Forty years of the fontan operation: A failed strategy

Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual

Volumn 13, Issue 1, 2010, Pages 96-100

  Rychik, Jack ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

PLACEBO; SILDENAFIL;

ARTICLE; BRONCHITIS; CENTRAL VENOUS PRESSURE; CLINICAL TRIAL; DRUG MECHANISM; FONTAN PROCEDURE; FUNCTIONAL STATUS; HEART OUTPUT; HUMAN; LIVER DISEASE; OUTCOME ASSESSMENT; PATHOGENESIS; PROTEIN LOSING GASTROENTEROPATHY; PULMONARY HYPERTENSION; STRATEGIC PLANNING; TREATMENT RESPONSE;

BRONCHITIS; CARDIAC OUTPUT; CENTRAL VENOUS PRESSURE; FONTAN PROCEDURE; HEART DEFECTS, CONGENITAL; HEART VENTRICLES; HUMANS; LIVER; LIVER DISEASES; POSTOPERATIVE COMPLICATIONS; POSTOPERATIVE PERIOD; PROTEIN-LOSING ENTEROPATHIES; TREATMENT FAILURE;

EID: 77949614808     PISSN: 10929126     EISSN: None     Source Type: Journal    
DOI: 10.1053/j.pcsu.2010.02.006     Document Type: Article

References (40)

1. Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax 1971, 26:240-248.
2. Kreutzer G., Galindez E., Bono H., et al. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973, 66:613-621.
3. de Leval M.R., Kilner P., Gewillig M., et al. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988, 96:682-695.
4. Khairy P., Fernandes S.M., Mayer J.E., et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008, 117:85-92.
5. Earing M.G., Cetta F., Driscoll D.J., et al. Long-term results of the Fontan operation for double-inlet left ventricle. Am J Cardiol 2005, 96:291-298.
6. Anderson P.A., Sleeper L.A., Mahony L., et al. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol 2008, 52:85-98.
7. Paridon S.M., Mitchell P.D., Colan S.D., et al. A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J Am Coll Cardiol 2008, 52:99-107.
8. McCrindle B.W., Williams R.V., Mitchell P.D., et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation 2006, 113:1123-1129.
9. Senzaki H., Masutani S., Ishido H., et al. Cardiac rest and reserve function in patients with Fontan circulation. J Am Coll Cardiol 2006, 47:2528-2535.
10. Hijazi Z.M., Fahey J.T., Kleinman C.S., et al. Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery. Circulation 1992, 86:196-202.
11. Mavroudis C., Zales V.R., Backer C.L., et al. Fenestrated Fontan with delayed catheter closure. Effects of volume loading and baffle fenestration on cardiac index and oxygen delivery. Circulation 1992, 86:II85-II92.
12. Rychik J., Rome J.J., Jacobs M.L. Late surgical fenestration for complications after the Fontan operation. Circulation 1997, 96:33-36.
13. Fogel M.A., Weinberg P.M., Chin A.J., et al. Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging. J Am Coll Cardiol 1996, 28:212-221.
14. Dilawar M., Bradley S.M., Saul J.P., et al. Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit fontan procedures. Pediatr Cardiol 2003, 24:284-288.
15. Szwast A., Tian Z., McCann M., et al. Right ventricular performance in the fetus with hypoplastic left heart syndrome. Ann Thorac Surg 2009, 87:1214-1219.
16. Senzaki H., Masutani S., Kobayashi J., et al. Ventricular afterload and ventricular work in fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with blalock-taussig shunts. Circulation 2002, 105:2885-2892.
17. Sundareswaran K.S., Kanter K.R., Kitajima H.D., et al. Impaired power output and cardiac index with hypoplastic left heart syndrome: a magnetic resonance imaging study. Ann Thorac Surg 2006, 82:1267-1277.
18. Cheung M.M., Smallhorn J.F., McCrindle B.W., et al. Non-invasive assessment of ventricular force-frequency relations in the univentricular circulation by tissue Doppler echocardiography: a novel method of assessing myocardial performance in congenital heart disease. Heart 2005, 91. 1338-1242.
19. Mahle W.T., Coon P.D., Wernovsky G., et al. Quantitative echocardiographic assessment of the performance of the functionally single right ventricle after the Fontan operation. Cardiol Young 2001, 11:399-406.
20. Penny D.J., Rigby M.L., Redington A.N. Abnormal patterns of intraventricular flow and diastolic filling after the Fontan operation: evidence for incoordinate ventricular wall motion. Br Heart J 1991, 66:375-378.
21. Cheung Y.F., Penny D.J., Redington A.N. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 2000, 83:420-424.
22. Friedrich-Rust M., Koch C., Rentzsch A., et al. Noninvasive assessment of liver fibrosis in patients with Fontan circulation using transient elastography and biochemical fibrosis markers. J Thorac Cardiovasc Surg 2008, 135:560-567.
23. Kendall T.J., Stedman B., Hacking N., et al. Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J Clin Pathol 2008, 61:504-508.
24. Ghaferi A.A., Hutchins G.M. Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 2005, 129:1348-1352.
25. Camposilvan S., Milanesi O., Stellin G., et al. Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg 2008, 86:177-182.
26. Kiesewetter C.H., Sheron N., Vettukattill J.J., et al. Hepatic changes in the failing Fontan circulation. Heart 2007, 93:579-584.
27. Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2007, 2:288-300.
28. Rychik J., Gui-Yang S. Relation of mesenteric vascular resistance after Fontan operation and protein-losing enteropathy. Am J Cardiol 2002, 90:672-674.
29. Ostrow A.M., Freeze H., Rychik J. Protein-losing enteropathy after fontan operation: investigations into possible pathophysiologic mechanisms. Ann Thorac Surg 2006, 82:695-700.
30. Murch S.H., Winyard P.J., Koletzko S., et al. Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition. Lancet 1996, 347:1299-1301.
31. Bode L., Eklund E.A., Murch S., et al. Heparan sulfate depletion amplifies TNF-alpha-induced protein leakage in an in vitro model of protein-losing enteropathy. Am J Physiol Gastrointest Liver Physiol 2005, 288:G1015-G1023.
32. Bode L., Murch S., Freeze H.H. Heparan sulfate plays a central role in a dynamic in vitro model of protein-losing enteropathy. J Biol Chem 2006, 281:7809-7815.
33. Humpl T., Reyes J.T., Holtby H., et al. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 2005, 111:3274-3280.
34. Uzun O., Wong J.K., Bhole V., et al. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan. Ann Thorac Surg 2006, 82:e39-e40.
35. Haseyama K., Satomi G., Yasukochi S., et al. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2006, 132:1232-1233.
36. Giardini A., Balducci A., Specchia S., et al. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008, 29:1681-1687.
37. Nagendran J., Archer S.L., Soliman D., et al. Phosphodiesterase type 5 is highly expressed in the hypertrophied human right ventricle, and acute inhibition of phosphodiesterase type 5 improves contractility. Circulation 2007, 116:238-248.
38. Rodefeld M.D., Boyd J.H., Myers C.D., et al. Cavopulmonary assist: circulatory support for the univentricular Fontan circulation. Ann Thorac Surg 2003, 76:1911-1916.
39. Throckmorton A.L., Ballman K.K., Myers C.D., et al. Mechanical cavopulmonary assist for the univentricular Fontan circulation using a novel folding propeller blood pump. ASAIO J 2007, 53:734-741.
40. Lacour-Gayet F.G., Lanning C.J., Stoica S., et al. An artificial right ventricle for failing fontan: in vitro and computational study. Ann Thorac Surg 2009, 88:170-176.