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[Chronic Heart Failure With Preserved Systolic Function and Reversible Dilatation of Cardiac Chambers].

Kardiologiia. 2016 06;56(6):102-108

Authors: Blagova OV, Volkov SV, Nedostup AV, Korobkov AO, Mostovoy IV, Sergushina NG, Gagarina NV, Mershina EA

Abstract
Diagnosis of dilation (D) cardiomyopathy (CMP) requires exclusion not only of inflammatory and genetically determined forms but also of some rare diseases. This 51 year old patient with history of moderate arterial hypertension approached a cardiologist because of new onset atrial fibrillation and dyspnea. Echocardiography detected dilation of all cardiac chambers with relatively preserved ejection fraction, causing suspicion of DCMP. Among conditions excluded were coronary atherosclerosis, congenital heart defect with left to right shunt, primary pulmonary hypertension, pulmonary embolism, hypertensive heart, tachycardia induced CMP, arrhythmogenic right ventricular dysplasia, noncompaction myocardium. Further examination revealed massive pelvic arteriovenous malformation with branches of right internal iliac artery and inferior mesenteric artery as feeding vessels. This malformation was considered the leading cause of DCMP. Successful multistage embolization of feeding arteries was associated with reduction of cardiac chambers, alleviation of valvular regurgitation and pulmonary hypertension, restoration of sinus rhythm. Presentation of this case is followed by discussion of possible mechanisms of heart failure with high cardiac output and preserved systolic function in patients with arteriovenous malformations with left to right shunt. Approaches to interventional treatment of these malformations are also discussed.

PMID: 28290856 [PubMed - indexed for MEDLINE]

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Clinical - microbiological characterization and risk factors of mortality in infective endocarditis from a tertiary care academic hospital in Southern India.

Indian Heart J. 2018 Mar - Apr;70(2):259-265

Authors: Subbaraju P, Rai S, Morakhia J, Midha G, Kamath A, Saravu K

Abstract
AIMS: To dissect the clinical-microbiological profile of Infective endocarditis (IE) population and to determine the risk factors for IE related mortality.
METHODS: A cohort study was conducted using relevant data from clinical records of patients (≥12years) with definite/possible IE from December 2007 to December 2013 and was analyzed using appropriate statistical tests.
RESULTS: In the cohort of 139 IE patients, mean age was 47.9±15.8years, with male preponderance (68.3%). Rheumatic heart disease was the commonest (30.9%) underlying cardiac lesion followed by mitral valve prolapse with mitral regurgitation (23.7%), degenerative valvular disease (23%), congenital heart disease (15.8%) and prosthetic valves (3.6%). Vegetations were detected in 94.2% cases. Blood cultures were positive in 69.8% cases, commonest organism isolated was α - hemolytic streptococci (30.9%) followed by Enterococcus (12.9%) and methicillin sensitive Staphylococcus aureus (10.8%). Complications observed were congestive cardiac failure (31.2%), acute kidney injury (25.9%), stroke (21.6%), septic shock (16.5%), embolic phenomenon non-stroke (8.6%), atrial fibrillation (5%) and ring abscess (2.9%). Mortality rate was 17.3%. Congestive cardiac failure, increase in the peak leucocyte count and stroke were the independent predictors of mortality.
CONCLUSIONS: This study reiterates the persistent dominance of rheumatic heart disease in the population studied and α - hemolytic Streptococci as the commonest responsible microorganism.

PMID: 29716704 [PubMed - indexed for MEDLINE]

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Physical Activity-Related Drivers of Perceived Health Status in Adults With Congenital Heart Disease.

Am J Cardiol. 2018 Jul 17;:

Authors: Ko JM, White KS, Kovacs AH, Tecson KM, Apers S, Luyckx K, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Subramanyan R, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Mackie AS, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, Callus E, Kutty S, Gandhi A, Moons P, Cedars AM, APPROACH-IS consortium and International Society for Adult Congenital Heart Disease (ISACHD)

Abstract
Data on the differential impact of physical activity on perceived health status (PHS) in a large adult congenital heart disease (ACHD) patient population are lacking. We conducted a cross-sectional assessment of 4,028 ACHD patients recruited from 24 ACHD-specialized centers in 15 countries across 5 continents to examine the association between physical activity and PHS in a large international cohort of ACHD patients. A linear analog scale of the EuroQol-5D 3 level version and the 12-item Short Form Health Survey-version 2 were used to assess self-reported health status and the Health-Behavior Scale-Congenital Heart Disease was used as a subjective measurement of physical activity type, participation, and level. Correlation analyses and Wilcoxon Rank Sum tests examined bivariate relations between sample characteristics and PHS scores. Then, multivariable models were constructed to understand the impact of physical activity on PHS. Only 30% of our sample achieved recommended physical activity levels. Physically active patients reported better PHS than sedentary patients; however, the amount of physical activity was not associated with PHS. Further statistical analyses demonstrated that specifically sport participation regardless of physical activity level was a predictor of PHS. In conclusion, the majority of ACHD patients across the world are physically inactive. Sport participation appears to be the primary physical activity-related driver of PHS. By promoting sport-related exercise ACHD specialists thus may improve PHS in ACHD patients.

PMID: 30139525 [PubMed - as supplied by publisher]

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Prediction of stenting related adverse events through patient-specific finite element modelling.

J Biomech. 2018 Aug 13;:

Authors: Caimi A, Sturla F, Pluchinotta FR, Giugno L, Secchi F, Votta E, Carminati M, Redaelli A

Abstract
Right ventricular outflow tract (RVOT) calcific obstruction is frequent after homograft conduit implantation to treat congenital heart disease. Stenting and percutaneous pulmonary valve implantation (PPVI) can relieve the obstruction and prolong the conduit lifespan, but require accurate pre-procedural evaluation to minimize the risk of coronary artery (CA) compression, stent fracture, conduit injury or arterial distortion. Herein, we test patient-specific finite element (FE) modeling as a tool to assess stenting feasibility and investigate clinically relevant risks associated to the percutaneous intervention. Three patients undergoing attempted PPVI due to calcific RVOT conduit failure were enrolled; the calcific RVOT, the aortic root and the proximal CA were segmented on CT scans for each patient. We numerically reproduced RVOT balloon angioplasty to test procedure feasibility and the subsequent RVOT pre-stenting expanding the stent through a balloon-in-balloon delivery system. Our FE framework predicted the occurrence of CA compression in the patient excluded from the real procedure. In the two patients undergoing RVOT stenting, numerical results were consistent with intraprocedural in-vivo fluoroscopic evidences. Furthermore, it quantified the stresses on the stent and on the relevant native structures, highlighting their marked dependence on the extent, shape and location of the calcific deposits. Stent deployment induced displacement and mechanical loading of the calcific deposits, also impacting on the adjacent anatomical structures. This novel workflow has the potential to tackle the analysis of complex RVOT clinical scenarios, pinpointing the procedure impact on the dysfunctional anatomy and elucidating potential periprocedural complications.

PMID: 30139536 [PubMed - as supplied by publisher]

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The Impact of Routine Angiographic Follow-Up in a Population of Patients Undergoing Percutaneous Coronary Intervention Within the Left Main Coronary Artery.

Angiology. 2016 09;67(8):742-8

Authors: Mencel G, Kowalczyk J, Lenarczyk R, Chodór P, Wąs T, Świerad M, Honisz G, Świątkowski A, Woźniak A, Kalarus Z, Sredniawa B

Abstract
Prognostic value of angiographic follow-up in patients undergoing percutaneous coronary interventions (PCIs) of the left main coronary artery (LMCA) still remains uncertain. The aim of the study was to compare clinical characteristics, mortality, and major cardiovascular events in patients with versus without angiographic follow-up after PCI of the LMCA as well as to identify independent risk factors for death after PCI of the LMCA. Study population consisted of 217 patients of 290 consecutive participants who underwent PCI of the LMCA and subsequently were divided into 2 groups: angiographic follow-up group (angio FU group, n = 155) and clinical follow-up group (clinical FU group, n = 62). In angio FU group, significantly lower mortality (19.4% vs 32.3%, P < .05) and higher repeated revascularization rates (PCI: 46.5% vs 8.1%, P < .001 and coronary artery bypass grafting: 12.9% vs 1.6%, P < .05) were observed. Independent risk factors for death were as follows: metal stent implantation (hazard ratio [HR]: 2.753), no angiographic follow-up (HR: 1.959), and an increase in serum creatinine level of 1 μmol/L (HR: 1.006). These preliminary data suggest that the lack of angiographic follow-up after PCI of the LMCA may result in higher long-term mortality.

PMID: 26514417 [PubMed - indexed for MEDLINE]

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Motivations and Features of Co-Parenting an Infant With Complex Congenital Heart Disease.

West J Nurs Res. 2018 08;40(8):1110-1130

Authors: Pridham K, Harrison TM, McKechnie AC, Brown R

Abstract
This study described co-parenting motivations and quality of co-parenting features for parents of infants with complex congenital heart disease (CCHD), including differences over time by illness severity. Existing transcripts of parenting experience at infant age 1 and 12 months for 23 parent couples were analyzed using directed content analysis. Data were quantitized for additional description. Six co-parenting motivations and five co-parenting features were identified. Two co-parenting motivations had not been previously described: Developing the Co-Parenting Relationship and Maintaining the Couple Relationship. Variability in quality of co-parenting features was evident. However, the majority of parents showed high ratings. Motivations and quality of co-parenting features changed over time and were related to illness severity. Our findings increase the understanding of what parents are working on as a couple to parent an infant with CCHD and the quality of this co-parenting. Several questions for further study are presented.

PMID: 28597724 [PubMed - indexed for MEDLINE]

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A novel NR2F2 loss-of-function mutation predisposes to congenital heart defect.

Eur J Med Genet. 2018 Apr;61(4):197-203

Authors: Qiao XH, Wang Q, Wang J, Liu XY, Xu YJ, Huang RT, Xue S, Li YJ, Zhang M, Qu XK, Li RG, Qiu XB, Yang YQ

Abstract
Congenital heart defect (CHD) is the most common type of birth defect in humans and a leading cause of infant morbidity and mortality. Previous studies have demonstrated that genetic defects play a pivotal role in the pathogenesis of CHD. However, the genetic basis of CHD remains poorly understood due to substantial genetic heterogeneity. In this study, the coding exons and splicing boundaries of the NR2F2 gene, which encodes a pleiotropic transcription factor required for normal cardiovascular development, were sequenced in 168 unrelated patients with CHD, and a novel mutation (c.247G > T, equivalent to p.G83X) was detected in a patient with double outlet right ventricle as well as ventricular septal defect. Genetic scanning of the mutation carrier's relatives available showed that the mutation was present in all affected family members but absent in unaffected family members. Analysis of the index patient's pedigree displayed that the mutation co-segregated with CHD, which was transmitted as an autosomal dominant trait with complete penetrance. The nonsense mutation was absent in 230 unrelated, ethnically-matched healthy individuals used as controls. Functional deciphers by using a dual-luciferase reporter assay system revealed that the mutant NR2F2 protein had no transcriptional activity as compared with its wild-type counterpart. Furthermore, the mutation abrogated the synergistic transcriptional activation between NR2F2 and GATA4, another core cardiac transcription factor associated with CHD. This study firstly associates NR2F2 loss-of-function mutation with an increased susceptibility to double outlet right ventricle in humans, which provides further significant insight into the molecular mechanisms underpinning CHD, suggesting potential implications for genetic counseling of CHD families and personalized treatment of CHD patients.

PMID: 29222010 [PubMed - indexed for MEDLINE]

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Long-Standing Cyanosis in Congenital Heart Disease Does not Cause Diffuse Myocardial Fibrosis.

Pediatr Cardiol. 2018 Jan;39(1):105-110

Authors: Kharabish A, Meierhofer C, Hadamitzky M, Nadjiri J, Martinoff S, Ewert P, Stern H

Abstract
The assumption of the presence of diffuse myocardial fibrosis in long-standing cyanotic congenital heart disease (CHD) inspired us to noninvasively determine the myocardial extracellular volume (ECV) using contrast CMR. T1 maps were measured pre and 10 min after the injection of 0.15 mmol/kg of gadolinium in 25 subjects. Seven patients with long-standing cyanotic CHD and no previous cardiac surgery (aged 16-53 years and oxygen saturations of 69-90%), nine normal subjects (aged 14-49 years), and nine patients with previously cyanotic CHD, who had been corrected by open heart surgery (aged 2 months-58 years, mean 9 years). Late gadolinium enhancement was performed to exclude scar areas. The T1 values were measured in the interventricular septum and in the left lateral or inferior ventricular wall, such that same areas were assessed in every patient in the pre- and post-contrast T1 scan. ECV was calculated according to ΔR1myocardium/ΔR1blood * (1 - hematocrit). Cyanotic patients had significantly lower ECV percentage than the previous cyanotic patients (septum: 22 ± 2.7% vs 35 ± 4.6%, p = 0.002; LV wall: 22 ± 2.2% vs 30 ± 3.7%, p = 0.01, respectively). No significant differences were found between cyanotic patients and normal controls (septum: 22 ± 2.7% vs 24 ± 1.4%, p = 0.44; LV wall: 22 ± 2.2% vs 24 ± 2%, p = 0.57, respectively). Long-standing cyanosis in CHD without cardiac surgery does not cause diffuse myocardial fibrosis or expansion of the myocardial ECV.

PMID: 28948316 [PubMed - indexed for MEDLINE]

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Biopsychosocial Predictors of Quality of Life Outcomes in Pediatric Congenital Heart Disease.

Pediatr Cardiol. 2018 Jan;39(1):79-88

Authors: Ernst MM, Marino BS, Cassedy A, Piazza-Waggoner C, Franklin RC, Brown K, Wray J

Abstract
The purpose of this prospective multi-center cross-sectional study was to identify key biopsychosocial factors that impact quality of life (QOL) of youth with congenital heart disease (CHD). Patient-parent pairs were recruited at a regular hospital follow-up visit. Patient- and parent-proxy-reported QOL were assessed using the Pediatric Cardiac Quality of Life Inventory (PCQLI). Wallander's and Varni's disability-stress coping model guided factor selection, which included disease factors, educational impairment, psychosocial stress, child psychological and parent/family factors. Measures utilized for these factors included the Pediatric Inventory for Parents, Self-Perception Profile for Children/Adolescents, Child Behavior Checklist, Revised Children's Manifest Anxiety Scale, Child PTSD Symptom Scale, State-Trait Anxiety Inventory, and Posttraumatic Diagnostic Scale. Ordinary least squares regression was applied to test the theoretical model, with backwards stepwise elimination process. The models accounted for a substantial amount of variance in QOL (Patient-reported PCQLI R 2 = 0.58, p < 0.001; Parent-proxy-reported PCQLI R 2 = 0.60, p < 0.001). For patient-reported QOL, disease factors, educational impairment, poor self-esteem, anxiety, patient posttraumatic stress, and parent posttraumatic stress were associated with lower QOL. For parent-proxy-report QOL, disease factors, educational impairment, greater parental medical stress, poorer child self-esteem, more child internalizing problems, and parent posttraumatic stress were associated with lower QOL. The results highlight that biopsychosocial factors account for over half the variance in QOL in CHD survivors. Assessing and treating psychological issues in the child and the parent may have a significant positive impact on QOL.

PMID: 28980091 [PubMed - indexed for MEDLINE]

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Quantification of pulmonary regurgitation and prediction of pulmonary valve replacement by echocardiography in patients with congenital heart defects in comparison to cardiac magnetic resonance imaging.

Int J Cardiovasc Imaging. 2018 Apr;34(4):607-613

Authors: Dellas C, Kammerer L, Gravenhorst V, Lotz J, Paul T, Steinmetz M

Abstract
Pulmonary regurgitation (PR) is common in patients with congenital heart defects (CHD) and contributes to morbidity and mortality in the long-term. We investigated in this retrospective analysis whether readily accessible echocardiographic parameters are useful for quantification of PR and for predicting pulmonary valve replacement (PVR) in comparison to the gold-standard phase contrast (PC) flow measurements from cardiovascular magnetic resonance (CMR). Continuous wave (CW) Doppler and colour flow images in echocardiograms from 53 patients with CHD were analysed. Slope and jet-to-RVOT ratio correlated significantly with CMR-assessed regurgitation fraction (RF), whereas pressure half time (PHT) showed an inverse correlation. Patients with mild PR in CMR had significantly higher PHT, lower slope and jet-to-RVOT ratio than patients with moderate or severe regurgitation. The AUC regarding PR severity was 0.778 for PHT (95% CI, 0.649-0.907; P = 0.007 for CMR-RF ≤ 35%), 0.744 for slope (95% CI, 0.603-0.885; P = 0.017 for CMR-RF > 35%) and 0.652 for jet-to-RVOT ratio (95% CI, 0.473-0.860; P = 0.168 for CMR-RF > 35%). The optimal cut-off values calculated from ROC analysis were 95 ms for PHT and 4.9 m/s2 for slope. In logistic regression analysis, slope emerged as the most valuable parameter for predicting the indication for PVR (OR 12.9, 95% CI, 1.8-90.9, P = 0.010). In conclusion, echocardiographic assessment of PR was feasible. Both parameters, PHT and in particular slope, were predictors for PVR. Thus, echocardiography appears appropriate in the management of patients with PR.

PMID: 29119274 [PubMed - indexed for MEDLINE]

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