Low agreement between cardiologists diagnosing left ventricular hypertrophy in children with end-stage renal disease.

BACKGROUND: Monitoring of the appearance of left ventricular hypertrophy (LVH) by echocardiography is currently recommended for in the management of children with End-stage renal disease (ESRD). In order to investigate the validity of this method in ESRD children, we assessed the intra- and inter-observer reproducibility of the diagnosis LVH. METHODS: Echocardiographic measurements in 92 children (0-18 years) with ESRD, made by original analysists, were reassessed offline, twice, by 3 independent observers. Smallest detectable changes (SDC) were calculated for continuous measurements of diastolic interventricular septum (IVSd), Left ventricle posterior wall thickness (LVPWd), Left ventricle end-diastolic diameter (LVEDd), and Left ventricle mass index (LVMI). Cohen's kappa was calculated to assess the reproducibility of LVH defined in two different ways. LVH(WT) was defined as Z-value of IVSd and/or LVPWd>2 and LVH(MI) was defined as LVMI> 103 g/m² for boys and >84 g/m² for girls. RESULTS: The intra-observer SDCs ranged from 1.6 to 1.7 mm, 2.0 to 2.6 mm and 17.7 to 30.5 g/m² for IVSd, LVPWd and LVMI, respectively. The inter-observer SDCs were 2.6 mm, 2.9 mm and 24.6 g/m² for IVSd, LVPWd and LVMI, respectively. Depending on the observer, the prevalence of LVH(WT) and LVH(MI) ranged from 2 to 30% and from 8 to 25%, respectively. Kappas ranged from 0.4 to 1.0 and from 0.1 to 0.5, for intra-and inter- observer reproducibility, respectively. CONCLUSIONS: Changes in diastolic wall thickness of less than 1.6 mm or LVMI less than 17.7 g/m² cannot be distinguished from measurement error in individual children, even when measured by the same observer. This limits the use of echocardiography to detect changes in wall thickness in children with ESRD in routine practice.

A multicentric study of disease-related stress, and perceived vulnerability, in parents of children with congenital cardiac disease.

Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need.

The effect of bosentan in patients with a failing Fontan circulation.

OBJECTIVES: To investigate the effect of bosentan in patients with a failing Fontan circulation. DESIGN: A multicentric open label, non-controlled study. SETTING: 5 tertiary care centres for congenital cardiology. PATIENTS: We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded. RESULTS: Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued. CONCLUSIONS: Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

MR imaging of right ventricular function after the Ross procedure for aortic valve replacement: initial experience.

PURPOSE: To prospectively assess right ventricular (RV) function after the Ross procedure by using magnetic resonance (MR) imaging. MATERIALS AND METHODS: The local ethics committee approved the study and informed consent was obtained from all participants prior to enrollment in the study. Seventeen patients (15 male, two female; mean age +/- standard deviation, 19 years +/- 3.9; imaging performed 8.3 years after surgery +/- 3.2) and 17 matched controls (15 male, two female; mean age +/- standard deviation, 20 years +/- 3.9) were studied by using MR imaging. Standard velocity-encoded and multisection multiphase imaging sequences were used to assess homograft valve function, systolic and diastolic RV function, and RV mass. The two-tailed Mann-Whitney U test and the Spearman rank correlation coefficient were used for statistical analysis. RESULTS: Minor degrees of homograft stenosis (peak flow velocity between 1.5 and 3.0 m/sec across the homograft valve) were found in 12 of 17 patients but not in controls (P < .001). A larger RV mass was present in Ross patients than in controls (17.0 g/m(2) +/- 4.8 vs 10.9 g/m(2) +/- 5.6, P = .004). In addition, impaired diastolic RV function was found, as shown by a decreased mean tricuspid valve early filling phase-atrial contraction phase (E/A) peak flow velocity ratio (1.56 +/- 0.75 vs 2.05 +/- 0.58, P = .03). Peak flow velocity across the homograft valve correlated with RV mass (r = 0.38, P = .03) and tricuspid valve E/A peak flow velocity ratio (r = 0.39, P = .02). RV systolic function was normal in Ross patients (mean RV ejection fraction, 52% +/- 8 vs 51% +/- 5; P = .74). CONCLUSION: RV hypertrophy and RV diastolic dysfunction are frequently observed in patients after the Ross procedure, even in the absence of overt homograft stenosis. RV systolic function is still preserved.

Aortic elasticity and left ventricular function after arterial switch operation: MR imaging--initial experience.

PURPOSE: To prospectively assess aortic dimensions, aortic elasticity, aortic valve competence, and left ventricular (LV) systolic function in patients after the arterial switch operation (ASO) by using magnetic resonance (MR) imaging. MATERIALS AND METHODS: Informed consent was obtained from all participants for this local ethics committee-approved study. Fifteen patients (11 male patients, four female patients; mean age, 16 years +/- 4 [standard deviation]; imaging performed 16.1 years after surgery +/- 3.7) and 15 age- and sex-matched control subjects (11 male subjects, four female subjects; mean age, 16 years +/- 4) were evaluated. Velocity-encoded MR imaging was used to assess aortic pulse wave velocity (PWV), and a balanced turbo-field-echo sequence was used to assess aortic root distensibility. Standard velocity-encoded and multisection-multiphase imaging sequences were used to assess aortic valve function, systolic LV function, and LV mass. The two-tailed Mann-Whitney U test and Spearman rank correlation coefficient were used for statistical analysis. RESULTS: Patients treated with the ASO showed aortic root dilatation at three predefined levels (mean difference, 5.7-9.4 mm; P < or = .007) and reduced aortic elasticity (PWV of aortic arch, 5.1 m/sec +/- 1.2 vs 3.9 m/sec +/- 0.7, P = .004; aortic root distensibility, [2.2 x 10(-3)] x mm Hg(-1) +/- 1.8 vs [4.9 x 10(-3)] x mm Hg(-1) +/- 2.9, P < .01) compared with control subjects. Minor degrees of aortic regurgitation (AR) were present (AR fraction, 5% +/- 3 in patients vs 1% +/- 1 in control subjects; P < .001). Patients had impaired systolic LV function (LV ejection fraction [LVEF], 51% +/- 6 vs 58% +/- 5 in control subjects; P = .003), in addition to enlarged LV dimensions (end-diastolic volume [EDV], 112 mL/m(2) +/- 13 vs 95 mL/m(2) +/- 16, P = .007; end-systolic volume [ESV], 54 mL/m(2) +/- 11 vs 39 mL/m(2) +/- 7, P < .001). Degree of AR predicted decreased LVEF (r = 0.41, P = .026) and was correlated with increased LV dimensions (LV EDV: r = 0.48, P = .008; LV ESV: r = 0.67, P < .001). CONCLUSION: Aortic root dilatation and reduced elasticity of the proximal aorta are frequently observed in patients who have undergone the ASO, in addition to minor degrees of AR, reduced LV systolic function, and increased LV dimensions.

Maternal global methylation status and risk of congenital heart diseases.

OBJECTIVE: To investigate whether the association between the maternal methylation status as reflected by low S-adenosylmethionine and high S-adenosylhomocysteine, is detrimental for cardiogenesis and congenital heart disease (CHD) in the offspring. METHODS: As part of a case-control study in the western part of the Netherlands, we evaluated 231 mothers of children with CHD and 315 control mothers of nonmalformed children. The total case group was analyzed and stratified into isolated (n=180) and nonisolated CHDs (n=51). The latter subgroup was further subdivided into Nonsyndromic (n=20), Down Syndrome (n=19), and Other Syndromes (n=12). A multivariable general linear model was used to test for differences between the case groups and controls. All analyses were adjusted for current B vitamin supplement use. RESULTS: Plasma total homocysteine was significantly different between the total case group (median, range 10.3, 4.0-43.8, P=.026) and the nonisolated cases (11.1, 5.5-43.8, P=.006) compared with the controls (10.0, 5.3-42.0). The subgroup of Down Syndrome presented significantly higher total homocysteine and S-adenosylhomocysteine levels and a lower S-adenosylmethionine/S-adenosylhomocysteine ratio than controls. CONCLUSION: Maternal hyperhomocysteinemia, and not hypomethylation, is a risk factor for having a child with CHD. Maternal hypomethylation, however, seems to be associated with offspring having CHD and Down syndrome.

Johanson-Blizzard syndrome caused by identical UBR1 mutations in two unrelated girls, one with a cardiomyopathy.

We report on two apparently unrelated girls with Johanson-Blizzard syndrome (JBS), in both children caused by a homozygous IVS26+5G>A mutation in the UBR1 gene. In both cases the parents are consanguineous and more sibs are affected. The somewhat mild phenotype (with no or slight mental retardation) in these two JBS families might be explained by residual UBR1 activity. One case has a dilated cardiomyopathy, a symptom only rarely reported in JBS, but of important clinical significance.

Maternal intake of fat, riboflavin and nicotinamide and the risk of having offspring with congenital heart defects.

BACKGROUND: With the exception of studies on folic acid, little evidence is available concerning other nutrients in the pathogenesis of congenital heart defects (CHDs). Fatty acids play a central role in embryonic development, and the B-vitamins riboflavin and nicotinamide are co-enzymes in lipid metabolism. AIM OF THE STUDY: To investigate associations between the maternal dietary intake of fats, riboflavin and nicotinamide, and CHD risk in the offspring. METHODS: A case-control family study was conducted in 276 mothers of a child with a CHD comprising of 190 outflow tract defects (OTD) and 86 non-outflow tract defects (non-OTD) and 324 control mothers of a non-malformed child. Mothers filled out general and food frequency questionnaires at 16 months after the index-pregnancy, as a proxy of the habitual food intake in the preconception period. Nutrient intakes (medians) were compared between cases and controls by Mann-Whitney U test. Odds ratios (OR) for the association between CHDs and nutrient intakes were estimated in a logistic regression model. RESULTS: Case mothers, in particular mothers of a child with OTD, had higher dietary intakes of saturated fat, 30.9 vs. 29.8 g/d; P < 0.05. Dietary intakes of riboflavin and nicotinamide were lower in mothers of a child with an OTD than in controls (1.32 vs. 1.41 mg/d; P < 0.05 and 14.6 vs. 15.1 mg/d; P < 0.05, respectively). Energy, unsaturated fat, cholesterol and folate intakes were comparable between the groups. Low dietary intakes of both riboflavin (<1.20 mg/d) and nicotinamide (<13.5 mg/d) increased more than two-fold the risk of a child with an OTD, especially in mothers who did not use vitamin supplements in the periconceptional period (OR 2.4, 95%CI 1.4-4.0). Increasing intakes of nicotinamide (OR 0.8, 95%CI 0.7-1.001, per unit standard deviation increase) decreased CHD risk independent of dietary folate intake. CONCLUSIONS: A maternal diet high in saturated fats and low in riboflavin and nicotinamide seems to contribute to CHD risk, in particular OTDs.

Transcatheter closure of perimembranous ventricular septal defects in infants and children using the Amplatzer perimembranous ventricular septal defect occluder.

There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

Aortic root dysfunctioning and its effect on left ventricular function in Ross procedure patients assessed with magnetic resonance imaging.

BACKGROUND: This study evaluated the diameters and distensibility of the aortic root as well as the degree of aortic regurgitation (AR) and its effect on left ventricular (LV) function in patients 8.2 +/- 3.1 years after they underwent the Ross procedure, with a comparison of these parameters between patients and matched healthy subjects. METHODS: Eighteen Ross procedure patients (16 male patients, age [mean +/- SD] 19.2 +/- 3.8 years) and 18 matched healthy subjects (16 male patients, age [mean +/- SD] 19.7 +/- 4.2 years) underwent magnetic resonance imaging. Measurements for diameters (at 4 levels) and the distensibility of the aortic root were performed using a steady-state free precession sequence. Aortic flow was assessed with a velocity-encoded phase-contrast sequence. Left ventricular systolic function was assessed with a gradient-echo sequence in the short-axis plane. Comparison of parameters was performed using the Mann-Whitney U test. Correlations between diameters, distensibility, AR fraction, and LV systolic function were expressed with Spearman rank correlation coefficients. Linear regression analysis was used to identify predictors of LV systolic dysfunction. RESULTS: Aortic root diameters were increased in Ross procedure patients as compared with healthy subjects (mean difference 6.3-11.6 mm, P < or = .02 at all 4 levels). Distensibility of the aortic root was lower in patients (1.9 +/- 1.1 vs 7.8 +/- 3.3 mm Hg(-1), P < .01). An AR fraction > 5% was present in 14 of the 18 patients (mean AR fraction 8% +/- 5% vs 1% +/- 1%, P < .01). Left ventricular ejection fraction was lower in patients (50% +/- 6% vs 57% +/- 6%, P < .01). Dilatation, decreased distensibility, and AR fraction were correlated with impaired LV systolic function (P < .05 for all). The AR fraction predicted impaired LV systolic function (P < .01). CONCLUSIONS: Magnetic resonance imaging shows dilatation and decreased distensibility of the aortic root, AR, and consequent impaired LV systolic function in patients after the Ross procedure.

Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of fallot.

BACKGROUND: Pulmonary regurgitation (PR) late after total correction for tetralogy of Fallot may lead to progressive right ventricular (RV) dilatation and an increased incidence of severe arrhythmias and sudden death. Timing of pulmonary valve replacement (PVR) is subject to discussion, because the effect of PVR on RV function in adults is unclear. In this study, MRI was used to assess the effect of PVR on RV function and PR. Clinical improvement was established by means of the NYHA classification. METHODS AND RESULTS: Twenty-six adult patients were included. Cardiac MRI was performed at a median of 5.1+/-3.4 months before and 7.4+/- 2.4 months after PVR. Mean preoperative PR was 46+/-10% (range, 25% to 64%). After PVR, 20 of 26 patients (77%) showed no residual PR, 5 patients showed mild residual PR, and 1 patient showed moderate PR. RV end-diastolic volume (RV-EDV) decreased from 305+/-87 to 210+/-62 mL (P<0.001), and RV end-systolic volume (RV-ESV) decreased from 181+/-67 to 121+/-58 mL (P<0.001). No significant change was found in RV-EF (42% versus 42%). However, RVEF corrected for regurgitations and shunting increased from 25.2+/-8.0% to 43.3+/-13.7% (P<0.001). Mean validity class improved from 2.0 to 1.3 (P<0.001). CONCLUSIONS: In adult patients with PR and RV dilatation, late after total correction of tetralogy of Fallot, MRI measurements show remarkable hemodynamic improvement of RV function after PVR and improvement of validity. We therefore advocate a less restrictive management concerning PVR in these patients.

Intraoperative stenting of pulmonary arteries.

OBJECTIVE: The surgical treatment of pulmonary artery stenoses in small children with complex cardiac lesions can be technically difficult. A hybrid-approach combining corrective surgery and intraoperative stent placement may be complementary in these patients. METHODS: Descriptive study in 11 small children (age: one week to 12 years, median of 12 months, weight: 2.5-20 kg) after previous cardiac surgery. Intraoperative stenting of pulmonary arteries was performed involving paediatric cardiologist and cardiac surgeon. Stenting was combined with repair of pulmonary atresia (n=2), right ventricular outflow and pulmonary artery reconstruction (n=3), unifocalisation of pulmonary arteries (n=1), revision of distal anastomosis of RV-PA-conduit after truncus repair (n=1), revision of distal anastomosis of RV-PA-conduit after AVSD/Fallot repair (n=1), aortic arch patch reconstruction after anatomical correction for transposition of the great arteries (n=1), bidirectional cavopulmonary anastomosis after Norwood I operation for hypoplastic left heart syndrome, (n=1) retrieval of a dislodged stent from the left pulmonary artery (n=1). In seven patients stenting was planned electively while in four patients it took place on an emergency base. RESULTS: No complications occurred during stent implantation. One patient died three weeks postoperatively from diffuse bleeding due to a coagulation disorder. Ten patients left hospital after the surgical intervention with concomitant stent implantation. Stent re-dilatation was necessary in 4 patients 2-24 months postoperatively. After a median follow-up of 15 months ranging from 3 weeks to 7.5 years all stents were patent as diagnosed by angiography in 6 patients and by colour-Doppler in all other patients. One year after stent placement one stent was removed and another surgically opened during re-operation for conduit replacement in the smallest patient from this series. There was one late death during operative right ventricular outflow-tract reconstruction after initial stent placement. CONCLUSIONS: With intraoperative stent placement surgically difficult patch augmentation of small and fragile pulmonary vessels during repair of complex cardiac lesions can be avoided. Stents recruit pulmonary vessels and keep them open and amenable to future percutaneous or surgical interventions.

Results of the Ross operation in a pediatric population.

OBJECTIVE: To analyse the results of the mid-term clinical and echocardiographic follow-up of the pediatric Ross operation. METHODS: Echo-Doppler follow-up of 53 consecutive pediatric Ross procedures performed between 1994 and 2003. Median age was 9.7 years at time of operation (2 weeks-17.7 years). Six patients were younger than 3 months. Median age at follow-up was 15.6 years. Aortic valve/left ventricular outflow tract (LVOT) anomalies were congenital in 49 (92%). Seventy percent had previous surgery or balloon valvuloplasty. Root replacement was used in all. Thirteen patients (25%) had LVOT enlargement. Mean cross-clamp time was 113 (69-189) minutes. RESULTS: Early mortality occurred in 3 patients after emergency surgery following balloon failure (n=1) and extended Ross following interrupted arch/VSD repair (n=2). Late mortality was due to LV fibroelastosis in 2 patients and complicated pulmonary artery stenting in another. RVOT reoperations were required because of late homograft obstruction in 2 patients and because of pulmonary artery stenosis in another. Five patients (9.4%) were reoperated for pulmonary autograft dilatation (n=3) and for leaflet fibrosis or perforation (n=2). Autografts were repaired in two patients, while a mechanical valve was inserted in 3 cases. At 9 years the actuarial survival and event free survival were 89 and 74%, respectively. At last follow-up 90% of autograft diameters indexed to body surface area was above the 90th percentile of normal aortic root diameters. LVOT and RVOT gradients were low and autograft insufficiency was trivial to mild in 84% and mild to moderate in 16%. Autograft stenosis was not noticed. CONCLUSIONS: The pediatric Ross procedure remains an important tool but autograft dilatation also occurs in the pediatric population. The significance of this finding has yet to be determined.

Deficiency of the vestibular spine in atrioventricular septal defects in human fetuses with down syndrome.

Data on the morphogenesis of atrioventricular septal defect (AVSD) in Down syndrome are lacking to support molecular studies on Down syndrome heart critical region. Therefore, we studied the development of complete AVSD in human embryos and fetuses with trisomy 21 using 3-dimensional graphic reconstructions and immunohistochemical markers. Eight trisomic hearts with AVSD and 10 normal hearts, ranging from 5 to 16 weeks' gestation, were examined. In AVSD, the muscular septum primum and venous valves develop normally, and the size and histology of the nonfused endocardial cushions also appear normal. However, the mass of extracardiac mesenchyme (vestibular spine), located at the dorsal mesocardium, is reduced and does not protrude ventrally along the right wall of the common pulmonary vein. As a result of this, the muscular septum primum and the right pulmonary ridge are seen as 2 separate septa that attach to the inferior endocardial cushion. Both the muscular septum primum and the superiorly fused venous valves (septum spurium) converge and are capped by a small rim of mesenchyme, which forms the roof of the persisting ostium primum and connects to cushions and the reduced vestibular spine. At 7 weeks, ventricular septation in AVSD is comparable to 5 to 6 weeks of normal cardiac development. At later stages, the septum spurium forms the anterosuperior limbus of the septum secundum and the mesenchymal cap becomes the bridging tendon that connects the bridging leaflets. Therefore, reduced expansion of the vestibular spine derived from the dorsal mesocardium appears to play an important role in the development of AVSD in Down syndrome.

Isolated unilateral absence of a pulmonary artery: a case report and review of the literature.

OBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. METHODS: A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. RESULTS: Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. CONCLUSION: Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.

The morphologic nature of noncommitted ventricular septal defects in specimens with double-outlet right ventricle.

BACKGROUND: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications. METHODS: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices. RESULTS: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle. CONCLUSION: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.

Employment in adults with congenital heart disease.

OBJECTIVE: To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. DESIGN: Cross-sectional study. SETTING: Patients were randomly selected from the archives of the Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. PATIENTS AND MAIN OUTCOME MEASURES: In total, 76 patients with complex CHD and 80 with mild CHD (age range, 17-32 years) completed a self-reported questionnaire on employment and handicaps, with reference data available (response rate, 70%). RESULTS: In the study groups, 45 (59%) of 76 patients with complex CHD had a paid job compared with 61 (76%) of 80 patients with mild CHD. Patients older than 25 years with complex CHD had significantly lower job participation (64%) than the general population (83%). Multiple logistic regression showed that type of CHD and level of education were significantly and independently related to job participation (odds ratio, 4.8; 99% confidence interval, 1.2-19.6; and odds ratio, 4.7; 99% confidence interval, 1.3-17.2, respectively). Of the 76 patients with complex CHD, 42 (55%) experienced disease-related career problems, in contrast to only 1 patient with mild CHD. Both CHD groups had more job-related mobility handicaps than did the reference group. However, in the mild CHD group, handicaps could be attributed to additional noncardiac diseases. CONCLUSIONS: Patients with complex CHD have reduced job participation compared with patients with mild CHD and the general population. Many receive disability benefits or experience career problems or job handicaps. Career counseling focusing on physical abilities and level of education may help prevent or reduce these job-related problems.

Validation and reproducibility of aortic pulse wave velocity as assessed with velocity-encoded MRI.

PURPOSE: To validate magnetic resonance imaging (MRI) assessment of aortic pulse wave velocity (PWV(MRI)) with PWV determined from invasive intra-aortic pressure measurements (PWV(INV)) and to test the reproducibility of the measurement by MRI. MATERIALS AND METHODS: PWV(MRI) was compared with PWV(INV) in 18 nonconsecutive patients scheduled for catheterization for suspected coronary artery disease. Reproducibility of PWV(MRI) was tested in 10 healthy volunteers who underwent repeated measurement of PWV(MRI) at a single occasion. Velocity-encoded MRI was performed on all participants to assess PWV(MRI) in the total aorta (Ao(total)), the proximal aorta (Ao(prox)), and the distal aorta (Ao(dist)). RESULTS: The results are expressed as mean +/- SD, Pearson correlation coefficient (PCC), and intraclass correlation (ICC). Good agreement between PWV(MRI) and PWV(INV) was found for Ao(total) (6.5 +/- 1.1 m/s vs. 6.1 +/- 0.8 m/s; PCC = 0.53), Ao(prox) (6.5 +/- 1.3 m/s vs. 6.2 +/- 1.1 m/s; PCC = 0.69), and for Ao(dist) (6.9 +/- 1.1 m/s vs. 6.1 +/- 1.0 m/s; PCC = 0.71). Reproducibility of PWV(MRI) was high for Ao(total) (4.3 +/- 0.5 m/s vs. 4.6 +/- 0.7 m/s; ICC = 0.90, P < 0.01), Ao(prox) (4.3 +/- 0.9 m/s vs. 4.7 +/- 1.0 m/s; ICC = 0.87, P < 0.01), and Ao(dist) (4.3 +/- 0.6 m/s vs. 4.4 +/- 0.8 m/s; ICC = 0.92, P < 0.01). CONCLUSION: MRI assessment of aortic pulse wave velocity shows good agreement with invasive pressure measurements and can be determined with high reproducibility.