MALPOSITION OF PULMONARY ARTERIES IN THE PRESENCE OF CONGENITAL HEART DISEASES WITH PULMONARY ARTERY STENOSIS: DIAGNOSIS AND EXPERIENCE OF SURGICAL TREATMENT
Objective. To present outcomes of diagnosis and experience of surgical treatment of patients with malposition of pulmonary arteries in the presence of congenital heart diseases (CHD) with pulmonary artery stenosis. Material and methods. From 1992 to 2013 in A.N. Bakoulev Scientific Center for Cardiovascular Surgery of Russian Academy of Medical Sciences 10 cases were diagnosed as having malposition of pulmonary arteries in the presence of CHD with reduced pulmonary blood flow. All 10 patients have had concomitant ventricular septal defect (VSD) and stenosis of PA. The total of 6 patients underwent palliative operations, while three of them underwent 2 or 3 palliative interventions. Median age of patients in this group made up 2.4 years. Three patients underwent radical repair of concomitant CHD after palliative interventions. Mean interval between palliative and radical operations made up 1.7 years. Seven patients underwent radical repair of concomitant CHD. Median age of 7 patients at the time of performance of radical repair made up 3.7 years. Results. Malposition of pulmonary arteries in the total of 10 patients was diagnosed by Doppler echocardiographic and angiocardiographic examination. Malposition with crisscross PA was diagnosed in 7 patients, malposition without crisscross PA was diagnosed in 3 patients. There was no hospital mortality after performance of palliative interventions and radical repair. Cardiopulmonary insufficiency having developed in postoperative period requested the administration of large doses of inotropic agents and prolonged mechanical ventilation of lungs, i.e. in 2 patients after palliative operations and in 5 patients after radical repair. Right ventricular outflow tract diameter measured on the operation table after finishing of repair of right ventricular outflow tract without closure of VSD in 3 patients made up 9.0 ± 1.0 mm (from 8 to 10 mm), Z-score from diameter of PA valve in the norm made up -2.7; 1.5 and -1.9 respectively, systolic pressure in PA after operation made up 29.0 ± 1.7 mm Hg with the systolic pressure in the aorta 96.7 ± 20.2 mm Hg. Before radical repair the Nakata index made up 280 ± 37 mm <sup>2</sup>/ m <sup>2</sup>, median Z-score of normal value of the Nakata index – –1.4. The systolic pressure in right ventricle made up 45.0 ± 14.6 mm Hg and the ratio of systolic pressure in ventricles after radical repair made up 0.50 ± 0.18. Conclusion. Recent methods of diagnosis allows in patient’s lifetime to diagnose the malposition of pulmonary arteries. The presence of pathology should not change the treatment strategy and repair of intracardiac defects has involved minimal risk.