某些具有完整室间隔(PA/IVS)或左心室显性房室道缺损(LDAVC)并伴有右心室发育不良(RV)和单室(1V)循环的肺动脉闭锁患者可能是转换为完全双心室(2V)修复或一个半心室修复(1.5V)的候选人。我们试图确定与成功从1V转换为1.5V或2V循环相关的术前心血管磁共振(CMR)发现。
在这项单中心回顾性研究中,如果在CMR时存在1V循环,然后进行旨在将其转换为1.5V或2V循环的手术干预,则包括患有PA/IVS或LDAVC且无截肢异常的受试者.转换失败定义为以下任一项:(1)最近随访时氧饱和度<90%,(2)转换回1.5V或1V循环,(3)死亡。
在PA/IVS队列中(n=15,中位年龄1.32岁),10例患者接受了1.5V循环和5例2V循环的手术转换。在尝试的1.5V组中,有两次失败,这些病例的RV质量较低(p=0.04)。在尝试的2V组中,有1次失败,与成功的CMR参数相比,没有显着差异。在成功的2例V组患者中,最小RV舒张末期容积(EDV)为27ml/m2.在LDAVC队列中(n=15,中位年龄1.0岁),1例患者接受手术转换为1.5V循环,14例患者接受2V循环。在尝试的1.5V组中,1转化失败并且具有15ml/m2的RVEDV。在尝试的2V组中,有两次失败,与成功病例相比,这些病例的RV:LV每搏输出量比(p=0.05)较小,RV射血分数较低(p=0.05).在成功的2例V组患者中,最小RVEDV为22ml/m2。
我们确定了与PA/IVS和LDAVC患者从1V循环成功转换为1.5V或2V循环相关的多个CMR参数。这些信息可以改善患者对转换程序的选择,并鼓励更大的研究来更好地定义CMR的作用。
Some patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1.5V or 2V circulation.
In this single center retrospective study, subjects with PA/IVS or LDAVC and no conotruncal abnormalities were included if they had a 1 V circulation at the time of CMR followed by a surgical intervention intended to convert them to a 1.5 V or 2 V circulation. Conversion failure was defined as any of the following: (1) oxygen saturation < 90% at the most recent follow-up, (2) conversion back to a 1.5 V or 1 V circulation, or (3) death.
In the PA/IVS cohort (n = 15, median age 1.32 years), 10 patients underwent surgical conversion to a 1.5 V circulation and 5 to a 2 V circulation. In the attempted 1.5 V group, there were 2 failures, and these cases had a lower RV mass (p = 0.04). In the attempted 2 V group, there was 1 failure, and no CMR parameters were significantly different compared to the successes. Among the successful 2 V group patients, the minimum RV end-diastolic volume (EDV) was 27 ml/m2. In the LDAVC cohort (n = 15, median age 1.0 years), 1 patient underwent surgical conversion to a 1.5 V circulation and 14 patients to a 2 V circulation. In the attempted 1.5 V group, the 1 conversion was a failure and had an RV EDV of 15 ml/m2. In the attempted 2 V group, there were 2 failures, and these cases had a smaller RV:LV stroke volume ratio (p = 0.05) and a lower RV ejection fraction (p = 0.05) compared to the successes. Among the successful 2 V group patients, the minimum RV EDV was 22 ml/m2.
We identified multiple CMR parameters associated with successful conversion from 1 V circulation to 1.5 V or 2 V circulation in patients with PA/IVS and LDAVC. This information may improve patient selection for conversion procedures and encourage larger studies to better define the role of CMR.