Hubert W. Vliegen

Preoperative Thresholds for Pulmonary Valve Replacement in Patients With Corrected Tetralogy of Fallot Using Cardiovascular Magnetic Resonance

Background— To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. Methods and Results— Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42±10% to 43±10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m2) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: &bgr;=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m2 for normalization of RV end-diastolic volume or 82 mL/m2 for RV end-systolic volume. Conclusions— Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m2 or RV end-systolic volume was <82 mL/m2.

Mortality in adult congenital heart disease

AIMS Mortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear. METHODS AND RESULTS The Dutch CONCOR national registry for adult congenital heart disease was linked to the national mortality registry. Coxs regression was used to assess mortality predictors. Of 6933 patients, 197 (2.8%) died during a follow-up of 24 865 patient-years. Compared with the general national population, there was excess mortality, particularly in the young. Median age at death was 48.8 years. Of all deaths, 77% had a cardiovascular origin; 45% were due to chronic heart failure (26%, age 51.0 years) or sudden death (19%, age 39.1 years). Age predicted mortality, as did gender, severity of defect, number of interventions, and number of complications [hazard ratio (HR) range 1.1-5.9, P < 0.05]. Several complications predicted all-cause mortality beyond the effects of age, gender, and congenital heart disease severity, i.e. endocarditis, supraventricular arrhythmias, ventricular arrhythmias, conduction disturbances, myocardial infarction, and pulmonary hypertension (HR range 1.4-3.1, P < 0.05). These risks were similar in patients above and below 40 years of age. Almost all complications predicted death due to heart failure (HR range 2.0-5.1, P < 0.05); conduction disturbances and pulmonary hypertension predicted sudden death (HR range 2.0-4.7, P < 0.05). CONCLUSION Mortality is increased in adults with congenital heart disease, particularly in the young. The vast majority die from cardiovascular causes. Mortality risk, particularly by heart failure, is increased by virtually all complications. Complications are equally hazardous in younger as in older patients.

Predictors of pregnancy complications in women with congenital heart disease

AIMS Data regarding pregnancy outcome in women with congenital heart disease (CHD) are limited. METHODS AND RESULTS In 1802 women with CHD, 1302 completed pregnancies were observed. Independent predictors of cardiac, obstetric, and neonatal complications were calculated using logistic regression. The most prevalent cardiac complications during pregnancy were arrhythmias (4.7%) and heart failure (1.6%). Factors independently associated with maternal cardiac complications were the presence of cyanotic heart disease (corrected/uncorrected) (P < 0.0001), the use of cardiac medication before pregnancy (P < 0.0001), and left heart obstruction (P < 0.0001). New characteristics were mechanical valve replacement (P = 0.0014), and systemic (P = 0.04) or pulmonary atrioventricular valve regurgitation related with the underlying (moderately) complex CHD (P = 0.03). A new risk score for cardiac complications is proposed. The most prevalent obstetric complications were hypertensive complications (12.2%). No correlation of maternal characteristics with adverse obstetric outcome was found. The most prevalent neonatal complications were premature birth (12%), small for gestational age (14%), and mortality (4%). Cyanotic heart disease (corrected/uncorrected) (P = 0.0003), mechanical valve replacement (P = 0.03), maternal smoking (P = 0.007), multiple gestation (P = 0.0014), and the use of cardiac medication (P = 0.0009) correlated with adverse neonatal outcome. CONCLUSION In our tertiary CHD cohort, cardiac, obstetric, and neonatal complications were frequently encountered, and (new) correlations of maternal baseline data with adverse outcome are reported. A new risk score for adverse cardiac complications is proposed, although prospective validation remains necessary.

Comparison of gated single-photon emission computed tomography with magnetic resonance imaging for evaluation of left ventricular function in ischemic cardiomyopathy.

To perform a head-to-head comparison between magnetic resonance imaging (MRI) and gated single-photon emission computed tomography (SPECT) for the evaluation of left ventricular (LV) function (LV ejection fraction [LVEF], LV volumes, and regional wall motion) in patients with ischemic cardiomyopathy, we studied 22 patients with chronic coronary artery disease and LV dysfunction. Multislice, multiphase echoplanar MRI was performed with Philips Gyroscan ACS-NT15. Image analysis was performed using the MASS software package to determine LV end-systolic volume, LV end-diastolic volume, and LVEF. The same parameters were calculated using quantitative gated SPECT software (QGS, Cedars-Sinai Medical Center). The different parameters were compared using linear regression, and correlation coefficients were calculated. Regional wall motion was also determined from both techniques, according to a 13-segment model and a 3-point scoring system (from 1 = normokinesia to 3 = akinesia or dyskinesia). A summed wall motion score was also calculated for MRI and gated SPECT. Good correlations were found between MRI and gated SPECT for all parameters: (1) summed wall motion scoreMRI versus summed wall motion scoreSPECT: y = 0.74x + 8.0, r = 0.88, p <0.01; (2) LV end-systolic volumeMRI versus LV end-systolic volumeSPECT: y = 0.94x - 12.3, r = 0.87, p <0.01; (3) LV end-diastolic volumeMRI versus LV end-diastolic volumeSPECT: y = 0.93x - 18.4, r = 0.84, p <0.01; and (4) LVEFMRI versus LVEFSPECT: y = 0.97x + 0.68, r = 0.90, p <0.01. For regional wall motion, an exact agreement of 83% was found, with a kappa statistic of 0.77 (95% confidence intervals 0.71 to 0.83, SE 0.03), indicating essentially excellent agreement. Thus, close and significant correlations were observed for assessment of LVEF, LV volumes, and regional wall motion by MRI and gated SPECT in patients with ischemic cardiomyopathy.

Functional and Metabolic Evaluation of the Athlete’s Heart By Magnetic Resonance Imaging and Dobutamine Stress Magnetic Resonance Spectroscopy

BACKGROUND The question of whether training-induced left ventricular hypertrophy in athletes is a physiological rather than a pathophysiological phenomenon remains unresolved. The purpose of the present study was to detect any abnormalities in cardiac function in hypertrophic hearts of elite cyclists and to examine the response of myocardial high-energy phosphate metabolism to high workloads induced by atropine-dobutamine stress. METHODS AND RESULTS We studied 21 elite cyclists and 12 healthy control subjects. Left ventricular mass, volume, and function were determined by cine MRI. Myocardial high-energy phosphates were examined by 31P magnetic resonance spectroscopy. There were no significant differences between cyclists and control subjects for left ventricular ejection fraction (59+/-5% versus 61+/-4%), left ventricular cardiac index (3.4+/-0.4 versus 3.4+/-0.4 L x min(-1) x m[-2]), peak early filling rate (562+/-93 versus 535+/-81 mL/s), peak atrial filling rate (315+/-93 versus 333+/-65 mL/s), ratio of early and atrial filling volumes (3.0+/-1.0 versus 2.6+/-0.6), mean acceleration gradient of early filling (5.2+/-1.4 versus 5.8+/-1.9 L/s2), mean deceleration gradient of early filling(-3.1 +/- 0.9 versus -3.2 +/- 0.7 L/s2), mean acceleration gradient of atrial filling (3.6+/-1.8 versus 4.5+/-1.7 L/s2), and atrial filling fraction (0.23+/-0.06 versus 0.26+/-0.04, respectively). Cyclists and control subjects showed similar decreases in the ratio of myocardial phosphocreatine to ATP measured with 31P magnetic resonance spectroscopy during atropine-dobutamine stress (1.41+/-0.20 versus 1.41+/-0.18 at rest to 1.21+/-0.20 versus 1.16+/-0.13 during stress, both P=NS). CONCLUSIONS Left ventricular hypertrophy in cyclists is not associated with significant abnormalities of cardiac function or metabolism as assessed by MRI and spectroscopy. These observations suggest that training-induced left ventricular hypertrophy in cyclists is predominantly a physiological phenomenon.

Health related quality of life and health status in adult survivors with previously operated complex congenital heart disease

Objective: To examine the impact of previously operated complex congenital heart disease on health related quality of life and subjective health status and to determine the relation between these parameters and physical status. Design: Cross sectional; information on medical follow up was sought retrospectively. Setting: Patients were randomly selected from the archives of the paediatric cardiology department, Leiden University Medical Centre, Leiden, The Netherlands, and approached irrespective of current cardiac care or hospital of follow up. Patients: Seventy eight patients with previously operated complex congenital heart disease (now aged 18–32 years) were compared with the general population. Main outcome measures: Health related quality of life was determined with a specifically developed questionnaire (Netherlands Organisation for Applied Scientific Research Academic Medical Centre (TNO-AZL) adult quality of life (TAAQOL)) and subjective health status was assessed with the 36 item short form health survey (SF-36). Physical status was determined with the objective physical index, Somerville index, and New York Heart Association functional class. Results: Health related quality of life of the patients was significantly worse than that of the general population in the dimensions gross motor functioning and vitality (p < 0.01). Correlations between health related quality of life and physical status were poor. Patients had significantly worse subjective health status than the general population in the dimensions physical functioning, role functioning physical, vitality, and general health perceptions (p < 0.01). Correlations between subjective health status and physical indices were weak. Conclusion: Adult survivors with previously operated complex congenital heart disease experienced limitations only in the physical dimensions of health related quality of life and subjective health status. Objectively measured medical variables were only weakly related to health related quality of life. These results indicate that, when evaluating health related quality of life, dedicated questionnaires such as the TAAQOL should be used.

Value of Fast Gradient Echo Magnetic Resonance Angiography as an Adjunct to Coronary Arteriography in Detecting and Confirming the Course of Clinically Significant Coronary Artery Anomalies

We investigated the additional value of magnetic resonance (MR) angiography in 12 patients with clinically significant coronary anomalies. In 5 patients, the referring cardiologist requested additional evaluation because coronary arteriography was inconclusive about the course of the anomaly. For comparison, 7 patients with known anomalous coronary arteries were collected from our database. In these patients, there had been no doubt about the course of the anomalous coronary arteries. MR angiography provided the diagnosis in all 5 patients in whom the diagnosis was inconclusive beforehand. From the 7 patients in whom diagnosis had been established previously by contrast arteriography, the anomaly was confirmed in 5 by MR angiography. In 1 patient, the initial diagnosis at contrast arteriography had to be changed as a result of MR angiography. In 1 patient, the MR image was of insufficient quality to be conclusive. In conclusion, in patients with angiographically suspected coronary anomalies, fast gradient echo MR angiography is a helpful adjunct to coronary arteriography in identifying and confirming the origin and course of clinically significant coronary anomalies. The additional value of fast gradient echo MR coronary angiography is the visualization of both the artery and its surrounding structures.

Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism

Background Chronic thromboembolic pulmonary hypertension after pulmonary embolism is associated with high morbidity and mortality. Understanding the incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism is important for evaluating the need for screening but is also a subject of debate because of different inclusion criteria among previous studies. We determined the incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism and the utility of a screening program for this disease. Design and Methods We conducted a cohort screening study in an unselected series of consecutive patients (n=866) diagnosed with acute pulmonary embolism between January 2001 and July 2007. All patients who had not been previously diagnosed with pulmonary hypertension (PH) and had survived until study inclusion were invited for echocardiography. Patients with echocardiographic suspicion of PH underwent complete work-up for chronic thromboembolic pulmonary hypertension, including ventilation-perfusion scintigraphy and right heart catheterization. Results After an average follow-up of 34 months of all 866 patients, PH was diagnosed in 19 patients by routine clinical care and in 10 by our screening program; 4 patients had chronic thromboembolic pulmonary hypertension, all diagnosed by routine clinical care. The cumulative incidence of chronic thromboembolic pulmonary hypertension after all cause pulmonary embolism was 0.57% (95% confidence interval [CI] 0.02–1.2%) and after unprovoked pulmonary embolism 1.5% (95% CI 0.08–3.1%). Conclusions Because of the low incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism and the very low yield of the echocardiography based screening program, wide scale implementation of prolonged follow-up including echocardiography of all patients with pulmonary embolism to detect chronic thromboembolic pulmonary hypertension does not seem to be warranted.

Pregnancy and delivery in women after Fontan palliation.

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.

Prognostic value of right ventricular longitudinal peak systolic strain in patients with pulmonary hypertension.

Background—Right ventricular (RV) function is an important prognostic marker in patients with pulmonary hypertension. The present evaluation assessed the prognostic value of RV longitudinal peak systolic strain (LPSS) in patients with pulmonary hypertension. Methods and Results—A total of 150 patients with pulmonary hypertension of different etiologies (mean age, 59±15 years; 37.3% male) were evaluated. RV fractional area change and tricuspid annular plane systolic excursion index were evaluated with 2-dimensional echocardiography. RV LPSS was assessed with speckle-tracking echocardiography. The patient population was categorized according to a RV LPSS value of –19%. Among several clinical and echocardiographic parameters, the significant determinants of all-cause mortality were evaluated. There were no significant differences in age, sex, pulmonary hypertension cause and left ventricular ejection fraction between patients with RV LPSS <−19% and patients with RV LPSS ≥−19%. However, patients with RV LPSS ≥−19% had significantly worse New York Heart Association functional class (2.7±0.6 versus 2.3±0.8; P=0.003) and lower tricuspid annular plane systolic excursion (16±4 mm versus 18±3 mm; P<0.001) than their counterparts. During a median follow-up of 2.6 years, 37 patients died. RV LPSS was a significant determinant of all-cause mortality (HR, 3.40; 95% CI, 1.19–9.72; P=0.02). Conclusions—In patients with pulmonary hypertension, RV LPSS is significantly associated with all-cause mortality. RV LPSS may be a valuable parameter for risk stratification of these patients. Future studies are needed to confirm these results in the pulmonary hypertension subgroups.