2.1.1 Pulmonary vein stenosis (narrowing) may be congenital or may be acquired after surgery to correct other congenital cardiac anomalies. It is rare and often associated with other cardiac abnormalities. Untreated, it
2.1.1 Pulmonary vein stenosis (narrowing) may be congenital or may be acquired after surgery to correct other congenital cardiac anomalies. It is rare and often associated with other cardiac abnormalities. Untreated, it leads to severe lung damage.
2.1.2 There is currently no reliable alternative treatment.
2.2.1 Balloon angioplasty of pulmonary vein stenosis, sometimes combined with stenting, is a palliative treatment for children with a very poor prognosis, or is sometimes a temporary measure for children awaiting further interventions. The procedure involves inserting a catheter into the narrowed area under radiological guidance. A balloon is then inflated to relieve the narrowing. A stent may be inserted after dilatation to maintain patency.
2.3.1 The evidence was limited to four very small, poor-quality case series, the largest including only five patients. The two largest studies found no benefit from the procedure in any patients. Another study of three patients found an immediate reduction in pulmonary vein pressure in all the patients, as well as angiographic evidence of relief of stenosis in one patient. However, this patient died of infection within 36 hours of surgery. For more details, refer to the Sources of evidence section.
2.3.2 The Specialist Advisors considered that this procedure may have only short-term efficacy (if any at all), and that recurrence rates may be high. They also noted, however, that there was almost no role for surgery in this condition, and that even a partial result from this procedure may offer palliation in this group of patients.
2.4.1 Some of the main adverse events reported in the studies included: venous tear leading to mediastinal haemorrhage in 20% (1/5) of patients; haemoptysis in 20% (1/5) of patients; death caused by infection in 33% (1/3) of patients; and puncture of the distal vein in 33% (1/3) of patients. For more details, refer to the Sources of evidence section.
2.4.2 The Specialist Advisors considered the main potential adverse events to be death, rupture of myocardium, rupture of pulmonary vein, cerebral or other systemic embolism, arrhythmias and sepsis.