Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases

Congenital heart block (CHB) can result in intrauterine cardiac failure leading to fetal or neonatal loss. To establish perinatal hemodynamic factors which might predict adverse outcome, six fetuses with CHB diagnosed between 20 and 30 gestational weeks were examined by echocardiography at 2-week intervals. Neonatal morbidity and outcome in infancy are detailed. The fetuses showed a significant decrease in ventricular rate (VR) with advancing gestation (60 ± 7 vs 51 ± 4 beats/min, p= 0.03). Cardiac decompensation defined as hydrops or pericardial effusion was associated with VR of lower than 55 beats/min in two fetuses. Three mothers had a therapeutic trial with a sympathomimetic and digoxin. Salbutamol increased VR 10% in one of three fetuses treated. Digoxin decreased pericardial effusion in one hydropic fetus with autoimmune myocarditis. In this fetus, poor left ventricular fractional shortening (LVFS) was accompanied with high umbilical artery resistance index (RI). High amniotic fluid erythropoietin indicated severe hypoxia preceding death. Pacemaker was indicated in all the newborns. At the age of 2 weeks all the surviving infants had tricuspid regurgitation and a shunt through foramen ovale due to asynchronized atrioventricular contraction. During the 12-month follow-up two of five surviving infants had no symptoms. One had symptomatic neonatal lupus. Two infants had patent ductus arteriosus, one with dilated cardiomyopathy. In conclusion, poor fetal outcome was associated with low VR, low LVFS, and high RI. Despite early pacing, morbidity was high in infancy due to cardiomyopathy and associated heart defects. Regular echocardiographic monitoring during pregnancy and after delivery is required in order to optimize care and timing of any interventions.     

Measurement of Atrial Septal Defect Size: A Comparative Study Between Three-Dimensional Transesophageal Echocardiography and the Standard Balloon Sizing Methods

Atrial septal defect (ASD) size measurement is of paramount importance for the successful deployment of a transcatheter septal occluder. The stretched balloon diameter (SBD) has long been regarded as the gold standard for selection of the size of any device. Three-dimensional (3-D) transesophageal echocardiography (TEE) can visualize the overall structure of the atrial septum, therefore rendering an accurate size of the ASD. In this study we aimed to validate the accuracy of ASD size measurement by 3-D TEE and to elucidate the reason for the difference between balloon sizing and 3-D measurement. Forty-one consecutive patients were enrolled in this protocol for ASD device closure using the Amplatzer septal occluder. Thirty-nine patients were diagnosed by 2-D transthoracic echocardiography as secundum ASD and 2 patients were diagnosed as patent foramen ovale. Two measurements of the balloon size were sequentially obtained by 2-D TEE after the balloon was fully inflated in the left atrium. First, no residual shunt across the septum could be seen while the balloon was pulled back against the septum. This measurement was called the balloon occlusive diameter (BOD). Second, with balloon deflation, a slight deformity of the balloon was seen just prior to its popping through the septum. This measurement was called the stretched balloon diameter (SBD). Three-dimensional TEE was performed in all patients at the beginning of the procedure before device deployment and within 15 minutes after device release. Three-dimensional TEE provided superior views of the ASDs, showing the spatial relationship between the ASD and the neighboring structures. For maximal ASD size measurement, balloon sizing was larger than 3-D TEE examination, whereas 2-D was smaller than the other two methods. The best correlation was found between 3-D TEE measurements and the BOD (r= 0.98, p < 0.0001). Three-dimensional TEE provides en face view of ASD; thus, it can accurately measure the size of ASD. Three-dimensional TEE measurement of ASD can be used instead of balloon sizing for the selection of transcatheter ASD occluder size.     

Distribution of Symptomatic Congenital Heart Disease in Hong Kong

Racial group studies have identified differences in the occurrence of congenital heart disease (CHD) among ethnic populations. The aim of this study was to characterize the proportionate frequency and clinical profile of children with symptomatic cardiac abnormalities in Hong Kong. The hospital records of 666, mainly Southern Chinese children with symptomatic CHD, who were 4 years of age or younger and who were admitted to Grantham Hospital, Hong Kong, in 1994 and 1995 were analyzed retrospectively. Left-to-right shunting (45.0%) and pulmonary outflow obstruction (34.4%) were the most frequently diagnosed categories, followed by left ventricular outflow obstruction (8.3%), transposition of the great arteries (4.2%), conditions with intracardiac mixing (3.9%), and other cardiac lesions (4.2%). Compared with Western studies, pulmonary outflow obstruction (p < 0.0001), particularly tetralogy of Fallot and critical pulmonary stenosis, were more frequent in Chinese children. In contrast with previous reports, coarctation of the aorta (5%) does not seem to be uncommon in Chinese patients. Conversely, aortic stenosis and hypoplastic left ventricle may be rare in these children (1% vs 3% and 3–7%). Other cardiac lesions showed no consistent racial difference in the frequency of occurrence. Chinese patients with Down's syndrome had ventricular septal defect (38%) as the predominant lesion followed by atrioventricular septal defect (25%). Western studies usually report a reverse pattern for these two lesions. The mortality rate for the total cohort was 7.5%. However, of those with conditions with intracardiac mixing and left ventricular outflow tract obstruction many did not survive childhood (20% and 21%, respectively).     

Radioangioscintigraphy and Doppler Echocardiography in the Quantification of Left-to-Right Shunt

The magnitude of left-to-right shunt in 55 children with isolated congenital heart disease [atrial septal defect (ASD) or ventricular septal defect (VSD) (muscular and perimembranous)] was estimated by two methods: radionuclide quantification and Doppler echocardiography [flow (L/min) = mean velocity × area × ejection time × heart rate]. We found little difference between the magnitude of left-to-right shunt obtained with Doppler echocardiography and that with radioangioscintigraphy for a whole group of patients (N= 55, −11.42% to 12.04%) and for subgroups of ASD (n= 24, −12.49% to 12.19%) and VSD (n= 31, −10.69% to 12.23%). These results indicate that Doppler echocardiography, in comparision with radioangioscintigraphy, is sufficiently accurate for clinical estimation of the Q _p/Q _s ratio in isolated congenital heart disease with left-to-right shunt.     

Results of Surgical Treatment of Congenital Heart Defects in Children with Down's Syndrome

We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ostium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 patients total correction was performed. The total death rate was 6%. Death in the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 months) are in NYHA class I or II. There were no reoperations. The postoperative course was complicated by pulmonary infections in 38% of patients, which converted to generalized infection in 10% and was the cause of death in 8% of patients. These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to eliminate problems connected with congestive heart failure and pulmonary hypertension.     

Congenital Heart Disease Among 815,569 Children Born Between 1980 and 1990 and Their 15-Year Survival: A Prospective Bohemia Survival Study

The objective of this study was to ascertain the prevalence and survival rate of children born with a heart defect. A total of 816,569 children live-born between 1980 and 1990 in Bohemia (52,478 km², population 6.314 million, western Czech Republic) were followed up and those with suspected heart disease referred to a center. Echocardiography was done in all of them. All dead children were autopsied. Congenital heart disease was found in 5030 of 816,569 children (6.16 per 1000 live births). The most frequent conditions were ventricular septal defect (41.59%), atrial septal defect (8.67%), aortic (7.77%) and pulmonary (5.81%) stenoses, transposition of the great arteries (5.39%), coarctation of the aorta (5.29%) and persistent ductus arteriosus (5.07%). The first week was survived by 92.46%, the first month by 89.14%, 6 months by 82.42%, and the first year of life by 80.02%, and 77.11% (95% CI 75.91–78.31%) survived to age 15 years. The best prognosis was found in pulmonary stenosis (15-year survival 95.55%), atrial septal defect (92.04%), persistent ductus arteriosus (90.59%), ventricular septal defect (89.37%) and aortic stenosis (88.39%). The worst results were attained in hypoplastic left heart, truncus arteriosus and pulmonary atresia with intact ventricular septum. In conclusion, the prevalence of congenital heart disease was 6.16 per 1000 live births; 77.11% of patients survived to age 15 years.     

Quality of Life 20 and 30 Years After Surgery in Patients Operated on for Tetralogy of Fallot and for Atrial Septal Defect

Follow-up studies on patients operated on for congenital heart defects have shown good anatomical results and long-term survival. To date, there have been few studies on such patients with regard to long-term psychosocial outcome and quality of life. In this study, two cohorts of patients operated on before the age of 15 years, one for tetralogy of Fallot (TOF) and the other for atrial septal defect (ASD), were investigated 20 and 30 years after operation regarding quality of life. The combined cohort had a higher educational level than average. There was no connection between quality of life and physical health as judged from the New York Heart Association classification. The TOF group rated their quality of life higher than the ASD group, but both groups had lower figures at the 30-year than at the 20-year follow-up. Fewer patients in the TOF than in the ASD group considered that their lives were affected by the heart disease. It is concluded that the severity of the heart disease is not necessarily congruent with estimated quality of live and that mild heart defects, such as ASD, can have a considerable impact on later life quality. Surprisingly few TOF patients were affected negatively, a finding which might reflect development of a specific coping strategy in these patients during childhood.     

Cardiorespiratory Exercise Tolerance in Asymptomatic Children with Ebstein's Anomaly

The aim of the study was to evaluate cardiorespiratory exercise tolerance in asymptomatic children with Ebstein's anomaly. Eleven children with a mean age of 9.6 years were prospectively studied by spirometry, cardiopulmonary exercise testing (bicycle ergometer n= 8, treadmill test n= 3), and contrast echocardiography. A right-to-left atrial shunt was detected by contrast echocardiography in 7 children (group 1), whereas no shunt was found in 4 (group 2). VO₂ max was decreased [84.5 (SD = 16.8)] and was strongly correlated to oxygen saturation in group 1 (p < 0.0001). Oxygen saturation at peak uptake was significantly decreased compared to baseline [97.4 (SD = 2.0) vs 90% (SD = 9.5%), p= 0.02] and was significantly lower in group 1 than in group 2 [85.7 (2.2) vs 98.2% (SD = 1.2%), p= 0.03]. Oxygen desaturation was related to a right-to-left atrial shunt (p= 0.01). Decreased VO₂ max was also correlated to the small size of the left ventricle (p= 0.05). We concluded that decreased exercise tolerance in children with asymptomatic Ebstein's anomaly is related to a right-to-left atrial shunt and to a small left ventricle. In case of poor exercise tolerance, a contrast echocardiography should be performed to detect an atrial septal defect.     

The Natural Course of Isolated Ventricular Septal Defect During Adolescence

Serial changes in patients with isolated VSD during adolescence have not previously been investigated. Hemodynamic status, diameter of the defect, and growth were studied yearly in 106 children with VSD. The mean duration of the follow-up was 13.16 years and ranged in 80% of subjects from 7 to 19 years (1395 patient years). The mean ages at pre- and postpuberty were 8.62 and 16.67, respectively. The presented longitudinal study, in which losses due to death and operation were minimal (4%), ideally reflected the natural history of VSD. Although weight showed retardation during prepuberty, this lag was caught up by the end of adolescence. Stature showed no retardation in pre- and postpuberty. Cardiothoracic ratio decreased significantly from a mean of 0.48 to 0.44 and showed normal variation. Although the mean defect diameter at prepuberty was 5.33 mm, this decreased to 2.7 postpubertally. The individual decrease (1.7 ± 2.34 mm) was significant (t= 5.349, p < 1/10⁵). The defect closed spontaneously in 24 (22.6%). In the 75 patients without pulmonary hypertension and with mild left-to-right shunting, 52 remained in the same class and spontaneous closure was observed in 23. In the 24 patients with moderate to severe left-to-right shunt, this decreased in 23 and only one remained stable. The 2 patients (1.9%) with Eisenmenger syndrome remained stable and 1 died. Aortic regurgitution developed in 10 patients (9.4%); however, this was of mild degree in most of them. No infective endocarditis was observed. It is concluded that patients with VSD should be followed closely through adolescence, because the diameter of the defect, as well as left-to-right shunting, can decrease, and it is concluded that the spontaneous closure of the defect is to be expected in a considerable 23%, and aortic prolapse or mild regurgitation may develop in approximately 10%.     

High Prevalence of Interatrial Communications During the First Three Months of Life

The objective of this study was to analyze retrospectively the results of color Doppler echocardiographic (CDE) investigations performed for the first time on a child under circumstances where there was unrestricted access to CDE. The records were obtained from a tertiary referral center at a university hospital serving a population of about one million. The patients were children (n= 284) ages 0 days to 15 years 9 months referred for CDE over a 1-year period. Heart abnormalities were found in 119 children (42%). Among the children <3 months old, a heart abnormality was detected by CDE in 71%. The most prevalent diagnosis was atrial septal defect, particularly during the first 3 months of life, where the intraatrial communications accounted for 78% of the heart abnormalities. It is concluded that there is high prevalence of atrial communications during the first 3 months of life. The spectrum of diagnoses among newborns differs from the previously reported frequencies of congenital heart diseases. Better equipment (CDE) seems to diagnose abnormalities that might not be diagnosed otherwise.     

Effects of gender,age, and heart rate on QT intervals in children

The objective of this study was to determine if gender, age, and heart rate affect corrected QT intervals in children. Electrocardiograms were obtained from 781 healthy children 10–18 years of age. Corrected QT intervals were significantly (p < 0.0005) greater for girls than for boys in the entire population and for each age group over 14 years. The corrected QT interval varied inversely with age and directly with heart rate. Hence gender, age, and heart rate should be considered when diagnosing long QT syndrome.     

Reversible Cardiomyopathy Secondary to α-Interferon in an Infant

Interferon-α (IFN-α) is a biological response modifier with antiviral and tumoral effect that is used in the treatment of chronic myelogenous leukemias. Adverse effects are well documented and cardiovascular disturbances mostly include hypotension and tachycardia and rarely cardiomyopathy. We report on an infant with chronic myelomonocytic leukemia (CML) diagnosed at 3 months of age who was treated with increasing IFN-α dosage (2.5–5.5 million U/m²/day) given subcutaneously for 7.5 months. At that age, he presented anorexia, general malaise, and nocturnal sweating for about a week, followed by respiratory distress and tachycardia. Diagnosis of congestive heart failure was suspected and documented by cardiomegaly and echographic changes of left ventricular dilated cardiomyopathy, with a 40% left ventricular ejection fraction (EF) and 20% fractional shortening (FS). He was treated with digoxin, furosemide, and angiotensin converting inhibitors, and IFN-α was discontinued. Progressive improvement of cardiac function was observed within 7 months of the events with normalization of the echocardiographic findings (EF 60%, FS 31%). We should emphasize the possibility of severe and reversible cardiac toxicity of IFN-α in infancy.     

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.     

Effects of Electrocardiography and Chest Radiography on the Accuracy of Preliminary Diagnosis of Common Congenital Cardiac Defects

The objective of this study was to compare the accuracy of the expert clinical examination for certain common cardiac defects with and without electrocardiogram (EKG) and chest radiogram (x-ray). The design of the study was a prospective, blinded comparison of diagnostic accuracy of the expert examination with and without EKG and x-ray, using echocardiography as the diagnostic standard. The setting of the study was the pediatric cardiology outpatient department. There were 749 outpatients with heart murmur under 21 years of age without prior echocardiography or pediatric cardiology consultation. The intervention was echocardiography as clinically indicated for evaluation of heart murmur of uncertain cause. Measurements were carried out using the incorporation of EKG and x-ray into multiple linear regression models to assess independent associations, if any, with the accuracy of clinical examination. Results were reported as the presence or absence of independent significant impact of availability of EKG and x-ray on examiner's diagnostic accuracy for innocent murmur, ventricular septal defect (VSD), pulmonary stenosis (PS), aortic valve disease, atrial septal defect (ASD), and patent ductus arteriosus. EKG enhanced detection of ASD and may have helped detect PS. X-ray enhanced detection of intermediate to large VSD. X-ray and EKG were otherwise without demonstrable independent advantage for defect-specific diagnosis. Routine use of one or both of these tests in the initial evaluation of heart murmur in the pediatric cardiology clinic should remain an option.     

Balloon Angioplasty of Native Coarctation of the Aorta in Infants and Neonates: Is It Worth the Hassle?

Balloon angioplasty (BA) for native coarctation of the aorta (CA) in infants and neonates remains controversial with a high incidence of restenosis. The purpose of this study is to analyze our acute and midterm results for BA of native CA in infants and neonates and try to identify factors that may be predictive of outcome. Between September 1991 and June 1999, 17 patients with CA underwent BA at a median age of 3 months (range 2 weeks–9 months) and median weight of 4.8 kg (range 2.8–7 kg). Fourteen patients had discrete CA and 3 had tubular hypoplasia. All patients were hemodynamically stable prior to BA and no patients had critical coarctation requiring prostaglandin E₁ infusion to maintain ductus arteriosus patency. Seven patients had other associated cardiac defects. All patients had significant initial improvement. The mean peak systolic gradient across the CA improved from 43 ± 15 mmHg to 10 ± 8 mmHg (p < 0.001), and the mean minimum diameter of the aortic lumen increased from 2.4 ± 0.9 mm to 5.2 ± 1.0 mm (p < 0.001). There was no mortality or major complication. At median follow-up interval of 2.7 years (0.15–7.75 years), 10 (59%) of 17 patients are clinically well and have an upper to lower limb systolic blood pressure difference of <20 mmHg. Seven (41%) of 17 patients developed significant restenosis (5 of these patients underwent repeat BA, which was successful in 3 patients). Four (24%) patients underwent surgical repair at a median age of 4.5 months (3–6.9 months) and a median time interval of 4 months (2–6.5 months) from the initial BA. All 3 patients with tubular hypoplasia type of CA underwent surgical repair. No patients developed aortic aneurysm following initial or repeat BA. All patients who underwent surgical repair were 1 month or less in age at the time of their initial BA. We conclude that BA of native CA in infants and neonates can be performed safely with low mortality and morbidity. It appears to offer the best results in patients who are older than 1 month with discrete CA and a well-developed aortic arch. Further restenosis of the discrete CA can be managed successfully by repeat BA.     

Radiofrequency Catheter Ablation of Tachycardia in Patients with Congenital Heart Disease

Patients with anomalies of the heart frequently suffer from arrhythmias that either are associated with a congenital heart defect or result from the course of the disease. For most of the bradyarrhythmias, appropriate timing of the initiation of treatment is more challenging than its eventual execution. In the case of tachycardias, technical aspects of treatment require more attention because the often imperative impact such tachycardias have on quality of life, morbidity, and mortality determine intervention timing. Increasingly, interventional electrophysiology is turned to as a potentially definitive and substrate-related treatment because of antiarrhythmic drug therapy's failure to prevent arrhythmia recurrences and the potential detrimental side effects from drug therapy seen in this particular patient population. Using the experience gained during the past 10 years in the treatment of patients with arrhythmias but without associated structural heart disease, several groups reported their results and difficulties with the application of such therapy to patients with congenital heart defects. In this report, we summarize our hospital's experience with transcatheter radiofrequency current application for treatment of various types of tachyarrhythmias in 139 children and adults with congenital heart defects, emphasizing the current limitations of such therapy and addressing the potential benefits expected from future technology. Patient ages ranged from 5 months to 76 years (mean 25.3 ± 17.7 years), including 56 children and adolescents less than 16 years of age. At least one attempt at surgical palliation or correction was made in 93 patients; the remaining 46 patients had no surgical intervention attempts. A total of 225 different tachycardias were found, 93 of which were based on a congenital arrhythmogenic substrate (e.g., an accessory pathway). Acquired substrates (e.g., scars or myocardial fibrosis) gave rise to the remaining 132 tachycardias. Radiofrequency current ablation (183 sessions) successfully treated 121 of 139 patients. Within a follow-up period of 21 months a recurrence of the intrinsically treated tachycardia was seen in 24 patients (10.7%); 13 of the 24 underwent a successful repeat session. There were no significant procedure-related complications. Young and adult patients with congenital heart disease can be safely and successfully treated for tachycardias with the use of radiofrequency current ablation. Because such treatment meets the specific needs of this patient group, early consideration for this therapy is recommended.     

Atrial Septal Defect: Spectrum of Care

Atrial septal defect (ASD) is a common congenital heart defect. Variability in management of this lesion exists among clinicians. A review of the literature reveals that there is lack of standard guidelines for the evaluation and management of patients with different types of ASDs. This survey-based study was conducted to test the uniformity of diagnostic and therapeutic approach to management of children with secundum, sinus venosus, and primum ASDs. Survey questionnaires were prepared to include questions regarding follow-up, diagnosis, and therapeutic intervention of different types and sizes of ASDs. Questions addressed follow-up visitations, type and frequency of investigative studies, pharmacological therapy, and choice of repair method. Surveys were sent out to all pediatric cardiology academic programs in the United States (n=48) and randomly selected international programs from Europe, Asia, and Australia (n=19). A total of 23 programs (34%) responded to the survey (15 from the United States and 8 internationally). A separate questionnaire was prepared for secundum, primum, and sinus venosus ASD. In each questionnaire, lesion types were subdivided into small, moderate, and large defect sizes to address differences of management approaches to each defect type and size. Results indicate that in secundum ASD, most participants use size of the defect and/or evidence of right-sided volume overload as criteria for defining small, moderate, and large defects. Frequency of follow-up does not vary with the type of lesion but is more frequent with larger defects. Most participants see patients with small defects at intervals of 6 months to 1 year and those with large defects at 3- to 6-month intervals. Age of patient and presence of symptoms determined the frequency of follow-up across all defects. Echocardiography was the most frequently used investigative modality in all defect sizes and types during follow-up visits (used by >80% for follow-up), followed by electrocardiography (ECG). There is a striking preference for the use of pharmacological therapy in primum ASD compared with secundum and sinus venosus ASD. The timing of repair was mainly dependent on patient age and symptomatology in different defects, with the presence of associated anomalies contributing to that in primum and sinus venosus ASD. Most participants use percutaneous approach to close secundum ASD (either as a first choice or as one of two choices depending on the presence of certain features). Before repair, participants use MRI or cardiac catheterization to fully evaluate a secundum ASD if it is large. These investigative modalities are not commonly used in primum and sinus venosus ASD. There is agreement on postoperative follow-up in different types of defects, with most participants continuing follow-up indefinitely, especially in larger defects.     

Familial Congenital Valvar Pulmonary Stenosis: Autosomal Dominant Inheritance

A mother and three of her four children, one girl and two boys, who had valvar pulmonary stenosis are described. One child had an associated septum secundum atrial septal defect, and another had an associated ventricular septal defect. An autosomal dominant mode of inheritance is likely for the valvar pulmonary stenosis. The association of additional cardiac lesions in two of the three siblings raises the possibility of closely associated genetic loci.     

Diagnostic and Interventional Trends in Tetralogy of Fallot and Transposition of the Great Arteries in a Population-Based Study

The goal of this study was to analyze diagnostic and interventional trends in tetralogy of Fallot and transposition of the great arteries in Malta for individuals born during 1920–1994 and calculate birth prevalences. The design was population based, in the setting of a regional hospital providing exclusive diagnostic and follow-up services for the entire population of Malta. Data collection and analysis was retrospective and included comparison with earlier epidemiological studies. Patients included were all Maltese live births diagnosed as having tetralogy of Fallot (TF) and transposition of the great arteries (TGA) born by the end of 1994. There were 109 cases of TF and 30 cases of TGA (n= 30). A significant decline in age at diagnosis and age at surgery was found for both lesions (p < 0.0001), which was associated with a significant decline in perioperative mortality (p= 0.005). Underascertainment was present prior to 1980, however, more cases were diagnosed due to improvement of postmortem services in the early 1980s followed by improvement of clinical services with eventually exclusive premortem diagnosis. The birth prevalence of TGA was 0.31/1000 live births, well within the range described in previous studies, unlike tetralogy of Fallot, which has been found in excess in this population in an earlier study. TF and TGA, the two lesions which comprise the majority of cyanotic congenital heart disease presenting in infancy, have been diagnosed and have undergone intervention at progressively earlier ages over the period under study. This decline was associated with a declining perioperative mortality.     

Trends of Childhood Infective Endocarditis in Israel with Emphasis on Children Under 2 Years of Age

This study retrospectively analyzed the characteristics of infective endocarditis (IE) in children in light of recent advances in pediatric cardiology. We evaluated 25 consecutive patients with IE from 1980 to 1991 at a tertiary Children's Medical Center in Israel. The incidence of IE increased significantly over the second half of the study period, owing mainly to an increase in the number of patients less than 2 years old. Concomitantly, the causative microorganisms changed considerably, with a decrease in viridans streptococci, an increase in staphylococci and other uncommon causes of IE, and increased antibiotic resistance. Children under the age of 2 years, previously considered uncommon IE patients, accounted for 47% of the IE cases during the second half of the study period. The infection often (78%) appeared in children with complex congenital heart diseases, commonly after early palliative or definitive cardiac surgery. Infants who underwent systemic-to-pulmonary shunting, especially with implantation of foreign materials, were at highest risks. IE was more often hospital-acquired (56% versus 6%) and acute (56% versus 44%) in the younger children than in the older ones and often lacked the typical clinical presentation of the disease. In addition, uncommon causative organisms were noted more frequently (67% versus 13%; p= 0.009) in the younger group. The study shows a significant increase in IE in infants and young children with distinctive underlying conditions, clinical presentation, and microbiologic findings, all of which should be considered currently for the diagnosis and treatment of pediatric IE.