Disturbances of Cardiopulmonary Circulatory Dynamics in Patients with Cystic Fibrosis
Authors:   Academician I.P. Pavlov First St. Petersburg State Medical University (a Federal Government-funded Educational Institution of Higher Education), Russian Federation Ministry of Health, Titova O.N. ,   St. Petersburg, Russian Federation, Kuzubova N.A. ,   Academician I.P. Pavlov First St. Petersburg State Medical University (a Federal Government-funded Educational Institution of Higher Education), Russian Federation Ministry of Health; St. Petersburg, Russian Federation, Alexandrov A.L. ,   Academician I.P. Pavlov First St. Petersburg State Medical University (a Federal Government-funded Educational Institution of Higher Education), Russian Federation Ministry of Health; St. Petersburg, Russian Federation, Perley V.E. ,   Academician I.P. Pavlov First St. Petersburg State Medical University (a Federal Government-funded Educational Institution of Higher Education), Russian Federation Ministry of Health; St. Petersburg, Russian Federation, Gichkin A.Yu. ,   Academician I.P. Pavlov First St. Petersburg State Medical University (a Federal Government-funded Educational Institution of Higher Education), Russian Federation Ministry of Health; St. Petersburg, Russian Federation
Published: January 2022

Study Objective: To use echodoplercardiography to identify the functional cardiopulmonary circulatory dynamics in patients with cystic fibrosis (CF). Study Design: Case control study. Materials and Methods. 48 adult patients with moderately to severely CF (19 male and 29 female patient aged 15–39 years old (mean age: 28 ± 1.1 years old) underwent ultrasound examination to identify their functional cardiopulmonary circulatory dynamics. The control group included 17 apparently healthy subjects aged 18 to 35 years old (mean age: 27 ± 2.7 years old). Patients were divided into two groups depending on the pulmonary artery (PA) pressure and presence of clinical signs of right ventricle (RV) failure: group 1 included 18 patients with systolic pressure in their PA of below 30 mm Hg (normal value) without any clinical signs of RV failure; group 2 included 11 patients with pulmonary hypertension (PH) at rest (systolic pressure in PA: above 30 mm Hg) without any signs of failure in their right heart; group 3 comprised 19 patients with PH and signs of RV failure. Study Results. RV hypertrophy and failure in patients with CF can be seen at relatively low pulmonary artery pressure, where systolic pressure is below 40–50 mm Hg; in many cases hypertrophy and dilatation were simultaneous. With aggravation of diastolic disorders in RV, patients with CF had increased contractive activity in their right atrium together with reduced Е/А ratio. Conclusion. In patients with CF, impaired diastolic function of the right heart often precedes systolic function disturbances, and the ratio of RV filling phases changes. Impaired RV diastolic function causes an increase in the contractive activity of right atrium, which reduces only at marked decompensation of the chronic pulmonary heart and contributes to a poor disease prognosis. Keywords: cystic fibrosis, echocardiography, dopplercardiometry, pulmonary hypertension, right ventricular failure, diastolic function.