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.

B-type natriuretic peptide levels predict long-term mortality in a large cohort of adults with congenital heart disease.

Background And Aims: Many adult patients with congenital heart disease (ACHD) are still afflicted by premature death. Previous reports suggested natriuretic peptides may identify ACHD patients with adverse outcome. The study investigated prognostic power of B-type natriuretic peptide (BNP) across the spectrum of ACHD in a large contemporary cohort.

Pregnancy in patients with the Fontan operation.

Improved survival rates for patients with a Fontan circulation has allowed more women with this complex cardiac physiology to contemplate pregnancy. However, pregnancy in women with a Fontan circulation is associated with a high risk of adverse maternal and foetal outcomes, high rates of miscarriage, and preterm delivery. Factors associated with a successful pregnancy outcome are younger age, normal body weight, absence of significant functional limitation, no Fontan-related complications, and well-functioning single ventricle physiology.

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.

Multifaceted Fontan Patients and Their Response to Pregnancy.

We present 4 patients with Fontan circulation who underwent successful pregnancies, albeit with complications that required close monitoring and timely intervention. Each Fontan patient presents with a unique clinical picture, making risk stratification challenging but all the more important.

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).

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.

Percutaneous intervention of severe native coarctation of the aorta presenting in pregnancy: a case report.

Background: Coarctation of the aorta (CoA) is one of the more common congenital heart defects affecting up to 5% of patients with congenital heart disease. Pregnant patients with unrepaired or severe re-coarctation are considered to be modified World Health Organisation (mWHO) IV, have the highest risk of maternal mortality and morbidity. The management of unrepaired CoA during pregnancy is influenced by a variety of factors which include the extent of the coarctation and coarctation characteristics, but due to paucity of data, it largely relies on expert opinion.

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.

Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block.

Background: Isolated congenital complete heart block (CCHB) is rare cardiac condition associated with maternal Anti-Rho antibodies. It is managed with permanent pacemaker insertion. We sought to determine the long-term outcomes of individuals with CCHB who had undergone pacemaker insertion.

Enhanced Assessment of Perioperative Mortality Risk in Adults With Congenital Heart Disease.

Background: In-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment.

Objectives: The purpose of this study was to assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery.

Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle.

Background: To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.

Methods: Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied.

Severe Left Ventricular Outflow Tract Obstruction Immediately After Surgical Repair of Ebstein's Anomaly.

A 52-year-old man following surgery for Ebstein's anomaly after repair developed acute hemodynamically significant left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve and severe mitral regurgitation. Fluid resuscitation and weaning of inotropes were unsuccessful. Left ventricular outflow tract obstruction and mitral regurgitation resolved by using esmolol.

Timely Pulmonary Valve Replacement May Allow Preservation of Left Ventricular Circumferential Strain in Patients with Tetralogy of Fallot.

Introduction: Patients with Tetralogy of Fallot (TOF) and pulmonary insufficiency and a dilated right ventricle (RV) may suffer from a reduction in left ventricular (LV) performance. It is not clear whether timely pulmonary valve replacement (PVR) preserves LV mechanics.

Methods: Ten TOF patients who underwent PVR were identified from hospital records, and pre- and postoperative cardiac magnetic resonance images were post-processed with a semi-automatic tissue tracking software.

Aortic length measurements for pulse wave velocity calculation: manual 2D vs automated 3D centreline extraction.

Background: Pulse wave velocity (PWV) is a biomarker for the intrinsic stiffness of the aortic wall, and has been shown to be predictive for cardiovascular events. It can be assessed using cardiovascular magnetic resonance (CMR) from the delay between phase-contrast flow waveforms at two or more locations in the aorta, and the distance on CMR images between those locations. This study aimed to investigate the impact of different distance measurement methods on PWV.

Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease.

Background: In patients with acquired heart failure, hypoalbuminaemia is associated with increased risk of death. The prevalence of hypoproteinaemia and hypoalbuminaemia and their relation to outcome in adult patients with congenital heart disease (ACHD) remains, however, unknown.

Methods: Data on patients with ACHD who underwent blood testing in our centre within the last 14 years were collected.