Cardiohepatic syndrome and its prognostic predictive ability in patients with pulmonary arterial hypertension.

With the development of progressive right ventricular dysfunction, pulmonary arterial hypertension (PAH) is one of the causes of type 2 cardiohepatic syndrome (CHS). Risk assessment, timely and effective management are crucial to improve survival in PAH. Thus, we aimed to evaluate the presence of CHS at diagnosis and its association with prognosis in patients with PAH. One hundred and eighteen consecutive incident patients with PAH between January 2013 and June 2021 were retrospectively included. The presence of CHS was assessed from blood tests taken during diagnostic evaluation and was defined as elevation of at least two of three cholestatic liver parameters; total bilirubin, alkaline phosphatase and gamma-glutamyl transferase. The primary endpoint was all-cause mortality. Patients were followed for a median period of 58 (32-96) months. 23.7% of the patients had CHS at diagnosis. Significantly more patients in CHS (+) group were in intermediate and high-risk categories according to 2015 ESC/ERS guideline, REVEAL 2.0 and REVEAL Lite 2 risk assessment methods (p = .02, .03 and <.001, respectively). The presence of CHS was identified as an independent predictor of mortality (HR: 2.17, 95% CI: 1.03-4.65, p = .03) along with older age (HR: 2.89, 95% CI: 1.50-5.56, p = .001) and higher WHO functional class (HR: 2.57, 95% CI: 1.07-6.22, p = .03). To conclude, presence of CHS at diagnosis in patients with PAH was associated with severe disease and poor prognosis independent of other well known risk factors. As a simple and easy parameter to assess from routinely taken blood tests, CHS should be evaluated in patients with PAH.

Pulmonary hypertension in patients with sarcoidosis: A single-center experience.

OBJECTIVE: Sarcoidosis is a systemic granulomatous disease rarely complicated by pulmonary hypertension (PH). The prevalence of PH in sarcoidosis is unclear and has differences between ethnic groups. This study aimed to investigate the prevalence and predictors of PH in a Turkish cohort. METHODS: The study included 55 patients with biopsy-proven sarcoidosis in a single center. All patients underwent detailed transthoracic echocardiography (TTE) to assess the probability of PH as recommended. Right heart catheterization (RHC) was performed for patients with intermediate-high risk of PH. Patients with mean pulmonary artery pressure >20 mm Hg by RHC were defined as PH. Demographic and clinical characteristics, laboratory data, spirometry, 6-min walk test, and TTE were compared between low and intermediate-high risk PH groups. RESULTS: The probability of PH was low with 47 patients. Eight patients had intermediate-high probability of PH, and two of them refused to undergo RHC. Of six intermediate-high probability patients, three had PH, and all of them had post-precapillary PH. The prevalence of PH in sarcoidosis was 5.5% (3/55). Six-minute walk distance (6 MWD) and diastolic parameters (E/A ratio, E' wave, and left atrial volume) were significantly lower, and New York Heart association class and N-terminal probrain natriuretic peptide (NT-proBNP) level were higher in intermediate-high risk PH patients compared with low-risk PH patients. CONCLUSION: The frequency of PH in sarcoidosis was 5.5% in a Turkish cohort. NT-proBNP, 6 MWD, diastolic function parameters, and myocardial strain parameters can be useful predictors of PH in patients with sarcoidosis, besides known echocardiographic parameters.

The effect of global longitudinal strain on impaired six-minute walk test performance in patients with sarcoidosis.

BACKGROUND: Sarcoidosis is a multisystem and granulomatous disease associated with impaired functional capacity as a result of pulmonary and cardiac involvement. Factors adversely effecting functional capacity in patients with sarcoidosis have not been systematically assessed including myocardial strain imaging on echocardiography which enable to diagnose subclinical cardiac dysfunction. We aimed to evaluate the effect of left and right ventricular global longitudinal strain (GLS) on submaximal exercise capacity in patients with sarcoidosis who do not have clinically manifest cardiac involvement. METHODS: Extracardiac biopsy proven 56 patients with sarcoidosis and 26 controls were included consecutively. Submaximal exercise capacity of the subjects was assessed with six-minute walk test (6 MWT). Pulmonary function tests and standard transthoracic and two-dimensional speckle tracking echocardiography were performed to the all subjects. Linear regression analysis was performed to find independent predictors of 6 MWT. RESULTS: Fifty-six patients (18% male) with a mean age of 52.5 ± 10.7 years were included. Patients with sarcoidosis had low 6 MWT performance and higher New York Heart Association classes and NT-proBNP levels. There were no significant differences between controls and patients with sarcoidosis in parameters of pulmonary function test. Biventricular GLS levels and biatrial reservoir and conduit function values were lower and systolic pulmonary artery pressure (SPAP) was significantly higher in patients with sarcoidosis as compared with controls. Older age and higher SPAP were found as independent predictors of poor 6 MWT performance. CONCLUSION: Although biventricular GLS levels were lower in the patients with sarcoidosis, only age and SPAP elevations were independent predictors of the submaximal exercise capacity. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1): 63-73).