A Case of Primary Pulmonary Hypertension with the Congenital Absence of Inferior Vena Cava.
10.4070/kcj.2000.30.3.346
- Author:
Kun Hyung KIM
;
Myung Ho JEONG
;
Shin Bae JOO
;
Seung Uk LEE
;
Nam Ho KIM
;
Jang Hyun CHO
;
Young Keun AHN
;
Yun Hyun KIM
;
Jeong Gwan CHO
;
Jong Chun PARK
;
Jung Chaee KANG
- Publication Type:Case Report
- Keywords:
Primary pulmonary hypertension;
Congenital Anomaly
- MeSH:
Diagnosis;
Female;
Humans;
Hypertension;
Hypertension, Pulmonary*;
Pulmonary Artery;
Vascular Resistance;
Veins;
Vena Cava, Inferior*;
Vena Cava, Superior;
Young Adult
- From:Korean Circulation Journal
2000;30(3):346-351
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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 pro-gressively and patients naturally die within three years after the initial diagnosis. PPH is characterized with mean pulmonary artery pressure over 25-30 mmHg and intractable hypertension despite any intensive therapeutic maneuvers. Associated congenital anomaly is very rare. We report a 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.
