Prevalence and pharmacologic management of familial hypercholesterolemia in an unselected contemporary cohort of patients with stable coronary artery disease

INTRODUCTION: Familial hypercholesterolemia (FH) is an inherited disorder characterized by elevated plasma levels of low-density lipoprotein cholesterol (LDL-C) associated with premature cardiovascular disease. METHODS: Using the data from the START (STable Coronary Artery Diseases RegisTry) study, a nationwide, prospective survey on patients with stable coronary artery disease (CAD), we described prevalence and lipid lowering strategies commonly employed in these patients. The study population was divided into "definite/probable FH," defined as a Dutch Lipid Clinic Network (DLCN) score ≥6, "possible FH" with DLCN 3-5, and "unlikely FH" in presence of a DLCN <3. RESULTS: Among the 4030 patients with the DLCN score available, 132 (3.3%) were classified as FH (2.3% with definite/probable and 1.0% with possible FH) and 3898 (96.7%) had unlikely FH. Patients with both definite/probable and possible FH were younger compared to patients not presenting FH. Mean on-treatment LDL-C levels were 107.8 ± 41.5, 84.4 ± 40.9, and 85.8 ± 32.3 (P < 0.0001) and a target of ≤70 mg/dL was reached in 10.9%, 30.0%, and 22.0% (P < 0.0001) of patents with definite/probable, possible FH, and unlikely FH, respectively. Statin therapy was prescribed in 85 (92.4%) patients with definite/probable FH, in 38 (95.0%) with possible FH, and in 3621 (92.9%) with unlikely FH (P = 0.86). The association of statin and ezetimibe, in absence of other lipid-lowering therapy, was more frequently used in patients with definite/probable FH compared to patients without FH (31.5% vs 17.5% vs 9.5%; P < 0.0001). CONCLUSIONS: In this large cohort of consecutive patients with stable CAD, FH was highly prevalent and generally undertreated with lipid lowering therapies

Prognostic relevance of a non-invasive evaluation of right ventricular function and pulmonary artery pressure in patients with chronic heart failure.

AIM: To determine the prognostic relevance of the echocardiographic evaluation of pulmonary artery systolic pressure (PASP) and tricuspid annular plane systolic excursion (TAPSE) in patients with chronic heart failure (CHF). Pulmonary hypertension (PH) and right ventricular (RV) dysfunction have both been associated with poor prognosis in CHF. METHODS AND RESULTS: A complete echocardiographic examination was performed in 658 outpatients with CHF and LVEF <45%. PASP was available in 544 (83%) patients, TAPSE in all patients, and E wave deceleration time (DT) in 643 (98%) patients. During a median follow-up period of 38 months, 125 patients died, 5 underwent urgent heart transplantation, and 5 had an appropriately detected and treated episode of ventricular fibrillation. At Cox survival analysis (composite endpoint was death, urgent heart transplantation, and ventricular fibrillation), patients with PASP ≥40 mmHg plus TAPSE ≤14 mm had a poorer prognosis than those with high PASP but preserved TAPSE; RV dysfunction associated with normal PASP did not carry additional risks. Similar results were obtained when patients were grouped on the basis of DT (restrictive vs. non restrictive) and TAPSE. CONCLUSIONS: A simple echocardiographic evaluation of PASP and RV function with TAPSE may improve risk stratification in patients with CHF. Importantly, if PASP cannot be recorded at echocardiography, a restrictive DT, measurable in the vast majority of patients, may be coupled with TAPSE to stratify patients