PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL HEART DISEASE: RATIONALE FOR MANAGEMENT ALGORITHM BASED ON A LITERATURE REVIEW

The substantial progress achieved in cardiology and paediatric cardiac surgery has led to a considerable increase of life expectancy of patients with congenital heart disease (CHD), and at the same time to growing number of adult patients developing pulmonary arterial hypertension (PAH) associated with congenital heart defects. Nowadays guidelines were developed and updated for PAH, but no expert recommendations exist for effective management of PAH associated with CHD. Thus, it is important to develop an algorithm for managing these patients, based on international standards and a comprehensive understanding of prognostic markers of worsening the course of pulmonary arterial hypertension (PAH), considering local cardiac tertiary hospitals' capabilities. The aim. To highlight the pathophysiology and clinical characteristics of PAH-CHD patients, identify prognostic markers for its onset and progression, and develop a management algorithm for patients. Results. The article presents the pathophysiology of PAH-CHD, the mechanisms of right ventricle adaptation to pressure overload, which determine the functional abilities of patients and prognosis, PAH formation, and progression after defects correction. The accurate identification of stages in PAH-CHD (volume overload, mixed, stage of pulmonary vascular disease) is essential for the timely determination of surgical intervention, selecting conservative treatment strategies, and predicting the outcomes of surgical defect correction. The type of defect, its size, and the volume of blood flow through the defect influence the onset and progression of PH-CHD. We review the pathophysiology and clinical features of PAH-CHD, as well as the reasons for the late diagnosis of heart failure at the stage of severe decompensation and irreversible remodelling of the lung vessels when diuretic therapy is not effective enough and inotropic support, circulatory mechanical support and heart / lung complex transplantation are required. The necessity of developing new scales for stratifying the risk of PAH-CHD is justified considering the particularities of the disease course and cardiac hemodynamic in different anatomical types of the defects. Based on this literature review, at the Municipal Enterprise «Dnipropetrovsk Regional Clinical Center for Diagnostics and Treatment» of the Dnipropetrovsk Regional Council» the algorithm for managing patients with PAH-CHD was devised and implemented into clinical practice. Conclusions. PAH is a common complication of CHD. The prevalence of PAH-CHD is substantial and it can manifest and progress even in patients with previously corrected heart defects. To effectively manage patients with PAH-CHD, an algorithm has been devised and implemented in clinical practice. It includes factors that have demonstrated associations with poor prognosis which are more than two hospitalizations for HF in the last year, pulmonary vascular resistance (PVR) (or index of PVR), mean pressure in the pulmonary artery, pulmonary capillary wedge pressure, left ventricular ejection fraction, heart failure with a reduced ejection fraction, right ventricle heart failure, NYHA FC, high risk of atrial fibrillation or flutter, 6-minute walking test distance, NTproBNP, highly sensitive C-reactive protein, B-lines on lung ultrasound, glomerular filtration rate, haemoglobin, platelet count, sodium, Model for End-Stage Liver Disease scale.