Case 51 cont'd
Common causes of unilateral V/Q mismatched defects are as follows:
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Pulmonary embolism (less common because the patient is usually to unstable to undergo for VQ scan)
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Fibrosing mediastinitis
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Hilar malignant tumor (blocking pulmonary artery)
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Pulmonary vein stenosis
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Pulmonary fibrosis (after unilateral lung transplant)
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Compression of the pulmonary artery by thoracic artery dissection
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Fibrothorax
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Swayer James
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Congenital anomalies i.e. pulmonary arteries or veins stenosis
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Complication of Fontan Sx.
Final Diagnosis :
Congenital heart disease with Fontan anastomosis complicated with right pulmonary artery occlusion.
Discussion:
The patient was diagnosed with congenital tricuspid atresia with concordant ventriculo-arterial connection who underwent
Corrective surgeries including: Waterston anastomosis, Bidirectional Glenn and Fontan procedure. The surgery complicated due to failing Fontan with significant cyanosis and occluded RPA. The patient finally underwent heart transplant for congenital heart disease with single lung (L) anastomosis, the right lung received perfusion from systemic circulation through bronchial arteries.
References:
1. Bowman, A.W., Albers, B.K., & Jain, M.K. (2018). Acquired Whole-lung Mismatched Perfusion Defects on Pulmonary Ventilation/Perfusion Scintigraphy. Indian Journal of Nuclear Medicine, 33, 312 - 316.