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Definition and Management of Segmental Pulmonary Hypertension.

J Am Heart Assoc. 2018 Jul 04;7(14):

Authors: Dimopoulos K, Diller GP, Opotowsky AR, D'Alto M, Gu H, Giannakoulas G, Budts W, Broberg CS, Veldtman G, Swan L, Beghetti M, Gatzoulis MA

PMID: 29973393 [PubMed - in process]

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Stroke volume ratio derived from magnetic resonance imaging as an indicator of interventricular dyssynchrony predicts future cardiac event in patients with biventricular Fontan circulation.

Heart Vessels. 2018 Jul 04;:

Authors: Takahashi T, Shiina Y, Nagao M, Inai K

The prognostic factors in patients with biventricular heart who underwent Fontan surgery remain unclear. This study wanted to assess the hypothesis that interventricular dyssynchrony evaluated by cardiac magnetic resonance imaging (MRI) can predict future cardiac events in patients with biventricular heart who have undergone Fontan surgery. We prospectively enrolled consecutive patients with biventricular Fontan circulation from 2003 to 2016, and performed protocolized cardiac MRI. We determined the stroke volume ratio (SVr) using the following formula to assess interventricular dyssynchrony: (stroke volume (SV) of the two whole ventricles)/(SV of the right ventricle + SV of the left ventricle), by tracing cine MRI data. If interventricular dyssynchrony existed, blood flowed and returned to each ventricle through the ventricular septal defect; therefore, the SVr in this instance should be less than 1.0. We enrolled 40 patients. SVr ranged from 0.81 to 1.0 (median 0.95). Low SVr (< 0.95) was associated with worse New York Heart Association functional class, longer QRS duration, right bundle branch block, low biventricular indexed stroke volume, and low biventricular ejection fraction. During the follow-up period (median 53.5 months), 10 cardiac events occurred (six cases of acute exacerbation of heart failure, three cases of supraventricular tachycardia, and one case of exacerbation of protein-losing enteropathy). Univariate analysis showed four clinical predictors: SVr < 0.95 [hazard ratio (HR) 9.3, 95% confidential interval (CI) 1.7-171.5]; biventricular ejection fraction < 0.45 (HR 9.4, 95% CI 2.2-65.3); left ventricular indexed end-diastolic volume > 73 mL/m2 (HR 4.5, 95% CI 1.1-15.7); and the presence of the aorta directly arising from the right ventricular conus (HR 5.8, 95% CI 1.1-106). SVr derived from MRI can predict future cardiac events in Fontan patients with biventricular hearts.

PMID: 29974198 [PubMed - as supplied by publisher]

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Unusual Atrioventricular Reentry Tachycardia in Congenitally Corrected Transposition of Great Arteries: A Novel Site for Catheter Ablation.

Circ Arrhythm Electrophysiol. 2016 06;9(6):

Authors: Noheria A, Asirvatham SJ, McLeod CJ

PMID: 27217343 [PubMed - indexed for MEDLINE]

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Fetal echocardiographic findings in right pulmonary artery to left atrium communication: a case report and review of the literature.

J Matern Fetal Neonatal Med. 2018 Feb;31(4):521-524

Authors: Ide T, Miyoshi T, Matsuyama TA, Kurosaki KI, Yoshimatsu J

Right pulmonary artery to left atrium communication (RPALA com) is a fatal congenital heart disease with right-to-left shunting. We present a rare case of RPALA com that was prenatally diagnosed and confirmed by autopsy. We focus on some unique echocardiographic findings for prenatal diagnosis of RPALA com by reviewing our case and the literature. Asymmetric cardiomegaly with a pear sign and dilatation of the pulmonary artery might be useful for screening tests. A larger stalk with the pear sign, high-velocity of shunt flow, and absence of a dilated pulmonary vein might support the prenatal diagnosis of RPALA com.

PMID: 28140722 [PubMed - indexed for MEDLINE]

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[Prenatally diagnosed case of Pallister‒Killian syndrome].

Orv Hetil. 2018 May;159(21):847-852

Authors: Tidrenczel Z, P Tardy E, Sarkadi E, Simon J, Beke A, Demeter J

Pallister-Killian syndrome (PKS) is a rare, sporadic genetic disorder that is caused by the mosaic presence of a supernumerary marker chromosome, isochromosome 12p. The syndrome is a polydysmorphic condition characterized by mental retardation, craniofacial dysmorphism, hypotonia, seizures, epilepsy and certain organic malformations (diaphragmatic hernia, congenital heart disease). Prenatal diagnosis is challenging due to the mosaic tissue-specific distribution of the chromosomal disorder and highly variable phenotype. Prenatal diagnosis is often accidental, however, appropriate laboratory techniques based on the second trimester ultrasound anomalies provide accurate prenatal diagnosis. We report a case of a 36-year-old primipara with second trimester ultrasound markers (polyhydramnion, ventriculomegaly, rhizomelic micromelia, abnormal facial profile). The patient underwent amniocentesis, the conventional karyotyping revealed a supernumerary chromosome in nearly 50 percent of amniocytes. FISH and targeted multicolour FISH probes verified mosaic tetrasomy of the short arm of chromosome 12 of the fetus. Fetopathological examinations and analysis of fetal tissues and blood confirmed the prenatal diagnosis. To our knowledge, this is the first reported case of prenatally diagnosed Pallister-Killian syndrome in Hungary. Orv Hetil. 2018; 159(21): 847-852.

PMID: 29779390 [PubMed - indexed for MEDLINE]

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Stem Cell Therapy for Hypoplastic Left Heart Syndrome: Mechanism, Clinical Application, and Future Directions.

Circ Res. 2018 Jul 06;123(2):288-300

Authors: Bittle GJ, Morales D, Deatrick KB, Parchment N, Saha P, Mishra R, Sharma S, Pietris N, Vasilenko A, Bor C, Ambastha C, Gunasekaran M, Li D, Kaushal S

Hypoplastic left heart syndrome is a type of congenital heart disease characterized by underdevelopment of the left ventricle, outflow tract, and aorta. The condition is fatal if aggressive palliative operations are not undertaken, but even after the complete 3-staged surgical palliation, there is significant morbidity because of progressive and ultimately intractable right ventricular failure. For this reason, there is interest in developing novel therapies for the management of right ventricular dysfunction in patients with hypoplastic left heart syndrome. Stem cell therapy may represent one such innovative approach. The field has identified numerous stem cell populations from different tissues (cardiac or bone marrow or umbilical cord blood), different age groups (adult versus neonate-derived), and different donors (autologous versus allogeneic), with preclinical and clinical experience demonstrating the potential utility of each cell type. Preclinical trials in small and large animal models have elucidated several mechanisms by which stem cells affect the injured myocardium. Our current understanding of stem cell activity is undergoing a shift from a paradigm based on cellular engraftment and differentiation to one recognizing a primarily paracrine effect. Recent studies have comprehensively evaluated the individual components of the stem cells' secretomes, shedding new light on the intracellular and extracellular pathways at the center of their therapeutic effects. This research has laid the groundwork for clinical application, and there are now several trials of stem cell therapies in pediatric populations that will provide important insights into the value of this therapeutic strategy in the management of hypoplastic left heart syndrome and other forms of congenital heart disease. This article reviews the many stem cell types applied to congenital heart disease, their preclinical investigation and the mechanisms by which they might affect right ventricular dysfunction in patients with hypoplastic left heart syndrome, and finally, the completed and ongoing clinical trials of stem cell therapy in patients with congenital heart disease.

PMID: 29976693 [PubMed - in process]

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Prevention of sudden cardiac death in patients with Tetralogy of Fallot: Risk assessment and long term outcome.

Int J Cardiol. 2018 Jun 28;:

Authors: Probst J, Diller GP, Reinecke H, Leitz P, Frommeyer G, Orwat S, Vormbrock J, Radke R, de Torres Alba F, Kaleschke G, Baumgartner H, Eckardt L, Wasmer K

BACKGROUND: In patients with repaired Tetralogy of Fallot (ToF), implantable cardioverter defibrillators (ICD) are considered reasonable in selected adults with multiple risk factors for sudden cardiac death.
PATIENTS AND METHODS: We performed a retrospective cohort study of all 174 patients with repaired ToF who are followed at the University Hospital of Muenster. We analyzed data according to the risk score previously proposed by Khairy and coworkers and patient outcome. We analyzed data separately for patients without previous sustained ventricular tachycardia (VT) (risk stratification group, n = 157) and patients with VT/secondary prevention ICD (n = 17).
RESULTS: In the risk stratification group, a mean of 4 ± 1 risk score parameters were available. All six risk parameters were known in 10%, five in 14%. Risk score increased with availability of parameters. 15 patients with secondary prevention ICD had a mean risk score of 6.3 ± 2.2 (range 2-10). 11 patients of the risk stratification group with primary prevention ICD had a mean risk score 5.8 ± 2.4 (range 3-8). During follow-up of up to 14 years, five patients died (3%): at age 58, two at 69 and two at 76 years.
CONCLUSION: In the majority of patients risk score variables were incomplete, severely limiting its applicability because the true score cannot be calculated. Risk scores were not different between patients with secondary prevention ICD and patients with ICD for primary prevention based on current guidelines. Standardization of follow-up and prospective evaluation of these standards in large prospective patient cohorts is desirable to improve risk stratification in patients with ToF.

PMID: 29980366 [PubMed - as supplied by publisher]

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Three-dimensional print facilitated ventricular tachycardia ablation in patient with corrected congenital heart disease.

Cardiol J. 2017;24(5):584-585

Authors: Lodziński P, Balsam P, Peller M, Gawałko M, Opolski G, Grabowski M

PMID: 29087541 [PubMed - indexed for MEDLINE]

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Does maternal environmental tobacco smoke interact with social-demographics and environmental factors on congenital heart defects?

Environ Pollut. 2018 Mar;234:214-222

Authors: Liu X, Nie Z, Chen J, Guo X, Ou Y, Chen G, Mai J, Gong W, Wu Y, Gao X, Qu Y, Bell EM, Lin S, Zhuang J

Congenital heart defects (CHDs) are a major cause of death in infancy and childhood. Major risk factors for most CHDs, particularly those resulting from the combination of environmental exposures with social determinants and behaviors, are still unknown. This study evaluated the main effect of maternal environmental tobacco smoke (ETS), and its interaction with social-demographics and environmental factors on CHDs in China. A population-based, matched case-control study of 9452 live-born infants and stillborn fetuses was conducted using the Guangdong Registry of Congenital Heart Disease data (2004-2014). The CHDs were evaluated by obstetrician, pediatrician, or cardiologist, and confirmed by cardia tomography/catheterization. Controls were randomly chosen from singleton newborns without any malformation, born in the same hospital as the cases and 1:1 matched by infant sex, time of conception, and parental residence (same city and town to ensure sufficient geographical distribution for analyses). Face-to-face interviews were conducted to collect information on demographics, behavior patterns, maternal disease/medication, and environmental exposures. Conditional logistic regression was used to estimate odds ratios and 95% confidence intervals of ETS exposure on CHDs while controlling for all risk factors. Interactive effects were evaluated using a multivariate delta method for maternal demographics, behavior, and environmental exposures on the ETS-CHD relationship. Mothers exposed to ETS during the first trimester of pregnancy were more likely to have infants with CHD than mothers who did not (aOR = 1.44, 95% CI 1.25-1.66). We also observed a significant dose-response relationship when mothers were exposed to ETS and an increasing number of risk factors and CHDs. There were greater than additive interactions for maternal ETS and migrant status, low household income and paternal alcohol consumption on CHDs. Maternal low education also modified the ETS-CHD association on the multiplicative scale. These findings may help to identify high-risk populations for CHD, providing an opportunity for targeted preventive interventions.

PMID: 29175685 [PubMed - indexed for MEDLINE]

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Importance of Non-invasive Right and Left Ventricular Variables on Exercise Capacity in Patients with Tetralogy of Fallot Hemodynamics.

Pediatr Cardiol. 2017 Dec;38(8):1569-1574

Authors: Meierhofer C, Tavakkoli T, Kühn A, Ulm K, Hager A, Müller J, Martinoff S, Ewert P, Stern H

Good quality of life correlates with a good exercise capacity in daily life in patients with tetralogy of Fallot (ToF). Patients after correction of ToF usually develop residual defects such as pulmonary regurgitation or stenosis of variable severity. However, the importance of different hemodynamic parameters and their impact on exercise capacity is unclear. We investigated several hemodynamic parameters measured by cardiovascular magnetic resonance (CMR) and echocardiography and evaluated which parameter has the most pronounced effect on maximal exercise capacity determined by cardiopulmonary exercise testing (CPET). 132 patients with ToF-like hemodynamics were tested during routine follow-up with CMR, echocardiography and CPET. Right and left ventricular volume data, ventricular ejection fraction and pulmonary regurgitation were evaluated by CMR. Echocardiographic pressure gradients in the right ventricular outflow tract and through the tricuspid valve were measured. All data were classified and correlated with the results of CPET evaluations of these patients. The analysis was performed using the Random Forest model. In this way, we calculated the importance of the different hemodynamic variables related to the maximal oxygen uptake in CPET (VO2%predicted). Right ventricular pressure showed the most important influence on maximal oxygen uptake, whereas pulmonary regurgitation and right ventricular enddiastolic volume were not important hemodynamic variables to predict maximal oxygen uptake in CPET. Maximal exercise capacity was only very weakly influenced by right ventricular enddiastolic volume and not at all by pulmonary regurgitation in patients with ToF. The variable with the most pronounced influence was the right ventricular pressure.

PMID: 28776135 [PubMed - indexed for MEDLINE]