弁置換により蛋白漏出性胃腸症が治癒した孤立性三尖弁閉鎖不全症の1例

A case of isolated tricuspid regurgitation associated with protein-losing gastroenteropathy

　15年前に敗血症に罹患，この際に感染性心内膜炎を合併し，三尖弁閉鎖不全から蛋白漏出性胃腸症が惹起されたと考えられる症例に三尖弁置換術を行ったところ，蛋白漏出性胃腸症の治癒がみられた。心疾患と蛋白漏出性胃腸症の関連，発症機転を論じる上で興味ある一例と考えられ，報告する。

A 43 year old woman was admitted to our hospital in April 1987 due to shortness of breath and pedal edema. She had a history of sepsis associated with the crisis of hyperthyroidism 15 years prior to the admission. Physical examination revealed a badly nourished with ascites : weight was 56 kg and he-ight 156 cm. The heart sounds were distant with mild holosystoric murmur (grade I /VI) at xiphoiste-rnum. The chest X-ray showed cardiomegaly (CTR : 72. 3%) with pleural effusion. The electrocardiog-ram showed atrial fibrillation, low voltage and ri-ght ventriculer hypertrophy. The echocardiogram showed marked dilatation of right atrium and vent-ricle with very short septal leaflet of tricuspid val-ve. The anterior and posterior leaflets were unde-tected. The tricuspid regurgitant doppler signal was recorded up to hepatic vein. No other abnor-malities were noted in other valves. The white cell count was 4900 with lymphocytopenia (26%; T-cell 82%, 13-cell 13%). Serum total protein was reduced to 3.4 g/dl with albumin 1. 64 g/dl. Immu-noelectrophoresis showed normal IgG, IgA and IgM. Proteinuria was not recognized. Fecal excre-tion of polyvinylpyrrolidone-¹³¹I (PVP) was eleva-ted to 2.8%, The systolic pressure in pulmonary artery, right ventricle, right atrium, superior and inferior vena cave were almost equal as 26 mmHg. The pulmonary arterial scintigraphy disclosed mul-tiple peripheral defects in both lungs. Two weeks after the operation of tricupid valve replacement based on the diagnosis of protein-losing enteropathy due to isolated tricuspid regurgitation, serum total protein and albumin were normalized to 6. 8 g/dl and 3. 6 g/dl respectively, but the lymphocytopenia was persistent. She become very well, with free of ascites and edema.

We strongly suspected from her clinical course that the cause of tricuspid regurgitation was due to infective endocarditis that might have occured in bacteremia, although it could not be proved in the histopathological findings of resected valve.

This case demonstrates that hypoproteinemia deri-ved from protein-losing enteropathy due to isolated tricuspid regurgitation can be cured by releasing the elevated pressure in right atrium. This is the first case with isolated tricuspid regurgitation asso-ciated with protein-losing enteropathy.