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Background Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. Objectives This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. Methods Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. Results From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. Conclusions COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity., Central Illustration

Introduction Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. Our aim was to investigate how the location of the shunt influences the outcome of these patients. Methods The REHAP registry is a Spanish multicentre voluntary registry of patients over 14 years of age, which includes patients with congenital heart disease and PAH, starting in 2007. Baseline characteristics, functional class, right catheterization data, treatment and survival were compared. We performed a cox regression model for assessing the impact of the type of shunt on prognosis, after looking for confusing and effect modifying variables. Results 664 patients were analyzed, their characteristics are detailed in the table. Mean follow up time was 10 years. We performed a survival analysis adjusted for the confounding variables: age (Hazard ratio (HR) 1.02 p Conclusion The type of shunt significantly affects prognosis in Eisenmenger patients, with a much worse outcome for those with pretricuspid shunts. Functional class and clinical group are also closely related to outcome. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): MSD, GSK

Background Risk models for primary prevention strategies in adult congenital heart disease (ACHD) must incorporate the heterogeneous risk for sudden cardiac death (SCD) and life-threatening ventricular arrhythmias (LTVA) as stratified by underlying lesion. Objectives To determine lesion-specific risk for SCD and LTVA in ACHD. Methods We analyzed 3311 ACHD patients (50% males) prospectively followed-up for 37510 person/years. SCD cases were confirmed by means of the Spanish National Death Registry. In addition, we identified all cases of resuscitated cardiac arrest or ventricular tachycardia requiring cardioversion. According to the incidence rate of the composite end-point of SCD and LTVA, lesions were stratified into four groups of risk. Cumulative freedom from SCD or LTVA in patients at high, moderate, low and very low risk were compared by using Cox regression model with left truncation. The c-index of this lesion-specific risk stratification was calculated by using the β-coefficients. The discriminative ability of this lesion-specific risk stratification was also tested in an external cohort of 203 SCD-LTVA cases and 2287 controls from 20 different centers. Results 71 patients experienced an event (53 SCD, 18 LTVA). Patients at highest risk (incidence rate >1%) were those with Rastelli procedure, severe coronary abnormalities, complex Fallot and cyanotic patients, either Eisenmenger or non-Eisenmenger; at moderate risk (incidence rate 0.25–1.0%) non-complex Fallot, Mustard/Senning repair, Fontan procedures and congenitally corrected transposition; at low risk (incidence rate 0.1–0.25%) Ebstein anomaly and left heart lesions; and at very low risk (incidence rate Lesion-specific risk and C-index Conclusions A lesion-specific risk stratification based on the incidence rate of SCD and LTVA was performed and validated. This approach could result in a more individualized risk assessment. Acknowledgement/Funding Instituto de Salud Carlos III, Ministerio de Economía y Competividad, Spain (Exp PI14/02099 and PI17/01327) and co-financed by FEDER

The use of non-invasive ventilation (NIV) in severe acute respiratory failure (ARF) due to community-acquired pneumonia (CAP) is controversial, and the risk factors for NIV failure in these patients are not well known. We assessed the characteristics and predictors of outcome of patients with CAP and severe ARF treated with NIV.We prospectively assessed 184 consecutive patients; 102 had "de novo" ARF, and 82 previous cardiac or respiratory disease. We defined successful NIV as avoidance of intubation and intensive care unit (ICU) survival at least 24 h in the ward. We assessed predictors of NIV failure and hospital mortality in multivariate analyses.Patients with "de novo" ARF failed NIV more frequently than patients with previous cardiac or respiratory disease (47, 46% versus 21, 26%, p = 0.007). Worsening radiologic infiltrate 24 h after admission, maximum Sepsis-Related Organ Failure Assessment (SOFA) score and, after 1 h of NIV, higher heart rate and lower PaO(2)/FiO(2) and bicarbonate independently predicted NIV failure. Likewise, maximum SOFA, NIV failure and older age independently predicted hospital mortality. Among intubated patients with "de novo" ARF, NIV duration was shorter in hospital survivors than non-survivors (32 ± 24 versus 78 ± 65 h, p = 0.014). In this group, longer duration of NIV before intubation was associated with decreased hospital survival (adjusted odds ratio 0.978, 95% confidence interval 0.962-0.995, p = 0.012). This association was not observed in patients with previous cardiac or respiratory disease.Successful NIV was strongly associated with better survival. If predictors for NIV failure are present, avoiding delayed intubation of patients with "de novo" ARF would potentially minimise mortality.

Migration of cardiac neural crest cells into the pharyngeal arches and the pharyngeal and splanchnic mesoderm contributes to the development of the cardiac outflow tract. The adrenal cortex is derived from the splanchnic mesoderm. Neuroblastoma is more prevalent in patients with congenital heart disease than in the general population, because both originate from embryonal neural crest-derived cells. Similarly, and in light of recent embryological findings, abnormal development or migration of splanchnic mesoderm, possibly due to an underlying genetic defect, could contribute to the association of adrenocortical carcinoma and tetralogy of Fallot. We present the case of a cardiologically asymptomatic 49-year-old woman with total correction of tetralogy of Fallot in the first year of life.

ResumenLa lesión de Dieulafoy es una anomalía vascular, rara pero potencialmente fatal de hemorragia gastrointestinal, y constituye menos del 2% de las causas de hemorragia digestiva aguda. El tratamiento endoscópico empleando diferentes métodos es el de elección teniendo que recurrir en pocos casos a la cirugía. Se presentan 2 casos de lesión de Dieulafoy con manejo endoscópico exitosoAbstractDieulafoy's lesion is a rare vascular anomaly with potentially fatal gastrointestinal bleeding that constitutes less than 2% of the causes of acute gastrointestinal bleeding. Endoscopic therapy using different methods is the treatment of choice, having to resort to surgery in rare cases. Two cases of Dieulafoy's lesion, with successful endoscopic management, are presented.