Observatory of Pulmonary Arterial Hypertension of Congenital Heart Disease

Observatory of Pulmonary Arterial Hypertension of Congenital Heart Disease

The complex congenital cardiac malformations are a significant number of different diseases, each having specific natural histories. The interface with pulmonary arterial hypertension (HTAP) is high since the physiology of many of these disorders comprises alterations in pulmonary vasculature.

This observatory is a cohort of 400 patients enrolled in 3 years, older than one month, having been informed and have agreed to participate in the study and with congenital heart disease other than patent foramen ovale as well as a diagnosis of pulmonary hypertension confirmed by cardiac catheterization.

The main objectives of this report are to know

• Incidence of congenital heart disease in HTAP France.
• Describe the natural history of HTAP in a large population of patients Congenital heart disease in France
• The characteristics of HTAP congenital heart disease
• Having a cohort study

Inclusion Criteria:

• The patient has a congenital heart disease other than patent foramen ovale
• The diagnosis of pulmonary hypertension was confirmed by cardiac catheterization. Only patients with Eisenmenger syndrome can be included without catheterization.
• The catheterization was done after 1 January 2009
• A mean pulmonary artery pressure > 25 mm Hg
• Pulmonary vascular resistances > 3 piece Wood m2
• Pulmonary capillary pressure available
• Consent for inclusion in the study must be signed by parents or legal guardians for minors, by the patient for adults.
• The patient he had a surgical procedure or interventional catheterization cardiac catheterization between his diagnosis and inclusion in the observatory? If yes, it can only be included if a new catheterization confirmed the persistence of HTAP at least 6 months after the procedure.
• Patient follow-up (at least once a year) in the center for its HTAP associated with congenital heart disease its