Multidisciplinary Management of Sternal Osteomyelitis Due to Klebsiella aerogenes after Open Heart Surgery in a Patient with Multiple Myeloma: A Case Report and Discussion of the Literature.

Sternal wound complications following cardiac surgery, including sternal dehiscence, mediastinitis, and osteomyelitis, pose significant challenges in terms of management and patient outcomes. We present a case report highlighting the complex management of a patient who underwent open heart surgery for severe aortic valve stenosis, followed by sternal wound dehiscence and sternum osteomyelitis due to extended spectrum beta lactamase (ESBL) producing Klebsiella aerogenes. A multiple myeloma diagnosis was also suspected at the positron emission tomography (PET) scan and confirmed with bone marrow biopsy. Multidisciplinary evaluation of the case led to a comprehensive treatment plan. To control the sternal osteomyelitis, total sternectomy was performed followed by immediate reconstruction with a bone (tibia) graft from the tissue bank and fixation with the minimal hardware possible. A microsurgical latissimus dorsi free flap was required to reconstruct the soft tissue defect. After 6 weeks of antibiotic treatment with ertapenem and fosfomycin based on a culture of intraoperative material, no clinical, imaging, or laboratory signs of infection were seen. Multiple myeloma treatment was then started. At 1 year of follow up, no recurrence of infection occurred, and the reconstruction was stable and closed. Multiple myeloma is under chronic treatment with novel agent combination, with an excellent haematological response.

Is Pulmonary Artery Pulsatility Index (PAPi) a Predictor of Outcome after Pulmonary Endarterectomy?

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.

Asymmetrical aortic root aneurism in patient with Filamin A mutation.

We report the case of a 28 years old woman with periventricular nodular heterotopia, due to Filamin A mutation. She had an asymmetrical aneurysm of the aortic root, involving, above all, noncoronary Valsalva sinus. She was asymptomatic and she had moderate aortic regurgitation. Reimplantation of the aortic valve with replacement of the aortic root was successfully accomplished. Filamin A is a protein that is encoded by the FLNA gene, which shows X-linked dominant inheritance. This protein is involved in neuronal migration, angiogenesis, cytoskeleton regulation, and cell signaling. Therefore, mutations of FLNA gene might result in brain, blood vessels, heart, and connective tissue disorders. A miscellany of cardiovascular abnormalities could be present in this subset of patients; cardiac symptoms may precede neurological manifestations. Aorta seems to be frequently affected. Consequently, in presence of FLNA gene mutations, cardiovascular evaluation should include vascular magnetic resonance imaging or computed tomography scan.

Aortic root aneurysm in a patient with Aarskog-Scott syndrome.

We present the case of a 69 years old man affected by Aarskog-Scott syndrome. He came to our attention for an aneurysm of the aortic root, with almost moderate aortic regurgitation; moderate mitral regurgitation was discovered during preoperative assessment. We performed a modified Bentall's procedure and mitral valve repair. A patent foramen ovale was closed. Aarskog-Scott syndrome is a complex developmental disorder, characterized by X-linked recessive hereditariness short stature, craniofacial abnormalities, hyperextension of the proximal interphalangeal joints, and genital malformations. Diagnosis is still a challenge, in light of various clinical pictures and features in common with other syndromes (i.e., Noonan, SHORT, and Robinow syndromes). It has been longly debated if cardiac surveillance is needed among the affected patients; it should be probably undertaken, in view of the higher incidence of congenital heart disease. Moreover, the presence of extremely flexible joints suggests the coexistence of a connective tissue disorder.

Therapeutic alternatives in chronic thromboembolic pulmonary hypertension: from pulmonary endarterectomy to balloon pulmonary angioplasty to medical therapy. State of the art from a multidisciplinary team.

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.

Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension: a systematic review.

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.

Impact of prior sternotomy on survival and allograft function after heart transplantation: A single center matched analysis.

BACKGROUND: Orthotopic heart transplantation (OHT) remains the gold standard for the treatment of end-stage heart failure. The number of patients who have had at least one prior sternotomy while awaiting transplantation has increased over the years reaching 50% in the last ISHLT registry report. We analysed our institutional transplant activity focusing on prior-sternotomy setting to identify the real burden of this preoperative variable and its potential consequences. METHODS: Between 2000 and 2020, a total of 512 consecutive adult patients underwent OHT. We divided them into two groups according to the previous sternotomy variable: a prior sternotomy group (PS-group, n = 131, 25.6%) and a heart transplant as first sternotomy group (FS-group, n = 381, 74.4%). After propensity score matching, a total of 106 matched-pairs were identified for the final analysis. RESULTS: The overall 30-day mortality was similar in the two groups (7.5% vs. 5.7%, p = .58). The prior sternotomy was not an independent risk factor for 90-day mortality (odds ratio: 0.89, p = .81). In the matched sample, prior cardiac surgery was not predictive for any major postoperative complication: primary graft failure, AKI, bleeding, acute respiratory insufficiency, need for extra-corporeal life support (p > .05). The log-rank test revealed no significant difference between the two groups in the unmatched and matched pools (p = .93 and 0.69 respectively. At univariable analysis prior sternotomy was not associated with an increased risk of posttransplant mortality (hazard ratio: 0.87, p = .599). CONCLUSIONS: Despite it increases surgical complexity, the reoperation alone does not represent a proper risk factor and among different co-variates that may affect post-OHT outcomes.

Late acute aortic dissection following cardiac transplantation in lupus-affected donor aorta.

We report the case of a young woman who underwent cardiac transplantation from systemic lupus erythematosus affected donor and who developed a type A aortic dissection limited only to the graft aortic wall 9 years after.

[Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: 5 years of experience in Italy]. / L'angioplastica delle arterie polmonari nel cuore polmonare cronico tromboembolico: 5 anni di esperienza in Italia.

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.

[Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension:a paradigmatic case report]. / Angioplastica delle arterie polmonari nel cuore polmonare cronico tromboembolico: un caso clinico paradigmatico.

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.

Ventricular assist devices implantation: surgical assessment and technical strategies.

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.

Mechanically Supported Early Graft Failure After Heart Transplantation.

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.

Donor risk analysis and validation in heart transplants: a single-centre experience.

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.

AVR in patients with anomalous course of the circumflex artery without prosthetic downsizing.

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.

Outflow graft tunneling through the transverse sinus for HeartWare HVAD implantation as bridge to heart transplantation strategy.

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.

Role and management of extracorporeal life support after surgery of chronic thromboembolic pulmonary hypertension.

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.