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PubMed1981

Extrahepatic biliary atresia associated with cyanotic congenital heart disease. Three case reports and a review.

Three patients with extrahepatic biliary atresia associated with cyanotic congenital heart disease are presented and their clinical courses reviewed. In these patients, the coincidence of cyanosis and jaundice caused an atypical green-appearing cutaneous discoloration which masked the early clinical detection of biliary atresia in one infant and cyanotic heart disease in the other two. In all infants, either partial or complete correction of the congenital cardiac lesion was possible. Two infants died from hepatic disease despite attempted surgical revision and one has satisfactory bile drainage one month following portoenterostomy. Awareness of the rare concurrence of cyanotic congenital heart disease and extrahepatic biliary atresia is helpful to the clinician when evaluating infants with atypical cutaneous discoloration. Early oversight of either underlying disease process can be avoided, and early evaluation and management of both entities can be possible.

Bile Ducts↗
PubMed1993

Abnormalities in skeletal muscle metabolism in cyanotic patients with congenital heart disease: a 31P nuclear magnetic resonance spectroscopy study.

1. Exercise tolerance is impaired in congenital heart disease. To examine the possible contribution of abnormalities in skeletal muscle bioenergetics, we used 31P nuclear magnetic resonance spectroscopy to investigate muscle metabolism in 10 subjects with congenital heart disease with cyanosis (median age 17.3 years) and in eight healthy age-matched control subjects. Spectra were collected from the gastrocnemius muscle at rest and during exercise and recovery. 2. In resting muscle there were significant elevations in cytosolic pH and in the cytosolic concentration of inorganic phosphate in the patients, and a strong positive correlation between cytosolic pH and blood haemoglobin concentration in all subjects. 3. During plantar flexion exercise the patients showed increased phosphocreatine depletion and cytosolic acidification over a shorter duration of exercise. The rise in calculated cytosolic ADP concentration was similar in both groups. 4. After cessation of exercise, the recovery half-times of phosphocreatine, ADP and phosphate were two to three times longer in the patients, and the initial rate of phosphocreatine resynthesis (a measure of the rate of mitochondrial ATP synthesis) was half the control value, consistent with a reduction in the effective maximum rate of oxidative ATP synthesis (expressed per volume of muscle). Also, recovery was faster in the young control subjects than in our earlier studies of older healthy control subjects. 5. The high phosphate concentration in resting muscle and the abnormalities found in exercise and recovery are consistent with a decrease in oxidative ATP synthesis due to reduced oxygen delivery by the blood in chronic hypoxaemia. The correlation between cytosolic pH and haemoglobin concentration remains to be explained.

Adolescent↗
PubMed1983

Cardiovascular effects of breathing 95 percent oxygen in children with congenital heart disease.

The hemodynamic effects of breathing 95% oxygen were evaluated in 26 children with congenital heart disease. Aortic, pulmonary arterial, right atrial, and pulmonary arterial wedge pressure, aortic and pulmonary artery oxygen saturation, and blood gas, cardiac index, and heart rate were measured in room air and after each patient had breathed 95% oxygen for 10 (n = 26) and 20 (n = 5) minutes. Measurements were repeated with the patient again breathing room air for 10 (n = 11) and 20 (n = 6) minutes. After 10 minutes of 95% oxygen, arterial partial pressure of oxygen increased from 85 +/- 13 to 420 +/- 89 torr (p less than 0.001). Aortic mean pressure increased from 80 +/- 10 to 83 +/- 10 mm Hg (p less than 0.01), and systemic vascular resistance increased from 20 +/- 7 to 26 +/- 8 U (p less than 0.001). The cardiac index decreased by 21% from 3.96 +/- 0.94 to 3.12 +/- 0.74 liters/min/m2 (p less than 0.001) and the stroke index decreased by 11% (p less than 0.001). A 23% decrease in oxygen consumption (p less than 0.001) was observed, and oxygen transport decreased from 763 +/- 179 to 600 +/- 161 ml O2/min/m2 (p less than 0.001). Cardiac index, stroke index, and systemic vascular resistance did not return to normal until 20 minutes after cessation of oxygen breathing. To determine whether reflex bradycardia is responsible for these oxygen-induced hemodynamic changes, heart rate was kept constant by atrial pacing in a second group of 5 patients. In these children, significant decreases in cardiac index, stroke index, and oxygen consumption, and increases in systemic vascular resistance also occurred with 95% oxygen. Thus, in children with acyanotic congenital heart disease, hyperoxia increases aortic pressure and systemic vascular resistance and decreases cardiac index, stroke index, oxygen consumption, and oxygen transport.

Adolescent↗
PubMed1999

Failure to diagnose congenital heart disease in infancy.

OBJECTIVE: To identify factors that predict failure to diagnose congenital heart disease in newborns. DESIGN: All fatal cases in the Baltimore-Washington Infant Study were compiled. The Baltimore-Washington Infant Study includes 4390 cases of infants with congenital cardiovascular malformations identified in a population-based study between 1981 and 1989 in the Baltimore-Washington metropolitan area. Death occurred in 800 such infants in the first year of life. In 76 of these infants, death occurred before diagnosis of heart disease. These cases were identified by community search of autopsy records. Their characteristics are compared with those of infants who died after a cardiac diagnosis was made. RESULTS: Infant characteristics (birth weight, gestational age, intrauterine growth retardation, and chromosomal anomaly) are associated with death of infants with congenital cardiovascular malformations and with death of such infants before diagnosis. Diagnoses of coarctation of the aorta, Ebstein's anomaly, atrial septal defect, and truncus arteriosus are overrepresented in infants found by community search, particularly in those infants without associated malformations. Paternal education is associated with failure to diagnose congenital heart disease in life but other sociodemographic characteristics of the infant's family are not. CONCLUSIONS: Diagnosis of congenital cardiovascular malformations requires close observation in the neonatal period. Analysis of age at death of infants with undiagnosed congenital cardiovascular malformation suggests that such infants may be at risk if discharged within the first 2 days of life.

Age Distribution↗
PubMed1983

Morphological and physiological changes of the eye in patients with congenital heart disease undergoing extracorporeal circulation.

We studied 76 patients with congenital heart disease, whose ages ranged from 2 to 39 years. The following investigations were carried out pre-operatively and post-operatively in all patients: visual acuity, cycloplegic refraction, orthoptic examination, examination of the fundus. Where collaboration was sufficient the following were also studied: kinetic and static perimetry, adaptation perimetry, colour vision, electroretinography. There were no differences before and after operation of visual acuity, adaptation perimetry, refraction, electroretinography or of the fundus. Post-operative changes were only found in 4 of the 65 patients in whom perimetry was performed. By comparison, an identical study of patients with acquired heart disease showed the percentage of functional ocular changes to be higher in this group.

Adolescent↗
PubMed1999

[Transesophageal electroconversion of atrial reentrant tachycardias early or late following surgery for congenital heart disease].

Atrial reentrant tachycardias (ART) are a potentially life-threatening complication in survivors of congenital heart disease surgery. From July 1993 to December 1997, temporary transesophageal pacing was used to convert 29 tachycardia episodes in 19 patients. At the time of the first tachycardia episode, patients' ages were 1 month to 26 years (mean 9.8 yrs). Time from operation to onset of first tachycardia episode ranged from 1 day to 19 years. Onset was within the first 2 weeks postoperatively in 6 patients and occurred later in 13 patients (1 to 19 years after operation). Postoperative pacemaker implantation had been performed in 2 pts; 17 of 19 pts were receiving antiarrhythmic medication. After placing a quadripolar transesophageal catheter, atrial and ventricular signals were recorded and atrial stimulation performed. Atrial cycle length of tachycardia ranged from 160-380 ms with 1:1 to 4:1 AV conduction. Temporary transesophageal pacing was performed following an algorithm starting with 4 extrastimuli (20 ms below atrial cycle length of tachycardia). Tachycardia terminated in 27 of 29 cases (93%) without complications. In 3 cases, conversion was achieved by pacing after amiodarone 5 mg/kg i.v. After tachycardia conversion, sinus- or pacemaker rhythm was present in 20 cases. In 9 cases atrial fibrillation was recorded; spontaneous conversion to sinus rhythm occurred after a maximum of 3 min (7 cases) or persisted and required direct current cardioversion (2 cases). In conclusion, transesophageal atrial pacing is an effective, relatively noninvasive method for conversion of atrial reentrant tachycardias after operation for congenital heart disease.

Adult↗
PubMed2000

Surgical intervention for complications of transcatheter dilation procedures in congenital heart disease.

BACKGROUND: Transcatheter interventions have assumed an important role in the management of many forms of congenital heart disease. While complications of transcatheter interventions are uncommon and usually minor, significant complications requiring operation do occur on occasion. The purpose of this report is to present our experiences with seven such complications, and to review the literature on this topic. METHODS: Seven patients who required operation after a transcatheter dilation procedure between 1992 and 1998 are described. Three patients required retrieval of retained foreign bodies (stents or balloons), and repair of the underlying abnormality. Two patients underwent repair of fistulas between 2 great vessels, or a great vessel and a cardiac chamber. One patient required operation for a postdilation aneurysm. One patient underwent urgent repair of severe aortic regurgitation after balloon aortic valvuloplasty. RESULTS: All patients survived and are doing well, with no further need for catheter or operative intervention, from 8 months to 6 years after operation. Additional reported complications requiring operation are discussed as well. CONCLUSIONS: Operation for complications of catheter interventions in congenital heart disease is seldom necessary. Though uncommon, a variety of such complications may occur, including vascular, valvar, intracardiac, and foreign body complications. When operation is required, results are typically very good.

Cardiac Catheterization↗
PubMedDate not supplied

Surgery for congenital heart disease in low-birth weight neonates: a comprehensive statewide Connecticut program to improve outcomes.

Low-birth weight (LBW) remains a significant risk factor in surgery for congenital heart disease (CHD). The timing of surgery and the choice of complete repair vs palliative measures are controversial issues. Delay of surgery to achieve weight gain may result in poorer outcomes. The results of a statewide, cardiac surgery protocol in LBW infants using specific selection criteria for repair vs palliation or delay over a 42-month period are reviewed. From September 1999 through February 2003, 32 low-birth weight infants (1,320 to 2,500 grams) underwent surgery for congenital heart disease in Connecticut. The congenital heart surgery programs in the two major pediatric centers in the state (Yale-New Haven Hospital and Connecticut Children's Medical Center) were amalgamated to form a joint program, with the same surgical team involved in all cases. Median gestational age was 34 weeks with 18 (60%) premature (< or = 37 wks). Median age at operation was eight days. Fourteen infants (44%) had recognized syndromes. Primary diagnoses included variant of single ventricle (8), ventricular septal defect (VSD) (4), tetralogy of Fallot (TOF) (2), pulmonary atresia (PA) with VSD (3), simple transposition of the great arteries (TGA) (1), TGA with VSD (TGA/VSD) (2), atrioventricular canal (AVC) (4), double outlet right ventricle (DORV) (3), aortic coarctation (CoA) (2), and interrupted aortic arch with VSD (IAA/VSD) (3). Eighteen patients (56%) were able to undergo procedures resulting in normal biventricular physiology. Eight patients (25%) had palliation for single ventricle, including two Norwood procedures. Seven patients potentially suitable for biventricular repair underwent palliative surgery because of contraindications to complete repair that were unrelated to weight. Follow-up (100%) ranged from two to 41 months. There were two early deaths (6%), one cardiac related (Norwood with ischemia), and one with sepsis. There was one early reintervention (PA stenosis following arterial switch). There were seven late deaths (22%), two cardiac related (one sudden death following Blalock-Taussig (BT) shunt, one sepsis related to low output), and four noncardiac. In three patients who received pulmonary artery banding (PAB), delay of surgery may have contributed to death due to progressive pulmonary disease. Of 23 long-term survivors, five have undergone biventricular repair following palliation, and two have had further palliation for univentricular heart physiology. A strategy of early surgical intervention favoring primary repair, or surgical palliation for those patients with single ventricle, results in good overall survival in symptomatic low-birth weight neonates with congenital heart disease. Delay in surgery due to LBW may not be beneficial in most cases and could result in lower overall survival and increased cost. A program utilizing statewide resources results in excellent outcomes for this challenging group of patients.

Connecticut↗
PubMedDate not supplied

Natural history of scoliosis in congenital heart disease.

We defined the natural history of scoliosis in congenital heart disease (CHD) with respect to the risk of progression and indications for treatment. We reviewed radiographs of 48 children with CHD and scoliosis. Thirty-seven had developmental scoliosis (77%), and 11 (23%) had congenital scoliosis. Children with congenital scoliosis can be managed in the same way as children without CHD. Developmental curves less than 30 degrees in children aged greater than 10 years showed no progression. Developmental curves greater than 30 degrees with onset in children aged less than 10 years and progression greater than 9 degrees per year require treatment.

Adolescent↗
PubMed1987

Prostaglandin E2 administration in infants with ductus-dependent cyanotic congenital heart disease.

Prostaglandin E2 was administered to 22 newborns with ductus-dependent cyanotic congenital heart disease. Twelve patients had pulmonary atresia and ten simple dextrotransposition of the great arteries. Patients were classified into two groups: group 1 (n = 11) received prostaglandin E2 by the intravenous route (dose: 0.01-0.05 microgram/kg per min); group 2 (n = 11) received prostaglandin E2 by the oral route (dose: 35-65 micrograms/kg per 1-4 h). Treatment lasted for 1-90 days. All infants except one of group 2 showed a significant (greater than 10 Torr) increase in PaO2 following PGE2 administration. The mean increase in PaO2 was higher (P less than 0.01) in group 1 (21.8 +/- 1.7, Torr) than in group 2 (15.8 +/- 1.5, Torr). PaO2 fell significantly (P less than 0.01) in five patients of group 1 who continued treatment orally with satisfactory (greater than 30 Torr) levels in four of them. Severe side effects were observed only in group 1. The data show that similarly to prostaglandin E1 infusions, prostaglandin E2, given i.v. or orally, is useful in the management of infants with ductus-dependent cyanotic congenital heart disease. Oral prostaglandin E2, administration is less effective than i.v. infusions, but can be used for long-term, therapy being more convenient and causing minimal morbidity.

Administration, Oral↗
PubMed1999

The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis. Working Party of the Italian Society of Pediatric Cardiology.

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341,647 surveyed livebirths. The new cases were registered using the same methodologic criteria of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8% and 8.1%; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler techniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.

Echocardiography, Doppler↗
PubMed1994

[Restriction fragment length polymorphism analysis of ventricular myosin light chain 1 gene in families with congenital heart disease].

Genomic DNAs isolated from 9 families and 13 unrelated individual patients with 4 different types of congenital heart disease were examined by Southern blot analysis using a cDNA probe which codes for the full length human ventricular myosin light chain 1. Restriction fragment length polymorphisms were detected in two families and were not associated with any type of congenital heart disease.

Blotting, Southern↗
PubMed2004

Prenatal diagnosis of congenital heart disease: clinical experience and analysis.

Over a five-year period, we reviewed 19 fetuses who were prenatally diagnosed with congenital heart disease, including hemodynamically significant arrhythmias. Five of them had fetal tachyarrhythmias, and 14 had structural heart disease. The outcomes were: six intrauterine deaths, five neonatal deaths, and three infant surgeries. Six of the fetuses had chromosomal abnormalities, four had extracardiac anomalies, and two had hydrops fetalis. Of the 96 neonates with congenital heart disease found during the study period, the overall detection rate was 20%; 16% of the neonates with structural cardiac defects and 83% of the neonates with arrhythmias. Some of the complex cardiac defects with normal fetal four-chamber view were difficult to detect prenatally. During the course of the pregnancy, 37% of the fetuses with prenatally diagnosed congenital heart disease were found to have intrauterine growth retardation, and 26% were found to have an abnormal amniotic fluid volume. In view of our findings, a comprehensive screening system should be more frequently considered in order to improve both detection rate and perinatal management.

Female↗
PubMedDate not supplied

The dilemmas of parents of adolescents and young adults with congenital heart disease.

OBJECTIVE: To provide a better understanding of parents' experiences as their children with congenital heart disease mature through adolescence and young adulthood. DESIGN: A qualitative pilot study. SETTING: The physician practices of the pediatric cardiology service of a large university medical center. SUBJECTS: Eight parents of adolescents and young adults with congenital heart disease. INTERVENTION: Each parent was separately interviewed with use of a semistructured interview guide. RESULTS: Our study has identified seven themes--the dilemmas of normality, disclosure dilemmas, the challenge of uncertainty, illness management dilemmas and strategies, social integration versus social isolation, the impact of illness on the family, and coping--with which parents have struggled through-out their adolescent's and young adult's life. It was not possible to determine whether the experiences described by these parents are unique. CONCLUSIONS: Parents experience distress, as outlined in the seven themes. They need assistance to determine what is "normal" for their child and how to monitor their child's health and safety. Further research is needed to develop specific interventions.

Adaptation, Psychological↗
PubMed2004

Resolution of calcific brown fat necrosis associated with prostaglandin therapy for cyanotic congenital heart disease in neonates: report of two cases.

Prostaglandin E1 (PGE1) administration for palliation of cyanotic congenital heart disease in neonates has been associated with radiographically visible necrosis of brown fat about the neck and shoulder girdles. However, the natural history of this process has not been described. We present two patients with cyanotic congenital heart disease, treated preoperatively with prostaglandin E2 (PGE2), both of whom developed dramatic calcific brown fat necrosis. This process slowly resolved over time in both patients.

Adipose Tissue, Brown↗
PubMed1987

Fetal echocardiographic screening for congenital heart disease: the importance of the four-chamber view.

The four-chamber view of the heart has been proposed as a screening method for obstetrical sonographers to assess the fetus for the presence of congenital heart disease. We examined the fetuses in 1022 pregnancies and found 74 structurally abnormal hearts. Seventy-one of these (96%) were found to have an abnormality as seen in the four-chamber view. The four-chamber view of the heart had 92% sensitivity and 99.7% specificity in the detection of congenital heart disease. The positive predictive value was 95.8%, and the negative predictive value was 99.4%. We conclude that the four-chamber view may be a useful screen for the presence of congenital heart disease and should be included as a part of all routine obstetric ultrasound examinations.

Echocardiography↗
PubMed1992

Congenital heart disease in the offspring and maternal habits and home exposures during pregnancy.

To test the effect of maternal habits and home exposures during early pregnancy on the occurrence of congenital heart disease in the offspring, 406 cases and 756 controls were studied. The cases included all cardiovascular malformations detected in Finland during 1982-1983, while the healthy controls were randomly selected from all babies born during the same period. Case and control mothers were interviewed after delivery using a structured and pre-tested questionnaire. Maternal overall drug consumption during the first trimester was as prevalent among case mothers (13.3%) as controls (14.6%). Neither was the risk of congenital heart disease associated with maternal use of contraceptive pills, salicylates, diazepam, or sweetening agents separately. Maternal exposures to disinfectants, dyes, lacquers, paints, pesticides, or glues at home were equally prevalent in case and control groups. Several earlier miscarriages was a predictor of an infant born with congenital heart disease (OR = 2.7, CI95 = 1.4-5.3). Maternal ultrasound examination was performed during the first 16 weeks of pregnancy more often among the case group (28.3%) than among the control group (22.0%). However, the association between ultrasound examination and the risk of congenital heart disease in the offspring was not statistically significant (OR = 1.2, 95% confidence interval 0.9-1.7) when adjusted for confounding factors such as the threat of miscarriage in logistic regression analysis. It is concluded that maternal ultrasound examination, intake of some common drugs, and exposure to a number of environmental factors at home during early pregnancy are probably not harmful for the developing fetal heart.

Case-Control Studies↗