Ozer Necla, Deveci Onur Sinan, Kaya Ergün Bariş, Demircin Metin
Department of Cardiology, Medicine Faculty of Hacettepe University, Ankara, Turkey.
Turk Kardiyol Dern Ars. 2009 Sep;37(6):421-4.
Acquired pulmonary stenosis is rare in adults and may be missed unless a high index of suspicion is present. Extrinsic pulmonic stenosis is even rarer and predominantly caused by external thoracic masses creating non-dynamic obstruction of the right ventricular outflow tract. A 20-year-old female was referred to our center with a cystic mass detected by transthoracic echocardiography and thoracic computed tomography, in the superoanterior mediastinum, 5.5 x 5.5 x 7 cm in size, causing main pulmonary trunk compression. Repeat transthoracic echocardiography demonstrated a mass causing compression of the main pulmonary artery immediately after the pulmonary valve. Continuous wave Doppler showed a peak systolic gradient of 65 mmHg and a mean gradient of 37 mmHg in the pulmonary artery at the site of compression. She underwent an open thoracotomy via a midline sternotomy. The mass was firmly attached to the pericardium. Its largest diameter was 15 cm; it surrounded the left phrenic nerve completely and invaded the outer wall of the pulmonary artery and aorta. The mass could only be partly dissected. The pathological diagnosis of the mass was stage IIa nodular sclerosing Hodgkin's lymphoma. The patient received postoperative chemotherapy and thoracal radiotherapy. She was in remission without any cardiac complaint.
获得性肺动脉狭窄在成人中罕见，除非高度怀疑否则可能被漏诊。外在性肺狭窄更为罕见，主要由胸外肿块引起，导致右心室流出道非动态梗阻。一名20岁女性因经胸超声心动图和胸部计算机断层扫描发现一个囊性肿块而被转诊至我们中心，该肿块位于前上纵隔，大小为5.5×5.5×7cm，导致主肺动脉受压。重复经胸超声心动图显示，该肿块在肺动脉瓣后立即压迫主肺动脉。连续波多普勒显示，在受压部位肺动脉的收缩期峰值梯度为65mmHg，平均梯度为37mmHg。她通过正中胸骨切开术接受了开胸手术。肿块与心包紧密相连。其最大直径为15cm；它完全包裹了左膈神经，并侵犯了肺动脉和主动脉的外壁。肿块只能部分切除。肿块的病理诊断为IIa期结节硬化型霍奇金淋巴瘤。患者接受了术后化疗和胸部放疗。她处于缓解期，无任何心脏不适。
