Long QT syndrome: an indication for cervicothoracic sympathectomy

Beta-blockade represents the primary treatment modality in patients with long QT syndrome, but left cervicothoracic sympathectomy (LCS) has been employed in refractory cases and in cases with malignant arrhythmias. LCS was performed in ten patients (six male, four female) with long QT syndrome, ranging in age from 1 month to 40 years (median 15 years). Familial long QT syndrome was present in seven patients (70%). The mean(s.e.m.) (range) preoperative corrected QT interval (QTc) was 0.52(0.01) (0.46-0.60)s. The mean(s.e.m.) duration of symptoms was 4.4(1.1) years with a mean(s.e.m.) of 4.1(0.9) syncopal episodes and 1.2(0.2) cardiac arrests per patient. LCS was carried out for refractory symptoms on beta-blockers in nine cases; a single patient was unable to tolerate beta-blockers and LCS represented the primary treatment modality. A left supraclavicular approach was utilized in each patient, resecting a portion of the stellate and all of the T2 and T3 ganglia. The median(range) length of hospitalization following operation was 2(2-6) days. There were no unexpected complications of operation, although nine (90%) of the patients developed Horner's syndrome. The QTc decreased a mean(s.e.m.) of 0.03(0.01)s following operation (P < 0.01). The frequency of symptomatic episodes decreased from a mean(s.e.m.) of 7.1(3.1)/year before LCS to 0.1(0.1) after operation (P < 0.001). Patients have been followed for a mean of 1.3(0.3) years, and all but one patient remains symptom-free; the youngest patient died suddenly 10 months after surgery. These results suggest that LCS is associated with significant clinical benefits in patients with long QT syndrome and the procedure should be considered when symptoms are refractory and malignant, or when contraindications to beta-blockers are present.