Bicuspid aortic valve disease and competitive sports: key considerations and challenges

2019 ◽  
pp. 233-238
Author(s):  
Benjamin S. Wessler ◽  
Natesa G. Pandian
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Dilation ◽  
Valvular Stenosis ◽  
Valvular Function ◽  
A Minor ◽  
Valve Lesion

Bicuspid aortic valve (BAV) is a common congenital disorder. It could simply be a minor anatomic abnormality or be associated with progressive aortic stenosis, aortic regurgitation, and aortic dilation. If an athlete is recognized to have a BAV, questions arise with regard to whether they can pursue their selected sports, particularly elite athletic activity, and what type of follow-up examinations are necessary and how often should be done. Valvular disorders such as the degree of aortic stenosis and aortic regurgitation, aortic size, and coexisting disorders are also influencing factors. The absence of robust controlled studies, which are difficult to perform, make decision-making difficult, although recommendations by expert panels provide some guidance. The general consensus is that athletes with BAV with normal valvular function and no aortic dilation can participate in all athletic activities. Those with mild aortic dilation should undergo annual screening, some more frequently than others. Those with moderate or severe valvular stenosis or regurgitation should be managed based on the haemodynamic impact of the valve lesion. Athletes with coexisting lesions or syndromes should be evaluated comprehensively. The overall recommendation to an individual athlete should incorporate many factors and employ a multidisciplinary approach.

scholarly journals The Immediate And Intermediate Results Of Percutaneous Balloon Aortic Valvuloplasty In Patients With Congenital Valvular Aortic Stenosis

2019 ◽  
Vol 13 (2) ◽  
pp. 51-55
Author(s):  
Hussein A. Alwahab
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Pressure Gradient ◽  
Aortic Regurgitation ◽  
Balloon Aortic Valvuloplasty ◽  
Maximum Peak ◽  
Percutaneous Balloon ◽  
Aortic Valvuloplasty ◽  
Valvular Aortic Stenosis

Background: Aortic valve stenosis results from minor to severe degrees of aortic valve maldevelopment. This stenosis causes mild to severe obstruction of the left ventricular outflow . Objectives : to study the immediate and intermediate results of percutaneous balloon aortic valvuloplasty in patients with congenital valvular aortic stenosis . Type of the study: A prospective study. Methods: The study was done on thirty five patients with congenital valvular aortic stenosis who had percutaneous balloon aortic valvuloplasty  in Ibn Al- Bitar Center for Cardiac Surgery from May 2009 to February 2011. Results: Twenty seven patients were male (77.2%) and 8 patients were female (22.8%), male to female ratio 3.5/ 1, . The aortic valve was bicuspid in 18 patients (51.4%) while 17 patients ( 48.6%) had tricuspid aortic valve. Balloon aortic valvuloplasty was successful in 30 patients (85.7%),. Maximum peak instantaneous Doppler pressure gradient across the aortic valve 24 hours postprocedural echocardiography showed reduction which is statistically significant. New aortic regurgitation had occurred in 15 patients ( 42.8%), it was mild in 9 patients ( 25.6%), moderate in 5 patients ( 14.3%) and severe in 1 patient( 2.9%) which is statistically significant. The follow up of 12.57 ± 3.88 ( 3- 22) months after intervention was done for all patients using echocardiography Doppler study, reveal the maximum peak instantaneous Doppler pressure gradient  across the aortic valve was raised  which is statistically significant. The aortic regurgitation was present in 18 patients (51.4%) , it was mild in 9 patients (25.7%) , moderate in 6 patients (17.1%) and severe in 3 patients  ( 8.6%). No mortality had been reported during the procedure or on follow up.                                                                                  Conclusion: Aortic balloon valvuloplasty is safe and effective procedure in the treatment of congenital valvular aortic stenosis but mild aortic regurgitation is the most common immediate complication of aortic balloon dilatation and progressive aortic regurgitation is a major problem during the intermediate follow up.                                                                                                                                               

scholarly journals Balloon Valvuloplasty for Congenital Aortic Stenosis: Experience at a Tertiary Center in a Developing Country

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Fatme A. Charafeddine ◽  
Haytham Bou Houssein ◽  
Nadine B. Kibbi ◽  
Issam M. El-Rassi ◽  
Anas M. Tabbakh ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Developing Country ◽  
Aortic Regurgitation ◽  
Developed Countries ◽  
Left Ventricular ◽  
Complication Rates ◽  
Congenital Aortic Stenosis ◽  
Tertiary Center

Background. Aortic valve stenosis accounts for 3–6% of congenital heart disease. Balloon aortic valvuloplasty (BAV) is the preferred therapeutic intervention in many centers. However, most of the reported data are from developed countries. Materials and Methods. We performed a retrospective single-center study involving consecutive eligible neonates and infants with congenital aortic stenosis admitted for percutaneous BAV between January 2005 and January 2016 to our tertiary center. We evaluated the short- and mid-term outcomes associated with the use of BAV as a treatment for congenital aortic stenosis (CAS) at a tertiary center in a developing country. Similarly, we compared these outcomes to those reported in developed countries. Results. During the study period, a total of thirty patients, newborns (n = 15) and infants/children (n = 15), underwent BAV. Left ventricular systolic dysfunction was present in 56% of the patients. Isolated AS was present in 19 patients (63%). Associated anomalies were present in 11 patients (37%): seven (21%) had coarctation of the aorta, two (6%) had restrictive ventricular septal defects, one had mild Ebstein anomaly, one had Shone’s syndrome, and one had cleft mitral valve. BAV was not associated with perioperative or immediate postoperative mortality. Immediately following the valvuloplasty, a more than mild aortic regurgitation was noted only in two patients (7%). A none-to-mild aortic regurgitation was noted in the remaining 93%. One patient died three months after the procedure. At a mean follow-up of 7 years, twenty patients (69%) had more than mild aortic regurgitation, and four patients (13%) required surgical intervention. Kaplan–Meier freedom from aortic valve reintervention was 97% at 1 year and 87% at 10 years of follow-up. Conclusion. Based on outcomes encountered at a tertiary center in a developing country, BAV is an effective and safe modality associated with low complication rates comparable to those reported in developed countries.

Abstract 18719: Relationship Between Proximal Aorta Morphology and Progression Rate of Aortic Stenosis

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Romain Capoulade ◽  
Philipp Bartko ◽  
Jonathan G Teoh ◽  
Elisa Teo ◽  
Yong H Park ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Bicuspid Aortic Valve ◽  
Morphological Changes ◽  
Progression Rate ◽  
Normal Range ◽  
Sinotubular Junction ◽  
Arterial Hemodynamics ◽  
Different Levels

Background: Morphological changes of the proximal aorta, such as effacement of the sinotubular junction (STJ), may result in increased mechanical stress on the aortic valve leaflets and contribute to calcification and progression of aortic stenosis (AS). The aim of this study was to examine the association between abnormal morphology of proximal aorta and AS progression rate. Methods: Between 2010 and 2012, 426 patients with mild to moderate AS (peak aortic jet velocity >2.5 and <4 m/s) and LVEF≥50% with at least two years of follow up were included in this study. Aortic dimensions were measured at 3 different levels: sinus of Valsalva (SVal), STJ and ascending aorta (Aa). The ratios of SVal by STJ (SVal/STJ) and Aa by STJ (Aa/STJ) were used to determine degree of aortic deformity with smaller ratios consistent with greater perturbation of normal geometry. SVal/STJ<1.13 and Aa/STJ<1.09 were defined as significant low ratios per normal range reported in Guidelines. AS progression rate was assessed by annualized increase in mean gradient (MG; follow-up time = 3.2±0.8 yrs). Results: Mean age was 71±13 yrs and 64% were male. 16% had bicuspid aortic valve and MG was 21±8 mmHg. SVal, STJ and Aa dimensions were respectively 33±4 mm, 27±4 mm and 36±5 mm. Mean SVal/STJ ratio was 1.21±0.15 and Aa/STJ ratio was 1.29±0.19. Patients with significant low ratios had faster AS progression (p≤0.05; figure). After adjustment for age, gender, hypertension, diabetes, renal disease, bicuspid aortic valve, baseline MG, LVEF, aortic regurgitation and indexed STJ, SVal/STJ (p=0.025) or Aa/STJ (p=0.027) were independently associated with faster AS progression. Conclusion: Abnormal aortic root geometry such as effacement of the sinotubular junction is a strong and independent predictor of faster AS progression, regardless of arterial hemodynamics, aortic valve phenotype and baseline AS severity. This finding suggests an interrelation between proximal aorta morphology and stenosis progression.

scholarly journals Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease

Heart ◽  
2017 ◽  
Vol 104 (7) ◽  
pp. 566-573 ◽  
Author(s):  
Arturo Evangelista ◽  
Pastora Gallego ◽  
Francisco Calvo-Iglesias ◽  
Javier Bermejo ◽  
Juan Robledo-Carmona ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Clinical Predictors ◽  
Valve Dysfunction ◽  
Tertiary Hospitals ◽  
Male Sex

ObjectiveBicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications.MethodsEight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established.ResultsThree BAV morphotypes were identified: right–left coronary cusp fusion (RL) in 72.9%, right–non-coronary (RN) in 24.1% and left–non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN.ConclusionsNormofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.

scholarly journals Bicuspid aortic valve with severe aortic stenosis: a case report

2016 ◽  
Vol 44 (2) ◽  
pp. 105-108
Author(s):  
Redoy Ranjan ◽  
Md Mushfiqur Rahman ◽  
Omar Sadeque Khan ◽  
Md Aftabuddin ◽  
Asit Baran Adhikary
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Replacement ◽  
Aortic Regurgitation ◽  
Valve Replacement ◽  
Bicuspid Aortic Valve ◽  
Severe Aortic Stenosis ◽  
Color Doppler ◽  
Flow Through ◽  
High Record

A bicuspid aortic valve (BAV) can be a serious disorder of heart valve in which the valve only has two leaflets or flaps that control blood flow through the heart. Between one and two percent of all people have this defect and it affects more men than women. This report presents a case of severe aortic stenosis with mild to moderate aortic regurgitation due to bicuspid aortic valve with hypertension. A 37 years old male presented with high record of blood pressure and occasional shortness of breath on exertion. Echocardiography (Color Doppler) revealed severe aortic stenosis with mild to moderate aortic regurgitation due to bicuspid aortic valve with moderately severe concentric LV wall hypertrophy. Surgical treatment (aortic valve replacement) was scheduled based on echocardiography findings. On surgical resection a well defined bicuspid aortic valve was found with calcification and friable valve leaflet. Histopathology of valve tissue shows large areas of calcification. Patient was discharged from hospital on 7th POD with an advice to attend cardiac surgery OPD after 1 month. Aortic valve replacement must be considered in this type of lesion.Bangladesh Med J. 2015 May; 44 (2): 105-108

A Larger Aortic Annulus Causes Aortic Regurgitation and a Smaller Aortic Annulus Causes Aortic Stenosis in Bicuspid Aortic Valve

2008 ◽  
Vol 25 (3) ◽  
pp. 242-248 ◽  
Author(s):  
Makoto Sonoda ◽  
Katsu Takenaka ◽  
Kansei Uno ◽  
Aya Ebihara ◽  
Ryozo Nagai
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Annulus

scholarly journals Progression of Aortic Stenosis in Patients with Bicuspid Aortic Valve

2021 ◽  
Author(s):  
Michael Shang ◽  
Arianna Kahler-Quesada ◽  
Makoto Mori ◽  
Sameh Yousef ◽  
Arnar Geirsson ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Bicuspid Aortic Valve ◽  
Proportional Hazards Model ◽  
Cox Proportional Hazards Model ◽  
Rapid Progression ◽  
Progression Rate

Background: Bicuspid aortic valve is the most common congenital heart defect and predisposes patients to developing aortic stenosis more frequently and at a younger age than the general population. However, the influence of bicuspid aortic valve on the rate of progression of aortic stenosis remains unclear. Methods: In 236 patients (177 tricuspid aortic valve, 59 bicuspid aortic valve) matched by initial severity of mild or moderate aortic stenosis, we retrospectively analyzed baseline echocardiogram at diagnosis with latest available follow-up echocardiogram. Baseline comorbidities, annualized progression rate of hemodynamic parameters, and hazard of aortic valve replacement were compared between valve phenotypes. Results: Median echocardiographic follow-up was 2.6 (IQR 1.6-4.2) years. Patients with tricuspid aortic stenosis were significantly older with more frequent comorbid hypertension and congestive heart failure. Median annualized progression rate of mean gradient was 2.3 (IQR 0.6-5.0) mmHg/year vs. 1.5 (IQR 0.5-4.1) mmHg/year (p=0.5), and that of peak velocity was 0.14 (IQR 0-0.31) m/s/year vs. 0.10 (IQR 0.04-0.26) m/s/year (p=0.7) for tricuspid vs. bicuspid aortic valve, respectively. On multivariate analyses, bicuspid aortic valve was not significantly associated with more rapid progression of aortic stenosis. In a stepwise Cox proportional hazards model adjusted for baseline mean gradient, bicuspid aortic valve was associated with increased hazard of aortic valve replacement (HR: 1.7, 95% CI [1.0, 3.0], p=0.049). Conclusion: Bicuspid aortic valve may not significantly predispose patients to more rapid progression of mild or moderate aortic stenosis. Guidelines for echocardiographic surveillance of aortic stenosis need not be influenced by valve phenotype.

scholarly journals Long-term outcomes in patients undergoing transcatheter aortic valve implantation: insights on clinical outcomes and prognostic markers from a large cohort of patients

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
M Munoz-Garcia ◽  
E Munoz-Garcia ◽  
A J Munoz Garcia ◽  
A J Dominguez-Franco ◽  
J H Alonso-Briales ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Aortic Valve ◽  
Aortic Stenosis ◽  
Clinical Outcomes ◽  
Aortic Regurgitation ◽  
Severe Aortic Stenosis ◽  
Vascular Complications ◽  
Transcatheter Aortic Valve

Abstract   Transcatheter Aortic valve Replacement (TAVR) has emerged as paradigm shift in the treatment of patients with severe symptomatic aortic stenosis. Clinical and performance data on long-term TAVR are still limited. The aim of this study was to determine the survival and the factors predicting mortality after TAVR with the first and second generation of prostheses. Methods From April 2008 to December 2019, the auto-expandible prostheses were implanted in 765 patients with symptomatic severe aortic stenosis with deemed high risk. The first generation prostheses included CoreValve and Accutrak System and the second prostheses included Evolut R and Pro. Results The mean age was 79.4±6.6 years. The logistic EuroSCORE and STS score were 17.1±11% and 5.7±3.9%, respectively. The implantation success rate was 98.87%. In-hospital mortality was 3.7%, and the combined endpoint of death, vascular complications, myocardial infarction or stroke had a rate of 15.1%. The clinical outcomes in according to prosthesis generation were, for pacemaker requirement (CoreValve vs. Accutrak system vs. Evolut R vs. and Evolut pro) 35.3% vs. 26.1% vs. 14.3% vs. 14%, p=0.001; and the paravalvular aortic regurgitation, were: none 28% vs. 44.8% vs. 43.3% vs. 58; mild 40% vs. 32.3% vs. 30.8% vs. 35.2%; moderate 32% vs. 20.3 vs. 23.9% vs. 5.7%; severe 0% vs. 2.6% vs. 0% vs. 1.1, p 0.001 The late mortality (beyond 30 days) was 35.9%. Survival at 1, 3, 5, 7 and 9 years were 88.9%, 76.1%, 61.1%, 44% and 32.6% respectively, after a mean follow-up of 42.3±27 months. The NYHA functional class improved from 3.1±0.6 to 1.77±0.7 in the follow-up. At 5 years, 5 patients had severe prosthetic valve dysfunction (severe stenosis and moderate transvalvular regurgitation The predictors of cumulative mortality were: Charlson index [HR 1.25 (95% CI 1.077–1.461), p=0.004], Readmision Heart Failure [HR 3.02 (95% CI 1.554–5.879), p=0.001], stroke post-TAVR [HR 3.472 (95% CI 1.115–10.53), p=0.032], residual aortic regurgitation [HR 1.45 (95% CI 1.093–1.934), p=0,010], and severe pulmonary hypertension [HR −0.983 (95% CI 0.645–0.423) p=0.032]. Conclusions TAVR is associated with significant survival benefit throughout 3.09 years of follow-up. Survival during follow-up depends particularly among patients with associated comorbidities and cardiac markers such as aortic regurgitation or pulmonary hypertension FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals Aortic stenosis of a bicuspid aortic valve in a patient with Klippel–Feil syndrome: a case report

2020 ◽  
Vol 4 (3) ◽  
pp. 1-4
Author(s):  
Rory F L Hammond ◽  
Sara Jasionowska ◽  
Wael I Awad
Keyword(s):  
Cervical Spine ◽  
Aortic Valve ◽  
Aortic Stenosis ◽  
Valve Replacement ◽  
Bicuspid Aortic Valve ◽  
Uneventful Recovery ◽  
Caucasian Woman ◽  
Surgical Aortic Valve Replacement ◽  
First Case

Abstract Background Klippel–Feil syndrome (KFS) is a rare congenital anomaly of the cervical spine, which is associated with a number of cardiovascular malformations, including coarctation of the aorta, bicuspid aortic valve (BAoV), and aortic aneurysm. Operative management of aortic stenosis of a BAoV in a patient with KFS has not been previously reported. Case summary A 54-year-old Caucasian woman with known KFS presented to her local hospital for elective cholecystectomy. An ejection systolic murmur was found incidentally on preoperative workup, which was confirmed to be due to a severely stenosed BAoV. The cholecystectomy was cancelled, and the patient was referred to our centre and accepted for surgical aortic valve replacement (AVR) based on symptomatic and prognostic grounds. Anaesthetic review of cervical spine imaging showed fusion of the C2–C6 vertebral bodies and a desiccated bulging disc at C4–C5 but no significant foraminal narrowing in the lower cervical spine. Valve replacement with a mechanical aortic prosthesis resulted in an uneventful recovery and the patient was discharged home to follow-up. Discussion We report the first case of severe aortic valve stenosis requiring AVR in a Klippel–Feil patient, in whom the aortic valve was confirmed to be bicuspid. This report provides further evidence of an association of KFS with BAoV and strengthens the case for screening and follow-up of KFS patients for BAoV and other cardiovascular pathologies, the consequences of which may be serious.

Abstract 5863: Rate and Correlates of Aortic Regurgitation Progression in a Cohort of 4128 Patients with Mild or Mild to Moderate Aortic Regurgitation

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Reena Patel ◽  
Ashvin Kamath ◽  
Padmini Varadarajan ◽  
Srikanth Krishnan ◽  
Ramdas G Pai
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Clinical Decision Making ◽  
Aortic Surgery ◽  
Clinical Decision ◽  
The Elderly ◽  
List Type ◽  
Average Increase

Background : Though the natural history of aortic stenosis is well studied, rate of progression of aortic regurgitation (AR) is not clear. This information is valuable for clinical decision making in patients with milder degrees of AR undergoing non aortic valve cardiac surgery. Methods : Our echocardiographic database between the years 1993 and 2007 was screened for patients with 1 or 2+ AR who had follow up echocardiograms at least 3 months later. AR severity was graded 1–4 based on standard criteria. A change in AR grade was annualized. Results : Of the 4128 patients, 3266 had 1+ and 862 had 2+ AR on the initial echocardiogram: age was 67±15 years, duration of follow up 4.2±2.7 years. Of those with 1+ AR on the initial echocardiogram, 95% had no change over a mean interval of 4.2 years and 2% increased the grade by ≥1+ per year. The average increase in AR grade was 0.04 per year. Of those with 2+ AR on initial echo, 90% had no change over this period and 2% increased the grade by ≥1+ per year. The average increase in grade was 0.07 per year. In the whole cohort, the AR progression correlated positively with age (p=0.03), ventricular septal thickness (p<0.0001), stroke distance (p=0.0003), increased transaortic velocity (p=0.01) and gradient (0.01) and initial AR severity (p<0.0001). Conclusions : The rate of AR progression is extremely slow and prophylactic aortic valve replacement during non aortic surgery may not be indicated in those with 1 or 2+ AR. AR progression seems to be higher in the elderly and those with aortic stenosis, higher cardiac output and greater AR severity. On the average, it would take 25 years to progress from grade 1+ to 2+ AR and 14 years to progress from grade 2+ to 3+ assuming linear progression.

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