Evaluation of Latent Pulmonary Arterial Hypertension in Congenital Shunt Lesions
Evaluation of Latent Pulmonary Arterial Hypertension in Congenital Shunt Lesions
Prospective, monocentric study for the evaluation of latent pulmonary arterial hypertension
 in patients with congenital shunt lesions lost to follow-up. Lost to follow-up is defined as
 latest clinical control ≥ 5 years.
Pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD) usually
 develops secondary to chronic volume overload of the pulmonary circulation following left to
 right shunt. This overload leads to elevated pulmonary artery pressure (PAP) and later to
 increased pulmonary vascular resistance (PVR). This causes pressure overload in the right
 heart, and thereby right ventricular (RV) and right atrial (RA) dysfunction, which may
 implicate considerable morbidity and even mortality. Since PAH nowadays is mostly detected
 when symptoms occur and PAP are elevated, the disease already evolved to an advanced
 (partially irreversible) stage and treatment is often initiated too late.

 Dismissal from follow-up after a surgical correction of simple CHD was customized in the
 seventies and eighties. There is no literature available that learns us whether these
 patients really need follow-up or not. A substantial number must have insidiously developed
 PAH or mild pulmonary vascular disease (PVD) and still are prone to develop PAH later in
 life. It is relevant to recall these patients dismissed from follow-up in the past, because
 they might carry a lot of useful information on the natural history of PAH development. Focus
 will lie mainly on patients with simple shunt lesions, as atrial septal defect (ASD) and
 ventricular septal defect (VSD).
Inclusion Criteria:

 -  Previous repair for secundum ASD, VSD

 Exclusion Criteria:

 -  Other congenital heart disease

 -  Chronic lung disease or total lung capacity < 80% of predicted value

 -  History of pulmonary embolism