Predictors of Bicuspid Aortic Valve-Associated Aortopathy in Childhood: A Report From the MIBAVA Consortium

BACKGROUND: Bicuspid aortic valve (BAV) is the most prevalent congenital heart defect affecting 1% to 2% of the population. It is associated with ascending aorta dilatation. Valve morphology, aortic stenosis (AS), and aortic insufficiency (AI) have been proposed as potential risk factors; however, evaluating their role is difficult, as these factors are inherently related. The aim of this study was to determine whether BAV morphology and dysfunction are independent determinants for ascending aorta dilatation in pediatric patients. METHODS: A multicenter, retrospective, cross-sectional study of pediatric BAV patients followed since 2004 was performed. Imaging data were assessed for BAV morphology, severity of AS and AI, history of coarctation, and aortic dimensions. Associations were determined using multivariable regression analysis. A subset of patients undergoing aortic interventions (balloon dilation or Ross) were assessed longitudinally. RESULTS: Data were obtained from 2122 patients (68% male; median age 10.2 years). Fifty percent of patients had ascending aorta dilatation. Right and noncoronary cusp fusion, increasing AS and AI, and older age were independently associated with ascending aorta dilatation. A history of coarctation was associated with less ascending aorta dilatation. In patients with neither AS nor AI, 37% had ascending aorta dilatation (4% severe). No complications related to aortic dilatation occurred in this cohort. Aortic CONCLUSIONS: In this large pediatric cohort of patients with BAV, valve morphology, AS, and AI are independently associated with ascending aorta dilatation, suggesting that hemodynamic factors influence aortopathy. However, even in BAVs with no AS or AI, there is significant ascending aorta dilatation independent of valve morphology. Interventions that led to changes in degree of AI and AS did not seem to influence change in aortic dimensions. The current BAV cohort can be used as a reference group for expected changes in aortic dimensions during childhood

Middle Aortic Syndrome and Renal Artery Stenosis: Disease Beyond the Arch

Background Middle aortic syndrome (MAS) is a rare childhood disease, often associated with renal artery stenosis (RAS). The etiology is unknown in most cases, but genetic and inflammatory causes have been described. Management of the associated hypertension can be medical, endovascular, or surgical, with variable success. Aims Our aims were to 1) evaluate management, and outcomes of MAS and/or RAS by etiology; 2) assess the peripheral vascular involvement and aortic disease; and 3) evaluate cardiac structure, function, and myocardial mechanics. Methods Aim 1: we conducted a systematic review of 630 MAS cases, and a retrospective cohort study of 93 children with MAS and/or RAS managed at the Hospital for Sick Children (HSC). Aim 2: a cross-sectional prospective study of 35 children with MAS and/or RAS was initiated at HSC (2014-2016). Carotid intima-media thickness (CIMT) and pulse wave velocity (PWV) were assessed using B-mode ultrasound and applanation tonometry. Aim 3: two-dimensional echocardiography and speckle-tracking echocardiography were used to assess left ventricular mass (LVM), diastolic function (E/a ratio) and myocardial strain. All cardiovascular measurements were compared to age, sex, and body surface area- matched healthy children. Results Of 630 cases in the literature, 70% had RAS, and the aortic disease was confined to the peri-renal aorta. Of 93 children managed at HSC, 70% received endovascular or surgical intervention, with a higher risk of intervention in children with unknown disease compared to those with genetic and inflammatory causes (HR=3, 95% CI [2,6]). Hypertension persisted in 65% of all patients for 2 [0.4-5] years after management. CIMT was increased in children with MAS and/or RAS compared to controls (0.54Âą0.10 vs. 0.44Âą0.05 mm, pPh.D

Longitudinal Assessment of Myocardial Function in Childhood Chronic Kidney Disease, During Dialysis, and Following Kidney Transplantation

BACKGROUND: Childhood chronic kidney disease (CKD) and dialysis are associated with increased long-term cardiovascular risk. We examined subclinical alterations in myocardial mechanics longitudinally in children with CKD, during dialysis, and following renal transplantation. METHODS: Forty-eight children with CKD (stage III or higher) who received kidney transplants from 2008 to 2014 were included in a retrospective study and compared to 192 age- and sex-matched healthy children. Measurements of cardiac systolic and diastolic function were performed, and global longitudinal strain (GLS) and circumferential strain (GCS) were measured by speckle-tracking echocardiography at CKD, during dialysis, and 1 year following kidney transplantation. Mixed-effects modeling examined changes in GLS and GCS over different disease stages. RESULTS: Children with CKD had a mean age of 10 ± 5 years and 67% were male. Eighteen children received preemptive transplantation. Children with CKD had increased left ventricular mass, lower GLS, and impaired diastolic function (lower E/A ratio and E\u27 velocities) than healthy children. Changes in left ventricular diastolic parameters persisted during dialysis and after renal transplantation. Dialysis was associated with reduced GLS compared to CKD (β = 1.6, 95% confidence interval 0.2-3.0); however, this was not significant after adjustment for systolic blood pressure and CKD duration. Post-transplantation GLS levels were similar to those at CKD assessment. GCS was unchanged during dialysis but significantly improved following transplantation. CONCLUSIONS: There are differences in diastolic parameters in childhood CKD that persist during dialysis and after transplantation. Systolic parameters are preserved, with significant improvement in systolic myocardial deformation following transplantation. The impact of persistent diastolic changes on long-term outcomes requires further investigation