Preoperative determinants of recovery time in adult Fallot patients after late pulmonary valve replacement.
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Age Factors↗
Biomedical subjects
Hubert W Vliegen
Publications and source records attributed to Hubert W Vliegen.
Long-term effect of pulmonary valve replacement on QRS duration in patients with corrected tetralogy of Fallot.
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.
Death, Sudden, Cardiac↗
Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study.
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.
Adult↗
Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum.
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.
Adult↗
Pregnancy after biventricular repair for pulmonary atresia with ventricular septal defect.
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.
Adult↗
Long-term follow-up of homograft function after pulmonary valve replacement in patients with tetralogy of Fallot.
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.
Adult↗
Effects of volume and/or pressure overload secondary to congenital heart disease (tetralogy of fallot or pulmonary stenosis) on right ventricular function using cardiovascular magnetic resonance and B-type natriuretic peptide levels.
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.
Adolescent↗
Cardiovascular magnetic resonance in the follow-up of patients with corrected tetralogy of Fallot: a review.
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.
Coronary Aneurysm↗
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.
Adolescent↗
Massive pulmonary embolism with cardiac arrest after an intracardiac electrophysiological study: a strong case for venous thromboprophylaxis.
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.
Anticoagulants↗
Intraatrial repair of transposition of the great arteries: use of MR imaging after exercise to evaluate regional systemic right ventricular function.
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.
Adult↗
Time course of diastolic and systolic function improvement after pulmonary valve replacement in adult patients with tetralogy of Fallot.
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.
Adolescent↗
Risk of complications during pregnancy after Senning or Mustard (atrial) repair of complete transposition of the great arteries.
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.
Abortion, Spontaneous↗
Global and regional left ventricular function assessment with 16-detector row CT: comparison with echocardiography and cardiovascular magnetic resonance.
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.
Aged↗
Right ventricular function late after total repair of tetralogy of Fallot.
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.
Humans↗
Reduction of QRS duration after pulmonary valve replacement in adult Fallot patients is related to reduction of right ventricular volume.
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.
Adult↗
Comprehensive assessment of patients after coronary artery bypass grafting by 16-detector-row computed tomography.
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.
Aged↗
Comparison of segmental and global systemic ventricular function at rest and during dobutamine stress between patients with transposition and congenitally corrected transposition.
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.
Adolescent↗