Abstract
Aim of this article to determine indications for radical surgical treatment of complete atrioventricular canal
(CAVC) complicated by Eisenmenger syndrome (ES) based on results of invasive monitoring of pulmonary
artery pressure (PAP).
Material and methods. From 2004 to 2015, 46 patients with CAVC complicated by ES were examined and
treated. Mean age was 53.3±45.1 (6–180) months. Mean body weight was 20.1±9.5 (5–40) kg. There were
21 (45.7%) males and 25 (54.3%) females. Determination of operability in patients with CAVC complicated
by ES was based on results of invasive monitoring of PAP. Pulmonary artery hypertension (PAH) stage more
than 80% was the indication for invasive monitoring of PAP, which was performed in all patients divided into
three groups, according to results of the test: one-step approach (n=22), two-step approach (n=14) and
inoperable patients (n=10).
Results. The first group have demonstrated decrease of the mean systolic PAP after radical surgical treatment
from 92.6±8.6 to 59.4±16.4 mm Hg while mortality due to PAH crisis (PHC) took place in 3 (13.6%)
patients in the early postoperative period. Simultaneously, there was only 1 (6.6%) death due to PAH crisis
after the first step of surgical correction in the second group withal other 14 patients have demonstrated significant
decrease of the mean systolic PAP from 90.3±18.4 initially before surgery up to 37.1±12.1 after the
second step of surgical correction (p<0.001). The third group was recognized as inoperable.
Conclusion. Our results allow to consider the two-step surgical approach according to results of the invasive
monitoring of PAP as more favorable tactics of surgical treatment in CAVC complicated by ES. It is expediently
to divide radical surgical treatment in CAVC complicated by ES into two steps with pulmonary artery
banding as primary procedure.
References
-
Bockeria L.A., Gorbachevskiy S.V., Belkina M.V.,Kireeva I.N. Invasive pulmonary artery pressure moni-toring for pulmonary artery hypertension diagnostics incongenital heart disease patients. Guidelines for physi-cians. Moscow; 2004 (in Russ.)
- Gorbachevskiy S.V., Mal'tsev S.G., Koledinskiy D.G.,Gorchakova A.I., Belkina M.V., Sukal'skiy S.V., Kire-eva I.N. Therapeutic test of pulmonary artery hyperten-sion in prognosis of conotruncal heart defects surgicalcorrection results. Detskie Bolezni Serdtsa i Sosudov(Children's Heart and Vascular Diseases).2004; 4: 45–8(in Russ.).
- Gorbachevskiy S.V., Belkina M.V., Smal'ts A.A., Mara-sulov Sh.I., Mal'tsev S.G., Samsonova O.F., Koledin-skiy D.G. Invasive pulmonary artery pressure monito-ring – an objective method for assess the severity of pul-monary artery hypertension in children with congenitalheart disease. Detskie Bolezni Serdtsa i Sosudov (Child-ren's Heart and Vascular Diseases).2006; 4: 77–81(in Russ.).
- Bockeria L.A., Gorbachevskiy S.V., Shkol'nikova M.A.Classification of pulmonary artery hypertension. Guide-lines on pulmonary artery hypertension in children.Moscow; 2013; 27–51, 153–5 (in Russ.).
- Chazova I.E., Martynyuk T.V. Pulmonary arteryhypertension. Moscow; 2015; 155–237, 293–309(in Russ.).
- Simonneau G., Robbins I.M., Beghetti M., Channick R.N.,
Delcroix M., Denton C.P. et al. Updated clinical classification
of pulmonary hypertension. J. Am. Coll.
Cardiol. 2009; 54 (1 Suppl): 43–54. DOI: 10.1016/j.jacc.2009.04.012
- Wood P. The Eisenmenger syndrome or pulmonary
hypertension with reversed central shunt. Br. Med. J.
1958; 2 (5099): 755–62.
- Vongpatanasin W., Brickner M.E., Hillis L.D., Lange R.A.
The Eisenmenger syndrome in adults. Ann. Intern. Med.
1998; 128 (9): 745–55.
- Duffels M.G., Engelfriet P.M., Berger R.M., van Loon
R.L., Hoendermis E., Vriend J.W. et al. Pulmonary
arterial hypertension in congenital heart disease: an epidemiologic
perspective from a Dutch registry. Int.
J. Cardiol. 2007; 120; 198–204. DOI: 10.1016/j.ijcard.2006.09.017
- Humbert M., Sitbon O., Chaouat A., Bertocchi M.,
Habib G., Gressin V. et al. Pulmonary arterial hypertension
in France: results from a national registry. Am.
J. Respir. Crit. Care Med. 2006; 173: 1023–30.
DOI: 0.1164/rccm.200510-1668OC
- Franklin J.W., Patel M.D. An approach to pulmonary
arterial hypertension in the adult patient with congenital
heart disease/ June 13, 2013. American College of
Cardiology//www.acc.org/latest-in-cardiology/article/
2014/07/18/12/56
- Silverman N., Levitsky S., Fisher E., DuBrow I., Hastreiter
A., Scagliotti D. Efficacy of pulmonary artery
banding in infants with complete atrioventricular canal.
Circulation. 1983; 68: II-148–II-153.
- Epstein M.L., Moller J.H., Amplatz K., Nicoloff D.M.
Pulmonary artery banding in infants with complete atrioventricular
canal. J. Thorac. Cardiovasc. Surg. 1979;
78: 28–31.
- Borowski A., Zeuchner M., Schickendantz S., Korb H.
Efficacy of pulmonary artery banding in prevention of
pulmonary vascular obstructive disease. Cardiology.
1994; 85: 207–15. DOI: 10.1159/000176677
- Bockeria L.A., Gorbachevskiy S.V., Abdulatipova I.V.Pediatric hypertensive vascular disease of the lung, asso-ciated with congenital heart disease. Clinical recom-mendations for diagnosis and treatment. Moscow;2014: 16–7.
- Liu Y.L., Hu S.S., Shen X.D., Li S.J., Wang X., Yan J.
et al. Midterm results of arterial switch operation in
older patients with severe pulmonary hypertension.
Ann. Thorac. Surg. 2010; 90 (3): 848–55. DOI: 10.1016/j.athoracsur.2010.03.114.
- Huang J.B., Liu Y.L., Yu C.T. Lung biopsy findings in
previously inoperable patients with severe pulmonary
hypertension associated with congenital heart disease.
Int. J. Cardiol. 2011
About the authors
- Gorbachevskiy Sergey Valer'evich, Dr. Med. Sci., Professor, Chief of the Department for Surgical Treatment of Heart Disease With Concomitant Progressive Pulmonary Artery Hypertension; orcid.org/0000-0002-4193-3320;
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Mazhidov Ulugbek Abdisattarovich, Cardiovascular Surgeon; orcid.org/0000-0003-2883-8019;
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Gorchakova Alla Ivanovna , Cand. Med. Sci., Senior Researcher;
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Khal'vani Mariya Yur'evna, Cardiovascular Surgeon; orcid.org/0000-0003-0335-4472
