title: Severe pulmonary arterial hypertension in an adult patient with total anomalous pulmonary venous connection operated in infancy.
abstract: The goal of total anomalous pulmonary venous connection repair is to obtain an unobstructed communication between the pulmonary veins and the left atrium and removing intracardiac shunting. However, pulmonary venous obstruction orstenosis may be seen in 5-10% of patients, is usually evident in the first 6 months following surgery and may lead to pulmonary congestion, pulmonary arterial hypertension, and late mortality. In such cases, early intervention may be indicated before irreversible secondary changes occur. We present the case and the therapeutic approach of an adolescent patient with total anomalous pulmonary venous drainage to the superior vena cava operated in infancy who developed pulmonary venous obstruction and secondary severe pulmonary arterial hypertension.
authors.forename: Efrén
authors.initials: E
authors.lastname: Martinez-Quintana
authors.forename: Fayna
authors.initials: F
authors.lastname: Rodríguez-González
mesh_terms: D000293:Adolescent; D000714:Anastomosis, Surgical / Q000009:adverse effects; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004359:Drug Therapy, Combination; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D011666:Pulmonary Valve Stenosis / Q000150:complications*; D011667:Pulmonary Veins / Q000002:abnormalities / Q000601:surgery; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate / Q000627:therapeutic use*; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome; D014683:Vena Cava, Superior / Q000601:surgery
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000068677:Sildenafil Citrate; D000077300:Bosentan