Babu-Narayan Sonya V, Prati Daniele, Rydman Riikka, Dimopoulos Konstantinos, Diller Gerhard-Paul, Uebing Anselm, Henein Michael Y, Kilner Philip J, Gatzoulis Michael A, Li Wei
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.
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.
Int J Cardiol. 2016 Oct 1;220:382-8. doi: 10.1016/j.ijcard.2016.06.090. Epub 2016 Jun 23.
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.
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).
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.
在大动脉转位的Mustard手术后晚期，系统性右心室（RV）功能障碍和心源性猝死仍然是棘手的问题。这种关系的确切机制可能是多因素的，包括心肌纤维化。多普勒超声心动图进一步揭示了延迟钆增强（LGE）心血管磁共振（CMR）显示的纤维化在晚期发病中的作用。
对22例连续患者（平均年龄28±8岁）进行二维超声心动图检查，并通过LGE CMR进行评估。13/22例患者（59%）存在LGE，这与室间隔缩短和延长延迟（P=0.002和P=0.049）、系统性右心室等容收缩时间延长（P=0.024）以及系统性右心室游离壁和室间隔运动幅度降低（P=0.027和P=0.005）有关。系统性右心室总等容时间延长，但与LGE范围无关。LGE范围与电机械延迟和不同步的标志物有关（右心室游离壁缩短和延长延迟开始；r分别为0.73和P=0.004，以及r=0.62和P=0.041），并且与QRS波时限r=0.68，P<0.01），与右心室游离壁收缩期缩短速度呈负相关（r=-0.59和P=0.042）。LGE的存在还与运动能力降低、≥轻度三尖瓣反流和更多心律失常有关（P=0.008、P=0.014和P=0.040）。右心室游离壁运动幅度和收缩期组织多普勒速度与CMR得出的右心室射血分数有关（r分别为0.51，P=0.015，和r=0.77，P<0.001）。
Mustard修复术后，心肌纤维化与不同步、右心室长轴功能障碍和三尖瓣反流有关。系统性右心室功能的超声心动图测量可放心用于Mustard手术后的系列随访。
