1 6 , 1 2 ; 3 78 w w w . r e a n i m a t o l o g y . c o mПациентка, 17 лет, была оперирована по поводу тетрады Фалло с атрезией легочной артерии. В раннем послеоперационном периоде наблюдалась клиника гиперволемии малого круга кровообращения. После проведения интенсивной терапии с применением методов неинвазивной вентиляции легких пациентка в удовлетворительном состоянии переведена из ОРИТ на 5 е сутки. Ключевые слова: тетрада Фалло; атрезия легочной артерии; операция Растелли; гиперволемия малого круга кровообращения; неинвазивная искусственная вентиляция легких17 year old patient with hypervolemia of lesser (pulmonary) circulation was operated for tetralogy of Fallout with pulmonary atresia. She was successfully treated by intensive therapy using techniques of noninvasive venti lation with regard to clinical status of hypovolemia of the lesser (pulmonary) circulatory system in the early post operative period.

In the article, in respect of the comparative aspect, there are considered data of non-invasive general clinical diagnostic methods, as well as angiography in patients with adjacent heart defects as tetralogy of Fallot and double outlet right ventricle (Fallot-type). 196 patients with Fallot-type DORV were included in the first group and 124 patients with tetralogy of Fallot – in the second comparative group. The results of general clinical diagnostic methods had descriptive form. The gold standard for differential diagnosis is the echocardiographic study, which, when using the original method, showed the high reliability of differential diagnosis of Fallot-type DORV.

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.