Clinical trial NCT02260362: Observatory of Pulmonary Arterial Hypertension of Congenital Heart Disease
Official title: Observatory of Pulmonary Arterial Hypertension of Congenital Heart Disease
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Pulmonary Arterial Hypertension of Congenital Heart Disease
Lead sponsor: French Cardiology Society
Sponsor class: OTHER
First posted: 2014-10-09
Enrollment: 349
Sex: ALL
Minimum age (years): 1
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: All patients with Pulmonary Arterial Hypertension of Congenital Heart Disease supported by one of the 20 centers participating in the observatory during the period incusion and respecting al the selection criteria

Brief summary: 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


Eligibility criteria: 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

Primary outcomes:
measure: Incidence HTAP of congenital heart disease in France.
timeFrame: 3 years
Secondary outcomes:
Other outcomes:
Locations:
city: Paris
country: France
facility: Hopital Necker Enfants Malades
Study officials:
References:
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citation: van Loon RL, Roofthooft MT, Hillege HL, ten Harkel AD, van Osch-Gevers M, Delhaas T, Kapusta L, Strengers JL, Rammeloo L, Clur SA, Mulder BJ, Berger RM. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation. 2011 Oct 18;124(16):1755-64. doi: 10.1161/CIRCULATIONAHA.110.969584. Epub 2011 Sep 26.
citation: Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, van der Velde ET, Bresser P, Mulder BJ. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007 Aug 21;120(2):198-204. doi: 10.1016/j.ijcard.2006.09.017. Epub 2006 Dec 19.