Nair Ashvin Krishna, Haranal Maruti, Elkhatim Ibrahim Mukhtar, Dillon Jeswant, Hew Chee Chin, Sivalingam Sivakumar
Department of Cardiothoracic Surgery, Institut Jantung Negara, Kuala Lumpur, Malaysia.
Ann Pediatr Cardiol. 2020 Jul-Sep;13(3):212-219. doi: 10.4103/apc.APC_111_19. Epub 2020 Jun 23.
Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms. We report the mid-term outcomes of surgical correction of patients with APVS.
A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients.
The overall 10-year survival was 82.1%. There was 81.1% overall freedom from re-intervention at 10 years. No statistically significant difference was found in 10-year survival ( = 0.464) and reoperation rates ( = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival ( = 0.039) and freedom from re-intervention ( = 0.016) compared to infants. Patients without respiratory complications had 100% 10-year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications.
There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems.
肺动脉瓣缺如综合征（APVS）是法洛四联症的一种变体，其特征为肺动脉瘤样扩张，压迫气管支气管树，导致呼吸道症状。我们报告了APVS患者手术矫正的中期结果。
2001年至2015年间共有27例患者接受手术，平均随访6.4±4.1年。27例患者中，14例（51.9%）为婴儿。修复时的中位年龄为9.8个月。6例患者（22.2%）术前需要插管，11例患者（40.7%）有呼吸窘迫症状。15例患者（55.6%）采用带瓣管道置换肺动脉瓣，6例患者（22.2%）采用单叶瓣，6例患者（22.2%）采用跨环补片。所有患者均进行了肺动脉缩窄成形术。
总体10年生存率为82.1%。10年时总体再次干预的自由度为81.1%。在带瓣管道、单叶瓣或跨环补片技术之间，10年生存率（P = 0.464）和再次手术率（P = 0.129）未发现统计学显著差异。与婴儿相比，年龄较大的儿童在统计学上有显著更长的生存期（P = 0.039）和免于再次干预的时间（P = 0.016）。无呼吸并发症的患者10年生存率为100%，10年再次手术自由度为93.3%，而有呼吸并发症的患者分别为55.6%和60.1%。
多年来APVS的手术结果有所改善。然而，管理婴儿和有持续呼吸问题的患者仍然是一项挑战。
