[Aortic atresia with normal left ventricle. Echocardiographic and anatomical study of a case].
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Biomedical subjects
Publications and source records attributed to A Cabrera.
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Three cases of congenital cerebral arteriovenous malformation are presented. They all developed congestive heart failure in the first day of life. The main clinical findings were a continuous murmur heard over the scalp, an ejection murmur at the upper left sternal border, gallop rhythm, cardiomegaly and hepatomegaly. Diagnosis was confirmed in all by carotideal arterography. Two had aneurysm of the vena cerebri interna Galeni and in the other, the aneurysm was localized in the area irrigated by the sylvian artery. The two former died after surgery was considered not feasible. The latter had his malformation resected. Two years after operation he is doing well.
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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Seven cases of isolated atrial septal defect, "ostium secundum" type, are presented. All had clinical symptoms of congestive heart failure and represented five per cent of the cases studied in our department with atrial septal defect. In three patients associated extracardiac anomalies were found. All had cardiomegaly, increased pulmonary vascularity, right ventricular hypertrophy and splitted second sound. Echocardiographic findings were: paradoxal movement of the interventricular septum and ventricular dyastolic overflow. Medium left to right shunt was 3.08. In four cases a massive filling of the right atrium was obtained, through, the selective injection of contrast material, in the pulmonary artery. None of the patients, responded to medical treatment alone and five of then went to surgery with significant improvement, in their clinical manifestations, and normal growth and weight curves in their follow-up. Importance in survival of early surgery is emphasized.
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Total anomalous pulmonary venous drainage is one of the cardiac malformations requiring early surgical correction. In our series of nine cases, 89% were under three months of age and none above six months. Clinically, two types were present: without obstruction and with obstruction to the pulmonary venous flow. In the non obstructive group, all had mild cyanosis, heart failure, cardiomegaly and systolic murmur. In the obstructive group, they had severe cyanosis, heart failure, small heart and pulmonary fields with a "ground glass" or mottled appearance on chest X-ray due to hypertension and edema. The second heart found was split in both groups. An increased right sided O2 saturation and pulmonary hypertension are due to the total anomalous pulmonary venous connection to the right atrium. Right atrium pressures were superior than left atrium pressures in two patients, requiring atrioseptostomy in one patient. Total surgical correction was performed in three patients, with one patient surviving; this patient is well one year after operation. It is noteworthy in our series the early symptomatology. Symptoms begun at birth in 77% of the cases. It was striking as well the absence of cases with drainage to the right atrium or the coronary sinus.
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Four cases of congenital pulmonary lymphangiectasis were observed during a five year period. This represents an incidence of 1.11 per 10,000 cases among alive newborns and of 53 per 10,000 cases among pediatric necropsias performed during the same period. One case was observed in the clinical context of a generalized hemangiolymphangiomatosis, another was associated to tetralogy of Fallot and the remaining two cases were associated to obstruction of pulmonary venous return. Although one of the pathogenic theories generally accepted in the formation of pulmonary lymphangiectasis points to the presence of either hypertension or obstruction of pulmonary venous drainage, it is possible that such situation is only circumstantial. Among 40 personal cases of obstruction of pulmonary venous return proved anatomically, only in the two cases presented were pulmonary lymphangiectasis demonstrated. The presence of dysplastic elements in pulmonary tissue in cases of lymphangiectasis suggests that a more possible mechanism is a primary defect in the development of pulmonary lymphatics.
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Two cases of aberrant left pulmonary artery are described. One of them died on the third day of life and had esophageal atresia and Fallot tetralogy as associated anomalies. Outstanding symptoms of this anomaly were bouts of respiratory difficulty with espiratory wheezing. During these episodes radiographic findings were right or bilateral lung hyperaireation and middle lobe atelectasis. Upper respiratory infections usually preceded these bouts of respiratory difficulty. Diagnosis was established by the presence of a notch on the anterior wall of the esophagus, diminished pulmonary perfusion of the left lung as shown by pulmonary gammagraphy and characteristic findings in pulmonary arteriography. During the last 12 months the first case, now aged three years, has had a spontaneous regression of symptoms.
Twenty-nine consecutive spontaneous attacks of paroxysmal supraventricular tachycardia (PSVT) in 14 infants (mean age 4.4 months) were treated with verapamil. No infant had associated heart disease. Verapamil 1-2 mg i.v was administered over 30 seconds. The dosage varied according to the weight of the infant. Within 60 seconds sinus rhythm was obtained in 28 instances (96.5%). No significant complications were observed. The high effectiveness, rapid action and lack of undesirable side effects observed in this series suggest that verapamil is the drug of choice in the treatment of PSVT in infants without underlying heart disease.
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Authors present the results of angiocardiographic study on 30 patients with complete A-V canal. This study is divided in two parts: First, they define general concepts such as aortic suspension, anterior common valve-theoretical right border angle, left and right regurgitation, etc. Secondly, they classify A-V canal in two groups according to the following characteristics: 1) Anterior common valve normal, moderate suspension of the aorta less than 50%, rough right border of the left ventricle, angulation less than 45 degrees, medial interventricular communication and left regurgitation. 2) Common anterior valve small, important suspension of the aorta greater than or equal to 50%, smooth right border, angulation greater than or equal to 45 degrees, large communication and left and right regurgitation. Differential data between interventricular communication and valvular regurgitation are commented. They emphasize how important the posterior smooth septum and the common anterior valve are in producing the suspension of the aorta and also in the narrowing and elongation of the infundibulum. Finally, some considerations about the surgical implications according to the type of canal are made.
We report a case of aortic atresia with biventricular hypertrophy and death due to cardiac insufficiency (at 5 1/2 days of age). In the necropsy a normally sized left ventricle was observed, with endocardiac fibroelastosis, anomalous mitral arcade, intact interventricular septum and atresia of the aortic valve. The ascending aorta was moderately hypoplasic; there was a foramen ovale and persistent ductus arteriosus. We believe that this is the first case that has been reported of this anomaly occurring with a normal left ventricle and intact ventricular septum.
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