Browsing by Subject "Pulmonary arteriovenous malformation"

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    A case report of hepatopulmonary syndrome in hereditary hemorrhagic telangiectasia (HHT): Not all right-to-left shunting in HHT is due to pulmonary arteriovenous malformations
    (Wolters Kluwer, 2018-08) Krishnan, Sheila; Lahm, Tim; Medicine, School of Medicine
    RATIONALE: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by abnormal vessel growth that results in telangiectasias and arteriovenous malformations (AVMs) in the skin, mucosa, and viscera. Up to 30% of patients with HHT exhibit pulmonary AVMs (PAVMs), clinically manifesting as right-to-left shunting and hypoxemia. PATIENT CONCERNS: We report an unusual and novel case of a patient with HHT who lacked clinical sequelae of portal hypertension but presented to clinic with hypoxemia without dyspnea. DIAGNOSES: Diagnostic workup revealed noncardiac right-to-left shunting due to hepatopulmonary syndrome (HPS) from HHT-induced portal hypertension rather than PAVMs. The diagnosis was confirmed by the absence of PAVMs on chest computed tomography and evidence of elevated portal pressures as noted by the presence of small esophageal varices on upper endoscopy and histologic findings on liver biopsy. INTERVENTION: Due to the patient's mild symptoms, no further intervention was required. He was closely followed up in the outpatient setting for changes in symptoms and underwent annual screening for development of PAVMs. OUTCOMES: The patient continues to do well clinically. He has not experienced worsening hypoxemia or dyspnea and has not developed PAVMs. LESSONS: Given that management of hypoxemia in HPS drastically differs from that of hypoxemia due to PAVMs, this case demonstrates the importance of evaluating HHT patients for HPS if they exhibit impaired oxygenation and noncardiac right-to-leftshunting in the setting of hepatic arteriovenous shunting.
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    Management of coexisting patent foramen ovale and pulmonary arteriovenous malformation: a case report of sequential closure
    (Oxford University Press, 2025-01-29) Yagasaki, Hiroto; Suzuki, Takeki; Watanabe, Keitaro; Watanabe, Ryota; Noda, Toshiyuki; Medicine, School of Medicine
    Background: Concurrent patent foramen ovale (PFO) and pulmonary arteriovenous malformation (PAVM) are rare but can cause paradoxical embolism and platypnoea-orthodeoxia syndrome (POS). Case summary: A 72-year-old female with embolic stroke history developed positional dyspnoea. Evaluation revealed right-to-left shunting through PFO and PAVM in the right middle lobe. Orthodeoxia was confirmed by 6% SpO2 decrease upon position change. A staged approach was adopted: PFO closure with Amplatzer™ Occluder, followed by PAVM embolization 1 month later. Symptoms improved significantly post-procedure. No residual shunting or symptoms have been observed during the 2-year follow-up. Discussion: This case emphasizes thorough evaluation in patients with cryptogenic stroke and POS, especially when symptoms persist. It demonstrates the effectiveness of staged treatment for concurrent PFO and PAVM, highlighting the importance of individualized strategies and long-term follow-up.