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PubMed1986

Renal glomerular size in infants with congenital heart disease and in cases of sudden infant death syndrome.

Recurrent apnoea and chronic hypoventilation have been implicated in the pathogenesis of the sudden infant death syndrome (SIDS) and markers of chronic hypoxaemia have been reported in such infants at post mortem examination. Markers of chronic hypoxaemia are common in cyanotic congenital heart disease. Glomerular enlargement in congenital heart disease is said to be related to hypoxaemia although the precise mechanism whereby this occurs is not clear. We have established a normal range of glomerular size for the postperinatal period and confirmed glomerular enlargement to be a common finding in children with congenital heart disease of similar age. In contrast glomerular size in SIDS is not different from controls. The results question the role of significant chronic hypoxaemia being involved in these deaths.

Autopsy↗
PubMed1993

Implantation and intermediate-term follow-up of stents in congenital heart disease.

BACKGROUND: Balloon-expandable stents (Johnson and Johnson Interventional Systems) have been in use for congenital heart disease since late 1989. They have made possible treatment in previously untreatable branch pulmonary artery stenoses and systemic venous stenosis. The purpose of this report is to detail the results and intermediate-term follow-up of stents used for treatment of congenital heart disease. METHODS AND RESULTS: Eighty-five patients underwent placement of 121 stents in Houston and Boston. Fifty-eight patients had stents put in pulmonary arteries, nine had stents in conduits or outflow tracts, and 21 had stents in venous stenoses or narrowed Fontan anastomoses. (Three patients had stents in two locations.) These stent procedures resulted in gradient reduction from 55.2 +/- 33.3 to 14.2 +/- 13.5 mm Hg in pulmonary arteries, from 41.4 +/- 26.0 to 20.7 +/- 17.0 mm Hg in conduits or outflow tracts, and from 9.8 +/- 6.9 to 2.4 +/- 3.1 mm Hg in venous stenoses or Fontan anastomoses. Diameter of narrowings increased from 4.6 +/- 2.3 to 11.3 +/- 3.2 mm in the pulmonary artery, from 8.8 +/- 3.6 to 12.7 +/- 2.6 in conduits, and from 3.8 +/- 2.9 to 11.3 +/- 2.8 in venous stenoses. Follow-up has shown stent fracture in one patient, restenosis in one, and sudden death in one. Recatheterization has been done in 38 patients an average of 8.6 months after stent installation. Compared with immediately postimplant data, there was no significant change in luminal diameter or pressure gradient. Redilation was performed in 14 patients (17 stents) 1 week to 24 months after implantation (mean, 10.2 months), with a small but significant increase in stenosis diameter. CONCLUSIONS: We conclude that stent treatment of vascular stenoses in congenital heart disease retains efficacy at medium-term follow-up and offers a much-improved outlook for patients with these lesions.

Adolescent↗
PubMed2001

[Congenital heart disease in adults. Part I. Non-cyanotic heart diseases].

Over the last decades major advances have been made in the diagnosis and treatment of congenital heart disease. Therefore, many children with simple and complex congenital heart defects can now survive to adulthood and represent an expanding population. Most of them are not "cured" and need specialized follow-up. This two-part review presents the more common acyanotic and cyanotic congenital heart diseases that adult cardiologists are likely to face in their daily practice and discusses recent treatment advances.

Adult↗
PubMed1976

Congenital extracardial malformations accompanying congenital heart disease.

A statistical review of 1,354 autopsies performed in cases of congenital heart disease in three major Italian hospitals was carried out. In 446 cases one or more extracardial malformations were found; those most commonly encountered were malformations of the urinary tract, anomalies of the respiratory tract, malformations of the intestine and spleen and trisomy 21. Ventricular septal defects and persistent common atrioventricular canals occurred in a higher percentage of cases with extracardial malformations than in those where congenital heart defects were the only malformations. The reverse was true of endocardial fibroelastosis and transposition of the great vessels. Ventricular septal defects are particularly common in the Klippel-Feil syndrome, in trisomy 18 and, together with a persistent common atrioventricular canal and atrial septal defects, in trisomy 21. The typical picture of so-called "congenital heart disease associated with asplenia" was found in cases with splenic agenesis, either with or without situs inversus viscerum, but also in cases with situs inversus viscerum without asplenia.

Abnormalities, Multiple↗
PubMed1990

Echo-Doppler and color-flow imaging in congenital heart disease.

Enormous strides in diagnosis and management have allowed many patients with congenital heart disease to reach adulthood. Proper care of these individuals requires knowledge of the anatomic and hemodynamic faults of the original defect, the dynamic changes that occur with time, and the effects of adult diseases or surgical intervention on that physiology. Echocardiographic and Doppler assessment of these patients has revolutionized their management. This article has discussed individual abnormalities such as shunts, obstructive or regurgitant lesions, and great-vessel and chamber malpositions, as well as selective aspects of more complex malformations. In the patient with congenital heart disease, whether unoperated or postoperative, a complete echo-Doppler examination includes complete anatomic delineation of intra- and extracardiac structures using combined two-dimensional and color-flow imaging, assessment of chamber sizes and myocardial function, and complementary pulsed and continuous-wave Doppler and color-flow imaging to search for and quantitate valvular, subvalvular or supravalvular stenosis or regurgitation; extra- and intracardiac conduit stenosis; pulmonary and systemic venous obstruction; transseptal or patch shunts; and intracardiac hemodynamics.

Adolescent↗
PubMed2006

Arrhythmias in adult congenital heart disease.

Recent advances in pediatric cardiology and cardiac surgery have allowed a rapidly expanding population of patients with congenital heart disease to thrive well into their adult years. Often after prolonged uneventful clinical courses as children and adolescents, arrhythmias may surface later in life. These arrhythmias are a major source of morbidity. In addition, sudden death is the leading cause of mortality in adult patients with congenital heart disease. This review highlights the various types of brady- and tachyarrhythmias encountered in the more common forms of adult congenital heart disease and explores prognostic implications and therapeutic options.

Adult↗
PubMed2004

[Interventional therapy for complex congenital heart disease].

OBJECTIVE: The advances in interventional cardiac catheterization have changed the therapeutic strategy for many patients with congenital heart diseases. The aim of this study was to evaluate the role of therapeutic cardiac catheterization in the treatment of complex congenital heart diseases. METHODS: Balloon atrial septostomy (BAS) was performed in 59 children using Rashkind balloon catheter. Static balloon dilatation of the atrial septum was performed in 2 children with hypoplastic right heart syndrome. One child with pulmonary artery stenosis at the suture lines after arterial switch was treated with balloon dilatation. Percutaneous balloon pulmonary valvuloplasty (PBPV) and balloon angioplasty were performed in 15 children with tetralogy of Fallot (TOF). Transcatheter coil embolization was performed in 18 children with systemic to pulmonary collateral vessels and 5 children with B-T shunts before surgical procedures. Transcatheter closure of fenestration with Amplatzer septal occluder device was performed in 1 child who had undergone Fontan procedure. RESULTS: In 46 children with transposition of great arteries (TGA), the arterial oxygen saturation (SaO(2)) was increased from 0.57 +/- 0.17 to 0.76 +/- 0.13 (t = 14.58, P < 0.01) after BAS. The pressure gradients across left and right atrium were less than 2 mmHg. The created atrial septal defects were 5 - 20 mm in size. In 10 children with pulmonary atresia with intact ventricular septum (PA/IVS), the arterial oxygen saturation did not change after BAS (t = 1.57, P > 0.05), but the pressure gradients across left and right atrium were less than 2 mmHg. In children with TOF, the arterial oxygen saturation was increased by 15 percent after PBPV and pulmonary valvular stenosis was relieved. In 14 of 18 children with systemic to pulmonary collateral vessels and 5 children with B-T shunts, complete occlusion was accomplished and the procedures were successful. In the child who had undergone Fontan procedure, the fenestration was occluded successfully and no complication was observed. CONCLUSION: In management of complex congenital heart diseases, combination of surgical procedure and interventional catheterization therapy could be suggested to have better outcome.

Cardiac Catheterization↗
PubMed2001

Congenital heart disease in Papua New Guinean children.

The aim of the study was to analyse critically the programme for surgical management of children in Papua New Guinea (PNG) with congenital heart disease. A hospital record-based analysis was undertaken to document the pattern, management and short-term outcome of surgery in PNG children referred with a diagnosis of congenital heart disease to the Royal Alexandra Hospital for Children in Sydney, Australia. On admission, physical examination, chest radiogram, electrocardiogram, cross-sectional echocardiogram and, in most cases, cardiac catheterization were performed. Of the 170 children referred over the 17-year period, 1978-1994, 165 were confirmed to have congenital heart disease and were included in the study. Their ages ranged from 2 months to 16 years (median 5.5) and the male to female ratio was 1:1. One-sixth had delayed milestones and one-fifth long-term wasting. A large number were tachypnoeic, in heart failure or had pulmonary hypertension on admission. Ventricular septal defect, 34%, tetralogy of Fallot, 23%, and patent ductus arteriosus, 16.4%, were the predominant defects. lesions such as aortic stenosis, coarctation of the aorta and transposition of the great arteries are under-represented. Altogether, 133 children (81%) had surgery; 75% were open- and 25% closed-heart operations. The complications were unremarkable and the mortality rate (6%) acceptable for the era. The programme was therefore very successful for a small proportion of children born in PNG with congenital heart disease.

Adolescent↗
PubMed2000

Physical activity in children and adolescents with congenital heart disease. Aspects of measurements with an activity monitor.

The aim of the present study was to evaluate different aspects of a device designed to monitor physical activity. Measurements of different axes and placement of the monitor were tested using a treadmill with ranging increments in incline or speed. The monitor was also used to assess the level of physical activity among children and adolescents with congenital heart disease and in healthy controls at the same age. The results indicate that the monitor is a valid and reproducible instrument for measurements of physical activity. The study revealed that the level of activity was higher for healthy boys than healthy girls (p<0.0001). Boys with congenital heart disease also displayed higher values compared to girls with congenital heart disease, although the difference was not significant (p=0.067). Healthy boys revealed a significantly higher level of activity than did boys with congenital heart disease (p=0.003), but no such difference was found in girls (p=0.757). Nor were any differences found between younger and older individuals among patients with congenital heart disease. Young healthy controls, however, showed significantly higher levels of activity than their older counterparts. There were differences in activity monitored during the week, with lower activity in the weekends, but the activity on the same day in different weeks seemed stable. Neither were there any differences between measurements over whole weeks. The results indicate that the Computer Science & Application monitor is a valid instrument for assessing physical activity. The monitor may also be used, therefore, to validate the levels of physical activity level in children with congenital heart disease after medical and surgical treatment.

Adolescent↗
PubMed2004

[Expression of the heat shock protein-70 in the myocardial cells of cyanosis congenital heart diseases].

OBJECTIVE: To evaluate the expression and myocardial protection of heat shock protein-70 (HSP70) in the myocardial cells of cyanosis congenital heart diseases. METHODS: The study enrolled 24 patients with congenital heart disease: 12 cases of non-cyanosis (group A), and the other 12 cases of cyanosis (group B). During the operation the samples of myocardium in the right atrium were attained at 0 min ischemia, 20 min ischemia, and 20 min reperfusion. They were used to evaluate the content of HSP70 by immunohistochemistry and activation of SOD and content of MDA by chemochromatometry. RESULTS: The content of HSP70 in myocardial cells during the operation was not significantly different in both groups (P > 0. 05). The content of HSP70 significantly decreased in group B compared with that of group A (P <0.05). The activity of SOD significantly decreased and the content of MDA significantly increased in group B during the operation (P < 0.05). CONCLUSION: Decreased expression of HSP70 is associated with the decreased tolerance to ischemia-reperfusion injury in myocardial cells of cyanosis congenital heart disease.

Double Outlet Right Ventricle↗
PubMed1996

Total repair of congenital heart disease in the very young.

Total repair of congenital heart disease in the very young is nearly always feasible when two balanced ventricles are present. Refinements in antenatal and fetal cardiology, and progress in pediatric cardiac surgery have improved both the surgical results and the long-term outcome of these critically ill infants.

Anastomosis, Surgical↗
PubMed1996

Ablation of 'incisional' reentrant atrial tachycardia complicating surgery for congenital heart disease. Use of entrainment to define a critical isthmus of conduction.

BACKGROUND: Intra-atrial reentrant tachycardia occurs frequently after surgery for congenital heart disease and is difficult to treat. We tested the hypotheses that intra-atrial reentrant tachycardia in patients who had undergone prior reparative surgery for congenital heart disease could be successfully ablated by targeting a protected isthmus of conduction bounded by natural and surgically created barriers and that entrainment techniques could be used to identify these zones. METHODS AND RESULTS: Eighteen consecutive patients with 26 intra-atrial reentrant tachycardias complicating surgery for congenital heart disease (9 atrial septal defect repair, 4 Fontan, 2 Mustard, 2 Senning, and 1 Rastelli procedure) underwent electrophysiological study and ablation attempts. Mapping of activation was facilitated by the deployment of catheters with multiple electrodes. Sites for ablation were sought that demonstrated entrainment with concealed fusion and at which the postpacing interval minus the tachycardia cycle length and the stimulus to P wave minus the activation time were < 30 ms. These sites were considered to be within a narrow isthmus critical to the tachycardia mechanism. Anatomic barriers bordering the critical isthmus of conduction were identified on anatomic grounds, by the presence of areas of electrical silence or by the demonstration of split potentials signifying a line of block. Success was achieved in 15 patients with 21 arrhythmias. The median number of radiofrequency applications was 5. There was a wide range of activation times at successful sites (-30 to -250 ms). At a mean duration of follow-up of 17 +/- 8 months, 11 patients were asymptomatic and 9 did not require antiarrhythmia therapy. CONCLUSIONS: Successful ablation of intra-atrial reentrant tachycardia complicating surgery for congenital heart disease may be achieved by creation of an ablative lesion in a critical isthmus of conduction bounded by anatomic barriers. This isthmus may be identified by the presence of entrainment with concealed fusion and an analysis of the relationship between the postpacing interval and the tachycardia cycle length and between the activation time and the stimulus time. Because this isthmus is invariably confined on at least one aspect by a surgical repair site that is of central importance to the tachycardia mechanism, we suggest that this type of arrhythmia be given the descriptive designation of "incisional reentry."

Adolescent↗
PubMed1994

Platelet-leukocyte activation and modulation of adhesion receptors in pediatric patients with congenital heart disease undergoing cardiopulmonary bypass.

Cardiopulmonary bypass has been shown in adults to activate platelets and leukocytes, lead to the formation of circulating platelet-leukocyte conjugates, and alter adhesive receptors on both cell types. Pediatric patients with congenital heart disease undergoing cardiopulmonary bypass, however, have not been extensively studied and may represent a group at particular clinical risk for bleeding and pulmonary dysfunction. We studied 13 patients with congenital heart disease undergoing operations necessitating bypass, 7 with cyanotic and 6 with noncyanotic congenital heart disease. We determined that (1) the surface density of platelet glycoprotein Ib was significantly lower at baseline and throughout bypass in patients with cyanotic heart disease than in noncyanotic patients; (2) platelet glycoprotein Ib in both cyanotic and noncyanotic congenital heart disease decreased significantly during bypass, with a nadir of 75% of baseline values; (3) platelets were activated to a high degree, comparable with that seen in adults; (4) mean circulating monocyte-platelet conjugates rose significantly during bypass, increasing from 36% to 66% by the end of bypass, whereas neutrophil-platelet conjugates and lymphocyte-platelet conjugates declined; and (5) both monocytes and neutrophils were activated by cardiopulmonary bypass, as assessed by increased surface expression of CD11b and, in the case of monocytes, CD11b expression continued to increase even after termination of bypass. Patients with cyanotic and noncyanotic heart disease did not differ with respect to platelet or leukocyte activation or the formation of platelet-leukocyte conjugates. We conclude that in children with congenital heart disease cardiopulmonary bypass causes loss of platelet adhesion receptors, activation of platelets, formation of platelet-leukocyte conjugates, and leukocyte activation. Cyanotic and noncyanotic patients are qualitatively similarly affected; however, cyanotic patients demonstrate a baseline deficit in the platelet adhesion receptor glycoprotein Ib. These cellular changes may contribute to both the hemostatic and inflammatory complications associated with cardiopulmonary bypass.

Blood Platelets↗
PubMed2000

3D-shaded surface rendering of gadolinium-enhanced MR angiography in congenital heart disease.

BACKGROUND: Gadolinium-enhanced three-dimensional (3D) MR angiography is a useful imaging technique for patients with congenital heart disease. OBJECTIVE: This study sought to determine the added value of creating 3D shaded surface displays compared to standard maximal intensity projection (MIP) and multiplanar reformatting (MPR) techniques when analyzing 3D MR angiography data. MATERIALS AND METHODS: Seventeen patients (range, 3 months to 51 years old) with a variety of congenital cardiovascular defects underwent gadolinium-enhanced 3D MR angiography of the thorax. Color-coded 3D shaded surface models were rendered from the image data using manual segmentation and computer-based algorithms. Models could be rotated, translocated, or zoomed interactively by the viewer. Information available from the 3D models was compared to analysis based on viewing standard MIP/ MPR displays. RESULTS: Median postprocessing time for the 3D models was 6 h (range, 3-25 h) compared to approximately 20 min for MIP/MPR viewing. No additional diagnostic information was gained from 3D model analysis. All major findings with MIP/MPR postprocessing were also apparent on the 3D models. Qualitatively, the 3D models were more easily interpreted and enabled adjacent vessels to be distinguished more readily. CONCLUSION: Routine use of 3D shaded surface reconstructions for visualization of contrast enhanced MR angiography in congenital heart disease cannot be recommended. 3D surface rendering may be more useful for presenting complex anatomy to an audience unfamiliar with congenital heart disease and as an educational tool.

Adolescent↗
PubMed2006

Time-resolved contrast-enhanced MR angiography of the thorax in adults with congenital heart disease.

OBJECTIVE: The aim of this study was to evaluate the diagnostic value of time-resolved contrast-enhanced MR angiography in adults with congenital heart disease. SUBJECTS AND METHODS: Twenty patients with congenital heart disease (mean age, 38 +/- 14 years; range, 16-73 years) underwent contrast-enhanced turbo fast low-angle shot MR angiography. Thirty consecutive coronal 3D slabs with a frame rate of 1-second duration were acquired. The mask defined as the first data set was subtracted from subsequent images. Image quality was evaluated using a 5-point scale (from 1, not assessable, to 5, excellent image quality). Twelve diagnostic parameters yielded 1 point each in case of correct diagnosis (binary analysis into normal or abnormal) and were summarized into three categories: anatomy of the main thoracic vessels (maximum, 5 points), sequential cardiac anatomy (maximum, 5 points), and shunt detection (maximum, 2 points). The results were compared with a combined clinical reference comprising medical or surgical reports and other imaging studies. Diagnostic accuracies were calculated for each of the parameters as well as for the three categories. RESULTS: The mean image quality was 3.7 +/- 1.0. Using a binary approach, 220 (92%) of the 240 single diagnostic parameters could be analyzed. The percentage of maximum diagnostic points, the sensitivity, the specificity, and the positive and the negative predictive values were all 100% for the anatomy of the main thoracic vessels; 97%, 87%, 100%, 100%, and 96% for sequential cardiac anatomy; and 93%, 93%, 92%, 88%, and 96% for shunt detection. CONCLUSION: Time-resolved contrast-enhanced MR angiography provides, in one breath-hold, anatomic and qualitative functional information in adult patients with congenital heart disease. The high diagnostic accuracy allows the investigator to tailor subsequent specific MR sequences within the same session.

Adolescent↗
PubMedDate not supplied

Use of platinum microcoils to embolize vascular abnormalities in children with congenital heart disease.

Many vascular anomalies found in children with congenital heart disease can be embolized with catheter-delivered steel coils. Platinum microcoils delivered through a microcatheter broaden the spectrum of lesions amenable to transcatheter closure. Platinum microcoil delivery technique is described, and representative cases demonstrating microcoil utility and benefits are presented.

Arterio-Arterial Fistula↗
PubMed2000

A study of children's concepts of their internal bodies: a comparison of children with and without congenital heart disease.

This study examined the following: (1) the knowledge children with and without congenital heart disease have about their internal bodies, (2) the relationship of this knowledge with age, disease severity, and previous health care experiences, and (3) how the differences between beliefs about the interior body and the value assigned to specific body parts differed between children with congenital heart disease and their unaffected peers. One hundred participants, 50 with cardiac disease and 50 comparisons, between 5 and 15 years of age, completed the inside the Body Test; the severity of the conditions of the children with cardiac problems was evaluated with the Permanent Impairment Evaluation Guide. Results indicated that there were no differences between the 2 groups on knowledge of body parts, body-part function, nor the values that were assigned. Implications for practice are discussed.

Adolescent↗
PubMed1993

Psychosocial concerns of adults with congenital heart disease. Employability and insurability.

Addressing the psychosocial concerns of the growing population of adults with congenital heart disease presents a unique challenge to health care providers. These patients frequently need information about their disease with emphasis on how it may affect their sexuality, childbearing, and risk of having children with congenital heart disease. These patients also face problems in securing employment that will provide health insurance coverage for their cardiac conditions.

Employment↗