Mizuno Masanori, Ohuchi Hideo, Kagisaki Koji, Miyazaki Aya, Ishibashi-Ueda Hatsue, Yamada Osamu
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan.
Pediatr Int. 2014 Aug;56(4):630-3. doi: 10.1111/ped.12374.
We treated four postoperative adults with congenital heart disease with severe restrictive hemodynamics (RH), and performed decortication (DC) with the anticipation of some relief of the RH. The catheterizations before DC showed high central venous, and right and left ventricular end-diastolic pressures with "dip-and-plateau" pressure waveforms in the right and left ventricles. Upon myocardial histopathologic examination, moderate myocardial fibrotic change was demonstrated in two of three cases. DC led to decrease in type B natriuretic peptide levels in all cases, resulting in a decline in the central venous, right and left ventricular end-diastolic pressures in three cases. Successful DC-related relief of RH, dilatation of the ventricles with decline in central and end-diastolic pressures, was observed in only one case. Our limited DC-related hemodynamic improvement indicates a complexity of the severe RH, which may represent a unique intractable heart failure pathophysiology in intractable postoperative adult congenital heart disease.
我们对4例患有严重限制性血流动力学(RH)的先天性心脏病术后成人患者进行了治疗,并进行了剥脱术(DC),预期能缓解部分RH。DC术前的导管检查显示中心静脉压以及左右心室舒张末期压力升高,左右心室出现“下陷-高原”压力波形。在心肌组织病理学检查中,3例中有2例表现为中度心肌纤维化改变。DC使所有病例的B型利钠肽水平降低,3例患者的中心静脉压以及左右心室舒张末期压力下降。仅1例患者通过DC成功缓解了RH,心室扩张,中心静脉压和舒张末期压力下降。我们有限的与DC相关的血流动力学改善表明严重RH的复杂性,这可能代表了难治性成人先天性心脏病术后独特的难治性心力衰竭病理生理学。