Department of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.
Department of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass.
J Thorac Cardiovasc Surg. 2023 Sep;166(3):933-942.e3. doi: 10.1016/j.jtcvs.2023.01.018. Epub 2023 Jan 23.
A subset of patients with borderline hypoplastic left heart may be candidates for single to biventricular conversion, but long-term morbidity and mortality persist. Prior studies have shown conflicting results regarding the association of preoperative diastolic dysfunction and outcome, and patient selection remains challenging.
Patients with borderline hypoplastic left heart undergoing biventricular conversion from 2005 to 2017 were included. Cox regression identified preoperative factors associated with a composite outcome of time to mortality, heart transplant, takedown to single ventricle circulation, or hemodynamic failure (defined as left ventricular end-diastolic pressure >20 mm Hg, mean pulmonary artery pressure >35 mm Hg, or pulmonary vascular resistance >6 international Woods units).
Among 43 patients, 20 (46%) met the outcome, with a median time to outcome of 5.2 years. On univariate analysis, endocardial fibroelastosis, lower left ventricular end-diastolic volume/body surface area (when <50 mL/m), lower left ventricular stroke volume/body surface area (when <32 mL/m), and lower left:right ventricular stroke volume ratio (when <0.7) were associated with outcome; higher preoperative left ventricular end-diastolic pressure was not. Multivariable analysis demonstrated that endocardial fibroelastosis (hazard ratio, 5.1, 95% confidence interval, 1.5-22.7, P = .033) and left ventricular stroke volume/body surface area 28 mL/m or less (hazard ratio, 4.3, 95% confidence interval, 1.5-12.3, P = .006) were independently associated with a higher hazard of the outcome. Approximately all patients (86%) with endocardial fibroelastosis and left ventricular stroke volume/body surface area 28 mL/m or less met the outcome compared with 10% of those without endocardial fibroelastosis and with higher stroke volume/body surface area.
History of endocardial fibroelastosis and smaller left ventricular stroke volume/body surface area are independent factors associated with adverse outcomes among patients with borderline hypoplastic left heart undergoing biventricular conversion. Normal preoperative left ventricular end-diastolic pressure is insufficient to reassure against diastolic dysfunction after biventricular conversion.
有一部分左心发育不良临界患者可能适合进行单心室到双心室转换，但长期的发病率和死亡率仍持续存在。先前的研究表明，术前舒张功能障碍与结果之间的关联存在矛盾，患者的选择仍然具有挑战性。
纳入了 2005 年至 2017 年期间接受双心室转换的左心发育不良临界患者。Cox 回归分析确定了与死亡率、心脏移植、转换为单心室循环或血流动力学衰竭（定义为左心室舒张末期压>20mmHg、平均肺动脉压>35mmHg 或肺血管阻力>6 国际伍兹单位）的复合结局相关的术前因素。
在 43 例患者中，有 20 例（46%）达到了研究终点，中位时间为 5.2 年。在单因素分析中，心内膜弹力纤维增生症、较低的左心室舒张末期容积/体表面积（<50mL/m）、较低的左心室每搏输出量/体表面积（<32mL/m）和较低的左心室/右心室每搏输出量比值（<0.7）与结局相关；较高的术前左心室舒张末期压则无相关性。多变量分析表明，心内膜弹力纤维增生症（风险比，5.1，95%置信区间，1.5-22.7，P=0.033）和左心室每搏输出量/体表面积 28mL/m 或更小（风险比，4.3，95%置信区间，1.5-12.3，P=0.006）与结局的风险更高独立相关。与没有心内膜弹力纤维增生症和更高的每搏输出量/体表面积的患者相比，大约所有有心内膜弹力纤维增生症和左心室每搏输出量/体表面积 28mL/m 或更小的患者（86%）都达到了研究终点。
左心发育不良临界患者行双心室转换后，心内膜弹力纤维增生症病史和较小的左心室每搏输出量/体表面积是与不良结局相关的独立因素。术前正常的左心室舒张末期压不足以保证双心室转换后舒张功能正常。
