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Kevin Pham, PGY-3 July 18 th, 2014. HPI Chief complaint: shortness of breath 74 yo man was “admitted to the hospital because of pulmonary HTN” and “worsened.

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Presentation on theme: "Kevin Pham, PGY-3 July 18 th, 2014. HPI Chief complaint: shortness of breath 74 yo man was “admitted to the hospital because of pulmonary HTN” and “worsened."— Presentation transcript:

1 Kevin Pham, PGY-3 July 18 th, 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, isosorbide dinitrate, 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 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, isosorbide dinitrate, 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 Enterobacter aerogenes 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

27

28

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.


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