Ross-Kabbani Operation in an Infant with Mitral Valve Dysplasia

Background. Mitral valve replacement can be very difficult to obtain in infants because the valve annulus diameter can be smaller than the available prosthesis. Case Report. We describe the case of a 2-month-old female weighing 3.5 kg affected by mitral valve dysplasia leading to severe valve stenosis. Despite full medication, the clinical conditions were critical and surgery was undertaken. The mitral valve was unsuitable for repair and the orifice of mitral anulus was 12 mm, too small for a mechanical prosthesis. Therefore, a Ross-Kabbani operation was undertaken, replacing the mitral valve with the pulmonary autograft and reconstructing the right ventricular outflow tract with an etherograft. Results. The postoperative course was uneventful and the clinical conditions are good at 4-month follow-up. Conclusion. The Ross-Kabbani operation can be an interesting alternative to mitral valve replacement in infants when valve repair is not achievable and there is little space for an intra-annular mechanical prosthesis implant

488 Candidacy for heart transplantation in adult congenital heart disease patients: a single-centre, retrospective, cohort study

Abstract Aims End-stage heart failure (HF) is the leading cause of death in adult congenital heart disease (ACHD) population. Heart transplantation (HTx) improves prognosis in ACHD end-stage HF but candidacy evaluation, referral pattern, and correct listing timing are not fully elucidated in this population. To evaluate factors associated to refusal from Htx in ACHD patients with end-stage HF referred for HTx evaluation. Methods and results This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients undergone HTx between 2000 and 2013. Refusal from HTx served as primary study endpoint. Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013. The main indication to HTx in patients with single ventricle physiology was Fontan failure, while in patients with systemic left ventricle and systemic right ventricle physiology, it was systemic ventricular dysfunction. We compared clinical, anatomical and demographic data of 41 patients accepted for transplantation with 15 patients refused after screening. Risk factors for refusal were: coexistence of multiple high risk features [odds ratio (OR): 3.6; 95% confidence interval (CI): 1.1–12.9; P 0.048]; anatomical factors (OR: 14.5; 95% CI: 3.1–68.4; P 0.001), out-of-centre ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). Survival in patients accepted for HTx was significantly higher than survival in patients declined from HTx with landmark comparison at 20, 40 and 60 months of 87%, 78%, and 72% vs. 70%, 59%, and 20%, respectively. HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1–8.3; P 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, coexistence of multiple conventional HTx high risk factors and out-of-centre referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy and to reduce referral delay in tertiary centre are strongly necessary for this growing population

Traumatic rupture of the thoracic aorta: Ten years of delayed management

ObjectiveTraumatic rupture of the thoracic aorta is a highly fatal condition in which patient outcome is strongly conditioned by other associated injuries. Delayed aortic treatment has been proposed to improve results.MethodsThe charts of 69 patients with traumatic rupture of the thoracic aorta observed between 1980 and 2003 were reviewed. Patients were grouped according the timing of repair: group I, immediate repair (21 patients); and group II, delayed repair (48 patients). In group II, 45 patients were treated surgically or by endovascular procedure.ResultsIn-hospital mortalities were 4 of 21 patients (19%) in group I and 2 of 48 patients (4.2%) in group II. There were 3 cases of paraplegia in group I and none in group II.ConclusionImprovement of patient outcome with traumatic rupture of the thoracic aorta can be achieved by delaying surgical repair until after management of major associated injuries if there are no signs of impending rupture. Endovascular treatment is feasible and safe and may represent a valid alternative to open surgery in selected cases

Extracorporeal Membrane Oxygenation Support as Treatment for Early Graft Failure After Heart Transplantation

Early graft failure (EGF) is a major risk factor for death after heart transplantation (Htx) accounting for >40% of deaths within 30 days postoperatively. According to the last International Society for Heart and Lung Transplantation (ISHLT) consensus statement, the graft dysfunction (GD) is to be classified into primary (PGD), in case of an unknown triggering factor or secondary (SGD) where there is a discernible cause such as acute rejection, pulmonary hypertension, or known surgical complications. The diagnosis of GD is to be made within 24 h after completion of Htx surgery and a severity scale for GD should include mild, moderate, or severe grades based on specified criteria. Mechanical circulatory support (MCS) for GD should be considered when medical management is not sufficient to support the newly transplanted graft. Currently, extra‐corporeal membrane oxygenation (ECMO) is widely accepted as treatment of severe EGF, given its easy and quick setup, the system versatility, the optimal end‐organ perfusion provided, and the possibility of both biventricular and lung assistance by usage of a low‐cost single pump

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study

: Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population

Planeamiento estratégico de la Provincia de Castrovirreyna

El Plan Estratégico de la provincia de Castrovirreyna se realizó con base en el Modelo Secuencial del Proceso Estratégico, el cual desarrolló el Dr. Fernando D’Alessio Ipinza, quien partió de un análisis de la situación actual, para llegar a una situación futura deseada, por medio del establecimiento de la visión, misión, valores, objetivos de largo plazo, corto plazo y estrategias asociadas al entorno y sus fortalezas, debilidades, oportunidades y amenazas. Castrovirreyna es una provincia que se conforma de 13 distritos, es predominantemente rural y tiene un alto grado de pobreza y pobreza extrema, posee abundantes recursos que podrían permitirle mejorar su posición, debido a que no tiene la promoción e inversión necesaria para capitalizar la riqueza de su patrimonio en actividades productivas que le permitan desarrollarse y crecer. La propuesta del Plan se basa en el desarrollo de la infraestructura (vial y de comunicaciones) y de los servicios básicos (salud, educación e internet) que sirva de soporte e incremente el nivel de vida y la competitividad de la provincia; el desarrollo de sectores económicos como agropecuario, acuícola y turismo, a través de la mejora de la tecnología de producción de especies propias de la provincia y los beneficios tributarios, otorgados desde el Estado y las capacitaciones e incentivos a la generación de clústeres, también la participación de la población en todos los procesos de la búsqueda del desarrollo provincial. Asimismo, se orienta a motivar a las nuevas generaciones al emprendimiento teniendo en cuenta las potencialidades de la provincia, y de esta manera poder reducir las brechas de pobreza y desigualdad existentesThe Strategic Plan of the province of Castrovirreyna has been made based on the sequential model of the Strategic Process developed by Dr. Fernando D’Alessio Ipinza, based on an analysis of the current situation, to reach a desired future situation, through the establishment of vision, mission, values, long-term goals, short-term and related to the environment and their strategies strengths, weaknesses, opportunities and threats. Castrovirreyna is a province that is composed of 13 districts, it’s predominantly rural and has a high degree of poverty and extreme poverty, it has abundant resources that could enable it to improve its position because it does not have the promotion and investment needed to capitalize on the wealth of its assets in activities productive that allow develop and grow. Plan’s proposal is based on the development of the infrastructure (roads and communications) and of the basic services (health, education and internet) to serve as a support and increase the standard of living and competitiveness of the province; the development of economic sectors such as agriculture, aquaculture and tourism through improved production technology of species native of the province and the tax benefits granted from the State and trainings and incentives to generate clusters, also participation of the population in all processes of the search for provincial development. Additional aims to encourage new generations to entrepreneurship taking into account the potential of the province, and thus to reduce the gaps existing poverty and inequalityTesi

Ductal ligation timing and neonatal outcomes: a 12-year bicentric comparison.

Funder: Alma Mater Studiorum - Università di BolognaPatent ductus arteriosus (PDA) is common among extremely preterm infants. In selected cases, surgical PDA ligation may be required. The timing for PDA ligation may depend upon a variety of factors, with potential clinical implications. We aimed to investigate the impact of different surgical PDA managements on ligation timing and neonatal outcomes. Inborn infants < 32 weeks of gestation and < 1500 g admitted at two tertiary Neonatal Intensive Care Units that underwent PDA ligation between 2007 and 2018 were enrolled in this retrospective cohort study and split into the following groups based on their surgical management: on-site bedside PDA ligation (ONS) vs. referral to an off-site pediatric cardiac surgery (OFS). Neonatal characteristics, surgical timing, and clinical outcomes of the enrolled infants were compared between the groups. Multivariate analysis was performed to evaluate the impact of PDA ligation timing on significantly different outcomes. Seventy-eight neonates (ONS, n = 39; OFS, n = 39) were included. Infants in the ONS group underwent PDA ligation significantly earlier than those in the OFS group (median age 12 vs. 36 days, p < 0.001) with no increase in postoperative mortality and complications. The multivariate analysis revealed a significant association between PDA ligation timing, late-onset sepsis prevalence (OR 1.045, 0.032), and oxygen need at discharge (OR 1.037, p = 0.025).Conclusions: Compared with off-site surgery, on-site bedside ligation allows an earlier surgical closure of PDA, with no apparent increase in mortality or complications. Earlier PDA ligation may contribute to reduced rates of late-onset sepsis and post-discharge home oxygen therapy, with possible cost-benefit implications. What is known: • Ineffective or contraindicated pharmacological closure of a hemodynamically significant PDA may require a surgical ligation. • Available literature comparing the effect of early vs. late PDA ligation on the main neonatal morbidities has yield contrasting results. What is new: • The availability of a cardiac surgery service performing bedside PDA ligation allows an earlier intervention compared to patient referral to an off-site center, with no difference in postoperative mortality and complications compared to off-site surgery. • Earlier PDA ligation was associated with a lower prevalence of late-onset sepsis and of oxygen need at discharge, with possible cost-benefit implications

PREDICT identifies precipitating events associated with the clinical course of acutely decompensated cirrhosis

Background & Aims: Acute decompensation (AD) of cirrhosis may present without acute-on-chronic liver failure (ACLF) (ADNo ACLF), or with ACLF (AD-ACLF), defined by organ failure(s). Herein, we aimed to analyze and characterize the precipitants leading to both of these AD phenotypes. Methods: The multicenter, prospective, observational PREDICT study (NCT03056612) included 1,273 non-electively hospitalized patients with AD (No ACLF = 1,071; ACLF = 202). Medical history, clinical data and laboratory data were collected at enrolment and during 90-day follow-up, with particular attention given to the following characteristics of precipitants: induction of organ dysfunction or failure, systemic inflammation, chronology, intensity, and relationship to outcome. Results: Among various clinical events, 4 distinct events were precipitants consistently related to AD: proven bacterial infections, severe alcoholic hepatitis, gastrointestinal bleeding with shock and toxic encephalopathy. Among patients with precipitants in the AD-No ACLF cohort and the AD-ACLF cohort (38% and 71%, respectively), almost all (96% and 97%, respectively) showed proven bacterial infection and severe alcoholic hepatitis, either alone or in combination with other events. Survival was similar in patients with proven bacterial infections or severe alcoholic hepatitis in both AD phenotypes. The number of precipitants was associated with significantly increased 90day mortality and was paralleled by increasing levels of surrogates for systemic inflammation. Importantly, adequate first-line antibiotic treatment of proven bacterial infections was associated with a lower ACLF development rate and lower 90-day mortality. Conclusions: This study identified precipitants that are significantly associated with a distinct clinical course and prognosis in patients with AD. Specific preventive and therapeutic strategies targeting these events may improve outcomes in patients with decompensated cirrhosis. Lay summary: Acute decompensation (AD) of cirrhosis is characterized by a rapid deterioration in patient health. Herein, we aimed to analyze the precipitating events that cause AD in patients with cirrhosis. Proven bacterial infections and severe alcoholic hepatitis, either alone or in combination, accounted for almost all (96-97%) cases of AD and acute-on-chronic liver failure. Whilst the type of precipitant was not associated with mortality, the number of precipitant(s) was. This study identified precipitants that are significantly associated with a distinct clinical course and prognosis of patients with AD. Specific preventive and therapeutic strategies targeting these events may improve patient outcomes. (c) 2020 European Association for the Study of the Liver. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)

The PREDICT study uncovers three clinical courses of acutely decompensated cirrhosis that have distinct pathophysiology

Acute decompensation (AD) of cirrhosis is defined as the acute development of ascites, gastrointestinal hemorrhage, hepatic encephalopathy, infection or any combination thereof, requiring hospitalization. The presence of organ failure(s) in patients with AD defines acute-on-chronic liver failure (ACLF). The PREDICT study is a European, prospective, observational study, designed to characterize the clinical course of AD and to identify predictors of ACLF. A total of 1,071 patients with AD were enrolled. We collected detailed pre-specified information on the 3-month period prior to enrollment, and clinical and laboratory data at enrollment. Patients were then closely followed up for 3 months. Outcomes (liver transplantation and death) at 1 year were also recorded. Three groups of patients were identified. Pre-ACLF patients (n = 218) developed ACLF and had 3-month and 1-year mortality rates of 53.7% and 67.4%, respectively. Unstable decompensated cirrhosis (UDC) patients (n = 233) required ≥1 readmission but did not develop ACLF and had mortality rates of 21.0% and 35.6%, respectively. Stable decompensated cirrhosis (SDC) patients (n = 620) were not readmitted, did not develop ACLF and had a 1-year mortality rate of only 9.5%. The 3 groups differed significantly regarding the grade and course of systemic inflammation (high-grade at enrollment with aggravation during follow-up in pre-ACLF; low-grade at enrollment with subsequent steady-course in UDC; and low-grade at enrollment with subsequent improvement in SDC) and the prevalence of surrogates of severe portal hypertension throughout the study (high in UDC vs. low in pre-ACLF and SDC). Acute decompensation without ACLF is a heterogeneous condition with 3 different clinical courses and 2 major pathophysiological mechanisms: systemic inflammation and portal hypertension. Predicting the development of ACLF remains a major future challenge. ClinicalTrials.gov number: NCT03056612. Lay summary: Herein, we describe, for the first time, 3 different clinical courses of acute decompensation (AD) of cirrhosis after hospital admission. The first clinical course includes patients who develop acute-on-chronic liver failure (ACLF) and have a high short-term risk of death - termed pre-ACLF. The second clinical course (unstable decompensated cirrhosis) includes patients requiring frequent hospitalizations unrelated to ACLF and is associated with a lower mortality risk than pre-ACLF. Finally, the third clinical course (stable decompensated cirrhosis), includes two-thirds of all patients admitted to hospital with AD - patients in this group rarely require hospital admission and have a much lower 1-year mortality risk