Hauser Michael, Meierhofer Christian, Schwaiger Markus, Vogt Manfred, Kaemmerer Harald, Kuehn Andreas
Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center, Franz-Schrank Strasse 2 80638, Germany.
Circ J. 2015;79(2):425-31. doi: 10.1253/circj.CJ-14-0716. Epub 2014 Dec 19.
Dysfunction of the morphologic systemic right ventricle (RV) is a sequela in long-term survivors with transposition of the great arteries (TGA) after atrial switch operation (AtSO). Impairment of myocardial blood flow (MBF) and coronary flow reserve (CFR) are hypothesized as predisposing factors.
The study group comprised 20 patients after AtSO (22.7 ± 5.03 years) and 15 individuals with congenitally corrected transposition (ccTGA) (30.6 ± 19.4 years). MBF was quantified by positron emission tomography; controls for coronary flow were 11 healthy volunteers (26.2 ± 5.1 years). Exercise capacity, ventricular mass, function and end-diastolic volume assessed by coronary magnetic resonance (CMR), hemodynamic parameters assessed by cardiac catheterization and echocardiography, and B-type natriuretic peptide levels correlated with MBF. At rest, MBF did not differ between patients and healthy volunteers (MBFrestml·100 g(-1)·min(-1); ccTGA: 75 ± 14 vs. AtSO: 73 ± 16 vs. controls: 77 ± 15; NS). After vasodilatation, MBF increased significantly, but was significantly lower in ccTGA and AtSO groups compared with controls (MBFstressml·100 g(-1)·min(-1); ccTGA: 198 ± 38 vs. AtSO: 167 ± 46 vs. controls 310 ± 74; P<0.001). In ccTGA, CFR correlated significantly with clinical, CMR, echocardiographic and hemodynamic parameters, but for AtSO patients no significant correlation could be calculated.
In patients with ccTGA, maximal coronary blood flow is attenuated and significantly correlated with ventricular function, whereas dysfunction of the morphologic systemic RV after AtSO is a multifactorial problem.
形态学上的体循环右心室(RV)功能障碍是大动脉转位(TGA)患者在心房调转手术(AtSO)后长期存活者的后遗症。心肌血流(MBF)和冠状动脉血流储备(CFR)受损被认为是易感因素。
研究组包括20例AtSO术后患者(22.7±5.03岁)和15例先天性矫正型大动脉转位(ccTGA)患者(30.6±19.4岁)。通过正电子发射断层扫描对MBF进行定量;冠状动脉血流的对照为11名健康志愿者(26.2±5.1岁)。通过冠状动脉磁共振(CMR)评估运动能力、心室质量、功能和舒张末期容积,通过心导管检查和超声心动图评估血流动力学参数,B型利钠肽水平与MBF相关。静息时,患者与健康志愿者的MBF无差异(静息MBF,ml·100 g⁻¹·min⁻¹;ccTGA:75±14,AtSO:73±16,对照组:77±15;无显著性差异)。血管扩张后,MBF显著增加,但ccTGA组和AtSO组与对照组相比显著降低(应激MBF,ml·100 g⁻¹·min⁻¹;ccTGA:198±38,AtSO:167±46,对照组:310±74;P<0.001)。在ccTGA中,CFR与临床、CMR、超声心动图和血流动力学参数显著相关,但对于AtSO患者,无法计算出显著相关性。
在ccTGA患者中,最大冠状动脉血流减弱且与心室功能显著相关,而AtSO术后形态学上的体循环RV功能障碍是一个多因素问题。