Bicuspid aortic valve: different clinical profiles for subjects with versus without repaired aortic coarctation

ObjectivesA small proportion of patients undergoing bicuspid aortic valve (BAV) intervention have had prior repair of aortic coarctation (CoA). We aimed to describe phenotypic differences between BAV patients, comparing those with versus those without previous coarctation repair.Methods556 adults with BAV who had undergone aortic valve and/or ascending aortic surgery were identified, and relevant clinical and operative details were retrospectively analysed.ResultsOf the total cohort, 532 patients (95.7%) had isolated BAV (‘BAV-only’), and 24 (4.3%) had had a previous successful CoA repair (‘BAV-CoA’). The median age at surgery was significantly lower in BAV-CoA patients compared with BAV-only (median, IQR: 40 years, 26–57 vs 62 years, 51–69, p<0.001). Indications for surgery also differed, with BAV-CoA patients much more likely to undergo surgery for aortic regurgitation (BAV-CoA 38% vs BAV-only 13%, p<0.001); patients with isolated BAV were more likely to require surgery for aortic stenosis (BAV-only 75% vs BAV-CoA 50%, p<0.001). Two different BAV morphotypes were commoner in the BAV-CoA group; type 0 valves (24% vs 8%, p<0.05) and type 2 valves (12% vs 3%, p<0.05). The proportion of patients undergoing concomitant aortic surgery at the time of valve surgery were similar (BAV-only 38% vs BAV-CoA 42%, p=0.8).ConclusionIn adult patients undergoing aortic valve surgery for BAV disease, those with a prior history of repaired CoA underwent surgery at a very much younger age, and a higher proportion required intervention for aortic regurgitation.

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Aortic regurgitation

ESC CardioMed ◽

10.1093/med/9780198784906.003.0765 ◽

2018 ◽

pp. 1634-1641

Author(s):

Pilar Tornos Mas ◽

Emmanuel Lansac

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Aortic Surgery ◽

Valve Surgery ◽

Aortic Dilatation ◽

Concomitant Surgery ◽

Asymptomatic Patients ◽

Aortic Pathology ◽

Definition Of ◽

And Function

Evaluation of aortic regurgitation requires consideration of valve morphology, mechanism and severity of regurgitation and assessment of aortic dilatation. In asymptomatic patients with severe aortic regurgitation, follow-up of symptomatic status and LV size and function is mandatory. The strongest indication for valve surgery is the presence of symptoms and/or the documentation of LVEF <50% and/or end-systolic diameter =50 mm. In patients with dilated aorta, definition of aortic pathology and accurate measurements of aortic diameters are crucial. Surgery is recommended whenever aortic dilation is = 55 mm or = 50 mm in patients with bicuspid aortic valves and Marfan syndrome or =45 mm when additional risk factors are present. For patients who have an indication for valve surgery, an aortic diameter of ≥45 mm is considered to indicate concomitant surgery of the aorta. Aortic valve repair and valve-sparing aortic surgery instead of aortic valve replacement should be considered in selected cases in experienced centres.

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When should a rare inherited connective tissue disorder be suspected in bicuspid aortic valve by primary-care internists and cardiologists? Proposal of a score

Internal and Emergency Medicine ◽

10.1007/s11739-020-02458-1 ◽

2020 ◽

Author(s):

Guglielmina Pepe ◽

Betti Giusti ◽

Stefania Colonna ◽

Maria Pia Fugazzaro ◽

Elena Sticchi ◽

...

Keyword(s):

Primary Care ◽

Aortic Valve ◽

Connective Tissue ◽

Bicuspid Aortic Valve ◽

Aortic Surgery ◽

Connective Tissue Disorder ◽

Tertiary Center ◽

History Of ◽

Selected Traits ◽

Size Threshold

Abstract Size threshold for aortic surgery in bicuspid aortic valve (BAV) is debated. Connective tissue disorders (CTDs) are claimed as a clinical turning point, suggesting early surgery in BAV patients with CTD. Thus, we aimed at developing a score to detect high risk of carrying CTDs in consecutive BAVs from primary care. Ninety-eight BAVs without ectopia lentis or personal/family history of aortic dissection were studied at the Marfan syndrome Tuscany Referral Center. Findings were compared with those detected in 84 Marfan patients matched for sex and age. We selected traits with high statistical difference between MFS and BAV easily obtainable by cardiologists and primary-care internists: mitral valve prolapse, myopia ≥ 3DO, pectus carenatum, pes planus, wrist and thumb signs, and difference between aortic size at root and ascending aorta ≥ 4 mm. Clustering of ≥ 3 of these manifestations were more frequent in Marfan patients than in BAVs (71.4% vs 6.1%, p < 0.0001) resulting into an Odds Ratio to be affected by MFS of 38.3 (95% confidence intervals 14.8–99.3, p < 0.0001). We propose a score assembling simple clinical and echocardiographic variables resulting in an appropriate referral pattern of BAVs from a primary-care setting to a tertiary center to evaluate the presence of a potential, major CTD.

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Ascending aortic dissection in a pregnant patient with neonatally repaired coarctation of aorta and bicuspid aortic valve

BMJ Case Reports ◽

10.1136/bcr-2019-233367 ◽

2019 ◽

Vol 12 (12) ◽

pp. e233367

Author(s):

Mark Philip Cassar ◽

Asad Shabbir ◽

Elizabeth Orchard ◽

James Stirrup

Keyword(s):

Aortic Valve ◽

Aortic Dissection ◽

Bicuspid Aortic Valve ◽

Aortic Surgery ◽

Pregnant Patient ◽

Haemodynamic Instability ◽

Coarctation Of Aorta ◽

Blurred Vision ◽

Life Threatening ◽

History Of

Aortic dissection is characterised by a tear in the intimal and medial layers of the endovascular aortic wall which propagates distally. Here, we discuss the case of a 35-year-old woman who was 37 weeks pregnant and presented with dizziness and blurred vision. She had a history of a neonatal end-to-end repair of a coarctation of aorta, a known bicuspid aortic valve and a dilated ascending aorta under surveillance. A transthoracic echocardiogram revealed an ascending aortic dissection. An emergency CT aortogram was performed which confirmed the diagnosis. The patient underwent emergency caesarean section and aortic surgery, with a good outcome for mother and baby. The case highlights the atypical nature of presentation and the absence of haemodynamic instability. Atypical and unexplained symptoms on a background of congenital heart disease should trigger a referral to cardiology with thorough investigation, often with echocardiography, to exclude rare and life-threatening complications.

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Differences in Aortopathy in Patients with a Bicuspid Aortic Valve with or without Aortic Coarctation

Journal of Clinical Medicine ◽

10.3390/jcm9020290 ◽

2020 ◽

Vol 9 (2) ◽

pp. 290

Author(s):

Anthonie Duijnhouwer ◽

Allard van den Hoven ◽

Remy Merkx ◽

Michiel Schokking ◽

Roland van Kimmenade ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Dissection ◽

Bicuspid Aortic Valve ◽

Aortic Coarctation ◽

Care Center ◽

Aortic Surgery ◽

Tertiary Care ◽

Aortic Diameter ◽

Aortic Dilatation ◽

Significant Difference

Objective: The combination of aortic coarctation (CoA) and bicuspid aortic valve (BAV) is assumed to be associated with a higher risk of ascending aortic dilatation and type A dissection, and current European Society of Cardiology (ESC) guidelines advise therefore to operate at a lower threshold in the presence of CoA. The aim of our study is to evaluate whether the coexistence of CoA in BAV patients is indeed associated with a higher risk of ascending aortic events (AAE). Methods: In a retrospective study, all adult BAV patients visiting the outpatient clinic of our tertiary care center between February 2003 and February 2019 were included. The primary end point was an ascending aortic event (AAE) defined as ascending aortic dissection/rupture or preventive surgery. The secondary end points were aortic dilatation and aortic growth. Results: In total, 499 BAV patients (43.7% female, age 40.3 ± 15.7 years) were included, of which 121 (24%) had a history of CoA (cBAV). An aortic event occurred in 38 (7.6%) patients at a mean age of 49.0 ± 13.6 years. In the isolated BAV group (iBAV), significantly more AAE occurred, but this was mainly driven by aortic valve dysfunction as indication for aortic surgery. There was no significant difference in the occurrence of dissection or severely dilated ascending aorta (>50 mm) between the iBAV and cBAV patients (p = 0.56). The aortic diameter was significantly smaller in the cBAV group (30.3 ± 6.9 mm versus 35.7 ± 7.6 mm; p < 0.001). The median aortic diameter increase was 0.23 (interquartile range (IQR): 0.0–0.67) mm/year and was not significantly different between both groups (p = 0.74). Conclusion: Coexistence of CoA in BAV patients was not associated with a higher risk of aortic dissection, preventive aortic surgery, aortic dilatation, or more rapid aorta growth. This study suggests that CoA is not a risk factor in BAV patients, and the advice to operate at lower diameter should be reevaluated.

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Genetic diseases of the aorta: aortic diseases related to Marfan syndrome and other genetic abnormalities

ESC CardioMed ◽

10.1093/med/9780198784906.003.0623_update_001 ◽

2018 ◽

pp. 2611-2613

Author(s):

Bernard Iung

Keyword(s):

Aortic Valve ◽

Aortic Dissection ◽

Bicuspid Aortic Valve ◽

Aortic Surgery ◽

Genetic Diseases ◽

Rapid Progression ◽

Aortic Diseases ◽

Genetic Abnormalities ◽

History Of ◽

Associated Risk Factors

Bicuspid aortic valve affects 1–2% of babies at birth and is frequently associated with thoracic aortic aneurysm but the risk of aortic dissection is low. Indications for aortic surgery are based on studies on natural history. Aortic surgery is recommended when the maximum aortic diameter is greater than 55 mm in patients with a bicuspid aortic valve. Intervention is indicated at an earlier stage when there are associated risk factors, in particular familial history of aortic dissection or rapid progression, or if there is an indication for aortic valve replacement. The choice between partial or total replacement of the aorta should be based on anatomy, but also take into account the patient’s age and operative risk.

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Genetic diseases of the aorta: aortic diseases related to Marfan syndrome and other genetic abnormalities

ESC CardioMed ◽

10.1093/med/9780198784906.003.0623 ◽

2018 ◽

pp. 2611-2613

Author(s):

Bernard Iung

Keyword(s):

Aortic Valve ◽

Aortic Dissection ◽

Bicuspid Aortic Valve ◽

Aortic Surgery ◽

Genetic Diseases ◽

Rapid Progression ◽

Aortic Diseases ◽

Genetic Abnormalities ◽

History Of ◽

Associated Risk Factors

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Repair of Bicuspid Aortic Valve in the Presence of Endocarditis and Leaflet Perforation

Texas Heart Institute Journal ◽

10.14503/thij-12-3049 ◽

2014 ◽

Vol 41 (1) ◽

pp. 67-69

Author(s):

William D.T. Kent ◽

Hadi D. Toeg ◽

Jehangir J. Appoo

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Bicuspid Aortic Valve ◽

Recurrent Infection ◽

Aortic Valve Repair ◽

Valve Repair ◽

Simplified Approach ◽

Severe Aortic Regurgitation ◽

History Of ◽

Leaflet Coaptation

Aortic valve repair can be a good option in younger patients who have severe aortic regurgitation. A systematic, disease-directed approach can simplify repair. This case report describes how a simplified approach can be successfully applied to complex pathologic conditions of the aortic valve. A 49-year-old man with a bicuspid aortic valve and a history of endocarditis presented with severe aortic regurgitation and evidence of recurrent infection. Intraoperatively, we found congenital and degenerative aortic anatomy with endocarditis and perforation. We performed aortic valve repair to enable leaflet coaptation and to adjust the coaptation height. After 24 months, the patient remained well, with an intact repair and trivial aortic regurgitation. We describe our systematic repair approach and rationales for targeting repairs to identified lesions. To our knowledge, this is the first description of complex aortic valve repairs in a patient who had simultaneous congenital, degenerative, and infectious conditions.

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Prognostic implications of left atrial dilation in aortic regurgitation due to bicuspid aortic valve

Heart ◽

10.1136/heartjnl-2020-318907 ◽

2021 ◽

pp. heartjnl-2020-318907

Author(s):

Steele C Butcher ◽

Federico Fortuni ◽

William Kong ◽

E Mara Vollema ◽

Francesca Prevedello ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Bicuspid Aortic Valve ◽

Left Atrial ◽

Valve Surgery ◽

Volume Index ◽

Aortic Valve Surgery ◽

Event Free Survival ◽

Free Survival

ObjectiveTo investigate the prognostic value of left atrial volume index (LAVI) in patients with moderate to severe aortic regurgitation (AR) and bicuspid aortic valve (BAV).Methods554 individuals (45 (IQR 33–57) years, 80% male) with BAV and moderate or severe AR were selected from an international, multicentre registry. The association between LAVI and the combined endpoint of all-cause mortality or aortic valve surgery was investigated with Cox proportional hazard regression analyses.ResultsDilated LAVI was observed in 181 (32.7%) patients. The mean indexed aortic annulus, sinus of Valsalva, sinotubular junction and ascending aorta diameters were 13.0±2.0 mm/m2, 19.4±3.7 mm/m2, 16.5±3.8 mm/m2 and 20.4±4.5 mm/m2, respectively. After a median follow-up of 23 (4–82) months, 272 patients underwent aortic valve surgery (89%) or died (11%). When compared with patients with normal LAVI (<35 mL/m2), those with a dilated LAVI (≥35 mL/m2) had significantly higher rates of aortic valve surgery or mortality (43% and 60% vs 23% and 36%, at 1 and 5 years of follow-up, respectively, p<0.001). Dilated LAVI was independently associated with reduced event-free survival (HR=1.450, 95% CI 1.085 to 1.938, p=0.012) after adjustment for LV ejection fraction, aortic root diameter, LV end-diastolic diameter and LV end-systolic diameter.ConclusionsIn this large, multicentre registry of patients with BAV and moderate to severe AR, left atrial dilation was independently associated with reduced event-free survival. The role of this parameter for the risk stratification of individuals with significant AR merits further investigation.

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A heart in a heart: a case report of spontaneous flail of bicuspid aortic valve

Journal of Medical Case Reports ◽

10.1186/s13256-021-03211-8 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Khadije Mohammadi ◽

Mahsa Akrami ◽

Marzieh Mirtajaddini

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Bicuspid Aortic Valve ◽

Past Medical History ◽

Severe Aortic Regurgitation ◽

Case Summary ◽

History Of ◽

Old Persian ◽

Acquired Abnormalities ◽

Supporting Structures

Abstract Background Aortic regurgitation is attributed to congenital and acquired abnormalities of the aortic valve or aortic valve supporting structures. The most common cause of aortic regurgitation is atherosclerotic degeneration of the valve, especially in the presence of a bicuspid aortic valve. Case summary A 25-year-old Persian man with no past medical history, developed dyspnea since 1 week before his first visit to the physician. He was an active person up to this time, and had no history of trauma or chest pain. Transthoracic echocardiography showed severe aortic regurgitation in the context of flail bicuspid aortic valve, with no evidence of endocarditis. Laboratory tests including blood cultures were negative for infection. The patient underwent aortic valve replacement and the diagnosis was confirmed at time of surgery. Conclusion This case represents noninfective and nontraumatic spontaneous flail of bicuspid aortic valve.

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