Outcome after aortic valve replacement in children: A systematic review and meta-analysis Etnel, Jonathan R.G., BSc; Elmont, Lisa C., BSc; Ertekin, Ebru, BSc ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
01/2016, Letnik:
151, Številka:
1
Journal Article
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Abstract Objective Despite an increasing interest in pediatric aortic valve repair, aortic valve replacement in children may be unavoidable. The evidence on outcome after pediatric aortic valve ...replacement is limited and usually reported in small case series. This systematic review and meta-analysis aims to provide an overview of reported outcome of pediatric patients after aortic valve replacement. Methods A systematic literature search for publications reporting outcome after pediatric aortic valve replacement published between January 1990 and May 2015 was conducted. Studies written in English with a study size of more than 30 patients were included. Results Thirty-four publications reporting on 42 cohorts were included in this review: 26 concerning the Ross procedure (n = 2409), 13 concerning mechanical prosthesis aortic valve replacement (n = 696), and 3 concerning homograft aortic valve replacement (n = 224). There were no studies on bioprostheses that met our inclusion criteria. The pooled mean patient age was 9.4 years, 12.8 years, and 8.9 years for Ross, mechanical prosthesis, and homograft recipients, respectively. Pooled mean follow-up was 6.6 years. The Ross procedure was associated with lower early (4.20%; 95% confidence interval CI, 3.37-5.22 vs 7.34%; 95% CI, 5.21-10.34 vs 12.82%; 95% CI, 8.91-18.46) and late mortality (0.64%/y; 95% CI, 0.49-0.84 vs 1.23%/y; 95% CI, 0.85-1.79 vs 1.59%/y; 95% CI, 1.03-2.46) compared with mechanical prosthesis aortic valve replacement and homograft aortic valve replacement, respectively. No significantly different aortic valve reoperation rates were observed between the Ross procedure and mechanical prosthesis aortic valve replacement (1.60%/y; 95% CI, 1.27-2.02 vs 1.07%/y; 95% CI, 0.68-1.68, respectively), whereas homograft aortic valve replacement was associated with significantly higher aortic valve reoperation rates (5.44%/y; 95% CI, 4.24-6.98). The Ross procedure–associated right ventricular outflow tract reoperation rate was 1.91% per year (95% CI, 1.50-2.44). Conclusions This systematic review illustrates that all currently available aortic valve substitutes are associated with suboptimal results in children, reflecting the urgent need for reliable and durable repair techniques and innovative replacement solutions for this challenging group of patients.
Abstract Background Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms ...involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes, have revealed an important contribution of disturbed transforming growth factor (TGF)-β signaling. Objectives This study sought to discover a novel gene causing syndromic aortic aneurysms in order to unravel the underlying pathogenesis. Methods We combined genome-wide linkage analysis, exome sequencing, and candidate gene Sanger sequencing in a total of 470 index cases with thoracic aortic aneurysms. Extensive cardiological examination, including physical examination, electrocardiography, and transthoracic echocardiography was performed. In adults, imaging of the entire aorta using computed tomography or magnetic resonance imaging was done. Results Here, we report on 43 patients from 11 families with syndromic presentations of aortic aneurysms caused by TGFB3 mutations. We demonstrate that TGFB3 mutations are associated with significant cardiovascular involvement, including thoracic/abdominal aortic aneurysm and dissection, and mitral valve disease. Other systemic features overlap clinically with Loeys-Dietz, Shprintzen-Goldberg, and Marfan syndromes, including cleft palate, bifid uvula, skeletal overgrowth, cervical spine instability and clubfoot deformity. In line with previous observations in aortic wall tissues of patients with mutations in effectors of TGF-β signaling ( TGFBR1/2, SMAD3, and TGFB2 ), we confirm a paradoxical up-regulation of both canonical and noncanonical TGF-β signaling in association with up-regulation of the expression of TGF-β ligands. Conclusions Our findings emphasize the broad clinical variability associated with TGFB3 mutations and highlight the importance of early recognition of the disease because of high cardiovascular risk.
Objectives The purpose of this study was describe the cardiovascular phenotype of the aneurysms-osteoarthritis syndrome (AOS) and to provide clinical recommendations. Background AOS, caused by ...pathogenic SMAD3 variants, is a recently described autosomal dominant syndrome characterized by aneurysms and arterial tortuosity in combination with osteoarthritis. Methods AOS patients in participating centers underwent extensive cardiovascular evaluation, including imaging, arterial stiffness measurements, and biochemical studies. Results We included 44 AOS patients from 7 families with pathogenic SMAD3 variants (mean age: 42 ± 17 years). In 71%, an aortic root aneurysm was found. In 33%, aneurysms in other arteries in the thorax and abdomen were diagnosed, and in 48%, arterial tortuosity was diagnosed. In 16 patients, cerebrovascular imaging was performed, and cerebrovascular abnormalities were detected in 56% of them. Fifteen deaths occurred at a mean age of 54 ± 15 years. The main cause of death was aortic dissection (9 of 15; 60%), which occurred at mildly increased aortic diameters (range: 40 to 63 mm). Furthermore, cardiac abnormalities were diagnosed, such as congenital heart defects (6%), mitral valve abnormalities (51%), left ventricular hypertrophy (19%), and atrial fibrillation (22%). N-terminal brain natriuretic peptide (NT-proBNP) was significantly higher in AOS patients compared with matched controls (p < 0.001). Aortic pulse wave velocity was high-normal (9.2 ± 2.2 m/s), indicating increased aortic stiffness, which strongly correlated with NT-proBNP (r = 0.731, p = 0.005). Conclusions AOS predisposes patients to aggressive and widespread cardiovascular disease and is associated with high mortality. Dissections can occur at relatively mildly increased aortic diameters; therefore, early elective repair of the ascending aorta should be considered. Moreover, cerebrovascular abnormalities were encountered in most patients.
Highlights • With 4D flow, any plane of interest can be interactively chosen for quantitative measurements. • Anatomical and flow data is obtained during an approximately 10-minute free-breathing ...scan. • 4D CMR flow measurements correlated well with the 2D PC ones. • Eddy current correction is important for good results with 4D flow
Objectives The aim of this study was to identify which patients with a systemic right ventricle are at risk for clinical events. Background In patients with congenitally or atrially corrected ...transposition of the great arteries, worsening of the systemic right ventricle is accompanied by clinical events such as clinical heart failure or the occurrence of arrhythmia. Methods At baseline, all subjects underwent electrocardiography, echocardiography, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging. Clinical events comprised death, vascular events, tricuspid regurgitation requiring surgery, worsening heart failure, and (supra)ventricular arrhythmia. A Cox proportional hazards analysis was used to assess the most valuable determinants of clinical events. Results A total of 88 patients with a mean age of 33 years were included in the study. Sixty-five percent were men, and 28% had congenitally corrected transposition of the great arteries. During a follow-up period of 4.3 years, 31 patients (35%) experienced 46 clinical events for an annual risk of 12%. Right ventricular end-diastolic volume index measured by means of cardiovascular magnetic resonance imaging or multirow detector computed tomography (hazard ratio: 1.20; p < 0.01) and peak exercise systolic blood pressure (hazard ratio: 0.86; p = 0.02) were the strongest determinants of clinical events. Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg were most likely to experience clinical events with an annual event rate of 19% versus 0.9% in patients without these risk factors. Conclusions Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg had a 20-fold higher annual event rate than patients without these risk factors. Regular cardiovascular magnetic resonance imaging and exercise testing are important in the risk assessment of these patients.
Background In women with corrected tetralogy of Fallot (ToF), pregnancy is associated with maternal cardiac, obstetric, and offspring complications. Our aim is to investigate the magnitude and ...determinants of pregnancy outcome in women with corrected ToF. Methods In this retrospective international multicenter study using 2 congenital heart disease registries, 204 women with corrected ToF were identified. Within this group, 74 women had 157 pregnancies, including 30 miscarriages and 4 terminations of pregnancy. Detailed information on each completed pregnancy (n = 123) was obtained using medical records and supplementary interviews. Results Cardiovascular events occurred during 10 (8.1%) pregnancies, mainly (supra)ventricular arrhythmias. Obstetric and offspring events occurred in 73 (58.9%) and 42 (33.9%) pregnancies, respectively, including offspring mortality in 8 (6.4%). The most important predictor was use of cardiac medication before pregnancy (odds ratio for cardiac events 11.7, 95% CI 2.2-62.7; odds ratio for offspring events 8.4, 95% CI 1.4-48.6). In pregnancies with cardiovascular events, significantly more small-for-gestational-age children were born ( P value < .01). Conclusions Cardiovascular, obstetric, and offspring events occur frequently during pregnancies in women with ToF. Maternal use of cardiovascular medication is associated with pregnancy outcome, and maternal cardiovascular events during pregnancy are highly associated with offspring events.
Allografts in aortic position: insights from a 27-year single-center prospective study Arabkhani, Bardia, MD, MSc; Bekkers, Jos A., MD, PhD; Andrinopoulou, Eleni-Rosalina, PhD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
12/2016, Letnik:
152, Številka:
6
Journal Article
Recenzirano
Odprti dostop
Abstract Objective Over the past decades the indication for allograft implantation in aortic position has evolved. The purpose of this study is to report long-term survival, allograft durability, and ...potential risk factors. Methods Between 1987 and 2010, 353 patients underwent aortic valve replacements using allograft (92 subcoronary, 261 root replacement; 98% aortic allografts). Patient characteristics, survival, valve durability, and valve-related events were analyzed. Additionally, patients were followed with standardized echocardiography. A joint modeling approach was used to detect the effect of (echocardiographic) variables on mortality and reoperation hazard. Results Mean age was 45 years (range 1 month to 84 years); 71% males. Etiology: endocarditis 32% (active 22%), congenital 31%, degenerative 9%, aneurysm/dissection 12%, rheumatic 6%, and prosthetic valve failure 10%. Hospital mortality: 5.9% (N = 21). During follow-up (mean 12 years, range 0-24, 99% complete), 113 patients died. Twenty-year cumulative survival was 41% (95% CI: 32-50). Valve-related reoperations occurred in 117 patients: 100 SVD, 9 NSVD, and 8 endocarditis. Competing-risk analysis predicted that at 20 years 31% died, and 30% were alive without reoperation. Younger patient-age was associated with increased reoperation. During follow-up left ventricular dilatation and severe aortic regurgitation were associated with mortality (p= 0.006 and 0.005 respectively), and ≥ grade 3 aortic regurgitation during follow-up was associated with reoperation risk (p= 0.001). Conclusions After almost 3 decades of experience with allografts in aortic position, the indication for use has become selective, mainly because of progressive SVD over time. In case of complex aortic root pathology and active endocarditis allografts may still be useful.
Bicuspid aortic valve (BAV) is a common congenital heart defect. Patients with BAV are at risk for long-term complications such as valve stenosis and regurgitation. This study aimed to investigate ...(1) sex differences in blood and imaging biomarkers and (2) describe the long-term prognostic value of blood and echocardiographic biomarkers.
Patients were included from two prospective observational cohort studies, patients underwent venous blood sampling and transthoracic echocardiography including speckle tracking. Analyzed blood biomarkers were: RDW, creatinine, CRP, TroponinT, NT-proBNP and TGF-β. Sex differences were analyzed at baseline. Associations between biomarkers and arrhythmia-free and intervention-free survival were determined by Cox regression, adjusted for age and sex.
182 BAV patients were included (median age 34 (IQR: 23-46) years, 55.5% male). CRP, NT-proBNP and RDW were higher in women while creatinine, TroponinT and TGF-β were higher among men. After a median follow-up time of 6.9 (IQR: 6.5-9.9) years, arrhythmia-free and intervention-free survival were respectively 81.0% and 73.1%. NT-proBNP was associated with both arrhythmia-free and intervention-free survival (HR 1.94, p=0.005 and HR 2.06, p=0.002 respectively). On echocardiography higher LA size, LVEDD, LV mass index and E/e’ ratio were associated with lower arrhythmia-free survival, while higher LA size, LV mass index, aortic valve peak velocity and aortic regurgitation were associated with lower intervention-free survival.
Differences were observed in blood biomarkers between men and women with BAV. Besides LV systolic parameters, diastolic LV function and NT-proBNP should have a more prominent role as prognostic markers in clinical care.
Objectives The aim of this study was to determine the value of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in adults with congenital heart disease (CHD) and investigate its relationship ...with ventricular function and exercise capacity. Background NT-proBNP may detect early deterioration in cardiac function. Methods In this cross-sectional study, extensive echocardiography, exercise testing, and NT-proBNP measurements were performed on the same day in consecutive adult patients with CHD. Results In total, 475 patients were included in this study (mean age of 34 ± 12 years, 57% male, 90% New York Heart Association class I). The median NT-proBNP level was 15.1 pmol/l (interquartile range IQR: 7.1 to 31.3 pmol/l), and the NT-proBNP level was >14 pmol/l in 53% of patients. The highest NT-proBNP levels were observed in patients with Fontan circulation (36.1 pmol/l IQR: 14.4 to 103.8 pmol/l) and a systemic right ventricle (RV) (31.1 pmol/l IQR: 21.8 to 56.0 pmol/l), and the lowest values were seen in patients with aortic coarctation (7.3 pmol/l IQR: 2.8 to 19.5 pmol/l). NT-proBNP levels correlated with age (r = 0.39, p < 0.001) and were higher in women (median of 21.7 vs. 10.4 pmol/l; p < 0.001). In patients with aortic stenosis or aortic coarctation, NT-proBNP levels correlated with diastolic function parameters of E/E′ ratio (r = 0.40, p < 0.001) and left atrial dimension (r = 0.36, p < 0.001). In patients with a systemic RV, NT-proBNP levels correlated with RV annulus diameter (r = 0.31, p = 0.024). In patients with tetralogy of Fallot, the strongest correlations were observed with left atrial dimension (r = 0.46, p < 0.001) and left ventricular ejection fraction (r = 0.37, p < 0.001). NT-proBNP levels were associated with exercise capacity (n = 198) (maximum workload: β = −0.08, p = 0.021) and peak oxygen uptake (β = −0.012, p = 0.011) in a multivariable regression model adjusted for age and sex. Conclusions NT-proBNP levels in adults with CHD clearly differ by diagnosis and are related to echocardiographic parameters and exercise capacity. Disease-specific correlations contribute to the understanding of the main hemodynamic problems per diagnosis. Follow-up data are needed to elucidate the additional prognostic value.
The objective of this study was to test the feasibility, accuracy, and reproducibility of the assessment of right ventricular (RV) volumes and ejection fraction (EF) using real-time three-dimensional ...echocardiographic (RT3DE) imaging in patients with congenital heart disease (CHD), using cardiac magnetic resonance (CMR) as a reference.
RT3DE data sets and short-axis cine CMR images were obtained in 62 consecutive patients (mean age, 26.9 +/- 10.4 years; 65% men) with various CHDs. RV volumetric quantification was done using semiautomated 3-dimensional border detection for RT3DE images and manual tracing of contours in multiple slices for CMR images.
Adequate RV RT3DE data sets could be analyzed in 50 of 62 patients (81%). The time needed for RV acquisition and analysis was less for RT3DE imaging than for CMR (P < .001). Compared with CMR, RT3DE imaging underestimated RV end-diastolic and end-systolic volumes and EF by 34 +/- 65 mL, 11 +/- 55 mL, and 4 +/- 13% (P < .05) with 95% limits of agreement of +/-131 mL, +/-109 mL, and +/-27%, as shown by Bland-Altman analyses, with highly significant correlations (r = 0.93, r = 0.91, and r = 0.74, respectively, P < .001). Interobserver variability was 1 +/- 15%, 6 +/- 17%, and 8 +/- 13% for end-diastolic and end-systolic volumes and EF, respectively.
In the majority of unselected patients with complex CHD, RT3DE imaging provides a fast and reproducible assessment of RV volumes and EF with fair to good accuracy compared with CMR reference data when using current commercially available hardware and software. Further studies are warranted to confirm our data in similar and other patient populations to establish its use in clinical practice.
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