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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: To review the role of intermittent positive pressure breathing in the cardiac surgery setting. Methods: This review was conducted by searching seven major databases. A search string was built using the key words 'intermittent positive pressure breathing', and 'cardiac surgery' matched with the Boolean operator AND. Filters were selected for the publication dates January 2006 to January 2016. In each database, the search was conducted in the fields of the article title, abstract and keywords. Results: In total, seven citations that fulfilled the inclusion criteria were included in the final review. There was consensus among the authors regarding the inspiratory pressure values at which intermittent positive pressure breathing should be administered (15-30 cm H2O). The duration of treatment ranged between 15-30 minutes divided into one to three daily sessions, or was carried out considering the number of respirations. With regard to the treatment of pulmonary atelectasis, intermittent positive pressure breathing was shown to be effective after coronary artery bypass grafting, and it was the most cited postoperative intervention used to treat either pulmonary atelectasis and oxygen impairment, in cardiac surgery patients. Conclusions: The findings of this review seem to confirm that intermittent positive pressure breathing is still currently being used postoperatively in the cardiac surgery setting to treat surgery-related pulmonary complications. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. 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. 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). 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. [ABSTRACT FROM AUTHOR]

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. doi: 10.1111/jocs.12480 (J Card Surg 2015;30:201-208) [ABSTRACT FROM AUTHOR]

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. 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. 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). 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. [ABSTRACT FROM AUTHOR]

β-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 β-thalassaemia patients. In this case report we present a successful mitral valve replacement (MVR) in a patient with a severe form of β-thalassaemia. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. • Vascular remodeling is an early and intrinsic feature of HCM. • Vessels are equally altered in spite of the phenotypic expression of the disease. • Interstitial fibrosis prevails in HOCM, replacement fibrosis is distinctive of ES-HCM. • In ES-HCM, vasculopathy shows a more stringent association with scar-like fibrosis. • Microvascular ischemia may cause myocardial scarring and promote ES progression. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Two centrifugal pumps, the Rota Flow ( Maquet, Jostra Medizintechnik AG, Hirrlingen, Germany) and Levitronix Centri Mag ( 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 Rota Flow ( n = 213) or Centri Mag ( 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 Rota Flow and Centri Mag 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. [ABSTRACT FROM AUTHOR]

Abstract: Objective: The purpose of this study was to examine our experience with the frozen elephant trunk in patients with chronic aortic dissection. Methods: In our Institution, between January 2007 and August 2010, 49 patients (mean age: 59.6±9.0 years) underwent total arch replacement with the frozen elephant trunk technique for chronic aortic dissection (type A, n =2; residual type A, n =37; type B, n =10). Forty patients (81.6%) patients had undergone previous cardiovascular procedures. Associated cardiac procedures were indicated in 21 (42.8%) patients. Brain protection was achieved with antegrade selective cerebral perfusion in all cases. Results: Hospital mortality (n =5) was 10.2%. Postoperative serious complications included coma (n =3; 6.1%), paraplegia (n =2; 4.1%), respiratory failure (n =6; 12.2%), and definitive dialysis (n =2; 4.1%). Follow-up was 100% completed (mean period: 12.9±11.7 months). The estimated 1- and 3-year survival rates were 91.2±4.2% and 81.6±6.5%, respectively. Endovascular extension was required in 11 (22.4%) patients, with technical success of 100%. Complete thrombosis of the peri-stent false lumen was achieved in 82.9% of cases, with significant reduction of the false lumen diameter (preoperative: 36±11mm; postoperative: 24±17mm; p =0.001) and increase of the true lumen diameter (preoperative: 15±5mm; postoperative: 26±6mm; p =0.001). Conclusions: The frozen elephant trunk technique, allowing treatment of extensive disease of the thoracic aorta, was associated with encouraging short- and midterm results. Longer-term follow-up is warranted. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]