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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: 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.)

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.)

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.

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.