Right ventricular workload assessed by FDG-PET in a patient with residual VSD and infundibular pulmonary stenosis after repair of tetralogy of Fallot
Right ventricular workload assessed by FDG-PET in a patient with residual VSD and infundibular pulmonary stenosis after repair of tetralogy of Fallot
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FDG-PET评估法洛四联症修复术后残余室间隔缺损伴漏斗部肺动脉狭窄患者的右心室负荷
DOI：
10.1007/s12350-017-0906-z
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发表时间：
2018
期刊：
影响因子：
2.4
通讯作者：
Fukumoto Y
中科院分区：
文献类型：
--
作者：
Nakamura T;Tahara N;Tahara A;Honda A;Igata S;Bekki M;Sugiyama Y;Sun J;Kumagai E;Kurata S;Fujimoto K;Abe T;Kato S;Tanaka H;Fukumoto Y
一名58岁男性因心悸和昏厥被转诊至我院，他在8岁时接受了Blalock-Taussig分流术，并在11岁时接受了膜周室间隔缺损（VSD）修补术和右心室（RV）流出道（RVOT）重建术治疗法洛四联症（TOF）。24小时霍尔特监测显示频繁的非持续性室性心动过速。超声心动图显示残余VSD、肺动脉狭窄（PS）和三尖瓣返流，压力梯度为79 mmHg（图1 A、B）。计算机断层血管造影（CTA）发现RVOT阻塞仍然存在（图1C;箭头）。心导管检查证实肺动脉至RV的压力梯度为56 mmHg，计算的分流分数（Qp/Qs）为2.01。18F-氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）结合CTA显示RV游离壁和RVOT中的FDG摄取强烈，表明RV工作负荷增加（图1D;箭头）。因此，我们决定手术重建右室流出道梗阻并关闭残余室间隔缺损。单尖瓣肺动脉瓣未接受治疗，以避免肺动脉返流加重（图1 E）。手术矫正后，Qp/Qs比值和肺动脉与RV压力梯度分别降至1.43和23 mmHg。连续FDG-PET/CTA扫描显示，手术治疗后与血流动力学改善相关的RV工作负荷显著缓解（图1F;箭头）。切除的RVOT的组织学分析显示，心肌细胞存在重度心肌肥大、核变性、肌原纤维稀疏、纤维化和紊乱，以及肌浆空泡化，间质组织存在胶原纤维增生（图1 G、H）。葡萄糖转运蛋白（GLUT）-4，而不是GLUT-1和-3，在切除的RVOT中得到了药物化学证实（图1 I-K）。在心肌细胞的细胞质中观察到过碘酸-希夫（PAS）染色的增加，其被过碘酸酶消化（图1 L，M）。因此，我们认为右室游离壁和右室流出道的FDG摄取反映了本例中心肌葡萄糖代谢的升高。TOF修复的自然史与RV衰竭和再次手术的相关发生率相关。1压力超负荷导致代谢从葡萄糖氧化转变为糖酵解，最终导致RV衰竭，
A 58-year-old male was referred to our hospital due to palpitation and faintness, who had undergone placement of a Blalock-Taussig shunt at the age of 8 and patch closure for a perimembranous ventricular septal defect (VSD) and reconstruction of the right ventricular (RV) outflow tract (RVOT) at the age of 11 for tetralogy of Fallot (TOF). A 24-hour Holter monitor showed frequent runs of non-sustained ventricular tachycardia. Echocardiography revealed residual VSD, pulmonary stenosis (PS), and tricuspid regurgitation with pressure gradient of 79 mmHg (Figure 1 A, B). Computed tomographic angiography (CTA) found that RVOT obstruction remained (Figure 1 C; arrowhead). Cardiac catheterization confirmed pressure gradient of 56 mmHg from pulmonary artery to RV and calculated shunt fraction (Qp/Qs) of 2.01. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) combined with CTA demonstrated intense FDG uptake in the RV free wall and RVOT indicating the increased RV workload (Figure 1 D; arrows). Therefore, we decided to surgically reconstruct the RVOT obstruction and close the residual VSD. A monocusp pulmonary valve was untreated to avoid the incidence of pulmonary regurgitation aggravation (Figure 1 E). After the surgical correction, the Qp/Qs ratio and pulmonary artery to RV pressure gradient reduced to 1.43 and 23 mmHg, respectively. Serial FDG-PET/CTA scan demonstrated a marked resolution of RV workload associating with hemodynamic improvement after the surgical treatment (Figure 1 F; arrows). Histopathological analyses of the excised RVOT revealed the cardiomyocytes with severe myocardial hypertrophy, nuclear degeneration, myofibrillar rarefaction, fibrillization and disarray, as well as sarcoplasmic vacuolization, and the interstitial tissue with collagen fiber hyperplasia (Figure 1 G, H). Glucose transporter (GLUT)-4, but not GLUT-1 and-3, was immunohistochemically proven in the excised RVOT (Figure 1 I–K). An increase in periodic acid-Schiff (PAS) staining was observed in the cytoplasm of cardiomyocytes, which were digested by diastase (Figure 1 L, M). Therefore, we consider that FDG uptake in the RV free wall and RVOT reflected the elevated myocardial glucose metabolism in this case. The natural history of repair of TOF is associated with a concerning incidence of RV failure and reoperation. 1 Pressure overload leads to metabolic shift from glucose oxidation to glycolysis and finally to RV failure,