[Experience in surgical treatment of congenital defects of the heart].
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Proportions of infants of birthweight 501-2000 g born in 1979-1981 who had defects recognisable at birth were ascertained by examining hospital records and tracing survivors. Twenty-seven per cent of 230 stillbirths, 8.4% of 1411 livebirths, 4.5% of 1117 neonatal survivors and 3.6% of 1074 two-year survivors had a congenital defect, birth prevalence rising with lower birthweight only in stillbirths. Among livebirths, birth prevalence was increased in growth retarded infants, in singletons and in infants of mothers who had not had a previous reproductive loss. Twenty-four per cent and 57% of neonatal deaths in the 1001-1500 g and 1501-2000 g birthweight groups respectively were associated with a congenital defect. Major neurological impairments were directly attributable to congenital defects in 10% of 71 survivors; surgery and hospital admissions were far more frequent in infants with congenital defects than others. The 1501-2000 g group accounted for 77% of all survivors with a congenital defect. Further improvements in the prognosis of low birthweight infants will depend to a large extent on prenatal prevention of disease.
The authors give a basic embriological classification of congenital arterial defects. Congenital abnormalities of the arterial system (arising in an embryo of 12 to 30 mm) affect the primary segments, and comprise excessive involution of the antibrachial trunk, of the popliteal artery, of the tibio-peroneal trunk and peroneal artery, abnormal or persistence of the interosseus artery, of the artery of sciatic nerve, and of the peroneal artery, a high axillary origin of the radial or antecubital artery, renal polar arteries arising directly from the aorta, and agenesis of the palmar or plantar arches. In the second generation arteries, they comprise agenesis or hypogenesis of the radial, antecubital or tibial arteries, and gross hypogenesis of the trunk of the renal artery with an immature kidney. Arrested or mal-development in the foetus can give rise to coarctation and stenosis of the thoracic aorta and large vessels, to segmental agenesis with huge collaterals, to imperforate osita, especially in the mesenteric arteries, to sudden variations in diameter of the renal arteries, and to parietal dyplasia and aneurysms in vessels in the residual undifferentiated mesenchymatous tissue, and diffuse dilatation or stenosis. The authors give their experience for a cardiological clinic, adding arterial hypertension and arterioparenchymatous dysplasia of the kidneys, Raynaud's, Takayasbu's and Burger's disease, and intermittent claudication of the lower limbs; they indicate the areas which they are currently studying.
Congenital defect of the muscular layer of the small intestine is a rare cause of spontaneous bowel perforation in premature infants. During the last 12 years we have observed four similar cases. We describe the most recent one, a premature infant who developed two abdominal events. On her 2nd day of life, spontaneous perforation of the distal ileum due to focal absence of the muscular layer occurred. Several weeks later she developed the typical clinical and histological picture of necrotizing enterocolitis. The clinical and histological characteristics of the two different conditions are compared, and the 24 cases reported in the literature are discussed. We conclude that focal absence of intestinal musculature may be not such a rare entity as is commonly believed.
Congenital defects of the pericardium are extremely rare and frequently difficult to diagnose. We present a diagnosis of partial congenital defect of the left pericardium based on a characteristic chest X-ray and left ventriculogram. Additionally, we report findings of a diastolic, migratory coronary stenosis on coronary arteriography which, to date, has not been reported. Confirmation of the diagnosis by mediastinoscopy was followed by thoracoscopic pericardiectomy.
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