문맥고혈압에 동반된 폐고혈압은 비교적 드문 질환으로 1951년 Mantz와 Craig가 처음 보고한 후 꾸준히 보고되고 있으나 국내엔 아직 보고된 바가 없다. 최근 간경변증으로 인한 복수와 호흡곤란을 주소로 내원한 49세 여자 환자에서 폐고혈압이 동반되어 있었던 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2 % incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances, normally metabolized by the liver, may have gained access to pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.
