scholarly journals Diffuse Supravalvular Aortic Stenosis: Surgical Repair in Adulthood

2009 ◽  
Vol 2009 ◽  
pp. 1-3 ◽  
Author(s):  
Giovanni Ferlan ◽  
Claudio De Pasquale ◽  
Concetta Losito ◽  
Annalisa Fiorella ◽  
Nicola Marraudino ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Three Dimensional ◽  
Ascending Aorta ◽  
Supravalvular Aortic Stenosis ◽  
Mechanical Prosthesis ◽  
Computed Tomographic ◽  
Valve Function ◽  
Normal Shape

We present the case of a 54-year-old woman in which a diffuse congenital supravalvular aortic stenosis (SVAS) was associated with a severe aortic valve incompetence and heavy calcification of the aortic annulus. Repair consisted in resection of the ascending aorta, patch augmentation of the hypoplastic aortic root and annulus, placement of a 20 mm Dacron tubular graft (Vascutek, Renfrewshire, UK) and aortic valve replacement with a mechanical prosthesis (Sorin, Turin, Italy). Follow-up echocardiography demonstrated normal prosthetic valve function and a postoperative three-dimensional computed tomographic scan showed a normal shape of the reconstructed ascending aorta.

Surgical Experience with Doty's Repair for Supravalvular Aortic Stenosis

1996 ◽  
Vol 4 (1) ◽  
pp. 29-32
Author(s):  
Balram Airan ◽  
Sunil K Kaushal ◽  
Anil Bhan ◽  
Panangipalli Venugopal
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Coronary Sinus ◽  
Innominate Artery ◽  
Left Ventricular ◽  
Supravalvular Aortic Stenosis ◽  
Coarctation Of Aorta ◽  
Valve Function ◽  
Aortic Obstruction ◽  
The Right

The surgical treatment of severe supravalvular aortic stenosis by conventional, asymmetrical, one point patch aortoplasty across the narrowed area has been associated with a substantial incidence of residual stenosis and reoperations. Almost symmetrical relief of such stenosis was achieved with Doty's extended aortoplasty in 5 patients in whom left ventricular aortic gradient was reduced from 60–170 nun Hg to 0–25 mm Hg. This technique of inserting an inverted V-shaped gusset across the stenosed segment into the right coronary sinus and non-coronary sinus, restores the aortic root geometry, provides more predictable relief of aortic obstruction and also improves aortic valve function. Associated procedures involved relief of coarctation of aorta and innominate artery stenosis in 1 case and repair of aortic valve and mitral valve in another case.

scholarly journals Quadricuspid Aortic Valve Combined with Moderate Ascending Aortic Dilatation

Aorta ◽  
2015 ◽  
Vol 03 (06) ◽  
pp. 187-190 ◽  
Author(s):  
Vladimir Uspenskiy ◽  
Alexei Osadchii ◽  
Mikhail Gordeev
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Insufficiency ◽  
Computed Tomographic Angiography ◽  
Aortic Dilatation ◽  
Quadricuspid Aortic Valve ◽  
Severe Aortic Regurgitation ◽  
Computed Tomographic ◽  
Tomographic Angiography

AbstractThe quadricuspid aortic valve is a very uncommon malformation associated with aortic insufficiency, aortic stenosis, endocarditis, and ascending aortic dilatation. We report four cases of this aortic valve malformation. One patient with severe aortic regurgitation and moderate aortic dilatation required aortic valve replacement. Three patients had mild or moderate aortic insufficiency combined with moderate ascending aortic dilatation. These patients were referred to follow-up. The presented cases demonstrate that this aortic valve malformation may not be as rare as it appears and that attention must be paid to any quadricuspid findings during computed tomographic angiography and echocardiography.

Pseudoaneurysm of the Ascending Aorta Combined with Aortic Valve Infective Endocarditis following Cardiac Operations

2011 ◽  
Vol 14 (1) ◽  
pp. 70
Author(s):  
Shengli Jiang ◽  
Tao Zhang ◽  
Bojun Li ◽  
Chonglei Rei ◽  
Tingting Chen ◽  
...  
Keyword(s):  
Infective Endocarditis ◽  
Aortic Valve ◽  
Circulatory Arrest ◽  
Ascending Aorta ◽  
Hypothermic Circulatory Arrest ◽  
Mechanical Prosthesis ◽  
Computed Tomographic ◽  
Life Threatening ◽  
Femorofemoral Bypass ◽  
Cardiac Operations

Background and Objective: Aortic pseudoaneurysms are rare but life-threatening complications of aortic procedures. Operation on the femorofemoral bypass with hypothermic circulatory arrest has been the method of choice. Iatrogenic ascending aorta pseudoaneurysm combined with infective endocarditis of the aortic valve has never been reported.Materials and Methods: We describe a case of a pseudoaneurysm of the ascending aorta at the site of an aortotomy site concomitant with infective endocarditis of the aortic valve. A contrast computed tomographic scan was the investigation technology of choice.Results: The operation was performed on femorofemoral bypass without hypothermic circulatory arrest, which provided safe re-entry and an opportunity to replace the infected aortic valve with a mechanical prosthesis and to repair the aortic defect with a patch.Conclusions: The ascending aorta pseudoaneurysm can be safely operated on with femorofemoral bypass without hypothermic cardiac arrest.

P1778Changes in size of ascending aorta in patients with bicuspid aortic valves

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M V Garcia-Ruiz ◽  
V M Becerra Munoz ◽  
J Robledo-Carmona ◽  
I Rodriguez-Bailon ◽  
E De Teresa Galvan
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Coarctation ◽  
Severe Aortic Stenosis ◽  
Adult Congenital Heart Disease ◽  
Type A ◽  
Ascending Aorta ◽  
P Value ◽  
Type B

Abstract Introduction It is well known that bicuspid aortic valve (BAV) is associated with premature valve dysfunction and ascending aorta dilation. However, limited data still exists regarding the rate of growth of the ascending aorta and the risk factors associated with it in these patients. Methods We analyzed prospectively baseline characteristics and echocardiographic data from 192 adult patients with BAV followed in an Adult Congenital Heart Disease Unit from 2007 until 2018. The exclusion criteria were: patient's without at least 2 echocardiographic examinations >6 months apart, and patients with aortic valve and/or ascending aorta surgery at baseline o at follow up (except aortic coarctation). Following the ASE guidelines two cardiologists experts in echocardiography made all the studies at baseline and follow-up where they measured the aortic root and ascending aortic diameters, as well as typified the aortic valve and analyzed the degree of stenosis (AS) and regurgitation (AR). Statistical analysis was performed using Stata 13.1, and a p value of ≤0.05 was considered significant. Results 97 patients were finally included (70 male, mean age 37.56±18.9 years). 66 patients had type A BAV (68.04%), 21 type B (21.7%), 2 type C (2.06%) and 7 unicuspid (7.22%). 19 patients had hypertension (19.59%) and 9 had a corrected aortic coarctation. In baseline echocardiogram, 20 had aortic stenosis ≥ moderate (20.6%) and 38 regurgitation ≥ grade II (39.18%). Mean diameter of ascending aorta was 35.78mm ± 6.73. With a mean follow-up time of 50.53±27.05 months, mean rate of diameter progression was 0.76 mm/year at the proximal ascending aorta (95% CI 0.6 to 1.2). Progression was significantly higher in patients with moderate or severe aortic stenosis (0.65 vs. 1.17 mm/year, p=0.021). Age, sex, hypertension, AR and high baseline diameters (≥40mm or ≥45mm) were not associated with progression. In a direct comparison between the two more frequent forms of BAV, Type A and Type B, rate of diameter progression was significantly higher in Type B (0.61 vs. 1.05 mm/year, p=0.044). In a multivariate analysis, none of the variables statistically associated to major progression were found to be predictors of growth of the ascending aorta. Conclussions In our population of BAV patients, mean rate of diameter progression at the proximal ascending aorta was 0.76 mm/year. Moderate-severe AS, and having type B BAV were associated, but not predictors, of higher growth rates.

P4642Prevalence and clinical significancy of bicuspid aortic valve diagnosed in the first year of life: a long term follow up in the paediatric age

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Cresti ◽  
S Sparla ◽  
S Stefanelli ◽  
R Navarri ◽  
P Baratta ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Insufficiency ◽  
Ascending Aorta ◽  
First Year ◽  
Live Births ◽  
Long Term Follow Up ◽  
Paediatric Age ◽  
First Year Of Life

Abstract Introduction and objectives Bicuspid aortic valve (BAV) is the most common congenital malformation, with an estimated prevalence ranging from 0,48 to 2%. Most of the studies describe complications associated with BAV, including aortic stenosis and regurgitation, infective endocarditis and aortic dilation and dissection. Very few studies have been performed in the neonatal and paediatric age, most lacking in long-term follow up. The aim of our study was to explore the prevalence of BAV and the progression of aortic valve disease and aortic dilation in paediatric patients followed from January 1996 to August 2018. Methods A prospective collection of Congenital Heart Disease (CHD) diagnosed in the first year of life was performed in our institution from January 1996 to August 2018. Patients affected by BAV were then followed yearly. BAV morphology, aortic valve dysfunction and aortic dimensions were measured. Comparisons were performed between right-left cusp fusion (R-L) and right-non coronary cusp fusion (R-N), according to Shaefer's classification. Rate of change of the ascending aorta size over time was analyzed, and aortic size values were normalized as the number of standard deviations above or below the mean size expected for body surface area (z scores). Results A total of 35,310 live births were screened. Incidence rate of total CHD, including small ventricular septal defects and BAV was19,57%. Sixty had BAV (8,73% of CHD), with an incidence of 17/1,000 live births. Male/Female ratio was 1.4. A positive family history was present in 5 (8.3%). In 12 patients (24%) BAV was associated to other CHD (9 Aortic Coarctation, 1 Atrio-Ventricular Septal Defect, and in two cases a critical neonatal aortic stenosis). BAV was isolated in 48 cases (80%), with an incidence of 13.6/1.000 live births. According to Shaefer's classification the most common was type 1 (R-L), with Right to Left cusps fusion (R-L 76%, R-N 21% and L-N 3%). Among patients with isolated BAV, after a median follow up of 11 [1–21] years an aortic stenosis was observed in 5 patients (10.4%, none severe), an aortic insufficiency in 13 (27%, in one case severe). A dilated ascending aorta (z-score>2) was found in 11 patients (23%). An indication cardiac surgery due to severe aortic insufficiency was present in one case. No patient had indication for aortic aneurysm surgery. Conclusions Paediatric patients with isolated BAV warrant medical follow-up but progression towards severe valve dysfunction and/or dilation of the ascending aorta warranting cardiac surgery is very low.

Abstract 19440: Reintervention Rates Following Balloon Aortic Valvuloplasty versus Surgical Valve Repair in Isolated Congenital Aortic Valve Stenosis - A Meta-analysis

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
May T Saung ◽  
Courtney McCracken ◽  
Ritu Sachdeva ◽  
Christopher J Petit
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Stenosis ◽  
Meta Analysis ◽  
Valve Repair ◽  
Balloon Aortic Valvuloplasty ◽  
Congenital Aortic Stenosis ◽  
Primary Indication ◽  
Aortic Valvuloplasty

Introduction: The optimal treatment for congenital aortic stenosis (AS) is debated despite decades of experience with both balloon aortic valvuloplasty (BAV) and surgical aortic valve repair (SAV). While BAV has been the mainstay of therapy for AS, recent single-center reports suggest optimal results following SAV. Hypothesis: We propose that reintervention rates following SAV and BAV are equivalent. Methods: We queried Medline, EMBASE and Web of Science for eligible studies using the keywords: “congenital aortic stenosis”, “balloon valvotomy”, “aortic valve stenosis surgery” and “treatment outcome or reintervention”. Studies were excluded when cohort size was <20 pts, when follow-up was < 2.5 yrs from primary intervention, and when primary indication was not AS (e.g. SAV in the setting of aortic valve regurgitation (AR)). Outcomes analyzed included death, reintervention and moderate or severe AR. Analysis was performed using Comprehensive Meta Analysis v3 using random effects models. Results: A total of 20 studies were included in our meta-analysis: SAV alone (n=3), BAV alone (n=12), and both (n=5). The mean age at BAV was 3.1 years (range, 4 days - 7 years) with a mean follow-up duration of 6.8 years, while mean age at SAV was 2.8 years (range, 14.2 days - 7.1 years) with a mean follow-up duration of 9.1 years. Mortality rates following BAV and SAV were 12.3% (95% CI: 7.7 - 19.1) and 10.2% (95% CI: 7.0 - 14.5), respectively (p=0.27). Reintervention following initial procedure for treatment of AS was higher following BAV (35.7% [95% CI: 29 - 43.1]) compared to SAV (25.2% [95% CI: 19.9 - 31.3])(p=0.012). Long-term and mid-term follow-up in these studies showed moderate to severe AR was present in 24.1% and 28.1% of BAV and SAV patients, respectively. Conclusions: Notwithstanding publication bias, both survival rates and development of late AR following BAV and SAV are similar. However, reintervention rates are significantly higher following BAV compared to SAV.

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