Taalaibek Kudaiberdiev¹, Irina Akhmedova¹, Bakyt Imanov^1,2, Damirbek Abibillaev¹, Zhunus Kadyraliev¹, Nazgul Shoonaeva³, Chinara Satarova³, Gulzada Imanalieva¹, Kayirnisa Tilemanbetova¹, Zarema Zhakypova¹, Gulnaz Tursunbekova¹

¹Scientific Research Institute of Heart Surgery and Organ Transplantation

²National Center for Cardiology and Therapy named after M.M.Mirrakhimov

³Kyrgyz State Medical Institute for Requalification

Abstract

In this article, the results of 3-year research program on primary diagnostics of congenital heart defects in children < 1 year of age are presented.

Key words: congenital heart disease, echocardiography, diagnosis, cardiac surgical care

              The congenital heart diseases (CHD) are defined as proposed by Mitchell et al: “a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance” (1). The frequency of CHD at birth (sometimes referred to as the prevalence per childbirth) depends on how the population is studied (2). According to the World Health Organization (WHO), 1.35 million newborns with CHD are born each year (3).

             When examining newborns using the echocardiography, small defects (small defects of the interventricular and interatrial septa, small sized patent ductus arteriosus (PDA)) predominate. With the inclusion of all forms of CHD into study, the prevalence of CHD, according to Hoffman and Kaplan (4) can reach 75 cases per 1000 live births. Performing an echocardiogram in all newborns revealed that about 5% have small ventricular septal defects, most of which spontaneously close for up to a year. Vast majority of newborns have delayed closure of the PDA (4). In 90% of cases CHDs are found in general populations without any particular risk factor, and only in 10% of cases CHD is associated with certain risk factors (5).

               Early detection of CHD and timely cardiac surgery is essential for prognosis in children with heart defects. The total mortality from CHD without intervention is sufficiently high. Usually, in most low- and middle-income countries, the diagnosis of CHD is considerably delayed (6). The average age of the primary diagnosis establishment can vary from the first day of life to almost 80 years (7).

          A previous study of CHD in Kyrgyz Republic territory showed that only 14% of children had been diagnosed before discharge from the maternity hospital, and 86% of patients had a delayed diagnosis (8).

The aim of study is evaluation of the scope of problem and gaining of novel statistical data on prevalence and detectability of CHD in various regions of Kyrgyz Republic

Objectives:       

1.            To assess the significance of contribution of various risk factors in complex CHD formation in intrauterine period

2.            To evaluate the prevalence of CHD in newborns of Kyrgyz Republic

3.            To determine the causes of disability and early mortality in adults with CHD.

The study was conducted in all territories of the Kyrgyz Republic (KR), in the high-altitude, mid-altitude and low-altitude regions, including the most remote areas (Table 1). The researchers organized more than 60 on-site evaluation of the local population.

The population of the country, according to the National Statistics Committee, is 6.4 million citizens. The average annual birth rate in the country over the past three years is 158.160 thousand annually.

Investigation was achieved by 2D transthoracic echocardiography assessment (US scanners of General Electric Vivid Е9, Phillips CX – 50 (portable)), prenatal screening was conducted by convex transducer and fetal echo program. Included groups were: newborns of 0-28 days, children under 1 year, pregnant women with more than 20^th week gestation.

The lack of data after birth is due to failure of the information from maternity hospitals

II. Primary workup of CHD in newborns and children under 1 year:

The study included newborns and children under 1 year old in the investigated area. The presence of a structural anomaly of the heart was regarded as the presence of CHD. Patients with congenital rhythm disturbances, cardiomyopathy, myocarditis, additional persistent vena cava, patent foramen ovale, acquired valvular heart disease were excluded.

A total of 4177 patients were examined, 523 had a primary diagnosis of congenital heart disease, which was 12.52%.

Figure 3. CHD forms detected in primary examination of newborns (0-28 days)

ASD – atrial septal defect, AV – aortic valve, AVSD – atrioventricular septal defect, CHD – congenital heart disease, CoAo – coarctation of aorta, DORV – double-outlet right ventricle, PA- pulmonary atresia, PAPVC – partial anomalous pulmonary vein connection, PDA – patent dusctus arteriosus, PH – pulmonary hypertension, PS – pulmonary stenosis, SV – single ventricle, TAPVC – total anomalous pulmonary vein connection, TGA – transposition of great arteries, TOF – tetralogy of Fallot, VSD – ventricular septal defect