Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

BACKGROUND: Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. METHODS: We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. RESULTS: The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort (P =.0001). Patients achieving 2 or 3 noninva-sive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P =.02; hazard ratio: 4.258, CI: 1.143-15.860, P =.031; hazard ratio: 0.095, CI: 0.013-0.672, P =.018, respectively). CONCLUSIONS: Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies.

Assessment of atrial electromechanical delay and P wave dispersion in patients with chronic obstructive pulmonary disease.

OBJECTIVE: Chronic obstructive pulmonary disease (COPD) is associated with atrial fibrillation (AF) and reduced forced expiratory volume (FEV1) is an independent predictor for new onset AF. The aims of this study were (1) to analyze the atrial electromechanical delay (AEMD) and P wave dispersion which are two predictors of AF development in patients with COPD and without any cardiovascular disease, and (2) to assess the relationship of those with pulmonary functions as quantified by FEV1 measurements. METHODS: The study included 41 patients with COPD (33 male; mean age: 51 years) and 32 healthy controls. P wave dispersion was calculated as the difference between the maximum and minimum P wave duration in a 12-lead surface electrocardiography (ECG) recording. AEMD, defined as the time interval from the P wave onset on the ECG to the initiation of the late diastolic (Am) wave using a tissue Doppler examination, was measured from the lateral mitral annulus (LAEMD), septal annulus (SAEMD), and tricuspid lateral annulus (TAEMD). RESULTS: P wave dispersion was significantly longer in the COPD group than those in the controls (76±19 ms vs. 45±10 ms; p<0.001). All of the AEMD measurements demonstrated significant prolongation in patients with COPD (LAEMD: 74±9 ms vs. 64±11 ms; SAEMD: 66±10 ms vs. 57±12 ms; and TAEMD: 65±9 ms vs. 46±7 ms; p<0.001 for all). The only correlation with FEV1 was observed in the TAEMD values of the COPD group (rs: -401; p<0.009). CONCLUSION: Both P wave dispersion and AEMD parameters were significantly longer in COPD patients without any established structural or functional cardiac abnormalities, indicating an increased tendency for AF development, beginning from the initial stages of the disease.

Risk assessment and survival of patients with pulmonary hypertension: Multicenter experience in Turkey.

OBJECTIVE: Risk stratification continues to evolve in pulmonary arterial hypertension (PAH). Our aim was to further confirm the risk assessment strategy in our cohort and to determine the most reliable model. METHODS: We enrolled incident patients with idiopathic PAH (IPAH), heritable, drug-induced, congenital heart disease (CHD), connective tissue diseases (CTD) subsets, and chronic thromboembolic pulmonary hypertension (CTEPH) from January 2008 to February 2018. Data from the baseline and subsequent follow-ups within 1 year of diagnosis were included. An abbreviated risk assessment strategy was applied using the following variables: functional class (FC), 6-minute walk distance (6 MWD), N-terminal pro-brain natriuretic peptide (NT-proBNP) or BNP, right atrial (RA) area, pericardial effusion, the mean RA pressure, cardiac index, and mixed venous oxygen saturation. Three different methods were applied to categorize patients. RESULTS: A total of 189 subjects (46+-17 years, 23% male) were included. Sixty-one patients had died. The survival differed significantly between the risk groups both at diagnosis and during the follow-up. Patients with a low-risk profile had a better survival rate. An abbreviated risk assessment tool predicted mortality at early follow-up in the entire group and CHD, CTD subsets, and CTEPH, separately. An overall mortality among risk categories was significantly different according to each categorization method. The most reliable model comprised FC, 6 MWD, NT pro-BNP/BNP, and the RA area at the follow-up. CONCLUSION: The abbreviated risk assessment tool may be valid for the PAH subsets and CTEPH. Echocardiographic variables do matter. A model comprising FC, 6 MWD, NT pro-BNP/BNP, and the RA area at the follow-up could be useful for better prognostication.