Complete heart block in a patient with acute lymphoblastic leukaemia: teicoplanin as a possible cause and review of literature.

WHAT IS KNOWN AND OBJECTIVE: Teicoplanin is a glycopeptide antibiotic used against documented or presumed methicillin-resistant infections. We report a 31-month-old boy with acute lymphocytic leukaemia who developed permanent complete atrioventricular block (CAVB) necessitating pacemaker insertion after receiving teicoplanin for Staphylococcus epidermidis bacteremia. CASE SUMMARY: Clinical assessment of the child revealed febrile neutropenia. After thorough assessment and work-up, the patient was started on teicoplanin intravenously after which he had sudden onset of bradycardia. Electrocardiography showed CAVB that eventually required permanent pacemaker insertion. Twenty-nine months from the incident, the patient is doing well. WHAT IS NEW AND CONCLUSION: We report on a case of teicoplanin-associated CAVB in a child with acute lymphoblastic leukaemia (ALL). This is one of only two similar cases reported in the literature. Teicoplanin remains the most probable cause. The use of teicoplanin should be approached cautiously in the setting of immunosuppression. Whether VZV contributed and teicoplanin triggered remains speculative. Physicians should be aware of this possible complication.

Ocular pathology in congenital heart disease.

PURPOSE: To describe the ocular findings in subjects with congenital heart disease (CHD). METHODS: In a prospective study, the same observer examined 240 consecutive patients with CHD admitted to the medical centre. Two independent geneticists performed identification of syndromes. RESULTS: The commonest anatomic cardiac anomalies were ventricular or atrial septal defects (62), tetralogy of Fallot (39), pulmonary stenosis (25), and transposition of the great arteries (24). The heart lesions were divided physiologically into volume overload (90), cyanotic (87), and obstructive (63). In all, 105 syndromic subjects included the velocardiofacial syndrome (18), Down's syndrome (17), CHARGE association (6), DiGeorge syndrome (5), Williams syndrome (3), Edwards syndrome (3), Noonan syndrome (3), VACTERL association (2), and Patau syndrome (trisomy 13) (2). The paediatric team recognized 51 patients as syndromic. Two independent geneticists recognized additional 54 patients as syndromic. Positive eye findings were present in 55% (132) and included retinal vascular tortuosity (46), optic disc hypoplasia (30), trichomegaly (15), congenital ptosis (12), strabismus (11), retinal haemorrhages (8), prominent eyes (7), and congenital cataract (6). There was a strong correlation between the retinal vascular tortuosity and both a low haematocrit (P=0.000) and a low arterial oxygen saturation (P=0.002). CONCLUSIONS: Patients with CHD are at a high risk for ocular pathology and need screening for various ocular abnormalities.

A new patient with pure trisomy 4p resulting from isochromosome formation and whole arm translocation.

Short arm isochromosome formation with translocation of the entire long arm of the same chromosome is an unusual constitutional abnormality that has been observed, to our knowledge, in 18 cases. Only one of these previously reported cases involved chromosome 4, resulting in pure trisomy 4p. Pure trisomy 4p has been reported in a number of cases, the majority of them due to familial chromosome rearrangements, and is associated with a distinct pattern of abnormal findings. We report here a second case of a de novo chromosome 4 whole arm translocation with short-arm isochromosome formation, which we have delineated further by FISH studies.

Doppler characterization of left ventricular diastolic function in beta-thalassaemia major. Evidence for an early stage of impaired relaxation.

AIMS: Doppler echocardiographic studies of left ventricular diastolic function in patients with thalassaemia major have shown conflicting findings. This study was undertaken to compare Doppler echocardiographic parameters of diastolic function among a group of patients with thalassaemia major, a group with thalassaemia intermedia and a group of normal individuals. METHODS AND RESULTS: 50 patients with thalassaemia major, 38 patients with thalassaemia intermedia and 29 normal subjects were studied. All had normal systolic function. The thalassaemia intermedia patients had larger body surface area and left ventricular mass index than the thalassaemia major patients but less than the controls. The ratios between peak early and late mitral diastolic flow (E/A ratio) were comparable between the three groups. The haematocrit levels were comparable in the two study groups, but the ferritin levels were significantly higher in the thalassaemia major group (P<0.001). Using multiple regression analysis to correct for the influence of heart rate, age and body surface area, we found a prolonged isovolumic relaxation time (P<0.03) and a lower E wave (P<0.001) in the thalassaemia major group as compared to the thalassaemia intermedia group. The isovolumic relaxation time also differed significantly between the thalassaemia groups and the control (P<0.001), suggesting a state of impaired relaxation most notable in thalassaemia major that is probably due to iron overload. CONCLUSION: In patients with thalassaemia major and normal systolic function who have iron overload, the earliest sign of diastolic dysfunction is an impairment in left ventricular relaxation manifested as a prolonged isovolumic relaxation time.

Treatment of rheumatic carditis with intravenous gammaglobulin: is there a beneficial effect?

Rheumatic carditis is a major manifestation of acute rheumatic fever. Conventional therapy includes the use of salicylates and steroids. To date, however, such therapy has not been proven to have a clear benefit in reducing valvar heart disease. We report the use of high-dose intravenous immunoglobulin in two chidlren with acute rheumatic carditis in whom we have been able to document the beneficial effect.

Cerebral mycotic aneurysm in a child with Down's syndrome: a unique association.

Mycotic aneurysms are rare complications in patients with infective endocarditis, particularly in the pediatric population. We report a case of mycotic aneurysm of the middle cerebral artery complicating bacterial endocarditis in a child with Down's syndrome. The patient was successfully treated medically without the need for surgical intervention.

Cardiac disease in children in Lebanon: the AUB-MC Children's Cardiac Registry experience.

OBJECTIVE: To study the epidemiology of cardiac disease in children and their outcome in Lebanon, we established a Children's Cardiac Registry Center (CCRC) at the American University of Beirut-Medical Center. DESIGN/METHODS: The CCRC included prospectively all pediatric patients with congenital heart disease (CHD) and/or acquired heart disease (AHD) who were evaluated at our center, between March 1, 1997 and July 31, 2000. RESULTS: Out of the 1000 patients with cardiac anomalies enrolled in the CCRC, 917 (91.7%) had CHD and the rest had AHD. Ventricular septal defect was the most common cardiac malformation with a relative frequency of 25.3%, followed by pulmonary stenosis (14.6%), aortic anomalies (8%), ASD (8%) and tetralogy of Fallot (7.8%). Complex cardiac lesions like HLHS, TGA and AVC had lower frequencies at 0.4%, 3.7% and 3.5% respectively. The most common AHD was rheumatic heart disease (42.2%). 34.9% of the registry patients with CHD and 10.8% with AHD underwent surgical intervention. There were 4.8% and 2.4% mortality rates in the CHD and AHD groups, respectively during the 40-month study period. CONCLUSION: The prevalence of many of the cardiac malformations in the CCRC was similar to that reported in the literature. However, some of the complex cardiac lesions were less common. The outcome of the two groups of patients is comparable to the outcome of children with cardiac malformation from developed countries. The establishment of a registry at the national level is important. Appropriate identification of the cardiac disease, its epidemiology, and outcome is of utmost importance in guiding adequate care.

Paediatric infective endocarditis: 19-year experience at a tertiary care hospital in a developing country.

A retrospective study was undertaken to study children who presented with infective endocarditis (IE) to a university teaching hospital in Beirut, Lebanon, between January 1977 and May 1995. Of 41 patients with IE (24F, 17M), 28 (68%) were diagnosed between 1977 and 1985. Patients' ages ranged from 3 to 18 y (mean age 11.3+/-2.8 y), and 13 patients were <10 y of age. Clinical presentations included: fever (in 88%), heart failure (in 39%), neurologic findings (in 20%) and embolic phenomena (in 22%). Nineteen patients (46%) had underlying congenital heart disease (CHD) with tetralogy of Fallot and pulmonary stenosis being the most common. Sixteen patients (39%) had underlying rheumatic heart disease (RHD). A total of 5 children (12%) with normal cardiac anatomy had IE. One had underlying acquired viral myocarditis with mitral insufficiency. Echocardiography showed vegetations in 60%. Blood cultures were positive in 31 patients (76%). IE occurred in three patients following cardiac surgery. In one patient it occurred within 2 mo of surgery and in the other two it occurred within 6 mo. Streptococcus viridans and Staphylococcus aureus were the two most commonly isolated bacteria. Overall mortality rate was 29% (not statistically significant between patients presenting between 1977-1985 and 1986-1995; p = 0.17). There was no statistically significant difference in mortality among the groups (five in the group with CHD, six with RHD and one with structurally normal heart). This study demonstrates that RHD is an important underlying cause of IE in children in our community. This finding is similar to those in other developing countries and different from those in developed countries. Distribution of pathogens and CHD in our study is comparable to some reports in the literature, except for the higher proportion of patients with underlying pulmonary stenosis. Bacterial endocarditis prophylaxis should be emphasized in patients with RHD or pulmonary stenosis.

Rheumatic fever in children: a 15-year experience in a developing country.

Clinical data from 91 patients with rheumatic fever (RF), who were hospitalized at a tertiary hospital in Lebanon between 1980 and 1995, were reviewed retrospectively. Age on hospitalization was 11.1+/-2.9 years (mean +/- SD, range 3-17 years). Nineteen patients were <6 years of age. Manifestations included carditis (93%), arthritis (39%), Sydenham's chorea (2%), erythema marginatum (4%), subcutaneous nodules (1%), fever (62%), arthralgia (55%), and acute congestive heart failure (CHF) on initial presentation (44%). Pericardial effusion occurred in 11%. There was positive family history of RF in 14%. Mitral insufficiency and aortic insufficiency occurred in 67 and 35%, respectively. Both mitral and aortic valves were involved in 30% of cases. Tricuspid insufficiency developed in 3% and pulmonary insufficiency in 1%. Mitral stenosis developed in 19%. Twenty-eight patients underwent surgical intervention: mitral valve repair and commissurotomy in 9/91 (10%), mitral valve replacement in 18/91 (20%), and aortic valve replacement in 9/91 (10%). Overall mortality was 12%: 5 following surgical intervention (3 after mitral valve surgery and 2 after mitral and aortic valve surgery). All patients that died had CHF on initial presentation (p = 0.006). This study includes hospitalized patients with predominant rheumatic heart disease. Initial presentation with CHF is a risk factor for surgical intervention and mortality. A significant high surgical intervention rate is noted that is probably related to the nature of the selected group studied. This study emphasizes the significant morbidity and death in patients with RF and carditis.

Foreign body retrieval in children with respiratory symptoms and no history of aspiration.

To asses the role of bronchoscopy and its yield in children with respiratory symptoms and no typical history of radiological evidence of foreign body aspiration (FBA), we reviewed the records of all patients who underwent bronchoscopy at our institution between 1985 and 1995 and had no history of choking, witnessed FBA or clear radiological evidence of FBA (N = 54, age range 4 months to 16 years). These were subdivided into two subgroups, those with positive bronchoscopy, and those with negative bronchoscopy. Respiratory symptoms and their duration, signs and radiological findings did not differ in both subgroups.

Congenital heart disease at a tertiary care center in Lebanon.

OBJECTIVE AND METHODS: To study the epidemiology of congenital heart disease (CHD) at the American University of Beirut-Medical Center, we reviewed the medical records of all cardiac patients seen at our outpatient cardiology clinic (OPD) between 1980 and 1995. The charts of all patients with CHD seen as inpatients and/or outpatients at our center during the year 1995 were also reviewed. A cardiologist evaluated all patients and the diagnosis was confirmed at least by echocardiography. The frequency of CHD was reported among three groups: 1980-1995 OPD groups (Group A); the group with CHD seen during the year 1995 (Group B); and (Group C), a subgroup of group B, included all newborns with CHD born at our hospital during the year 1995. Stillbirth and premature infants with the diagnosis of patent ductus arteriosus were excluded from the study. RESULTS: Group A included 883 patients. 344 patients were evaluated in Group B, with a mean age of 3.8 years. The incidence of CHD was 11.5/1,000 live births at our center. There was a relatively low prevalence of complex lesions (i.e., hypoplastic left heart syndrome, transposition of the great arteries) and a relatively high prevalence of the simpler cardiac malformation (i.e., ventricular and atrial septal defects, pulmonary stenosis) in Groups A and B. CONCLUSION: The relatively low prevalence of complex cardiac lesions in our study is probably related to the age of the studied patients, and reflects the high mortality of these complex lesions in our country early in life. The incidence of CHD of 11.5/1,000 live births at our center is higher than that reported in the literature, with evidence of more frequent ventricular septal defects and pulmonary atresia lesions. This may be related to high rate of consanguinity in our population. This review underscores the need for a national cardiac registry center for children in a developing country like Lebanon. Such a database will allow referral and care of complex cardiac lesions.

Transient hypertrophic cardiomyopathy in the newborn following multiple doses of antenatal corticosteroids.

Postnatal exposure to steroids has been associated with hypertrophic cardiomyopathy (HCM) in the newborn. Such an effect has not been described in infants born to mothers who received antenatal steroids. We report three newborns whose mothers were treated with betamethasone prenatally in different doses, duration of time, and who developed various degrees of HCM diagnosed by echocardiography. There was no maternal evidence of diabetes except for one infant whose mother had a normal fasting and post-prandial blood glucose prior to steroid therapy, but an abnormal one hour postprandial glucose after 8 weeks of betamethasone therapy, with a normal HbA1 C level. There was no family history of HCM, no history of maternal intake of other relevant medications, and no hypertension in all three newborns. Follow-up echocardiography revealed complete resolution of the HCM changes in all infants. We suggest that repeated antenatal maternal steroid intake may cause changes of HCM in the newborn. These changes appear to be dose- and duration-related and are mostly reversible. Further prospective controlled studies to evaluate these observations and to investigate potential mechanisms are warranted.

Necrotising infection of the orofacial tissues in neonates (noma neonatorum). Case report.

Noma neonatorum should be differentiated from noma, in that it is typically a disease of seriously ill premature infants whose birth weight was low, and is caused by Pseudomonas aerugenosa septicaemia. We know of only two case reports of noma neonatorum involving newborn infants born at full term, so we report here another case of noma neonatorum in a neonate born at full term. In addition we describe the differences between noma neonatorum and noma (cancrum oris), a clinically related entity.

Double-outlet right ventricle (tetralogy of Fallot type) associated with anomalous origin of the left coronary artery from the right pulmonary artery: report of successful total repair in a 2-month-Old infant.

The case reported herein demonstrates the rare association of double-outlet right ventricle (tetralogy of Fallot type) with anomalous origin of the left coronary artery from the pulmonary artery. It is the first reported successful total surgical repair in an infant.

Dextrocardia and corrected transposition of the great arteries (I,D,D) in a case of Kartagener's syndrome: a unique association.

Kartagener's syndrome (KS) usually includes mirror-image dextrocardia. The incidence of congenital heart disease in KS is comparable with that in the general population. This paper reports on a case of Kartagener's syndrome associated with dextrocardia, corrected transposition of the great arteries (I,D,D), ventricular septal defect, and valvar pulmonary stenosis in an 8-year-old girl.

Intrapericardial teratoma in an adult: a rare presentation.

BACKGROUND: Intrapericardial teratomas are rare and usually present early in infancy or childhood. PROCEDURE: We describe herein a rare case of an adult patient with an intrapericardial teratoma who presented with fever, cardiac arrhythmias, and oppressive substernal chest pain. Preoperative diagnosis was suggested by echocardiography and computerized tomography of the chest. The tumor weighed 530 g and its histologic features were those of a mature cystic teratoma. It was excised totally and 10 years' follow-up revealed no evidence of residual disease. DISCUSSION: Our patient is one of the very few adult patients with intrapericardial teratomas who was treated successfully with surgery. Both echocardiography and tomography of the chest suggested the diagnosis and delineated the relationship of the tumor to the great vessels. CONCLUSION: The diagnosis of Intrapericardial teratomas is suspected by echocardiography and/or tomography of the chest and confirmed by specific histologic features. These tumors should be excised whenever detected.

In utero management of hydrops fetalis caused by critical aortic stenosis.

Hydrops fetalis is rarely associated with congestive heart failure caused by obstructive left-sided heart lesions. There are rare cases of live born neonates with critical congenital valvar aortic stenosis and hydrops reported in the literature, all with fatal outcomes. This report describes, to the best of our knowledge, the first two newborns who were diagnosed prenatally to have hydrops fetalis caused by critical valvar aortic stenosis, who were treated prenatally with digoxin and who postnatally had successful percutaneous balloon aortic valvuloplasty. Both patients had not only left but right ventricular dysfunction. We speculate that right ventricular dysfunction was a contributing factor in the development of hydrops in these patients and in utero medical therapy with digoxin is associated with resolution of the hydrops before delivery.

Effect of early versus delayed hypoxic environment on neonatal rabbits.

We developed a low-cost, nonsurgical small animal model simulating the condition of cyanotic heart disease. Six groups of New Zealand white rabbits were studied: Group (1-C), 1-week-old control rabbits (n = 9) reared in room air; Group (1-H), 1-week-old rabbits placed in a hypoxic environment (10% O2) at birth (n = 5); Group (1-H-3), 1-week-old rabbits (n = 11) reared in room air for 3 days and then placed under hypoxic conditions identical to those for Group (1-C); Group (4-C), 4-week-old control rabbits (n = 12); Group (4-H), 4-week-old rabbits placed in hypoxia since birth (n = 11); Group (4-H-3), 4-week-old rabbits kept in room air after birth for 3 days (n = 7) before being exposed to hypoxia. Animals were anesthetized, heparinized, and instrumented for measurement of hemodynamic parameters. Right ventricular (RV) hypertrophy and hematocrit were assessed. Lung tissue was analyzed using quantitative morphometric techniques to assess arterial size, number, and muscularity. Group (1-H) and Group (4-H) developed RV hypertrophy, pulmonary hypertension, and erythrocytosis. The RV hypertrophy developed rapidly, as early as 1 week of age and became so pronounced by 4 weeks as to result in high mortality rate (35%). None of the animals in Groups (1-H-3) or (4-H-3) died while chronic changes of hypoxemia still developed. Placing rabbits after birth in room air for 3 days before exposing them to hypoxia appeared to play a protective role, moderating the development of pulmonary hypertension and severe RV hypertrophy. The effects of hypoxia appear to be at least partially dependent on the time of exposure. Utilizing our low-cost model will allow future work, including study of the effects of chronic hypoxemia on systemic ventricle exposed to an ischemic insult.