Atorvastatin Effect on Aortic Dilatation and Valvular Calcification Progression in Bicuspid Aortic Valve (BICATOR): A Randomized Clinical Trial.

BACKGROUND: Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the benefit of statins in reducing these complications. This study aimed to determine whether atorvastatin treatment is effective in reducing the growth of aortic diameters in bicuspid aortic valve and if it slows the progression of valve calcification. METHODS: In a randomized clinical trial, 220 patients with bicuspid aortic valve (43 women; 46±13 years of age) were included and treated with either 20 mg of atorvastatin per day or placebo for 3 years. Inclusion criteria were ≥18 years of age, nonsevere valvular dysfunction, nonsevere valve calcification, and ascending aorta diameter ≤50 mm. Computed tomography and echocardiography studies were performed at baseline and after 3 years of treatment. RESULTS: During follow-up, 28 patients (12.7%) discontinued medical treatment (15 on atorvastatin and 13 taking placebo). Thus, 192 patients completed the 36 months of treatment. Low-density lipoprotein cholesterol levels decreased significantly in the atorvastatin group (median [interquartile range], -30 mg/dL [-51.65 to -1.75 mg/dL] versus 6 mg/dL [-4, 22.5 mg/dL]; P<0.001). The maximum ascending aorta diameter increased with no differences between groups: 0.65 mm (95% CI, 0.45-0.85) in the atorvastatin group and 0.74 mm (95% CI, 0.45-1.04) in the placebo group (P=0.613). Similarly, no significant differences were found for the progression of the aortic valve calcium score (P=0.167) or valvular dysfunction. CONCLUSIONS: Among patients with bicuspid aortic valve without severe valvular dysfunction, atorvastatin treatment was not effective in reducing the progression of ascending aorta dilation and aortic valve calcification during 3 years of treatment despite a significant reduction in low-density lipoprotein cholesterol levels. REGISTRATION: URL: https://www.clinicaltrialsregister.eu; Unique identifier: 2015-001808-57. URL: https://www.clinicaltrials.gov; Unique identifier: NCT02679261.

The genetic component of bicuspid aortic valve and aortic dilation. An exome-wide association study.

BACKGROUND: Bicuspid aortic valve is the most common cardiovascular congenital malformation affecting 2% of the general population. The incidence of life-threatening complications, the high heritability, and familial clustering rates support the interest in identifying risk or protective genetic factors. The main objective of the present study was to identify population-based genetic variation associated with bicuspid aortic valve and concomitant ascending aortic dilation. MATERIALS AND METHODS: A cross-sectional exome-wide association study was conducted in 565 Spanish cases and 484 controls. Single-marker and gene-based association analyses enriched for low frequency and rare genetic variants were performed on this discovery stage cohort and for the subsets of cases with and without ascending aortic dilation. Discovery-stage association signals and additional markers indirectly associated with bicuspid aortic valve, were genotyped in a replication cohort that comprised 895 Caucasian cases and 1483 controls. RESULTS: Although none of the association signals were consistent across series, the involvement of HMCN2 in calcium metabolism and valve degeneration caused by calcium deposit, and a nominal but not genome-wide significant association, supported it as an interesting gene for follow-up studies on the genetic susceptibility to bicuspid aortic valve. CONCLUSIONS: The absence of a genome-wide significant association signal shows this valvular malformation may be more genetically complex than previously believed. Exhaustive phenotypic characterization, even larger datasets, and collaborative efforts are needed to detect the combination of rare variants conferring risk which, along with specific environmental factors, could be causing the development of this disease.

Infective endocarditis in patients with bicuspid aortic valve: Clinical characteristics, complications, and prognosis. / Endocarditis infecciosa sobre válvula aórtica bicúspide: características clínicas, complicaciones y pronóstico.

INTRODUCTION: Bicuspid aortic valve (BAV) is the most frequent congenital cardiac disease. It is associated to a higher risk of cardiovascular complications, including infective endocarditis (IE). METHODS: Retrospective, observational and single centre study that included all patients with IE diagnosed between 1996 and 2014. An analysis was made of the epidemiological, clinical, microbiological and echocardiographic data, complications during hospital admission, need for surgery, in-hospital mortality, and 1-year follow-up. Cases with endocarditis on prosthetic valves or other locations were excluded, as well as those for which the aortic valve morphology had not been accurately defined. A comparative statistical analysis was performed between BAV and tricuspid (TAV). RESULTS: Of a total of 328 cases with IE, 118 (35.67%) were on aortic valve, with 18 (16.22%) of them being BAV. The BAV cases were younger than TAV (51±19.06 vs. 60.83±15.73 years, P=.021) and they had less comorbidity (Charlson 0.67±0.77 vs. 1.44±1.64, P=.03).). There was a higher tendency of Staphylococcal origin (38.9 vs. 21.5%, P=.137), and 55.6% showed peri-valvular complications (TAV 16.1%, P=.001), in particular, abscesses (38.9 vs.16.1%, P=.047). BAV was the only predictive factor of peri-valvular complications (OR 7.87, 95% CI; 2.38-26.64, P=.001). Patients with BAV had more surgery during their admission (83.3 vs. 44.1%, P=.004), had less in-hospital mortality, with no statistical significance (5.6 vs. 25.8%, P=.069), and 1-year survival was significantly superior (93.8 vs 69.3%, P=.048). CONCLUSIONS: Patients with IE on BAV are young, with low comorbidity. They frequently present with peri-valvular complications and they often require early surgery. Compared to TAV cases, in-hospital mortality is lower and 1-year survival is significantly higher.

Familial clustering of bicuspid aortic valve and its relationship with aortic dilation in first-degree relatives.

OBJECTIVE: Bicuspid aortic valve (BAV) is the most common congenital heart disease. This study aimed to determine the prevalence rate of BAV in first-degree relatives (FDR) and the inheritance pattern according to different morphotypes and aortic dilation. METHODS: BAV probands were consecutively studied at eight tertiary referral centres. After sequential screening, FDR were included in the study. The BAV morphotype, aortic dilation and aortic phenotype were assessed by transthoracic echocardiography. RESULTS: Seven hundred and twenty-four FDR of 256 BAV probands agreed to undergo family screening. The prevalence of BAV was 6.4% in FDR (9.2% in men, 3.5% in women, p=0.002). Aortic dilation was diagnosed in 9.6% of FRD with tricuspid aortic valves (TAV), with a root phenotype in 2.7% and tubular in 6.9% and more frequently in the presence of arterial hypertension (OR 4.48; CI 95% 2.51 to 7.99; p=0.0001) and valvular regurgitation (OR 5.87, CI 95% 1.37 to 25.16; p=0.025). The heritability (h2 ) of BAV was highly significant (0.47; p=0.002); however, no concordance was observed among valve morphotypes. Aortic dilation heritability was not significant. CONCLUSIONS: The BAV prevalence rate in FDR was low (6.4%) but aortic dilation was observed in 9.6% of FDR with TAV. The heritability of BAV was high without concordance in valve morphotypes, and aortic dilation heritability was not observed. Patients with BAV should be made aware of its familial pattern.

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

OBJECTIVE: Bicuspid 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. METHODS: Eight 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. RESULTS: Three 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. CONCLUSIONS: Normofunctional 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.

Increased blood levels of transforming growth factor ß in patients with aortic dilatation.

OBJECTIVES: Recent studies have shown that patients with syndromic thoracic aortic aneurysm, particularly patients with bicuspid aortic valve, have increased blood levels of transforming growth factor ß1 (TGF-ß1), indicating this molecule as a prognostic biomarker. However, it is not known whether TGF-ß1 is also elevated in the blood of patients with tricuspid aortic valve and aortic dilatation. METHODS: We analysed the plasma levels of TGF-ß1 in 52 patients with tricuspid or bicuspid aortic valve and with normal or dilated ascending aorta who underwent cardiac surgery in our hospital. RESULTS: TGF-ß1 blood level was significantly increased two-fold in patients with tricuspid aortic valve and dilated aorta compared to patients with tricuspid aortic valve and normal aorta. CONCLUSIONS: Our results suggest that TGF-ß1 blood levels may serve as a prognostic biomarker for patients with syndromic and non-syndromic thoracic aortic aneurysm. Further studies with larger cohorts of patients should be performed to confirm these results.

Fibrillin 2 is upregulated in the ascending aorta of patients with bicuspid aortic valve.

OBJECTIVES: Bicuspid aortic valve (BAV) is the most prevalent congenital cardiac malformation, frequently associated with aortic dilatation (AD). The molecular mechanisms involved in AD and its aetiological link with BAV formation are poorly understood. Altered fibrillin-1 (FBN1) and metalloprotease-2, -9 (MMP2,9) protein activities have been suggested to be involved in BAV aortopathy. In addition, FBN2 participates in embryonic valve formation, but its possible involvement in BAV-associated AD has never been explored. In this report, we evaluate the expression levels of MMP2,9 and FBN1,2 in the ascending aorta of patients with normal or dilated aortas and with tricuspid aortic valve (TAV) or BAV, using appropriate tissue-specific reference genes. METHODS: Gene expression was quantified by real-time quantitative polymerase chain reaction in 52 patients, using one or three reference genes previously validated in the same patient population. RESULTS: FBN2 expression was significantly increased in the aortas of patients with BAV compared with individuals with TAV (0.178 ± 0.042 vs 0.096 ± 0.021, P = 0.015), whereas differences in FBN1 did not reach statistical significance (1.946 ± 0.228 vs 1.430 ± 0.114, P = 0.090). When four groups of samples were considered, FBN2 expression was significantly higher in patients with BAV and AD compared with patients with TAV and AD (0.164 ± 0.035 vs 0.074 ± 0.027, P = 0.040). No significant differences were found when FBN1/FBN2 ratio, and MMP2 and MMP9 expression levels were analysed. No linear relationship between aortic diameter and gene expression levels were found. CONCLUSIONS: BAV patients have an increased FBN (especially FBN2) gene expression level in the ascending aorta, irrespective of dilatation, whereas MMP expression does not change significantly. These results add a new piece of information to the pathophysiology of BAV disease and point to FBN2 as a new molecular player.

Clinical and echocardiographic course in tako-tsubo cardiomyopathy: Long-term follow-up from a multicenter study.

OBJECTIVES: To jointly describe clinical characteristics, ECG and echocardiographic findings, and adverse cardiovascular events in patients with tako-tsubo cardiomyopathy (TC) in the long-term. METHODS: Longitudinal multicenter study including retrospective analysis of clinical and ECG data, and follow-up evaluation with clinical interview, electrocardiogram and echocardiogram. RESULTS: Data from 66 cases of TC were available for analysis of clinical and adverse cardiovascular events, and 56 of them completed the follow-up visit including electrocardiogram and echocardiogram. Most patients (97%) were asymptomatic or oligosymptomatic (NYHA I [58%] or II [39%], respectively) at follow-up (median time: 3.7 [1.8-6.6] years). The vast majority of individual QRS complex and repolarization abnormalities had disappeared (87% with no ECG abnormalities at follow-up). On echocardiography, left ventricular ejection fraction was ≥50% in all patients (mean: 63±6%). Wall motion abnormalities were observed in 4 patients (7%; 3 with apical wall motion abnormalities and 1 with mild global hypokinesia). Long-term outcomes were as follows: 4 deaths (6%), 2 cardiovascular and 2 non-cardiovascular; no atrial fibrillation development; no stroke events; 5 acute recurrence events of TC (8%). Globally, 57 patients (86%) had a clinical course free from adverse cardiovascular events. CONCLUSIONS: After a long period following the admission event, patients discharged from TC remain asymptomatic or minimally symptomatic, and feature a low prevalence of both ECG and left ventricular wall motion abnormalities; moreover, the latter lead to a very mild impairment of ejection fraction. Among cardiovascular adverse events, recurrence of the TC event appears to play the most significant role.

Selection of reference genes for quantitative real time PCR (qPCR) assays in tissue from human ascending aorta.

Dilatation of the ascending aorta (AAD) is a prevalent aortopathy that occurs frequently associated with bicuspid aortic valve (BAV), the most common human congenital cardiac malformation. The molecular mechanisms leading to AAD associated with BAV are still poorly understood. The search for differentially expressed genes in diseased tissue by quantitative real-time PCR (qPCR) is an invaluable tool to fill this gap. However, studies dedicated to identify reference genes necessary for normalization of mRNA expression in aortic tissue are scarce. In this report, we evaluate the qPCR expression of six candidate reference genes in tissue from the ascending aorta of 52 patients with a variety of clinical and demographic characteristics, normal and dilated aortas, and different morphologies of the aortic valve (normal aorta and normal valve n = 30; dilated aorta and normal valve n = 10; normal aorta and BAV n = 4; dilated aorta and BAV n = 8). The expression stability of the candidate reference genes was determined with three statistical algorithms, GeNorm, NormFinder and Bestkeeper. The expression analyses showed that the most stable genes for the three algorithms employed were CDKN1ß, POLR2A and CASC3, independently of the structure of the aorta and the valve morphology. In conclusion, we propose the use of these three genes as reference genes for mRNA expression analysis in human ascending aorta. However, we suggest searching for specific reference genes when conducting qPCR experiments with new cohort of samples.

Hereditary patterns of bicuspid aortic valve in a hundred families.

BACKGROUND: To study the following characteristics of bicuspid aortic valves (BAVs): 1) the recurrence rate in our population, 2) patterns of hereditary transmission in different BAV morphologies and 3) the aortic dimensions of BAVs in first-degree relatives (FDRs). METHODS: A cross-sectional, prospective study of 100 consecutive families of BAV patients attending a university hospital. The following aortic valve morphologies were analysed and categorised: fusion of the right and left coronary cusps (BAV type A), right and noncoronary cusps (type B) and of the left and noncoronary cusps (type C). RESULTS: There were 553 subjects studied, 100 cases with a BAV (46.8±15 years, 66% male, type 67% A, 32% B and 1% C; 42% with aortic dilatation), 348 FDRs (44.8% male), and 105 healthy control subjects (50% male). We detected 16 BAVs among 348 FDRs. The recurrence rates were 15% for families, 4.6% for FDRs, 7.05% in men and 2.60% in women. The morphologic concordance in family members was 68.8%. The aortic dimensions in 270 adult FDRs with a tricuspid aortic valve were significantly smaller compared with BAV patients (sinus index diameter 1.60±0.19 cm/m(2) vs. 1.82±0.29 cm/m(2), p<0.001; tubular index diameter 1.51±0.23 cm/m(2) vs. 2.00±0.45 cm/m(2), p<0.001) and similar to 103 control subjects(sinus index diameter 1.60±0.19 cm/m(2) vs. 1.59±0.17 cm/m(2), p=0.600 and tubular index diameter 1.51±0.23 cm/m(2) vs. 1.53±0.18 cm/m(2), p=0.519). CONCLUSIONS: In our population, the BAV recurrence rate in FDRs was low (4.6%). The hereditary transmission of morphologic BAV types seems by chance, and the aortic dimensions in tricuspid FDRs are normal.

Endocarditis infecciosa sobre válvula aórtica bicúspide: características clínicas, complicaciones y pronóstico / Infective endocarditis in patients with bicuspid aortic valve: Clinical characteristics, complications, and prognosis

Introducción: La válvula aórtica bicúspide (VAB) es la malformación cardíaca congénita más frecuente. Se asocia a un mayor riesgo de complicaciones cardiovasculares, entre las que se incluye la endocarditis infecciosa (EI). Métodos: Estudio observacional, unicéntrico de cohorte, que incluye de forma prospectiva a todos los pacientes ingresados por EI entre 1996 y 2014. Se analizan datos epidemiológicos, clínicos, microbiológicos, ecocardiográficos, complicaciones durante la hospitalización, necesidad quirúrgica, mortalidad intrahospitalaria y seguimiento a un año. Se excluyen los casos con endocarditis sobre válvulas protésicas o en otras localizaciones, y aquellos de cuya válvula aórtica no se tienen datos certeros acerca de su morfología. Se ha realizado un análisis estadístico comparativo entre VAB y tricúspide (VAT). Resultados: De un total de 328 casos con EI, 118 (35,67%) fueron sobre válvula aórtica. Tenían VAB 18 (16,22%). Los casos con VAB eran más jóvenes que los portadores de VAT (51±19,06 vs. 60,83±15,73 años, p=0,021) y tenían menos comorbilidad (índice de Charlson 0,67±0,77 vs. 1,44±1,64, p=0,03). En el grupo con VAB observamos tendencia a EI causada por Staphylococcus spp. (38,9 vs. 21,5%, p=0,137). Con diferencia estadística, hubo más complicaciones perivalvulares entre los casos con VAB (55,6% vs. 16,1%, p=0,001) predominando los abscesos (38,9 vs. 16,1%, p=0,047). Ser portador de VAB fue el único factor predictor de las mismas (OR 7,87, IC del 95%, 2,38-26,64, p=0,001). Los pacientes con VAB se operaron más (83,3 vs. 44,1%, p=0,004) y la mortalidad durante el ingreso hospitalario fue menor, aunque no alcanzó significación estadística (5,6 vs. 25,8%, p=0,069). La supervivencia a un año fue significativamente superior en el grupo de VAB (93,8 vs 69,3%, p=0,048). Conclusiones:Los pacientes con EI sobre VAB son jóvenes, con poca comorbilidad asociada. Tienen frecuentemente complicaciones perivalvulares por lo que requieren cirugía precoz. La mortalidad intrahospitalaria comparada con EI sobre VAT es menor y la supervivencia a un año es significativamente mayor (AU)

Introduction: Bicuspid aortic valve (BAV) is the most frequent congenital cardiac disease. It is associated to a higher risk of cardiovascular complications, including infective endocarditis (IE). Methods: Retrospective, observational and single centre study that included all patients with IE diagnosed between 1996 and 2014. An analysis was made of the epidemiological, clinical, microbiological and echocardiographic data, complications during hospital admission, need for surgery, in-hospital mortality, and 1-year follow-up. Cases with endocarditis on prosthetic valves or other locations were excluded, as well as those for which the aortic valve morphology had not been accurately defined. A comparative statistical analysis was performed between BAV and tricuspid (TAV). Results: Of a total of 328 cases with IE, 118 (35.67%) were on aortic valve, with 18 (16.22%) of them being BAV. The BAV cases were younger than TAV (51±19.06 vs. 60.83±15.73 years, P=.021) and they had less comorbidity (Charlson 0.67±0.77 vs. 1.44±1.64, P=.03).). There was a higher tendency of Staphylococcal origin (38.9 vs. 21.5%, P=.137), and 55.6% showed peri-valvular complications (TAV 16.1%, P=.001), in particular, abscesses (38.9 vs.16.1%, P=.047). BAV was the only predictive factor of peri-valvular complications (OR 7.87, 95% CI; 2.38-26.64, P=.001). Patients with BAV had more surgery during their admission (83.3 vs. 44.1%, P=.004), had less in-hospital mortality, with no statistical significance (5.6 vs. 25.8%, P=.069), and 1-year survival was significantly superior (93.8 vs 69.3%, P=.048). Conclusions: Patients with IE on BAV are young, with low comorbidity. They frequently present with peri-valvular complications and they often require early surgery. Compared to TAV cases, in-hospital mortality is lower and 1-year survival is significantly higher(AU)

Válvula aórtica bicúspide: resultados a largo plazo de la cirugía reparadora / Long-term Results with Reconstructiva Surgery of the Bicuspid Aortic Valve

Introducción: La válvula aórtica bicúspide es la anomalía congénita cardíaca más frecuente. En pacientes con requerimienro de cirugía, el tratamiento tradicional ha sido la sustitución valvular. La introducción del concepto de reparación de la válvula aórtica bicúspide ha llevado a nuestro grupo a reproducir las técnicas de reparación, las cuales se han estandarizado y aplicado de manera homogénea en nuestras instituciones. Objetivos: Revisar la experiencia conjunta de tres centros, con la presentación de los resultados a mediano y a largo plazos de la reparación valvular. Material y métodos: Entre octubre de 1995 y febrero de 2013 se intervinieron 666 pacientes con válvulas bicúspides e insuficiencia aórtica y/o aneurisma de la aorta. De ellos, 254 presentaban insuficiencia aórtica aislada y 412, aneurisma o disección. Se reconstruyó la válvula en todos los pacientes (en 254 como procedimiento aislado, en 281 "remodelación de la raíz", en 129 remodelación de la unión sinotubular y en 2 "reimplantación"). Resultados: La mortalidad fue de 3/666 (0,5%): 1/254 (0,4%) tras reparación valvular aislada y 2/410 (0,5%) tras reparación más reemplazo de la aorta. En pacientes con cirugía asociada (coronaria, reparación mitral/tricúspide) fue de 1/77 (1,3%). Durante el seguimiento murieron 12 pacientes (supervivencia a los 10 años: 95%). Las libertades de reoperación y de sustitución valvular a los 10 y 15 años fueron del 80% y 77% y del 86% y 83%, respectivamente. La libertad de reoperación a los 10 años fue superior en el reemplazo de la raíz (86%) o la aorta tubular (84%) en comparación con la reparación aislada (74%; p = 0,005). La libertad de cualquier complicación relacionada con la válvula fue del 80% y 77% a los 10 y 15 años, respectivamente, y fue mejor para reparación incluyendo "remodelación de la raíz" (87% y 82%) que para reparación aislada (77% y 77%; p = 0,04). Conclusiones: La reparación de la válvula aórtica bicúspide es un procedimiento seguro y duradero, con una incidencia baja de "complicaciones relacionadas con la válvula" a mediano y a largo plazos.

Introduction: Bicuspid aortic valve is the most common congenital heart disease. Traditionally, aortic valve replacement has been the approach for patients requiring surgery. After introduction of the bicuspid aortic valve repair concept, our group began reproducing these techniques, which have been standardized and homogeneously applied at our institutions. Objectives: The aim of this study was to review the conjoint experience of three centers and show the mid- and long-term results of bicuspid aortic valve repair. Methods: Between October 1995 and February 2013, 666 patients with bicuspid aortic valve underwent surgery for aortic regurgitation and/or aortic aneurysm. Isolated aortic regurgitation was present in 254 patients, and 412 had aortic aneurysm or dissection. The valve was reconstructed in all the patients (isolated valve repair in 254, "remodelling of the aortic root" in 281, remodelling of the sinotubular junction in 129 and "reimplantation" technique in 2). Results: Mortality was 3/666 (0.5%): 1/254 (0.4%) after isolated valve repair and 2/410 (0.5%) after valve repair plus aortic replacement. In patients with combined procedures (coronary revascularization or mitral/tricuspid valve repair), mortality was 1/77 (1.3%). During follow-up, 12 patients died (10-year survival: 95%). Freedom from reoperation and from aortic valve replacement at 10 and 15 years were 80% and 77%, and 86% and 83%, respectively Freedom from reoperation at 10 years was higher with aortic root (86%) or tubular aorta (84%) replacement, compared with isolated valve repair (74%; p = 0.005). Freedom from any valve-related complication was 80% and 77% at 10 and 15 years, respectively, and was better for valve repair including "remodelling of the aortic root" (87% and 82%) than for isolated repair (77% and 77%; p = 0.04). Conclusions: Bicuspid aortic valve repair is a safe, long-lasting procedure, with a low incidence of mid- and long-term "valve-related complications".

Seguimiento a largo plazo tras la ablación con radiofrecuencia de taquicardias ventriculares en pacientes portadores de un desfibrilador automático implantable / Radiofrequency catheter ablation of ventricular tachycardia in patients with an implantable defibrillator. Long-term follow-up

Introducción y objetivos. La aparición de episodios frecuentes de taquicardia ventricular es un problema para los pacientes portadores de un desfibrilador implantable y en ocasiones requiere la combinación de un tratamiento antiarrítmico o una ablación con radiofrecuencia. Hemos analizado los resultados de esta técnica en el grupo de pacientes portadores de desfibrilador por taquicardia ventricular sincopal previa que presentaron taquicardia ventricular frecuente o incesante. Pacientes y método. Se realizaron 18 procedimientos de ablación en 11 varones de 67,64 ± 5,87 años con estas características; 10 pacientes habían presentado un infarto de miocardio 15,50 ± 5,08 años antes y otro tenía una displasia arritmogénica del ventrículo derecho. Hasta la ablación se produjeron 591,67 ± 1.020,34 episodios de taquicardia ventricular (rango, 7-2.604).Resultados. El éxito electrofisiológico inicial fue del72,73% (n = 8). Tras un seguimiento de 39,10 ± 24,70meses, el número de descargas del desfibrilador disminuyó significativamente en todos los pacientes y pasó de52,82 ± 35,73 (rango, 7-130) a 0,64 ± 1,03 (rango, 0-3)(p = 0,001); 9 pacientes presentaron posteriormente alguna taquicardia ventricular; 6 enfermos necesitaron nuevos intentos de ablación (2 por fracaso inicial, 3 por recurrencia y 1 por una taquicardia diferente). Con independencia del resultado electrofisiológico, todos los pacientes presentaron buena evolución clínica a largo plazo. Conclusiones. La ablación con radiofrecuencia interrumpe de manera eficaz la situación de taquicardia ventricular incesante o muy recurrente y reduce significativamente las descargas del desfibrilador, incluso tras el fracaso electrofisiológico, y es especialmente útil en estas situaciones críticas, cuando otras terapias resultan ineficaces o insuficientes. Dado que nuestros pacientes son mayoritariamente isquémicos y muy proclives a presentar nuevos eventos arrítmicos durante su seguimiento, la ablación no se consideró sustitutiva, sino complementaria del desfibrilador implantable (AU)

Introduction and objectives. The frequent occurrence of ventricular tachycardia can be a serious problem for patients with an implantable defibrillator, and may necessitate adjuvant antiarrhythmic therapy or radiofrequency catheter ablation. We analyzed the long-term results obtained with this latter therapy in patients suffering from frequent or continuous ventricular tachycardia. Patients and method. Eighteen ablation procedures were performed in 11 patients who had a defibrillator implanted because of previous syncopal ventricular tachycardia. All were men, aged 67.64 (5.87) years; 10 patients had had a myocardial infarction 15.50 (5.08) years earlier, and one suffered from arrhythmogenic right ventricular dysplasia. Results. Electrophysiologically, treatment was initially successful in 8 patients (72.73%). After a follow-up period of 39.10 (24.70) months, the number of defibrillator discharges decreased significantly in all patients, from 52.82(35.73) to 0.64 (1.03) (P=.001). During follow-up, ventricular tachycardia occurred in nine patients. In five, it took the same form as the ablated ventricular tachycardia. Six patients needed additional ablation procedures: two because of initial failure, three because of recurrence, and one because a different ventricular tachycardia occurred. In addition to the good electrophysiological results obtained, long-term clinical evolution was favorable in all patients. Conclusions. Radiofrequency ablation successfully disrupts frequent or continuous ventricular tachycardias and significantly reduces the defibrillator discharge rate even when ablation has failed electrophysiologically. It is particularly useful in these latter critical situations, in which other therapies are not sufficiently effective. Because our patients mainly had ischemic heart disease and were highly susceptible to new arrhythmias during follow up, ablation complemented rather than replaced the implantable defibrillator (AU)