Department of Thoracic and Cardiovascular Surgery, Hirosaki University School of Medicine, 5 Zaifucho, Hirosaki, Aomori, 036-8562, Japan.
Department of Cardiovascular Surgery, Ibaraki Clinical Education and Training Center, University of Tsukuba Hospital, Tsukuba, Ibaraki, 305-8576, Japan.
J Cardiothorac Surg. 2020 Sep 29;15(1):279. doi: 10.1186/s13019-020-01335-4.
We evaluated additional pulmonary blood flow at the time of bidirectional cavopulmonary shunt and its effects on the Fontan procedure and long-term outcome of Fontan circulation and liver function.
We included 22 patients (16 boys, 6 girls) having undergone bidirectional cavopulmonary shunt with additional pulmonary blood flow between April 2002 and January 2016. Mean age and body weight were 20 ± 13 months and 7.5 ± 6.5 kg, respectively. We retrospectively evaluated the patients' clinical data, including cardiac catheterization data, liver function, and liver fibrosis markers.
All patients were alive with a New York Heart Association status of I at the long-term follow-up. Changes between pre-bidirectional cavopulmonary shunt and 101 months after the Fontan procedure included the following: the cardiothoracic ratio of chest X-ray decreased from 52.2 ± 3.9% to 41.8 ± 5.9% (p < 0.001); systemic ventricle end-diastolic pressure decreased from 11.4 ± 3.2 mmHg to 6.9 ± 3.6 mmHg (p < 0.001); and the pulmonary artery index decreased from 485.1 ± 272.3 to 269.5 ± 100.5 (p = 0.02). Type IV collagen, hyaluronic acid, and procollagen levels increased over the normal range 116 months after the Fontan procedure.
The additional pulmonary blood flow at the time of bidirectional cavopulmonary shunt may contribute to pulmonary arterial growth at the Fontan procedure with low pulmonary arterial resistance and without ventricle volume overload. The Fontan circulation was well-maintained at the long-term follow-up, while liver fibrosis markers were above their normal values.
评估双向腔静脉肺动脉吻合术时的额外肺血流量及其对 Fontan 手术、Fontan 循环的长期结果和肝功能的影响。
我们纳入了 2002 年 4 月至 2016 年 1 月期间接受双向腔静脉肺动脉吻合术且合并额外肺血流量的 22 例患者（男 16 例，女 6 例）。平均年龄和体质量分别为 20 ± 13 个月和 7.5 ± 6.5kg。我们回顾性评估了患者的临床资料，包括心导管检查数据、肝功能和肝纤维化标志物。
所有患者在长期随访时均存活且纽约心脏协会心功能分级为 I 级。在双向腔静脉肺动脉吻合术前和 Fontan 手术后 101 个月时的变化包括：胸部 X 射线的心胸比从 52.2 ± 3.9%降至 41.8 ± 5.9%（p<0.001）；左心室舒张末期压力从 11.4 ± 3.2mmHg 降至 6.9 ± 3.6mmHg（p<0.001）；肺动脉指数从 485.1 ± 272.3 降至 269.5 ± 100.5（p=0.02）。Fontan 手术后 116 个月时，IV 型胶原、透明质酸和前胶原水平超过正常值。
双向腔静脉肺动脉吻合术时的额外肺血流量可能有助于在 Fontan 手术中促进肺动脉生长，同时保持肺动脉阻力低和心室容积无超负荷。Fontan 循环在长期随访中保持良好，而肝纤维化标志物高于正常值。
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