Eli Zalzstein

Aortic dissection in children and young adults: diagnosis, patients at risk, and outcomes.

OBJECTIVE To heighten the awareness of pediatricians and pediatric cardiologists to aortic dissection, a potentially dangerous medical condition. METHODS We reviewed the charts of 13 patients, seen in four medical centers, who suffered acute or chronic aortic dissection over the period 1970 through 2000 whilst under the age of 25 years. RESULTS There were seven male and six female patients, with the mean age at diagnosis being 12.1 years, with a range from one day to 25 years. Congenital cardiac defects were present in five patients, and Marfan syndrome in four. In three of the patients with congenital cardiac defects, aortic dissection developed as a complication of medical procedures. In three patients, dissection followed blunt trauma to the chest. We could not identify any risk factors in one patient. The presenting symptoms included chest pain in four patients, abdominal pain and signs of ischemic bowel in two, non-palpable femoral pulses in one, and obstruction of the superior caval vein in one. Angiography and magnetic resonance imaging were the main diagnostic tools. Overall mortality was 38%. Only six patients had successful surgical outcomes. CONCLUSION Due to the rarity of aortic dissection a high index of suspicion is required to reach the diagnosis in a timely manner. It should be considered in young patients complaining of chest pain in association with Marfan syndrome, anomalies of the aortic valve and arch, and chest trauma.

Advanced atrioventricular conduction block in acute rheumatic fever

We carried out a retrospective case control analysis to evaluate the outcome, and the need for treatment, of problems with atrioventricular conduction occurring during an acute attack of rheumatic fever, assessing the occurrence of second and third atrioventricular block versus first degree block. We reviewed and analysed the clinical, electrocardiographic and echocardiographic records of all children diagnosed in a single institute as having acute rheumatic fever during a period of seven consecutive years. During the period from October, 1994, through October, 2001, 65 children meeting the modified Jones criterions for acute rheumatic fever were hospitalized in the Soroka University Medical Center, Israel. First-degree atrioventricular block was identified in 72.3% of the children, and resolved with no specific treatment other than non-steroidal anti-inflammatory medications. Second-degree atrioventricular block of Mobitz type I, was observed in one child (1.5%), which progressed from first-degree block, and subsequently resolved. Complete atrioventricuar block was found in 3 children (4.6%), one progressing from Mobitz type I second-degree block, and two being seen as the first presentation. Of the three children with complete atrioventricular block, one patient was not treated, the second was treated with aspirin, and the final one with combined aspirin and steroids. The disturbances of conduction resolved in all three. We conclude that advanced atrioventricular block is rare during acute rheumatic fever. If occurring, block appears to be temporary, and resolves with conventional anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when syncope or clinical symptoms persist.

Successful treatment of infective endocarditis with recombinant tissue plasminogen activator

OBJECTIVES In a prospective study, we examined the effect of treatment with recombinant tissue plasminogen activator (r-TPA) on survival and morbidity in a series of high-risk children with infectious endocarditis (IE) after prolonged treatment with indwelling catheters. We hypothesized that r-TPA is an adjunctive therapy for dissolution of infected thrombi in drug-resistant IE. STUDY DESIGN In the prospective 3-year study (1998-2001), we identified high-risk children with chronic illness and prolonged treatment with indwelling catheters who developed IE and overwhelming sepsis. Patients were allocated to receive r-TPA after persistent and enlarging intracardiac vegetations and failure to respond to conventional medical management. Complications associated with treatment, survival, and cardiac morbidity were observed. RESULTS Seven infants were treated prospectively with r-TPA. All infants responded promptly to treatment, with resolution of the intracardiac vegetations within 3 to 4 days of commencement and without any adverse complications. All patients survived without long-term cardiac morbidity. CONCLUSION Recombinant tissue plasminogen activator may offer a safe alternative to surgical intervention in the high-risk infant with IE.

Prenatal diagnosis of Shone's syndrome: parental counseling and clinical outcome.

To describe a series of fetuses diagnosed as having Shones syndrome, which includes four cardiac defects and for which there is a wide variety of clinical presentations, surgical treatments and outcomes, and to discuss the counseling strategy.

Methotrexate in recurrent postpericardiotomy syndrome.

The postpericardiotomy syndrome occurs in up to one-third of children undergoing cardiac surgery. Its treatment includes anti-inflammatory agents, diuresis, and drainage of effusions. Administration of steroids can have a dramatic effect, but is limited by adverse effects. Usually the syndrome lasts weeks only, and persistence beyond six months is exceptional. We describe a rare case of chronic postpericardiotomy syndrome, with recurrent pericardial effusions and steroid dependency, that was treated successfully with a low weekly dose of methotrexate.

Cardiac manifestations following electrocution in children

BACKGROUND Electrical injury can result in a variety of cardiac abnormalities. We evaluate the cardiac effects in patients injured by electric shock and treated in our medical centre. METHODS We reviewed retrospectively the findings in 52 children, aged from 7 months to 17 years, with a mean age of 10.1 +/- 5.1 years, all evaluated and treated for accidental electric shock from January, 1992, through July, 2004. Relevant data regarding clinical presentation, electrocardiogram recording and cardiac enzymes was compiled. We also evaluated the echocardiographic findings, clinical course, treatment, and outcome. RESULTS Syncope had been the presenting symptom in 17 children (33%), asystole in 1 patient, and ventricular fibrillation or tachycardia in 2 patients. Characteristic changes of acute ischaemia of the anterior wall on the basis of changes in the ST segments were noted in 2 patients. Total creatine phosphokinase was measured in 33 children (63%), and was elevated in 20. Creatine phosphokinase-MB was measured in 11 patients, and was abnormal in six (54%). Troponin was measured in three children, and was significantly high in one (33%). Cardiopulmonary resuscitation and mechanical ventilation for a significant period was necessary in 5 patients, of whom 4 (80%) survived. None of the survivors was left with any cardiac disability following the acute event. CONCLUSIONS Significant cardiac damage and complications are rare in children and young adults who survive incidental electrocution. Most of the cardiac events are observed during the acute phase and immediately subsequent to electrocution. No delayed complications are anticipated.

Recombinant tissue plasminogen activator as a novel treatment option for infective endocarditis: a retrospective clinical study in 32 children.

Infective endocarditis is a life-threatening infectious syndrome, with high morbidity and mortality. Current treatments for infective endocarditis include intravenous antibiotics, surgery, and involve a lengthy hospital stay. We hypothesised that adjunctive recombinant tissue plasminogen activator treatment for infective endocarditis may facilitate faster resolution of vegetations and clearance of positive blood cultures, and therefore decrease morbidity and mortality. This retrospective study included follow-up of patients, from 1997 through 2014, including clinical presentation, causative organism, length of treatment, morbidity, and mortality. We identified 32 patients, all of whom were diagnosed with endocarditis and were treated by recombinant tissue plasminogen activator. Among all, 27 patients (93%) had positive blood cultures, with the most frequent organisms being Staphylococcus epidermis (nine patients), Staphylococcus aureus (six patients), and Candida (nine patients). Upon treatment, in 31 patients (97%), resolution of vegetations and clearance of blood cultures occurred within hours to few days. Out of 32 patients, one patient (3%) died and three patients (9%) suffered embolic or haemorrhagic events, possibly related to the recombinant tissue plasminogen activator. None of the patients required surgical intervention to assist vegetation resolution. In conclusion, it appears that recombinant tissue plasminogen activator may become an adjunctive treatment for infective endocarditis and may decrease morbidity as compared with current guidelines. Prospective multi-centre studies are required to validate our findings.

Digoxin concentration in saliva and plasma in infants, children, and adolescents with heart disease.

BACKGROUND Because of its narrow therapeutic index, therapeutic monitoring of digoxin is important in the management of infants and children receiving the drug for cardiac failure or arrhythmias, or following accidental ingestion. Whether saliva can replace plasma in the therapeutic monitoring of digoxin therapy in children is unclear. OBJECTIVE This study assessed the value of determining saliva digoxin concentration in infants, children, and adolescents. METHODS Infants, children, and adolescents receiving digoxin for various indications, whose digoxin dosage had remained unchanged for ≥10 days, and whose compliance was good according to the parents were enrolled. Digoxin concentration was measured in paired specimens of citric acid-stimulated mixed saliva and plasma obtained simultaneously. RESULTS Eighteen children (10 boys, 8 girls; mean [SD] age, 42.3 [53.1] months [range, 2 months-14 years]) were included in the study. Digoxin therapy was administered for cardiac failure due to dilated cardiomyopathy in 9 patients (50.0%), ventricular septal defect in 4 (22.2%), supraventricular tachycardia in 3 (16.7%), and after cardiac surgery in 2 (11.1%). Digoxin concentration in the 20 paired specimens obtained varied from 0.0 to 0.92 ng/mL (mean [SD], 0.25 [0.26] ng/mL) in saliva and from 0.27 to 1.54 ng/mL (mean [SD], 0.77 [0.40] ng/mL) in plasma. The mean plasma/saliva digoxin concentration ratio was 2.8. CONCLUSIONS This study of infants, children, and adolescents receiving digoxin for a variety of indications and whose dose was unchanged for ≥10 days showed that marked individual variability in the saliva/plasma concentration ratio precludes the use of saliva in predicting the plasma digoxin concentration. The value of saliva digoxin (as opposed to plasma digoxin) measurements in the assessment of the cardiac effects of the drug in children remains to be determined.

Streptobacillus moniliformis Endocarditis: An Unusual Case of Pulmonary Valve Erosion Resulting in Free Pulmonary Regurgitation and Aneurysm.

Endocarditis is a consideration in the differential diagnosis when masses are seen on echocardiography in a patient with congenital heart disease. We present a case of insidious development of endocarditis caused by Streptobacillus moniliformis in a seven-month-old baby after a rat bite, when the baby was three months of age.

Positional cyanosis in infants: an unusual presentation of right-sided cardiac masses

Positional cyanosis is an uncommon finding in young patients. We report three infants who presented with positional cyanosis due to a pedunculated tumour in the right heart. Arterial desaturation was the result of right-to-left shunting at the level of the oval foramen caused by obstruction and/or insufficiency of the tricuspid valve. The obstruction at the level of the tricuspid valve was variable because of the pedunculated nature of the tumours, which gave them considerable mobility. Hence, the degree of right-to-left shunting was dependent on the position of the patient. In all the patients, surgical resection of the tumours resolved the cyanosis.