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PubMed2006

Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease.

This article focuses first on the process of transition and transfer of care of young adults with complex congenital heart disease. It defines the transition process and briefly discusses its history. It reviews the important aspects of transition, outlines the key elements of a successful transition program, and provides a curriculum appropriate for the young adult with congenital heart disease. Finally, it identifies the barriers to transfer of care, discusses the importance of a policy on timing, outlines the components of adult provider services that may be needed, and reviews the steps to an orderly transfer process.

Adolescent↗
PubMed1978

Surgical correction of congenital heart disease in the adult: experience with 139 patients.

Patients over 18 years of age who have undergone a surgical correction of a congenital cardiac malformation during the period 1968 through 1977 have been reviewed. Those patients with calcific aortic stenosis which was thought, but not proved, to have arisen in a congenitally malformed aortic valve, were excluded from the review. Patients with cystic medial necrosis of the aorta were similarly excluded. There were 139 patients in the series (age range from 18 years to 67 years). The most common defects were those involving the intra-atrial septum and the related great veins, 50%. Abnormalities involving the great arteries including patent ductus arteriosus and coarctation of the aorta accounted for 19%. Common defects of conal development including ventricular septal defects and Tetralogy of Fallot malformations accounted for 15%. Valvular abnormalities including pulmonic stenosis, aortic valve abnormalities and Ebstein's malformation of tricuspid valve accounted for 11.5%. Complex congenital malformations were relatively uncommon, 4%. There were two patients with a combination of acquired and congenital heart disease. There were two operative deaths in the series, both occurring in patients with complex forms of congenital heart disease (multiple ventricular septal defects, double outlet right ventricle). There were two additional postoperative hospital deaths, one occurring following repair of an atrial septal defect from massive pulmonary embolus, and another occurring six weeks following a Fontan procedure performed for tricuspid atresia. Thus, the hospital mortality for the series was 2.9%. This reviewed series reveals the incidence of operable congenital heart defects appearing in an adult cardiac surgical practice and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

Adolescent↗
PubMed1996

[Tridimensional echocardiography: general principles and clinical applications (with special reference to congenital heart diseases)].

Methods of three-dimensional reconstruction of cardiac images and emerging clinical applications are described, with special reference to congenital heart disease. Compared to other techniques providing dynamic three-dimensional visualization of heart structures, echocardiography has advantages of better temporal resolution and minor cost. Current applications include assessment of volumes and global and regional ventricular performance, evaluation of time motion of valves and other dynamic processes together with color-flow images, and dissection of the heart into specific areas of interest, such as atrial septum, mitral inflow and aortic outflow. In complex congenital heart disease, three-dimensional reconstruction provides an interpretation of the mode of atrioventricular and ventriculoarterial connections, with simulations of a "surgeon's view" of the heart. Defects as atrioventricular canal, double inlet left ventricle and tricuspid atresia can be examined in details concerning the architecture of valvar and subvalvar apparatus and presence and importance of ventricular septal defect. Other congenital malformations such as transposition of great arteries, truncus, coarctation syndrome, and double outlet right ventricle can be assessed in regard to interventricular communication and infundibular obstruction. Multiplane transesophageal probes are best suitable to this sort of electronic vivisection, but rotational and linear scanning methods have also given useful images of cardiac structures in children as well as adults. Validation studies are in progress on the reliability of a number of quantitative parameters that can be derived from the three-dimensional data set.

Adult↗
PubMed2005

Association of the C677T methylenetetrahydrofolate reductase mutation with congenital heart diseases.

BACKGROUND: To investigate whether the cytosine-to-thymine mutation at base 677 of the gene for methylenetetrahydrofolate reductase (MTHFR) is associated with congenital heart diseases (CHD), using high throughput heteroduplex analysis based upon the powerful technique of denaturing high-performance liquid chromatography. METHODS: We investigated the MTHFR genotype of a cytosine-to-thymine mutation at base 677 for 213 patients of CHDs as confirmed by cardiac catheterization and also for 195 healthy controls. RESULTS: The overall genotype frequencies of the MTHFR C677T polymorphism were not significantly different between the CHD patients and the healthy control (P = 0.345). Furthermore, taking various subgroups of CHD patients into consideration, we noted a significantly increased proportion of homozygous TT genotypes for patients suffering from valvular pulmonary stenosis (PS) or pulmonary atresia with an intact ventricular septum (PA + IVS) (p = 0.0005). For patients revealing heterotaxy syndrome, a conotruncal anomaly including tetralogy of Fallot, an interruption of the aortic arch, persistent truncus arteriosus, and aortopulmonary window, no statistically significant difference existed. CONCLUSIONS: The discrepancy in the distribution of MTHFR genotypes amongst various subtypes of CHD reflects some heterogeneity in the developmental mechanism of CHD. The increased percentage of homozygous TT genotypes might contribute to the pathogenesis of valvular PS and PA + IVS.

Asian People↗
PubMed1984

Pulmonary wedge angiography for the evaluation of the pulmonary vascular bed in congenital heart disease.

Pulmonary wedge angiograms (PWA) were analyzed qualitatively and quantitatively to evaluate the pulmonary vascular bed in 40 patients with congenital heart disease. The patients were divided into four groups: group 1, normal pulmonary hemodynamics; group 2, increased pulmonary blood flow; group 3, pulmonary hypertension (PH); and group 4, PH with elevated pulmonary vascular resistance. The PWA were analyzed for tapering, pulmonary circulation time (PCT) index, and vascular image. The tapering was normal in groups 1 and 2 and was more rapid in groups 3 and 4. The PCT index was slightly lower in groups 3 and 4 than in groups 1 and 2. With 100% oxygen, the tapering became less severe and the PCT index increased in group 3. However in group 4, these parameters did not improve with 100% oxygen, suggesting that more advanced vascular changes had developed. These studies confirm the usefulness of the PWA in evaluating the pulmonary vascular bed of patients with congenital heart disease and PH.

Angiography↗
PubMed1993

Percutaneous coil embolization of superfluous vascular connections in patients with congenital heart disease.

Transcatheter Gianturco coil embolization of 26 superfluous but hemodynamically significant vascular connections was attempted in 19 patients with congenital heart disease. There were 18 aortopulmonary collaterals, two vessels involved in pulmonary sequestration, two surgical aortopulmonary shunts (one inadvertently connected from the left subclavian artery to a left upper pulmonary vein), two cases of patent ductus arteriosus, one regurgitant left ventricular-to-descending aorta conduit, and a left superior vena cava-to-left atrium post Fontan procedure. The mean ratio of coil to vessel diameter was 1.6 +/- 0.7, and a mean of 1.5 +/- 1.1 coils were used per vessel. Occlusion was achieved in 24 of 26 attempts (92%), being complete in 21 (81%) and near complete (> 80% decrease in flow) in three (12%). Two attempts failed; a tortuous aortopulmonary collateral was engaged by the delivery catheter but the coil could not be advanced past an acute angle, and another aortopulmonary collateral had a narrow origin and enlarged distally, resulting in coil migration to a distal pulmonary artery branch. One patient had a transient decrease in leg pulses that resolved spontaneously. Fever occurred in four patients (21%) within 12 hours of coil placement, and resolved in all without sequelae or complications. A variety of undesirable vascular connections in patients with congenital heart disease can be safely and effectively treated by percutaneous coil embolization.

Aorta↗
PubMed1990

Orthotopic heart transplantation for complex congenital heart disease.

Orthotopic heart transplantation has become standard therapy for end-stage cardiomyopathy in children and adults, but there has been much less experience with transplantation for complex congenital heart disease. In this report experience with orthotopic transplantation in seven children with various forms of complex congenital heart disease is reviewed. Diagnoses included hypoplastic left heart syndrome in two (after stage I palliation), left ventricular diverticulum in one, single ventricle in two (dextrocardia, atrial situs inversus, and total anomalous pulmonary venous return in one patient and post-Fontan repair in the second), D-transposition of the great arteries and ventricular septal defect (post-Senning repair and ventricular septal defect closure) in one, and Ebstein's anomaly with biventricular dysplasia in one. Six of the seven were hospital survivors and there has been one late death at 2 1/3 years. Modifications of the standard operative technique to fit the anatomic variations in these defects are reviewed.

Adolescent↗
PubMed2005

Usefulness of plasma B-type natriuretic peptide to identify ventricular dysfunction in pediatric and adult patients with congenital heart disease.

The usefulness of B-type natriuretic peptide (BNP) levels to assess ventricular dysfunction in children and the congenital heart disease population remains largely unknown. We retrospectively analyzed 62 patients with or without known heart disease who had plasma BNP measured for the investigation of new or severity grading of known ventricular dysfunction. BNP levels were significantly higher in patients with ventricular dysfunction (mean 623 +/- 146 pg/ml, range 5 to 5,000) than in patients without ventricular dysfunction (mean 22 +/- 5 pg/ml, range 5 to 63; p <0.01). Using a cutoff of 40 pg/ml, BNP levels detected heart disease associated with ventricular dysfunction at a sensitivity of 85%, specificity of 81%, positive predictive value of 92%, and negative predictive value of 68%. The degree of BNP elevation was also associated with the severity of heart failure and high ventricular filling pressures. Plasma BNP elevation can be a reliable test in children and young adults with various kinds of congenital heart disease resulting in ventricular dysfunction.

Adolescent↗
PubMed2000

Clinical and cardiorespiratory assessment in children with Down syndrome without congenital heart disease.

OBJECTIVE: To assess the clinical and functional status of a young Down syndrome (DS) population without congenital heart disease. DESIGN: Prospective study of children with DS and control subjects. SETTING: Bambino Gesù Children's Hospital, Rome, Italy. PARTICIPANTS AND METHODS: Forty-two children with DS (mean +/- SD age, 9.8 +/- 3.6 years) underwent genetic, clinical, neuropsychological (IQ), and cardiorespiratory evaluation. Cardiorespiratory fitness was assessed with a treadmill test and a lung function test to determine forced vital capacity, first-second forced expiratory volume, and peak expiratory flow. Data were expressed as a percentage of the predicted values for control children. To assess cooperation during exercise testing, we devised a compliance scale assigning a score according to the subjects' ability to understand instructions, ability to walk and run as required, and need for vocal encouragement. RESULTS: Eighteen (43%) of 42 children with DS were obese, 10 (24%) were short, and 17 (40%) had microcephaly. On the Leiter International Performance Scale, 2 of 35 subjects had a normal IQ score (80-120); all others had low IQ scores (79 to <40). Five subjects did not undergo cardiorespiratory assessment. Eighteen of 37 subjects completed lung function tests: the results for 10 were unremarkable, and results from 8 revealed reduced forced vital capacity because of poor compliance. The subjects tested had low exercise tolerance (mean +/- SD tolerance percentage, 61% +/- 12%), mild tachycardia (maximal heart rate, 91% +/- 4%), and a mild hypertensive response (maximal blood pressure, 89% +/- 8%). Compliance scores correlated significantly with exercise time and age but not with IQ. CONCLUSIONS: Clinical and cardiorespiratory assessment is feasible in subjects with DS without congenital heart disease and should be useful in gauging their fitness level for safe physical activity.

Adolescent↗
PubMed1977

Pulmonary changes in congenital heart disease of childhood: histometrical and histological studies.

One hundred and fifty lungs from the cases below 15 years of age with various congenital heart diseases and 80 controls were used for histometrical and histological studies. Cases with congenital heart disease were divided into two groups of the increased and the decreased pulmonary blood flow. In the former group, the thickness of the pulmonary arterial media was the same as that of controls in the neonatal period, and through the wall thickness gradually decreased in a pattern seen in controls, the thickness was constantly larger than that of controls. In some cases, the media increased gradually within 6 months after birth. Pneumonia and massive pulmonary hemorrhage were seen in a higher incidence in autopsy cases. Pneumonia in younger infants was histologically characteristic and possibly more correlated to their death. In the latter group, most of the cases were with the thinner medias of the pulmonary arteries. Massive pulmonary hemorrhage was not common in the latter group.

Child↗
PubMed1999

Postoperative evaluation of congenital heart disease by magnetic resonance imaging.

In the last four decades the survival of patients with corrected or palliated congenital heart disease has increased dramatically. However, post-operative abnormalities frequently occur and therefore a noninvasive imaging tool is mandatory for the timely detection of morphological as well as functional abnormalities. Magnetic resonance imaging (MRI) is ideally suited for the noninvasive diagnosis and post-operative follow-up of congenital heart disease. Spin-echo MRI is able to visualize structures that may be difficult to assess with other noninvasive image modalities and is sensitive in the detection of post-interventional stenoses or aneurysms. Because the function of the ventricles may deteriorate over time after correction or palliation of a congenital cardiac malformation, the use of gradient-echo MRI is essential in the follow-up after correction or palliation, as no other conventional technique allows such detailed evaluation of ventricular function, without geometrical assumptions. Phase-contrast MRI is well suited to assess valvular function, allowing accurate measurement of regurgitation or stenosis. Shunt quantification is another application of phase-contrast MRI. J. Magn. Reson. Imaging 1999;10:656-666.

Follow-Up Studies↗
PubMed2005

Impact of congenital heart disease on cerebrovascular blood flow dynamics in the fetus.

OBJECTIVES: Neurological abnormalities are present in some children after repair of congenital heart disease (CHD). Recently, structural brain abnormalities have been identified in infants prior to cardiac surgery. By altering in utero blood flow patterns, the type of CHD may impact upon cerebrovascular flow dynamics prior to birth. We sought to determine whether left- and right-sided obstructive congenital heart lesions modify cerebrovascular flow dynamics in the fetus. METHODS: Pulsed Doppler was used to measure blood flow velocities in the umbilical (UA) and middle cerebral (MCA) arteries in 172 fetuses from 20 to 39 weeks' gestational age referred for fetal echocardiography. Pulsatility index (PI), an indicator of downstream vascular resistance, was determined by (peak systolic velocity--end-diastolic velocity)/mean velocity. RESULTS: Fetuses with hypoplastic left heart syndrome (HLHS; n = 28) had decreased MCA-PI (P = 0.009) compared to normal fetuses (n = 114). Fetuses with right-sided obstructive lesions (RSOL; n = 17) had increased MCA-PI (P = 0.001) when compared to fetuses with HLHS. The UA-PI was elevated in fetuses with RSOLs (P = 0.045). CONCLUSIONS: Cerebrovascular resistance is lower than normal in fetuses with HLHS, a condition in which cerebral perfusion occurs retrograde via the ductus arteriosus. Fetuses with RSOL had significantly higher cerebrovascular resistance compared to fetuses with HLHS. The type of CHD impacts upon fetal cerebrovascular blood flow distribution and this may have implications for later development of neurological sequelae.

Blood Flow Velocity↗
PubMed1996

Lung perfusion scintigraphy in patients with congenital heart disease: sensitivity and important pitfalls.

The lung perfusion scans requested over a 5-year period for children with known congenital heart disease were reviewed retrospectively. Altogether, 102 lung perfusion scans were performed and comparable pulmonary arteriograms were available in 63 cases. There was agreement between scintigraphy and angiography in 50 (79%) cases and discrepancy in 13 (21%) cases. Scintigraphy was more sensitive in detecting small perfusion defects, particularly those seen only on oblique views. Pulmonary arteriography was more specific in identifying the cause of the perfusion abnormalities. The two techniques yield complementary information about lung perfusion. Perfusion scintigraphy is safe in children with congenital heart disease even with known pulmonary to systemic shunting. Many pitfalls could be avoided by careful choice of injection site, by knowledge of previous surgery and comparison with relevant angiograms.

Adolescent↗
PubMed2002

The incidence of congenital heart disease.

This study was designed to determine the reasons for the variability of the incidence of congenital heart disease (CHD), estimate its true value and provide data about the incidence of specific major forms of CHD. The incidence of CHD in different studies varies from about 4/1,000 to 50/1,000 live births. The relative frequency of different major forms of CHD also differs greatly from study to study. In addition, another 20/1,000 live births have bicuspid aortic valves, isolated anomalous lobar pulmonary veins or a silent patent ductus arteriosus. The incidences reported in 62 studies published after 1955 were examined. Attention was paid to the ways in which the studies were conducted, with special reference to the increased use of echocardiography in the neonatal nursery. The total incidence of CHD was related to the relative frequency of ventricular septal defects (VSDs), the most common type of CHD. The incidences of individual major forms of CHD were determined from 44 studies. The incidence of CHD depends primarily on the number of small VSDs included in the series, and this number in turn depends upon how early the diagnosis is made. If major forms of CHD are stratified into trivial, moderate and severe categories, the variation in incidence depends mainly on the number of trivial lesions included. The incidence of moderate and severe forms of CHD is about 6/1,000 live births (19/1,000 live births if the potentially serious bicuspid aortic valve is included), and of all forms increases to 75/1,000 live births if tiny muscular VSDs present at birth and other trivial lesions are included. Given the causes of variation, there is no evidence for differences in incidence in different countries or times.

Ductus Arteriosus, Patent↗
PubMedDate not supplied

Surgical experience with congenital heart disease in Down's syndrome.

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.

Adolescent↗
PubMed1998

The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease.

UNLABELLED: The role of inhaled nitric oxide in the immediate post-bypass period after surgical repair of congenital heart disease is uncertain. In a controlled, randomized, double-blind study, we tested the hypothesis that inhaled nitric oxide (NO) would reduce pulmonary hypertension immediately after surgical repair of congenital heart disease in 40 patients with preoperative evidence of pulmonary hypertension (mean pulmonary arterial pressure [MPAP] exceeding 50% of mean systemic arterial pressure [MSAP]). Patients were then followed in the intensive care unit (ICU) to document the incidence of severe pulmonary hypertension. Of the patients, 36% (n = 13) emerged from bypass with MPAP > 50% MSAP. In these patients, inhaled NO reduced MPAP by 19% (P = 0.008) versus an increase of 9% in the placebo group. No effect on MPAP was observed in patients emerging from bypass without pulmonary hypertension (n = 23). Inhaled NO was required five times in the ICU, always in the patients who had emerged from cardiopulmonary bypass with pulmonary hypertension (5 of 13 [38%] versus 0 of 23). We conclude that, in infants and children undergoing congenital heart surgery, inhaled NO selectively reduces MPAP in patients who emerge from cardiopulmonary bypass with pulmonary hypertension and has no effect on those who emerge without it. IMPLICATIONS: In a randomized double-blind study, inhaled nitric oxide selectively reduced pulmonary artery pressures in pediatric patients who developed pulmonary hypertension (high blood pressure in the lungs) immediately after cardiopulmonary bypass and surgical repair.

Administration, Inhalation↗
PubMed1995

A comparison of transesophageal and transthoracic echocardiographic assessment of left ventricular function in pediatric patients with congenital heart disease.

OBJECTIVE: To determine the quantitative utility of transesophageal echocardiographic assessments of left ventricular function in pediatric patients with congenital heart disease by evaluating the variability between observers and between echocardiographic windows. DESIGN: Retrospective, blinded analysis. SETTING: University-associated pediatric hospital. PARTICIPANTS: Transthoracic and transesophageal echocardiographic images of 25 pediatric patients with congenital heart disease were reviewed. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: End-diastolic area, end-systolic area, and fractional area change were measured from short-axis images of the left ventricle at the midpapillary level by two separate investigators. These measurements were compared by the method of Bland and Altman and Sheiner and Beal. Significant differences in measurements of end-diastolic and end-systolic area by different observers were noted, but they were systematic. A similar situation was noted for the comparison of transthoracic and transesophageal measurements of end-diastolic and end-systolic area. In the comparison of fractional area change between observers or windows, bias and absolute prediction error were lower, with 95% confidence limits of bias or absolute prediction error of 10% or less. CONCLUSIONS: The potential error in the measurement of fractional area change in 10% under optimal conditions. This would suggest that the assessment of ventricular function in the operating room or intensive care unit, under less than optimal conditions, should be viewed as a qualitative, rather quantitative, measurement. There may be significant interobserver and interwindow variability.

Bias↗