Key result
Patients with PAH-CHD and Eisenmenger syndrome experienced a significantly longer time from symptom onset to diagnosis compared to patients with IPAH (3 years vs 10.5 months; p=0.0006).
Why the study?
To analyze the clinical, functional, and hemodynamic profile of patients with PAH-CHD and Eisenmenger syndrome compared to IPAH, and evaluate drug therapy patterns.
Observational (n=40)
Yes
p-value: p=0.0006
Patients with Eisenmenger syndrome experience a significantly longer delay in diagnosis and have lower oxygen saturation compared to those with idiopathic pulmonary hypertension.
Small registry comparison of PAH-CHD versus IPAH profiles; leaves open tailored therapy questions in Eisenmenger syndrome.
Aim. To conduct a comprehensive analysis of the clinical, functional, hemodynamic profile of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and Eisenmenger syndrome, as compared with the group of idiopathic pulmonary hypertension (IPAH) according to the Russian Registry, and also to study the features of drug therapy. Materials and methods. The study involved 20 patients with PAH-CHD and Eisenmenger syndrome and 20 patients with IPAH over 18 years old. All patients had been entered in the Russian registry (NCT03707561). A comparative analysis of clinical, functional, hemodynamic parameters was held. The diagnosis was established in accordance with the algorithm proposed in the Russian guidelines the diagnosis and treatment of PH (2016). Results. In this study, 20 patients with PAH-CHD (16 women and 4 men) and Eisenmenger syndrome and 20 patients with IPAH (18 women and 2 men) were analyzed. Patients were comparable in age and sex. The time from the onset of symptoms to the final diagnosis in the PAH-CHD group was significantly longer: on average, it took three years to establish the diagnosis, whereas in patients with IPAH, on average, after 10.5 months, the correct diagnosis was made (p=0,0006). In the group of patients with Eisenmenger syndrome, there was a significant decrease in SpO2 both at rest and under exertion and averaged 86.5±9.7% at rest and 70±12.3% after exercise (p=0,0002). Conclusions. Patients with Eisenmenger syndrome compared with the IPAH group are characterized by a longer duration of symptoms until the diagnosis, and lower oxygen saturation. When estimating the parameters of pressure in the pulmonary artery (sPAP, dPAP, mPAP), according to the data of right heart catheterization and echo, significantly higher values of pressure in the pulmonary artery and lower values of SpO2 were detected.
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Gratsianskaya et al. (2019) conducted an observational in Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and Eisenmenger syndrome, and idiopathic pulmonary hypertension (IPAH) (n=40). PAH-CHD and Eisenmenger syndrome vs. Idiopathic pulmonary hypertension (IPAH) was evaluated on Time from onset of symptoms to final diagnosis (p=0.0006). Patients with PAH-CHD and Eisenmenger syndrome experienced a significantly longer time from symptom onset to diagnosis compared to patients with IPAH (3 years vs 10.5 months; p=0.0006).
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