Is there a renoprotective value to leukodepletion during heart valve surgery? A randomized controlled trial (ROLO).

BACKGROUND: Acute Kidney Injury (AKI) adversely affects outcomes after cardiac surgery. A major mediator of AKI is the activation of leukocytes through exposure to the cardiopulmonary bypass circuit. We evaluate the use of leukodepletion filters throughout bypass to protect against post-operative AKI by removing activated leukocytes during cardiac surgery. METHODS: This is a single-centre, double-blind, randomized controlled trial comparing the use of leukodepletion versus a standard arterial filter throughout bypass. Elective adult patients undergoing heart valve surgery with or without concomitant procedures were investigated. The primary clinical outcome measured was the development of AKI according to the KDIGO criteria. Secondary measures included biomarkers of renal tubular damage (urinary Retinol Binding Protein and Kidney Injury Molecule-1), glomerular kidney injury (urinary Micro Albumin and serum Cystatin C) and urinary Neutrophil Gelatinase Associated Lipocalin, as well as the length of hospital stay and quality of life measures through EQ-5D-5L questionnaires. RESULTS: The ROLO trial randomized 64 participants with a rate of recruitment higher than anticipated (57% achieved, 40% anticipated). The incidence of AKI was greater in the leukodepletion filter group (44% versus 23%, risk difference 21, 95% CI - 2 to 44%). This clinical finding was supported by biomarker levels especially by a tendency toward glomerular insult at 48 h, demonstrated by a raised serum Cystatin C (mean difference 0.11, 95% CI 0.00 to 0.23, p = 0.068) in the leukodepleted group. There was however no clear association between the incidence or severity of AKI and length of hospital stay. On average, health related quality of life returned to pre-operative levels in both groups within 3 months of surgery. CONCLUSIONS: Leukocyte depletion during cardiopulmonary bypass does not significantly reduce the incidence of AKI after valvular heart surgery. Other methods to ameliorate renal dysfunction after cardiac surgery need to be investigated. TRIAL REGISTRATION: The trial was registered by the International Standard Randomized Controlled Trial Number Registry ISRCTN42121335 . Registered on the 18 February 2014. The trial was run by the Bristol Clinical Trials and Evaluation Unit. This trial was financially supported by the National Institute of Health Research (Research for Patient Benefit), award ID: PB-PG-0711-25,090.

Retrospective Review of a Prothrombin Complex Concentrate (Beriplex P/N) for the Management of Perioperative Bleeding Unrelated to Oral Anticoagulation.

A multicenter, retrospective, observational study of 4-factor prothrombin complex concentrate (PCC) and/or fresh frozen plasma (FFP) use within routine clinical care unrelated to vitamin K antagonists was conducted. The PCC was administered preprocedure for correction of coagulopathy (prophylactic cohort) and treatment of bleeding postsurgery (treatment cohort). Of the 445 patients included, 40 were in the prophylactic cohort (PCC alone [n = 16], PCC and FFP [n = 5], FFP alone [n = 19]) and 405 were in the treatment cohort (PCC alone [n = 228], PCC and FFP [n = 123], FFP alone [n = 54]). Cardiovascular surgery was the most common setting. PCC doses ranged between 500 and 5000 IU. Effectiveness (assessed retrospectively) was reported as effective in 93.0% in the PCC-only group (95% confidence interval, 89.1% to 95.9%), 78.9% (70.8% to 85.6%) with PCC and FFP, and 86.3% (76.2% to 93.2%) with FFP alone. In the treatment cohort, international normalized ratio was significantly reduced in all 3 groups. In patients who received PCC, the rate of thromboembolic events (1.9%) was below rates in the literature for similar procedures. PCCs offer a potential alternative to FFP in the management of perioperative bleeding unrelated to oral anticoagulant therapy.

Balloon assisted retraction of a migrated CoreValve Evolut R bioprosthesis during cardiac arrest.

We describe a case of balloon assisted retraction of a migrated CoreValve Evolut R bioprosthesis during trans-femoral TAVI.

Effect of Opioids vs NSAIDs and Larger vs Smaller Chest Tube Size on Pain Control and Pleurodesis Efficacy Among Patients With Malignant Pleural Effusion: The TIME1 Randomized Clinical Trial.

IMPORTANCE: For treatment of malignant pleural effusion, nonsteroidal anti-inflammatory drugs (NSAIDs) are avoided because they may reduce pleurodesis efficacy. Smaller chest tubes may be less painful than larger tubes, but efficacy in pleurodesis has not been proven. OBJECTIVE: To assess the effect of chest tube size and analgesia (NSAIDs vs opiates) on pain and clinical efficacy related to pleurodesis in patients with malignant pleural effusion. DESIGN, SETTING, AND PARTICIPANTS: A 2×2 factorial phase 3 randomized clinical trial among 320 patients requiring pleurodesis in 16 UK hospitals from 2007 to 2013. INTERVENTIONS: Patients undergoing thoracoscopy (n = 206; clinical decision if biopsy was required) received a 24F chest tube and were randomized to receive opiates (n = 103) vs NSAIDs (n = 103), and those not undergoing thoracoscopy (n = 114) were randomized to 1 of 4 groups (24F chest tube and opioids [n = 28]; 24F chest tube and NSAIDs [n = 29]; 12F chest tube and opioids [n = 29]; or 12F chest tube and NSAIDs [n = 28]). MAIN OUTCOMES AND MEASURES: Pain while chest tube was in place (0- to 100-mm visual analog scale [VAS] 4 times/d; superiority comparison) and pleurodesis efficacy at 3 months (failure defined as need for further pleural intervention; noninferiority comparison; margin, 15%). RESULTS: Pain scores in the opiate group (n = 150) vs the NSAID group (n = 144) were not significantly different (mean VAS score, 23.8 mm vs 22.1 mm; adjusted difference, -1.5 mm; 95% CI, -5.0 to 2.0 mm; P = .40), but the NSAID group required more rescue analgesia (26.3% vs 38.1%; rate ratio, 2.1; 95% CI, 1.3-3.4; P = .003). Pleurodesis failure occurred in 30 patients (20%) in the opiate group and 33 (23%) in the NSAID group, meeting criteria for noninferiority (difference, -3%; 1-sided 95% CI, -10% to ∞; P = .004 for noninferiority). Pain scores were lower among patients in the 12F chest tube group (n = 54) vs the 24F group (n = 56) (mean VAS score, 22.0 mm vs 26.8 mm; adjusted difference, -6.0 mm; 95% CI, -11.7 to -0.2 mm; P = .04) and 12F chest tubes vs 24F chest tubes were associated with higher pleurodesis failure (30% vs 24%), failing to meet noninferiority criteria (difference, -6%; 1-sided 95% CI, -20% to ∞; P = .14 for noninferiority). Complications during chest tube insertion occurred more commonly with 12F tubes (14% vs 24%; odds ratio, 1.91; P = .20). CONCLUSIONS AND RELEVANCE: Use of NSAIDs vs opiates resulted in no significant difference in pain scores but was associated with more rescue medication. NSAID use resulted in noninferior rates of pleurodesis efficacy at 3 months. Placement of 12F chest tubes vs 24F chest tubes was associated with a statistically significant but clinically modest reduction in pain but failed to meet noninferiority criteria for pleurodesis efficacy. TRIAL REGISTRATION: isrctn.org Identifier: ISRCTN33288337.

Staged hybrid treatment for contained rupture of a large right coronary artery aneurysm.

We describe a staged approach to the management of a rare acute condition--contained rupture of a large right coronary artery aneurysm. A covered stent was deployed percutaneously to isolate the aneurysm at presentation followed by planned coronary bypass grafting. Treatment interval was complicated by new-onset pulmonary tuberculosis and subacute thrombosis of the covered stent leading to nonfatal inferior myocardial infarction. Coronary surgery was performed after complete antitubercular treatment and resolution of the acute pericarditis/thrombosis as a consequence of the contained rupture. The advantages of this staged approach included the following: (a) The covered stent prevented both acute myocardial infarction and progressive pseudoaneurysm expansion in the acute phase. (b) Deferred surgery was rendered technically less hazardous while avoiding the undesirable option of having to exclude an extremely calcified dominant right coronary artery. The patient made an excellent postoperative recovery with complete resolution of her symptoms at 6 weeks' follow-up.

Duplicated common femoral artery can be safely cannulated for femorofemoral cardiopulmonary bypass.

The duplicated common femoral artery can be safely cannulated for femorofemoral bypass, but we recommend postoperative imaging to identify potential complications. We found no previous reports of duplicated common femoral artery.

Leukodepletion for patients undergoing heart valve surgery.

BACKGROUND: There is some evidence for the benefits of leukodepletion in patients undergoing coronary artery surgery. Its effectiveness in higher risk patients, such as those undergoing heart valve surgery, particularly in terms of overall clinical outcomes, is currently unclear. OBJECTIVES: To assess the beneficial and harmful effects of leukodepletion on clinical, patient-reported and economic outcomes in patients undergoing heart valve surgery. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (2013, Issue 3 of 12) in The Cochrane Library, the NHS Economic Evaluations Database (1960 to April 2013), MEDLINE Ovid (1946 to April week 2 2013), EMBASE Ovid (1947 to Week 15 2013), CINAHL (1982 to April 2013) and Web of Science (1970 to 17 April 2013) on 19 April 2013. We also searched the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP), the US National Institutes of Health (NIH) clinical trials database and the International Standard Randomised Controlled Trial Number Register (ISRCTN) in April 2013 for ongoing studies. No language or time period restrictions were applied. We examined the reference lists of all included randomised controlled trials and contacted authors of identified trials. We searched the 'grey' literature at OpenGrey and handsearched relevant conference proceedings. SELECTION CRITERIA: Randomised controlled trials comparing a leukocyte-depleting arterial line filter with a standard arterial line filter, on the arterial outflow of the heart-lung bypass circuit, in elective patients undergoing heart valve surgery. DATA COLLECTION AND ANALYSIS: Data were collected on the study characteristics, three primary outcomes (1. post-operative in-hospital all-cause mortality within three months, 2. post-operative all-cause mortality excluding inpatient mortality < 30 days, 3. length of stay in hospital, 4. adverse events and serious adverse events) and seven secondary outcomes (1. tubular or glomerular kidney injury, 2. validated health-related quality of life scales, 3. validated renal injury scales, 4. use of continuous veno-venous haemo-filtration, 5. length of stay in intensive care, 6. costs of care). Data were extracted by one author and verified by a second author. Insufficient data were available to perform a meta-analysis or sensitivity analysis. MAIN RESULTS: Eight studies were eligible for inclusion in the review but data on prespecified review outcomes were available from only one, modestly powered (24 participants) study (Hurst 1997). There were no differences between a leuko-depleting versus standard filter in length of stay in the intensive care unit (ICU) (mean difference (MD) 0.80 days; 95% confidence interval (CI) -0.24 to 1.84) or length of hospital stay (MD 0.20 days; 95% CI -1.78 to 2.18). AUTHORS' CONCLUSIONS: There are currently insufficient good quality trials with valve surgery patients to inform recommendations for changes in clinical practice. A future National Institute for Health Research (NIHR)-funded feasibility study (recruiting mid-year 2013) comparing leukodepletion with a standard arterial line filter in patients undergoing elective heart valve surgery (the ROLO trial) will be the largest study to date and will make a significant contribution to future updates of this review.

Early and late prognostic implications of coronary artery bypass timing after myocardial infarction.

OBJECTIVES: The optimal timing of coronary artery bypass grafting (CABG) after myocardial infarction (MI) is still controversial. With advances in perioperative care and myocardial protection, CABG is not infrequently undertaken sooner. Although CABG soon after MI is associated with high morbidity and mortality, the impact of CABG timing on late survival is not clear. METHODS: We analysed prospectively collected data for 8320 patients who underwent primary CABG from 1996 through 2010. Operative outcomes and late survival were compared between patient categories based on MI-to-CABG days: groups A (0-30, n = 658), B (31-60, n = 734), C (>90, n = 2698) and D (no MI, n = 4230). The effect of the timing of surgery on survival was determined using multivariate and Kaplan-Meier analyses. RESULTS: As the MI-to-CABG interval increased, the frequency of urgent/emergency operations decreased and hospital mortality (A, 3.5% vs B, 2.6% vs C, 1.2%, vs D, 1.1%, P < 0.0001) steadily declined. In general, patients who had CABG within 90 days of MI had more cardiac morbidity and co-morbidities. Expectedly, therefore, postoperative organ system dysfunction (cardiac, renal, respiratory and neurological) was more frequent in these groups. Reoperation for bleeding was similar for all groups, but blood product transfusion decreased as the MI-to-CABG days increased. The 10-year survival improved with the MI-to-CABG interval (A, 72.2% vs B, 73.4% vs C, 75.8% vs D, 81.4%, P < 0.0001). By multivariate analysis, the MI-to-CABG interval was not a risk factor for operative or late mortality. However, less frequent were left internal mammary artery use, non-elective surgery and high blood transfusion rates; all more often associated with shorter MI-to-CABG intervals. CONCLUSIONS: Early and late mortality risk for CABG declines with increasing interval from MI for reasons indirectly linked to the timing of surgery. Our findings emphasize the importance of preoperative organ system optimization and consistent left internal mammary artery use, regardless of the proximity of surgery to MI or the exigency of surgery.

A surgical strategy for cor triatriatum atrial septal defect--A1 lam subclass.

Atrial fibrillation and a heart murmur were diagnosed in a 68-year-old woman during a routine medical examination. She presented 2 years later with pulmonary edema. A transthoracic echocardiography examination revealed a tunneled atrial septal defect (ASD) and severe tricuspid regurgitation. Transesophageal echocardiography and 3-dimensional computed tomography evaluations revealed multiple intracardiac defects, including abnormal atrial septation suggestive of a typical cor triatriatum sinistrum (A1 Lam subclass), a rare congenital defect in adults. The patient underwent tricuspid valve repair with concomitant closure of the ASD by using the cor triatriatum curtain to form an autologous transposition flap. The intraoperative transesophageal and predischarge imaging evaluations confirmed an excellent repair. The patient made a swift recovery and demonstrated improvement in her symptoms at follow-up. This previously undescribed technique eliminates the need for a prosthetic implant and is applicable in >80% of cor triatriatum cases in which an ASD exists.

In patients undergoing video-assisted thoracoscopic surgery excision, what is the best way to locate a subcentimetre solitary pulmonary nodule in order to achieve successful excision?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'In patients undergoing video-assisted thoracoscopic surgery (VATS) excision, what is the best way to locate a subcentimetre solitary pulmonary nodule (PN) in order to achieve successful excision?' Altogether, 107 papers were found using the reported search, of which 19 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers were tabulated. The hook-wire technique showed a varied success rate ranging from 58 to 97.6% and a relatively higher failure rate due to wire dislodgement. The most common complication of this method was pneumothorax. CT-guided spiral-wire localization displayed a success rate of 86% with the added advantage of providing more stability than the hook-wire technique and permitting manipulation. Radio-guided localization techniques and fluoroscopic-aided methods using contrast media displayed consistently high sensitivities with few complications. The radio-guided technique had the benefit of allowing a longer time-period between the staining of the nodule and the operation. Ultrasonography showed sensitivities ranging from 92.6 to 100%; however, it is highly operator-dependent. Finger palpation was shown to achieve suboptimal results and should be avoided. We concluded that radio-guided surgery is a preferable method. It showed high accuracy with minimal complications and operator dependence in detecting subcentimetre PNs when compared with other techniques such as ultrasonography, finger palpation, fluoroscopic, hook-wire, spiral-wire and microcoil localization.

The impact of major perioperative renal insult on long-term renal function and survival after cardiac surgery.

Temporary renal replacement therapy (RRT) facilitates recovery from a major perioperative renal injury and, although RRT can improve the hospital outcome, it is not known as to whether it mitigates long-term renal sequelae. Therefore, we investigated the risk of long-term dialysis after RRT post-cardiac surgery. We analysed prospectively the data collected for all hospital survivors who received RRT following cardiac surgery between March 1996 and July 2010, excluding those on dialysis preoperatively or with a functioning renal transplant. The follow-up data were obtained for all surviving patients. The mean age of the 82 patients was 68.6 ± 9.9 years, and 60 (73%) were male. Severe pre-existing renal dysfunction with a serum creatinine level of >200 µmol/l was present in 15 (18%) patients and diabetes in 31 (38%) patients. Operative procedures included redo surgery (n = 11, 13%) and thoracic aortic surgery (n = 9, 11%). During a 13.4-year follow-up, there were 38 late deaths. Only three patients with severe preoperative renal dysfunction received dialysis. The Kaplan-Meier 5- and 7-year survival rates for this patient cohort were 54% and 38%, respectively. In conclusion, a major renal insult requiring temporary RRT after cardiac surgery does not increase the risk for renal dialysis in the long term for patients with normal renal function preoperatively.

Is a fully heparin-bonded cardiopulmonary bypass circuit superior to a standard cardiopulmonary bypass circuit?

A best-evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Is a fully heparin bonded cardiopulmonary bypass circuit superior to a standard cardiopulmonary bypass circuit?' Altogether more than 792 papers were found using the reported search, of which 13 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated (Table 1). The studies analysed show that perfusion with heparin-coated and heparin-polymer-coated bypass does not increase the risk of adverse effects but reduces blood loss, re-operation rates, ventilation time, length of intensive care unit (ICU) and hospital stay and is also associated with improved biocompatibility, as evidenced by platelet preservation, reduced leucocyte and complement activation, and proinflammatory cytokine production. The various coated circuits have comparable biocompatibility as evaluated by a range of inflammatory markers and clinical outcomes. Three studies documented a significant decrease in post-operative blood loss (P = 0.001-0.54) and a meta-analysis found that perfusion with a heparin-bonded circuit resulted in a reduction in blood transfusion requirements (20%), ventilation time (P < 0.01), length of time in the ICU (P < 0.01) and also hospital stay (P = 0.02). Two studies found reduced levels of polymorphonuclear elastase (P < 0.018-0.001) and two trials concluded that the use of heparin-coated circuits in combination with low-dose systemic heparin (activated clotting time >250) resulted in the greatest clinical benefit and improvement in inflammation. One study documented significant platelet preservation with the use of third-generation heparin-polymer-bonded circuits (P ≤ 0.05). We conclude that despite heparin-bonded and newer third-generation heparin-polymer-bonded cardiopulmonary bypass circuits having a greater cost per person, their improved clinical outcomes and biocompatibility in patients undergoing cardiac surgery make them a preferable option to standard non-heparin-bonded circuits.

Risk modeling for ventricular assist device support in post-cardiotomy shock.

BACKGROUND: Post-cardiotomy shock (PCS) has a complex etiology. Although treatment with inotrops and intra-aortic balloon pump (IABP) support improves cardiac performance, end-organ injuries are common and lead to prolonged ICU stay, extended hospitalization and increased mortality. Early consideration of mechanical circulatory support may prevent such complications and improve outcome. METHODS: Between January 1997 and January 2002, 321 patients required IABP and inotropic support for PCS following coronary artery bypass grafting (CABG) at our institution. Perioperative variables including age, mixed venous saturation (MVO2), inotropic requirements and LV function were analyzed using multivariate statistical methods. All explanatory variables with a univariate p value <0.10 were entered into a stepwise logistic regression model to predict hospital mortality. Odds ratios from significant variables (p < 0.05) in the regression model were used to compose a risk score. RESULTS: Overall hospital mortality was 16%. The independent risk factors for mortality in this population were: MVO2 < 60% (OR = 3.2), milrinone > 0.5 µg/kg/min (OR = 3.2), age > 75 (OR = 2.7), adrenaline > 0.1 µg/kg/min (OR = 1.5). A 15-point risk score was developed based on the regression model. Hospital mortality in patients with a score >6 was 46% (n = 13/28), 3-6 was 31% (n = 9/29) and <3 was 11% (n = 29/264). CONCLUSIONS: A significant proportion of patients with PCS continue to face high mortality despite IABP and inotropic support. Advanced age, heavy inotropic dependency and poor oxygen delivery all predicted increased risk for death. Further investigation is needed to assess whether early institution of VAD support could improve outcome in this high-risk group of patients.

Does getting smokers to stop smoking before lung resections reduce their risk?

A best-evidence topic in thoracic surgery was written according to a structured protocol. The question of whether the incidence of major pulmonary morbidity after lung resection was associated with the timing of smoking cessation was addressed. Overall 49 papers were found using the reported search outlined below, of which 7 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. In most studies, smoking abstinence was shown to reduce the incidence of post-operative pulmonary complications (PPCs) such as pneumonia, respiratory distress, atelectasis, air leakage, bronchopleural fistula and re-intubation. The timing of cessation is not clearly identified, although there is some evidence showing reduction in risk of PPCs with increasing interval since cessation. Two studies suggested that smoking abstinence for at least 4 weeks prior to surgery was necessary in order to reduce the incidence of major pulmonary events. Furthermore, it was also shown that a pre-operative smoke-free period of >10 weeks produced complication rates similar to those of patients who had never smoked. We conclude that smoking cessation reduces the risk of PPCs. All patients should be advised and counseled to stop smoking before any form of lung resection.

Complete excision of giant calcified saphenous vein graft aneurysm in redo coronary artery bypass grafting.

A 73-year-old female who first underwent coronary artery bypass graft surgery in 1987 represented in 2008 with recurrent angina. Coronary angiography demonstrated a giant calcified aneurysm of the saphenous vein graft (SVG) to the right coronary artery bracketed by severe stenoses in addition to severe disease in the native vessels. Following 64-slice computed tomography coronary angiography, total resection of the aneurysm with en-bloc excision of the contagious right atrial free wall and ascending aorta were accomplished during redo coronary revascularization. The pathophysiology of SVG aneurysm, the clinical relevance and therapeutic approaches will be discussed in light of this case.

Use of contrast-enhanced computed tomography for management of a late-onset spontaneous massive chest wall hematoma.

This is a rare case of a 68-year-old woman who was rehospitalized after uneventful redo double-valve surgery. An 8.3 × 12.9 × 16.4 cm tense right chest wall hematoma was diagnosed. This was precipitated by a single cough. Contrast-enhanced computed tomography revealed a bleeding source. Hematoma evacuation and hemostasis following emergency warfarin reversal produced an excellent outcome.

Off-pump coronary artery bypass in poland syndrome with dextrocardia: case report.

Poland Syndrome is a congenital disorder characterised by hypoplasia of the pectoral muscles along with upper extremity deformities. We encountered a patient with Poland syndrome associated with dextrocardia and also failed pectus excavatum repairs who presented to us with symptomatic ischaemic heart disease requiring intervention. He underwent successful off-pump coronary artery bypass surgery (OPCABG). As far as we are aware, this is the first case report of OPCABG in a case of Poland syndrome with dextrocardia. We describe here the management of this complex patient and wish to emphasise that the off-pump option is feasible in dextrocardia with some technical modifications.

Extensive aneurysms of sinuses of Valsalva precluding valve sparing aortic root reimplantation (David procedure).

A 55-year-old female noticed worsening exertional dyspnoea for two years. She was born with cleft palate and profound deafness. Significant physical findings included dysmorphism with micrognathia and acrocephaly and congenital deafness. Transthoracic echocardiogram revealed aneurysms involving the right and the non-coronary sinuses of Valsalva. Despite that, the native aortic valve retained preserved geometry. Computed tomography (CT)-scan demonstrated multiple aneurysms arising from all three sinuses of Valsalva. This displaced the right ventricle (RV) caudally and indented the RV outflow tract. A valve-sparing root reimplantation was planned. However, intraoperatively the root aneurysms were found to be very extensive such that no healthy tissue remained along the insertion lines of the aortic valve leaflets. The aortic annulus was not dilated (2 cm) and the left ventricular outlow tract was not involved in the disease process. Consequently, despite the presence of macroscopically normal leaflets and relatively undisturbed annular geometry, we were unable to reimplant the native aortic valve and proceeded to a modified Bentall procedure. Histologically, significant medial degeneration with loss of elastin and muscle was identified in the aortic sinus wall. Similar changes were also found affecting the native leaflets coupled with increased fibrous thickening.

Sinus of valsalva aneurysm in Blau's syndrome.

Blau syndrome is a rare granulomatous disorder inherited in an autosomal dominant manner characterized by the early appearance of granulomatous arthritis, skin rash and anterior uveitis. There are very few data on the cardiovascular manifestations of Blau syndrome. Here we report the first case of sinus of valsava aneurysm in Blau syndrome. In isolated unruptured aneurysms of a sinus of Valsalva without compromise of the aortic valve and/or the coronary ostia, repair may be accomplished by simple placation of the aneurysm or excision of the aneurysm(s) and patch closure of the defect(s) between the aortic annulus and the sinu-vascular ridge. Because of the particular conditions in our case, the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall's technique.