Pulmonary Hypertension With Unilateral Pulmonary Vein Atresia.

The recently revised European Society of Cardiology/European Respiratory Society guidelines changed the diagnostic criteria for pulmonary hypertension from a mean pulmonary arterial pressure (PAP) of 25 mmHg or greater to more than 20 mmHg, emphasizing early diagnosis.1) Patients with cardiac malformations with shunts may develop pulmonary arterial hypertension (PAH) due to right ventricle (RV) volume/pressure overload due to the shunts. However, in patients who underwent cardiac surgery for congenital cardiac anomaly at birth, the state of the vascular structure and hemodynamic changes may vary, complicating the diagnosis and classification of pulmonary hypertension.