Management of Pulmonary Arterial Hypertension in Children

Paediatric Respiratory Reviews

Volumn 11, Issue 4, 2010, Pages 240-245

  Roofthooft, M T R ^a   van Loon, R L E ^a   Berger, R M F ^a  

^a UNIVERSITY OF GRONINGEN   (Netherlands)

Author keywords

Paediatrics; Pulmonary Hypertension; Pulmonary Vascular Disease; Therapy

Indexed keywords

AMBRISENTAN; ANTICOAGULANT AGENT; BERAPROST; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; DIGOXIN; DIURETIC AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; PHOSPHODIESTERASE V INHIBITOR; PLACEBO; PROSTACYCLIN; PROSTANOID; SILDENAFIL; SITAXSENTAN; TADALAFIL; UNIPROST;

ABDOMINAL CRAMP; AIRWAY RESISTANCE; BRONCHOSPASM; CHILD; CLINICAL TRIAL; CONGENITAL HEART DISEASE; CONNECTIVE TISSUE DISEASE; CONTINUOUS INFUSION; DIARRHEA; DRUG EFFICACY; DRUG HALF LIFE; DRUG MECHANISM; DRUG WITHDRAWAL; EXERCISE; HEADACHE; HEART LUNG TRANSPLANTATION; HEART RIGHT VENTRICLE FAILURE; HOT FLUSH; HUMAN; HYPOTENSION; IDIOPATHIC DISEASE; INFECTION PREVENTION; LUNG HEMODYNAMICS; MYALGIA; OXYGEN THERAPY; PAIN; PATIENT COMPLIANCE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; PULMONARY SHUNT; REVIEW; SIDE EFFECT; SURVIVAL;

EID: 78649433855     PISSN: 15260542     EISSN: 15260550     Source Type: Journal    
DOI: 10.1016/j.prrv.2010.07.002     Document Type: Review

References (55)

1. D'Alonzo G.E., Barst R.J., Ayres S.M., et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann. Intern. Med. 1991, 115:343-349.
2. van Loon R.L., Roofthooft M.T., Osch-Gevers M., et al. Clinical characterization of pediatric pulmonary hypertension: complex presentation and diagnosis. J. Pediatr. 2009, 155:176-182.
3. Simonneau G., Robbins I.M., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2009, 54:S43-S54.
4. Beghetti M., Hoeper M.M., Kiely D.G., et al. Safety experience with bosentan in 146 children 2-11 years old with pulmonary arterial hypertension: results from the European Postmarketing Surveillance program. Pediatr. Res. 2008, 64:200-204.
5. Barst R.J., Gibbs J.S., Ghofrani H.A., et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2009, 54:S78-S84.
6. Galie N., Hoeper M.M., Humbert M., et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur. Respir. J. 2009, 34:1219-1263.
7. Galie N., Hoeper M.M., Humbert M., et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur. Heart J. 2009, 30:2493-2537.
8. Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N. Engl. J. Med. 1992, 327:76-81.
9. Fuster V., Steele P.M., Edwards W.D., Gersh B.J., McGoon M.D., Frye R.L. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984, 70:580-587.
10. Donti A., Formigari R., Ragni L., Manes A., Galie N., Picchio F.M. Pulmonary arterial hypertension in the pediatric age. J. Cardiovasc. Med. (Hagerstown.) 2007, 8:72-77.
11. Young G. New anticoagulants in children. Hematology. Am. Soc. Hematol. Educ. Program. 2008, 245-250.
12. Sandoval J., Aguirre J.S., Pulido T., et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am. J. Respir. Crit Care Med. 2001, 164:1682-1687.
13. Bowyer J.J., Busst C.M., Denison D.M., Shinebourne E.A. Effect of long term oxygen treatment at home in children with pulmonary vascular disease. Br. Heart J. 1986, 55:385-390.
14. Hunt S.A., Abraham W.T., Chin M.H., et al. 2009 focused update incorporated into the ACC/AHA 2005 Guidelines for the Diagnosis and Management of Heart Failure in Adults: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines: developed in collaboration with the International Society for Heart and Lung Transplantation. Circulation 2009, 119:e391-e479.
15. Haworth S.G. The management of pulmonary hypertension in children. Arch. Dis. Child 2008, 93:620-625.
16. Widlitz A., Barst R.J. Pulmonary arterial hypertension in children. Eur. Respir. J. 2003, 21:155-176.
17. Barst R.J., Maislin G., Fishman A.P. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999, 99:1197-1208.
18. Sitbon O., Humbert M., Jais X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005, 111:3105-3111.
19. Tuder R.M., Cool C.D., Geraci M.W., et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am. J. Respir. Crit Care Med. 1999, 159:1925-1932.
20. Christman B.W., McPherson C.D., Newman J.H., et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N. Engl. J. Med. 1992, 327:70-75.
21. Barst R.J., Rubin L.J., Long W.A., et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N. Engl. J. Med. 1996, 334:296-302.
22. Lammers A.E., Hislop A.A., Flynn Y., Haworth S.G. Epoprostenol treatment in children with severe pulmonary hypertension. Heart 2007, 93:739-743.
23. Steffen R.P., de la Mata M. The effects of I5AU8I, a chemically stable prostacyclin analog, on the cardiovascular and renin-angiotensin systems of anesthetized dogs [corrected]. Prostaglandins Leukot. Essent. Fatty Acids 1991, 43:277-286.
24. McNulty M.J., Sailstad J.M., Steffen R.P. The pharmacokinetics and pharmacodynamics of the prostacyclin analog 15AU81 in the anesthetized beagle dog. Prostaglandins Leukot. Essent. Fatty Acids 1993, 48:159-166.
25. McLaughlin V.V., Gaine S.P., Barst R.J., et al. Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension. J. Cardiovasc. Pharmacol. 2003, 41:293-299.
26. Vachiery J.L., Naeije R. Treprostinil for pulmonary hypertension. Expert. Rev. Cardiovasc. Ther. 2004, 2:183-191.
27. Simonneau G., Barst R.J., Galie N., et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am. J. Respir. Crit Care Med. 2002, 165:800-804.
28. Oudiz R.J., Schilz R.J., Barst R.J., et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004, 126:420-427.
29. Barst R.J., Galie N., Naeije R., et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur. Respir. J. 2006, 28:1195-1203.
30. Fitscha P., Tiso B., Krais T., Sinzinger H. Effect of iloprost on in vivo and in vitro platelet function in patients with peripheral vascular disease (PVD). Adv. Prostaglandin Thromboxane Leukot. Res. 1987, 17A:450-454.
31. Olschewski H., Simonneau G., Galie N., et al. Inhaled iloprost for severe pulmonary hypertension. N. Engl. J. Med. 2002, 347:322-329.
32. Ivy D.D., Doran A.K., Smith K.J., et al. Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2008, 51:161-169.
33. Galie N., Humbert M., Vachiery J.L., et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J. Am. Coll. Cardiol. 2002, 39:1496-1502.
34. Barst R.J., McGoon M., McLaughlin V., et al. Beraprost therapy for pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2003, 41:2119-2125.
35. Barst R.J., Ivy D., Dingemanse J., et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin. Pharmacol. Ther. 2003, 73:372-382.
36. Rosenzweig E.B., Ivy D.D., Widlitz A., et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2005, 46:697-704.
37. Segal E.S., Valette C., Oster L., et al. Risk management strategies in the postmarketing period: safety experience with the US and European bosentan surveillance programmes. Drug Saf 2005, 28:971-980.
38. Beghetti M., Haworth S.G., Bonnet D., et al. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study. Br. J. Clin. Pharmacol. 2009, 68:948-955.
39. Barst R.J., Langleben D., Badesch D., et al. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J. Am. Coll. Cardiol. 2006, 47:2049-2056.
40. Barst R.J., Langleben D., Frost A., et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am. J. Respir. Crit Care Med. 2004, 169:441-447.
41. Benza R.L., Barst R.J., Galie N., et al. Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival. Chest 2008, 134:775-782.
42. Barst R.J., Rich S., Widlitz A., Horn E.M., McLaughlin V., McFarlin J. Clinical efficacy of sitaxsentan, an endothelin-A receptor antagonist, in patients with pulmonary arterial hypertension: open-label pilot study. Chest 2002, 121:1860-1868.
43. Galie N., Olschewski H., Oudiz R.J., et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008, 117:3010-3019.
44. Cheng J.W. Ambrisentan for the management of pulmonary arterial hypertension. Clin. Ther. 2008, 30:825-833.
45. McGoon M.D., Frost A.E., Oudiz R.J., et al. Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities. Chest 2009, 135:122-129.
46. Leibovitch L., Matok I., Paret G. Therapeutic applications of sildenafil citrate in the management of paediatric pulmonary hypertension. Drugs 2007, 67:57-73.
47. Kass D.A., Champion H.C., Beavo J.A. Phosphodiesterase type 5: expanding roles in cardiovascular regulation. Circ. Res. 2007, 101:1084-1095.
48. Wharton J., Strange J.W., Moller G.M., et al. Antiproliferative effects of phosphodiesterase type 5 inhibition in human pulmonary artery cells. Am. J. Respir. Crit Care Med. 2005, 172:105-113.
49. Galie N., Ghofrani H.A., Torbicki A., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N. Engl. J. Med. 2005, 353:2148-2157.
50. Stocker C., Penny D.J., Brizard C.P., Cochrane A.D., Soto R., Shekerdemian L.S. Intravenous sildenafil and inhaled nitric oxide: a randomised trial in infants after cardiac surgery. Intensive Care Med. 2003, 29:1996-2003.
51. Schulze-Neick I., Hartenstein P., Li J., et al. Intravenous sildenafil is a potent pulmonary vasodilator in children with congenital heart disease. Circulation 2003, 108(Suppl 1):II167-II173.
52. Humpl T., Reyes J.T., Holtby H., Stephens D., Adatia I. 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.
53. Galie N., Brundage B.H., Ghofrani H.A., et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009, 119:2894-2903.
54. Blanc J., Vouhe P., Bonnet D. Potts shunt in patients with pulmonary hypertension. N. Engl. J. Med. 2004, 350:623.
55. Aurora P., Edwards L.B., Christie J.D., et al. Registry of the International Society for Heart and Lung Transplantation: Twelfth Official Pediatric Lung and Heart/Lung Transplantation Report-2009. J. Heart Lung Transplant. 2009, 28:1023-1030.