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Elevated circulating levels of inflammatory cytokines and bacterial endotoxin in adults with congenital heart disease.

Chronic heart failure is a state of immune activation, and endotoxin is a potential trigger for cytokine production. Our aim was to study whether immune activation and endotoxemia occur in adults with congenital heart disease. We prospectively measured tumor necrosis factor (TNF)-alpha, soluble TNF receptors (sTNFR-1, sTNFR-2), interleukin-6, interleukin-10, endotoxin, and soluble CD14 levels in 52 consecutive adults with congenital heart disease (age 34 +/- 2 years [mean +/- SEM]) and 18 healthy controls (age 31 +/- 1 years). A variety of congenital heart lesions were studied: single ventricle physiology (n = 15), systemic right ventricle (n = 7), tetralogy of Fallot (n = 20), and "other" congenital heart disease (n = 10). Patients were subgrouped into asymptomatic (New York Heart Association [NYHA] class I, n = 11), mild (NYHA class II, n = 30), and moderate/severe (NYHA class III/IV, n = 11) categories. Patients had elevated TNF and interleukin-6 levels compared with controls (TNF 2.8 vs 2.1 pg/ml, p <0.05; interleukin-6 8.5 vs 5.7 pg/ml, p <0.001). TNF levels were higher in patients with moderate/severe symptoms compared with patients who were asymptomatic or had mild symptoms (p <0.05). Soluble TNFR-1 levels related directly to the degree of systemic ventricular impairment (p <0.05). There were no significant differences in sTNFR-1, sTNFR-2, interleukin-10, or sCD14 levels between patients and controls. Endotoxin levels were greater in patients with congenital heart disease versus controls (0.40 vs 0.26 endotoxin units/ml, p <0.0001). Thus, adults with congenital heart disease have elevated levels of inflammatory cytokines and bacterial endotoxin, which relate to functional status. Congenital heart disease in adults may be amenable to novel anti-inflammatory therapies in selected patients.

Adult↗

Ultrarapid opioid detoxification of two children with congenital heart disease.

The cases of two children with congenital heart disease and severe opioid dependency who underwent ultrarapid opioid detoxification are presented. This technique entails rapid opioid reversal with the opioid antagonist naloxone while under general anesthesia. The procedures were not technically difficult to perform and both children were successfully detoxified at the end of the procedure. In the weeks following the procedure, the first child exhibited accelerated neurodevelopment. Ultrarapid opioid detoxification is possible in children and may have a neurodevelopmental advantage over a prolonged wean.

Analgesics, Opioid↗

Haematological considerations in patients with cyanotic congenital heart disease: a review.

In patients with cyanotic congenital heart disease (CCHD), the need for prior antibiotic prophylaxis for infective endocarditis is well known to dentists, but not many dentists are aware of the associated haemorrhagic tendencies in such patients. Haemostatic abnormalities associated with CCHD are an important aspect that is often overlooked by both physicians and dentists. We briefly review the literature to highlight the importance of more elaborate haematological evaluation in patients with CCHD, prior to any oral surgical procedures.

Blood Coagulation Disorders↗

An unusual presentation of congenital heart disease.

We describe an unusual presentation of congenital heart disease mimicking preeclampsia in a young, gravid girl. The diagnosis of Shone's complex was confirmed by echocardiography. This complex is manifested by multiple levels of obstruction involving the left side of the heart and the systemic circulation. It is extremely rare, especially in pregnant adolescents. We briefly describe the patient's clinical history, physical examination, and treatment, as well as our clinical decisions regarding her case. The discussion focuses on the spectrum of findings in Shone's complex and the physiologic impact of therapy on this patient.

Adolescent↗

The role of the clinical specialist in an adult congenital heart disease program.

As the specialty of adult congenital heart disease (CHD) develops, there is concern regarding the training of the health professional who will be responsible for patient management. Integral to the program is the role of the clinical nurse specialist. An advanced practice role, the clinical nurse specialist provides her or his expertise to both the daily operation of the program and to the clinical management and education of patients and their families. The educational preparation and roles of a clinical specialist in adult CHD are described.

Adult↗

Current status of surgery for congenital heart diseases.

Results of the surgical treatment of congenital heart diseases have improved significantly in the recent past, with an overall hospital mortality rate of less than 4 per cent. This has been due to improved preoperative diagnosis, intraoperative management, and postoperative care. Aggressive intervention in early infancy and attempts at total repair rather than palliation in cases such as ventricular septal defect, tetralogy of Fallot, and others has optimized the long-term outcome. The introduction of new procedures has made it possible to treat patients who were considered unoperable in the past, such as tricuspid atresia, single ventricle, and hypoplastic left ventricle. Continued progress in the field of cardiac transplantation promises hope for the few remaining unoperable lesions.

Heart Defects, Congenital↗

Birth weight of children with congenital heart disease.

Birth weights of 843 children with congenital heart disease (CHD) were compared to the respective data of a normal West German population. On average, the CHD group had significantly lower birth weights, but the weight deficit was far less pronounced than in previous studies. The decrease in birth weight was distinct only in children with tetralogy of Fallot and atrial septal defect. Compared to normal newborns, patients with CHD were more often small for gestational age (15.0%) or had a low (less than 2500 g) birth weight (8.6%). The prevalence of prematurity was not increased. Extracardiac malformations were not significantly more common in CHD patients, who were small for gestational age, than in CHD patients with normal birth weight.

Birth Weight↗

[Left ventricular pressure response to acute volumetric overload in congenital heart disease (author's transl)].

Fifty patients with congenital heart disease were studied hemodynamically to evaluate their functionally dynamic situation. The peak systolic left ventricular pressure and the left ventricular endiastolic pressure were analyzed in basal conditions and following the volumetric overload of the left ventricular angiogram as parameters reflecting afterload and preload, respectively. Highly significant correlations were obtained among those parameters in all patients and taking in consideration different syndromic groups. Regression lines obtained from these correlations permitted to establish, in a given patient, a quantitative prediction of response to volumetric overload which can estimate qualitative situation of his ventricle into the Starling's curve.

Adolescent↗

Evaluation of late results of surgical treatment of congenital heart disease.

Operative mortality from surgical treatment of congenital heart disease has been steadily reduced over the past 20 years. During the same period it has become clear that a proportion of survivors have residual clinical problems. Some of these are due to imperfect repair, a number being due to factors beyond present surgical control, and some are the consequence of associated cardiovascular defects. The chief problems can be elucidated by an analysis of the results of surgical treatment of six cardiac malformations: simple pulmonary valve stenosis, coarctation of the aorta, secundum atrial septal defect, isolated ventricular septal defect, tetralogy of Fallot and transposition of the great arteries. Recognition of the sequelae of preoperative hemodynamic strain and apparently minor associated malformations is important, and it is possible to anticipate such factors. Long-term follow-up of patients after operation is particularly important.

Adolescent↗

Progression of congenital heart disease in the prenatal period.

BACKGROUND: Prenatal echocardiography has shown evidence of prenatal development of congenital heart disease. Prenatal cardiac anatomy, chamber size and function change during gestation, so that the appearance of cardiac structure in abnormal hearts may be different from that which is usually seen postnatally. METHODS: Published prenatal echocardiographic studies were reviewed and in utero development of congenital heart disease from midtrimester to the early postnatal period is discussed. RESULTS: The growth of the great vessels and ventricles is reduced in fetuses with ventricular outflow obstruction. Valve regurgitation may progress. The foramen ovale and ductus arteriosus have been reported to become restrictive in utero in several settings. Pulmonary vascular obstructive changes may progress prenatally. Fetal arrhythmia (both bradycardia and tachycardia) may develop in utero. Development of congestive heart failure is a very important issue during follow up of fetuses with significant cardiac or extracardiac problems. Some may progress to fetal hydrops and prognosis of the affected fetuses is usually very poor. CONCLUSIONS: Correct knowledge of possible development is important for accurate prenatal diagnosis. Information on prenatal progression of the cardiac anomaly is also important to make plans for follow up and perinatal management, to predict outcomes and to counsel family. Furthermore, the benefits of prenatal treatment instead of postnatal treatment should be assessed by the accurate prediction of the progression of the cardiac problem in utero. Further extensive studies using a large number of cases is required to predict progression accurately. In addition, further studies for elucidating the mechanisms of progression is important to provide better outcomes for fetuses with various congenital heart diseases.

Arrhythmias, Cardiac↗

Incidence of congenital heart disease: I. Postnatal incidence.

The incidence of congenital heart disease (CHD) in the Western industrialized world has varied from a low value of about 3 to 5 per 1000 live births to about 12 per 1000 live births. Most of the lower incidence figures were obtained before there were sufficiently well trained pediatric cardiologists and before the success of cardiac surgery put a premium on early and correct diagnosis of CHD. The advent of echocardiography with Doppler color flow measurements has made it possible to diagnose lesions that are asymptomatic, minor, and even without murmurs. Given these differences, there does not appear to have been a significant increase in the incidence of CHD over the last 20-30 years. The incidence of CHD in underdeveloped countries is not known, but the distribution of different lesions is fairly similar to those in developed countries except perhaps for fewer with aortic stenosis and coarctation of the aorta.

Child, Preschool↗