Abstract

To optimize the surgical strategy and individualized treatment for scimitar syndrome (SS) by summarizing the clinical outcomes of the pericardial tunnel technique for the vertical form of SS at a single centre. The vertical form was defined as an angle of scimitar vein (SV) insertion to the inferior vena cava (θ) ≤ 45°, whereas the horizontal form was defined as θ > 45°. Nine patients with vertical form were operated on from June 2011 to June 2017. The mean age of patients during surgery was 3.1 ± 3.63 (range 0.3-12) years. Five patients were with infantile form and 4 with childhood/adult form per Dupuis' classification. The pericardial tunnel technique entailed attaching the SV to the right lateral pericardium and directing blood flow to the left atrium through a left atriotomy inside the pericardial tunnel. All patients had associated intracardiac anomalies repaired concomitantly; 3 patients had major collateral arteries occluded or ligated. There was no in-hospital postoperative death or residual SV obstruction. The postoperative ventilation time was 88.3 ± 63.8 (range 36-264) h. Eight survivors were followed up for 33.4 ± 19.2 (range 2-72) months, with 1 lost to follow-up. Echocardiography demonstrated that 7 survivors had no pulmonary hypertension and 1 had mild pulmonary hypertension; none developed SV obstruction during the follow-ups. The pericardial tunnel technique is suitable for the vertical form of SS and is a reasonable option for individualized treatment of SS.

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