Showing papers in "Pediatric Cardiology in 2008"

Feeding Difficulties and Growth Delay in Children with Hypoplastic Left Heart Syndrome versus d-Transposition of the Great Arteries

Abstract: The objective of this study was to identify the incidence of feeding difficulties in infants with hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (d-TGA). Congenital heart disease is a risk factor for growth failure. The etiologies include poor caloric intake, inability to utilize calories effectively, and increased metabolic demands. The goals of our study were to (1) identify feeding difficulties in infants with HLHS and d-TGA and (2) assess their growth in the first year of life. We performed a chart review of 27 consecutive infants with HLHS and 26 with d-TGA. Descriptive statistics were generated for demographic and clinical variables within each group and are presented as means ± standard deviations. HLHS and d-TGA groups were compared on time to achieving nutritional goals using the log rank test, on complication rate using the chi-square test, and on weight using the t-test. A significance level of 0.05 was used for all tests. Birth weight was similar for both the HLHS and d-TGA groups (3.19 ± 0.69 vs 3.35 ± 0.65 kg, respectively; p = 0.38). Infants with HLHS weighed less than those with d-TGA at l month (3.29 ± 0.58 vs 3.70 ± 0.60 kg, respectively; p = 0.021), 6 months (6.27 ± 1.06 vs 7.31 ± 1.02 kg, p = 0.003), and 12 months of age (8.40 ± 1.11 vs 9.49 ± 1.01 kg, p = 0.006). Time to achieving full caloric intake (at least 100 kcal/kg/day) for the HLHS group (24 ± 11.9 days) was significantly longer than for the d-TGA group (12.0 ± 11.2 days, p < 0.001). In addition, infants with HLHS had a higher incidence of feeding-related complications that those with d-TGA (48 vs 4%, respectively; p = 0.001). Compared to the d-TGA group, infants with HLHS weighed less at follow-up, took longer to reach nutritional goals, and had a much higher incidence of feeding-related complications.

Hospital mortality for Norwood and arterial switch operations as a function of institutional volume.

Abstract: Regionalization of complex surgical procedures to high-volume centers is a model for improving hospital survival. We analyzed the effect of institutional volume on hospital mortality for the Norwood and arterial switch operations (ASO) as representative high-complexity neonatal cardiac procedures. Analysis of discharge data from the 2003 Kids’ Inpatient Database (KID) was conducted. Association between institutional volume and in-hospital mortality was examined for the ASO or Norwood procedure. Logistic regression analysis was performed to calculate the probability of hospital mortality for both procedures.

Pulmonary hypertension in children and adolescents with sickle cell disease

Abstract: The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 25 m/s Twenty-four (4615%) SCD patients had increased PAP (ie, TRV ≥25 m/s), and 6 (115%) had significant PHTN (ie, TRV ≥30 m/s) Pulmonary disease was marginally associated with PHTN (odds ratio 280 and confidence interval 088 to 886; p = 00795) As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD

Ultrasound-guided versus landmark-guided femoral vein access in pediatric cardiac catheterization.

Abstract: Background This study aimed to evaluate whether an ultrasound-guided technique can improve upon a landmark-guided technique in achieving femoral vein access in pediatric cardiac catheterization.

Assessment of Myocardial Function in Pediatric Patients with Operated Tetralogy of Fallot: Preliminary Results with 2D Strain Echocardiography

Abstract: The global myocardial function in patients after repair of tetralogy of Fallot (TOF) can be assessed by cardiovascular magnetic resonance (CMR) and measurement of B-type natriuretic peptides. Two-dimensional echocardiography-derived strain and strain rate (2D strain) facilitate the assessment of regional myocardial function. We evaluated myocardial function in 16 children with residual severe pulmonary valve regurgitation and right ventricular (RV) volume overload after TOF repair before, 1 month after, and 6 months after pulmonary valve replacement (PVR). In 2D strain echocardiography preoperatively, the longitudinal systolic RV strain was reduced (p < 0.05). One month after PVR, longitudinal systolic RV strain decreased further (p < 0.05), while systolic and early diastolic radial left ventricular strain and strain rate increased (each p < 0.05), followed by a return toward preoperative values after 6 months. Six months after PVR, preoperatively elevated RV end-diastolic volume (p < 0.01) assessed by CMR and N-terminal pro-B-type natriuretic peptide (p < 0.05) decreased. In conclusion, the impairment of the regional myocardial after TOF repair and transient changes after PVR can be subtly analyzed by 2D strain echocardiography in addition to the established assessment of myocardial function with CMR and measurement of B-type natriuretic peptides.

Cardiac malformations are increased in infants of mothers with epilepsy.

Abstract: We aimed to ascertain the prevalence of cardiac malformation (CM) and its association with antenatal exposure to an antiepileptic drug (AED) in infants of mothers with epilepsy (IMEs). Women with epilepsy (WWE) are enrolled in Kerala Registry of Epilepsy and Pregnancy (KREP) in the prepregnancy or early pregnancy period and are followed up with a standard protocol until the IMEs are 6 years old. At 3 months postpartum, a cardiologist, blinded to the AED exposure, carried out a clinical examination and echocardiography on all live-born babies. Patent foramen ovale (PFO) and interatrial septal defects of < 5 mm in size were excluded from CM. Details of maternal epilepsy, folate usage, AED exposure in the first trimester, and newborn characteristics were abstracted from the records of the KREP. We examined 462 babies. Maternal epilepsy was generalized in 201 (43.50%) or localization related in 241 (52.2%). The AED exposure was monotherapy in 262 (56.7%)—carbamazepine (112), valproate (71), phenobarbitone (43), phenytoin (31), and clonazepam (2)—and polytherapy in 126 (27.3%). Seventy-four infants (16.01%) had no AED exposure. There were 36 infants with CM (7.8%; 95% confidence interval: 5.5–10.6). CMs included atrial septal defect (26; 72.2%), tetrology of Fallot (3; 8.3%), patent ductus arteriosus and pulmonic stenosis (2 each; 5.6%), and ventricular septal defect, tricuspid regurgitation, transposition of great arteries (1 each; 2.8%). CMs were significantly more for IMEs with premature birth (p < .003). There was no association between CM and maternal age, epilepsy syndrome, seizure frequency during pregnancy, and folate use. CMs were more frequent with polytherapy (13; 10.3%) compared to monotherapy (17; 6.5%). Those with valproate exposure had a trend (not statistically significant) toward higher frequency of CM compared to IMEs on other AEDs as monotherapy.

Warfarin therapy for giant aneurysm prevents myocardial infarction in Kawasaki disease.

Abstract: We retrospectively investigated the effect of warfarin therapy in improving the clinical outcome of Kawasaki disease (KD) patients with giant coronary aneurysms (GAs). We followed 2350 KD patients from 1973 to 2004. The GAs (> or =8 mm in diameter) were diagnosed by coronary angiography. Sixty-eight patients (54 males and 14 females) were retrospectively studied. Patients were divided into two groups. One group consisted of 19 patients with 33 branches treated with a combination of low-dose aspirin and warfarin (target international normalized ratio: 1.5 - 2.5 IU). The second group consisted of 49 patients with 102 branches treated with low-dose aspirin without warfarin. The incidence of myocardial infarction was significantly less in the combination therapy group than in patients treated with aspirin without warfarin (1 patient vs. 16 patients, p < 0.05). Sudden death occurred in seven patients taking aspirin without warfarin, but none of the patients receiving warfarin died. No major bleeding events occurred in either group. Combination therapy of warfarin with aspirin is associated with a decreased risk of myocardial infarction in KD patients with GAs.

Treatment Strategies for Pediatric Patients with Primary Cardiac and Pericardial Tumors: A 30-Year Review

Abstract: This study reviewed different types of primary cardiac and mediastinal tumors in infants and children as well as their clinical presentation and management. Altogether, 34 consecutive patients followed from 1976 through December 2005 were analyzed. Of these 34 patients, 14 (41%) underwent surgery and 20 (59%) with rhabdomyomas were managed conservatively. Histologic examination of the surgically resected tumors showed teratoma in four patients, rhabdomyoma in four patients, and hemangioma in two patients. Myxoma, myofibroma, neuroblastoma, and malignant fibrous histiocytoma were encountered in one patient each. Follow-up evaluation was completed for 97% of the patients and extended up to 30 years. Half of the nonsurgical patients with rhabdomyoma showed partial or complete spontaneous regression. One patient died after resection of a malignant histiocytoma, and one patient required a tumor-related reoperation. Freedom from tumor-related reoperation after 10 years was 91% ± 8.7%. Of the survivors, 85% were New York Heart Association (NYHA) class 1, and 100% had sinus rhythm. Spontaneous tumor regression is common in rhabdomyoma and surgery, and is indicated only for symptomatic patients with hemodynamically significant intracardiac obstruction. For all other benign primary cardiac tumors, complete resection usually can be accomplished with good results. Patients with giant tumor masses compressing or infiltrating the heart frequently cannot undergo complete resection. For these patients, restoration/preservation of sufficient heart function is the primary goal. Malignant tumors are extremely rare in pediatric patients and have a very poor prognosis.

Impact of Postoperative Hyperglycemia following Surgical Repair of Congenital Cardiac Defects

Abstract: The objective of this study was to determine the prevalence of postoperative hyperglycemia in pediatric patients following surgery for congenital cardiac defects and its impact on morbidity and mortality. It was designed as a retrospective cohort study in a pediatric intensive care unit of a university-affiliated free-standing children’s hospital. A cohort of 213 patients who underwent 237 surgical procedures for repair or palliation of congenital cardiac defects comprised the study. Postoperative blood glucose measurements and all clinical and laboratory data were compiled for the first 10 days after surgery. The intensity and duration of hyperglycemia were analyzed for association with hospital morbidities and mortality. Mild and severe hyperglycemia were highly prevalent in our cohort (97% and 78%, respectively). Survivors had significantly lower peak (289.7 ± 180.77 mg/dl vs. 386 ± 147.95 mg/dl), mean (110.13 ± 36.22 mg/dl vs. 146.75 ± 57.12 mg/dl), and duration (2.59 ± 2.3 days vs. 5.35 ± 2.8 days) of hyperglycemia compared to nonsurvivors. Duration of hyperglycemia was independently associated with morbidity [odds ratio (OR): 1.95; p < 0.001] and mortality (OR: 1.41; p = 0.03) by multivariate logistic regression. Hyperglycemia is common in children following surgical repair or palliation of congenital cardiac defects. Postoperative hyperglycemia is associated with increased morbidity and mortality in these patients.

Cardiac Remodeling After Enzyme Replacement Therapy with Acid α-Glucosidase for Infants with Pompe Disease

Abstract: Background Infantile Pompe disease (glycogen storage disease type 2) is a fatal disorder caused by deficiency of acid α-glucosidase. This deficiency results in glycogen accumulation in the lysosomes of many tissues including cardiac muscle. The disease is characterized by profound hypotonia, poor growth, organomegaly, and cardiomegaly. Severe hypertrophic cardiomyopathy often is present in early infancy, and most patients die of cardiac or respiratory failure in the first year of life. This report describes the cardiac response of infants with Pompe disease to a phase 2 trial of enzyme replacement therapy (ERT).

Modeling the Fontan circulation: where we are and where we need to go.

Abstract: The Fontan procedure and its subsequent modifications over the past 30 years can be described as a class of surgical procedures for patients born with complex congenital heart disease exhibiting a single-ventricle physiology. The long-term outcome for children currently undergoing a Fontan procedure remains worrisome because of multiple late morbidities observed. Despite significant modeling efforts spanning three decades, improvements to the Fontan procedure have occurred without comprehensive validation from these modeling studies. Careful examination shows that modeling studies to date offer only a “glimpse through a keyhole” into understanding and modeling a representative range of the variations in anatomy and physiology that exist in Fontan patients. Suggestions for future investigations are provided.

Chest pain: characteristics of children/adolescents.

Abstract: Chest pain (CP) in children/adolescents is a common referral for the pediatric cardiologist. A group of 263 patients (141 males/122 females, mean age = 13.4 years, range = 5–22 years) with the primary complaint of CP underwent evaluation in the cardiac stress lab at Children’s Hospital of Wisconsin. Echocardiograms at rest were obtained in 70% of patients with no significant cardiac abnormalities identified. Endurance time (EXT) and oxygen consumption (VO2/kg) were below predicted in 26% and 46%, respectively. Reactive airway disease (RAD) as a preexisting condition was reported in 19% of patients, but abnormal resting pulmonary function (PFTs) were found in 26% (n = 68), with 48/68 never having the diagnosis of RAD. At risk of overweight (BMI >85th percentile), was seen in 28% of the cohort, with 16% identified as being overweight (BMI >95th percentile). A significant difference in RAD (p < 0.01) was seen in African Americans (AA) and decreased EXT (p = 0.01) was seen in Hispanics (H). VO2/kg was significantly reduced in both AA and H (p < 0.01). These results identify both racial and age-related differences in the etiology of CP in children. Most importantly, true cardiac pathology is extremely rare. AOW, deconditioning, and respiratory compromise play important roles in CP. The need for comprehensive cardiopulmonary monitoring is emphasized by these findings.

Echocardiography and the neonatologist.

Abstract: Pediatric echocardiography as performed and interpreted by pediatric cardiologists provides details of cardiac structure and function as well as hemodynamic data. Functional echocardiography, in contrast to echocardiography as performed by the cardiologist, is the bedside use of cardiac ultrasound to follow functional and hemodynamic changes longitudinally. Data reflecting cardiac function and systemic and pulmonary blood flow in critically ill preterm and term neonates can be monitored using this method. Functional echocardiography is being developed and driven by neonatologists as an extension of their clinical skills. A wealth of hemodynamic information can be derived from functional echocardiography used for the sick neonate, which provides clinical information different from the assumed underlying physiology. Lack of access to appropriate training programs and interdisciplinary politics is limiting the use of this potentially valuable clinical information. Without the use of functional echocardiography, clinicians are left to speculate as to the underlying pathophysiology of circulatory compromise, and the assumptions they make often are incorrect. For functional echocardiography to fulfill its clinical potential, it needs to be available at any time and at short notice in the neonatal intensive care unit (NICU). Because most NICUs do not have external diagnostic services to provide longitudinal hemodynamic follow-up assessment at the bedside, neonatologists should be able to develop appropriate echocardiographic skills in close collaboration with their cardiologist colleagues.

Endothelial Dysfunction and Oxidant Status in Pediatric Patients with Hemoglobin E-β Thalassemia

Abstract: Thalassemia major is characterized by anemia, iron overload, and oxidant damage to major organs, especially the cardiovascular system. Oxidative stress is ultimately involved in endothelial dysfunction, a condition which is evident in adults suffering from various cardiovascular diseases including thalassemia. We investigated endothelial function in pediatric patients with hemoglobin E-β thalassemia (HbE-β thalassemia), who have been exposed to excessive iron and oxidative stress for much shorter period than adults with thalassemia. We recruited 22 blood transfusion-dependent HbE-β thalassemia patients aged 11.8 ± 2.9 years and 20 healthy controls aged 12.1 ± 1.7 years. Oxidant status was determined, and endothelial function was assessed by a forearm blood flow technique. Oxidative stress was increased in the thalassemic patients, as blood glutathione (GSH) and ratios of reduced GSH to GSH disulfide were markedly reduced, and superoxide anion released from blood cells was highly elevated. Oxidative stress response, assessed by γ-glutamylcysteine ligase activity, was increased approximately twofold in thalassemia patients. Basal forearm blood flow was significantly increased in patients compared with controls (7.3 ± 1.8 vs 6.0 ± 1.8 ml/100 ml tissue/min, respectively), whereas forearm vasodilatory response to reactive hyperemia was depressed by 50% in patients compared with controls. Endothelial function is impaired in young thalassemia patients, and impaired endothelial function is associated with oxidant stress.

Safety and efficacy of carvedilol therapy for patients with dilated cardiomyopathy secondary to muscular dystrophy.

Abstract: Background By the age of 20 years, almost all patients with Duchenne’s or Becker’s muscular dystrophy have experienced dilated cardiomyopathy (DCM), a condition that contributes significantly to their morbidity and mortality. Although studies have shown carvedilol to be an effective therapy for patients with other forms of DCM, few data exist concerning its safety and efficacy for patients with muscular dystrophy. This study aimed to evaluate the safety and efficacy of carvedilol for patients with DCM.

Relation of Left Ventricular Hypertrophy to Microalbuminuria and C-Reactive Protein in Children and Adolescents with Essential Hypertension

Abstract: Microalbuminuria (MA) and C-reactive protein (CRP) levels are predictors of increased risk for left ventricular hypertrophy (LVH). Whether the strength of association between CRP and LVH is comparable to that of MA in hypertensive children is unknown. CRP and MA were measured in 64 children and adolescents with essential hypertension (HTN). In the entire population, CRP and MA showed positive relations with body mass index (BMI) (r = 0.30, p = 0.04 and r = 0.32, p = 0.04, respectively), systolic blood pressure (SBP) (r = 0.63, p = 0.03 and r = 0.58, p = 0.03, respectively), and LVH (r = 0.86, p < 0.001 and r = 0.81, p < 0.001, respectively). Patients with LVH (n = 23) had significantly higher BMI (p = 0.32), increased SBP (p = 0.031), and higher levels of CRP (p < 0.001) and MA (p < 0.001) compared with those without LVH. Multiple linear regression analysis demonstrated that CRP (r = 2.11, p < 0.001), MA (r = 1.94, p < 0.003), BMI (r = 0.53, p = 0.02), and SBP (r = 0.48, p = 0.04) were significantly associated with LVH. By analysis of covariance, CRP and MA were significantly different between patients who had LVH and those without LVH after adjustment for age, gender, BMI, SBP, SBP index, and diastolic blood pressure (p < 0.001 for the two markers). In conclusion, the strength of association between LVH and CRP is comparable to that of MA in children and adolescents with essential HTN.

Treatment of Pulmonary Hypertension in Children with Chronic Lung Disease with Newer Oral Therapies

Abstract: Chronic lung disease (CLD) is often complicated by chronic pulmonary vascular changes and pulmonary hypertension (PH) in young children. Current therapies for severe PH in such patients, including oxygen, inhaled nitric oxide, and parenteral prostacyclin, are often suboptimal, cumbersome, and expensive. Recently, oral endothelin receptor blockers and phosphodiesterase-5 inhibitors have been used successfully to control and reverse pulmonary vascular disease in idiopathic PH, but the use and efficacy of these agents in pediatric CLD have not been previously reported. We report a series of six children with CLD and severe PH treated with bosentan (six of six) and sildenafil (four of six). Vascular reactivity was assessed by cardiac catheterization prior to and after 6 months of therapy. Serial echocardiography was also used to assess response. Patients have been treated for 2.1-2.9 years (mean, 2.53 years). Response to therapy has included improvement in oxygenation, symptoms, echocardiographic parameters, and hemodynamics by cardiac catheterization. Transiently elevated liver enzymes were noted associated with viral respiratory infections in two subjects; no other adverse effects were noted. Three patients with large cardiac right-to-left shunts prior to therapy had subsequent shunt reversal, two of whom underwent shunt closure later. Oral therapy with bosentan alone or in combination with sildenafil improves PH in patients with CLD over a period of 3-4 years.

Multidetector CT Angiography and 3D Reconstruction in Young Children with Coarctation of the Aorta

Abstract: The purpose of this study was to assess the reliability of multidetector CT (MDCT) angiography and three-dimensional (3D) reconstruction in patients with coarctation of the aorta. Sixteen patients (median age, 2 months; range, 15 days to 28 months) with suspected coarctation of the aorta underwent both color Doppler echocardiography (CDE) and MDCT. In addition to the CT axial slices, 3D reconstructions such as volume rendering and multiple planar reformation were used to diagnose coarctation and associated cardiac abnormalities. The sensitivities of diagnosis were evaluated compared with MDCT and echocardiography and surgical findings. Sixteen cases were diagnosed as coarctation of the aorta by MDCT. This included hypoplasia of the aortic arch (n = 5). The results were confirmed at the time of surgery. Diagnosis sensitivities of coarctation were 87.5% for axial and 100% for both multiplanar and 3D volume-rendered images. Moreover, MDCT was able to clearly display the location and extent of the coarctation. Focal defects were observed in 14 cases, and diffuse defects in 2 cases. The sensitivity of MDCT diagnosis for coarctation of the aorta was 100%, which was higher than that of CDE (87.5%). Ventricular septum defect was diagnosed by MDCT in 11 cases, with 1 missed, and the sensitivity was 91.7% (11/12), which was lower than that of CDE (100%; 12/12). In addition, six patients had patent ductus arteriosus. Two patients in this study had collateral vessel formation. We conclude that CTA with 3D reconstruction represents a reliable noninvasive technique for the assessment of coarctation. It may serve as a noninvasive diagnostic tool before intervention or surgical treatment.

Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome.

Abstract: Historically, outcomes of patients with heterotaxy syndrome and congenital heart disease have been poor. Published series include patients treated over many decades or focus on specific patient/operative subgroups. This study was performed to evaluate midterm outcomes and determine anatomic risk factors for death in patients with all types of heterotaxy syndrome treated after 1985. We reviewed all infants with heterotaxy born between 1985 and 1997, presenting to one institution at or =5 years. Of 102 study patients (46 with asplenia phenotype, 56 with polysplenia phenotype), 48 (47%) died at a median age of 0.6 months, 12 without intervention. Survivors were followed for a median of 12.8 years (5-21.7 years). Independent predictors of mortality included obstructed totally anomalous pulmonary venous connection (TAPVC) (OR, 7.8; 95% CI, 1.9-32.9; p = 0.005), mild or greater atrioventricular (AV) valve regurgitation at presentation (OR, 3.5; 95% CI, 1.0-12.1; p = 0.03), and common AV canal (OR, 3.1; 95% CI, 1.1-8.5; p = 0.03). Sixteen patients developed pulmonary vein stenosis at a median age of 2 months, with 5 (31%) alive at follow-up. In patients with TAPVC, the mean indexed sum of pulmonary vein diameters was lower among patients who died than in survivors (42.3 +/- 8.3 vs 49.5 +/- 10.1 mm/m2; p = 0.02). Mortality remains high among patients with heterotaxy treated in the modern surgical era, particularly those with obstructed TAPVC. Pulmonary vein stenosis is common after repair of TAPVC in patients with heterotaxy, may be diagnosed beyond infancy, and is associated with poor outcomes.

Palivizumab use in subjects with congenital heart disease: results from the 2000-2004 Palivizumab Outcomes Registry.

Abstract: The Palivizumab Outcomes Registry prospectively collected data on 19,548 subjects who received respiratory syncytial virus (RSV) prophylaxis with palivizumab during the 2000–2004 RSV seasons. We evaluated the characteristics of enrolled registry subjects with congenital heart disease (CHD) over the four RSV seasons and examined additional information on these subjects collected in the 2002–2004 seasons. The percentage of registry subjects with CHD increased from 4.8% (102/2116) in the first season to 11.4% (688/6050) in the last season. Across all four seasons, 1500 subjects with CHD were enrolled, 71% of whom had acyanotic CHD. The proportion with cyanotic CHD increased from 19.6% (20/102) in the 2000–2001 season to 37.5% (258/688) in the 2003–2004 season, while the proportion of all CHD in the registry more than doubled during this time. The cumulative RSV hospitalization rate was 1.9% among patients with CHD who received prophylaxis. Among subjects with cyanotic and acyanotic CHD, hospitalization rates were 2.6% and 1.6%, respectively. Prospective data collected in the Palivizumab Outcomes Registry provide the largest published dataset available on infants with CHD receiving palivizumab and show low hospitalization rates and use consistent with prelicensure clinical trial data and revised American Academy of Pediatrics guidelines.

64-Slice Multidetector-Row Computed Tomographic Angiography for Evaluating Congenital Heart Disease

Abstract: This study aimed to assess critically the role of 64-slice multidetector-row computed tomographic (MDCT) angiography for evaluating congenital heart disease. The study enrolled 60 consecutive patients (median age, 4.7 years; median weight, 16.5 kg) with congenital heart disease who underwent 64-slice MDCT angiography during the period June 2006 through September 2007. The results were classified as diagnostic categories, and the impact of the procedure on strategizing management was critically analyzed. In each of the groups, the current technique offered a clear advantage over conventional imaging and provided specific clues for surgical/interventional management. A management algorithm was evolved based on questions frequently asked about pulmonary artery anatomy. The correlation with surgical anatomy in all cases that involved surgery was excellent. Early results suggest that 64-slice MDCT angiography is a major breakthrough in cardiovascular imaging with an important diagnostic and decision-aiding role. Diagnostic cardiac catheterization, especially for evaluating great vessel anomalies, could be largely replaced by the described technique for congenital heart disease.

Etiology, management, and outcome of pediatric pericardial effusions.

Abstract: The objective of this study was to determine the contemporary etiologies, treatment, and outcomes of moderate and large pericardial effusions in pediatric patients. We reviewed pediatric patients with moderate or large effusions diagnosed at Children's Hospital Boston. Effusion size was determined in offline review of echocardiograms. One hundred sixteen patients with moderate or large pericardial effusions were identified. The age range was 1 day to 17.8 years (median 8.6). The size of the pericardial effusions ranged from 0.5 to 4.7 cm (median 2.1). Neoplastic disease was present in 39% of patients, collagen vascular disease in 9%, renal disease in 8%, bacterial infection in 3%, and human immunodeficiency virus (HIV) in 2%; 37% were idiopathic. Pericardial drainage procedures were performed in 47 patients (41%). Of these, 29 (63%) had recurrent effusions leading to repeat drainage in 12 (41%). Pericardial effusions resolved within 3 months in 83% of patients who underwent drainage and in 91% of patients who did not. In summary, pediatric pericardial effusions were rarely caused by bacterial infections in this study population and were more frequently idiopathic or associated with neoplastic disease. Pericardial effusions often reaccumulated after drainage. The majority of both drained and undrained effusions resolved within 3 months.

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

Abstract: Background This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD).

Parental Understanding of Neonatal Congenital Heart Disease

Abstract: Background This study aimed to evaluate the impact of prenatal diagnosis on parental understanding of congenital heart disease (CHD) in newborns. Methods Consenting parents of newborns with CHD answered questions about the cardiac lesion, surgical repair, follow-up management, risk for CHD in future children, and maternal education before neonatal intensive care unit (NICU) discharge. A total understanding score was calculated (0–10) as the sum of five subscores: physician score, CHD score, surgery score, follow-up score, and reproduction score. Each category was scored as 0 (none correct), 1 (some correct), or 2 (all correct). The prenatal and postnatal diagnoses scores were compared. Results From June 2006 to November 2006, 50 families completed the questionnaire. Of these 50 families, 26 reported a prenatal diagnosis. The mean infant age when the parents were approached was 17.3 ± 13.3 days. The summary understanding score for the entire group was 6.3 ± 2.4 of 10. Multivariate regression analysis demonstrated a difference in scores between prenatal and postnatal diagnosis groups (p = 0.02) when control was used for maternal education. Prenatal diagnosis and maternal education (p < 0.01) had independent effects on the score. Conclusion Prenatal diagnosis increases parental understanding of neonatal CHD. Nevertheless, parental understanding remains suboptimal.

Variability in the Preoperative Management of Infants with Hypoplastic Left Heart Syndrome

Abstract: Infants with hypoplastic left heart syndrome (HLHS) commonly undergo initial surgical palliation during the first week of life. Few data exist on optimal preoperative management strategies; therefore, the management of these infants prior to surgery is anecdotal and variable. To more fully define this variability in preoperative care of infants with HLHS, a survey was designed to describe current preoperative management practices in the infant with HLHS. The questionnaire explored management styles as well as preoperative monitoring techniques and characteristics of the respondent's health care institution. The responses were compiled and are reported. A striking lack of consistency in preoperative management techniques for infants with HLHS is apparent. The impact of these preoperative strategies is unknown. Despite challenges in anatomic and hemodynamic variability at presentation, a prospective randomized controlled trial comparing ventilatory management techniques, enteral feeding strategies, and the utility of various monitoring tools on short- and long-term outcome is needed.

Differences in arterial compliance among normotensive adolescent groups: Collins arterial compliance in adolescents.

Abstract: Decreased arterial compliance is an important predictor of cardiovascular risk. Pulse wave velocity correlates well with arterial compliance. Gender and ethnic differences in adult populations have been described. However, few data are available evaluating arterial compliance in adolescent subjects. Using a simple noninvasive oscillometric technique, brachial–ankle pulse wave velocity (baPWV) was measured as an index of arterial stiffness. Measurements were performed on a cross-sectional (65% African American, 52% female) sample of 205 normotensive (blood pressure <95% for gender, height, and age) adolescents with a mean age of 15.9 years (range, 12–21 years). The 205 adolescent subjects include 106 females and 99 males. In these adolescents, the mean baPWV was higher for males (1,096 cm/s) than for females (1,039 cm/s; p < 0.0024; 95% confidence interval [CI], 0.2051–0.9349), and for African Americans (1,080 cm/s) than for whites (1,040 cm/s; p < 0.0438; 95% CI, 0.0112–0.7888). Multiple regression analyses found a three-way interaction among gender, ethnicity, and age. The effect of age on baPWV was greater among African Americans (slope = 18.1 cm/s/year) and males (slope = 21.6) than among whites (slope = 11.0) and females (slope = 11.3), although these differences did not reach statistical significance. Differences in arterial compliance are already present and detectable in normotensive adolescent subjects. Decreased arterial compliance among adolescent groups correlates with the known adult risk for cardiovascular events among the same ethnic and gender groups.

Electrocardiographic Criteria for Left Ventricular Hypertrophy in Children

Abstract: Previous studies to determine the sensitivity of the electrocardiogram (ECG) for left ventricular hypertrophy (LVH) in children had their imperfections: they were not done on an unselected hospital population, several criteria used in adults were not applied to children, and obsolete limits of normal for the ECG parameters were used. Furthermore, left ventricular mass (LVM) was taken as the reference standard for LVH, with no regard for other clinical evidence. The study population consisted of 832 children from whom a 12-lead ECG and an M-mode echocardiogram were taken on the same day. The validity of the ECG criteria was judged on the basis of an abnormal LVM index, either alone or in combination with other clinical evidence. The ECG criteria were based on recently established age-dependent normal limits. At 95% specificity, the ECG criteria have low sensitivities (<25%) when an elevated LVM index is taken as the reference for LVH. When clinical evidence is also taken into account, the sensitivity improved considerably (<43%). Sensitivities could be further improved when ECG parameters were combined. The sensitivity of the pediatric ECG in detecting LVH is low but depends strongly on the definition of the reference used for validation.

Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population

Abstract: An anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (interarterial coronary artery [IACA]) is a rare congenital anomaly associated with sudden cardiac death. The cardiac and surgical databases at a single pediatric institution were reviewed for a description of the clinical profiles and associated risk factors of this coronary anomaly. From 1994 to 2006, IACA was diagnosed for 31 patients with a median age of 6.2 years (range, birth to 16 years). The symptoms for 6 (19%) of the 10 patients (32%) presenting with symptoms were deemed to be cardiac in origin. The symptoms for the remaining 21 patients (68%) were incidental findings. Of the 31 patients, 29 (94%) had normal resting electrocardiograms (ECG). A total of 17 patients underwent dobutamine stress echo. None had wall motion abnormalities, but two had ECG changes indicating ischemia, and 4 had abnormal coronary flow, as detected by Doppler echocardiography. Seven patients, with either acute symptoms or testing suggestive of ischemia, underwent surgery. All seven had an interarterial left main coronary artery. There was one cardiac-related death. No sudden death was found in either the surgery or nonsurgery group during the mean follow-up period of 23 and 58 months, respectively. Whether surgical intervention modifies the natural history of the anomaly or not remains to be determined.

Prognostic value of B-type natriuretic peptide in surgical palliation of children with single-ventricle congenital heart disease.

Abstract: The objective of this prospective study was to assess the prognostic role of perioperative B-type natriuretic peptide (BNP) levels in infants and children with single-ventricle congenital heart disease undergoing Norwood, bidirectional cavopulmonary anastomosis (BCPA), or Fontan operation. BNP levels were measured at baseline, after cardiopulmonary bypass, 6 to 12 hours after surgery, and then daily until indwelling vascular catheters were removed. Outcome measures included length of mechanical ventilation, inotropic support, and hospital stay. Twenty subjects underwent 23 surgical procedures (13 Norwood, 5 BCPA, and 5 Fontan). BNP levels were significantly higher in patients undergoing a Norwood procedure compared with a BCPA or Fontan procedure (p < 0.01). BNP levels measured 6 to 12 hours after surgery were predictive of duration of hospitalization (p = 0.005) and inotropic support (p = 0.01). An increase in BNP level within 48 hours of extubation was observed in 92% of patients undergoing a Norwood procedure. Early postoperative BNP levels correlate significantly with the ensuing duration of inotropic support and length of hospitalization. An increase in BNP after extubation may be reflective of the degree of underlying cardiopulmonary instability. Further investigation is necessary to define this important relation.

Competition between native flow and graft flow after coronary artery bypass grafting. Impact on indications for coronary artery bypass grafting for localized stenosis with giant aneurysms due to Kawasaki disease.

Abstract: We report the postoperative course of native and graft flow after coronary artery bypass grafting (CABG) in two patients with giant aneurysms and localized stenosis due to Kawasaki disease (KD). Although both patients had undergone CABG to the left anterior descending artery (LAD) with the left internal thoracic artery (ITA), at 5 and 10 years old, respectively, the ITA grafts were occluded 1 month postsurgery. However, when the two patients suffered complete occlusion of the native LAD more than 10 years after surgery, angiograms showed that the ITA grafts had reopened. We believe that this postoperative course reflects competition between the native artery flow and graft flow after CABG. CABG in patients with severely delayed coronary flows or recurrence of thrombus in giant aneurysms was ineffective in preventing myocardial infarction or damage. We conclude that CABG in giant aneurysm without significant localized stenosis should be avoided.