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<p>Pulmonary arteriovenous malformations (PAVMs) are rare vascular anomalies characterized by direct communications between pulmonary arteries and veins, bypassing the capillary system. While often asymptomatic, PAVMs can lead to significant clinical manifestations, including hypoxemia, cyanosis, and paradoxical embolism. Their association with hereditary hemorrhagic telangiectasia (HHT) is well-documented. A 52-year-old female presented with a 7-year history of intermittent chest pain, cough, and progressive dyspnea, worsening over the past 3&#xa0;months. Examination revealed central cyanosis, grade 4 digital clubbing, mucocutaneous telangiectasia, and severe hypoxemia (SpO₂ 81% on room air) refractory to high-flow oxygen therapy. Chest imaging identified a rounded opacity in the right lower lung zone, and HRCT with contrast demonstrated a large pulmonary arteriovenous malformation (PAVM) in the posterior basal segment of the right lower lobe, with an additional smaller lesion in the lingular segment. Contrast-enhanced echocardiography confirmed a right-to-left extracardiac shunt. Secondary erythrocytosis was noted without other systemic abnormalities. There was no family history suggestive of hereditary hemorrhagic telangiectasia (HHT), and genetic testing was declined. The patient underwent successful right lower lobectomy, resulting in complete resolution of hypoxemia. Histopathology confirmed PAVM. At 1-year follow-up, the patient remained asymptomatic with normal oxygenation and no recurrence. Pulmonary arteriovenous malformations are a rare but critical cause of refractory hypoxemia. This case emphasizes the importance of early recognition, advanced imaging, and a multidisciplinary approach to management. While embolization is the standard therapy, surgical resection remains essential for large or complex lesions. Timely diagnosis and definitive treatment are key to preventing serious complications, with long-term follow-up crucial for sustained clinical outcomes.</p>

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Refractory hypoxemia due to a large pulmonary arteriovenous malformation: a case report

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年7月15日

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  • Rohan Chauhan,
  • Arshnoor Kaur Chug,
  • Kushal Sood,
  • Neeru Bansal,
  • Muhammad Umar,
  • Hafiza Tooba Siddiqui

摘要

Pulmonary arteriovenous malformations (PAVMs) are rare vascular anomalies characterized by direct communications between pulmonary arteries and veins, bypassing the capillary system. While often asymptomatic, PAVMs can lead to significant clinical manifestations, including hypoxemia, cyanosis, and paradoxical embolism. Their association with hereditary hemorrhagic telangiectasia (HHT) is well-documented. A 52-year-old female presented with a 7-year history of intermittent chest pain, cough, and progressive dyspnea, worsening over the past 3 months. Examination revealed central cyanosis, grade 4 digital clubbing, mucocutaneous telangiectasia, and severe hypoxemia (SpO₂ 81% on room air) refractory to high-flow oxygen therapy. Chest imaging identified a rounded opacity in the right lower lung zone, and HRCT with contrast demonstrated a large pulmonary arteriovenous malformation (PAVM) in the posterior basal segment of the right lower lobe, with an additional smaller lesion in the lingular segment. Contrast-enhanced echocardiography confirmed a right-to-left extracardiac shunt. Secondary erythrocytosis was noted without other systemic abnormalities. There was no family history suggestive of hereditary hemorrhagic telangiectasia (HHT), and genetic testing was declined. The patient underwent successful right lower lobectomy, resulting in complete resolution of hypoxemia. Histopathology confirmed PAVM. At 1-year follow-up, the patient remained asymptomatic with normal oxygenation and no recurrence. Pulmonary arteriovenous malformations are a rare but critical cause of refractory hypoxemia. This case emphasizes the importance of early recognition, advanced imaging, and a multidisciplinary approach to management. While embolization is the standard therapy, surgical resection remains essential for large or complex lesions. Timely diagnosis and definitive treatment are key to preventing serious complications, with long-term follow-up crucial for sustained clinical outcomes.