell ME, Ghanayem NS, Pelech AN, Marla R, Hoffman GM (2009) Fontan palliation in the modern era: factors impacting mortality and morbidity. Ann Thorac Surg 88:1291–1299CrossRefPubMed Tweddell JS, Nersesian M, Mussatto KA, Nugent M, Simpson P, Mitchell ME, Ghanayem NS, Pelech AN, Marla R, Hoffman GM (2009) Fontan palliation in the modern era: factors impacting mortality and morbidity. Ann Thorac Surg 88:1291–1299CrossRefPubMed
2.
Zurück zum Zitat Khairy P, Fernandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE, Landzberg MJ (2008) Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 117:85–92CrossRefPubMed Khairy P, Fernandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE, Landzberg MJ (2008) Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 117:85–92CrossRefPubMed
3.
Zurück zum Zitat Downing TE, Allen KY, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Faerber JA, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ (2017) Long-term survival after the Fontan operation: twenty years of experience at a single center. J Thorac Cardiovasc Surg 154:243–253 e242CrossRefPubMed Downing TE, Allen KY, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Faerber JA, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ (2017) Long-term survival after the Fontan operation: twenty years of experience at a single center. J Thorac Cardiovasc Surg 154:243–253 e242CrossRefPubMed
4.
Zurück zum Zitat Rychik J, Goldberg DJ (2014) Late consequences of the Fontan operation. Circulation 130:1525–1528CrossRefPubMed Rychik J, Goldberg DJ (2014) Late consequences of the Fontan operation. Circulation 130:1525–1528CrossRefPubMed
5.
Zurück zum Zitat Rychik J, Goldberg D, Rand E, Semeao E, Russo P, Dori Y, Dodds K (2013) End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis. Cardiol Young 23:831–840CrossRefPubMed Rychik J, Goldberg D, Rand E, Semeao E, Russo P, Dori Y, Dodds K (2013) End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis. Cardiol Young 23:831–840CrossRefPubMed
6.
Zurück zum Zitat Khiabani RH, Whitehead KK, Han D, Restrepo M, Tang E, Bethel J, Paridon SM, Fogel MA, Yoganathan AP (2015) Exercise capacity in single-ventricle patients after Fontan correlates with haemodynamic energy loss in TCPC. Heart 101:139–143CrossRefPubMed Khiabani RH, Whitehead KK, Han D, Restrepo M, Tang E, Bethel J, Paridon SM, Fogel MA, Yoganathan AP (2015) Exercise capacity in single-ventricle patients after Fontan correlates with haemodynamic energy loss in TCPC. Heart 101:139–143CrossRefPubMed
7.
Zurück zum Zitat Avitabile CM, Goldberg DJ, Dodds K, Dori Y, Ravishankar C, Rychik J (2014) A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. Ann Thorac Surg 98:634–640CrossRefPubMedPubMedCentral Avitabile CM, Goldberg DJ, Dodds K, Dori Y, Ravishankar C, Rychik J (2014) A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. Ann Thorac Surg 98:634–640CrossRefPubMedPubMedCentral
8.
Zurück zum Zitat Goldberg DJ, Surrey LF, Glatz AC, Dodds K, O’Byrne ML, Lin HC, Fogel M, Rome JJ, Rand EB, Russo P, Rychik J (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 6 Goldberg DJ, Surrey LF, Glatz AC, Dodds K, O’Byrne ML, Lin HC, Fogel M, Rome JJ, Rand EB, Russo P, Rychik J (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 6
9.
Zurück zum Zitat John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F (2014) Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol 64:54–62CrossRefPubMed John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F (2014) Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol 64:54–62CrossRefPubMed
10.
Zurück zum Zitat Opotowsky AR, Baraona FR, Mc Causland FR, Loukas B, Landzberg E, Landzberg MJ, Sabbisetti V, Waikar SS (2017) Estimated glomerular filtration rate and urine biomarkers in patients with single-ventricle Fontan circulation. Heart 103:434–442CrossRefPubMed Opotowsky AR, Baraona FR, Mc Causland FR, Loukas B, Landzberg E, Landzberg MJ, Sabbisetti V, Waikar SS (2017) Estimated glomerular filtration rate and urine biomarkers in patients with single-ventricle Fontan circulation. Heart 103:434–442CrossRefPubMed
11.
Zurück zum Zitat Sharma S, Ruebner RL, Furth SL, Dodds KM, Rychik J, Goldberg DJ (2016) Assessment of kidney function in survivors following Fontan palliation. Congenit Heart Dis 11:630–636CrossRefPubMed Sharma S, Ruebner RL, Furth SL, Dodds KM, Rychik J, Goldberg DJ (2016) Assessment of kidney function in survivors following Fontan palliation. Congenit Heart Dis 11:630–636CrossRefPubMed
12.
Zurück zum Zitat Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson WH Jr, Li J, Smith SE, Bellinger DC, Mahle WT, American Heart Association Congenital Heart Defects Committee CoCDitYCoCN, Stroke C (2012) Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 126:1143–1172CrossRefPubMed Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson WH Jr, Li J, Smith SE, Bellinger DC, Mahle WT, American Heart Association Congenital Heart Defects Committee CoCDitYCoCN, Stroke C (2012) Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 126:1143–1172CrossRefPubMed
13.
Zurück zum Zitat Giglia TM, Massicotte MP, Tweddell JS, Barst RJ, Bauman M, Erickson CC, Feltes TF, Foster E, Hinoki K, Ichord RN, Kreutzer J, McCrindle BW, Newburger JW, Tabbutt S, Todd JL, Webb CL, American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young CoC, Stroke Nursing CoE, Prevention, Stroke C (2013) Prevention and treatment of thrombosis in pediatric and congenital heart disease: a scientific statement from the American Heart Association. Circulation 128:2622–2703CrossRefPubMed Giglia TM, Massicotte MP, Tweddell JS, Barst RJ, Bauman M, Erickson CC, Feltes TF, Foster E, Hinoki K, Ichord RN, Kreutzer J, McCrindle BW, Newburger JW, Tabbutt S, Todd JL, Webb CL, American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young CoC, Stroke Nursing CoE, Prevention, Stroke C (2013) Prevention and treatment of thrombosis in pediatric and congenital heart disease: a scientific statement from the American Heart Association. Circulation 128:2622–2703CrossRefPubMed
14.
Zurück zum Zitat Graham TP Jr, Driscoll DJ, Gersony WM, Newburger JW, Rocchini A, Towbin JA (2005) Task Force 2: congenital heart disease. J Am Coll Cardiol 45:1326–1333CrossRefPubMed Graham TP Jr, Driscoll DJ, Gersony WM, Newburger JW, Rocchini A, Towbin JA (2005) Task Force 2: congenital heart disease. J Am Coll Cardiol 45:1326–1333CrossRefPubMed
15.
Zurück zum Zitat Asrani SK, Warnes CA, Kamath PS (2013) Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 368:1756–1757CrossRefPubMed Asrani SK, Warnes CA, Kamath PS (2013) Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 368:1756–1757CrossRefPubMed
Metadaten
Titel
Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey
verfasst von
Michael V. Di Maria
David W. Brown
Frank Cetta
Salil Ginde
David Goldberg
Shaji C. Menon
Heather M. Phelps
Jack Rychik
Kurt R. Schumacher
Philip Thrush
Gruschen Veldtman
Gail Wright
Adel K. Younoszai
Publikationsdatum
29.08.2018
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 1/2019
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-018-1966-9

Weitere Artikel der Ausgabe 1/2019

Pediatric Cardiology 1/2019 Zur Ausgabe

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.