APPROACH TO ACUTE HYPOXIC RESPIRATORY FAILURE IN A PATIENT WITH COPD AND UNDERLYING HISTORY OF HEREDITARY HEMORRHAGIC TELENGIECTASIA WITH PULOMARY AV MALFORMATIONS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Hereditary Hemorrhagic Telangiectasia is a multisystem disease which results in AV Malformations and telangiectasia. It has autosomal dominant inheritance prevalence of 1 5000 to 10,000. Patients with Pulmonary AVMs can present hypoxia, more post exercise sedentary position improvement on standing. presenting hypoxia history HHT should undergo thorough evaluation including radiological imaging echocardiography rule out recurrence sequelae such as pulmonary hypertension or arterial hypertension, respectively. CASE PRESENTATION: We case 66 y/o male Past medical AVM s/p repair presented complaints dyspnea cough sputum production. In ER patient was hypoxic SPO2 88% room air. Patient had coarse rhonchi, scattered wheeze telangiectasia the nose hands. Routine labs were normal. Legionella antigen, SARS COVID PCR negative. Chest x-ray revealed peri hilar basilar reticular opacities. CT chest contrast prior right lower lobe almost complete resolution signs severe COPD emphysema. Transthoracic echocardiogram ruled artery hypertension. treated for exacerbation albuterol/ ipratropium nebulization, steroids, oxygen support azithromycin. DISCUSSION: familial genetic mutations endoglin gene chromosome 9q33-q34 ALK-1 12q13.2 50% all patients develop malformations. Clinical finding includes respiratory failure. lead left shunt formation impairing effective gas exchange leading interestingly these participate high intensity physical activity despite being severely hypoxic, this been attributed hemodynamic compensatory responses. 3 This paradoxical emboli causing strokes brain abscess. Management trans catheter coil vaso-occlusion artery. Recanalization development growth untreated known, warranting long term follow up. itself life threating condition, its course be further complicated by chronic lung like COPD. These not explained shunting, it underlying conditions, our patient's case, CONCLUSIONS: While known cause COPD, worsening due output cardiac failure Therefore, crucial have detailed malformations new onset REFERENCE #1: Mary E. Meek, M.D., James C. D.O., Michael V. Beheshti, M.D. [Google Scholar, PubMed] #2: Sabba, M. Gallitelli, G. Pasculli, P. Supressa, F. Resta Tafaro. HHT: A rare broad spectrum clinical aspects [PubMed] #3: Dupius-Girod S., Cottin V, Shovlin The hereditary hemorrhagic Scholar] DISCLOSURES: No relevant relationships Khushdeep Chahal, source=Web Response priyanka kapoor,