Outcomes of Ultrafiltration in community-based hospitals.

OBJECTIVE: We sought to examine outcomes of ultrafiltration in real world community-based hospital settings. BACKGROUND: Ultrafiltration (UF) is an accepted therapeutic option for advanced decompensated heart failure (ADHF). the feasibility of UF in a community hospital setting, by general cardiologists in a start-up program had not been objectively evaluated. METHODS: We retrospectively analyzed the first-year cohort of ADHF patients treated with UF from 10/1/2019 to 10/1/2020, which totaled 30 patients, utilizing the CHF Solutions Aquadex FlexFlow™ System with active UF rate titration. RESULTS: Baseline patient characteristics were similar to RCTs: mean age 63, 73% male; 27% female; 53% Caucasian; 47% African American; 77% had LVEF ≤ 40. The baseline mean serum creatinine (Cr) was 1.84 ±0.62 mg/dL, mean GFR of 36.95 ±9.60 ml/min. HF re-admission rates were not significantly different than prior studies (17.2% at 30 d, 23.3% at 60 d, but in our cohort, per patient HF re-admission rates were reduced significantly by 60 d (0.30 p = 0.017). CONCLUSION: Our analysis showed success with UF in mainstream setting with reproducible results of significant volume loss without adverse renal effect, mitigation of recurrent Hdmissions, and remarkable subjective clinical benefit.

Prevalence of lymphedema among Anderson-Fabry disease patients: A report from the Fabry registry.

BACKGROUND: Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease due to a genetic variation in the α-galactosidase A (GLA) gene. As a result, the activity of the α-galactosidase A (AGAL-A) enzyme is reduced or absent, which causes sphingolipid deposition within different body parts. AFD typically manifests with cardiovascular, renal, cerebrovascular, and dermatologic involvement. Lymphedema is caused by sphingolipid deposition within lymphatics. Lymphedema can cause intolerable pain and limit daily activities. Very limited data exist on lymphedema in AFD patients. METHODS: Using data from the Fabry Registry (NCT00196742) with 7671 patients included (44% males and 56% females), we analyzed the prevalence of lymphedema among AFD patients who were ever assessed for lymphedema and studied the age of first reported lymphedema. Additionally, we assessed whether patients received AFD-specific treatment at some point during their clinical course. The data was stratified by gender and phenotype. RESULTS: Our study showed that lymphedema occurred in 16.5% of the Fabry Registry patients who were ever assessed for lymphedema (n = 5487). Male patients when compared to female patient have higher prevalence (21.7% vs 12.7%) and experienced lymphedema at a younger age (median age at first reported lymphedema of 43.7 vs 51.7 years). When compared to other phenotypes, classic phenotype has the highest prevalence of lymphedema with the earliest reported lymphedema. Among those who reported lymphedema, 84.5% received AFD-specific treatment during their clinical course. CONCLUSIONS: Lymphedema is a common manifestation of AFD in both genders, with a tendency to present later in female patients. Recognition of lymphedema can offer an important opportunity for intervention and potential impact on associated morbidity. Additional future studies are needed to characterize the clinical implications of lymphedema in AFD patients and identify additional treatment options for this growing population.

Barth Syndrome: Psychosocial Impact and Quality of Life Assessment.

BACKGROUND: Barth syndrome (BTHS) is a rare X-linked genetic disease that affects multiple systems and leads to complex clinical manifestations. Although a considerable amount of research has focused on the physical aspects of the disease, less has focused on the psychosocial impact and quality of life (QoL) in BTHS. METHODS: The current study investigated caregiver- (n = 10) and self-reported (n = 16) psychological well-being and QoL in a cohort of BTHS-affected patients and families. Participants completed the depression and anxiety components of the Patient-Reported Outcomes Information System (PROMIS) Short Form 8A and Health-related quality of life (HRQoL) surveys at enrollment and again during a follow-up period ranging from 6 to 36 months after baseline. RESULTS: Quality of life changed significantly over time and the various domains with some improvement and some decline. Among the available caregiver-patient dyad data, there was a trend toward discordance between caregiver and self-reported outcomes. Most notably, patients reported improvement in HRQoL, while caregivers reported declines. This suggests that there may be differences in perceived quality of life between the patients and parents, though our study is limited by small sample size. CONCLUSION: Our study provides valuable insights into the impacts of psychosocial and mental health aspects of BTHS. Implications of these findings include incorporating longitudinal assessment of QoL and screening for psychological symptoms in BTHS care to identify interventions that may drastically impact health status and the course of the disease.

A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment.

Amyloidosis is a group of disorders that can affect almost any organ due to the misfolding of proteins with their subsequent deposition in various tissues, leading to various disease manifestations based on the location. When the heart is involved, amyloidosis can manifest with a multitude of presentations such as heart failure, arrhythmias, orthostatic hypotension, syncope, and pre-syncope. Diagnosis of cardiac amyloidosis can be difficult due to the non-specific nature of symptoms and the relative rarity of the disease. Amyloidosis can remain undiagnosed for years, leading to its high morbidity and mortality due to this delay in diagnosis. Newer imaging modalities, such as cardiac magnetic resonance imaging, advanced echocardiography, and biomarkers, make a timely cardiac amyloidosis diagnosis more feasible. Many treatment options are available, which have provided new hope for this patient population. This manuscript will review the pathology, diagnosis, and treatment options available for cardiac amyloidosis and provide a comprehensive overview of this complicated disease process.

Drug-eluting versus nondrug peripheral vascular interventions.

Introduction: Drug-eluting (DRUG) peripheral vascular interventions (PVIs) are associated with higher patency rates than nondrug (NONDRUG) PVIs. Recent data raised safety concerns with using DRUG devices in PVIs. Material and methods: The study population was extracted from the 2016 Nationwide Readmissions Database using the International Classification of Diseases, tenth edition, clinical modifications/procedure coding system codes for PVI, DRUG and NONDRUG devices, and in-hospital procedural complications. Study endpoints included in-hospital all-cause mortality, length of index hospitalization, acute kidney injury (AKI), amputation, compartment syndrome, vascular complications, bleeding, and blood transfusion. Propensity matching was used to adjust for baseline characteristics. Results: 49,883 discharged patients who underwent lower extremity arterial PVI were identified, 25.3% DRUG and 74.7% NONDRUG PVI. Mean age was 68.3 years and 40.6% were female. Critical limb ischemia was reported in 33.2%, claudication in 7.6%, and acute limb ischemia in 0.1%. In comparison to the NONDRUG group, the DRUG group was associated with lower in-hospital all-cause mortality (2.2 vs. 2.9%, p < 0.001), shorter length of index hospitalization (8.3 vs. 8.6 days, p = 0.001), bleeding (12.0% vs. 13.5%, p < 0.001), and need for blood transfusion (10.1% vs. 11.0%, p = 0.004). There was no significant difference in terms of AKI (17.3% vs. 18.0%, p = 0.10), amputation (15.3% vs. 15.4%, p = 0.63), compartment syndrome (0.5% vs. 0.6%, p = 0.07), or vascular complications (0.8% vs. 0.8%, p = 0.50). After propensity matching, the mortality benefit was no longer present. Conclusions: DRUG PVI was associated with lower in-hospital all-cause mortality, bleeding events and shorter length of index hospitalization and comparable vascular-related complications. However, this mortality benefit was no longer present after propensity matching.

Complex interventions in the failing heart.

PURPOSE OF REVIEW: Heart failure is a complex clinical syndrome with a substantial disease burden. We aim to review the interventional aspects of management of advanced heart failure, focusing on the role of management of coronary artery disease, valvular heart disease, and mechanical circulatory support. RECENT FINDINGS: The patients with coronary artery disease and heart failure requiring revascularization are at higher risk than the rest of the general population. Coronary artery bypass grafting or percutaneous intervention can be used depending on varied patient characteristics and coronary anatomy. Transcatheter aortic valve implantation, Mitraclip and transcatheter ventricular restoration help in tackling the valvular and left ventricular remodeling concerns often seen in this patient population. Temporary hemodynamic circulatory support helps stabilizing patients in cardiogenic shock while long-term support can help bridge them to more definitive therapies. SUMMARY: The management strategies in this disease state are ever evolving with robust evidence coming in support of interventional therapies whenever deemed appropriate. It is the multidisciplinary patient-centered approach, which yields maximum benefit out of these complex interventions.

Rare and unusual presentation of gastrocardiac syndrome.

Gastrointestinal pathology can cause cardiac symptoms and disorders. We present a case of a patient who had worsening of her palpitations with food intake. She was found to have a high burden of premature ventricular contractions in the setting of hiatal hernia and gastro-oesophageal reflux disease. After extensive investigations and ruling out cardiac causes, her arrhythmia resolved with the surgical correction of hiatal hernia.

An Unusual Presentation of Median Arcuate Ligament Syndrome.

Median arcuate ligament syndrome (MALS) is a rare benign condition typically affecting young females. It usually presents with abdominal symptoms of pain, nausea, and unintentional weight loss. They are usually diagnosed incidentally on CT of the abdomen done for abdominal pain. Here we present a rare case of MALS which presented with an anginal type of chest pain without any abdominal symptoms leading to an extensive workup and incidental diagnosis.

Non-ischemic Cardiomyopathy: A Rare Adverse Effect of Clozapine.

Clozapine is a dibenzodiazepine antipsychotic used for treatment-resistant schizophrenia. Its association with several side effects such as agranulocytosis, seizure, and insulin resistance is well known. Cardiac side effects such as myocarditis and cardiomyopathy are less common and have been seldom reported. Here we report an unusual case of clozapine-induced nonischemic dilated cardiomyopathy. A 50-year-old female with treatment-resistant schizophrenia on clozapine presented with gradually worsening shortness of breath, productive cough, and pleuritic chest pain. She was found to have non-ischemic dilated cardiomyopathy due to clozapine use as no other causative factor was found. Clozapine was gradually tapered and then discontinued. Repeat echocardiogram in three months revealed improvement in ejection fraction. This case is unique as it outlines clozapine as a rare cause of nonischemic cardiomyopathy, as discontinuation of the drug showed improvement in symptoms and heart function.

The First 4 Years of Postmarketing Safety Surveillance Related to the MitraClip Device: A United States Food and Drug Administration MAUDE Experience.

OBJECTIVE: The MitraClip (Abbott) is a commercially available device to perform percutaneous transcatheter mitral valve repair (TMVR) for patients with symptomatic mitral regurgitation (MR). Recent data support its role in appropriately selected patients with functional MR, and its use is poised to increase. However, limited safety data in "real-world" practice are available after market introduction. METHODS: We queried all available adverse event reports from the publicly available Manufacturer and User Facility Device Experience (MAUDE) database including "injuries" and "deaths" from October 2013 (date of Food and Drug Administration [FDA] premarket approval) to September 2017 using the following search limits: brand name ("MitraClip") and product code ("NKM," a unique FDA designation linked to MitraClip). RESULTS: During the first 4 years after FDA approval, MAUDE received 200 death reports and 1666 injury reports containing 2974 unique adverse events. Of all death reports, 21% described deaths occurring >1 year post MitraClip and 30% included limited details. The top three known causes of death included complications requiring rescue high-risk surgery, clip detachment or unsuccessful clip placement, and damage to the valvular apparatus. Similar non-fatal events were reported. Additional procedures or surgical intervention were required in 227 injury events (8%). CONCLUSIONS: While injuries reported to the FDA have steadily increased with more widespread use of TMVR, device- or procedure-related death reports have accrued more slowly, corroborating a potential institutional or operator learning curve with this device. However, in light of incomplete and poor data quality, higher-fidelity systems of postmarketing safety surveillance are needed in the evaluation of emerging devices.

A right atrial fat ball: Rare case of cardiac lipoma.

Due to varied presentation, a high index of suspicion is needed for diagnosis of cardiac lipoma. Treatment should only be reserved for symptomatic patients. This case acknowledges the importance of cardiac MRI in making the diagnosis of cardiac lipoma and further delineating the management options available.

Slit in the Coronaries: A Case of Spontaneous Coronary Artery Dissection.

Spontaneous coronary artery dissection (SCAD) is a noniatrogenic epicardial coronary artery dissection unrelated to an atherosclerotic disease process. SCAD is responsible for a small percentage of acute coronary syndrome (ACS) cases. The left anterior descending (LAD) artery is the most common artery affected in SCAD, although any coronary artery can be affected. We present an interesting case of SCAD presenting as an ST-elevation myocardial infarction complicated with dissection extending to the left main and distal LAD requiring emergent coronary artery bypass grafting. Our case emphasizes the importance of considering SCAD as a cause for ACS, especially in young patients with minimal atherosclerotic risk factors. Also, a very high recurrence rate demands strict follow-up and multidisciplinary decision making in the population impacted with this rare entity.

Pericardial Effusion: Rare Adverse Effect of Clozapine.

Clozapine is a dibenzodiazepine antipsychotic used for resistant schizophrenia, which is known to be associated with side effects such as agranulocytosis, seizures, weight gain, and less commonly myocarditis/ cardiomyopathy. We present a case of a 20-year-old female who presented with chest pain, shortness of breath, and cough. She was later found to have clozapine-induced pericardial effusion that resolved after discontinuation of clozapine therapy. Our case discloses the importance to consider clozapine in the differential diagnosis of pericardial effusion as discontinuation of the drug leads to resolution of effusion, with no need for further treatment.

A Case of Left Ventricular Noncompaction Presenting as Atrial Fibrillation.

Ventricular noncompaction is a rare congenital cardiomyopathy resulting from failure in cardiac embryogenesis. Both left and right ventricular involvement has been reported in nature. We present a case of a 69-year-old male presenting with atrial fibrillation complicated with in-hospital cardiac arrest in the setting of left ventricular noncompaction (LVNC). Our case demonstrates the fatal nature of this disease entity thus demanding a high index of suspicion for early detection and treatment.