A 59-year-old African American female with a history of pulmonary tuberculosis treated twenty-one years ago, active tobacco use, and untreated hepatitis C presented with three days of coughing up frank red blood. This was preceded by a month of viral-like upper respiratory symptoms, chills, unintentional weight loss and night sweats. She denied productive cough, lower extremity swelling, shortness of breath or chest pain. She was an active smoker of 0.25 packs per day for forty-five years (11.25 pack year smoking history) with additional alcohol use estimating four beers per day since she was a teenager. She denied any illicit drug use.
Upon presentation she was in no acute distress with normal mental status. Vitals on admission revealed a temperature of 98.2 F, heart Rate of 89 bpm, blood pressure of 144/80 mmHg, respiratory rate of 16 while saturating 96% breathing ambient air. There was a prominent S1, split S2 with prominent P2, and a decrescendo-crescendo diastolic murmur loudest at the apex. Pulmonary exam revealed bibasilar crackles. Her abdomen was soft, non-tender and without organomegaly. There was no cyanosis or clubbing of the extremities.
Complete blood count revealed: WBC of 6.2, Hb of 12.9, Hct of 40.1, PLT of 318. PT was 11.9 seconds, INR was 1.1. Hepatic panel was normal with an AST of 27 U/L, ALT of 23 U/L, Alk phos of 60 U/L & Total Bilirubin of 0.9 mg/dL.
A chest x-ray (CXR) (Fig. 1) revealed fibronodular changes in both apices without infiltrates or effusions. A chest computer tomography angiogram (CTA) (Fig. 2) revealed extensive ground glass opacities, bilateral calcified pulmonary nodules most pronounced in the right upper lobe, and a dilated left atrium. No pulmonary embolism or arteriovenous malformations (AVM) were seen.
A transthoracic echocardiogram (TTE) revealed a left ventricular ejection fraction of 35-45%, mild left ventricular hypertrophy, and severe mitral valve stenosis with moderate mitral valve regurgitation.
Sputum AFB smear, blood cultures, HIV, ANA, ANCA and anti-GBM antibodies were obtained and all negative. Rheumatoid factor was elevated. Bronchoscopy (Fig. 3) revealed engorged veins in the trachea and bilateral proximal bronchial trees.
Results of the clinical work-up made infection, malignancy, TB, vasculitis or AVM unlikely. The dilated left atrium and severe mitral stenosis seen on TTE was confirmed by transesophageal echocardiogram (TEE), which showed a mean gradient of 12 mmHg and Wilkins score of 11 (see Fig. 4, Fig. 5, Fig. 6). Left heart catheterization was performed and showed no evidence of coronary artery disease.
Given these findings, the patient's massive hemoptysis was attributed to severe mitral valve stenosis in the setting of rheumatic heart disease. Given her Wilkins score of 11, she was not a candidate for balloon valvuloplasty, and ultimately underwent successful mitral valve replacement with a pericardial bioprosthetic valve and resection of anterior and posterior papillary muscles.