Constrictive pericarditis presented by generalized edema (anasarca).
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Biomedical subjects
Publications and source records attributed to M Frand.
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Left superior vena cava (LSVC) to the left atrium is a rare congenital cardiac complex, which may appear as an isolated anomaly, or as part of more complex cardiac anomalies. Traditionally, an intraatrial baffle was the preferred surgical technique. Although this technique has proved reliable and successful, acute ligation and extracardiac repair are simpler and easier solutions, requiring less myocardial ischemic time. We present 3 patients who underwent simple ligation and discuss the literature for other extracardiac options of surgical repair. Our patients had short transient congestion in the left upper part of their body that resolved completely after a few weeks, without further complications. We believe that either acute ligation or extracardiac repair is a much simpler yet effective solution to divert the left caval flow to the lesser circulation.
The incidence of congenital heart defects is 0.8% of liveborn infants. Of these 13% are ostium secundum atrial septal defects (ASD) which can be successfully repaired by open heart surgery with less than 1% mortality. However, morbidity associated with cardiac surgery is universal. Transcatheter closure of ASDs was introduced 2 decades ago to decrease surgical complications, hospital stay and cost. Few devices have undergone clinical trials but all of them have been associated with instances of failure and complications. The most important mechanism for acute failure was selection of cases with too large a defect or selection of a defective device. In the past decade great progress has been made with the aid of transesophageal echocardiography (TEE) and modifications of the device. We present our experience in our first 5 patients. They ranged in age from 4 to 27 years; the ASD diameter was 11-15 mm and the device diameter was 17-33 mm. All transcatheter closures were performed on the same day without any complications, and all patients were discharged home after 2 days of hospitalization. We conclude that transcatheter occlusion of ASDs up to 15 mm is feasible, relatively safe and effective. This transcatheter method appears to be a viable alternative to surgery for some patients with secundum atrial septal defects.
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Adenosine has been approved for intravenous use for paroxysmal supraventricular tachycardias (SVT) in adults and children. However, effectiveness and safety of intravenous adenosine in preterm infants are not well established. Thirteen episodes of SVT in three preterm and two full-term neonates were treated with intravenous adenosine. All had narrow QRS tachycardia at 230 to 260 beats/min. Adenosine prepared as a sterile 1 mg/mL solution was given as an intravenous bolus starting at 0.05 mg/kg, and increased by 0.05 mg/kg until tachycardia was terminated. Termination of tachycardia was achieved within 12 to 25 seconds in all patients. In one, termination of SVT was followed by temporary suppression of the sinus node, followed by resumption of normal sinus rhythm. No other side effects were noted. Adenosine is a safe and effective agent for treating preterm infants with SVT. However, further investigation of adenosine in this group of patients is warranted.
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Two-dimensional color-coded Doppler examination revealed a cranial arteriovenous (AV) malformation in a critically ill newborn with intractable congestive heart failure. This case emphasizes the value of color-coded Doppler in the diagnosis of this rare malformation.
Cerebrovascular accident (CVA) complicating cyanotic congenital heart disease (CCHD) is most frequently attributed to cerebral venous thrombosis, only rarely to in situ cerebral arterial thrombosis. Cerebrovascular accident due to paradoxical embolization from an inferior vena cava (IVC) thrombus in a patient with tetralogy of Fallot (TF) is described. This case is the first antemortem documentation of a venous embolic source that caused paradoxical embolization to the brain in a patient with CCHD, and illustrates the importance of noninvasive screening for a venous source of emboli in these patients.
4 patients with enlarged tonsils and adenoids associated with pectus excavatum are presented. 2 also had signs of pulmonary hypertension and congestive heart failure. All 4 underwent adenoidectomy and tonsillectomy. Respiratory distress and congestive heart failure were rapidly relieved, while the pectus deformity improved gradually and completely disappeared in 6 months to 2 years after surgery.
The performance of cross-sectional echocardiography on every child with a heart murmur poses a heavy load and economic burden on pediatric cardiology services. We prospectively studied 100 consecutive patients newly referred to our clinic for evaluation of a heart murmur. After a thorough clinical assessment and review of the electrocardiogram and chest radiograms, 50 patients were diagnosed as having "innocent murmurs". Cross-sectional echocardiography confirmed the initial diagnosis in 48 patients (96%). Two patients had bicuspid aortic valve. Among the patients with echocardiographically proved innocent murmur, left ventricular anomalous bands were demonstrated in 15 patients (31%), right ventricular anomalous bands in 5 patients (10%) and increased left ventricular shortening fractions (greater than or equal to 0.5) in 10 patients (21%). Among the remaining 50 patients, the initial diagnosis was "suspected heart disease" in 10 patients, and "pathological murmurs" in 40 patients. Cross-sectional echocardiography was most useful in these patients providing the diagnosis in 80% (eight) of the former group and 95% (38) of the later group. Echocardiography need not be routinely performed in children initially diagnosed, by a pediatric cardiologist, as having an "innocent murmur".
Thirty-five episodes of infective endocarditis in 35 children with congenital heart disease, from January 1965 to December 1984, were reviewed. The incidence of infective endocarditis in cyanotic congenital heart defects increased with a concomitant decrease in the frequency of lesions with left to right shunt. The incidence of postoperative endocarditis decreased from 1:52 during the first decade to 1:1033 during the second decade. The time interval between onset of symptoms and the establishment of diagnosis and treatment shortened from 7.8 +/- 3.2 weeks (mean +/- SD) to 2.1 +/- 0.9 weeks. During the latter decade, positive blood cultures were obtained in 100% of patients, as compared with 60% during the former decade (P less than 0.05). Echocardiography demonstrated vegetations in 70% of the cases. Over the entire time period, streptococcus viridans was the most frequent pathogen (46%) and staphylococcus aureus second most frequent (17%). Six patients died, all were under two years of age and all had congestive heart failure. Early surgical correction of the congenital heart disease may offer the best form of prevention.
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The clinical and echocardiographic manifestations in 25 patients with Marfan's syndrome diagnosed during infancy and childhood (mean [+/- SD] age, 8.1 +/- 4.8 years; range 0 to 16 years) were evaluated. Twenty-one patients (84%) had a midsystolic click, 11 patients (44%) had mitral regurgitation (MR), and five patients (20%) had combined MR and aortic regurgitation (AR). Echocardiography demonstrated mitral valve prolapse in all 25 patients, aortic root dilatation in 20 patients (80%), AR in seven patients (28%), and aortic aneurysm in five patients (20%). During the follow-up period (mean, 5 +/- 4.5 years), progressive AR and aortic aneurysm were documented in four patients, progressive MR in three patients, and progressive aortic root dilatation in two patients. Five patients (22%) died during the follow-up period. Among patients with a positive family history of Marfan's syndrome, MR was less frequent as compared with sporadic cases (29.4% vs 75%, respectively). Progressive cardiovascular involvement was more frequent among patients diagnosed before 10 years of age compared with those diagnosed later (60% vs 12.5%, respectively). Cardiovascular involvement was a common feature of childhood Marfan's syndrome, causing significant morbidity and mortality. Sporadic cases and children diagnosed before 10 years of age represented a particularly high-risk group.
Ehlers-Danlos syndrome (EDS) is clinically and genetically a heterogeneous disorder of connective tissue. Eleven different types of EDS have been documented, several of which have major cardiovascular complications as part of their clinical manifestations. The purpose of this report is to call attention to a form of EDS with minimal external features but severe internal vascular complications.
Fourteen infants with congenital heart disease were investigated for failure to thrive. Assessment of intestinal function revealed minor absorptive abnormalities (mild steatorrhea in three patients, bile salt loss in four patients), delayed gastric emptying, and abnormal triglyceride loading tests. Low caloric intake (88.3 +/- 19.3 kcal/kg/day) seemed the main reason for failure to gain weight. Weight accession and cardiorespiratory rates were monitored daily during voluntary intake, a high-caloric diet by mouth, and nasogastric tube feeding. Providing 169 +/- 29 kcal/kg/day by tube resulted in weight gain with mild and transient elevation of respiratory rate at the end of the meal and increased heart rate 90 min after the meal. This regimen is a metabolically inexpensive and efficient method of supporting weight gain in children with congenital heart disease.
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Thirty-two pediatric patients with invasive Hemophilus influenzae type B (HITB) infections were evaluated according to the frequency of resistant strains and the clinical profile. The incidence of resistant strains was 28% (9/32), all of them due to beta-lactamase-producing bacteria. The rate of ampicillin resistance was significantly higher among patients who had received beta-lactam antibiotics. The initial treatment of HITB infections in our region should therefore include chloramphenicol; the indiscriminate day-to-day use of beta-lactam antibiotics should be reconsidered.