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PubMed2005

[The best of congenital heart disease in 2004].

The year 2004 was notable, as were the previous years, for advances in diagnosis, treatment and follow-up of certain congenital cardiac diseases with studies carried out in the foetus, the child and the adult. Foetal cardiology is in constant development: the malformations are better detected, left ventricular function is evaluated by echocardiography, and, consequently, foetal cardiac failure can be treated. Interventional procedures have started in the foetus with results that require confirmation. In children and adults, publications of interventional catheterisation are as numerous as before: percutaneous closure of atrial septal defects, closure of patent foramen ovale in cases of transient ischaemic cerebral attacks, closure of congenital or acquired (iatrogenic, post-infarct or traumatic) ventricular septal defects, percutaneous valve replacement, maintenance of patent ductus arteriosus. Many other interventional procedures have been carried out in operated and unoperated congenital heart lesions: angioplasty, embolisation, valvular stenosis. Adult congenital heart disease may pose problems of arrhythmia, during pregnancy for example. Cardiac resynchronisation, which is well developed in adults with cardiac failure, also has indications in congenital heart disease of children and adults. Pregnancy is possible in women with tetralogy of Fallot, operated or not, with a maternal risk of left ventricular dysfunction and progression of pulmonary regurgitation. After a Mustard procedure, pregnancy may aggravate symptoms and NYHA Class but it is usually well tolerated. Finally, let's cite one publication which showed, if it was still necessary, that maternal and foetal risk in pregnancy is high in patients with an Eisenmenger syndrome.

Cardiac Catheterization↗
PubMed1981

Incidence of congenital heart disease among children in a selected population sample.

The incidence of congenital heart disease [CHD] among infants and children has been studied since 1976. The sample represents more than one third of all infants born annually during the years under review. In 1976, 354 of all newborns had a CHD, confirmed by autopsy in 105, as compared with 285 newborns with CHD in 1977, confirmed by autopsy in 62, the incidence in 1976 and 1977 thus being 9.48% and 7.67%, respectively. The most common diseases were ventricular septal defect (38.7% and 39.65%), atrial septal defect (9.04% and 7.72%) and left-to-right shunt (8.47 and 11.23%). The incidence of the remaining CHD was less than 5%. During the two years under review and unspecified heart diseases represented 12.15% (1976) and 7.72% (1977). Mortality within the first month of birth in 1976 and 1977 was 62.8% and 51.6%, respectively. The difference shows that the incidence of CHD in 1976 was higher than in 1977.

Age Factors↗
PubMed1995

Video-assisted thoracoscopic surgery for congenital heart disease.

Video-assisted endoscopic techniques have reduced operative trauma in adult thoracic and general surgery, but applications in children with congenital heart disease have been limited. We report the development of video-assisted thoracic surgery procedures for neonates and infants with cardiovascular disease. Endoscopic instruments and techniques for pediatric cardiovascular procedures were designed and tested in the animal laboratory. Forty-eight operations were subsequently performed in 46 pediatric patients ranging in age from 2 hours to 14 years (median 9 months), weighing from 575 grams to 54 kg (median 8.5 kg). Clinical applications included seven different surgical procedures: patent ductus arteriosus interruption in infants (n = 26) and premature neonates (n = 5), vascular ring division (n = 8), pericardial drainage and resection (n = 3), arterial and venous collateral interruption (n = 2), thoracic duct ligation (n = 2), epicardial pacemaker lead insertion (n = 1), and diagnostic thoracoscopy (n = 1). There was no operative mortality. Technical success, defined as a video-assisted procedure completed without incising chest wall muscle or spreading the ribs, was achieved in 39 of 48 procedures (82%), with thoracotomy required to complete nine procedures. Most patients (22/25, 88%) undergoing elective ductus ligation were extubated in the operating room and discharged from the hospital within 48 hours of the operation. Eight of the last 10 patients having ductus ligation were discharged on the first postoperative day. Residual ductal flow was assessed by (1) transesophageal echocardiography in the operating room (incidence: 0/25, 0%, 70% CL 0% to 7.3%); (2) discharge auscultation (incidence: 1/30, 3%, 70% CL 0.5% to 10.8%); and (3) follow-up Doppler echocardiography (incidence: 3/25, 12%, 70% CL 5.4% to 22.6%). Video-assisted thoracoscopic techniques can be safely applied to pediatric patients with patent ductus arteriosus and vascular rings and may become an effective addition to the staged management of more complex forms of congenital heart disease.

Adolescent↗
PubMed1998

Adenosine induced transient cardiac standstill in catheter interventional procedures for congenital heart disease.

OBJECTIVE: To describe the use of intravenous adenosine to create transient cardiac standstill during balloon dilatation procedures for congenital heart defects. SETTING: A tertiary paediatric cardiac centre. DESIGN AND PATIENTS: This was a prospective pilot study. Thirteen patients born with congenital heart disease and who had stenotic lesions requiring relief were considered for the technique. All were suitable for balloon dilatation. Their ages ranged from 2 months to 30 years, mean (SD) 9.9 (9.8) years. The dose of adenosine varied from 0.125 mg/kg to 0.555 mg/kg, mean 0.33 (0.127). RESULTS: Two patients only developed sinus bradycardia in response to adenosine, which may have been related to the technique of administration. The other 11 experienced a period of asystole, which ranged from 2.4 to 10.8 seconds, mean 4.99 (2.27), and a total atrioventricular block period of 5.0 to 21.2 seconds, mean 9.47 (4.64). The interval between adenosine injection and the onset of asystole varied from 2.4 to 15.8 seconds, mean 8.05 (3.6), depending on cannula size, site of administration, and cardiac output. The peak gradient across the stenotic lesions fell from 52.3 (23.7) to 17.8 (11.9) mm Hg (p < 0.001). Apart from one short episode of atrial fibrillation there were no complications. CONCLUSIONS: Intravenous adenosine is a safe and effective agent for creating transient cardiac standstill during balloon dilatation procedures for congenital heart disease. This achieves stability which is likely to improve results and reduce complications. It may have applications in other fields of cardiac intervention where an immobile heart is desirable during the critical phase of a procedure.

Adenosine↗
PubMed2004

Individual quality of life in adults with congenital heart disease: a paradigm shift.

AIMS: During the last decade, a paradigm shift has emerged in the measurement of quality of life, from the use of standard questionnaires towards a more individualized approach. Therefore, this study examined individual quality of life in adults with congenital heart disease and explored potential differences with those reported by matched, healthy control subjects. METHODS AND RESULTS: We examined 579 adults with congenital heart disease. A subsample of 514 of these patients was matched for age, gender, educational level, and employment status with 446 healthy counterparts. Individual quality of life was assessed using the Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW). Twelve domains affecting patients' quality of life were identified. Family, job/education, friends, health, and leisure time were the most prominent quality of life domains. Significantly fewer patients than control subjects considered financial means and material well-being and future to be important determinants of quality of life. CONCLUSION: Assessment of quality of life in adults with congenital heart disease that focusses on the individual is appropriate for obtaining in-depth information on issues relevant for patients' quality of life. This represents a paradigm shift in the measurement of this concept.

Adolescent↗
PubMed1991

Congenital heart disease and fetal thoracoabdominal anomalies: associations in utero and the importance of cytogenetic analysis.

We examined the frequency with which congenital heart disease (CHD) and cytogenetic abnormalities were found associated with omphalocele, gastroschisis, duodenal atresia and posterior diaphragmatic hernias. We performed fetal echocardiograms on 80 patients with these diagnoses and found congenital heart disease in 13 of 37 with omphalocele (35%), 2 of 17 with gastroschisis (12%), 4 of 15 with duodenal atresia (27%), and 2 of 11 with posterior diaphragmatic hernia (18%). Karyotypes were obtained in 74 and were abnormal in 24 (32%). Although most fetuses with these extracardiac malformations and abnormal karyotypes had associated CHD, many did not. Normal karyotypes were found in 69% of fetuses with CHD and omphalocele, and 50% of fetuses with CHD and duodenal atresia. We conclude that CHD may be present in fetuses with extracardiac malformations whether or not the karyotype is normal and that the prenatal evaluation of fetuses with these lesions should include both karyotype and fetal echocardiography. Although karyotypes play an important role in prenatal diagnosis, they are not predictive of normal cardiac structure when normal in the abnormalities studied. Even when the karyotype is normal in the presence of these abnormalities, fetal echocardiography is indicated.

Abdominal Muscles↗
PubMed1996

Inhaled nitric oxide in the management of congenital heart disease.

Inhaled nitric oxide, with a threshold of perhaps only a few parts per million, is a selective pulmonary vasodilator in patients with congenital heart disease and increased pulmonary vascular resistance. Multiple reports suggest that it may be useful in managing postoperative pulmonary hypertension in the cardiac patient, although it is unknown to what extent inhaled nitric oxide can actually reduce morbidity and mortality in this setting. This agent also holds promise for evaluating patients with pulmonary hypertension prior to heart transplantation. Although special care is needed to avoid toxicity related to excess inhaled nitric oxide or nitric dioxide or increased methemoglobin, the risk of complications with inhaled nitric oxide therapy appears to be very low. Inhaled nitric oxide will likely continue to play a significant role in the pre-and postoperative management of patients with congenital heart disease.

Heart Defects, Congenital↗
PubMed2006

[Consultation of congenital heart diseases in pediatric cardiology in Mauritania].

OBJECTIVES: The aim of this study is to describe the epidemiological profile of congenital heart diseases in Mauritanian children. MATERIAL AND METHODS: Our population was composed of children referred by the different cardiologists of the country on the occasion of two missions of pediatric cardiologists in December 2001 and November 2002. All patients had physical examination, chest radiogram, electrocardiogram, and echocardiogram. RESULTS: There were 84 patients aged between 2 months and 16 years. Mean age was 6.1 +/- 5.0 years. Forty eight children (57.1%) were males and 36 (43.9%) females (sex ratio was 1.3). Ventricular septal defect (17%), tetralogy of Fallot (13%) and pulmonary stenosis (12%) were the predominant pathologiese. Surgery was initially indicated for in 61 infants (73%), realized in 22 of them (26%) and considered out of date in 10 patients (12%). CONCLUSION: Congenital heart diseases pose a major problem of treatment in Mauritania. They are dominated by the left to right shunts. The age of diagnosis is relatively late (6 years) exposing to the risk of complications. Therefore, a team of paediatric cardiologists on the spot and the setting up of cardiovascular surgery facilities are necessary.

Adolescent↗
PubMed2004

Management of aortopulmonary collaterals in children following cardiac transplantation for complex congenital heart disease.

BACKGROUND: Heart transplantation (HTx) is increasingly utilized as therapy for end-stage cyanotic congenital heart disease. This study investigates the presence and impact of aortopulmonary collaterals (APCs) associated with cyanotic heart disease on the early post-operative course of patients undergoing transplantation. High output cardiac failure due to residual aortopulmonary collaterals can affect outcome following heart transplantation. METHODS: Seven patients with hemodynamically significant APCs post-transplant were identified among 40 patients with cyanotic congenital heart disease undergoing HTx. The peri- and intra-operative courses of these patients were reviewed. All 7 patients required prolonged inotropic support despite normal ventricular function and no allograft rejection; 5 were ventilator-dependent due to significant pulmonary vascular congestion. Selective angiography demonstrated the presence of significant aortopulmonary collaterals at 7 to 19 days post-transplant. Coil embolization of aortopulmonary collaterals was performed in all patients; a mean of 6 (2 to 16) vessels/patient were embolized. RESULTS: After embolization, pulmonary edema resolved and heart size normalized in all patients; inotropic support was weaned within 2 to 10 days in 5 patients. One patient developed transient renal failure secondary to excessive contrast load and another had enterococcal sepsis within 24 hours after the procedure. All patients were asymptomatic from 4 to 10 years of follow-up post-HTx. CONCLUSIONS: Aortopulmonary collaterals should be considered a cause of early donor heart failure in children following HTx for cyanotic congenital heart disease. Early detection and treatment of aortopulmonary collaterals by coil embolization is necessary to improve the post-transplant course in these complex patients.

Adolescent↗
PubMed2005

Optimization of 3D contrast-enhanced pulmonary magnetic resonance angiography in pediatric patients with congenital heart disease.

Contrast kinetics were studied in the main pulmonary artery (MPA) and ascending aorta (AAo) of 12 children with congenital heart disease. This information was used to optimize the timing of data acquisition for contrast-enhanced MR angiography in these vessels. To reduce contrast-agent dosage in these fragile patients, contrast enhancement was measured during routine diagnostic 3D magnetic resonance (MR) angiography instead of using test-bolus methods. This was possible by acquiring 2D cross-sectional images of the MPA and AAo during the 3D scan. Time-to-peak in the MPA and AAo was 4.9 +/- 2.2 and 6.1 +/- 2.2 s, respectively, while the transit time between the two vessels was 4.5 +/- 0.6 s. A point-spread-function analysis showed that intravascular signal strength was maximized if data acquisition began 4.7 +/- 2.3 s after the first arrival of contrast in the MPA and 5.6 +/- 2.3 s in the AAo. Little signal loss and artifact resulted when longer acquisition delays were used because contrast-agent clearance was slow. Based on these results, MR angiography of both the MPA and the AAo in children with congenital heart disease can be performed using elliptic-centric k-space sampling and a trigger delay of 7.9 s after contrast arrival in the AAo (i.e., time-to-peak signal strength in the AAo plus one SD to account for intersubject variability).

Adolescent↗
PubMed2002

Internet use in families with children requiring cardiac surgery for congenital heart disease.

OBJECTIVE: We sought to document the prevalence of Internet access and usage patterns among families who have children with congenital heart disease presenting for cardiac surgery. DESIGN: A cross-sectional study was performed utilizing a questionnaire (10 questions) as the study tool. Statistical analysis was performed on all completed questionnaires. The chi(2) test was used to evaluate categorical variables and the t test to evaluate continuous variables. RESULTS: Two hundred seventy-five questionnaires were completed and analyzed. Thirty-seven percent (102/275) of the children had a cyanotic congenital heart defect. There were 21 children with Trisomy 21. Fifty-eight percent (160/275) of families had access to the Internet. The most common locations for accessing the Internet were home (80%; 129/160) and work (51%; 82/160). There were no significant differences in Internet access with regards to underlying individual congenital heart defect, cyanotic versus acyanotic heart defects, or congenital heart defects with functional univentricular hearts versus biventricular hearts. Families with older children (12--24 years) were more likely to have Internet access. Families of children undergoing placement of a right ventricle to pulmonary artery conduit were more likely to have Internet access. Of the 160 families with Internet access, 58% (93/160) used the Internet to obtain information related to their child's cardiac diagnosis. Eighty-two percent (76/93) characterized locating cardiology-related information as easy. Six parents created interactive personal Web sites specifically related to their child's congenital heart defect. Although families with older patients (12--24 years) were more likely to have access to the Internet, this did not translate into greater use of the Internet to obtain cardiology-related information. Among families who accessed the Internet for cardiology-related information, 95% (88/93) of families characterized the information as helpful or very helpful in furthering the understanding of their child's heart defect. CONCLUSION: Families are utilizing the Internet to educate themselves about congenital heart disease. Most parents consider the process easy and the information obtained helpful to the understanding of their child's congenital heart defect and surgery. Internet use in this patient population is expected to increase. Our vigilance in providing accurate Internet references, as well as in identifying inaccurate Internet information available to our patients and their parents, is of paramount importance.

Adolescent↗
PubMed1986

Interventional procedures in congenital heart disease.

Balloon septostomy changed the natural history of transposition of the great arteries. Relatively little more occurred in catheter treatment of congenital heart disease until the development of the balloon catheter for transluminal angioplasty. Modification of these catheters led to successful treatment of valvular pulmonic stenosis, peripheral pulmonic stenosis, and coarctation restenosis. Possible future applications of balloon valvuloplasty include valvar aortic stenosis, stenotic shunts, and palliative operations. Pulmonary arteriovenous malformations are well managed by detachable balloon techniques, and balloons and coils are used for occluding large systemic collaterals. Interventional techniques have made a significant impact on the treatment of congenital heart disease, and it is anticipated that as technology improves further advances will occur.

Adult↗
PubMed1995

Advances in surgical care of infants and children with congenital heart disease.

Recent important technical developments in the field of surgery for congenital heart disease have included the Ross pulmonary autograft replacement of the aortic valve, video-assisted thoracoscopic surgery, and the double-switch and switch-Rastelli procedure for congenitally corrected transposition. Although the growth potential of the pulmonary autograft has been confirmed, an important incidence of late aortic regurgitation has been noted. Expanding indications for video-assisted thoracoscopic surgery have been described, including division of the vascular ring. Late hemodynamic assessment after the double-switch or switch-Rastelli procedure will encourage increased application of this repair. Both clinical and laboratory studies have focused on neurologic and developmental outcome after cardiac surgery. Perioperative seizures in young infants have been found to be associated with impaired psychomotor development at 1 year of age. In the area of perioperative management, the antifibrinolytic agents tranexamic acid, epsilon-aminocaproic acid, and aprotinin have been found to be useful in reducing postoperative hemorrhage. Nitric oxide is useful in reducing postoperative pulmonary hypertension. Late clinical follow-up studies of patients with single ventricle have revealed an important incidence of pulmonary arteriovenous malformations after a bidirectional Glenn shunt and atrial flutter after a Fontan procedure. Late assessment of patients after the arterial switch procedure for transposition has revealed preservation of ventricular function and an extremely low incidence of late arrhythmias.

Antifibrinolytic Agents↗
PubMed2005

Inhaled nitric oxide for the postoperative management of pulmonary hypertension in infants and children with congenital heart disease.

BACKGROUND: Nitric oxide (NO) is a prevalent molecule in the human body responsible for many physiologic activities including pulmonary vasodilation. An exogenous, inhaled form (iNO) exists that mimics this action without directly affecting systemic blood pressure. This therapy has been implemented in the treatment of pulmonary hypertension. This review examines the efficacy of iNO in the postoperative management of infants and children with congenital heart disease. OBJECTIVES: To compare the effects of postoperative iNO versus placebo and/or conventional management on infants and children with congenital heart disease. The primary outcome was mortality, while secondary outcomes included length of hospital stay, assessment of neurodevelopmental disability, number of pulmonary hypertensive crises (PHTC), changes in haemodynamics including mean pulmonary arterial pressure (MPAP), mean arterial pressure (MAP), and heart rate (HR), changes in oxygenation measured as the ratio PaO2:FiO2, and measurement of maximum methaemoglobin level as a marker of toxicity. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, Issue 3, 2004), MEDLINE (1966 to 2004), and EMBASE (1980 to 2004). We included abstracts and all languages. SELECTION CRITERIA: We included randomized and quasi-randomized controlled trials comparing iNO with placebo and conventional management, or both. Trials included only children with congenital heart disease requiring surgery and complicated by pulmonary hypertension. DATA COLLECTION AND ANALYSIS: Data were collected on mortality, number of PHTC, changes in MPAP, MAP, HR, and PaO2:FiO2, and maximum methaemoglobin level, while data on long-term mortality, neurodevelopmental disability, and length of hospital stay were unavailable. We performed subgroup analysis by age and method of control. We performed sensitivity analysis using studies of highest methodologic quality. MAIN RESULTS: We included four randomized trials. We observed no differences between groups with respect to mortality (P = 0.50), PHTC (P = 0.79), change in MPAP (P = 0.16), MAP (P = 0.40), HR (P = 1.00), or PaO2:FiO2 (P = 0.46). There was a significant reduction in MPAP in the subgroup of patients from birth to three months (P = 0.005), although this finding was based on a small number of patients (N = 23). AUTHORS' CONCLUSIONS: We observed no differences with the use of iNO as compared with control in the majority of outcomes reviewed. No data were available for analysis with respect to several clinical outcomes including long-term mortality and neurodevelopmental outcome. We found it difficult to draw valid conclusions because of concerns regarding methodologic quality, bias, sample size, and heterogeneity.

Administration, Inhalation↗
PubMed1991

Hemodynamic effects of prostaglandin E1 in patients with congenital heart disease and pulmonary hypertension.

Investigations were performed to determine whether prostaglandin E1 (PGE1) would cause a favorable change in pulmonary hemodynamics in pediatric patients with congenital heart disease and elevated pulmonary vascular resistance. Twenty-seven patients (ages 0.5 months to 19 years) were studied. PGE1 caused a significant decrease in mean pulmonary artery pressure with 0.05 microgram/kg/min and 0.1 microgram/kg/min infusions (p = .0004). There were significant increases in pulmonary blood flow (p = .0007), and decreases in pulmonary vascular resistance (p = .0001); changes in systemic pressure, resistance, oxygen consumption, and heart rate were minimal. Twenty-three patients underwent heart surgery after catheterization, nineteen of these survived. The four nonsurvivors had higher initial pulmonary resistance (PVR) and less decline in PVR with PGE1 than survivors. This study shows that PGE1 is an effective pulmonary vasodilator in patients with congenital heart disease and elevated pulmonary vascular resistance.

Adolescent↗
PubMedDate not supplied

Measurement and validity of the ventilatory threshold in patients with congenital heart disease.

The purpose of the present study was to measure oxygen uptake (VO2) at the ventilatory threshold (VT) in patients with congenital heart disease using a progressive exercise protocol on a treadmill and to evaluate the validity and feasibility of this procedure. Eight control subjects and seventeen patients performed a maximal exercise test with breath-by-breath measurement of ventilation and gas exchange variables. VT(VE) was determined by the change in the ventilatory equivalent for VO2 and carbon dioxide output, VT(V-sl) by the V-slope method, and the lactate threshold (LT) by the change in blood lactate concentration; these parameters were determined in 100%, 88%, and 96% of subjects, respectively. The interobserver error among three evaluators was not significant, and LT was correlated with each VT (r = 0.97, 0.92; p = 0.0001) and with peak VO2 (r = 0.91; p = 0.0001). The VTs were correlated with each other when expressed as milliliter per minute and milliliters per kilogram per minute. It was concluded that a progressive exercise protocol on a treadmill was a feasible procedure for determining the VTs in most individuals and that VTs were valid, useful parameters for evaluating submaximal exercise tolerance in patients with congenital heart disease.

Adolescent↗
PubMed1990

Ultrasound in the diagnosis of diaphragmatic paralysis after operation for congenital heart disease.

Phrenic nerve palsy is a recognised complication of operation for congenital heart disease in children. The accuracy of ultrasound in assessing diaphragmatic motion was prospectively compared with fluoroscopy in 16 patients in whom phrenic nerve palsy was suspected. Ultrasound successfully identified the five patients with phrenic nerve palsy; there were no false positive or false negative diagnoses. Ultrasound was as effective as fluoroscopy in the diagnosis of abnormalities of diaphragmatic motion.

Child↗