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Children with primary pulmonary hypertension (PHT) are a high-risk group who require assessment by cardiac catheterization under anaesthesia. Complications, including death, have occurred during anaesthesia in these patients, but the true risk has not been quantified.The clinical records of children with PHT undergoing general anaesthesia for pulmonary vascular resistance studies were reviewed retrospectively. Data collected included pre-catheter measures of severity of disease, details of clinical management, and complications occurring within 24 h of the start of anaesthesia.During the past 5 yr, 75 consecutive patients were catheterized and usable records were available in 70. The age range was 0.1–18 yr (mean 7.1). Four children required external cardiac massage [6% (95% confident limits 1–11%)] and one of these died. Of the four, two had an arrhythmia related to the mechanical effects of catheterization, one was hypotensive during anaesthesia and the other had fatal cardiac failure in recovery. All four had severe PHT as judged by echocardiographic estimation of tricuspid regurgitant jet velocity > 4 m s−1.Resuscitation or death occurred in 6% of cases. Any associated risk factors could not be determined because the number of complications was too small. Risks may be highest in children with severe idiopathic PHT and symptoms of chest pain, syncope, or dizziness.