Dextrocardia in Adults with Congenital Heart Disease.

BACKGROUND/OBJECTIVES: Dextrocardia is rare in the general population, and may be associated with significant additional cardiac malformations. We aimed to identify the prevalence and patterns of additional cardiac defects, as well as the associated long-term morbidity and mortality, in adult patients with dextrocardia, in a specialised Adult Congenital Heart Disease (ACHD) service. METHODS: A retrospective study of patients with dextrocardia managed by our tertiary ACHD service, since January 2000, was performed. Medical records were reviewed and the National Death Index was consulted to confirm survival status. RESULTS: Of 3698 adults in our ACHD Service, 19 (0.5%) had dextrocardia. Mean follow-up duration was 7±7.5 years. The mean age at last review was 36.8±10.5 years (range 20-63 years). Situs was solitus in 14 (74%) and inversus in five (26%). Eleven patients (58%) had functional single ventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA) discordance and two had VA discordance only. Four patients with two ventricles had AV-VA discordance. All patients had at least one additional cardiac malformation. Fourteen patients (74%) required surgical intervention. Eleven patients (58%) underwent a Fontan-type operation. Five patients (26%) required ablation procedures for arrhythmia. One patient had infective endocarditis and two deaths occurred, both in patients who also had AV-VA discordance. CONCLUSION: Dextrocardia remains a rare finding in adults, even in a highly select group of patients with known congenital heart disease. Those with associated congenital heart abnormalities are likely to have complex lesions, which may require multiple surgical and medical interventions. Despite this, our series demonstrated that patients surviving to adulthood and then managed in an ACHD centre may have good medium-term survival.

What Is New in Spontaneous Coronary Artery Dissection?

Spontaneous coronary artery dissection (SCAD) is a condition that leads to tearing of the coronary vessel wall in the absence of trauma, iatrogenic injury, or atherosclerosis. SCAD is an important cause of myocardial infarction in young women, leading to significant cardiovascular morbidity and mortality. Within cohorts of women aged around 50 years on average, who experience acute coronary syndrome, the prevalence of SCAD is 22.5%- 35%. Over the past decade, SCAD research has expanded rapidly, leading to improved understanding of this condition. In this review, we provide a summary of the current body of knowledge, highlight areas of ongoing research, and identify existing knowledge gaps. Specifically, we provide a focused update on the pathogenesis of SCAD, including genetic and associated conditions, clinical presentation and diagnosis, prognosis, and short-term and long-term management. Highlighted areas include the following: insights from recent genome-wide association studies; intracoronary imaging for the diagnosis of SCAD; the role of cardiac computed tomography angiography to assess for vessel healing; revascularization strategies and challenges; cardiogenic shock in SCAD; and the increasingly recognized burden of anxiety, depression, and posttraumatic stress disorder among SCAD patients.

La dissection spontanée de l'artère coronaire (DSAC) est un trouble qui survient lorsque la paroi d'un vaisseau coronarien se déchire en l'absence de traumatisme, de lésion iatrogène ou d'athérosclérose. La DSAC est une cause importante d'infarctus du myocarde chez les jeunes femmes, menant à des taux importants de morbidité et de mortalité cardiovasculaires. Dans des cohortes de femmes âgées d'environ 50 ans, en moyenne, qui ont subi un syndrome coronarien aigu, la prévalence de DSAC était de 22,5 % à 35 %. Au cours de la dernière décennie, la recherche sur la DSAC s'est accélérée, permettant de mieux comprendre cette affection. Dans cette analyse, nous présentons un résumé du corpus de connaissances actuel, mettons l'accent sur les principaux domaines de la recherche en cours et cernons les lacunes à combler. Plus particulièrement, nous présentons une mise à jour ciblée sur la pathogenèse de la DSAC, comme les causes génétiques ou autres, les manifestations et le diagnostic cliniques, le pronostic ainsi que la prise en charge à court et à long terme. Les domaines mis de l'avant sont les suivants : résultats des récentes études d'association menées sur l'ensemble du génome; imagerie intracoronarienne pour le diagnostic de DSAC; rôle de l'angiotomodensitométrie cardiaque dans l'évaluation de la guérison des vaisseaux; stratégies et difficultés de revascularisation; choc cardiogène dans la DSAC; et fardeau croissant associé à l'anxiété, la dépression et le stress post-traumatique chez les patients atteints de DSAC.

Spontaneous coronary artery dissection (SCAD): A contemporary review.

Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of myocardial infarction that most frequently affects younger women, making it an important cause of morbidity and mortality within these demographics. The evolution of intracoronary imaging, improved diagnosis with coronary angiography, and ongoing research efforts and attention via social media, has led to increasing recognition of this previously underdiagnosed condition. In this review, we provide a summary of the current body of knowledge, as well as focused updates on the pathogenesis of SCAD, insights on genetic susceptibility, contemporary diagnostic tools, and immediate, short- and long-term management.

Prevalence and prognostic impact of tricuspid regurgitation in patients with cardiac implantable electronic devices: From the national echocardiography database of Australia.

AIMS: We sought to analyse the distribution of TR severity and survival in a large cohort of adults with CIED leads. METHODS: The distribution of TR severity was analysed in 18,797 adults (mean age 73.8+/-13.9, 63.3% men) with CIED leads undergoing echocardiography across 25 centres. Survival status and cause of death were linked and the relationship between TR severity and mortality during 2.6 (interquartile range 1.1-4.6) years follow-up examined. Data from 439,558 individuals (mean age 62.1 ±17.8 years, 51.5% men) without a CIED were subsequently incorporated in a pooled cohort analysis. RESULTS: Overall, 8,824/18,797 individuals (47%) with a CIED had no/trivial TR; 5,490 (29.2%) mild TR; 3,068 (16.3%) moderate TR; and 1,415 (7.5%) severe TR. Moderate or greater TR was independently associated with age, female sex, atrial fibrillation and significant left heart disease (p<0.001 for all). 8,868 individuals (47.2%) died from any cause (43.2% from cardiovascular causes). Individuals with moderate or severe TR had a 1.6 to 2.5-fold increased risk of all-cause mortality in adjusted models, compared to those with no TR (p<0.001 for both). In the pooled cohort analysis, CIEDs were associated with a near 2-fold (95% CI 1.81-1.99; p<0.001) increased probability of moderate or greater TR, on adjusted basis. However, the mortality associated with moderate or greater TR did not differ significantly with respect to the presence or absence of a device lead. CONCLUSIONS: Moderate or greater TR is more prevalent in those with CIED's, even in adjusted models, and was independently associated with incremental risks for all-cause and cardiovascular mortality.

Prevalence and determinants of tricuspid regurgitation after repair of tetralogy of Fallot.

BACKGROUND: The prevalence and determinants of tricuspid regurgitation (TR) in patients with repaired Tetralogy of Fallot (rTOF) remain incompletely understood. OBJECTIVES: To explore the prevalence of and factors associated with TR in patients with rTOF, specifically, the relationship of right ventricular (RV) dilatation with TR severity. METHODS: Patients (≥17 yrs) with rTOF referred to our service (2000-2019) were identified. Those with severe pulmonary stenosis, significant shunt, or previous tricuspid valve surgery were excluded. Using standard cardiac MRI protocols, RV, right atrial (RA) and tricuspid valve (TV) parameters were measured and compared. RESULTS: 68 consecutively eligible patients with rTOF were included in the study (27 ± 9 yrs., 35% female). Despite substantial RV volume overload (mean RVEDVi 153 mL/m2), the majority of the cohort (78%) had no or only mild TR. RA volumes, tenting height/area and annular diameter were normal (4.9 ± 2.0 mm, 1.1 ± 1.0 cm2 and 32.4 ± 6.2 mm, respectively). There was no significant correlation of TR fraction with RVEDVi (r = 0.13; p = 0.30), RVEF (r = 0.09; p = 0.44) or tricuspid annular diameter (r = 0.07; p = 0.62). Only RAVi showed a weak but significant correlation with TR fraction (0.29; p = 0.03). In a pooled cohort analysis, including both rTOF patients and adults with a dilated RV from pre-tricuspid shunt lesions, only rTOF was independently associated with higher TR fraction (p = 0.017). CONCLUSION: Despite substantial RV dilatation in a cohort with rTOF, there was surprisingly little TR. We found poor correlation between RVEDVi, RA volumes, tricuspid annular dilatation and the presence of significant TR. These findings question commonly held notions regarding the pathophysiology of functional TR in these patients.

Longitudinal assessment of structural phenotype in Brugada syndrome using cardiac magnetic resonance imaging.

Background: Despite historically being considered a channelopathy, subtle structural changes have been reported in Brugada syndrome (BrS) on histopathology and cardiac magnetic resonance (CMR) imaging. It is not known if these structural changes progress over time. Objective: The study sought to assess if structural changes in BrS evolve over time with serial CMR assessment and to investigate the utility of parametric mapping techniques to identify diffuse fibrosis in BrS. Methods: Patients with a diagnosis of BrS based on international guidelines and normal CMR at least 3 years prior to the study period were invited to undergo repeat CMR. CMR images were analyzed de novo and compared at baseline and follow-up. Results: Eighteen patients with BrS (72% men; mean age at follow-up 47.4 ± 8.9 years) underwent serial CMR with an average of 5.0 ± 1.7 years between scans. No patients had late gadolinium enhancement (LGE) on baseline CMR, but 4 (22%) developed LGE on follow-up, typically localized to the right ventricular (RV) side of the basal septum. RV end-systolic volume increased over time (P = .04) and was associated with a trend toward reduction in RV ejection fraction (P = .07). Four patients showed a reduction in RV ejection fraction >10%. There was no evidence of diffuse myocardial fibrosis observed on parametric mapping. Conclusions: Structural changes may evolve over time with development of focal fibrosis, evidenced by LGE on CMR in a significant proportion of patients with BrS. These findings have implications for our understanding of the pathological substrate in BrS and the longitudinal evaluation of patients with BrS.

Adverse Prognostic Impact of Even Mild or Moderate Tricuspid Regurgitation: Insights from the National Echocardiography Database of Australia.

BACKGROUND: The prevalence and prognostic impact of tricuspid regurgitation (TR) remain incompletely characterized. METHODS: The distribution of TR severity was analyzed in 439,558 adults (mean age, 62.1 ± 17.8 years; 51.5% men) being investigated for heart disease, from 2000 to 2019, by 25 centers contributing to the National Echocardiography Database of Australia. Survival status and cause of death were ascertained in all adults from the National Death Index of Australia. The relationship between TR severity and mortality was examined. RESULTS: Of those studied, 311,604 (70.9%) had no/trivial TR; 94,172 (21.4%), mild TR; 26,056 (5.9%), moderate TR; and 7,726 (1.8%), severe TR. During a median 4.1 years (interquartile range, 2.2-7.0 years) of follow-up, 109,004 died (49% from cardiovascular causes). Moderate or greater TR was associated with older age and female sex (P < .001). Individuals with moderate and severe TR had a 2.0- to 3.2-fold increased risk of all-cause long-term mortality after adjustment for age and sex compared with those with no/trivial TR (P < .001 for both comparisons). Even those with mild TR had a significantly increased risk for mortality (hazard ratio [HR] = 1.29; 95% CI, 1.27-1.31). In fully adjusted models, including for RV systolic pressure, atrial fibrillation, and significant left heart disease, there remained a 1.24- to 2.65-fold increased risk of mortality with mild (HR = 1.24; 95% CI, 1.23-1.26), moderate (HR = 1.72; 95% CI, 1.68-1.75), or severe TR (HR = 2.65; 95% CI, 2.57-2.73), compared with those with no/trivial TR (P < .001 for all). CONCLUSIONS: Tricuspid regurgitation is a common condition in adults referred for echocardiography. Moreover, even in the presence of other cardiac disease, increasing grades of TR are independently associated with increasing risks of cardiovascular and all-cause mortality. Furthermore, we show that even mild TR is independently associated with a significant increase in mortality.

Right ventricular volume and its relationship to functional tricuspid regurgitation.

BACKGROUND: Significant right ventricular (RV) dilatation has long been considered integral to the pathogenesis of functional tricuspid regurgitation (FTR). OBJECTIVES: To explore the relationship of RV dilatation and FTR in patients with 'pure' RV volume overload. METHODS: Patients (>17yrs) with RV dilatation due to pre-tricuspid shunts (atrial septal defect; ASD and/or partial anomalous pulmonary venous drainage; PAPVD) referred to our service (2000-2019) were retrospectively identified. Those with pulmonary hypertension, primum ASD or left-heart disease were excluded. Using standard cardiac MRI protocols, RV, right atrial and TV parameters were measured and compared. RESULTS: Of 52 consecutively eligible patients (42 ± 15yrs, 25 males), 25 had ASDs, 13 had PAPVD and 14 had both conditions. All were in sinus rhythm and none had pulmonary regurgitation. Left and right ventricular ejection fractions were normal (LVEF 63 ± 8%, RVEF 56 ± 8%). Indexed RV end-diastolic volumes (RVEDVi) were moderately increased (males 148 ± 33 mL/m2 and females 141 ± 42 mL/m2, range 95-267 mL/m2). Despite substantial RV volume overload, no patients had severe tricuspid regurgitation (TR). Only two had > mild TR. There was a weak correlation between tricuspid annular diameter and both degree of RV dilatation (r = 0.37; p = 0.01) and degree of TR (r = 0.38; p = 0.006). There was a similarly poor correlation between right atrial dimensions and the degree of TR (r = 0.34; p = 0.02). CONCLUSION: When RV dilatation is simply due to volume overload, we find that significant TR is extremely rare. This gives an important and novel insight; that RV dilatation per se does not result in FTR.

Long-term importance of right ventricular outflow tract patch function in patients with pulmonary regurgitation.

OBJECTIVE: Chronic pulmonary regurgitation (PR) has deleterious effects on right ventricular (RV) function in repaired tetralogy of Fallot (ToF). However, there are little data regarding right ventricular outflow tract (RVOT) contractile dysfunction in response to chronic PR and on both RV and LV volumes and function. METHODS: We retrospectively identified consecutive patients with PR who were referred for magnetic resonance imaging quantification of "free PR" detected on echocardiography between 2003 and 2008. Patients had ToF and a transannular patch procedure (n = 30, 25.1 ± 1.2 years) or PR resulting from valvar pulmonary stenosis treated with surgical or percutaneous valvotomy (n = 30, 26.6 ± 1.8 years). RESULTS: The ToF and the PS groups were well matched for age at scan, age at repair surgery in ToF or initial valvotomy in PS, duration of exposure to PR, body surface area, heart rate, PR fraction, net forward pulmonary artery flow, and main and branch pulmonary artery dimensions. Severe PR fractions were identified in both groups (ToF: 40% ± 1% vs PS: 37% ± 2%, P = .2). Indexed RV and LV end-diastolic volumes were similar for both ToF and PS groups (RV end-diastolic volume index: 137 ± 6 mL/m(2) vs 128 ± 5 mL/m(2), P = .2, and LV end-diastolic volume index: 72 ± 2 mL/m(2) vs 67 ± 2 mL/m(2), P = .1, respectively). RV mass was also similar between groups (95 ± 5 g vs 81 ± 6 g, respectively, P = .08). However, indexed RV and LV end-systolic volumes were consistently higher in ToF when compared with PS (RV end-systolic volume index: 70 ± 5 mL/m(2) vs 54 ± 3 mL/m(2), P < .01, and LV end-systolic volume index: 29 ± 1 mL/m(2) vs 22 ± 1 mL/m(2), P < .01, respectively). These changes were reflected in lower biventricular systolic function in patients with ToF when compared with PS (RV ejection fraction: 52% ± 1.5% vs 59% ± 1%, P < .001, and LV ejection fraction: 61% ± 1% vs 67 ± 1%, P < .001, respectively). Although RV transannular plane systolic excursion was not significantly different between the groups (P = .86), the RV outflow tract was considered contractile in only 50% of patients with ToF compared with 93% of patients with PS (P = .0004). RV volumes and function were similar when only patients with contractile RV outflow tracts were compared. CONCLUSIONS: RV outflow tract patch dysfunction in repaired ToF is responsible for higher end-systolic volumes and thus lower global measures of ventricular systolic function. These findings were not evident in cases of PS treated with valvotomy with comparable amount of PR. These observations highlight the importance of the initial repair surgery in ToF for late outcomes.