congenital aortic stenosis
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2021 ◽  
pp. 021849232110504
Author(s):  
Motonori Ishidou ◽  
Keiichi Hirose ◽  
Akio Ikai ◽  
Kisaburo Sakamoto

A boy was diagnosed with a unicuspid aortic valve with severe stenosis at birth. Percutaneous balloon aortic valvuloplasty was performed four times; however, he had severe heart and growth failure. Thus, aortic valve repair was performed at age 2 years and 6 months. He weighed 6.6 kg. The aortic valve annulus diameter was 9.8 mm (z value = 0.43). We report on a small toddler with congenital aortic stenosis who was successfully treated with autologous pericardial aortic valve leaflet reconstruction using the open-sleeve technique.


2021 ◽  
Vol 14 (7) ◽  
pp. e243878
Author(s):  
Ahmed Ashraf Abdelhamid ◽  
Takaaki Kobayashi ◽  
Joseph Tholany ◽  
Poorani Sekar

A 29-year-old man with a history of congenital aortic stenosis and mechanical aortic valve replacement with previous Cutibacterium acnes prosthetic valve endocarditis (PVE) presented with a 2-week history of fevers and night sweats. Transoesophageal echocardiogram revealed a 0.6 cm×0.5 cm vegetation on the mechanical aortic valve. An anaerobic blood culture became positive for C. acnes 6 days after the blood cultures were obtained. He did not have any surgical intervention. He was successfully treated with 6 weeks of ceftriaxone, followed by chronic suppression with oral doxycycline. Despite its low virulence, a growing number of C. acnes PVE cases have been reported, owing to its biofilm production. When clinical suspicion is high, extending culture incubation duration beyond the standard 5 days might be helpful. Most cases are treated with surgical repair or replacement in conjunction with antibiotics, but medical therapy alone has been documented as being successful.


2021 ◽  
Vol 4 (12) ◽  
pp. 01-28
Author(s):  
P. Syamasundar Rao

Balloon aortic valvuloplasty (BAV) provides an excellent alternative to surgical intervention and has become the preferred intervention for initial palliation for aortic stenosis in neonates, infants, children, adolescents, and young adults. The elderly patients with calcific aortic stenosis do not benefit from BAV. With the exception of neonates, most patients can be discharged home within 24-hours of the procedure. Although there is definitive evidence for pressure gradient relief immediately after as well as at follow-up and postponement of surgical intervention following BAV, the progression of aortic insufficiency at late follow up remain a major concern. In the neonatal population, severe aortic insufficiency may develop requiring surgical intervention. Despite these limitations, balloon aortic valvuloplasty is currently considered as therapeutic procedure of choice in the management of congenital aortic stenosis in the pediatric and young adult population. Careful follow-up to detect recurrence of stenosis and development of significant aortic insufficiency is recommended.


Author(s):  
Takashi Kido ◽  
Alvise Guariento ◽  
Ilias P. Doulamis ◽  
Diego Porras ◽  
Christopher W. Baird ◽  
...  

Background: We sought to identify predictive factors for aortic valve (AoV) surgery after neonatal balloon aortic valvuloplasty (BAV) and characterize clinical outcomes of AoV surgery after neonatal BAV. Methods: Time-to-event analysis identified predictors for AoV surgery after neonatal BAV. Clinical outcomes of AoV surgery following neonatal BAV were examined. Results: This study included 96 consecutive patients who underwent neonatal BAV for congenital aortic stenosis between 1998 and 2018, in 26 of whom a fetal BAV had been performed. Fifty-six patients underwent AoV surgery at a median age of 2.0 years. Significant risk factors for AoV surgery in univariate Cox regression (result presented as hazard ratio [HR], [95% CI]; P value) were a history of fetal BAV (HR, 4.05 [95% CI, 2.19–7.40]; P <0.001), AoV annulus diameter Z score (HR, 0.56 [95% CI, 0.43–0.75]; P =0.001), the presence of endocardial fibroelastosis (HR, 2.61 [95% CI, 1.48–4.51]; P =0.001), severe left ventricular dysfunction before neonatal BAV (HR, 1.75 [95% CI, 1.03–2.97]; P =0.04), and recent era (HR, 3.08 [95% CI, 1.68–5.91]; P =0.0002) in the entire cohort. Area under the receiver operating characteristic curve and Youden J index analysis identified a cutoff value for AoV annulus diameter Z score of −2.6 in patients without fetal BAV. In 24 patients with midterm cardiac catheterization data, univariate linear regression analysis (result presented as B coefficient [95% CI]; P ) showed that the presence of greater-than-moderate aortic regurgitation immediately after BAV (B coefficient, 4.8 [95% CI, 1.0–8.6]; P =0.018) and before AoV surgery (B coefficient, 6.1 [95% CI, 2.2–10.0]; P =0.004) were significant risk factors for elevated left ventricular end-diastolic pressure after AoV surgery, while concomitant endocardial fibroelastosis resection at AoV surgery had a protective effect (B coefficient, −3.8 [95% CI, −7.6 to −0.06]; P =0.05). Conclusions: A small AoV annulus diameter Z score with a cutoff value of −2.6 and a history of fetal BAV were significantly associated with AoV surgery after neonatal BAV. Concomitant endocardial fibroelastosis resection is recommended at AoV surgery following neonatal BAV to improve left ventricular diastolic function.


2021 ◽  
pp. 1-7
Author(s):  
Benjamin C. Auld ◽  
Julia S. Donald ◽  
Naychi Lwin ◽  
Kim Betts ◽  
Nelson O. Alphonso ◽  
...  

Abstract Background: Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon centre bias with limited relevant, comparative literature. Objectives: This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches. Methods: Retrospective analysis of patients with congenital aortic valve stenosis who underwent balloon valvuloplasty (Queensland Children’s Hospital, Brisbane) or surgical valvotomy (Royal Children’s Hospital, Melbourne) between 2005 and 2016. Patients were excluded if pre-intervention assessment indicated ineligibility to either group. Propensity score matching was performed based on age, weight, and valve morphology. Results: Sixty-five balloon patients and seventy-seven surgical patients were included. Overall, the groups were well matched with 18 neonates/25 infants in the balloon group and 17 neonates/28 infants in the surgical group. Median age at balloon was 92 days (range 2 days – 18.8 years) compared to 167 days (range 0 days – 18.1 years) for surgery (rank-sum p = 0.08). Mean follow-up was 5.3 years. There was one late balloon death and two early surgical deaths due to left ventricular failure. There was no significant difference in freedom from reintervention at latest follow-up (69% in the balloon group and 70% in the surgical group, p = 1.0). Conclusions: Contemporary analysis of balloon aortic valvuloplasty and surgical aortic valvotomy shows no difference in overall reintervention rates in the medium term. Balloon valvuloplasty performs well across all age groups, achieving delay or avoidance of surgical intervention.


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