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[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↗

Transmural atrial pacing in patients with postoperative congenital heart disease.

INTRODUCTION: Some patients with postoperative congenital heart disease require permanent cardiac pacing, but the use of transvenous or epicardial pacing leads may be limited by type of cardiac malformation, venous connections, body size, or fibrosis. Transmural atrial pacing may provide an alternative in difficult patients, but to date has been described in only a few articles with small patient numbers, and data from lead performance are lacking. METHODS AND RESULTS: Records were reviewed in 18 consecutive patients (4 months to 21 years old) with postoperative congenital heart disease receiving transmural atrial pacing leads from July 1994 to December 1996. Implantation materials and techniques were described. Lead sensing and capture thresholds obtained acutely and during short-term follow-up (mean: 11.0 months) were evaluated, and comparisons were made between patients with postoperative Fontan anatomy and non-Fontan patients, and between patients receiving steroid-eluting and nonsteroid leads. Overall, the median acute sensing and capture thresholds of transmural leads were 4.1 m V and 0.7 V at 0.5 msec, respectively. Median follow-up thresholds were 2.8 m V and 0.8 V, respectively. Performance of leads in Fontan patients was similar to those in non-Fontan patients. Steroid-eluting leads had a chronic capture threshold of 0.6 V versus 0.9 V for nonsteroid leads (P = 0.038). CONCLUSION: Transmural atrial pacing leads were successfully implanted in patients with diverse ages and types of postoperative congenital heart disease. Lead performance was acceptable both acutely and during the first year of follow-up.

Adolescent↗

The adolescent with simple or corrected congenital heart disease.

An ever-increasing number of congenital heart disease patients are surviving into and beyond adolescence. Despite the encouraging cardiac results, with the vast majority asymptomatic and requiring no medication and having few, if any, limitations, there are many challenges for this patient population. They are likely to find obstacles to attaining education, social development, health and life insurance, and employment. Most of these obstacles are attributable to the stresses upon children with congenital heart disease and their families, and the prejudices of a society that knows little about congenital heart disease. Practitioners caring for these young people must be familiar with these issues and knowledgeable about the facts of individual cases, so that they may anticipate needs and offer support to their patients in their progress toward adulthood.

Adolescent↗

[A post-mortem analysis of 712 cases of congenital heart disease (author's transl)].

Congenital cardiovascular malformations were found in 712 cases (32.1%) out of 2159 autopsies, performed on children less than 16 years old, at the Department of Pathology of the Institute of Pediatrics in Warsaw since 1959. A detailed retrospective analysis of all the cases was performed. The author's own classification of congenital heart diseases was used in this analysis. The incidence of particular heart malformations or syndromes was presented. The most frequent ones were: transposition of the great arteries, tetralogy of Fallot and ventricular septal defects, in a decreasing frequency. An analysis of the age distribution of various cardiac malformations and a short analysis of the causes of death was made. The incidence of congenital heart diseases accompanied by other congenital malformations was analysed.

Adolescent↗

Congenital heart disease: a 10 year cohort.

In order to describe the epidemiology of congenital heart disease in Western Australia, a retrospective, population-based cohort study was conducted using data collected from multiple sources of ascertainment by the Western Australian Birth Defects Registry. The prevalence of congenital heart disease was 7.65 per 1000 total births. Nine per cent of all cases had a chromosomal disorder, another 17% had extracardiac defects in addition to congenital heart disease, and 75% had isolated congenital heart disease. Congenital heart disease was more common in Aboriginal compared with non-Aboriginal infants, and in multiple births compared with singletons. Compared with infants weighing 3000 to 3499 g, infants of lower birthweight were more likely to have congenital heart disease, and infants in the heaviest category (> or = 3500 g) were less likely to have congenital heart disease. Three per cent of all stillbirths were known to have a cardiac defect, as were 15.2% of neonatal deaths and 10.8% of post-neonatal deaths.

Birth Weight↗

Report of the National Heart, Lung, and Blood Institute Working Group on research in adult congenital heart disease.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease.

Adult↗

Intracranial aneurysms and cervicocephalic arterial dissections associated with congenital heart disease.

OBJECTIVE: The number of patients with congenital heart disease who survive to adolescence and adulthood continues to increase. We review our experience with noninfectious intracranial aneurysms and cervicocephalic arterial dissections in patients with congenital heart disease, expanding the clinical spectrum of the cerebrovascular abnormalities that may be encountered in this group of patients. METHODS: All patients with congenital heart disease and intracranial aneurysms of cervicocephalic arterial dissections, who were evaluated at the Mayo Clinic between 1969 and 1992, were identified. RESULTS: Congenital heart disease was diagnosed in 3 (8%) of 36 children with intracranial aneurysms, in 5 (0.3%) of 1994 adults with intracranial aneurysms, in 1 (4%) of 25 children with cervicocephalic arterial dissections, and in 5 (2%) of 250 adults with cervicocephalic arterial dissections. The mean age of the 14 patients was 32 years (range, 13-58 yr). The congenital heart disorders consisted of complex cardiac anomalies in three patients (truncus arteriosus, transposition of the great arteries, and tricuspid atresia in one patient each), pulmonic valve or arterial stenosis in two patients, aortic coarctation in four patients, and bicuspid aortic valve in five patients. Only one patient had an intracranial aneurysm and coarctation of the aorta. CONCLUSION: Individuals with a variety of congenital heart disorders may be at an increased risk of intracranial aneurysm development and cervicocephalic arterial dissection, particularly in adolescence. The muscular arteries of the head and neck are derived from neural crest cells and the neural crest is also of major importance in early cardiac development, suggesting that an abnormality of the neural crest may be the common pathogenetic factor explaining this association.

Adolescent↗

Effect of detailed fetal echocardiography as part of routine prenatal ultrasonographic screening on detection of congenital heart disease.

BACKGROUND: Cardiac abnormalities are frequently not detected by routine ultrasound screening examinations. Although detailed fetal echocardiography is more sensitive in detection of congenital heart disease, it is used only for high-risk cases. The main aim of this study was to assess the prenatal detection of congenital heart disease by detailed fetal echocardiography in an unselected, consecutive group of pregnant women. METHODS: Between Jan 1, 1993, and Sept 30, 1994, all women who attended our antenatal-care unit were routinely offered a detailed fetal echocardiography examination at 18-28 weeks' gestation. 3085 consecutive women were screened: 2181 were screening cases with no known risk factor for congenital heart disease; 540 had maternal risk factors for congenital heart disease, such as a family history or coexisting maternal disease; 364 had sonographically detected abnormalities. The examination included the four-chamber view, outflow-tract scan, and colour-flow mapping; doppler and M-mode investigations were also done when appropriate. FINDINGS: 46 cases of congenital heart disease were detected prenatally by echocardiography-15 in the group with no risk factors, three in the group with maternal risk factors, and 28 in the group with sonographic abnormalities. Postnatal assessments found six further cases of congenital heart disease that had not been detected prenatally, but these were all minor cases. There were no false-positive diagnoses (sensitivity 85.5%, specificity 100%). The incidence of congenital heart disease in screening cases with no risk factors and in those with maternal risk factors was low (6.9% per 1000, 5.6 per 1000) and similar to the expected overall incidence of 8.0 per 1000 livebirths in the general population. In the group with sonographic abnormalities congenital heart disease was found significantly more often (79.9 per 1000). INTERPRETATION: Inclusion of detailed fetal echocardiography as a screening examination has a substantial effect on detection of congenital heart disease since a major proportion of prenatally detectable cases occur in a low-risk population.

Adult↗

Congenital heart disease diagnosed in Guadeloupe.

An epidemiologic survey of congenital heart disease was performed in Guadeloupe, French West Indies, during the 3-year period 1988-1990. This survey, the first on congenital heart disease from the Caribbean, found an overall rate of 6.08 congenital heart disease cases per 1,000 live births, a high rate of hypoplastic left heart disease, and a rate of coarctation of the aorta similar to rates reported in developed countries.

Female↗

Chromosome 22q11 microdeletion and congenital heart disease--a survey in a paediatric population.

UNLABELLED: Congenital heart disease is a common finding in patients with microdeletion of chromosome 22q11. To determine if the deletion is an epidemiologically important cause of congenital heart disease, we studied a consecutive series of children attending a paediatric cardiac clinic and of neonates diagnosed as having structural congenital heart disease. Venous blood samples were tested by fluorescent in-situ hybridisation analysis for microdeletion of chromosome 22q11 using probe D22S75. Each patient was examined for the other clinical features associated with microdeletion of chromosome 22q11, and any family history of congenital heart disease recorded. Of 151 families approached, 111 participated and a fluorescent in-situ hybridisation result achieved in 87. One patient with microdeletion of chromosome 22q11 was identified; the clinical features were those of DiGeorge syndrome. Two patients with CHARGE association, two with nasal speech, ten with high arched palate, and 15 with minor facial dysmorphic features had no deletion. CONCLUSION: Microdeletion of chromosome 22q11 detected by probe D22S75 is rare in this consecutive series of patients with structural congenital heart disease.

Child↗

Incidence of congenital heart disease in Qatari children.

We surveyed the incidence of congenital heart disease in 49887 native live born children in the period between 1984 to 1994 in Qatar. Each child with clinically suspected congenital heart disease underwent echocardiographic examination. Magnetic resonance imaging, cardiac catheterization and surgical intervention were done at the discretion of the patient's pediatric cardiologist. Virtually no postmortem examinations were performed. Children with congenital heart disease were entered into a computerized database and were then followed for 1-11 years. Congenital heart disease was diagnosed in 610 of 49,887 children for an incidence of 12.23/1000 live births. The reasons for the high incidence were high proportion of small muscular ventricular septal defects discovered before the time of their spontaneous closure, referral to and follow up by a single group of pediatric cardiologists, location of the pediatric cardiology service in the same setting where nearly all of the deliveries took place, freely available health care service, and echocardiographic examination of every child with a clinical diagnosis of congenital heart disease.

Female↗

Risk of pulmonary tuberculosis in children with congenital heart disease.

Children with low-flow congenital heart lesions are reported to have an increased incidence of pulmonary tuberculosis. The aim of this study was to investigate if children with congenital heart disease have an increased incidence of pulmonary tuberculosis and to determine if patients with certain heart conditions are more susceptible to pulmonary tuberculosis than others. This retrospective study over a 6-year period showed that pulmonary tuberculosis was 2.5-fold more common in children with congenital heart disease than in normal children from the same community. Children with congenital pulmonary stenosis had a prevalence equal to those with acyanotic (ventricular and atrial septal defects) and cyanotic (transposition of the great arteries) high-flow heart lesions, whereas there were no cases of tuberculosis in children with low-flow cyanotic heart lesions such as tetralogy of Fallot. Cardiac surgery had to be postponed as a result of pulmonary tuberculosis in 7.2% of all patients in whom it was required. Over the 6-year period of the study, cardiac surgery had to be delayed in 60% of cases with pulmonary tuberculosis and congenital heart lesions so antituberculosis therapy could be completed. Physicians treating children with congenital heart lesions should maintain a high index of suspicion for the development of pulmonary tuberculosis, especially in those with acyanotic and cyanotic high-flow lesions and pulmonary stenosis.

Adolescent↗

The total correction of congenital heart disease in infants.

If radical correction of congenital heart disease can be performed with a low risk in the first year of life the advantages are obvious. Total correction avoids the latent risks of the underlying lesion and the risks of palliative surgery, and relieves the parents and family of the psychological pressure of a major illness. Palliative surgery, although offering survival, may include the long term problems of the palliative operation itself, and the possibility that cases may be lost to further treatment or follow-up examination after a successful palliative operation. For these reasons, to be desirable, a palliative operation should offer a survival chance that is at least 10% better than the corrective procedure in that patient at that time. That is, any corrective procedure with a mortality rate less than 10% is to be perferred at any age to a palliative operation. An accurate assessment of risk demands a complete diagnostic study, and a knowledge of the natural history of that form of congenital heart disease. The problems of infants with congenital heart disease are not primarily caused by age of size. The main problem is that of natural selection.

Acute Kidney Injury↗

Evaluation of canine congenital heart disease using an echocardiographic algorithm.

Evaluation of canine congenital heart disease presents a diagnostic challenge to many ultrasonographers. To assist clinicians attempting to examine these patients, an echocardiographic algorithm containing the six most common canine congenital heart diseases (i.e., patent ductus arteriosus, subaortic stenosis, pulmonic stenosis, ventricular septal defect, tricuspid dysplasia, and tetralogy of Fallot) is presented. The algorithm focuses on the underlying disease pathogenesis and the resultant changes in cardiac structure and function that can be readily identified during echocardiographic examination. Use of this algorithm provides a framework from which the ultrasonographer gains both experience and confidence in diagnosing congenital heart disease via echocardiography. This algorithm is supported by a number of still figures within the article as well as real-time echocardiographic images available for viewing at AAHA's website, www.aahanet.org.

Algorithms↗

Nutritional treatment of congenital heart disease.

Twelve of 13 patients with congenital heart disease given continuous enteral nutrition displayed normal growth; cardiac function remained stable or improved in 10 in spite of the water load (146 +/- 22 ml/kg/day). This is safe treatment for malnutrition in congenital heart disease.

Body Height↗

Evaluation of syncope in adult congenital heart disease.

Approximately one million adults in the United States have congenital heart disease. The practitioner who sees one of these patients presenting with syncope must have a thorough understanding of: the underlying disease process; the associated congenital heart abnormalities; the type of surgery performed; the potential surgical residua; and the natural and unnatural history of this type of heart disease. Several types of congenital heart disease including: atrial septal defects; ventricular septal defects; left-sided obstructive lesions; Ebstein Anomaly; tetralogy of Fallot; and transposition of the great arteries are reviewed.

Journal Article↗

[Intestinal malformations and congenital heart diseases].

A series of 21 patients with both congenital heart disease and intestinal malformation seen over a 12-year period is reported. The intestinal malformations were: anorectal malformations (11 cases), duodenal atresia (5), omphalocele (4) and common mesentery (1). Congenital heart diseases consisted of: atrial septal defect (ASD) (10 cases), ventricular septal defect (VSD) (2), tetralogy of Fallot (2) and miscellaneous cardiopathies. In patients with anorectal malformations ASD and VSD predominated (6/11 cases) and multiple malformations syndromes were present in 8 cases, including trisomy 13, Vater syndrome, skeletal (4), neurological (3) and renal abnormalities (3); 3 children died. Duodenal atresia was always associated with left-to-right shunt: VSD (3), ductus arteriosus (2), complete atrioventricular canal (1) and trisomy 21 (2); one child died. Omphalocele coexisted with VSD (2), tetralogy of Fallot (1), dual outlet right ventricle (1), trisomy 21 (1) and multiple malformations syndromes (3); 2 children died. The patient with common mesentery had left-to-right shunt. Comparison of this series with data from the literature showed that children with congenital heart disease have a much higher incidence of intestinal malformations than those with normal heart and that they frequently present with multiple malformations (chromosome aberrations or multiple organ lesions). This multiple malformation complex is particularly common in anorectal malformations where the incidence of congenital heart diseases is 9 to 14%, with predominance of VSD and tetralogy of Fallot. In patients with duodenal atresia trisomy 21 is extremely frequent, and the incidence of cardiopathies is 18%; in the absence of trisomy 21 the cardiopathy is complex.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗