Pathoanatomic Findings and Treatment During Hypertrophic Obstructive Cardiomyopathy Surgery: The Role of Mitral Valve.

BACKGROUND: 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). METHODS: 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. RESULTS: 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 (p<0.0001), the LVOT gradient from 88±35 to 20±18mmHg (p<0.0001), mitral valve regurgitation from grade 3±1 to 1±0.7 (p<0.0001), and septum thickness from 18±3 to 14±2mm (p<0.0001). CONCLUSIONS: 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.

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

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.

Mitral Regurgitation Grading in the Operating Room: A Systematic Review and Meta-analysis Comparing Preoperative and Intraoperative Assessments During Cardiac Surgery.

OBJECTIVE: To assess differences in mitral regurgitation (MR) grade between the preoperative and the intraoperative evaluations. DESIGN: Systematic review and meta-analysis of 6 observational studies found from MEDLINE and EMBASE. SETTING: Cardiac surgery. PARTICIPANTS: One hundred thirty-seven patients. INTERVENTION: 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). MEASUREMENTS AND MAIN RESULTS: 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, p < 0.00001), 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. CONCLUSIONS: 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.

Functional assessment and quality of life before and after pulmonary endoarterectomy.

PURPOSE: The study investigates Quality of Life (QOL) and correlation with functional status of patients affected by Chronic Thromboembolic Pulmonary Hypertension who undergo Pulmonary Endoarterectomy. METHODS: We investigated with an observational design (before surgery, three and twelve months afterwards) the hemodynamic data (NYHA class, mean pulmonary arterial pressure, cardiac output and pulmonary vascular resistance), the functional status (using the 6-Minute Walk Test) and the QOL, using three questionnaires: Medical Outcome Study Short Form-36 (SF-36), Minnesota Living with Heart Failure Questionnaire (MLHFQ), Saint George Respiratory Questionnaire (SGRQ). We report the results of forty-nine patients. RESULTS: After surgery there was an improvement on functional and hemodynamic parameters and on QOL. The physical domain (PCS) of SF-36 was weakly but significantly associated with all functional parameters. There was no association between functional parameters and mental domain (MCS) of SF-36 or SGRQ. The improvement in 6-Minute Walk Distance was associated with an increase in MLHFQ. CONCLUSIONS: Both QOL and submaximal exercise tolerance improve after surgery. However only the physical domains of SF-36 appear to be significantly associated to the functional data.

Pulmonary arterial compliance and exercise capacity after pulmonary endarterectomy.

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<0.0001); the model showed good discrimination (Harrell's c=0.84) and calibration (shrinkage coefficient=0.91). Poor exercise capacity at 3 months was loosely associated with higher death rate during subsequent survival (Harrell's c=0.61). In conclusion, after successful PEA, reduced pulmonary arterial compliance is an important determinant of exercise capacity in association with the age and sex of the patients, and the extent of recovery of both cardiac and respiratory function. However, exercise capacity does not explain a large proportion of the effect of surgery on subsequent survival.

Impact of systematic diabetes screening on peri-operative infections in patients undergoing cardiac surgery.

Detection of high glycated hemoglobin (A1c) is associated with worse postoperative outcomes, including predisposition to develop systemic and local infectious events. Diabetes and infectious Outcomes in Cardiac Surgery (DOCS) study is a retrospective case-control study aimed to assess in DM and non-DM cardiac surgery patients if a new screening and management model, consisting of systematic A1c evaluation followed by a specialized DM consult, could reduce perioperative infections and 30-days mortality. Effective July 2021, all patients admitted to the cardiac surgery of IRCCS ISMETT were tested for A1c. According to the new protocol, glucose values of patients with A1c ≥ 6% or with known diabetes were monitored. The diabetes team was activated to manage therapy daily until discharge or provide indications for the diagnostic-therapeutic process. Propensity score was used to match 573 patients managed according to the new protocol (the Screen+ Group) to 573 patients admitted before July 2021 and subjected to the traditional management (Screen-). Perioperative prevalence of infections from any cause, including surgical wound infections (SWI), was significantly lower in the Screen+ as compared with the Screen- matched patients (66 [11%] vs. 103 [18%] p = 0.003). No significant difference was observed in 30-day mortality. A1c analysis identified undiagnosed DM in 12% of patients without known metabolic conditions. In a population of patients undergoing cardiac surgery, systematic A1c evaluation at admission followed by specialist DM management reduces perioperative infectious complications, including SWI. Furthermore, A1c screening for patients undergoing cardiac surgery unmasks unknown DM and enhances risk stratification.

Risk factors for surgical site infection following cardiac surgery in a region endemic for multidrug resistant organisms.

OBJECTIVES: To identify risk factors for surgical site infections following cardiosurgery in an area endemic for multidrug resistant organisms. DESIGN: Single-center, historical cohort study including patients who underwent cardiosurgery during a 6-year period (2014-2020). SETTING: Joint Commission International accredited, multiorgan transplant center in Palermo, Italy. MAIN OUTCOME MEASURES: Surgical site infection was the main outcome. RESULTS: On a total of 3609 cardiosurgery patients, 184 developed surgical site infection (5.1 %). Intestinal colonization with multidrug resistant organisms was more frequent in patients with surgical site infections (69.6 % vs. 33.3 %; p < 0.001). About half of surgical site infections were caused by Gram-negative bacteria (n = 97; 52.7 %). Fifty surgical site infections were caused by multidrug resistant organisms (27.1 %), with extended-spectrum Beta-lactamase-producing Enterobacterales (n = 16; 8.7 %) and carbapenem-resistant Enterobacterales (n = 26; 14.1 %) being the predominant resistance problem. However, in only 24 of surgical site infections caused by multidrug resistant organisms (48 %), mostly carbapenem-resistant Enterobacterales (n = 22), a pathogen match between the rectal surveillance culture and surgical site infections clinical culture was demonstrated. Nevertheless, multivariate logistic regression analysis identified a rectal swab culture positive for multidrug resistant organisms as an independent risk factor for SSI (odds ratio 3.95, 95 % confidence interval 2.79-5.60). Other independent risk factors were female sex, chronic dialysis, diabetes mellitus, previous cardiosurgery, previous myocardial infarction, being overweight/obese, and longer intubation time. CONCLUSION: In an area endemic for carbapenem-resistant Enterobacterales, intestinal colonization with multidrug resistant organisms was recognized as independent risk factor for surgical site infections. IMPLICATIONS FOR CLINICAL PRACTICE: No causal relationship between colonization with resistant pathogens and subsequent infection could be demonstrated. However, from a broader epidemiological perspective, having a positive multidrug resistant organisms colonization status appeared a risk factor for surgical site infections. Therefore, strict infection control measures to prevent cross-transmission remain pivotal (e.g., nasal decolonization, hand hygiene, and skin antisepsis).

Surgical treatment of chronic thromboembolic pulmonary hypertension.

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 <5% in experienced centres.

Resolutive pulmonary endarterectomy in a non-compliant patient with systemic lupus erythematosus and antiphospholipid syndrome.

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.

Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry.

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.

Septal Thickness Does Not Impact Outcome After Hypertrophic Obstructive Cardiomyopathy Surgery (Septal Myectomy and Subvalvular Mitral Apparatus Remodeling): A 15-Years of Experience.

Background: The aim of this study was to assess the impact of septal thickness on long-term outcomes of surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) and correction of mitral subvalvular anomalies. Methods: Sixty-six consecutive patients (58 ± 12 years, 56% female) undergoing extended septal myectomy and subvalvular mitral apparatus remodeling from 2007 to 2021 were retrospectively reviewed. Patients were divided into 2 groups according to septal thickness: moderate [< 18 mm, 29 patients (44%)] and severe [≥ 18 mm, 37 patients (56%)]. End points included survival, symptom improvement, reduction of left ventricle outflow tract (LVOT) gradient, resolution of mitral regurgitation (MR), and reoperation. Results: The mean interventricular septal thickness was 19 ± 3 mm, 15.8 ± 0.8 mm in patients with moderate and 21.4 ± 3.2 mm in those with severe hypertrophy. Preoperative data, intraoperative variables, postoperative complication rates, pre-discharge echocardiographic and clinical parameters did not differ between the two study groups [except for procedures involving the posterior mitral leaflet (p = 0.033) and septal thickness after myectomy (p = 0.0001)]. Subvalvular apparatus remodeling (secondary chordae of mitral valve resection and papillary muscle and muscularis trabecula procedures including resection, splitting, and elongation) was invariably added to septal myectomy (100%). Four (6%) procedures involved the posterior mitral leaflets. Mitral valve replacement was carried out in two patients (3%, p = 0.4). Reoperation for persistent MR was necessary in one patient (1%, p = 0.4). Neither iatrogenic ventricular septal defect nor in-hospital mortality occurred. During follow-up (mean 4.8 ± 3.8 years), two deaths occurred. NYHA class was reduced from 2.9 ± 0.7 to 1.6 ± 0.6 (p < 0.0001), the LVOT gradient from 89.7 ± 34.5 to 16.3 ± 8.8 mmHg (p < 0.0001), mitral valve regurgitation grade from 2.5 ± 1 to 1.2 ± 0.5 (p < 0.0001), and septal thickness from 18.9 ± 3.7 to 13.9 ± 2.7 mm (p < 0.0001). Conclusions: Regardless of septal thickness, subvalvular apparatus remodeling with concomitant septal myectomy can provide satisfactory long-term outcomes in terms of symptom improvement, LVOT obstruction relief, and MR resolution (without mitral valve replacement in most cases) in patients with HOCM.

Long-term outcome after pulmonary endarterectomy.

RATIONALE: 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). OBJECTIVES: To prospectively evaluate long-term outcome of patients with CTEPH treated with PEA. METHODS: 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. MEASUREMENTS AND MAIN RESULTS: 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 (P < 0.001) and persisted during follow-up; exercise tolerance progressively increased over time (P < 0.001). 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. CONCLUSIONS: After PEA, long-term survival and cardiopulmonary function recovery is excellent in most patients.

Multidisciplinary approach in the management of uterine intravenous leiomyomatosis with intracardiac extension: case report and review of literature.

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.

[Surgical management of hypertrophic obstructive cardiomyopathy: the role of mitral valve]. / Trattamento chirurgico della cardiomiopatia ipertrofica ostruttiva: il ruolo della valvola mitrale.

Hypertrophic obstructive cardiomyopathy (HOCM) is the most frequently inherited cardiovascular disease (prevalence in the general population of 1/500) and is characterized by significant left ventricular hypertrophy, especially in the interventricular septum, combined with small-volume cardiac cavities. Transaortic surgical septal myectomy is the most commonly used technique to treat HOCM, and is associated with low operative morbidity and mortality and a reduction of the outflow gradients. The composite operative mortality of only 0.4% (17/3695 patients) from 5 major high-volume centers in North America highlights the role of dedicated HOCM units. The involvement of the mitral valve in the pathophysiology of HOCM has been addressed as systolic anterior motion (SAM)-related left ventricular outflow tract obstruction. Hypertrophic cardiomyopathy mitral malformations include leaflet elongation and a wide array of malformations of the papillary muscles and chordae that can be detected by transthoracic and transesophageal echocardiography and by cardiac magnetic resonance. Because they participate fundamentally in the predisposition to SAM, they have increasingly been repaired surgically.

Pulmonary endarterectomy in the elderly: safety, efficacy and risk factors.

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.

Use of responder threshold criteria to evaluate the response to treatment in the phase III CHEST-1 study.

BACKGROUND: In 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. METHODS: Patients 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/m(2), pulmonary vascular resistance <500 dyn∙sec∙cm(-5), mixed venous oxygen saturation ≥65%, World Health Organization functional class I/II, N-terminal pro-brain natriuretic peptide <1,800 pg/ml, and right atrial pressure <8 mm Hg. RESULTS: Riociguat increased the proportion of patients with 6MWD ≥380 m, World Health Organization functional class I/II, and pulmonary vascular resistance <500 dyn∙sec∙cm(-5) from 37%, 34%, and 25% at baseline to 58%, 57%, and 50% at Week 16, whereas there was little change in placebo-treated patients (6MWD ≥380 m, 43% vs 44%; World Health Organization functional class I/II, 29% vs 38%; pulmonary vascular resistance <500 dyn∙sec∙cm(-5), 27% vs 26%). Similar changes were observed for thresholds for cardiac index, mixed venous oxygen saturation, N-terminal pro-brain natriuretic peptide, and right atrial pressure. CONCLUSIONS: In this exploratory analysis, riociguat increased the proportion of patients with inoperable chronic thromboembolic pulmonary hypertension or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy achieving criteria defining a positive response to therapy.

Surgical treatment of primary pulmonary artery sarcoma.

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.

Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension.

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.

Aortic valve replacement performed twice through ministernotomy 15 years after lung transplantation.

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.