|
| HOME | SUBSCRIPTIONS | CURRENT ISSUE | PAST ISSUES | CARDIOSOURCE | SEARCH | HELP | FEEDBACK |
|
|
|
|||||||||
|
|||||||||||
|
J Am Coll Cardiol, 1995; 25:466-474 © 1995 by the American College of Cardiology Foundation |
Cardiopulmonary Department, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico DF, Mexico.
OBJECTIVES. This study characterized mortality in a group of Mexican children (n = 18, mean [+/- SD] age 9.9 +/- 3 years) with primary pulmonary hypertension and investigated the factors associated with their survival. BACKGROUND. Primary pulmonary hypertension is a progressive, fatal disease of unknown cause. Establishing the diagnosis earlier in life may influence prognosis. METHODS. A dynamic cohort of children with primary pulmonary hypertension were enrolled between December 1977 and May 1991 and followed up through September 1992. Measurements included hemodynamic and pulmonary function variables in addition to demographic data, medical history and response to vasodilator treatment. We also compared the survival estimates of these children with those of our adult patients with primary pulmonary hypertension (n = 42, mean age 27.9 +/- 8.5 years). RESULTS. Baseline mean (+/- SD) pulmonary artery pressure was similar in children and adults (66 +/- 15 vs. 65 +/- 18 mm Hg, p = NS), but a higher cardiac index resulted in a lower mean pulmonary vascular resistance index in children (18 +/- 7 vs. 26 +/- 12 U/m2, p < 0.01). The proportion of patients who had a positive hemodynamic response to vasodilator treatment was higher in children than in adults (41% vs. 25%). Estimated median survival in children was 4.12 years (95% confidence interval [CI] 0.75 to 8.66) and 3.12 years in adults (95% CI 0.5 to 13.25, chi-square log-rank 0.81, p = NS). Elevated right atrial pressure (rate ratio 10.2) and decreased stroke volume index (rate ratio 32.9) were the only significant predictors of mortality (Cox proportional hazards model). CONCLUSIONS. Children with primary pulmonary hypertension have a poor survival expectancy, which does not appear to differ from that in adults with primary pulmonary hypertension. Mortality in childhood primary pulmonary hypertension is also associated with variables that assess right ventricular dysfunction.
This article has been cited by other articles:
|
|
 |
|
  M. D. Puchalski, J. S. Lozier, D. J. Bradley, L. L. Minich, and L. Y. Tani Electrocardiography in the Diagnosis of Right Ventricular Hypertrophy in Children Pediatrics, September 1, 2006; 118(3): 1052 - 1055. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. B. Rosenzweig, D. D. Ivy, A. Widlitz, A. Doran, L. R. Claussen, D. Yung, S. H. Abman, A. Morganti, N. Nguyen, and R. J. Barst Effects of Long-Term Bosentan in Children With Pulmonary Arterial Hypertension J. Am. Coll. Cardiol., August 16, 2005; 46(4): 697 - 704. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Adatia Improving the Outcome of Childhood Pulmonary Arterial Hypertension: The Effect of Bosentan in the Setting of a Dedicated Pulmonary Hypertension Clinic J. Am. Coll. Cardiol., August 16, 2005; 46(4): 705 - 706. [Full Text] [PDF]
|
|
|
|
 |
|
 A Rashid and D Ivy Severe paediatric pulmonary hypertension: new management strategies Arch. Dis. Child., January 1, 2005; 90(1): 92 - 98. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Task Force members, N. Galie, A. Torbicki, R. Barst, P. Dartevelle, S. Haworth, T. Higenbottam, H. Olschewski, A. Peacock, G. Pietra, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension: The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology Eur. Heart J., December 2, 2004; 25(24): 2243 - 2278. [Full Text] [PDF]
|
|
|
|
 |
|
 D. Yung, A. C. Widlitz, E. B. Rosenzweig, D. Kerstein, G. Maislin, and R. J. Barst Outcomes in Children With Idiopathic Pulmonary Arterial Hypertension Circulation, August 10, 2004; 110(6): 660 - 665. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. B. Badesch, S. H. Abman, G. S. Ahearn, R. J. Barst, D. C. McCrory, G. Simonneau, and V. V. McLaughlin Medical Therapy For Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines Chest, July 1, 2004; 126(1_suppl): 35S - 62S. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, K. W. Presberg, R. L. Doyle, S. H. Abman, D. C. McCrory, T. Fortin, and G. Ahearn Prognosis of Pulmonary Arterial Hypertension*: ACCP Evidence-Based Clinical Practice Guidelines Chest, July 1, 2004; 126(1_suppl): 78S - 92S. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M Emmel, B Keuth, and S Schickendantz Paradoxical increase of pulmonary vascular resistance during testing of inhaled iloprost Heart, January 1, 2004; 90(1): e2 - 2. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Widlitz and R.J. Barst Pulmonary arterial hypertension in children Eur. Respir. J., January 1, 2003; 21(1): 155 - 176. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C J McMahon, J Kadkin, and M R Nihill Rapid regression of primary pulmonary hypertension Heart, July 1, 2001; 86(1): e1 - 1. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. Barst, G. Maislin, and A. P. Fishman Vasodilator Therapy for Primary Pulmonary Hypertension in Children Circulation, March 9, 1999; 99(9): 1197 - 1208. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. R. O'Hagan, P. C. Stillwell, and A. Arroliga Airway Responsiveness to Inhaled Albuterol in Patients wit Pulmonary Hypertension Clinical Pediatrics, January 1, 1999; 38(1): 27 - 33. [Abstract] [PDF]
|
|
|
|
|
|
J. Sandoval, J. Gaspar, T. Pulido, E. Bautista, M. L. Martinez-Guerra, M. Zeballos, A. Palomar, and A. Gomez Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension: A therapeutic alternative for patients nonresponsive to vasodilator treatment J. Am. Coll. Cardiol., August 1, 1998; 32(2): 297 - 304. [Abstract] [Full Text] [PDF]
|
|
| HOME | SUBSCRIPTIONS | CURRENT ISSUE | PAST ISSUES | CARDIOSOURCE | SEARCH | HELP | FEEDBACK |
