A 58 year-old male with an episode of syncope and an 8 month history of progressive dyspnea was brought to The Johns Hopkins Hospital Emergency Department by his family. The history is obtained from family.

The patient was a 58 year-old Caucasian male who had not been followed by a medical doctor for over 30 years. He was brought to the emergency room by his family following an episode of loss of consciousness. For approximately 6 months prior to admission he had complained of increasing shortness of breath, dyspnea on exertion, and cough. The cough was occasionally productive of non- purulent pink frothy phlegm. He denied fevers, chills, or night sweats. Over the past 6 months his family reported that he became progressively more immobile due to these symptoms. They think he had lost approximately 40 pounds due to loss of appetite. However, he also complained of progressive lower extremity swelling, abdominal swelling over this period of time. He had been sleeping in a chair for 2-3 months. The patient refused to seek medical attention despite his family's urging.

His syncopal episode occurred while he was walking to the kitchen and was preceded by some lightheadedness. His family reported no seizure activity. He was unconscious for less than a minute and was alert immediately afterwards.

No prior medical history

No surgical history

No medications

42-pack-years of tobacco use. He only drank white Zinfandel wine socially and never used illicit drugs. He was sickly as a child and was exempted from military duty. He was a retired accountant and did not participate in active sports other than speed chess. He was never married and lived alone until this past year when he moved in with a sister and her husband due to failing health.

There is no history of cardiac, pulmonary, hematologic, autoimmune or neurologic diseases.

NKDA

General: 52 kg, cachectic, alert in mild respiratory distress supine
Vital Signs: Blood pressure: 103/84; HR 85 irregular, RR 26, Temp 370 C
HEENT: Temporal wasting
Neck: JVP >10 cm, no carotid bruits
Cardiac: irreg/irreg rhythm, PMI laterally displaced, S3 gallop, III/VI holosystolic ejection murmur, pulses thready
Chest: Rales at right and left bases
Abdomen: Abdomen slightly distended with possible ascites. Bowel sounds present, no abdominal tenderness
GI: External hemorrhoids, stool heme negative
Skin: Lower extremities with chronic venous stasis changes and 2-3+ edema
Neuro: Decreased muscle strength, bilateral lower extremities, alert but somnolent.

Na 139, Cl 97, K 4.1, BUN 66, Cr 1.1, Glu 101, Ca 8.3,
pH 7.50, pCO2 35, pO2 116 (nasal O2),
Lactic acid 2.7, Total Bili 4.2, AST 99, ALT 137, Alk Phos 113
WBC 9710, Hct 42%, Hgb 13.8, MCV 83.7, Plt 121,000
Cardiac enzymes normal, PTT 32.3, PT 13.7
Cholesterol 133, Triglycerides 92, HDL 20, LDL 95

CXR: Bilateral pleural effusions with vascular prominence (Image 1)
Electrocardiogram: Atrial fibrillation followed by sinus rhythm with nonspecific ST- and T-wave changes
Chest CT (admission): Moderate bilateral pleural effusions, rounded interlobar effusion in right minor fissure, patchy ground glass infiltrates bilaterally probably representing mild edema, heart moderately enlarged with prominence of left ventricle and left atrium, moderate atherosclerotic changes in thoracic aorta (Images 2 and 3).
Abdominal CT: Gallstones and calcified aorta
Head CT: Unremarkable
Echocardiogram (Day 2, while on vasopressors): The left ventricle is mildly dilated. Apical echoes consistent with trabeculae are noted. Cannot rule out associated thrombi. There is borderline concentric left ventricular hypertrophy. Left ventricular systolic function is severely reduced. EF 20- 25%. The transmitral spectral Doppler flow pattern is suggestive of restrictive physiology. The transmitral spectral Doppler flow pattern is abnormal for age. There is severe global hypokinesis of the left ventricle. The right ventricle is mild to moderately dilated. The right ventricular systolic function is moderate to severely reduced. The left atrium is moderately dilated. The right atrium is mildly dilated. The reduced mitral leaflet separation suggests decreased flow through the mitral valve and poor cardiac output. There is moderate to severe mitral regurgitation. RVSP 37 mm Hg. Doppler findings suggest mild pulmonary hypertension. The aortic valve is trileaflet. The aortic valve opens well. There is discrete nodular thickening of the non- coronary cusp. No hemodynamically significant valvular aortic stenosis. Trace aortic regurgitation. The aortic root is normal size. Trace pericardial effusion.

While in the emergency department, he developed atrial fibrillation with a rapid ventricular response over 150/min. Oxygen saturation decreased, and he required intubation and mechanical ventilation. He was admitted to the coronary care unit. There was no evidence of acute myocardial infarction. He received diuresis, low dose ACE inhibitors, aspirin. Cardioversion briefly restored sinus rhythm. He was started on amiodarone because of recurrent atrial fibrillation. Due to progressive hypotension and shock, he was started on vasopressors. Blood, sputum, and urine cultures were negative. Despite these efforts, he continued to experience severe hypotension and worsening organ failure. On day 3 of admission, his family requested withdrawal of intensive care and he expired. With permission of the family, a complete autopsy was performed.

What was the cause(s) of the patients' 6 months of cardiopulmonary symptoms?
What was the most likely cause of death?