Brauner R A, Laks H, Drinkwater D C, Scholl F, McCaffery S
Division of Cardiothoracic Surgery, UCLA School of Medicine 90095, USA.
Ann Thorac Surg. 1997 Sep;64(3):721-9. doi: 10.1016/s0003-4975(97)00632-2.
The outcome of children with multilevel left heart obstructions (Shone's anomaly) is generally poor. Literature is scarce, consisting mainly of case reports. The mitral disease may be the predominant factor affecting outcome.
Surgical results in 19 consecutive patients are presented, with a median follow-up of 8 years. Mitral stenosis was present in all, with parachute deformity in 12 patients. Supramitral rings were found in 9 patients. Other features included subaortic stenosis (15 patients), valvar aortic stenosis (9), bicuspid aortic valve (16), and coarctation (13 patients). The patients underwent 46 surgical procedures, including 18 mitral operations (9 replacements, 9 repairs).
There were three in-hospital (16%) and two late (10.5%) deaths. Of the 5 nonsurvivors, 4 patients (80%) had predominant mitral disease and moderate to severe pulmonary hypertension, versus 4 (28.5%) and 5 (36%) survivors, respectively (p = not significant). Valve repair was the final procedure in 9 survivors. The other 5 patients had repeated valve replacements (1), aortoventriculoplasty with valve replacements (2), or no mitral operation (2). Freedom from mitral reoperation was 78% (7 of 9 patients) after repair procedures and 43% (3 of 7 patients) after replacement. At follow-up, 10 patients (71.4%) are in New York Heart Association functional class I and the other 4 in class II and III. Six (43%) await reoperation due to recurrent aortic (4) or subaortic (1) stenosis and recoarctation (2). Echocardiography reveals mild mitral stenosis or regurgitation in 3 patients after repair (33%). Four are considered free of residual disease (21% of all).
Late outcome in Shone's anomaly seems to correlate with the predominance of mitral valve involvement and the degree of pulmonary hypertension. Valve repair is indicated whenever feasible and should be considered before the occurrence of pulmonary hypertension.
患有多级左心梗阻（肖恩综合征）的儿童预后通常较差。相关文献稀少，主要为病例报告。二尖瓣疾病可能是影响预后的主要因素。
介绍了连续19例患者的手术结果，中位随访时间为8年。所有患者均存在二尖瓣狭窄，其中12例为降落伞样畸形。9例患者发现二尖瓣上环形狭窄。其他特征包括主动脉瓣下狭窄（15例）、主动脉瓣狭窄（9例）、二叶式主动脉瓣（16例）和主动脉缩窄（13例）。患者接受了46次外科手术，包括18次二尖瓣手术（9次置换，9次修复）。
有3例住院死亡（16%）和2例晚期死亡（10.5%）。在5例非幸存者中，4例患者（80%）主要患有二尖瓣疾病且伴有中度至重度肺动脉高压，而幸存者中分别为4例（28.5%）和5例（36%）（p值无统计学意义）。瓣膜修复是9例幸存者的最终手术方式。另外5例患者进行了重复瓣膜置换（1例）、主动脉心室成形术联合瓣膜置换（2例）或未进行二尖瓣手术（2例）。修复手术后二尖瓣再次手术的免再手术率为78%（9例患者中的7例），置换后为43%（7例患者中的3例）。随访时，10例患者（71.4%）纽约心脏协会心功能分级为I级，另外4例为II级和III级。6例（4�%）因复发性主动脉（4例）或主动脉瓣下（1例）狭窄及主动脉缩窄复发（2例）等待再次手术。超声心动图显示3例修复后的患者存在轻度二尖瓣狭窄或反流（33%）。4例被认为无残余疾病（占所有患者的21%）。
肖恩综合征的晚期预后似乎与二尖瓣受累的程度和肺动脉高压的程度相关。只要可行，应进行瓣膜修复，且应在肺动脉高压发生之前考虑。
