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Rationale: The pathogenesis of bicuspid aortic valve (BAV)–associated aortopathy is poorly understood, and no prognostic biomarker is currently available. Objective: We aimed to identify putative circulating biomarkers pathogenetically and prognostically linked to bicuspid aortopathy. Methods and Results: By reverse transcription polymerase chain reaction, we evaluated gene expression variations (versus normal aorta) of transforming growth factor-β1 (TGF-β1), connective tissue growth factor, matrix metalloproteinase-2 (MMP-2), MMP-14, endoglin (ENG), and superoxide dismutase 3 in ascending aorta samples from 50 tricuspid and 70 patients with BAV undergoing surgery for aortic stenosis (aorta diameter ≤45 mm: BAV non-dil or >45 mm: BAV dil ). Expression changes of the TGF-β1 active dimer and ENG were analyzed also by Western blot in ascending aorta samples from other 10 tricuspid aortic valve, 10 BAV non-dil , and 10 BAV dil patients. The serum concentration of study targets was assessed through ELISA and the ratio of serum TGF-β1/ENG (T/E) was evaluated. All BAV non-dil patients underwent follow-up echocardiography to assess aortic growth rate. In BAV non-dil patients, TGF-β1 and MMP-2 gene expression increased significantly, whereas MMP-14 and ENG expression decreased versus controls. Expression changes were confirmed at protein level for TGF-β1 and ENG. TGF-β1 serum concentration significantly decreased in tricuspid aortic valve and BAV non-dil patients versus healthy subjects. ENG serum concentration decreased in all patients, more markedly in BAV dil . A significant increase of the T/E ratio versus healthy subjects was unique of patients with BAV. In BAV non-dil patients, a T/E ≥9 was independently associated in multivariable analysis with higher MMP-2 and lower superoxide dismutase 3 gene expression, independent of age and aortic diameter. A significant correlation was observed between baseline T/E ratio and aortic diameter growth rate in BAV non-dil patients ( r =0.66, P Conclusions: The novel evidence of a possible value of the T/E ratio as a biomarker of BAV aortopathy was presented: further validation studies are warranted.

Unresponsive pulmonary hypertension (PH) implies poor posttransplant outcomes. Data on late adaptation of the right ventricle (RV) are still few. This study evaluated three-yr RV function and remodeling, exercise capacity, and hemodynamic data in a selected group of patients initially disqualified because of PH. Between May 2005 and December 2009, 31 consecutive patients were qualified for oral sildenafil because of unresponsive PH at baseline right heart catheterization (RHC). After a 12-wk trial, RHC disclosed PH reversibility (mean PVR: 5.41 ± 3 Wood units, mean TPG 14.5 ± 5.6 mmHg, and mean systolic PAP 68.9 ± 15.1 mmHg), allowing listing even though as high-risk procedures. All patients underwent heart transplantation. RV failure developed in three patients (9.6%), and hospital mortality was 3.2%. Protocol RHC disclosed pulmonary hemodynamic profile normalization within the third postoperative month, allowing weaning from sildenafil in the 30 hospital survivors. One- and three-yr RHCs confirmed stable PH reversal (n = 26, all three-yr survivors). Parameters of late RV function and remodeling proved satisfactory. Parameters of functional capacity (Vo2 peak 19.7 ± 3.6 mL/kg/min and slope VE/Vco2 34.8 ± 2.7) proved homogeneous to those measured in transplant recipients with normal preoperative pulmonary artery pressure. Oral sildenafil is effective in allowing candidacy, safe transplantation, and long-term survival in PH recipients initially disqualified.

Background Degenerative mitral valve (MV) bileaflet prolapse (DMVBP) is recognized as one of the most complex lesions to be treated by reconstructive surgery. In the present study, we report our long-term results with reconstructive surgery for DMVBP. Methods From 2000 to 2011, 140 patients with MV regurgitation due to DMVBP were treated at our institution. Mean age was 56.4 ± 14.5 years (range 16 to 84). Of the 140 study patients, 24 (17%) were in functional class I, 48 (34%) in class II, 60 (43%) in III, and 8 (6%) in class IV of the New York Heart Association. The MV leaflets were reconstructed without using prosthetic material, maintaining the normal shape and dimension of the valve ring and inter-papillary distance. The standard surgical procedure included the excision of the most elongated or ruptured chordae area of the posterior leaflet, with subsequent transposition of second-order chordae from the posterior leaflet to the most elongated or ruptured chordae area of the anterior leaflet. This procedure was performed in 123 patients. A para-commissural edge-to-edge was performed in 16 patients and a triangular resection of the anterior MV leaflet in 1 patient. The posterior leaflet was reconstructed with different techniques: a longitudinal suture of the annulus and residual scallops in 86 patients, a Z-plasty suture in 51 and a sliding suture of the residual posterior scallops in 3 patients. A posterior trygon-to-trygon annuloplasty was performed with an autologous pericardium strip in all patients. During follow-up, serial echocardiograms were obtained once a year. Results There were no hospital deaths. Mean follow-up was 6.42 ± 3.1 years. Of the 140 study patients, 7 developed severe (3+), 10 moderate (2+) and 18 mild (1+) MV regurgitation. Two patients were reoperated within 6 months; 1 after 8 years and 1 after 10 years for recurrence of severe MV regurgitation. At 12 years after the initial surgical procedure, overall survival was 95.8%, freedom from MV re-intervention was 91.8% and freedom from late recurrence of 2+ or greater MV regurgitation was 78.7%. All patients had a satisfactory residual MV area, leaflets motion, and inter-papillary muscle distance. No patient developed left ventricular outflow tract obstruction. Conclusions Our results confirm the effectiveness of anatomic reconstruction in the DMVBP. Survival rate after mitral valve repair is identical to that of the general population when surgery is performed in asymptomatic or pauci-symptomatic patients; the identification of this kind of patient is required followed by a therapeutic strategy for early surgery. This management approach in patients with DMVBP leads to optimal long-term results of MV repair.

Objectives This study sought to identify risk factors for rapid growth of the ascending aorta in patients with bicuspid aortic valve (BAV) disease, taking into account its phenotypic variability. Background Phenotypic heterogeneity of BAV-related aortopathy has recently been widely recognized. However, few studies have addressed the determinants of aortic growth so far, not distinguishing among morphological phenotypes. Methods Serial retrospective data on 133 adult outpatients with BAV undergoing echocardiographic follow-up were analyzed to search for factors associated with aortic diameter growth over time and with rapid aortic growth (fifth quintile of growth rate distribution), focusing on the impact of different valve morphotypes (i.e., cusp fusion pattern: right-left coronary [RL] and right-noncoronary [RN]) and previously defined aortic phenotypes (nondilated aorta, ascending dilation, root dilation). Results The RL pattern was present in 69% of patients with BAV and RN in 31%. At baseline, an ascending dilation phenotype was observed in 57% of patients and a root phenotype in 13.5%. No patient with RN-BAV had a root dilation phenotype at either baseline or last examination. Follow-up time averaged 4.0 ± 2.7 years (535 patient-years). The mean growth rate was 0.3 mm/year at the sinuses and 0.6 mm/year at the ascending level. Aortic regurgitation predicted an increase in ascending diameter over time (odds ratio [OR]: 2.3; p = 0.03). Root phenotype at presentation, not absolute baseline diameter, was an independent predictor of fast progression (>0.9 mm/year) for the ascending tract (OR: 14; p = 0.001). Fast growth was rarely seen in patients with the RL morphotype and ascending phenotype (6% at the root and 10% at the ascending level). Conclusions In patients with BAV, the root phenotype (aortic dilation predominantly at the sinuses, with normal or less dilated ascending tract) may be a marker of more severe aortopathy, warranting closer surveillance and earlier treatment. The more common ascending phenotype proved to be a more stable disease entity, generally with slower progression. © 2013 by the American College of Cardiology Foundation Published by Elsevierinc .

Nowadays, approximately every patient affected from end-stage acute or chronic heart failure may be a potential candidate for MCS; therefore, a careful patient selection for MCS is crucial to establish an effective MCS program, having an impact on both the number of patient treated and the outcomes and the costs of the entire program.

Objectives Both preoperative (disease-related) and operative (management-related) variables make the assessment of the outcomes of acute type A aortic dissection (ATAAD) surgery a difficult task. Our aim was to evaluate the impact of operative factors, including arterial cannulation site, route of cerebral perfusion and surgeon's specific experience with ATAAD ('aortic surgeon'), on the early results of surgical management, with particular attention to neurological injury. Methods Penn classification was used to identify clinically homogeneous risk groups of ATAAD patients undergoing surgery. Between January 2007 and June 2014, 111 of 183 ATAAD patients treated with open surgery in a single centre were in Penn Class Aa (no ischaemic complications at presentation). They were divided in two groups depending on the arterial cannulation site: femoral artery (FemA; 56 patients) or right axillary artery (RAxA; 55 patients). Study outcomes included: 30-day mortality, major adverse cardiac and cerebrovascular events at 30 days, neurological complications and in particular, patterns of stroke as defined by Bamford classification. Results No significant differences in preoperative variables were observed between cannulation-site groups, except for myocardial ischaemic time (60.9 ± 30.4 min in the RAxA group vs 81.7 ± 52.3 in the FemA group, P = 0.014) and cerebral perfusion time (42.1 ± 25.5 min in the RAxA group vs 52.9 ± 32.6 in the FemA group, P = 0.048). Outcomes in terms of mortality and neurological injury did not differ except for a higher incidence of lacunar cerebral infarction (LACI) in the RAxA group (14.5 vs 3.6%, P = 0.043), mainly but not exclusively explained by a higher incidence of LACI in unilateral (17.2%) than in bilateral cerebral perfusion (6.9%) within the RAxA group. The 'non-aortic surgeon' was associated instead with 30-day mortality and composite outcome in multivariable analysis (respectively, OR 6.40, P = 0.002 and OR 4.68, P = 0.001). Conclusions The RAxA cannulation and FemA cannulation are associated with comparable 30-day mortality following surgery for aortic dissection. However, the possible higher risk of LACI-type strokes in the RAxA group, especially when associated with unilateral brain perfusion, should be considered when RAxA cannulation is performed in ATAAD. The hypothesis that more experienced surgeons may produce better earlier outcomes warrants further investigation.

Objectives: Different methods to classify the anatomical configurations of the aorta with bicuspid aortic valve (BAV) have been proposed. We aimed to test them in terms of descriptive power (i.e. capability to identify different clusters of patients with unique associations of anatomo-clinical features) and possible prognostic significance. Methods: A consecutive echocardiographic series of 696 BAV patients (mean age 48 ± 16 years, male:female ratio 3:1) was analysed. Three possible schemes for classification of the patterns of aortic dimensions were compared. One defined the aortic shape as 'N' (ascending < sinuses > sinotubular junction (STJ)), 'A' (ascending > sinuses > STJ) or 'E' (sinotubular ≥ sinuses), the second as 'non-dilated', 'ascending phenotype' (dilated, with ascending > sinuses) or 'root phenotype' (dilated, with sinuses > ascending) and the third as normal, 'type I' (dilated only at the ascending tract), 'type II' (dilated at both ascending and sinus levels) or 'type III' (dilated only at the sinuses). We evaluated the correlation with valve morphotypes (right-left fusion, right-non-coronary fusion) and patient characteristics. In a smaller longitudinal study (n = 150), the occurrence of fast growth of the aorta (fifth quintile: ≤1 mm/year) during follow-up (5 ± 3 years) in the various phenotypes was assessed. Results: The three classification methods proved meaningful in terms of association with valve morphotypes: significant associations were found between right-left-coronary BAV and the root phenotype (P < 0.001) and between the right-non-coronary BAV and the shapes A and E (P1 mm/year) of the ascending diameter (50% root phenotype patients; P = 0.005). The association with the N type was weaker (P = 0.055); no association was found with types from the other classification scheme (P = 0.42). Conclusions: When tested on a large population, three previously suggested phenotypic classifications of the BAV aorta proved to categorize patients into significantly different clusters, but only the classification system distinguishing between ascending phenotype and root phenotype showed a potential prognostic value. Phenotypic class of the aorta could be a factor to integrate in future comprehensive models for risk stratification of BAV aortopathy. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Primary cardiac tumors do not occur frequently, and only one quarter of them, chiefly sarcomas, are malignant. Patients with angiosarcoma typically have a shorter survival time than do patients with other sarcomas, and the prognosis for survival depends strictly on the stage of the disease at the time of diagnosis and the possibility of complete surgical excision. Chemotherapy and radiotherapy have well-established postoperative roles because of the high probability of metastasis. We report the case of a 25-year-old man who presented with pericardial effusion and echocardiographic evidence of an intracavitary right atrial mass but without the bulky, infiltrative growth typical of this location of the disease. Malignancy was suggested by the clinical presentation, the location of the mass in the right side of the heart, and the absence of conditions favoring thrombus formation. After complete surgical excision, the mass was confirmed to be an angiosarcoma. Conventional adjuvant chemotherapy and maintenance therapy with inhibitors of CD117 (c-kit) and vascular endothelial growth factor relieved the patient's clinical symptoms and enabled his long-term, disease-free survival. In addition to reporting this case, we discuss aspects of the diagnosis and treatment of angiosarcoma.

Objectives: The aim of this study was to assess the early and long-term outcomes of a previously introduced technique of reduction aortoplasty for asymmetric ascending aortic dilatation. Different indication criteria for reduction ascending aortoplasty have been previously adopted by others, thus another purpose was to identify the patient profile for whom this approach may be best suited. Methods: Between January 2001 and December 2010, reduction ascending aortoplasty with “waistcoat technique” was performed in 156 patients (mean age 62±12 years, 61% male) with asymmetric dilatation of the ascending aorta (prevailing at the convexity of the supracoronary tract). Eighty-seven patients had a tricuspid aortic valve (TAV), 69 a bicuspid aortic valve (BAV). Aortoplasty was associated to aortic valve replacement in 60% cases. Preoperative, intraoperative, early postoperative and follow-up data were analysed. Comparisons were performed between groups of valve morphology (TAV versus BAV) and subgroups of baseline valve function. In patients with a follow-up time >1 year the annual growth of the ascending tract was calculated and compared between subgroups. The independent predictors of growth velocity were assessed by multivariable linear regression analysis. Results: Mean cross-clamp and cardiopulmonary bypass times were 39 ± 18 and 69 ± 29 min, respectively. Hospital death was 1.9%. In no case, postoperative death or any early complication was causally related to the aortoplasty procedure. The mean postoperative ascending diameter was 3.1 ± 0.3 (versus preoperative 5.2 ± 0.8 cm, P< 0.001). Mean follow-up time was 4 ± 2.5 years (maximum 10 years): 7-year survival was 95 ± 2%; 7-year freedom from aortic events 94 ± 4%. Redilatation (ascending diameter exceeding 4.5 cm) occurred in two patients, acute dissection in one: all three preoperatively had significant aortic regurgitation. The mean ascending aortic diameter at last follow-up was 3.4 ± 0.5 cm; median diameter progression was 0.4 mm/year, with no significant difference between TAV and BAV and no patient reaching 0.5 cm/year. With TAV, the only determinant of aortic growth rate was normal preoperative valve function (P= 0.04); with BAV, the degree of regurgitation at preoperative echocardiography (P= 0.001). Conclusions: Waistcoat aortoplasty proved a safe and durable treatment for patients with asymmetric non-syndromic non-familial ascending aorta dilatation. The technique showed its best durability in aortic stenosis patients and in patients with normofunctional BAV.

OBJECTIVES: Unresponsive pulmonary hypertension (PH) may contraindicate heart transplant since it implies poor early outcomes. The present study reports the effectiveness of oral perioperative sildenafil in allowing heart transplant candidacy and surgery in a selected group of patients initially deemed ineligible because of PH. METHODS: Between May 2005 and December 2009, 31 consecutive patients (5 females, 9 with a history of idiopatic cardiomyopathy and 16 with a history of coronary artery disease, 10 with previous sternotomies, 71.42 ± 27.69 ml/min/m 2 mean pre-operative epidermal growth factor receptor) were qualified for oral sildenafil because of unresponsive PH at baseline right heart catheterization (RHC). After a 12-week trial, RHC disclosed PH reversibility (mean pulmonary vascular resistance index: 9.57 ± 4.07 WU, mean transpulmonary gradient 14.47 ± 5.66 mmHg and mean systolic pulmonary artery pressure: 68.96 ± 15.15 mmHg), allowing listing despite a higher risk for early post-transplant RV failure. Transplant protocol included donor/recipient size matching ≥0.8 and inhaled nitric oxide in the early post-operative period followed by reinstitution of oral sildenafil. RESULTS: All patients underwent heart transplantation. Mean overall graft ischaemic time was 179 ± 47min; mean donor recipient weight ratio was 1.04 ± 0.17. Right ventricular failure developed in three patients (9.6%) and hospital mortality was 3.2%. Protocol RHC disclosed pulmonary haemodynamic profile normalization within the third post-operative month allowing weaning from sildenafi li n the 30 hospital survivors. One-year RHC confirmed PH reversal (n= 29 patients, all who survived up to 1year). CONCLUSIONS: This pilot prospective uncontrolled trial suggests that oral sildenafil is effective in allowing candidacy, safe transplantation and post-operative pulmonary profile normalization in potential recipients initially disqualified because of PH.

gavenoinformationaboutanyliverproblemandnoscreeningforhepaticfunctionwasperformedbythistime.Therewasnosymtomorsignofcongestiveheartfailureinthepreoperativeperiod.Results: The patient underwent coronary artery bypass graftingprocedure. Laboatory findings on postoperative 1st day and 2nddayrevealedelevatedliverfunctiontests(AST1430,ALT880,LDH3360andAST6680,ALT2600,LDH9150,respectively).Thepatientdiedonthe2ndpostoperativedayduetofulminanthepaticfailureand metabolic encephalopathy. Postoperative period was highlyprogressivewithaggressiveworseningofhepaticfunctionswhichcausedmultiorganfailureandeventuallydeath.Conclusions: Fulminanthepaticfailureisarareconditionseenafteropenheartsurgeryisconstantlyprogressiveandfatal.PreoperativeHbsAgpositivityshouldbetakenunderconsiderationasastrictriskfactor. Careful preoperative evaluation should be performed andHbsAgpositivityshouldforcethedoctorstoeveluateliverfunctionin detail. To minimize fulminant hepatic failure, further effortsshould focus on off-pump surgery, shorter X-clamp time, shortercardiopulmonary bypass time, avoidance of potential hepatotoxicmedication.PP-266SURGICAL MANAGEMENT OF INFECTED RIGHT ATRIALTHROMBUS RELATED TO TEMPORARY UNTUNNELLEDHEMODIALYSIS CATHETER IN END-STAGE RENAL DISEASEA.I.Rezk.

Heart transplant is the golden standard in the management of end-stage heart failure. Recent studies have pointed out the role of nutritional issues in patients evaluated for heart transplant listing. In particular, extremes in body habitus, cachexia and obesity, have been characterized and identified as independent prognostic factors and clinically relevant target for therapeutic interventions. Effects of such conditions exert a prognostic implication well beyond waiting time up to early post transplant setting. Changes in posttransplant clinical conditions and nutritional status have been recently described in their pattern of presentation and implications on weight gain, reversal of preoperative cachexia and early and late morbidity and mortality. New onset diabetes mellitus and metabolic syndrome have been disclosed as relevant clinical conditions in this setting. Implications for tailoring of immunosuppressive therapy and dietary prescription emerged as main stem of long term recipient management. All this issues have been reviewed focusing on the clinical relevance of this growing body of knowledge and emphasizing the role of a multidisciplinary approach for selection and management of heart transplant recipients.