Child, Adolescent – ABC CARDIOL

IJCS

Original Article

Cardiovascular Risk Factors in Children and Adolescents with Fontan Circulation

Sandra Mari Barbiero et al. Rafael B. Carloto Danielly Steffen Pereira Gabriela C. Schwantes … Marcela Menuci Guimarães Maíra Ribas Goulart Daniela Schneid Schuh Lucia Campos Pellanda

Abstract

Background:

Long-term outcomes of patients with Fontan circulation are uncertain regarding the prevalence and role of risk factors (RFs) such as increased body mass index (BMI), arterial hypertension, and hypercholesterolemia.

Objectives:

To describe the prevalence of RFs in patients with univentricular heart, with variable follow-up times.

Methods:

This mixed cohort study was performed with 66 patients, who underwent blood count, fasting blood glucose, C-reactive protein (CRP), and lipid profile tests; systolic/diastolic blood pressure (SBP/DBP) measurements; and anthropometric and sociodemographic data collection. Cardiovascular RFs among first-degree relatives and physical activity habits were also assessed. Prevalence was described using proportions, with a 95% confidence interval. Continuous variables (height, weight, age, SBP, DBP) were described as means and standard deviations (m±SD). Associations between RFs were assessed using chi-squared or Fisher’s exact tests. Spearman’s correlation was used for analyzing CRP and the presence of 2 or more RFs. The Shapiro-Wilk test was used to check for data normality. Statistical significance considered p<0.05.

Results:

In our population, 19.7% were overweight, mean SBP was 89.44±37.4, and mean DBP was 60.0±26.08. The most prevalent diseases in the interviewees’ families were systemic arterial hypertension (30.3%), obesity (16.7%), and 2 or more cardiovascular RFs among first-degree relatives (13.8%). We observed a trend towards significance between the presence of 2 familial RFs and overweight, as well as a risk profile for cardiovascular disease. There was an association between the BMI percentile, the presence of 2 or more RFs (p<0.05), and CRP (p<0.01).

Conclusions:

Overweight is common in patients with univentricular heart, being related to more than 2 cardiovascular RFs among first-degree relatives; physical inactivity and changes in lipid profiles are also frequent.

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ABC

Case Report

Spontaneous Coronary Artery Dissection in a Patient with Cerebrotendinous Xanthomatosis

Maria Júlia Silveira Souto et al. Marcos Antônio Almeida-Santos Eduardo José Pereira Ferreira Luiz Flávio Galvão Gonçalves … Joselina Luzia Menezes Oliveira Antônio Carlos Sobral Sousa

Introduction

Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive disease characterized by the formation of xanthomatous lesions in many tissues, particularly the brain and tendons. The disorder is a consequence of the reduced production of bile acids, predominantly chenodeoxycholic acid (CDCA), and an increased formation of cholestanol. Common clinical manifestations include infant-onset diarrhea and juvenile-onset bilateral cataract, usually followed by tendon xanthomas and progressive neurological dysfunction. The final diagnosis is based on biochemical abnormalities, including elevated plasma cholestanol level and increased levels of bile alcohol in urine associated with a diminished biliary concentration of CDCA. The treatment is based on oral supplementation of CDCA, which, if initiated early, can prevent major clinical problems, as it produces a reduction in cholestanol synthesis and plasma levels.

Cardiovascular impairment in patients with CTX is mostly associated with premature atherosclerosis. Blood lipid analysis in patients with CTX revealed dramatically high levels of 27-hydroxycholesterol and low levels of high-density lipoprotein cholesterol (HDL), which place these patients at a high risk of cardiovascular disease.

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