Summary Background Since December, 2019, Wuhan, China, has experienced an outbreak of coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Epidemiological and clinical characteristics of patients with COVID-19 have been reported but risk factors for mortality and a detailed clinical course of illness, including viral shedding, have not been well described. Methods In this retrospective, multicentre cohort study, we included all adult inpatients (≥18 years old) with laboratory-confirmed COVID-19 from Jinyintan Hospital and Wuhan Pulmonary Hospital (Wuhan, China) who had been discharged or had died by Jan 31, 2020. Demographic, clinical, treatment, and laboratory data, including serial samples for viral RNA detection, were extracted from electronic medical records and compared between survivors and non-survivors. We used univariable and multivariable logistic regression methods to explore the risk factors associated with in-hospital death. Findings 191 patients (135 from Jinyintan Hospital and 56 from Wuhan Pulmonary Hospital) were included in this study, of whom 137 were discharged and 54 died in hospital. 91 (48%) patients had a comorbidity, with hypertension being the most common (58 [30%] patients), followed by diabetes (36 [19%] patients) and coronary heart disease (15 [8%] patients). Multivariable regression showed increasing odds of in-hospital death associated with older age (odds ratio 1·10, 95% CI 1·03–1·17, per year increase; p=0·0043), higher Sequential Organ Failure Assessment (SOFA) score (5·65, 2·61–12·23; p Interpretation The potential risk factors of older age, high SOFA score, and d-dimer greater than 1 μg/mL could help clinicians to identify patients with poor prognosis at an early stage. Prolonged viral shedding provides the rationale for a strategy of isolation of infected patients and optimal antiviral interventions in the future. Funding Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences; National Science Grant for Distinguished Young Scholars; National Key Research and Development Program of China; The Beijing Science and Technology Project; and Major Projects of National Science and Technology on New Drug Creation and Development.

Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study

Prevention of infective endocarditis

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.

Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

n Society of Echocardiography designates this educational activity for of 1.5 AMA PRA Category 1 Credits . Physicians should only claim credit te with the extent of their participation in the activity. CCI recognize the ASE’s certificates and have agreed to honor the credit their registry requirements for sonographers. n Society of Echocardiography is committed to ensuring that its educan and all sponsored educational programs are not influenced by the special y corporation or individual, and itsmandate is to retain only those authors ial interests can be effectively resolved to maintain the goals and educaty of the activity. Although amonetary or professional affiliationwith a cors not necessarily influence an author’s presentation, the Essential Areas and e ACCME require that any relationships that could possibly conflict with al value of the activity be resolved prior to publication and disclosed to . Disclosures of faculty and commercial support relationships, if any, dicated. ience: is designed for all cardiovascular physicians and cardiac sonographers with erest and knowledge base in the field of echocardiography; in addition, reschers, clinicians, intensivists, and other medical professionals with a spein cardiac ultrasound will find this activity beneficial.

Recommendations for Quantification Methods during the Performance of a Pediatric Echocardiogram: A Report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. ©RSNA, 2016.

Congenital Variants and Anomalies of the Aortic Arch

Thrombotic and Embolic Complications Associated With Atrial Arrhythmia After Fontan Operation: Role of Prophylactic Therapy

Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation

Background
Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV.

Methods
The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention.

Results
Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8–9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P < .04).

Conclusions
The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD.

Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects

Background In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. Methods The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016. Cusp fusion morphology was determined from the echocardiographic studies, while coexisting cardiac defects and genetic syndromes were determined from chart review. Results A total of 1,037 patients with BAV were identified: 550 (53%) had an isolated BAV, 299 (29%) had BAV and a coexisting congenital heart defect, and 188 (18%) had BAV and a coexisting genetic syndrome or disorder. There were no differences in distribution of morphology across the three groups. However, right-noncoronary (RN) cusp fusion was the predominant morphology associated with Down syndrome ( P  = .002) and right-left (RL) cusp fusion was the predominant morphology associated with Turner syndrome ( P  = .02), DiGeorge syndrome ( P  = .02), and Shone syndrome ( P  = .0007), when compared with valve morphology in patients with isolated BAV. Isolated BAV patients with RN cusp fusion had larger ascending aorta diameter ( P  = .001) and higher number of patients with ≥ moderate aortic regurgitation ( P  = .02), while those with RL cusp fusion had larger sinus of Valsalva diameter ( P  = .0006). Conclusions Morphological subtypes of BAV are associated with different genetic syndromes, suggesting distinct perturbations of developmental pathways in aortic valve malformation.

Talha Niaz

Papers

Thrombotic and Embolic Complications Associated With Atrial Arrhythmia After Fontan Operation: Role of Prophylactic Therapy

Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation

Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects

Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes

Outcomes in adult Fontan patients with atrial tachyarrhythmias