[Complete congenital atrioventricular block. Presentation of 20 cases and review of the literature].
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Biomedical subjects
Publications and source records attributed to E Pastor.
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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.
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.
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The authors present their experience on fast atrial rhythms. Fourteen patients were studied in the first three months of life, nine of them having congestive heart failure. The electrophysiological basis of the reentrance tachycardia at A-V junction is discussed. The pharmacological treatment is reviewed, emphasizing the usefulness of verapamil. They comment on the good prognosis of the disorder, provided there is not associated congenital heart disease.
We have studied 14 patients of corrected transposition, 12 with situs solitus and 2 with situs inversus. In all the patients, excepting one, a clinical, radiological and angiocardiographical study was performed. The associated anomalies were determining elements of the clinical situation; the patients with pulmonary obstruction and interventricular communication had cianosis, while the 4 patients with aortic obstruction or anomalies type left Ebstein show cardiac insufficiency. The simple radiological study helped us to determinate the ascendent aorta in the left side of the heart, in 9 cases. It was unusual to see the droped hilium in the right hemitorax. The more significants electrical signs were: The presence of complete A-V block, Q-waves in D2, D3 and aVF, absence of right waves in precordial leads in most of the patients and a negative T wave in a VL. Summarizing, authors believe that in this type of defects selective injection of contrast in both ventricles is the best method to establish the difference between corrected transposition and other cardiopathies with the aorta situated at the left side of the pulmonary artery.
To know how patients perceive the information leaflets about medicines, it has been carried out a study in a sample of 65 patients admitted to a hospital for chronic respiratory or cardiac diseases. A 47.7% of patients expressed that the main information provided by the leaflet was such concerning to the purpose of the drug, whereas a 18.5% pointed out such concerning to side effects and management of unexpected circumstances. Sixty three percent of patients expressed that leaflets did not provide them new information. A relevant percentage of patients did not know the meaning of some terms frequently used in leaflets and package-inserts (80% of mistakes in the interpretation of "take the drug on an empty stomach"). It is concluded that the information leaflets can be useful in the framework of a good physician-patient relationship, as a reminder, and to reinforce the knowledge that the patient already has. It is considered that the most appropriate time for the education of chronic patients about drugs is when the treatment is prescribed for the first time.
We report on a newborn infant with right ventricular diverticulum associated to dilated myocardiopathy, who was diagnosed by two-dimensional echocardiography within 24 hours after vaginal delivery. We believe this case represents the youngest one ever diagnosed with this disease.
Eight patients (4 male, 4 female) with persistent truncs arteriosus diagnosed by two-dimensional echocardiography are presented. Age ranged from 1 to 19 days. Diagnosis was based on the demonstration of a single vessel giving rise to the aorta, pulmonary arteries and coronary circulation. In 6 patients we were able to define the types of the truncus: in 4 patients corresponded to type I (with partial presence of aorto-pulmonary septum) and in 2 cases corresponded to type II (without existence of septum). All cases had a dilated truncus (1.13 +/- 0.06 cm, range 1.05-1.3 cm), with moderate overriding (52% range 30%-65%) and an infundibular ventricular septal defect (0.6 +/- 0.08 cm, range 0.45-0.85 cm). In 2 cases we could also demonstrate the presence of an interruption of the aortic aorta. We conclude that two-dimensional echocardiography (specially parasternal long-short axis views and subcostal for right or leftout flow tracts axis views) gives sufficient information about this cardiac malformation, which can be completed by the use of pulsed Doppler ultrasound.
Mitral valve prolapse is frequent in childhood. The use of two-dimensional echocardiography may enable more accurate diagnosis and assessment of the degree of valve involvement. Twenty five (1.9%) of all children studied by two-dimensional echocardiography fulfilled the diagnostic criteria for mitral valve prolapse. In 48% it was associated to a different congenital heart as normality. The apical four chamber and parasternal long and short axis views were used, and cases were graded according to the severity of the prolapse. Diagnosis was made in 44% of cases by the apical four chamber view, which was the best projection to detect the abnormality. Cases of moderate and severe prolapse were also detected in the parasternal long axis view. All patients were asymptomatic but they were all controlled detect progression to mitral insufficiency of the appearance of other complications.
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