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Cardiovascular malformation in infant deaths. 10-year clinical and epidemiological study.

The infant mortality from cardiovascular malformations in a region with a population of 2 million inhabitants during a 10-year period has been studied. The study involved validation of the diagnoses and judgement whether the cardiovascular malformation was the dominating or a contributing cause of death. It is shown that the incidence of fatal cardiovascular malformations is probably overestimated in the official death statistics. Evaluation of the clinical findings and necropsy reports are important aids in obtaining more reliable figures of the incidence. The rate of referral of infants with cardiovascular malformations has increased during the period of this study, so that the number of operable lesions not referred has decreased. The infant mortality rate, found in this study, of 1.33 per 1000 liveborn babies constitutes about 20 per cent of all liveborn infants with cardiovascular malformations. The most common lesions found in those who died belonged to the group constituting the hypoplastic left heart syndrome. The proportion of this type of malformation, about 20 per cent of all those dying, is higher than in other similar studies. This difference can probably be explained by variations in selection and classification.

Aortic Coarctation↗

Non-invasive estimation by cross sectional echocardiography of myocardial damage in cardiomyopathy.

Retrospective and prospective studies of high resolution cross sectional echocardiograms were undertaken in order to establish an ultrasonic method for the non-invasive estimation of degeneration and fibrosis of the endomyocardium in cases of cardiomyopathy. When the echocardiograms of the ventricular wall were compared with the histological specimens intense abnormal echoes were seen at the sites of myocardial degeneration and fibrosis of the ventricular wall. The abnormal echoes classified into five types: types I, II, III-1, III-2, and III-3. Type I and type III-1 echoes were the strongest followed by those of types II and III-2, and then those of type III-3. The intensity of the abnormal echoes was 5-20 decibels stronger than that from intact tissue and was closely related to the consistency and density of the diseased tissue. These findings strongly suggest that the boundary between degeneration or fibrosis and the intact normal myocardium was the source of the abnormal myocardial echoes and that the extent and the pattern of the distribution of the sites of degeneration and fibrosis in the myocardium were reflected in the echo patterns. Thus the tissue characteristics of the sites of degeneration or fibrosis of the myocardium may be determined non-invasively by measuring the echo intensity.

Adolescent↗

Fatal Lyme carditis and endodermal heterotopia of the atrioventricular node.

A fatal case of Lyme carditis occurring in a Suffolk farmworker is reported. Post-mortem examination of the heart showed pericarditis, focal myocarditis and prominent endocardial and interstitial fibrosis. The additional finding of endodermal heterotopia ('mesothelioma') of the atrioventricular node raises the possibility that this could also be related to Lyme infection and account for the relatively frequent occurrence of atrioventricular block in this condition. Lyme disease should always be considered in a case of atrioventricular block, particularly in a young patient from a rural area. The heart block tends to improve and therefore only temporary pacing may be required.

Adult↗

Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis.

BACKGROUND: Transcatheter balloon aortic valvuloplasty (BAVP) has become the first-line treatment for critical aortic stenosis (AS) in neonates. However, little is known about the growth and function of left heart structures or about patterns of reintervention on the left heart after neonatal BAVP. METHODS AND RESULTS: Between 1985 and 2002, 113 patients underwent neonatal BAVP at < or =60 days of age. There were 16 early deaths (14%), with a significant decrease from 1985 to 1993 (22%) to 1994 to 2002 (4%), and 6 patients had successful early conversion to a univentricular circulation. In the short term, the mean relative gradient reduction was 54+/-26%, and significant aortic regurgitation (AR) developed in 15% of patients. The 91 early survivors with a biventricular circulation were followed up for 6.3+/-5.3 years, during which time there was a steady increase in the frequency of significant AR. Freedom from moderate or severe AR was 65% at 5 years. In almost all patients with a baseline aortic annulus z score less than -1, the annulus diameter increased to within the normal range within 1 to 2 years. Similarly, left ventricular (LV) end-diastolic dimension z scores, which ranged from -5 to 7.5 before BAVP, normalized within 1 to 2 years in nearly all patients with a predilation z score less than -1. Among early survivors with a biventricular circulation, reintervention-free survival on the LV outflow tract was 65% at 1 year and 48% at 5 years, with younger age, higher pre- and post-BAVP gradients, and a larger balloon-annulus diameter ratio associated with decreased reintervention-free survival (P<0.01). Seventeen surgical interventions were performed on the aortic valve in 15 patients, including replacement in 7. Survival free from aortic valve replacement was 84% at 5 years. CONCLUSIONS: BAVP for AS during the first 60 days of life results in short-term relief of AS in the majority of patients. Among early survivors, initially small left heart structures may be associated with worse subacute outcomes but typically normalize within 1 year. Reintervention for residual/recurrent AS or iatrogenic AR is relatively common, particularly during the first year after BAVP, but aortic valve replacement during early childhood is seldom necessary.

Aortic Valve↗