Kawasaki disease (KD), an acute, self-limiting, medium-sized arterial vasculitis, is now the most common cause of acquired heart disease in childhood in the developed world. In this review, we discuss the diagnosis of KD, predicting resistance to traditional therapy and treatment options in refractory or high-risk disease. We also highlight ongoing clinical trials and other potential avenues of research which may prove beneficial in managing children, especially those with resistant KD.

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1–16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3–9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.

The highest incidence of sepsis across all age groups occurs in neonates leading to substantial mortality and morbidity. Cardiovascular dysfunction frequently complicates neonatal sepsis including biventricular systolic and/or diastolic dysfunction, vasoregulatory failure, and pulmonary arterial hypertension. The haemodynamic response in neonatal sepsis can be hyperdynamic or hypodynamic and the underlying pathophysiological mechanisms are heterogeneous. The diagnosis and definition of both neonatal sepsis and cardiovascular dysfunction complicating neonatal sepsis are challenging and not consensus-based. Future developments in neonatal sepsis management will be facilitated by common definitions and datasets especially in neonatal cardiovascular optimisation. Impact Cardiovascular dysfunction is common in neonatal sepsis but there is no consensus-based definition, making calculating the incidence and designing clinical trials challenging. Neonatal cardiovascular dysfunction is related to the inflammatory response, which can directly target myocyte function and systemic haemodynamics.

Ischaemic heart disease is the most common cause of death in males and the second in the female gender. Yet we often only focus on identification and treatment of this foremost cause of death in adulthood. The review asks the question what form of coronary disease do we encounter in childhood, what predisposing factors give rise to atherosclerosis and what strategies in childhood could we employ to detect and reduce atherosclerosis development in later life.

Background: The study was aimed at assessing clinical status and outcome of patients affected by aorto-left ventricular tunnel (ALVT). Methods: A systematic search of keywords relating to ALVT was conducted to identify papers published between 1965 and February 2024 present on Pubmed/Medline and Scopus. Results: A total of 109 studies, which in all consisted of case reports and case series comprising 177 patients (64.2% males, p < 0.02) met the inclusion criteria. The median age of patients was 9.5 ± 8.9 years. Initial diagnosis was based on echocardiographic findings in 86.4% of patients, and confirmed by computed tomography (CT) and/or magnetic resonance imaging (MRI) in 17%. Of the 177 patients identified, 47.1% were diagnosed with a heart murmur and 32.4% with congestive heart failure. Associated cardiac abnormalities were detected in 39.8% (unicuspid/bicuspid aortic valve with or without stenosis/atresia in 14.8%, coronary artery abnormalities in 9.6%). A total of 90.3% of patients underwent surgery, whilst 4.5% were treated by means of transcatheter closure. Outcomes were largely favorable (death was reported in 5.7%). Mild residual aortic regurgitation continued to be present in 22.7% of the sample. In terms of statistics, no risk factors for death were found. Conclusions: ALVT, an extremely rare congenital cardiac abnormality, may be diagnosed in both newborns and adults. Initial diagnostic observations are usually made using echocardiography, and subsequently refined by means of catheterization, CT or MRI. Surgery should be performed as soon as possible following diagnosis, particularly due to the inefficacy of medical treatment. In selected cases, transcatheter closure may represent a valid option. The condition is associated with a high mortality rate. Moreover, complications, particularly in the form of residual aortic valve regurgitation, may hamper postoperative prognosis. Due to the rarity of the disease, the setting up of an international registry is recommended.