The autonomic condition of children with congenital hypothyroidism as indicated by the analysis of heart rate variability.

Early treatment by thyroid hormone in congenital hypothyroidism (CH) prevents for mental retardation, but the autonomic condition could well remain impaired. The aim of this study was to get insights about the cardiovascular regulation of CH children by analysing their heart rate variability (HRV) data. RR-interval data of 34 early treated CH children, 24 to 48 months of age, were collected at rest in a clinical environment. In all these cases, CH resulted from an abnormal development of thyroid gland. Data from 29 age matched-controls were also collected in a scholar environment. Short time series were then analysed in time and frequency domains. Detrended fluctuation analysis (DFA) was also applied to obtain the scaling exponent α(1). We found that the HRV obtained from children with CH is diminished as suggested by statistical differences (p<0.05) in the following parameters: RMSDD, pNN20, total power, LF and HF. However, no significant differences were found in the LF/HF ratio, the central frequencies of the LF and HF bands, as well as in α(1). Given that the significant differences were basically reflected in static parameters rather than in the frequency distribution or scale-invariant behaviour of the studied data, we consider that the analysis points toward the effect of treatment on rising heart rate by the thyroid hormone, and not necessarily toward any residual autonomic dysfunction, as the dominant underlying cause of the diminished HRV exhibited by CH children.

Repeatability of heart rate variability in congenital hypothyroidism as analysed by detrended fluctuation analysis.

The analysis of heart rate fluctuations, or heart rate variability (HRV), may be applied to explore children's neurodevelopment. However, previous studies have reported poor reliability (repeatability) of HRV measures in children at rest and during light exercise. Whether the reliability can be improved by controlling variables such as physical activity, breathing rate and tidal volume, or by selecting non-conventional techniques for analysing the data remains as an open question. We evaluated the short-term repeatability of RR-interval data from medicated children with congenital hypothyroidism (CH). The alpha(1) exponents, obtained by detrended fluctuation analysis (DFA), from the data of 21 children collected at two different sessions were compared. Elapsed days between sessions were 59 +/- 33, and data were obtained during 10 min, trying to restrict the children's activity while being seated. We found statistical agreement between the means of alpha(1) exponents for each session (p = 0.94) and no bias with a low-coefficient variation (9.1%); an intraclass correlation coefficient ri = 0.48 ([0.14 0.72], 95% confidence interval) was also estimated. These findings, which were compared with results obtained by conventional time and frequency techniques, indicate the existence of agreement between the alpha(1) exponents obtained at each session, thereby providing support concerning the repeatability of HRV data as analysed by DFA in children with congenital hypothyroidism. Of particular interest was also the agreement found by using the central frequency of the high-frequency band and the parameter pNN20, both showing better or similar ri than alpha(1) (0.77 [0.57 0.89] and 0.51 [0.17 0.74], respectively), yet considerably better repeatability than other conventional time and frequency parameters.

Multiple arterial anomalies in the newborn infant. Echocardiographic and angiographic diagnosis.

Multiple arterial anomalies characterized by tortuosity and rolling of the pulmonary arteries and aorta were diagnosed on echocardiography in an asymptomatic newborn infant with a phenotype suggesting Ehlers-Danlos syndrome. These changes were later confirmed on angiography, which also showed peripheral vascular abnormalities. The electrocardiogram showed a probable hemiblock of the left anterosuperior branch, and the chest x-ray showed an excavated pulmonary trunk with normal pulmonary flow.

Right coronary artery fistula with congestive heart failure in the neonate. Doppler echocardiographic diagnosis and closure with detachable balloon.

We report here a case of coronary artery fistula in a neonate with clinical signs of heart failure. The electrocardiogram showed signs of left ventricular hypertrophy and diffuse alterations in ventricular repolarization. Chest X-ray showed an enlargement of the cardiac silhouette with an increase in pulmonary flow. After echocardiographic diagnosis and angiographic confirmation, closure of the fistulous trajectory was performed with a detachable balloon with an early and late successful outcome.

Association of pulmonary atresia with intact ventricular septum and aortic valve stenosis. Prenatal diagnosis.

A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81 mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.

[Right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Absence of origin of the coronary arteries from the aorta]. / Circulação coronária dependente do ventrículo direito na atresia pulmonar com septo interventricular íntegro. Ausência da origem das artérias coronárias da aorta.

This report describes the clinical, echocardiographic and angiographic aspects of a five-day old boy with pulmonary atresia and intact ventricular septum. Both the echocardiogram and the aortography did not show any coronary arteries arising from the aorta. Two-dimensional echocardiography was able to identify the coronary arteries originating from the right ventricle and so did the right ventricular angiogram. No retrograde flow into the aorta or pulmonary trunk was identified after opacification of the coronary arteries. As far as we know this is the first case diagnosed by echocardiography, and is a vivid example of the necessity of identifying the coronary arteries in patients with pulmonary atresia and intact ventricular septum.

[Anomalous origin of the right pulmonary artery from the ascending aorta (Hemitruncus)]. / Origem Anômala da artéria pulmonar direita em aorta ascendente (Hemitruncus).

The origin of the right pulmonary artery from the ascending aorta is a rare cogenital anomaly, with very few reports in the literature. We describe two cases of this rare malformation, one of them, associated with interruption of the aortic arch (type B). In both cases, the diagnosis was made by two-dimensional echocardiography, with angiographic confirmation in one of them. The origin of the right pulmonary artery was close to the aortic valve, anomaly pathogenetically distinct from the type that arises close to the innominate artery.

Origem anômala da artéria pulmonar direita em aorta ascendente (hemitruncus) / Anomalous origin of right pulmonary artery from ascending aorta (hemitruncus)

A origem da artéria pulmonar direita em aorta ascendente é uma anomalia congênita rara, com poucos casos descritos na literatura. Descrevemos dois casos desta malforamçäo, um associado à interrupçäo de arco aórtico tipo B. Em ambos, o diagnóstico foi realizado mediante ecocardigrafia bidimensional, com confirmaçäo angiográfica em um. A origem da artéria pulmonar direita era próxima à valva aórtica, anomalia patogeneticamente diferente da origem perto da artéria inominada.

Persistência do canal arterial e endarterite da artéria pulmonar / Patent ductus arteriosus and pulmonary artery endarteritis

Descrevemos uma paciente de sete anos com diagnóstico ecocardiográfico e confirmação cirúrgica de endarterite de artéria pulmonar, citada como complicação da persistência do canal arterial e ilustramos a necessidade de ressecção cirúrgica completa do processo infeccioso para evitar outras complicaçöes, como embolia pulmonar.

[Patent ductus arteriosus and pulmonary artery endarteritis]. / Persistência do canal arterial e endarterite da artéria pulmonar.

We describe a seven year old girl with echocardiographic diagnosis of pulmonary artery endarteritis as a complication of a patent ductus arteriosus, confirmed at surgery. This case illustrates the necessity of complete surgical resection of the infectious source as a way to avoid other complications such as pulmonary embolism.