Background Congenital aortic stenosis (ConAoS) accounts for approximately 4%–8% of all congenital heart diseases. Chronic pressure overload may result in myocardial stiffening, leading to impaired cardiac filling, reduced contractility and ultimately contributing to the onset of symptomatic heart failure. Shear wave elastography (SWE), using high-frame-rate echocardiography, allows visualisation of myocardial shear waves. By measuring shear wave propagation velocity (SWV), this technique may enable non-invasive assessment of myocardial stiffness. This study aimed to explore the feasibility of SWE in adults with ConAoS and to compare SWV with healthy volunteers (HVs).Methods In 68 adult patients with ConAoS—including 17 patients after aortic valve replacement (AVR)—and 19 HVs, high-frame-rate recordings were acquired in the parasternal long-axis view. Custom software was used to measure SWV by manually placing M-mode lines through the interventricular septum after aortic valve closure.Results SWE feasibility was 73% in ConAoS patients with a native aortic valve (NV), 53% post-AVR and 100% in HVs. Median SWV was 4.7 m/s (3.2–5.5) in NV patients, 3.7 m/s (3.1–5.4) post-AVR and 3.4 m/s (3.0–4.4) in HVs (p=0.200). Patients with moderate-to-severe ConAoS demonstrated higher SWV compared with HVs (4.7 m/s (3.4–5.6) vs 3.4 m/s (3.0–4.4); p=0.035).Conclusions SWE is feasible in the majority of ConAoS patients. Higher SWV in moderate-to-severe ConAoS patients, despite preserved diastolic function in most cases, may reflect early myocardial mechanical alterations and provide a promising non-invasive marker for myocardial stiffening. Further technical optimisation and cross-platform validation are essential for clinical translation.

In recent years, remarkable advancements in paediatric cardiology and surgical techniques have dramatically increased the survival of patients with congenital heart disease. As a result, adults with congenital heart disease (ACHD) are now living longer, and many, despite having residual lesions and sequelae, may have near-normal life expectancy. Adults with congenital heart disease patients should no longer be viewed merely as survivors; instead, medical care should shift its focus from simply prolonging life to enhancing quality of life and overall well-being. Moreover, as ACHD population ages, they are increasingly exposed to a burden of acquired cardiovascular and extracardiac disease, making early intervention on modifiable risk factors a key priority. Herewith, we provide an overview of the multiple aspects that should be considered in this holistic approach to guide everyday issues of ACHD patients, including nutrition, physical activity, sexual health, cancer prevention, respiratory disease, and infective endocarditis.

BACKGROUND:The hemodynamic changes during pregnancy can be challenging in women with underlying heart disease, particularly in women with impaired left ventricular function (LVF, left ventricular ejection fraction <40%). OBJECTIVES:The aim of this study was to describe the cardiac, obstetric, and fetal outcomes of pregnancy in women with impaired LVF. METHODS:ROPAC (Registry Of Pregnancy and Cardiac disease) includes an international, prospective, observational cohort of pregnancies in women with heart disease. Cardiac, obstetric, and fetal outcomes were analyzed in 251 patients with impaired LVF. The primary endpoint was the occurrence of major adverse cardiac events (MACE) including maternal death, supraventricular or ventricular arrhythmias, heart failure, aortic dissection, endocarditis, ischemic coronary event, and other thromboembolic events. Logistic regression was used to determine variables associated with poor outcomes. RESULTS:Median follow-up duration was 7 (6-11) months. Maternal mortality occurred in 6/251 (2.4%, 1%-5%) and heart failure in 67/251 (27%, 21%-33%) patients. Ventricular tachyarrhythmias occurred in 11/251 (4%, 2%-8%) patients. Eighty-one of 251 (32%, 27%-38%) patients experienced at least one MACE during pregnancy or up to 6 months postpartum. Obstetric complications were common, including preterm birth in 67/251 (27%, 22%-33%) and low birthweight in 65/251 (26%, 21%-32%). Patients with cardiomyopathy were at higher risk of cardiovascular complications with 4.3% mortality and nearly 40% risk of MACE during pregnancy. Prepregnancy signs of heart failure (OR: 2.67; 1.3-5.6), atrial fibrillation (OR: 6.32; 3.0-13.3), and an NYHA functional class >II (OR: 6.06; 2.2-16.6) were associated with poor cardiac outcomes. CONCLUSIONS:Women with impaired LVF are at increased risk of complications, particularly heart failure, tachyarrhythmias, and premature delivery with low birth weight.

BACKGROUND:Left ventricular (LV) remodeling is becoming increasingly important in assessing the impact of aortic stenosis (AS). However, data on structural LV remodeling in congenital AS patients is scarce. Therefore, this study aimed to describe LV remodeling before and after aortic valve replacement (AVR) in adults with congenital AS and identify markers associated with myocardial fibrosis. METHODS AND RESULTS:In this prospective study, adult patients with congenital AS were included with 1) at least mild AS (Vmax ≥2.5 m/s) or 2) prior AVR due to congenital AS. Cardiovascular Magnetic Resonance (CMR) was performed to assess structural LV remodeling. Results were compared with CMR data from 20 healthy controls. Data from 73 patients were included (median age 43 [30-52] years, 67% male), of whom 20 patients were post-AVR. LV systolic function was preserved in all patients. LV mass index was significantly higher in patients with a native valve than in controls (70 ± 19 vs. 59 ± 8 g/m2, p = 0.023). Late gadolinium enhancement (LGE) was present in 19 patients (26%): 13 with a native valve and 6 post-AVR (25% vs. 30%, p = 0.64). Native T1 and extracellular volume (ECV) did not differ significantly from healthy controls. In patients post-AVR, LV mass index and matrix volume were significantly lower compared to patients with a native valve. CONCLUSION:LGE was present in a quarter of adults with congenital AS. However, ECV in patients with native valves did not differ significantly from healthy controls. LV dimensions were lower in patients post-AVR, but LGE persisted, indicating LV remodeling even after AVR.

OBJECTIVES:Data on long-term outcomes after surgical repair of pulmonary valve stenosis are limited. This study evaluated survival, clinical outcomes and quality of life (QoL) after surgery during childhood. METHODS:Single centre, longitudinal cohort study evaluating consecutive patients with pulmonary valve stenosis who underwent surgical repair between 1968-1980 and were evaluated every decade since 1990. RESULTS:Of the original cohort of 89 operated patients, 11 died (12%), including 2 who died within 30 days postsurgery (2%), and 7 (8%) were lost to follow-up. Survival at 50 years follow-up was 87%, which was not significantly different from the GDP. Of the remaining 71 survivors, 32 refrained earlier from participating in this cohort study, leaving 39 eligible, of whom 34 (87%) participated again (50% male, median age 48 years) with a median follow-up of 45 (range 40-52) years. Event-free survival was 50%, with supraventricular tachycardia (14%) and reintervention (13%) being the most frequent events, although less frequently in the last 10 years. At last follow-up, biventricular function was preserved in most patients. Reduced right and left ventricular ejection fraction (EF) was found in 33% and 13%, respectively. Exercise capacity and maximum rate of oxygen consumption were mildly impaired in 14% and 32% of patients. Patients who underwent an infundibulectomy during initial surgery were significantly more likely to undergo reintervention (HR=8.32, p=0.003). Patient-reported QoL scores remained stable over time and consistently exceeded those of the age-matched GDP. CONCLUSION:Fifty-year survival after surgery for pulmonary valve stenosis was excellent and comparable to the GDP. Most patients maintained preserved ventricular function, functional capacity and excellent QoL. Routine lifelong follow-up may not be necessary for all patients, but should be considered for those who underwent an infundibulectomy or have residual lesions.

Background Patients with adult congenital heart disease (ACHD) frequently develop late complications despite early surgical repair, including ventricular dysfunction and arrhythmias. In conditions with predominant right-sided pathology such as atrial septal defect (ASD), pulmonary stenosis (PS) and Tetralogy of Fallot (ToF), leading to right ventricular dilatation or systolic/diastolic dysfunction, ventricular interdependence may result in impaired left ventricular filling and left atrial (LA) dysfunction, despite the absence of primary left-sided disease. LA strain (LAS) has demonstrated prognostic value in acquired heart disease but its role in ACHD remains incompletely defined.Objective To evaluate the prognostic significance of LAS in adults with repaired right-sided CHD.Methods This single-centre longitudinal study includes patients with ACHD such as ASD, PS and ToF from the Quality of Life study at the Erasmus Medical Center. All patients underwent surgical repair during childhood between 1968 and 1980. Detailed cardiological evaluation, including STE-derived assessment of LA reservoir (LASr), conduit and contractile (LASct) function, was performed in 2012. Clinical outcomes were collected over a 10-year follow-up period, including mortality, reinterventions, heart failure and symptomatic tachyarrhythmias (supraventricular or ventricular) requiring treatment.Results A total of 195 patients were included (ASD=78 (40%); PS=45 (23%); ToF=72 (37%)), with a mean age of 40.7±5.2 years at the time of evaluation. Patients with ToF exhibited more frequent interventricular conduction abnormalities at baseline and had 2.7-fold higher odds of reaching the composite endpoint compared with patients with ASD. In Cox regression analyses, LASr and LASct were independently associated with symptomatic tachyarrhythmias (LASr: HR=0.952, p=0.013; LASct: HR=0.936, p=0.046), and these associations remained significant after adjustment for left ventricular ejection fraction, LA volume index or N-terminal pro B-type natriuretic peptide.Conclusion LAS provides independent prognostic information after surgical repair of right-sided CHD, particularly for predicting symptomatic tachyarrhythmias. Impaired LA deformation identifies patients at increased risk, underscoring the clinical relevance of LA mechanics in ACHD follow-up.

BACKGROUND:Women with left ventricular outflow tract obstruction (LVOTO) are at risk of adverse pregnancy outcomes, which have been related to the severity of the LVOTO. However, the physiological adaptations of pregnancy increase the gradient across an LVOTO, often altering the clinical perception of risk. Here, we examined how the LVOTO gradient changes during pregnancy, investigating whether a greater increase in the gradient is associated with worse pregnancy outcome and whether any increase in the gradient is transient, reversing after the end of the pregnancy. METHODS:The study was conducted at three tertiary centres. Data were collected from 125 pregnancies in 100 women with a range of causes of LVOTO, including bicuspid aortic valve (n=71), trileaflet aortic stenosis (n=23), prosthetic valve replacement (n=18) and subvalvular stenosis (n=8). Measurements included echocardiographic parameters (peak and mean LVOT gradients, left ventricular ejection fraction) and pregnancy outcomes. The primary endpoints were the change in gradient and pregnancy outcomes. RESULTS:The mean (19.5±11.9 mm Hg to 23.8±14.9 mm Hg) and peak (31.6±19.2 mm Hg to 40.6±27.7 mm Hg) gradients increased significantly during pregnancy and in a minority of cases this rise persisted postpartum. Pregnancy complications were unrelated to baseline gradient severity or the increase in gradient observed during pregnancy. Counter to our hypothesis, there was no association between the increase in gradient and adverse pregnancy outcomes. CONCLUSIONS:The physiological changes of pregnancy resulted in an increase in the gradient across the LVOTO, which was not associated with any adverse obstetric outcomes. In some cases, the increase in the gradient only partially reversed after pregnancy, implying that pregnancy may induce a structural change in the lesion, giving rise to LVOTO.

BACKGROUND:Cardiac resynchronisation therapy (CRT) is a key treatment for heart failure (HF) in acquired heart disease, but its benefits in adults with congenital heart disease and a systemic right ventricle (sRV) remain unclear. This study aimed to assess whether CRT improves outcomes in patients with sRV. METHODS:This is an international, retrospective study including patients >18 years from 33 centres with transposition of the great arteries (TGA) following atrial switch operation and congenitally corrected TGA. The primary endpoint included overall survival and survival free from HF. The secondary endpoint was a composite of death, hospitalisation for HF, heart transplant, mechanical support and ventricular tachycardia/implantable cardioverter-defibrillator therapies. RESULTS:We identified 105 out of 1721 patients (3.5%) who underwent CRT. Median follow-up after CRT implant was 4.6 (1.6-8) years. QRS improvement was limited to those with previous pacing (167±35 vs 154±28 ms; p=0.002). Following CRT, there was no significant change in B-type natriuretic peptide values, peak VO2 and tricuspid regurgitation severity by echocardiography. CRT complications occurred in 10 (9.5%), though they were usually minor. Patients with CRT were propensity-matched to controls according to age, sex, anatomy, presence of complex disease, previous HF and sRV dysfunction at baseline. At univariable analysis, CRT (HR 4.39-95%, CI 1.6 to 11.9; p=0.003), older age and moderate-to-severe sRV dysfunction at baseline were predictive of death, while CRT (HR 3-95%, CI 1.3 to 7; p=0.01) and sRV dysfunction were associated with HF admission. By multivariable analysis, CRT (HR 8.8-95%, CI 2.9 to 26.6; p=0.0001) and age (HR 1.1%-95%, CI 1.01 to 1.15; p<0.0001) were independently associated with poorer outcome. CONCLUSION:In this retrospective study in the largest population thus far described with an sRV, CRT implant was not associated with improved survival, even after controlling for key confounders.

Background Despite growing interest in male–female differences in cardiovascular disease, evidence in infective endocarditis (IE) is limited and contradictory.Methods This prospective study included all patients with definite or possible valvular IE discussed by the endocarditis team from 2016 to 2025. Baseline characteristics, diagnostics, treatment and outcomes were compared between sexes. A Cox model was conducted to assess survival adjusted for baseline characteristics, IE type and treatment.Results The cohort included 791 patients with definite or possible IE (72.8% males, 27.2% females). Age was not different (male: 67 (IQR 56–75), female: 71 (IQR 55–78) years, p=0.07). Females more often had hypertension (46.0% vs 35.2%, p=0.07) and mitral valve IE (45.6% vs 32.3%, p<0.001). Males had more predisposing conditions (71.5% vs 60.5%, p=0.004) and more aortic valve IE (71.0% vs 62.3%, p=0.02) and Cutibacterium species (5.7% vs 1.4%, p=0.007). There was no difference in indication for surgery. However, males were more often treated surgically (41.1% vs 29.3%, p=0.002), and females with an indication for surgery were more often treated conservatively (22.5% vs 37.0%, p=0.006). After a median follow-up of 2.1 (IQR 0.4–4.4) years, females had higher mortality (adjusted HR (aHR) 1.39 (95% CI 1.03 to 1.89), p=0.03). Surgery was associated with higher mortality in males (aHR 1.32 (95% CI 0.90 to 1.94) vs aHR 0.62 (95% CI 0.32 to 1.20), p value for interaction=0.03).Conclusions Differences were observed in baseline characteristics, IE type and survival. Females had worse overall adjusted survival, suggesting a less favourable overall prognosis. In terms of surgical decision-making, surgery was withheld more often in females despite surgical indication, and after excluding these patients, surgery appeared more protective in females than in males. These findings may reflect sex differences in surgical selection.

Aims:Sex-related differences in ascending aorta aneurysm (AscAA) pathology and the underlying mechanisms remain incompletely understood. In this study, sex-specific patterns of aortic media degeneration (MD) in idiopathic AscAA were investigated. Methods and Results:Aortic tissue and blood samples were obtained from 32 patients undergoing elective AscAA resection (mean age 65.6 years, 25% women). Two donor aortas served as healthy controls. Tissues were evaluated using a modified consensus scoring system assessing elastin integrity, mucoid extracellular matrix accumulation (MEMA), smooth muscle cell organization, and collagen remodelling. Immunohistochemistry was used to quantify extracellular matrix and remodelling markers. Outcome parameters included aortic diameter, immunohistochemical profiling, MD scores, and sex hormone profiling. Absolute ascending aortic diameters did not differ between sexes. Women exhibited significantly more advanced and diffuse MD across all sampled locations, with higher total MD scores before and after adjustment for aortic size. This was attributed to increased elastin degradation, MEMA, and elevated local MMP2 expression, consistent with a degradative remodelling profile. In contrast, men displayed a more focal and inflammatory phenotype, characterized by increased MMP9, cKIT, and CD68 expression, and relatively thicker media. Circulating hormone levels were reduced in both sexes but were not directly associated with MD severity. However, female-specific decreases in DHEA and male-specific decreases in testosterone, AMH, and androstenedione were observed. Conclusion:This study reveals pronounced sex-specific differences in AscAA wall degeneration. Women demonstrate a structurally more vulnerable aortic wall at comparable dilatation, fuelling discussion for revision of female treatment plans.

Cardiovascular disease (CVD) remains the leading cause of mortality in women, yet sex-specific risk factors are usually not included in conventional predictive models. Specifically, heart failure (HF) in women may be influenced by sex-specific hormones and pathologies that need to be addressed to improve prevention and treatment. This expert consensus statement aims to provide a comprehensive roadmap for HF prevention and management across specific conditions affecting women during their life-course. Each section focuses on the impact of a specific female condition on CVD and how to prevent and manage HF in specific settings: 1. Pregnancy with a specific focus on how to deal with hypertensive disorders in the acute and chronic setting and how to prevent and treat Peripartum Cardiomyopathy (PPCM); 2. Gynecological conditions predisposing to HF, such as Polycystic Ovary Syndrome (PCOS), endometriosis, and the menopausal transition. Emphasis is placed on chronic inflammation, metabolic dysfunction, and the "window of opportunity" for Menopausal Hormone Therapy (MHT); 3. Cardio-Oncology: mitigating Cancer Therapy-Related Cardiac Dysfunction (CTRCD) in breast and gynecological cancers, focusing on female-specific cardiotoxicity profiles, the importance of subclinical detection of cardiac dysfunction and the implementation of cardioprotective strategies (ACE-inhibitors, Beta-blockers, SGLT2 inhibitors) during cardiotoxic treatments. Lifestyle interventions such as the DASH diet and exercise-based rehabilitation are highlighted as essential for maintaining cardiac reserve.

Aims:To compare transthoracic echocardiography (TTE), 2D and 4D flow cardiovascular magnetic resonance (CMR) for the evaluation of valve function and flow measurements after percutaneous pulmonary valve implantation (PPVI). Methods and results:In this multicentre exploratory observational study, 29 patients who underwent PPVI were planned for TTE and CMR with 2D and 4D flow measurements after PPVI. Peak velocity was measured with all techniques. Net and regurgitating flow measurements were measured with CMR distal to the transcatheter pulmonary valve (TPV). Median peak velocity of the TPV with TTE was 2.5 (2.0-3.1) m/s, with 2D flow CMR 2.3 (2.0-3.2) m/s, and with 4D flow 2.3 (2.0-3.0) m/s. There was a good correlation for the peak velocity of the TPV between TTE and 2D flow CMR (r = 0.68, P < 0.001) and a moderate correlation for peak velocity between TTE and 4D flow CMR (r = 0.56, P = 0.003), and between 2D flow and 4D flow CMR (r = 0.56, P = 0.006). For peak velocity measurements, no proportional bias was observed between the three imaging techniques. For the CMR flow measurements, a strong correlation was observed between 2D and 4D flow CMR, for both net flow (r = 0.87, P < 0.001) and regurgitation fraction (r = 0.96, P < 0.001) of the TPV. Net flow through the left pulmonary artery correlated strongly between 2D and 4D flow CMR (r = 0.85, P < 0.001), and there was a good correlation for the right pulmonary artery (r = 0.65, P < 0.01). For net flow measurements of the TPV, we found an underestimation of flow measurements at higher flow rates with 4D flow CMR, compared to 2D flow CMR. Conclusion:TTE, 2D flow CMR, and 4D flow CMR are all feasible and reliable imaging techniques after PPVI, concordant in predominantly mild-to-moderate stenosis. These findings strengthen the use of 2D and 4D flow CMR as a tool for post-PPVI assessment, despite stent-induced artefacts, although reliability in more advanced valve dysfunction remains uncertain.

OBJECTIVES:Acute type A aortic dissection (ATAAD) carries high mortality, with emergency surgery being the cornerstone of treatment. The German Registry for Acute Type A Aortic Dissection (GERAADA)-score is advocated in guidelines to predict 30-day mortality after ATAAD surgery. This study investigates its performance in a Dutch cohort, with an emphasis on malperfusion definitions, age groups, and sex. METHODS:Adults undergoing emergency surgery for ATAAD at 5 Dutch centres (2007-2024) were included in a multicentre database. External validation of the GERAADA-score was performed with these data, using discrimination (area under the curve [AUC]) and calibration (Brier score, Hosmer-Lemeshow test, and calibration plots). A logistic regression with GERAADA variables was fitted on the study population, and assumptions were checked. Subgroup analyses were conducted based on sex, age groups, and malperfusion definitions (including imaging and clinical definitions). RESULTS:A total of 1,146 patients underwent emergency surgery for ATAAD. Observed early mortality was 16.9% (n = 194). Of 1,130 patients included in the external validation cohort, 92.2% had low-intermediate risk (GERAADA-score ≤ 30%). The GERAADA-score showed moderate discrimination (AUC = 0.649, 95% confidence interval = 0.604-0.694), with a higher AUC for younger patients (50-59 years). The malperfusion definition including ischaemia confirmed by imaging showed the best discriminative power. Calibration was good (Hosmer-Lemeshow, P = .754, Brier score = 0.131). Logistic regression identified age, catecholamine use, ventilation support, and coronary and peripheral malperfusion as independent risk factors for 30-day mortality, with signs of multicollinearity between preoperative catecholamine use and resuscitation. CONCLUSIONS:In the Dutch setting, the GERAADA-score demonstrated moderate discriminative power and good calibration across relevant subgroups. Adaptations of the GERAADA-score, including conducting a haemodynamic instability variable, may be considered to avoid redundant predictions and boost reproducibility.

OBJECTIVES:To investigate the optimal valve substitute for young women requiring aortic valve replacement (AVR), allowing improved future valve-related outcomes for mother and foetus during pregnancy. METHODS:A systematic search was performed for publications between 1998 and 2025 reporting women experiencing pregnancy after AVR with a pulmonary autograft (Ross-procedure), homograft, bioprosthesis (xenograft), or mechanical valve. Pooled proportions were calculated to determine maternal, valvular and foetal outcomes during pregnancy using generalized linear mixed models. RESULTS:Thirteen studies reporting 356 pregnancies in 251 women (pooled mean age at pregnancy 29.1 ± 4.8 years) after AVR with a pulmonary autograft (70 women, 119 pregnancies), homograft (73 women, 99 pregnancies), bioprosthesis (37 women, 50 pregnancies), or mechanical valve (71 women, 88 pregnancies) were included. During pregnancy, valve-related reintervention in women with a bioprosthesis was 2.7% (95% CI, 0.4-16.9) at 5.1 ± 2.5 years after AVR. This was not observed in women with pulmonary autografts (7.7 ± 4.2 years after AVR) and homografts (4.1 ± 3.3 years after AVR). Reintervention for valve thrombosis (4.9% [95% CI, 1.6-14.0]) and maternal death (1.1% [95% CI, 0.2-7.6]) occurred only in women with mechanical valves (8.1 ± 4.5 years after AVR). Pooled probability of liveborn delivery was 71.7% (95% CI, 59.2-81.6) in women with a mechanical valve, compared to 90.6% (95% CI, 72.4-97.3), 92.3% (95% CI, 56.0-99.1), and 82.9% (95% CI, 53.3-95.4) in women with an autograft, homograft, and bioprosthesis respectively. CONCLUSIONS:Maternal mortality and valve thrombosis during pregnancy occurred only in women with mechanical valves. Although no statistical comparisons were made, pregnancies in women with pulmonary autograft, homograft or bioprosthesis showed acceptable maternal and foetal outcomes. These descriptive findings provide foundations for further investigation of tissue-valve function before, during and after pregnancy, aiming for more support of current guidelines.