A familial syndrome of pericarditis, arthritis, and camptodactyly.
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Biomedical subjects
Publications and source records attributed to A Zghaib.
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Congenital absence of pulmonary valve leaflets is an uncommon condition usually associated with ventricular septal defect and an obstructive pulmonary valve ring. Twenty-one patients with these malformations are described. Twenty had an associated ventricular septal defect with ventriculoarterial concordance, and one also had transposition of the great arteries, ventricular septal defect, and obstructive pulmonary valve ring. The clinical features, cardiac catheterisation findings, and angiocardiographic results are presented. Twelve patients underwent cardiac surgery. Three patients died, one in the early, and the other two in the late postoperative period. The results, according to the surgical technique employed and postoperative cardiac catheterisation findings, showed that patients in whom the bioprostheses were implanted in the pulmonary position had a better late follow-up.
We report 41 patients with myocardial infarction who were less than forty years old and that had been studied by coronary angiography. 97.5% were male mostly in their thirties. Coronary risk factors in this group were similar to the old one; excepting for mental stress present in 75% of our patients. There was not predominant infarction site. We observed different disturbances of the cardiac rhythm but no patient had congestive heart failure or cardiogenic shock. Mortality due to the infarct itself was none .61% of the cases had univascular lesions or normal coronary angiography and only 12% had trivascular lesions. The patients with normal coronary angiography had no significant difference in the mayor coronary risk factors and in our group we found patients with arterial hypertension, hyperlipidemia, cigarette smoking and obesity. We suggest that mental stress is an important coronary risk factor; the evolution of these patients is favorable and the mortality is low as compared with previous reports.
The natural history of valvular pulmonary stenosis is modified by the surgical treatment of these patients. In order to assess these changes we studied the clinical and hemodynamic features of 91 patients that were treated surgically. Hemodynamic studies were performed postoperatively in 62 of them. All patients had preoperatively a right ventricular systolic pressure above 70 mm Hg and were divided into three groups according to the transvalvular gradient. The first group had less than 50 mm Hg, the second group between 50 and 80 mm Hg, and the third group more than 80 mm Hg gradient. Correlation of these values with asymptomatic (49 patients) and with symptomatic (42 patients) cases showed significant values (p less than 0.001). Postoperatively only 2 patients remained with mild degree effort dyspnea. There was also a significant correlation (p less than 0.005) between the systolic gradient and the cardiothoracic ratio in the studied groups. The T wave in lead VF showed a predictive value in relation to the systolic gradient (p less than 0.001). Reduction of the systolic gradient after surgery showed very significant values when compared to the preoperative gradient (p less than 0.001). The overall mortality was 5.5%.
he recent advances in surgical treatment of congenital heart disease, permits the survival of those cases to adult life. Infective endocarditis in the childhood is becoming increasingly important. We studied 32 cases in the pediatric cardiology ward at the INC between 1977 and 1981. There was a male predominance of 62.5%. Rheumatic heart disease (40.7%), congenital heart disease (15.6%) and postoperative cases (43.7%) were the underlying conditions. There was an average time of 43 days delay from the first symptom to diagnosis. Blood cultures were taken in 29 cases (90.6%). Only half of them were positive. Staphylococcus and streptococcus were the commonest isolates. Peripheral and pulmonary embolism (11 cases-34.4%) were the most frequent complications. In those cases with negative blood culture the most successful antibiotic combination was ampicillin or oxacillin plus aminoglucosides (56%). There were 17 deaths (53%).
Due to the lack of specificity of the clinical picture in the right-sided infective endocarditis, the correct diagnosis is rarely made. We reviewed 30 cases with right-sided or right and left infective endocarditis, treated in the INC from 1946 to 1982. The average age was 20 years. Rheumatic fever (53%), congenital heart disease (40%) and cardiac prostheses (7%) were the more common underlying diseases. The diagnosis was made on an average 7.3 months after the first symptom. Heart failure (93%), fever (76%), weight loss (73%), haemoptysis (66%) and general malaise (53%) were the predominant symptoms. There was no diagnostic suspicion in 9 patients (30%) and in 7 from 16 with negative blood culture, the infection was exclusively right-sided. Peripheral and pulmonary embolism was the most frequent complication. (66%) There were 29 deaths (96.6%). In all of them the diagnosis was confirmed in the postmortem examination. Heart failure and septic shock were the main causes of death. Almost all patients were infected with gram-negative germs and staphylococcus Aureus. This diagnosis should be suspected in a patient with known heart disease, who develops unexplained heart failure, moreover if pulmonary emboli are a feature. The diversity of the isolated germs is different from other publication that have shown staphylococcus as the most prevalent microorganism. This difference can be explained by the lack of drug abuse in our cases. The mortality rate is higher than in the left sided endocarditis.
We describe two patients with under-and-over ventricles. Both were diagnosed angiographically. One has atrial, situs solitus with discordant atrioventricular and ventriculoarterial connections and a ventricular septal defect. The second has atrial situs inversus with concordant atrioventricular connections and double-outlet right ventricle. We review the literature and analyze the probable embryogenesis of the malformation.
We report two cases with double outlet right ventricle and subaortic restrictive ventricular septal defect. Both had atrial situs solitus and atrioventricular concordance. There were no other associated defects. Both cases were diagnosed by means of an angiocardiographic study and case two was also studied anatomically. Additional data for the diagnosis were findings of left ventricular overload by the EKG; left atrial enlargement and signs of venocapillary hypertension by the chest X-rays; left ventricular/right ventricular pressure gradient by the hemodynamic study and the angiographic findings. One case had mitro-aortic discontinuity. Case two had a double infundibulum, being the left one the responsible for the restrictive ventricular septal defect. We conclude that double-outlet right ventricle with restrictive ventricular septal defect cases have their own clinical, hemodynamic and angiographic features which have to be accurately studied in order to offer an adequate surgical treatment.
We are reporting a case of Ebstein's anomaly of the tricuspid valve and another case of a congenital double tricuspid lesion, both associated with pulmonary valve stenosis and with similar hemodynamics and physiopathology. Given that there are few reported cases in the literature, we analyzed the clinical and electrocardiographic findings of both. The utility of the echocardiogram is discussed in the diagnosis of such abnormalities and the angiographic and hemodynamic data commented upon. It is emphasized that right ventricular hypertension (105 mm Hg, and 98 mmHg, respectively) modifies the cardiopathic hemodynamics and its natural history, that was favorably modified by isolated corrective surgery of the pulmonary obstruction. The postoperatory transvalvular gradients were measured at 27 and 11 mm Hg, respectively. After having revised the pertinent bibliographic literature, we commented upon the factors that give such a high mortality rate in Ebstein's surgery and the congenital abnormalities which are most frequently associated.
Four cases of hypoplastic right ventricle with intact ventricular septum are presented. All of them were diagnosed by means of an angiographic study, and one case was examined postmortem. The anatomic, physiologic and clinical features of this malformation are analyzed and the differential diagnosis with similar congenital cardiac diseases is discussed.
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We describe the cardiovascular abnormalities found at autopsy in patients with AIDS and a description of the opportunistic infections in these cases studied between January 1988 and August 1993. There were 51 cases with such diagnosis. Pericardial effusion appeared in 9, pleural effusion in 7, myocarditis in 5, 7 with pericarditis, endocarditis in 6, left ventricular hypertrophy in 20, right ventricular hypertrophy in 21 and evidence of atherosclerosis in 15. Thus, data of cardiovascular damage was present in 42.7% of our patients. The cardiovascular abnormalities in this group are common, in contrast to the paucity of clinical findings. Diagnosis of cardiac pathology was made in only 12% of them. So in every case with diagnosis of AIDS, a careful clinical examination and cardiac diagnostic oriented tests must be done for detection of these abnormalities.