Articles

Introduction: Klippel-Feil syndrome (KFS), is a bone disorder characterized by the abnormal joining (fusion) of two or more spinal bones in the neck (cervical vertebrae), which is present from birth. Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. In some individuals, KFS can be associated with a variety of additional symptoms and physical abnormalities which contribute in the deterioration and complication of the condition of the child. Aim of presentation: Here, we report five children from Kosovo with KFS associated with different heart abnormalities, clinical presentation, diagnosis, management, and outcomes of selected conditions in resources-limited settings. Methods: Retrospectively we analysed medical reports of five children, diagnosed at different age with congenital disease and clinical and lab signs of Klippel-Feil syndrome. Conclusion: Basing on our cases, all diagnosed in a small country as a Kosovo, we can conclude that KFS is not such a rare condition. In addition, such syndrome is not so rarely associated with different congenital heart disease. In four cases cardiac surgery was indicated and successfully was done abroad Kosovo in the lack of such services in Kosovo.

Background: Current guidelines for diagnosis and management of heart failure (HF) rely on clinical findings and natriuretic peptide values, but evidence suggests that recently identified cardiac biomarkers may aid in early detection of HF and improve risk stratification. The aim of this study was to assess the diagnostic and prognostic utility of multiple biomarkers in patients with HF and left ventricular systolic dysfunction (LVSD). Methods: High-sensitivity cardiac troponin I (cTnI), N-terminal pro b-type natriuretic peptide (NT-proBNP), interleukin-6 (IL-6), endothelin-1 (ET-1), pro-matrix metalloproteinase-9 (pMMP-9), and tumor necrosis factor-alpha (TNF-α) were measured using single-molecule counting technology in 200 patients with varying stages of HF. Plasma detection with cross-sectional associations of biomarkers across all HF stages, and advanced-therapy and transplant-free survival were assessed using multivariate analysis and Cox regression analyses, respectively. Results: NTproBNP, pMMP-9, IL-6 were elevated in early, asymptomatic stages of HF, and increased with HF severity. Higher circulating levels of combined IL-6, NTproBNP, and cTnI predicted significantly worse survival at 1500-day follow-up. Cox regression analysis adjusted for ACC/AHA HF stages demonstrated that a higher concentration of IL-6 and cTnI conferred greater risks in terms of time to death, implantation of left ventricular assist device (LVAD), or heart transplantation. Conclusion: Biomarkers of inflammation, LV remodeling, and myocardial injury were elevated in HF and increased with HF severity. Patients had a significantly higher risk of serious cardiac events if multiple biomarkers were elevated. These findings support measuring NTproBNP, cTnI and IL-6 among patients with HF and LVSD for diagnostic and prognostic purposes.

Blunt chest trauma leads to a wide range of lesions, relatively minor parietal injuries to potentially fatal cardiac lesions, making diagnosis and management difficult. The diagnosis is currently facilitated by imaging, however, these lesions may go unnoticed and be discovered late through complications. We report the case of a neglected heart wound revealed by a heart failure. This case is notable due to a favourable outcome despite a delay in diagnosis due to a lack of pericardial effusion and the absence of cardiac symptoms, and a long delay from injury to appropriate treatment in the presence of a penetrating cardiac wound deep enough to cause a muscular ventricular septal defect and lacerate the anterior mitral leaflet.

Background: Early diagnosis and improved facilities are necessary for determining the optimal timing of surgery and other interventions in children with congenital heart diseases in Nigeria. This is because late presentation, late diagnosis and delayed surgery can lead to mortality and affect the quality of life among these children. Objectives: This review article is aimed at enumerating the timing of cardiac surgeries and other interventions and to seek if there is any factor associated with the timing of cardiac surgery. Methods: A search on PubMed database, World Health Organization libraries, Google scholar, TRIP database, and reference lists of selected articles on timing of cardiac surgery in children was done. The Cochrane Database of Systematic Reviews was also searched. We noted few data from African setting. Key words such as timing of cardiac surgery; children, congenital heart defect were used. Conclusion: Appropriate timing for cardiac surgery in children with congenital heart disease is very important as late surgical intervention could result in several morbidities and mortality.

Background: Growing evidence has revealed that fear and anxiety related situations could affect cardiac parameters. P wave dispersion (Pd) is an important index. In the present study, we aimed to evaluate Pd values in patients with premenstrual dysphoric disorder. Methods: The study was composed of twenty-five female patients with premenstrual dysphoric disorder and same number of healthy controls. Pd, Pmin and Pmax values were determined by electrocardiogram (ECG) in the subjects. Results: It was found that patients with premenstrual dysphoric disorder had considerably higher Pmax and Pmin values compared to those of healthy subjects. Pd was also significantly higher in patients with premenstrual dysphoric disorders than that of healthy subjects. Conclusion: Study suggests that patients with premenstrual dysphoric disorder seems to have increased Pd, as can be seen in anxiety and fear related clinical conditions, considering that this group of patients have an increased trend to cardiac abnormalities, particularly cardiac arrythmias. To access strong conclusion, it is required novel studies with larger samples.

Background: The prognostic significance of impaired left ventricular (LV) relaxation and increased LV stiffness as precursor of heart failure with preserved ejection fraction and death is still largely unknown in apparently healthy subjects. Methods: We constituted a cohort of 353 patients with normal ejection fraction (>45%) and no significant heart disease, based on a total of 3,575 consecutive left-sided heart catheterizations performed. We measured peak negative first derivative of LV pressure (-dP/dt) and operating chamber stiffness (Κ) using a validated equation. Patients were categorized as having: 1) normal diastolic function, 2) isolated relaxation abnormalities (-dP/dt > 1860mm Hg/sec and K <0.025mm Hg/ml), or 3) predominant stiff heart (K ≥0.025mm Hg/ml). Results: During a follow-up of at least 5 years, the incidence of the primary composite endpoint (death, major arterial event, heart failure, and arrhythmia) was 23.2% (82 patients). Compared to isolated relaxation abnormalities, predominant stiff heart showed stronger prognostic significance for all events (p=0.002), namely heart failure (HR, 2.9; p=0.0499), cardiac death (HR, 5.8; p=0.03), and heart failure and cardiac death combined (HR, 3.7; p=0.003). Conclusion: In this apparently healthy population referred to our center for cardiac catheterization, the prevalence of diastolic dysfunction was very high. Moreover, predominant stiff heart was a better predictor of cardiovascular outcomes than isolated relaxation abnormalities.

We report a challenging patient with Williams syndrome and severe coarctation of the aorta. As in a few similar cases reported, several surgical and catheter interventions for recoarctation, intrastent intimal proliferation and stenosis of the left sub-clavian artery were required. Aortic patch angioplasty is planned for the future in a grower child.

Background: There is an important shortage of blood in the greatest blood banks worldwide to meet up with requirements for numerous medical interventions. Limited studies have associated regular blood donation to the lowering of lipid function parameters. Assessing the lipid function is a classical method of evaluating an individual’s risk for coronary heart disease. Objective: The general goal of the study is to determine lipid and hematological profile among blood donors in European Gaza Hospital, Palestine. Materials and Methods: This study was a case-control study that involved 120 male, 40 of whom were regular blood donors (study group), 40 first time donors and 40 non- donors (control group) aged between 18-60 years. A volume of 5ml venous blood was drawn from each fasting participant into a dry biochemistry screw-capped tube. This was allowed to clot and the serum was used to determine total cholesterol (TC), triglycerides, High-density lipoprotein cholesterol (HDL-C), Low-density lipoprotein cholesterol (HDL-C), while HDL-C/LDL-C and TC/LDL ratio were calculated by using the following formula. Anthropometric parameters (weight, height) of donors were measured using standard protocol. The height (in meter), weight (in kilogram) were used to calculate the body mass index (BMI) using the following formula. BMI= weight (kg)/ (height in meter)² and blood was collected from each participant in EDTA (for hematocrit, ESR). Three groups were matched for age and BMI. Data were analyzed using SPSS version 23. Chi-square (χ²) was used to compare the relationship between categorical variables, ANOVA was used to measure the difference between means. Data were summarized using tables, pie charts, histograms. A P-value < 0.05 was considered to be statistically significant for all tests conducted. Results: The mean total cholesterol (169±10.85 mg/dl), triglycerides (116±9.73 mg/dl), HDL (54±2.5 mg/dl ), LDL (92±11.4mg/dl), LDL/HDL ratio (1.73±0.25) and TC/HDL ratio (3.16±0.26) were lower in the regular blood donors than the first time donors(198±10.13, 179±5.82, 42.33±1.6, 120±11.2, 2.85±0.36, 4.7±0.40) and non- donors (202±10.19, 180±12.68, 41.75±1.4, 125±11.7, 2.99±0.33, 4.86±0.32) respectively and statistically significant (P < 0.05).The mean ESR (6.63±0.87mm/hr) was lower statistically significant in the regular blood donors than the first time donors (7.40±1.17) and non- donors (7.60±1.48) respectively (P < 0.05). The mean HCT (42.98±0.86%) was lower statistically significant in the regular blood donors than the first time donors (44.63±0.90) and non- donors (44.75±0.74, P < 0.05). Conclusion: Regular donors have reduced risk of developing coronary heart disease as reflected by the low total cholesterol, triglycerides, LDL-c, LDL-c/HDL-c ratio, TC/HDL-c ratio and HCT and high HDL. BMI in regular donor was less than the donor for the first time and did not donate, but did not reach the statistical significance. Also in our study regular donors have reduced risk of developing inflammation as reflected by low ESR.

Background: Persistent left atrial thrombus remains a contra indication to transeptal valves in valves procedure. We aimed to perform a double valves in valves replacement through transapical access with cerebral angiography control during the procedure just after implantation. Our case shows the feasibility of this strategy and the management of right ventricle laceration successfully treated after extra corporeal membrane oxygenation implantation and local hemostasis. We reported a feasibility case report of successful double valves in valves implantation through transapical access with 6 months of clinical and computed tomography follow up.

Cardiomyopathy is a heart muscle disease with structural and functional myocardial abnormalities in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease. However, it has become clear that diverse etiologies and clinical manifestations (e.g. arrhythmogenic right-ventricular cardiomyopathy/dysplasia (ARVC/D), ARVD/C, left-ventricular non-compaction cardiomyopathy (LVNC)) are responsible for the clinical picture of dilated cardiomyopathy (DCM). The American Heart Association (AHA) classification grouped cardiomyopathies into genetic, mixed and acquired forms, while the European Society of Cardiology (ESC) classification proposed the subgrouping of each major type of cardiomyopathy into familial or genetic, and nonfamilial or nongenetic, forms [1-4]. Cardiomyopathies are clinically heterogeneous diseases, and there are differences in sex, age of onset, rate of progression, risk of development of overt heart failure and likelihood of sudden death within each cardiomyopathy subtype [5]. Because of the complex etiology and clinical presentation, the diagnostic spectrum in cardiomyopathies spans the entire range of non-invasive and invasive cardiological examination techniques including genetic analysis. The exact verification of certain cardiomyopathies necessitates additional investigations. So, histological, immunohistological and molecular biological/virological investigations of endomyocardial biopsies are the gold standard to confirm the diagnosis of an inflammatory cardiomyopathy (DCMi) [6-10]. This review focuses on myocarditis and inflammatory cardiomyopathies underlying an immune-mediated process or persistent viral infection.