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器官变异综合征患者的双心室修补

Biventricular repair in patients with heterotaxy syndrome

Lim HG, Bacha EA, Marx GR 2009/5/31 18:19:25

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J Thorac Cardiovasc Surg, 2009,

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Objective:
Complex intracardiac and extracardiac anatomy is often confronted during biventricular repair in patients with heterotaxy syndrome. We examined factors affecting surgical outcomes in these patients.

Methods:
Between January 1990 and July 2007, 371 patients received a diagnosis of heterotaxy syndrome; 91 (91/371, 24.5%) underwent biventricular repair. Left atrial isomerism was present in 73% (66/91) and right atrial isomerism in 10% (9/91), with indeterminate atrial anatomy in 17% (16/91). Median age at biventricular repair was 6.8 months (5 days to 22.3 years). Systemic venous anomalies were present in 75 patients, pulmonary venous anomalies in 26, and endocardial cushion defects in 36. Transposition complexes were present in 15 patients with atrioventricular discordance in 10; 8 underwent double switch, 2 received a physiologic repair, 2 underwent arterial switch, and 3 underwent the Rastelli operation. Other conotruncal anomalies included double-outlet right ventricle in 10 patients, tetralogy of Fallot in 3, and hemitruncus in 2. Separation of systemic from pulmonary venous return included intra-atrial baffling in 48 patients and extracardiac grafting in 2. Combined lesions were common, occurring in 99% (90/91). Statistical analysis with Kaplan–Meier and Cox proportional hazards models were performed.

Results:
Average follow-up was 44.9±57.5 months (3 days to 189.3 months). Kaplan–Meier estimated survival was 93.4% at 10 years; unbalanced complete atrioventricular canal was the only risk factor for mortality (P=.006). Subsequent procedures were common with a 10-year freedom from reoperation or reintervention of 38%±7.5%. Arrhythmias occurred in 36 (39.6%) patients; bradyarrhythmia in 27 (29.7%) and tachyarrhythmia in 15 (16.5%). Freedom from any arrhythmia was 53.9%±6.7% at 10 years.

Conclusions:
Excellent survival for patients with heterotaxy undergoing biventricular repair can be expected, even for multiple, complex lesions. Reintervention is common, and arrhythmia is a long-term concern. This experience shows that patients with heterotaxy syndrome and complex cardiac anatomy can be considered for biventricular repair. Patients with unbalanced complete atrioventricular canal are a high-risk group for which selection criteria are particularly important.

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疾病资源中心

高血压	慢性阻塞性肺病
脂肪性肝病	糖尿病
卒中	肺癌
冠心病	子宫内膜异位症
结直肠癌	乳腺癌
心力衰竭	心律失常

病例分析 [栏目介绍]

甲状腺功能亢进合并黄疸

王燕燕王曙

上海交通大学附属瑞金医院内分泌科

患者，女，69岁。2009年1月无明显诱因下出现乏力，当时程度较轻，未予以重视。2009年3月患者乏力症状加重，尿色逐渐加深，大便习惯改变，颜色变淡。4月18日入我院感染科治疗，诉轻度头晕、心慌，体重减轻10kg。无肝区疼痛，无发热，无腹痛、腹泻、腹胀、里急后重，无恶性、呕吐等。入院半月前于外院就诊，查肝功能：ALT 601IU/L，AST 785IU/L，TBIL 97.7umol/L，白蛋白 41g/L，甲状腺功能：游离T3 30.6pmol/L，游离T4 51.9pmol/L，心电图示快速房颤。

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