Background: Pulmonary hypertension (PH) with congenital heart disease (CHD) affects the functional capacity (FC), quality of life, and survival. However, the importance of different echocardiographic parameters and their correlation with FC is unclear. Methods and Results: A custom-made sheet for 34 consecutive patients with PH due to CHD was made to include patient's demographic data, underlying cardiac disorder, and FC by 6-min walk test (6MWT). The patients were subdivided into Group 1 with 6MWT < 330 m and Group 2 with 6MWT > 330 m. A cutoff value of 330 m was selected because it reflected the survival and outcome of patients in many studies before. Left ventricle global radial strain, baseline saturation, and saturation after 6MWT showed a significant strong positive correlation with 6MWT ( r = 0.755, 0.714, and 0.721, P = 0.001, 0.000, and 0.000, respectively). Multiple regression analysis using a multivariate model showed that the mean pulmonary artery pressure (MPAP) and baseline saturation are the most independent predictors of the FC ( P = 0.028 and 0.049, respectively), with a cutoff point for MPAP > 30 mmHg (area under the curve [AUC]: 0.85) with a sensitivity and specificity of 69.23% and 95.24%, respectively, and cutoff point for saturation < 94% (AUC: 0.852) with a sensitivity and specificity of 92.31% and 76.19%, respectively. Conclusion: The MPAP and the baseline oxygen saturation were the most independent predictors of impaired FC. They can be used for risk stratification and as surrogate predictors of outcome in this group of patients.
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