Population, genetic, and clinical studies demonstrated a causative and continuous, from other plasma lipoproteins independent relationship between elevated plasma lipoprotein (a) [Lp(a)] concentration and the development of cardiovascular disease (CVD), mainly those related to athe-rosclerotic CVD, and calcific aortic stenosis. Currently, a strong international consensus is still lacking regarding the single value which would be commonly used to define hyperlipoproteinemia (a). Its prevalence in the general population is estimated to be in the range of 10%–35% in accordance with the most commonly used threshold levels (>30 or >50 mg/dL). Since elevated Lp(a) can be of special importance in patients with some genetic disorders, as well as in individuals with otherwise controlled major risk factors, the identification and establishment of the proper therapeutic interventions that would lower Lp(a) levels and lead to CVD risk reduction could be very important. The majority of the classical lipid-lowering agents (statins, ezetimibe, and fibrates), as well as nutraceuticals (CoQ10 and garlic), appear to have no significant effect on its plasma levels, whereas for the drugs with the demonstrated Lp(a)-lowering effects (aspirin, niacin, and estrogens), their clinical efficacy in reducing cardiovascular (CV) events has not been unequivocally proven yet. Both Lp(a) apheresis and proprotein convertase subtilisin/kexin type 9 inhibitors can reduce the plasma Lp(a) by approximately 20%–30% on average, in parallel with much larger reduction of low-density lipoprotein cholesterol (up to 70%), what puts us in a difficulty to conclude about the true contribution of lowered Lp(a) to the reduction of CV events. The most recent advancement in the field is the introduction of the novel apolipoprotein (a) [apo(a)] antisense oligonucleotide therapy targeting apo(a), which has already proven itself as being very effective in decreasing plasma Lp(a) (by even >90%), but should be further tested in clinical trials. The aim of this review was to present some of the most important accessible scientific data, as well as dilemmas related to the currently and potentially in the near future more widely available therapeutic options for the management of hyperlipoproteinemia (a).

The need for aspirin therapy as part of primary prevention of cardiovascular (CV) disease is currently being highly debated, especially after 3 studies in different settings reported that a reduction in ischemic events is largely counterbalanced by an increase in bleeding events. One possible explanation for these results is the progressive reduction in the risk of major adverse cardiovascular events (MACE) as a result of primary prevention, which has accompanied global education programs that have led to patients smoking less, exercising more, and increasingly undertaking lipid-lowering therapies. Based on a meta-regression of the benefits and harmful effects of aspirin therapy in primary prevention as a function of the 10-year risk of MACE, we favor a differentiated and personalized approach that acknowledged differences between patients and emphasized an individualized assessment of benefits and risks. Following general preventive measures (physical exercise, cessation of smoking, treatment of hypertension and hypercholesterolemia, etc.), an individualized approach to prescribing aspirin is still warranted. When patients are less than 70 years of age, clinicians should assess the 10-year CV risk. Aspirin treatment should be considered only when the CV risk is very high and the bleeding risk is low, after taking into account the patient’s preferences.

Objective: Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism which arises due to deficient or absent activity of lysosomal α-galactosidase A (α-Gal A). This may be associated with increased left ventricular (LV) wall thickness and may mimic the morphological features of hypertrophic cardiomyopathy. The purpose of this study was to define the ratio of occurrence of FD to the manifestation of unexplained left ventricular hypertrophy (LVH).
Methods: We studied a prospectively assembled a consecutive cohort of 190 patients with unexplained LVH on echocardiography. The criterion for LVH diagnosis was a maximum LV wall thickness of 13 mm or greater. All patients were tested for mutations in the GLA gene.
Results: The majority of patients were male (n=119, 63%) and the mean patient age was 47.2±15 years. In 190 patients diagnosed with LVH, we identified 2 patients (1.05%) with documented GLA mutations [c.427G>A (p.A143T)(p.Ala143Thr)] and [c.937G>T (p.D313Y)(p.Asp313Tyr)]. After the family screening, 3 additional patients with FD were identified in 2 families, including 5 individuals who are now receiving enzyme replacement therapy.
Conclusion: We identified 2 index patients with FD and unexplained LVH. Cardiologists should, therefore, be aware of FD in cases of unexplained LVH. Family screening is crucial for the earlier identification of unaffected new patients who may benefit from enzyme replacement therapy.

Objective: Currently, there are limited comparative data concerning long-term major clinical outcomes following the angiotensin-converting-enzyme inhibitors (ACEIs) and angiotensin II type 1 (AT1) receptor blockers (ARBs) therapy in patients with acute myocardial infarction (AMI) with dyslipidemia after a successful stent implantation. Therefore, we investigated major clinical outcomes for 2 years following the ACEIs and ARBs therapy in these patients.
Methods: A total of 3015 patients with AMI who underwent a successful stent implantation and were prescribed ACEIs (n=2175) or ARBs (n=840) were enrolled into the study from the Korea AMI Registry (KAMIR). The major clinical endpoint was the occurrence of major adverse cardiac events (MACEs) defined as all-cause death, recurrent myocardial infarction (Re-MI), and any repeat-revascularization-comprised target lesion revascularization (TLR), target vessel revascularization (TVR), and non-TVR.
Results: After the adjustment, the cumulative incidence of all-cause death in the ARBs group was significantly higher than in the ACEIs group [adjusted hazard ratio (aHR), 2.277; 95% confidence interval (CI), 1.154–4.495; p=0.018]. The cumulative incidences of MACEs (aHR, 1.305; 95% CI, 0.911–1.869; p=0.146), cardiac death, Re-MI, any repeat revascularization, TLR, TVR, and non-TVR were similar between the two groups. In addition, an advanced age (≥65 years), decreased left ventricular ejection fraction (<50%), and cardiopulmonary resuscitation on admission were meaningful independent predictors for all-cause death in this study.
Conclusion: ACEIs were a preferred treatment modality when compared to ARBs for patients with AMI with dyslipidemia who underwent a successful stent implantation to reduce the incidences of all-cause death during a 2-year follow-up. However, additional research is required to determine the clinical implications of these results.

Objective: Cardiovascular disease (CVD) is the leading cause of death during pregnancy. Therefore, its detection early in pregnancy is of great importance. In this study, we aimed to investigate the rate and spectrum of CVD among healthy women in the first trimester of pregnancy.
Methods: A total of 900 women in the first trimester of pregnancy, who attended the antenatal outpatient clinic of a tertiary care center in Ankara, Turkey, for a routine pregnancy examination, were recruited into this prospective study. Patients with a history of chronic systemic diseases, CVD, and/or a family history of an early onset CVD, and multiple pregnancies were excluded. Patients who were included in the study underwent electrocardiography and transthoracic echocardiography by the same cardiologist.
Results: The rate of newly diagnosed CVD cases among asymptomatic pregnant women without any cardiac or clinical risk factors was 5.2% (n=47). The following CVDs were detected: rheumatic heart disease (n=26; 55.3%), congenital heart disease (n=13; 27.6%), and isolated valve disease (moderate and severe) (n=8; 17%). The mitral valve prolapse was detected in 32 women (3.5%) and atrial septal aneurysm in 51 women (5.67%). Thirty women (3.3%) had arrhythmia.
Conclusion: The CVD rate of 5.2% among healthy women in the first trimester of pregnancy shows that the clinicians must keep in mind that during pregnancy, physiological changes in the cardiovascular system may aggravate an undiagnosed disease, and they should be alert even in case of mild cardiac symptoms that may interfere with pregnancy complaints.

Objective: Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of pulmonary hypertension (PH). We aimed to investigate the outcome of CTEPH patients who were followed-up by a PH outpatient clinic.
Methods: We screened the medical records of 29 PH patients who were followed-up by a PH outpatient clinic between 2009 and 2018. The patients’ demographics and their clinical, echocardiographic, and hemodynamic characteristics were recorded.
Results: Our study group consisted of 16 females (55.2%) and 13 males (44.8%). The mean age was 59.5±13.7 years and the median follow-up duration was 44 months (1-113 months). The mean initial 6-minute walking distance (6MWD) was 321.4±119.9 m. The initial median N-terminal pro brain natriuretic peptide (NT-proBNP) level was 2468 pg/mL (46.1-20.564 pg/mL). All patients were on oral anticoagulant therapy. Pulmonary endarterectomy (PEA) was performed in 17 of 29 patients (58.6%). Twelve patients (41.4%) were not operated upon due to distal disease, comorbidities, or their own preference. The operated patients were younger than the non-operated patients (55 years & 65 years, p=0.04). At the follow-up, the 6MWD in the operated patients increased (+76 m) and decreased in non-operated patients (-46 m). The survival rate at 10-year follow-up was 58.6% for the whole group. Twelve patients died during the follow-up period. While 7 of 12 not-operated patients died (58.3%), just 5 of 17 operated patients (4 perioperatively and 1 at follow-up) died (29%). Advanced-stage final functional capacity (FC) [New York Heart Association (NYHA) III-IV], inoperability, lower final 6MWD, higher final NT-proBNP, and reduced tricuspid annular plane systolic excursion (TAPSE) were associated with an increased mortality rate. Univariate Cox regression analysis showed that patients with NYHA I-II final FC showed a 166-fold decreased mortality rate.
Conclusion: The long-term prognosis of operated patients is better than the outcome of not-operated patients. The strongest predictor associated with mortality was a worse final FC (NYHA III-IV).