Koga Hiroshi, Hidaka Tomoko, Miyako Kenichi, Suga Naohiro, Takahashi Noboru
Department of Pediatrics, National Hospital Organization Beppu Medical Center, 1473 Oaza-Uchikamado, Beppu, Oita, Japan.
Pediatr Cardiol. 2010 Nov;31(8):1186-90. doi: 10.1007/s00246-010-9787-5. Epub 2010 Sep 3.
Unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although UAPA has been reported previously, its age-related pathogenesis and symptoms remain unclear. This retrospective cohort study included cases of UAPA reported in Japan at medical meetings or in the literature from 1990 through 2009. Patients with other congenital cardiac defects were excluded from the study. Clinical status was assessed according to age, and the clinical course of patients with isolated UAPA was compared with that of patients who had UAPA with a patent ductus arteriosus (PDA). Of the 92 patients with UAPA identified, 78 had isolated UAPA (14 with PDA). Hemoptysis and collateral arteries were observed in 0 and 13% of patients with isolated UAPA who were younger than 1 year, as compared with 24 and 50% of those 20 years of age or older, respectively. Pulmonary hypertension was present in 5% of the patients aged 1 to 19 years. Among patients 20 years or older, however, 32% had pulmonary hypertension, and 8% died. Compared with isolated UAPA, UAPA with PDA was associated with an earlier diagnosis (median age, 20 vs. 0 years; p = 0.002), a higher prevalence of pulmonary hypertension (22% vs. 86%; p < 0.0001), and a higher mortality rate (4% vs. 21%; p = 0.046). Collateral artery formation and pulmonary hypertension progress with age in patients with UAPA. Early diagnosis and revascularization may prevent the age-related progression of UAPA.
单侧肺动脉缺如（UAPA）是一种罕见的先天性异常。尽管此前已有关于UAPA的报道，但其与年龄相关的发病机制和症状仍不清楚。这项回顾性队列研究纳入了1990年至2009年在日本医学会议或文献中报道的UAPA病例。其他先天性心脏缺陷患者被排除在研究之外。根据年龄评估临床状况，并将孤立性UAPA患者的临床病程与合并动脉导管未闭（PDA）的UAPA患者进行比较。在确诊的92例UAPA患者中，78例为孤立性UAPA（14例合并PDA）。1岁以下孤立性UAPA患者咯血和侧支动脉的发生率分别为0和13%，而20岁及以上患者的发生率分别为24%和50%。1至19岁患者中5%存在肺动脉高压。然而，在20岁及以上患者中，32%存在肺动脉高压，8%死亡。与孤立性UAPA相比，合并PDA的UAPA诊断更早（中位年龄，20岁对0岁；p = 0.002），肺动脉高压患病率更高（22%对86%；p < 0.0001），死亡率更高（4%对21%；p = 0.046）。UAPA患者侧支动脉形成和肺动脉高压随年龄进展。早期诊断和血运重建可能预防UAPA与年龄相关的进展。
