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.

Clinical Impact of Changes in Hemodynamic Indices of Contractile Function During Treatment of Acute Decompensated Heart Failure.

BACKGROUND: The objective of this work was to determine the impact of improving right ventricular versus left ventricular stroke work indexes (RVSWI vs LVSWI) during therapy for acute decompensated heart failure (ADHF). METHODS AND RESULTS: Cox proportional hazards regression and logistic regression were used to analyze key factors associated with outcomes in 175 patients (mean age 56.7 ± 13.6 years, 29.1% female) with hemodynamic data from the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness trial. In this cohort, 28.6% and 69.7%, respectively, experienced the outcomes of death, transplantation, or ventricular assist device implantatation (DVADTX) and DVADTX or HF rehospitalization (DVADTXHF) during 6 months of follow-up. Increasing RVSWI (ΔRVSWI) from baseline to discharge was associated with a decrease in DVADTXHF (hazard ratio [HR] 0.923, 95% confidence interval [CI] 0.871-0.979) per 0.1 mm Hg⋅L⋅m-2 increase); however, increasing LVSWI (ΔLVSWI) had only a nonsignificant association with decreased DVADTXHF (P = .11) In a multivariable model, patients with ΔRVSWI ≤1.07 mm Hg⋅L⋅m-2 and ΔLVSWI ≤4.57 mm Hg⋅L⋅m-2 had a >2-fold risk of DVADTXHF (HR 2.05, 95% CI 1.23-3.41; P = .006). CONCLUSION: Compared with left ventricular stroke work, increasing right ventricular stroke work during treatment of ADHF was associated with better outcomes. The results promise to inform optimal hemodynamic targets for ADHF.

Diastolic pulmonary gradient predicts outcomes in group 1 pulmonary hypertension (analysis of the NIH primary pulmonary hypertension registry).

BACKGROUND: Diastolic pulmonary gradient (DPG), calculated as the difference between pulmonary artery diastolic pressure and mean pulmonary capillary wedge pressure ≥ 7 mmHg is associated with pulmonary vascular disease and portends poor prognosis in heart failure (HF). The prognostic relevance of DPG in group 1 pulmonary hypertension (PH) is uncertain. METHODS: Using the Pulmonary Hypertension Connection (PHC) risk equation for 225 patients in the NIH-PPH, the 5-year probability of death was calculated, which was then compared with DPG using a Cox proportional hazards model. Kaplan-Meier survival curves were determined for two cohorts using the median DPG of 30 mmHg as cutoff, and significance was tested using the log-rank test. RESULTS: The mean age was 38.1 ± 16.0 years old, 63% female, and 72% were "white". The mean DPG was 31.6 mmHg ± 13.8 mm Hg and only 1.8% had a DPG <7 mm Hg. Increasing DPG was significantly associated with increased 5-year mortality even after adjustment for the PHC risk equation (HR 1.29 per 10 mm Hg increase). When DPG was dichotomized based on the median of 30 mm Hg, the HR for DPG >30 mm Hg with respect to 5-year mortality was 2.03. After adjustment for pulmonary artery systolic pressure (PASP), increasing DPG remained significantly associated with decreased 5 years survival (HR 1.99 for DPG > 30 mm Hg). CONCLUSIONS: DPG is independently associated with survival in group 1 PH patients even after adjustment for the PHC risk equation or PASP. Patients with increased DPG had a 2-fold increased risk of mortality. The use of DPG for guiding treatment and prognosis in group 1 PH should be further investigated.

A case of familial calcific aortic and mitral stenosis in association with hereditary sclerosing poikiloderma.

Hereditary sclerosing poikiloderma is a rare, familial disease with the primary clinical features being dermatologic. Widespread poikiloderma, as well as linear hyperkeratotic and sclerotic bands, tends to be the most common sign of this disease. It has been suggested that cardiac involvement may represent an important element of this disorder; however, this has not been well studied. We confirm here a case of hereditary sclerosing poikiloderma in a patient and his family with significant cardiac involvement characterized by heavily calcified stenotic aortic and mitral valves on echocardiography. Due to the patient's symptomatic severe valvular disease, he underwent simultaneous aortic and mitral valve replacement. Histopathologic analysis of the valves confirmed severe calcification of the aortic and mitral valve leaflets, suggesting a potential common mechanism between the cardiac and skin pathology of this disease. Multiple other family members had presented with similar cardiac and skin manifestations. Further research is needed to better understand the cardiac pathophysiology of this disease.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for 11%-22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.