Abstract Introduction Thromboangiitis obliterans (TAO), or Buerger disease, is a non-atherosclerotic vasculitis linked to tobacco use, occurring predominantly in males and typically affecting peripheral arteries. Pulmonary artery (PA) involvement is exceptionally rare, with few prior cases reported. We present a 38-year-old female with TAO and severe pre-capillary pulmonary hypertension (PH), PA dilation, severe tricuspid regurgitation (TR), and right-sided heart failure (RHF), suspected to be secondary to TAO. Case Presentation A 38-year-old female with TAO, chronic leg wounds, and active tobacco/cannabis use presented for worsening pain from a right leg ulcer. Imaging confirmed osteomyelitis; she underwent right above-knee amputation. Despite denying dyspnea or chest pain, she was tachycardic with marked edema. Electrocardiograms showed atrial arrhythmia and right ventricle (RV) hypertrophy. Chest computed tomography angiogram (CTA) excluded pulmonary embolism but revealed cardiomegaly, right atrium (RA)/RV enlargement, septal bowing, and a severely ectatic main PA (4.0 cm). Echocardiography confirmed severe PH (RV systolic pressure 80 mmHg), severe TR, and RA/RV dilatation with RV dysfunction. Given her high-risk PH (REVEAL 2.0 score ≥9) and no rheumatology follow-up, autoimmune and thrombophilia workup was initiated: erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) were elevated; antinuclear antibody (ANA), anti-Smith, anti-U1-ribonucleoprotein, anti-chromatin, and rheumatoid factor were positive; C4 complement was low. Thrombophilia panel showed low antithrombin III, low protein C, positive lupus anticoagulant, and elevated homocysteine. Antineutrophil cytoplasmic antibody (ANCA) testing was inconclusive. Management included metoprolol, apixaban, bumetanide, outpatient referrals, and smoking cessation counseling. Discussion No evidence of left heart disease, thromboembolism, or parenchymal lung pathology was found in our patient, raising suspicion that her PH and RHF were secondary to TAO. Although TAO typically affects small- and medium-size peripheral arteries, sporadic involvement of larger visceral arteries (e.g., aortoiliac, cerebral, coronary, renal, and mesenteric) has been reported, supporting its characterization as a generalized vascular disease. PA involvement remains exceedingly rare; our review of literature identified only one prior accessible case of TAO associated with unexplained PH. This severe presentation in a relatively young patient highlights the importance of maintaining a high index of suspicion for atypical systemic complications of TAO, including central cardiopulmonary involvement. Conclusions Pulmonary artery involvement in TAO is an exceptional and poorly documented phenomenon. Our case may represent such a manifestation, though histopathology is required to confirm etiology and exclude other vasculitides. The patient’s young age further emphasizes the need for early recognition of uncommon presentations in TAO. This abstract is funded by: None
DeBrota et al. (Fri,) studied this question.