Thrombosis of native rheumatic mitral valve: A case report.

Spontaneous native mitral valve leaflet thrombosis is an exceedingly rare phenomenon. Here, we describe the case of a 71-year-old woman with rheumatic mitral stenosis who presented with cardiogenic shock. She was found to have a thrombus on her native mitral valve despite being on anticoagulation and without a clear associated hypercoagulable comorbidity. The patient underwent mitral valve replacement with favorable outcomes. This case sheds light on the inflammatory and prothrombotic nature of rheumatic valvular disease.

HeartMate II pump exchange with HeartMate III implantation to the descending aorta.

Removal of the HeartMate II left ventricular assist device (LVAD) usually requires a sternotomy. We report a case of HeartMate III LVAD implantation to the descending aorta via a left thoracotomy while leaving most of the HeartMate II device in place to avoid redo-sternotomy.

Left Ventricular Assist Devices or Inotropes for Decreasing Pulmonary Vascular Resistance in Patients with Pulmonary Hypertension Listed for Heart Transplantation.

BACKGROUND: Fixed pulmonary hypertension is common in patients with advanced heart failure and is a contraindication for heart transplantation. Left ventricular assist devices (LVAD) and inotropes have been used to reduce pulmonary vascular resistance (PVR) and allow transplantation. However, little is known about the efficacy of this strategy. METHODS: We queried the United Network for Organ Sharing registry for all adult patients (age ≥18 years) listed for primary heart transplantation (2008-2014) with PVR of >5 wood units (WU) or transpulmonary gradient >16 mmHg who were treated with LVAD or IV inotropes as status 1a, 1b, or 7. We compared waitlist mortality/delisting and absolute changes in hemodynamics between listing and transplantation. RESULTS: Of 18,009 patients listed during the study period, 1016 were included in the analysis (393 LVAD, 623 inotropes), with a mean age of 52.9 ± 11.6 years, 74% male, and 38% had ischemic etiology. Mean PVR was 5.7 ± 2.4 WU and transpulmonary pressure gradient 19.3 ± 5.3 mmHg. Compared with the inotrope group, LVAD patients were more likely listed as status 1A (32.8% vs 18.1%, P < .001), had lower PVR (5.3 WU vs 5.9 WU, P = .001), and higher cardiac output (4.1 vs 3.6 L/min, P < .001). After a mean of 239 days, PVR decreased by 1.71 WU in the LVAD group vs 1.85 WU in the inotrope group (P = .52). PVR normalization (<2.5 WU) occurred at similar rates among those treated with inotropes and LVAD (30.7% vs 35.6%, P = .228). Waitlist mortality was similar between LVAD and inotropes (adjusted P = .837). Absolute PVR and transpulmonary pressure gradient reductions correlated with time on the waitlist (P < .001 for both comparisons). CONCLUSION: Only about one-third of patients with fixed pulmonary hypertension achieve normalization of PVR before transplant with either LVAD or inotropes. Similar waitlist mortality was observed among patients bridged with either strategy.

Non-invasive Imaging in the Evaluation of Cardiac Allograft Vasculopathy in Heart Transplantation: A Systematic Review.

Cardiac allograft vasculopathy (CAV) is the leading cause of long-term graft dysfunction in patients with heart transplantation and is linked with significant morbidity and mortality. Currently, the gold standard for diagnosing CAV is coronary imaging with intravascular ultrasound during traditional invasive coronary angiography. Invasive imaging, however, carries increased procedural risk and expense to patients in addition to requiring an experienced interventionalist. With the improvements in non-invasive cardiac imaging modalities such as transthoracic echocardiography, computed tomography, magnetic resonance imaging and positron emission tomography, an alternative non-invasive imaging approach for the early detection of CAV may be feasible. In this systematic review, we explored the literature to investigate the utility of non-invasive imaging in diagnosis of CAV in >3000 patients across 49 studies. We also discuss the strengths and weaknesses for each imaging modality. Overall, all 4 imaging modalities show good to excellent accuracy for identifying CAV with significant variations across studies. Majority of the studies compared non-invasive imaging with invasive coronary angiography without intravascular imaging. In summary, non-invasive imaging modalities offer an alternative approach to invasive coronary imaging for CAV. Future studies should investigate longitudinal non-invasive protocols in low-risk patients after heart transplantation.

MELD score is predictive of 90-day mortality after veno-arterial extracorporeal membrane oxygenation support.

BACKGROUND: The Model for End-Stage Liver Disease (MELD) score was originally described as a marker of survival in chronic liver disease. More recently, MELD and its derivatives, MELD excluding INR (MELD-XI) and MELD with sodium (MELD-Na), have been applied more broadly as outcome predictors in heart transplant, left ventricular assist device placement, heart failure, and cardiogenic shock, with additional promising data to support the use of these scores for prediction of survival in those undergoing veno-arterial extracorporeal membrane oxygenation (VA ECMO). METHODS: This study assessed the prognostic impact of MELD in patients with cardiogenic shock undergoing VA ECMO via a single-center retrospective review from January 2014 to March 2020. MELD, MELD-XI, and MELD-Na scores were calculated using laboratory values collected within 48 h of VA ECMO initiation. Multivariate Cox regression analyses determined the association between MELD scores and the primary outcome of 90-day mortality. Receiver operating characteristics (ROC) were used to estimate the discriminatory power for MELD in comparison with previously validated SAVE score. RESULTS: Of the 194 patients, median MELD was 20.1 (13.7-26.2), and 90-day mortality was 62.1%. There was a significant association between MELD score and mortality up to 90 days (hazard ratio (HR) = 1.945, 95% confidence interval (95% CI) = 1.244-3.041, p = 0.004) after adjustment for age, indication for VA ECMO, and sex. The prognostic significance of MELD score for 90-day mortality revealed an AUC of 0.645 (95% CI = 0.565-0.725, p < 0.001). MELD-Na score and MELD-XI score were not associated with mortality. CONCLUSION: MELD score accurately predicts long-term mortality and may be utilized as a valuable decision-making tool in patients undergoing VA ECMO.

Causes and predictors of 30-day readmissions in patients with cardiogenic shock requiring extracorporeal membrane oxygenation support.

BACKGROUND: Cardiogenic shock is associated with significant mortality, morbidity, and healthcare cost. Utilization of extracorporeal membrane oxygenation in cardiogenic shock has increased in the United States. We sought to identify the rates and predictors of hospital readmissions in patients with cardiogenic shock after weaning from extracorporeal membrane oxygenation. METHODS: Using the 2016 Nationwide Readmission Database, we identified all patients (⩾18 years) with cardiogenic shock (ICD-10 CM R57.0) that have been implanted with extracorporeal membrane oxygenation (ICD-10-PSC of 5A15223) and were discharged alive (January-November 2016). We explored the rates, causes, and predictors of all-cause readmissions within 30 days. RESULTS: Out of 69,040 admissions with cardiogenic shock, 1641 (2.4%) underwent extracorporeal membrane oxygenation (581 were implanted during or after cardiac surgery). A total of 734 (44.7%) patients of all extracorporeal membrane oxygenations survived to discharge, and 661 were available for analysis. Out of those, 158 (23.9%) were readmitted within 30 days of discharge. More than 50% of these readmissions happened within the first 11 days. Out of 158 patients who were readmitted, 12 (7.4%) died during the readmission hospitalization. Leading causes of readmission were cardiovascular (31.6%) (heart failure: 24.1%, arrhythmia: 20.6%, neurovascular: 10.3%, hypertension: 10.3%, and endocarditis: 6.8%), followed by complications of medical/device care (17.7%), infection (11.3%), and gastrointestinal/liver (10.1%) complications. Factors associated with readmissions include the following: discharge to skilled nursing facility or with home healthcare (odds ratio: 2.10; 95% confidence interval: 1.18-3.74), durable ventricular assisted device implantation, asthma, and chronic liver disease. CONCLUSION: Patients with cardiogenic shock who underwent extracorporeal membrane oxygenation had a readmission rate. Identifying patients at high risk of readmissions might help improve outcomes.

Life-threatening presentations of propionic acidemia due to the Amish PCCB founder variant.

Although individuals of Amish descent with propionic acidemia (PA) are generally thought to have a milder disease phenotype, we now have a better understanding of the natural history of PA in this population. Here we describe two Amish patients with emergent presentations of PA, one with metabolic decompensation and another with cardiogenic shock. PA can present with life-threatening metabolic decompensation or an adult-onset severe cardiomyopathy. We discuss critical clinical implications of this observation.

Gender differences in the onset of diabetic neuropathy.

OBJECTIVE: Diabetic neuropathy is one of the more common complications plaguing individuals with type 2 diabetes. The development and progression of such complications are responsible for much of the morbidity and mortality related to this disease. Few studies have evaluated age at onset of diabetic neuropathy between genders. A difference in the progression of diabetic neuropathy between men and women may exist. This investigation evaluated gender differences in the age at onset of neuropathy among patients with type 2 diabetes. METHODS: The study, a retrospective chart analysis, reviewed 376 inpatient and outpatient medical records between January 2004 and January 2006 from a Cleveland, Ohio, hospital. Onset of neuropathy was determined by the date the neuropathy International Classification of Diseases, Ninth Revision code was first included in the medical chart; for this study, onset was equated with the date of first identification. Data were analyzed via a tailed independent t test. RESULTS: Of the 376 inpatient and outpatient charts reviewed, 156 were for male patients and 220 were for female patients (41% and 59%, respectively). All patients had type 2 diabetes; however, 23% (n=86) required insulin therapy at the time of the study. Males developed neuropathic complications at 63 years, approximately 4 years earlier than did females (at 67 years). The t test revealed a statistically significant difference in age at onset of diabetic neuropathy between the male and female subjects. CONCLUSIONS: This study demonstrates that the males in the study population developed neuropathy earlier than did the females. It may then be hypothesized that earlier interventions in the male population may improve disease outcomes.

Incidence and risk of heart failure in systemic lupus erythematosus.

BACKGROUND: Although case series suggest a higher burden of cardiovascular risk factors in patients with systemic lupus erythematosus (SLE) compared with the general population, the association between SLE and heart failure (HF) remains undefined. We sought to investigate the incidence and risk of HF in patients with SLE. METHODS: In April 2016, we performed a retrospective cohort analysis using the Explorys platform, which provides aggregated electronic medical record data from 26 major integrated healthcare systems across the USA from 1999 to present. Demographic and regression analyses were performed to assess the impact of SLE on HF incidence. RESULTS: Among 45 284 540 individuals in the database, we identified 95 400 (0.21%) with SLE and 98 900 (0.22%) with a new diagnosis of HF between May 2015 and April 2016. HF incidence was markedly higher in the SLE group compared with controls (0.97% vs 0.22%, relative risk (RR): 4.6 (95% CI 4.3 to 4.9)), as were other cardiovascular risk factors. In regression analysis, SLE was an independent predictor of HF (adjusted OR: 3.17 (2.63 to 3.83), p<0.0001). RR of HF was highest in young males with SLE (65.2 (35.3 to 120.5) for age 20-24), with an overall trend of increasing absolute risk but decreasing RR with advancing age in both sexes. Renal involvement in SLE correlated with earlier and higher incidence of HF. CONCLUSIONS: The findings of this study suggest that patients with SLE have significantly higher risk of developing HF and a worse cardiovascular risk profile compared with the general population. These results need to be confirmed by prospective studies.

Clinical epidemiology of heart failure with preserved ejection fraction (HFpEF) in comparatively young hospitalized patients.

BACKGROUND: While heart failure with preserved ejection fraction (HFpEF) is primarily a disease of old age, risk factors that contribute to HFpEF are not limited to older patients. The objectives of this population-based observational study were to describe the clinical epidemiology of HFpEF in younger (<65 years) as compared with older (≥65 years) patients hospitalized with acute decompensated heart failure. METHODS AND RESULTS: We reviewed the medical records of residents of central Massachusetts hospitalized with HFpEF at all 11 greater Worcester (MA) medical centers during the 5 study years of 1995, 2000, 2002, 2004, and 2006. Among the 2398 patients hospitalized with confirmed HFpEF, 357 (14.9%) were <65 years old. Younger patients were more likely to be male, non-Caucasian, obese, and to have a history of diabetes and chronic kidney disease than older patients with HFpEF. Younger patients hospitalized with HFpEF were less likely to have received commonly prescribed cardiac medications, had a longer hospital stay, and experienced significantly lower post-discharge death rates than older hospitalized patients. CONCLUSION: While HFpEF is predominantly a disease of old age, data from longitudinal studies remain needed to identify risk factors in younger individuals that may predispose them to the development of HFpEF.

Heart failure in patients with human immunodeficiency virus infection: Epidemiology and management disparities.

BACKGROUND: Persons living with HIV are at a higher risk of cardiovascular disease despite effective antiretroviral therapy and dramatic reductions in AIDS-related conditions. We sought to identify the epidemiology of heart failure (HF) among persons living with HIV in the United States in an era of contemporary antiretroviral therapy. METHODS: Explorys is an electronic healthcare database that aggregates medical records from 23 healthcare systems nationwide. Using systemized nomenclature of medicine-clinical terms (SNOMED-CT), we identified adult patients (age>18), who had active records over the past year (September 2014-September 2015). We described the prevalence of HF in HIV patients by demographics and treatment and compared them to HIV-uninfected controls. RESULTS: Overall, there were 36,400 patients with HIV and 12,208,430 controls. The overall prevalence of HF was 7.2% in HIV and 4.4% in controls (RR 1.66 [1.60-1.72], p<0.0001). The relative risk of HF associated with HIV infection was higher among women and younger age groups. Patients receiving antiretroviral therapy had only marginally lower risk (6.4% vs. 7.7%, p<0.0001) of HF compared to those who were untreated. Compared to uninfected patients with HF, HIV patients with HF were less likely to receive antiplatelet drugs, statins, diuretics, and ACE/ARBs (p<0.0001 for all comparisons). For patients with HIV and HF, receiving care from a cardiologist was associated with higher use of antiplatelets, statins, betablockers, ACE/ARBs, and diuretics. CONCLUSIONS: Persons with HIV are at higher risk for HF in this large contemporary sample that includes both men and women. Although the prevalence of heart failure is higher in older HIV patients, the relative risk associated with HIV is highest in young people and in women. HIV patients are less likely to have HF optimally treated, but cardiology referral was associated with higher treatment rates.

Effect of Hepatitis C Positivity on Survival in Adult Patients Undergoing Heart Transplantation (from the United Network for Organ Sharing Database).

Concerns exist regarding orthotropic heart transplantation in hepatitis C virus (HCV) seropositive recipients. Thus, a national registry was accessed to evaluate early and late outcome in HCV seropositive recipients undergoing heart transplant. Retrospective analysis of the United Network for Organ Sharing registry (1991 to 2014) was performed to evaluate recipient profile and clinical outcome of patients with HCV seropositive (HCV +ve) and seronegative (HCV -ve). Adjusted results of early mortality and late survival were compared between cohorts. From 23,507 patients (mean age 52 years; 75% men), 481 (2%) were HCV +ve (mean age 52 years; 77% men). Annual proportion of HCV +ve recipients was comparable over the study period (range 1.3% to 2.7%; p = 0.2). The HCV +ve cohort had more African-American (22% vs 17%; p = 0.01), previous left ventricular assist device utilization (21% vs 14%; p <0.01) and more hepatitis B core Ag+ve recipients (17% vs 5%; p <0.01). However, both cohorts were comparable in terms of extracorporeal membrane oxygenator usage (p = 0.7), inotropic support (p = 0.2), intraaortic balloon pump (p = 0.7) support, serum creatinine (p = 0.7), and serum bilirubin (p = 0.7). Proportion of status 1A patients was similar (24% HCV + vs 21% HCV -); however, wait time for HCV +ve recipients were longer (mean 23 vs 19 days; p <0.01). Among donor variables, age (p = 0.8), hepatitis B status (p = 0.4), and Center for Diseases Control high-risk status (p = 0.9) were comparable in both cohorts. At a median follow-up of 4 years, 67% patients were alive, 28% died, and 1.1% were retransplanted (3.4% missing). Overall survival was worse in the HCV+ cohort (64.3% vs 72.9% and 43.2% vs 55% at 5 and 10 years; p <0.01), respectively. Late renal (odds ratio [OR] 1.2 [1 to 1.6]; p = 0.02) and liver dysfunction (odds ratio 4.5 [1.2 to 15.7]; p = 0.01) occurs more frequently in HCV +ve recipients. On adjusted analysis, HCV seropositivity is associated with poorer survival (hazard ratio for mortality 1.4 [1.1 to 1.6]; p <0.001). In conclusion, a small proportion of patients receiving a heart transplant in the United States have hepatitis C. Despite comparable preoperative hepatic function, hepatitis C seropositive recipients demonstrate poorer long-term survival.

A late presentation of an anomalous left coronary artery originating from the pulmonary artery (ALCAPA): A case study and review of the literature.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital condition which can manifest as various cardiac symptoms. CASE REPORT: A 66-year-old woman who presented for pre-operative surgical risk assessment for hip surgery underwent a nuclear stress test which revealed a large reversible anterior defect. At coronary angiography she was found to have ALCAPA. CONCLUSIONS: This is a rare case of ALCAPA due to the patient's age. Survival to adulthood is possible and patients may remain relatively asymptomatic for years..

Weight and mortality following heart failure hospitalization among diabetic patients.

BACKGROUND: Type 2 diabetes is an important risk factor for heart failure and is common among patients with heart failure. The impact of weight on prognosis after hospitalization for acute heart failure among patients with diabetes is unknown. The objective of this study was to examine all-cause mortality in relation to weight status among patients with type 2 diabetes hospitalized for decompensated heart failure. METHODS: The Worcester Heart Failure Study included adults admitted with acute heart failure to all metropolitan Worcester medical centers in 1995 and 2000. The weight status of 1644 patients with diabetes (history of type 2 diabetes in medical record or admission serum glucose ≥200 mg/dL) was categorized using body mass index calculated from height and weight at admission. Survival status was ascertained at 1 and 5 years after hospital admission. RESULTS: Sixty-five percent of patients were overweight or obese and 3% were underweight. Underweight patients had 50% higher odds of all-cause mortality within 5 years of hospitalization for acute heart failure than normal weight patients. Class I and II obesity were associated with 20% and 40% lower odds of dying. Overweight and Class III obesity were not associated with mortality. Results were similar for mortality within 1 year of hospitalization for acute heart failure. CONCLUSIONS: The mechanisms underlying the association between weight status and mortality are not fully understood. Additional research is needed to explore the effects of body composition, recent weight changes, and prognosis after hospitalization for heart failure among patients with diabetes.