Results of Hemodynamic Correction in Patients with Double Outlet Right Ventricle

  • Kh. K. Abralov Republic Specialized Scientific Practical Medical Center of Surgery named after V. Vakhidov, Tashkent, Uzbekistan
  • O. Kh. Karimov Republic Specialized Scientific Practical Medical Center of Surgery named after V. Vakhidov, Tashkent, Uzbekistan
  • S. O. Siromakha National M. M. Amosov Institute of Cardiovascular Surgery National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
  • I. V. Dziuriy National M. M. Amosov Institute of Cardiovascular Surgery National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
  • Ya. P. Truba National M. M. Amosov Institute of Cardiovascular Surgery National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
  • V. V. Lazorishinets National M. M. Amosov Institute of Cardiovascular Surgery National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
Keywords: congenital heart disease, double outlet right ventricle, echocardiography, diagnosis, pediatric surgery

Abstract

Aim. To analyze results of hemodynamic correction in surgical treatment of double outlet right ventricle (DORV).

Маterials and methods. For the period from January 1996 to September 2017, 31 (6.03 % of total number of patients with DORV) patients underwent hemodynamic correction of DORV. The age of the patients ranged from 1 to 19 years (71.2 ± 50.5 months on the average). The weight of the patients ranged from 9 to 41 kg (19.6 ± 11.3 kg on the average). Of these, 19 (61.3 %) were male patients and 12 (38.7 %) were female patients. The overwhelming majority (25 (80.6 %)) of the patients were diagnosed with transposition-type DORV. The anatomy of DORV with non-committed ventricular septal defect was observed in 5 (16.1 %) patients. In one patient (3.1 %), the anatomy of DORV (in the form of tetralogy of Fallot) was combined with tricuspid valve atresia.

Results. The main reasons of hemodynamic correction in 16 (51.6 %) cases was LV hypoplasia. In 2 cases it was combined with tricuspid valve (TV) straddling, and in 2 cases it was an integral part of the unbalanced form of complete atrio-ventricular communication (AVC). In one case (3.1 %), the unbalanced form of complete AVC was combined with a mixed form of the common ventricle. The mixed form of the common ventricle was the reason of hemodynamic correction in 9 (29 %) patients. In 2 (6.2 %) cases, hemodynamic correction was performed due to the anatomy of the RV hypoplasia. In the remaining 2 patients, anatomy of the common ventricle was not diagnosed, but a combination of other concomitant defects was a contraindication to biventricular correction. Palliative operations (Blalock-Taussig shunt, BTS) as the first stage of correction were performed in 16 (51.6 %) patients. In 2 patients with LV outflow tract obstruction, systemic-pulmonary anastomosis was applied in combination with plastic repair of the great vessel roots using the proprietary technique for elimination of the left ventricular outflow tract (LVOT) stenosis. Bidirectional cavopulmonary anastomosis (BCPA) was applied in 29 (93.5 %) cases. Of these, 4 (13.8 %) patients subsequently underwent total cavopulmonary anastomosis (TCPA) procedure. Two patients with good hemodynamic parameters underwent TCPA without prior palliative procedures.

Conclusion. Palliative surgery as the first stage to hemodynamic correction is accompanied by significant improvement in hemodynamic parameters of patients. Application of BCPA as the second stage of hemodynamic correction provides good results and is required to prepare the patient for TCPA. The long-term period is characterized by improvement in the quality of life in patients with complex DORV. In the long-term period, 85.2 % of patients are classified as NYHA FC I.

References

  1. Kirklin JW, Barratt-Boyes BG. Double-outlet right ventricle. In: Kirklin JW, Barratt-Boyes BG, editors. Cardiac surgery. 3rd ed. New York: Churchill Livingston; 2007. p. 1469–500.
  2. Tchervenkov C, Walters HL 3rd, Chu VF. Congenital Heart Surgery Nomenclature and Database Project: Double outlet left ventricle. Ann Thorac Surg. 2000 Apr;69(4 Suppl):S264–9.
  3. Anderson RH, Cook AC. Morphology of the functionally univentricular heart. Cardiol Young 2004;14(Suppl. 1):3– 12.
  4. Trusler GA, MacGregor D, Mustard WT. Cavopulmonary anastomosis for cyanotic congenital heart disease. J Thorac Cardiovasc Surg. 1971;62:803–9.
  5. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240–8. https://doi.org/10.1136/thx.26.3.240
  6. Yie K, Sung S, Kim D, Woo J. Bidirectional cavopulmonary shunt as a rescue procedure for right ventricular endomyocardial fibrosis. Interact Cardiovasc Thorac Surg. 2004 Mar;3(1):86–8. https://doi.org/10.1016/S1569-9293(03)00189-0
  7. Anderson RH, Ho SY. Which hearts are unsuitable for biventricular correction? Ann Thorac Surg 1998;66(2):621– 6.
  8. Villemain O, Bonnet D, Houyel L, Vergnat M, Ladouceur M, Lambert V, et al. Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous? Semin Thorac Cardiovasc Surg. Spring 2016;28(1):69–77. https:// doi.org/10.1053/j.semtcvs.2016.01.007
  9. Tjark Ebels. Double-outlet right ventricle univentricular repaire. EACTS/ESTS joint meeting. Postgraduate courses; 2001 Sept 16–19; Lisbon. p. 61–3.
  10. Jaquiss R, Imamura M. Single ventricle physiology: surgical options, indications and outcomes. Curr Opin Cardiol. 2009 Mar;24(2):113–8. https://doi.org/10.1097/HCO.0b013e328323d85a
Published
2019-05-22
How to Cite
Abralov, K. K., Karimov, O. K., Siromakha, S. O., Dziuriy, I. V., Truba, Y. P., & Lazorishinets, V. V. (2019). Results of Hemodynamic Correction in Patients with Double Outlet Right Ventricle. Ukrainian Journal of Cardiovascular Surgery, (2 (35), 55-60. https://doi.org/10.30702/ujcvs/19.3505/042055-060