Register
Forgot your password?

Effectiveness of two-step surgical approach in completeatrioventricular canal complicated by Eisenmenger syndrome

Authors: S.V. Gorbachevskiy, U.A. Mazhidov, A.I. Gorchakova, M.Yu. Khal'vani

Company: Bakoulev National Medical Research Center for Cardiovascular Surgery, Rublevskoe shosse, 135, Moscow, 121552, Russian Federation

Type:  Original Research


DOI: https://doi.org/10.24022/0236-2791-2018-60-4-294-300

For citation: Gorbachevskiy S.V., Mazhidov U.A., Gorchakova A.I., Khal’vani M.Yu. Effectiveness of two-step surgical approach in complete atrioventricular canal complicated by Eisenmenger syndrome. Russian Journal of Thoracic and Cardiovascular Surgery. 2018; 60 (4): 294–300 (in Russ.). DOI: 10.24022/0236-2791-2018-60-4-294-300

Received / Accepted:  19.04.2018/04.05.2018

Keywords: atrioventricular septal defect pulmonary artery hypertension congenital heart disease Eisenmenger syndrome

Download
Full text:  

 

Abstract

Objective. 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, 47 patients with CAVC complicated by ES were examined and treated. Mean age was 53.3±45.1 (6–180) months, mean body weight was 17.1±9.5 (3.5–40) kilograms. There were 21 (44.7%) males and 26 (55.3%) females. Determination of operability in patients with CAVC complicated by ES was based on the results of invasive monitoring of PAP. Pulmonary artery hypertension (PAH) more than 80% was the indication for invasive monitoring of PAP which was performed in all patients divided into three groups according to the results of the test: one-step approach (n = 22), two-step approach (n =15), and inoperable patients (n =10).

Results. The 1st group demonstrated the decrease of the mean systolic PAP after radical surgical treatment from 92.6±8.6 to 59.4 ±16.4 mm Hg, but 3 (13.6%) patients died due to PAH crisis in the early postoperative period. In the 2nd group there was only 1 (6.6%) death due to PAH crisis after the first step of surgical correction, while other 14 patients demonstrated significant decrease of the mean systolic PAP from 90.3±18.4 initially up to 37.1±12.1 after the second step of surgical correction (p<0.001). The 3rd group was recognized as inoperable.

Conclusion. Our results allow to consider the two-step surgical approach after 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

  1. Бокерия Л.А., Горбачевский С.В., Белкина М.В., Киреева И.Н. Инвазивный мониторинг давления в легочной артерии в диагностике легочной гипертензии у больных с врожденными пороками сердца. Методические рекомендации для врачей. М.: НЦССХ им. А.Н. Бакулева РАМН; 2004. [Bockeria L.А., Gorbachevskiy S.V., Belkina М.V., Kireeva I.N. Invasive monitoring of pulmonary artery pressure in the diagnosis of pulmonary hypertension in patients with congenital heart disease. Мoscow; 2004 (in Russ.).]
  2. Горбачевский С.В., Мальцев С.Г., Колединский Д.Г., Горчакова А.И., Белкина М.В., Сукальский С.В., Киреева И.Н. Медикаментозный тестовый анализ легочной гипертензии в прогнозе результатов хирургической коррекции пороков конотрункусa. Детские болезни сердца и сосудов. 2004; 4: 72–6. [Gorbachevskiy S.V., Mal'tsev S.G., Koledinskiy D.G., Gorchakova А.I., Belkina М.V., Sukal'skiy S.В., Kireeva I.N. Drug test-analysis of pulmonary hypertension in the prediction of results of surgical correction of conotruncal defects. Children's Heart and Vascular Diseases. 2004; 4: 72–6 (in Russ.).]
  3. Горбачевский С.В., Белкина М.В., Колединский Д.Г., Шмальц А.А., Марасулов Ш.И., Мальцев С.Г., Самсонова О.А. Инвазивный мониторинг давления в легочной артерии – объективный метод оценки степени тяжести легочной гипертензии у детей с врожденными пороками сердца. Детские болезни сердца и сосудов. 2006; 4: 77–81. [Gorbachevskiy S.V., Belkina М.V., Koledinskiy D.G., Shmaltz А.А., Marasulov Sh.I., Mal'tsev S.G., Samsonova О.А. Invasive monitoring of pulmonary artery pressure is an objective method for assessing the severity of pulmonary hypertension in children with congenital heart disease. Children's Heart and Vascular Diseases. 2006; 4: 77–81 (in Russ.).]
  4. Бокерия Л.А., Горбачевский С.В., Школьникова М.А. (ред.) Легочная гипертензия у детей. М.; 2013. [Bockeria L.А., Gorbachevskiy S.V., Shkol'nikova М.А. (Eds.) Pulmonary hypertension in children. Мoscow; 2013 (in Russ.).]
  5. Чазова И.Е., Мартынюк Т.В. Легочная гипертензия. М.: Практика; 2015: 155–237, 293–309. [Chazova I.Е., Martynyuk Т.V. Pulmonary hypertension. Мoscow: Praktika; 2015: 155–237, 293–309 (in Russ.).]
  6. 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
  7. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br. Med. J. 1958; 2 (5099): 755–62.
  8. Vongpatanasin W., Brickner M.E., Hillis L.D., Lange R.A. The Eisenmenger syndrome in adults. Ann. Intern. Med. 1998; 128 (9): 745–55. DOI: 10.7326/0003-4819-128-9-199805010-00008
  9. 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 (2); 198–204. DOI: 10.1016/j.ijcard.2006.09.017
  10. 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 (9); 1023–30. DOI: 10.1164/rccm.200510-1668OC
  11. Franklin W.J., Patel M.D. An approach to pulmonary arterial hypertension in the adult patient with congenital heart disease. Am. Coll. Cardiol. 2013; Jun. 13.
  12. 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 (3 Pt. 2): II148–53.
  13. 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 (1): 28–31.
  14. Borowski A., Zeuchner M., Schickendantz S., Korb H. Efficacy of pulmonary artery banding in prevention of pulmonary vascular obstructive disease. Cardiology. 1994; 85 (3–4): 207–15. DOI: 10.1159/000176677
  15. Педиатрическая гипертензионная сосудистая болезнь легких, ассоциированная с врожденными пороками сердца. Клинические рекомендации по диагностике и лечению. М.; 2014: 16–7. [Pediatric hypertensive vascular lung disease associated with congenital heart disease. Clinical practice guidelines for the diagnosis and treatment. Мoscow; 2014: 16–7 (in Russ.).]
  16. 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
  17. Huang J.B., Liu Y.L., Yu C.T., Lv X.D., Du M., Wang Q., Kong B. Lung biopsy findings in previously inoperable patients with severe pulmonary hypertension associated with congenital heart disease. Int. J. Cardiol. 151 (1): 76–83. DOI: 10.1016/j.ijcard.2010.04.094

About Authors

  • Sergey V. Gorbachevskiy, Dr. Med. Sc., Professor, Head of Department, orcid.org/0000-0002-4193-3320;
  • Ulugbek A. Mazhidov, Cardiovascular Surgeon, orcid.org/0000-0003-2883-8019;
  • Alla I. Gorchakova , Cand. Med. Sc., Senior Researcher;
  • Maria Yu. Khal’vani, Cardiovascular Surgeon, orcid.org/0000-0003-0335-4472

Chief Editor

Elena Z. Golukhova, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, Director of Bakoulev National Medical Research Center for Cardiovascular Surgery


Sort by