PMID- 27390959 OWN - NLM STAT- MEDLINE DCOM- 20170829 LR - 20220129 IS - 1874-1754 (Electronic) IS - 0167-5273 (Linking) VI - 220 DP - 2016 Oct 1 TI - Dyssynchrony and electromechanical delay are associated with focal fibrosis in the systemic right ventricle - Insights from echocardiography. PG - 382-8 LID - S0167-5273(16)31100-7 [pii] LID - 10.1016/j.ijcard.2016.06.090 [doi] AB - BACKGROUND: Systemic right ventricular (RV) dysfunction and sudden cardiac death remain problematic late after Mustard operation for transposition of the great arteries. The exact mechanism for that relationship is likely to be multifactorial including myocardial fibrosis. Doppler echocardiography gives further insights into the role of fibrosis shown by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in late morbidity. METHODS AND RESULTS: Twenty-two consecutive patients, mean age 28+/-8years, were studied with 2D echocardiography, and also assessed by LGE CMR. The presence of LGE in 13/22 patients (59%) was related to delayed septal shortening and lengthening (P=0.002 &P=0.049), prolonged systemic RV isovolumic contraction time (P=0.024) and reduced systemic RV free wall and septal excursion (P=0.027 &P=0.005). The systemic RV total isovolumic time was prolonged but not related to extent of LGE. LGE extent was related to markers of electromechanical delay and dyssynchrony (delayed onset of RV free wall shortening and lengthening; r=0.73 &P=0.004 and r=0.62 &P=0.041, respectively, and QRS duration r=0.68, P<0.01) and was inversely related to systolic RV free wall shortening velocity (r=-0.59 &P=0.042). The presence of LGE was also related to lower exercise capacity, >/=mild tricuspid regurgitation and more arrhythmia (P=0.008, P=0.014 and P=0.040). RV free wall excursion and systolic tissue Doppler velocity were related to CMR derived RV ejection fraction (r=0.51, P=0.015, and r=0.77, P=<0.001, respectively). CONCLUSION: Post Mustard repair, myocardial fibrosis is related to dyssynchrony, RV long axis dysfunction and tricuspid regurgitation. Echocardiographic measurements of systemic RV function can be confidently used in serial follow-up following Mustard operation. CI - Copyright (c) 2016 Elsevier Ireland Ltd. All rights reserved. FAU - Babu-Narayan, Sonya V AU - Babu-Narayan SV AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. FAU - Prati, Daniele AU - Prati D AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK; Department of Biomedical and Surgical Sciences, Section of Cardiology, Universita' degli Studi di Verona, Verona, Italy. FAU - Rydman, Riikka AU - Rydman R AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK; Department of Molecular Medicine and Surgery, Section of Clinical Physiology, Karolinska Institutet, Stockholm, Sweden. FAU - Dimopoulos, Konstantinos AU - Dimopoulos K AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. FAU - Diller, Gerhard-Paul AU - Diller GP AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. FAU - Uebing, Anselm AU - Uebing A AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. FAU - Henein, Michael Y AU - Henein MY AD - National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. FAU - Kilner, Philip J AU - Kilner PJ AD - NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. FAU - Gatzoulis, Michael A AU - Gatzoulis MA AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. FAU - Li, Wei AU - Li W AD - Adult Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, Dovehouse Street, London SW3 6LY, UK. Electronic address: w.li@rbht.uk. LA - eng GR - FS/11/38/28864/BHF_/British Heart Foundation/United Kingdom PT - Journal Article DEP - 20160623 PL - Netherlands TA - Int J Cardiol JT - International journal of cardiology JID - 8200291 SB - IM MH - Adult MH - Cohort Studies MH - *Echocardiography/methods MH - Electrocardiography/methods MH - Female MH - Fibrosis/diagnostic imaging/physiopathology MH - Follow-Up Studies MH - Humans MH - Longitudinal Studies MH - Male MH - Ventricular Dysfunction, Right/*diagnostic imaging/*physiopathology MH - Young Adult OTO - NOTNLM OT - Fibrosis OT - Late gadolinium enhancement OT - Long axis function OT - Systemic right ventricle OT - Tissue Doppler imaging OT - Transposition of the great arteries EDAT- 2016/07/09 06:00 MHDA- 2017/08/30 06:00 CRDT- 2016/07/09 06:00 PHST- 2016/05/24 00:00 [received] PHST- 2016/06/21 00:00 [accepted] PHST- 2016/07/09 06:00 [entrez] PHST- 2016/07/09 06:00 [pubmed] PHST- 2017/08/30 06:00 [medline] AID - S0167-5273(16)31100-7 [pii] AID - 10.1016/j.ijcard.2016.06.090 [doi] PST - ppublish SO - Int J Cardiol. 2016 Oct 1;220:382-8. doi: 10.1016/j.ijcard.2016.06.090. Epub 2016 Jun 23.