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KMID : 0368120000300030346
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Korean Circulation Journal
2000 Volume.30 No. 3 p.346 ~ p.351
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A Case of Primary Pulmonary Hypertension with the Congenital Absence of Inferior Vena Cava
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1김건형/1Kun Hyung Kim
1정명호/1주신배/1이승욱/1김남호/1조장현/1안영근/1조정관/1박종춘/1강정채/2김윤현/1Myung Ho Jeong/1Shin Bae Joo/1Seung Uk Lee/1Nam Ho Kim/1Jang Hyun Cho/1Young Keun Ahn/1Jeong Gwan Cho/1Jong Chun Park/1Jung Chaee Kang/2Yun Hyun Kim
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Abstract
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Primary pulmonary hypertension (PPH) is one of rare and fatal vascular disorder. The
exact mechanisms for the increased pulmonary vascular resistance and pressure in PPH
are unknown. The disease is advanced progressively and patients naturally die within
three years after initial diagnosis. PPH is characterized with mean pulmonary artery
pressure over 25-30 mmHg and intractable hyertension despite any intensive therapeutic
maneuvers. Associated congenital anomaly is very rare. We report 19 year-old female
patient who diagnosed as PPH 10 years ago and suffered from severe pulmonary
hypertension (100 mmHg of pulmonary arterial pressure) with the congenital absence of
inferior vena cava. Her venous blood from lower body was drained through azygous
vein into superior vena cava.
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KEYWORD
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