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Incidence and outcome of infective endocarditis following percutaneous versus surgical pulmonary valve replacement.

Catheter Cardiovasc Interv. 2018 02 01;91(2):277-284

Authors: Lluri G, Levi DS, Miller E, Hageman A, Sinha S, Sadeghi S, Reemtsen B, Laks H, Biniwale R, Salem M, Fishbein GA, Aboulhosn J

Abstract
OBJECTIVES: To provide a comparison of the outcome of infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TPVR) versus surgical pulmonary valve replacement (SPVR).
BACKGROUND: Although TPVR is thought to be associated with a higher risk of IE than SPVR, there is paucity of data to support this.
METHODS: Patients who underwent TPVR or SPVR at UCLA between October 2010 and September 2016 were included and retrospectively analyzed.
RESULTS: Three hundred forty-two patients underwent PVR at UCLA including 134 SPVR and 208 TPVR. Patients undergoing TPVR were more likely to have had a history of endocarditis than those undergoing SPVR (5.3% vs. 0.7%, P = 0.03) and a right ventricle to pulmonary artery (RV to PA) conduit (37% vs. 17%, P = 0.0001). Two SPVR and seven TPVR patients developed IE with a 4-year freedom from endocarditis of 94.0% in the SPVR versus 84% in the TPVR group (P = 0.13). In patients who underwent TPVR and developed endocarditis, the mean gradient across the RVOT prior to intervention was higher (28.1 ± 4.5 vs. 17.4 ± 0.6 mmHg, P = 0.02) and were more likely to have a conduit (71% vs. 36%, P = 0.049).
CONCLUSIONS: In this study, patients undergoing TPVR were not at a higher risk of IE than patients undergoing SPVR. TPVR patients were more likely to have had a prior history of IE and RV-PA conduit. The patients at highest risk were those with stenotic RV to PA conduits who were treated with TPVR.

PMID: 28895275 [PubMed - indexed for MEDLINE]

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New York Heart Association (NYHA) classification in adults with congenital heart disease: relation to objective measures of exercise and outcome.

Eur Heart J Qual Care Clin Outcomes. 2018 01 01;4(1):51-58

Authors: Bredy C, Ministeri M, Kempny A, Alonso-Gonzalez R, Swan L, Uebing A, Diller GP, Gatzoulis MA, Dimopoulos K

Abstract
Aims: The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality.
Methods and results: This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P < 0.0001). Although a large number of 'asymptomatic' (NYHA class 1) patients did not achieve a 'normal' peak VO2, the NYHA class was a strong predictor of mortality, with an 8.7-fold increased mortality risk in class 3 compared with class 1 (hazard ratio 8.68, 95% confidence interval: 5.26-14.35, P < 0.0001).
Conclusion: Despite underestimating the degree of limitation in some ACHD patients, NYHA classification remains a valuable clinical tool. It correlates with objective measures of exercise and the severity of underlying cardiac disease, as well as mid- to long-term mortality and should, thus, be into incorporated the routine assessment and risk stratification of these patients.

PMID: 28950356 [PubMed - indexed for MEDLINE]

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A Functional Aryl Hydrocarbon Receptor Genetic Variant, Alone and in Combination with Parental Exposure, is a Risk Factor for Congenital Heart Disease.

Cardiovasc Toxicol. 2018 06;18(3):261-267

Authors: Pulignani S, Borghini A, Vecoli C, Foffa I, Ait-Ali L, Andreassi MG

Abstract
Recent experimental studies showed that ablation of the aryl hydrocarbon receptor (AhR) as well as its activation by exogenous ligands disrupt the molecular networks involved in heart formation and function, leading to congenital heart disease (CHD). However, no evidence is available about the role of AhR in humans. We assessed the prevalence of a functional AhR genetic variant (p.Arg554Lys) in CHD patients as well as its joint effects with parental exposure. A total of 128 CHD patients (76 males; age 6.2 ± 6.7 years) and 274 controls (160 males; age at birth) were genotyped for the AhR polymorphism by using the TaqMan® Drug Metabolism Genotyping assay. Both case and control parents completed a structured questionnaire on demographic, lifestyle and preconception exposures. Genotype (p = 0.001) and allele (p < 0.0001) distributions of AhR p.Arg554Lys differed significantly between patients and controls. A significant elevated CHD risk was found under dominant (OR = 2.9, 95% CI 1.9-4.6, p < 0.0001) and additive genetic models (OR = 6.2, 95% CI 2-19, p = 0.001). There was a significant interaction between 554-Lys allele and paternal smoking exposure (ORsmoking = 1.6, 95% CI = 0.9-2.9; ORallele = 2.6, 95% CI = 1.3-5; ORinteraction = 4.9, 95% CI = 2.4-9.9, p interaction < 0.0001). Additionally, 554-Lys allele exacerbated the effect of maternal periconceptional exposure (ORexposure = 1.6, 95% CI = 0.8-3; ORallele = 2.6, 95% CI = 1.5-4.5; ORinteraction = 5.7; 95% CI = 2.6-12, p interaction < 0.0001). Our findings showed that the AhR p.Arg554Lys polymorphism, alone and in combination with parental exposures, is associated with the CHD risk, highlighting the significant role of AhR in the cardiovascular development.

PMID: 29185192 [PubMed - indexed for MEDLINE]

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Comprehensive microRNA profiling reveals potential augmentation of the IL1 pathway in rheumatic heart valve disease.

BMC Cardiovasc Disord. 2018 03 16;18(1):53

Authors: Lu Q, Sun Y, Duan Y, Li B, Xia J, Yu S, Zhang G

Abstract
BACKGROUND: Valvular heart disease is a leading cause of cardiovascular mortality, especially in China. More than a half of valvular heart diseases are caused by acute rheumatic fever. microRNA is involved in many physiological and pathological processes. However, the miRNA profile of the rheumatic valvular heart disease is unknown. This research is to discuss microRNAs and their target gene pathways involved in rheumatic heart valve disease.
METHODS: Serum miRNA from one healthy individual and four rheumatic heart disease patients were sequenced. Specific differentially expressed miRNAs were quantified by Q-PCR in 40 patients, with 20 low-to-moderate rheumatic mitral valve stenosis patients and 20 severe mitral valve stenosis patients. The target relationship between certain miRNA and predicted target genes were analysis by Luciferase reporter assay. The IL-1β and IL1R1 expression levels were analyzed by immunohistochemistry and western blot in the mitral valve from surgery of mitral valve replacement.
RESULTS: The results showed that 13 and 91 miRNAs were commonly upregulated or downregulated in all four patients. Nine miRNAs, 1 upregulated and 8 downregulated, that had a similar fold change in all 4 patients were selected for quantitative PCR verification. The results showed similar results from miRNA sequencing. Within these 9 tested miRNAs, hsa-miR-205-3p and hsa-miR-3909 showed a low degree of dispersion between the members of each group. Hsa miR-205-3p and hsa-miR-3909 were predicted to target the 3'UTR of IL-1β and IL1R1 respectively. This was verified by luciferase reporter assays. Immunohistochemistry and Western blot results showed that the mitral valve from rheumatic valve heart disease showed higher levels of IL- 1β and IL1R1 expression compared with congenital heart valve disease. This suggested a difference between rheumatic heart valve disease and other types of heart valve diseases, with more inflammatory responses in the former.
CONCLUSION: In the present study, by next generation sequencing of miRNAs, it was revealed that interleukin 1β and interleukin 1 receptor 1 was involved in rheumatic heart diseases. And this is useful for diagnosis and understanding of mechanism of rheumatic heart disease.

PMID: 29548280 [PubMed - indexed for MEDLINE]

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Asymptomatic young man with an incidental murmur.

Heart. 2018 08;104(15):1307

Authors: Shojaeifard M, Pouraliakbar HR, Houshmand G

Abstract
CLINICAL INTRODUCTION: A 32-year old man was referred to our institution for transthoracic echocardiography (TTE) following detection of an incidental murmur on physical examination before blood donation. He was asymptomatic with no significant medical history. Physical examination revealed dual heart sounds with a grade II/VI systolic murmur heard in the left sternal border. An ECG was in normal sinus rhythm. TTE was performed (figure 1A-C, online supplementary videos 1-4) followed by cardiac CT angiography (CTA) (figure 1D,E).heartjnl;104/15/1307/F1F1F1Figure 1(A) Transthoracic echocardiography, parasternal left ventricular long axis view. (B) Colour Doppler of modified short axis in the mid-left ventricular level. (C) Doppler flow velocity profile. (D) Cardiac CT angiography (CTA) sagittal reconstruction. (E) Three-dimensional CTA reconstruction of the heart.
QUESTION: What is the diagnosis?Pericardial cyst.Ventricular septal defect.Kawasaki.Anomalous left coronary artery from pulmonary artery (ALCAPA).

PMID: 29853486 [PubMed - indexed for MEDLINE]

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Contraceptive Choices in the Immediate Postpartum Period in Women With Cardiac Disease.

Am J Cardiol. 2019 Jan 25;:

Authors: Sobhani NC, Schultz H, Kheiwa A, Killion M, Parikh NI, Harris IS, Gonzalez JM, Agarwal A

Abstract
Maternal cardiac disease (MCD) is associated with increased maternal and neonatal morbidity and mortality. Because unplanned pregnancies are especially risky, active use of reliable contraception is critical in this population. Studies in the noncardiac population have demonstrated that the postpartum period is an ideal time to address contraceptive plans. This retrospective cohort study was designed to describe contraceptive choices in women with MCD in the immediate postpartum period and to identify factors associated with specific contraceptive plans. We included women with MCD who delivered from January 2008 to September 2017 at a tertiary care institution with a multidisciplinary obstetrics and cardiology team. Maternal demographics, specifics of MCD, obstetrical outcomes, and contraceptive plans were obtained through chart review. Contraceptive plans were categorized into highly reliable methods (sterilization or long-acting reversible contraceptive methods) or less reliable methods (nonlong-acting reversible contraceptive methods or no contraception). In the 254 women included in this study, 40% planned to use highly reliable methods, while 60% planned to use less reliable methods. Women with cardiomyopathy were more likely to choose a highly reliable method of contraception (adjusted odds ratio 2.6, 95% confidence interval 1.2 to 5.7), a reassuring finding, given the particularly high risk of poor pregnancy outcome with this diagnosis. There were no differences in other cardiac diagnoses between the 2 contraceptive groups. In conclusion, the finding that <50% of postpartum women with MCD plan to use a highly reliable method of contraception warrants further examination to identify and address barriers to reliable contraceptive plans in this high-risk population.

PMID: 30712772 [PubMed - as supplied by publisher]

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Atrial fibrillation in adults with congenital heart disease.

Int J Cardiol. 2019 Jan 26;:

Authors: Waldmann V, Laredo M, Abadir S, Mondésert B, Khairy P

Abstract
A convergence of epidemiological and clinical features has prompted a precipitous rise in the prevalence of atrial fibrillation (AF) in the expanding population of adults with congenital heart disease. Herein, we synthesize the current state of knowledge on epidemiological features, associated morbidities, and pathophysiological insights regarding AF in adults with congenital heart disease. Management issues are examined including surgical, pharmacological, and catheter-based therapies. Throughout, knowledge gaps and avenues for future research are identified. Although AF has been coined the next arrhythmic epidemic to strike adults with congenital heart disease, it has already usurped atrial macroreentrant tachycardia as the most common presenting arrhythmia over the age of 50 years. Much remains to be discovered about the mitigating role of types of congenital defects, residual hemodynamic lesions, surgical sequelae, and ramifications of shunts and cyanosis on mechanisms and determinants of AF. Thromboprophylaxis is the cornerstone of pharmacological management, with anticoagulation recommended in patients with moderate or complex congenital heart disease and those with significant valve disease or risk factors for stroke. Considering the limited success with antiarrhythmic drugs, catheter ablation is increasingly performed. Non-pulmonary vein sources, focal and reentrant arrhythmias, appear to be important triggers for AF in this population. As such, they should be identified and addressed during catheter ablation interventions. The nascent literature on electrical isolation of pulmonary veins suggests that it is feasible and safe, although initial success rates appear to be modest. A more thorough understanding of underlying mechanisms and substrates carries the potential to further improve outcomes.

PMID: 30712848 [PubMed - as supplied by publisher]

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Sarcopenia in adults with congenital heart disease: Nutritional status, dietary intake, and resistance training.

J Cardiol. 2019 Jan 31;:

Authors: Shiina Y, Matsumoto N, Okamura D, Takahashi Y, Kijima Y, Fukuda T, Kawamatsu N, Nishihata Y, Komiyama N, Niwa K

Abstract
BACKGROUND: This study aimed (1) to assess the nutritional status and dietary intake, (2) compare the body composition and nutritional intake between sarcopenia and non-sarcopenia, and (3) evaluate the effects of resistance training and amino acid intake in adults with congenital heart disease (CHD).
METHODS: Study 1 In total, 172 adults with CHD were prospectively enrolled. The Food Frequency Questionnaire was used, and body composition analysis was conducted. Study 2: Thirty of 172 adult patients with CHD were divided into two groups: amino acid intake plus resistance training (group A) and amino acid intake only (group B) for 2 months.
RESULTS: Study 1: Skeletal muscle mass index was lower in adults with CHD compared to healthy Japanese. Calorie, protein, and fat intake in adults with CHD was higher than those in the National Nutritive Intake Investigation; however, the difference in carbohydrate or salt intake was non-significant. Study 2: In adults with CHD in group A, body fat percentage, edema index, and N-terminal prohormone of brain natriuretic peptide improved, and body weight, skeletal muscle mass index, and basic metabolism increased after the intervention. There was no improvement after intervention for group B.
CONCLUSIONS: According to this study, adults with CHD have higher calorie, protein, and fat intake than those in a national survey despite decreased skeletal muscle mass. Amino acid intake plus resistance training positively improved body fat percentage, skeletal muscle mass, and edema in adults with CHD. Appropriate nutritional education and resistance training guidelines should be provided.

PMID: 30713126 [PubMed - as supplied by publisher]

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Education as important predictor for successful employment in adults with congenital heart disease worldwide.

Congenit Heart Dis. 2019 Feb 04;:

Authors: Sluman MA, Apers S, Sluiter JK, Nieuwenhuijsen K, Moons P, Luyckx K, Kovacs AH, Thomet C, Budts W, Enomoto J, Yang HL, Jackson JL, Khairy P, Cook SC, Subramanyan R, Alday L, Eriksen K, Dellborg M, Berghammer M, Mattsson E, Mackie AS, Menahem S, Caruana M, Gosney K, Soufi A, Fernandes SM, White KS, Callus E, Kutty S, Bouma BJ, Mulder BJM, APPROACH-IS consortium, the International Society for Adult Congenital Heart Disease (ISACHD)

Abstract
BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort.
METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models.
RESULTS: Median age was 32 years (IQR 25-42) and 94% of patients had at least a high school degree. Overall employment rate was 69%, but varied substantially among countries. Higher education (OR 1.99-3.69) and having a partner (OR 1.72) were associated with more employment; female sex (OR 0.66, worse NYHA functional class (OR 0.67-0.13), and a history of congestive heart failure (OR 0.74) were associated with less employment. Limitations at work were reported in 34% and were associated with female sex (OR 1.36), increasing age (OR 1.03 per year), more severe CHD (OR 1.31-2.10), and a history of congestive heart failure (OR 1.57) or mental disorders (OR 2.26). Only a university degree was associated with fewer limitations at work (OR 0.62).
CONCLUSIONS: There are genuine differences in the impact of CHD on employment status in different countries. Although the majority of adult CHD patients are employed, limitations at work are common. Education appears to be the main predictor for successful employment and should therefore be encouraged in patients with CHD.

PMID: 30714326 [PubMed - as supplied by publisher]

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Successful everolimus-eluting stent implantation into the left main trunk stenosis in the anomalous coronary artery after neo-aortic valve replacement in a 6-year-old boy.

Cardiol Young. 2019 Feb 04;:1-3

Authors: Asada D, Tomita H, Fujii T

Abstract
CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.

PMID: 30714565 [PubMed - as supplied by publisher]

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