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PubMed1998

The effects of congenital heart disease on the lungs.

The alterations in anatomy and physiology, which often accompany congenital heart disease, may have a major impact on the child's lungs. These changes in pulmonary blood flow and ventilation are usually demonstrable on chest radiography and should be recognized as manifestations of the underlying lesion and not mistaken for primary lung disease. Correlation with the clinical history and physical examination are essential for correct interpretation, but radiological findings should be viewed objectively and without bias to provide accurate and valuable information to the treating physician.

Child↗
PubMed2001

Evaluation of internal behaviors of children with congenital heart disease.

This study was designed to determine how internal behaviors of children with congenital heart disease were handled by their mothers. The study group included the mothers of 96 children randomly selected (ages ranging from 4 to 11 years) who were being monitored at the Institute of Cardiology. A 21-item questionnaire was administered to obtain information about the mother and the child. Internal subsets of the Child Behavior Check List (CBCL)/4-18 were performed, including withdrawn aggressive behaviors, somatic complaints, and anxiety/depression tests. The results showed that factors influencing withdrawn aggressive behavior were low parent education (p =.000), poor economic status of the family (p =.02), and aggravation of the hemodynamic status of the disease (p =.003). The factors influencing somatic complaints were low parent education (p =.000) and severity of the hemodynamic status of the disease (p =.02). An increase in the number of children in the family seemed to have an effect on anxiety/depression levels (p =.009).

Adolescent↗
PubMed2001

The role of embolization therapy in the treatment of congenital heart disease.

Embolization therapy plays a significant role in the treatment and palliation of congenital heart disease. Currently, there are several devices available to occlude these superfluous vascular connections. This article reviews the indications, results and the current technology available for transcatheter embolization of vessels.

Catheterization↗
PubMed1999

Prospective diagnosis of alveolar capillary dysplasia in infants with congenital heart disease.

Alveolar capillary dysplasia (ACD) is a lethal pulmonary disorder found in newborns that is characterized by severe pulmonary hypertension and hypoxemia. We report on the clinical behavior of this disorder in a series of patients and its association with congenital heart disease, especially left heart obstructive disease; we also report a prospective diagnosis of ACD by lung biopsy in a newborn with congenital heart disease, which prevented futile and prolonged medical intervention.

Arteriovenous Malformations↗
PubMed2004

Validity, reliability and responsiveness of the "Schedule for the Evaluation of Individual Quality of Life-Direct Weighting" (SEIQoL-DW) in congenital heart disease.

BACKGROUND: The 'Schedule for the Evaluation of Individual Quality of Life-Direct Weighting' (SEIQoL-DW) is an instrument developed to measure individual quality of life. Although this instrument has been used in numerous studies, data on validity and reliability are sparse. This study aimed to examine aspects of validity, reliability and responsiveness of the SEIQoL-DW on data obtained in adults with congenital heart disease, by using the new standards of psychological testing. METHODS: We evaluated validity evidence based on test content, internal structure, and relations to other variables, as well as the stability and responsiveness of the SEIQoL-DW. Evidence was provided by both theoretical considerations and empirical data. Empirical data were acquired from two studies. Firstly, using a cross-sectional study design, we included 629 patients with congenital heart disease. Secondly, 130 of the 629 initially included patients readministered the questionnaires approximately one year after the first data collection. In addition to the SEIQoL-DW, linear analog scales were used to assess overall quality of life and perceived health. RESULTS: We found that the SEIQoL-DW is not a valid measure of quality of life, but rather assesses determinants that contribute to individuals' quality of life. The SEIQoL-DW consistently proved to be valid and reliable to assess those determinants. However, responsiveness in patients with congenital heart disease may be problematic. CONCLUSION: Based on theoretical and empirical considerations, the SEIQoL-DW cannot be considered as a quality of life instrument. Nonetheless, it is a valid and reliable instrument to explore determinants for patients' quality of life.

Adaptation, Psychological↗
PubMedDate not supplied

Auditory brainstem responses in congenital heart disease.

To evaluate the effect of chronic hypoxemia on brainstem maturation, auditory brainstem responses were examined in 70 children (32 with and 38 without cyanosis) who had congenital heart disease. Ninety-one age-matched normal children served as controls. At 1-3 months of age, the I-V interpeak latencies of cyanotic infants (mean +/- S.D.; 5.17 +/- 0.17 ms) were more prolonged than were those of controls (4.95 +/- 0.11 ms) and those without cyanosis (4.84 +/- 0.22 ms; P < .05; P < .01). At 4-11 months of age, the I-V interpeak latencies of cyanotic infants (4.85 +/- 0.13 ms) were more prolonged than were those of controls (4.67 +/- 0.19 ms) and those not experiencing cyanosis (4.5 +/- 0.17 ms; P < .05; P < .01). In the cyanotic children, there was a significant negative correlation between the I-V interpeak latency and oxygen partial pressure (P < .01) or oxygen saturation (P < .05). Three of the 70 patients (4.3%) with congenital heart disease had absent auditory brainstem response. These data indicate that chronic hypoxemia may be one of the factors in retarded brainstem maturation.

Brain Stem↗
PubMed1993

Congenital heart disease in the adult.

With improved medical and surgical care, more patients with congenital heart disease are now surviving to adulthood and presenting with previously unobserved problems. This review discusses the course of older operated and unoperated patients as far as physical and psychosocial problems and suggests methods of dealing with these previously unencountered clinical situations.

Adult↗
PubMed2002

Consensus-based method for risk adjustment for surgery for congenital heart disease.

OBJECTIVE: The aim was to develop a consensus-based method of risk adjustment for in-hospital mortality among children younger than 18 years after surgery for congenital heart disease (designated RACHS-1). METHODS: An 11-member national panel of pediatric cardiologists and cardiac surgeons used clinical judgment to place surgical procedures into six risk categories. Categories were refined after review of information from the Pediatric Cardiac Care Consortium and three statewide hospital discharge data sets. The effects of including additional clinical variables were explored by comparing areas under receiver-operator characteristic curves. RESULTS: Among 4602 surgical patients in the Pediatric Cardiac Care Consortium data set and 4493 in the hospital discharge data, 3767 (81.9%) and 3832 (85.3%), respectively, had a single cardiac procedure, and 98.5% and 89.2%, respectively, were able to be assigned to one of six risk categories defined by the panel. Mortality rates showed expected trends (P <.001). For the Pediatric Cardiac Care Consortium data, mortality rates were 0.4% in category 1, 3.8% in 2, 8.5% in 3, 19.4% in 4, and 47.7% in 6; rates were similar in the hospital discharge data. There were too few cases in category 5 to estimate mortality rates. In multivariable models, younger age, prematurity, and the presence of a major noncardiac structural anomaly added to the risk of in-hospital death predicted by risk category alone. Best performance was obtained when cases with multiple procedures were placed in the risk category of the most complex procedure. CONCLUSION: The RACHS-1 method should adjust for baseline risk differences and allow meaningful comparisons of in-hospital mortality for groups of children undergoing surgery for congenital heart disease.

Cardiac Surgical Procedures↗
PubMed1996

Diagnostic problems in a case with mucometrocolpos, polydactyly, congenital heart disease, and skeletal dysplasia.

Mucometrocolpos is the distention of the uterus and vagina caused by obstruction to the drainage of genital secretions. Although most cases of mucometrocolpos are sporadic, it may be part of an autosomal recessive condition, known as McKusick-Kaufman syndrome (MKS), including postaxial polydactyly and congenital heart disease as main findings. The diagnosis may be difficult when the presence of additional findings creates an overlap with other syndromes. We report on a female infant with mucometrocolpos, postaxial polydactyly, congenital heart disease, short limbs, short ribs, and chest constriction. The clinicopathological findings are described and discussed in the context of the phenotypic spectrums of MKS and mucometrocolpos concomitant with Ellis van Creveld syndrome.

Abnormalities, Multiple↗
PubMed1994

[Cardiac transplantation for old congenital heart diseases after multiple surgery].

Of the 100 consecutive patients undergoing cardiac transplantation between January 1988 and October 1993, 4 patients had terminal cardiac failure related to congenital heart disease after multiple prior palliative procedures (transposition of the great arteries, N = 1, tricuspid atresia, N = 1, single ventricle, N = 2). The prior palliative or curative operations (average 3.75 procedures per patient) modified essentially the systemic venous return and the pulmonary arteries. The technique of "subtotal" cardiac transplantation enabled anatomical reconstruction without prosthetic material in all cases by extensive usage of the donor tissue. There was no hospital mortality. There were no specific postoperative complications. The long-term results were comparable to those of the rest of the transplanted population. Patients with congenital heart disease in a terminal condition should be considered as candidates for cardiac transplantation. The difficulties related to anatomical abnormalities caused by prior surgery may be overcome and should not be considered a contra-indication to transplantation, providing pulmonary arterial resistances are taken into consideration.

Adolescent↗
PubMed1993

Contrast echocardiography during cardiac catheterization in patients with congenital heart diseases.

Contrast echocardiography performed during cardiac catheterization has mostly been limited to a few published case reports. We studied 37 patients with congenital heart disease to assess the capability of the method to diagnose cardiac shunts and valve regurgitation. Injections of 5% dextrose in water through an angiographic catheter were made to evaluate septal integrity and valve competence compared with conventional contrast angiography. An overall sensitivity of 93% and specificity of 78% were found. In four cases of atrial septal defect and seven of mitral regurgitation, the sensitivity was 100%. It was slightly lower for eleven cases of ventricular septal defect (91%) and four of patent ductus arteriosus (75%). When assessing aortic, tricuspid and pulmonary valve competence, the method proved to be more sensitive than conventional angiography to detect mild regurgitation. Contrast echocardiography is a sensitive and safe technique that may be used in association with conventional angiography reducing the need for radiographic contrast and ionizing radiation time.

Adolescent↗
PubMed2006

Exercise intolerance in adults with congenital heart disease.

This article describes the ways to assess exercise capacity in adults with congenital heart disease (ACHD) and the impact of exercise intolerance in the population. It also discusses the likely pathogenesis of exercise intolerance in ACHD, the similarities between ACHD and acquired heart failure, and potential therapeutic options.

Adult↗
PubMed2003

Congenital heart disease in adults: catheterization laboratory considerations.

Congenital heart defects are the most common birth defects and represent an increasing proportion of adolescent and adult patients followed by cardiologists. While many of these patients have undergone successful palliative or corrective surgery with excellent functional results, most of them still require careful follow-up. Further, even complex lesions may first be diagnosed in adolescence and adulthood. Therefore, cardiologists caring for adults need to become more familiar with these defects. Assessment of the patient with known or suspected congenital heart defects requires a careful history, physical examination, and noninvasive assessment. In addition, the catheterization laboratory remains a critical venue for diagnosis and, increasingly, therapy. Pressure measurements, oximetry, and angiography remain cornerstones of diagnosis in selected patients and a variety of interventional procedures have become viable therapeutic alternatives in both pre- and postoperative patients.

Adult↗
PubMed1987

Extracardiac malformations and congenital heart disease: frequency and patterns of associations.

Congenital Heart Malformations (CHM) can be present together with Extracardiac Malformations (ECM) in a single individual, however the frequency and the patterns of associations are not well defined because the casistics of the literature are little comparable. The diagnosis of CHM has been demonstrated with cardiac catheterization in any case. Between January 1st, 1976 and December 31st, 1983 cardiac catheterization has been performed in 1012 children and 971 of them were affected by Congenital there was at least one ECM as well (group B); 311 ECM were present in group B. VSD (p less than 0.05) and Atrioventricular Septal Defects (AV.SD) (p less than 0.01) resulted associated with ECM while T.F. (p less than 0.05) and isolated TGA (p less than 0.01) showed the tendency to occur in isolation. VDS resulted associated with gastrointestinal anomalies (p less than 0.05), AV.SD with T21 (p less than 0.001), aortic valve (p less than 0.05) and supra-valve (p less than 0.001) stenosis with Nervous System abnormalities. Pulmonary stenosis showed little tendency to occur together with T21 (p less than 0.05).

Abnormalities, Multiple↗
PubMed1965

THE ACID-BASE STATUS IN CYANOTIC CONGENITAL HEART DISEASE.

The relation between oxygen consumption, metabolic status and prognosis was studied in two infants with identically low arterial oxygen tensions (20 mm. Hg) due to cyanotic congenital heart disease. The first patient had low oxygen consumption, arterial blood acidosis and increased arterial lactate, and died at the age of 36 hours. The second had normal oxygen consumption, arterial acid-base balance, lactate and pyruvate, and survived. Since arterial oxygen tensions were similar in both, it was concluded that compensatory factors, such as cardiac output, pulmonary and systemic blood flow and increased oxygen saturation at normal pH levels (Bohr effect), are important in maintaining tissue oxidation and preventing anaerobiosis and lactate production. The importance of a knowledge of acid-base status in the immediate prognosis of cyanotic congenital heart disease is stressed. The treatment of acidosis by buffer therapy may be an important palliative, improving cardiac output and tissue oxidation, and should be undertaken as soon as possible before irreversible cellular damage has occurred.

Acid-Base Equilibrium↗
PubMed1986

Superior vena caval abnormalities: their occurrence rate, associated cardiac abnormalities and angiographic classification in a paediatric population with congenital heart disease.

Angiography of the superior systemic veins was performed on 510 patients with congenital heart disease. An analysis of these angiograms was carried out and a simple angiographic classification of superior vena caval anomalies is presented. Eleven per cent of cases had complete bilateral superior vena cavae, a frequency of occurrence higher than that previously reported. An analysis of associated congenital heart lesions revealed a higher than average association of bilateral superior vena cavae with atrioventricular septal defects and double-outlet right ventricle, but this was seen rarely in cases of transposition of the great arteries. Bilateral superior vena cavae also occurred very frequently (72%) in patients with situs abnormalities. Femoral vein catheterisation is likely to facilitate diagnosis and appropriate radiological signs are discussed.

Adolescent↗
PubMed1994

[Comparison of the ultrastructural and clinical studies on myocardium in patients with congenital heart disease].

Right ventricle myocardium of 25 cases of tetralogy of Fallot and 11 cases of ventricle septal defect were studied by both light and electron microscopy. Normal human myocardium from 4 cases were used as the control. The resulting data indicate that the mean muscle diameter correlated significantly with the course of disease, mean pressure of right ventricle and diameter of ventricle septal defect. In 6 cases with congenital heart disease, the myocardium was atrophic and presented with 2 types of ultrastructural features: accumulation of Z-line material or hyperplasia of the mitochondria. The significance of these ultrastructural findings in congenital heart disease is discussed.

Heart Septal Defects, Ventricular↗