Objective: We investigated the frequency of different bicuspid aortic valve disease (BAV) phenotypes,the associated valvular pathologies, and the aortopathy phenotypes, using 2-dimensional (2D) transthoracic, 2D transesophageal echocardiography (TEE) and 3-dimensional (3D) TEE.
Methods: A total of 154 patients with BAV were included. Five BAV phenotypes were detected. To better define valvular pathologies, binary classifications of BAV were used: BAV with antero-posterior commisural line (BAV-AP) and right-left commissural line (BAV-RL). Aortopathy phenotype was classified according to the involved tract(s).
Results: Of the patients, 53.2% had type 1, 16.2% type 2, 15.6% type 3, 1.3% type 4, and 13.6% had type 5 BAV. The prevalence of BAV-AP and BAV-RL was 68.2% and 31.8%, respectively. No difference was detected with respect to aortic regurgitation between BAV-AP and BAV-RL (p=0.9), but the BAV-RL group had an increased propensity to have a stenotic aortic valve (p=0.003). The indexed aortic diameter was larger in BAV-AP cases than BAV-RL at the sinus of Valsalva (p=0.008). In patients with dilatation of the root and tubular portion, a predominance of BAV-AP versus BAV-RL was observed (85% vs 15%). A markedly low prevalence of the root phenotype (3.2%) was observed. In 90.1% of the patients, 2D TEE was sufficient to classify BAV phenotypes; further 3D imaging was needed in 9.9% of the cases.
Conclusion: There may be racial differences in the frequency of valvular and aortopathy phenotypes in patients with BAV. BAV phenotypes differ with respect to aortic stenosis and aortopathy phenotypes. TEE may have good diagnostic utility in differentiating BAV phenotypes.

Objective: The pathophysiological mechanism of in-stent restenosis (ISR) is different from atherosclerosis of native coronary arteries. The aim of this study was to evaluate the relationship between ISR and the extent of coronary artery disease (CAD), and to identify other risk factors associated with ISR in ST-segment elevation myocardial infarction (STEMI) patients.
Methods: A total of 372 consecutive patients presenting with first acute STEMI who were successfully treated with primary percutaneous coronary intervention within 12 hours from the onset of symptoms and who had an angiographic follow-up at 3 months were included in the study. The extent of CAD was calculated using the Gensini score.
Results: The incidence of ISR observed in our group of patients was 23.4% (n=87). The mean Gensini score was significantly higher in patients with ISR when compared with group without restenosis (69 [range: 51–90] vs 42 [range: 32–61]; p<0.001). The presence of diabetes mellitus, left ventricular ejection fraction (LVEF), and low-density lipoprotein cholesterol (LDL-C) level differed significantly between the 2 groups (p<0.05 for all). Stent diameter and stent length were found to be significantly different between the ISR group and the no-restenosis group (p<0.05 for both). In multivariate logistic regression analysis, the Gensini score, stent diameter, stent length, LVEF, and LDL-C were independently associated with ISR.
Conclusion: Despite the differences in the underlying pathophysiological mechanism of ISR and native coronary atherosclerosis, patients with a greater extent of CAD should be considered candidates for future stent restenosis.

Objective: Right ventricular (RV) functions are clinically important in acute pulmonary embolism (APE). Measurement of systolic function of the right ventricular outflow tract (RVOT) with echocardiography is a simple method to evaluate RV function. The aim of this study was to determine the relationship between RVOT systolic function and the Pulmonary Embolism Severity Index (PESI).
Methods: A total of 151 patients diagnosed with APE by pulmonary computed tomography angiography or ventilation/perfusion scintigraphy were included. Patients were assigned to 2 groups based on the simplified PESI (sPESI): sPESI <1 (n=85) and sPESI ≥1 (n=66). RV conventional parameters, RVOT dimensions, and fractional shortening (RVOT-FS) were also measured.
Results: Mean age was similar between sPESI <1 and >1 patients (58.7±12.9 years vs. 61.1±12.7 years, respectively). Frequency of male gender was significantly higher in PESI <1 group (61.2% vs. 40.2%, p=0.013). No significant differences were found between the groups in fasting glucose, serum creatinine, hemoglobin, C-reactive protein, erythrocyte sedimentation rate, troponin, and D-dimer levels, and left ventricular ejection fraction. RVOT-FS was higher in patients with sPESI <1 than in patients with sPESI ≥1 (34.41±3.56 vs. 22.98±4.22), and this difference was significant (p<0.001). Tricuspid annular plane systolic excursion values were lower and pulmonary artery systolic pressure values were higher in the sPESI ≥1 group, which was also statistically significant (p<0.05). Mortality occurred in 7 patients with sPESI <1 and in 16 patients with sPESI ≥1. The mortality rate was higher in patients with lower RVOT-FS, and a RVOT-FS <0.22 predicted mortality with a sensitivity of 54.5% (AUC: 0.674, 95% CI 0.552-0.796; p=0.009).
Conclusion: The RVOT-FS is a noninvasive measurement of RV systolic function, is well-correlated with the sPESI score, and associated with mortality in patients with APE. This easily applied measurement may be used to predict short-term mortality in patients with APE.

Objective: Sudden cardiac death in athletes is one of the most tragic health events seen both in our country and all over the world. In some of those athletes, there is no obvious structural abnormality. Dynamic changes in intracardiac hemodynamics during exercise may be a cause for sudden death in these athletes, the impact of exercise on intracardiac gradient and cardiac hemodynamic parameters in athletes was compared with healthy controls.
Methods: A total of 21 professional male athletes and 21 healthy male controls were included in the study. Transthoracic echocardiography was performed in all participants both at rest and maximal exercise level to assess the intraventricular gradient (IVG) and cardiac systolic and diastolic functions. Abnormal IVG was defined as gradient of >30 mm Hg at peak exercise level.
Results: Both groups reached the level of predicted maximum exercise. There was no exercise limiting symptom among participants during exercise test. The athletes revealed a higher maximum peak systolic IVG at baseline and after exercise in comparison with the control group. None of the participants showed an abnormal IVG level.
Conclusion: Our results showed that there was no dynamic intraventricular obstruction with aerobic exercise in subjects with a structurally normal heart.

Objective: Lifelong statin treatment is recommended in patients with cardiovascular diseases, but drug adherence is a significant problem. The aim of this study was to investigate factors related to statin discontinuation in high-risk patients with coronary artery disease (CAD) diagnosed by coronary angiography.
Methods: A total of 300 consecutive patients who were followed-up with a diagnosis of CAD were recruited. Patients were categorized as statin adherent or statin non-adherent (patients interrupting statin therapy >30 days). Study participants completed a questionnaire regarding demographic characteristics, medical history, knowledge of statin treatment, and factors related to statin discontinuation.
Results: In all, 160 patients (53.3%) were found to be statin adherent. Of those, 122 patients had suffered myocardial infarction, and atorvastatin was the most prescribed statin on discharge. Among the study population, 26% were illiterate and 55% had graduated from primary school. Only 39 cases (13%) cases had a low-density lipoprotein-cholesterol level <70 mg/dL. In 60% of the statin non-adherent patients, the reason for statin discontinuation was physician discontinuation of the statin prescription. In 14%, the patient stopped the therapy after cholesterol parameters had been reduced or reached the normal range. Only 8% of participants reported that negative information received from TV programs and social media was responsible for the decision to terminate drug use.
Conclusion: Our findings demonstrated that the importance of intensive statin treatment in CAD patients has not been recognized by patients or many physicians. Adherence to statin treatment and success of the therapy is low, leading to unnecessarily high cholesterol levels in patients with CAD.

Objective: Familial hypercholesterolemia (FH) is a life-threatening genetic disease associated with elevated low-density lipoprotein cholesterol (LDL-C) and premature coronary heart disease that is undiagnosed and undertreated around the world. This study aimed to examine the demographic characteristics, awareness, and treatment adherence of undiagnosed or undertreated FH patients based on laboratory records.
Methods: In a 16-month retrospective survey using laboratory records, patients with elevated LDL-C (>250 mg/dL) were identified (n=395). Patients younger than 18 years of age or with secondary causes of dyslipidemia were excluded (n=98). In all, 297 patients were called and asked to participate in a phone interview regarding their demographic characteristics, awareness of dyslipidemia, and treatment adherence.
Results: A total of 147 patients (mean age: 51.7±16.6 years; 59.2% female) completed the interview. The mean LDL-C level of the patients was 292.8±49.9 mg/dL. According to the Dutch Lipid Clinic Network criteria, 18.4% of the patients had definite FH, 66.0% had probable FH, and 15.6% had possible FH. Although the majority of the patients (93.9%) were aware of their high LDL-C level, only about half of them (n=75; 51.0%) were in treatment. Of all the patients who were interviewed, 21% (n=31) had never taken medication to lower their LDL-C, and 28% (n=41) had stopped taking a lipid-lowering drug.
Conclusion: This pilot study revealed that a significant number of FH patients were not taking statins despite having a very high LDL-C level. Nationwide detection of likely FH patients using hospital records and interviewing them via a phone survey may help to better understand and manage these high-risk patients.

Coronary artery perforation (CAP) is a rare, but potentially mortal possible complication of percutaneous coronary intervention. There are several treatment options for this complication, including prolonged balloon dilatation, use of a coronary stent graft, and bypass surgery. In this case report, a 65-year-old female patient who was admitted to the catheter laboratory with a diagnosis of acute coronary syndrome, was presented. Coronary angiography revealed total occlusion in the mid segment of the right coronary artery and a drug-eluting stent was implanted under 12 atm of pressure following pre-dilatation with a perfusion balloon. In order to perform defragmentation of the thrombus shifted into the proximal stent segment, post-dilatation was performed with a stent balloon (4–6 atm). After post-dilatation, an Ellis Class II perforation developed. In order to control the bleeding, a coronary stent graft was implanted at the perforation area. The rupture was sealed. Control coronary angiography 40 days later indicated that the stent graft was patent, but an arteriovenous fistula (AVF) draining to the right ventricle was detected. To the best of our knowledge, this is the first case of AVF seen as a late complication of CAP treated with a stent graft.

A 51-year-old male patient presented with frequent and symptomatic premature ventricular complexes (PVCs) that had induced cardiomyopathy. Radiofrequency (RF) catheter ablation of the origin of the PVCs was planned and the patient underwent electrophysiological study. During activation mapping, the earliest ventricular activation was identified at the His bundle region. Small His bundle electrogram recording by the distal ablation electrode during sinus rhythm revealed that the earliest ventricular activation during PVC preceded the QRS onset by 58 milliseconds. The PVCs were successfully eliminated without occurrence of atrioventricular block using incremental application of RF energy to the para-Hisian region. At the conclusion of a 5-month follow-up period, the patient remained asymptomatic and transthoracic echocardiography demonstrated an improvement in left ventricular ejection fraction.

Defibrillation threshold (DFT) testing is an important part of ICD implantation. After placement of the ICD generator, a DFT test is performed to evaluate the integrity of the ICD system and to confirm a successful defibrillation safety margin. More than 6% of ICDs implanted are not within the DFT safety margin. Presently described is the case of a patient with a high DFT and some of the methods that can be used to manage this circumstance, including the use of a subcutaneous array.

An unconscious, 25-year-old, male patient was brought to the emergency department. The patient’s electrocardiography demonstrated a wide QRS interval and first-degree atrioventricular block. He was being treated with propafenone twice daily (450 mg) for paroxysmal atrial fibrillation. The patient had consumed a substantial amount of alcohol the day before. He recovered after supportive management with sodium bicarbonate and inotropic therapy. In the presently described case, treatment resulted in quick normalization of QRS interval and stabilization of hemodynamic status.

Primary cardiac lymphoma (PCL) is one of the rarest tumors of the heart. The most common type is diffuse, large B-cell lymphoma. Most often, the right atrium and the right ventricle are involved, and if not diagnosed and treated in time, it can be fatal. In this case, a female patient underwent an urgent operation for a large, infiltrative, right atrial mass. Extensive resection of the lateral walls of both atria and the interatrial septum as well as reconstruction were performed successfully. The pathological evaluation suggested PCL. The aim of this case is to raise awareness of this disease and to highlight clinical and surgical approaches.

Williams syndrome is a rare neurodevelopmental disorder characterized by mental retardation, growth deficiency, hypercalcemia, cardiac defects, and a distinctive facial appearance. Cardiovascular abnormalities are present in approximately 80% of Williams syndrome patients. Surgical treatment is generally performed for supravalvular aortic stenosis, aortic coarctation, pulmonary artery stenosis, or ventricular septal defect. In rare cases, diffuse hypoplasia of the aortic arch with a normal left ventricular outflow tract and ascending aorta may be diagnosed in early childhood. Described herein is the case of a 16-month-old female with Williams syndrome and diffuse hypoplasia of the aortic arch and isthmus, and concomitant pulmonary stenosis and a ventricular septal defect. The patient underwent a successful surgical repair of the aortic arch with a modified pericardial patch technique.