Hughes Marina L, Tsang Victor T, Kostolny Martin, Giardini Alessandro, Muthurangu Vivek, Taylor Andrew M, Brown Katherine
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, NHS Trust, London, United Kingdom.
Cardiol Young. 2011 Dec;21(6):646-53. doi: 10.1017/S104795111100059X. Epub 2011 May 31.
This study describes single-unit experience and explores risk stratification, with protocolised inter-stage cardiac magnetic resonance imaging for infants with hypoplastic left heart syndrome. Survival was retrospectively analysed among the cohort of locally followed survivors of Norwood Stage I procedure, who underwent magnetic resonance imaging under general anaesthesia from 2003 to 2008. This included 32 patients: 17 with Sano conduit and 15 with arterio-pulmonary shunt. The median (inter-quartile range) age and weight at scan were 3.1 (2.6-4.6) months and 5.0 (4.5-5.3) kilograms, respectively. Using morphologic definitions, the median coarctation index was 0.71 (0.57-0.83). The degree of proximal right and left pulmonary artery narrowing was 25% (14-44%) and 25% (11-50%), respectively. The median right ventricular ejection fraction was 54% (48-59%). The ejection fraction was not related to the coarctation index or to pulmonary artery narrowing. Patients were followed up for a median of 19.2 (10.8-46.0) months, during which 13 (41%) had an intervention in addition to routine Norwood Stage II surgery and seven died. Risk of death was related to reduced right ventricular ejection fraction, with a hazard ratio of 0.91 (95% confidence interval 0.85-0.98, p = 0.02), and the cumulative number of focal stenoses of neo-aortic arch and pulmonary arteries (hazard ratio 2.71, 95% confidence interval 1.14-6.44, p = 0.02).
In addition to comprehensive three-dimensional morphologic imaging, inter-stage cardiac magnetic resonance imaging provides a ventricular functional index that may predict outcome in patients with hypoplastic left heart syndrome. Measures to preserve right ventricular systolic function and relieve stenoses are paramount within the complex management strategies for these patients.
本研究描述了单病例经验，并探讨了风险分层，采用标准化的阶段性心脏磁共振成像对左心发育不全综合征婴儿进行评估。对在当地接受随访的诺伍德一期手术存活者队列进行回顾性生存分析，这些患者于2003年至2008年在全身麻醉下接受了磁共振成像检查。这包括32例患者：17例采用桑诺导管，15例采用动肺分流术。扫描时的中位（四分位间距）年龄和体重分别为3.1（2.6 - 4.6）个月和5.0（4.5 - 5.3）千克。根据形态学定义，中位缩窄指数为0.71（0.57 - 0.83）。右肺动脉和左肺动脉近端狭窄程度分别为25%（14 - 44%）和25%（11 - 50%）。右心室射血分数中位值为54%（48 - 59%）。射血分数与缩窄指数或肺动脉狭窄无关。患者随访中位时间为19.2（10.8 - 46.0）个月，在此期间，13例（41%）除了接受常规诺伍德二期手术外还接受了干预，7例死亡。死亡风险与右心室射血分数降低有关，风险比为0.91（95%置信区间0.85 - 0.98，p = 0.02），以及新主动脉弓和肺动脉局灶性狭窄的累积数量有关（风险比2.71，95%置信区间1.14 - 6.44，p = 0.02）。
除了全面的三维形态学成像外，阶段性心脏磁共振成像还提供了一个心室功能指标，可预测左心发育不全综合征患者的预后。在这些患者的复杂管理策略中，保留右心室收缩功能和缓解狭窄的措施至关重要。
