Background: Evaluation of left ventricular (LV) function in patients with idiopathic premature ventricular contraction (PVC) with preserved LV ejection fraction (LVEF), especially in the subclinical stage, may be of great importance in terms of directing early treatment. Methods: A total of 122 patients, retrospectively recruited, were divided into three groups according to PVC burden: Group 1; 5% ≤ PVC < 10%, Group 2; 10% ≤ PVC < 15%, and Group 3; 15% ≤ PVC. Transthoracic echocardiography (TTE) was performed to evaluate LV parameters. Results: LV-GLS (Global longitudinal strain) was found to be significantly lower in groups 2 and 3 with high PVC burden (18.9% ± 1.4, 17.5% ± 2.1, 16.3% ± 1.3; p < 0.001, respectively). Correlation analysis revealed a positive and significant correlation between PVC burden and deterioration in LV-GLS (r: 0.536; p < 0.001). In addition, PVC burden was found to be an independent predictor of LV-GLS deterioration in multiple linear regression analysis (β: 0.525, p < 0.001). The ROC curve analysis demonstrated that a PVC burden cut-off value of 8.4% was associated with a LV-GLS deterioration greater than −18, with a specificity of 75.4% and a sensitivity of 75.4% (AUC: 0.81 [0.73–0.88] 95% CI; p < 0.001). Conclusions: PVC burden was an independent predictor of deterioration in LV-GLS. The presence of LV-GLS deterioration due to PVC burden emphasizes the necessity for closer patient monitoring, observation of the response to pharmacological treatment, and evaluation of early invasive treatment strategies in selected patient groups.

Pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD) is a severely progressive condition with complex pathogenesis. The aim of this study was to evaluate the contribution of genetic variants to the development of PAH in patients with APAH-CHD. Fifteen children and twenty-seven adults diagnosed with APAH-CHD were enrolled. Targeted next-generation sequencing was performed on PAH-associated genes ( ABCC8 , ACVRL1 , AQP1 , ATP13A3 , BMPR2 , CAV1 , GDF2 , GGCX , EIF2AK4 , ENG , KCNK3 , KDR , KLK1 , SMAD1 , SMAD4 , SMAD9 , SOX17 , TBX4 , TET2 ). A total of 21 distinct variants across 11 different genes were detected in 17 of the 42 patients. ( ABCC8 = 2, ACVRL1 = 1, ATP13A3 = 2, BMPR2 = 4, GGCX = 1, EIF2AK4 = 2, ENG = 1, KDR = 3, SMAD1 = 1, SMAD9 = 1, TET2 = 3). Five of the patients with the mutation were under the age of 18, and 12 patients were adults. The most common CHD in patients with detected variants was VSD. PAH-related genetic variants were not uncommon in APAH-CHD patients. Our study identified 12 novel variants that may help to understand the genetic basis of APAH-CHD. Trial Registration The study has been registered on ClinicalTrials.gov with the identification number NCT05550389.

Background and Objectives: Cachexia is a systemic wasting syndrome associated with poor outcomes in chronic diseases, including heart failure (HF). Although the cachexia index (CXI), which integrates skeletal muscle mass, serum albumin, and the neutrophil-to-lymphocyte ratio, has shown prognostic value in oncology, its clinical significance in HF remains poorly defined. Materials and Methods: This retrospective single-center cohort study evaluated a selected subgroup of adults hospitalized with decompensated heart failure between January 2020 and January 2025 who had undergone abdominal computed tomography within the preceding 6 months, enabling CT-based body composition assessment. Skeletal muscle index was measured at the L3 vertebral level, and CXI was calculated as (SMI × serum albumin)/neutrophil-to-lymphocyte ratio. Patients were followed for all-cause mortality and HF-related rehospitalizations. Results: A total of 127 patients were included (mean age 70.45 ± 12.73 years; 51.2% male). CXI showed excellent discrimination for mortality (AUC 0.951; 95% CI 0.905–0.996), with an optimal cut-off value of <20.87. Patients with low CXI had significantly higher all-cause mortality (80.5% vs. 4.7%, p < 0.001) and more HF-related hospitalizations [4 (3–5) vs. 0.5 (0–1), p < 0.001] than those with high CXI. Conclusions: In patients hospitalized with decompensated HF, low CXI was strongly associated with all-cause mortality and recurrent hospitalization, suggesting that CXI may serve as an integrative prognostic marker in this population.

Background: Cardiac amyloidosis is more common than previously thought with an incidence of up to 15% in aortic stenosis and heart failure with preserved ejection fraction. Pacemaker need in these patients ranges from 9.5% to 20%; however, its prevalence and clinical relevance in patients with unexplained cardiac conduction defects remain unclear. Methods: This retrospective, single-center cohort study evaluated 1107 patients who underwent intracardiac device implantation for unexplained cardiac conduction defects between 2015 and 2024. Patients with secondary conduction defects or known cardiomyopathy were excluded. The prognostic value of the T-AMYLO score and associated red flag findings were assessed in relation to the composite primary endpoint: all-cause mortality, non-fatal myocardial infarction, and non-fatal stroke. Results: Over a median of 58 months for follow-up, 460 patients experienced a primary event, including 346 deaths. Higher event rates were observed in older males, those with atrioventricular block, and patients receiving single-lead ventricular devices. T-AMYLO score and the presence of red flag findings, particularly aortic valve disease, AV block, peripheral neuropathy, low voltages and increased septal thickness were significantly associated with adverse outcomes. Multivariate Cox regression identified elevated T-AMYLO score (HR: 1.06, p = 0.012), aortic valve disease (HR: 1.29, p = 0.016), and AV block (HR: 1.43, p = 0.009) as independent predictors of mortality. Survival analyses confirmed a stepwise decline in prognosis with an increasing T-AMYLO risk group and red flag burden (p < 0.001). Conclusion: These findings highlight the importance of incorporating T-AMYLO scoring and red flags assessment in patients with conduction defects to improve early detection of cardiac amyloidosis and guide risk stratification for outcomes.

Background/Objectives: Patients receiving a cardiac implantable electronic device (CIED) for conduction disease may remain at substantial long-term risk despite preserved systolic function at implantation. The prognostic association of sodium–glucose cotransporter-2 (SGLT2) inhibitor use in this population is uncertain. We therefore examined the relationship between early documented SGLT2 inhibitor use and subsequent all-cause mortality in patients with preserved left ventricular ejection fraction (LVEF) undergoing CIED implantation for conduction disease. Methods: In this retrospective single-center cohort study, we screened consecutive adults who underwent CIED implantation for conduction disease between January 2018 and June 2024. Patients with LVEF ≥ 50% and complete clinical, laboratory, electrocardiographic, and echocardiographic data were included. SGLT2 inhibitor exposure was defined as documented initiation or active use within 3 months after implantation. The primary endpoint was all-cause mortality. Associations were evaluated using multivariable Cox proportional-hazards regression and Kaplan–Meier analyses. Results: Among 2176 screened patients, 540 fulfilled the eligibility criteria, of whom 206 (38.1%) had documented SGLT2 inhibitor exposure. During a median observation period of 64.2 months (range, 7–90 months), 185 deaths occurred. SGLT2 inhibitor exposure was associated with a lower adjusted hazard of death (HR, 0.48; 95% CI, 0.38–0.69; p < 0.001). Survival also differed between exposure groups in the overall cohort (log-rank p = 0.006) and in patients without either heart failure or diabetes mellitus (log-rank p = 0.020). Conclusions: In patients with preserved LVEF undergoing CIED implantation for conduction disease, documented SGLT2 inhibitor use was independently associated with lower all-cause mortality. These observational findings are hypothesis-generating and warrant confirmation in prospective studies.

Pulmonary hypertension (PH) is a major health problem with increasing awareness. Although most common cause of PH is left heart disease (Group 2 PH), life-threatening complications occur mostly in Group 1 (pulmonary arterial hypertension) and Group 4 (chronic thromboembolic PH) patients. Although external compression of the left main coronary artery (LMCA) due to pulmonary artery dilatation is rare, it is a life-threatening complication since it causes myocardial ischemia and sudden cardiac death. In addition, PH is more than a single clinical entity due to its complex mechanism in which more than one subgroup may develop over time in the same patient. This complex mechanism challenges us when diagnosing the patient and faces us with life-threatening complications. In this case; we report a pulmonary arterial hypertension patient applied to our clinic with progressive dyspnea and recent angina, after detection of LMCA ostial stenosis, the patient was treated with intravascular ultrasound -guided stent implantation. In the further follow-ups, the patient underwent the pulmonary endarterectomy operation due to the diagnosis of chronic thromboembolic pulmonary hypertension secondary to newly diagnosed primary antiphospholipid syndrome.