Biomedical subjects
Publications and source records attributed to E Pastor.
We report the first case of a child with an aortic rupture into the pericardial cavity secondary to a dissecting aneurysm developing in the context of aortic coarctation. The combination was diagnosed by cross-sectional echocardiography.
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Between February 1983 and December 1985 authors have studied 26 newborn infants with severe congenital heart disease who received prostaglandin E1 infusion as therapy. Onset of therapy was before 5 days of age in 15 patients, between 5 and 7 days of age in 7 cases and after 14 days of age in the remaining ones. Initial dose was 0.05 mcg/kg/minutes and mean duration of infusion was 131.9 +/- 19.0 hours. Patients were divided into 3 groups according to pulmonary and systemic blood flows: Group I, with cyanosis and diminished pulmonary blood flow (12 cases); Group II, with diminished systemic blood flow (10 cases); and group III, with cyanosis and normal or increased pulmonary blood flow (4 cases). Most patients of groups I (10/12) and II (9/10) improved levels of blood PO2: mean increments of 17.8 +/- 7.4 and 17.5 +/- 13.3 torr respectively. Peripheral pulses also improved in four extremities in patients from group II; systolic and diastolic pressures increased 16 +/- 9.6 and 5 +/- 2.2 mmHg, respectively. No improvement was observed in patients from group III, with exception of patients with transposition of great vessels. Most frequent complication of prostaglandin infusion was hyperthermia, which was observed in 16 cases but disappeared in all with moderate reduction of doses. Appropriate surgery was performed in 19 patients, with an overall survival of 52.6%. In conclusion, prostaglandin infusion appears to be a very valued tool to improve the hemodynamic condition in newborn infants with severe congenital heart disease and thus allow the practice of corrective surgery during first month of life.
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Sixty-four preterm infants (birth weight 1,393 +/- 483 g g and gestational age 30.1 +/- 2.7 weeks) developed a symptomatic ductus arteriosus, and were treated with intravenous indomethacin. Mechanical ventilation was needed by all of them, and 98% had IRDS. Pharmacologic ductus closure rate was 87%. Sixty-four per cent of patients had a permanent closure after first trial, and 74% after a second course of the drug. Ductus closure rate was found to be greater in patients with higher birth weight and gestational age. It was also higher in patients treated in the first week of life. Closure rate was higher in this group of patients treated with intravenous indomethacin, than in a previous group of a similar birth weight and gestational age, who received the drug by oral route (87% versus 64%). Incidence of necrotizing enterocolitis was found to be similar in both groups. Follow-up data showed that four infants (8.5%) had severe neurological sequelae, and another three cases (6.4%) developed severe respiratory problems.
We present the pathological study of 17 cases of mitral atresia with patent aortic valve and their surgical implications. The study is based on the type of atrioventricular connection, presence or absence of ventricular septum, size of the left ventricle and relation of the aorta with the aforementioned ventricle. We have classified mitral atresia into two groups by assessing these data. Group A) Both ventricles and atrioventricular connections are present and there is ventricular septal defect. The aorta may connect with an either normal or hypoplastic left ventricle or it may emerge in double outlet from the right ventricle. Group B) Absent left ventricle and atrioventricular connection. The aorta emerges from the single ventricle or the rudimentary chamber. Thirteen cases belonged to group A and four to group B. The pathological structure of mitral atresia requires the decompression of the left atrium by atrioseptostomy during the neonatal period to enable survival. On a second stage a palliative correction should be carried out, which; depending on ventricular size and outlet of the vessels, will be a valvular prosthesis left atrium-left ventricle or the modified atriopulmonary technique connecting the new left atrium with the systemic ventricle and aorta after resecting the auricular septum and connecting the right atrium with the pulmonary artery.
Right ventricular hypoplasia without additional congenital heart disease is an uncommon abnormality. It is characterized by partial absence of right ventricular inflow tract, both tricuspid and pulmonary valves being normally shaped. We report three cases; a newborn, an infant and a twelve years old boy. All of them had cyanosis and right atrial enlargement and both of the younger patients had electrocardiographic signs suggesting right ventricular hypoplasia. Right atrial mean pressure were raised. A differential diagnosis should be stablished with all sorts of right ventricular hypoplasia with cyanosis and with restrictive myocardiopathyes. Surgery for symptomatic patient should be palliative during first months of life and corrective from age one year onwards.
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Authors present angiocardiographic study of 19 patients with univentricular heart. It was realized during the neonatal period in 13 cases and between the six first six months in the rest. To determine the existence of an only ventricle contrast was injected in the right and left atrium and the ventricle through the right and left atrioventricular valve. The bulbo-ventricular foramen was orientated in all cases from back-front wards. Those of left ventricle type had a right anterior, anterior-superior or left-anterior rudimentary chamber. Depending on its' morphology they were classified in three types: left, right or indeterminate. In these series fourteen patients had left ventricle morphology. All cases had a rudimentary chamber: 10 with transposition great vessel and four with normal related great vessels. Six presented normal situs (five with levocardia , and eight ambiguous situs (six with asplenia, four with dextrocardia). In nine atrio ventricular valves were anomalous. In the indeterminate type, two had a normal situs, two with polysplenia and two asplenia. Cardiac apex was oriented to the right in two (one asplenia). The two patients with polysplenia and one with normal situs and single atrium had a common ventricular valve. Four had an anterior aorta and three pulmonary obstruction.
Surgical correction of pulmonary artery sling with vascular ring in a 2 1/2-year-old boy is described. Despite the high mortality rate of this entity, the post-operative course was uneventful and since hospital discharge, the boy has remained asymptomatic.
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We describe 13 cases of Ebstein's anomaly of the tricuspid valve. Seventy percent of there cases were detected at one month of age. Ten patients died and 9 presented associated cardiac anomalies. Signs of right heart failure were the clinical date that made possible the diagnosis of the disease in the most severe cases in the neonatal period. Only two patients had alteration of the cardiac rhythm: One had tachycardia that ended with ventricular fibrillation and the other had a 2:1 heart block. Echocardiography was very useful to establish the diagnosis: one hundred percent of the patients showed a delay in closing of the tricuspid in relation to the mitral valve, with a range of 0,03 to 0,06 seconds. Angiocardiographic studies revealed a tricuspid valve displacement in 8 cases, and double contour image in 6. The septal and posterior leaflets were hypoplasic in the 10 anatomic cases and in 80 percent of them it was possible to differentiate the cords from the papillary muscles.
Sixty-one preterm infants (birthweight, 1.529 +/- 450g; gestational age, 31,9 +/- 2,4 weeks) developed a large DAP. Seventy-eight per cent required mechanical ventilation and 75,5% had hyaline membrane disease. Pharmacological closure of the DAP with oral indomethacin was attempted on 31. A steady improvement was achieved in 64,5%. The postnatal age of responders was lower than that of non-responders. Neonatal survival was over 90% but long-term survival dropped to 64,5% linked to a high incidence of bronchopulmonary dysplasia. The patients with bronchopulmonary dysplasia had received indomethacin later than the others. It was found that postnatal age at the time of indomethacin administration correlated with the days of mechanical ventilation later required by the survivors.
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Authors described three infants under three months of age with cardiac rhabdomyoma observed during a period of eight years. There was no clinical or pathological evidence of tuberous sclerosis. During this period of time they found two cases of tuberous sclerosis, without associated rhabdomyoma, among 2,457 necropsies. On the other hand none of 11 children with tuberous sclerosis under observation in this centre had any clinical evidence of rhabdomyoma. Presenting symptoms of the three cases were cyanosis, heart failure, heart murmurs and alterations of heart rhythm. Diagnosis was suspected on the bases of eco and angiocardiographic studies and was finally confirmed by necropsy. Surgical exeresis of the tumor was attempted in one of the cases, but was not possible because it was too large.
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