Abstract
Introduction. Pulmonary arterial hypertension (PAH) is a common complication of congenital heart diseases (CHD), with most cases
occurring in patient with congenital cardiac shunts. Increased pulmonary vascular resistance affects the postoperative period, determines the duration of mechanical ventilation and frequency of complications associated with it, the possibility of pulmonary hypertensive crisis.
Material and methods. 87 patients of the first year of life were including in this retrospective observational study. All patients underwent radical correction for CHD and had confirmed high PAH in the early postoperative period. Infants were divided in 2 groups:
group 1 consisted of 77 patients, who didn't received bosentan, and group 2 of 10 patients with bosentan administration. The median
weight amounted 3.8 kg and 3.9 kg in the first and second groups respectively. All patients underwent radical correction of heart
defects: septal defects, atrioventricular canal, total arterial trunk, coarctation of the aorta, total anomalous pulmonary venous
drainage, aortopulmonary window.
Results. Patients of the second group had a lower systolic pulmonary artery pressure (MPAP) after 48 hours of operation (p=0.023,
95% confidence inteval (CI) [0.55-0.96] odds ratio (OR) - 0.73). The management patients with bosentan in complex therapy of
pulmonary hypertension in the early postoperative period allowed to reduce the duration of mechanical ventilation (p=0.01, 95% CI
[0.92-0.99], OR=0.96), duration the use of inotropic agents (p=0.046, 95% CI [0.06-0.98], OR=0.25) and the need for prolonged
muscle relaxation (p=0.017, 95% CI [0.04-0.74], OR=0.17).
Conclusion. The use of bosentan as part of complex management treatment of patients after correction of CHD has resulted in faster
decreasing of MPAP, reducing the duration of mechanical lung ventilation, inotropic support and muscle relaxante.
References
- Rubin L.J. Primary pulmonary hypertension. N. Engl.
J. Med. 1997; 336: 1 1 1 - 1 7 .
- HumbertM., MorrellN.W., ArcherS.L., Stenmark K.R.,
MacLean M.R., Lang I.M. et al. Cellular and molecular
pathobiology of pulmonary arterial hypertension. J.
Am. Coll. Cardiol. 2 0 0 4 ; 4 3 : 1 3 - 2 4 .
- Beghetti M . , Tissot C. Pulmonary Hypertension in
Congenital Shunts. Rev. Esp. Cardiol. 2010; 63:
1 1 7 9 - 9 3 .
- Galie N., HoeperM.M., Humbert M. et al. Guidelines for
the diagnosis and treatment of pulmonary hypertension. Eur. Respir. J. 2009; 34: 1 2 1 9 - 6 3 .
- Yung D., Widlitz A.C., Rosenzweig E.B., Kerstein D.,
Maislin G., Barst R.J. Outcomes i n c h i l d r e n w i t h idiopathic
pulmonary arterial hypertension. Circulation.
2 0 0 4 . 110: 6 6 0 - 5 .
- Dan-Chen Wu, Hong-Da Zhang, Zhi-Cheng Jing.
Pediatric pulmonary arterial hypertension. Curr.
Hypertens. Rep. 2013; 15: 6 0 6 - 1 3 .
- Beghetti M. Bosentan i n pediatric patients with pulmonary arterial hypertension. Curr. Vasc. Pharmacol.
2009; 7: 2 2 5 - 3 3 .
- Бураковский В.И., Бухарин В.А., Плотникова Л.Р.
Л е г о ч н а я г и п е р т е н з и я при в р о ж д е н н ы х п о р о к а х
с е р д ц а . М.: Медицина; 1975.
- Barst R.J., Channick R., Ivy D., Goldstein B. Clinical
perspectives w i t h l o n g - t e rm pulsed inhaled nitric oxide
for the treatment of pulmonary arterial hypertension.
Pulm. Circ. 2 0 1 2 ; 2 ( 2 ) : 1 3 9 - 1 4 7 .
- Davidson D., Barefield E.S., Kattwinkel J. et a l . Inhaled
nitric oxide for the early treatment of persistent pulmonary hypertension of the t e rm newborn: a randomized, double-masked, placebo-controlled, doseresponse,
multicenter study. The I-NO/PPHN Study
Group. Pediatrics. 1998; 1 0 1 : 3 2 5 - 3 4 .
- Горбачевский С.В. П р о б л е м а л е г о ч н о й г и п е р т е н з и и
в х и р у р г и и ВПС у д е т е й р а н н е г о в о з р а с т а : А в т о р е ф .
д и с . ... д - р а мед. н а у к . М.; 1997.
- Буров А.Л., Жиркова Ю.В., Титков К.В., Теплякова О.В., Никифоров Д.В., Плахова Н.А., Мацкевич Е.Г. и д р . Л е г о ч н а я г и п е р т е н з и я у н о в о р о ж д е н ных: к л и н и к а , д и а г н о с т и к а , л е ч е н и е . М.; 2 0 1 1 .
- Мартынюк Т.В., Коносова И.Д., Чазова И.Е. Соврем е н н ы е п о д х о д ы к м е д и к а м е н т о з н о м у л е ч е н и ю легочной г и п е р т е н з и и . Consilium medicum. 2003; 5: 1-4.
- Widlitz A., Barst R.J. Pulmonary arterial hypertension
i n children. Eur. Respir. J. 2003; 2 1 : 1 5 5 - 7 6 .
- Yamaki S., Abe A., Tabayashi K., Endo M., Mohri H.,
Takahashi T. Inoperable pulmonary vascular disease in
infants with congenital heart disease. Ann. Thorac.
Surg. 1998; 66 ( 5 ) : 1 5 6 5 - 7 0 .
- Barst R.J., Ivy D., Dingemanse J., Widlitz A.,
Schmitt K., Doran A. et al. Pharmacokinetics, safety,
and effects of bosentan in pediatric patients w i t h pulmonary arterial hypertension. Clin. Pharmacol. Ther.
2003; 73: 3 7 2 - 8 2 .
- Rosenzweig E.B., Ivy D.D., Widlitz A., Doran A., Claussen
L.R., YungD. et al. Effects of l o n g - t e rm bosentan in
children w i t h pulmonary arterial hypertension. J. Am.
Coll. Cardiol. 2 0 0 5 ; 4 6 : 6 9 7 - 7 0 4 .
- Maiya S., Hislop A.A., Flynn Y., Haworth S.G.
Response to bosentan in children with pulmonary
hypertension. Heart. 2006; 92: 6 6 4 - 7 0 .
- Beghetti M., Hoeper M.M., Kiely D.G., Carlsen J.,
Schwierin B., Segal E.S. et al. Safety experience w i t h
bosentan in 146 children 2 - 1 1 years old with pulm o n a r y arterial hypertension: results from the
European Postmarketing Surveillance program. 2007.
Eur. Respir. J. 30: 3 3 8 - 4 4 .
- Агапитов Л.И. Д и а г н о с т и к а и л е ч е н и е л е г о ч н о й а р т
е р и а л ь н о й г и п е р т е н з и и у д е т е й . Лечащий врач.
2014; 4: 5 2 - 6 8 .
- Takatsuki S., Rosenzweig E.B., Zuckerman W. et a l .
Clinical safety, p h a r m a c o k i n e t i c s , and efficacy of
ambrisentan t h e r a p y i n c h i l d r e n w i t h p u l m o n a r y arterial h y p e r t e n s i o n . Pediatr. Pulmonol. 2 0 1 3 ; 4 8 :
2 7 - 3 4 .