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What pediatric residents should learn (or what pediatricians should know) about congenital heart disease.

Technologic advances in the care of children with congenital heart disease have resulted in improved care, younger ages at completion of treatment, and improved outcomes. A gap between what the pediatrician needs to do to identify a child with congenital heart disease and the specialized care provided by a cardiologist has developed over time. This has altered physician roles and the training needs for general pediatricians. This article outlines a model for training that emphasizes the skills necessary for pediatricians to identify infants and children early in life with congenital heart disease consistent with advances in diagnosis and management.

Child↗
PubMed2005

Scoliosis after median sternotomy in children with congenital heart disease.

STUDY DESIGN: A retrospective review of spinal deformity in patients with congenital heart disease surgically treated through a median sternotomy before the age of 8 years. Assessment was done on chest roentgenograms at skeletal maturity. OBJECTIVES: To determine if patients surgically treated through a median sternotomy present a higher prevalence of spinal deformity. SUMMARY OF BACKGROUND DATA: Congenital heart disease is associated with a higher prevalence of scoliosis. The etiology of scoliosis in this group of patients is unknown. Thoracotomy causes scoliosis, but median sternotomy has not been identified as a causal agent in these patients. METHODS: Chest roentgenograms were done after skeletal maturity in 128 patients to assess for the presence of a spinal deformity in the sagittal or coronal plane as a result of a median sternotomy for treatment of congenital heart disease before the age of 8 years in patients without any prior radiographic evidence of spinal or costal deformity before surgery. RESULTS: Forty-four (34.3%) of these patients had scoliosis greater than 10 degrees , 16 of them (12.5%) had curves greater than 20 degrees , and 33 (25.8%) had thoracic kyphosis lesser than 20 degrees . Patients operated before the age of 18 months had a significantly increased risk of developing scoliosis than those operated at a later age (odds ratio = 3.5; confidence interval = 1.3-9.6; P = 0.016). The presence of scoliosis was not related to the type of congenital heart disease. CONCLUSIONS: There is a high prevalence of scoliosis in patients with congenital heart disease surgically treated through a median sternotomy. The prevalence of scoliosis increases in patients operated at an earlier age.

Adolescent↗
PubMed1994

Energy expenditure in congenital heart disease.

Growth failure is a well recognised consequence of severe congenital heart disease. Total daily energy expenditure (TDEE) was investigated in eight infants with severe congenital heart disease to determine whether an increase in this parameter is an important factor in their failure to thrive, and to estimate the energy intake that would be required to allow normal growth. The infants were studied over a seven day period before surgery using the doubly labelled water method. Growth failure was evident; their mean age standardised body mass index was 80% of the expected value. Mean TDEE was 425 kJ/kg, significantly greater than in healthy infants (mean TDEE/kg SD score = +1.4; 95% confidence interval +0.27 to +2.57). In contrast, their energy intake was only 82% of the estimated average requirements. It was estimated that in early infancy a gross energy intake of 600 kJ/kg/day is required for normal growth in patients with congenital heart disease. This is unlikely to be achieved by energy supplements alone and early recourse to nasogastric feeding should be considered.

Cardiac Surgical Procedures↗
PubMed2006

The accuracy of chest radiographs in the detection of congenital heart disease and in the diagnosis of specific congenital cardiac lesions.

BACKGROUND: Congenital heart disease (CHD) is a significant cause of morbidity and mortality in pediatric patients. Traditional teaching holds that specific types of CHD can be diagnosed on the chest radiograph (CXR) through pattern recognition. OBJECTIVE: To determine the accuracy of radiologists in detecting CHD on the CXR. MATERIALS AND METHODS: This study was a blinded retrospective review of chest radiographs from 281 patients (<12 years) by five pediatric radiologists from three institutions. Thirteen groups were evaluated that included 12 categories of CHD and a control group of patients without heart disease. Radiographs were assessed for heart size, heart and mediastinal shape and vascularity. Clinical information, angiography, echocardiograms and surgery were used as the gold standard for definitive diagnosis. RESULTS: The average accuracy of the five readers in distinguishing normal from CHD patients was 78% (range of 72% to 82%). The overall measure of accuracy in distinguishing specific congenital cardiac lesions among 13 groups of patients was 71% (range of 63% to 79%). CONCLUSION: CXR alone is not diagnostic of specific cardiac lesions, with a low accuracy of only 71%. We believe that less emphasis should be placed on the use of radiographs alone in diagnosing specific congenital cardiac lesions.

Child↗
PubMed2004

Heartbeat sensitivity in adults with congenital heart disease.

This study tested the hypothesis that patients with a congenital heart disease are sensitive regarding heartbeat perception, reflected in enhanced attention for heartbeat, estimation of own heart rate, and a vulnerability to become anxious by listening to heartbeat sounds. Twenty adults with a congenital heart disease, and 20 healthy controls conducted 3 experimental tasks: a concentration task during distraction by heartbeat sounds, own heart rate estimation, and exposure to different patterns of heartbeat sounds. The results showed that patients were more distracted by heartbeat, and were also worse at estimating heart rate than controls. However, heartbeat sounds did not evoke anxiety. In conclusion, patients with a congenital heart disease may differ from controls in heartbeat perception, but there was no support for obsessive monitoring for heartbeat or excessive reactions to heartbeat sounds.

Adolescent↗
PubMed2004

Physiologic risk factors for respiratory viral infections and immunoprophylaxis for respiratory syncytial virus in young children with congenital heart disease.

BACKGROUND: Infants and children with congenital heart disease (CHD) are a population at increased risk for infection with respiratory syncytial virus (RSV) and its inherent complications compared with the general population. RSV infection increases morbidity from respiratory complications in young CHD patients. METHODS: In an effort to guide strategies for reducing RSV-associated risk in patients with CHD, predisposing factors for severe RSV as well as experimental approaches for prevention were reviewed. CONCLUSIONS: Risk factors that may exacerbate the adverse effects of respiratory disease in young CHD patients include compromised cardiorespiratory status at baseline, altered pulmonary mechanics, potential cyanosis and/or pulmonary hypertension and ventilation-perfusion mismatch. Furthermore patients with such conditions are at increased risk from RSV lower respiratory tract infection, making RSV prevention in patients with CHD an important goal. Because the efficacy and safety of active immunization for RSV have not yet been established, a number of approaches toward passive immunoprophylaxis have been investigated. Monthly prophylaxis with palivizumab, a humanized murine monoclonal anti-F glycoprotein antibody preparation, reduced the rate of hospitalization in children with CHD by 45%, as well as hospital time and oxygen use in those admitted. Thus palivizumab prophylaxis should be considered in young patients with hemodynamically significant CHD.

Antibodies, Monoclonal↗
PubMed1985

Upper limb malformations associated with congenital heart disease.

Upper limb malformations occur in patients with congenital heart disease as multifactorial, chromosomal or teratogenic combinations, and can be predictable (nonrandom) or sporadic (random) associations. The cardiac and limb defects represent either essential features of a syndrome or less common or less significant components. A practical classification is proposed based on the frequency and relative consistency of upper limb and cardiac associations. Recognition of upper limb malformations helps to identify accompanying cardiac disease and aids in the choice of diagnostic and therapeutic interventions in determination of prognosis and in genetic counseling. Upper limb malformations are the commonest skeletal abnormalities in patients with congenital heart disease, but it is also important to know with which limb defects concordance is low.

Abnormalities, Multiple↗
PubMed2006

Recommendations for participation in competitive and leisure sports in patients with congenital heart disease: a consensus document.

BACKGROUND: Physical activity is important for patients with congenital heart disease. The aim of this paper is to provide a consensus document for participation in competitive or leisure sport activity in children and adults with congenital heart disease. METHODS: The recommendations are based on expert consensus meetings, personal experience of the contributing authors and an updated review of the literature regarding exercise performance and risk stratification in patients with congenital heart disease. RESULTS: Physical performance and exercise tolerance is close to normal in patients with simple lesions with successful repair or no need for therapy. Most patients with complex lesions have some degree of residual disease, making them less suitable for participation in competitive sport. CONCLUSION: Regular exercise at recommended levels can be performed and should be encouraged in all patients with congenital heart disease. Many can attend sports with no restrictions. Special concern should be given to those patients with a significant ventricular dysfunction or recent history or risk of arrhythmia.

Adult↗
PubMed2004

Irrigated-tip catheter ablation of intraatrial reentrant tachycardia in patients late after surgery of congenital heart disease.

OBJECTIVES: The aim of this study was to evaluate irrigated-tip catheter for ablation of intraatrial reentrant tachycardias late after surgical repair of congenital heart disease. BACKGROUND: In congenital heart disease patients, the right atrium can be markedly enlarged with areas of low blood flow. Radiofrequency (RF) lesion creation may be hampered by insufficient electrode cooling at sites with low blood flow. METHODS: Thirty-six consecutive patients with intraatrial reentrant tachycardia refractory to antiarrhythmic therapy from two centers were included in the study. Entrainment pacing and electroanatomic mapping (CARTO) were used to delineate reentrant circuits and critical isthmus sites. RF ablation was performed using an irrigated-tip catheter (Navistar Thermocool). RESULTS: Fifty-two intraatrial reentrant tachycardia circuits were identified, and 48 were targeted with RF ablation. RF ablation was performed using a mean of 13 +/- 11 irrigated RF applications per tachycardia isthmus with a mean power of 36 +/- 8 W. In a historical control group of congenital heart disease patients managed with conventional catheter ablation, the number of lesions per isthmus was higher (23 +/- 11) and mean power was lower (27 +/- 14 W). Acute success was achieved in 45 intraatrial reentrant tachycardias (94% of targeted tachycardias and 87% of all tachycardias). After a mean follow-up of 17 +/- 7 months, 33 (92%) of 36 patients were free of recurrence. Five patients (14%) developed paroxysmal atrial fibrillation. CONCLUSIONS: The combination of modern techniques including electroanatomic mapping and catheter irrigation allows safe and highly effective ablation of intraatrial reentrant tachycardia in patients with surgically repaired congenital heart disease.

Adolescent↗
PubMed1985

Intraoperative color flow mapping by real-time two-dimensional Doppler echocardiography for evaluation of valvular and congenital heart disease and vascular disease.

We have developed real-time two-dimensional Doppler echocardiography, which can display color flow mapping on a two-dimensional echocardiogram. Intraoperative two-dimensional Doppler echocardiography was performed during cardiovascular operations before and after the definitive procedure in 33 patients, 14 with valvular disease, eight with congenital heart disease, and 11 with vascular disease. Its clinical usefulness was evaluated. In patients with valvular heart disease, 13 valve replacements, 10 valvuloplasties, and four untouched lesions were assessed. No paravalvular leakage and three instances of minimal physiological transvalvular leakage from St. Jude Medical valves in the mitral position were noticed. Regurgitation after valvuloplasty was graded by extent of the regurgitant flow. This grading, comparable to postoperative grading, indicated no need for an additional procedure. In congenital heart disease, preoperative diagnoses were confirmed. The effect of the corrective operation was evaluated and no significant leakage or stenosis was found. Interatrial shunt flow was shown to increase after operative balloon atrial septostomy in a patient with dextro-transposition of the great arteries. In 11 patients with dissecting aortic aneurysm, the aneurysm was totally visualized in the operative field, including the structure and flow dynamics. In two patients, the preoperative cineangiographic diagnosis regarding involvement of dissection was corrected. After the vascular procedure, sufficient flow in the major aortic branches was confirmed in all patients and minimal leakage at the suture line was noticed in four patients. In conclusion, intraoperative color flow mapping by two-dimensional Doppler echocardiography has enabled the precise diagnosis and the necessary operation to be determined before cardiopulmonary bypass. It has also allowed the effects of the operation to be assessed before chest closure.

Adult↗
PubMed2005

Congenital heart disease in a population of dizygotic twins: an echocardiographic study.

BACKGROUND: Congenital heart disease (CHD) is the most common malformation in the fetal and neonatal period but little is known about its cause. The distribution analysis of CHD in dizygotic twins could provide a useful tool to evaluate the role of genetic and environmental factors in the development of CHD. Dizygotic twins are siblings with different genes, growing together in the same womb. AIM OF STUDY: To investigate the occurrence of CHD in a large sample of dizygotic twins of nonconsanguineous healthy parents, comparing the data from non-twin patients. METHODS: From January 1999 to December 2002, we enrolled 1743 CHD patients with, at least 1 sibling, and 66 pairs of dizygotic twins, referred to our tertiary center. The diagnosis of CHD was based on clinical and echocardiographic evaluation. RESULTS: Considering only the sibling nearest in age for each non-twin patient the recurrence was 67/1743 (3.8%). Among these 67 patients, 35 (52.2%) had a sibling with the same or similar CHD. Conversely, considering all 1886 siblings, recurrence of CHD in the non-twin group was 70/1743 (4%). Of the 70 patients, 36 (36/70, 51.4%) had a sibling with the same suspected pathogenic mechanism of CHD. In 9/66 pairs of twins (13.6%), both siblings had a CHD. In the nine pairs of twins in whom both siblings had a CHD, the percentage of concordance (based on the suspected pathogenic mechanism) for CHD was 100% (p<0.05). CONCLUSIONS: Our findings suggest that the higher recurrence and concordance of CHD found in dizygotic twins could depend on some poorly identified environmental risk during the pregnancy.

Adult↗
PubMed2002

[Trichothiodystrophy and congenital heart disease in two sisters].

INTRODUCTION: Trichothiodystrophy refers to a heterogeneous group of autosomal recessive genodermatosis of unknown etiology. Patients with trichothiodystrophy have alopecia with typical hair dysplasia ("tiger-tail"), and abnormally low sulfur content. Numerous unrelated neuroectodermal defects have been observed in trichothiodystrophy. We report here trichothiodystrophy and congenital heart disease in two sisters. CASES REPORT: Two sisters were born as collodion babies and presented ichthyosis and alopecia. The diagnosis of trichothiodystrophy was confirmed by polarizing microscopy of hair and low sulfur content. Both had congenital heart disease. DISCUSSION: Cardiovascular defects have rarely been reported in trichothiodystrophy. The association trichothiodystrophy - congenital heart disease has never been described. The low incidence of both conditions suggests that these abnormalities are linked etiologically rather than by chance. Abnormal Notch signaling, or contiguous gene deletion syndrome could lead to combined phenotype as observed in our family.

Alopecia↗
PubMed1993

Infective endocarditis in the adult with congenital heart disease.

Advances in the diagnosis and therapy of congenital heart disease in the past few decades have allowed the survival of young adults who are a fertile substrate for developing bacterial endocarditis. In addition to valvular involvement, young adults with congenital disease may develop infection on the ventricular septum, or in a vascular shunt, a patent ductus arteriosus, or coarctation of the aorta. The incidence of endocarditis in children and adults with congenital heart disease is higher than with rheumatic heart disease or mitral valve prolapse. Infecting organisms, diagnosis, and therapy are similar to acquired heart disease, but the physiologic variations and patient population often add a unique flavor to the management of this group.

Adult↗
PubMed1989

Association of congenital heart disease, blepharoptosis, and short stature.

We found 12 patients with congenital heart disease of unknown etiology complicated with blepharoptosis during a period from Sept. 1, 1981 to April 30, 1989. All the patients with congenital heart disease were acyanotic, including 10 with short stature. Among these 10, abnormalities of high frequency were intrauterine growth retardation (5 cases), mental retardation (5), microcephaly (3), epicanthus (4), high arched palate (4), sacral dimple (4), and distal axial triradius (3). It is postulated that the association of congenital heart disease, blepharoptosis and short stature might indicate pathogenetic relationships.

Blepharoptosis↗
PubMed1980

Persistent left superior vena cava. Incidence, associated congenital heart defects and frontal plane P-wave axis in a paediatric population with congenital heart disease.

Among 3 671 patients aged 0-15 years with congenital heart disease diagnosed by cardiac catheterization and/or autopsy, 49 or 1.3% had a persistent left superior vena cava (PLSBC). The distribution of PLSVC among patients with various types of congenital heart disease did not suggest any causal relationship except in patients with pulmonary atresia and in patients with anomalous pulmonary venous connection, in whom PLSVC was found more frequently than expected by a mere chance relationship. Abnormal frontal plane P-wave suggesting ectopic pacemaker activity was found in 35% of the patients with PLSVC. The potential importance of PLSVC for the surgical risk during operation for congenital heart disease and for instability of the cardiac rhythm is emphasized.

Adolescent↗
PubMed2001

Aerobic capacity in adults with various congenital heart diseases.

As an increasing number of patients with congenital heart disease reach adulthood, more information is needed regarding outcomes. The first signs of impaired heart function may appear during exercise testing. The aim of the present study was to establish mean values for maximal oxygen uptake in adults with various congenital heart diseases. Patients from 6 major diagnostic groups were identified, including patients with atrial septal defect (ASD, n = 93), transposition of the great arteries corrected with the Mustard procedure (n = 84), congenitally corrected transposition of the great arteries (CCTGA, n = 41), Tetralogy of Fallot (n = 168), Ebstein's anomaly (n = 37), and Modified Fontan procedure (n = 52). Diminished maximal oxygen uptake was found in all diagnostic groups across age compared with healthy subjects. A significant decrease in maximal oxygen uptake with aging was found in those with ASD (p <0.0001), CCTGA (p = 0.01), and Tetralogy of Fallot (p <0.0001). There was no significant decline, however, in Ebstein's anomaly (p = 0.270), Fontan procedure (p = 0.182), and in the Mustard patients (p = 0.188). All patients achieved significantly lower heart rates than predicted (mean for all groups, p <0.0001). Forced vital capacity values (3.51 L, mean SD +/- 1.02) were lower than predicted values (4.10 L, mean SD +/- 0.90, p <0.0001) for all patients groups except those with ASD. Mean values, however, were within the accepted 20% range of variance. This study showed diminished aerobic capacity in all diagnostic groups when compared with a healthy population. The maximal oxygen uptake values across age groups can be used as reference values in patients with similar diagnoses and as the basis for further research.

Adolescent↗