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Objectives: To investigate the age at menarche, the prevalence of menstrual cycle (interval) disorders, and determinants in women with congenital heart disease (CHD)., Design: Using two CHD registries, 1802 (82%) of the 2196 women with CHD contacted (aged 18-58 years) provided written informed consent. After exclusion of patients with genetic disorders known to be associated with menstrual cycle disorders, 1593 eligible patients remained. Interviews by telephone and reviews of medical records were conducted., Results: Overall, the age at menarche was slightly increased in women with CHD (13.3 vs. 13.1 years in the general population), mainly attributable to an increased prevalence of primary amenorrhea (n = 147; 9.2%). Other menstrual cycle disorders were documented: secondary amenorrhea (n = 181, 11.4%), polymenorrhea (n = 103, 6.5%), oligomenorrhea (n = 90, 5.6%), and menorrhagia (n = 117, 6.5%). The occurrence of these disorders also depended on the presence of cyanotic heart disease, surgical status, the number of surgical interventions, and the severity of CHD., Discussion: Menstrual cycle disturbances, in particular primary amenorrhea, were frequently observed in this population. Patients with complex (cyanotic) heart disease needing repeated surgical interventions prior to menarche are especially at risk.

Background: Pregnancy in women with congenital aortic stenosis (AS) is associated with increased cardiac complications. Data on non-cardiac complications are limited, and this information is crucial for prenatal counselling and perinatal care. The aim of this study was to present the maternal and perinatal outcome of pregnancy in women with congenital AS., Methods: By review of the Dutch CONCOR national registry and a local Belgian tertiary care centre database, 35 women with congenital AS with a history of completed pregnancy before aortic valve replacement were enrolled in this study. Medical history and maternal and perinatal outcome were determined., Results: Thirty-five women had 58 pregnancies resulting in 53 successful pregnancies, three miscarriages, and two abortions. The most serious cardiac complications were heart failure (n=2, 3.8%) and atrial arrhythmia (n=3, 5.7%). Although cardiac complications were present (9.4%), obstetric (22.6%) and perinatal (24.5%) complications were observed more often. A total of six pregnancies (11.3%) were complicated by hypertension-related disorders, including one case of eclampsia. Furthermore, 7 premature births (13.2%) and 7 small-for-gestational-age births (13.2%) were encountered. Pregnancy in women with severe AS was characterized by an increased incidence of heart failure and premature labour, and shorter pregnancy duration. Older women (>30 years) were at increased risk of perinatal events (odds ratio 4.38, 95% confidence interval 1.02 to 18.81)., Conclusions: Pregnancy is generally well tolerated in women with congenital AS. Importantly, an excess of obstetric and perinatal complications was found, requiring more meticulous attention.

The study aimed to assess whether the 12-lead ECG-derived ventricular gradient, a vectorial representation of ventricular action potential duration heterogeneity directed toward the area of shortest action potential duration, can improve ECG diagnosis of chronic right ventricular (RV) pressure load. ECGs from 72 pulmonary arterial hypertension patients recorded <30 days before onset of therapy were compared with ECGs from matched healthy control subjects (n = 144). Conventional ECG criteria for increased RV pressure load were compared with the ventricular gradient. In 38 patients a cardiac magnetic resonance (CMR) study had been performed within 24 h of the ECG. By multivariable analysis, combined use of conventional ECG parameters (rsr' or rsR' in V1, R/S > 1 with R > 0.5 mV in V1, and QRS axis >90 degrees ) had a sensitivity of 89% and a specificity of 93% for presence of chronic RV pressure load. However, the ventricular gradient not only had a higher diagnostic accuracy for chronic RV pressure load by receiver operating characteristic analysis [areas under the curve (AUC) = 0.993, SE 0.004 vs. AUC = 0.945, SE 0.021, P < 0.05], but also discriminated between mild-to-moderate and severe RV pressure load. CMR identified an inverse relation between the ventricular gradient and RV mass, and a trend toward a similar relation with RV volume. In conclusion, chronically increased RV pressure load is electrocardiographically reflected by an altered ventricular gradient associated with RV remodeling-related changes in ventricular action potential duration heterogeneity. The use of the ventricular gradient allows ECG detection of even mildly increased RV pressure load.

Objective: To assess the effect of pulmonary valve replacement (PVR) on the repolarization of patients with tetralogy of Fallot., Background: Pulmonary valve regurgitation may cause right ventricular failure in adult patients with Fallot's tetralogy. In these patients, prolonged depolarization and disturbed repolarization are associated with ventricular arrhythmias and sudden cardiac death., Methods: Thirty Fallot patients (age 32+/-9 years, 19 male) eligible for PVR were studied with cardiac magnetic resonance imaging (CMR) before and 6 months after PVR. Electrocardiograms obtained during initial and follow-up CMR were analyzed and occurrence of ventricular arrhythmias was studied., Results: Right ventricular end-diastolic volume (RV EDV) decreased from 322+/-87 to 215+/-57 ml after PVR (P<0.0001). The spatial QRS-T angle normalized from 117+/-34 to 100+/-35 degrees , P=0.0004 (normal angle <105 degrees). QT dispersion and T-wave complexity did not change significantly. T-wave amplitude decreased from 376+/-121 to 329+/-100 microV (P=0.01). T-wave area decreased from 43+/-15 to 38+/-13 microV s (P=0.02). Decreases in T-wave amplitude and area were most prominent in the right precordial leads overlying the RV. Three patients had sustained ventricular arrhythmias and one patient died suddenly. These patients had a QRS duration >160 ms. No severe ventricular arrhythmias were found in patients with a RV EDV <220 ml, QRS-T angle <100 degrees , QT dispersion <60 ms or T-wave complexity <0.30., Conclusion: Normal repolarization indices may be associated with the absence of severe ventricular arrhythmias. PVR in Fallot patients with dilated right ventricles has a beneficial effect on electrocardiographic indices of repolarization heterogeneity.

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., ((c) RSNA, 2007.)

Objectives: To investigate outcome of pregnancy and fertility in women with double outlet right ventricle (DORV)., Methods: Using 2 congenital heart disease registries, 21 female patients with DORV (aged 18-39 years) were retrospectively identified. Detailed recordings of each patient and their completed (>20 weeks gestation) pregnancies were recorded., Results: Overall, 10 patients had 19 pregnancies, including 3 spontaneous miscarriages (16%). During the 16 live birth pregnancies, primarily (serious) noncardiac complications were observed, e.g. premature labor/delivery (n = 7 and n = 3, respectively), small for gestational age (n = 4), preeclampsia (n = 2) and recurrence of congenital heart disease (n = 2). Except for postpartum endocarditis and deterioration of subpulmonary obstruction, only mild cardiac complication pregnancies were recorded. Two women with children reported secondary female infertility. Several menstrual cycle disorders were reported: secondary amenorrhea (n = 4), primary amenorrhea (n = 3) and oligomenorrhea (n = 2)., Conclusion: Successful pregnancy in women with DORV is possible. Primarily noncardiac complications were observed and only few (minor) cardiac complications. Infertility and menstrual cycle disorders appear to be more prevalent.

Aims: To investigate outcome and complications of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (CHD) and to identify predictors of (in-) appropriate shocks., Methods and Results: Sixty-four CHD patients >/= 18 years at first ICD implantation [63% tetralogy of Fallot (TOF) and age at implantation 37 +/- 13 years] were identified using the Dutch adult CHD registry and a Belgian tertiary care centre database. Median follow-up duration was 3.7 years. Early complications included pocket haematoma (n = 3), lead failure (n = 2), and pneumothorax (n = 2). Late complications occurred in 11 (17%) patients, including lead failure (n = 6) and and electrical storm (n = 3). Overall, 30 device-related re-interventions were performed in 20 patients (31%), including four premature generator changes and seven lead replacements. Half of the patients received one or more shocks, and 46 shocks in 15 patients (23%) were classified as appropriate. One hundred and sixty shocks in 26 patients (41%) were classified as inappropriate. No predictors of (in-)appropriate shocks were identified, except TOF being associated with less appropriate shocks than patients with other CHD (HR 0.29, P = 0.02)., Conclusion: The ICD provided effective therapy in a quarter of adults with CHD with low complication rates. The incidence of inappropriate shocks, however, appeared to be excessive and warrants further attention.

The study aim was to assess three-dimensional electrocardiogram (ECG) changes during development of pulmonary arterial hypertension (PAH). PAH was induced in male Wistar rats (n = 23) using monocrotaline (MCT; 40 mg/kg sc). Untreated healthy rats served as controls (n = 5). ECGs were recorded with an orthogonal three-lead system on days 0, 14, and 25 and analyzed with dedicated computer software. In addition, left ventricular (LV)-to-right ventricular (RV) fractional shortening ratio was determined using echocardiography. Invasively measured RV systolic pressure was 49 (SD 10) mmHg on day 14 and 64 (SD 10) mmHg on day 25 vs. 25 (SD 2) mmHg in controls (both P < 0.001). Baseline ECGs of controls and MCT rats were similar, and ECGs of controls did not change over time. In MCT rats, ECG changes were already present on day 14 but more explicit on day 25: increased RV electromotive forces decreased mean QRS-vector magnitude and changed QRS-axis orientation. Important changes in action potential duration distribution and repolarization sequence were reflected by a decreased spatial ventricular gradient magnitude and increased QRS-T spatial angle. On day 25, LV-to-RV fractional shortening ratio was increased, and RV hypertrophy was found, but not on day 14. In conclusion, developing PAH is characterized by early ECG changes preceding RV hypertrophy, whereas severe PAH is marked by profound ECG changes associated with anatomical and functional changes in the RV. Three-dimensional ECG analysis appears to be very sensitive to early changes in RV afterload.

Background and Aim of the Study: The pulmonary autograft has been recommended as the valve of choice for aortic valve replacement (AVR) in young women contemplating pregnancy. However, current information on maternal and perinatal outcome of pregnancy in women with pulmonary autograft valve replacement is limited., Methods: Using a nationwide Dutch registry (CONCOR) and a local Belgian tertiary care center database, 17 women (age range: 18 to 45 years) with pulmonary autograft valve replacement were enrolled into the study. Twelve pregnancies were observed among five different women, including one miscarriage and one elective abortion., Results: Clinically significant (non-)cardiac complications were documented in two of 10 completed pregnancies. Complications included: (i) placental abruption necessitating Cesarean delivery at 29 weeks' gestation, further complicated by postpartum hemorrhage; and (ii) preterm premature rupture of the membranes resulting in premature delivery at 29 weeks' gestation with postpartum demise of the immature born child. Two women reported primary female infertility, but both became pregnant after hormonal substitution therapy. Four women reported irregularities of their natural menstrual cycle (menorrhagia, dysmenorrhea, polymenorrhea, oligomenorrhea, or amenorrhea)., Conclusion: Successful pregnancy in women with pulmonary autograft valve replacement is possible, although serious and clinically significant events occurred during gestation. Infertility and menstrual cycle disorders appear to be more prevalent.

A search of peer-reviewed literature was conducted to identify reports that provide data on complications associated with pregnancy in women with structural congenital heart disease (CHD). This review describes the outcome of 2,491 pregnancies, including 377 miscarriages (15%) and 114 elective abortions (5%). Important cardiac complications were seen in 11% of the pregnancies. Obstetric complications do not appear to be more prevalent. In complex CHD, premature delivery rates are high, and more children are small for gestational age. The offspring mortality was high throughout the spectrum and was related to the relatively high rate of premature delivery and recurrence of CHD.

Objective: To analyse the long-term course of QRS duration after pulmonary valve replacement in patients with a previous correction for tetralogy of Fallot., Setting: Tertiary referral centres., Methods: In a retrospective study, 99 adult patients with tetralogy of Fallot, who had undergone a first pulmonary valve replacement late after initial total correction, were identified from the CONCOR (CONgenital CORvitia) registry. Computer-generated QRS durations were obtained from 12-lead electrocardiogram ECG reports in the medical records. A mixed linear regression model was used to analyse the course of QRS duration over time and to identify risk factors for increase in QRS duration over time. Composite end point was created from sudden cardiac death, ventricular tachycardia or implantable cardioverter-defibrillator discharge., Results: In total, 99 patients (57% men, mean (SD) age at pulmonary valve replacement 29 (11) years) with a median follow-up of 4.9 (0.1-16) years were analysed. In patients with preoperative QRS <120 ms, surgery caused no significant change in QRS duration (increase 1.3 (7.9) ms; p = 0.65), and after surgery, QRS duration remained stable over time (increase 0.0064 (0.059) ms/year; p = 0.98). By contrast, in patients with a preoperative QRS of 150-180 ms or QRS > or =180 ms, surgery resulted in QRS shortening (mean decrease 9.9 (SE 4.3) ms, p = 0.021, and 12.2 (SE 2) ms; p<0.001, respectively). During follow-up, a QRS widening 1.1(1.3) ms/year (p<0.001) in both groups was observed. In patients with a preoperative QRS > or =180 ms, no significant difference was observed in the number of patients reaching the composite end point compared with patients with a preoperative QRS of 150-180 ms (25% vs 7%; p = 0.08). However, the former more often reached QRS > or =180 ms again after surgery compared with the latter (53% vs 13%; p = 0.02, respectively). None of the patients with a preoperative QRS > or =180 ms died during follow-up., Conclusion: In our study, we observed a decrease in QRS duration directly after surgery, followed by a steady increase, in patients with a preoperative QRS >150 ms. The beneficial effect of pulmonary valve replacement on QRS duration was transient. The risk of developing ventricular arrhythmias after surgery was substantial when preoperative QRS was > or =180 ms, but mortality remained low.

Background: Predicting changes in right ventricular (RV) size and function after pulmonary valve replacement (PVR) is important for timely reintervention in adult tetralogy of Fallot patients., Methods: We analyzed the influence of pulmonary regurgitation severity and RV size and function before PVR on the outcome of RV size and function after PVR in 27 adult Fallot patients who had cardiac magnetic resonance imaging before and after PVR. RV dimensions were indexed for body surface area., Results: Pulmonary regurgitation (48% +/- 11% of RV stroke volume) was not related to RV dimensions and function before PVR. Moreover, severity of pulmonary regurgitation did not influence changes in RV dimensions after PVR. The indexed RV end-systolic volume before PVR (mean, 98 mL/m2; range, 52 to 235 mL/m2) best predicted the indexed RV end-systolic volume after PVR (mean, 59 mL/m2; range, 24 to 132 mL/m2, r = 0.78, p < 0.001) and the indexed RV end-diastolic volume after PVR (mean, 107 mL/m2; range, 70 to 170 mL/m2, r = 0.73, p < 0.001). Baseline RV ejection fraction corrected for valvular insufficiencies and shunting (21% +/- 7%) best predicted the RV ejection fraction after PVR (43% +/- 10%, r = 0.77, p < 0.001)., Conclusions: Timing of PVR should be based on indexed RV end-systolic volume and corrected RV ejection fraction rather than on severity of pulmonary regurgitation.

The aim was to validate a magnetic resonance high-resolution, phase-contrast sequence for quantifying flow in small and large vessels and to demonstrate its feasibility to measure flow in coronary artery bypass grafts. A breathhold, echo planar imaging (EPI) sequence was developed and validated in a flow phantom using a fast field echo (FFE) sequence as reference. In 17 volunteers aortic flow was measured using both sequences. In 5 patients flow in the left internal mammary artery (LIMA) and aorta was measured at rest and during adenosine stress, and coronary flow reserve (CFR) was calculated; in 7 patients, vein graft flow velocity was measured. In the flow phantom measurements, the EPI sequence yielded an excellent correlation with the FFE sequence (r = 0.99; p < 0.001 for all parameters). In healthy volunteers, aortic volume flow correlated well (r = 0.88; p < 0.01), with a minor overestimation. It was feasible to measure flow velocity in the LIMA and vein grafts of the 12 patients. The high-resolution, breathhold cardiovascular magnetic resonance velocity-encoded sequence correlated well with a free-breathing, FFE sequence in a flow phantom and in the aortae of healthy volunteers. Using the EPI sequence, it is feasible to measure flow velocity in both LIMA and vein grafts, and in the aorta.

Background: Information on the outcome of pregnancy in patients with pulmonary valvar stenosis is scarce, mostly limited to cardiac complications observed during pregnancy., Objectives: To investigate the magnitude and determinants of non-cardiac and fetal risks during pregnancy of women with isolated pulmonary valvar stenosis., Methods: Using the nationwide registry (CONgenital CORvitia), 106 women with (un-)corrected pulmonary valvar stenosis receiving care in six tertiary medical centres in The Netherlands were included. A total of 51 women had 108 pregnancies, including 21 (19%) miscarriages and 6 elective abortions., Results: In the 81 completed (>20 weeks of gestation) pregnancies, we observed a high number of hypertension-related disorders (n = 12, 15%, including pre-eclampsia (n = 4) and eclampsia (n = 2)), premature deliveries (n = 14, 17%, including one twin) and thromboembolic events (n = 3, 3.7%). Furthermore, recurrence of congenital heart defects in the offspring was detected in three children (3.7%, pulmonary valvar stenosis (n = 2) and complete transposition of the great arteries in combination with anencephaly). In addition to the intrauterine fetal demise of the transposition child, three other children died shortly after birth owing to immaturity, hydrocephalus combined with prematurity and meningitis (overall offspring mortality, 4.8%)., Conclusion: In this largest report on pregnancy in women with (un-) corrected isolated pulmonary valvar stenosis, an excessive number of (serious) non-cardiac complications and mortality were observed in the offspring.

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.

Objectives: To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders., Design and Patients: Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18-45 years): atriopulmonary anastomosis (n = 23), atrioventricular connection (n = 5) and total cavopulmonary connection (n = 10)., Results: Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live-birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non-specified secondary infertility (n = 2), uterus bicornis (n = 1) and related to endometriosis (n = 1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n = 15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population)., Conclusion: Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.

Aims: To compare multidetector row computed tomography (MDCT) global and regional left ventricular (LV) function assessment with echocardiography and cardiovascular magnetic resonance (CMR)., Methods and Results: In 25 patients, who were referred for noninvasive angiography with 16-detector row CT, LV function assessment was also performed. A subsequent echocardiogram was performed, and in a subgroup of patients, CMR examination was completed to evaluate LV function. For global function assessment, the LV ejection fraction (LVEF) was calculated. Regional LV function was scored using a 17-segment model and a 4-point scoring system. MDCT agreed well with echocardiography for the assessment of LVEF (r=0.96; bias 0.54%; p<0.0001) and regional LV function (kappa=0.78). Eight patients had no contra-indications and gave informed consent for CMR examination. A fair correlation between MDCT and CMR was demonstrated in the assessment of LVEF (r=0.86; bias -1.5%; p<0.01). Regional LV function agreement between MDCT and CMR was good (kappa=0.86)., Conclusion: MDCT agreed well with both echocardiography and CMR in the assessment of global and regional LV function. Global and regional LV function may accurately be evaluated by 16-detector row CT, and can be added to a routine CT image analysis protocol without need for additional contrast or imaging time.

The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34 years) had biventricular repair for PAIVS. Three PAIVS patients had a total of 5 pregnancies, including 1 abortion and 1 ectopic pregnancy. Besides minor noncardiac complications, the 3 live-birth pregnancies were successful. None of the women reported infertility or irregularities (hypermenorrhea, dysmenorrhea, polymenorrhoea, oligomenorrhea, or amenorrhea) of their natural menstrual cycle. In conclusion, successful pregnancy in women with biventricular repair for PAIVS is possible, and only minor complications were observed. Infertility and menstrual cycle disorders do not appear to be more prevalent than usual.

Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD (aged 21 to 38 years) were identified. Ten pregnancies were observed in 5 different women with PAVSD, including 3 spontaneous miscarriages. Clinically significant (non)cardiac complications were documented in 3 of 7 completed pregnancies. These complications were: (1) atrioventricular reentry tachycardia with symptomatic right-sided heart failure; (2) eclampsia with hemolysis, elevated liver enzymes, and low platelets syndrome further complicated by abruptio placentae leading to premature delivery of a small-for-gestational-age child; and (3) premature delivery due to cervical insufficiency with antepartum demise of an immature child. Furthermore, none of the women reported infertility. Moreover, none of the women reported irregularities of their natural menstrual cycle (age at menarche 13 years; cycle duration 28 days), with the exception of delayed menarche (>16 years) in 2 patients. In conclusion, successful pregnancy in patients with biventricular repair of PAVSD is possible, although often complicated by serious clinically significant events. Infertility and menstrual cycle disorders do not appear to be more prevalent, except for a high incidence of primary amenorrhea.

Aims: To analyse the long-term outcomes after pulmonary valve replacement (PVR) in patients with a previous correction for tetralogy of Fallot., Methods and Results: In a retrospective study, 158 adult patients with a diagnosis of tetralogy of Fallot, who had undergone a PVR after initial total correction in childhood, were identified from the CONCOR (CONgenital CORvitia) registry. All patients underwent 175 PVRs between June 1986 and June 2005. To analyse the predictors for homograft dysfunction and adverse events (death, reoperations, balloon angioplasty), Cox-regression analysis was performed. Overall freedom from significant homograft dysfunction was 66% after 5 years and 47% after 10 years. We could not identify predictors for combined homograft dysfunction. Event-free survival was 78% at 10 years and 68% at 15 years after PVR. Both early significant pulmonary regurgitation (PR) (HR 6.8, P = 0.017) and pulmonary stenosis (PS) (HR 4.0, P = 0.037) after surgery were associated with adverse events. When analysing direct post-operative PR or PS, we observed that in patients with severe, pre-operative PR, right ventricular aneurysm/patch resection resulted in a lower post-operative PR (mean difference grade 0.38 +/- 0.14, P = 0.01). Less significant post-operative PS was associated with a higher diameter of the homograft (HR 0.37, P = 0.006)., Conclusion: While 47% of the patients in our study were free from homograft dysfunction at 10 years after PVR, event-free survival after PVR remained fairly good (78%). Significant residual lesions directly after surgery influenced event-free survival. A smaller diameter of the pulmonary homograft and severe pre-surgical PR were related to early homograft dysfunction after surgery.

The aim of this study was to analyze the effect of pressure and/or volume overload on right ventricular (RV) function and brain natriuretic peptide (BNP) levels in patients with surgically corrected congenital heart disease. Forty-two consecutive patients aged 17 to 57 years (median 30) with congenital heart disease (32 with tetralogy of Fallot and 10 with pulmonary stenosis) were examined. The RV systolic pressure was estimated using Doppler echocardiography. Cardiovascular magnetic resonance imaging was used to obtain the RV volumes, ejection fraction (EF) and corrected EF (cEF). Plasma BNP levels were determined by immunoradiometric assay. Patients were categorized as having volume overload when pulmonary regurgitation was > or =10% and pressure overload when the RV systolic pressure was >40 mm Hg. Patients with RV volume overload had a lower RVEF compared with patients with pressure overload (p = 0.02) and lower left ventricular EF (p <0.001). BNP was higher in patients with volume overload than in patients with pressure overload (p = 0.002). BNP correlated with pulmonary regurgitation, RVEF, RV cEF, and left ventricular EF. In linear regression analysis, RV cEF was an independent predictor for BNP, after adjustment for age. Without the parameter of RV cEF in the regression model, pulmonary regurgitation and RVEF were independently associated with BNP level, after adjustment for age. In conclusion, patients with RV volume overload had higher BNP levels and lower RV function than patients with RV pressure overload. BNP levels were independently associated with the degree of RV volume overload and RV function.

Cardiovascular magnetic resonance (CMR) is becoming an important tool in the clinical management of patients with congenital heart disease. Because of the diverse problems patients may face after initial correction for tetralogy of Fallot and the large amount of CMR techniques that can be applied, creating a patient-orientated imaging protocol is a difficult issue. Although it is still not certain what the impact of some parameters, provided by CMR, should be on clinical decision making, new techniques are being developed and applied. In this report, we review the current clinical issues in patients with tetralogy of Fallot and review the current implication and limitations of CMR in this patient category.

A 47-year-old woman was referred to our hospital for an intracardiac electrophysiological study. The procedure was complicated by cardiac arrest due to massive pulmonary embolism, which only successfully resolved after the administration of a thrombolytic agent. This case suggests that there is a real risk of venous thromboembolism in certain patients during electrophysiological studies. Therefore, a consensus should be reached concerning the prevention of venous thromboembolism during these procedures.

Purpose: To evaluate retrospectively the presence of fibrosis and largest diameter of the right ventricular outflow tract (RVOT) by using delayed enhancement magnetic resonance (MR) imaging in patients who had undergone initial correction for tetralogy of Fallot., Materials and Methods: MR imaging was performed in 24 consecutive patients (16 male, eight female; mean age, 25 years; age range, 13-47 years) with corrected tetralogy of Fallot. The study protocol was approved by the local ethics committee, and informed consent was not required. Fifteen minutes after injection of 0.2 mmol/kg gadopentetate dimeglumine, an inversion-recovery turbo field-echo sequence was applied for detection of delayed enhancement. Right ventricular volumes, ejection fraction, and anterior-posterior diameter of the RVOT were calculated. Mann-Whitney nonparametric testing was used to compare measurements of ventricular volume, function, and anterior-posterior diameter of the RVOT in the presence or absence of delayed enhancement. Correlation was tested with Pearson coefficient., Results: Delayed enhancement was seen in 17 patients in the RVOT. During initial surgery, transannular patching was performed in 13 (76%) of 17 patients, RVOT patching in one (6%) of 17 patients, and the Brock procedure in two (12%) of 17 patients. In one patient, the type of initial RVOT repair was unknown. Patients with delayed enhancement in the RVOT, as compared with those without delayed enhancement in the RVOT, had increased RVOT diameter (32 mm +/- 7 [standard deviation] vs 22 mm +/- 3, P < .01), decreased right ventricular ejection fraction (43% +/- 6.3 vs 54% +/- 10, P < .001), and increased end-diastolic volume (175 mL/m2 +/- 42 vs 118 mL/m2 +/- 34, P < .01). The diameter of the RVOT correlated with increased right ventricular end-systolic volume (R = 0.86) and was inversely related to ejection fraction (R = -0.65)., Conclusion: Delayed enhancement occurs frequently in patients after correction for tetralogy of Fallot. Delayed enhancement in the RVOT was associated with RVOT dilatation, which adversely affects right ventricular hemodynamics., (RSNA, 2005)

Purpose: To prospectively assess regional systemic right ventricular (RV) function at rest and in response to exercise by using magnetic resonance (MR) imaging in patients who have undergone surgical correction at the atrial level for transposition of the great arteries (TGA)., Materials and Methods: Informed consent was obtained, and the medical review board approved this study. In 25 adult patients (mean age, 25.8 years +/- 4.7 [standard deviation]; 13 men) who had undergone correction for TGA (23.4 years +/- 4.9 after surgery) and 11 healthy volunteers (mean age, 27.4 years +/- 2.7; six men), systemic ventricular function was assessed with MR imaging (turbo field echo-planar imaging) at rest and during supine bicycle exercise. Regional wall thickness and wall thickening of the systemic RV were assessed and compared with those of the left ventricle in healthy volunteers by two investigators working together. Regional wall parameters were calculated by using the three-dimensional centerline method. Independent-samples t test and paired-samples t test were used for statistical analysis., Results: Ejection fraction of the systemic RV did not increase after exercise (56% +/- 8 at rest to 55% +/- 7 after exercise, P = .196). Mean RV wall thickening was impaired in patients with TGA at all levels both at rest and in response to exercise (P < .05). Moreover, the free wall and the anterior wall of the systemic RV had a smaller end-systolic thickness and a diminished thickening at rest and after exercise compared with findings in the normal left ventricle (P < .05)., Conclusion: The systemic RV of patients after intraatrial correction for TGA reveals regional functional disturbances at rest and in response to exercise., (RSNA, 2005)

Aims: To investigate magnitude and determinants of risks during pregnancy in women with Mustard or Senning repair for complete transposition of the great arteries (TGA)., Methods and Results: Using a nationwide registry (CONCOR), 70 women with Senning (23%) or Mustard (77%) repair for TGA were enrolled. A total of 28 patients had 69 pregnancies (two twins), including 17 spontaneous miscarriages and three elective abortions. During 39 of the 49 completed pregnancies, complications were observed. The most important cardiac complication was clinically significant arrhythmia (n=11, 22%), especially occurring in patients with a prior history of arrhythmia. Important general pregnancy complications were preeclampsia (n=5, 10.2%) and pregnancy-induced hypertension (n=4, 8.2%). Obstetric complications included premature rupture of membranes (n=7, 14.3%), premature labour (n=12, 24.4%), premature delivery (n=16, 31.4%), and thrombo-embolic complications (n=2, 4.1%). Mean (singleton) pregnancy duration was 36+/-5 weeks. Eleven of the 51 children (21.6%) were small for gestational age. Foetal and neonatal mortality combined was 11.8% (n=6). No recurrence of congenital heart disease in the offspring was documented., Conclusion: In this largest report on pregnancy in women with atrial-corrected TGA to date, a high incidence of obstetric complications and mortality in the offspring was observed.

Objectives: The aim of this research was to assess right ventricular diastolic and systolic function before and after pulmonary valve replacement (PVR) in adult patients after repair of tetralogy of Fallot., Background: Pulmonary valve replacement (PVR) in adult patients late after repair of tetralogy of Fallot leads to rapid improvement of right ventricular (RV) systolic function., Methods: A total of 16 patients and 8 healthy subjects were included. Median age at initial repair was 4.9 (0.9 to 13.1) years, and mean age at PVR was 28.7 (19.5 to 45.6) years. Cardiac magnetic resonance imaging was performed before and 8 and 22 months after PVR. Right ventricular volumes and function as well as RV in- and outflow patterns were assessed., Results: The volume of the early filling of the RV (Evol) increased from 49.8 +/- 14.7 ml to 53.8 +/- 19.3 ml (not significant) and 62.0 +/- 18.9 ml, respectively (p < 0.05), whereas the volume of the atrial contraction (Avol) remained unchanged. Consequently, the Evol/Avol ratio increased from 1.4 +/- 0.7 before PVR to 1.6 +/- 0.7 at 8 months (not significant) and 2.3 +/- 1.2 at 22 months (p < 0.01). The Evol/Avol ratio was not significantly different from the healthy subjects at 22 months, indicating late recovery of diastolic function. Systolic function improved rapidly after PVR; the indexed RV end-systolic volume decreased from 93.7 +/- 33.0 ml/m2 to 60.9 +/- 18.4 ml/m2 (p < 0.01) and 54.8 +/- 21.0 ml/m2 (p < 0.01)., Conclusions: In adult patients late after total repair of Fallot, PVR leads to late improvement of diastolic function. We speculate that the rapid volume unloading after PVR increases systolic performance, whereas improvement in diastolic function requires long-term remodeling.

Background: Multidetector-row computed tomography (MDCT) is a versatile modality to evaluate stenoses in native coronary arteries and bypass grafts. Acquired MDCT data can additionally be used to assess left ventricular ejection fraction (LVEF). The purpose was to use MDCT for the assessment of bypass graft and coronary artery disease combined with evaluation of LVEF., Methods: Twenty-five patients underwent 16-detector-row CT examination and coronary angiography. Bypass grafts and nongrafted coronary artery segments at MDCT were evaluated on eligibility, patency, and > or = 50% stenosis. The MDCT data set was used to calculate LVEF and was divided into patients with no/subendocardial/transmural myocardial infarctions (MIs)., Results: Ninety vessels were evaluated: 14 arterial grafts/53 vein grafts/23 nongrafted vessels. Of 225 segments, 17 were ineligible for evaluation because of metal clips. With MDCT, patency in segments of arterial grafts/vein grafts/nongrafted vessels could be evaluated with high accuracy in 100%/100%/97% of segments. In arterial grafts, stenoses > or = 50% did not occur at angiography, which was for all eligible segments correctly diagnosed at MDCT. Stenosis > or = 50% could be correctly detected by MDCT with a sensitivity/specificity of 100%/94% for vein grafts and 100%/89% for nongrafted vessels. Negative predictive value was 100% for vein grafts and nongrafted vessels. In patients with transmural MI, MDCT revealed a significant lower LVEF as compared with patients without or with subendocardial MI (P < .05)., Conclusion: Comprehensive assessment of bypass grafts, nongrafted vessels, and LVEF is feasible with MDCT. Owing to the high negative predictive value this noninvasive approach may be used as gatekeeper before coronary angiography.

Background: Coronary angiography is considered the gold standard in evaluating vein graft disease; however, angiography does not allow assessment of hemodynamic consequences of lesions. In this study hemodynamic consequences of significant stenoses in vein grafts were evaluated by Doppler velocity assessment, and results were compared with single photon emission computed tomography (SPECT) perfusion imaging., Methods and Results: Angiography was performed in 58 patients after coronary artery bypass grafting because of recurrent chest pain. During the procedure, Doppler velocity measurements were acquired before and after administration of adenosine. Of 58 patients (with 78 vein grafts), 20 patients (with 24 vein grafts) underwent SPECT perfusion imaging. Grafts were divided into those with nonsignificant percent diameter stenosis (< 50%) (n = 49) and those with significant percent diameter stenosis (> or =50%) (n = 29). When a cutoff value for coronary flow velocity reserve (CFVR) of 1.8 was applied, modest agreement (69%, kappa = 0.25, P < .05) between CFVR and angiography was shown. Agreement between SPECT and angiography was also modest (63%, kappa = 0.28, P = not significant). SPECT and CFVR provided comparable information in 20 of 24 grafts with available SPECT, illustrating good agreement (83%, kappa = 0.61, P = .001)., Conclusions: Significant stenoses in vein grafts require further exploration to assess their hemodynamic significance. The Doppler velocity results agreed better with SPECT perfusion imaging than with percent diameter stenosis in the evaluation of vein graft function.

Aims: Late after total correction, Fallot patients with a long QRS duration are prone to serious arrhythmias and sudden cardiac death. Pulmonary regurgitation is a common cause of right ventricular (RV) failure and QRS lengthening. We studied the effects of pulmonary valve replacement (PVR) on QRS duration and RV volume., Methods and Results: Twenty-six consecutive Fallot patients were evaluated both pre-operatively and 6-12 months post-operatively by cardiac magnetic resonance (CMR). In this study, we present the computer-assisted analysis of the standard 12-lead electrocardiograms closest in time to the CMR studies. For the whole group, QRS duration shortened by 6+/-8 ms, from 151+/-30 to 144+/-29 ms (P=0.002). QRS duration decreased in 18 of 26 patients by 10+/-6 ms, from 152+/-32 to 142+/-31 ms. QRS duration remained constant or increased slightly in eight of 26 patients by 3+/-3 ms, from 148+/-27 to 151+/-25 ms. CMR showed a decrease in RV end-diastolic volume from 305+/-87 to 210+/-62 mL (P=0.000004). QRS duration changes correlated with RV end-diastolic volume changes (r=0.54, P=0.01)., Conclusion: Our study shows that PVR reduces QRS duration. The amount of QRS reduction is related to the success of the operation, as expressed by the reduction in RV end-diastolic volume.

The aim of the study was to evaluate segmental and global right ventricular function at rest and during stress in patients having a systemic morphologically right ventricle. We studied 17 patients after atrial correction for transposition, 13 with congenitally corrected transposition, and 11 age-matched controls using cardiovascular magnetic resonance at rest and during stress with dobutamine given at 15 micrograms per kilogram per minute. Blood was drawn to obtain levels of brain natriuretic peptide. Right ventricular ejection fraction was calculated, and wall-motion and wall-thickening were assessed, in 4 segments of a midventricular slice. The findings for the systemic right ventricle were compared to the left ventricle in controls. Patients with transposition showed a decreased ejection fraction at rest (57 percent versus 69 percent, p equal to 0.005), decreased wall motion of the anterior, lateral and septal wall (p less than 0.01, p less than 0.01, and p less than 0.01) and decreased thickening of the lateral wall (p less than 0.01). Patients with congenitally corrected transposition showed normal ejection fraction, wall thickening, and wall motion at rest. During dobutamine stress, we found no significant differences in wall motion and thickening between the two groups. Ejection fraction, also increased to comparable values during stress, at 67 percent versus 66 percent, p not being significant. In both groups, we observed similarly increased levels of brain natriuretic peptide (p equal to 0.02 and 0.03, respectively). We conclude that only patients with transposition showed segmental wall motion and wall thickening abnormalities at rest. After dobutamine stress, however, segmental and global right ventricular dysfunction was similar in both groups.

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance.

Because patients with hypertension are at increased risk for coronary artery disease, early and noninvasive identification of the disease in patients with hypertension is important. Recently, multislice computed tomography (MSCT) has been demonstrated to allow both noninvasive coronary angiography and assessment of left ventricular function. The purpose of the present study therefore was to demonstrate the feasibility of this approach in patients with hypertension with known or suspected coronary artery disease and to compare the results to invasive coronary angiography and 2-dimensional echocardiography, respectively. MSCT was performed in 31 patients with confirmed hypertension. From the MSCT images, the presence of significant coronary stenoses (>or=50% luminal narrowing) and regional wall motion abnormalities were evaluated and compared with invasive coronary angiography and 2-dimensional echocardiography. In addition, left ventricular ejection fraction was calculated from the MSCT images. A total of 243 (88%) coronary artery segments could be evaluated with MSCT. Sensitivity and specificity for the detection of significant coronary artery stenoses were 93% and 96%. On a per-patient basis, MSCT was accurate in 28 (90%) patients. Mean left ventricular ejection fraction was 46+/-14% (range, 16% to 64%). The agreement for assessing regional wall motion was 91% (kappa statistic, 0.81). In conclusion, simultaneous, noninvasive evaluation of coronary artery stenoses and left ventricular function with MSCT is accurate in patients with hypertension. This noninvasive approach may allow triage of patient treatment in terms of conservative versus invasive management.

Cardiovascular magnetic resonance (CMR) with flow velocity mapping has emerged as a noninvasive method to measure flow in saphenous vein coronary artery bypass grafts. The aim of the current study was to retrospectively test two previously described analysis methods on a large CMR data set and to compare their diagnostic accuracy in detecting diseased vein grafts. In 125 vein grafts of 68 patients, volume flow parameters (volume flow, systolic and diastolic peak flow, diastolic-to-systolic flow ratio at rest and during adenosine stress, and flow reserve) were derived from the velocity maps. Method 1 implemented basal flow < 20 ml/min or flow reserve < 2, yielding a sensitivity and specificity of 70% and 38% in the detection of a diseased graft or recipient vessel. Method 2 used receiver operating characteristic (ROC) curve analysis and implemented all significant volume flow parameters in a logistic regression model, yielding a sensitivity of 74% with a specificity of 68% in the detection of a diseased graft or recipient vessel. Evaluating single and sequential grafts separately, this method yielded a sensitivity and specificity of 79% and 87% for single grafts, and 62% and 94% for sequential grafts in the detection of > or = 50% stenosis in grafts or recipient vessels. Cut-off values were formulated for the respective volume flow parameters, which maximally separate grafts with and without > or = 50% stenosis. Using ROC curve analysis with logistic regression the specificity of the analysis method improved considerably. For the current data set the best results were acquired when single and sequential grafts were separately analyzed.

Background: In patients with an intra-atrial correction for transposition of the great arteries (TGA) an abnormal response to stress testing is common. However, hemodynamic responses may vary substantially when different stress tests are used. We compared the hemodynamic response to dobutamine stress with the response to physical exercise in patients and controls., Methods: Thirty-nine patients and 25 age/sex-matched control subjects underwent either dobutamine stress (15 microg/kg/min) or submaximal physical exercise cardiovascular magnetic resonance. End-systolic and end-diastolic right ventricular volumes (ESV; EDV) were determined. Five representative patients underwent both stress tests. For these patients, wall thickening reserve was calculated as systolic wall thickening during stress minus systolic wall thickening at rest., Results: In controls, dobutamine stress and physical exercise showed similar responses: stroke volume, cardiac output, and ejection fraction increased significantly, whereas ESV decreased significantly and EDV was unchanged. In patients, stroke volume did not increase with either dobutamine or exercise (-8.6% vs. 2.9%). Ejection fraction increased significantly with dobutamine (16%, p < 0.001) but tended to decrease during exercise (-2.1%, P = NS). EDV and ESV decreased during dobutamine but were unchanged during exercise. (-22% vs. 5.0%, P < 0.001; -36% vs. 9.0%, P < 0.01 respectively). Wall thickening reserve was higher with dobutamine than with exercise (0.9 mm vs. -0.6 mm, P = 0.02)., Conclusion: Dobutamine stress and physical exercise cannot be used interchangeably for assessment of systolic and diastolic function in patients with an intra-atrial correction for TGA. This may have consequences for the use of different stress CMR approaches in the clinical setting.

Purpose: To assess the time course of right ventricular (RV) function improvement after pulmonary valve replacement (PVR) in patients 25.2 years +/- 7.0 after repair of tetralogy of Fallot., Materials and Methods: The medical ethics committee approved this study, and informed consent was obtained. Cardiac magnetic resonance (MR) imaging was performed before, 7 months after, and 19 months after PVR in 25 consecutive patients with tetralogy of Fallot with a 1.5-T MR imager. RV function was assessed with gradient-echo sequences in the short-axis plane. Pulmonary flow was assessed with a velocity-encoded phase-contrast sequence. Paired t test was used to evaluate follow-up data. Independent samples t test was used to assess differences based on the presence of recurrent pulmonary regurgitation (PR)., Results: Mean indexed RV end-diastolic volume decreased from 166.9 mL/m(2) +/- 41.3 before PVR to 113.5 mL/m(2)+/- 35.7 (P < .001) at 7-month follow-up and 111.7 mL/m(2)+/- 41.1 (P = .46) at 19-month follow-up. The RV ejection fraction was corrected for PR and improved from 25.0% +/- 7.7 before surgery to 44.1% +/- 11.9 (P < .001) and 45.2% +/- 11.1 (P = .39), at 7- and 19-month follow-up, respectively. Recurrent PR after PVR was found in 11 patients; 14 patients did not have recurrent PR. Total reduction of indexed RV end-diastolic volume at 19 months follow-up was more prominent in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05). Furthermore, improvement of RV ejection fraction corrected for regurgitation was more marked in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05)., Conclusion: In patients with tetralogy of Fallot, RV function improves rapidly after PVR and is sustained at 19-month follow-up in most patients; however, recurrence of PR after PVR appears to reduce recovery of RV systolic function., ((c) RSNA, 2004.)

Objective: Early identification of coronary artery disease (CAD) in patients with diabetes is important because these patients are at increased risk for CAD and have worse outcome than nondiabetic patients after CAD is diagnosed. Recently, noninvasive coronary angiography and assessment of left ventricular function has been demonstrated with multislice computed tomography (MSCT). The purpose of the present study was to validate this approach in patients with type 2 diabetes., Research Design and Methods: MSCT was performed in 30 patients with confirmed type 2 diabetes. From the MSCT images, coronary artery stenoses (> or =50% luminal narrowing) and left ventricular function (left ventricular ejection fraction, regional wall motion) were evaluated and compared with results of conventional angiography and two-dimensional echocardiography., Results: Two hundred twenty of 256 coronary artery segments (86%) were interpretable with MSCT. In these segments, sensitivity and specificity for detection of coronary artery stenoses were 95%. Including the uninterpretable segments, sensitivity and specificity were 81 and 82%, respectively. Bland-Altman analysis in the comparison of left ventricular ejection fractions demonstrated a mean difference of -0.48 +/- 3.8% for MSCT and echocardiography, which was not significantly different from 0. Agreement between the two modalities for assessment of regional contractile function was excellent (91%, kappa statistic 0.81)., Conclusions: Accurate noninvasive evaluation of both the coronary arteries and left ventricular function with MSCT is feasible in patients with type 2 diabetes. This noninvasive approach may allow optimal identification of high-risk patients.

Objectives: This study was designed to perform a head-to-head comparison between single-photon emission computed tomography (SPECT) and cardiovascular magnetic resonance (CMR) to evaluate hemodynamic significance of angiographic findings in bypass grafts., Background: The hemodynamic significance of a bypass graft stenosis may not always accurately be determined from the coronary angiogram. A variety of diagnostic tests (invasive or noninvasive) can further characterize the hemodynamic consequence of a lesion., Methods: Fifty-seven arterial and vein grafts in 25 patients were evaluated by angiography, SPECT perfusion imaging, and coronary flow velocity reserve determination by CMR. Based on angiography and SPECT, four different groups could be identified: 1) no significant stenosis (<50%), normal perfusion; 2) significant stenosis (>/=50%), abnormal perfusion; 3) significant stenosis, normal perfusion (no hemodynamic significance); and 4) no significant stenosis, abnormal perfusion (suggesting microvascular disease)., Results: A complete evaluation was obtained in 46 grafts. Single-photon emission computed tomography and CMR provided similar information in 37 of 46 grafts (80%), illustrating good agreement (kappa = 0.61, p < 0.001). Eight grafts perfused a territory with scar tissue. When agreement between SPECT and CMR was restricted to grafts without scar tissue, it improved to 84% (kappa = 0.68). Integration of angiography with SPECT categorized 14 lesions in group 1, 23 in group 2, 6 in group 3, and 3 in group 4. Single-photon emission computed tomography and CMR agreement per group was 86%, 78%, 100%, and 33%, respectively., Conclusions: Head-to-head comparison showed good agreement between SPECT and CMR for functional evaluation of bypass grafts. Cardiovascular magnetic resonance may offer an alternative method to SPECT for functional characterization of angiographic lesions.

Forty-nine patients with previous bypass surgery underwent coronary angiography and cardiovascular magnetic resonance (MR) imaging of single-vein bypass grafts. Volume flow and velocity analyses were performed and compared on MR velocity maps. Bland-Altman analysis showed close agreement between the two types of analysis. Comparison of areas under the receiver operating characteristic curve revealed no significant differences between the analyses for detection of stenoses of 70% or greater. Diagnostic accuracy for volume flow and velocity parameters was 92% and 93%, respectively. Velocity analysis appears to be the preferred method, because it is less time-consuming and has a similar diagnostic accuracy to volume flow analysis.

Intracoronary stent implantation is a frequently performed procedure in the treatment of stenoses in coronary arteries, but in-stent restenosis occurs in approximately 10% to 15% of patients. A noninvasive diagnostic procedure to evaluate in-stent restenosis would therefore be of great benefit. We investigated the feasibility of assessing stent patency with 16-slice computed tomography. Multislice computed tomography (MSCT) was performed in 22 patients with previously implanted stents. For each stent, assessability was determined and related to stent type and diameter. Subsequently, the presence of significant restenosis was determined in the evaluable stents. In addition, peristent lumina (5 mm proximal and distal to the stent) were evaluated. Conventional angiography in combination with quantitative coronary angiography served as the standard of reference. MSCT was performed successfully in all but 1 patient. Of 65 stents, 50 (77%) were determined assessable. Uninterpretable stents tended to have a thicker strut and/or a smaller diameter. In the evaluable stents, 7 of 9 stenoses were detected and the absence of restenosis was correctly identified in all 41 patent stents, resulting in a sensitivity and specificity of 78% and 100%, respectively. Sensitivity and specificity for the detection of peristent stenosis were 75% and 96%, respectively. In conclusion, MSCT may be useful in the assessment of stent patency and may function as a gatekeeper before invasive diagnostic procedures.

Objective: To assess with magnetic resonance imaging (MRI) cardiovascular function in response to exercise in patients after atrial correction of transposition of the great arteries (TGA)., Methods: Cardiac function at rest and during submaximal exercise was assessed with MRI in 27 patients with TGA (mean (SD) age 26 (5) years) late (23 (2) years) after atrial correction and in 14 control participants (25 (5) years old)., Results: At rest, only right ventricular ejection fraction was significantly lower in patients than in controls (56 (7)% v 65 (7)%, p < 0.05). In response to exercise, increases in right ventricular end diastolic (155 (55) ml to 163 (57) ml, p < 0.05) and right ventricular end systolic volumes (70 (34) ml to 75 (36) ml, p < 0.05) were observed in patients. Furthermore, right and left ventricular stroke volumes and ejection fraction did not increase significantly in patients. Changes in right ventricular ejection fraction with exercise correlated with diminished exercise capacity (r = 0.43, p < 0.05)., Conclusions: In patients with atrially corrected TGA, MRI showed an abnormal response to exercise of both systemic right and left ventricles. Exercise MRI provides a tool for close monitoring of cardiovascular function in these patients, who are at risk for late death.

This study represents the development and validation of a cardiac-specific module of the generic health-related quality of life (HRQoL) instrument, the TAAQOL (TNO/AZL Adult Quality Of Life), for young adults with congenital heart disease (CHD). Items were selected based on literature, an explorative previous study in CHD patients, interviews with patients, and the advice of experts. The newly developed Congenital Heart Disease-TNO/AZL Adult Quality of Life (CHD-TAAQOL) was tested in 156 patients with mild or complex CHD and consisted of three hypothesised subject scales: 'Symptoms' (9 items), 'Impact Cardiac Surveillance' (7 items), and 'Worries' (10 items). Cronbach's alpha for the three scales were 0.77, 0.78, and 0.82, respectively. Scale structure was confirmed by Principal Component Analysis, corrected item-scale and interscale correlations. Overall, 55% of reported health status problems were associated with negative emotions, which is an argument for assessing HRQoL as a concept distinct from health status. Convergent validity with validated generic instruments (TAAQOL and Short Form-36, SF-36) showed satisfactory coefficients. Discriminant validity was proven by significantly higher scores for mild CHD patients compared with those with complex CHD. In conclusion, the CHD-TAAQOL module together with the generic TAAQOL can be used to assess group differences for cardiac-specific HRQoL in young adults with CHD. Testing psychometric properties of the CHD-TAAQOL shows satisfactory results. However, to detect changes in HRQoL over time, further research is needed.

Growth hormone therapy in patients with idiopathic dilated cardiomyopathy and ischemic cardiac failure has revealed varying effects on systolic function, probably related to the response in serum insulin-like growth factor I (IGF-I) levels. As diastolic function has not been studied thoroughly, we studied the effects of 6 months of recombinant human growth hormone (rh GH) treatment on systolic and diastolic function in patients with ischemic cardiac failure, using cardiovascular magnetic resonance (MR) imaging. Nineteen patients with ischemic cardiac failure (left ventricular ejection fraction (LVEF), <40%) were studied in a randomized trial. Nine patients received 6 months treatment with growth hormone (2 IU/day). Systolic and diastolic function were assessed at baseline and after 26 weeks by cardiovascular MR imaging. No differences were found in systolic and diastolic function between rh GH treated patients and controls. No change was observed in left ventricular mass index (LVMI), end-diastolic volume, end-systolic-volume and ejection fraction. The treated patients showed no clinical improvement. Six months of treatment with growth hormone therapy in ischemic cardiac failure has no favorable effects on LVMI, on systolic and diastolic function.

Purpose: To provide functional reference values in single and sequential vein grafts by using magnetic resonance (MR) flow mapping and to examine the effect of percutaneous intervention (PCI) on coronary artery bypass graft function., Materials and Methods: Fast MR flow mapping at baseline and during adenosine-induced stress was performed in 39 nonstenotic single vein grafts and 20 nonstenotic sequential vein grafts, as well as in 15 stenotic vein grafts before and 7.3 weeks +/- 1.5 after successful PCI. We evaluated the following parameters (in terms of mean values +/- SDs): average peak velocity (APV) at baseline, stress APV, and velocity reserve. Parameters in nonstenotic single and sequential vein grafts were compared by means of unpaired two-tailed Student t testing. To evaluate changes in velocities before and after PCI, a paired two-tailed Student t test was used. P <.05 was considered to indicate a statistically significant difference., Results: Reference values in single vein grafts for baseline APV, stress APV, and velocity reserve were 8.6 cm/sec +/- 3.4, 20.2 cm/sec +/- 9.5, and 2.4 +/- 0.8, respectively. In sequential vein grafts, significantly higher values for baseline APV (12.2 cm/sec +/- 5.0) and stress APV (27.2 cm/sec +/- 10.6) but a similar velocity reserve (2.3 +/- 0.7) were found. Significant improvements were observed after PCI in baseline APV (before PCI: 9.2 cm/sec +/- 6.6; after PCI: 12.9 cm/sec +/- 7.9; P =.008) and stress APV (before PCI: 12.9 cm/sec +/- 6.3; after PCI: 27.1 cm/sec +/- 13.9; P <.001). No improvement in velocity reserve was observed., Conclusion: Significantly higher absolute velocity and flow values were observed in sequential versus single vein grafts, underscoring the need for separate functional reference values for different graft types. Graft function showed significant improvement after PCI to the point that it was restored or nearly restored to reference values.

Purpose: Evaluation of different electrocardiographic criteria for left ventricular hypertrophy (ECG-LVH criteria) using left ventricular mass index (LVMI) determined by magnetic resonance imaging (MRI). In addition, the relation between LVMI regression after aortic valve replacement and corresponding ECG changes regarding LVH was studied., Methods: A group of 31 patients with severe aortic valve disease was studied to assess the presence of ECG-LVH and to measure LVMI and LV end-diastolic volume index (LVEDVI); 13 patients were restudied at 9.8 +/- 2.7 months after aortic valve replacement., Results: Three criteria had a sensitivity of 100% (SV1 + RV5 or RV6 > 3.0 mV; SV1 or SV2 + RV5 > or = 3.5 mV; SV1 or SV2 + RV5 or RV6 > 3.5 mV), at the cost of specificity (50%, 44.4% and 44.4%, respectively). The R in I > 1.4 mV had a specificity of 100%, at the cost of both sensitivity and accuracy (13.6% and 54.5%, respectively). The Romhilt-Estes point score system, using > or = 4 points as cut-off value for LVH, provided the overall best accuracy of 79.5%. The number of Romhilt-Estes points also showed the best correlation with LVMI, both before and after aortic valve replacement (r = 0.81 and r = 0.67, respectively)., Conclusions: Substantial differences in sensitivity, specificity, accuracy, and correlation with LVMI were found among several ECG-LVH criteria. No single criterion performed optimally since the highest sensitivity, specificity, accuracy, and correlation with LVMI were found with different criteria. The present study may therefore contribute to a more targeted use of the existing ECG-LVH criteria.