Naslov
Congenital pulmonary arteriovenous fistula in Childhood

Autori
Batinica, Stipe ; Luetić, Tomislav ; Marinović, Branko ; Popović, Ljiljana ; Župančić, Božidar ; Bradić, Ivan

Izvornik
Acta medica Croatica. Supplement (1331-1638) 51 (1997); 86-86

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, stručni

Ključne riječi
pulmonary arteriovenous fistula; children

Sažetak
Congenital pulmonary arteriovenous fistula is a rare malformation in which pulmonary arteries and veins communicate, shunting the pulmonary and systemic circulations. Fistulas may be relatively well circumscribed with dilated afferent and efferent vessels or they may be diffuse racemose malformations involving an entire lobe or a major portion of a lobe. The clinical manifestation depend on the magnitude of the right to left shunt. When the shunted blood exceeds 20-30% of the cardiac output, cyanosis is usually observed. Cyanosis and dyspnea with bronchopulmonary symtoms are common clinical features in children. The hypoxia leads to polycytaemia and digital clubbing. Complications occur in children in more than 1/3 of cases, with a lethality of 40%. It is impossible to predict which patients will develop serious complications, so all cases, even asymptomatic, should be considered severe.Diagnosis can be established by physical examination and chest radiography. Pulmonary angiography, contrast echocardiography and radionuclide perfusion on lung scintigraphy can be used as screening methods. Cardiac catheterization helps in differentiating it from cardiac lesions. Once the diagnosis has been established, surgical treatment is recommended, even in asymptomatic paatients. Postoperative morbidity and mortality are very low. Conservative therapy should be reserved for inoperable cases. From 1980 until april 1997, five patients with congenital pulmonary arteriovenous fistula were seen at the Dept. of pediatric surgery, University Hospital Centre, School of medicine, Zagreb. Their age ranged from 1, 3 do 12, 5 years ( 1 boy and 4 girls). All of them had symptoms of blu child with no signs of heart failure. In one of girls the fistula wqas associated with hereditary hemorrhagic teleangiectasia ( Rendu-Osler-Weber disease). After surgical procedure, four patienst had no postoperative complications and they are well. The last girl developed symptoms of cerebral embolism with consecutive hemiplegia, 32 hours after the oeration. She in recovering well All patients became symptom-free with disappearance of cyanosis.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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