Evaluation of the Pulmonary Vascular Reactivity Test in Patients With Pulmonary Arterial Hypertension and a Cardiac Shunt

Inclusion Criteria: * Patient child or adult, (regardless of age and weight) , which presents with heart disease with a shunt, * Patient who requires, during current care, a hemodynamic exploration by catheterization to assess pulmonary vascular resistance (due to doubt on the operability of the patient on the usual clinical and echo cardiographic data) . * Informed consent signed by the patient or at least one holder of parental authority, and the investigator * Patient affiliated or benefiting from a social security scheme Exclusion Criteria: * Patient with a specific treatment for PAH (the prostacyclin derivatives, antagonists of endothelium receptors and inhibitors of phosphodiesterase 5). * Patient participating in another research with exclusion period * Known allergy to acetylcholine or adenosine * Contraindication to the use of KRENOSIN (adenosine) * atrioventricular block second or third degree, with the exception of patients with cardiac pacemaker. * dysfunction sinoatrial (the headset disease) except patients with a pacemaker * chronic obstructive pulmonary disease with bronchospasm (eg bronchial asthma) * long QT Syndrome * severe arterial hypotension * known adenosine hypersensitivity * Pregnant or patient of childbearing potential not using an effective contraception