Pulmonary Hypertension

Pulmonary hypertension is characterized by increased pulmonary vascular resistance and elevated pulmonary arterial pressure that can lead to abnormalities in cardiac structure and function. Right ventricular hypertrophy and depressed ventricular function, low cardiac output, and pericardial effusion are among the more common sequelae. Morbidity, disability, and mortality associated with pulmonary hypertension are generally a function of these cardiac abnormalities, with right heart failure a major cause of death. Pulmonary hypertension can be primary (or idiopathic) or it can be secondary to several causes including lung disease causing chronic hypoxia or chronic thromboembolic disease.

Both generic and respiratory condition-specific instruments have been used to measure HRQL in pulmonary hypertension.

Published studies of health-related quality of life (HRQL) in patients with pulmonary hypertension are rare, with the few studies that exist focusing on primary pulmonary hypertension. To date (October 1999), three papers have appeared in the literature. A fourth paper evaluated quality of life outcomes using the New York Heart Association classification.

Generic Health-Related Quality of Life Instruments Used in Pulmonary Hypertension

Nottingham Health Profile

• Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundate BH et al. A comparision of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996;334(5):296-302.
• Hinderliter AL, Willis PW IV, Barst RJ. Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Circulation. 1997;95:1479-86.

Medical Outcomes Study SF-36

• Archibald CJ, Auger WR, Fedullo PF, Channick RN, Kerr KM, Jamieson SW, Kapelanski DP, Watt CN, Moser KM. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med. 1999;160:523-8.

Condition-Specific Health-Related Quality of Life Instruments Used in Pulmonary Hypertension

Chronic Heart Failure Questionnaire

• Hinderliter AL, Willis PW IV, Barst RJ. Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Circulation. 1997;95:1479-86.
• Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundate BH et al. A comparision of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996;334(5):296-302.

Minnesota Living with Heart Failure Questionnaire

• Barst RJ, McGoon M, McLaughlin V, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2003;41(12):2119-25.

New York Heart Association (NYHA) classification

• Frank H, Mlczoch J, Huber K, Schuster E, Gurtner HP, Kneussl M. The effect of anticoagulant therapy in primary and anorectic drug-induced pulmonary hypertension. Chest. 1997;112:714-21.