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PubMed1995

Congenital heart disease in the adult patient: the value of plain film chest radiology.

Congenital heart disease (CHD) is a major clinical problem in children, occurring in 0.8% of newborns (1). In the past, most patients with CHD died in infancy. With improved surgical and postoperative care, as well as more accurate preoperative evaluation, the overall 10-year survival rate is > 90%. As a result, > 500,000 adults in the United States have surgically treated CHD (2). In addition, at least 150,000 adults are thought to have unrecognized, misdiagnosed, or recognized but untreated CHD (3,4). Diagnostic imaging procedures for the evaluation of CHD include plain film radiology, fluoroscopy, angiocardiography, echocardiography, scintigraphy, computed tomography (CT), and magnetic resonance imaging (MRI). Each has unique as well as overlapping abilities to characterize precisely cardiovascular anatomy and pathophysiology. We emphasize those congenital cardiac disorders found predominantly in adults. In addition, conditions that are usually unrecognized in childhood, "slip through the system", and become clinically recognized in adults for the first time, often with an atypical clinical presentation, are discussed (5). The interpreter of the plain film radiograph has a unique opportunity to identify and often characterize the severity of a congenital cardiac disorder that may be unrecognized by the patient's physician. Important clues found on plain film radiographs will suggest either additional studies to pin-point the type of CHD more precisely or that no additional studies are needed because the recognized lesion is incidental and not of clinical significance.

Adult↗
PubMed1985

Evaluation of oral and low dose intravenous prostaglandin E2 in management of ductus dependent congenital heart disease.

Prostaglandin E2 was given orally to 59 infants with ductus dependent congenital heart disease, and intravenous infusions were substituted for varying periods in 27 of them. An additional three neonates received intravenous treatment alone. Mean oral maintenance dose was 27 micrograms/kg per hour and the mean intravenous dose was 0.005 micrograms/kg per minute. Mean duration of treatment was 49 days (range 16 hours to 272 days). Oral treatment was almost always effective and was especially suitable for long term use. Low dose intravenous treatment was readily substituted when indicated. Complications were usually 'minor'. Growth of the infants and of their pulmonary arteries facilitated later surgical management.

Administration, Oral↗
PubMed1986

Congenital heart disease and extracardiac anomalies: associations and indications for fetal echocardiography.

Fetal echocardiography is a well-established technique for the prenatal identification of congenital heart disease. One of the indications for its use is the presence of extracardiac anomalies, as such coexistent defects may have important implications for obstetric and neonatal management. We have reviewed the obstetric and pediatric literature to examine reported associations. If a fetus is suspected to have hydrocephalus, microcephaly, holoprosencephaly, agenesis of the corpus callosum, Meckel-Gruber syndrome, esophageal atresia, duodenal atresia, diaphragmatic hernia, omphalocele, or renal dysplasia, cardiac evaluation should be pursued. Furthermore, echocardiography may be of help in differential diagnosis of some anomalies (for instance, skeletal dysplasias). Maternal diabetes and phenylketonuria, as well as exposure to phenytoin, trimethadione, or isotretinoin, may result in multiple systemic defects, including congenital heart disease.

Abdominal Muscles↗
PubMed2000

[Anesthetic management for a patient with mental retardation and unexamined complex congenital heart disease].

An 18-year old female with mental retardation and unexamined complex congenital heart disease received dental care under general anesthesia. Anesthesia was induced and maintained successfully without any significant hemodynamic changes with inhalation of nitrous oxide, oxygen (FIO2 0.25-0.3) and sevoflurane after a heavy premedication (morphine 10 mg, scopolamine 0.3 mg and midazolam 5 mg i.m.). After induction of anesthesia, cardiac anomaly was diagnosed by transesophageal echocardiography as TGA, VSD, PS, and operation was completed without any problem. Two points are considered important in this case; first, to appropriately estimate preoperative cardiac function and second, to adequately manage anesthesia to avoid any hemodynamic fluctuation.

Abnormalities, Multiple↗
PubMed2006

Iron deficiency in a patient with extreme erythrocytosis due to cyanotic congenital heart disease.

Erythrocytosis is an adaptive response to improve oxygen transport in cyanotic congenital heart disease (CCHD). However, at highly increased hematocrit levels patients may experience hyperviscosity symptoms. Iron deficiency in CCHD patients is often overlooked due to elevated hemoglobin concentrations. A 29-year-old male with CCHD was readmitted to our outpatient clinic. Red blood cells (11.65*10(12)/L), hemoglobin (25.7 g/dL), and hematocrit (80%) were extremely elevated. Measurements of iron supply showed a constellation typical for iron deficiency with low ferritin (13.2 microg/L), and high sTfR (20 mg/L). We present a case of extremely high red blood cell counts with concomitant iron deficiency. For appropriate management and to avoid misinterpretation of the iron status, ferritin and sTfR should always accomplish laboratory examination of CCHD patients.

Adult↗
PubMed1999

Arrhythmias in the athlete with congenital heart disease: guidelines for participation.

Advances in the diagnosis and early management of congenital heart disease in recent decades have led to increasing numbers of individuals being sufficiently well to participate in social as well as truly competitive sports. Physicians are increasingly asked whether such participation is safe, advisable, and efficacious, yet few guidelines exist to help them make these decisions. There are three apparent subgroups of patients: (a) those with mild or repaired problems, who function normally or nearly so and may fully participate; (b) those with severe functional deficit or known high risk, for whom strenuous exertion must be strictly proscribed; and (c) those who fall in between, with some limitations and some risk--these patients present a great challenge to the wisdom and clinical skill of the physician.

Arrhythmias, Cardiac↗
PubMed1999

Interventional cardiac catheterization advances in nonsurgical approaches to congenital heart disease.

Interventional cardiac catheterization offers increased therapeutic options to many patients with congenital heart disease. Over the past year encouraging results have been reported in hundreds of patients with interatrial communications, patent ductus arteriosus, and pulmonary artery stenosis requiring stenting. The Amplatzer (AGA Medical Corporation, Golden Valley, MN, USA) family of atrial septal, ventricular septal, and ductus occluding devices shows particular promise. Other studies support the efficacy and safety of established procedures such as balloon valvuloplasty and coarctation angioplasty. In some areas, such as transcatheter perforation of atretic pulmonary valves and endovascular stenting for coarctation of the aorta, our technical abilities may be advancing faster than our understanding of the ultimate clinical impact of our treatment. As interventional cardiac catheterization procedures continue to develop, they must be evaluated carefully against alternative treatments.

Angioplasty, Balloon↗
PubMed1985

The pattern and frequency of congenital heart disease among blacks.

The types of cardiac malformations in 804 black patients of all ages in whom the diagnosis was confirmed by cardiac catheterization, surgery or autopsy are reported. The most frequent anomalies were ventricular septal defect, patent ductus arteriosus and tetralogy of Fallot. Among infants under 1 year of age, complete transposition of the great vessels accounted for the third-largest group of malformations. While pulmonary venous anomalies were extremely rare and hypoplastic left heart defects rare in the neonate, it is not considered that a racial predilection for differences in the frequency of various cardiac malformations exists. The frequency of coarctation of the aorta in the group as a whole was not lower than that found in studies among whites. Among black patients of all ages with heart disease (studied in a hospital environment) congenital cardiac malformations ranked as the second most common form of heart disease with a frequency of 26%. Among children aged 15 years or less, congenital heart disease ranked first with a frequency of 53%. It is suggested that a diagnosis of congenital heart disease is not made in the majority of blacks born with such malformations.

Adolescent↗
PubMed2005

Gynecological health of females with congenital heart disease.

Because of major advances in diagnostic and surgical methods, females with congenital heart disease (CHD) now survive into and beyond their reproductive years. Management of pregnancy in this patient population is well described, but gynecologic management such as menstruation, contraception and menopause have received scanty attention. Accordingly, the gynecologic health issues confronting these patients are described. Menstrual patterns in acyanotic females with CHD are similar to the general population, but cyanotic females have menstrual irregularities including amenorrhea, which implies anovulation and an increased risk of uterine carcinoma. Anticoagulants predispose to heavy vaginal bleeding and corpus luteum rupture. Contraceptives must be selected according to individual patient profiles. Hormone replacement therapy is warranted for relief of menopausal symptoms as in the general population and should be relatively safe because estrogen dose is low.

Abortion, Induced↗
PubMed1998

Growing up with congenital heart disease: the dilemmas of adolescents and young adults [see comment].

Advances in diagnosis, medical management and surgical intervention have improved the longevity and quality of life for children with congenital heart disease. Despite this, research studies specifically examining the psychosocial concerns of adolescents and young adults with congenital heart disease are few. To explore the subjective experiences and dilemmas of this population during the transition from adolescence to young adulthood, we interviewed, using a semi-structured protocol, a convenience sample of nine adolescents and young adults. Using analytic procedures inherent in Grounded Theory methodology, seven themes were identified: the dilemma of normality; dilemmas in disclosure; dilemmas in strategies for management of illness; the challenge of social integration versus social isolation; the challenge of dependence versus independence; the challenge of uncertainty; and strategies for coping. An understanding of these experiences by health professionals can be beneficial in helping this clinical population as they grow up and face the challenges of an uncertain, yet promising, future.

Adaptation, Psychological↗
PubMed1990

Alpha 1-acid glycoprotein and the binding of lidocaine in children with congenital heart disease.

The purpose of this study was to evaluate the effect of congenital heart disease (CHD) on the serum concentrations of alpha 1-acid glycoprotein (alpha 1-AGP) and the serum binding of lidocaine. Thirteen children with acyanotic CHD and 12 children with cyanotic CHD were studied and compared with 28 children without heart disease (control). The mean (+/- SD) serum concentration of alpha 1-AGP, as determined by radial immunodiffusion, did not differ significantly among the three groups. Five minutes after administration of 1.5 mg.kg-1 intravenous lidocaine, the free fraction of lidocaine correlated inversely and linearly with the serum concentration of alpha 1-AGP in children with acyanotic CHD (r2 = 0.74; P less than 0.001) cyanotic CHD (r2 = 0.57; P less than 0.005), and control (r2 = 0.63; P less than 0.001). The slopes and intercepts of the linear regressions did not differ significantly among the three groups. We conclude that the serum concentration of alpha 1-AGP in children with CHD does not differ quantitatively or qualitatively from that in children without CHD.

Adolescent↗
PubMed1998

[Radionuclide pulmonary perfusion imaging in evaluating pulmonary hypertension in patients with congenital heart disease].

OBJECTIVE: To evaluate the degree of pulmonary hypertension in patients with congenital heart disease (CHD) by using radionuclide pulmonary perfusion imaging. METHOD: The pulmonary perfusion in 81 patients with left to right cardiac shunts was examined by scintigram with macroaggregates of (99m)Tc-labeled human serum albumin (PPS). PPS was analyzed for (1) lung regional radionuclide count (LRC), (2) right upper and lower lung count ratio (RULR), (3) kidney count ratio (KCR). The results were compared with those of cardiac catheterization exam. RESULT: There were correlation between mean pulmonary arterial pressure (mPAP), total pulmonary resistance (TPR), and PPS (KCR, RULR with mPAP gamma = 0.25, 0.35; LRC, KCR with TPR gamma = -0.34, 0.29). Five patients repeated PPS test after early operation. There were no changes in PPS. CONCLUSION: The degree of reversibility of pulmonary hypertension in patients with CHD could be quantitatively analysed by PPS.

Adolescent↗
PubMed1986

Consanguinity and congenital heart disease in the rural Arab population in northern Israel.

The incidence of congenital heart disease (CHD) was examined in relation to the consanguinity of the parents. The study was performed in five Arab villages in the Western Galilee, Israel, where consanguineous matings are known to be very high. All children up to the age of 7 years were included in this study; there were 1,546 children, 32% were the offspring of consanguineous marriages including first- and second-degree cousins. A higher percentage of isolated CHD was found in the offspring of consanguineous marriages: 2.81% out of 498 children compared to 1.24% in 1,048 offspring of unrelated parents. Among 373 children whose parents were first cousins the percentage of CHD rose to 3.22. The differences in CHD frequencies were found to be statistically significant. CHD is believed to have a multifactorial background. This study shows that the genetic influence is an important factor in the etiology of such malformations.

Child↗
PubMed2001

Are infant behavioural feeding difficulties associated with congenital heart disease?

AIM: To compare the feeding patterns and difficulties of infants with congenital heart disease (CHD) and healthy controls. Information was gathered via parental questionnaires. METHODS: A matched case controlled study of 64 infants with CHD compared with 64 healthy controls. RESULTS: The main findings were: (1) Feeding patterns: mothers with infants with CHD used bottle-feeding as a first method of feeding their babies more often (CHD, 20%, controls, 2%); (2) Specific feeding difficulties: (a) infants with CHD were significantly more breathless when feeding (CHD = 16%, controls, 0%), (b) had more vomiting at mealtimes (CHD = 23%, controls = 11%), but (c) had significantly less spitting (CHD = 19%, controls, 41%); and (3) infants with CHD showed significantly reduced growth. CONCLUSIONS: The feeding difficulties are related to the organic condition and not specific difficulties in mother-infant interaction. Professional support may be required for mothers of infants with CHD to maintain feeding routines and to deal with the difficulties that arise.

Breast Feeding↗
PubMed2000

Accuracy and limitations of transabdominal fetal echocardiography at 12-15 weeks of gestation in a population at high risk for congenital heart disease.

OBJECTIVE: Evaluation of transabdominal fetal echocardiography at 12-15 weeks of gestation. DESIGN: Retrospective analysis. SETTING: Tertiary fetal cardiology unit. SAMPLE: Two hundred twenty-nine consecutive fetuses imaged at 12-15 weeks of gestation over a 45-month period. METHODS: Retrospective analysis of echocardiography and autopsy reports. MAIN OUTCOME MEASURES: Accuracy of early echocardiography for the detection of abnormalities of the cardiac connections. RESULTS: Diagnostic images were obtained in 226/229 fetuses (98.7%). Abnormalities of the cardiac connections were detected in 13 fetuses (5.7%) on the initial scan. Where information was available (n = 11), the echocardiographic findings were confirmed at autopsy or postnatally. In two of the 13 cases of congenital heart disease, repeat echocardiography was necessary to provide additional cardiological information. Of the 213 cases in whom a normal initial report was issued, four (1.7%) had congenital heart disease diagnosed later in pregnancy (n = 3) or postnatally (n = 1). Three of these fetuses had haemodynamically insignificant ventricular septal defects and one developed a dilated cardiomyopathy later in gestation. CONCLUSIONS: Transabdominal fetal echocardiography can be performed at 12-15 weeks of gestation permitting accurate early detection of major congenital heart defects in a high risk population. Some forms of congenital heart disease, usually minor, may not be detectable at such an early stage.

Echocardiography↗
PubMed1991

The effects of high-energy feeding on energy balance and growth in infants with congenital heart disease and failure to thrive.

Failure to thrive (FTT) in infants with congenital heart disease (CHD) can be attributed to their low energy intakes and high resting energy expenditures. Energy intake, energy expenditure and growth were studied in infants with CHD on normal formula feeds and then on feeds supplemented with glucose polymer to see whether supplementation improved energy retention and growth. Mean gross energy intakes increased by 31.7% on high-energy feeding and mean weight gain improved from 1.3 g/kg per d on control to 5.8 g/kg per d on high-energy feeding. Resting oxygen consumption (VO2 ml/kg per min) was not significantly different on the two feeding regimens, although respiratory quotient rose on high-energy feeding reflecting the increased carbohydrate intake. Estimated energy costs of growth on high-energy feeding fell within the previously described range for normal infants. It is recommended that infants with CHD known to be associated with FTT are fed on high-energy diets from the time of diagnosis in order to optimize growth.

Energy Metabolism↗
PubMed2001

[Clinical characteristics of congenital heart disease diagnosed during neonatal period].

We have analyzed 43 newborn babies with congenital heart disease (CHD) over the last two years. The occurrence rate of CHD was 7.5 per 1,000 live births in our maternity hospital, and 4.5% of all babies admitted to our NICU. Low birth weight (<2,500g), and prematurity (<37 weeks of gestation) led to occurrence rates of 34% and 26%, respectively. Cardiac symptoms (heart murmur and cyanosis) accounted for 47% of all cases in which CHD was discovered, and extracardiac symptoms accounted for 40%. Prenatal diagnosis was made in two patients. Twenty babies were transferred to the pediatric cardiac care unit in the general hospital from our maternity hospital during their neonatal period. Dividing them into two categories by hemodynamic state, acyanotic type made up 72% of all patients; VSDs (Ventricular Septal Defects) were the most frequent anomaly. Cyanotic type made up only 28%, but included all 4 cases of neonatal death. Twelve babies underwent surgery during the study period, and 7 of them reached final anatomical correction. Early detection of the cardiac anomaly, including prenatal diagnosis, plays an important role in improving the prognosis of patients, and comprehensive therapeutic strategies are essential for neonatal CHD.

Cardiac Surgical Procedures↗
PubMed2002

Circadian and seasonal variation of malignant arrhythmias in a pediatric and congenital heart disease population.

INTRODUCTION: Recent studies in adult populations have revealed seasonal variation in the frequency of acute cardiovascular events, including life-threatening arrhythmias, demonstrating increased events during winter and early spring. Trends in the time of day that arrhythmias occur also were noted. We sought to establish whether pediatric and young adult congenital heart disease implantable cardioverter defibrillator (ICD) recipients have circadian or seasonal variability in shock frequency, similar to adult populations. METHODS AND RESULTS: Data from ICD patients at six pediatric centers in North America were analyzed to assess the timing of life-threatening arrhythmias. The populations consisted of children and adults with congenital heart disease and ICDs placed for malignant arrhythmias. Data were considered in 46 patients who received appropriate therapy (total 139 episodes) for ventricular tachycardia or ventricular fibrillation. Multiple variables were analyzed, including time of day, day of week, and month of year. In contrast to previously studied adult patients, fewer events occurred in the early morning (7.5%), with the most therapies occurring between 6 P.M. and midnight (35%). An increased frequency of therapies was observed in the fall and winter (September-January), representing 60% of all appropriate shocks. Unlike adult populations, Mondays did not have an increased frequency of malignant arrhythmias. CONCLUSION: Pediatric and adult congenital heart disease populations have moderate seasonal and 24-hour variation in ICD event rate, with some distinctly different peaks than those seen in typical adult ICD populations. These findings suggest circadian variation in arrhythmia vulnerability that may differ from conventional occupational, physical, or emotional stressors. (J Cardiovasc Electrophysiol, Vol. 13, pp.

Adolescent↗