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Objective The object of the present study is to evaluate factors precluding heart transplantation (HTx) in adult congenital heart disease patients (ACHD) with end-stage heart failure (HF) referred for HTx evaluation. Methods This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients receiving HTx between 2001 and 2013. HTx refusal due to poor candidacy status for excess risk of mortality after transplantation, served as the main study outcome. Results Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013. We compared clinical, anatomical and demographic data of 41 patients suitable for transplantation (including both listed/transplanted patients, not yet listed because too well and patients who chose to refuse transplantation) with 15 patients refused after screening (excluding 4 patients with ongoing screening). Risk factors for refusal were: multiple high risk features (odds ratio [OR]: 3.6; 95% confidence interval [CI]: 1.1 to 12.9; p 0.048); anatomical factors (OR: 14.5; 95% CI: 3.1 to 68.4; p 0.001), out-of-center ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1 to 8.3; p 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, multiple conventional HTx high risk factors and out-of-center referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy are strongly necessary for this growing population. Funding Acknowledgement Type of funding sources: None.

The growing population of women with heart disease of reproductive age has been associated with an increasing number of high-risk pregnancies. Pregnant women with heart disease are a very heterogeneous population, with different risks for maternal cardiovascular, obstetric, and foetal complications. Adverse cardiovascular events during pregnancy pose significant clinical challenges, with uncertainties regarding diagnostic and therapeutic approaches potentially compromising maternal and foetal health. This review summarizes best practice for the treatment of common cardiovascular complications during pregnancy, based on expert opinion, current guidelines, and available evidence. Topics covered include heart failure (HF), arrhythmias, coronary artery disease, aortic and thromboembolic events, and the management of mechanical heart valves during pregnancy. Cardiovascular pathology is the leading cause of non-obstetric morbidity and mortality during pregnancy in developed countries. For women with pre-existing cardiac conditions, preconception counselling and structured follow-up during pregnancy are important measures for reducing the risk of acute cardiovascular complications during gestation and at the time of delivery. However, many women do not receive pre-pregnancy counselling often due to gaps in what should be lifelong care, and physicians are increasingly encountering pregnant women who present acutely with cardiac complications, including HF, arrhythmias, aortic events, coronary syndromes, and bleeding or thrombotic events. This review provides a summary of recommendations on the management of acute cardiovascular complication during pregnancy, based on available literature and expert opinion. This article covers the diagnosis, risk stratification, and therapy and is organized according to the clinical presentation and the type of complication, providing a reference for the practicing cardiologist, obstetrician, and acute medicine specialist, while highlighting areas of need and potential future research.

Background Ebstein anomaly is a rare and heterogeneous congenital heart defect affecting the tricuspid valve and right ventricular (RV) myocardium. Few studies have analysed the electrocardiographic features of Ebstein anomaly and none has addressed correlations with disease severity. Methods Patients with Ebstein anomaly who had undergone electrocardiography and cardiac magnetic resonance (CMR) within 6 weeks between 2001 and 2009 were included. Exclusion criteria were: associated congenital cardiac defect, previous RV myoplasty and/or reduction surgery, class I anti-arrhythmic drug therapy, and paced/pre-excited QRS. Standard electrocardiogram (ECG) findings were correlated with CMR-based RV measures and clinical profile. Results The mean age of the 63 study patients was 22 ± 13 years. An RV conduction delay (rsR′ pattern in right precordial leads) was present in 45 patients (71%). The QRS duration correlated with anatomic RV diastolic volume ( r = +0.56, P < 0.0001) and inversely with RV ejection fraction (EF; r = −0.62, P < 0.0001). The presence of QRS fractionation predicted greater atrialized RV volume (80 ± 31 vs. 45 ± 37 mL/m2, P < 0.001). Normal QRS duration was associated with smaller anatomic RV diastolic volume (150 ± 57 vs. 256 ± 100 mL/m2; P < 0.0001), higher RV EF (48 ± 6 vs. 34 ± 14%; P < 0.0001), higher oxygen consumption (VO2) at cardiopulmonary exercise (25.8 vs. 21.8 mL/kg/min, P = 0.05) and lower incidence of oxygen desaturation with exercise (25 vs. 65%, P = 0.02). Conclusion Delayed and prolonged depolarization of the RV is common in patients with Ebstein anomaly. The QRS duration is a marker of RV enlargement and dysfunction. QRS fractionation is associated with a greater atrialized RV volume. A preserved surface ECG identifies a subset of patients with Ebstein anomaly with mild morphological and functional abnormalities and better clinical profile.