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PubMed1994

[Total cavopulmonary anastomosis in the surgical treatment of congenital heart defects with a functional single ventricle].

Total cavopulmonary connection (TCPC) allows surgical treatment of patients with complex univentricular heart disease. Between 1991 and 1993 this operation was performed in 15 cyanotic children with complex heart defects. Age range was 3.5-14 (median 8.3) years. In 5 children with increased operative risk a calibrated interatrial fenestration was created intraoperatively to allow systemic venous decompression. Three patients (20%) died. In remaining 12 patients a significant increase in arterial oxygen saturation and decrease of polycythemia was encountered. All patients remain in a good clinical condition 1-25 months after operation. Noninvasive investigation including echocardiography confirmed good function of the heart.

Adolescent↗
PubMed1989

Occurrence of congenital heart defects in siblings of patients with univentricular heart and tricuspid atresia.

Because of the rarity of univentricular heart and tricuspid atresia, no study of a large number of patients has been made to determine the empiric occurrence risks of a congenital heart defect in their siblings. A retrospective chart review was performed of 223 patients with univentricular heart and 113 with classic tricuspid atresia evaluated from 1982 to 1987. Thirty-four patients with univentricular heart and 17 with tricuspid atresia had no siblings and were excluded. Eleven of the total 388 siblings (2.8%) of the 189 patients with univentricular heart had a congenital heart defect. Of the siblings born after the index case of univentricular heart, 7 of 169 (4.1%) had a congenital heart defect. Two of the 210 siblings (1.0%) of the 96 patients with tricuspid atresia had a congenital heart defect. The patients with univentricular heart were subdivided into 4 groups: (1) double-inlet left ventricle; (2) complex univentricular heart with single or common inlet, or with a ventricle of common or right ventricular morphology; (3) complex univentricular heart with asplenia; and (4) complex univentricular heart with polysplenia. One of the 202 siblings (0.5%) of the 102 patients with double-inlet left ventricle had a congenital heart defect, compared with 7 of the 140 siblings (5.0%) of the 69 patients with complex univentricular heart, 1 of the 29 siblings (3.4%) of the 14 patients with complex univentricular heart and asplenia, and 2 of the 7 siblings (28.6%) of the 4 patients with complex univentricular heart and polysplenia.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2003

The developing heart and congenital heart defects: a make or break situation.

Congenital heart defects are common in humans, but the underlying basis for these defects is not well understood. It has been clear that abnormal heart development is at the root of these diseases, but the genes involved have remained elusive until recently. This review focuses on recent advances in our understanding of mammalian heart formation, and how some of these processes, when disrupted, lead to congenital heart defects.

Animals↗
PubMed2006

Prognostic value of plasma N-terminal pro-brain natriuretic peptide in children with congenital heart defects and open-heart surgery.

OBJECTIVE: To assess whether preoperative and postoperative plasma levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) predict postoperative outcome of open-heart surgery in children. STUDY DESIGN: A prospective observational study was conducted with 40 children with congenital heart defects who were undergoing elective open-heart surgery. Plasma levels of NT-proBNP, troponin T, lactate, C-reactive protein, and total neutrophil cell counts were measured before, during, and 1 and 3 hours after the end of cardiopulmonary bypass grafting (CPB). Outcomes were assessed by means of the kind, dosage, and duration of inotropic drug use during the postoperative period, lactate concentrations, and the duration of mechanical ventilation. RESULTS: Preoperative levels of NT-proBNP were significantly increased irrespective of the type of congenital heart defect and the age of the patient. Preoperative NT-proBNP levels were higher in patients receiving prolonged postoperative inotropic drug therapy (r = 0.56, P = .0003). By means of multivariate analysis with the duration of inotropic therapy as the dependent variable, a significant impact of preoperative NT-proBNP levels, the presence of a cyanotic heart defect, the risk adjustment for congenital heart surgery score, duration of CPB time, and postoperative lactate levels were demonstrated (R squared = 76.8%, P <.0001). CONCLUSION: Preoperative NT-proBNP levels were associated with complicated postoperative outcome in children who underwent low-risk open-heart surgery. This marker may therefore be a useful tool in risk stratification of patients with congenital heart defects.

Biomarkers↗
PubMedDate not supplied

Surgical management of congenital heart defects: current trends.

Surgical treatment for congenital heart disease has become available over the last five decades. Palliative procedures have been designed to improve physiologic abnormalities, for example systemic artery (or venous) to pulmonary artery shunts of various types to increase the pulmonary blood flow, pulmonary artery constriction (banding) to decrease the pulmonary blood flow, and surgical or transcatheter atrial septostomy to augment intracardiac mixing. These can be performed with a low mortality. The majority of congenital heart defects can be corrected by open heart surgical techniques; some require prior palliation and others can be operated without prior palliative surgery. Recent surgical advances include early total surgical correction for tetralogy of Fallot, arterial switch procedure for transposition of the great arteries, Fontan operation and its modifications for tricuspid atresia and single ventricle, new operations for hypoplastic left heart syndrome, newer prosthetic valves, myocardial preservation and cardiac transplantation.

Cardiac Surgical Procedures↗
PubMed1987

Abnormal endothelial factor VIII associated with pulmonary hypertension and congenital heart defects.

In patients with pulmonary hypertension associated with congenital heart defects, ultrastructural abnormalities are observed in endothelial cells, which suggest heightened metabolic function. If endothelial production of the von Willebrand factor (vWF) is increased, this may be associated with abnormal interactions with platelets leading to worsening of the pulmonary hypertension. We therefore evaluated vWF in 30 patients with pulmonary hypertension (25 with congenital heart defects) and in 30 individuals with normal pulmonary arterial pressure (12 with congenital heart defects). We measured the antigenic (vWF: Ag) and biologic (VWF: rist) activity of vWF in plasma and assessed endothelial vWF: Ag directly by an immunoperoxidase stain applied to lung biopsy tissue. Because of considerable variance and small size, the group of five patients with pulmonary hypertension and without congenital heart defects were excluded from statistical analyses. Patients with pulmonary hypertension and congenital heart defects had significant higher vWF: Ag levels than individuals with normal pulmonary arterial pressure without congenital heart defects (p less than .05), whereas values in those with normal pressure and congenital heart defects were intermediate. In lung biopsy tissue available from 29 patients in this study and from 11 others we previously reported, immunostain of pulmonary arterial endothelium for vWF was intense (suggesting increased production) in 29 of 32 with pulmonary hypertension and congenital heart defects and in only one of eight with normal pulmonary arterial pressure and congenital heart defects (p less than .01). Only three patients with congenital heart defects and pulmonary hypertension and increased vWF: Ag, however, had increased vWF: rist. Compatible with this discrepancy was a loss of vWF high-molecular weight forms as determined by both crossed immunoelectrophoresis and multimeric analysis. Our results suggest that increased vWF in most patients with congenital heart defects and pulmonary hypertension is associated with increased production of a biologically deficient molecule lacking high-molecular weight forms.

Adolescent↗
PubMedDate not supplied

Geographical distribution of congenital heart defects in Saudi Arabia.

BACKGROUND: Congenital heart defects (CHD), which are caused by abnormalities early in fetal life, encompass over 50 diagnoses. Since the detailed etiology is unknown, the geographical distribution of defects might suggest likely risk factors. METHODS: The geographical distribution of 5 865 Saudi Arabian nationals with CHD was studied by cross-matching their residential provinces and towns with a geographical information system provided by the General Directorate for Military Survey. Population data were obtained from the 1413H census. RESULTS: CHD cases were mostly distributed across the provinces in proportion to their total population but due to their size and inhomogeneity, province-based thematic maps were found to be misleading. City-based maps were preferable and showed similar geographic distributions for cases registered in successive years. Thematic maps of the distribution of the CHD burden highlighted the southwestern provinces, nearthe border with Yemen, and the northeast section of the Eastern Province. CONCLUSIONS: Patterns of disease in Saudi Arabia are best studied at the level of individual towns and villages. The CHD registry has already attained good national coverage and can therefore support nationwide epidemiological studies. Southwestern Saudi Arabia and the northern part of the Eastern Province appear to exhibit a higher burden of CHD.

Heart Defects, Congenital↗
PubMed1975

[Oxygen transport in the blood of children with congenital heart defects].

In 55 children with congenital heart defects (aged 6 months and older), hematocrit, hemoglobin concentration, acid-base status, oxygen pressure, oxygen saturation, and 2,3-DPG content of erythrocytes were determined in blood taken from the vena cava superior. The data were correlated with each other as well as with parameters obtained during cardiac catheterization. Whereas an increased hematocrit was seen only at a difference in arteriovenous oxygen saturation of more than 30%, or a central venous saturation below 65%, a close correlation between 2,3-DPG concentration in erythrocytes and these parameters was obtained already in the normal range (r = 0.79, and r = 0.78, respectively). A relatively close correlation was also found between 2,3-DPG concentration and half-maximal oxygen saturation pressure (T50 value; r equals 0.73), and the T50 values and the central venous oxygen saturation (r equals 0.61), respectively. Because of the shift to the right of the O2-binding curve the functional oxygen transport capacity was increased by an average 15% in children with acyanotic heart defects (mean T50 value of 31.6 mm Hg), and by 40% in children with cyanotic heart defects (mean T50 value of 34.6 mm Hg), as compared to healthy children (mean T50 value of 28.1 mm Hg). A marked influence on the position of the O2-binding curve and hence on oxygen release of the blood in the periphery is therefore exerted by the 2,3-DPG concentration, whose effect was about twice that of the Bohr effect in the pH range measured.

Biological Transport↗
PubMed2003

Temporal variability in birth prevalence of congenital heart defects as recorded by a general birth defects registry.

OBJECTIVE: Our aim was to examine the temporal variability in congenital heart defect (CHD) birth prevalence from 1980 to 2000 in Emilia-Romagna, Italy. METHODS: The study population consisted of all infants, surveyed by the Emilia-Romagna birth defects registry (Indagine Malformazioni conpenite in Emilia-Romagna [IMER]), who were affected by CHDs. A simplified classification into "simple" and "complex " CHD was adopted. A comparison with another epidemiologic study using different methodology in the same area was performed. RESULTS: From 1980 to 2000, IMER ascertained 2442 live births with CHD of 480,793 infants born, with an average CHD birth prevalence of 5.1% (Range, 3.1% to 7.5%). A significant increase in prevalence of simple CHD during the second decade of the study was demonstrated because of an increased recognition of "minor" cardiac lesions among the simple CHD. The birth prevalence of complex CHD remained stable. CONCLUSIONS: The apparent increase in live births with CHD results mainly from the current widespread availability of color Doppler echocardiography, which allows the early detection of the "minor" cardiac defects. Other differences are the result of the sources of ascertainment, diagnostic criteria, system of classification, and especially the age limit for enrolling infants with suspected CHD.

Female↗