Jeremy A. Mazurek

Enhancing the accuracy of echocardiography in the diagnosis of pulmonary arterial hypertension: looking at the heart to learn about the lungs.

Purpose of review Although routine use of Doppler echocardiography has led to an increased recognition of pulmonary hypertension, the role of Doppler echocardiography has largely remained as a screening tool with the primary emphasis on the presence or absence of an increased Doppler-estimated pulmonary artery systolic pressure (PASP). However, the utility of Doppler echocardiography in the workup of pulmonary hypertension extends far beyond that of a screening tool, with the integration of relevant Doppler echocardiography parameters providing a wealth of hemodynamic insight into not only if a patient has pulmonary hypertension, but why they have pulmonary hypertension. This review summarizes some of the recent advances in the use of Doppler echocardiography in evaluating the pathophysiology of pulmonary hypertension. Recent findings Distinguishing pulmonary hypertension related to pulmonary vascular disease (i.e. pulmonary arterial hypertension, pulmonary hypertension due to lung disease, or chronic thromboembolic pulmonary hypertension; PHPVD), from those with left heart disease associated pulmonary hypertension (pulmonary venous hypertension; PVH) is crucial as workup and treatment options differ dramatically. Recent studies have identified easily obtainable Doppler echocardiography parameters that can reliably distinguish between PHPVD and PVH, allowing for rapid triage of patients with evidence of PHPVD to invasive right heart catheterization whiles avoiding invasive investigation and the inappropriate use of pulmonary hypertension specific therapy in patients with PVH. Summary This review highlights the importance of integrating two-dimensional and Doppler parameters in order to inform the clinician as to the hemodynamic cause of pulmonary hypertension, thus enhancing the diagnostic accuracy of Doppler echocardiography, rapidly identifying those with PHPVD and right heart dysfunction and assisting in triage of patients to further invasive hemodynamic assessment.

Understanding Heart Failure

Heart failure (HF) is a growing global health concern that affects more than 20 million people worldwide. With an ever-growing segment of the population over the age of 65, the prevalence of HF and its associated costs are expected to increase exponentially over the next decade. Advances in the understanding of the pathophysiology and treatment of HF have resulted in the ability to enhance both the quantity and the quality of life of patients with HF. This article reviews the current understanding of the pathophysiology, cause, classification, and treatment of HF and describes areas of uncertainty that demand future study.

Mortality and readmission rates in patients hospitalized for acute decompensated heart failure: a comparison between cardiology and general-medicine service outcomes in an underserved population.

With recent legislation imposing penalties on hospitals for above‐average 30‐day all‐cause readmissions for patients with acute decompensated heart failure (ADHF), there is concern these penalties will more heavily impact hospitals serving socioeconomically vulnerable and underserved populations.

Right ventricular response to pulsatile load is associated with early right heart failure and mortality after left ventricular assist device

BACKGROUND Right ventricular (RV) adaptation to afterload is crucial for patients undergoing continuous-flow left ventricular assist device (cf-LVAD) implantation. We hypothesized that stratifying patients by RV pulsatile load, using pulmonary arterial compliance (PAC), and RV response to load, using the ratio of central venous to pulmonary capillary wedge pressure (CVP:PCWP), would identify patients at high risk for early right heart failure (RHF) and 6-month mortality after cf-LVAD. METHODS During the period from January 2008 to June 2014, we identified 151 patients at our center with complete hemodynamics prior to cf-LVAD. Pulsatile load was estimated using PAC indexed to body surface area (BSA), according to the formula: indexed PAC (PACi) = [SV / (PAsystolic - PAdiastolic)] / BSA, where SV is stroke volume and PA is pulmonary artery. Patients were divided into 4 hemodynamic groups by PACi and CVP:PCWP. RHF was defined as the need for unplanned RVAD, inotropic support ≥14 days or death due to RHF within 14 days. Risk factors for RHF and 6-month mortality were examined using logistic regression and Cox proportional hazards modeling. RESULTS Sixty-one patients (40.4%) developed RHF and 34 patients (22.5%) died within 6 months. Patients with RHF had lower PACi (0.92 vs 1.17 ml/mm Hg/m2, p = 0.008) and higher CVP:PCWP (0.48 vs 0.37, p = 0.001). Higher PACi was associated with reduced risk of RHF (adjusted odds ratio [adj-OR] 0.61, 95% confidence interval [CI] 0.39 to 0.94, p = 0.025) and low PACi with increased risk of 6-month mortality (adjusted hazard ratio [adj-HR] 3.18, 95% CI 1.40 to 7.25, p = 0.006). Compared to patients with low load (high PACi) and adequate right heart response to load (low CVP:PCWP), patients with low PACi and high CVP:PCWP had an increased risk of RHF (OR 4.74, 95% CI 1.23 to 18.24, p = 0.02) and 6-month mortality (HR 8.68, 95% CI 2.79 to 26.99, p < 0.001). CONCLUSIONS A hemodynamic profile combining RV pulsatile load and response to load identifies patients at high risk for RHF and 6-month mortality after cf-LVAD.

Successful cardiac transplantation outcomes in patients with adult congenital heart disease

Objectives The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). Background Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT. Methods The Penn Congenital Transplant Database comprises a cohort of patients with ACHD who underwent OHT between March 2010 and April 2016. We performed a retrospective cohort study of the 20 consecutive patients. Original cardiac diagnoses include single ventricle palliated with Fontan (n=8), dextro-transposition of the great arteries after atrial switch (n=4), tetralogy of Fallot (n=4), pulmonary atresia (n=1), Ebstein anomaly (n=1), unrepaired ventricular septal defect (n=1) and Noonan syndrome with coarctation of the aorta (n=1). Results Eight patients required pretransplant inotropes and two required pretransplant mechanical support. Nine patients underwent heart–liver transplant and three underwent heart–lung transplant. Three patients required postoperative mechanical circulatory support. Patients were followed for an average of 38 months as of April 2016, with 100% survival at 30 days and 1 year and 94% overall survival (19/20 patients). Conclusions ACHD–OHT patients require highly specialised, complex and multidisciplinary healthcare. The success of our programme is attributed to using team-based, patient-centred care including our multidisciplinary staff and specialists across programmes and departments.

Follow-up tricuspid annular plane systolic excursion predicts survival in pulmonary arterial hypertension:

Few studies have examined the utility of serial echocardiography in the evaluation, management, and prognosis of patients with pulmonary arterial hypertension (PAH). Therefore, we sought to evaluate the prognostic significance of follow-up tricuspid annular plane systolic excursion (TAPSE) in PAH. We prospectively studied 70 consecutive patients with PAH who underwent baseline right heart catheterization (RHC) and transthoracic echocardiogram, who survived to follow-up echocardiogram after initiation of PAH therapy. Baseline TAPSE was 1.6 ± 0.5 cm which increased to 2.0 ± 0.4 cm on follow-up (P < 0.0001). The cohort was dichotomized by TAPSE at one-year follow-up: Group 1 (n = 37): follow-up TAPSE ≥ 2 cm; Group 2 (n = 33): follow-up TAPSE < 2 cm. Group 1 participants were significantly more likely to reach WHO functional class I–II status and achieve a higher six-minute walk distance on follow-up. Of the 68 patients who survived more than one year, 18 died (26.5%) over a median follow-up of 941 days (range, 3–2311 days), with significantly higher mortality in Group 2 versus Group 1 (41.9% vs. 13.5%; P = 0.003). While baseline TAPSE stratified at 2 cm did not predict survival in this cohort, TAPSE ≥ 2 cm at follow-up strongly predicted survival in bivariable models (hazard ratio, 0.21; 95% confidence interval, 0.08–0.60). In conclusion, follow-up TAPSE ≥ 2 cm is a prognostic marker and potential treatment target in a PAH population.

Understanding Heart Failure.

Heart failure (HF) is a growing global health concern that affects more than 20 million people worldwide. With an ever-growing segment of the population over the age of 65, the prevalence of HF and its associated costs are expected to increase exponentially over the next decade. Advances in the understanding of the pathophysiology and treatment of HF have resulted in the ability to enhance both the quantity and the quality of life of patients with HF. This article reviews the current understanding of the pathophysiology, cause, classification, and treatment of HF and describes areas of uncertainty that demand future study.

Relaxin levels in pulmonary hypertension: a comparison between pulmonary arterial hypertension and diastolic heart failure-induced pulmonary hypertension.

Jeremy A. Mazurek, MD, Benjamin D. Horne, PhD, MPH, Iosif Kelesidis, MD, Jason N. Salamon, MD, and Ronald Zolty, MD, PhD From the Department of Medicine, Cardiovascular Division, Hospital of the University of Pennsylvania, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania; Intermountain Heart Institute, Intermountain Medical Center, Salt Lake City, Utah; Department of Medicine, Division of Cardiology, Montefiore Medical Center; and the Department of Medicine, Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, New York

Prognostic Implications of Changes in Albumin Following Left Ventricular Assist Device Implantation in Patients With Severe Heart Failure

Mechanical assist devices have emerged as an established therapeutic option for patients with end-stage heart failure. Because preimplant hypoalbuminemia is a known risk factor for adverse outcomes, we hypothesized that change in albumin may be a prognostic indicator in patients with continuous-flow left ventricular assist devices (cfLVADs). This is a retrospective single-center study of patients who underwent cfLVAD implantation (HeartMate II and HeartWare HVAD) at an academic center from 2008 to 2014. After excluding those who died, were transplanted, or hospitalized during the first 3 months post-implant, albumin values were obtained and stratified by an increase or a decrease from pre-implant to 3 months post-implant on 171 (81% male, mean age 57 ± 16 years) patients (139 for the survival analysis and 90 for the hospitalization analysis). Decrease in albumin from pre-implant to 3 months after implant correlated with increased mortality (hazard ratio 2.93, confidence interval 1.57 to 5.44, p <0.01) and reduced time to next hospitalization (hazard ratio 1.70, confidence interval 1.03 to 2.81, p = 0.04). The Kaplan-Meier survival curve estimated a 49.43% 2-year survival rate in those whose albumin decreased versus 83.62% in those whose albumin increased over 3 months (p <0.01). Improved outcomes were seen in patients whose albumin normalized versus patients whose albumin remained normal over 3 months. In conclusion, our study is the first to demonstrate the importance of change in albumin from pre- to postimplant on the prognosis of cfLVAD patients. Future studies are needed to determine whether therapeutic intervention to improve albumin post-implant will prevent hospitalizations and improve outcomes.

Predicting Long Term Outcome in Patients Treated With Continuous Flow Left Ventricular Assist Device: The Penn—Columbia Risk Score

Background Predicting which patients are unlikely to benefit from continuous flow left ventricular assist device (LVAD) treatment is crucial for the identification of appropriate patients. Previously developed scoring systems are limited to past eras of device or restricted to specific devices. Our objective was to create a risk model for patients treated with continuous flow LVAD based on the preimplant variables. Methods and Results We performed a retrospective analysis of all patients implanted with a continuous flow LVAD between 2006 and 2014 at the University of Pennsylvania and included a total of 210 patients (male 78%; mean age, 56±15; mean follow‐up, 465±486 days). From all plausible preoperative covariates, we performed univariate Cox regression analysis for covariates affecting the odds of 1‐year survival following implantation (P<0.2). These variables were included in a multivariable model and dropped if significance rose above P=0.2. From this base model, we performed step‐wise forward and backward selection for other covariates that improved power by minimizing Akaike Information Criteria while maximizing the Harrell Concordance Index. We then used Kaplan–Meier curves, the log‐rank test, and Cox proportional hazard models to assess internal validity of the scoring system and its ability to stratify survival. A final optimized model was identified based on clinical and echocardiographic parameters preceding LVAD implantation. One‐year mortality was significantly higher in patients with higher risk scores (hazard ratio, 1.38; P=0.004). This hazard ratio represents the multiplied risk of death for every increase of 1 point in the risk score. The risk score was validated in a separate patient cohort of 260 patients at Columbia University, which confirmed the prognostic utility of this risk score (P=0.0237). Conclusion We present a novel risk score and its validation for prediction of long‐term survival in patients with current types of continuous flow LVAD support.