Outcomes of Isolated Tricuspid Valve Surgery.

BACKGROUND: Isolated tricuspid valve surgery is a rarely performed procedure and traditionally is associated with a bad prognosis, although its clinical outcomes still are little known. The aim of this study was to assess the short- and long-term clinical outcomes obtained at our center after isolated tricuspid valve surgery as treatment for severe tricuspid regurgitation. METHODS: This retrospective study included 71 consecutive patients with severe tricuspid regurgitation who underwent isolated tricuspid valve surgery between December 1996 and December 2017. Perioperative and long-term mortality, tricuspid valve reoperation, and functional class were analyzed after follow up. RESULTS: Regarding surgery, 7% of patients received a De Vega annuloplasty, 14.1% an annuloplasty ring, 11.3% a mechanical prosthesis, and 67.6% a biological prosthesis. Perioperative mortality was 12.7% and no variable was shown to be predictive of this event. After a median follow up of 45.5 months, long-term mortality was 36.6%, and the multivariate analysis identified atrial fibrillation as the only predictor (Hazard Ratio 3.014, 95% confidence interval 1.06-8.566; P = 0.038). At the end of follow up, 63.6% of survivors had functional class I. CONCLUSIONS: Isolated tricuspid valve surgery was infrequent in our center. Perioperative mortality was high, as was long-term mortality. However, a high percentage of survivors were barely symptomatic after follow up.

The Prognostic Value of Previous Coronary Stent Implantation in Patients Undergoing Myocardial Revascularization Surgery.

BACKGROUND: Currently, studies are underway to determine whether coronary stent implantation with percutaneous transluminal coronary angioplasty before a coronary artery bypass graft (CABG) influences the prognosis of surgery. This study aimed to assess the need for future revascularisation or all-cause mortality as a composite endpoint after CABG surgery among patients with previous stent implantation. METHODS: A retrospective, non-randomised study was performed on 721 patients who underwent CABGin our centre between 2012 and 2017. This single-centre study compared two groups: 1) the previous stent group, patients with previous stent implantation (n=144), and 2) the non-previous stent group, patients without previous stent implantation (n=577). RESULTS: After a median follow-up of 36 months, the previous stent group presented a decreased combined event-free survival at 1, 3 and 5 years compared with the non-previous stent group (67.4, 43.5 and 23.0% vs. 91.0, 80.3 and 63.0%, respectively; p<0.01). There was also higher mortality in the previous stent group than in the non-previous stent group (96.1, 90.5 and 79.4 vs. 91.9, 75.9 and 51.0, respectively; p=0.01). The multivariable analysis of demographics, baseline comorbidity and surgical data showed previous stent implantation as an independent predictor of the composite endpoint (Hazard Ratio=3.00 and 95% confident interval=2.09-4.32; p<0.01). CONCLUSION: Patients with percutaneous coronary intervention before CABG present higher comorbidities and clinical events during follow-up than those who do not undergo stenting.

Survival and predictive factors of mortality after 30 days in patients treated with percutaneous implantation of the CoreValve aortic prosthesis.

BACKGROUND: Few data exist on the clinical impact of transcatheter aortic valve implantation (TAVI) in patients with symptomatic aortic stenosis and a high surgical risk. The aim of this study was to determine the survival and the factors predicting mortality after 30 days post-TAVI with the CoreValve prosthesis (Medtronic, Minneapolis, MN). METHODS: From April 2008 to October 2010, the CoreValve prosthesis (Medtronic) was implanted in 133 consecutive high-risk surgical patients with symptomatic severe aortic stenosis. RESULTS: The mean age was 79.5 ± 6.7 years. The logistic European System for Cardiac Operative Risk Evaluation was 21.5% ± 14%. The implantation success rate was 97.7%. In-hospital mortality was 4.5%, and the combined end point of death, vascular complications, myocardial infarction, or stroke had a rate of 9%. Survival at 12 and 24 months was 84.5% and 79%, respectively, after a mean follow-up of 11.3 ± 8 months. The New York Heart Association functional class improved from 3.3 ± 0.5 to 1.18 ± 0.4 and remained stable at 1 year. A high Charlson index (hazard ratio [HR] 1.44, 95% CI 1.09-1.89, P < .01) and a worse Karnofsky score before the procedure (HR 0.95, 95% CI 0.92-0.99, P = .021) were predictors of mortality after 30 days. CONCLUSIONS: Transcatheter aortic valve implantation with the CoreValve prosthesis for patients with aortic stenosis and a high surgical risk is a safe, efficient option resulting in a medium-term clinical improvement. Survival during follow-up depends on the associated comorbidities. Early mortality beyond 30 days is predicted by preoperative comorbidity scores and the functional status of the patient.

The importance of genotype-phenotype correlation in the clinical management of Marfan syndrome.

BACKGROUND: Marfan syndrome (MFS) is a disorder of autosomal dominant inheritance, in which aortic root dilation is the main cause of morbidity and mortality. Fibrillin-1 (FBN-1) gene mutations are found in more than 90% of MFS cases. The aim of our study was to summarise variants in FBN-1 and establish the genotype-phenotype correlation, with particular interest in the onset of aortic events, in a broad population of patients with an initial clinical suspicion of MFS. MATERIAL AND METHODS: This single centre prospective cohort study included all patients presenting variants in the FBN-1 gene who visited a Hereditary Aortopathy clinic between September 2010 and October 2016. RESULTS: The study included 90 patients with FBN-1 variants corresponding to 58 non-interrelated families. Of the 57 FBN-1 variants found, 25 (43.9%) had previously been described, 23 of which had been identified as associated with MFS, while the the remainder are described for the first time. For 84 patients (93.3%), it was possible to give a definite diagnosis of Marfan syndrome in accordance with Ghent criteria. 44 of them had missense mutations, 6 of whom had suffered an aortic event (with either prophylactic surgery for aneurysm or dissection), whereas 20 of the 35 patients with truncating mutations had suffered an event (13.6% vs. 57.1%, p < 0.001). These events tended to occur at earlier ages in patients with truncating compared to those with missense mutations, although not significantly (41.33 ± 3.77 vs. 37.5 ± 9.62 years, p = 0.162). CONCLUSIONS: Patients with MFS and truncating variants in FBN-1 presented a higher proportion of aortic events, compared to a more benign course in patients with missense mutations. Genetic findings could, therefore, have importance not only in the diagnosis, but also in risk stratification and clinical management of patients with suspected MFS.

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.

[Transcatheter closure of aorto-left atrial fistula using an Amplatzer device]. / Cierre percutáneo de una fístula entre aorta y aurícula izquierda mediante dispositivo Amplatzer.

Aorto-left atrial fistulas are rare. We describe the case of a patient in whom transcatheter closure of an aorto-left atrial fistula was carried out using an Amplatzer septal occluder. The patient had previously undergone cardiac surgery twice because of a recurrent left atrial myxoma. Closure of this type of fistula using a transcatheter device should be considered when the location and size of the fistula are appropriate and there are no associated abnormalities. The risk of surgery must also be taken into account.

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.

Resultados del tratamiento quirúrgico de la insuficiencia tricuspídea grave en una serie contemporánea / Outcomes after surgical treatment of severe tricuspid regurgitation in a contemporary series

No disponible

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.

Arteria subclavia izquierda como abordaje para el implante de prótesis aórtica CoreValve / No disponible

No disponible

Cierre percutáneo de una fístula entre aorta y aurícula izquierda mediante dispositivo Amplatzer / Transcatheter closure of aorto-left atrial fistula using an amplatzer device

Las fístulas entre la aorta y la aurícula izquierda son excepcionales. Describimos un caso tras cirugía en dos ocasiones de un mixoma auricular izquierdo con posterior recidiva, que fue tratado mediante una prótesis Amplatzer para el cierre de la comunicación interventricular. El cierre percutáneo de estas fístulas debe valorarse en función del riesgo quirúrgico y cuando la localización y el tamaño sean adecuados, en ausencia de anomalías asociadas (AU)

Aorto-left atrial fistulas are rare. We describe the case of a patient in whom transcatheter closure of an aorto-left atrial fistula was carried out using an Amplatzer septal occluder. The patient had previously undergone cardiac surgery twice because of a recurrent left atrial myxoma. Closure of this type of fistula using a transcatheter device should be considered when the location and size of the fistula are appropriate and there are no associated abnormalities. The risk of surgery must also be taken into account (AU)

Regurgitación valvular causada por fármacos anorexígenos / Valvular regurgitation caused by anorectic agents

La enfermedad cardíaca valvular asociada al uso de fármacos anorexígenos es una entidad clínica recientemente descrita. Presentamos el caso de una paciente de 46 años con regurgitación severa mitral, aórtica y tricúspide que había estado tomando fenfluramina y dexfenfluramina durante 2 años. Requirió tratamiento quirúrgico con recambio de las tres válvulas por prótesis mecánicas. El antecedente de toma de fármacos anorexígenos, las características ecocardiográficas de las válvulas afectadas y los hallazgos macroscópicos e histopatológicos sugieren una fuerte asociación entre los anorexígenos y la enfermedad valvular de la paciente (AU)