Khasanova K.A.1,2, Ternovoy S.K.2.3, Abrahamyan M.A.1.4


1 - Morozov Children's City Clinical Hospital of the Moscow Health Department.

2 - I.M. Sechenov First Moscow State Medical University (Sechenov University).

3 - A.L. Myasnikova Institute of Clinical Cardiology, National Medical Research Center of Cardiology.

4 - Russian Peoples' Friendship University. Moscow, Russia.


ouble-outlet right ventricle (DORV) is a conotruncal congenital heart defect. In the natural course up to 90% of children are in serious condition due to severe pulmonary hypertension by the 1st year. The management for children with DORV depends on the presence of associated anomalies and the anatomical type of the defect, which must be determined using cardiac imaging techniques at the preoperative stage.

Purpose. To evaluate the capabilities of TTE, CT and MRI of the heart in the diagnosis and surgical planning double-outlet right ventricle in children.

Materials and methods. The study included 28 children with an incoming diagnosis –  DORV. The age of the patients was 68 (39; 129) days. All children underwent TTE, 21 – cardiac CT, 10 – cardiac MRI, 7 – conventional angiography. The capabilities of the techniques in diagnosing the defect, determining its type and combined intra- and extracardiac anomalies were assessed and compared. For children with DORV – type tetralogy of Fallot (TF), the severity of hypoplasia of the pulmonary artery system were additionally assessed. All data from were compared with the results of conventional angiography and surgical data. The interval between TTE, CT, cardio-MRI and conventional angiography or open surgery was less than 10 days.

Results. In determining combined intracardial anomalies, the accuracy of TTE was 100% (AUC 100%), CT – 94% (AUC 93.7%), MRI – 97% (AUC 93%). The accuracy of TTE in determining combined extracardiac anomalies was 92%. MRI has proven to be a highly accurate tool in identifying intracardiac abnormalities: 95.2% accuracy (92.4% AUC). CT didn’t have erroneous results in determining extracardiac anomalies (AUC 100%). The obtained results of measurements of the distal parts of the right and left PA according to TTE, were significantly different from the indicators of conventional angiography and surgery with an underestimation of vessel diameters (p = 0.022).

Discussion. Our results demonstrate the high effectiveness of TTE in diagnosing DORV and determining the anatomical type of the defect, however,  the method  has limited  diagnostic  capabil-


ities in assessing a number of surgical risks when planning radical correction of complex types of defect (type “tetralogy of Fallot”, type “transposition of the great vessels”, Taussing Bing anomaly). Tomographic diagnostic methods (CT and MRI of the heart) have proven to be highly accurate tools in making a diagnosis and in identifying combined intra- and extracardiac anomalies, which play a decisive role in planning open surgical correction of double outlet vessels from the right ventricle.

Conclusion. For patients with DORV – TF type and the TMA type (Taussing-Bing anomaly), TTE should be supplemented with tomographic studies for correct morphometric analysis of the pulmonary artery system and assessment of combined anomalies of the aortic arch and pulmonary arteries. In all other cases, TTE can be considered as final tool in planning the DORV surgery. Conventional angiography should be used in children with double-outlet right ventricle only if it is necessary to clarify the functional rather than anatomical parameters of the defect.

The choice between preoperative CT and MRI for children with double-outlet right ventricle should be made based on the general condition of the child and the presence of contraindications to prolong stay under anesthesia and/or administration of radiocontrast agents during cardiac CT.


Keywords: double-outlet right ventricle, DORV, Taussing-Bing anomaly, VSD, ventricular septal defect, congenital heart defects, CT, MRI, TTE.


Corresponding author: Khasanova K.A., e-mail: Этот e-mail адрес защищен от спам-ботов, для его просмотра у Вас должен быть включен Javascript


For citation: Khasanova K.A., Ternovoy S.K., Abrahamyan M.A. Preoperative planning for the correction of double outlet vessels from the right ventricle in children: the possibilities of modern cardiac imaging methods. REJR 2024; 14(1):98-109. DOI: 10.21569/2222-7415-2024-14-1-98-109.

Received:        10.01.24 Accepted:       12.02.24