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[Cyanosis for more than 4 years in a girl aged 4 years and 3 months]
Mao-Lan Wu1, Xiang-Rong Zheng, Yan-Tong Zhu
1Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China. xrzheng@csu.edu.cn.
Insights
A pediatric patient with congenital pulmonary arteriovenous fistula experienced cyanosis and shortness of breath. Interventional embolization successfully resolved symptoms, restoring normal oxygen levels and activity tolerance.
Area of Science:
- Cardiology
- Pulmonology
- Pediatric Medicine
Background:
- Congenital pulmonary arteriovenous fistula (PAVF) is a rare vascular anomaly.
- Early diagnosis and treatment are crucial for managing associated cyanosis and hypoxemia.
Observation:
- A 4-year-old girl presented with lifelong cyanosis, exertional dyspnea, and acrocyanosis.
- Physical examination revealed acropachy and toe deformity.
- Laboratory tests showed elevated hemoglobin and reduced arterial oxygen pressure.
Findings:
- CT and MRI revealed a large pulmonary arteriovenous malformation in the right lower lung.
- Diagnosis confirmed as congenital pulmonary arteriovenous fistula.
- Successful interventional embolization of the pulmonary arterial fistula was performed.
Implications:
- Interventional embolization is an effective treatment for pediatric congenital pulmonary arteriovenous fistula.
- Treatment significantly improves cyanosis, dyspnea, and overall patient well-being.
- Restoration of normal oxygen saturation and enhanced activity tolerance observed post-procedure.
Abstract:
A girl, aged 4 years and 3 months, presented with cyanosis of the lips shortly after birth. She then experienced shortness of breath after activity 1 year ago and acrocyanosis 3 months ago, with obvious acropachy and toe deformity. Laboratory examinations revealed an increase in hemoglobin (178 g/L) and a reduction in arterial partial pressure of oxygen (37.7 mm Hg). Plain and contrast-enhanced CT scans of the lungs showed a large area of dense shadow and multiple nodules with clear boundaries in the right lower lung, as well as thickening of the arteries and dilatation of the veins in the right lower lung. Magnetic resonance angiography of the pulmonary artery showed large arteriovenous malformation in the lung. The child was diagnosed with congenital pulmonary arteriovenous fistula and was given interventional embolization of the pulmonary arterial fistula. The child was followed up at 3 months after surgery. The symptoms of shortness of breath and cyanosis disappeared, and activity tolerance, heart rate, hemoglobin, red blood cell count, and transcutaneous oxygen saturation all returned to normal.
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