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• W2080223048 abstract "Pulmonary atresia with intact ventricular septum is a rare congenital cardiac lesion comprising less than one per cent of all congenital cardiac abnormalities (7). Nevertheless, in an infant who is cyanotic and in distress during the neonatal period this condition is more likely to be present than tricuspid atresia or tetralogy of Fallot (8). The major anatomical abnormality is fusion of the pulmonary valve cusps into a membrane which does not allow blood to leave the right ventricle in the usual manner. The systemic return to the right atrium is shunted into the left atrium through a patent foramen ovale or an atrial septal defect. The left ventricle pumps into the systemic circulation and also into the lungs by way of a patent ductus arteriosus. Clinically, there are neonatal cyanosis and dyspnea. No murmurs are heard in most cases. Occasionally, a systolic murmur may be produced by tricuspid insufficiency or the patent ductus arteriosus. The second heart sound is single and is produced by closure of the aortic valve. The plain roentgenograms show ischemic lungs and a variable degree of cardiomegaly. Right atrial enlargement is frequently apparent. Although the left atrium, left ventricle, and aorta are enlarged, this is usually not obvious on the plain roentgenograms. The main pulmonary artery is small, resulting in concavity of the left upper cardiac border. The appearance is not readily distinguishable from tricuspid atresia or Ebstein's malformation of the tricuspid valve. Angiocardiographically and anatomically, two varieties of this lesion have been described (1, 2). They have been designated as Types I and II. In Type II, the tricuspid valve is not competent. The right ventricle is of normal size or enlarged. In Type I, the tricuspid valve is competent. The wall of the right ventricle becomes greatly hypertrophied, while the right ventricular cavity is diminutive. These changes are due to the greatly increased pressures which develop inside this chamber. It is in Type I cases that intramyocardial sinusoidal channels have been demonstrated. These have a characteristic appearance on the angiocardiogram. No description of these sinusoids could be found in the radiological literature. The purpose of this report is to describe two cases in which intramyocardial sinusoids were found and to discuss their significance. Case Reports Case I: A 5-week-old girl had cyanosis of the hands and feet at birth, and subsequently upon crying. Generalized cyanosis and respiratory distress developed two days prior to admission. The respiratory rate on the day of admission was 80. No murmurs were heard. The peripheral pulses were normal, and the liver was not enlarged. The electrocardiogram disclosed left ventricular hypertrophy. Roentgenograms of the chest revealed the heart to be within normal limits in overall size with prominence of the right atrium." @default.
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• W2080223048 date "1966-03-01" @default.
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• W2080223048 title "Myocardial Sinusoids in Pulmonary Valvular Atresia" @default.
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• W2080223048 doi "https://doi.org/10.1148/86.3.421" @default.
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