Medical advances have extended the lives of adults with CHD, but transitioning to adult healthcare remains challenging. Identifying factors related to lapses in care is crucial for adult females due to their unique healthcare needs. We examined the relationships among length of lapses in primary care and cardiology services with quality of life and perceived health status for adult females with CHD. A convergent mixed-methods design was used to examine thirty adult females with CHD, aged 21-30. Descriptive statistics and correlational analysis were used to examine perceived health status and quality of life based on lapse of care durations. Thematic analysis was used for qualitative data. A 12-month or greater lapse in care occurred in 46.7% of participants for cardiology and 60% for primary care, and 30.0% lacked an established adult primary care provider. Participants cited healthcare system barriers, provider shortages, and difficult transitions from paediatric providers as contributing factors to lapses in care. Despite lapses, most participants reported similar or better physical health scores than the general population. However, those with either no or prolonged lapse in cardiology care had lower mental health scores. Participants with primary care lapses greater than 6 months reported better quality-of-life scores than those with uninterrupted care. Significant gaps, which contribute to lapses of care, persist in transition care for females with CHD. Findings highlight the need for structured transition planning, improved primary care access, and an integrated primary-specialty care model to improve transition.

Coarctation of the aorta accounts for approximately 6-8% of CHDs, typically manifesting as narrowing of the proximal thoracic aorta. Clinical and haemodynamic effects vary depending on the severity and associated anomalies. We aimed to compare the outcomes of surgical versus percutaneous interventions in patients with coarctation of the aorta and to identify factors influencing the choice of treatment strategy. We retrospectively analysed the medical records of 120 patients diagnosed and treated for coarctation of the aorta at Ankara University Faculty of Medicine, Department of Pediatric Cardiology over a 12-year period. Patients were grouped by age (0-4 months and >4 months). Clinical and echocardiographic data were reviewed. Treatment selection was based on American Heart Association 2011 guidelines, considering age, weight, and anatomy. Surgical repair was preferred in infants <4 months; balloon angioplasty or stent implantation was used in older patients. Procedural success and complications were assessed. A total of 62% were male, the median age at diagnosis was 1.1 months, and 67% were diagnosed before 4 months of age. The most common symptom was a cardiac murmur (62.5%). Initially, balloon angioplasty was performed in 50%, surgical repair in 45%, and stent implantation in 5%. The acute success rate was 98.3%. Complications occurred in 13.3%, including thrombosis (7.5%) and aneurysm (2.5%). Recoarctation developed in 43% and was significantly higher after balloon angioplasty compared to surgery (55% vs. 29.6%, p = 0.004), and in patients <4 months (52.2% vs. 25.5%, p = 0.014). Management strategies typically included surgical repair in infants <4 months, balloon angioplasty in older children, and stenting in those ≥25 kg, while treatment was ultimately individualised. Recoarctation was the most frequent complication, especially among infants under four months and after balloon angioplasty. Surgical repair was associated with a lower risk of recoarctation in early infancy. Percutaneous stent implantation for coarctation is an effective and safe procedure, but it is limited by the patient's weight. Individualised treatment based on age, anatomy, and clinical status is essential to optimise outcomes.

Mutations in genes encoding sarcomeric proteins account for 50-60% of familial hypertrophic cardiomyopathy cases. However, the molecular pathogenesis in approximately one-third of patients remains unidentified. We describe the case of a 15-year-old female who presented with intermittent palpitations and had a significant paternal cardiovascular history. She was diagnosed with hypertrophic cardiomyopathy, confirmed by echocardiogram and cardiac MRI. Genetic testing revealed a variant of unknown significance in exon 7 of the FHL1 gene and exon 43 of the ANK2 gene.

The peer review process is fundamental to academic publishing, guaranteeing the integrity and quality of the research upon which we depend. However, it is also infamous for its sluggishness-occasionally excruciatingly so. For numerous authors, the prolonged wait for feedback on their articles might seem interminable, particularly when they are enthusiastic about disseminating innovative discoveries to the public. But why exactly does peer review take so long? The reasons are complex and multifaceted, involving challenges faced by editors, reviewers, and authors alike. By understanding these challenges, we can start to see the bigger picture and work towards solutions that might speed things up."Patience requires knowing not just the cost of delay, but also the benefit of delay""The two most powerful warriors are patience and time."-Leo Tolstoy"Lost time is never found again." - Benjamin Franklin.

Exercise interventions through cardiac fitness programmes improve aerobic capacity and quality of life in paediatric patients with heart disease. The aim of this study was to characterise the landscape of paediatric cardiac fitness programmes via the Global Coalition for Fitness and Congenital Heart Disease (GloCo), an international network of experts providing exercise-based interventions for children and young adults with congenital and acquired heart disease. A survey was developed and distributed electronically to GloCo members and was completed by 40/53 individuals (response rate 75%), including 23 centres in 6 countries, predominantly US based. Programmes were similar with regard to duration, session frequency, and eligible patient populations but varied with regard to mode of delivery, equipment, incentives, funding sources, and cost. At the time of completion, 14 (61%) centres had enrolled at least 1 patient, and 4 (17%) centres were developing programmes. Respondents felt that cardiac fitness programmes are effective at improving quality of life/wellness (87%), cardiovascular fitness parameters (67%), and neuromuscular strength (47%) and that the most important outcomes were quality of life/wellness, exercise testing parameters, and strength/flexibility assessments. Paediatric cardiac fitness programmes focusing on exercise interventions represent a growing international field with diverse applications. Significant variability in their structure and implementation creates barriers to developing consensus guidelines and achieving standardised care. This study underscores this variability and the need for increased collaboration across centres. Multicentre research is essential to determining the optimal design of these programmes, which in turn will allow for the development of comprehensive, evidence-based guidelines.

The impact of arch anatomy on the prognosis of aortic coarctation of the aorta (COA) is not well established. We aim to assess the relationship between arch anatomy and the short- and long-term prognosis after surgical repair. Patients with COA operated on the period between 11/2007 and 03/2016 were retrospectively recruited. Anatomical analysis of the aortic arch was done using Multidetector CT with measurement of the inter-branch distances between left common carotid artery, innominate artery, and left subclavian. We classified patients into group I, whose distance ratio (LCCA- IA)/(LSCA-IA) is short and less than 50%, and group II with such ratio ≥ 50%. Seventy-three patients were recruited. The distance (LCCA-IA) had a range of Zero (common origin) to 22.3 mm. The distance ratio (LCCA-IA)/(LSCA-IA) ranged between Zero and 89%. Group I had a significantly higher incidence of adverse outcomes, including recoarctation, re-elevation of blood pressure, and re-elevation of pressure gradient, compared to Group II (p = 0.0001, 0.011, and 0.014, respectively). A positive correlation exists between the distance ratio and the residual SPG across the repaired segment (P = 0.0001). Only the anatomical distance ratio (LCCA-IA)/(LSCA-IA) can independently predict recoarctation in the long term. There is a strong association between the anatomical distance LCCA-IA and recoarctation. This novel parameter is the only anatomical independent predictor of recoarctation.

The timing for intervention in patients with significant chronic aortic regurgitation is based on adult guidelines and criteria which may not apply to children. There is limited data on the use of cardiac MRI parameters to guide surgical decision-making in paediatrics. We examined associations between MRI quantification of aortic regurgitation and left ventricular volumetric function and the need for surgical intervention. Forty children and young adults with aortic regurgitation who had undergone cardiac MRI were divided into two groups based on aortic valve surgery (n = 20) or no surgery (n = 20). Ventricular volumetric functional parameters and aortic regurgitant volume and fraction were collected. Differences in MRI parameters between the groups were compared using unpaired t-tests. Receiver operating characteristic analysis identified MRI cut-off values with discriminatory ability towards primary end point of surgery (area under the curve > 0.7). Patients who underwent surgery had significantly larger ventricular volumes and aortic regurgitant fraction than those without surgery. Aortic regurgitant fraction and volume had the highest discriminatory power (0.93 and 0.92, respectively) between the two groups, followed by indexed left ventricular volumes (end-diastolic volume 0.85 and end-systolic volume 0.89). Current guidelines for surgical intervention in children with chronic aortic regurgitation are limited. Our findings suggest potential MRI-based threshold values that may aid in surgical decision-making and highlight the need future research for aortic valve surgery in children with chronic aortic regurgitation.

This retrospective study aimed to investigate the prevalence of arrhythmia in patients presenting with palpitation to the paediatric emergency department of our hospital, which serves as an arrhythmia centre and to share the principles of their management. Patients presenting with palpitations were retrospectively reviewed. Those diagnosed with arrhythmias received appropriate emergency interventions. Cardiac electrophysiological studies and ablation were performed when indicated. Among 534 paediatric patients evaluated for palpitations, 140 (26.2%) were diagnosed with arrhythmias requiring antiarrhythmic treatment (Group 1). Within this group, 61 patients described palpitations lasting longer than one hour and/or heart rates too rapid to count, compared to only 35 patients in the not requiring antiarrhythmic treatment group (Group 2) (p < 0.001). Group 1 also demonstrated significantly higher rates of isolated palpitations (a single episode without accompanying symptoms or recurrent occurrences), recurrent palpitations, and palpitations ongoing at the time of paediatric emergency department admission (all p < 0.001). Our study supports that, as in adults, the probability of arrhythmia increases in children when palpitations persist for more than an hour, occur at an uncountable rapid rate, present as isolated or recurrent episodes, or continue at the time of admission. This data highlights the importance of taking a detailed medical history once again. To our knowledge, this is one of the few studies to comprehensively examine both the acute management and long-term outcomes of arrhythmia in children, including the role of ablation therapy, making it a potentially valuable contribution to the existing literature.

Isolated atrioventricular discordance with ventriculoarterial concordance, or ventricular inversion, is a rare congenital cardiac anomaly that produces transposition-like physiology. We report a case of prenatally diagnosed ventricular inversion presenting with profound systemic hypoperfusion secondary to a large patent ductus arteriosus (PDA). Initial management included prostaglandin E1 to maintain ductal patency and balloon atrial septostomy to promote intracardiac mixing. Despite these measures, the patient developed significant pulmonary overcirculation and systemic steal, necessitating urgent transcatheter PDA closure. This intervention resulted in immediate hemodynamic improvement and stabilization, allowing for subsequent definitive repair with VSD closure and Senning atrial switch. The case underscores the importance of individualized hemodynamic assessment and the potential for transcatheter ductal closure to temporize systemic perfusion in select patients with ventricular inversion prior to anatomic correction.

The combination of a right aortic arch with a vascular ring and coarctation of the aorta is a rare association, presenting a unique management challenge for the primary team. A thorough literature review revealed only seven published case reports, with three cases reported in the neonatal period with similar anatomy. This distinctive anatomy inspires inquiry into the development of coarctation in the context of a right aortic arch and vascular ring, as well as the best approach to surgical management. We encountered a similar case in a neonate with a combination of these malformations and a unique aortic arch branching pattern. Cardiac CT was instrumental in the diagnosis and surgical planning. This article reviews the variations in anatomy, clinical presentation, imaging findings, and management challenges encountered in the reported cases. This comprehensive review aims to assist the primary team in making informed decisions when treating these complex patients.