Abstract
Objective. To present surgical correction experience of the A.N. Bakulev SCCVS, RAMS for such sophisticated congenital
heart defect as anomalous origin of left coronary artery from pulmonary artery (Bland-White-Garland syndrome
- BWGS).
Material and methods. Surgical results of 127 patients of various age categories who had underwent coronary
blood supply repair for the above defect at the period between 1972 and 2009 were analyzed. Mean age of the
patients at the moment of the surgery was 62.5±98.6 months (mean 18 months), body weight was 14.6±17.2 kg
months (mean 8 kg), body surface area was 0.56±0.46 m2 (mean 0.44 m2). Examined patients included 42 male
(33.1%) patients and 85 female patients (66.9%). All patients were divided into two groups depending on the final
aim of the performed procedure: to create single coronary (n=26) or two coronary system of myocardial perfusion.
Surgical treatment of developed complications of disease was performed together with coronary correction pathology,
particularly hemodynamically significant regurgitation on the mitral valve - 26 patients (20.5%), 9 (34.6%)
had valve-sparing procedures and 17 (65.4%) had the valve replacement of prosthesis device.
The presence of left ventricular aneurysm in 11 patients (8.7%) was the cause for supplementary intracardiac
manoeuvres according to its correction with the use of the following methods: 1) aneurysmorrhaphy - 2 patients
(1.5%); 2) aneurysmoplasty of LV (Jatene procedure) - 3 patients (2.4%); 3) aneurysmoplasty of LV (Dor procedure)
- 6 patients (4.8%).
60 patients had long-term follow-up that is 62.5% of the total number of discharged patients. The follow up period
was from 3 to 25 years; mean value was 52.4±49.3 months (Me - 36.0 months).
Results. 31 patients died in the close postoperative period: 6 (23.1%) patients died after the surgery creating single
coronary myocardial blood supply system (ligation or suturing abnormal LCA outlet) and 25 (24.7%) - following
the operation restoring two coronary system (p=0.955). Analysis of the late postoperative period showed significant
survival reduction on the 5-6 th year of life in the group of patients with single coronary myocardial blood supply
system.
Conclusion. Restoration of the two-coronary myocardial blood supply system using various techniques
is «the golden standard» of the surgical treatment for СБУГ today. Method choice was determined first of all
by the defect anatomy, clinical state of the patient as well as experience and the surgeon's devotion to one
of methods. Preferred coronary blood supply normalization technique for small children is direct implantation
of the left coronary artery (LCA) to aorta (Ao). For surgical correction for abnormal LCA outlet from LA it is
necessary to remove mitral regurgitation with valve sparing operations to all the patients with initial mitral valve
incompetence with regurgitation over 2(+) and if it is impossible to perform them to implant prosthetic cardiac
valve.
