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患者男性,18岁,诉劳力性呼吸困难5年。体检:肺动脉瓣区可闻及续较久的喷射性收缩期杂音,肺动脉瓣第2音减轻,血压125/80,胸部X线检查心脏明显增大,二维超声心动图检查示显著右室肥厚,舒张末期右室游离壁厚3.2cm,室间隔厚3.4cm,左室游离壁正常(1.0cm),肺动脉瓣明显增厚。右心导管检查示右室压力260/21mmHg,导管不能越过肺动脉瓣。右室造影示显著右室肥厚伴明显小梁形成,肺动脉瓣明显增厚、圆凸,提示肺动脉瓣狭窄。
Male patient, 18 years old, v. Exertional dyspnea for 5 years. Physical examination: pulmonary valve area can be heard and continued for a long time jet systolic murmur, pulmonary valve relief second tone, blood pressure 125/80, chest X-ray examination significantly increased heart, two-dimensional echocardiography showed significant right ventricular hypertrophy , End-diastolic right ventricular free wall thickness 3.2cm, interventricular septum thickness 3.4cm, normal left ventricular free wall (1.0cm), pulmonary valve was significantly thicker. Right heart catheterization showed right ventricular pressure 260 / 21mmHg, the catheter can not cross the pulmonary valve. Right ventricular angiography showed significant right ventricular hypertrophy with significant trabecular formation, pulmonary valve was significantly thicker, convex, suggesting pulmonary valve stenosis.