scholarly journals Developmental changes in bicuspid aortic valve and associated aortopathy in small children

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
J Boesgaard Norsk ◽  
A S Sillesen ◽  
A Axelsson Raja ◽  
M Munk Paerregaard ◽  
C Pihl ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Flow Velocity ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Early Years ◽  
Z Score ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Valve Annulus

Abstract Background The prevalence of bicuspid aortic valve (BAV) in newborns is 0.8%. BAV is associated with an increased risk of aortic valve dysfunction and aortopathy. Aortopathy with increased aortic diameters has recently been reported in newborns with BAV. As most patients with BAV are diagnosed in adulthood the development of BAV and associated aortopathy during early years of life is not well described. Purpose The purpose of the study was to assess changes in aortic valve function and aortic dimensions at two to four years of age in children diagnosed with BAV neonatally. Methods Children with BAV were included from a population-based cohort study, in which newborns (n≈25,000) underwent standardized transthoracic echocardiography (TTE). Follow-up TTE was performed and analyzed according to established guidelines. Neonatal (baseline) and follow-up data were compared. Diameters were indexed to body surface area (BSA). Z-scores were calculated using formulas from the Pediatric Heart Network Echocardiogram Database. Results At follow-up 101 newborns with BAV (mean age 2 years 5 months, SD 11 months), 75% male) were examined from May 2019 to April 2021. From baseline to follow-up there was an increase in the BSA-adjusted diameter of the aortic valve annulus (z-score −0.19 at baseline vs 0.95 at follow up, p<0.001), and of the sino-tubular junction (z-score 0.16 at baseline vs 0.43 at follow up, p<0.05). Conversely, there was a decrease in the Z-score for the diameter measured 1 cm from the valve annulus (z-score 1.31 at baseline vs 0.68 at follow up, p<0.001) and at the widest point of the visualized ascending aorta (z-score 2.44 at baseline vs 1.45 at follow up, p<0.001). There was no significant change in the diameter of the sinus of Valsalva (z-score 0.83 at baseline vs 0.80 at follow up, p=0.92). The number of children with at least one aortic z-score >3 was 31 (30.6%) at baseline and 17 (17.8%) at follow up. At baseline mild aortic valve regurgitation was observed in 18 children (17.8%) and in 23 children at follow up (23%). Mean maximum systolic velocities across the aortic valve were 1.03 m/s at baseline (SD 0.24) and 1.11 m/s at follow up (SD 0.27), p<0.05. Aortic stenosis, defined as flow velocity >2.5 m/s was seen in one child both at baseline and at follow-up. Conclusion In children diagnosed with BAV neonatally, re-examination at the age of 2.5 years showed significant increases in the diameter of the aortic valve annulus and the sino-tubular junction, but a significant decrease in the diameter of the ascending aorta. The maximum blood flow velocity across the aortic valve increased and more children had developed mild aortic valve regurgitation. Thus, the bicuspid aortic valve and the associated aortopathy seem to undergo remodeling during early childhood. FUNDunding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The Danish Children's Heart Foundation, Boernehjertefonden.

Prevalence of aortic valve regurgitation in a large population-based study of newborns

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Farooqui ◽  
J.K Bjerrekaer ◽  
M.F Boerresen ◽  
R.O Voegg ◽  
C.A Pihl ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Population Based ◽  
Left Ventricular ◽  
Ascending Aorta ◽  
Large Cohort ◽  
Funding Source ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
True Prevalence

Abstract Background Congenital aortic valve regurgitation (AVR) has been estimated to be present in 0.25 per 1,000 live births globally. The true prevalence in an unselected large-scale cohort remains unknown. Presence of congenital AVR could indicate a structural valve abnormality and may result in long-term strain on the heart. Aim To find the true prevalence of AVR in a large cohort of unselected newborns. Methods Between April 2016 and October 2018, 25,752 newborns were consecutively included in a population-based prospective multicenter cohort and examined with transthoracic echocardiography within the first 30 days of life. Examinations with AVR were categorized as trivial AVR by the presence of a trivial diastolic transvalvular flow, or as non-trivial AVR by the presence of a significantly larger and visually observable transvalvular flow. In newborns with non-trivial AVR, the vena contracta, the duration of the transvalvular flow, and the duration of the diastole were measured. The cases were matched 1:4 with newborns with no AVR based on sex, gestational age at birth, birth weight and age at time of examination. Results AVR was identified in 329 newborns (1.3%). Of these, 260 were classified as trivial AVR, 69 (0.27%) were non-trivial AVR. Non-trivial AVR had significantly larger aortic roots at all points of measurement compared to controls (mean±SD): aortic valve annulus (7.3 (0.73) vs. 7.0 (0.63) mm), Sinus of Valsalva (10.3 (1.0) vs. 9.7 (1.0) mm), sino-tubular junction (8.4 (0.8) vs. 8 (0.8) mm), and ascending aorta (10 (0.9) vs. 9.4 (1.1) mm) (p<0.01 for all). Left ventricular dimensions and function did not significantly differ between the cases and controls. The presence of a non-trivial AVR was highly associated with a bicuspid aortic valve (9 (13.0%) vs. 3 (1.1%), p<0.001). Conclusions We found a prevalence of congenital AVR of 1.3% in a large cohort of unselected newborns, which is higher than previously reported. One in five of all AVR found were classified as non-trivial. Newborns with non-trivial AVR presented with larger aortic root and ascending aorta dimensions. Newborns with non-trivial AVR also had a ten-fold higher occurrence of bicuspid aortic valve compared to controls. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Hjerteforeningen

scholarly journals Comparison of bicuspid and tricuspid aortic valve repair

2020 ◽  
Author(s):  
Radosław Gocoł ◽  
Jarosław Bis ◽  
Marcin Malinowski ◽  
Joanna Ciosek ◽  
Damian Hudziak ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Hazard Ratio ◽  
Aortic Valve Repair ◽  
Pericardial Patch ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Valve Repair ◽  
Tricuspid Aortic Valve ◽  
Valve Annulus ◽  
Annulus Diameter

Abstract   OBJECTIVES The aim of this study was to compare the outcomes of tricuspid aortic valve (TAV) and bicuspid aortic valve (BAV) repair. METHODS We assessed mortality, freedom from reoperation and the rate of aortic valve regurgitation recurrence. Mortality in both groups was compared with expected survival, and risk factors for reoperation were identified. RESULTS From January 2010 to April 2020, a total of 368 elective aortic valve repair procedures were performed, including 223 (60.6%) in patients with TAV. The perioperative mortality was 0.7% in the BAV group and 3.6% in the TAV group (P = 0.079). Estimated survival at 5 years in the BAV versus TAV group was 97 ± 3% vs 80 ± 6%, respectively (P < 0.001). Freedom from reoperation at 5 years in the TAV versus BAV group was 96 ± 3% vs 93 ± 4%, respectively (P = 0.28). Grade 2 or more aortic valve regurgitation was noted in 9.9% of BAV patients and 11% of TAV patients (P = 0.66). Reoperation was predicted by cusp perforation [hazard ratio 15.86 (4.44–56.61); P < 0.001], the use of pericardial patch [hazard ratio 8.58 (1.96–37.53); P = 0.004] and aortic valve annulus diameter >27.5 mm [hazard ratio 3.07 (0.99–9.58); P = 0.053]. CONCLUSIONS BAV repair is as durable as TAV repair. BAV is not a predictor of a higher rate of reoperations. BAV repair yields survival comparable to expected. Cusp perforation, aortic valve annulus diameter >27.5 mm and the use of pericardial patch adversely impact long-term outcome of aortic valve repair.

scholarly journals Fate of mild-to-moderate bicuspid aortic valve disease untreated during ascending aorta replacement

2020 ◽  
Author(s):  
Alessandro Verzini ◽  
Marta Bargagna ◽  
Guido Ascione ◽  
Alessandra Sala ◽  
Davide Carino ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Term Survival ◽  
Kaplan Meier ◽  
Aortic Root Surgery ◽  
Increased Risk ◽  
Aorta Replacement ◽  
Ascending Aorta Replacement

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and it is responsible for an increased risk of developing aortic valve and ascending aorta complications. In case of mild to moderate BAV disease in patients undergoing supracoronary ascending aorta replacement, it is unclear whether a concomitant aortic valve replacement should be performed. Methods: From June 2002 to January 2020, 75 patients with mild-to-moderate BAV regurgitation (± mild-to-moderate stenosis) who underwent isolated supracoronary ascending aorta replacement were retrospectively analyze. Clinical and echocardiographic follow-up was 100% complete (mean: 7.4±3.9 years, max 16.4). Kaplan Meier estimates were employed to analyze long-term survival. Cumulative incidence function for time to re-operation, recurrence of aortic regurgitation (AR)≥3+ and aortic stenosis (AS) greater than moderate, with death as competing risk, were computed. Results: There was no hospital mortality and no cardiac death occurred. Overall survival at 12 years was 97.4±2.5%, 95% CI [83.16-99.63]. At follow-up there were no cases of aortic root surgery whereas 3 patients underwent AV replacement. At 12 years the CIF of reoperation was 2.6±2.5%, 95% CI [0.20-11.53]. At follow up, AR 3+/4+ was present in 1 pt and AS greater than moderate in 3. At 12 years the CIF of AR>2+/4+ was 5.1±4.98% and of AS>moderate 6.9±3.8%. Conclusions: In our study mild to moderate regurgitation of a BAV did not significantly worse at least up to 10 years after isolated supracoronary ascending aorta replacement.

scholarly journals Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

Heart ◽  
1992 ◽  
Vol 67 (6) ◽  
pp. 439-441 ◽  
Author(s):  
A S Sadee ◽  
A E Becker ◽  
H A Verheul ◽  
B Bouma ◽  
G Hoedemaker
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Pathological Correlation

Valve Repair for Bicuspid Aortic Valve Regurgitation: An Option?

2007 ◽  
Vol 60 (2) ◽  
pp. 209-212
Author(s):  
Irene Méndez ◽  
Belén Prado ◽  
Pastora Gallego ◽  
Antonio Castro ◽  
José M. Barquero ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Valve Repair

scholarly journals Impact of Mild Aortic Valve Disease on Late Outcomes after Replacement of the Ascending Aorta Case-control Study

2021 ◽  
Author(s):  
Ken Nakamura ◽  
Kouan Orii ◽  
Taichi Kondo ◽  
Mitsutaka Nakao ◽  
Makoto Wakatabe ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Stenosis ◽  
Aortic Valve Disease ◽  
Ascending Aorta ◽  
Valve Disease ◽  
Aortic Valve Area ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Tricuspid Aortic Valve ◽  
Valve Area

Abstract Background: The time course of mild aortic valve disease after replacement of the ascending aorta is unclear. We sought to clarify it.Methods: Between January 2011 and December 2016, 26 patients (9 bicuspid and 17 tricuspid aortic valve disease) underwent replacement of the enlarged ascending aorta alone. We compared the postoperative disease course between bicuspid and tricuspid aortic valve by transthoracic echocardiography and computed tomography performed at 73 ± 23 and 60 ± 23 months post-surgery, respectively.Results: The bicuspid group was younger than the tricuspid group (62.1 ± 4.8 vs 73.3 ± 4.8 years). Pathophysiology predominantly involved aortic valve stenosis and regurgitation in the bicuspid and tricuspid group, respectively. The peak and mean pressure gradient increased (P = .16, and P = .46) and the aortic valve area decreased significantly in the bicuspid group (P = .005). Two patients in the bicuspid group who required re-operation had an aortic valve area of 1.2 cm2 at initial operation. Seventy percent of patients in the tricuspid group had less than mild aortic valve regurgitation preoperatively, which improved up to 82% at follow-up. Freedom from re-operation was 66.7% and 100% at 8 years for the bicuspid and tricuspid group, respectively. Conclusions: Aortic valve replacement may be considered for patients with bicuspid aortic valve stenosis if the aortic valve area is less than 1.2 cm2, even if the general diagnosis is mild aortic valve stenosis at initial surgery. Even mild aortic valve regurgitation may be improved by surgical intervention in the ascending aorta.

scholarly journals Aortic tortuosity is related to aortic phenotype in patients with bicuspid aortic valve: a CT scan study of 83 cases

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
T Wallet ◽  
O Milleron ◽  
L Eliahou ◽  
J F Paul ◽  
F Arnoult ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Ascending Aorta ◽  
Ct Scans ◽  
Z Score ◽  
Funding Sources ◽  
The Relationship ◽  
Tubular Diameter ◽  
Tubular Dilatation

Abstract Background Although the incidence of aortic dissection is higher in patients with bicuspid aortic valve (BAV) compared to tricuspid aortic valve (TAV), risk stratification remains unclear. Guidelines focus on ascending aorta diameters, regardless of the location, and do not take into account the morphology of the aorta. Aortic tortuosity (AT) is emerging as a novel biomarker associated with more severe aortopathy in patients with Marfan syndrome. AT has not been accuretely assessed in BAV. Our aim is to describe the relationship between AT and ascending aortic phenotype in patients with BAV. Methods 83 patients (43±16 years, 19 women) diagnosed with BAV and without significant aortic valve disease nor prior aortic intervention were included. CT scans were retrospectively analysed with measurements of aortic diameters and aortic tortuosity. For 61 patients with abdominal images available, descending and total aortic length and tortuosity were measured. Results In our cohort, 62 (75%) patients presented a typical BAV. Pathological aorta (Root and/or tubular Z-score >2) was found in 80 patients (96%) and 67 (81%) presented a tubular dilatation. The aortic phenotype, the maximal aortic diameters and aortic tortuosity index were similar in typical and atypical BAV. Total aortic tortuosity index was correlated to Z-score tubular diameter (r=0.31; p=0,014) but not with Z-score Valsalva diameter (p=0,55). In patients with tubular dilatation (Z score >2), total aortic tortuosity index was higher than in patient without tubular dilatation (2.01 vs 1.85; p=0,015). Conclusion Total aortic tortuosity is associated with tubular dilatation but not with root dilatation in BAV patients suggesting that tubular phenotype may be at higher risk of complication in BAV. Further studies evaluating the association between aortic tortuosity and clinical outcomes in BAV are needed. FUNDunding Acknowledgement Type of funding sources: None.

Sign in / Sign up

Export Citation Format

Share Document