Is It Helpful to Apply Pulmonary Vasodilator Agents in Children after Fontan Surgery?_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

ZHANGYa-juan ,  YANJun , LIShou-jun , WANGQiang , YANPeng , JIANGRui

Pediatric Cardiac Center of Fu Wai Cardiovascular Hospital, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, P. R. China;

Corresponding author：

YANJun, Email: yanjun.1112@aliyun.com

Keywords：

Fontan procedure; Pulmonary vasodilator drugs; Bosentan; Sildenafil congenital heart defects

DOI：

10.7507/1007-4848.20150085

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Objective To investigate the effect and safety of the pulmonary vasodilators in pediatric patients after Fontan operation. Methods This retrospective study evaluated the clinical utility of pulmonary vasodilators in pediatric patients with Fontan surgery. Between January and December 2013, 42 consecutive patients with single ventricle physiology who underwent a modified Fontan procedure of total cavapulmonary collection (TCPC) were enrolled. After extubated oral intake started, 24 patients (the treated group) received the pulmonary vasodilator treatment, while 18 patients (the untreated group) didn't not receive the treatment. Results No inpatient death occurred after surgery. The primary endpoints were time of stay in hospital and time of chest tube drainage. There was no statistical difference between the two groups. Instead, patients in the treatment group seemed to have longer time of hospital stay (22 to 21 days) and chest tube drainage (14.0 to 8.5 days) than those in the untreated group. Compared with the untreated group, patients in the treatment group were younger (P=0.082) with no statistical difference, and had higher postoperative Lac with statistic difference (P=0.031), longer ventilation time with no statistical difference (P=0.050), and lower postoperative oxygen saturation with statistic difference (P=0.065). No clinically significant adverse events relating to pulmonary vasodilator therapy occurred during this study and, in particular, no significant abnormalities in hepatic, renal function tests were observed in pediatric Fontan patients. Conclusions Pulmonary vasodilator agents were found to be particularly used in the patients with serious conditions in our study. Our study results failed to show significant improvement of pulmonary vasodilator drugs after Fontan surgery in decreasing time of pleural drainage and time of stay in hospital.

Citation： ZHANGYa-juan, YANJun, LIShou-jun, WANGQiang, YANPeng, JIANGRui. Is It Helpful to Apply Pulmonary Vasodilator Agents in Children after Fontan Surgery?. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2015, 22(4): 306-310. doi: 10.7507/1007-4848.20150085 Copy

1.	Gewillig M.The Fontan circulation.Heart, 2005, 91(6):839-846.
2.	Khambadkone S.The Fontan pathway:What's down the road?Ann Pediatic Cardiol, 2008, 1(2):83-92.
3.	Khairy P, Fernandes SM, Mayer JE Jr, et al.Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.Circulation, 2008, 117(1):85-92.
4.	Almond CS, Mayer JE Jr, Thiagarajan RR, et al.Outcome after Fontan failure and takedown to an intermediate palliative circulation.Ann Thorac Surg, 2007, 84(3):880-887.
5.	Salvin JW, Scheurer MA, Laussen PC, et a1.Factors associated with prolonged recovery after the Fontan operation.Circulation, 2008, l18(Suppl 1):S171-S176.
6.	Earing MG, Cetta F, Driscoll DJ, et al.Long-term results of the Fontan operation for double-inlet left ventricle.Am J Cardiol, 2005, 96(2):291-298.
7.	Sitbon O, McLaughlin VV, Badesch DB, et al.Survival in patients with classⅢidiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol.Thorax, 2005, 60(12):1025-1030.
8.	Denton CP, Humbert M, Rubin L, et al.Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease:a subgroup analysis of the pivotal clinical trials and their open-label extensions.Ann Rheumatic Dis, 2006, 65(10):1336-1340.
9.	Denton CP, Pope JE, Peter HH, et al.Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases.Ann Rheumatic Dis, 2008, 67(9):1222-1228.
10.	Rosenzweig EB, Ivy DD, Widlitz A, et al.Effects of long-term bosentan in children with pulmonary arterial hypertension.J Am Coll Cardiol, 2005, 46(4):697-704.
11.	Galie N, Beghetti M, Gatzoulis MA, et al.Bosentan therapy in patients with Eisenmenger syndrome:a multicenter, double-blind, randomized, placebo-controlled study.Circulation, 2006, 114(1):48-54.
12.	Barst RJ, Langleben D, Badesch D, et al.Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan.J Am Coll Cardiol, 2006, 47(10):2049-2056.
13.	Barst RJ.A review of pulmonary arterial hypertension:role of ambrisentan.Vasc Health Risk Manag, 2007, 3(1):11-22.
14.	Benza RL, Mehta S, Keogh A, et al.Sitaxsentan treatment for patients with pulmonary arterial hypertension discontinuing bosentan.J Heart Lung Transplant, 2007, 26(1):63-69.
15.	Humpl T, Reyes JT, Holtby H, et al.Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension:twelve-month clinical trial of a single-drug, openlabel, pilot study.Circulation, 2005, 111(24):3274-3280.
16.	Apostolopoulou SC, Papagiannis J, Rammos S.Bosentan induces clinical, exercise and hemodynamic improvement in a pretransplant patient with plastic bronchitis after Fontan operation.J Heart Lung Transplant, 2005, 24(8):1174-1176.
17.	Miyaji K, Nagata N, Miyamoto T, et al.Combined therapy with inhaled nitric oxide and intravenous epoprostenol (prostacyclin) for critical pulmonary perfusion after the Fontan procedure.J Thorac Cardiovasc Surg, 2003, 125(2):437-439.
18.	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(5):1232-1233.
19.	Uzun O, Wong JK, Bhole V, et al.Resolution of proteinlosing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan.Ann Thorac Surg, 2006, 82(6):e39-e40.
20.	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(13):1681-1687.
21.	Samánek M.Children with congenital heart disease:probability of natural survival.Pediatr Cardiol, 1992, 13(3):152-158.
22.	Fontan F, Baudet E.Surgical repair of tricuspid atresia.Thorax, 1971, 26:240-248.
23.	De Leval MR.The Fontan circulation:a challenge to William Harvey?Nat Clin Pract Cardiovasc Med, 2005, 2(4):202-208.
24.	Hosein RBM, Clarke AJB, McGuirk SP, et al.Factors influencing early and late outcome following the Fontan procedure in the current era.The two commandments'? Eur J Cardio-Thoracic Surg, 2007, 31(3):344-353.
25.	Ono M, Boethig D, Goerler H, et al.Clinical outcome of patients 20 years after Fontan operation-effect of fenestration on late morbidity.Eur J Cardiothorac Surg, 2006, 30(6):923-929.
26.	McGuirk SP, Winlaw DS, Langley SM, et al.The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection.Eur J Cardiothorac Surg, 2003, 24(1):37-46.
27.	La Gerche A, Gewillig M.What limits cardiac performance during exercise in normal subjects and in healthy fontan patients?Int J Pediatr, 2010, 2010.pii:791291.doi:10.1155/2010/791291.Epub2010Sep7.
28.	Gewillig M, Brown SC, Eyskens B, et al.The Fontan circulation:who controls cardiac output?Interact Cardiovasc Thoracic Surg, 2009, 10(3):428-433.
29.	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(13):1681-1687.
30.	Ovaert C, Thijs D, Dewolf D, et al.The effect of bosentan in patients with a failing Fontan circulation.Cardiol Young, 2009, 19(4):331-339.
31.	Beghetti M.Fontan and the pulmonary circulation:a potential role for new pulmonary hypertension therapies.Heart, 2010, 96(12):911-916.
32.	Maarslet L, Møller MB, Dall R, et al.Lactate levels predict mortality and need for peritoneal dialysis in children undergoing congenital heart surgery.Acta Anaesthesiol Scand, 2012, 56(4):459-464.
33.	Hebert A.The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study.BMC Cardiovascular Disorders, 2013 May 11;13:36.doi:10.1186/1471-2261-13-36.
34.	Goldberg DJ, French B, McBride MG, et al.Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation:a randomized, double-blind, placebo-controlled, crossover trial.Circulation, 2011, 123(11):1185-1193.

1. Gewillig M.The Fontan circulation.Heart, 2005, 91(6):839-846.
2. Khambadkone S.The Fontan pathway:What's down the road?Ann Pediatic Cardiol, 2008, 1(2):83-92.
3. Khairy P, Fernandes SM, Mayer JE Jr, et al.Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.Circulation, 2008, 117(1):85-92.
4. Almond CS, Mayer JE Jr, Thiagarajan RR, et al.Outcome after Fontan failure and takedown to an intermediate palliative circulation.Ann Thorac Surg, 2007, 84(3):880-887.
5. Salvin JW, Scheurer MA, Laussen PC, et a1.Factors associated with prolonged recovery after the Fontan operation.Circulation, 2008, l18(Suppl 1):S171-S176.
6. Earing MG, Cetta F, Driscoll DJ, et al.Long-term results of the Fontan operation for double-inlet left ventricle.Am J Cardiol, 2005, 96(2):291-298.
7. Sitbon O, McLaughlin VV, Badesch DB, et al.Survival in patients with classⅢidiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol.Thorax, 2005, 60(12):1025-1030.
8. Denton CP, Humbert M, Rubin L, et al.Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease:a subgroup analysis of the pivotal clinical trials and their open-label extensions.Ann Rheumatic Dis, 2006, 65(10):1336-1340.
9. Denton CP, Pope JE, Peter HH, et al.Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases.Ann Rheumatic Dis, 2008, 67(9):1222-1228.
10. Rosenzweig EB, Ivy DD, Widlitz A, et al.Effects of long-term bosentan in children with pulmonary arterial hypertension.J Am Coll Cardiol, 2005, 46(4):697-704.
11. Galie N, Beghetti M, Gatzoulis MA, et al.Bosentan therapy in patients with Eisenmenger syndrome:a multicenter, double-blind, randomized, placebo-controlled study.Circulation, 2006, 114(1):48-54.
12. Barst RJ, Langleben D, Badesch D, et al.Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan.J Am Coll Cardiol, 2006, 47(10):2049-2056.
13. Barst RJ.A review of pulmonary arterial hypertension:role of ambrisentan.Vasc Health Risk Manag, 2007, 3(1):11-22.
14. Benza RL, Mehta S, Keogh A, et al.Sitaxsentan treatment for patients with pulmonary arterial hypertension discontinuing bosentan.J Heart Lung Transplant, 2007, 26(1):63-69.
15. Humpl T, Reyes JT, Holtby H, et al.Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension:twelve-month clinical trial of a single-drug, openlabel, pilot study.Circulation, 2005, 111(24):3274-3280.
16. Apostolopoulou SC, Papagiannis J, Rammos S.Bosentan induces clinical, exercise and hemodynamic improvement in a pretransplant patient with plastic bronchitis after Fontan operation.J Heart Lung Transplant, 2005, 24(8):1174-1176.
17. Miyaji K, Nagata N, Miyamoto T, et al.Combined therapy with inhaled nitric oxide and intravenous epoprostenol (prostacyclin) for critical pulmonary perfusion after the Fontan procedure.J Thorac Cardiovasc Surg, 2003, 125(2):437-439.
18. 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(5):1232-1233.
19. Uzun O, Wong JK, Bhole V, et al.Resolution of proteinlosing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan.Ann Thorac Surg, 2006, 82(6):e39-e40.
20. 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(13):1681-1687.
21. Samánek M.Children with congenital heart disease:probability of natural survival.Pediatr Cardiol, 1992, 13(3):152-158.
22. Fontan F, Baudet E.Surgical repair of tricuspid atresia.Thorax, 1971, 26:240-248.
23. De Leval MR.The Fontan circulation:a challenge to William Harvey?Nat Clin Pract Cardiovasc Med, 2005, 2(4):202-208.
24. Hosein RBM, Clarke AJB, McGuirk SP, et al.Factors influencing early and late outcome following the Fontan procedure in the current era.The two commandments'? Eur J Cardio-Thoracic Surg, 2007, 31(3):344-353.
25. Ono M, Boethig D, Goerler H, et al.Clinical outcome of patients 20 years after Fontan operation-effect of fenestration on late morbidity.Eur J Cardiothorac Surg, 2006, 30(6):923-929.
26. McGuirk SP, Winlaw DS, Langley SM, et al.The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection.Eur J Cardiothorac Surg, 2003, 24(1):37-46.
27. La Gerche A, Gewillig M.What limits cardiac performance during exercise in normal subjects and in healthy fontan patients?Int J Pediatr, 2010, 2010.pii:791291.doi:10.1155/2010/791291.Epub2010Sep7.
28. Gewillig M, Brown SC, Eyskens B, et al.The Fontan circulation:who controls cardiac output?Interact Cardiovasc Thoracic Surg, 2009, 10(3):428-433.
29. 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(13):1681-1687.
30. Ovaert C, Thijs D, Dewolf D, et al.The effect of bosentan in patients with a failing Fontan circulation.Cardiol Young, 2009, 19(4):331-339.
31. Beghetti M.Fontan and the pulmonary circulation:a potential role for new pulmonary hypertension therapies.Heart, 2010, 96(12):911-916.
32. Maarslet L, Møller MB, Dall R, et al.Lactate levels predict mortality and need for peritoneal dialysis in children undergoing congenital heart surgery.Acta Anaesthesiol Scand, 2012, 56(4):459-464.
33. Hebert A.The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study.BMC Cardiovascular Disorders, 2013 May 11;13:36.doi:10.1186/1471-2261-13-36.
34. Goldberg DJ, French B, McBride MG, et al.Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation:a randomized, double-blind, placebo-controlled, crossover trial.Circulation, 2011, 123(11):1185-1193.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Is It Helpful to Apply Pulmonary Vasodilator Agents in Children after Fontan Surgery?

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