Manmadhan Arun, Malhotra Sunil P, Weinberg Catherine R, Reyentovich Alex, Latson Larry A, Bhatla Puneet, Saric Muhamed
Department of Medicine, Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, NY, USA.
Department of Cardiothoracic Surgery, Division of Pediatric and Adult Congenital Cardiac Surgery, New York University School of Medicine, New York, NY, USA.
J Cardiothorac Surg. 2017 Oct 30;12(1):93. doi: 10.1186/s13019-017-0654-9.
Pulmonary artery intimal spindle cell sarcomas are rare and carry with them a poor prognosis and high rate of recurrence. In extremely rare cases, this tumor can infiltrate the pulmonic valve and manifest as adult-onset pulmonic stenosis.
We report an unusual case of a patient with symptomatic, adult-onset severe pulmonic stenosis who was referred for possible balloon valvuloplasty but was subsequently found to have pulmonary artery intimal sarcoma infiltrating the pulmonary valve leading to progressive exertional dyspnea.
The presence of adult-onset pulmonic stenosis should prompt the clinician to investigate further as most cases of pulmonic stenosis are congenital in nature and present early in life. Careful diagnostic evaluation in concert with multimodal imaging should take place to arrive at the correct and challenging diagnosis of sarcoma-induced adult-onset severe pulmonic stenosis. Given the poor prognosis and rapid progression of disease, early diagnosis is crucial.
肺动脉内膜梭形细胞肉瘤罕见，预后不良且复发率高。在极为罕见的情况下，这种肿瘤可浸润肺动脉瓣并表现为成人期肺动脉狭窄。
我们报告了一例不同寻常的病例，一名患有症状性成人期重度肺动脉狭窄的患者因可能进行球囊瓣膜成形术前来就诊，但随后发现患有浸润肺动脉瓣的肺动脉内膜肉瘤，导致进行性劳力性呼吸困难。
成人期肺动脉狭窄的出现应促使临床医生进一步调查，因为大多数肺动脉狭窄病例本质上是先天性的且在生命早期出现。应结合多模态成像进行仔细的诊断评估，以得出肉瘤所致成人期重度肺动脉狭窄这一正确且具有挑战性的诊断。鉴于预后不良和疾病进展迅速，早期诊断至关重要。
