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Long-Term Survival of Individuals Born With Congenital Heart Disease: A Systematic Review and Meta-Analysis.

J Am Heart Assoc. 2016 Jun 16;5(6):

Authors: Best KE, Rankin J

BACKGROUND: Estimates of long-term survival are required to adequately assess the variety of health and social services required by those with congenital heart disease (CHD) throughout their lives.
METHODS AND RESULTS: Medline, Embase, and Scopus were searched from inception to June 2015 using MeSH headings and keywords. Population-based studies that ascertained all persons born with CHD within a predefined area and reported survival estimates at ≥5 years were included. Unadjusted survival estimates for each CHD subtype at ages 1 year, 5 years, 10 years, and so forth were extracted. Pooled survival estimates for each age were calculated using meta-analyses. Metaregression was performed to examine the impact of study period on survival. Of 7840 identified articles, 16 met the inclusion criteria. Among those with CHD, pooled 1-year survival was 87.0% (95% CI 82.1-91.2), pooled 5-year survival was 85.4% (95% CI 79.4-90.5), and pooled 10-year survival was 81.4% (95% CI 73.8-87.9). There was significant heterogeneity of survival estimates among articles (P<0.001 for 1-, 5-, and 10-year survival). A more recent study period was significantly associated with greater survival at ages 1 year (P=0.047), 5 years (P=0.013), and 10 years (P=0.046). Survival varied by CHD subtype, with 5-year survival being greatest for those with ventricular septal defect (96.3%, 95% CI 93.7-98.2) and lowest for those with hypoplastic left heart (12.5%, 95% CI 0.0-41.4).
CONCLUSIONS: Among persons with CHD, the mortality rate is greatest during the first year of life; however, this systematic review and meta-analysis showed that survival decreases gradually after infancy and into adulthood.

PMID: 27312802 [PubMed - indexed for MEDLINE]

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Recommendations on the Echocardiographic Assessment of Aortic Valve Stenosis: A Focused Update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

J Am Soc Echocardiogr. 2017 Apr;30(4):372-392

Authors: Baumgartner H, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F, Otto CM

Echocardiography is the key tool for the diagnosis and evaluation of aortic stenosis. Because clinical decision-making is based on the echocardiographic assessment of its severity, it is essential that standards are adopted to maintain accuracy and consistency across echocardiographic laboratories. Detailed recommendations for the echocardiographic assessment of valve stenosis were published by the European Association of Echocardiography and the American Society of Echocardiography in 2009. In the meantime, numerous new studies on aortic stenosis have been published with particular new insights into the difficult subgroup of low gradient aortic stenosis making an update of recommendations necessary. The document focuses in particular on the optimization of left ventricular outflow tract assessment, low flow, low gradient aortic stenosis with preserved ejection fraction, a new classification of aortic stenosis by gradient, flow and ejection fraction, and a grading algorithm for an integrated and stepwise approach of aortic stenosis assessment in clinical practice.

PMID: 28385280 [PubMed - indexed for MEDLINE]

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A prospective 5-year study of the frequency of arrhythmias during serial exercise testing and clinical follow-up after Melody valve implant.

Heart Rhythm. 2016 Nov;13(11):2135-2141

Authors: Priromprintr B, Silka MJ, Rhodes J, Batra AS

BACKGROUND: Although percutaneous Melody valve implant has become an accepted alternative to surgical pulmonary valve replacement in patients with congenital heart disease, the benefit regarding frequency and severity of arrhythmias remains undefined.
OBJECTIVE: The purpose of this study was to evaluate the impact of Melody valve implant on the type and frequency of arrhythmias during cardiopulmonary exercise testing (CPET) and subsequent clinical outcome.
METHODS: As part of the phase I Melody valve clinical trial, 136 patients with congenital heart disease underwent prospective serial evaluation including CPET before implant, 6 months after implant, and annually thereafter for 5 years. Arrhythmias were defined as premature ventricular complexes (PVCs) and supraventricular or ventricular tachycardia (VT).
RESULTS: Before Melody implant, PVCs occurred in 55 patients (40%) and nonsustained ventricular tachycardia (NSVT) in 1 patient during CPET. Median age at valve implantation was 19.0 years (range 7-53 years). During median follow-up of 4.9 years (range 0.8-7.3 years), there was no significant change in the proportion of patients with PVCs during CPET at any follow-up interval (40%-45%). However, postimplant, NSVT occurred in 18 patients, including 8 during CPET. Diagnoses in the patients with NSVT were tetralogy of Fallot (11), transposition (2), and post-Ross procedure (5). Improved hemodynamic status was not associated with resolution or prevention of arrhythmias.
CONCLUSION: Despite improvement in hemodynamics, Melody valve implant was not associated with resolution or prevention of arrhythmias during CPET. PVCs or VT may be related to pathologic hypertrophy, fibrosis, dilation, or possible mechanical effects of the Melody device.

PMID: 27453127 [PubMed - indexed for MEDLINE]

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Old but not forgotten: Creative use of abandoned epicardial leads after more than 2 decades.

HeartRhythm Case Rep. 2017 Dec;3(12):559-561

Authors: Bjorkman K, Clancy JF, Elder RW

PMID: 29296574 [PubMed]

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Pulmonary valve and right ventricular outflow tract surgery in adults: 23-year experience.

Kardiol Pol. 2018 Jan 03;:

Authors: Szczechowicz M, Mkalaluh S, Farag M, Mashhour A, Loukanov T, Kolcz J, Karck M, Weymann A

BACKGROUND: Surgery of the pulmonary valve, right ventricular outflow tract, and pulmonary artery falls under the domain of paediatric cardiac surgery. However, 97 adult patients underwent such operations in our establishment from 1993 to 2016.
AIM: This study aims to analyse preoperative risk factors, intraoperative data, postoperative outcomes, and long-term survival to identify the potential predictors of mortality and high-risk patients.
 METHODS: We divided our patient cohort into three groups in accordance with surgical indications: 17 patients with pulmonary valve endocarditis (group A), 70 patients with congenital defects involving the pulmonary valve (group B), and 10 patients who underwent pulmonary valve surgery for other indications, like tumor or other acquired valvular disease (group C).
 RESULTS: Gender distribution was comparable in all the three groups, with about 40% of the total number of patients being female. The mean age was 35.9 ±15.7 years. Sixty patients (61.9%) had a history of cardiac surgery. Various concomitant cardiac surgical procedures were necessary in 49 cases (50.5%). There were two in-hospital deaths (11.8%) in group A, two (2.9%) in group B, and none in group C. Within the mean follow-up time of 6.6 ±7.2 years, three patients (17.7%) in group A, two (2.9%) in group B, and four (40%) in group C died.
 CONCLUSIONS: Adult patients with pulmonary valve disease are often previously heart-operated and often need concomitant procedures. The operative risk in patients with pulmonary valve endocarditis is high. Surgery of congenital defects of the pulmonary valve is safe and can be performed with excellent outcomes.

PMID: 29297190 [PubMed - as supplied by publisher]

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Isolated double orifice mitral valve in a young asymptomatic woman.

J Cardiovasc Med (Hagerstown). 2016 Dec;17 Suppl 2:e161-e163

Authors: Pizzino F, Piccione MC, Trio O, Zito C, Monaco M, Carerj S

: Double orifice mitral valve (DOMV) is a rare congenital malformation of the mitral apparatus frequently associated with other congenital malformations of the heart, whereas the isolated form is rare. The prevalence is about 1% of congenital heart disease; valve stenosis and regurgitation are common findings. Echocardiography represents the best method to diagnose DOMV, providing both anatomical and functional data. We report a case of a young woman presenting with an isolated DOMV, in the absence of stenosis and significant regurgitation. The dysmorphic valve has been evaluated with both two-dimensional and three-dimensional echocardiography, providing impressive and detailed images of the peculiar anatomical configuration of the valve.

PMID: 26204470 [PubMed - indexed for MEDLINE]

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Does Survival on the Heart Transplant Waiting List Depend on the Underlying Heart Disease?

JACC Heart Fail. 2016 Sep;4(9):689-97

Authors: Hsich EM, Rogers JG, McNamara DM, Taylor DO, Starling RC, Blackstone EH, Schold JD

OBJECTIVES: The aim of this study was to identify differences in survival on the basis of type of heart disease while awaiting orthotopic heart transplantation (OHT).
BACKGROUND: Patients with restrictive cardiomyopathy (RCM), congenital heart disease (CHD), or hypertrophic cardiomyopathy (HCM) may be at a disadvantage while awaiting OHT because they often are poor candidates for mechanical circulatory support and/or inotropes.
METHODS: The study included all adults in the Scientific Registry of Transplant Recipients database awaiting OHT from 2004 to 2014, and outcomes were evaluated on the basis of type of heart disease. The primary endpoint was time to all-cause mortality, censored at last patient follow-up and time of transplantation. Multivariate Cox proportional hazards modeling was performed to evaluate survival by type of cardiomyopathy.
RESULTS: There were 14,447 patients with DCM, 823 with RCM, 11,799 with ischemic cardiomyopathy (ICM), 602 with HCM, 964 with CHD, 584 with valvular disease, and 1,528 in the "other" category (including 1,216 for retransplantation). During median follow-up of 3.7 months, 4,943 patients died (1,253 women, 3,690 men). After adjusting for possible confounding variables including age, renal function, inotropes, mechanical ventilation, and mechanical circulatory support, the adjusted hazard ratios by diagnoses relative to DCM were 1.70 for RCM (95% confidence interval [CI]: 1.43 to 2.02), 1.10 for ICM (95% CI: 1.03 to 1.18), 1.23 for HCM (95% CI: 0.98 to 1.54), 1.30 for valvular disease (95% CI: 1.07 to 1.57), 1.37 for CHD (95% CI: 1.17 to 1.61), and 1.51 for "other" diagnoses (95% CI: 1.34 to 1.69). Sex was a significant modifier of mortality for ICM, RCM, and "other" diagnoses (p < 0.05 for interaction).
CONCLUSIONS: In the United States, patients with RCM, CHD, or prior heart transplantation had a higher risk for death while awaiting OHT than patients with DCM, ICM, HCM, or valvular heart disease.

PMID: 27179836 [PubMed - indexed for MEDLINE]

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Congenital Heart Disease With and Without Cyanotic Potential and the Long-term Risk of Diabetes Mellitus: A Population-Based Follow-up Study.

J Am Heart Assoc. 2016 Jul 08;5(7):

Authors: Madsen NL, Marino BS, Woo JG, Thomsen RW, Videbœk J, Laursen HB, Olsen M

BACKGROUND: Long-term survival for persons born with congenital heart disease (CHD) is improved, but limited knowledge exists of this growing population's acquired cardiovascular risk profile. This study's purpose was to assess CHD survivors' risk for type 2 diabetes mellitus (T2DM) with attention to the impact of cyanotic CHD.
METHODS AND RESULTS: This population-based cohort study included Danish subjects with CHD who were born between 1963 and 1980 and were alive at age 30 years. For each CHD case, we identified 10 individuals from the general population matched by sex and birth year, by using the Danish Civil Registration System. Complete follow-up was obtained through Danish public registries for death, emigration, and T2DM (diagnosis and prescriptions record). We computed cumulative incidences and hazard ratios of developing T2DM after age 30 for 5149 CHD subjects compared with the general population. After adjusting for CHD severity, as well as age, sex, preterm birth, and extracardiac defects, we analyzed the impact of cyanotic compared with acyanotic CHD. By age 45 years, the cumulative incidence of T2DM after age 30 was 4% among subjects with CHD. Subjects with CHD were more likely to develop T2DM than the general population (hazard raio 1.4, 95% CI 1.1-1.6). Subjects CHD who had cyanotic defects were more likely to develop T2DM than were subjects with acyanotic CHD (hazard ratio 1.9, 95% CI 1.1-3.3).
CONCLUSIONS: CHD survivors had an increased risk of developing T2DM after age 30. Patients with cyanotic CHD are at particular risk. Given the cardiovascular health burden of T2DM, attention to its development in CHD survivors seems warranted.

PMID: 27402234 [PubMed - indexed for MEDLINE]

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Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study.

Arch Cardiovasc Dis. 2017 May;110(5):303-316

Authors: Hascoet S, Fournier E, Jaïs X, Le Gloan L, Dauphin C, Houeijeh A, Godart F, Iriart X, Richard A, Radojevic J, Amedro P, Bosser G, Souletie N, Bernard Y, Moceri P, Bouvaist H, Mauran P, Barre E, Basquin A, Karsenty C, Bonnet D, Iserin L, Sitbon O, Petit J, Fadel E, Humbert M, Ladouceur M

BACKGROUND: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial.
AIMS: To investigate outcomes in patients with ES, and their relationship with PAH-SDT.
METHODS: Retrospective, observational, nationwide, multicentre cohort study.
RESULTS: We included 340 patients with ES: genetic syndrome (n=119; 35.3%); pretricuspid defect (n=75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE5I]) 46.7%; dual therapy (ERA+PDE5I) 40.9%; triple therapy (ERA+PDE5I+prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0-9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4-47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8-21.6%] and 46.4% [95% confidence interval 38.2-55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P=0.0001 and P=0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P<0.001 and P<0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P=0.002, P=0.01 and P=0.04, respectively), and one or two PAH-SDTs with a lower risk of events (P=0.009).
CONCLUSIONS: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.

PMID: 28286190 [PubMed - indexed for MEDLINE]

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Real-time three dimensional CT and MRI to guide interventions for congenital heart disease and acquired pulmonary vein stenosis.

Int J Cardiovasc Imaging. 2017 Oct;33(10):1619-1626

Authors: Suntharos P, Setser RM, Bradley-Skelton S, Prieto LR

To validate the feasibility and spatial accuracy of pre-procedural 3D images to 3D rotational fluoroscopy registration to guide interventional procedures in patients with congenital heart disease and acquired pulmonary vein stenosis. Cardiac interventions in patients with congenital and structural heart disease require complex catheter manipulation. Current technology allows registration of the anatomy obtained from 3D CT and/or MRI to be overlaid onto fluoroscopy. Thirty patients scheduled for interventional procedures from 12/2012 to 8/2015 were prospectively recruited. A C-arm CT using a biplane C-arm system (Artis zee, VC14H, Siemens Healthcare) was acquired to enable 3D3D registration with pre-procedural images. Following successful image fusion, the anatomic landmarks marked in pre-procedural images were overlaid on live fluoroscopy. The accuracy of image registration was determined by measuring the distance between overlay markers and a reference point in the image. The clinical utility of the registration was evaluated as either "High", "Medium" or "None". Seventeen patients with congenital heart disease and 13 with acquired pulmonary vein stenosis were enrolled. Accuracy and benefit of registration were not evaluated in two patients due to suboptimal images. The distance between the marker and the actual anatomical location was 0-2 mm in 18 (64%), 2-4 mm in 3 (11%) and >4 mm in 7 (25%) patients. 3D3D registration was highly beneficial in 18 (64%), intermediate in 3 (11%), and not beneficial in 7 (25%) patients. 3D3D registration can facilitate complex congenital and structural interventions. It may reduce procedure time, radiation and contrast dose.

PMID: 28455631 [PubMed - indexed for MEDLINE]