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CMR feature tracking left ventricular strain-rate predicts ventricular tachyarrhythmia, but not deterioration of ventricular function in patients with repaired tetralogy of Fallot.

Int J Cardiol. 2019 11 15;295:1-6

Authors: Hagdorn QAJ, Vos JDL, Beurskens NEG, Gorter TM, Meyer SL, van Melle JP, Berger RMF, Willems TP

BACKGROUND: Myocardial strain has been shown to predict outcome in various cardiovascular diseases, including congenital heart diseases. The aim of this study was to evaluate the predictive value of cardiac magnetic resonance (CMR) feature-tracking derived strain parameters in repaired tetralogy of Fallot (rTOF) patients for developing ventricular tachycardia (VT) and deterioration of ventricular function.
METHODS: Patients with rTOF who underwent CMR investigation were included. Strain and strain-rate of both ventricles were assessed using CMR feature tracking. The primary outcome was a composite of the occurrence of sustained VT or non-sustained VT requiring invasive therapy. The secondary outcome was analyzed in patients that underwent a second CMR after 1.5 to 3.5 years. Deterioration was defined as reduction (≥10%) in right ventricular (RV) ejection fraction, reduction (≥10%) in left ventricular (LV) ejection fraction or increase (≥30 mL/m2) in indexed RV end-diastolic volume compared to baseline.
RESULTS: 172 patients (median age 24.3 years, 54 patients <18 years) were included. Throughout a median follow-up of 7.4 years, 9 patients (4.5%) experienced the primary endpoint of VT. Multivariate Cox-regression analysis showed that LV systolic circumferential strain-rate was independently predictive of primary outcome (p = 0.023). 70 patients underwent a serial CMR, of whom 14 patients (20%) showed ventricular deterioration. Logistic regression showed no predictive value of strain and strain-rate parameters.
CONCLUSIONS: In patients with rTOF, LV systolic circumferential strain-rate is an independent predictor for the development of VT. Ventricular strain parameters did not predict deterioration of ventricular function in the studied population.

PMID: 31402156 [PubMed - indexed for MEDLINE]

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Insights from circulating microRNAs in cardiovascular entities in turner syndrome patients.

PLoS One. 2020;15(4):e0231402

Authors: Abu-Halima M, Oberhoffer FS, El Rahman MA, Jung AM, Zemlin M, Rohrer TR, Kahraman M, Keller A, Meese E, Abdul-Khaliq H

BACKGROUND: Turner syndrome (TS) is a chromosomal disorder, in which a female is partially or entirely missing one of the two X chromosomes, with a prevalence of 1:2500 live female births. The present study aims to identify a circulating microRNA (miRNA) signature for TS patients with and without congenital heart disease (CHD).
METHODS: Microarray platform interrogating 2549 miRNAs were used to detect the miRNA abundance levels in the blood of 33 TS patients and 14 age-matched healthy volunteer controls (HVs). The differentially abundant miRNAs between the two groups were further validated by RT-qPCR.
RESULTS: We identified 60 differentially abundant miRNA in the blood of TS patients compared to HVs, from which, 41 and 19 miRNAs showed a higher and a lower abundance levels in TS patients compared to HVs, respectively. RT-qPCR confirmed the significantly higher abundance levels of eight miRNAs namely miR-374b-5p, miR-199a-5p, miR-340-3p, miR-125b-5p, miR-30e-3p, miR-126-3p, miR-5695, and miR-26b-5p in TS patients as compared with the HVs. The abundance level of miR-5695 was higher in TS patients displaying CHD as compared to TS patients without CHD (p = 0.0265; log2-fold change 1.99); whereas, the abundance level of miR-126-3p was lower in TS patients with congenital aortic valve disease (AVD) compared to TS patients without BAV (p = 0.0139, log2-fold change 1.52). The clinical feature statistics revealed that miR-126-3p had a significant correlation with sinotubular junction Z-score (r = 0.42; p = 0.0154).
CONCLUSION: The identified circulating miRNAs signature for TS patients with manifestations associated with cardiovascular diseases provide new insights into the molecular mechanism of TS that may guide the development of novel diagnostic approaches.

PMID: 32271829 [PubMed - indexed for MEDLINE]

Cardiac resynchronization therapy for the failing systemic right ventricle: A systematic review.

Int J Cardiol. 2020 Jul 06;:

Authors: Kharbanda RK, Moore JP, Taverne YJHJ, Bramer WM, Bogers AJJC, de Groot NMS

Patients with a systemic right ventricle (SRV) are at high risk for development of heart failure early in life. An SRV is encountered in patients with congenitally corrected transposition of the great arteries (CCTGA) or dextro-transposition of the great arteries (DTGA) with previous atrial switch repair (Mustard or Senning procedure). Progressive heart failure is one of the leading cause of mortality in these patients. Therefore, cardiac resynchronization therapy (CRT) has gained increasing momentum for use in this challenging congenital heart disease (CHD) population. However, current guidelines differ in recommendations for CRT in patients with an SRV as evidence supporting CRT has thus far only been described in case reports and retrospectively in relatively small study populations. In fact, the European Society of Cardiology Guideline for the management of grown-up congenital heart disease consider CRT to be 'experimental' in this population. This systematic review critically summarizes current literature on CRT in SRV patients and provides future perspectives for further research in this challenging and growing CHD population.

PMID: 32645324 [PubMed - as supplied by publisher]

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[Risk factors for death and the clinical features of different subtypes of patients with pulmonary arterial hypertension related to congenital heart disease].

Zhonghua Xin Xue Guan Bing Za Zhi. 2020 Apr 24;48(4):315-322

Authors: Xu ZY, Li QQ, Zhang C, Zhang HS, Gu H

Objective: To explore the risk factors for death in patients with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) and the clinical characteristics of different subtypes in patients with PAH-CHD. Methods: It was a retrospective study. A total of 507 PAH-CHD patients, who were hospitalized in the Department of Pediatric Cardiology of Beijing Anzhen Hospital between September 2005 and May 2019, were included. Patients were divided into 4 subgroups: (1) Eisenmenger syndrome(ES) group. (2) PAH associated with prevalent systemic-to-pulmonary shunts(SP) group. (3) PAH associated with small defects(SD) group. (4) PAH after defect correction(CD) group. According to the complexity of cardiac malformation, patients were divided into simple-medium complex malformation group and complex malformation group. According to the location of shunts, patients were divided into pre-tricuspid group, post-tricuspid group, and mixed group or complex deformity group. Baseline clinical data of patients were collected from the electronic medical record system. Demographic data (age, gender, etc.), percutaneous oxygen saturation(SPO(2)), New York Heart Association(NYHA) cardiac function classification, 6 minutes walking distance(6MWD), and B type natriuretic peptide(BNP), systolic pulmonary arterial pressure(sPAP) estimated by echocardiography and mean pulmonary artery pressure (mPAP), mean right atrial pressure(mRAP), cardiac index(CI), and calculated pulmonary vascular resistance (PVR) estimated by right heart catheterization were compared among various groups. The results of regular follow-up of all enrolled patients were collected, including the status of monotherapy or combination of PAH-targeted drugs during the follow-up period, cardiac-related adverse events(hemopysis, syncope, edema, arrhythmia, etc.) and primary endpoint event(all-cause death) were obtained and analyzed. Risk factors for all-cause death were analyzed using univariate and multivariate Cox regression analysis model. Results: The median age at diagnosis was 23.1(13.9,32.1) years, 345 cases(68.0%) were female. Two hunderds and thirty-five cases(46.4%) were diagnosed with ES; 193 cases(38.1%) were diagnosed with CD, 47 cases (9.3%) were diagnosed with SD. Among them, 32 cases(6.3%) were in the SP group. All 507 patients underwent echocardiography examination, there were significant differences in sPAP among different clinical subgroups(P<0.001). A total of 289 patients(57.0%) received right heart catheterization examination, the results showed that the ES group had the highest mPAP and PVR and the lowest mRAP(all P<0.001), the CD group had the highest mRAP and CI(both P<0.001). The 6MWD in the ES group was significantly shorter than that in the SP, SD, and CD groups(all P<0.001). The proportion of patients with NYHA class Ⅲ/Ⅳ was higher in SD group than in SP group(P<0.001), which was similar between SD, ES and CD groups (P values were 0.077 and 0.072, respectively). At admission, the proportion of patients with NYHA class Ⅰ/Ⅱwas the highest in SP group(96.9% (31/32) ), followed by CD group (85.5%(165/193)) and the ES group(85.1%(200/235)), and the SD group(75.0%(35/47)). The BNP level at admission was also higher in SD group than in SP, ES and CD groups(P<0.001). Of the 507 patients, 379(74.8%) patients received PAH-targeted drug therapy at the last follow-up, and the treatment plan was mainly monotherapy(75.7%(287/379)). The median follow-up time was 3.6(2.0, 5.6) years and 37(7.3%) patients died, including 13 in the CD group, 17 in the ES group, and 7 in the SD group. No deaths occurred in the SP group. Right heart failure was the most common cause of death(11(29.7%)), followed by severe hemoptysis dyspnea(7(18.9%)), sudden cardiac death(6(16.2%)), and pulmonary hypertensive crisis(4(10.8%)). Kaplan-Meier curve showed that survival rates of end-point-free events at 1, 3, 5 and 10 years after diagnosis of PAH were 98.0%, 95.4%, 89.9%, and 84.4%, respectively; there were statistically significant differences in survival among the subgroups(P=0.026); there was no significant difference in the survival rate between the ES group and the CD group(P=0.918), and both were higher than the SD group(P values were 0.011 and 0.013, respectively). Univariate Cox regression analysis showed that NYHA class Ⅲ/Ⅳ and BNP>100 ng/L at admission were the risk factors for all-cause death in patients with PAH-CHD(HR=6.452, 95%CI 3.378-12.346, P<0.001, and HR=2.481, 95%CI 1.225-5.025, P=0.012). Multivariate Cox regression analysis showed that NYHA class Ⅲ/Ⅳ was an independent risk factor for all-cause death in patients with PAH-CHD(HR=4.998, 95%CI 1.246-20.055, P=0.023). Conclusions: PAH-CHD patients with different clinical subtypes have different clinical symptoms, cardiac functional class, hemodynamic characteristics, and mid to long-term survival rates. SP patients have the best prognosis, outcome of ES and CD patients is similar, and SD patients have the worst prognosis. NYHA class Ⅲ/Ⅳ is an independent risk factor for all-cause death in patients with PAH-CHD.

PMID: 32370483 [PubMed - indexed for MEDLINE]

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Invasive Hemodynamic Characteristics in Patients Undergoing Transcatheter Tricuspid Valve-In-Valve Implantation for Treatment of Tricuspid Stenosis.

World J Pediatr Congenit Heart Surg. 2020 Jul;11(4):411-416

Authors: Patel K, Sadeghi S, Aboulhosn J

BACKGROUND: We sought to describe invasive hemodynamic measurements in patients with tricuspid stenosis (TS) undergoing transcatheter tricuspid valve-in-valve (TVIV) implantation immediately pre- and postimplantation. Development of TS in patients who have undergone surgical tricuspid valve replacement with a bioprosthetic valve is a serious complication that leads to elevated right atrial (RA) pressures and decreased cardiac output. Transcatheter TVIV implantation is a viable alternative to surgical tricuspid valve replacement, but data on the hemodynamic consequences of TVIV for the treatment of severe TS are currently limited to echocardiographic assessment of Doppler-derived gradients.
METHODS: Eleven patients undergoing transcatheter TVIV implantation with moderate to severe bioprosthetic valve stenosis were selected for retrospective review. Right atrial mean pressure, right ventricular (RV) systolic and end-diastolic pressure, mean diastolic RA-RV pressure gradient, pulmonary artery capillary wedge pressure, pulmonary artery systolic, end-diastolic and mean pressures, and pulmonary artery pulsatility index (PAPi) both before and after transcatheter valve placement were collected from catheterization reports.
RESULTS: After transcatheter TVIV implantation, the mean TS gradient decreased significantly (P < .01), while the mean RV end-diastolic pressure increased (P = .046). Pulmonary artery pulsatility index also increased as the TS was relieved (P = .039).
CONCLUSIONS: Tricuspid valve-in-valve implantation results in immediate relief of TS, leading to increased RV preload with resultant augmentation of RV and pulmonary pressures. Increased PAPi following the procedure demonstrates acute improvement in RV output but remains low due to the failure of the RA pressure to decline significantly immediately following intervention.

PMID: 32645780 [PubMed - in process]

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Combined Transcatheter Tricuspid and Pulmonary Valve Replacement.

World J Pediatr Congenit Heart Surg. 2020 Jul;11(4):432-437

Authors: Small AJ, Aksoy O, Levi DS, Salem MM, Yang EH, Aboulhosn JA

BACKGROUND: For patients with combined tricuspid and pulmonary valve disease, operative intervention carries high mortality risk. Published reports of combined transcatheter tricuspid and pulmonary valve replacement have been limited to single cases.
METHODS: A retrospective chart review was performed including all patients undergoing combined transcatheter tricuspid and pulmonary valve replacement at the Ahmanson/UCLA Adult Congenital Heart Disease Center.
RESULTS: Combined transcatheter tricuspid and pulmonary valve replacement was undertaken in five adult patients (median age: 46 years; range: 24-64 years). Three had congenital heart disease and two had carcinoid syndrome. Four patients had previous surgical tricuspid valve replacement and one had a surgical incomplete annuloplasty ring. Four patients had previous surgical pulmonary valve replacement and one had a right ventricle-to-pulmonary artery homograft conduit. Two patients underwent Medtronic Melody valve implantation in both tricuspid and pulmonary positions and three underwent Edwards Sapien S3 implantation in both tricuspid and pulmonary positions. Valve implantation was successful in all. Follow-up ranged from 0.9 to 3.0 years. One patient underwent redo transcatheter tricuspid valve replacement 12 months after the first transcatheter intervention for progressive regurgitation of a Melody valve. This patient died 2.5 years after combined valve placement of complications from refractory heart failure. The remaining patients were alive and free of valve reintervention at follow-up.
CONCLUSIONS: Combined transcatheter tricuspid and pulmonary valve replacement can be performed successfully, with an acceptable complication rate. This strategy is a feasible option for appropriately selected patients.

PMID: 32645786 [PubMed - in process]

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Characteristics of Adults With Congenital Heart Defects in the United States.

J Am Coll Cardiol. 2020 Jul 14;76(2):175-182

Authors: Gurvitz M, Dunn JE, Bhatt A, Book WM, Glidewell J, Hogue C, Lin AE, Lui G, McGarry C, Raskind-Hood C, Van Zutphen A, Zaidi A, Jenkins K, Riehle-Colarusso T

BACKGROUND: In the United States, >1 million adults are living with congenital heart defects (CHDs), but gaps exist in understanding the health care needs of this growing population.
OBJECTIVES: This study assessed the demographics, comorbidities, and health care use of adults ages 20 to 64 years with CHDs.
METHODS: Adults with International Classification of Disease-9th Revision-Clinical Modification CHD-coded health care encounters between January 1, 2008 (January 1, 2009 for Massachusetts) and December 31, 2010 were identified from multiple data sources at 3 U.S. sites: Emory University (EU) in Atlanta, Georgia (5 counties), Massachusetts Department of Public Health (statewide), and New York State Department of Health (11 counties). Demographics, insurance type, comorbidities, and encounter data were collected. CHDs were categorized as severe or not severe, excluding cases with isolated atrial septal defect and/or patent foramen ovale.
RESULTS: CHD severity and comorbidities varied across sites, with up to 20% of adults having severe CHD and >50% having ≥1 additional cardiovascular comorbidity. Most adults had ≥1 outpatient encounters (80% EU, 90% Massachusetts, and 53% New York). Insurance type differed across sites, with Massachusetts having a large proportion of Medicaid (75%) and EU and New York having large proportions of private insurance (44% EU, 67% New York). Estimated proportions of adults with CHD-coded health care encounters varied greatly by location, with 1.2 (EU), 10 (Massachusetts), and 0.6 (New York) per 1,000 adults based on 2010 census data.
CONCLUSIONS: This was the first surveillance effort of adults with CHD-coded inpatient and outpatient health care encounters in 3 U.S. geographic locations using both administrative and clinical data sources. This information will provide a clearer understanding of health care use in this growing population.

PMID: 32646567 [PubMed - in process]

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ACHD Care in the United States: Complex Patients, Even More Complex Health Care System.

J Am Coll Cardiol. 2020 Jul 14;76(2):183-185

Authors: Tompkins R, Khan A

PMID: 32646568 [PubMed - in process]

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Genetics in congenital heart disease. Are we ready for it?

Rev Esp Cardiol (Engl Ed). 2020 Jul 06;:

Authors: De Backer J, Callewaert B, Muiño Mosquera L

Genetics has rightly acquired an important place in almost all medical disciplines in recent years and this is certainly the case in the field of congenital cardiology. Not only has this led to greater insight into the pathophysiology of congenital heart defects but it also has a beneficial impact on patient management. Integration of clinical genetics in multidisciplinary centers of expertise for CHD is therefore a clear recommendation. Adult and pediatric cardiologists play a crucial role in the process of genetic evaluation of patients and families and should have be familiar with red flags for referral for further clinical genetic elaboration, counseling, and eventual testing. Some basic knowledge is also important for the correct interpretation of genetic testing results. In this review article, we provide a practical overview of what genetic evaluation entails, which type of genetic tests are possible today, and how this can be used in practice for the individual patient.

PMID: 32646792 [PubMed - as supplied by publisher]

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Lifetime Burden of Adult Congenital Heart Disease in the USA Using a Microsimulation Model.

Pediatr Cardiol. 2020 Jul 10;:

Authors: Gong CL, Zhao H, Wei Y, Tysinger B, Goldman DP, Williams RG

Congenital heart defects (CHD) represent a growing burden of illness among adults. We estimated the lifetime health, education, labor, and social outcomes of adults with CHD in the USA using the Future Adult Model, a dynamic microsimulation model that has been used to study the lifetime impacts of a variety of chronic diseases. We simulated a cohort of adult heads of households > 25 years old derived from the Panel Survey of Income Dynamics who reported a childhood heart problem as a proxy for CHD and calculated life expectancy, disability-free and quality-adjusted life years, lifetime earnings, education attainment, employment, development of chronic disease, medical spending, and disability insurance claiming status. Total burden of disease was estimated by comparing to a healthy cohort with no childhood heart problem. Eighty-seven individuals reporting a childhood heart problem were identified from the PSID and were used to generate the synthetic cohort simulated in the model. Life expectancy, disability-free, quality-adjusted, and discounted quality-adjusted life years were an average 4.6, 6.7, 5.3, and 1.4 years lower than in healthy adults. Lung disease, cancer, and severe mental distress were more common compared to healthy individuals. The CHD cohort earned $237,800 less in lifetime earnings and incurred higher average total medical spend by $66,600 compared to healthy individuals. Compared to healthy adults, the total burden of CHD is over $500K per adult. Despite being among the healthiest adults with CHD, there are significant decrements in life expectancy, employment, and lifetime earnings, with concomitant increases in medical spend.

PMID: 32651615 [PubMed - as supplied by publisher]