An Unusual Cause of Aortic Regurgitation in a Patient With Bicuspid Aortic Valve.

Aortic fibrous strands are considered residual tissue from aortic valve development. Rupture of these strands is an important albeit uncommon cause of aortic regurgitation (AR). The authors describe a 67-year-old man who was admitted to the authors' hospital with sudden onset shortness of breath and diagnosed with severe AR. The patient was scheduled for Bentall surgery. The transesophageal echocardiogram (TEE) found multiple fibrous strands that were present in multiple locations of the aortic valve, some of which were ruptured. Ruptured fibrous strands are in the differential diagnosis in patients presenting with acute AR without a more conventional explanation, and TEE is instrumental in securing the diagnosis.

Aortic root replacement with bicuspid valve reimplantation: Are outcomes and valve durability comparable to those of tricuspid valve reimplantation?

OBJECTIVES: To assess intermediate-term outcomes of aortic root replacement with valve-sparing reimplantation of bicuspid aortic valves (BAV), compared with tricuspid aortic valves (TAV). METHODS: From January 2002 to July 2017, 92 adults underwent aortic root replacement with BAV reimplantation and 515 with TAV reimplantation at the Cleveland Clinic. Balancing-score matching based on 28 preoperative variables yielded 71 well-matched BAV and TAV pairs (77% of possible pairs) for comparison of postoperative mortality and morbidity, longitudinal echocardiogram data, aortic valve reoperation, and survival. RESULTS: In the BAV group, 1 hospital death occurred (1.1%); mortality among all reimplantations was 0.2%. Among matched patients, procedural morbidity was low and similar between BAV and TAV groups (1 stroke in TAV group; renal failure requiring dialysis, 1 patient each; red cell transfusion, 25% each). Five-year results: Severe aortic regurgitation was present in 7.4% of the BAV group and 2.9% of the TAV group (P = .7); 39% of BAV and 65% of TAV patients had none. Higher mean gradients (10 vs 7.4 mm Hg; P = .001) and left ventricular mass index (111 vs 101 g/m2; P = .5) were present in BAV patients. Freedom from aortic valve reoperation was 94% in the BAV group and 98% in the TAV group (P = .10), and survival was 100% and 95%, respectively (P = .07). CONCLUSIONS: Both BAV and TAV reimplantations can be performed with equal safety and good midterm outcomes; however, the constellation of higher gradients, less ventricular reverse remodeling, and more aortic valve reoperations with BAV reimplantations raises concerns requiring continued long-term surveillance.

Natural History and Outcomes of Nonreplaced Aortic Sinuses in Patients With Bicuspid Aortic Valves.

BACKGROUND: Enlargement of the sinus of Valsalva (SOV) is common in patients with bicuspid aortic valves (BAVs), and management at the time of aortic valve replacement (AVR) and concomitant ascending aorta replacement/repair is controversial. METHODS: Between January 2000 and July 2017, 400 patients with BAVs underwent AVR and concomitant ascending aorta repair (graft replacement, 79%; aortoplasty, 21%). To assess the impact of the initial SOV dimension on future dilatation and outcomes, patients were stratified into 2 groups: SOV of less than 40 mm (SOV<40 mm) (n = 209) and SOV of 40 mm or larger (SOV≥40 mm) (n = 191). RESULTS: Patients with SOV≥40 mm were older and more often male. At a median follow-up of 8.1 years (interquartile range, 7.4-9.1 years), 6 patients underwent reoperations on the ascending or sinus portion of the aorta due to aneurysmal dilatation, and enlargement of the sinus was the primary indication for operation in 1 patient. Adjusted analysis showed that baseline SOV and SOV dimension over time were not associated with late outcomes. A gradual increase in SOV diameter over time was identified (P = .004). Patients with smaller baseline SOV diameters showed an initial early decrease in diameter, followed by gradual increase, whereas those with larger baseline diameters had a stable early phase, followed by gradual dilatation. CONCLUSIONS: Ascending aorta replacement may lead to an initial remodeling/stabilizing effect on the spared bicuspid aortic root, which is more pronounced in patients with lower SOV diameters. In addition, our data demonstrate that the retained aortic sinuses enlarge slowly, and within the limited follow-up of our study, SOV diameter was not a risk factor for survival or reoperation.

Prognostic Implications of Bicuspid and Tricuspid Aortic Valve Phenotype on Progression of Moderate Aortic Stenosis and Ascending Aorta Dilatation.

Studies on the impact of aortic valve anatomy (bicuspid aortic valve [BAV] or tricuspid aortic valve [TAV]) on the progression of moderate aortic stenosis (AS) and ascending aorta (AA) dilatation and its prognostic implications are limited. From 1991 to 2016, 288 asymptomatic patients with moderate AS detected during index echocardiography with at least 1 year of echocardiographic follow-up were retrospectively studied. Baseline clinical and echocardiographic characteristics were compared between patients with BAV (n = 80) and patients with TAV (n = 208). Co-primary outcomes were 1-year hemodynamic and anatomic progression of AS and AA dilatation. Secondary end points were the incidence of AA rapid progressors, all-cause mortality, aortic valve replacement, and congestive heart failure. Determinants of AS progression, AA diameters, AA dilatation, and prognostic outcomes were evaluated. Similar 1-year progression of the aortic valve peak velocity, Vmax (9 ± 18 vs 9 ± 23 cm/s), mean gradient (1.5 ± 2.3 vs 1.3 ± 3.2 mm Hg), and aortic valve area (AVA) (-0.04 ± 0.09 vs -0.05 ± 0.10 cm2) were noted for BAV and TAV groups, respectively. One-year progressions of AA were similar at Valsalva (0.11 ± 0.88 vs 0.14 ± 1.10 mm) and tubular levels (0.12 ± 0.68 vs 0.30 ± 1.51 mm) in BAV and TAV groups, respectively. A trend toward increased rapid AA progression in patients with BAV (31.3%) was observed compared with patients with TAV (14.8%, p = 0.099). BAV was associated with progression of Vmax (ß = 0.17, p = 0.036), the dimensionless index (ß = -0.17, p = 0.008), and AVA (ß = -0.14, p = 0.048), but not mean gradient after adjusting for age, baseline severity indexes, gender, hypertension, diabetes, and body surface area. Although BAV was a determinant of larger baseline AA diameter, there was no significant association between BAV and AA rapid progressors. Adjusted Kaplan-Meier curves demonstrated no differences in congestive heart failure, aortic valve replacement, or mortality between valve morphology. In conclusion, there was a similar 1-year disease progression in terms of AVA, Vmax, mean gradient, and AA diameters between patients with BAV and patients with TAV. BAV was associated with a significant increase in Vmax, dimensionless index, and AVA after adjusting for important confounders. Close and prolonged follow-up is warranted in both groups of patients.

Aortic annulus sizing in bicuspid and tricuspid aortic valves using CT in patients with surgical aortic valve replacement.

The purpose of this study was to evaluate whether bicuspid anatomy affects the discrepancy between CT-derived annular size and intraoperative size. We retrospectively analyzed annular measurements in 667 patients who underwent surgical aortic valve replacement (AVR). Preoperative CT measurements of the aortic annulus were compared to surgically implanted valve sizes. To evaluate whether the bicuspid valve affects the differences between CT annulus diameter and surgical AVR size, patients with diameter larger by > 10% (CT-Lg group) on CT, compared to surgical AVR size, were compared with those having size difference < 10% (CT-Sim group). Propensity score matching yielded 183 matched patients from each group. Bicuspid aortic valve annulus parameters significantly correlated with surgical aortic valve size (r = 0.52-0.71; for all, p < 0.01). The most representative measurements corresponded to surgical aortic valve size were area-derived diameters in tricuspid aortic valve (r = 0.69, p < 0.001) and bicuspid without raphe (r = 0.71, p < 0.001), and perimeter-derived diameter in bicuspid with raphe (r = 0.63, p < 0.001). After propensity score matching, native valve type was not different between CT-Sim and CT-Lg groups. In multivariable analysis, the difference between CT-derived diameter and surgical AVR size was affected by the operator factor and types of prosthesis. Bicuspid aortic annulus diameters measured on CT showed a significant correlation with surgical aortic valve size. The difference between CT-derived diameter and surgical AVR size is affected by operator factor and the types of prosthesis but not affected by the bicuspid valve.

Sex Differences in Children and Young Adults With Bicuspid Aortic Valve Disease in First Two Decades of Life.

OBJECTIVE: To elucidate sex differences in valve morphology, disease phenotype, progression, and outcomes among children and young adults with bicuspid aortic valve (BAV). PATIENTS AND METHODS: This is a retrospective cohort study examining all children and young adults (aged ≤22 years) with isolated BAV diagnosed, by excluding patients with concomitant congenital heart defects or genetic syndromes, from January 1, 1990, through December 1, 2016, at Mayo Clinic in Rochester, Minnesota. RESULTS: Of 1010 patients with BAV, 558 had isolated BAV. Distributions of morphology were right-left in 65.8% (n=367), right-noncoronary in 34% (n=190), and left-noncoronary cusp fusion in 0.2% (n=1) of patients; with no sex differences. Male to female ratio was 3:1. At the first echocardiographic evaluation in the study, there were no sex differences in terms of frequency of aortic valve stenosis or regurgitation. However, males had significantly higher grades of aortic valve regurgitation at 17 years of age onward (P<.0001). Males had significantly larger mid-ascending aorta (P=.01) and sinus of Valsalva dimensions (z score; P=.0001) as compared with females, with a novel finding of peak aortic dimensions around 8 years of age. Males also had more than 2-fold higher risk for sinus of Valsalva dilation (z score >2) as compared with females (odds ratio, 2.3; 95% CI, 1.2 to 4.2; P=.01). There were no significant sex differences in the primary cardiac outcomes of interventions on aortic valve and/or aorta, aortic dissection, or death. CONCLUSION: In children and young adults with BAV, males have a higher grade of aortic regurgitation in late adolescence, significantly larger aortic dimensions, different patterns of aortic growth, and more frequent sinus of Valsalva dilation as compared with females. Overall, the rate of primary cardiac events is lower in young patients, with no significant sex differences.

Bicuspid aortic valve disease is associated with abnormal wall shear stress, viscous energy loss, and pressure drop within the ascending thoracic aorta: A cross-sectional study.

ABSTRACT: Bicuspid aortic valve (BAV) disease has significant gaps in its clinical management practices. To highlight the potential utility of advanced hemodynamic biomarkers in strengthening BAV assessment, we used 4-dimentional flow magnetic resonance imaging to investigate altered hemodynamics in the ascending aorta (AAo).A total of 32 healthy controls and 53 age-matched BAV patients underwent cardiac magnetic resonance imaging at 3T, with cine imaging and 4D-flow. Analysis planes were placed along 3D-segmented aortas at the left ventricular outflow tract (LVOT), sinuses of Valsalva, mid-ascending aorta (MAA), and proximal to the first aortic branch. Locations were analyzed for aortic diameter (normalized to body surface area), pressure drop (PD), viscous energy loss (EL), and wall shear stress (WSS) sub-vectors (axial wall shear stress, circumferential wall shear stress [WSSC], magnitude wall shear stress). Student's t tests, or non-parametric equivalents, compared parameters between cohorts. Univariable and multivariable analyses explored the associations of AAo diameter with hemodynamics within the BAV cohort.Compared to control cohort, BAV patients showed significantly greater PD (MAA: 9.5 ±â€Š8.0 vs 2.8 ±â€Š2.4 mm Hg; P < .01), EL (from LVOT-AA1: 7.39 ±â€Š4.57 mW vs 2.90 ±â€Š1.07 mW; P < .01), and WSSC (MAA: 0.3 ±â€Š0.1 vs 0.2 ±â€Š0.06 Pa; P ≤ .01) throughout the AAo. Correlational analyses revealed an inverse association between AAo diameter and both magnitude wall shear stress and axial wall shear stress.BAV patients exhibited increased PD, EL, and WSSC in the AAo, and an inverse association between AAo diameter and WSS sub-vectors. This demonstrated the impact of PD, EL, and WSS in BAV disease and the importance of altered hemodynamics in aortic remodelling.

Update in Biomolecular and Genetic Bases of Bicuspid Aortopathy.

Bicuspid aortic valve (BAV) associated with aortopathy is the most common congenital heart disease in the general population. Far from being a simple harmless valve malformation, it can be a complex and heterogeneous disease and a source of chronic and acute pathology (early valvular disease, aneurysm, dissection). In the previous years, intense research has been carried out to find out and understand its mechanisms, but the pathophysiology of the disease is still not fully understood and many questions remain open. Recent studies have discovered several genetic mutations involved in the development of valvular and aortic malformations, but still cannot explain more than 5-10% of cases. Other studies have also focused on molecular alterations and cellular processes (TGF-ß pathway, microRNAs, degradation of the extracellular matrix, metalloproteinases, etc.), being a field in constant search and development, looking for a therapeutic target to prevent the development of the disease. Increased knowledge about this multifaceted disorder, derived from both basic and clinical research, may influence the diagnosis, follow-up, prognosis, and therapies of affected patients in the near future. This review focuses on the latest and outstanding developments on the molecular and genetic investigations of the bicuspid aortopathy.

Prenatal cardiac biometry and flow assessment in fetuses with bicuspid aortic valve at 20 weeks' gestation: multicenter cohort study.

OBJECTIVE: To investigate prenatal changes in cardiac biometric and flow parameters in fetuses with bicuspid aortic valve (BAV) diagnosed neonatally compared with controls with normal cardiac anatomy. METHODS: This analysis was conducted as part of the Copenhagen Baby Heart Study, a multicenter cohort study of 25 556 neonates that underwent second-trimester anomaly scan at 18 + 0 to 22 + 6 weeks' gestation and neonatal echocardiography within 4 weeks after birth, in Copenhagen University Hospital Herlev, Hvidovre Hospital and Rigshospitalet in greater Copenhagen, between April 2016 and October 2018. From February 2017 (Rigshospitalet) and September 2017 (Herlev and Hvidovre hospitals), the protocol for second-trimester screening of the heart was extended to include evaluation of the four-chamber view, with assessment of flow across the atrioventricular valves, sagittal view of the aortic arch and midumbilical artery and ductus venosus pulsatility indices. All images were evaluated by two investigators, and cardiac biometric and flow parameters were measured and compared between cases with BAV and controls. All cases with neonatal BAV were assessed by a specialist. Maternal characteristics and first- and second-trimester biomarkers were also compared between the two groups. RESULTS: Fifty-five infants with BAV and 8316 controls with normal cardiac anatomy were identified during the study period and assessed using the extended prenatal cardiac imaging protocol. There were three times as many mothers who smoked before pregnancy in the group with BAV as in the control group (9.1% vs 2.7%; P = 0.003). All other baseline characteristics were similar between the two groups. Fetuses with BAV, compared with controls, had a significantly larger diameter of the aorta at the level of the aortic valve (3.1 mm vs 3.0 mm (mean difference, 0.12 mm (95% CI, 0.03-0.21 mm))) and the pulmonary artery at the level of the pulmonary valve (4.1 mm vs 3.9 mm (mean difference, 0.15 mm (95% CI, 0.03-0.28 mm))). Following conversion of the diameter measurements of the aorta and pulmonary artery to Z-scores and Bonferroni correction, the differences between the two groups were no longer statistically significant. Pregnancy-associated plasma protein-A (PAPP-A) multiples of the median (MoM) was significantly lower in the BAV group than in the control group (0.85 vs 1.03; P = 0.04). CONCLUSIONS: Our findings suggest that fetuses with BAV may have a larger aortic diameter at the level of the aortic valve, measured in the left-ventricular-outflow-tract view, and a larger pulmonary artery diameter at the level of the pulmonary valve, measured in the three-vessel view, at 20 weeks' gestation. Moreover, we found an association of maternal smoking and low PAPP-A MoM with BAV. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Relative survival after aortic valve surgery in patients with bicuspid aortic valves.

OBJECTIVES: The objective of this cohort study was to analyse long-term relative survival in patients with bicuspid aortic valve (BAV) who underwent aortic valve surgery. METHODS: We studied 865 patients with BAVs who participated in three prospective cohort studies of elective, open-heart, aortic valve surgery at the Karolinska University Hospital, Stockholm, Sweden, between 2007 and 2020. The expected survival for the age, sex and calendar year-matched general Swedish population was obtained from the Human Mortality Database. The Ederer II method was used to calculate relative survival, which was used as an estimate of cause-specific survival. RESULTS: No differences were found in the observed versus expected survival at 1, 5, 10 or 12 years: 99%, 94%, 83% and 76% vs 99%, 93%, 84% and 80%, respectively. The relative survival at 1, 5, 10 and 12 years was 100% (95% CI 99% to 100%), 101% (95% CI 99% to 103%), 99% (95% CI 95% to 103%) and 95% (95% CI 87% to 102%), respectively. The relative survival at the end of follow-up tended to be lower for women than men (86% vs 95%). The mean follow-up was 6.3 years (maximum 13.3 years). CONCLUSIONS: The survival of patients with BAV following aortic valve surgery was excellent and similar to that of the general population. Our results suggest that the timing of surgery according to current guidelines is correct and provide robust long-term survival rates, as well as important information about the natural history of BAV in patients following aortic valve surgery.

Ex Vivo Analysis of a Porcine Bicuspid Aortic Valve and Aneurysm Disease Model.

We identified an extremely rare congenital porcine type 0 lateral bicuspid aortic valve from a fresh porcine heart. Using a 3-dimensionally printed ex vivo left heart simulator, we analyzed valvular hemodynamics at baseline, in an aortic aneurysm disease model, and after valve-sparing root replacement. We showed that bicuspid aortic valve regurgitation due to aortic aneurysm can be successfully repaired without significant hemodynamic impairment using the valve-sparing root replacement technique in an individualized approach. Our results provide direct hemodynamic evidence supporting the use of valve-sparing root replacement for patients with bicuspid aortic valve regurgitation.

Transcatheter and surgical aortic valve replacement in patients with bicuspid aortic valve.

OBJECTIVES: To compare the outcomes after surgical (SAVR) and transcatheter aortic valve replacement (TAVR) for severe stenosis of bicuspid aortic valve (BAV). METHODS: We evaluated the early and mid-term outcome of patients with stenotic BAV who underwent SAVR or TAVR for aortic stenosis from the nationwide FinnValve registry. RESULTS: The FinnValve registry included 6463 AS patients and 1023 (15.8%) of them had BAV. SAVR was performed in 920 patients and TAVR in 103 patients with BAV. In the overall series, device success after TAVR was comparable to SAVR (94.2% vs. 97.1%, p = 0.115). TAVR was associated with increased rate of mild-to-severe paravalvular regurgitation (PVR) (19.4% vs. 7.9%, p < 0.0001) and of moderate-to-severe PVR (2.9% vs. 0.7%, p = 0.053). When newer-generation TAVR devices were evaluated, mild-to-severe PVR (11.9% vs. 7.9%, p = 0.223) and moderate-to-severe PVR (0% vs. 0.7%, p = 1.000) were comparable to SAVR. Type 1 N-L and type 2 L-R/R-N were the BAV morphologies with higher incidence of mild-to-severe PVR (37.5% and 100%, adjusted for new-generation prostheses p = 0.025) compared to other types of BAVs. Among 75 propensity score-matched cohorts, 30-day mortality was 1.3% after TAVR and 5.3% after SAVR (p = 0.375), and 2-year mortality was 9.7% after TAVR and 18.7% after SAVR (p = 0.268) CONCLUSIONS: In patients with stenotic BAV, TAVR seems to achieve early and mid-term results comparable to SAVR. Type 1 N-L and type 2 L-R/R-N BAV morphologies had higher incidence of PVR. Larger studies evaluating different phenotypes of BAV are needed to confirm these findings. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT03385915.

A de novo pathogenic BMP2 variant-related phenotype with the novel finding of bicuspid aortic valve.

A rare autosomal dominant syndrome with craniofacial dysmorphisms, skeletal abnormalities, short stature, and congenital heart defects has recently been described, associated with monoallelic truncating and frameshift bone morphogenetic protein 2 (BMP2) variants and deletions. We describe a patient harboring a novel de novo BMP2 nonsense variant, who exhibited craniofacial and skeletal features previously described for this trait and the novel findings of bicuspid aortic valve (BAV) and aortic root and ascending aortic aneurysm. This first instance of aortic valve involvement provides another potential cause of BAV and confirms the role of BMP2 in left ventricular outflow development.

Dilatation of the Ascending Aorta in Turner Syndrome: Influence of Bicuspid Aortic Valve Morphology and Body Composition.

BACKGROUND: Aortic dilatation and bicuspid aortic valve (BAV) are frequent in Turner syndrome (TS). Due to short stature, aortic size index (ASI)-ascending aortic diameter (AD)/body surface area (BSA)-is used to identify aortic dilatation in TS patients. We sought to: 1) describe echocardiographic findings in the largest cohort of Australian women with TS; 2) assess if ASI progresses differently with age in TS BAV compared to non-syndromic BAV; and 3) determine whether adjustment of AD for body composition may be superior to BSA indexation. METHODS: Transthoracic echocardiography (TTE) data were retrospectively collected on 125 women with TS. Body composition was quantified by dual energy X-ray absorptiometry (DXA) in 60 women within 6 months of baseline TTE. Age-matched females with non-syndromic BAV (n=170) were used as controls for TS patients with BAV. RESULTS: Mean age of TS women was 28±16 years, and mean height and BSA were 141.6±21.7 cm and 1.4±0.4 m2, respectively. Mean AD was 2.5±0.8 cm, and ASI 2.0±0.6 cm/m2. Aortic dilatation (ASI >2.0 cm/m2) was present in 42 (34%) patients. Turner syndrome women with BAV (n=34; 27%) had a larger ASI than those with tri-leaflet AV (2.2±0.4 cm/m2 vs. 1.7±0.3 cm/m2, p<0.001). In the pooled BAV cohort, TS patients had a higher baseline ASI (2.2±0.4 cm/m2 vs. 2.1±0.3 cm/m2, p=0.02) and greater increase in ASI with age (0.21 mm/m2/year vs. 0.10 mm/m2/year, p=0.01) compared to non-syndromic BAV patients. DXA fat-free mass (r=0.33, p=0.01) and lean mass (r=0.32, p=0.02) correlated with AD, as did BSA (r=0.62, p<0.001). CONCLUSION: Turner syndrome women with BAV have a greater degree of baseline aortic dilatation and a twofold faster increase in aortic dimension with age when compared to matched women with non-syndromic BAV. Several DXA-derived body composition parameters correlate with aortic size in TS, however BSA appears to be the most robust method of indexation.

Contemporary management of severe symptomatic bicuspid aortic valve stenosis: the BiTri Registry.

AIMS: A greater number of patients with bicuspid aortic valves (BAV) may be identified and treated as indications for transcatheter aortic valve implantation (TAVI) are expected to expand to younger patients. We evaluated the contemporary frequency and management of symptomatic patients with stenotic BAV in a multicenter European registry. METHODS: Between November 2017 and February 2018, all consecutive patients admitted for symptomatic aortic stenosis across six high-volume European hospitals were prospectively enrolled in the BiTri registry. RESULTS: Of the 832 patients, 17% (n = 138) had a BAV. The most frequent BAV phenotypes were type 1 (left--right coronary cusps fusion 64%) and type 1 (right-noncoronary cusps fusion 17%). Type 0 and type 2 accounted for 12 and 2%, respectively. When compared with tricuspid patients (n = 694), BAV patients were younger, with lower surgical risk. The transthoracic echocardiography (TTE) identified BAV in 64% of patients. Multisliced computed tomography (MSCT) additionally completed the diagnosis in 20% of patients. Surgical inspection finally identified the remaining undiagnosed 16% of BAV. A combination of TTE and MSCT was the most common diagnosis method for BAV. Surgical aortic valve replacement (SAVR) was the predominant therapeutic option for BAV (70%) whilst TAVI was performed in 26%. CONCLUSION: BAV is frequently observed in symptomatic patients with aortic stenosis. These patients are younger, have a lower risk profile and are predominantly treated with SAVR as compared with tricuspid patients. However, TAVI is performed in almost one-third of BAV patients in contemporary European practice. TTE combined with MSCT identified 84% of BAV.

Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects. / Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos.

La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.

Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos / Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects

Resumen La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.

Abstract The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.