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A case of diffuse pulmonary arteriovenous fistula
K Hirata1, H Yamagishi, K Akioka
1First Department of Internal Medicine, Osaka City University Medical School, Osaka, Japan.
Japanese Circulation Journal
|July 16, 1999
Summary
This case study reports the first adult with microscopic pulmonary arteriovenous fistulas causing persistent cyanosis after congenital heart defect surgery. Heart-lung transplantation may be the only effective treatment for this rare condition.
Area of Science:
- Cardiology
- Pulmonology
- Medical Imaging
Background:
- A 30-year-old woman presented with persistent dyspnea and cyanosis post-surgery for atrial septal defect and partial anomalous pulmonary venous drainage.
- Congenital heart anomalies can lead to complex circulatory issues and persistent symptoms even after corrective procedures.
Observation:
- Diagnostic angiography revealed a rare double inferior vena cava (IVC) anomaly.
- The double IVC drained predominantly into the superior vena cava via enlarged azygos and hemiazygos veins.
- Pulmonary function tests showed significant oxygen desaturation and lung scintigraphy indicated widespread perfusion defects.
Findings:
- The findings suggested diffuse, microscopic pulmonary arteriovenous fistulas (PAVF) as the cause of the patient's ongoing cyanosis.
- This represents the first reported adult case of diffuse microscopic PAVF.
- Normal pulmonary arterial pressure contrasted with significant pulmonary venous oxygen desaturation.
Implications:
- The complex venous anomaly and diffuse PAVF make surgical correction challenging.
- Resection of PAVF or corrective surgery for the venous anomaly were not indicated.
- Heart-lung transplantation is considered the potential sole therapeutic option for symptom relief.