Cardiac catheterization in pulmonary arterial hypertension: Tips and tricks to enhance diagnosis and guide therapy.

Cardiac catheterization (CC) is essential for the diagnosis of pulmonary hypertension (PH), and for its characterisation. It allows distinction between pre- and post-capillary PH which, when integrated with other non-invasive data, facilitates classification into one of the 5 diagnostic groups defined by international PH guidelines. CC also provides valuable information for the risk stratification of patients with PH, guiding management and the type and intensity of treatment.

Atherosclerotic Risk Factor Prevalence in Adults With Congenital Heart Disease: A Meta-Analysis.

Background: The risk of atherosclerotic cardiovascular disease (ASCVD) in adults with congenital heart disease (ACHD) is comparable to that of the general population and is driven by traditional ASCVD risk factors.

Objectives: The aim of the study was to estimate the prevalence of traditional ASCVD risk factors (hypertension, dyslipidemia, diabetes mellitus [DM], obesity, smoking, and physical inactivity) in ACHD and compare it with the general population.

Methods: A systematic literature search was conducted up to May 15, 2024, to identify studies (with or without control group) reporting the prevalence of ASCVD risk factors in ACHD.

Pregnancy in women with a Fontan circulation: Short and long-term outcomes.

Background: The Fontan circulation procedure is the palliative surgery of choice for patients with single ventricle physiology, many of whom are now reaching childbearing age due to advances in care. Our study examines the impact of pregnancy on women with Fontan circulation, assessing both short and long-term outcomes.

Methods: We retrospectively analysed pregnancies in women with Fontan circulation at our centre from 2005 to 2023, including a matched non-pregnant, nulliparous cohort for comparison.

Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management.

Patients with pulmonary hypertension associated with a left-right shunt include a wide spectrum of pathophysiological substrates, ranging from those characterized by pulmonary over-circulation to those with advanced pulmonary vascular disease. The former group may benefit from shunt repair in carefully selected cases but, when advanced pulmonary vascular disease has developed, defect closure should be avoided, and pulmonary vasodilators may be used to improve effort tolerance and hemodynamics. There is a paucity of evidence, however, to support decision-making in the care of these patients.

Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

Aims: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking.

Methods And Results: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.

Adult congenital heart disease training in Europe: current status, disparities and potential solutions.

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.

Repair of Aortic Regurgitation in Young Adults: Sooner Rather Than Later.

Background Establishing surgical criteria for aortic valve replacement (AVR) in severe aortic regurgitation in young adults is challenging due to the lack of evidence-based recommendations. We studied indications for AVR in young adults with severe aortic regurgitation and their outcomes, as well as the relationship between presurgical echocardiographic parameters and postoperative left ventricular (LV) size, function, clinical events, and valve-related complications. Methods and Results Data were collected retrospectively on 172 consecutive adult patients who underwent AVR or repair for severe aortic regurgitation between 2005 and 2019 in a tertiary cardiac center (age at surgery 29 [22-41] years, 81% male).

Prognostic significance of subpulmonary left ventricular size and function in patients with a systemic right ventricle.

Aims: To assess the additional prognostic significance of echocardiographic parameters of subpulmonary left ventricular (LV) size and function in patients with a systemic right ventricle (SRV).

Methods And Results: All adults with an SRV who underwent transthoracic echocardiography in 2010-18 at a large tertiary centre were identified. Biventricular size and function were assessed at the most recent examination.

Management of pulmonary arterial hypertension during pregnancy.

Introduction: Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure >20 mmHg and pulmonary vascular resistance >2 Wood Units (WU) on right-heart catheterization. Pregnancy is generally contraindicated in PAH, it is associated with high maternal mortality. Despite current recommendations, the number of women with PAH wishing to become pregnant is increasing.

Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes.

Aims: Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021.

Transition to adult care of young people with congenital heart disease: impact of a service on knowledge and self-care skills and correlates of a successful transition.

Aims: Less than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition.

Methods And Results: Overall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.

Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease.

Long-term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension.

To assess the long-term prognostic value of cardiac catheterization and acute vasodilator testing (AVT) with inhaled iloprost in children with idiopathic pulmonary arterial hypertension (IPAH). Data on 81 consecutive children with IPAH referred to our center who underwent cardiac catheterization and AVT between June 2008 and August 2019 were collected. The correlation between the invasive hemodynamic data and transplant-free survival was analyzed.