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首页> 外文期刊>Circulation journal >Hemodynamic consequences of a swinging, infarcted ventricular septum.
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Hemodynamic consequences of a swinging, infarcted ventricular septum.

机译：摆动的梗死性室间隔的血流动力学后果。

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摘要

Noonan syndrome presents with dysmorphic facial features, short stature, and cardiac abnormalities (most commonly pulmonic stenosis and hypertrophic cardiomyopathy). This report describes a rare case accompanied by a secundum atrial septal defect (ASD) and a ventricular septal aneurysm causing right ventricular (RV) pressure gradient. A 29-year-old mentally retarded man was admitted to hospital with exertional dyspnea. His somatic features included short stature (148 cm), hypertelorism, a shield chest, and thoracic scoliosis. Echocardiogram showed a secundum ASD with bidirectional shunting and a ventricular septum bulging toward the left ventricle in diastole, and then toward the RV in systole causing obliteration of the RV. The peak pressure gradient measured across the RV outflow by continuous wave Doppler was 30 mmHg. Cardiac catheterization revealed an elevated RV pressure without pulmonary hypertension and confirmed the pressure gradient. Right ventriculography revealed the septal excursion towardthe RV in systole, leaving only a small residual cavity in the inflow and outflow regions of the RV. The ASD was closed with an autologous pericardial patch. A thin, fibrous portion of the ventricular septum was resected and replaced with a Dacron patch. From the histological examination, the RV cavity obliteration turned out to be produced by the excursion of the infarcted ventricular septum.
机译：Noonan综合征表现出面部畸形，身材矮小和心脏异常（最常见的是肺动脉狭窄和肥厚型心肌病）。该报告描述了一种罕见的病例，伴有继发性房间隔缺损（ASD）和室间隔动脉瘤，引起右心室（RV）压力梯度。一名29岁的智障男子因劳累性呼吸困难入院。他的躯体特征包括身材矮小（148厘米），肢端亢进，盾形胸和胸椎侧弯。超声心动图显示具有双向分流的继发性室间隔缺损（ASD），并且室间隔向舒张期的左心室凸出，然后向收缩期的右室凸出，从而导致右室闭塞。通过连续波多普勒测得的整个RV流出的峰值压力梯度为30 mmHg。心脏导管检查显示RV压力升高，无肺动脉高压，并证实了压力梯度。右心室造影显示室间隔向右室偏移，在右室的流入和流出区域仅留下一个小的残留腔。 ASD用自体心包膜封闭。切除室间隔的纤细纤维部分，并用Dacron贴片代替。从组织学检查，发现右室腔闭塞是由梗死的室间隔的偏移引起的。

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