Papers by William Ravekes
American Journal of Medical Genetics, Feb 26, 2013
Previous data suggest women are at increased risk of death from aortic dissection. Therefore, we ... more Previous data suggest women are at increased risk of death from aortic dissection. Therefore, we analyzed data from the GenTAC registry, the NIH-sponsored program that collects information about individuals with genetically triggered thoracic aortic aneurysms and cardiovascular conditions. We performed crosssectional analyses in adults with Marfan syndrome (MFS), familial thoracic aortic aneurysm or dissection (FTAAD), bicuspid aortic valve (BAV) with thoracic aortic aneurysm or dissection, and subjects under 50 years of age with thoracic aortic aneurysm or dissection (TAAD <50 years). Women comprised 32% of 1,449 subjects and were 21% of subjects with BAV, 34% with FTAAD, 22% with TAAD <50 years, and 47% with MFS. Thoracic aortic dissections occurred with equal gender frequency yet women with BAV had more extensive dissections. Aortic size was smaller in women but was similar after controlling for BSA. Age at operation for aortic valve dysfunction, aneurysm or dissection did not differ by gender. Multivariate analysis (adjusting for age, BSA, hypertension, study site, diabetes, and subgroup diagnoses) showed that women had fewer total aortic surgeries (OR ¼ 0.65, P < 0.01) and were less likely to receive angiotensin converting enzyme inhibitors (ACEi; OR ¼ 0.68, P < 0.05). As in BAV, other genetically triggered aortic diseases such as FTAAD and TAAD <50 are more common in males. In women, decreased prevalence of aortic operations and less treatment with ACEi may be due to their smaller absolute aortic diameters. Longitudinal studies are needed to determine if women are at higher risk for adverse events.
Prenatal Diagnosis, Feb 1, 2011
European Heart Journal - Cardiovascular Imaging
Funding Acknowledgements Type of funding sources: None. Background Surveillance strategies for ao... more Funding Acknowledgements Type of funding sources: None. Background Surveillance strategies for aortic disease based on the current guidelines support the utilization of maximum aortic diameters to evaluate the risk of aortic dissection, however there remains a need for improved imaging markers for this risk stratification. Purpose This study aimed to compare cardiac magnetic resonance (CMR)-derived ventriculo-vascular properties in patients with Marfan syndrome against normal controls, and to determine the relationship between aortic area strain and other clinical variables in this population. Methods Ascending (AAAS) and descending aortic area (DAAS) strain and left ventricular (LV) longitudinal (LS) and circumferential (CS) strain were retrospectively obtained from 20 CMR studies from 17 patients with Marfan syndrome. Aortic area strain was estimated using maximal and minimal luminal areas obtained from cross-sectional aortic images at the level of the pulmonary artery and was cal...
American Journal of Cardiology, Oct 1, 2017
American Journal of Cardiology, 2012
The tricuspid annular peak systolic velocity (TAPSV) is an echocardiographic measurement assessin... more The tricuspid annular peak systolic velocity (TAPSV) is an echocardiographic measurement assessing right ventricular systolic function in children and adults. We determined the growth-related changes of the TAPSV to establish the references values for the entire pediatric age group. A prospective study was conducted of a group of 860 healthy pediatric patients (age 1 day to 18 years; body surface area [BSA] 0.14 to 2.30 m 2). We determined the effects of age, gender, and BSA on the TAPSV values. Stepwise linear multiple regression analysis was used to estimate the TAPSV from the age, BSA, and gender. A correlation of normal TAPSV with normal tricuspid annular plane systolic excursion values was performed. The TAPSV ranged from a mean of 7.2 cm/s (z score ؎ 2: 4.8 to 9.5 cm/s) in the newborn to 14.3 cm/s (z score ؎ 2: 10.6 to 18.6 cm/s) in the 18-year-old adolescent. The TAPSV values showed a positive correlation with age and BSA, with a nonlinear course. No significant difference was found in the TAPSV values according to gender. A significant correlation was found between the TAPSV and tricuspid annular plane systolic excursion values in our pediatric population. In conclusion, the z scores of the TAPSV values were calculated, and percentile charts were established to serve as reference data for patients with congenital heart disease.
American Heart Journal, Jul 1, 2012
Background Longitudinal myocardial function has gained more interest in the last years. The mitra... more Background Longitudinal myocardial function has gained more interest in the last years. The mitral annular plane systolic excursion (MAPSE) is an echocardiographic measurement to assess left ventricular (LV) long-axis function in adults. The aim of this study was to evaluate MAPSE values in a healthy pediatric population and to propose reference values. Methods A prospective study was conducted in a group of 558 healthy children and adolescents (age day 1 to 18 years) (body surface area [BSA] 0.18-2.21 m²). We determined the effects of age and BSA on MAPSE values and a possible correlation of MAPSE values with LV ejection fraction values. Results The MAPSE ranged from a mean of 0.57 cm (z-score ± 2: 0.38-0.76 cm) in neonates to 1.63 cm (z-score ±2: 1.31-1.95 cm) in 18-year-old adolescents. The MAPSE values showed a positive correlation with age (r = 0.87, P b .001) and BSA (r = 0.89, P b .001) with a nonlinear course. There was no significant difference in MAPSE values between females or males. A positive correlation was found between MAPSE values and LV ejection fraction values (r = 0.28, P b .001). Conclusions Z-scores of MAPSE values were calculated, and percentile charts were established to serve as reference data in patients with congenital heart disease or heart failure in the future.
Pediatric Cardiology, Mar 24, 2006
The objective of this study was to determine if distortion of the left subclavian artery course d... more The objective of this study was to determine if distortion of the left subclavian artery course distinguishes double aortic arch with atretic left dorsal aorta from right aortic arch with mirror image branching. We performed a retrospective case series at a tertiary care center. Twenty-six patients undergoing magnetic resonance imaging for suspicion of a vascular ring were identified, 6 of whom had an atretic left dorsal aorta confirmed by surgical inspection. Six patients with the diagnosis of right aortic arch with mirror image branching were identified for comparison. The course of the left subclavian artery was assessed using surface-rendered magnetic resonance angiography (MRA) and axial fast spin echo images. All patients with double aortic arch had clinical symptoms suggestive of esophageal or tracheal compression. Six patients had double aortic arch, 3 of whom had an atretic left dorsal aorta. In these 3 patients, the branching patterns on MRA mimicked right aortic arch mirror image branching except for the distortion of the initial course of the left subclavian artery. Surgical observation confirmed the presence of an atretic left dorsal aorta that resulted in tension on the left subclavian artery pulling it posteriorly and inferiorly and completing the vascular ring. Patients with right aortic arch mirror image branching demonstrated no such subclavian artery distortion, and these patients did not have clinical symptoms suggestive of a vascular ring. Our results demonstrate that left subclavian artery distortion due to traction by an atretic left arch is an important diagnostic finding in the evaluation 6 patients with suspected vascular rings.
Journal of The American Society of Echocardiography, Oct 1, 2012
Background: Tricuspid annular peak systolic velocity (S 0), as an echocardiographic index to asse... more Background: Tricuspid annular peak systolic velocity (S 0), as an echocardiographic index to assess right ventricular (RV) systolic function, has not been investigated thoroughly in children and young adults with repaired tetralogy of Fallot (TOF) and pulmonary artery hypertension secondary to congenital heart disease (PAH-CHD). Methods: S 0 values in patients with TOF (n = 183) and PAH-CHD (n = 55) were compared with those in normal subjects. S 0 values were compared with RV ejection fraction and RV end-diastolic volume index (RVEDVi) determined by magnetic resonance imaging. Results: S 0 values became significantly reduced in PAH-CHD patients after 10.4 years of age and after 13.6 years of age in patients with TOF compared with the lower boundary of the 62-SD interval of normal subjects. Significant positive correlations between S 0 and RV ejection fraction were seen in patients with TOF (r = 0.66, P < .001) and those with PAH-CHD (r = 0.82, P < .001). Significant negative correlations between S 0 and RVEDVi were also seen in patients with repaired TOF (r = À0.29, P = .002) and in those with PAH-CHD (r =-0.59, P < .001). Conclusions: Although initially preserved, in this prospective study, impaired S 0 values with increasing age were found in patients with repaired TOF and PAH-CHD. Persistent pressure overload in patients with PAH-CHD as well as volume overload in those with repaired TOF might lead to systolic RV functional impairment and increased RVEDVi. The validity of S 0 data was supported by magnetic resonance imaging data (RVEDVi and RV ejection fraction).
Thoracic and Cardiovascular Surgeon, Oct 16, 2013
Quantitative determination of right ventricular (RV) function has gained more interest over the l... more Quantitative determination of right ventricular (RV) function has gained more interest over the last years. The RV outflow tract systolic excursion (RVOT SE) has been recently proposed as an echocardiographic tool to assess RV systolic function in adults. We aimed to determine growth-related changes of RVOT SE in children and to establish references values. Study design A prospective study was conducted in a group of 711 healthy paediatric patients (age: 1 day to 18 years). We determined the effects of age and body surface area (BSA) on RVOT SE values. RVOT SE values were further correlated with the established RV systolic function parameters tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (S'). Results The RVOT SE ranged from a mean of 3.4 mm in neonates to 9.5 mm in 18-year-old adolescents. The RVOT SE values showed a positive correlation with age (r ¼ 0.90, P , 0.001) and BSA (r ¼ 0.91, P , 0.001). A significant positive correlation was seen between RVOT SE and TAPSE (r ¼ 0.93, P , 0.001) and also between RVOT SE and S' (r ¼ 0.86, P , 0.001) in our patients. Conclusion RVOT SE provides a simple measure and, in combination with long-axis excursion parameters TAPSE and S', a comprehensive assessment of RV systolic function. Z-scores of RVOT SE values were calculated, and percentile charts were established to serve as reference data.
Pediatric Cardiology, Nov 18, 2004
We evaluated the aortic outflow tract (AOT) and coronary artery dimensions in pediatric patients ... more We evaluated the aortic outflow tract (AOT) and coronary artery dimensions in pediatric patients with unicommissural aortic valves. A retrospective review of an echocardiographic database identified 37 patients with unicommissural aortic valves. A total of 115 echocardiograms were reviewed, and the right coronary artery (RCA), left main coronary artery (LM), left anterior descending coronary artery aortic valve annulus, aortic root, sinotubular junction (STJ), and ascending aorta were measured and z scores determined. The aortic stenosis peak gradient and the amount of aortic regurgitation (AR) were also measured. The RCA diameter (z score, 1.85 ± 1.8, p = 0.03) and LM diameter (z score, 1.74 ± 1.47, p = 0.04) are significantly dilated, as are all the AOT measurements: aortic annulus (2.02 ± 1.9, p = 0.02), aortic root (2.25 ± 1.9, p = 0.02), STJ (2.22 ± 1.74, p = 0.01), and ascending aorta (4.38 ± 2.03, p < 0.001). Longitudinal follow-up showed that there was no significant variation over time in any variable. The AOT measurements were significantly correlated with each other. A trend was found in which an increasing amount of AR gave an increase in AOT measurements. The aortic gradient was not significantly associated with any measurement. Our study population demonstrated significant dilatation of the RCA and LM as well as the AOT. The dilatation of the AOT structures is likely caused by the same mechanism that accounts for the AOT dilatation in patients with bicommissural aortic valves. Dilatation of the coronary arteries may represent an intrinsic abnormality in the vessel wall. Further studies are needed to define possible changes.
The Annals of Thoracic Surgery, Oct 1, 2013
The primary outcome was 1-year survival. All causes of death were incorporated into the survival ... more The primary outcome was 1-year survival. All causes of death were incorporated into the survival analyses. Secondary outcomes included 30-day and 6-month survival, as well as rates of postoperative complications. The complications examined included renal failure requiring dialysis, stroke, all-cause reoperation, infection, and rejection. Postoperative was defined as occurring during the same admission as the HTx. Data Analysis A trend analysis was initially performed examining annual rates of nonbridged HTx, bridge to HTx with
Cardiology in The Young, Dec 1, 2015
UNDIAGNOSED ANOMALOUS CORONARY ARTERIES are a well-recognised cause of sudden cardiac death in ch... more UNDIAGNOSED ANOMALOUS CORONARY ARTERIES are a well-recognised cause of sudden cardiac death in children. Anomalous coronary patterns include origin of a coronary artery from the contralateral sinus and a single coronary artery. All patients with an anomalous left main coronary artery origin from the right sinus of Valsalva are generally referred for surgical repair, whereas operative decisions pertaining to patients with an anomalous right coronary artery origin remain controversial. Generally, patients with symptoms of ischaemia or arrhythmia with either form of the anomaly are referred for surgical repair, whereas asymptomatic patients with an anomalous right coronary artery pose a management dilemma. In addition, a review of the literature documents case reports of familial clustering, thus sparking the question of whether first-degree relatives should undergo screening. Here, we provide clinical examples of familial clustering of congenital abnormalities of the coronary arteries. In discussing these patients, we hope to assess the risk of sudden death in patients with anomalous coronary arteries, the risk for first-degree relatives, the best screening modalities to establish diagnosis, and, if echocardiographic assessment of the coronary arteries is unclear, how far to pursue diagnosis. Clinical cases
International Journal of Cardiovascular Imaging, Jul 3, 2013
Aim of this prospective study was to evaluate longitudinal systolic left ventricular (LV)-right v... more Aim of this prospective study was to evaluate longitudinal systolic left ventricular (LV)-right ventricular (RV) interaction using M-mode compared to magnetic resonance imaging (MRI) data in 146 pediatric and adults with operated tetralogy of Fallot (TOF). We determined biventricular measures of longitudinal M-mode echocardiography [i.e. tricuspid annular plane systolic excursion (TAPSE); the mitral annular plane systolic excursion (MAPSE)] compared to longitudinal function parameters using MRI. M-mode data were compared to established normal z-score values. We found a good correlation between MAPSE and LVEF values (r = 0.788; p \0.001). Correlations between MRI derived MAPSE and M-mode guided MAPSE (r = 0.879, p \ 0.001), and between MRI derived TAPSE and M-mode guided TAPSE were significant (r = 0.780, p \ 0.001). While the LVEF was normal in patients with a normal RVEF, the LVEF was decreased in patients with significantly reduced RVEF. Patients with a significantly dilated RV (RVED-Vi [ 150 ml/m 2) showed a significantly reduced mean MAPSE of 1.30 ± 0.26 cm. LV longitudinal function decreases below-2 SD of normal MAPSE z-score values after a mean of 22 postoperative years. Our data confirm progressive adverse RV-LV interaction in the long-term follow-up of TOF. We show that simple M-mode measurement of the longitudinal LV function (i.e. MAPSE) is a sufficient surrogate for estimation of LVEF. Therefore determination of the MAPSE is a helpful additional tool for LV systolic function assessment late after TOF repair.
Pulmonary circulation, 2012
Research Ar ticle INTRODUCTION Pulmonary hypertension (PH) is a complex disease with a heterogene... more Research Ar ticle INTRODUCTION Pulmonary hypertension (PH) is a complex disease with a heterogeneous group of etiologies in the neonatal and pediatric populations. The most common cause of acute pulmonary vascular crisis in children is persistent PH of the newborn (PPHN)-postpartum persistence of
Clinical Research in Cardiology, 2011
We briefly would like to comment on the Article ''Tricuspid annular plane systolic excursion and ... more We briefly would like to comment on the Article ''Tricuspid annular plane systolic excursion and right ventricular ejection fraction in pediatric and adolescent patients with tetralogy of Fallot, patients with atrial septal defect, and age-matched normal subjects'' by Koestenberger et al. [1]. The authors report on the investigations on TAPSE in a patient cohort after tetralogy of Fallot repair, in atrial septum defect and in age-matched healthy controls. Regarding the patients after repair, a reduction in TAPSE after 7 years postoperatively was found. Moreover, a weak correlation between TAPSE and the RV-EF as measured by MRI was calculated.
The Journal of Heart and Lung Transplantation, 2018
Improved renal function following ventricular assist device (VAD) placement is seen in some, but ... more Improved renal function following ventricular assist device (VAD) placement is seen in some, but not all VAD recipients. Failure to improve may indicate enduring renal injury. We hypothesized that VAD implantation results in improved renal function in the majority of pediatric patients with heart failure and that failure to improve would predict poor renal outcomes after heart transplantation (HT). Methods: All VAD patients enrolled in both the PEDIMACS and PHTS registries between 2012 and 2016 were considered for a linkage analysis. Following implant, persistent acute kidney injury (P-AKI), defined as a serum creatinine (SCr) > /= 1.5x baseline, was assessed at post-op day 7. Estimated glomerular filtration rate (eGFR) was determined at implant, 30 days after implantation, and 12 months post-HT. The relationship between P-AKI and eGFR was used to predict the need for dialysis and post-HT chronic kidney disease (CKD). Results: 207 patients were eligible for analysis, including 52 (25%) with an eGFR < 60 mL/min/1.73 m 2 at the time of implantation. P-AKI was seen in 21 (10%) at 7 days, including 13 (62%) who had pre-implant CKD. Mean eGFR 1 month post-implant was 131.70 +/-61.03. Seventy-four percent (105/141) experienced a significant increase (> 10 mL/min/1.73m 2) in eGFR over the first post-VAD month. SCr data was available for 133 patients at 1-year post-HT, for whom 10 (7.52%) had CKD (eGFR < 60); 50 (37.59%) were "at risk" for CKD (eGFR < 90 but > 60). In the first post-HT year, 11 patients required dialysis. Failure to experience an increase in eGFR following VAD placement was predictive of the need for dialysis after HT and/or CKD at 12 months post-HT (OR 1.82, 95% CI 0.999-3.317, p= 0.05, table 1).
Circulation, 2016
Background: The influence of gender and age on phenotypic features of individuals with Marfan syn... more Background: The influence of gender and age on phenotypic features of individuals with Marfan syndrome has not been systematically examined in a large cohort of both children and adults. Methods: W...
Neonatology, 2013
Access to full text and tables of contents, including tentative ones for forthcoming issues: www.... more Access to full text and tables of contents, including tentative ones for forthcoming issues: www.karger.com/neo_issues 141 Neonatal Morbidity after Exchange Transfusion for Red Cell Alloimmune Hemolytic Disease
Arteriosclerosis, Thrombosis, and Vascular Biology, 2014
Background: Little is known about reliable predictors of thoracic aortic dissection or rupture (T... more Background: Little is known about reliable predictors of thoracic aortic dissection or rupture (TAD) in children and young adults. We sought to determine whether elevated vertebral artery tortuosity is a biomarker of TAD or aortic surgery at an early age. Methods: We identified 208 patients ≤50 years old in the GenTAC Registry who had ≥2.5 cm of either vertebral artery visualized on a computed tomography angiogram (CTA). In a blinded fashion, using a volume-rendered projection, each patient’s vertebral artery tortuosity index (VTI) was calculated using the larger distance factor (% by which actual length exceeds straight-line length) of the two vertebral arteries. We then investigated associations between VTI and freedom from prophylactic or post-TAD surgery. Results: Subjects included 73 with Marfan syndrome (MS), 34 with bicuspid aortic valve, 18 with Loeys-Dietz syndrome (LDS), 16 with familial thoracic aneurysms and dissections, 12 with Ehlers-Danlos syndrome type IV (EDS), 6 wi...
Circulation, 2015
Background: The NHLBI Pediatric Heart Network randomized trial of atenolol vs. losartan in Marfan... more Background: The NHLBI Pediatric Heart Network randomized trial of atenolol vs. losartan in Marfan Syndrome demonstrated no significant treatment difference in the rate of change in body surface area adjusted maximum aortic root diameter z score (AoRz). Objectives: To report trial results on aortic stiffness and to determine whether aortic stiffness predicts clinical outcome and change in AoRz. Methods: 608 patients (6 mo - 25 yr) who met original Ghent criteria and had AoRz > 3 were enrolled. Echocardiograms obtained at 0, 6, 12, 24 & 36 months were centrally interpreted. Aortic dimensions were measured by 2D imaging, and stiffness indices were calculated for aortic root (AoR) and ascending aorta (AA). Where appropriate, stiffness measurements were indexed to 1/sqrt(R-R interval) to adjust for heart rate. Data were analyzed by multivariable mixed effects modeling and Cox regression. Results: The rate of change over three years in heart rate-adjusted AoR stiffness index differed b...
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Papers by William Ravekes