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PubMed2003

Birth cohort effect of the mortality rate from congenital heart disease in Japan.

BACKGROUND: The mortality rate from congenital heart disease in Japan had decreased partly because the great improvement of medical treatment and care. Therefore, the patients who would have died of congenital heart disease in younger age in the past may be alive to be adult, and the number of adults with congenital heart disease might increase. If the management of such adults did not improve, the mortality rate from congenital heart disease might increase because of the increased number. METHODS: Using the Japanese vital statistics from 1960 through 1999, we observed the time series of the mortality rate from congenital heart disease by age and sex. The birth cohort consisted of those who were bone in 5-year period starting in 1960. The mortality rate was the sum of the number of death in every five years divided by the population of the center year. RESULTS: The transition of the mortality rate from congenital heart disease for age group 0 to 4 years was decreased since 1973. Other age groups showed decreased mortality rate since late 1960's. The birth cohort analyses showed that the mortality rate of each birth cohort was decreased as time passed, especially the age group 5 to 9 years old and 10 to 14 years old. CONCLUSION: Birth cohort effect of mortality from congenital heart disease exists in Japan.

Adolescent↗
PubMed2006

[Multislice CT scan in congenital heart disease].

The management of congenital cardiovascular malformations is a diagnostic challenge. It requires accurate assessment of the intra- and extra-cardiac malformations. Three-dimensional imaging by the multislice CT scanner is now a routine investigation for congenital heart disease, complementary to echocardiography, and, increasingly, an alternative to conventional angiography. Three dimensional imaging is particularly useful in the diagnosis of complex congenital heart disease, in the preparation of complex investigations of interventional cardiac catheterisation and the postoperative evaluation of surgical repair. This report describes the different acquisition protocols adapted for children and illustrates the present role of volumic CT scanner in congenital heart disease by clinical examples of everyday clinical practice.

Child↗
PubMed1999

Congenital heart disease: six decades of progress.

Congenital heart malformations occur in approximately 1 in 100 live births. Since Robert Gross successfully ligated a persistent ductus arteriosus 60 years ago, there has been enormous progress in the surgical management of even the most complex lesions. More recently, the interventional cardiologist armed with balloons, stents, coils, umbrellas, and laser beams is providing an alternative to surgery for many lesions. Echocardiography combined with Doppler studies is now the most informative diagnostic modality, and its usefulness is being expanded by transesophageal studies. Magnetic resonance imaging is helpful for some lesions not well visualized on echo. Some complex lesions continue to challenge caregivers, especially the hypoplastic left heart syndrome and several variants of pulmonary atresia. Neurological complications in patients with congenital heart disease are not uncommon and are the main theme of this issue.

Cardiac Surgical Procedures↗
PubMed1993

Risk of stroke in adults with cyanotic congenital heart disease.

BACKGROUND: Adults with cyanotic congenital heart disease and elevated hematocrit levels are often phlebotomized because of an assumed risk of cerebral arterial thrombotic stroke. Whether a relation exists between hematocrit level, symptomatic erythrocytosis (hyperviscosity), and stroke remains to be established in this patient population. METHODS AND RESULTS: Accordingly, 112 cyanotic patients 19-74 years old (mean, 36 +/- 11.7 years) in the UCLA Adult Congenital Heart Disease Center Registry were selected for study by virtue of continuous observation for 1-12 years (total, 748 patient-years). Patients with independent risk factors for embolic or vasospastic stroke were excluded. The study patients were then divided into two groups: 1) compensated erythrocytosis (stable hematocrit levels of 46.0-72.7% [mean, 57.5 +/- 7.2%], iron replete, absent or mild hyperviscosity symptoms), and 2) decompensated erythrocytosis (unstable rising hematocrit levels of 61.5-75.0% [mean, 69.5 +/- 10.6%], iron deficiency, marked-to-severe hyperviscosity symptoms). No patient with either compensated or decompensated erythrocytosis, irrespective of hematocrit level, iron stores, or the presence, degree, or recurrence of cerebral hyperviscosity symptoms, progressed to clinical evidence of a complete stroke (cerebral arterial thrombosis with brain infarction). CONCLUSIONS: Because a risk of stroke caused by cerebral arterial thrombosis was not demonstrated, because the circulatory effects of phlebotomy are transient, and because of the untoward sequelae of phlebotomy-induced iron deficiency, we recommend phlebotomy for the temporary relief of significant, intrusive hyperviscosity symptoms but not for the hematocrit level per se. According to our data, phlebotomy is not warranted to reduce an assumed risk of stroke because that risk did not materialize.

Adult↗
PubMed2001

Evaluation of prenatal diagnosis of congenital heart diseases by ultrasound: experience from 20 European registries.

OBJECTIVES: To evaluate prenatal diagnosis of congenital heart diseases by ultrasound investigation in well-defined European populations. DESIGN: Data from 20 registries of congenital malformations in 12 European countries were included. The prenatal ultrasound screening programs in the countries ranged from no routine screening to three ultrasound investigations per patient routinely performed. RESULTS: There were 2454 cases with congenital heart disease with an overall prenatal detection rate of 25%. Termination of pregnancy was performed in 293 cases (12%). There was considerable variation in prenatal detection rate between regions, with the lowest detection rates being in countries without ultrasound screening (11%) and in Eastern European countries (Croatia, Lithuania and Ukraine; 8%). In Western European countries with ultrasound screening, detection rate ranged from 19-48%. There was a significant difference in prenatal detection rate and proportion of induced abortions between isolated congenital heart disease and congenital heart disease associated with chromosome anomalies, multiple malformations and syndromes (P < 0.0001). There were 1694 cases with isolated congenital heart disease of which 16% were diagnosed prenatally. Malformations affecting the size of the ventricles were detected prenatally in half of the cases. CONCLUSIONS: Prenatal detection rate of congenital heart disease varies significantly between countries even with the same screening recommendations. The presence of associated malformations significantly increases the prenatal detection rate.

Abortion, Induced↗
PubMed2002

The index of pulmonary vascular disease in children with congenital heart disease: relationship to clinical and haemodynamic findings.

OBJECTIVE: We asked whether a scoring system [index of pulmonary vascular disease (IPVD)] that quantifies the individual pulmonary vascular pathology would relate to postoperative survival in patients with congenital heart disease and pulmonary hypertension (PH). METHODS: Lung biopsy specimens from 28 patients at a median age of 6 months (1 month to 21 years) were analysed qualitatively and morphometrically. The IPVD and other morphometric parameters were related to haemodynamic findings and survival. RESULTS: Mean pulmonary artery pressure (PAP) was 44 mmHg (15-72 mmHg), and the resistance to pulmonary perfusion was 5 U x m(2) (0.9-14 U x m(2)). There were three early (in-hospital) and three late deaths during the follow-up period of 2.5 years (6 months to 7 years). Incipient plexiform lesions were observed in one infant with trisomy 21 and complete atrioventricular septal defect (cAVSD). An IPVD score above the upper critical limit (>2.2) was not observed during the first year of life. On discriminant analysis, morphometric parameters could not predict mortality ( P=0.08). CONCLUSIONS: The IPVD is not helpful to predict surgical mortality during the first year of life. Patients with trisomy 21 and cAVSD may show advanced pulmonary vascular disease in infancy.

Child↗
PubMed1984

Pulmonary vascular disease in different types of congenital heart disease. Implications for interpretation of lung biopsy findings in early childhood.

Pulmonary vascular structure was studied by analysing serial reconstructions of the arterial pathways and random sections of tissue in the lungs of 16 children who died with different types of congenital heart disease and pulmonary hypertension. Cases of ventricular septal defect showed an appreciable increase in muscularity of both preacinar and intra-acinar (respiratory unit) arteries, and intimal proliferation was infrequent and mild. By contrast, cases of transposition of the great arteries with ventricular septal defect and atrioventricular septal defect showed an increase in preacinar muscularity, a short heavily muscularised arterial segment containing intimal proliferation at the entrance to the acinus, whereas the intra-acinar arteries beyond showed only a moderate increase in muscularity. In these children who were less than 1 year of age an increase in pulmonary vascular resistance was due to strategically placed small areas of intimal proliferation and not to widespread obliterative pulmonary vascular disease. The study demonstrated and explained differences in the appearance of the peripheral pulmonary arteries in different types of congenital heart disease, which help interpret the findings of lung biopsies.

Adolescent↗
PubMed2001

Epidemiological study of congenital heart disease.

A community based survey of congenital heart disease was carried out on a random sample of 11,833 children below 15 years in Delhi, India. Congenital heart disease was diagnosed on clinical history and/or clinical examination. Out of the examined sample, 50 were found suffering from congenital heart disease, giving an overall prevalence of 4.2/1000 (4.6/1000 in boys and 3.7/1000 in girls). The prevalence rate was slightly higher than other studies carried out in the country. The reason is obvious. The present study is community based while other studies were hospital or school based. The type of lesions were similar to those reported from other studies. The lesions in order of frequency were ventricular septal defect (46%), atrial septal defect (18%), patent ductus arteriosus (14%), Fallot's tetrology (10%), aortic stenosis (4%), and pulmonary stenosis (4%). Like other studies ventricular septal defect was the commonest lesion. The prevalence rate was higher in the age group 0-4 years and 5-9 years in boys whereas it was higher in adolescent age group (10-14 years) in girls.

Adolescent↗
PubMed2005

Mechanism of myocardial microvessel formation in cyanotic congenital heart disease.

BACKGROUND: Patients with cyanotic congenital heart disease (C-CHD) usually have myocardial thickening and fibrosis, both of which can affect the course of surgical management. Hypoxia and ischemia may stimulate microvessel formation in the myocardium, which may accelerate the myocardial thickening and fibrosis. Whether hyperplasia of microvessels occurs in the myocardium of C-CHD was investigated in this report. METHODS AND RESULTS: The patients were divided into 2 groups; the C-CHD group (n = 22), and the acyanotic congenital heart disease (A-CHD) group (n = 24). The microvessels and vascular endothelial growth factor (VEGF) mRNA of the myocardium were detected by immunohistochemical staining assay and real-time quantitative reverse transcriptase polymeric chain reaction, respectively. The serum VEGF levels were measured by using enzyme-linked immunosorbent assay. The results were that: (1) the number of microvessels in the myocardium were more in the C-CHD group than in A-CHD group; (2) the serum VEGF levels in the C-CHD group vs the A-CHD group were higher in the preoperative period (p < 0.001), but there was no difference after operation; and (3) VEGF protein and the expression of VEGF mRNA in the myocardium were higher in the C-CHD group than in the A-CHD group (p < 0.01). CONCLUSIONS: Myocardial microvessels formed in the myocardium of patients with C-CHD, possibly mediated by increasing VEGF levels (for this group of patients).

Adolescent↗
PubMed1984

The incidence of urinary tract malformations in congenital heart disease.

A statistically significant number of congenital heart disease patients were evaluated for urinary tract anomalies. Twelve percent (160 cases among 1340 patients) had such associated anomalies. However, no association between a specific congenital heart disease and a particular urinary tract anomaly could be found. The results were very similar to those reported in clinical series, but slightly higher than those found at autopsy. The percentage of urinary tract malformations varied over a wide range, with some congenital heart disease having a much higher association than others. However, the total average incidence of urinary tract malformation was similar to that of the general population.

Adolescent↗
PubMed1999

Spinal fusion in patients with congenital heart disease. Predictors of outcome.

The strong association between congenital heart disease and spinal deformity is well established, but data on the risks and outcome of spinal fusion surgery in patients with congenital heart disease are scarce. The purpose of this study was to identify predictors of perioperative risk and outcome in a large series of children and adolescents with congenital heart disease who underwent spinal fusion for scoliosis or kyphosis. In the authors' retrospective analysis of 74 consecutive patients with congenital heart disease undergoing spinal fusion, there were two deaths (2.7%) and 18 significant complications (24.3%) in the perioperative period. Preoperative cyanosis (arterial oxygen saturation < 90% at rest) with uncorrected or incompletely corrected congenital heart disease was associated with both deaths. Complications occurred in nine of 18 (50%) patients with cyanosis and in 11 of 56 (20%) patients without cyanosis. As judged by multivariate analysis the best predictors of perioperative outcome were the overall physical status of the patient as represented by the American Society of Anesthesiologists' preoperative score and a higher rate of intraoperative blood loss. Seventeen of 43 patients (40%) with an American Society of Anesthesiologists score of 3 or higher experienced complications including two perioperative deaths. Successful spinal fusion and correction were achieved in 97% of patients. Children and adolescents with congenital heart disease can undergo elective spinal fusion with risks that relate to overall cardiac status. Careful assessment of preoperative status by pediatric cardiologists and cardiac anesthesiologists familiar with surgical treatment of patients with congenital heart disease will assist the orthopaedic surgeon in providing the most realistic estimate of risk.

Adolescent↗
PubMedDate not supplied

[Morphometric study of the lungs (correlation of alveolar and interstitial tissue) in children with congenital heart diseases].

116 autopsies have been performed. Congenital heart diseases caused premature death of these children. Morphometric assessment of the correlation of total alveolar space and interstitial tissue volume was made. This correlation doesn't differ between a group of children with congenital heart diseases characterized by increased lung circulation and a control one consisting of healthy children. Considerable decrease in total alveolar space and increase in interstitial tissue volume has been noted in children with congenital heart diseases characterized by decreased lung circulation. It proves that hypoxia in children with such conditions is also caused by decrease in number of alveoli.

Autopsy↗
PubMed2006

The impact of fetal echocardiography on the prevalence of liveborn congenital heart disease.

Fetal echocardiography allows for early detection of congenital heart disease, and pregnancy termination may be an option in cases of complex defects. In the current study, the most important factors contributing to the diagnosis and termination of affected pregnancies are reviewed and their combined effect on the future prevalence of liveborn congenital heart disease is evaluated. The relative reduction of the prevalence of the most severe forms of congenital heart disease is estimated as the product of the probability that (1) a fetal cardiac screening is performed (p (evaluation)), (2) an affected pregnancy is detected (P (detection)), (3) pregnancy termination is decided following antenatal diagnosis (P (decision)). In areas where termination of pregnancy is a realistic and supported option, a universal sonographic screening of all pregnancies (P (evaluation) = 1), with an average reported sensitivity of 35% and a termination rate of 43% following antenatal diagnosis, would result in a 15% overall reduction of the prevalence of the most severe forms of congenital heart disease. However, wide variability exists regarding the defect-specific estimates (2-50% prevalence relative reduction) due to considerable differences in the reported diagnostic sensitivity and termination rates associated with each heart defect. If an earlier diagnosis could be achieved, which is reported to be associated with an average 1.4-fold increased probability of termination, the overall reduction of the prevalence of congenital heart disease could approach 21%. As the skills of obstetric and pediatric cardiology sonographers improve, fetal echocardiography is expected to have a substantial impact on the future epidemiology of liveborn congenital heart disease.

Echocardiography↗
PubMed2006

[Critical congenital heart diseases in Tuzla Canton area].

Critical congenital heart diseases (CHD) represent extensive and complex possibilities changes of morphology and homodynamic on the heart, which vital jeopardize child at the time of birth so as at the first weeks of its life or at the first months of its life too. The purpose of this study is to determine frequency, structure and the mode of medical treatment critical UAS in the Tuzla Canton area to harmonies with experiences and results we have reached in order to treat children who have congenital heart disease. There was discovered CHD in the 493 (8,15 per 1000 live-born) from 60.493 live-born children in Tuzla Canton area during the 10-year period start at 01.01.1995. Critical congenital heart disease was discovered in 142 children what represent total prevalence of 2,35 per 1000 live-born children. Yearly prevalence was pretty consistent in observed period of the time and it was from 1,36 to 3,24 per 1000 live born children. The most frequent UAS was complete atrio ventricular canal, which has proved in the 22,4% critical UAS. The more then one-third (36,6 %) children with critical UAS have had extra cardiac anomalies and syndromes. The balloon septostomy was performed in only two of our patients, in 41 (28,8 %) cardiac surgery was performed. Only one of the children underwent surgery in the neonatal period. In 8 patients who were no surgery treated developed pulmonary hypertension, one of them had bacterial endocarditic. Of the total 142 children with critical CHD 99 (69,7%) died. The 92 of them died before surgery, 7 of them died after the surgery; 3 of the 7 in the early after surgery period; 4 of the 7 died because of complications in a later period. Timely discovering critical UAS in Tuzla Canton area is failure in the area prenatal diagnosis. The treatment of patients is inappropriate and without possibilities of emergency invasive cardiac treatment and surgical treatment.

Adolescent↗
PubMedDate not supplied

Immunologic profile of patients with protein-losing enteropathy complicating congenital heart disease.

The immunologic profile of patients with congenital heart disease complicated by protein-losing enteropathy (PLE) is undefined. The aim of this study was to assess the lymphocyte subpopulation and immunglobulin (Ig) pattern in patients with PLE complicating congenital heart disease. The immunologic profile of six patients with congenital heart disease complicated by PLE was compared to that of controls without PLE matched for age and cardiac interventions. Enteric protein loss was documented by Tc99m-labeled albumin scintigraphy. The lymphocyte subpopulations were enumerated using flow cytometry, whereas serum IgG, IgA, and IgM concentrations were measured by the turbidimetric technique. The cardiac diagnoses included complex cyanotic heart disease post-Fontan procedure (n = 3), and one each of tetralogy of Fallot, restrictive cardiomyopathy, and valvar pulmonary stenosis. In patients with PLE, the T lymphocyte (CD3+) count was significantly lower (300 +/- 186 vs 2070 +/- 1171/microl, p = 0.017); both the helper/inducer lymphocytes (CD4+) (127 +/- 158 vs 927+/- 377/microl, p = 0.006) and suppressor/cytotoxic lymphocytes (CD8+) (129 +/- 49 vs 850 +/- 695/microl, p = 0.057) reduced with reversal of CD4(+)/CD8(+) ratio (0.81 +/- 0.68 1.64 +/- 0.89, p = 0.027). Furthermore, IgG level was significantly reduced (5.12 +/- 2.84 vs 12.5 +/- 1.58 g/L, p = 0.005) and IgA level tended to be lower (1.36 +/- 1.37 vs 2.50 +/- 0.80 g/L, p = 0.095). In contrast, the B lymphocyte (CD19+) count (340 +/- 151 vs 618 +/- 427/microl, p = 0.25), natural killer cell count (CD16(+) 56(+) CD3(-)) (252 +/- 212 vs 276 +/- 251/microl, p = 0.85), and IgM level (0.98 +/- 0.59 vs 1.12 +/- 0.25 g/L, p = 0.67) were similar for both groups. None of the patients developed opportunistic or severe viral infections. Abnormal immunologic profile of both the cellular and humoral arms of the immune system occurs in patients with congenital heart disease complicated by PLE. Nonetheless, these abnormalities perhaps appear quantitative rather than qualitative in nature, although further functional studies of antibody production and lymphocyte proliferation assays are required to support this proposition.

Adolescent↗
PubMedDate not supplied

Congenital heart disease in adolescence.

Adolescents and young adults with congenital heart disease are increasingly commonly seen in cardiological practice. Their problems are particular and often unique. Their management requires training and experience in the management of congenital heart disease by the attending cardiologist. In these days of clinical governance, management of ACHD patients must be supervised by clinicians specifically trained in handling congenital cardiac malformation.

Adolescent↗
PubMed1995

Scoliosis and congenital heart disease.

STUDY DESIGN: This study analyzed clinical characteristics of scoliosis associated with congenital heart disease. Chest roentgenograms were reviewed to determine frequency, type, onset, and progression of scoliosis associated with congenital heart disease. OBJECTIVES: To determine which patients with scoliosis and congenital heart disease should be followed-up carefully. SUMMARY OF BACKGROUND DATA: Although several studies have been reported on scoliosis and congenital heart disease, the etiology of scoliosis in patients with congenital heart disease is still unknown. Furthermore, it has been difficult to determine the progression of scoliosis. METHODS: Chest roentgenograms of 680 patients who underwent cardiac operations because of congenital heart disease were evaluated. RESULTS: Seventy-four (10.9%) of these patients had scoliosis of more than 10 degrees, although most of them had mild curves. Scoliosis in patients under 10 years old undergoing cardiac operations was more severe than in those older than 10 years who underwent surgery. Scoliosis associated with congenital heart disease showed no particular features. Patients with patent ductus arteriosus showed left convex, high thoracic scoliosis after surgery. CONCLUSIONS: The mechanisms of onset of scoliosis in patients with congenital heart disease were not simple and were affected by several factors, including age at operation, side of approach, and type of congenital heart disease.

Adolescent↗
PubMed2003

[Pregnancy and delivery in women with congenital heart disease].

OBJECTIVES: Perinatal care of women with congenital heart disease is very important for obstetrician. DESIGN: The purpose of this study was to analyse the course of pregnancy, delivery and after birth period of women with congenital heart disease. MATERIAL AND METHODS: A group of 35 pregnancies and deliveries in patients with congenital heart disease cured in 1998-2003 in the Department of Obstetrics and of Medical University in Gdansk were analysed. The control group was a total of 1657 deliveries in the Department of Obstetrics in the 1998. For statistical analysis of the results the T-Student test was used with the significance level p = 0.05. RESULTS: Time of pregnancy, birth weight and length were statistically different to the control group. CONCLUSIONS: We found no cardiological complications during pregnancy in patients with congenital heart disease at I and II stage NYHA. Time of pregnancy, birth weight and length were statistically different to the control group.

Adult↗