M. A. Galli

Analysis of congenital heart defects in 87 consecutive patients with Brachmann-de Lange syndrome.

Congenital heart defects (CHDs) have been estimated to occur in ∼20% of patients with Brachmann‐de Lange syndrome (BDLS, also known as Cornelia de Lange syndrome, OMIM 122470). We report on the results of a prospective echocardiographic evaluation of a cohort of 87 Italian BDLS patients with longitudinal follow‐up from 5 to 12 years. A cardiac anomaly was identified in 29/87 (33.3%) including 28 (32.2%) patients with a structural CHD, and an additional patient (1.2%) with isolated non‐obstructive hypertrophic cardiomyopathy (HCM). Of the 28 patients with a CHD, 12 (42.9%) had an isolated obstructive CHD, 10 of which were pulmonary stenosis (36%), 8 (28.6%) had an isolated left to right shunt, and the remainder showed a combination of structural anomalies. Overall incidence of pulmonary stenosis was 39% (11/28). Isolated late‐onset mitral or tricuspid valve dysplasia, albeit hemodynamically insignificant, was detected at follow‐up examination in 4 (14.3%) patients older than 10 years, previously known to be normal. In contrast to previous studies, only two patients required surgery, one for closure of a large perimembranous ventricular septal defect (VSD) and associated ASD closure (1), and another for VSD closure and relief of pulmonary valve stenosis (1). The remainder are receiving medical follow‐up. We believe that the overall frequency (33.3%) and evidence of 4 late onset dysplastic valves anomalies justifies both echocardiographic assessment in all BDLS patients at the first diagnostic assessment, and later on during medical follow‐up.

Differences between office and ambulatory blood pressures in children and adolescents attending a hospital hypertension clinic.

Background and objectives: Information on ambulatory blood pressure monitoring (ABPM) in children is scarce. While in adults office BP (OBP) is higher than ABP and the difference increases as OBP increases, information in children suggests that at this young age ABP is no lower and often higher than OBP. This study was aimed at describing OBP–ABP differences in a cohort of children of different ages and BPs, and investigating whether OBP–ABP differences are dependent on age or OBP level. Methods: We retrospectively compared OBP and 24-h, daytime and night-time ABP in 433 children and adolescents aged 4–18 years, referred to our hospital clinic. Results: OBP was found to be significantly lower than 24-h and daytime ABP in the low age tertile (4–10 years) but not in the medium and high tertiles. OBP was also lower than ABP in normotensive patients (n = 182), but higher than ABP in untreated hypertensive patients (n = 92) despite similar ages. Continuous analyses showed a weak correlation of OBP–ABP differences with age, and a much stronger correlation with OBP so that 24-h ABP was higher than OBP at OBP values less than 117/73 mmHg and lower than OBP at higher OBP values. Logistic regression analysis indicates that also in children OBP accounts for most of the OBP–ABP difference. Conclusion: There is a common relation both in children and adults between OBP and ABP. It is only because high OBP is common in the elderly, and the lowest OBP is usually found in young children that large positive OBP–ABP differences have been associated with old age, and negative differences with childhood. OBP–ABP differences, often defined as white-coat effect, can have different directions and are likely to be largely due to regression to the mean.

Flecainide as first-line treatment for supraventricular tachycardia in newborns.

Background Flecainide for the treatment of supraventricular tachycardia (SVT) in newborns is still controversial because of its potentially severe proarrhythmic effects. Methods and results Between January 2004 and December 2006, we used flecainide to treat 20 consecutive newborns (15 males) with paroxysmal SVT without any structural heart disease. Their age at hospitalization was 11.5 ± 11.1 days. The intravenous administration of flecainide (1 mg/kg) effectively restored sinus rhythm in all the patients. Once stable sinus rhythm had been restored, the drug was administered orally at a dose of 2 mg/kg/day twice daily, which was uptitrated as the patients gained weight. The patients were followed up for up to 24 months with clinical evaluations, baseline ECG and 24-h Holter monitoring every 3 months. There were neither deaths nor any episodes of heart failure or sustained ventricular tachycardia during follow-up. SVT were completely controlled in 17 patients (85%), with an oral dose of 3.35 ± 1.35 mg/kg/day of flecainide; in the remaining three patients with refractory arrhythmias, propranolol was added for optimal treatment. No significant increase in the duration of QRS (70 ± 1.09 vs. 63.8 ± 1.87 ms, P = NS) or any significant QTc prolongation (413 ± 7.4 vs. 412.6 ± 8.01 ms, P = NS) was observed. One patient developed an incomplete right bundle branch block promptly reverted by reducing the dose. Conclusion This preliminary experience indicates that flecainide is well tolerated and effective as first-line treatment for paroxysmal SVT in newborns without structural heart disease.

A rare case of neonatal cyanosis due 'cor triatriatum dexter' and a review of the literature.

Cor triatriatum dexter is a rare cardiac congenital malformation in which a membrane divides the right atrium into two chambers. We describe one case of isolated cor triatriatum dexter, symptomatic for severe central cyanosis, in which the membrane was identified before surgery by means of transthoracic echocardiography alone, and successfully removed surgically at 1 month of life. We discuss this case and review the literature.

A Guide to Neonatal and Pediatric ECGs

A Guide to Neonatal and Pediatric ECGs - Libros de Medicina - Cardiologia pediatrica - 103,99

Applications for Pediatric ECGs

The role of the ECG in pediatric cardiac evaluation is expanding and guide lines have been published to help establish directions on their use. The value of the ECG is clear in the initial evaluation of a patient suffering from cardiopathy or suspected cardiopathy, as well as in the follow-up for a cardiac patient or one who takes prescriptions with possible effects on the heart.

ECG Reading Method

The pediatric ECG reading method is based on the fact that the morphology of a normal ECG varies with age. The electrical activity of the heart reflects hemodynamic cardiac changes, which are at their height in the first month of life and which continue, in part, through the first year of life and beyond.

Left Ventricular Overload

In the left ventricle, left ventricular systolic and diastolic overload refer to pressure overload and volume overload. These two hemodynamic situations cause left ventricular hypertrophy and left ventricular dilatation respectively. From an electrocardiographical standpoint they can be considered together since they are both expressed by the same observation: the appearance of the “adult pattern” of left ventricular prevalence on an ECG, before the age of 2 and with the S and R wave’s pathological voltage in V1 and V6 precordial leads.

Normal Parameters of Pediatric ECGs

Along with the heart rhythm evaluation, the following basic parameters are initially considered when reading an ECG: Heart rate PR interval QRS frontal axis QRS duration QT and QTc (heart rate corrected) interval.

Right Ventricular Overload

Right ventricular systolic overload refers to an overload of systolic pressure in the right ventricle and it indicates a right ventricle that pumps more than the normal 30 mmHg while developing systolic pressure. This hemodynamic situation appears in the following conditions: 1. All congenital heart defects that obstruct right ventricular outflow, i.e. significant or critical pulmonary valve stenosis, pulmonary atresia, and Tetralogy of Fallot 2. Congenital heart defects causing pulmonary hypertension from a high pulmonary blood flow with low pulmonary vascular resistance, for example a large ventricular septal defect, double-outlet right ventricle, complete form of atrioventricular septal defect, total anomalous pulmonary venous drainage 3. Congenital heart defects complicated by pulmonary artery hypertension due to progressive structural abnormalities in the pulmonary vascular bed, i.e. obstructive pulmonary vascular disease.