Introduction Current management of heart failure in patients with sickle cell disease hinges on optimizing the underlying hematological process, primarily with the use of hydroxyurea, in addition to the use of guideline-directed medical therapy (GDMT) for heart failure. There is, however, limited data on the impact of hydroxyurea on hospitalizations due to heart failure in these patients. Hypothesis We hypothesized that the use of hydroxyurea in sickle cell patients with heart failure would reduce heart failure hospitalizations. Methods A retrospective analysis was conducted on 101 patients with sickle cell disease and heart failure between August of 2023 and January of 2024. Demographic data, comorbidities, echocardiographic findings, GDMT, and outcomes, including vaso-occlusive crisis (VOC) episodes, and hospitalizations due to heart failure were assessed. Results Among the 101 patients in the study, 55 were on hydroxyurea, while 46 were not. There were no significant differences in baseline characteristics between the two groups. When compared to patients not on hydoxyurea, patients on hydroxyurea had a trend towards fewer VOC episodes per year (2.2 vs. 3.6, p = 0.135) and heart failure hospitalizations (0.7 vs. 1.3, p = 0.179), although these differences were not statistically significant. Conclusion Our study suggests a benefit of hydroxyurea in reducing hospitalizations for sickle cell cardiomyopathy patients. Further investigation with larger sample sizes is needed to confirm these findings and provide more evidence regarding the efficacy of hydroxyurea to warrant its use in this patient population.

Cardiac sarcoidosis (CS), an inflammatory disorder characterized by granulomatous inflammation, can manifest as heart failure (HF), ventricular arrhythmias, and conduction disease (1). Despite immunosuppression and medical therapy for HF, a subset of CS patients will progress to end-stage HF requiring left ventricular assist device (LVAD) therapy and/or heart transplantation (HT) which are associated with excellent outcomes (2, 3). The optimal surveillance strategy for monitoring CS recrudescence after advanced heart failure therapies remains undefined.

Purpose: The differential impact of left ventricular (LV) size and body surface area (BSA) on survival following durable left ventricular assist device (d-LVAD) implantation has garnered increasing attention. We sought to determine the outcomes of d-LVAD recipients stratified by LV size and BSA in a single center analysis.

Introduction: Pulmonary Arterial Hypertension (PAH) therapies warrant uptitration when patients remain intermediate/high risk profile. We present 2 cases of patients on maximal oral therapies who underwent successful transition from selexipag to parenteral treprostinil, for which guidelines do not currently exist.

Introduction Black women have been underrepresented in left ventricular assist device (LVAD) clinical trials and published reports. The aim of this study was to examine the outcomes of Black women undergoing LVAD implantation. Methods We retrospectively identified Black women who underwent LVAD implantation at a single center between January 1, 2007 and October 31, 2020. Outcomes of patients who received HeartMate II (HMII), HeartWare (HVAD), and HeartMate 3 (HM3) devices were compared. Adverse events (AEs) for all patients were calculated as events per patient-year (EPPY). Reasons for readmissions, including incidence and rates, were reviewed. Results A total of 154 Black female patients underwent LVAD implantation during the study period. Sixty-six received HVAD, 36 received HMII, and 52 received HM3 devices (Figure 1). Median age for all patients was 57.7 years (IQR 46.9-64). At baseline, patients supported with HMII and HM3 were more likely to have diabetes compared to those supported with HVAD (HM3 50% vs HMII 50% vs HVAD 22.7%, p=0.003). HM3 recipients had a higher incidence of stroke pre-operatively (17.3% vs HMII 13.9% vs HVAD 3%, p=0.03). Post-operatively, HM3 recipients were on inotropes for longer duration (14 days vs HMII 9 days vs HVAD 8.5 days (IQR 5.3-16), p=0.004) and were placed on Epoprostenol at higher frequency (65.4% vs HMII 27.8% vs HVAD 39.4%, p=0.001). In regards to AEs, the incidence of LVAD-related infections was 25.3%, gastrointestinal bleeding was 33.8%, and cerebrovascular accidents was 14.3%. Twenty-nine pump thrombosis events occurred in 20 (13%) patients. Overall, patients supported with HM3 demonstrated lower rates of pump thrombosis (EPPY: 0.008 vs HMII 0.216 vs HVAD 0.062, p<0.001 and p=0.034, respectively)(Figure 1). HM3 patients experienced fewer readmissions for LVAD infection compared to HMII patients (EPPY: 0.19 vs HMII 0.49, p=0.001) as well as for cardiac arrhythmias (EPPY: 0.07 vs HMII 0.26, p=0.003). Hospitalization for LVAD alarms was significantly higher in the HM3 population (44% vs. HMII 19.4%, p<0.001). Conclusions Black Women LVAD recipients experienced differential outcomes post-LVAD by device type including rates of pump thrombosis, infection, and hospitalization for LVAD alarms. Additional studies are warranted to further examine racial and sex disparities in pre- and post-LVAD clinical outcomes.

Purpose Intra-aortic balloon pump (IABP) therapy is often used to bridge patients to durable left ventricular assist device (LVAD). We examined the hemodynamic changes following IABP insertion in cardiogenic shock patients requiring LVAD therapy. Methods We performed a retrospective observational study of patients receiving an IABP as a bridge to LVAD from 2015-2021. Hemodynamic metrics including right atrial pressure (RAP), pulmonary artery pressures (PAP), mixed venous oxygen saturation (SvO2), and cardiac output/indices (CO/CI) were collected at 1-hour, 24-hours, and 48-hours post-IABP insertion as well as day of insertion of the LVAD. Post-IABP hemodynamic measures were compared to the pre-IABP state. Heart failure-related labs were drawn prior to IABP and compared to 48 hours post-IABP. All p values < 0.05 were considered significant. Results A total of 104 patients were analyzed. Mean (SD) age was 55.8 (11.9) years old and median (IQR) days on IABP support was 4.5 (3.1). Improvement in RAP and PAP was noted at 24- and 48-hours post-IABP placement and on the day of LVAD placement. SvO2 and CO/CI were higher at all time points following IABP (Figure). All labs with the exception of sodium were improved at the 48-hour interval (Table). Conclusion IABP therapy improves hemodynamic profiles in cardiogenic shock patients bridged to LVAD as early as 1-hour post-placement and persists until the day of LVAD implantation. IABPs can serve as an effective platform to bridge selected patients to durable LVAD. Future research should be focused on the identification of predictors of IABP hemodynamic response in advanced heart failure patients.

Purpose Calcineurin inhibitors (CNI) are the mainstay of immunosuppression after heart transplantation. Atypical hemolytic uremic syndrome (aHUS), defined as the constellation of microangiopathic hemolytic anemia, thrombocytopenia and renal dysfunction, is a rare complication of CNI use following solid organ transplantation. This is the first case series to describe the clinical features, treatment and outcomes of patients with CNI-induced aHUS after heart transplantation. Methods Heart transplant recipients between 2014 and 2022 were retrospectively reviewed at our center; aHUS was defined by hemolytic anemia with elevated LDH, decreased haptoglobin, schistocytes on peripheral smear, thrombocytopenia and renal dysfunction. Results Of the 179 transplanted patients, 5 were identified to have clinically diagnosed CNI-induced aHUS (60% male, mean age 51.5 ± 11.8 years). Initial post-transplant immunosuppression was standardized as tacrolimus, mycophenolate mofetil and prednisone. Mean time from transplant to diagnosis of aHUS was 58.7 ± 34.2 days. Only one patient had abnormal ADAMST13 function. Upon diagnosis, tacrolimus was transitioned to cyclosporine in 2 patients, tacrolimus trough goals were decreased in 2 patients, and tacrolimus was transitioned to sirolimus in 1 patient. Monoclonal antibody therapy was the main therapeutic regimen (80%), with duration ranging 2 months to 6 years. Two patients received at least 2 sessions of plasmapheresis. Four of 5 experienced rehospitalization due to infection, and 2 of 5 ultimately died due to infection. Four required hemodialysis, of which one had renal recovery. Conclusion In this case series of CNI-induced aHUS, despite resolution of initial hemolytic anemia in all and monoclonal antibody therapy in most, a high frequency of dialysis and readmission for infection was observed. Optimal management remains unclear, and further studies are needed to guide immunosuppressive strategies in this complication.

Purpose Malnutrition and cardiac cachexia with low body mass index (BMI) are recognized pre-implant risk factors for adverse post-LVAD outcomes. The obesity paradox has defined a protective effect of higher BMI in terms of outcomes in heart failure (HF) patients. We evaluated the interaction of nutritional state (estimated by albumin) with BMI on LVAD outcomes. Methods A retrospective cohort study of patients implanted with continuous flow LVAD at our center between 2006 and 2022 was performed. Patients were stratified into four quartiles based on nutritional status and BMI. We considered malnourished to be albumin <3.5g/dL and well-nourished as albumin >3.5g/dL. We considered obesity to be a BMI >30kg/m2 and non-obese as BMI <30kg/m2. We created 4 groups - malnourished, obese (MN-Obese); malnourished, non-obese (MN-NonObese); well-nourished, obese (N-Obese); and well-nourished, non-obese (N-NonObese). We compared time to first HF exacerbation post-LVAD implantation amongst the 4 groups. Results 745 patients were included for analysis: 25% (n=185) MN-Obese, 45% (340) MN-NonObese, 14% (105) N-Obese and 16% (117) N-NonObese. Independent of nutritional status, obese patients were more likely to self-report as Black (p = 0.021), and have diabetes, hypertension and OSA (p = < 0.001 for all). Obese patients had higher incidence of first HF exacerbation than non-obese patients regardless of nutritional status (Figure). At 1 year, 20% of MN-NonObese and 13% of N-NonObese patients had been hospitalized compared to 32% of MN-Obese and 37% of N-Obese patients (p < 0.001). One year survival was similar between the groups. Conclusion Although there was no association with survival, obese LVAD patients have a higher rate of heart failure hospitalizations than non-obese. Malnutrition appears to increase the rate of hospitalization in non-obese LVAD patients, but not in the obese. Further investigation is warranted to understand the impact of BMI and nutritional status on long-term clinical outcomes with LVAD therapy.

Durable left ventricular assist devices (LVADs) provide circulatory support in patients with end-stage heart failure; however, complications include infection of the driveline exit site. Nontuberculous mycobacterial infections are rare in patients with LVADs, but they should be considered in those who have undergone device exchanges and have bacterial infections with driveline exit-site discharge but no fever or leukocytosis. We reviewed the charts of patients who had an LVAD implanted at our institution from January 2009 through December 2019, to identify those with a device-related nontuberculous mycobacterial infection. Collected data included patient demographics, premorbid conditions, infection type, previous device complications, treatment, and outcomes. We identified infections in 3 patients (mean age, 41 yr): Mycobacterium abscessus in 2 and M. chimaera in 1. All had a HeartMate II device and had undergone device exchanges for pump thrombosis or for driveline fault or infections. All presented with driveline exit-site discharge without fever or leukocytosis. The mean time between initial device implantation and diagnosis of a nontuberculous mycobacterial infection was 55 months. All 3 patients were treated with antibiotics and underwent localized surgical débridement; one underwent an additional device exchange. The M. abscessus infections disseminated, and both patients died; the patient with M. chimaera infection continued to take suppressive antibiotics. Nontuberculous mycobacterial infections are associated with high morbidity and mortality rates, warranting prompt diagnosis and treatment.

Infiltrative cardiomyopathies are an increasingly recognized cause of heart failure warranting systematic evaluation. Given overlap of clinical and imaging findings among etiologies of infiltrative cardiomyopathies, comprehensive evaluation, including a history and physical examination, advanced cardiac imaging, and sometimes endomyocardial biopsy, is required for diagnosis. We report a case of infiltrative cardiomyopathy in which endomyocardial biopsy confirmed diagnosis of cobalt-induced cardiomyopathy. The novel teaching points highlighted by this case report include identification of heavy-metal toxicity as a cause of infiltrative cardiomyopathy, and the outline of a diagnostic approach and management for cobalt-induced cardiomyopathy.

Cobalt toxicity is a rare cause of infiltrative cardiomyopathy. We describe a case of cobalt-induced cardiomyopathy that progressed to end stage heart failure requiring orthotopic heart transplantation. A 67 year old woman with history of bilateral cobalt-chromium hip replacements presented with dyspnea of 3 weeks duration. She was tachycardic and hypotensive, with elevated NT- proBNP. Electrocardiogram demonstrated sinus tachycardia, low voltage and right bundle branch block. Transthoracic echocardiogram revealed left ventricular ejection fraction of 20-25% with global hypokinesis and a moderate circumferential pericardial effusion. Left and right heart catheterization showed normal coronaries, elevated filling pressures and low cardiac index. Dobutamine infusion was initiated for management of cardiogenic shock. Cardiac magnetic resonance imaging (MRI) illustrated diffuse late gadolinium enhancement concerning for infiltrative cardiomyopathy. Extensive non-ischemic and rheumatologic workup was negative. Cobalt and chromium levels were significantly elevated at 121 mcg/L (Nl <=1mcg/L) and 46 mcg/L (Nl <=5 mcg/L) respectively, concerning for cobalt-induced cardiomyopathy. MRI of the hips showed pseudotumor formation surrounding the left prosthesis. She underwent left hip arthroplasty with removal of the source, and was placed on oral chelation therapy. She was discharged home on inotropic support. Despite decline in serum cobalt and chromium levels, the patient was readmitted with cardiogenic shock requiring a second inotropic agent. She was stabilized and evaluated for heart transplant. She underwent successful cardiac transplantation and was discharged to a rehab facility. Pathology of the native heart with electron microscopy revealed cardiomyocytes with enlarged distorted mitochondria, large lipid droplets and elevated tissue cobalt level of 0.95 mcg/g (reporting limit <0.018 mcg/g) consistent with cobalt-induced cardiomyopathy. Cobalt-induced cardiomyopathy is a potentially reversible cause of infiltrative cardiomyopathy, with the diagnosis hinging on obtaining a thorough medical history. Treatment includes routine heart failure management and removal of the cobalt source with the role of chelation therapy remaining unclear. Once progressed to end stage heart failure, preferred treatment is cardiac transplantation. Cobalt toxicity is a rare cause of infiltrative cardiomyopathy. We describe a case of cobalt-induced cardiomyopathy that progressed to end stage heart failure requiring orthotopic heart transplantation. A 67 year old woman with history of bilateral cobalt-chromium hip replacements presented with dyspnea of 3 weeks duration. She was tachycardic and hypotensive, with elevated NT- proBNP. Electrocardiogram demonstrated sinus tachycardia, low voltage and right bundle branch block. Transthoracic echocardiogram revealed left ventricular ejection fraction of 20-25% with global hypokinesis and a moderate circumferential pericardial effusion. Left and right heart catheterization showed normal coronaries, elevated filling pressures and low cardiac index. Dobutamine infusion was initiated for management of cardiogenic shock. Cardiac magnetic resonance imaging (MRI) illustrated diffuse late gadolinium enhancement concerning for infiltrative cardiomyopathy. Extensive non-ischemic and rheumatologic workup was negative. Cobalt and chromium levels were significantly elevated at 121 mcg/L (Nl <=1mcg/L) and 46 mcg/L (Nl <=5 mcg/L) respectively, concerning for cobalt-induced cardiomyopathy. MRI of the hips showed pseudotumor formation surrounding the left prosthesis. She underwent left hip arthroplasty with removal of the source, and was placed on oral chelation therapy. She was discharged home on inotropic support. Despite decline in serum cobalt and chromium levels, the patient was readmitted with cardiogenic shock requiring a second inotropic agent. She was stabilized and evaluated for heart transplant. She underwent successful cardiac transplantation and was discharged to a rehab facility. Pathology of the native heart with electron microscopy revealed cardiomyocytes with enlarged distorted mitochondria, large lipid droplets and elevated tissue cobalt level of 0.95 mcg/g (reporting limit <0.018 mcg/g) consistent with cobalt-induced cardiomyopathy. Cobalt-induced cardiomyopathy is a potentially reversible cause of infiltrative cardiomyopathy, with the diagnosis hinging on obtaining a thorough medical history. Treatment includes routine heart failure management and removal of the cobalt source with the role of chelation therapy remaining unclear. Once progressed to end stage heart failure, preferred treatment is cardiac transplantation.

Sarcoidosis is an increasingly prevalent systemic disease resulting in granulomatous inflammation. 1 Birnie DH Nery PB Ha AC Beanlands RS Cardiac sarcoidosis. J Am Coll Cardiol. 2016; 68: 411-421 Crossref PubMed Scopus (190) Google Scholar ,2 Kandolin R Lehtonen J Airaksinen J et al. Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study. Circulation. 2015; 131: 624-632 Crossref PubMed Scopus (228) Google Scholar Cardiac sarcoidosis (CS) can manifest as systolic heart failure (HF) resulting in increased morbidity and mortality. CS that progresses to end-stage HF may benefit from left ventricular assist device (LVAD) therapy to improve outcomes. 3 Crawford TC Okada DR Magruder JT et al. A contemporary analysis of heart transplantation and bridge-to-transplant mechanical circulatory support outcomes in cardiac sarcoidosis. J Card Fail. 2018; 24: 384-391 Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar The treatment of myocardial inflammation, as determined by 18-fludeoxyglucose positron emission tomography (FDG-PET), with immunosuppressive agents is central to prevent adverse cardiovascular events such as ventricular arrhythmias and even death. 4 Blankstein R Osborne M Naya M et al. Cardiac positron emission tomography enhances prognostic assessments of patients with suspected cardiac sarcoidosis. J Am Coll Cardiol. 2014; 63: 329-336 Crossref PubMed Scopus (378) Google Scholar Tumor necrosis factor-α (TNF-α) inhibitors are an established therapy for refractory extra CS, defined as not responsive to steroids and steroid-sparing agents such as methotrexate or mycophenolate. 5 Baughman RP Grutters JC New treatment strategies for pulmonary sarcoidosis: antimetabolites, biological drugs, and other treatment approaches. Lancet Respir Med. 2015; 3: 813-822 Abstract Full Text Full Text PDF PubMed Scopus (88) Google Scholar Recent publications have suggested a role for TNF-α inhibitors in the treatment of refractory CS, although safety concerns remain given Food and Drug Administration warnings regarding their use in moderate-to-severe HF. 6 Rosenthal DG Parwani P Murray TO et al. Long-term corticosteroid-sparing immunosuppression for cardiac sarcoidosis. J Am Heart Assoc. 2019; 8e010952 Crossref PubMed Scopus (21) Google Scholar , 7 Harper LJ McCarthy M Ribeiro Neto ML et al. Infliximab for refractory cardiac sarcoidosis. Am J Cardiol. 2019; 124: 1630-1635 Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar , 8 Page 2nd, RL O'Bryant CL Cheng D et al. Drugs that may cause or exacerbate heart failure: a scientific statement from the American Heart Association [published correction appears in Circulation 2016;134:e261]. Circulation. 2016; 134: e32-e69 Crossref PubMed Scopus (164) Google Scholar We present a case of refractory multisystem sarcoidosis complicated by advanced HF requiring LVAD therapy successfully treated with a TNF-α inhibitor, adalimumab.

Introduction HIV-associated pulmonary arterial hypertension (HIV-PAH) is an increasingly recognized complication of HIV, with a significant degree of associated morbidity and mortality. Given a wide array in the severity of clinical presentations, the prompt diagnosis and subsequent initiation of therapies has remained a challenge in this rare condition. Areas covered Diagnosis of HIV-PAH has been primarily established by transthoracic echocardiogram and right heart catheterization. There are several viral proteins that have been implicated in the pathogenesis of this condition that could be developed as either alternative or complimentary diagnostics in the evaluation of this condition. Therapy for HIV-PAH is centered on the management of HIV as well as the use of standard PAH therapies. The caveats in this distinct patient population include the consideration of drug-drug interactions of therapy with highly active antiretroviral therapy (HAART) and rare use of calcium channel blockers. Expert opinion Further research into the pathogenesis of HIV-PAH is warranted in order to develop diagnostics and therapy directed at the viral proteins implicated in this condition. This will be integral to the future evaluation and management of this unique patient population.

Video-assisted thoracoscopic surgery (VATS) with pleural decortication is a commonly performed procedure for patients with empyema and fibrosis. An 80-year-old female with coronary artery disease with left internal mammary bypass to left anterior descending artery and vein graft to left circumflex