Ayman Jubran

Clinical determinants and treatment gaps in familial hypercholesterolemia: Data from a multi-ethnic regional health service

Background Familial hypercholesterolemia is characterized by markedly increased low-density lipoprotein cholesterol and risk for premature atherosclerotic cardiovascular disease. Models of care vary and reflect differing health policies and resources. The availability of electronic databases may enable better identification and assessment of familial hypercholesterolemia in the community. Methods A regional healthcare database was utilized to identify patients with a high probability of familial hypercholesterolemia, clinically defined by age-dependent-peak low-density lipoprotein cholesterol cutoffs and exclusion of secondary causes of severe hypercholesterolemia. Clinical characteristics, low-density lipoprotein cholesterol goal attainment, and treatment gaps were investigated. Results Probable familial hypercholesterolemia was diagnosed in 1932 of 685,314 individuals (1:355; median age 47 years). Atherosclerotic cardiovascular disease was present in 16.3% of adults (38% in males aged 50–74 years). Median peak low-density lipoprotein cholesterol was 264 mg/dl (interquartile range 252–288). Statins and/or ezetimibe were prescribed to 83% of patients and high-intensity statins to 53%, whereas prescriptions were filled in 57% and 40% cases respectively over the last six months, p < 0.001. Treatment gaps were wider among ethnic minorities, younger individuals, and those without atherosclerotic cardiovascular disease. Low-density lipoprotein cholesterol < 100 mg/dl was attained in 10.1% overall and 28.7% of those with atherosclerotic cardiovascular disease. Predictors of low-density lipoprotein cholesterol goal attainment included recent issue of high-intensity statins, presence of atherosclerotic cardiovascular disease, diabetes, older age and lack of smoking. Conclusions The population with high probability for familial hypercholesterolemia was characterized by low attainment of low-density lipoprotein cholesterol treatment goals despite high prescription rates of lipid-lowering medications. Low utilization of intensified therapies, non-adherence, and ethnic disparities were contributing factors. These findings emphasize the need to improve awareness and quality of care of familial hypercholesterolemia in the community.

Clinical Features and Gaps in the Management of Probable Familial Hypercholesterolemia and Cardiovascular Disease

BACKGROUND Familial hypercholesterolemia (FH) is associated with premature atherosclerotic cardiovascular disease (ASCVD). The introduction of potent therapeutic agents underlies the importance of improving clinical diagnosis and treatment gaps in FH.Methods and Results:A regional database of 1,690 adult patients with high-probability FH based on age-dependent peak-low-density lipoprotein cholesterol (LDL-C) cut-offs and exclusion of secondary causes of severe hypercholesterolemia, was examined to explore the clinical manifestations and current needs in the management of ASCVD, which was present in 248 patients (15%), of whom 83% had coronary artery disease (CAD); 19%, stroke; and 13%, peripheral artery disease. ASCVD was associated with male gender, higher peak LDL-C, lower high-density lipoprotein cholesterol (HDL-C), and traditional risk factor burden. Despite high-intensity statin (prescribed in 83% and combined with ezetimibe in 42%), attainment of LDL-C treatment goals was low, and associated with treatment intensity and drug adherence. Multivessel CAD (adjusted hazard ratios (HR), 3.05; 95% CI: 1.65-5.64), myocardial infarction, and the presence of ≥1 traditional risk factor (HR, 2.59; 95% CI: 1.42-4.71), were associated with repeat coronary revascularizations, in contrast with peak LDL-C >300 mg/dL (HR, 1.13; 95% CI: 0.66-1.91). CONCLUSIONS Main manifestations of ASCVD in FH patients were premature, multivessel CAD with need for recurrent revascularization, associated with classical cardiovascular risk factors but not with peak LDL-C. In spite of intensive therapy with lipid-lowering agents, treatment gaps were significant, with low attainment of LDL-C treatment goals.

TRANSCATHETER AORTIC VALVE REPLACEMENT IN ONCOLOGY PATIENTS WITH AORTIC STENOSIS

Patients with malignancy and severe aortic stenosis (AS) are often denied from surgical aortic valve replacement (AVR). Transcatheter AVR (TAVR) may be a better option for these patients, but was not yet systematically evaluated. An international TAVR in Oncology Patients with AS (TOP AS) registry

Varying clinical presentations of anomalous origin of the left main coronary artery from the right coronary sinus with an interarterial course in adults

The diagnosis of anomalous origin of the left main coronary artery from the right coronary sinus with an interarterial course in children and adolescents is considered life-threatening and clinical guidelines recommend surgical correction. The prognostic implications of this diagnosis in adults are not clear. This anomaly may present in adults as sudden cardiac death or may be diagnosed incidentally. Treatment of this anomaly in adults should be tailored individually taking into account the clinical presentation and patient characteristics.

Aortic Root Intussusception During Transcatheter Aortic Valve Replacement

Accurate valve positioning during transcatheter aortic valve replacement (TAVR) is crucial. Valve migration into the left ventricle during deployment can be treated by pulling the valve toward the aortic root or by implanting a second valve. An 83-year-old woman with severe symptomatic aortic