TY - JOUR T1 - Adult congenital heart disease and pulmonary hypertension: management of a complex case JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 362 LP - 364 DO - 10.1183/09059180.00004912 VL - 21 IS - 126 AU - Curt J. Daniels Y1 - 2012/12/01 UR - http://err.ersjournals.com/content/21/126/362.abstract N2 - To the Editor:Patients with congenital heart disease (CHD) are at increased risk of developing pulmonary arterial hypertension (PAH). These patients have a particularly poor prognosis if untreated when compared with CHD patients without PAH [1]. This case study demonstrates how a complete work-up and continuous follow-up in expert centres is required to ensure that PAH-CHD patients are managed appropriately.A female patient initially presented at a local community hospital at 3 yrs of age when she had failed to thrive. Echocardiography showed the presence of a moderately sized ventricular septal defect (VSD) and pulmonary stenosis. She underwent surgery for pulmonary stenosis with right ventricle outflow tract (RVOT) augmentation and valvectomy. However, the VSD was left open for reasons that were not specified in the patient's notes. Following this procedure, the patient moved out of the area and was lost to follow-up.After 16 yrs, in 2006 and at the age of 19 yrs, she presented to a local cardiologist to enquire whether she could become pregnant. On assessment, she was found to have mild dyspnoea in World Health Organization functional class II/III, oxygen saturation of 91% on room air, loud P2 heart sounds, II/VI holosystolic murmur at the left sternal border, normal jugular venous pressure and no evidence of oedema. Transthoracic echocardiography (TTE) showed a mildly enlarged right ventricle with normal systolic function, a moderately sized malaligned VSD with left-to-right (systemic-to-pulmonary) shunting, and normal left ventricle size and systolic function. Based on these findings, the patient was referred for immediate VSD closure. At this time, pregnancy was not ruled out. The patient was subsequently referred to an adult CHD/pulmonary hypertension (PH) expert centre for further evaluation.At the adult CHD/PH evaluation, the patient complained of dyspnoea with hill walking, climbing stairs or when carrying heavy loads, … ER -