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Background: We investigated if the occurrence of preoperative right ventricular dysfunction is capable of influencing heart transplant results in terms of in-hospital mortality, incidence of primary graft dysfunction, and follow-up mortality., Methods: We retrospectively analyzed 517 patients who underwent heart transplant between January 2000 and December 2020. We defined right ventricular dysfunction (RVD), as central venous pressure (CVP) > 15 mm Hg and CVP/pulmonary capillary wedge pressure ratio > 0.63. We identified 2 subgroups in our population: 33 patients with preoperative RVD and 484 patients without RVD., Results: In-hospital mortality was 7.9%. Severe early graft failure occurred in 6.6% of patients, with 26 patients (5.1%) needing intra-aortic balloon pump and 17 patients (3.3%) needing extracorporeal membrane oxygenation support. Clinical variables that significantly influenced in-hospital mortality were age, peripheral artery disease, and bilirubin > 1.5 mg/dL, while hemodynamic variables influencing in-hospital mortality were CVP (odds ratio [OR], 1.09 [confidence interval {CI}, 1.03-1.15], P = .004], pulmonary artery systolic pressure (OR, 1.02 [CI, 1.00-1.04], P = .05), CVP/pulmonary capillary wedge pressure ratio (OR, 2.78 [CI, 1.14-6.80], P = .025), pulmonary vascular resistance (OR, 1.15 [CI, 1.01-1.32], P = .042), transpulmonary gradient (TPG) (OR, 1.11 [CI, 1.03-1.18], P = .003) , diastolic transpulmonary gradient (OR, 1.10 [CI, 1.02-1.20], P = .015], together with right ventricular dysfunction (OR, 3.56 [CI, 1.44-8.80], P = .011). On the other hand, clinical variables influencing the incidence of early graft failure were body mass index (calculated as weight in kilograms divided by height in meters squared) > 30, peripheral artery disease, bilirubin > 1.5 mg/dL, Model for End-Stage Liver Disease score excluding international normalized ratio before transplant, and preoperative extracorporeal membrane oxygenation support, while hemodynamic variables were pulmonary arterial systolic pressure (OR, 1.03 [CI, 1.00-1.05], P = .016), TPG (OR, 1.08 [1.01-1.17], P = .03), and right ventricular dysfunction (OR, 3.00 [CI, 1.07-8.40] P = .046). On the multivariable analysis, RVD and TPG were independent predictors of in-hospital mortality, while only TPG was a predictor of early graft failure. Follow-up mortality was 38.7% and was influenced by recipient age, recipient body mass index, and preoperative diabetes. Moreover, 1-, 5-, and 10-year survival of patients with preoperative RVD was significantly worse than patients without RVD (log-rank = 0.001)., Conclusions: In our population, RVD influenced both in-hospital and long-term results after heart transplant. For these reasons, it appears crucially important to optimize preoperative right ventricular function to improve these patients' outcomes., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Competing Interests: Funding Support and Author Disclosures Dr Palmerini has received personal fees from Abbott and Edwards Lifesciences outside the submitted work. Dr. Saia has received consulting fees from Abbott Vascular, Eli Lilly, AstraZeneca, and St. Jude Medical; and speaker fees from Abbott Vascular, Eli Lilly, AstraZeneca, St. Jude Medical, Terumo, Biosensors, Edwards Lifesciences, and Boston Scientific outside the submitted work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Pulmonary endarterectomy (PEA) is the gold standard therapy for chronic thromboembolic pulmonary hypertension (CTEPH). Traditionally, pulmonary vascular resistance (PVR) represents the main prognostic factor after surgery. The pulmonary artery pulsatility index (PAPi) has been proposed for the assessment of RV in advanced heart failure, but it has never been applied in CTEPH patients. The aim of the present study is to describe PAPi in patients who underwent PEA, before and after surgery, and to define its predictive impact on postoperative outcomes. Methods: We retrospectively reviewed 188 consecutive adult patients who underwent PEA, between December 2003 and December 2021. PAPi was calculated for 186 patients and reported. Patients were partitioned in two groups using median preoperative PAPi as cutoff value: Group 1 with PAPi ≤ 8.6 (n = 94) and Group 2 with PAPi > 8.6 (n = 92). The propensity-score-matched analysis identified 67 pairs: Early outcomes were compared between two groups. Results: Mean preoperative PAPi was 10.3 ± 7.2. Considering matched populations, no differences emerged in terms of postoperative hemodynamics; Group 1 demonstrated higher 90-day mortality significance (10.4% vs. 3.0%, p = 0.082); the need for mechanical circulatory support (MCS) was similar, but successful weaning was unlikely (25% vs. 85.7%, p = 0.032). Conclusions: Mean PAPi in the CTEPH population is higher than in other diseases. Low PAPi (≤8.6) seems to be associated with lower postoperative survival and successful weaning from MCS.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease with a very complex pathophysiology differing from other causes of pulmonary hypertension (PH). It is an infrequent consequence of acute pulmonary embolism that is frequently misdiagnosed. Pathogenesis has been related to coagulation abnormalities, infection or inflammation, although these disturbances can be absent in many cases. The hallmarks of CTEPH are thrombotic occlusion of pulmonary vessels, variable degree of ventricular dysfunction and secondary microvascular arteriopathy. The definition of CTEPH also includes an increase in mean pulmonary arterial pressure of more than 25 mmHg with a normal pulmonary capillary wedge of less than 15 mmHg. It is classified as World Health Organization group 4 PH, and is the only type that can be surgically cured by pulmonary endarterectomy (PEA). This operation needs to be carried out by a team with strong expertise, from the diagnostic and decisional pathway to the operation itself. However, because the disease has a very heterogeneous phenotype in terms of anatomy, degree of PH and the lack of a standard patient profile, not all cases of CTEPH can be treated by PEA. As a result, PH-directed medical therapy traditionally used for the other types of PH has been proposed and is utilized in CTEPH patients. Since 2015, we have been witnessing the rebirth of balloon pulmonary angioplasty, a technique first performed in 2001 but has since fallen out fashion due to major complications. The refinement of such techniques has allowed its safe utilization as a salvage therapy in inoperable patients. In the present keynote lecture, we will describe these therapeutic approaches and results., Competing Interests: Conflicts of Interest: The authors declare no conflicts of interest., (2022 Annals of Cardiothoracic Surgery. All rights reserved.)

Background: Pulmonary thromboendarterectomy (PTE) is the gold standard treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, the results are poorly quantified outside a few registry reports and several individual centers., Methods: A systematic review was performed searching five electronic databases assessing the outcomes for adult patients undergoing PTE for CTEPH. All articles that reported mortality data were included. Primary outcome measures were early/inpatient mortality; secondary outcomes were survival, pulmonary haemodynamics, morbidity and functional status following PTE for CTEPH. Results were pooled via a meta-analysis of proportions and meta-regression., Results: A total of 5,717 studies were identified, yielding sixty-one relevant papers. Thirty-day mortality ranged from 0.8% to 24.4%, and on meta-analysis was 8.4% [95% confidence interval (CI): 7.2-9.6%]. Mortality was noted to decrease with increasing center volume of PTE cases (P<0.01). Residual pulmonary hypertension was reported in 8.2% to 44.5% of patients., Conclusions: CTEPH is associated with acceptable short-term mortality and an improvement in pulmonary hemodynamics. With increasing volume of experience and ongoing developments over time peri-operative mortality continues to decrease., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2022 Annals of Cardiothoracic Surgery. All rights reserved.)

Background: Right ventricular dysfunction (RVD) is a major issue in patients with advanced heart failure because it precludes the implantation of left ventricular assist device, usually leaving heart transplantation (HTx) as the only available treatment option. The pulmonary artery pulsatility index (PAPi) is a hemodynamic parameter integrating information of right ventricular function and of pulmonary circulation. Our aim is to evaluate the association of preoperative RVD, hemodynamically defined as a low PAPi, with post-HTx survival., Methods and Results: Consecutive adult HTx recipient at 2 Italian transplant centers between 2000 and 2018 with available data on pre-HTx right heart catheterization were included retrospectively. RVD was defined as a value of PAPi lower than the 25th percentile of the study population. The association of RVD with the 1-year post-HTx mortality and other secondary end points were evaluated. Multivariate logistic regression was used to adjust for clinical and hemodynamic variables. Analyses stratified by pulmonary vascular resistance (PVR) status (≥3 Woods units vs <3 Woods units) were also performed. Among 657 HTx recipients (female 31.1%, age 53 ± 11 years), patients with pre-HTx RVD (PAPi of <1.68) had significantly lower 1-year survival rates (77.8% vs 87.1%, P = .005), also after adjusting for estimated glomerular filtration rate, total bilirubin, PVR, serum sodium, inotropes, and mechanical circulatory support at HTx (hazard ratio 2.0, 95% confidence interval, 1.3-3.1). RVD was also associated with post-HTx renal replacement therapy (hazard ratio 2.0, 95% confidence interval 1.05-3.30) and primary graft dysfunction (hazard ratio 1.7, , 95% confidence interval 1.02-3.30). When stratifying patients by estimated PVR status, RVD was associated with worse 1-year survival among patients with normal PVR (76.9% vs 88.3%, P = .003), but not in those with increased PVR (78.6% vs 83.2%, P = .49)., Conclusions: Preoperative RVD, evaluated through PAPi, is associated with mortality and morbidity after HTx, providing incremental prognostic value over traditional clinical and hemodynamic parameters., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Mechanical circulatory supports with left ventricular assist devices (LVAD) are nowadays an established treatment in end-stage heart failure for those patients who are waiting for an organ donation or are unsuitable for transplantation. The duration of LVAD support is variable, depending on the device, the intention to treat and the issues occurring during treatment, which can change the purpose treatment or accelerate the transplantation. Moreover, length of reported supports in the literature is heterogenous. In here, we present the clinical and surgical case of the longest LVAD support reported in the literature, as a bridge to transplantation, with axial pump Jarvik 2000 (Jarvik Heart, Inc, New York, NY)., (© 2021. The Japanese Society for Artificial Organs.)

Background: Balloon pulmonary angioplasty (BPA) represents a therapeutic option for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in patients who are not eligible for surgical pulmonary endarterectomy (PEA) or with persistent/recurrent symptomatic pulmonary arterial hypertension after PEA. This study evaluated the safety of BPA during 5 years of experience of the only Italian center systematically performing this procedure., Methods: The BPA program was activated at the S. Orsola Polyclinic in Bologna in June 2015. Life-threatening periprocedural complications were defined as: death <30 days, need for cardiopulmonary support, hemoptysis with the need for endotracheal intubation. Serious complications were vascular complications requiring surgical or percutaneous intervention. Other endpoints of interest were: hemoptysis, pulmonary vascular damage with or without hemoptysis, and pulmonary reperfusion injury with high-resolution computed tomography lung scan at 24 h., Results: From June 2015 to September 2020, 50 patients (45% male, median age 68 years), 42 inoperable and 8 with persistent/recurrent pulmonary hypertension after PEA, underwent 156 BPA procedures at our institution. There was one life-threatening complication (2% of patients, 0.06% of the procedures), i.e. severe hemoptysis requiring endotracheal intubation, and four serious complications (8% of the patients, 2.6% of the procedures), i.e. one pulmonary artery perforation requiring percutaneous treatment and three access-site vascular complications requiring surgery. There were no deaths <30 days. Pulmonary reperfusion injury occurred in 37 patients (74%) for a total of 96 sessions (62%). However, reperfusion injury was limited and with subclinical course in most cases., Conclusions: This study confirmed the relative safety of BPA in patients with CTEPH who are not candidates for heart surgery or with persistent pulmonary hypertension after PEA in the first large Italian experience.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex disease where organized pulmonary thrombi and progressive vascular remodeling of the pulmonary arterial tree act synergistically to increase pulmonary vascular resistance and cause pulmonary hypertension. Balloon pulmonary angioplasty (BPA) has gained a renewed interest for the treatment of patients with CTEPH who are not undergoing surgery with pulmonary endarterectomy (PEA) or with persistent/recurrent pulmonary hypertension after PEA and has shown promising results in several observational studies conducted to date. We describe the case of a 42-year-old man with inoperable CTEPH in NYHA functional class III who normalized functional capacity, hemodynamic profile and main hemodynamic parameters after three BPA sessions.

Along with the worldwide increase in continuous left ventricular assist device (LVAD) strategy adoption, more and more patients with demanding anatomical and clinical features are currently referred to heart failure (HF) departments for treatment. Thus surgeons have to deal, technically, with re-entry due to previous cardiac surgery procedures, porcelain aorta, peripheral vascular arterial disease, concomitant valvular or septal disease, biventricular failure. New surgical techniques and surgical tools have been developed to offer acceptable postoperative outcomes to all mechanical circulatory support recipients. Several less invasive and/or thoracotomic approaches for surgery combined with various LVAD inflow and outflow graft alternative anastomotic sites for system placement have been reported and described to solve complex clinical scenarios. Surgical techniques have been upgraded with further technical tips to preserve the native anatomy in case of re-entry for heart transplantation, myocardial recovery or device explant. The current continuous-flow miniaturized and intrapericardial devices provide versatility and technical advantages. However, the surgical planning requires a careful multidisciplinary evaluation which must be driven by a dedicated and well-trained Heart Failure team. Biventricular assist device (BVAD) implantation by adoption of the newer radial pumps might be a challenge. However, the results are encouraging thus remaining a valid option. This paper reviews and summarizes LVAD preoperative assessment and current surgical techniques for implantation., Competing Interests: Conflicts of Interest: The authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-325). The series “Heart Failure in the Young and Old: Insights into Various Therapies” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: The occurrence of early graft failure (EGF) after heart transplantation (Htx) often requires a mechanical circulatory support (MCS) therapy. The aims of our study were to identify risk factors of mechanically supported severe EGF and evaluate their impact on both early and late outcomes., Methods: Between January 2000 and December 2019, 499 consecutive adult patients underwent Htx at our institution. Severe EGF was defined as the need for extracorporeal life support (ECLS) within 24 hours after surgery. All available recipient and donor variables were retrospectively analyzed., Results: Overall, EGF occurred in 58 (11.6%) patients. Post-Htx peripheral or central ECLS was necessary in 32 (6.4%) cases. Independent predictors of severe EGF were, in the recipient group, preoperative transpulmonary gradient (TPG) >12 mm Hg (odds ratio [OR] 4.1, P = .013), preoperative inotropic score >10 (OR 7.3, P = .0001), and pre-Htx ECLS support (OR 5.2, P = .015), while in the donors, a Eurotransplant donor score ≥17 (OR 8.5, P = .005). The absence of EGF was related with a better survival at 1 year and 5 years (94% and 85%, respectively) compared with EGF requiring ECLS population (36% and 28% at 1 year and 5 years, respectively; P < .001). A five-year conditional survival rate did not differ significantly (85% no EGF vs 83% EGF requiring ECLS)., Conclusion: Both donor and recipient factors may influence EGF occurrence. Post-Htx ECLS may impact negatively early; however, patients weaned from ECLS eventually benefit from such a rescue treatment with outcomes comparable with Htx patients who did not suffer EGF., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: A heart transplant (Htx) remains the gold standard treatment for patients with advanced heart failure. Considering the limited availability of organs, donor risk scores might improve organ selection and allocation. The objective of the study was to compare United Network for Organ Sharing, RADIAL and Eurotransplant scoring models in calculating post-Htx outcomes in an Italian Htx population., Methods: Between January 2000 and December 2017, a total of 461 adult patients underwent Htxs. United Network for Organ Sharing, RADIAL and Eurotransplant scores were calculated. Clinical features and donor risk scores were tested to identify preoperative, intraoperative and postoperative risk variables and eventually validate the scores on our population., Results: Early graft failure was detected in 16.1% (74/461). Post-Htx extracorporeal life support was used in 11.1% (51/461). Of the donor-related factors, the use of noradrenaline (P = 0.015) negatively influenced early outcomes, whereas an ischaemic time >240 min (P = 0.037) influenced early graft failure occurrence. The Eurotransplant donor score did not impact outcomes; the RADIAL score significantly influenced both early and late mortality; and the United Network for Organ Sharing score influenced only late mortality. On the multivariable analysis, after adjustment of scores per cohort, noradrenaline infusion was the main independent predictor of in-hospital mortality for the donors, whereas age of the recipient [odds ratio (OR) 1.003, 1.003-1.081; P = 0.032] and use of preoperative extracorporeal membrane oxygenation (OR 3.320, 1.124-9.805; P = 0.030) were the main independent predictors for the recipients., Conclusions: None of the validated donor scoring systems fully behave as reliable predictors of transplant outcomes. According to our 'local only' graft selection, specific donor and recipient risk variables should be monitored in order to predict early and late outcomes satisfactorily., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

An anomalous origin of the left circumflex coronary artery that arises as a side branch of the right coronary artery and encircles the aortic annulus is usually an incidental finding. However, in patients undergoing aortic valve/root procedures, its existence can significantly complicate the surgical treatment. We report our operative strategy with three different prostheses without valve downsizing., (© 2020 Wiley Periodicals LLC.)

In this study, We report on the use of fluorine 18-fluorodeoxyglucose (F-FDG) positron emission tomography (PET) scan examinations for detecting potential COVID-19 respiratory syndrome in asymptomatic left ventricular assist device (LVAD) recipients. Thus, extreme caution and thoughtful approaches should be taken for a timely detection in delicate LVAD populations, especially if patients are living in a high-density COVID-19-infected area, and the potential intention for LVAD treatment is bridge to transplantation.

Postoperative rehabilitation is a cornerstone of the recovery pathway following left ventricular assist device implantation (LVAD), and patients are expected to conduct an autonomous life thanks to improved technology and increased knowledge of mechanical circulatory support. The primary purpose of the present study was to quantify clinical changes related to rehabilitation, in patients with LVAD: functional capacity, disability, and quality of life were identified as reliable outcomes to detect such changes. The current study was a scoping review conducted searching three primary databases, namely PubMed, Scopus, and Cochrane Library, from their inception until January 2020. After the selection process was completed, 12 citations were included in the present study. Three hundred eight three patients were included in the current analysis. Functional capacity, disability, and quality of life were investigated in 157, 215, 18 patients, respectively. Significant differences were found before and after rehabilitation. The mean walked distance at 6-Minute Walk Test improved from 319±96 to 412.8±86.2 metres (p<0.001), the mean score of the Functional Independence Measure from 68.4±11.8 to 92.5±10.8 points (p<0.001), the mean score of the Short Form-36 physical component from 32.7±29.9 to 55.5±24.7 points (p=0.009) and the mental component from 55.8±19.8 to 75.4±21.4 points (p=0.002). Postoperative rehabilitation is effective at improving functional capacity, disability, and quality of life in patients with left ventricular assist device; all these three domains are particularly expressive of the entity of patients' functional recovery.

Peripheral extracorporeal membrane oxygenation (ECMO) setting remains a valid option to treat cardiogenic shock (CS). We investigated a percutaneous approach to unload the left ventricle (LV) while on veno-arterial (v-a) peripheral ECMO support. Between 2017 and 2018, eight patients (three females, mean age: 49.6 years old, and five males, mean age: 58 years old, respectively) suffered refractory CS due to acute myocardial infarction (n = 4), acute myocarditis (n = 2), acute decompensation on chronic heart failure (n = 1), and primary graft failure after heart transplantation (Htx) (n = 1), respectively. After a multidisciplinary CS team discussion, it was decided to proceed with peripheral v-a ECMO placement and percutaneous LV venting via right internal jugular vein access to drain the pulmonary artery (PA), in the hybrid operating room. In a single postcardiotomy case, the PA trunk was vented centrally. Mean ECMO support time was 8.5 days. Seven (87.5%) patients were successfully weaned from ECMO and one (12.5%) successfully bridged to Htx. All patients were successfully discharged after treatment except for a single case who died due to sepsis. In case of not recommended usage of LV apical venting, the adoption of v-a peripheral ECMO support associated with percutaneous PA drainage enables the rapid onset of extracorporeal life support with an effective biventricular unloading.

Objective: To investigate differences in cardiac manifestations of patients affected by laminopathy, according to the presence or absence of neuromuscular involvement at presentation., Methods: We prospectively analyzed 40 consecutive patients with a diagnosis of laminopathy followed at a single centre between 1998 and 2017. Additionally, reports of clinical evaluations and tests prior to referral at our centre were retrospectively evaluated., Results: Clinical onset was cardiac in 26 cases and neuromuscular in 14. Patients with neuromuscular presentation experienced first symptoms earlier in life (11 vs 39 years; p <  0.0001) and developed atrial fibrillation/flutter (AF) and required pacemaker implantation at a younger age (28 vs 41 years [p = 0.013] and 30 vs 44 years [p = 0.086] respectively), despite a similar overall prevalence of AF (57% vs 65%; p = 0.735) and atrio-ventricular (A-V) block (50% vs 65%; p = 0.500). Those with a neuromuscular presentation developed a cardiomyopathy less frequently (43% vs 73%; p = 0.089) and had a lower rate of sustained ventricular tachyarrhythmias (7% vs 23%; p = 0.387). In patients with neuromuscular onset rhythm disturbances occurred usually before evidence of cardiomyopathy. Despite these differences, the need for heart transplantation and median age at intervention were similar in the two groups (29% vs 23% [p = 0.717] and 43 vs 46 years [p = 0.593] respectively)., Conclusions: In patients with laminopathy, the type of disease onset was a marker for a different natural history. Specifically, patients with neuromuscular presentation had an earlier cardiac involvement, characterized by a linear and progressive evolution from rhythm disorders (AF and/or A-V block) to cardiomyopathy.

Background: Although imaging techniques have demonstrated the existence of microvascular abnormalities in hypertrophic cardiomyopathy (HCM), a detailed histopathological assessment is lacking as well as a comparison between different phases of the disease. We aimed to compare microvasculopathy and myocardial fibrosis in hypertrophic obstructive cardiomyopathy (HOCM) versus end-stage (ES) HCM., Methods: 27 myectomy specimens of HOCM patients and 30 ES-HCM explanted hearts were analyzed. Myocardial fibrosis was quantitatively determined with dedicated software and qualitatively classified as scar-like or interstitial. Intramural coronary arteries were evaluated separately according to lumen diameter: 100-500 μ versus <100 μ. Microvasculopathy assessment included the description of medial and intimal abnormalities and stenosis grading. The two subgroups were compared considering only the anterobasal septum of ES explanted hearts., Results: Median value of fibrosis in the anterobasal septum of explanted hearts was 34.6% as opposed to 10.3% of myectomy specimens (p < 0.001). Scar-like fibrosis was widely found in ES hearts while interstitial fibrosis was distinctive of HOCM (p < 0.001). All slides showed 100-500 μ microvasculopathy without any differences between subgroups in terms of lumen narrowing, extent of the disease and type of parietal involvement. Among ES hearts these lesions were associated with scar-like fibrosis (p = 0.034). <100-μ microvasculopathy was also frequent with no differences between subgroups., Conclusions: Microvasculopathy is an intrinsic feature of HCM with similar characteristics across the natural phases of the disease. Conversely, myocardial fibrosis changes over time with ES hearts showing a three-fold greater amount, mainly scar-like. ES showed a closer association between microvasculopathy and replacement fibrosis., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)

Left ventricular assist device graft protection and its intraoperative orientation continues to be a major concern in bridge-to-transplant strategy. Different techniques have been described, including the adoption of a standard full sternotomy approach. We describe our institutional experience of placement of the with HeartWare HVAD® implantable continuous flow pump, with outflow graft tunnelling through the transverse sinus to prepare patients in need of eventual re-sternotomy. Surgical tips are provided in the tutorial videos both for HVAD® placement, and for explantation at the time of heart transplantation., (© The Author 2016. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Pulmonary endarterectomy (PEA) is a surgical intervention reserved for patients with chronic thromboembolic pulmonary hypertension (CTEPH). In some cases, temporary circulatory support [extracorporeal life support (ECLS)] is required after PEA. Rates of ECLS requirement varies between centers. Reasons for institution of ECLS include respiratory failure, cardiac failure (or both respiratory and cardiac failure), bleeding, and reperfusion edema. This article reviews the experience of ECLS after PEA from the current literature, as well as our own institution's experience as a CTEPH multidisciplinary center., Methods: A literature review was conducted along with a retrospective chart review from 15 years of our PEA program., Results: The literature demonstrates many different approaches are used for mechanically supporting patients who develop complications after PEA. Variations in approach stem from differing indications such as, respiratory failure rather than hemodynamic compromise (or vice versa), time of implantation (immediately in operating room or delayed after surgery) and many other causes. In our center, 12.3% (19/154) of patients need ECLS with extracorporeal membrane oxygenator (ECMO) after PEA procedure. Implantation was mainly in the operating room before or immediately after weaning from cardiopulmonary bypass and mostly peripheral cannulation was used. ECMO lasted an average of 11±8 days. And 52.6% (10 of 19 patients) of patients were weaned from ECLS and of this, 70% (7 of 10 patients) were discharged., Conclusions: In some cases of PEA, ECLS is needed post-operatively. Expert teams should consider this possibility pre-operatively based on predisposing characteristics. The need for ECMO shouldn't be "di per se" a contraindication to surgery but might be considered in the surgical risk estimation. The ideal setup is not fixed and depends on the center's practices as well as indication. Even though complications do occur with ECMO, in general, results are good, being a bridge to further recovery of pulmonary hypertension (PH) or also to transplantation., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Background: Primary graft dysfunction (P-GD) is the leading cause of early mortality after heart transplantation (HT). In this 2-center study we analyze outcomes and risk factors of P-GD according to the recent consensus conference classification endorsed by International Society for Heart and Lung Transplantation., Methods: We included all adult HTs performed between 1999 and 2013. P-GD was graded as mild, moderate, and severe, according to International Society for Heart and Lung Transplantation recommendations, and analyzed separately from secondary GD. The primary end point was the combined occurrence of in-hospital death or emergency retransplantation., Results: Early GD was found in 118 of 518 patients (23%), and 72 (13.9%) met the criteria for P-GD. Of these, 4 (5%) were mild, 33 (46%) moderate, and 35 (49%) severe and mostly characterized by biventricular involvement (78%). The end point occurred in 53 patients (10.2%). Overall, GD was a strong predictor of death-graft loss (odds ratio, 15.9; 95% confidence interval, 7.9-33.5; p < 0.01), with non-significant worse outcomes in P-GD (37%) vs secondary GD (27%) patients (p = 0.2). The study end point was more frequent in severe P-GD patients (65%) than in moderate (12%) or mild (0%; p < 0.01). Several known risk factors influenced the risk for P-GD, and the combination of specific donor and recipient risk factors accounted for approximately 22-times increased odds for P-GD. Donor age, recipient diabetes, ischemic time, and post-operative dialysis predicted non-recovery from P-GD., Conclusions: Consensus-defined P-GD identifies patients at major risk for early death and graft loss after HT, although the "mild" grade appeared under-represented and clinically irrelevant. The amplified negative effect of donor and recipient factors on P-GD risk underscores the need for appropriate donor-recipient match., (Copyright © 2017 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Early graft failure (EGF) is a major risk factor for death after heart transplantation (Htx). We investigated the predictive risk factors for moderate-to-severe EGF requiring an intra-aortic balloon pump (IABP) or extracorporeal membrane oxygenation (ECMO) circulatory support as treatment after Htx. Between January 2000 and December 2014, 412 consecutive adult patients underwent isolated Htx at our institution. Moderate and severe EGF were defined as the need for IABP and ECMO support, respectively, within 24 h after Htx. All available recipient and donor variables were analyzed to assess the risk of EGF occurrence. Overall, moderate-to-severe EGF occurred in 46 (11.1%) patients. Twenty-nine (63.04%) patients required peripheral or central ECMO support in the treatment of severe EGF and 17 (36.9%) patients required IABP support for the treatment of moderate EGF. The predictive risk factors for moderate-to-severe EGF in recipients, as assessed by logistic regression analysis, were a preoperative transpulmonary gradient > 12 mm Hg (odds ratio [OR] 5.2; P = 0.023), a preoperative inotropic score > 10 (OR 8.5; P = 0.0001), and preoperative ECMO support (OR 4.2; P = 0.012). For donors, the predictive risk factor was a donor score ≥ 17 (OR 8.3; P = 0.006). The absence of EGF was correlated with improved long-term survival: 94% at 1 year and 81% at 5 years without EGF versus 76% and 36% at 1 year (P < 0.001), and 70% and 28% at 5 years (P < 0.001) with EGF requiring IABP and ECMO support, respectively. In-hospital weaned and survived patients after IABP or ECMO treatment for moderate-to-severe EGF had a similar 5-year conditional survival rate as transplant patients who had not suffered EGF: 88% without EGF versus 84% with EGF treated with mechanical circulatory support devices (P = 0.08). The occurrence of EGF is a multifactorial deleterious event that depends on donor and recipient profiles. IABP and ECMO support are reliable treatment strategies, depending on the grade of EGF. Furthermore, surviving patients treated with IABP or ECMO have the same long-term conditional survival rate as patients who have not suffered EGF., (© 2016 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Background: RotaFlow and Levitronix CentriMag veno-arterial extracorporeal membrane oxygenation (ECMO) support systems have been investigated as treatment for refractory cardiogenic shock (CS)., Methods: Between 2004 and 2012, 119 consecutive adult patients were supported on RotaFlow (n = 104) or CentriMag (n = 15) ECMO at our institution (79 men; age 57.3 ± 12.5 years, range:19-78 years). Indications for support were: failure to wean from cardiopulmonary bypass in the setting of postcardiotomy (n = 47) and primary graft failure (n = 26); post-acute myocardial infarction CS (n = 11); acute myocarditis (n = 3); and CS on chronic heart failure (n = 32)., Results: A central ECMO setting was established in 64 (53.7%) patients while peripherally in 55 (46.2%). Overall mean support time was 10.9 ± 8.7 days (range:1-43 days). Forty-two (35.2%) patients died on ECMO. Overall success rate, in terms of survival on ECMO (n = 77), weaning from mechanical support (n = 51; 42.8%) and bridge to heart transplantation (n = 26; 21.8%), was 64.7%. Sixty-eight (57.1%) patients were successfully discharged. Stepwise logistic regression identified blood lactate level and CK-MB relative index at 72 h after ECMO initiation, and number of packed red blood cells (PRBCs) transfused on ECMO as significant predictors of mortality (p = 0.011, odds ratio [OR] = 2.48; 95% confidence interval [CI] = 1.11-3.12; p = 0.012, OR = 2.81, 95% CI = 1.02-2.53; and p = 0.012, OR = 1.94; 95% CI = 1.02-5.21; respectively). Central ECMO population had a higher rate of continuous veno-venous hemofiltration (CVVH) need and bleeding events when compared with the peripheral setting., Conclusions: Patients with a poor hemodynamic status may benefit by rapid insertion of veno-arterial ECMO. The blood lactate level, CK-MB relative index and PRBCs transfused should be strictly monitored during ECMO support., (© 2014 Wiley Periodicals, Inc.)

Background: The RotaFlow (Maquet, Jostra Medizintechnik AG, Hirrlingen, Germany) and Levitronix CentriMag (Levitronix LCC, Waltham, MA, USA) veno-arterial extracorporeal membrane oxygenation (ECMO) support systems have been investigated as treatment for refractory cardiogenic shock., Methods: Between 2004 and 2012, 119 consecutive adult patients were supported on RotaFlow (n=104) or CentriMag (n=15) ECMO at our Institution (79 men; mean age 57.3 ± 12.5 years, range 19-78 years). Indications for support were: failure to wean from cardiopulmonary bypass in the setting of postcardiotomy (n=47) and primary graft failure (n=26); post-acute myocardial infarction cardiogenic shock (n=11); acute myocarditis (n=3), and cardiogenic shock on chronic heart failure (n=32)., Results: A central ECMO setting was established in 64 (53.7%) patients while peripherally in 55 (46.2%). Overall mean support time was 10.9 ± 8.7 days (range 1-43 days). Forty-two (35.2%) patients died on ECMO. Overall success rate, in terms of survival on ECMO (n=77), weaning from mechanical support (n=51; 42.8%) and bridge to heart transplantation (n=26; 21.8%), was 64.7%. Sixty-eight (57.1%) patients were successfully discharged. Stepwise logistic regression identified blood lactate levels and creatine kinase-MB relative index at 72h after ECMO initiation, and number of packed red blood cells (PRBCs) transfused on ECMO as significant predictors of mortality. Central ECMO population had a higher rate of continuous veno-venous hemofiltration need and bleeding events compared with the peripheral setting., Conclusions: ECMO support provides encouraging results in different subsets of patients in cardiogenic shock. Blood lactate levels, creatine kinase-MB relative index and PRBCs transfused should be strictly monitored during veno-arterial ECMO running. Type of ECMO implantation, if peripheral or central, should be decided according to the specific patient subset.

Two centrifugal pumps, the RotaFlow (Maquet, Jostra Medizintechnik AG, Hirrlingen, Germany) and Levitronix CentriMag (Levitronix LCC, Waltham, MA, USA), used in central or peripheral veno-arterial extracorporeal membrane oxygenation (ECMO) support systems have been investigated, in terms of double-center experience, as treatment for patients with refractory cardiogenic shock (CS). Between January 2006 and December 2012, 228 consecutive adult patients were supported on RotaFlow (n=213) or CentriMag (n=15) ECMO, at our institutions (155 men; age 58.3±10.5 years, range: 19-84 years). Indications for support were: failure to wean from cardiopulmonary bypass in the setting of postcardiotomy (n=118) and primary donor graft failure (n=37); postacute myocardial infarction CS (n=27); acute myocarditis (n=6); and CS on chronic heart failure (n=40). A peripheral ECMO setting was established in 126 (55.2%) patients while it was established centrally in 102 (44.7%). Overall mean support time was 10.9±9.7 days (range: 1-43 days). Eighty-four (36.8%) patients died on ECMO. Overall success rate, in terms of survival on ECMO (n=144), weaning from mechanical support (n=107; 46.9%), bridge to mid-long-term ventricular assist device (n=6; 2.6%), and bridge to heart transplantation (n=31; 13.5%), was 63.1%. One hundred twenty-two (53.5%) patients were successfully discharged. Stepwise logistic regression identified blood lactate level and MB isoenzyme of creatine kinase (CK-MB) relative index at 72 h after ECMO initiation, and number of packed red blood cells (PRBCs) transfused on ECMO as significant predictors of mortality on ECMO (P=0.010, odds ratio [OR]=2.94; 95% confidence interval [CI]=1.10-3.14; P=0.010, OR=2.82, 95% CI=1.014-3.721; and P=0.011, OR=2.69; 95% CI=1.06-4.16, respectively). Central ECMO population had significantly higher rate of continuous veno-venous hemofiltration need and bleeding requiring surgery events compared with the peripheral ECMO setting population. No significant differences were seen by comparing the RotaFlow and CentriMag populations in terms of device performance. At follow-up, persistent heart failure with left ventricle ejection fraction (LVEF)≤40% was a risk factor after hospital discharge. Patients with a poor hemodynamic status may benefit from rapid central or peripheral insertion of ECMO. The blood lactate level, CK-MB relative index, and PRBCs transfused should be strictly monitored during ECMO support. In addition, early ventricular assist device placement or urgent listing for heart transplant should be considered in patients with persistent impaired LVEF after ECMO., (Copyright © 2014 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

The aim of this study is to investigate the blood perfusion and the inflammatory response of the myocardial infarct area after transplanting a hyaluronan-based scaffold (HYAFF(®) 11) with bone marrow mesenchymal stem cells (MSCs). Nine-week-old female pigs were subjected to a permanent left anterior descending coronary artery ligation for 4 weeks. According to the kind of the graft, the swine subjected to myocardial infarction were divided into the HYAFF(®) 11, MSCs, HYAFF(®) 11/MSCs and untreated groups. The animals were killed 8 weeks after coronary ligation. Scar perfusion, evaluated by Contrast Enhanced Ultrasound echography, was doubled in the HYAFF(®) 11/MSCs group and was comparable with the perfusion of the healthy, non-infarcted hearts. The inflammation score of the MSCs and HYAFF(®) 11/MSCs groups was near null, revealing the role of the grafted MSCs in attenuating the cell infiltration, but not the foreign reaction strictly localized around the fibres of the scaffold. Apart from the inflammatory response, the native tissue positively interacted with the HYAFF(®) 11/MSCs construct modifying the extracellular matrix with a reduced presence of collagene and increased amount of proteoglycans. The border-zone cardiomyocytes also reacted favourably to the graft as a lower degree of cellular damage was found. This study demonstrates that the transplantation in the myocardial infarct area of autologous MSCs supported by a hyaluronan-based scaffold restores blood perfusion and almost completely abolishes the inflammatory process following an infarction. These beneficial effects are superior to those obtained after grafting only the scaffold or MSCs, suggesting that a synergic action was achieved using the cell-integrated polymer construct., (© 2013 The Authors. Published by Foundation for Cellular and Molecular Medicine/Blackwell Publishing Ltd.)

Splitting the liver for two adults to increase the donor pool is still a debated issue, especially for combined organ transplantation. We described a case of liver-splitting procedure for two adults, which was successful even in the presence of combined organ transplantation. Three adult combined organ transplantations from one deceased donor were performed, with, use of split liver grafts in two patients: a combined heart-right split liver, a left kidney-left split liver, and a right kidney-pancreas transplantation. Despite a not perfect match between the graft type and recipient, the prevention of small-for-size syndrome by ligature of the splenic artery, and/or hemiportocaval shunt in the patient receiving the left split liver, and the maximal reduction of ischemia time were the main factors contributing to the success of the procedure. This is the first report of combined heart and split liver in two adults which may suggest new strategies for organ transplantations.

Objective: Extensive thoracic aortic aneurysms represent a challenging pathology in cardiac surgery. The frozen elephant trunk procedure, combining conventional surgery with endovascular techniques, allows single-stage treatment for such pathology. Here we present our surgical technique and results with the single-stage frozen elephant trunk procedure., Methods: Between January 2007 and December 2009, 67 patients were treated with the frozen elephant trunk procedure in our institution. Mean age was 61 ± 11 years. Indications for surgery included chronic aneurysm (n = 22, 32.8%), acute type A dissection (n = 4, 6.0%), acute type B dissection (n = 2, 3.0%), chronic type A dissection (n = 30, 44.8%), and chronic type B dissection (n = 9, 13.4%). Thirty-six patients (53.7%) had undergone 38 previous cardiac or aortic operations. Thirty-two associated aortic and cardiac operations were performed. Brain protection was achieved by means of antegrade selective cerebral perfusion and moderate hypothermia (26°C) in all cases., Results: In-hospital mortality was 13.4%. Postoperatively, permanent neurologic dysfunction (coma) occurred in 5 cases (7.5%), paraplegia in 2 (3.2%), and paraparesis in 3 (4.9%). Follow-up was 100% complete, with mean duration of 11.1 ± 8.4 months. The 1- and 2-year survivals were 76.7 ± 5.6% and 70.3 ± 8.0%, respectively. Ten patients (14.9%) required endovascular completion 2.3 ± 3.1 months after the first procedure, with 100% technical and procedural success., Conclusions: In contrast to the conventional elephant trunk technique, the frozen elephant trunk technique offers a potentially curative single-stage procedure for patients with extensive thoracic aortic disease, with encouraging short-term and midterm results., (Copyright © 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

A new bio-prosthetic valved conduit (Bio-Valsalva) has recently been introduced into surgical practice in order to offer a valid option for elderly patients undergoing composite aortic root replacement. The conduit is made up of a stentless porcine valve (elan valve) pre-sewn inside a triple layer Valsalva prosthesis and it is entirely preserved in a glutaraldehyde solution. In our Department, 21 patients (16 males, mean age 67.8+/-5.5 years) underwent aortic root replacement using the Bio-Valsalva prosthesis. Composite root replacement was extended to the hemiarch in three cases while a complete arch replacement was performed in two patients. Type A aortic dissection was present in two cases while a bicuspid aortic valve was detected in eight patients. In-hospital mortality was 4.7% (1 patient). Re-thoracotomy for bleeding was performed in one case. The median in-hospital stay was 12 days. The median follow-up was six months and is 100% complete. There were no re-operations or structural deterioration during this early phase of observation. The Bio-Valsalva graft, readily available in different sizes, demonstrates ease of implantability and shows good haemostatic characteristics. More patients and a longer follow-up are necessary to confirm the advantages of this graft.

beta-Thalassaemia is an inherited haemoglobin (Hb) disorder resulting in chronic haemolytic anaemia. The most anaemic patients require regular red blood cell (RBC) transfusions for survival but iron accumulation leads to multisystem dysfunction. Heart complications represent the leading cause of mortality in beta-thalassaemia patients. In this case report we present a successful mitral valve replacement (MVR) in a patient with a severe form of beta-thalassaemia.

Background: Although deep hypothermic circulatory arrest has been extensively used in neonates for aortic arch surgery, the brain and other organs might be adversely affected by prolonged ischemia and deep hypothermia., Methods: Between December 1997 and January 2005, 70 consecutive neonates underwent Norwood stage I procedure for hypoplastic left heart syndrome (group A, n = 30), or aortic arch repair for interruption or coarctation with arch hypoplasia (group B, n = 40), with antegrade selective cerebral perfusion (ASCP). Mean weights were 3.0 +/- 0.2 kg and 2.8 +/- 0.07 kg, and mean ages were 10 +/- 3.5 days and 14 +/- 10.6 days in groups A and B, respectively. Only 2 patients were older than 30 days. Core body temperature was lowered to 25 degrees C, and mean pump flow during ASCP was initiated at 10 to 20 mL/(kg x min) and adjusted to guarantee a radial/temporal artery pressure of 30 to 40 mm Hg and venous oxygen saturation of more than 70%. Hematocrit was maintained at 30%., Results: Early mortality was 17% (group A, 23%; group B, 12.5%; p = 0.19). Six late deaths occurred (3 in each group), and at 36 months, Kaplan-Meier overall survival was 64% +/- 9.2% in group A and 85% +/- 5.7% in group B. One patient had postoperative seizures. Age, weight, sex, prematurity, group A, and ASCP duration did not influence early mortality., Conclusions: Antegrade selective cerebral perfusion is a safe and effective procedure and might improve outcome of neonatal aortic arch surgery, minimizing neurologic impact without the need for deep hypothermia.

Aortic valve-sparing operations have provided very good clinical outcomes. However, the absence of the sinuses of Valsalva might limit valve durability. The Gelweave Valsalva prosthesis, which presents pre-fashioned neo-sinuses, has been designed in order to avoid early leaflets deterioration. We report our results in 63 patients who underwent valve-sparing operations (reimplantation technique) using the Gelweave Valsalva graft. The main indication was ascending aorta aneurysm or annuloaortic ectasia, with or without aortic insufficiency. The operation was performed also in cases of Marfan syndrome, Bicuspid Aortic Valve (BAV), and acute Type A dissection. In-hospital mortality was of 4.7%, and two thirds were acute Type A dissection patients (P=0.01). There were no late deaths. Three years freedom from grade 3-4 AI and freedom from late aortic valve replacement were 91.7+/-4.3% and 93.8+/-5.1%, respectively. Aortic valve-sparing operations show good results in patients electively operated for aortic root ectasia. Aortic cusps repair may lead to late failure. Even if the Gelweave Valsalva prosthesis is easy to implant and it also reproduces pseudosinuses, a long-term follow up is necessary to determine if this graft may reduce leaflets deterioration.

Patients who underwent isolated aortic valve replacement could come to attention for new onset aortic disease or progression of borderline alterations not corrected at the first operation, especially in the subset of bicuspid valve disease. We describe our technique in redo operations for aortic root disease, using only a vascular graft and sparing the previously implanted valve prosthesis. In case of normally functioning mechanical prosthesis, we always left the valve in situ and substituted the aortic root with a Dacron conduit, extending the replacement if necessary to the other diseased portions of the thoracic aorta.

Surgical treatment of the thoracic aorta combined with endovascular stent grafting may be an interesting approach to diseases of the thoracic aorta in high-risk patients. A patient with a chronic post-dissection aneurysm of the aortic arch and proximal descending aorta developed, after surgery, a perianastomotic leak of the distal suture of the graft. We successfully treated the leak by means of an endovascular stent graft procedure. These combined procedures may be useful to resolve surgical complications particularly in case of high operative risk.

Background: Composite valve graft replacement is currently the treatment of choice for a wide variety of lesions of the aortic root and the ascending aorta. In this study we report our experience with aortic root replacement using a composite graft., Methods: Between October 1978 and May 2001, 274 patients (79.6% male and 20.4% female) with a mean age of 53.5 years underwent composite graft replacement of the aortic root. One hundred sixty-one patients (70.8%) had annuloaortic ectasia and 46 (16.8%) aortic dissection. The classic Bentall technique was used in 94 patients (34.3%), the "button technique" in 172 patients (62.8%), and the Cabrol technique in 8 patients (2.9%)., Results: The early mortality rate was 6.9% (19 of 274 patients). Cardiopulmonary bypass time longer than 180 minutes and associated coronary artery bypass grafting were found to be independent risk factors of early mortality. The actuarial survival rate was 77.7% at 5 years and 63% at 10 years. The independent risk factors for late mortality were coronary artery disease, chronic renal failure, and postoperative dialysis. The actuarial freedom from reoperation on the remaining aorta was higher among patients without Marfan syndrome (94.6% versus 79.6% at 10 years, p = 0.008)., Conclusions: Composite valve graft replacement can be performed with low hospital mortality and morbidity. The button technique offers some advantages and should be used whenever possible. In case of acute aortic dissection root replacement is usually not necessary. Marfan patients should be treated with early root replacement before dissection occurs.