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Mentions: She had moderate cyanosis, but was relatively well and thriving until 4 years of age. Subsequently, she developed progressively severe cyanosis and polycythemia, with a hematocrit of 60% and oxygen saturation of 65-70%. She had moderate clubbing with a harsh IV/VI systolic ejection murmur at the right upper sternal border. Cardiac catheterization at 4 years of age showed the above-described anatomy, with low pulmonary artery pressures (mean of 10 mmHg). It was decided to proceed with bilateral anastomoses of the superior venae cavae to the pulmonary arteries which also connected the inferior vena caval flow to the pulmonary circulation through the left azygos vein (Kawashima operation), thus leaving only the hepatic veins draining to the systemic circulation. The main pulmonary artery was disconnected from the right ventricle. The postoperative oxygen saturation was 94%. Over the next 2 years, there was a progressive drop in the arterial oxygen saturation, to 79%. A repeat cardiac catheterization was performed which showed well functioning bilateral cavopulmonary anastomoses, normal central pulmonary arteries, with a diffuse opacification of the lung parenchyma and rapid appearance of contrast in the pulmonary venous circulation, with opacification of large pulmonary veins at the same time with central pulmonary arteries, suggesting diffuse pulmonary arteriovenous communications (Fig 2). The pulmonary artery pressure was normal. She was reoperated, and the hepatic veins were anastomosed through a 22 mm Gore-tex tube graft to the right pulmonary artery. Immediately postoperatively the saturation increased to 85-90%, and over the next six months, it increased further to 97%, suggesting resolution of the pulmonary arteriovenous communications.
Diagnosis and management of pulmonary arteriovenous malformations
Bottom Line: It can also complicate palliative surgery such as the Glenn operation for complex congenital heart disease with single ventricle physiology.Complications of the disease itself and of various modes of treatment are relatively common, and patients require close surveillance for possible recurrence, or development of new fistulas.In cases related to the Glenn operation, redirection of hepatic venous flow or heart transplantation may cure the problem.
Affiliation: Division of Pediatric Cardiology and Pediatric Cardiac Surgery, Onassis Cardiac Surgery Center.
Pulmonary arteriovenous malformation is a rare anomaly that presents in several different ways. It can present as an isolated finding, or more often in the context of hereditary haemorrhagic telangiectasia. It can also complicate palliative surgery such as the Glenn operation for complex congenital heart disease with single ventricle physiology. Its management includes transcatheter embolization, which is the preferred mode of therapy, surgery (including resection of the affected lobe, segment, or the fistula itself), or rarely, medical therapy. Complications of the disease itself and of various modes of treatment are relatively common, and patients require close surveillance for possible recurrence, or development of new fistulas. In cases related to the Glenn operation, redirection of hepatic venous flow or heart transplantation may cure the problem.