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To assess the role of the mitral valve apparatus (leaflets, chordae and papillary muscles, (PM)) in left ventricle outflow tract (LVOT) obstruction, and results of the surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM).Twenty-eight consecutive patients (58±11years, 53% female) undergoing HOCM surgery from 2007 to 2016 at our institute were retrospectively reviewed. Endpoints included the involvement of the mitral valve in LVOT obstruction, mortality, and changes in clinical and echocardiographic characteristics after HOCM surgery.Secondary chordae tendineae tractioning the anterior mitral leaflet to the interventricular septum, and systolic anterior motion were detected in 78% of the patients. Anomalous, hypertrophied, and fused PM with muscularis trabeculae hypertrophy were found in 50%, 25%, and 35% of the patients, respectively. Four patients had posterior leaflet redundancy. Secondary chordae (92%), PM, and muscularis trabeculae resection (71%), and PM splitting and elongation (28%) were added variably to septal myectomy (100%). Nine procedures (32%) on mitral valve leaflets were performed, involving six posterior and three anterior mitral leaflets. Long-term follow-up was 4±2.8years. There was no hospital mortality, and NYHA was reduced from 3±0.5 to 1±0.7 (p0.0001), the LVOT gradient from 88±35 to 20±18mmHg (p0.0001), mitral valve regurgitation from grade 3±1 to 1±0.7 (p0.0001), and septum thickness from 18±3 to 14±2mm (p0.0001).The mitral valve apparatus contributes with all its components variably to LVOT dynamic obstruction thus surgical correction in addition to extended myectomy is recommended to achieve the best outcome.

Uterine intravenous leiomyomatosis is an uncommon tumor, usually arising from the uterus, with nodular masses which extend intravascularly over variable distances and may reach the inferior vena cava, right atrium, and pulmonary arteries. Early diagnosis and surgical intervention are crucial as intracardiac leiomyomatosis not only causes cardiac symptoms but may result in pulmonary embolism and sudden death. Complete tumor resection is key in disease management, thus rendering cardiac-extending uterine intravenous leiomyomatosis one of the most challenging conditions for surgical treatment. The use of interventional radiology procedures can facilitate the surgical approach. We report the case of a massive pelvic recurrence of uterine leiomyomatosis with intracardiac extension and pulmonary embolism, analyzing management and surgical outcomes, highlighting the role of interventional radiology during the therapeutic pathway. Nonetheless, there are currently very few data available concerning the use of interventional radiology procedures in the therapeutic strategy of uterine intravenous leiomyomatosis with intracardiac extension.

BackgroundIn the Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase - Stimulator Trial 1 (CHEST-1) study, riociguat improved 6-minute walking distance (6MWD) vs placebo in patients with inoperable chronic thromboembolic pulmonary hypertension or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy. In this study, the proportion of patients who achieved responder thresholds that correlate with improved outcome in patients with pulmonary arterial hypertension was determined at baseline and at the end of CHEST-1.MethodsPatients received placebo or riociguat individually adjusted up to 2.5 mg 3 times a day for 16 weeks. Response criteria were defined as follows: 6MWD increase ≥40 m, 6MWD ≥380 m, cardiac index ≥2.5 liters/min/m2, pulmonary vascular resistance

To assess differences in mitral regurgitation (MR) grade between the preoperative and the intraoperative evaluations.Systematic review and meta-analysis of 6 observational studies found from MEDLINE and EMBASE.Cardiac surgery.One hundred thirty-seven patients.Comparison between the preoperative MR assessment and the intraoperative evaluation conducted under general anesthesia (GA), with or without "hemodynamic matching" (HM) (artificial increase of afterload).The primary outcome was the difference between the preoperative and intraoperative MR grade under "GA-only" or "after-HM." Secondary analyses addressed differences according to effective regurgitant orifice area (EROA), regurgitant volume (RVol), color-jet area, and vena contracta width. Risk of MR underestimation was found under "GA-only" (SMD: 0.55; 95% confidence interval [CI], 0.31-0.79, p0.00001), but not "after-HM" (SMD: -0.16; 95% CI, -0.46 to 0.13, p = 0.27). Under "GA-only", EROA had a trend toward underestimation (p = 0.07), RVol was reliable (p = 0.17), while reliance on color-jet area and vena contracta width incur risk of underestimation (both p = 0.003). After HM, EROA accurately reflected preoperative MR (p = 0.68) while RVol had a trend toward overestimation (p = 0.05). The overall reported incidence of misdiagnoses was slightly more common under "GA-only" (mean 48%, 39% underestimation, 9% overestimation; range: 32%-57%) than "after-HM" (mean 41%, 12% underestimation, 29% overestimation; range: 33%-50%). Only the minority of misdiagnoses were clinically relevant: underestimation was around 10% (both approaches), but 18% had clinically significant overestimation "after-HM" as compared with 3% under GA-only.Intraoperative assessment under "GA-only" significantly underestimated MR. A more accurate intraoperative evaluation can be obtained with afterload manipulation, although HM strategy carries high risk of clinically significant overestimation.

Background— Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. Methods and Results— A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90–95), 91% (95% CI, 87–93), and 89% (95% CI, 86–92) in operated patients (n=404), and only 88% (95% CI, 83–91), 79% (95% CI, 74–83), and 70% (95% CI, 64–76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension–targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49–11.62; P =0.0065 and HR, 4.76; 95% CI, 1.76–12.88; P =0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95–1.90; P =0.0992 and HR, 1.50; 95% CI, 1.20–1.88; P =0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36–6.69; P =0.0065 and HR, 2.15; 95% CI, 1.18–3.94; P =0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension–targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. Conclusions— The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients.

Objective An increasing number of elderly patients are referred for pulmonary endarterectomy. The distinction between operable and inoperable lesions has been challenged over time. Hence, we developed alternative cardiopulmonary bypass management and cerebral protection strategies to obtain satisfactory surgical results according to the changing patient features. Methods From April 1994 to March 2011, 347 pulmonary endarterectomies were performed at our center. We began with the technique championed by the San Diego Group, adopting a single period of deep hypothermic circulatory arrest for each side (group A). Since 2003, we began to perform short periods of intermittent deep hypothermic circulatory arrest followed by periods of reperfusion (group B). We then adopted moderate, instead of deep, hypothermia (group C). Finally, we modified our technique further performing shorter (5–7-minute) periods of circulatory arrest (group D). Results The hemodynamic results after surgery were excellent in all 4 groups. The patients' age increased significantly. A trend toward an increase in the number of Jamieson type 3 lesions was observed. Associated with our protocol changes, we observed better postoperative respiratory function, a reduction in the length of mechanical ventilation and postoperative infections, and a remarkable improvement in uneventful postoperative courses. Despite the increased total circulatory arrest time, a trend toward a reduction in the incidence of transient neurologic events was observed, and operative mortality was not affected. Conclusions In our experience, our alternative strategy resulted in a better combination of surgical accuracy and cerebral protection and improved outcomes.

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) have poor prognosis, and pulmonary endarterectomy (PEA) is considered the treatment of choice for this condition. We report a case and review the literature of successful PEA for CTEPH due to antiphospholipid syndrome associated with systemic lupus erythematosus. The definitive and decisive approach needed to treat this high-risk patient with a history of comorbidity, long-term illness and poor compliance was found with a therapy of PEA.

OBJECTIVES Antiphospholipid (a-PL) antibodies, especially IgG isotype, have been associated with a variety of neurological manifestations related to thrombotic mechanism and reactivity against nervous tissues. Furthermore, high titre of a-PL antibodies has been also correlated to chronic thromboembolic pulmonary hypertension (CTEPH) and, therefore, is frequently reported in patients undergoing pulmonary endarterectomy (PEA). The impact of a-PL antibodies in postoperative outcome following PEA, however, has not been clearly evaluated yet. In this paper, we investigated the impact of a high a-PL IgG titre (HAPT) on postoperative outcome following PEA. METHODS From April 1994 to October 2008, out of 204 patients undergoing PEA at our centre, 184 were prospectively screened for a-PL antibodies. According to the preoperative IgG titre, patients were divided into two groups: Group A (high a-PL antibodies titre - HAPT) with a-PL IgG titre >10 U/ml and Group B (low a-PL antibodies titre - LAPT) with a-PL IgG titre

Aims Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension, but there are few data in the literature about the results of this procedure in the elderly. In this study, we aimed to assess whether this type of surgery is effective and well tolerated for the elderly. Methods A total of 264 consecutive patients who underwent PEA between January 2008 and December 2012 were reviewed. PEA was performed under cardiopulmonary bypass and hypothermic ventricular fibrillation, with the aorta left unclamped. The population was dichotomized according to age into the following two groups: below 70 years (n = 176, younger patients) and at least 70-year-olds (n = 88, elderly patients). Regression models were used to identify predictors of hospital mortality and postoperative adverse events, and their interaction with age was tested. Results Hospital mortality was slightly, but not significantly higher in elderly patients (9.1 vs. 5.1%; P = 0.22). Effect modification by history of smoking and preoperative O2 therapy was present. The cumulative survival at 1, 2, and 4 years was 93, 92, and 91% among younger patients; and 88, 86, and 86% among older patients (P = 0.19). Clinical and hemodynamic improvement was similar in the two groups. Conclusion Despite a slightly higher short-term mortality, PEA is feasible and well tolerated for the vast majority of the elderly patients. Clinical and hemodynamic improvements are outstanding, with satisfactory long-term survival rates.

This study aims to evaluate whether thepresence or discovery of a BAV under ATAA repair would impactthe decision-making of the surgical procedure choice.

within the chest. For example, patients who have undergone Fontan palliation have only 1 atrioventricular valve, and a capacitance chamber must be constructed to adequately connect the TAH-t. Virtual implantation offers the ability to test several surgical approaches for device placement before the patient even enters the operating room. This study was limited by a single-center experience in a small patient cohort. In addition, the assessment by virtual implantation was retrospective, and no 50cc TAH-t device was actually implanted to confirm the predicted results of the virtual implantation because it was not available during the study. However, two 70cc TAH-t devices were placed successfully as predicted by virtual implantation. In conclusion, virtual compatibility testing allows device consideration for fit to be individualized and represents a movement away from using generalized assumptions about heart size, chest wall anatomy, and spatial relationships of cardiothoracic structures to determine fit. Disclosure statement

Patients with chronic thromboembolic pulmonary hypertension (CTEPH), despite successful pulmonary endarterectomy (PEA), can continue to suffer from a limitation in exercise capacity. The objective of this study was to assess whether pulmonary arterial compliance is a predictor of exercise capacity after PEA. Right heart haemodynamics, treadmill incremental exercise test, spirometry, carbon monoxide transfer factor, arterial blood gas and echocardiographic examinations were retrospectively analysed in a population of CTEPH patients who underwent PEA at a single centre. Baseline and 3-month haemodynamic data were available in 296 patients; 5-year follow-up data were available in 68 patients. In a multivariable model the following parameters were found to be independent predictors of exercise capacity after surgery: age, sex, pulmonary arterial compliance, tricuspid annular plane excursion, arterial oxygen tension and carbon monoxide transfer factor (p

Extracorporeal membrane oxygenation (ECMO) has become the standard of care for temporary life support in severe but potentially reversible cardiac or respiratory failure, when conventional treatments have failed. In the past 10 years, ECMO use has grown worldwide with improving outcomes, mostly in adults with severe acute respiratory failure. Since 2009, a further significant growth has occurred after the publication of the Conventional Ventilatory Support Versus ECMO for Severe Adult Respiratory Failure (CESAR) trial, a multicenter randomized-controlled study concluding that ECMO improves survival in acute respiratory distress syndrome and is cost-effective. In addition, the recent pandemic influenza A (H1N1) contributed to the wide use of ECMO, which has proven to be effective even in this peculiar subset of critically ill patients. More recently, with the introduction of the

It is likely that chronic thromboembolic pulmonary hypertension (CTEPH) is more prevalent than currently recognised. Imaging studies are fundamental to decision making with respect to operability. All patients with suspected CTEPH should be referred to an experienced surgical centre. Currently, there is no risk scoring stratification system to guide operability assessment and it is predominantly based on surgical experience. The aim of pulmonary endarterectomy (PEA) is the removal of obstructive material to immediately reduce pulmonary vascular resistance. PEA affords the best chance of cure, but is difficult to perfect. Recognition and clearance of distal segmental and subsegmental disease is the main problem. The basic surgical techniques include: median sternotomy incision, cardiopulmonary bypass, arteriotomy incisions within pericardium, and a true endarterectomy with meticulous full distal dissection. Deep hypothermic circulatory arrest is recommended as the best means of reducing blood flow in the pulmonary artery to allow a clear field for dissection. In the recent PEACOG (PEA and COGnition) trial there was no evidence of cognitive impairment post-PEA. Reperfusion pulmonary oedema and residual pulmonary hypertension are unique post-operative complications post-PEA and are associated with increased mortality. However, in-hospital mortality is now

After transplantation, steroids and calcineurin inhibitors together with end-stage renal failure may lead to associated cardiovascular diseases, particularly in long-term survivors. We present a case of aortic valve replacement 15 years after lung transplantation, followed by reoperative valve replacement for late infective endocarditis. Lung compliance and gas exchange were excellent during recovery. Despite adequate prophylaxis, immunosuppression and hemodialysis likely contributed to repeated episodes of sepsis, which caused detachment of the first aortic prosthesis. Despite the high mortality of prosthetic valve endocarditis, the postoperative course was uneventful and the patient is doing well at 24-month follow-up.

Background— Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. Methods and Results— The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%– 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension–targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. Conclusions— Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension–targeted treatments.

There are few follow-up studies on long-term cardiopulmonary function after pulmonary endarterectomy (PEA), the operation of choice for chronic thromboembolic pulmonary hypertension (CTEPH).To prospectively evaluate long-term outcome of patients with CTEPH treated with PEA.Between 1994 and 2006, 157 patients (mean age 55 yr) were treated with PEA at Pavia University Hospital. The patients were evaluated before PEA and at 3 months (n = 132), 1 year (n = 110), 2 years (n = 86), 3 years (n = 69), and 4 years (n = 49) afterward by NYHA class, right heart hemodynamic, spirometry, carbon monoxide transfer factor (Tl(CO)), arterial blood gas, and treadmill incremental exercise test.Cumulative survival was 84%. Within 3 months, 18 patients died in-hospital and 2 had lung transplantation; during long-term follow-up, 6 died, 1 had lung transplantation, and 3 had a second PEA (2.5 events per 100 person-years). NYHA class III-IV was the most important predictor of late death, lung transplant, or PEA redo (hazard ratio, 3.94). Extraordinary improvement in NYHA class, hemodynamic, and Pa(O(2)) were achieved in the first 3 months (P0.001) and persisted during follow-up; exercise tolerance progressively increased over time (P0.001). At 4 years, although 74% of the patients were in NYHA class I and none was in class IV, 24% had pulmonary vascular resistance greater than 500 dyne.s/cm(5) or Pa(O(2)) less than 60 mm Hg; they were significantly older and were more frequently in NYHA class III-IV 3 months after surgery than the others.After PEA, long-term survival and cardiopulmonary function recovery is excellent in most patients.

Pulmonary endarterectomy (PEA) is a technically demanding operation, currently performed in few centers worldwide. A bloodless surgical field is mandatory to identify the correct arterial dissection plane, which has to be extended toward subsegmental branches with limited visibility. Vessel perforation is a rare but potentially fatal complication, leading to uncontrollable airway bleeding. A general algorithm for the approach to pulmonary hemorrhage has been proposed by the group from the University of California at San Diego, whereas alternative managements are only briefly mentioned in the literature. Extracorporeal life support unloads pulmonary circulation but invariably requires anticoagulation, which is detrimental in pulmonary hemorrhage. We describe a novel technique for the treatment of intraoperative airway bleeding during PEA.

Objectives Chronic thromboembolic pulmonary hypertension can be cured by pulmonary endarterectomy. Operability assessment remains a major concern, because there are no well-defined criteria to discriminate proximal from distal obstructions, and surgical candidacy depends mostly on the surgeon's experience. The intraoperative classification of chronic thromboembolic pulmonary hypertension describes 4 types of lesions, based on anatomy and location. We describe our recent experience with the more distal (type 3) disease. Methods More than 500 pulmonary endarterectomies were performed at Foundation I.R.C.C.S. Policlinico San Matteo (Pavia, Italy). Because of recent changes in the patient population, 331 endarterectomies performed from January 2008 to December 2013 were analyzed. Two groups of patients were identified according to the intraoperative classification: proximal (type 1 and type 2 lesions, 221 patients) and distal (type 3 lesions, 110 patients). Results The number of endarterectomies for distal chronic thromboembolic pulmonary hypertension increased significantly over time (currently ∼37%). Deep venous thrombosis was confirmed as a risk factor for proximal disease, whereas patients with distal obstruction had a higher prevalence of indwelling intravascular devices. Overall hospital mortality was 6.9%, with no difference in the 2 groups. Postoperative survival was excellent. In all patients, surgery was followed by a significant and sustained improvement in hemodynamic, echocardiographic, and functional parameters, with no difference between proximal and distal cases. Conclusions Although distal chronic thromboembolic pulmonary hypertension represents the most challenging situation, the postoperative outcomes of both proximal and distal cases are excellent. The diagnosis of inoperable chronic thromboembolic pulmonary hypertension should be achieved only in experienced centers, because many patients who have been deemed inoperable might benefit from favorable surgical outcomes.

Objective Primary pulmonary artery sarcoma is a severe and underdiagnosed disease, with the clinical and surgical approach not clearly established. Only a few individual case reports or small series on this topic have been published. The aim of the present study was to report our surgical experience in this field. Methods From March 2004 to December 2012, 13 patients underwent surgery for pulmonary artery sarcoma at our institution. In 7 patients, the sarcoma was unilateral (53.8%), and in 6 (46.2%), the tumor had already extended to both lungs. The surgical strategy evolved over the years, but the 2 techniques used were always the same: pneumonectomy in 5 patients and pulmonary endarterectomy in 8. Results Two patients died in-hospital, both in the pneumonectomy group. The median length of the intensive care unit and hospital stay was 1 day (range, 1-10) and 14 days (range, 11-17) for the pneumonectomy group and 6 days (range, 3-23) and 19 days (range, 10-32) fort the pulmonary endarterectomy group, respectively. The median survival was 26.8 months after pneumonectomy and 6.6 months after pulmonary endarterectomy. Conclusions Primary pulmonary artery sarcoma has a poor prognosis. The surgical strategy at our institution included pneumonectomy, for possible radical resection, and palliative endarterectomy, to reduce symptoms and increase the life expectancy. The correct surgical approach must be evaluated individually, according to the tumor presentation, the presence of pulmonary hypertension, and the patient's clinical condition.