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Geographical variation and predictors of physical activity level in adults with congenital heart disease.

Int J Cardiol Heart Vasc. 2019 Mar;22:20-25

Authors: Larsson L, Johansson B, Sandberg C, Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Alday L, Eriksen K, Dellborg M, Berghammer M, Rempel G, Menahem S, Caruana M, Tomlin M, Soufi A, Fernandes SM, White K, Callus E, Kutty S, Moons P, APPROACH-IS Consortium, International Society for Adult Congenital Heart Disease (ISACHD)

Background: Physical activity is important to maintain and promote health. This is of particular interest in patients with congenital heart disease (CHD) where acquired heart disease should be prevented. The World Health Organization (WHO) recommends a minimum of 2.5 h/week of physical activity exceeding 3 metabolic equivalents (METS) to achieve positive health effects. It is unknown whether physical activity levels (PAL) in adult CHD patients differ by country of origin.
Methods: 3896 adults with CHD recruited from 15 countries over 5 continents completed self-reported instruments, including the Health Behaviour Scale (HBS-CHD), within the APPROACH-IS project. For each patient, we calculated whether WHO recommendations were achieved or not. Associated factors were investigated using Generalized Linear Mixed Models.
Results: On average, 31% reached the WHO recommendations but with a great variation between geographical areas (India: 10%-Norway: 53%). Predictors for physical activity level in line with the WHO recommendations, with country of residence as random effect, were male sex (OR 1.78, 95%CI 1.52-2.08), NYHA-class I (OR 3.10, 95%CI 1.71-5.62) and less complex disease (OR 1.46, 95%CI 1.16-1.83). In contrast, older age (OR 0.97, 95%CI 0.96-0.98), lower educational level (OR 0.41, 95%CI 0.26-0.64) and being unemployed (OR 0.57, 95%CI 0.42-0.77) were negatively associated with reaching WHO recommendations.
Conclusions: A significant proportion of patients with CHD did not reach the WHO physical activity recommendations. There was a large variation in physical activity level by country of origin. Based on identified predictors, vulnerable patients may be identified and offered specific behavioral interventions.

PMID: 30511012 [PubMed]

Ultrasound-Guided axillary venous access for pediatric and adult congenital lead implantation.

Pacing Clin Electrophysiol. 2018 Dec 04;:

Authors: Clark BC, Janson CM, Nappo L, Pass RH

BACKGROUND: Axillary venous access with ultrasound guidance for pediatric transvenous lead implantation may reduce risks for pneumothorax and hemothorax. The objective was to retrospectively evaluate ultrasound-guided axillary vein access as an alternative to the subclavian approach.
METHODS: The technique consists of ultrasonographic identification of the axillary vein at the delto-pectoral groove after initial contrast venography. A micropuncture kit is used for initial ultrasound-guided percutaneous access with fluoroscopic confirmation of wire position. Pocket creation is performed and sheath insertion and lead implantation proceed as usual. Demographic, procedural and radiation exposure data were collected and analyzed.
RESULTS: 16 patients (median age 13 years, range 8-50; median weight 56 kg, range 29-77) underwent lead implantation; 2 additional patients required fluoroscopy due to poor acoustic windows (89% success). 15/21 leads (71%) were ventricular; 50% of implants were pacemakers and 31% were dual-chamber. Median time to venous access was 13 minutes (inter-quartile range (IQR) 9.25 - 20.25) and median implant procedure time was 156 minutes (IQR 112 - 172). Median fluoroscopy time was 18.0 minutes (IQR 11.9 - 29.6), median air kerma 9.0 mGy (IQR 3.0 - 28.5) and median dose-area product 30.2 Gy-cm2 (IQR 16.1 - 234.5). One patient required generator pocket revision 2 days post-procedure without lead dislodgement. There were no other complications encountered.
CONCLUSIONS: Transvenous pacemaker and ICD lead implantation in the pediatric and adult congenital population through ultrasound-guided axillary venous access is safe and efficacious. This technique may provide a low-risk alternative for vascular access for pediatric implantation procedures. This article is protected by copyright. All rights reserved.

PMID: 30515865 [PubMed - as supplied by publisher]

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Risk stratification in congenital heart disease - A call for protocolised assessment and multicentre collaboration.

Int J Cardiol. 2018 Nov 20;:

Authors: Kempny A, Fraisse A, Dimopoulos K

PMID: 30503190 [PubMed - as supplied by publisher]

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Repeat Pulmonary Valve Replacement: Similar Intermediate-Term Outcomes With Surgical and Transcatheter Procedures.

JACC Cardiovasc Interv. 2018 Nov 23;:

Authors: Caughron H, Kim D, Kamioka N, Lerakis S, Yousef A, Maini A, Reginauld S, Sahu A, Shashidharan S, Jokhadar M, Rodriguez FH, Book WM, McConnell M, Block PC, Babaliaros V

OBJECTIVES: This study compares 30-day, 1-year, and 3-year echocardiographic findings and clinical outcomes of transcatheter pulmonary valve-in-valve replacement (TPVR) and repeat surgical pulmonary valve replacement (SPVR).
BACKGROUND: In patients with adult congenital heart disease and previous pulmonary valve replacement (PVR) who require redo PVR, it is unclear whether TPVR or repeat SPVR is the preferred strategy.
METHODS: We retrospectively identified 66 patients (TPVR, n = 36; SPVR, n = 30) with bioprosthetic pulmonary valves (PVs) who underwent either TPVR or repeat SPVR at Emory Healthcare from January 2007 to August 2017.
RESULTS: The TPVR cohort had fewer men and more patients with baseline New York Heart Association (NYHA) functional class III or IV. There was no difference in mortality, cardiovascular readmission, or post-procedural PV reintervention at 30 days, 1 year, or 3 years. Post-procedural echocardiographic findings showed no difference in mean PV gradients between the TPVR and SPVR groups at 30 days, 1 year, or 3 years. In the TPVR cohort, there was less right ventricular dysfunction at 30 days (2.9% vs. 46.7%; p < 0.01), despite higher baseline NYHA functional class in the SPVR cohort.
CONCLUSIONS: In patients with bioprosthetic PV dysfunction who underwent either TPVR or SPVR, there was no difference in mortality, cardiovascular readmission, or repeat PV intervention at 30 days, 1 year, or 3 years. Additionally, TPVR and SPVR had similar intermediate-term PV longevity, with no difference in PV gradients or PVR. The TPVR cohort also had less right ventricular dysfunction at 30 days despite a higher baseline NYHA functional classification. These intermediate-term results suggest that TPVR may be an attractive alternative to SPVR in patients with previous bioprosthetic surgical PVs.

PMID: 30503596 [PubMed - as supplied by publisher]

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Optimization of heart allocation: The transplant risk score.

Am J Transplant. 2018 Dec 02;:

Authors: Jasseron C, Legeai C, Jacquelinet C, Nubret-Le Coniat K, Flécher E, Cantrelle C, Audry B, Bastien O, Dorent R

The new French heart allocation system is designed to minimize waitlist mortality and extend the donor pool without a detrimental effect on post-transplant survival. This study was designed to construct a 1-year post-transplant graft-loss risk score incorporating recipient and donor characteristics. The study included all adult first single-organ recipients transplanted between 2010 and 2014 (N=1776). This population was randomly divided in a 2:1 ratio into derivation and validation cohorts. The association of variables with 1-year graft-loss was determined with a mixed Cox model with center as random effect. The predictors were used to generate a transplant-risk score (TRS). Donor-recipient matching was assessed using two separate recipient and donor-risk scores. Factors associated with 1-year graft-loss were recipient age >50, valvular cardiomyopathy and congenital heart disease, previous cardiac surgery, diabetes, mechanical ventilation, glomerular filtration rate and bilirubin, donor age >55, and donor female gender. The C-index of the final model was 0.70. Correlation between observed and predicted graft-loss rate was excellent for the overall cohort (r=0.90). Hearts from high-risk donors transplanted to low-risk recipients had similar survival as those from low-risk donors. The TRS provides an accurate prediction of 1-year graft-loss risk and allows optimal donor-recipient matching. This article is protected by copyright. All rights reserved.

PMID: 30506840 [PubMed - as supplied by publisher]

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Adult congenital heart disease at the Royal Brompton: A historical perspective and future directions discussed by Michael Gatzoulis.

Eur Heart J. 2018 Dec 01;39(45):3990-3992

Authors: Gatzoulis MA

PMID: 30508102 [PubMed - in process]

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The incidentally diagnosed adult congenital heart disease during routine medical health checkups in 27,897 Koreans at a single center over seven years.

BMC Cardiovasc Disord. 2018 Dec 05;18(1):223

Authors: Kwag EM, Lee JS, Kim SH

BACKGROUND: The rate of incidentally diagnosed congenital heart disease (CHD) in adulthood has not been reported. The aim of this study was to investigate the detection rate of CHD in adults by routine, general health checkups.
METHODS: Data was acquired from 222,401 patients older than 19 years who participated in general health checkups from January 2010 to December 2016. We excluded persons who did not undergo echocardiography during the general health checkups, who underwent echocardiography prior to the health checkups, and who were previously diagnosed with CHD.
RESULTS: Among the 27,897 patients, who were included in the final analysis, 293 cases were newly diagnosed as CHD, and the overall detection rate was 1.05%. The mean age of patients with CHD was 48.7 ± 21.5 years, and most of them were female (n = 187, 63.8%). More than two-thirds were between the third and fifth decade of life, and only six patients (2.04%) were older than 70 years. The most common type was bicuspid aortic valve (n = 155). Interestingly, Ebstein's anomaly that required surgical repair was detected in five persons.
CONCLUSIONS: During general health checkup, there were cases of severe CHD that required cardiac surgery upon diagnosis.

PMID: 30518327 [PubMed - in process]

A minimally-invasive hybrid approach for cardiac resynchronization of the systemic right ventricle.

Pacing Clin Electrophysiol. 2018 Dec 06;:

Authors: Moore JP, Gallotti RG, Shannon KM, Biniwale R

BACKGROUND: Patients with systemic right ventricles (RV) often develop progressive heart failure and may benefit from cardiac resynchronization therapy (CRT), however the optimal strategy for CRT has not been defined.
METHODS: A retrospective review of all patients with systemic RV failure undergoing a hybrid transcatheter-surgical approach was performed. Procedural technique and outcomes are reported.
RESULTS: Six patients underwent detailed EAM of the systemic RV followed by a new hybrid approach targeting latest endocardial activation followed by focused epicardial mapping. The exact site of latest endocardial activation was variable but localized to the basolateral RV in all cases. Sites of latest activation tended to be more superior during contralateral ventricular pacing versus intact AV conduction (p = 0.06). Latest endocardial activation at the targeted site occurred at 157 ms (IQR 120-181 ms) and corresponding epicardial activation 174 ms (IQR 140-198 ms), after the onset of the QRS complex. Following hybrid CRT, the QRS duration decreased from a median of 193 to 147 ms and the FAC increased from a median of 15.5 to 30% (p < 0.001). Patients were discharged to home after a median of 4 days. Of 3 patients initially referred for transplant evaluation, 2 (66%) no longer met criteria following CRT.
CONCLUSIONS: Whereas latest endocardial activation for the systemic RV appears to localize to the basolateral region, the optimal lead position may be variable. An approach utilizing endocardial mapping followed by a limited surgical incision and confirmation of latest activation may result in minimally invasive surgery and a favorable acute CRT response. This article is protected by copyright. All rights reserved.

PMID: 30520520 [PubMed - as supplied by publisher]

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The management of labour in women with cardiac disease: need for more evidence?

BJOG. 2017 Aug;124(9):1307-1309

Authors: Cauldwell M, Cox M, Gatzoulis M, Nelson-Piercy C, O'Brien P, Roos-Hesselink JW, Thorne S, Walker F, Johnson MR

PMID: 28218452 [PubMed - indexed for MEDLINE]

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Genomic study of severe fetal anomalies and discovery of GREB1L mutations in renal agenesis.

Genet Med. 2018 07;20(7):745-753

Authors: Boissel S, Fallet-Bianco C, Chitayat D, Kremer V, Nassif C, Rypens F, Delrue MA, Dal Soglio D, Oligny LL, Patey N, Flori E, Cloutier M, Dyment D, Campeau P, Karalis A, Nizard S, Fraser WD, Audibert F, Lemyre E, Rouleau GA, Hamdan FF, Kibar Z, Michaud JL

PURPOSE: Fetal anomalies represent a poorly studied group of developmental disorders. Our objective was to assess the impact of whole-exome sequencing (WES) on the investigation of these anomalies.
METHODS: We performed WES in 101 fetuses or stillborns who presented prenatally with severe anomalies, including renal a/dysgenesis, VACTERL association (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities), brain anomalies, suspected ciliopathies, multiple major malformations, and akinesia.
RESULTS: A molecular diagnosis was obtained in 19 cases (19%). In 13 of these cases, the diagnosis was not initially suspected by the clinicians because the phenotype was nonspecific or atypical, corresponding in some cases to the severe end of the spectrum of a known disease (e.g., MNX1-, RYR1-, or TUBB-related disorders). In addition, we identified likely pathogenic variants in genes (DSTYK, ACTB, and HIVEP2) previously associated with phenotypes that were substantially different from those found in our cases. Finally, we identified variants in novel candidate genes that were associated with perinatal lethality, including de novo mutations in GREB1L in two cases with bilateral renal agenesis, which represents a significant enrichment of such mutations in our cohort.
CONCLUSION: Our study opens a window on the distinctive genetic landscape associated with fetal anomalies and highlights the power-but also the challenges-of WES in prenatal diagnosis.

PMID: 29261186 [PubMed - indexed for MEDLINE]