Monika Kaldarárová, Tatiana Valkovičová, Iveta Šimková
Pulmonary hypertension may occur in various types of congenital heart diseases. Pulmonary arterial hypertension
(PAH) associated with congenital heart diseases is a specific form of pulmonary hypertension because it represents the
only preventable form of PAH. The size and location of the defect is important for the development of PAH. According
to the severity of PAH, or according to clinical and hemodynamic evaluation, it is classified as Eisenmenger syndrome,
representing the most severe form, PAH with left-to-right shunt, PAH associated with small defect and PAH after defect
correction. According to defect location pre-tricuspid and post-tricuspid defects can be distinguished. It is crucial to
consider the defect operability. In case of severe PAH without any possibility for surgical or interventional correction,
there remains a possibility for the so-called specific PAH therapy. The management of associated cyanosis related multisystemic
complications is also important. The final option is a lung (and heart) transplantation.