Objective: Left ventricular non-compaction is a rare cardiomyopathy following an early arrest in endomyocardial morphogenesis. This study aimed to present the clinical and electrocardiographic characteristics, diagnostic features, treatment strategies, effects of systolic dysfunction on clinical and diagnostic parameters, and follow-up of pediatric patients diagnosed with left ventricular non-compaction.
Methods: We retrospectively reviewed children with isolated left ventricular non-compaction at Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital from January 2010 to June 2020.
Results: Fifty-five children were diagnosed with left ventricular non-compaction. Thirty-two patients (58.2%) were male, and the median age of presentation was 8.5 years (1 month-17.9 years). The median follow-up of the study was 19 months (1-121 months). Fourteen (25.5%) presented with systolic dysfunction (ejection fraction < 45%), and 2 presented with resuscitated/aborted cardiac arrest. Electrocardiographic abnormalities were present in 78.2%. Fragmented QRS was observed in 6 patients, and QTc duration was 450 milliseconds and above in 17 patients (30.9%). Electrocardiographic abnormalities, low QRS voltage, fragmented QRS, and thrombus were common in patients with ejection fraction < 45% group. Atrial and ventricular arrhythmias (including ventricular fibrillation-VF) were found with similar frequency in both ejection fraction < 45% and ≥45% groups. One patient with a complete atrioventricular block and 1 with long QT syndrome and severe bradycardia underwent permanent pacemaker implantation. Five (9.1%) patients died.
Conclusions: Left ventricular non-compaction has heterogeneous clinical findings in childhood. It is essential to follow-up with the patients closely for the development of ventricular dysfunction or arrhythmias due to the progressive course of the disease. Further studies are needed since life-threatening ventricular arrhythmias can be seen, even in patients with preserved ejection fraction.
Fractional flow reserve derived from computed tomography decreases across severe coronary stenosis. The diagnostic accuracy of fractional flow reserve-derived computed tomography is high for severe coronary stenosis. In this report, we present a case of no significant fractional flow reserve-derived computed tomography changes even in severe coronary stenosis. A 75-year-old man showed severe stenosis (85% diameter stenosis) in the distal segment of the right coronary artery on both computed tomography angiography and invasive coronary angiography. However, fractional flow reserve-derived from computed tomography showed no significant changes from the proximal (0.97) to the distal (0.95) segments despite the presence of severe stenotic lesion. This patient had different features including the presence of a large acute marginal branch and significantly lower plaque components in the stenotic lesion compared with another patient who had coronary stenosis in the same segment. A large bifurcation branch and/or proportion of plaque components can affect fractional flow reserve-derived from computed tomography hemodynamics.
Objective: Isolated ostial diagonal stenoses are very rare lesions in which percutaneous intervention could cause significant vessel compromise, and the long-term results have been reported in a few studies. This study sought the characteristics and long-term follow-up of the patients with isolated osteal diagonal stenosis regarding percutaneous coronary intervention and presence of angina.
Methods: The study was an observational retrospective study conducted between January 2014 and December 2020. A total of 9769 patients who underwent coronary angiography were analyzed, and 87 patients had isolated diagonal stenosis. The patients were evaluated according to treatment modality and angina severity in long-term pattern.
Results: Median follow-up time was 36 months. A total of 54 (83.1%) patients were followed up with only medical treatment, and 11 (16.9%) patients underwent revascularization in addition to medical treatment. The degree of stenosis of the diagonal artery was significantly higher in the percutaneous coronary intervention group than medical group (P = 0.002) and the patients with wider reference diameter of diagonal artery complaint of more angina (P = 0.007). Class I angina was significantly higher in percutaneous coronary intervention group than medical and the patients with no angina were significantly higher in medical group than percutaneous coronary intervention group.
Conclusion: Percutaneous coronary intervention was mainly performed for diagonal arteries with a higher degree of stenosis; however, the patients who underwent percutaneous coronary intervention had angina more than 50% rates. Furthermore, the patients with ongoing angina had a larger diameter of the diagonal artery regardless of the type of treatment.
Objective: Beta thalassemia major is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Cardiac involvement is the main cause of death in patients. Speckle-tracking echocardiography is a feasible method for the evaluation of cardiac function via an assessment of the longitudinal deformation of the myocardium through the cardiac cycle. The aim of our study is to evaluate the association between vitamin D deficiency and deformation of the left ventricular myocardium measured by speckle-tracking echocardiography in children with thalassemia major.
Methods: In this prospective study, 33 thalassemic patients with vitamin D deficiency were enrolled. Cardiac magnetic resonance T2* value, conventional echocardiography, and speckle tracking, and also left ventricular longitudinal and circumferential strain values were measured. Myocardial functions of the patients with vitamin D deficiency or insufficiency were evaluated by speckle-tracking echocardiography before and after vitamin D replacement.
Results: The mean age of the patients was 15.4 ± 3.09 years. Vitamin D level was deficient in 30 (90%) and insufficient in 3 (10%) of them. Speckle-tracking analysis showed a significantly decreased absolute value of the left ventricular global longitudinal strain before vitamin D replacement. A significant improvement in the global longitudinal strain was detected after vitamin D replacement (P < 0.05). A statistically significant increase was observed in parameters showing left ventricular systolic and diastolic functions after vitamin D replacement.
Conclusion: Vitamin D deficiency is frequently observed and causes decreased contractility in thalassemic patients. In our study, we observed that our patients' cardiac functions had improved after vitamin D replacement therapy.
Objective: Curcumin is a bioactive compound that has well-known pharmacological activities. Numerous studies have shown that curcumin provides potential cardiovascular benefits through a variety of mechanisms.The present study aims to discuss different concentrations of curcumin's impact on mechanical functions and cardiac contractility in isolated perfused rat hearts.
Methods: The hearts were isolated under sodium thiopental (50 mg/kg) anesthesia and perfused with a modified Krebs-Henseleit solution (mK-Hs). After stabilization, curcumin was applied in concentrations of 0.1, 1, and 10 µM. In isolated rat hearts, indexes of + dP / dt max, LVDP, MAP, and LVEDP were evaluated for cardiac contractility and ventricular function.
Results: All curcumin concentrations reduced +dP/dtmax and LVDP. Ten µM curcumin also significantly decreased heart rate. Curcumin (1 and 10 µM) increased LVEDP and reduced MAP amplitude with a concomitant increase in MAP duration. Curcumin at all concentrations did not affect dMAPdtmax and dMAPdtmin.
Conclusion: Our results might suggest that curcumin at higher concentrations (≥ 1 µM) increases LVEDP with a negative chronotropic effect and decreases MAP amplitude with an increase in MAP duration. There is sufficient evidence from this study that Curcumin possesses an adverse inotropic action. Different disease models should support the pathophysiological role of Curcumin on cardiac contraction.
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