Katrijn Jansen

Differential left ventricular remodelling and longitudinal function distinguishes low flow from normal-flow preserved ejection fraction low-gradient severe aortic stenosis

AIMS There is uncertainty in identifying patients with severe aortic stenosis (AS) with preserved left ventricular (LV) ejection fraction, low flow, and low gradients (LFLG). Prior studies propose that these patients demonstrate significant concentric remodelling and decreased survival, while others suggest that they have features and survival similar to moderate AS. METHODS AND RESULTS We compared the clinical characteristics, echocardiographic features, and overall survival of LFLG AS patients (n = 38) to those with normal-flow, low-gradient (NFLG) severe AS (n = 75) and moderate AS (n = 70). Low-flow, low-gradient patients had the lowest end-diastolic volume index (43 vs. 54 vs. 54 mL/m², P < 0.001), highest relative wall thickness (RWT) (60 vs. 49 vs. 48%, P < 0.001), and lowest septal mitral annular displacement (1.0 vs. 1.5 vs. 1.5 cm, P < 0.001). New York Heart Association (NYHA) class III/IV symptoms were the most frequent in the LFLG group (29 vs. 11 vs. 3%, P < 0.001). Survival at 3 years was significantly lower in LFLG compared with NFLG (P = 0.006) and moderate AS (P = 0.002), but not different between NFLG and moderate AS (P = 0.49). Higher NYHA classification (HR 1.77, 95% CI 1.22-2.57), RWT > 50% (HR 3.28, 95% CI 1.33-8.1), and septal displacement <1.1 cm (HR 3.93, 95% CI 1.96-7.82) but not low flow were independent predictors of survival in Cox proportional hazards analysis. CONCLUSION Preserved ejection fraction, LFLG AS patients exhibit marked concentric remodelling and impaired longitudinal functional-features that predict their poor long-term survival. Normal-flow, low-gradient AS patients have outcomes similar to moderate AS.

A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot

Objective To determine the association of pulmonary valve replacement (PVR) with death and sustained ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTOF). Methods Subjects with rTOF and cardiac magnetic resonance from an international registry were included. A PVR propensity score was created to adjust for baseline differences. PVR consensus criteria were predefined as pulmonary regurgitation >25% and ≥2 of the following criteria: right ventricular (RV) end-diastolic volume >160 mL/m2, RV end-systolic volume >80 mL/m2, RV ejection fraction (EF) <47%, left ventricular EF <55% and QRS duration >160 ms. The primary outcome included (aborted) death and sustained VT. The secondary outcome included heart failure, non-sustained VT and sustained supraventricular tachycardia. Results In 977 rTOF subjects (age 26±15 years, 45% PVR, follow-up 5.3±3.1 years), the primary and secondary outcomes occurred in 41 and 88 subjects, respectively. The HR for subjects with versus without PVR (time-varying covariate) was 0.65 (95% CI 0.31 to 1.36; P=0.25) for the primary outcome and 1.43 (95% CI 0.83 to 2.46; P=0.19) for the secondary outcome after adjusting for propensity and other factors. In subjects (n=426) not meeting consensus criteria, the HR for subjects with (n=132) versus without (n=294) PVR was 2.53 (95% CI 0.79 to 8.06; P=0.12) for the primary outcome and 2.31 (95% CI 1.07 to 4.97; P=0.03) for the secondary outcome. Conclusion In this large multicentre rTOF cohort, PVR was not associated with a reduced rate of death and sustained VT at an average follow-up of 5.3 years. Additionally, there were more events after PVR compared with no PVR in subjects not meeting consensus criteria.

Clinical features and prognosis of patients with isolated severe aortic stenosis and valve area less than 1.0 cm2

Objective Current guidelines define severe aortic stenosis (AS) as an aortic valve area (AVA)≤1.0 cm2, but some authors have suggested that the AVA cut-off be decreased to 0.8 cm2. The aim of this study was, therefore, to better describe the clinical features and prognosis of patients with an AVA of 0.8–0.99 cm2. Methods Patients with isolated, severe AS and ejection fraction ≥55% with an AVA of 0.8–0.99 cm2 (n=105) were compared with those with an AVA<0.8 cm2 (n=155) and 1.0–1.3 cm2 (n=81). The endpoint of this study was a combination of death from any cause or aortic valve replacement at or before 3 years. Results Patients with an AVA of 0.8–0.99 cm2 group comprised predominantly normal-flow, low-gradient (NFLG) AS, while high gradients and low flow were more often observed with an AVA<0.8 cm2. The frequency of symptoms was not significantly different between an AVA of 0.8–0.99 cm2 and 1.0–1.3 cm2. The combined endpoint was achieved in 71%, 52% and 21% of patients with an AVA of 0.8 cm2, 0.8–0.99 cm2and 1.0–1.3 cm2, respectively (p<0.001). Among patients with an AVA of 0.8–0.99 cm2, NFLG AS was associated with a lower hazard (HR=0.40, 95% CI 0.23 to 0.68, p=0.001) of achieving the combined endpoint with outcomes similar to moderate AS in the first 1.5 years of follow-up. Patients with high-gradient or low-flow AS with an AVA of 0.8–0.99 cm2 had outcomes similar to those with an AVA<0.8 cm2. The sensitivity for the combined endpoint was 61% for an AVA cut-off of 0.8 cm2 and 91% for a cut-off of 1.0 cm2. Conclusions The outcomes of patients with AS with an AVA of 0.8–0.99 cm2 are variable and are more precisely defined by flow-gradient status. Our findings support the current AVA cut-off of 1.0 cm2.

Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review

OBJECTIVES Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population. METHODS We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death. RESULTS A total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease. CONCLUSIONS Women with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

LOW STROKE VOLUME IN PATIENTS WITH SEVERE AORTIC STENOSIS IS ASSOCIATED WITH ADVERSE LEFT VENTRICULAR REMODELING

The degree of left ventricular (LV) remodeling due to pressure overload varies significantly in patients with severe aortic stenosis (AS). Worsening compensatory adaptation of the left ventricle to pressure load may be manifested by differences in LV remodeling which results in decreased stroke

Left atrial volumes: should we reset the reference standard?

Left atrial (LA) volume has been shown to be a prognostic indicator of cardiovascular (CV) events and a marker of diastolic dysfunction [(1,2)][1]. Normative values for indexed LA volume (LAVi) have been established to be 22 ± 6 ml/m2 [(3)][2]. Yet, these values are on the basis of small sample