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Hypoplastic left heart syndrome (HLHS) is one of the most complex congenital heart defects (CHD), characterized by a hypoplastic left ventricle (LV), dominant right ventricle (RV) and small left-sided heart structures. The introduction of the Norwood operation has significantly improved outcomes, with 5-year survival reported up to 65 %. Despite these advances, post-operative morbidity and mortality remain high, and the long-term complications in adult survivors represent a challenge. The number of HLHS patients with Fontan circulation is expected to double in the next 20 years, leading to a growing population requiring specialized care from adult congenital heart disease (ACHD) teams.This article reviews current management strategies for HLHS, outlines potential long-term complications, and highlights existing knowledge gaps. Specific considerations in this population include the assessment of the neo-aorta and aortic arch, and systemic RV dysfunction in the setting of a Fontan circulation. The proposed surveillance strategy emphasizes the need for vigilant monitoring and timely intervention to treat the complications unique to this population, ensuring better outcomes for HLHS patients reaching adulthood.

Objectives This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe.Methods A questionnaire was sent to ACHD cardiologists from 34 European countries.Results Representatives from 31 of 34 countries (91%) responded. ACHD cardiology was recognised by the respective ministry of Health in two countries (7%) as a subspecialty. Two countries (7%) have formally recognised ACHD training programmes, 15 (48%) have informal (neither accredited nor certified) training and 14 (45%) have very limited or no programme. Twenty-five countries (81%) described training ACHD doctors ‘on the job’. The median number of ACHD centres per country was 4 (range 0–28), median number of ACHD surgical centres was 3 (0–26) and the median number of ACHD training centres was 2 (range 0–28). An established exit examination in ACHD was conducted in only one country (3%) and formal certification provided by two countries (7%). ACHD cardiologist number versus gross domestic product Pearson correlation coefficient=0.789 (p

Cardiovascular imaging represents a fundamental part in the management of pregnant women with known or suspected cardiovascular disease. However, the available imaging modalities may carry different risks to the mother and fetus. Echocardiography is the safest imaging modality in pregnancy. It is the method of choice for evaluation of valvular and congenital heart disease, ventricular function, aortic disease, and assessment of hemodynamic parameters. Magnetic resonance is relatively safe and is invaluable for the evaluation of complex congenital heart disease, aortic disease, cardiomyopathies and myocarditis. Gadolinium can be harmful to the fetus and should be limited in pregnancy. Radiation exposure can also be deleterious to the fetus. The risk depends on the radiation dose and the gestational age at the time of exposure. Although common cardiovascular procedures that use ionizing radiation usually do not exceed radiation dose associated with significant fetal adverse effects, they should, nevertheless, be avoided whenever possible. If indicated, they should be performed preferably after the 12th week of gestation. In pregnancy, computed tomography is utilized in the diagnosis of pulmonary embolism, while coronary angiography is indicated in acute coronary syndrome. When deciding on imaging modality in pregnancy, both maternal and fetal benefits and risks must be taken into consideration.

While virus epidemics are nothing new to man, the scale, speed of global spread and immediacy of the COVID-19 pandemic have been truly unprecedented [1]. The entire world has been turned on its head in less than a few months, with major implications beyond disease burden and loss of life, threatening the economic status quo and human psychosocial balance and wellbeing not only for patients, but for all of us. The primary aim of our Call for Action Viewpoint was to support and protect our adult congenital heart disease (ACHD) patients and their needs during these challenging and uncertain times. This goal had to be met while we, as individuals, teams, institutions and nations, came together in a global effort to combat this aggressive virus, that appears to spare no organs or systems, nor any borders, geographic or other. As with any crisis, there is always opportunity: we are submitting herewith a vision for a different and better model of ACHD care, and for a better life journey and health care experience for our patients, that should be in place in the aftermath of the Covid-19 pandemic. Many of the points and principles discussed in this article, need not be confined to ACHD patients, but have a broader reach. And we must not forget nor neglect the most vulnerable in society at this time, namely the elderly, disabled and other dependent or disadvantaged groups in this “We Are One” global operation. Last but not least, this maybe the time to take better care of ourselves (and others) and reflect on life.

The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success story of modern medicine. In the 1950s survival of children born with CHD was only approximately 15%, whereas nowadays more than 90% of these children survive well into adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients with CHD over 60 years old. Many adult patients are afflicted by residual haemodynamic lesions and also face additional opportunities and/or challenges such as pregnancy, acquired heart disease, non-cardiac pathology etc., necessitating integrated care and all medical disciplines. We are faced with a ‘tsunami’ in terms of adult CHD numbers, disease heterogeneity, and complexity of work and interventions needed. We need to secure resources, welcome more people in our field, learn from ‘marching with our patients’, and educate better patients, public, and ourselves so that every single patient with CHD, born anywhere in the world, may reach their full life potential.

Background Women are underrepresented in leading medical positions and academia. The gender-gap in authorship of congenital heart disease (CHD) publications remains unknown. As determinants of gender equity in this field are poorly characterised, we aimed to quantify and characterise publications in CHD and to assess factors associated with female representation in research.Methods and results We identified 35 118 CHD publications between 2006 and 2015 for which author gender could be ascertained. Overall, 25.0% of all authors were female. Women accounted for 30.2% and 20.8% of all first and senior authorship positions with great geographic heterogeneity. While globally female first and senior authorship increased by 0.8% and 0.6%/year, some geographic regions showed no improvement in gender representation. Significant predictors of female first authorship on logistic regression analysis were country gross domestic product, human development index, gender inequality index and a female senior author (p