Elsevier

Respiratory Medicine

Volume 104, Issue 11, November 2010, Pages 1588-1607
Respiratory Medicine

Review
Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: A systematic review of the literature

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Summary

Rationale

There is a lack of consensus on factors that predict mortality in idiopathic pulmonary arterial hypertension (IPAH). Tests that can accurately predict prognosis are needed to guide treatment and counsel patients.

Methods

We conducted a systematic review to identify factors that prognosticate mortality in IPAH. Study design, cohort size, comparison method, measured value, and statistical significance was extracted for eight pre-selected parameters [pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), mean right atrial pressure (mRAP), cardiac output, right ventricular end diastolic pressure, functional class, 6 min walk distance (6MWD), and diffusing capacity of carbon monoxide].

Results

107 factors have been associated with mortality in IPAH. A reproducible predictive association with mortality was demonstrated for only 10 factors: functional class (14 studies), heart rate (10 studies), 6MWD (8 studies), pericardial effusion (5 studies), mPAP (10 studies), mRAP (17 studies), cardiac index (13 studies), stroke volume index (4 studies), PVR (10 studies), mixed venous PaO2 or saturations (4 studies). Of the 8 factors chosen for detailed evaluation, there were at least half as many studies that evaluated the variable and did not find an association with mortality compared to those that did.

Conclusions

There is a large body of literature describing numerous factors that predict mortality in IPAH. Most factors have been assessed in very few studies. There are conflicting reports on the prognostic value of many factors. These discrepancies highlight the need to evaluate the literature in total when considering the utility of variables as prognostic factors in IPAH.

Keywords

Idiopathic pulmonary arterial hypertension
Prognosis
Survival
Mortality
Outcomes
Systematic review

Abbreviations

6MWD
6 min walk distance
CO
cardiac output
CI
cardiac index
CPET
cardiopulmonary exercise
DLCO
diffusing capacity of carbon monoxide
IPAH
idiopathic pulmonary arterial hypertension
JTG
John T Granton
JRS
John R Swiston
mPAP
mean pulmonary arterial pressure
mRAP
mean right atrial pressure
NIH
National Institutes of Health
NYHA
New York Heart Association
O2
oxygen
PAH
Pulmonary arterial hypertension
PCWP
pulmonary capillary wedge pressure
PO2
partial pressure of oxygen
PPH
primary pulmonary hypertension
PVR
pulmonary vascular resistance
RVEDP
right ventricular end diastolic pressure
SRJ
Sindhu R Johnson
WHO
World Health Organization

Cited by (0)

This work was performed at the University of British Columbia and the University of Toronto.