Subclinical Right Ventricular Dysfunction in Cystic Fibrosis

Abstract

Patients with severe cystic fibrosis can develop cor pulmonale, but little is known about the function of the right ventricle (RV) early in the disease. We hypothesized that such patients might have subclinical RV dysfunction, detectable by tissue Doppler echocardiography, and related to the severity of lung disease. We studied 21 clinically stable patients (Group 1), five patients with severe lung disease (Group 2), and 23 age-matched healthy subjects. Patients had impaired RV systolic function. The mean (SD) systolic velocities of the RV free wall were 8.9 (1.7) cm/s in Group 1, 7.7 (1.0) in Group 2, and 10.8 (1.9) in healthy subjects (p < 0.001). The velocities of the tricuspid annulus were less in patients (p < 0.0001). Patients had a greater isovolumic relaxation time (p < 0.001), indicating RV diastolic dysfunction. RV wall thickness was greater in patients (0.4 [0.1] versus 0.3 [0.1] cm/m(2), p < 0.01). RV systolic function was related to C-reactive protein (r = - 0.66, p < 0.001) and FEV(1) (r = 0.62, p = 0.003) and diastolic function to interleukin-6 (r = 0.64, p < 0.005). Patients with cystic fibrosis have subclinical RV dysfunction, which correlates with the severity of lung disease. Tissue Doppler echocardiography provides a quantifiable indicator useful for detection and monitoring of disease progression.

Left ventricular diastolic dysfunction in idiopathic pulmonary fibrosis: a tissue Doppler echocardiographic study

“…Conversely, LV systolic function, as measured by both standard 2D and TDI echocardiography, was preserved in the present IPF patients, another finding that is in keeping with previous reports evaluating LV systolic function in clinical entities that affect RV performance [4][5][6][7][8][9][10][11][12].…”

“…Right ventricular (RV) dysfunction has been well described in IPF patients, and in several conditions affecting pulmonary circulation, such as primary PH, chronic obstructive pulmonary disease, chronic thromboembolic PH, systemic sclerosis, systemic lupus erythematosus and cystic fibrosis [4][5][6][7][8][9][10]. Moreover, left ventricular (LV) diastolic dysfunction has been reported in some of these conditions [5-7, 11, 12], mainly as a consequence of RV pressure overload.…”

Validation of Myocardial Acceleration During Isovolumic Contraction as a Novel Noninvasive Index of Right Ventricular Contractility

“…The myocardial velocity during ejection (S wave) changed in a way similar to IVA during inotropic stimulation but failed to decrease during ␤-blockade, was affected by changes in preload and afterload, and did not change significantly with heart rate. Although RV function may be abnormal in many patients with acquired and congenital heart disease 15,16 as well as pulmonary hypertension and lung disease 17,18 and after heart transplantation, 19 all of these diseases may be associated with altered load. The potential advantage of a load-independent index is therefore obvious.…”

Prevalência de hipertensão pulmonar avaliada por ecocardiografia Doppler em uma população de pacientes adolescentes e adultos com fibrose cística

“…(8,9) The patients classified as having PH presented significantly lower clinical scores, as well as lower values for FEV 1 , FVC, and SpO 2 (at rest and at the end of the 6MWT), than did the patients classified as not having PH. The TRV correlated inversely with In the multiple linear regression analysis (stepwise method), clinical score, FEV 1 , radiographic One group of authors (8) studied 18 adult CF patients with severe pulmonary disease in order to achieve the following objectives: to determine the prevalence of PH and cardiac dysfunction; to study the relationship between cardiovascular abnormalities and hypoxemia; and to evaluate the impact of subclinical PH on survival.…”

“…Common findings in the evaluation of patients with PH include dilatation of the right heart chambers, right ventricular hypertrophy, paradoxical septal movement, and tricuspid insufficiency. (4)(5)(6)(7) According to some studies, (8,9) a large percentage of patients with CF present subclinical PH. The great phenotypic variability of the disease (10) justifies a systematic diagnostic approach to evaluate PH in a population of adolescent and adult patients in Brazil.…”