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Stroke in Adults With Coarctation of the Aorta: A National Population-Based Study.

J Am Heart Assoc. 2018 Jun 01;7(11):

Authors: Pickard SS, Gauvreau K, Gurvitz M, Gagne JJ, Opotowsky AR, Jenkins KJ, Prakash A

Abstract
BACKGROUND: Adults with repaired coarctation of the aorta (CoA) have reduced long-term survival compared with the general population. This study aimed to determine whether CoA is independently associated with premature ischemic and hemorrhagic stroke in the contemporary era.
METHODS AND RESULTS: This was a cross-sectional study utilizing the National Inpatient Sample database from 2005 to 2014. We hypothesized that patients with CoA are hospitalized with ischemic and hemorrhagic stroke at a younger age compared with the general population. To test this hypothesis, we compared the age at stroke in patients with and without a diagnosis of CoA using simple and multivariable weighted linear regression. Among 4 894 582 stroke discharges, 207 had a diagnosis of CoA. Patients with CoA had strokes at significantly younger age compared with patients without CoA: 18.9 years younger for all-cause stroke (P<0.001), 15.9 years younger for ischemic stroke (P<0.001), and 28.5 years younger for hemorrhagic stroke (P<0.001), after adjusting for potential confounders. There was no significant difference in the proportion of ischemic strokes between those with and without CoA (79.2% versus 83.0%, P=0.50). However, CoA patients had a higher proportion of subarachnoid hemorrhage (11.8% versus 4.8%, P=0.039) than those without CoA. Among patients who had a hemorrhagic stroke, the prevalence of unruptured intracranial aneurysms was higher in patients with CoA compared with those without CoA (23.3% versus 2.5%, P=0.002).
CONCLUSIONS: Patients with CoA have both ischemic and hemorrhagic strokes at significantly younger ages compared with the general population.

PMID: 29858370 [PubMed - in process]

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Risk of thromboembolic complications in adult congenital heart disease: A literature review.

Arch Cardiovasc Dis. 2018 May 30;:

Authors: Karsenty C, Zhao A, Marijon E, Ladouceur M

Abstract
Adult congenital heart disease (ACHD) is a constantly expanding population with challenging issues. Initial medical and surgical treatments are seldom curative, and the majority of patients still experience late sequelae and complications, especially thromboembolic events. These common and potentially life-threating adverse events are probably dramatically underdiagnosed. Better identification and understanding of thromboembolic risk factors are essential to prevent long-term related morbidities. In addition to specific situations associated with a high risk of thromboembolic events (Fontan circulation, cyanotic congenital heart disease), atrial arrhythmia has been recognized as an important risk factor for thromboembolic events in ACHD. Unlike in patients without ACHD, thromboembolic risk stratification scores, such as the CHA2DS2-VASc score, may not be applicable in ACHD. Overall, after a review of the scientific data published so far, it is clear that the complexity of the underlying congenital heart disease represents a major risk factor for thromboembolic events. As a consequence, prophylactic anticoagulation is indicated in patients with complex congenital heart disease and atrial arrhythmia, regardless of the other risk factors, as opposed to simple heart defects. The landscape of ACHD is an ongoing evolving process, and specific thromboembolic risk scores are needed, especially in the setting of simple heart defects; these should be coupled with specific trials or long-term follow-up of multicentre cohorts.

PMID: 29859704 [PubMed - as supplied by publisher]

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Impact of Percutaneous Pulmonary Valve Implantation on the Timing of Reintervention for Right Ventricular Outflow Tract Dysfunction.

Rev Esp Cardiol (Engl Ed). 2018 May 30;:

Authors: de Torres-Alba F, Kaleschke G, Baumgartner H

Abstract
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Early surgical repair has dramatically improved the outcome of this condition. However, despite the success of contemporary approaches with early complete repair, these are far from being curative and late complications are frequent. The most common complication is right ventricle outflow tract (RVOT) dysfunction, affecting most patients in the form of pulmonary regurgitation, pulmonary stenosis, or both, and can lead to development of symptoms of exercise intolerance, arrhythmias, and sudden cardiac death. Optimal timing of restoration of RVOT functionality in asymptomatic patients with RVOT dysfunction after TOF repair is still a matter of debate. Percutaneous pulmonary valve implantation, introduced almost 2 decades ago, has become a major game-changer in the treatment of RVOT dysfunction. In this article we review the pathophysiology, the current indications, and treatment options for RVOT dysfunction in patients after TOF repair with a focus on the role of percutaneous pulmonary valve implantation in the therapeutic approach to these patients.

PMID: 29859895 [PubMed - as supplied by publisher]

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Left atrial flutter after implantation of atrial septal occluder.

Europace. 2018 May 31;:

Authors: Zhao A, Karsenty C, Ladouceur M

PMID: 29860385 [PubMed - as supplied by publisher]

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The analysis of heterotaxy patients reveals new loss-of-function variants of GRK5.

Sci Rep. 2016 09 13;6:33231

Authors: Lessel D, Muhammad T, Casar Tena T, Moepps B, Burkhalter MD, Hitz MP, Toka O, Rentzsch A, Schubert S, Schalinski A, Bauer UM, Kubisch C, Ware SM, Philipp M

Abstract
G protein-coupled receptor kinase 5 (GRK5) is a regulator of cardiac performance and a potential therapeutic target in heart failure in the adult. Additionally, we have previously classified GRK5 as a determinant of left-right asymmetry and proper heart development using zebrafish. We thus aimed to identify GRK5 variants of functional significance by analysing 187 individuals with laterality defects (heterotaxy) that were associated with a congenital heart defect (CHD). Using Sanger sequencing we identified two moderately frequent variants in GRK5 with minor allele frequencies <10%, and seven very rare polymorphisms with minor allele frequencies <1%, two of which are novel variants. Given their evolutionarily conserved position in zebrafish, in-depth functional characterisation of four variants (p.Q41L, p.G298S, p.R304C and p.T425M) was performed. We tested the effects of these variants on normal subcellular localisation and the ability to desensitise receptor signalling as well as their ability to correct the left-right asymmetry defect upon Grk5l knockdown in zebrafish. While p.Q41L, p.R304C and p.T425M responded normally in the first two aspects, neither p.Q41L nor p.R304C were capable of rescuing the lateralisation phenotype. The fourth variant, p.G298S was identified as a complete loss-of-function variant in all assays and provides insight into the functions of GRK5.

PMID: 27618959 [PubMed - indexed for MEDLINE]

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Stenting of the ascending aorta revisited.

Catheter Cardiovasc Interv. 2017 Oct 01;90(4):626-630

Authors: Moiduddin NJ, Rios R, El-Said H, Moore JW

Abstract
Despite few institutions stenting the ascending aorta, it has been discouraged because of the proximity of the aortic valve, the coronary artery orifices, and the aortic arch branches. We describe a small case series of patients having acquired stenosis of the ascending that was relieved successfully by stenting. © 2017 Wiley Periodicals, Inc.

PMID: 28471087 [PubMed - indexed for MEDLINE]

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Outcomes of pulmonary arterial hypertension therapy in Australia: is monotherapy adequate?

Intern Med J. 2017 Oct;47(10):1124-1128

Authors: Moonen A, Garsia R, Youssef P, Torzillo P, Corte T, Boehm C, Cordina R, Celermajer D, Lau E

Abstract
BACKGROUND: In Australia, government-subsidised treatment of pulmonary arterial hypertension (PAH) is limited to monotherapy. Recent international guidelines advocate that initial combination therapy be considered for all symptomatic PAH patients.
AIM: To characterise 'real-life' outcomes in PAH patients initiated on monotherapy.
METHODS: We performed a retrospective analysis of 100 consecutive PAH patients at a single centre who were commenced on monotherapy for PAH between 2004 and 2015. The composite clinical end-point of 'treatment failure' was prospectively defined as (i) >15% fall in 6-min walk distance (6MWD) on follow up, (ii) physician judgement of inadequate treatment response, (iii) adverse drug effect requiring cessation and (iv) death or transplantation.
RESULTS: At initiation of therapy, mean age was 54 ± 18 years, and underlying diagnoses included idiopathic (36%), connective tissue disease-associated (37%) and congenital heart disease-associated-PAH (25%). Baseline 6MWD was 360 ± 140 m, and 75% were in either the New York Heart Association functional classes III or IV. Over a median follow up of 38 months (interquartile range 20-67), 62% of the subjects met the criteria for a clinical failure event. Median time to monotherapy failure was 24 months (95% confidence interval 14-34), with death or transplantation being the most common clinical failure event. Estimated 1-, 3- and 5-year survival rates from time of treatment initiation were 92, 75 and 66%.
CONCLUSION: The majority of patients failed initial monotherapy therapy within 2 years of treatment initiation. Broader access to approved PAH agents is needed to enable combination therapy in line with evidence-based international guidelines.

PMID: 28560817 [PubMed - indexed for MEDLINE]

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Ischemic heart disease in children and young adults with congenital heart disease in Sweden.

Int J Cardiol. 2017 Dec 01;248:143-148

Authors: Fedchenko M, Mandalenakis Z, Rosengren A, Lappas G, Eriksson P, Skoglund K, Dellborg M

Abstract
BACKGROUND: An increasing proportion of congenital heart disease (CoHD) patients survive to an age associated with increased risk of developing ischemic heart disease (IHD). The aim was to investigate the risk of developing IHD among children and young adults with CoHD.
METHODS: Using the Swedish National Patient Register, we created a cohort of all CoHD patients born between January 1970 and December 1993. Ten controls matched for age, sex, county were randomly selected from the general population for each patient (n=219,816). Patients and controls were followed from birth until first IHD event, death, or December 31, 2011.
RESULTS: We identified 21,982 patients with CoHD (51.6% men), mean follow-up was 26.4 (21.2-33.9) years. CoHD patients had 16.5 times higher risk of being hospitalized with or dying from IHD compared to controls (95% CI: 13.7-19.9), p<0.0001. Patients with conotruncal defects and severe nonconotruncal defects, had the highest IHD incidence rate (71.1 and 56.3 cases per 100,000 person-years, respectively, compared to 2.9 and 2.3 in controls). Hypertension and diabetes were less common among CoHD patients with IHD than among controls with IHD (hypertension 9.7% vs 19.7%, diabetes 1.8% vs 7.7% in CoHD patients and controls). Patients with aortic coarctation did not have a specific increase in the risk of developing IHD or acute myocardial infarction.
CONCLUSIONS: In this large case-control cohort study, the relative risk of developing IHD was markedly higher in CoHD patients than in controls. However, the absolute risk was low in both groups.

PMID: 28705603 [PubMed - indexed for MEDLINE]

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Abnormal right atrial performance in repaired tetralogy of Fallot: A CMR feature tracking analysis.

Int J Cardiol. 2017 Dec 01;248:136-142

Authors: Kutty S, Shang Q, Joseph N, Kowallick JT, Schuster A, Steinmetz M, Danford DA, Beerbaum P, Sarikouch S

Abstract
BACKGROUND: We hypothesized that right atrial (RA) performance is abnormal in repaired tetralogy of Fallot (TOF).
METHODS: TOF patients were prospectively enrolled for cardiovascular magnetic resonance (CMR), echocardiography and exercise stress following a standardized 14-center protocol. Peak RA longitudinal strain (RALS) and right ventricular longitudinal strain (RVLS) were measured using CMR feature tracking (FT) and correlated to RA and RV end diastolic volumes (EDVi) and ejection fraction (EF).
RESULTS: The cohort had 311 subjects: 171 TOF (94 male, age 18.2±8years) and 140 healthy controls (69 male, 16.4±11years). RAEDVi, RALS, RVEDVi, RVLS, RAEF, and RVEF in TOF were 60.8±17.1ml/m2, 13.6±5.7%, 120.3±30.3ml/m2, 12.3±4.2%, 32.5±9.9% and 51.2±8.4% and differed from respective indices in controls: 51.7±15.7ml/m2, 27±10.1%, 74±19.0ml/m2, 18.5±5.3%, 54±8% and 62.5±5.5% (p<0.001). RAEDVi and RALS correlated with RVLS (p=0.004, <0.001, r=0.2,0.3). RAEDVi was higher in older TOF, while RALS did not increase with age. RAEDVi but not RALS correlated with RV systolic pressure(r=0.2, 0). Neither RAEDVi nor RALS was associated with tricuspid regurgitation grade or peak oxygen uptake (r=0.1, 0). Positive correlation was observed for RVEDVi with RAEDVi (p=0.035, r=0.2) and a trend toward negative correlation with RALS (p=0.09, r=0.1).
CONCLUSION: RALS, RAEDVi and RAEF are abnormal in TOF. Reduced RALS indicates decreased RA reservoir function. Because they correlate with other functional RV indices, these abnormalities may represent RA diastolic burden from chronic RV dysfunction in TOF. The young cohort age might explain the absence of RALS correlation to tricuspid regurgitation and peak oxygen uptake.

PMID: 28712562 [PubMed - indexed for MEDLINE]

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Longitudinal sequential biventricular assessment in adults with transposition of the great arteries and relationship with adverse outcomes.

Int J Cardiol. 2017 Dec 01;248:131-135

Authors: Riahi M, Claman A, Kiess M, Orgad M, Human D, Chakrabarti S, Leipsic J, Grewal J

Abstract
BACKGROUND: In a cohort of congenitally corrected transposition of the great arteries (cc-TGA) and transposition of the great arteries after atrial switch procedure (d-TGA) the study objectives were: 1) to assess the change of quantitative systemic right ventricle (sRV) parameters over time and; 2) to examine the relationship of quantitative sRV parameters with adverse clinical outcomes.
METHODS AND RESULTS: Single-center cohort study that included 49 (39%) cc-TGA and 76 (61%) d-TGA patients >18years who had at least one MUGA sRV assessment, 18/39 had more than one respectively. The primary clinical endpoint was all-cause mortality, heart transplantation and/or heart failure hospitalization. At a median clinical follow-up of 7years following the first MUGA, the primary endpoint occurred more often in cc-TGA versus d-TGA patients (18 (36.7%) vs. 9 (11.8%), p=0.03). Median time between the MUGA assessments was 5.8 (cc-TGA) and 4.9years (d-TGA). At last MUGA follow-up: 6 (33%) cc-TGA/14 (36%) d-TGA patients showed a significant decline in sRVEF (>5%); 6 (33%) cc-TGA/17 (44%) d-TGA patients had a significant increase in sRVEDVi; and 7 (39%) cc-TGA/19 (49%) PA-TGA patients had a significant increase in sRVESVi. Baseline sRV parameters were not associated with the primary end point or sRV changes over time.
CONCLUSIONS: An important proportion of both patient cohorts demonstrated a significant change in sRV parameters over time and these are likely related to multiple factors that vary between individuals given population heterogeneity. The TGA patients have distinct clinical trajectories with increased adverse heart failure outcomes in the cc-TGA population and sRV parameters were not related to adverse heart failure events in either group.

PMID: 28818352 [PubMed - indexed for MEDLINE]

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