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Primary pulmonary hypertension in children: clinical characterization and survival
J Sandoval1, O Bauerle, A Gomez
1Cardiopulmonary Department, Instituto Nacional de Cardiología Ignacio Chávez, México DF, México.
Insights
Children with primary pulmonary hypertension (PPH) have poor survival, similar to adults with PPH. Early diagnosis and identifying right ventricular dysfunction are crucial for improving outcomes in pediatric PPH.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Childhood Mortality Studies
Background:
- Primary pulmonary hypertension (PPH) is a severe, progressive, and often fatal condition of unknown etiology.
- Earlier diagnosis of PPH in children may significantly impact prognosis and survival rates.
Purpose of the Study:
- To characterize mortality patterns in Mexican children diagnosed with primary pulmonary hypertension.
- To identify factors influencing survival in pediatric patients with PPH.
- To compare survival estimates between children and adults with PPH.
Main Methods:
- A cohort of 18 children with PPH (mean age 9.9 years) were followed from 1977-1992.
- Hemodynamic, pulmonary function, and treatment response data were collected.
- Survival data were compared with 42 adult PPH patients and analyzed using Cox proportional hazards models.
Main Results:
- Children with PPH showed a higher cardiac index and lower pulmonary vascular resistance index compared to adults.
- A greater proportion of children responded positively to vasodilator treatment (41%) versus adults (25%).
- Median survival was similar in children (4.12 years) and adults (3.12 years). Elevated right atrial pressure and decreased stroke volume index predicted mortality.
Conclusions:
- Children diagnosed with primary pulmonary hypertension exhibit a poor survival expectancy, comparable to adult patients.
- Mortality in childhood PPH is significantly associated with indicators of right ventricular dysfunction.
- Further research into early detection and management strategies for pediatric PPH is warranted.
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.