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1
Question:

An 86-year-old woman comes to the office due to difficulty breathing with exertion and nocturia.  She has had these symptoms for 6 months and they have been slowly worsening.  The patient has had no chest pain, palpitations, or syncope.  She has a history of chronic hypertension and takes lisinopril.  Blood pressure is 152/78 mm Hg, pulse is 82/min and regular, and respirations are 18/min.  Jugular venous pressure is 10 cm H2O.  The lungs are clear without crackles.  There are no heart murmurs.  The abdomen is mildly distended with no tenderness.  There is 2+ pitting edema in the patient's legs.  Brain natriuretic peptide concentration is 776 pg/mL (N <100 pg/mL).  D-dimer is normal.  Chest x-ray shows no cardiomegaly, normal interstitial markings, and no pulmonary edema.  Urinalysis shows 1+ protein but is otherwise normal.  Which of the following is the most likely diagnosis?

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Explanation:

This patient with nocturia and progressive exertional dyspnea has evidence of volume overload (eg, jugular venous distension [JVD], peripheral edema), which is suggestive of decompensated heart failure (DHF).  An elevated brain natriuretic peptide (BNP) level strengthens this clinical diagnosis, although some patients with DHF (especially those with obesity) may have falsely normal BNP levels.

The cause of dyspnea in DHF is multifactorial and involves more than pulmonary edema alone (eg, increased ventilatory demand, ventilatory and peripheral muscle dysfunction).  Therefore, the absence of pulmonary edema in this patient does not exclude the diagnosis of DHF because patients with slowly progressive decompensation may have minimal or no pulmonary edema.  This is because the pulmonary lymphatics can gradually increase fluid outflow rate up to 10 times the baseline when needed, effectively offloading fluid from the pulmonary venous system to the central venous system and allowing evidence of elevated central venous pressure (eg, JVD, peripheral edema) to be present despite minimal or no pulmonary edema.

Cardiomegaly may be absent if DHF is due to left ventricular hypertrophy (eg, in the setting of chronic hypertension) resulting in diastolic dysfunction (impaired filling).

(Choice A)  Although deconditioning can cause dyspnea on exertion, it would not explain JVD, peripheral edema, or elevated BNP.

(Choice C)  Interstitial lung disease (ILD) typically presents with dry cough and dyspnea with exertion.  Long-standing ILD can also lead to JVD and peripheral edema (from elevated central venous pressure caused by pulmonary hypertension).  However, lung auscultation typically reveals fine crackles, and chest x-ray is expected to show increased interstitial lung markings.

(Choice D)  Nephrotic syndrome typically presents with proteinuria and prominent peripheral edema resulting from loss of albumin and other proteins in the urine.  Dyspnea on exertion can occur, but JVD and elevated BNP are not expected because the hypoalbuminemia causes intravascular volume depletion.  Mild to moderate proteinuria is common in DHF, possibly related to increased angiotensin II activity.

(Choice E)  Pulmonary embolism typically causes sudden-onset dyspnea and often elevated BNP.  Pleuritic chest pain and sinus tachycardia (absent in this patient) are commonly present, and a normal D-dimer makes pulmonary embolism highly unlikely.

Educational objective:
Decompensated heart failure typically presents with dyspnea on exertion, jugular venous distension, and peripheral edema.  Pulmonary edema may be absent in slowly progressive decompensation because of increased lymphatic drainage.