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An increasing number of children with congenital heart disease are surviving into adulthood, creating new, unusual patients with different physiologic and anatomic problems for the adult cardiologist. This article discusses those lesions affecting primarily the right ventricle.
Growth failure in infants with congenital heart disease was investigated by studies of food intake, change in body weight, oxygen consumption, carbon dioxide production, and lean body mass. Infants with congenital heart disease weighed less initially and gained less weight during observation than normal infants. The daily intake of calories per kilogram body weight was inadequate for some infants and considered generally adequate for others. Lean body mass was normal, and the quantity of oxygen used for metabolism was similar in both groups. Infants with congenital heart disease were not found to be hypermetabolic when oxygen consumption was related to lean body mass. The growth failure seen in these infants appears to be most appropriately related to inadequate calorie intake rather than to any other factor studied.
OBJECTIVE: Vascular endothelial growth factor and basic fibroblast growth factor are potent stimulators of angiogenesis. Children with cyanotic congenital heart disease often experience the development of widespread formation of collateral blood vessels, which may represent a form of abnormal angiogenesis. We undertook the present study to determine whether children with cyanotic congenital heart disease have elevated serum levels of vascular endothelial growth factor and basic fibroblast growth factor. METHODS: Serum was obtained from 22 children with cyanotic congenital heart disease and 19 children with acyanotic heart disease during cardiac catheterization. Samples were taken from the superior vena cava, inferior vena cava, and a systemic artery. Vascular endothelial growth factor and basic fibroblast growth factor levels were measured in the serum from each of these sites by enzyme-linked immunosorbent assay. RESULTS: Vascular endothelial growth factor was significantly elevated in the superior vena cava (P =.04) and systemic artery (P =.02) but not in the inferior vena cava (P =.2) of children with cyanotic congenital heart disease compared to children with acyanotic heart disease. The mean vascular endothelial growth factor level, determined by averaging the means of all 3 sites, was also significantly elevated (P =.03). Basic fibroblast growth factor was only significantly elevated in the systemic artery (P =.02). CONCLUSION: Children with cyanotic congenital heart disease have elevated systemic levels of vascular endothelial growth factor. These findings suggest that the widespread formation of collateral vessels in these children may be mediated by vascular endothelial growth factor.
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Pregnancy rates for mothers of infants with conotruncal cardiac malformations and mothers of healthy control infants were compared for a 3-year period. Mothers of infants who died of congenital heart disease had the highest pregnancy rates, followed by control mothers, and then by mothers of surviving infants with congenital heart disease. Comparison in relation to the number of living children showed similar subsequent pregnancy rates for mothers of deceased infants with congenital heart disease and mothers of control infants, but lower rates for mothers of surviving infants with congenital heart disease. "Replacement" of a deceased infant occurs frequently, apparently in order to achieve a desired family size, whereas decreased reproduction in families of living infants with congenital heart disease may reflect the psychosocial and economic impact of the continuing care of a child with severe heart disease.
AIM: To assess whether four-dimensional (4D) spatiotemporal image correlation (STIC) can provide unique and novel information for diagnosing complex congenital heart diseases. METHODS: A dynamic interactive 4D ultrasound system was used to examine 21 fetuses with complex congenital heart diseases and 25 fetuses with no apparent anomalies referred for prenatal diagnosis and management. Data acquisition was achieved by an automatic sweep device capable of image capture across the entire fetal heart and thorax. An offline analysis of the acquired volume was carried out to examine the fetal cardiac anatomy with multiplanar mode and surface rendering. A simplified "spin" technique was used to evaluate the outflow tracts of the fetal heart. Results. Successful acquisition was possible in all 46 fetuses. STIC volume datasets were displayed interactively as a series of 3 orthogonal planes, with the ability of the examiner to control the pivot point, rotational axis, and parallel shift of each image plane inside the volume. The reformatted slices or surface-rendered images allowed the visualization of certain structures and projections not demonstrated with conventional fetal echocardiography. The information derived from STIC was either beneficial (8 patients) or crucial (5 patients) for establishing the diagnosis of congenital heart diseases in the study group. Conclusions. This study demonstrated that the 4D STIC could provide unique and independent information from the routine use of fetal echocardiography that served for diagnosis of complex congenital heart diseases.
OBJECTIVE: To examine the presence of depression and anxiety in adults with congenital heart disease and the association of medical severity with depression and anxiety. DESIGN: Prospective, pilot study. SETTING: An adult outpatient cardiology clinic in university-affiliated children's hospital in Northeast. PATIENTS: Twenty-two adult patients with congenital heart disease followed in an adult cardiology clinic. Patients were selected who had no evidence of emotional or behavioral difficulties, (ie, no symptoms of depression or anxiety). Outcome Measures Standardized semi-structured psychiatric interview with structured checklist eliciting Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) diagnostic criteria for depressive and anxiety disorders, Brief Symptom Inventory (BSI), and the Cardiologist's Perception of Medical Severity scale. RESULTS: Among patients who had been assumed to be "well adjusted," 36.4% were experiencing a diagnosable psychiatric disorder, with anxiety or depressive symptoms being prominent. There were 6 patients (27.3%) who had BSI scores above 63 indicating pathological emotional functioning. There was significant convergent validity between the clinical diagnoses of depression and anxiety using both diagnostic interviews to identify DSM-IV diagnostic conditions and the BSI. There were significant associations between patient's medical severity scores and with the DSM-IV diagnosis of depression and the BSI global index score and depression subscale. CONCLUSIONS: This pilot study raises important concerns about the emotional functioning of many adults facing congenital heart disease, particularly those with complex lesions. From a clinical perspective, this work suggests that health care professionals should be alert for emotional difficulties and the possible need for psychological intervention for adult survivors of congenital heart disease even among those that are seemingly emotionally well adjusted.
BACKGROUND: Pulmonary arterial hypertension (PAH) associated with congenital heart disease is usually the result of a large systemic-to-pulmonary shunt, and often leads to right ventricular failure and early death. The purpose of this study was to determine the prevalence of PAH among adult patients included in a national registry of congenital heart disease and to assess the relation between patient characteristics and PAH. METHODS: Patients with PAH associated with a septal defect were identified from the registry. Gender, age, underlying diagnosis, previous closure, age at repair and NYHA classification were recorded. PAH was defined as a systolic pulmonary arterial pressure (sPAP) greater than 40 mm Hg, estimated by means of echocardiographical evaluation. RESULTS: The prevalence of PAH among all 5970 registered adult patients with congenital heart disease was 4.2%. Of 1824 patients with a septal defect in the registry, 112 patients (6.1%) had PAH. Median age of these patients was 38 years (range 18-81 years) and 40% were male. Of these patients, 58% had the Eisenmenger syndrome. Among the patients with a previously closed septal defect, 30 had PAH (3%). Ventricular septal defect (VSD) was the most frequent underlying defect (42%) among patients with PAH and a septal defect. Female sex (Odds ratio=1.5, p=0.001) and sPAP (Odds ratio=0.04, p<0.001) were independently associated with a decreased functional class. CONCLUSION: PAH is common in adult patients with congenital heart disease. In our registry the prevalence of PAH in septal defects is around 6%. More than half of these patients have the Eisenmenger syndrome, which accounts for 1% of the total population in the CONCOR registry. Whether the prevalence of PAH will decrease in the future as a result of early detection and intervention remains to be awaited.
13 cases of congenital heart diseases in patients having atrial situs inversus and dextrocardia, all studied by means of angiography, are described following an up to date segmental approach. The type of atrioventricular connection was concordant in the 11 biventricular hearts; double inlet in the 2 univentricular hearts. The mode was always via two atrioventricular valves. In two cases the atrioventricular flows realized a criss-cross condition, being horizontal the interventricular septum, superior the morphologically right ventricle and inferior the morphologically left ventricle. In none of the univentricular hearts was an accessory chamber identified; they were both considered to be of indeterminate type. Ventriculo-arterial connections were: concordant in 3 cases (23%), including one case of Tetralogy with pulmonary atresia; discordant in 3 cases (23%); double outlet in 7 cases *54%). The aortic valve was in the assumed position (left and posterior to the pulmonary valve) only in the 3 cases with concordant as a rule. A ventricular septal defect was actually found in all cases; in one it was associated with pulmonary vascular disease. Obstruction to the pulmonary flow (subvalvular, valvular or vascular) was ascertained in 85% of cases, whereas aortic obstruction was never found. Overall, important congenital heart malformations appear to be frequently associated with situs inversus and dextrocardia. The ventriculo-arterial junction is identified as the most affected point.
AIM: To investigate the performance of routine neonatal and 6 week examinations for detecting congenital heart disease. METHODS: A retrospective review of findings on clinical examination was conducted of a cohort of live born infants with congenital heart disease in one health region in 1987-94. RESULTS: Of 1590 babies with congenital heart disease, 523 (33%) presented before neonatal examination because of symptoms or non-cardiac abnormalities. 1061 underwent routine neonatal examination which was abnormal in 476 (45%), but only 170 were referred directly for diagnosis. Of 876 discharged with no diagnosis, 306 presented or died undiagnosed before 6 weeks. At 6 weeks 252 of 569 babies underwent a second routine examination which was abnormal in 164 (65%). CONCLUSIONS: Routine neonatal examination fails to detect more than half of babies with heart disease; examination at 6 weeks misses one third. A normal examination does not exclude heart disease. Babies with murmurs at neonatal or 6 week examinations should be referred for early paediatric cardiological evaluation which will result either in a definitive diagnosis of congenital heart disease or in authoritative reassurance of normal cardiac anatomy and function.
To clarify which hemodynamic measurement correlates best with lung mechanics in infants with congenital heart disease and left-to-right shunts, dynamic pulmonary function tests and echocardiography were performed in 26 infants with such disease (study infants) and in 37 normal, healthy infants (control infants). The tidal volume and pulmonary compliance (CL) were lower and airway resistance higher in infants with congenital heart disease than in control infants. A significant correlation was demonstrated between CL, expiratory resistance (Re), and the right pulmonary artery-to-aortic size ratio (RPA/DAO). CL and Re also correlated well with the corrected acceleration time square root of RR ratio (ACT/square root of RR: ACT, acceleration time and RR: length of the cardiac cycle) of pulmonary flow velocity. Stepwise multiple regression analysis revealed that RPA/DAO correlated best with both CL and Re. It is concluded that infants with congenital heart disease and left-to-right shunts have lower lung compliance and higher expiratory airway resistance than normal children, and that RPA/DAO is the echocardiographic parameter that correlates best with the changes in lung mechanics.
The high risk of vaso-occlusive events in children younger than 4 years with cyanotic congenital heart disease and polycythaemia has been attributed to increased thromboxane (Tx) A2 formation. In older children with cyanotic congenital heart disease, however, the risk of vaso-occlusive events is much lower. We therefore hypothesized that the formation of TxA2 and prostacyclin is not disturbed in this age group. We measured urinary excretion of stable index metabolites of in vivo TxA2 and prostacyclin formation by gas chromatography-mass spectrometry in nine children (age 5.9-14.4, median 8.7 years) with cyanotic congenital heart disease, and in nine healthy, age-matched control subjects. The patients excreted less 2,3-dinor-TxB2 (systemic TxA2 formation, P = 0.03), 2,3-dinor-6-keto-PGF1 alpha (systemic prostacyclin formation. P = 0.03) and TxB2 (renal TxA2 formation, P = 0.01) than the control subjects. We conclude that in children older than 5 years with cyanotic congenital heart disease, endogenous synthesis of TxA2 and prostacyclin is not stimulated. This result may explain the lower risk of vaso-occlusive events in this age group as compared with younger children. In addition, our results suggest that chronic hypoxaemia may affect the in vivo formation of TxA2 and prostacyclin and the metabolic disposition of TxB2.
PURPOSE OF REVIEW: This paper reviews the past year's literature on computerized outcomes analysis for congenital heart disease. RECENT FINDINGS: This review focuses on recent advances in four areas of computerized outcomes analysis for congenital heart disease: nomenclature, database, complexity adjustment, and data verification. A common nomenclature, along with a common core minimal dataset, were adopted by the European Association for Cardio-thoracic Surgery and the Society of Thoracic Surgeons and published in 2000 in the Annals of Thoracic Surgery; the thrust towards the establishment of an international nomenclature is now being developed further by the International Society for Nomenclature of Pediatric and Congenital Heart Disease [http://www.IPCCC.net]. This common nomenclature and common minimum database dataset, created by the International Congenital Heart Surgery Nomenclature and Database Project, are now used by the European Association for Cardio-thoracic Surgery and Society of Thoracic Surgeons since 1998, this nomenclature and database have been used to analyze outcomes of over 40 000 patients. Two major multi-institutional efforts have attempted to measure case complexity: the Risk Adjustment in Congenital Heart Surgery-1 and the Aristotle Complexity Score. Collaborative efforts involving the European Association for Cardio-thoracic Surgery and Society of Thoracic Surgeons are underway to develop mechanisms to verify data completeness and accuracy. SUMMARY: Methods of congenital heart disease outcomes analysis continue to evolve, with continued advances in four areas: nomenclature, database, complexity adjustment, and data verification.