Korean Circ J.  2003 Jun;33(6):507-512. 10.4070/kcj.2003.33.6.507.

A Clinical Review of Primary Pulmonary Hypertension

• Lee WD
• Kim DS
• Lee JH
• Cho KI
• Cho KH
• Kim DK
• Kim DI
• Lee YM
• Park JS
• Kim YJ
• Cha TJ
• Lee JW

• KMID: 1858818
• DOI: http://doi.org/10.4070/kcj.2003.33.6.507

Abstract

BACKGROUND AND OBJECTIVES
Primary (idiopathic) pulmonary hypertension is a rare, progressive and fatal disease. It has been defined, by the World Health Organization, as a mean pulmonary arterial pressure greater than 25 mmHg at rest, or greater than 30 mmHg during exercise, without the apparent cause of secondary pulmonary hypertension. This study was performed to better understanding the clinical presentation, natural history and prognosis of primary pulmonary hypertension.
SUBJECTS AND METHODS
A total of 18 patients, who were diagnosed as primary pulmonary hypertension, at three University Hospitals, were retrospectively reviewed. All patients had undergone echocardiography and cardiac catheterization.
RESULTS
With the patients there was a male: female ratio of 1:8, ranging in age between 10 and 50 years. The most common presenting symptom was dyspnea on exertion, with other symptoms comprising of fatigue in 11, chest pain in 5, syncope in 3 and hemoptysis in 2. The ECG & echocardiography reflected the presence of right-sided heart enlargement. The average right ventricular systolic pressure, from Doppler echocardiography, was 73.6+/-18.8 mmHg. The mean pulmonary artery pressure and pulmonary capillary wedge pressure were 52.9+/-18.4 and 9.2+/-3.1 mmHg, respectively. The survival times were within 30 and 21 to 60 months in 9 and the remaining patients, respectively.
CONCLUSION
We conclude that primary pulmonary hypertension is common in female patients in their third to fifth decades. This study also showed a poor prognosis, as in other reports.

Keyword

Pulmonary hypertension; Primary

MeSH Terms

Arterial Pressure
Blood Pressure
Cardiac Catheterization
Cardiac Catheters
Cardiomegaly
Chest Pain
Dyspnea
Echocardiography
Echocardiography, Doppler
Electrocardiography
Fatigue
Female
Hemoptysis
Hospitals, University
Humans
Hypertension, Pulmonary*
Male
Natural History
Prognosis
Pulmonary Artery
Pulmonary Wedge Pressure
Retrospective Studies
Syncope
World Health Organization