Features of pulmonary circulation in children with congenital pulmonary venolobar syndrom

Abstract

Introduction. Despite the continuous improvement of methods for prenatal diagnosis of fetal malformations, at present, the limitations of intrauterine verifcation of vascular lung anomalies and relevance in postnatal typing of all components of pathological pulmonary hemodynamic, especially in the case of complex, combined vascular malformations, remain. Detailed and expert assessment of the anatomy of the pulmonary vessels, intrapulmonary hemodynamic is necessary to minimize the time for the surgical correction of the defect in order to avoid the development of irreversible pulmonary hypertension. One of the most rare and diffcult to diagnose forms of a combination of vascular abnormalities is congenital pulmonary venolobar syndrome (CPVS), which combines venous, arterial, pulmonary and cardiac pathology, to one degree or another coexisting in one patient.Objective – analysis of clinical material and data of different diagnostic methods in children with CPVS. This everything will help to optimize diagnostics and choose the most safe and informative method to visualize combination of pulmonary and cardiovascular diseases and to determine the specifcity of pulmonary circulation. Furthermore, it will allow to minimize all risks and negative consequences for patient’s body.Material and methods. We reviewed the records of 11 children (age from 14 days to 17 years old) with intraoperative verifcation of CPVS. We systematized all variants of vascular anomalies of lungs in children with CPVS, its possible combinationswith other abnormalities. We performed the comparison of capability and diagnostic signifcance of plain radiography, echocardiography, selective angiography and CT­scan in determining the specifcity of pulmonary circulation in children with CPVS and assessment of combination of the malformation components. Results. Antenatal echocardiography and postnatal diagnostics using echocardiography, plain radiography and selective angiography is extremely limited. We suggested the diagnostic algorithm using CT­scan with the proof of the great representativeness. Obligatory components of CPVS were identifed: reduction of the right lung volume, anomalous pulmonary venous drainage (scimitar syndrome) and non­obligate components of malformation: aplasia or hypoplasia of the right pulmonary artery, systemic pulmonary arterial supply with differential pulmonary perfusion, pulmonary sequestration, other congenital heart abnormalities.Conclusion. CPVS is a rare congenital malformation. Antenatal diagnosis of all components of CVLS is impossible. MDCTAG is the most effective diagnostic method that allows to identify all components of CPVS in postnatal period. This method does not require additional radiodiagnostics and invasive diagnostic procedures.

Синдром Ятагана: Подходы К Диагностике И Ведению

“…Despite the continuous improvement of the methods of prenatal diagnosis of fetal malformations, the limited possibilities of intrauterine verification of vascular anoma lies of the lungs still take place. One of the most rare and difficult to diagnose forms of combination of vascular anomalies is partial abnormal pulmonary vein drainage, which is sometimes a component of scimitar syndrome (ICD-10: Q26.8) [1].…”

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Âèïàäîê ³ç ïðàêòèêè / Case ReportDespite the continuous improvement of the methods of prenatal diagnosis of fetal malformations, the limited possibilities of intrauterine verification of vascular anoma lies of the lungs still take place. One of the most rare and difficult to diagnose forms of combination of vascular anomalies is partial abnormal pulmonary vein drainage, which is sometimes a component of scimitar syndrome (ICD-10: Q26.8) [1].Scimitar syndrome (hypogenetic pulmonary syndrome, venolobar syndrome or "yataghan" syndrome) belongs to a group of venolobar syndromes associated with lung sequestration and congenital heart disease. This is a congenital anomaly characterized by the fact that one or more pul-monary veins drain not into the left atrium, as normal, but directly into the inferior vena cava above or below the diaphragm.

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