Claudiu Ghiragosian

Background: Totally endoscopic techniques have become increasingly popular in cardiac surgery, with minimally invasive mitral valve repair emerging as an effective alternative to median sternotomy. This approach could be particularly advantageous for patients with Noonan syndrome, who often present with structural thoracic anomalies and other comorbidities like bleeding disorders. Endoscopic mitral valve surgery is rapidly establishing itself as the new standard of care for mitral valve operations, demonstrating both safety and efficacy. Noonan syndrome is an autosomal-dominant multisystem disorder with variable expression and is the second most common syndromic cause of congenital heart disease, surpassed only by Down syndrome. A wide spectrum of cardiovascular phenotypes is associated with Noonan syndrome, including pulmonary valve stenosis (often with dysplastic valves), hypertrophic cardiomyopathy, secundum atrial septal defect and mitral valve abnormalities. Methods: Given the limited data in the literature regarding the experience of other centers with endoscopic mitral surgery in patients with this condition, we aim to present the case of a 46-year-old male with a known diagnosis of Noonan syndrome who presented to a cardiologist with a 6-month history of dyspnea and fatigue. Transthoracic echocardiography revealed severe mitral regurgitation. Following multidisciplinary discussions within the Heart Team and after obtaining informed consent from the patient and his family, the decision was made to proceed with totally endoscopic mitral valve repair. Results: The patient experienced an uneventful postoperative course and was discharged 8 days after the procedure. In this case, endoscopic surgery was essential for successfully repairing the mitral valve. Structural abnormalities, such as chest wall deformities causing heart malrotation and atypical positioning, significantly impaired visualization. Conclusions: The endoscopic approach provided superior access to the mitral valve, enabling precise and effective repair. Additionally, it offered benefits such as improved esthetic outcomes, faster recovery, and a reduced risk of exacerbating thoracic deformities due to improper sternal bone healing.

Background/Objectives: Bicuspid aortic valve (BAV) is a prevalent congenital heart defect that continues to present a significant challenge in the management of paediatric patients. The assessment of left ventricle systolic function is typically conducted through the measurement of the left ventricular ejection fraction. Currently, left ventricle global longitudinal strain (LV GLS) is regarded as a more sensitive indicator, enabling the quantitative assessment of global and segmental ventricular function through the determination of myocardial deformation. Methods: A prospective study was conducted between 10 January 2023 and 10 January 2024 in a tertiary paediatric cardiology referral centre. The study enrolled children aged 6 to 17 years with BAV who were undergoing periodic evaluation, as well as a control group. The primary objective was to analyse the systolic function (global and segmental LV) using the classical method (LV EF) and speckle tracking echocardiography (STE). Results: The study group comprised 73 patients with a mean age of 13 years and was predominantly male. The control group comprised 55 patients. The phenotype IB with aortic regurgitation (AR) was the most prevalent. The results of the STE evaluation in the control group demonstrated mean GLS values between −22.1% and −22.8%. A comparison of the BAV group and the control group revealed a significant difference in GLS for the apical four-chamber view (p = 0.022). Conclusions: Although the analysis of global LV function demonstrated normal values of EF in patients with BAV, the strain analysis revealed significantly reduced strain in the inferior segment and in the apical four-chamber view, as well as in the anterior segment. Further investigation is required to determine whether reduced LV GLS in paediatric patients with BAV will ultimately result in the development of clinical heart failure. Additionally, it is necessary to ascertain whether this can identify patients with subclinical heart failure and whether early detection can result in a reduction in morbidity.

Background/Objectives: Bicuspid aortic valve (BAV) is a prevalent congenital heart defect that continues to present a significant challenge in the management of paediatric patients. The assessment of left ventricle systolic function is typically conducted through the measurement of the left ventricular ejection fraction. Currently, left ventricle global longitudinal strain (LV GLS) is regarded as a more sensitive indicator, enabling the quantitative assessment of global and segmental ventricular function through the determination of myocardial deformation. Methods: A prospective study was conducted between 10 January 2023 and 10 January 2024 in a tertiary paediatric cardiology referral centre. The study enrolled children aged 6 to 17 years with BAV who were undergoing periodic evaluation, as well as a control group. The primary objective is to analyse the systolic function (global and segmental LV) using the classical method (LV EF) and speckle tracking echocardiography (STE). Results: The study group comprised 73 patients with a mean age of 13 years, with a male predominance. The control group comprised 55 patients. The phenotype IB with aortic regurgitation (AR) was the most prevalent. The results of the STE evaluation in the control group demonstrate mean GLS values between -22.1% and -22.8%. A comparison of the BAV group and the control group revealed a significant difference in GLS for the apical four-chamber view (p = 0.022).

Background: This study focuses on two of the most prevalent congenital heart defects: bicuspid aortic valve (BAV) and coarctation of the aorta (CoA). In severe forms, CoA is an emergency that can become life-threatening. Both diseases are linked to aortic vasculopathy, which represents a significant contributor to morbidity and mortality in young patients with congenital heart disease. The aim of this study was to investigate whether there are differences between patients with CoA and BAV, and patients with CoA and without BAV in terms of outcome and echocardiographic parameters, and to evaluate the efficacy of different echocardiographic parameters in predicting CoA. Methods: A retrospective and prospective observational analytic study was conducted between January 2018 and October 2024 at a tertiary pediatric cardiology referral center enrolling patients aged between 0 and 17 years, diagnosed with CoA, who underwent surgical CoA repair. The common carotid-subclavian artery index (CSAI) and the aortic isthmus-descending aorta index (I/D) were calculated based on echocardiographic measurements. Results: A total of 101 patients underwent surgical or interventional treatment for CoA in the studied period, 58.4% being neonates. Based on echocardiographic examination, 68.3% of patients had BAV with CoA. The mean value of the carotid-subclavian index was 0.95 in neonates, compared to 1.11 in children (p = 0.035). No statistically significant difference was observed in the mean value of the aortic isthmus-descending aorta index between the two groups (p = 0.237). Conclusions: The implications of the coexistence of the two conditions are not yet fully understood. This study did not find significant echocardiographic differences between patients with CoA and BAV and patients with CoA and without BAV. Further long-term, multicenter, multi-arm studies with larger groups are required in these areas.

The race for an ideal cardioplegic solution has remained enthusiastic since the beginning of the modern cardiac surgery era. The Bretschneider solution, belonging to the “intracellular cardioplegic” group, is safe and practical in myocardial protection during ischemic time. Over time, some particular concerns have arisen regarding the effects on cardiac metabolism and postoperative myocardial functioning. This paper reviews the most important standpoints in terms of theoretical and practical analyses.