Heart transplantation in muscular dystrophy: Single-center analysis.

INTRODUCTION: Cardiac involvement may occur in many forms of muscular dystrophy (MD). While cardiac disease may progress to warrant heart transplantation (HTx), there may be contraindications related to extra-cardiac disease including pulmonary and skeletal muscle involvement that limit overall survival and impairs post-transplant rehabilitation efforts. This study describes the MD HTx experience at a single high-volume center. METHODS: We examined the clinical characteristics and outcomes of patients with MD with heart failure (HF) (n = 28), patients with MD status post HTx (n = 20) and non-MD HTx control group (n = 40) matched 2:1 for age at transplant, sex, listing status, and antibody sensitization. RESULTS: Patients with MD who underwent HTx had increased ventilator days (2 vs. 1 days, p = .013), increased hospital length of stay (20 vs. 12 days, p = .022), and increased discharge to inpatient rehab (60% vs. 8%, p < .001). By 1 year post HTx, patients with MD more often required assistive devices for walking (55% vs. 10%, p = .01). Nonetheless, post-HTx survival was similar at 1 year (100% vs. 97.5%, p = .48) and 5 years (95.0% vs. 87.5%, p = .36). Of the HTx recipients with MD, 95% were followed by a neurologist, 60% by a neuromuscular specialist as part of the Muscular Dystrophy Association Clinic at our center. CONCLUSION: Transplantation is a feasible option for patients with MD and advanced HF. MD patients who undergo transplantation may benefit from multidisciplinary specialized care to optimize MD-related morbidity.

Prognostic Value of Left Ventricular Global Longitudinal Strain in Transcatheter Edge-to-Edge Repair for Chronic Primary Mitral Regurgitation.

AIMS: Left ventricular global longitudinal strain (LVGLS) is a known outcome predictor in transcatheter edge-to-edge repair (TEER) for functional mitral regurgitation (MR). We aimed to assess its prognostic yield in the setting of TEER for chronic primary MR. METHODS AND RESULTS: We conducted a single-center, retrospective analysis of 323 consecutive patients undergoing isolated, first-time procedures. Stratified by baseline LVGLS quartiles (≤-19%, -18.9-(-16)%, -15.9-(-12)%, > -12%), the cohort was evaluated for the primary composite outcome of all-cause mortality or heart failure hospitalizations, as well as secondary endpoints consisting of mitral reinterventions and the persistence of significant residual MR and/or functional disability - all along the first year after intervention. Subjects with worse (i.e., less negative) LVGLS exhibited higher comorbidity, more advanced HF, and elevated procedural risk. Post-TEER, those belonging to the worst LVGLS quartile group sustained increased mortality (16.9 vs 6.3%, Log-Rank p = 0.005, HR 1.75, 95% CI 1.08-4.74, p = 0.041) and, when affected by LV dysfunction/dilatation, more primary outcome events (21.1 vs 11.5%, Log-Rank p = 0.037, HR 1.68, 95% CI 1.02-5.46, p = 0.047). No association was demonstrated with other endpoints. Upon exploratory analysis, 1-month postprocedural LVGLS directly correlated with and was worse than its baseline counterpart by 1.6%, and a more impaired 1-month value - but not the presence/extent of deterioration - conferred heightened risk for the primary outcome. CONCLUSION: TEER for chronic primary MR is feasible, safe, and efficacious irrespective of baseline LVGLS. Yet, worse baseline LVGLS forecasts a less favorable postprocedural course, presumably reflecting a higher-risk patient profile.

Dyslipidemia in midlife women: Approach and considerations during the menopausal transition.

Dyslipidemia is an established risk factor for cardiovascular disease (CVD), which remains the leading cause of morbidity and mortality in women globally. The incidence of dyslipidemia increases over a woman's lifespan, with adverse changes around the time of menopause. Menopause, and the years leading up to the final menstrual period, is a time of estrogen fluctuation and ultimately estrogen deficiency, which has been associated with proatherogenic changes in the lipid profile. Independent of aging, menopausal status is associated with elevations in serum total cholesterol, LDL cholesterol, apolipoproteins, and triglycerides, and decreases in HDL cholesterol (HDL-C). Emerging research also suggests that after menopause there is a loss of functional HDL cardioprotective properties. Early initiation of menopausal hormone therapy (MHT) confers a favorable effect on lipid profile, though this does not translate into improved CVD outcomes and therefore guidelines do not indicate it for primary or secondary prevention of CVD. At the time of menopause, special consideration should be given to women with conditions more associated with CVD, including polycystic ovarian syndrome, premature menopause, early menopause, premature ovarian insufficiency, and familial hypercholesterolemia. Statins remain the mainstay of dyslipidemia therapy, though novel lipid-lowering agents are emerging. This review provides an overview of lipid alterations observed during the menopausal transition, summarizes the current evidence on the role of estrogen and progestogen on lipids, identifies special populations of women at especially high risk for lipid dysregulation at menopause, and describes approaches to the screening and treatment of midlife women.

Scleroderma Renal Crisis Associated With Microangiopathic Hemolytic Anemia in a Patient With Seronegative Scleroderma and Monoclonal Gammopathy.

Systemic sclerosis with negative serology, particularly that complicated by scleroderma renal crisis (SRC), is rarely encountered. We describe a patient with seronegative systemic sclerosis who developed acute kidney injury, proteinuria, and hypertensive emergency following motor vehicle-related trauma and in the setting of nonsteroidal anti-inflammatory drug use. Findings on physical examination, imaging, and skin biopsy led to a clinical diagnosis of scleroderma, despite the lack of supportive laboratory data. IgG lambda paraproteinemia was detected on workup. Bone marrow biopsy showed plasmacytosis and trace lambda-restricted plasma cells consistent with monoclonal gammopathy of undetermined significance. Chemotherapy was initially started given concern for myeloma with cast nephropathy but was later stopped after a kidney biopsy revealed thrombotic microangiopathy (TMA). The SRC associated with TMA was ultimately diagnosed, though atypical hemolytic uremic syndrome (aHUS) induced perhaps by monoclonal gammopathy or hypertension was also possible. Captopril and eculizumab were initiated for SRC and aHUS, respectively. Despite therapy, renal function did not recover, and the patient required hemodialysis indefinitely. This case highlights clinical features common to both SRC and aHUS as well as points out a few ways to differentiate between them.

MRI of surgical flaps in pelvic reconstructive surgery: a pictorial review of normal and abnormal findings.

Surgical flaps are commonly used for pelvic reconstruction in a subgroup of patients with locally advanced or recurrent anorectal and gynecologic malignancies and following complications of pelvic irradiation. Surgical scenarios where flaps may be placed include (but are not limited to) extended or radical abdominal perineal resection (APR) and total pelvic exenteration (PE). Surgical flaps in pelvic reconstruction serve several functions, including reducing dead space and providing structural support, facilitating wound closure and cosmetic appearance, enhancing the postsurgical healing process, protecting anastomoses and helping to prevent adhesions of organs and viscera to adjacent structures and the pelvic side wall. The most commonly used surgical flaps in pelvic reconstruction surgery include the VRAM (Vertical Rectus Abdominis Muscle), MRAM (Modified Rectus Abdominis Myocutaneous flap), gracilis, sartorius and omental flaps. Surgical flaps can be mistaken for recurrent or residual tumor by radiologists who are not familiar with the appearance or surgical methods of flap placement, since flaps may have a mass-like appearance on cross sectional imaging, including CT and MRI. Recurrent neoplasm may be difficult to differentiate from postoperative changes of flap placement and associated postsurgical anatomic distortion. This review article focuses on understanding the nuances of surgically placed pelvic flaps and identifying their normal and abnormal appearances on magnetic resonance imaging (MRI) along a time continuum. Postsurgical complications, including hematoma, postoperative fluid collections, infection, ischemia, and necrosis as well as tumor recurrence on the initial and follow-up magnetic resonance imaging are illustrated and discussed.