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PubMed2001

MR and cine-MR imaging in the diagnosis of infantile congenital heart disease.

OBJECTIVE: To assess the effectiveness of magnetic resonance imaging (MRI) and cine magnetic resonance imaging (cine-MRI) in the diagnosis of infantile congenital heart disease. METHODS: A total of 34 cases were studied with MRI and cine-MRI. The data were analyzed and compared with those of two-dimensional echocardiography (2DE), cardioangiography (CAG) and surgery. RESULTS: The size of the defect or its caliber obtained from MRI in 6 patients with left to right shunt congenital heart disease was compatible with that observed in surgery (P = 0.924). Comparison of cine-MRI and CAG in 28 patients with complicated congenital heart disease showed that the diagnosis of 27 cases by cine-MRI was the same as that by CAG. CONCLUSION: Both MRI and cine-MRI play an important role in diagnosing infantile congenital heart disease.

Adolescent↗
PubMed2004

Interventional cardiac catheterisation in congenital heart disease.

As a result of recent technological advances, more types of congenital heart disease are amenable to treatment in the cardiac catheter laboratory than ever before.1 Improved imaging techniques allow for better selection of patients, and the development of a wide range of devices specifically for use in children means that many patients can avoid surgery altogether, while those with complex congenital heart disease may require fewer or less complex surgical procedures.2 This allows for a quicker recovery and a shorter hospital stay, and gives many patients an improved quality of life in the short to medium term. However, the long term outcome for many of the newer forms of intervention is still unknown.

Cardiac Catheterization↗
PubMed1985

Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study.

The Baltimore-Washington Infant Study is a regional epidemiologic study of congenital heart disease. Among Infants born in the study area in 1981 and 1982, 664 had a diagnosis of congenital heart disease confirmed in the first year of life by echocardiography, cardiac catheterization, cardiac surgery, or autopsy. The prevalence rate was 3.7/1,000 livebirths for all cases and 2.4/1,000 livebirths for cases confirmed by invasive methods only. Diagnosis-specific prevalence rates of congenital heart disease are compared with those of eight previous case series. Changing diagnostic categorizations in the time span covered and methodological differences resulted in great variation of the data. However, the data of the New England Infant Cardiac Program which used the same case discovery methods showed similar occurrences of major morphologic abnormalities, suggesting that these are stable basic estimates in the eastern United States. For all case series, the rate of confirmed congenital heart disease was approximately 4/1,000 livebirths over the 40-year time span.

Cardiac Catheterization↗
PubMed1964

ESTIMATES OF INCIDENCE AND PREVALENCE OF MONGOLISM AND OF CONGENITAL HEART DISEASE IN BRITISH COLUMBIA.

There is a need for accurate ascertainment of incidence and prevalence rates of congenital anomalies. In British Columbia the Registry for Handicapped Children and Adults used in conjunction with vital records has proved a valuable source of information. Birth notifications alone cannot be relied upon for incidence data. It was found that seven times as many cases of congenital heart disease were registered subsequently as were reported at birth. The estimated minimal incidence rates of mongolism and congenital heart disease per 1000 live births were 1.46 and 4.75, respectively. The well-known association of maternal age with mongolism was confirmed. Twice as many babies with congenital heart disease (without mongolism) were born to mothers over 39 years of age as would be expected on the basis of the maternal age distribution for all live births in the population. Prevalence estimates of these two diseases compared favourably with other published estimates.

Adult↗
PubMed1993

Thymic epithelial abnormalities in patients with congenital heart disease and Down's syndrome.

Among 85 children (< 5 years) operated on for congenital heart disease, we found 15 with structural changes of the thymus consisting of epithelial ducts and cysts. Nine of the children had Down's syndrome. In only two of these did we find epithelial abnormalities. In conclusion, these changes were not more frequent in children with Down's syndrome than in other patients with congenital heart disease.

Child, Preschool↗
PubMedDate not supplied

[Epidemiological study of congenital heart diseases in the province of Parma during the years 1972-80 (author's transl)].

We have carried out an epidemiological-statistical study about congenital heart disease on 33.245 live-born infants in Parma from 1972 to 1980. 327 newborns with congenital heart disease were found; the incidence was 9,8/1000 live-born. The lowest value was 5,8/1000 live-born in 1972, the highest was 14,5/1000 live-born in 1977. In the majority of cases diagnosis was only clinical VSD, PDA, ecc.), whereas in the more complex cardiac malformations and in patients with severe congestive heart failure diagnosis was confirmed at cardiac catheterization and at surgery. In 60 cases diagnosis was confirmed also at autopsy. The congenital heart disease most frequently observed was VSD (33%), followed by PDA (13,4%) and TGA (8,8%), ecc. Clinical status at 1 year of age in all patients was evaluated. The Authors think that infant cardiac program is necessary to improve the results on congenital heart disease control.

Europe↗
PubMed2004

Measurement of tracheal size in children with congenital heart disease by computed tomography.

BACKGROUND: To establish a reference of tracheal size in children with congenital heart disease to allow detection of airway stenosis. METHODS: We conducted a retrospective study using existing data from children referred for computed tomography (CT) scanning. From January 1999 to February 2001, 540 consecutive children with congenital heart disease who received electron beam CT scanning at our hospital were considered eligible for inclusion in the study. After exclusion criteria were considered, 99 children (50 girls and 49 boys; aged 4 days to 16 years 10 months) were enrolled in the study. Tracheal width was measured at three levels on CT images. The relationship between tracheal width and the patient's height, weight, age, and sex were analyzed by multiple regression and formula transformation. RESULTS: Height was the most effective parameter for predicting the transverse diameter of the intrathoracic trachea, and tracheal size could be predicted based on height using the established equations. In addition, tracheal width increased from the thoracic inlet to the carina. Reference curves based on the subject's height were created for convenient use. CONCLUSIONS: Tracheal stenosis in children with congenital heart disease may be diagnosed by comparing the size of the trachea of the individual to the 95% confidence interval of predicted values based on the patient's height.

Adolescent↗
PubMed1995

Benefits and potential risks of atrial antitachycardia pacing after repair of congenital heart disease.

UNLABELLED: Atrial reentry tachycardia is common after surgical repair of congenital heart disease. The arrhythmia is often difficult to treat and is occasionally life-threatening. This study reports experience with atrial antitachycardia (AAIT mode) pacing for the management of atrial reentry tachycardia, with emphasis on the risks and benefits of automatic pacing therapy. Eighteen patients (2-32 years of age) with a variety of congenital heart lesions underwent atrial antitachycardia pacemaker placement for recurrent atrial tachycardia that was amenable to pace termination prior to the implantation procedure. An appropriate antitachycardia program was determined by repeated induction and termination of atrial tachycardia using the noninvasive programmed stimulation mode of the pacemaker. Over 4-30 months of follow-up, 6 patients had 189 episodes of tachycardia successfully converted with AAI-T pacing, 4 patients had 8 episodes of tachycardia detected but not successfully converted, and 8 patients had no episodes of tachycardia with antibradycardia pacing alone. The number of patients receiving pharmacological therapy other than digoxin or beta blockade fell from 12 to 6. Two subjects died suddenly, 1 while wearing a Holter monitor. In both, tachycardia was detected and pace cardioversion attempted. CONCLUSIONS: Atrial antitachycardia pacing is a useful tool in the management of patients with congenital heart disease and atrial arrhythmias; however, in selected cases, it may not prevent and may even exacerbate the lethal complications of the tachycardia. Antitachycardia function evaluation is recommended under varying levels of autonomic stress prior to institution of automatic therapy.

Adolescent↗
PubMed1998

Airway compression in children due to congenital heart disease: value of flexible fiberoptic bronchoscopic assessment.

OBJECTIVE: To evaluate the frequency and severity of airway compression due to congenital heart disease in children and validate the use of the fiberoptic bronchoscope to assess them. DESIGN: A retrospective study. SETTING: A single-institutional study in a university hospital. PARTICIPANTS: Seventy-two children with congenital heart disease. INTERVENTIONS: Airway endoscopy was performed in an awake child in cases of clinical and/or radiologic respiratory signs or in cases of preoperative assessment of a cardiac abnormality that is known to accompany airway compression. MEASUREMENTS AND MAIN RESULTS: Endoscopy was well tolerated; 71% of the children had endoscopic abnormalities and 50% had airway compression. The locations of these compressions are the same as those described in the literature in the cases of vascular rings and left-to-right shunts. The other endoscopic findings were laryngeal and bronchial abnormalities, tracheobronchial malacia, respiratory signs of gastroesophageal reflux, and positive bacteriologic sputum samples. CONCLUSION: Endoscopy in an awake patient is the only way to evaluate the functional component of a compression due to malacia; the resulting collapse of the airway can cause trapping of air and secretions. Furthermore, fiberoptic bronchoscopy offers a complete examination of the airways and can help detect airway abnormalities that are potential causes of complications. Fiberoptic bronchoscopy is a suitable and well-tolerated examination that is easy to perform at the bedside of the child. This technique optimizes the preoperative assessment of children with congenital heart disease.

Adolescent↗
PubMed2000

Renal function after cardiopulmonary bypass surgery in cyanotic congenital heart disease.

UNLABELLED: We studied perioperative renal damage in 22 patients with long-standing cyanotic congenital heart disease. BACKGROUND: [corrected] Postoperative acute renal failure is a major complication of cardiac surgery associated with poor prognosis. Our study was designed to identify risk factors for renal failure in patients with cyanotic congenital heart disease. PATIENTS: 22 cyanotic patients with a oxygen saturation of 82% (38-92%), age 14 years (5-42 years) and six controls with atrial septal defect, age 37 years (28-66 years) were investigated with repeated urinary analyses. RESULTS: Before operation, six of 22 of the cyanotic patients had albuminuria. Postoperatively three patients developed acute renal failure including glomerular and tubular damage. Urinary albumin analysis >1000 mg g(-1) creatinine after 24 h and N-acteyl-beta-D-glucosaminidase analysis >100 U g(-1) creatinine after 48 h predicted dialysis requirement. In noncyanotic controls only one preoperative and none of the postoperative analyses were pathological. CONCLUSIONS: Patients with cyanotic congenital heart disease are at risk for acute renal failure, which can be prognosed by urine analysis already 24 h after surgery. Cyanotic glomerulopathy should be known before surgery. To lower the risk, cardiopulmonary bypass time should be kept as short as possible and adequate hydration should be maintained in combination with extended diuretic therapy.

Acetylglucosaminidase↗
PubMed2005

Ocular pathology in congenital heart disease.

PURPOSE: To describe the ocular findings in subjects with congenital heart disease (CHD). METHODS: In a prospective study, the same observer examined 240 consecutive patients with CHD admitted to the medical centre. Two independent geneticists performed identification of syndromes. RESULTS: The commonest anatomic cardiac anomalies were ventricular or atrial septal defects (62), tetralogy of Fallot (39), pulmonary stenosis (25), and transposition of the great arteries (24). The heart lesions were divided physiologically into volume overload (90), cyanotic (87), and obstructive (63). In all, 105 syndromic subjects included the velocardiofacial syndrome (18), Down's syndrome (17), CHARGE association (6), DiGeorge syndrome (5), Williams syndrome (3), Edwards syndrome (3), Noonan syndrome (3), VACTERL association (2), and Patau syndrome (trisomy 13) (2). The paediatric team recognized 51 patients as syndromic. Two independent geneticists recognized additional 54 patients as syndromic. Positive eye findings were present in 55% (132) and included retinal vascular tortuosity (46), optic disc hypoplasia (30), trichomegaly (15), congenital ptosis (12), strabismus (11), retinal haemorrhages (8), prominent eyes (7), and congenital cataract (6). There was a strong correlation between the retinal vascular tortuosity and both a low haematocrit (P=0.000) and a low arterial oxygen saturation (P=0.002). CONCLUSIONS: Patients with CHD are at a high risk for ocular pathology and need screening for various ocular abnormalities.

Child, Preschool↗
PubMedDate not supplied

[Nutritional recovery in infants with congenital heart disease and severe malnutrition using a hypercaloric diet].

Protein-energy malnutrition is commonly observed in infants with congenital heart disease (CHD). The effect of a hypercaloric formula on nutritional recovery was evaluated in malnourished infants with congenital heart diseases. We retrospectively studied 30 infants (mean age 9 months, range 2 to 21) with CHD along 60 to 90 days in a closed nutritional recovery center. All patients were fed a hypercaloric whole cow's milk formula with sucrose, butter oil and corn starch (1.29 kcal.ml). Mean daily energy intake was 220 +/- 19.8 kcal.kg body weight.day. Mean weight gain was 2.7 g.kg.day. No effect of cyanosis, cardiac failure, degree of malnutrition or number of illness-free days was observed on nutritional recovery. Patients older than 9 months had significantly greater weight gain than younger infants. Those infants with worse CHD and severe intercurrent illnesses had the worst nutritional recovery. We suggest that a hypercaloric formula induces good nutritional weight gain infants with severe congenital heart disease.

Administration, Oral↗
PubMed1992

Potential impact of population screening for prenatal diagnosis of congenital heart disease.

The potential impact of prenatal screening for the detection of congenital heart disease (CHD) was assessed by prospective analysis of 428 consecutive infant admissions to a supraregional centre; 28 (6.5%) did not have CHD and were excluded from analysis. Of the 400 cases with CDH, 396 (99%) underwent fetal ultrasonography but scanning was performed only before 18 weeks' gestation in 200 (50%). One hundred and forty nine (37%) of all cardiac abnormalities and 149/283 (53%) of severe abnormalities were considered to be detectable prenatally in a screening echocardiographic four chamber view had this technique been used. Prenatal diagnosis of severe CHD actually occurred in only eight (2%) cases and was after 30 weeks' gestation in all. There were 181/347 (52%) of all mothers and 177/253 (70%) of the subgroup with severe abnormalities who expressed an opinion volunteered their preference for termination of pregnancy if mid-trimester diagnosis had been available. Mid-trimester detection of congenital heart disease rarely occurs at present despite fetal ultrasound scanning in almost all pregnancies. More than half of all severe congenital heart defects seen in infancy are potentially detectable by screening. Major training at primary scan level and modification of the timing of existing fetal anomaly scanning would be required for a screening programme to be effective.

Female↗
PubMed2003

Transitioning adolescents with congenital heart disease into adult health care.

The effort and commitment dedicated to the treatment of children with congenital heart disease over the past 50 years has been astounding. Therefore, it is imperative to ensure these young patients who have survived the varying challenges of their conditions a smooth transition into a new world of adult health care. The key to the long-term management of an increasing number of children surviving congenital heart disease is the effective coordination of care from pediatric to adult health care. Strategies for facilitating the transition of the adolescent with congenital heart disease into adult health care practices are presented in this paper.

Adaptation, Psychological↗
PubMed1999

Sequential segmental approach to fetal congenital heart disease.

The sequential segmental approach is now universally used in the diagnosis of congenital heart disease. Its utilization during fetal sonography has not been well described. In this review, we show how the fetal heart can be approached in a sequential segmental manner by using six basic sonographic views. The transverse view of the fetal upper abdomen is obtained to determine the arrangement of the abdominal organs, which, in most cases, provides the important clues to the determination of the atrial arrangement. The four-chamber view is obtained to evaluate the atrioventricular junctions. The views of the left and right ventricular outflow tracts are obtained to evaluate the ventriculoarterial junctions. The three-vessel view and the aortic arch view are obtained for the evaluation of the arrangement and size of the great arteries, which provides the additional clues to the diagnosis of the abnormalities involving the ventriculoarterial junctions and the great arteries. The standard protocol to acquire these six basic views is also introduced.

Echocardiography↗
PubMedDate not supplied

Response to prostaglandin E1 in neonates with intracranial arteriovenous malformation treated for suspected congenital heart disease.

Two neonates with cardiovascular symptoms associated with intracranial AVM were initially considered to have ductal-dependent congenital heart disease. Prostaglandin E1 (PGE1) infusion, initiated to achieve patency of the ductus arteriosus, produced improved oxygenation and cardiovascular status in both infants. Other vascular effects of PGE1, including pulmonary and extracranial systemic vasodilation, likely accounted for these unique observations in the infants with intracranial AVM. Not only may the signs and symptoms of congenital heart disease be imitated by intracranial AVM, but improved oxygenation and cardiovascular status with PGE1 infusion used for suspected congenital heart disease may be observed as well.

Alprostadil↗
PubMed1999

Evolution and long term outcome in cases with fetal diagnosis of congenital heart disease: Italian multicentre study. Fetal Cardiology Study Group of the Italian Society of Pediatric Cardiology.

OBJECTIVES: To analyse the evolution and outcome in utero and after birth of infants with a fetal diagnosis of congenital heart disease. DESIGN: Inclusion criteria were the fetal diagnosis of congenital heart disease, confirmed postnatally or postmortem, and a complete follow up in utero and after birth. SETTING: 20 centres operating prenatal echocardiographic screening. PATIENTS: 847 cases were included in the study. Gestational age at diagnosis ranged from 15-39 weeks; in 370 cases (43.7%) the diagnosis was made before 24 weeks' gestation. RESULTS: 245/847 cases (28.9%) were terminated during pregnancy, 227 following early diagnosis; 128/245 cases (52. 2%) had associated anomalies and 117/245 (47.8%) had serious congenital heart disease. Of the remaining 602 cases that continued the pregnancy, 72 (11.9%) died in utero, 259 (43%) died postnatally (83 after surgery or invasive procedures), and 271 infants (45%) survived and presently range in age from 18 months to 13 years old. The mortality rate was higher in cases with associated extracardiac or chromosomal anomalies (68% and 74% of cases continuing pregnancy, respectively), and in cases with heart failure and complex cardiac defects. CONCLUSIONS: The data confirm a relevant fetal and postnatal loss in cases with complex congenital heart disease, and major clinical use of prenatal diagnosis in the management of ductus dependent anomalies. Negative prognostic factors for the outcome were associated anomalies and heart failure.

Abortion, Therapeutic↗