Atrial Septal Stent Fenestration in the Management of Idiopathic Pulmonary Hypertension

Objectives: Assess the clinical impact of atrial septal stent fenestration in the management of patients with severe idiopathic pulmonary hypertension. Methods: Three patients (median age 12 years; range 11–13 years; weight 34 kg; range 28–41 kg) with severe idiopathic pulmonary hypertension accompanied by exertional syncope were catheterised with the creation of a fenestration between the left and right atria. Clinical symptoms, 6-min walk test (6MW) with resting and post exercise saturations and echocardiographic data were reviewed pre and post fenestration creation. Results: Two patients were stented and one underwent static balloon dilation; due to recurrent bacterial infections a stent was not implanted. Follow-up was complete at a median of 126 days (range 109–362 days). There were no complications. All patients had resolution of exertional syncope; 2/3 were asymptomatic. Mean 6MW distance increased from 447m to 538m (p value = NS); resting saturations fell from 96% to 94% (p value = NS) and post exercise saturations fell from 84% to 76% as expected (p value = NS). No significant change in levels of pulmonary hypertension estimated by tricuspid regurgitant gradient (87 mmHg to 76 mmHg) or left ventricular fractional shortening (33–37%). Conclusions: Atrial septal fenestration was well tolerated in our patients. It appeared to prevent collapse in severe idiopathic pulmonary hypertension and improved exercise tolerance as measured by 6MW with the expected fall in exercise saturations. Stent fenestration may allow longer term patency and titratability of the fenestration. Performed earlier in the course of the illness it may be better tolerated, facilitating a gradual tolerance of desaturation.