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新生兒肺動脈瓣膜閉鎖合併腫塊樣右心室血栓:一解剖病例報告

Right Ventricular Thrombus Mimicking a Tumor Mass in a Newborn with Pulmonary Atresia - an Autopsy Report

摘要


嬰兒時期心臟內腫塊相當少見,其原因可為腫瘤、血栓和細菌性心內膜炎的贅生物。本例為11天大之男嬰,因出生第二日開始即有呼吸困難,嘴唇和指尖發紺而轉至本院。理學檢查可聽到第二度收縮期心雜音,有時為連續性心雜音。心電圖正常,胸部X光顯示肺紋減少,雙相超音波心圖在右心室腔有一腫瘤樣影像。病理解剖發現肺動脈閉鎖瓣下有一小血栓 (0.3×0.2 cm),開放性動脈導管 (1.0×0.5 cm),有孔狀之卵圓孔,右心室發育不全,右心室壁肥厚,右心室腔內有一球狀腫塊樣血栓 (2.0×1.3×1.0 cm)。右心室腔血流鬱滯加上低血氧症可能是造成右心室血栓形成之原因。

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並列摘要


Intracardiac masses in infancy are not common. Tumors, thrombi and vegetations of bacterial endocarditis are exceedingly rare in this age group. These masses are seldom diagnosed before the infant’s death. Two-dimensional echocardiography is a noninvasive technique that can detect and differentiate intracardiac masses that occur in infants. It can serially detect changes in the size or location of the masses, thus guiding subsequent medical or surgical intervention. An 11-day-old male newborn had suffered from lip and finger nail cyanosis, and dyspnea since 2 days old. Physical examination revealed subcostal retractions and no heart murmur during admission but a grade II/VI systolic murmur with a sometime grade II/VI continuous murmur which could be heard at the left upper sternal border 3 days after admission. The liver was palpable 2cm below the right costal margin. The electro-cardiogram was normal. The chest X-ray showed a normal sized heart (CTR=0.58) with decreased pulmonary vascularity. Arterial blood gases revealed hypoxemia (PO2 24.1 mmHg) and metabolic acidosis, even though the patient was receiving O2 at 50%. The two-dimensional echocardiogram from the parasternal long axis view showed an echo-dense tumor mass in the right ventricular cavity. At autopsy, a complex congenital heart disease was found. The pathologic findings were: patent ductus arteriosus (1.0×0.5cm), fenestrated foramen ovale, unopening pulmonary valve with thrombus (0.3×0.2cm), right ventricular hypoplasia and hypertrophy of the right ventricular wall (1 cm in thickness). A ball like, organized and calcified throumbus, measuring 2.0×1.3×1.0 cm was found in the right ventricular cavity. It was hypothesized that stasis of the right ventricular blood flow with hypoplasia and hypofunction of the right ventricle, and pulmonary valve unopening plus hypoxia were factors which contributed to the formation of the thrombus in this case.

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