Hereditary angioedema in cardiac surgery: Perioperative management considerations for a rare disease.

INTRODUCTION: Hereditary Angioedema is a rare disease caused by C1 esterase inhibitor deficiency leading to diffuse and potentially life-threatening oedema formation. Preventing attacks is critical, particularly for patients undergoing cardiac surgery. CASE REPORT: We report a case of a 71-years-old woman with a history of Hereditary Angioedema scheduled for open-heart surgery on Cardiopulmonary Bypass. Multidisciplinar teamwork and patient-targeted strategy were crucial to obtain a favorable outcome. DISCUSSION: Cardiac surgery is a major stressor for Angioedema attacks because of Complement cascade and inflammatory response activation leading to potential life-threatening oedema formation. In literature only few cases of complex open heart surgery under Cardiopulmonary Bypass are described. CONCLUSION: Continuous updating and multidisciplinarity are key elements to manage patients with Hereditary Angioedema in cardiac surgery in order to reduce morbidity and mortality.

Sleeve Technique Combined With Aortic Valve Replacement: A Simpler Alternative to the Bentall Procedure.

Aortic root disease can be treated with different surgical techniques. The surgical approach of choice depends on patients' comorbidities and aortic valve conditions. We describe an operation combining a Sleeve aortic rootplasty with an aortic valve replacement, as an alternative to the classical Bentall procedure. The patient, a 63-year-old man, was admitted to our institution in April 2019 with severe aortic regurgitation, left ventricle dysfunction (ejection fraction = 44%), and aortic root ectasia (476 mm). Since a sparing aortic valve procedure was judged not feasible because of the thickness and retraction of the leaflets, we decided to perform a procedure through a sleeve operation to treat the aortic ectasia and a standard bioprosthetic aortic valve replacement to treat the aortic regurgitation. The patient had an uneventful postoperative course and was discharged on the sixth postoperative day with aspirin as sole antiplatelet treatment. This procedure may be considered an alternative to the Bentall operation. In fact, it may also offer complete treatment for the aortic root disease, avoiding coronary complications due to coronary ostia manipulation and reimplantation.

Use of new cannulated screws for primary sternal closure in high risk patients for sternal dehiscence.

BACKGROUND: Median sternotomy is still the approach of choice for heart surgery and the most common sternal closure is conventional six-wire technique. Mediastinitis is the frightening complication of the median sternotomy and occurs from 1% up to 2.4% of cases with a mortality rate which ranges from 14% up to 47%. Many methods have been suggested to reinforce the sternum with the common goal to improve sternal stability and prevent mediastinitis, but there is not consensus among cardiac surgeons on the optimal way to close the sternum. For this reason, we report our experience with a sternal device that, although not entirely new as a concept, has new technical features and plays a new role in preventing sternal dehiscence, according to the most recent findings on mechanism which leads to sternal dehiscence. METHODS: We enrolled 62 high risk patients for sternal dehiscence (patients with 2 well established historical risk factors), and we closed the sternum of the patients with a new surgical option consisting of passing, in a conventional six-wire sternal closure, the last sixth steel wire (Ø 1.0 mm, n° 5) through a couple of titanium cannulated screws (Ø 5.5 mm, self-tap-ping with a length from 10 to 16 mm and 2-mm increments) inserted into the 5th or 6th couple of rib cartilages. RESULTS: Primary chest closure with cannulated screws was performed on 48 males and 14 females. The average age of patients was 67.6 years (range, 43-88 years). The average follow-up was 12.8±16.2 months (range, 1-41 months). Chronic obstructive pulmonary disease (COPD) was the most common preoperative risk factor. The average number of risk factors was 2.4 (range, 2-4). Coronary artery bypass grafting (CABG) was the most common cardiac procedure. There were no peri-operative deaths. BMI >30 Kg/m2 and diabetes were the associated of risk factors predisposing to superficial skin dehiscence. CONCLUSIONS: In our clinical experience with high risk patients, this surgical option suggests that there is evidence of an effective stabilization of standard sternal closure and this option seems a promising technique to prevent the domino effect which starts from the xifoid bone and proceeds to the manubrium with the effect of leading to sternal instability which is the first step to mediastinitis.

Role of different vascular approaches on transcatheter aortic valve implantation outcome: a single-center study.

OBJECTIVE: To compare different vascular approaches on clinical outcome of patients undergoing transcatheter aortic valve implantation (TAVI) with self-expandable bioprosthesis. METHODS: We included all the patients undergoing CoreValve implantation at our institute between September 2007 and March 2014. They were divided into four groups based on the vascular approach: percutaneous transfemoral (pTF), cut-down transfemoral (cTF), transaxillary (TAx) and transaortic (TAo). Clinical outcomes were evaluated according to Valve Academic Research Consortium-2 recommendations. RESULTS: Out of 322 consecutive patients, 170 (53%) underwent pTF, 76 (23%) cTF, 32 (10%) TAx and 44 (14%) TAo approach. Although the TAx and TAo patients had a higher risk profile, they had a similar outcome compared with the pTF and cTF groups; in particular, there were no differences regarding cardiovascular and all-cause mortality at 30 days, 1 and 2 years, as well as stroke, myocardial infarction, bleeding, major vascular complications, permanent pacemaker implantation and acute kidney injury rates. The observed device success rate was higher in the TAo than in the other approaches (88.6 versus 65.9, 68.7 and 76.3% in the pTF, cTF and TAx groups, respectively; P = 0.019). No differences occurred regarding 30-day early safety and 1-year clinical efficacy across the four groups. Fluoroscopy time, amount of contrast medium used and minor vascular complications were significantly higher in pTF patients, as well as in-hospital stay in the TAo group. Atrial fibrillation and prosthetic valve regurgitation, but not the vascular approach, were independent predictors of all-cause mortality. CONCLUSION: A more invasive vascular approach, for CoreValve implantation, even in higher risk patients, does not affect early-term, mid-term and long-term outcomes.

Vitamin D and endothelial vasodilation in older individuals: data from the PIVUS study.

CONTEXT: Vitamin D plays a role in a wide range of extraskeletal processes, including vascular function. Endothelial dysfunction is a predictor of cardiovascular disease, especially in older subjects. However, the relationship between vitamin D levels and indexes of endothelial vasodilation has never been fully addressed in older individuals. OBJECTIVE: The objective of this study was to examine the association between vitamin D and endothelial function in a large community-based sample of older subjects. METHODS: This cross-sectional study involved 852 community-dwelling men and women aged 70 years from the Prospective Study of the Vasculature in Uppsala Seniors (PIVUS), with complete data on vascular function and 25-hydroxyvitamin D. We evaluated endothelium-dependent vasodilation by an invasive forearm technique with acetylcholine, endothelium-independent vasodilation by sodium nitroprussiate, flow-mediated vasodilation, and the pulse wave analysis (reflectance index). Vitamin D levels were measured by chemiluminescence. We used multivariate regression models adjusted for body mass index (model 1) and for multiple confounders (high-sensitivity C-reactive protein, insulin, total cholesterol, high-density lipoprotein-cholesterol, low-density lipoprotein-cholesterol, smoking, sex hormones, season of blood collection, hypertension, diabetes, cardiovascular medications and diseases, statin usage, plasma calcium and calcium intake, PTH, physical exercise, liver and kidney function tests, albumin; model 2). RESULTS: In women, but not in men, vitamin D levels were positively associated with endothelium-independent vasodilation in both model 1 (ß ± SE = 1.41 ± 0.54; P = .001), and model 2 (ß ± SE = 2.01 ± 0.68; P = .003).We found no significant relationship between vitamin D levels and endothelium-dependent vasodilation, flow-mediated vasodilation, and reflectance index in both sexes. CONCLUSIONS: In older women, but not in men, vitamin D is positively and independently associated with EIDV.

Mitral valve repair or replacement for ischemic mitral regurgitation? The Italian Study on the Treatment of Ischemic Mitral Regurgitation (ISTIMIR).

OBJECTIVE: It is uncertain whether mitral valve replacement is really inferior to mitral valve repair for the treatment of chronic ischemic mitral regurgitation. This multicenter study aimed at providing a contribution to this issue. METHODS: Of 1006 patients with chronic ischemic mitral regurgitation and impaired left ventricular function (ejection fraction < 40%) operated on at 13 Italian institutions between 1996 and 2011, 298 (29.6%) underwent mitral valve replacement whereas 708 (70.4%) received mitral valve repair. Propensity scores were calculated by a nonparsimonious multivariable logistic regression, and 244 pairs of patients were matched successfully using calipers of width 0.2 standard deviation of the logit of the propensity scores. The postmatching median standardized difference was 0.024 (range, 0-0.037) and in none of the covariates did it exceed 10%. RESULTS: Early deaths were 3.3% (n = 8) in mitral valve repair versus 5.3% (n = 13) in mitral valve replacement (P = .32). Eight-year survival was 81.6% ± 2.8% and 79.6% ± 4.8% (P = .42), respectively. Actual freedom from all-cause reoperation and valve-related reoperation were 64.3% ± 4.3% versus 80% ± 4.1%, and 71.3% ± 3.5% versus 85.5% ± 3.9 in mitral valve repair and mitral valve replacement, respectively (P < .001). Actual freedom from all valve-related complications was 68.3% ± 3.1% versus 69.9% ± 3.3% in mitral valve repair and mitral valve replacement, respectively (P = .78). Left ventricular function did not improved significantly, and it was comparable in the 2 groups postoperatively (36.9% vs 38.5%, P = .66). At competing regression analysis, mitral valve repair was a strong predictor of reoperation (hazard ratio, 2.84; P < .001). CONCLUSIONS: Mitral valve replacement is a suitable option for patients with chronic ischemic mitral regurgitation and impaired left ventricular function. It provides better results in terms of freedom from reoperation with comparable valve-related complication rates.

Triple-orifice valve repair in severe Barlow disease with multiple-jet mitral regurgitation: report of mid-term experience.

OBJECTIVES: Barlow disease represents a surgical challenge for mitral valve repair (MR) in the presence of mitral insufficiency (MI) with multiple regurgitant jets. We hereby present our mid-term experience using a modified edge-to-edge technique to address this peculiar MI. METHODS: From March 2003 till December 2010, 25 consecutive patients (mean age 54 ± 7 years, 14 males) affected by severe Barlow disease with multiple regurgitant jets were submitted to MR. Preoperative transesophageal echo (TEE) in all the cases showed at least 2 regurgitant jets, involving one or both leaflets in more than one segment. In all the patients, a triple orifice valve (TOV) repair with annuloplasty was performed. Intra-operative TEE and postoperative transthoracic echocardiography (TTE) were carried out to evaluate results of the TOV repair. RESULTS: There was no in-hospital death and one late death (non-cardiac related). At intra-operative TEE, the three orifices showed a mean total valve area of 2.9 ± 0.1cm(2) (range 2.5-3.3 cm(2)) with no residual regurgitation (2 cases of trivial MI) and no sign of valve stenosis (mean transvalvular gradient 4.6 ± 1.5 mmHg). At follow up (mean 38 ± 22 months), TTE showed favourable MR and no recurrence of significant MI (6 cases of trivial and 1 of mild MI). Stress TTE was performed in 5 cases showing persistent effective valve function (2 cases of trivial MI at peak exercise). All the patients showed significant NYHA functional class improvement. CONCLUSIONS: This report indicates that the TOV technique is effective in correcting complex Barlow mitral valves with multiple jets. Further studies are required to confirm long-term applicability and durability in more numerous clinical cases.

Refractory spasm of coronary arteries and grafted conduits after isolated coronary artery bypass surgery.

BACKGROUND: Refractory vascular spasm (RVS) concomitantly involving the entire coronary artery system and grafted conduits after coronary artery bypass grafting (CABG) surgery is a rare, but dreadful event. No consensus exists in terms of appropriate management. METHODS: Between 1986 and 2009, 5,762 patients underwent isolated CABG at our institution, and 7 patients experienced RVS involving the coronary arteries and implanted conduits. Mean age was 65.6 years and 3 were female. All patients received from 3 to 5 distal anastomoses, including use of the left internal mammary artery. During the same time period, 18 patients experienced perioperative vasospasm of a single coronary artery or of a grafted conduit. RESULTS: All diffuse RVS events occurred between 3 and 8 hours after surgery. All patients had diffuse ischemic-like electrocardiographic changes, and 5 patients rapidly developed cardiogenic shock in the intensive care unit. Angiography was quickly performed in all patients and showed diffuse RVS involving either the native coronary arteries or the anastomosed arterial and venous conduits. The first 5 patients of this series died in the catheterization lab due to rapidly evolving refractory cardiogenic shock and unresponsive cardiac arrest, despite intraaortic counterpulsation and aggressive pharmacologic interventions (selective vasodilators and systemic inotropes). In the last 2 patients, extracorporeal membrane oxygenation was quickly instituted (1 in the catheterization lab, 1 in the operating room) and RVS could be successfully managed with complete resolution of ongoing vasospasm. In the single vascular spasm, there was only 1 death for refractory cardiac arrest, whereas all the other patients were successfully treated with direct infusion of vasodilators. CONCLUSIONS: Diffuse RVS after CABG is a rare but lethal condition. Our experience, although limited, indicates that in such cases an aggressive treatment, that is, prompt extracorporeal membrane oxygenation institution and controlled cardiocirculatory assistance, represents the preferred solution to face such a dramatic event and may save patient lives.

Type 2 diabetes mellitus is associated with faster degeneration of bioprosthetic valve: results from a propensity score-matched Italian multicenter study.

BACKGROUND: The present study was aimed at determining the impact of type 2 diabetes mellitus (DM) on postoperative bioprosthetic structural valve degeneration. METHODS AND RESULTS: Twelve Italian centers participated in the study. Patient data refer to bioprosthetic implantations performed from November 1988 to December 2009, which resulted in 6184 patients (mean age 71.3±5.4 years, 60.1% male) being enrolled. Of these patients, 1731 (27.9%) had type 2 DM. The propensity score-matching algorithm successfully matched 1113 patients with type 2 DM with the same number of no-DM patients. The postmatching standard differences were less than 0.1 for each of the covariates, and 64.2% of DM patients were matched. The early (30 days) mortality rate was 7.8% (n=87) versus 2.9% (n=33) in patients with or without type 2 DM (P<0.001), respectively. Seven-year freedom from valve deterioration was significantly lower in patients with DM (73.2% [95% confidence interval, 61.6-85.5] versus 95.4% [95% confidence interval, 83.9-100], P<0.001). In Cox regression models with robust SEs that accounted for the clustering of matched pairs, DM was the strongest predictor of structural valve degeneration (hazard ratio 2.39 [95% confidence interval 2.28-3.52]). When we allowed for interaction between type 2 DM and other key risk factors, DM remained a significant predictor beyond any potentially associated variable. CONCLUSIONS: Patients with type 2 DM undergoing bioprosthetic valve implantation are at high risk of early and long-term mortality, as well as of structural valve degeneration.

Human fibrinogen/thrombin-coated collagen patch to control intraoperative severe pulmonary hemorrhage and air leakage after correction of a ruptured thoracic aortic aneurysm.

Lung erosion represents a dreadful complication in patients affected by thoracic aortic aneurysms. Intraoperative occurrence of severe bleeding and extensive air leakage from the pulmonary erosion is usually quite particularly dangerous and challenging because of fragile lung tissue to be repaired, hypo-coagulative state, and the need for full mechanical ventilation. We report the case of a patient who had uncontrollable pulmonary bleeding and marked air leakage from an aortic aneurysm-induced lung erosion and laceration, which were effectively treated with a hemostatic patch (Tachosil, Nycomed, Linz, Austria) after unsuccessful conventional surgical approaches.

Assessment of a continuous blood gas monitoring system in animals during circulatory stress.

BACKGROUND: The study was aimed to determine the measurement accuracy of The CDI™ blood parameter monitoring system 500 (Terumo Cardiovascular Systems Corporation, Ann Arbor MI) in the real-time continuous measurement of arterial blood gases under different cardiocirculatory stress conditions METHODS: Inotropic stimulation (Dobutamine 2.5 and 5 µg/kg/min), vasoconstriction (Arginine-vasopressin 4, 8 and 16 IU/h), hemorrhage (-10%, -20%, -35%, and -50% of the theoretical volemia), and volume resuscitation were induced in ten swine (57.4 ± 10.7 Kg).Intermittent blood gas assessments were carried out using a routine gas analyzer at any experimental phase and compared with values obtained at the same time settings during continuous monitoring with CDI™ 500 system. The Bland-Altman analysis was employed. RESULTS: Bias and precision for pO2 were - 0.06 kPa and 0.22 kPa, respectively (r2 = 0.96); pCO2 - 0.02 kPa and 0.15 kPa, respectively; pH -0.001 and 0.01 units, respectively ( r2 = 0.96). The analysis showed very good agreement for SO2 (bias 0.04,precision 0.33, r2 = 0.95), Base excess (bias 0.04,precision 0.28, r2 = 0.98), HCO3 (bias 0.05,precision 0.62, r2 = 0.92),hemoglobin (bias 0.02,precision 0.23, r2 = 0.96) and K+ (bias 0.02, precision 0.27, r2 = 0.93). The sensor was reliable throughout the experiment during hemodynamic variations. CONCLUSIONS: Continuous blood gas analysis with the CDI™ 500 system was reliable and it might represent a new useful tool to accurately and timely monitor gas exchange in critically ill patients. Nonetheless, our findings need to be confirmed by larger studies to prove its reliability in the clinical setting.

Left ventricular pseudoaneurysm and α1-antitrypsin enzyme deficiency: another pathological correlation.

Alpha1-antitrypsin deficiency (A1ATD) is usually linked to chronic lung dysfunction and chronic liver disease. Recent evidence have shown involvement also of the cardiovascular system. Indeed, dissection of the thoracic aorta or of the coronary arteries, and intra-cerebral aneurysms has been shown to represent concomitant vascular adverse events and apparently related to the impaired vascular wall integrity, strictly related to the elastase levels which are usually modulated by the A1AT. The case herein reported describes a patient affected by A1ATD associated with a large pseudoaneurysm of the left ventricle without any known predisposing factors to pseudoaneurysm development (coronary artery disease, acute myocardial infarction, taruma and myocarditis) indicating that the connective tissue impairment documented in A1ATD may also affect the myocardial structure, ultimately leading to weakened wall integrity, sudden rupture and pseudoaneurysm formation successfully managed by a surgical approach.

Does preoperative tethering symmetry affect left ventricular reverse remodeling after restrictive annuloplasty?

BACKGROUND: We hypothesized that a preoperative symmetric pattern with anterior mitral leaflet (AML) tethering predominance is related to lack of LVRR after restrictive annuloplasty. METHODS: In 300 patients with surgical annuloplasty for chronic ischemic mitral regurgitation the AML and posterior mitral leaflet (PML) tethering angles were quantified and patients were divided on the basis of the preoperative anterior/posterior tethering angle ratio: there were 144 patients with symmetric (Group 1) and 156 with asymmetric (Group 2) preoperative tethering pattern patients underwent echocardiography preoperatively, at discharge and at follow-up appointments (6 months [IQR 5-8 months]; late, 48 months [15-63 months]). Reverse remodeling was defined as a reduction in left ventricular end systolic volume index >15%. RESULTS: LVRR was higher in the asymmetric group at discharge (69.2% vs. 9.7%, p<0.001), early (70.55% vs. 10.45%, p<0.001 and late follow up (81.4% vs. 4.8%, p<0.001). At multivariable regression analysis corrected by significant key factors of LVRR, symmetric leaflet tethering (OR, 4.8 [95% CI 2.9-5.6], p<0.001), anterior tethering angle alpha'<39.5 degrees (OR, 5.0 [95% CI 2.0-6.6], p<0.001), coaptation height<11 mm (OR, 2.5 [95% CI 1.1-3.3], p=0.006) and coaptation length > or =8 mm at the end of procedure (OR, 2.0 [CI 0.8-3.0], p=0.01) were independent predictors of LVRR. Compared with patients with asymmetric pattern (adjusted OR 0.2 [95% CI 0.03-1.6), those with symmetric pattern had >4-fold odds for lack of LVRR. CONCLUSIONS: The preoperative symmetric pattern with AML prevalence was strongly associated with lack of reverse remodeling after annuloplasty. An accurate echocardiographic evaluation of the tethering mechanisms should be incorporated into clinical risk assessment and prediction models.

Atorvastatin attenuates post-implant tissue degeneration of cardiac prosthetic valve bovine pericardial tissue in a subcutaneous animal model.

AIMS: The aim of our study was to examine the effects of statin therapy (atorvastatin) on post-implant structural changes of bovine pericardial tissue in a subcutaneous animal model. METHODS AND RESULTS: Sixty male C57BL/6 mice underwent subcutaneous dorsal implantation of bovine pericardial fragments. Animals were randomized to treatment with atorvastatin (50 mg/kg) (statin group - SG) or to vehicle (control group - CG). After 1.5 months, all fragments were explanted and submitted to histopathological assessment (semi-quantitative analysis) to elucidate extent of inflammatory infiltrate, signs of tissue injury, or presence of microcalcification. Calcium determination of the implanted pericardial tissue was also performed by inductively coupled plasma mass spectrometry (ICP-MS) assessment. ICP-MS analysis showed that pericardial fragments in SG had significantly (p<0.01) less calcium content than CG (625+/-142 vs. 962+/-590 microg/g, respectively). Light microscopy showed marked inflammatory infiltrates and tissue injury of pericardial specimens in CG animals, whereas SG animals maintained a better preserved original pericardial structure. CONCLUSIONS: Our findings indicate that atorvastatin significantly attenuates the post-implant structural degeneration of artificial valve bovine pericardial tissue in a subcutaneous animal model. Further observations are mandatory to assess the effects of statins on the implanted bioprosthetic valve tissue in the blood circulation.

Infectious endocarditis during pregnancy, problems in the decision-making process: a case report.

Infective endocarditis in pregnancy has a low incidence, often being associated with a previous history of rheumatic or congenital heart disease. In most reports the disease tends to run a subacute course and to appear more frequently in the third trimester of pregnancy. We present the case of a 36-year-old woman with large vegetations on the mitral valve due to infective endocarditis detected at the 32(nd) week of her first pregnancy. The difficulties in selecting the appropriate management strategy, particularly optimal time and mode of delivery, optimal time and type of valve surgery, are emphasized.

Unknown ankylosing spondylitis with only cardiac involvement in patient with surgical indication: Call for patient and family members immunological screening.

Aortic regurgitation (AR) and first-degree atrioventricular heart block (FDAVB) are encountered in ankylosing spondylitis (AS). This rheumatological disease also presents in 90% of the cases an immunogenetic marker that is Human Leucocyte Antigen-B27 (HLA-B27). In this report we describe a case of a patient presenting with AR, FDAVB, aneurysm and thinning of the ascending aortic wall, aneurysm of the sinuses of Valsalva and inferior myocardial infarction-like electrocardiographic pattern with unknown cardiac AS and absence of other AS-related systemic manifestations.