Increased Pulmonary-Systemic Pulse Pressure Ratio Is Associated With Increased Mortality in Group 1 Pulmonary Hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is characterised by remodelling of the pulmonary vasculature leading to right ventricular (RV) failure. The failing RV, through interventricular uncoupling, deleteriously impacts the left ventricle and overall cardiac efficiency. We hypothesised that the ratio of the pulmonary artery pulse pressure to the systemic pulse pressure ("pulmonary-systemic pulse pressure ratio", or PS-PPR) would be associated with mortality in PAH. METHODS: We conducted a retrospective analysis of 262 patients in the National Institute of Health Primary Pulmonary Hypertension Registry (NIH-PPH). We evaluated the association between the PS-PPR and mortality after adjustment for the Pulmonary Hypertension Connection (PHC) risk equation. RESULTS: Among 262 patients (mean age 37.5±15.8years, 62.2% female), median PS-PPR was 1.04 (IQR 0.79-1.30). In the Cox proportional hazards regression model, each one unit increase in the PS-PPR was associated with more than a two-fold increase in mortality during follow-up (HR 2.06, 95% CI 1.40-3.02, p=0.0002), and this association of PS-PPR with mortality remained significant in the multivariable Cox model adjusted for the PHC risk equation, mean pulmonary artery pressure, and body mass index (BMI) (adjusted HR 1.81, 95% CI 1.13-2.88, p=0.01). Furthermore, PS-PPR in the upper quartile (>1.30) versus quartiles 1-3 was associated with a 68% increase in mortality after adjustment for these same covariates (adjusted HR 1.68, 95% CI 1.13-2.50, p=0.01). CONCLUSIONS: Pulmonary-systemic pulse pressure ratio, a marker of biventricular efficiency, is associated with survival in PAH even after adjustment for the PHC risk equation. Further studies are needed on the wider applications of PS-PPR in PAH patients.

Right atrial to left atrial volume index ratio is associated with increased mortality in patients with pulmonary hypertension.

BACKGROUND: Pulmonary hypertension (PH) is characterized by increased pulmonary vascular resistance leading to right heart failure. Elevated right atrial (RA) pressure reflects right ventricular (RV) pressure overload and is an established risk factor for mortality in PH. We hypothesized that PH patients with an increased ratio of RA to LA volume index (RAVI/LAVI), would have increased mortality. METHODS: We evaluated the association of RAVI/LAVI with mortality in 124 patients seen at a single academic center's PH clinic after adjusting for the REVEAL risk score, an established risk score in PH. LA and RA volume indices were measured in the four-and two-chamber views by two independent researchers. Multivariable logistic regression was used to model the independent association of RAVI/LAVI with survival. RESULTS: Among 124 patients (mean age 62 ± 12.7 years, 68.6% female), each unit increase in RAVI/LAVI was associated with a nearly twofold increase in mortality (OR: 1.91, 95% CI: 1.20-3.04). In a multivariable logistic regression, each unit increase in RAVI/LAVI was associated with a nearly twofold increase in mortality (OR: 1.73, 95% CI: 1.003-2.998). Furthermore, RAVI/LAVI in the highest quartile (>1.42) was significantly associated with elevated right atrial pressure (RAP) to pulmonary artery wedge pressure ratio (RAP/PAWP) (0.76 ± 0.41, P = 0.02) compared with the lowest quartile (<0.77), suggesting an interaction between invasive hemodynamic data, atrial structural changes, and mortality in PH. CONCLUSIONS: Increased RAVI/LAVI in PH is associated with decreased survival and accounts for atrial structural remodeling related to invasive hemodynamics. These findings support further study of this index in predicting outcomes in PH.

Off-label Use of Direct Oral Anticoagulants Compared With Warfarin for Left Ventricular Thrombi.

Importance: Left ventricular (LV) thrombi can arise in patients with ischemic and nonischemic cardiomyopathies. Anticoagulation is thought to reduce the risk of stroke or systemic embolism (SSE), but there are no high-quality data on the effectiveness of direct oral anticoagulants (DOACs) for this indication. Objective: To compare the outcomes associated with DOAC use and warfarin use for the treatment of LV thrombi. Design, Setting, and Participants: A cohort study was performed at 3 tertiary care academic medical centers among 514 eligible patients with echocardiographically diagnosed LV thrombi between October 1, 2013, and March 31, 2019. Follow-up was performed through the end of the study period. Exposures: Type and duration of anticoagulant use. Main Outcomes and Measures: Clinically apparent SSE. Results: A total of 514 patients (379 men; mean [SD] age, 58.4 [14.8] years) with LV thrombi were identified, including 300 who received warfarin and 185 who received a DOAC (64 patients switched treatment between these groups). The median follow-up across the patient cohort was 351 days (interquartile range, 51-866 days). On unadjusted analysis, DOAC treatment vs warfarin use (hazard ratio [HR], 2.71; 95% CI, 1.31-5.57; P = .01) and prior SSE (HR, 2.13; 95% CI, 1.22-3.72; P = .01) were associated with SSE. On multivariable analysis, anticoagulation with DOAC vs warfarin (HR, 2.64; 95% CI, 1.28-5.43; P = .01) and prior SSE (HR, 2.07; 95% CI, 1.17-3.66; P = .01) remained significantly associated with SSE. Conclusions and Relevance: In this multicenter cohort study of anticoagulation strategies for LV thrombi, DOAC treatment was associated with a higher risk of SSE compared with warfarin use, even after adjustment for other factors. These results challenge the assumption of DOAC equivalence with warfarin for LV thrombi and highlight the need for prospective randomized clinical trials to determine the most effective treatment strategies for LV thrombi.

Isolated Marked Inferior Vena Cava Dilatation: Unusual Presentation or Underrecognized Common Phenomenon?

Inferior vena cava (IVC) diameter and respirophasic variation are commonly used echocardiographic indices to estimate right atrial pressure. While dilatation of the IVC and reduced collapsibility have traditionally been associated with elevated right heart filling pressures, the significance of isolated IVC dilatation in the absence of raised filling pressures remains poorly understood. We present a case of an asymptomatic 28-year-old male incidentally found to have IVC dilatation, reduced inspiratory collapse, and normal right heart pressures.

Decreased pulmonary arterial proportional pulse pressure is associated with increased mortality in group 1 pulmonary hypertension.

BACKGROUND: This study evaluated the utility of a novel index, pulmonary arterial (PA) proportional pulse pressure (PAPP; range 0-1, defined as [PA systolic pressure - PA diastolic pressure] / PA systolic pressure), in predicting mortality in patients with World Health Organization group 1 pulmonary hypertension (PH). HYPOTHESIS: Low PAPP is associated with increased 5-year mortality independent of a validated contemporary risk-prediction equation (Pulmonary Hypertension Connection [PHC] equation). METHODS: In a group of 262 patients in the National Institutes of Health Primary Pulmonary Hypertension (NIH-PPH) Registry, PAPP and the PHC risk equation were used to predict mortality during 5 years of follow-up using Cox proportional hazards models. Kaplan-Meier survival curves were used to compare mortality among PAPP quartiles, and significance was tested using the log-rank test. RESULTS: Patients in the lowest quartile (PAPP ≤0.47) had a significantly higher 5-year mortality than did patients in higher quartiles (log-rank P = 0.016). In a Cox model adjusted for the PHC equation, PAPP remained significantly associated with 5-year mortality (hazard ratio: 0.74 per 0.10 increase in PAPP, 95% confidence interval: 0.61-0.90). The χ2 statistic for the single PAPP covariate in this model was 8.8 (P = 0.003), which compared favorably with the χ2 statistic of 15.2 (P < 0.0001) for the multivariable PHC equation. CONCLUSIONS: PAPP, an index of ventricular-arterial coupling, is independently associated with survival in World Health Organization group 1 PH. The use of this easily measurable index for guiding risk stratification needs further investigation.