Academy Case Presentation

1. Academy Case Presentation Kevin Pham, PGY-3 July 18th, 2014

2. HPI • Chief complaint: shortness of breath • 74 yo man was “admitted to the hospital because of pulmonary HTN” and “worsened right sided heart failure.”

3. HPI • Seven years prior to admission, patient was admitted for a dog bite. • Inspiratory wheezing, digital clubbing, cyanosis, and increased anteriorposterior diameter of thorax on exam. • Chest X-ray shows increased marking in bilateral bases and decreases marking in upper fields. • Patient was asymptomatic at this time

4. HPI • Three years prior to admission, patient was admitted for increased dyspnea. • ABG: pH 7.44, PaCO2 14, PaO2 49 on room air • PFTs: FVC 73% predicted, FEV1 66% predicted, FEV1/FVC 90%, DLCO 9% predicted • Patient did not improve with bronchodilators and was discharged on home oxygen

5. HPI • Two years prior to admission, patient was admitted for chest discomfort and dyspnea. • Patient was discharge on Lasix, isosorbidedinitrate, and oxygen

6. HPI • Fifteen months prior to admission, patient was admitted for dyspnea again. • Increase jugular venous pressure, increased anteroposterior thoracic diameter, hyperresonance, diminished breath sound, inspiratory crackles at right lung base, liver 3 cm below right coastal margin, acral cyanosis, digital clubbing, 3+ pitting edema bilateral legs. • ABG: pH 7.54, PaCO2 22, PaO2 43

7. Pulmonary function testing 60% predicted 73% predicted 105% predicted 175% predicted 117% predicted 13% predicted

8. Chest X-ray

9. Chest X-ray • Slight cardiac enlargement • Hyperexpanded • Decreased vascular markings in left upper lobe • Fine linear opacities in bilateral bases • Small left lung pleural plaque • Central pulmonary arteries are dilated

10. HPI • Patient was discharged with beclomethasone inhaler, albuterol inhaler, Lasix, nitroglycerin, isosorbidedinitrate, and oxygen

11. HPI • Three weeks later, patient stopped using inhalers and was not using oxygen as prescribed. • Adherence advised

12. HPI • Eight months prior to admission, cardiac echocardiography was done and showed: • Right ventricular hypertrophy • There was no intracardiac shunt

13. HPI • Two months prior to admission, a radionuclide pulmonary scan performed.

14. V/Q scan • Multiple areas of decreased perfusion which filled in with equilibrium. • Perfusion defects matched with ventilation defects

15. HPI • Patient worked in a naval shipyard in welding for 10 years. • Patient worked in construction and demolition for 37 years. • He also worked in vicinity of sandblasting and fiberglass before. • He smoked tobacco 1-2 packs daily for 45 years. He quit 5 years prior to admission. • No history of tuberculosis or prior exposure. • No family history of pulmonary disease

16. Review of Systems • Chronic productive cough with small amount of sputum • Patient denies fever, weight loss, hemoptysis, allergic symptoms, or chest pain.

17. Physical Examination • Vitals: T 36.1 C, P 90, BP 125/90, RR 28 (no pulse ox reported) • General: thin, cyanotic in moderate pulmonary distress. • HEENT: facial flushing, multiple telangiecstases. JVD 10 cm with hepatojugular reflex • Pulmonary: faint breath sound, clear bilaterally, prolonged expiration. • Cardiovascular: regular rhythms, pronounced P2, grade 2 holosystolic murmur over left lower left sternal border increased with inspiration. • Abdomen: liver palpable 3 cm below right costal margin. • Extremities: 3+ pitting edema with increased skin pigmentation and digital clubbing bilaterally.

18. Laboratory • HCT 48.3 CBC otherwise is normal • CO2 21, BUN 25, Cr not reported, BMP otherwise is normal • Total bilirubin 1.6, direct bilirubin 1.1, LFTs otherwise normal • Normal coagulation panel. • Normal UA. • ABG: 7.34/44/413 on 100% oxygen

19. EKG • Pertinent for axis 120 degree, evidence of right atrial enlargement and right ventricular hypertrophy.

20. Pulmonary Arterial Line • Right atrial mean pressure 20 mm Hg • Right ventricular pressure 100/25 mm Hg • Pulmonary arterial pressure 95/50 mm Hg • Wedge pressure 10 mm Hg

21. Hospital Course • Hydralazine, captopril, and nitroprusside given with no effects on pulmonary pressure. • Nifedipine given with slight improvement in pulmonary pressure. • Warfarin started

22. Pulmonary Function Testing

23. Hospital Day 9 • Worsening dyspnea with diffused wheezing. • ABG: 7.50/28/32 on 100% oxygen • Intubated. • Solumedrol, thephylline, terbutaline, metaproterenol. • Chest X-ray with airspace conslidation at right lung base. • Ampicillin and gentamicin started. • Hemoptysis developed. • Heparin drip started in place of Coumadin. • Lower extremities ultrasound negative for DVTs.

24. Hospital Day 18 • Heparin drip stopped. • IVC filter placed. • Sputum culture positive for Enterobacteraerogenes and Pseudomonas maltiphilia. • CXR with partial clearance of airspace consolidation. • Weaning failed multiple times. • Creatinine and BUN progressively increased. • Repeat CXR showed no change. • Patient expired on Hospital Day 28

25. Case Discussion

26. Autopsy

29. Pathology • Upper lobes with marked panacinar emphysema • Apical segments completed comprised of small bullae • Middle lobe, lingula, and lower lobes with interstitial fibrosis. Fibrosis was fine reticulated with no honeycombing. • Large number of asbestos bodies present. • Pathology consistent with chronic bronchitis.

30. Reference • Scully, RE, et al. Case 32-1986. New England Journal of Medicine. 315;17:437-449.