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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]

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Ebstein's anomaly in adults: Modified cone reconstruction of the tricuspid valve is associated with promising outcomes.

Arch Cardiovasc Dis. 2017 May;110(5):325-333

Authors: Belli E, Rabot M, Petit J, Gouton M

BACKGROUND: Ebstein's anomaly is a complex malformation. Justification of a procedure in mildly symptomatic adults is debatable: repair techniques are demanding and valve replacement is associated with poorer outcome.
OBJECTIVES: We report our initial experience with the cone procedure versus medical follow-up.
METHODS: Patients aged≥15years with Ebstein's anomaly were enrolled during 2007-2014. The cone procedure was performed in consecutive patients with severe tricuspid regurgitation (TR); those with less severe disease did not undergo surgery, although some underwent percutaneous catheter atrial septal defect closure.
RESULTS: The cone procedure was performed in 20 patients (mean age 34.3±14.4years; TR grade 3.3±0.7) because of impaired functional capacity: six New York Heart Association (NYHA) class II, 14 class III. No surgical patient died during a mean (range) follow-up of 2.8 (0.5-5.0) years. One patient required subsequent repair for suture dehiscence 6 months postoperatively. All patients presented with mild or less TR at last echocardiographic follow-up. NYHA functional class was significantly improved at follow-up (P<0.0001): 16 NYHA class I and four class II. Of 24 non-surgical patients (mean age 37.3±16.9 years; TR grade 2.2±0.8), seven underwent percutaneous ASD closure. During a mean (range) follow-up of 4.8 (3.6-5.0) years, two patients died: one sudden death and one stroke.
CONCLUSION: The cone repair of the tricuspid valve in adults with Ebstein's anomaly provided excellent mid-term results and significantly improved functional status. This procedure might be considered even in mildly symptomatic patients in the presence of severe valve regurgitation.

PMID: 28566198 [PubMed - indexed for MEDLINE]

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Cardiac T1 mapping in congenital heart disease: bolus vs. infusion protocols for measurements of myocardial extracellular volume fraction.

Int J Cardiovasc Imaging. 2017 Dec;33(12):1961-1968

Authors: Al-Wakeel-Marquard N, Rastin S, Muench F, O H-Ici D, Yilmaz S, Berger F, Kuehne T, Messroghli DR

Myocardial extracellular volume fraction (ECV) reflecting diffuse myocardial fibrosis can be measured with T1 mapping cardiovascular magnetic resonance (CMR) before and after the application of a gadolinium-based extracellular contrast agent. The equilibrium between blood and myocardium contrast concentration required for ECV measurements can be obtained with a primed contrast infusion (equilibrium contrast-CMR). We hypothesized that equilibrium can also be achieved with a single contrast bolus to accurately measure diffuse myocardial fibrosis in patients with congenital heart disease (CHD). Healthy controls (n = 17; median age 24.0 years) and patients with CHD (n = 19; 25.0 years) were prospectively enrolled. Using modified Look-Locker inversion recovery T1 mapping before, 15 min after bolus injection, and during constant infusion of gadolinium-DOTA, T1 values were obtained for blood pool and myocardium of the left ventricle (LV), the interventricular septum (IVS), and the right ventricle (RV) in a single midventricular plane in short axis or in transverse orientation. ECV of LV, IVS and RV by bolus-only and bolus-infusion correlated significantly in CHD patients (r = 0.94, 0.95, and 0.74; p < 0.01, respectively) and healthy controls (r = 0.96, 0.89, and 0.64; p < 0.05, respectively). Bland-Altman plots revealed no significant bias between the techniques for any of the analyzed regions. ECV of LV and RV myocardium measured by bolus-only T1 mapping agrees well with bolus-infusion measurements in patients with CHD. The use of a bolus-only approach facilitates the integration of ECV measurements into existing CMR imaging protocols, allowing for assessment of diffuse myocardial fibrosis in CHD in clinical routine.

PMID: 28620681 [PubMed - indexed for MEDLINE]

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Transthoracic 3D echocardiographic left heart chamber quantification in patients with bicuspid aortic valve disease.

Int J Cardiovasc Imaging. 2017 Dec;33(12):1895-1903

Authors: van den Hoven AT, Mc-Ghie JS, Chelu RG, Duijnhouwer AL, Baggen VJM, Coenen A, Vletter WB, Dijkshoorn ML, van den Bosch AE, Roos-Hesselink JW

Integration of volumetric heart chamber quantification by 3D echocardiography into clinical practice has been hampered by several factors which a new fully automated algorithm (Left Heart Model, (LHM)) may help overcome. This study therefore aims to evaluate the feasibility and accuracy of the LHM software in quantifying left atrial and left ventricular volumes and left ventricular ejection fraction in a cohort of patients with a bicuspid aortic valve. Patients with a bicuspid aortic valve were prospectively included. All patients underwent 2D and 3D transthoracic echocardiography and computed tomography. Left atrial and ventricular volumes were obtained using the automated program, which did not require manual contour detection. For comparison manual and semi-automated measurements were performed using conventional 2D and 3D datasets. 53 patients were included, in four of those patients no 3D dataset could be acquired. Additionally, 12 patients were excluded based on poor imaging quality. Left ventricular end-diastolic and end-systolic volumes and ejection fraction calculated by the LHM correlated well with manual 2D and 3D measurements (Pearson's r between 0.43 and 0.97, p < 0.05). Left atrial volume (LAV) also correlated significantly although LHM did estimate larger LAV compared to both 2DE and 3DE (Pearson's r between 0.61 and 0.81, p < 0.01). The fully automated software works well in a real-world setting and helps to overcome some of the major hurdles in integrating 3D analysis into daily practice, as it is user-independent and highly reproducible in a group of patients with a clearly defined and well-studied valvular abnormality.

PMID: 28631106 [PubMed - indexed for MEDLINE]

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Comparison of systemic right ventricular function in transposition of the great arteries after atrial switch and congenitally corrected transposition of the great arteries.

Int J Cardiovasc Imaging. 2017 Dec;33(12):1993-2001

Authors: Morcos M, Kilner PJ, Sahn DJ, Litt HI, Valsangiacomo-Buechel ER, Sheehan FH

In patients with transposition of the great arteries corrected by interatrial baffle (TGA) and those with congenitally corrected transposition of the great arteries (ccTGA) the right ventricle (RV) is subjected to systemic pressure and fails prematurely. Previous studies have demonstrated RV dysfunction may be more pronounced in patients with TGA. The present study sought to compare patients with TGA and ccTGA using three-dimensional (3D) techniques to comprehensively analyze the shape, volume, global and regional function in the systemic RV. We compared RV size, shape, and regional and global function in 25 patients with TGA, 17 patients with ccTGA, and 9 normal subjects. The RVs were reconstructed from cardiac Magnetic Resonance Images for 3D analyses. Compared to normal, the RV in TGA and ccTGA was dilated, rounded, and reduced in function. Compared to each other, TGA and ccTGA patients had similar RV size and shape. Global RV function was lower in TGA than ccTGA when assessed from ejection fraction (EF) (30 ± 7 vs. 35 ± 7, p = 0.02) and from normalized tricuspid annular systolic plane excursion (TAPSE) (0.10 ± 0.04 vs. 0.18 ± 0.04, p < 0.01). Basilar RV function was poorer in the TGA patients when compared to ccTGA. The systemic RVs in both TGA and ccTGA are dilated, spherical, and poorly functioning. Compared to ccTGA, TGA RVs have reduced TAPSE and worse basilar hypokinesis.

PMID: 28668979 [PubMed - indexed for MEDLINE]

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The Center for Epidemiologic Studies Depression Scale is an adequate screening instrument for depression and anxiety disorder in adults with congential heart disease.

Health Qual Life Outcomes. 2017 Sep 05;15(1):176

Authors: Moon JR, Huh J, Song J, Kang IS, Park SW, Chang SA, Yang JH, Jun TG

BACKGROUND: The Center for Epidemiological Studies Depression Scale (CES-D) is an instrument that is commonly used to screen for depression in patients with chronic disease, but the characteristics of the CES-D in adults with congenital heart disease (CHD) have not yet been studied. The aim of this study was to investigate the criterion validities and the predictive powers of the CES-D for depression and anxiety disorders in adults with CHD.
METHODS: Two hundred patients were screened with the CES-D and secondarily interviewed with a diagnostic instrument, i.e., the Mini International Neuropsychiatric Instrument. The sensitivity and specificity values of the CES-D were calculated by cross-tabulation at different cutoff scores. Receiver operating characteristic (ROC) curves were used to assess the optimal cutoff point for each disorder and to assess the predictive power of the instrument.
RESULTS: The CES-D exhibited satisfactory criterion validities for depression and for all combinations of depression and/or anxiety. With a desired sensitivity of at least 80%, the optimal cutoff scores were 18. The predictive power of the CES-D in the patients was best for major depression and dysthymia (area under the ROC curve: 0.92) followed by the score for any combination of depression and/or anxiety (0.88).
CONCLUSION: The use of CES-D to simultaneously screen for both depression and anxiety disorders may be useful in adults with CHD.
TRIAL REGISTRATION: CESDEP 212. Registered 2 March 2014 (retrospectively registered).

PMID: 28874154 [PubMed - indexed for MEDLINE]

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Cardiac arrest and ventricular arrhythmia in adults with Ebstein anomaly and left ventricular non-compaction.

J Cardiol. 2018 Jan 02;:

Authors: Kumor M, Lipczyńska M, Biernacka EK, Klisiewicz A, Wójcik A, Konka M, Kożuch K, Szymański P, Hoffman P

BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC).
AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA.
METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features). We reviewed echocardiograms and CMR scans for concomitant morphological abnormalities (LVNC, PDA - patent ductus arteriosus, VSD - ventricular septal defect, ASD - atrial septal defect, mitral valve prolapse, BAV - bicuspid aortic valve, CoA - coarctation of aorta).
RESULTS: The studied group consisted of 84 consecutive patients (mean age 38±15 years, 50 women) with EA. We found four patients (4.8%) with LVNC, two of them had cardiac arrest, one had VT, and one was symptomless, but had QTc prolongation in Holter recordings. Concomitant abnormalities were VSD (4.8%), PDA (1.2%), CoA (1.2%), mitral valve prolapse (1.2%), and BAV (2.4%). The most common anomaly was ASD type II - 23 patients (27.3%) and WPW - Wolff-Parkinson-White's syndrome - 9 patients (10.7%).
CONCLUSIONS: Non-compaction is a notable abnormality in adult patients with EA and it may affect their prognosis. Although other concomitant lesions were more common, only patients with LVNC suffered from cardiac arrest or ventricular arrhythmia.

PMID: 29305187 [PubMed - as supplied by publisher]