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Malnutrition in hospitalized children with congenital heart disease.

OBJECTIVE: To determine the prevalence of malnutrition among hospitalized children with congenital heart disease by age, disease process, and clinical status. DESIGN: Cross-sectional, retrospective chart review. SETTING: Pediatric cardiology units at a 150-bed tertiary care teaching hospital in Ann Arbor, Mich. PATIENTS: Patients (n = 160) were randomly selected from consecutive admissions to the Pediatric Cardiology and Thoracic Surgery Services during a 1-year period. INTERVENTION: None. MAIN OUTCOME MEASURES: Acute and chronic malnutrition, assessed by comparing the patients' weight and height with established means. RESULTS: Acute and chronic malnutrition occurred in 33% and 64% of the patients, respectively. Age, diagnostic category, and symptoms were associated with malnutrition. Eighty percent of infants presented with acute malnutrition compared with 18% of patients of other ages (P < .001). Malnutrition affected 60% of patients with left-to-right shunts, 53% of patients with complex heart disease, and no patients with primary rhythm disturbances. Acute malnutrition affected 11% and chronic malnutrition affected 50% of patients with left-sided heart obstruction. Acute or chronic malnutrition occurred in 70% or more of patients with cyanosis and/or congestive heart failure but in only 30% of patients with neither (P < .001). CONCLUSION: Malnutrition in hospitalized children with congenital heart disease remains common, highlighting the importance of nutritional screening and intervention.

Acute Disease↗

[The magnetic resonance tomography of complex congenital heart diseases in adults].

An increasing number of patients with complex congenital heart disease reaches adulthood, because treatment and patient outcome have improved considerably in recent years. Monitoring of these patients requires both definition of cardiac anatomy and assessment of function with good reproducibility. Complications after surgical repair such as restenoses of pulmonary arteries after surgical repair of tetralogy of Fallot, ventricular hypertrophy, stenoses or leakage of baffles, or stenosis and aneurysms of anastomoses have to be detected at an early stage. Magnetic resonance imaging (MRI) is an excellent tool to monitor these patients because of its noninvasive nature, its good interstudy and interobserver reproducibility, and because it allows assessment of both cardiac anatomy and function. This paper reviews the current applications of MRI in complex congenital heart disease in adults.

Adult↗

Cardiac resynchronization therapy in congenital heart disease.

While cardiac resynchronization therapy (CRT) is of proven benefit in selected patients with severe ischemic or dilated cardiomyopathy, refractory symptoms, and conduction delay, extrapolation to congenital heart disease is not straightforward. This rapidly expanding patient population commonly suffers from heart failure, particularly in the presence of a single or systemic right ventricle. Surgical repair may also contribute to ventricular asynchrony. In this systematic review, the current state of knowledge regarding CRT in congenital heart disease is presented. Issues specific to congenital heart disease including right bundle branch block, right (pulmonary) ventricular dysfunction, systemic right ventricular dysfunction, and single ventricle dysfunction are explored. Evidence-based CRT applications for each of these particular conditions are reviewed. Initial experience with CRT in the acute postoperative setting and longer-term, including our own, is elaborated. Unlike standard indications based on multiple randomized clinical trials, supporting evidence for CRT in congenital heart disease is limited to case reports, case series, and small experimental crossover studies in the acute postoperative setting. The heterogeneous patient population, technical limitations from patient size, vascular access issues, and unique forms of ventricular asynchrony further obscure the selection of potential beneficiaries. Despite these limitations, experience thus far has been favorable. Quality of current data precludes definitive evidence-based recommendations, but optimistic initial results suggest that research endeavors in this field should be pursued. Multicenter prospective collaborative efforts are to be encouraged.

Cardiac Pacing, Artificial↗

Training in the care of adult patients with congenital heart disease.

The growing population of patients with congenital heart lesions surviving to adulthood necessitates a fresh look at the clinical training required to care for these patients. Physicians in pediatric and medical cardiology, general pediatrics, general medicine, family practice, obstetrics and gynecology and other specialities will all have a role in the care of these patients. Most likely, training will need to be incorporated into existing pathways with a clear delineation of a body of knowledge necessary to assimilate to complete the training program. The authors favor a certificate of added qualification for the treatment and management of adults with congenital heart disease to encourage excellence in preparation of physicians to care for this complex patient population.

Adult↗

ECG determinants in adult patients with chronic right ventricular pressure overload caused by congenital heart disease: relation with plasma neurohormones and MRI parameters.

OBJECTIVE: To examine retrospectively the changes in ECG parameters over time and their correlation with other quantitative right ventricular (RV) function parameters in patients with chronic RV pressure overload caused by congenital heart disease. METHODS: 48 patients with chronic RV pressure overload caused by the following congenital heart diseases were studied: nine with congenitally corrected transposition of the great arteries (TGA), 12 with surgically corrected TGA, and 27 with a subpulmonary pressure overloaded RV. QRS duration and dispersion were measured manually from standard ECG recorded twice within five years. RV end diastolic volume (EDV) and RV mass were determined by magnetic resonance imaging. Brain natriuretic peptide (BNP) plasma concentrations were measured. RESULTS: QRS duration and QRS dispersion increased in all patient groups during the follow up period. QRS duration increased significantly in the congenitally corrected TGA (p = 0.04) and the subpulmonary pressure overloaded RV groups (p = 0.01). QRS dispersion increased significantly in patients with surgically corrected TGA (p = 0.03) and in the subpulmonary pressure overloaded RV group (p = 0.02). A significant correlation was found between QRS duration and RVEDV (r = 0.71, p < 0.0001). RV mass was significantly correlated with QRS duration in patients with tetralogy of Fallot (r = 0.67, p = 0.01). Mean (SD) plasma brain natriuretic peptide concentrations (6.6 (5.4) pmol/l) were increased compared with normal reference values but no correlation was found with ECG parameters or RV systolic pressure. No malignant arrhythmia or sudden death occurred. CONCLUSIONS: ECG parameters worsened gradually in asymptomatic or minimally symptomatic patients with chronic RV pressure overload, regardless of the nature of their congenital heart disease. In all patients, a significant positive correlation was found between QRS duration and RVEDV. In patients with tetralogy of Fallot there was also a correlation between QRS duration and RV mass.

Adult↗

Quantitation of the global right ventricular function in children with normal heart and congenital heart disease: a right ventricular myocardial performance index.

Although the assessment of right ventricular (RV) function is important in the clinical management of children with congenital heart disease, available imaging techniques have been limited because of the complex geometry of the right ventricle. A new Doppler index combining systolic and diastolic time intervals (the Tei index) has been reported to be useful for the assessment of global RV function in adults. However, normal values in children, age-related changes, and the clinical utility of the Tei index with regard to congenital heart disease have not been demonstrated. The purpose of this study was to prospectively assess RV function in children with normal heart and congenital heart disease using the Tei index. The subjects included 150 healthy children and 43 patients with congenital heart disease (35 patients with atrial septal defects and 8 patients who had had a Senning operation). The index was defined as the sum of isovolumetric contraction time and isovolumetric relaxation time divided by ejection time and was measured from conventional RV outflow and inflow Doppler velocity profiles. The Tei index was not affected by age in healthy children (0.24 +/- 0.04). There was a significant difference in index rating between patients who had had a Senning operation (0.58 +/- 0.09) and healthy children (p < 0.01), but there was no significant difference between children with atrial septal defect (0.25 +/- 0.13) and healthy children. The Tei index is a feasible approach to use when assessing global RV function in children with congenital heart disease.

Adolescent↗

Congenital heart disease in the adult: clinical approach.

The remarkable advances in diagnostic techniques and in the surgical and medical care of infants and children with congenital heart disease have resulted in an impressive increase in longevity. Congenital heart disease in adults has emerged as a specialized area of cardiovascular interest. In response, special multidisciplinary facilities have been developed for the comprehensive care of adults with congenital heart disease. The scope of the discipline is relatively broad and includes unoperated survival patterns; survival patterns after surgery or interventional catheterization; a host of medical considerations; surgical considerations (operation and reoperation, cardiac catheterization as a therapeutic intervention, and noncardiac surgery); and the important problems of postoperative residua and sequelae. The purpose of this communication is to call attention to this subspecially and to excite the interest of radiologists in certain imaging aspects of congenital heart disease in adults.

Adult↗

Congenital heart disease; comments regarding incidence and natural history.

The incidence of congenital heart disease is approximately 1 per cent of all live births. Approximately 60 per cent of patients who die of congenital heart disease do so at less than two years of age. Very few patients with such lesions live beyond 45 years at the very most. In about 70 per cent of patients who are born with cardiac anomalies, the lesions are either of kinds that are already being operated upon successfully or for which operations are now being attempted and often are helpful.

Cardiovascular Abnormalities↗

Trends in congenital heart disease in Dallas County births. 1971-1984.

To examine the changes in birth cohort prevalence rates and severity of congenital heart disease, we studied children with congenital heart disease born to blacks, whites, and Mexican-Americans in Dallas County from 1971 through 1984. Diagnoses were made by pediatric cardiologists' clinical evaluations, echocardiography, catheterization, surgery, or autopsy. During this study period, 2,509 of 379,561 liveborn infants were diagnosed, a prevalence rate of 6.6/1000. The rates for whites was significantly higher than for blacks or Mexican-Americans--7.2/1,000, 5.6/1,000, and 5.9/1,000, respectively. The rate for severe cases requiring cardiac catheterization or surgery or undergoing autopsy was 3.1/1,000 and did not differ among the three groups. The time trend for rates of congenital heart disease suggested an apparent increase in prevalence rate during the 1970s; however, the prevalence rate of severe forms remained relatively stable. This indicates that the apparent rise in prevalence could be accounted for by an increase in detection of mild cases. These findings were interpreted as reflecting a greater tendency for pediatricians to refer asymptomatic children with significant heart murmurs to a pediatric cardiologist.

Black People↗

Diet and growth in congenital heart disease.

To assess the relationship between diet and growth in congenital heart disease we studied nutrient intake, body measurements, and cardiac status in 568 affected ambulatory patients less than 11 years of age. Most had mild heart disease, 104 were cyanotic, and only 10 were in congestive heart failure. Major disturbances of growth were uncommon. For the entire group body weight was below normal but only in those studied before 2 years of age; rate of growth and weight gain were normal over the period preceding the dietary study. There was no statistically significant relationship between intake of calories, protein, or other nutrients and growth or gain, analyzing the entire group, or analyzing patients subgrouped according to age, severity of heart disease, or severity of growth retardation. Body size and growth were diminished in cyanosed compared with noncyanosed children but cardiac status had no effect on nutrient intake. We conclude that in children with growth failure associated with congenital heart disease, nutrient intake is not an important factor limiting their growth.

Body Height↗

Adult patients' knowledge about their congenital heart disease.

OBJECTIVE: To assess patient knowledge about their congenital cardiovascular disease. DESIGN: Fifty consecutive patients (25 women) attending the Adult Congenital Heart Disease Clinic, University of Alberta, Edmonton, Alberta, filled out a questionnaire that tested knowledge about their heart defect and endocarditis prophylaxis. The patients ranged in age from 18 to 60 years (mean 25). The most frequent cardiovascular anomalies were obstruction of the left ventricular outflow tract (13), coarctation of the aorta (10), tetralogy of Fallot (six), transposition of the great arteries (five) and Marfan syndrome (four). Patients had been seen on average by three cardiologists (range one to six) and had made an average of seven clinic visits (range one to 22) since the age of 17 years. Eight patients had been followed at other clinics in the past. MAIN RESULTS: Fifty-four per cent of patients knew their diagnosis. Forty-four per cent could explain the defect in lay language, and another 48% made an attempt. When given a heart diagram, 26% marked their defect correctly and 28% made an attempt. Terms 'endocarditis' and 'antibiotic prophylaxis' were known to 16% and 22% of patients, respectively. Fifty-eight per cent could name at least one situation that carries a risk of 'infection in the heart'. CONCLUSION: Adults with congenital heart disease have poor knowledge of their heart defects and the importance of endocarditis and antibiotic prophylaxis. Although time consuming, patient education should be a part of every clinic visit. Repetitive and structured patient education may improve patients' knowledge and, hence, participation in their health care.

Adolescent↗

Effect of congenital heart disease on neurodevelopmental outcomes within multiple-gestation births.

OBJECTIVES: We sought to assess the effect of congenital heart disease requiring surgical intervention with cardiopulmonary bypass at 6 months of age or less on developmental outcomes and growth at 1 year of age while controlling for socioeconomic status, prematurity, home environment, and parental intelligence. METHODS: We performed within-family comparison of 11 multiple-gestation births in which one child had congenital heart disease. At 1 year of age, the Bayley Scales of Infant Development II were administered, and growth parameters were assessed. Paired comparisons were made by using fixed effects regression conditioned on family. RESULTS: The multiple-gestation subjects were mildly premature on average (mean gestational age, 35.4 +/- 3.0 weeks). At 1 year of age, children with congenital heart disease scored lower on the Mental Development Index (85.0 +/- 19.3 vs 93.9 +/- 16.0, P = .037) and the Psychomotor Development Index (76.6 +/- 16.9 vs 91.3 +/- 14.9, P = .015) on the Bayley Scales of Infant Development II than did their siblings without congenital heart disease. There were no differences between siblings in weight, height, or head circumference. CONCLUSIONS: The presence of congenital heart disease requiring surgical intervention with cardiopulmonary bypass at 6 months of age or less is associated with a deficit in developmental achievement at 1 year of age, as measured by using the Bayley Scales of Infant Development II.

Female↗

[Application of cardiovascular magnetic resonance in congenital heart disease].

Cardiovascular magnetic resonance (CVMR) is the best non invasive imaging technique for the diagnosis of congenital heart disease (CHD) as it gives important information concerning anatomy and function of the heart in the same exploration. This method is useful for the follow up of the patient with congenital heart disease. The most important benefits of CVMR are the non invasiveness of the same and the lack of ionic radiation. The objective of this article is to demonstrate that congenital heart disease can be diagnosed with CVMR with a higher precision compared with other cardiovascular imaging techniques.

Cardiovascular System↗