Holodiastolic flow reversal in the descending aorta can complicate acute myocarditis, indicating decreased cardiac output and possible aortitis. This pathology is related to the disappearance of the 'Windkessel' phenomenon, normally enabling the aortic walls to store potential energy during systole, which is released to the thoracic aorta blood flow during diastole. Furthermore, severe low-output syndrome or distributive shock can render the aortic walls incapable of exploiting their elasticity, which usually enhances the power of aortic blood flow. Moreover, the increased release of catecholamines, or their large-scale administration, causes vasoconstriction, mainly in the abdominal aorta and iliac-femoral branches. This increases peripheral resistance, but to a lesser extent in the supra-aortic branches, whose network becomes competitive and remains supplied with a sufficient amount of blood. From a pathological point of view, aortitis can be caused by an increase in the release of inflammatory cytokines, enhanced by ischemia resulting from a decrease in blood supply from the vasa vasorum, a consequence of shock. This pathology, easily detected by Echocardiography, must be considered an important sign of shock and is worthy of further investigation using 4D MRI aortic angiography.

Advancements in pediatric heart failure management and mechanical circulatory support devices necessitate evaluating survival outcomes for children on the heart transplant waiting list. This study examines survival rates of pediatric patients (ages 0-18) on the heart transplant waiting list from 1987-2023. A retrospective analysis of the United Network for Organ Sharing (UNOS) database was conducted for survival of pediatric candidates (ages 0-18) listed for heart transplant from 1987 to 2023. Patients over 18 and combined transplant candidates were excluded, resulting in a total of 18,536 candidates studied. These candidates were followed from listing to death, transplantation, or clinical improvement warranting removal from the waitlist. Analysis involved unadjusted and adjusted survival rates, with Kaplan-Meier survival curves and competing risk analysis models used to assess survival. For competing risk analysis, the primary outcome was waitlist mortality, while transplantation and removal from the waiting list due to improvement were competing outcomes. Variables analyzed include demographic data, body mass index (BMI), dialysis, extracorporeal membrane oxygenation (ECMO), use of ventricular assist devices (VADs), and functional status at listing. One-year survival for pediatric waitlist candidates improved from 21.1% in 1987-1990 to 70.5% in 2021-2023 (P < .05). Adjusted analyses showed a reduction in mortality risk across eras. Survival for pediatric heart transplant candidates has significantly improved over time. Policy changes in allocation and mechanical support advancements have contributed to enhanced outcomes. Future research should refine pediatric transplant criteria and further investigate assist devices' role in improving waitlist survival.

Surgical outcome data on adolescent and adult congenital heart disease (CHD) undergoing cardiac surgery remains scarce in developing economies. The study aims to evaluate the clinical characteristics and postoperative outcomes in this population, with a focus on identifying risk factors to guide quality improvement initiatives. This retrospective cohort study analyzed 569 CHD patients (aged > 12years) who underwent surgery at a tertiary center (2018-2023). Data included demographics, surgical details, and clinical outcomes. Major adverse events (MAEs) were defined as cardiopulmonary resuscitation, chest reopening for resuscitation, extracorporeal membrane oxygenation, or death. Univariate and multivariate logistic regressions were performed to identify predictors of MAEs. The median age at surgery was 19.7years; 42.4% underwent redo sternotomy. Complications occurred in 60.1% of patients, with MAEs in 4.9% and mortality in 2.1%. Multivariate analysis identified significant postoperative bleeding (OR 583.62, 95% CI 16.24-20,970.32) as the main predictor of MAEs. Redo sternotomy doubled the risk of a significant postoperative bleeding (15.4% vs. 6.7%,p < 0.001). The strong association between redo sternotomy, significant postoperative bleeding and MAEs provided the evidence base for a focused quality initiative. The findings served as the direct impetus for developing and implementing a multidisciplinary institutional protocol aimed at mitigating this primary risk. This structured framework offers a replicable model for programs in other resource-limited settings.

Improvements in outcomes for congenital heart disease (CHD) have resulted in a growing adolescent/adult CHD population. Subsequently, education on CHD causes and inheritance has been identified as important. This study aimed to determine population-specific understanding and preferences of CHD causes and inheritance education. CHD adolescents aged 13-18years, their parent, and clinicians involved in the care of patients with CHD completed a purpose-designed, online survey outlining their current understanding of CHD genetics and inheritance and their preferences for education content and delivery. Approximately 50% of participants were unsure if CHD was caused by genetic factors, and 25% believed CHD was not genetic. Many participants (63% (19/30) adolescents, 81% (26/32) parents) wanted to know more about CHD causes, with most considering this important. Over 60% of participants indicated that CHD causes had not been discussed with them, despite 74% of clinicians (17/23) indicating they discuss this. Information on genetic causes of CHD and recurrence risks, delivered in-person with parents present, was the preferred option for the content and delivery of this information. Most clinicians indicated genetics education should be introduced between 12 and 14years, whereas adolescents and parents preferred this to occur between 14 and 16years. Participants want to know more about CHD causes and inheritance, in an outpatient setting with parents in attendance. The findings of this study support the development of a specialized genetics education program about CHD causes and inheritance for adolescents with CHD. Further, it provides important insight into the content, timing, and delivery of this information.

Infective endocarditis (IE) remains a significant cause of morbidity and mortality in pediatric patients with congenital heart disease (CHD), however, data on its epidemiology, risk factors, symptoms, complications, and outcomes remain scarce. This retrospective multicenter study analyzed pediatric patients (< 18years) with CHD diagnosed with IE between 2008 and 2023. Clinical data-including demographic information, cardiac history, echocardiographic findings, microbiological results, treatment strategies, and outcomes-were collected and analyzed. A total of 41 patients were included. The most common risk factors were prior cardiac surgery or percutaneous intervention, prosthetic materials, and significant valvulopathies. A significant association was observed between vegetation size and embolic events, with larger vegetation conferring a higher embolic risk. Staphylococcus aureus and Coagulase-negative staphylococci were the most frequently isolated microorganisms. Fever, new onset or worsening valvulopathies, and vegetation or abscesses formation were the predominant clinical presentation. Aggressive medical and surgical management resulted in favorable outcome in most patients. While IE remains a major challenge in pediatric CHD patients, early detection and timely intervention are crucial for optimizing outcomes, particularly in patients with large vegetations. Future research should aim to identify additional risk factors and refine strategy for prevention and treatment in this high-risk population.

Most pediatric patients with valve reconstruction require reoperation after discharge, primarily due to valve structural degeneration or infective endocarditis, which is caused by valve leaflet material problems. Previous studies generally believe that glutaraldehyde-solidified autologous pericardial tissue is superior to other biological tissues; however, it is regrettable that it lacks growth potential. It is urgent to develop new valve materials with certain growth potential, without anticoagulation and rejection. The right atrial appendage tissue is a native tissue and can be used immediately without any chemical fixation. This tissue is stretchable, elastic, and relatively thin. It has many benefits such as easy access, low price, safe and effective, short learning curve, strong reproducibility, no need for anticoagulation, no immunogenicity, and certain growth potential, which are worthy of promotion in many developing countries with limited medical resources. This review summarizes the pathophysiological mechanism, past and current status of right atrial appendage tissue for valve reconstruction in children, and prospects it, provides the evidence-based medical basis for the surgical treatment of valve diseases in children, and opens up another direction for the surgical repair of valve diseases in children.

Postural orthostatic tachycardia syndrome (POTS) and neurocardiogenic syncope (NCS) are frequently observed causes of orthostatic intolerance (OI). Clinical experience reveals patients with overlapping symptoms of both. This observation led to the hypothesis of orthostatic tachycardia hypotensive syndrome (OTHS), a distinct OI variant, combines POTS and NCS features. This study aims to define and characterize it. A retrospective chart review of patients presenting with OI and underwent head up tilt (HUT) between 2014 and 2020. We extracted demographic data, findings during HUT including heart rate (HR), systolic blood pressure (SBP), stroke volume (SV), near infrared spectroscopy (NIRS), syncope, cardiac asystole, and convulsions. We divided the subjects into three groups: POTS, NCS, and OTHS. We included patients with POTS (n = 90), NCS(n = 86), and OTHS (n = 101). POTS patients showed higher HR (baseline, recovery, minimum) vs. OTHS (p = 0.047, < 0.001, < 0.001), while OTHS patients had higher HR (5min, 10min, minimum, maximum) vs. NCS (p = 0.047, < 0.001, < 0.001, < 0.001). Minimum SBP was higher in POTS vs. OTHS (p < 0.001), and OTHS patients had higher SV (baseline, recovery, minimum, maximum) vs. POTS (p = 0.006, < 0.001, 0.002, 0.005). Patients with POTS have lower baseline NIRS compared to NCS and OTHS (p = < 0.032, < 0.011). Asystole was significantly more frequent in the NCS group (n = 24, 27.9%) than in the OTHS group (n = 9, 8.9%), with p < 0.001. OTHS is a form of OI characterized by initial orthostatic tachycardia with increased HR > 30-40bpm followed by hypotension leading to syncope.

Serum biomarkers have emerged as tools for diagnosis and management in adult heart disease but are less investigated in the pediatric population. This exploratory study reports biomarker profiles in unrepaired tetralogy of Fallot (TOF), repaired TOF (rTOF), hypoplastic left heart syndrome (HLHS) following Fontan surgery, and healthy controls. We compared circulating biomarker patterns between TOF, rTOF, HLHS, and control groups, aiming to characterize potential disease-specific profiles and generate hypotheses for future research. We prospectively enrolled subjects and collected single-time blood samples for analysis. We measured: microRNA-21 (miR-21), soluble suppression of tumorigenicity-2 (sST-2), galectin-3 (Gal-3), procollagen type-I carboxy-terminal pro-peptide (PICP), procollagen type-III amino-terminal pro-peptide (PIIINP), metalloproteinases (MMP-1/MMP-9), and NT-proBNP. We included 207 patients: TOF (n = 75), rTOF (n = 60), HLHS (n = 11), and healthy controls (n = 60). Compared to the younger controls, TOF patients had higher PICP, MMP-1, and NT-proBNP. Compared to older controls, rTOF patients had higher PIIINP, MMP-1, MMP-9, and NT-proBNP; and HLHS patients had higher PIIINP and MMP-1. Collagen metabolism biomarkers and MMP-1 were elevated across disease groups. Gal-3 was associated with age in HLHS. No disease-specific patterns were observed; however, differences from controls suggest cardiac remodeling in TOF and HLHS.