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Pulmonary arteriovenous fistulas. Case presentations and clinical recognition
Insights
Pulmonary arteriovenous fistulas can cause cyanosis and dyspnea in young patients. Pulmonary angiography remains essential for diagnosing these vascular malformations, especially when cardiac disease is absent.
Area of Science:
- Cardiology
- Pulmonary Medicine
- Vascular Surgery
Background:
- Congenital and traumatic pulmonary arteriovenous fistulas (PAVF) are rare vascular malformations.
- Early diagnosis and management are crucial for preventing complications.
Observation:
- Six young patients (5-25 years) presented with congenital PAVF, exhibiting cyanosis, digital clubbing, and exertional dyspnea.
- Two patients also had telangiectasia; one patient had a traumatic intrapulmonary arteriovenous fistula.
- All patients underwent cardiac catheterization and pulmonary angiography, with no evidence of pulmonary hypertension.
Findings:
- Angiography revealed diffuse PAVF in both lungs in four patients and localized fistulas in one lung segment in two.
- Radionuclide angiography and perfusion scintigraphy detected significant right-to-left shunting in three patients.
- Pulmonary angiography is definitive for characterizing PAVF, while noninvasive methods offer supportive data.
Implications:
- Pulmonary arteriovenous fistulas should be considered in the differential diagnosis of cyanotic patients without cardiac disease.
- Accurate diagnosis via pulmonary angiography is vital for appropriate treatment planning.
- Understanding PAVF presentation aids in timely intervention and improved patient outcomes.
Abstract:
This report describes characteristic features and clinical presentation of six young patients (ranging in age from five to 25 years) with various congenital forms of pulmonary arteriovenous fistulas as well as one 21-year old patient with traumatic intrapulmonary arteriovenous fistula. The former six patients were cyanotic, had clubbing of the digits and exertional dyspnea; two, additionally, had telangiectasia. All patients underwent cardiac catheterization and pulmonary angiography. None had pulmonary hypertension. Angiographically, four had diffuse arteriovenous fistulas in both lungs (Figures 1 and 3 to 5) and two had arteriovenous fistulas localized in one pulmonary segment. Four patients were additionally studied with radionuclide angiography and pulmonary perfusion scintigraphy. In three of these, substantial right-to-left shunt in the region of the lungs was detected; the diagnosis could not be established in only one of the latter with a small traumatic intrapulmonary arteriovenous shunt of less than 10%. While noninvasive methods provide adjunctive diagnostic support, pulmonary angiography is still required to assess, with certainty, the nature and localization of the lesion. In cyanotic patients with no evidence of cardiac disease, differential diagnostic consideration should be given to pulmonary arteriovenous fistulas.