Letter to the Editor
Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma

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Abstract

A 48-year-old male patient presented with dyspnea on exertion. Patient was found to have pulmonary hypertension. Myocardial biopsy showed amyloidosis and further work-up revealed Salmon-Durie stage 1A multiple myeloma. Patient had no other clinical manifestations of amyloidosis. It is possible that the pulmonary hypertension is caused by amyloid deposition into pulmonary arteries as the arterial amyloid deposition is common in AL amyloidosis. Treatment with sildenafil led to hemodynamic and symptomatic improvement.

Introduction

Clinical evidence of cardiac involvement occurs in ∼ 50% of the patients with AL amyloidosis. Amyloidosis may lead to systolic, diastolic or restrictive cardiac dysfunction. In addition to amyloid fibril deposition into cardiac tissue, deposition may occur also in the coronary arteries and pulmonary blood vessels [1]. Pulmonary hypertension is a rare late manifestation of amyloidosis [1], [2]. We describe a patient with AL amyloidosis and pulmonary hypertension without any other clinical manifestation.

Section snippets

Case

48-year-old previously healthy male presented to a community hospital with a 6-month history of NYHA II-level dyspnea on exertion.

In a clinical exam, blood pressure was 140/72 and HR 82 bpm. BMI was 26. Clinical exam was significant for loud S2. ECG had a rSR' alteration in lead V1 consistent with right ventricular hypertrophy. Laboratory studies were normal but total serum protein was elevated.

In bicycle ergometry exercise tolerance test, patient reached a maximal load of 150 W and the blood

Discussion

Our patient presented with exertional dyspnea that was found to be due to pulmonary hypertension. Extensive work-up of the various secondary causes of pulmonary hypertension did not reveal evidence of either of left or right heart failure. Neither did we find evidence of chronic pulmonary embolism, lung disease or shunt. Elevated plasma protein levels led ultimately to a diagnosis of stage 1A multiple myeloma and amyloidosis.

The deposition of light chains appears to have tissue specificity that

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    Pulmonary hypertension from prominent vascular involvement in diffuse amyloidosis

    Arch Intern Med

    (1988)
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