CLINICAL STUDY: PRIMARY PULMONARY HYPERTENSION
Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension
Ronald J. Raymond, MD*,
Alan L. Hinderliter, MD*,*,
Park W. Willis, IV, MD*,
David Ralph, MD ,
Edgar J. Caldwell, MD ,
William Williams, MD,
Neil A. Ettinger, MD ,
Nicholas S. Hill, MD||,
Warren R. Summer, MD¶,
Bennett de Boisblanc, MD¶,
Todd Schwartz, MS#,
Gary Koch, PhD#,
Linda M. Clayton, Pharm D**,
Maria M. Jöbsis, BA**,
James W. Crow, PhD,
Walker Long, MD Primary Pulmonary Hypertension Study Group
* University of North Carolina, Chapel Hill, North Carolina, USA
University of Washington, Seattle, Washington, USA
Maine Medical Center, Portland, Maine, USA
Washington University, St. Louis, Missouri, USA
|| Rhode Island Hospital, Providence, Rhode Island, USA
¶ Louisiana State University Medical Center, New Orleans, Louisana, USA
# Department of Biostatistics, University of North Carolina, Chapel Hill, North Carolina, USA
** GlaxoWellcome Inc., Research Triangle Park, North Carolina, USA
United Therapeutics, Inc., Chapel Hill, North Carolina, USA
Cato Research Ltd., Durham, North Carolina, USA
Manuscript received August 8, 2001;
revised manuscript received January 3, 2002,
accepted January 16, 2002.
* Reprint requests and correspondence: Dr. Alan L. Hinderliter, Division of Cardiology, University of North Carolina, CB # 7075, 338 Burnett-Womack, Chapel Hill, North Carolina 27599-7075, USA
hinderli{at}med.unc.edu
OBJECTIVES: The aim of this study was to evaluate the relationships between echocardiographic findings and clinical outcomes in patients with severe primary pulmonary hypertension (PPH).
BACKGROUND: Primary pulmonary hypertension is associated with abnormalities of right heart structure and function that contribute to the poor prognosis of the disease. Echocardiographic abnormalities associated with PPH have been described, but the prognostic significance of these findings remains poorly characterized.
METHODS: Echocardiographic studies, invasive hemodynamic measurements and 6-min walk tests were performed and outcomes prospectively followed in 81 patients with severe PPH. Subjects were participants in a 12-week randomized trial examining the effects of prostacyclin plus conventional therapy compared with conventional therapy alone.
RESULTS: During the mean follow-up period of 36.9 ± 15.4 months, 20 patients died and 21 patients underwent transplantation. Pericardial effusion (p = 0.003) and indexed right atrial area (p = 0.005) were predictors of mortality. Pericardial effusion (p = 0.017), indexed right atrial area (p = 0.012) and the degree of septal shift in diastole (p = 0.004) were predictors of a composite end point of death or transplantation. In multivariable analyses incorporating clinical, hemodynamic and echocardiographic variables, pericardial effusion and an enlarged right atrium remained predictors of adverse outcomes. Six-minute walk results, mixed venous oxygen saturation and initial treatment randomization were also independently associated with a poor prognosis.
CONCLUSIONS: Pericardial effusion, right atrial enlargement and septal displacement are echocardiographic abnormalities that reflect the severity of right heart failure and predict adverse outcomes in patients with severe PPH. These characteristics may help identify patients appropriate for more intensive medical therapy or earlier transplantation.
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Abbreviations and Acronyms
| | CI | | confidence interval | | HR | | hazard ratio | | NYHA | | New York Heart Association | | PPH | | primary pulmonary hypertension | | RA | | right atrium | | RVEDA | | right ventricular end-diastolic area | | RVESA | | right ventricular end-systolic area | | TR | | tricuspid regurgitation |
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