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Double-chambered left ventricle: coronary embolism as the first presentation of an extremely unusual cardiac anomaly.

J Cardiovasc Med (Hagerstown). 2017 10;18(10):790-792

Authors: Dallaglio PD, Claver E, Di Marco A, Alió J, Hidalgo A, Cequier A

PMID: 23549276 [PubMed - indexed for MEDLINE]

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Catheter Ablation of Atrial Fibrillation: An Overview for Clinicians.

Adv Ther. 2017 Aug;34(8):1897-1917

Authors: Mujović N, Marinković M, Lenarczyk R, Tilz R, Potpara TS

Catheter ablation (CA) of atrial fibrillation (AF) is currently one of the most commonly performed electrophysiology procedures. Ablation of paroxysmal AF is based on the elimination of triggers by pulmonary vein isolation (PVI), while different strategies for additional AF substrate modification on top of PVI have been proposed for ablation of persistent AF. Nowadays, various technologies for AF ablation are available. The radiofrequency point-by-point ablation navigated by electro-anatomical mapping system and cryo-balloon technology are comparable in terms of the efficacy and safety of the PVI procedure. Long-term success of AF ablation including multiple procedures varies from 50 to 80%. Arrhythmia recurrences commonly occur, mostly due to PV reconnection. The recurrences are particularly common in patients with non-paroxysmal AF, dilated left atrium and the "early recurrence" of AF within the first 2-3 post-procedural months. In addition, this complex procedure can be accompanied by serious complications, such as cardiac tamponade, stroke, atrio-esophageal fistula and PV stenosis. Therefore, CA represents a second-line treatment option after a trial of antiarrhythmic drug(s). Good candidates for the procedure are relatively younger patients with symptomatic and frequent episodes of AF, with no significant structural heart disease and no significant left atrial enlargement. Randomized trials demonstrated the superiority of ablation compared to antiarrhythmic drugs in terms of improving the quality of life and symptoms in AF patients. However, nonrandomized studies reported additional clinical benefits from ablation over drug therapy in selected AF patients, such as the reduction of the mortality and stroke rates and the recovery of tachyarrhythmia-induced cardiomyopathy. Future research should enable the creation of more durable ablative lesions and the selection of the optimal lesion set in each patient according to the degree of atrial remodeling. This could provide better long-term CA success and expand indications for the procedure, especially among the patients with non-paroxysmal AF.

PMID: 28733782 [PubMed - indexed for MEDLINE]

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A comparison of echocardiographic variables of right ventricular function with exercise capacity after bosentan treatment in patients with pulmonary arterial hypertension: Results from a multicenter, prospective, cohort study.

J Clin Ultrasound. 2017 Jan;45(1):28-34

Authors: Kim H, Bae Lee J, Park JH, Yoo BS, Son JW, Yang DH, Lee BR

PURPOSE: Bosentan reduces pulmonary arterial pressure and improves exercise capacity in patients with pulmonary arterial hypertension (PAH). However, there are limited data regarding the extent to which the changes in echocardiographic variables reflect improvements in exercise capacity. We aimed to assess the improvement of echocardiographic variables and exercise capacity after 6 months of bosentan treatment for PAH.
METHODS: We performed a prospective study from June 2012 to June 2015 in seven participating medical centers. Echocardiography, including tissue Doppler imaging (TDI) and the 6-minute walk test distance (6MWD), was performed at baseline and after 6 months of bosentan treatment.
RESULTS: We analyzed 19 patients with PAH: seven with congenital shunt, six with collagen vascular disease, and six with idiopathic PAH. After bosentan treatment, mean 6MWD increased by 50 meters. Right ventricle (RV) systolic pressure, tricuspid annular plane systolic excursion, myocardial performance index (MPI) derived from TDI (MPI-TDI) of RV and left ventricle (LV), RV fractional area change, and RV ejection fraction were significantly improved. In particular, the magnitude of RV and LV MPI-TDI showed good correlation with changes in the 6MWD.
CONCLUSIONS: The magnitude of RV and LV MPI-TDI was strongly associated with improvements in exercise capacity. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:28-34, 2017.

PMID: 27619758 [PubMed - indexed for MEDLINE]

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[2017 ESC/EACTS Guidelines for the management of valvular heart disease].

Kardiol Pol. 2018;76(1):1-62

Authors: Baumgartner H, Falk V, Bax JJ, Bonis M, Hamm C, Holm PJ, Iung B, Lancellotti P, Lansac E, Munoz DR, Rosenhek R, Sjögren J, Mas PT, Vahanian A, Walther T, Wendler O, Windecker S, Zamorano JL

PMID: 29399765 [PubMed - in process]

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Optimizing patient care and outcomes through the congenital heart center of the 21st century.

Congenit Heart Dis. 2018 Feb 05;:

Authors: Anderson JB, Chowdhury D, Connor JA, Daniels CJ, Fleishman CE, Gaies M, Jacobs J, Kugler J, Madsen N, Beekman RH, Lihn S, Stewart-Huey K, Vincent R, Campbell R

Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day. This was an open participation meeting for clinicians, administrators, patients/parents to propose metrics that optimize patient care and outcomes for a state-of-the-art congenital heart center of the 21st century. Care center collaboration helps overcome the barrier of relative small volumes at any given program. Patients and families have become active collaborative partners with care centers in the definition of acute and longitudinal outcomes and our quality metrics. Understanding programmatic metrics that create an environment to provide outstanding congenital heart care will allow centers to improve their structure, processes and ultimately outcomes, leading to an increasing number of centers that provide excellent care. This manuscript provides background, as well listing of proposed specialty domain quality metrics for centers, and thus serves as an updated baseline for the ongoing dynamic process of optimizing care and realizing patient value.

PMID: 29400005 [PubMed - as supplied by publisher]

General Concepts in Adult Congenital Heart Disease.

Balkan Med J. 2018 Jan 20;35(1):18-29

Authors: Mutluer FO, Çeliker A

Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

PMID: 29400306 [PubMed - in process]

Pregnancy and delivery outcomes from patients with repaired anomalous origin of the left coronary artery from the pulmonary artery.

J Obstet Gynaecol Res. 2018 Feb 05;:

Authors: Kanoh M, Inai K, Shinohara T, Shimada E, Shimizu M, Tomimatsu H, Ogawa M, Nakanishi T

AIM: We investigated the clinical courses before and during pregnancy and after delivery in patients with repaired anomalous origin of the left coronary artery from the pulmonary artery to determine the impact of the hemodynamic changes and cardiac function on the selection of the appropriate mode of delivery.
METHODS: Six patients who underwent coronary artery reimplantation delivered 10 infants. We scrutinized the patients' hemodynamic changes on echocardiographs and the plasma brain natriuretic peptide levels before and during pregnancy and after delivery, the perinatal outcomes and maternal and fetal events.
RESULTS: All patients were asymptomatic and categorized as having New York Heart Association functional class I before pregnancy. In 8 of 10 pregnancies, vaginal deliveries were performed; two elective cesarean sections were performed because of symptomatic heart failure. The hemodynamic parameters were stable throughout pregnancy and postdelivery, and no maternal or fetal events occurred in the patients who underwent vaginal deliveries. One cesarean section patient developed significant heart failure during the late second and third trimesters, which was accompanied by hemodynamic changes, including increased brain natriuretic peptide levels, left ventricular diastolic dysfunction and worsening arrhythmias, and thrombosis and post-partum hemorrhage occurred postdelivery. The baby had intrauterine growth retardation and small for gestational age. None of the babies had congenital anomalies.
CONCLUSION: Pregnancy was safe in most of the asymptomatic patients long after anomalous origin of the left coronary artery from the pulmonary artery was repaired. Symptomatic heart failure might occur during pregnancy in patients with persisting myocardial damage. Pregnancy and delivery should be carefully managed.

PMID: 29400413 [PubMed - as supplied by publisher]

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Congenital Double-Orifice Mitral Valve in Asymptomatic Patients.

Int Heart J. 2018 Jan 27;59(1):213-215

Authors: Liu S, Ren W, Ma C, Yang J

Congenital double-orifice mitral valve (DOMV) is a rare malformation that may affect both mitral valve and apparatus, and it is usually associated with other cardiac disorders. Asymptomatic DOMV is extremely rare. We present two similar cases of asymptomatic congenital double-orifice mitral valve. Both cases presented functionally normal mitral valve apparatus with two equally sized orifices, which were formed by a central fibrosis. In addition, final confirmatory diagnosis was made using three-dimensional (3D) echocardiography in both cases.

PMID: 29269712 [PubMed - indexed for MEDLINE]

Going beyond morphology in Ebstein's anomaly.

Int J Cardiol. 2018 Feb 02;:

Authors: Lombardi M, Pluchinotta FR

PMID: 29402524 [PubMed - as supplied by publisher]

In silico thrombin and factor Xa generation profiles in adult patients after Fontan operation.

Blood Coagul Fibrinolysis. 2018 Feb 05;:

Authors: Gissel M, Tomkiewicz-Pajak L, Podolec P, Hoffman P, Trojnarska O, Lipczyńska M, Undas A, Brummel-Ziedins KE

: Single-ventricle defects are associated with increased risk of thromboembolic events. To analyze the prothrombotic potential in a long-term follow-up on Fontan patients via plasma contribution to thrombin and factor (F)Xa generation profiles. Thrombin and FXa generation was simulated from plasma concentrations of FII, FV, FVII, FVIII, FIX, FX, antithrombin and tissue factor (TF) pathway inhibitor from Fontan patients (n = 48) and healthy controls (n = 34). TF and thrombin-antithrombin complex (TAT) were measured by ELISA. Fontan patients had significantly reduced procoagulant protein concentrations and increased anticoagulant protein concentrations over controls, resulting in a lowered procoagulant potential. However, Fontan patients showed increased hemostatic activation as evidenced by increased TF and TAT. Modeling this increased TF showed a more prothrombotic profile. Observed changes in procoagulant and anticoagulant proteins may be a compensatory mechanism aimed at mitigating the underlying disease effects characterized by elevated TF and TAT.

PMID: 29406387 [PubMed - as supplied by publisher]