Jariwala Pankaj, Giragani Suresh, Maturu Venkata Nagarjuna
Department of Cardiology, Yashoda Hospitals, Somajiguda, Hyderabad, Telangana, India.
Department of Radio-Diagnosis, Yashoda Hospitals, Somajiguda, Hyderabad, Telangana, India.
Lung India. 2021 Jan-Feb;38(1):86-87. doi: 10.4103/lungindia.lungindia_103_20.
Congenital isolated unilateral hypoplasia of the pulmonary artery (CIUPAH) can have late presentation in the adulthood, mostly diagnosed incidentally, as in our case. They may have symptoms such as exertional dyspnea, recurrent lung infections, and hemoptysis. Although the surgical management is a definitive treatment modality, it depends on the presence of reversible pulmonary hypertension and size of the pulmonary arteries; interventional and medical management can be attempted as a palliative therapy in cases where definitive surgical management is not feasible. We have discussed a rare case of isolated unilateral pulmonary artery hypoplasia which is a congenital anomaly of the lung vasculature without associated congenital heart disease manifested in adulthood.
先天性孤立性单侧肺动脉发育不全（CIUPAH）在成年期可能出现较晚，大多是偶然诊断出来的，就像我们的病例一样。患者可能有劳力性呼吸困难、反复肺部感染和咯血等症状。虽然手术治疗是一种确定性的治疗方式，但它取决于是否存在可逆性肺动脉高压以及肺动脉的大小；在无法进行确定性手术治疗的情况下，可尝试介入和药物治疗作为姑息性疗法。我们讨论了一例罕见的孤立性单侧肺动脉发育不全病例，它是一种肺部血管系统的先天性异常，成年期出现且无相关先天性心脏病。
