Pulmonary Hypertension in Patients With Amyloidosis

doi:10.1378/chest.120.5.1735

Chest

Volume 120, Issue 5, November 2001, Pages 1735-1738

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Pulmonary hypertension (PH) with right-sided cardiac failure is a rare complication of amyloidosis, and its natural history is not well-defined. The aim of our study was to evaluate patients who were seen at our institution who had PH and amyloidosis and to describe the natural history of this complication. The study was a retrospective chart review of patients seen at the Mayo Clinic with both PH and amyloidosis listed as major diagnoses between January 1, 1980, and December 31, 1999. Patients with known causes of PH were excluded. Five patients met our criteria (four women and one man). Four patients had light-chain amyloidosis and one had amyloid A deposition secondary to familial Mediterranean fever. All patients had symptoms related to PH without echocardiographic evidence of left ventricular dysfunction. The median survival time after the diagnosis of amyloidosis was 2.8 years, and PH was found a median of 73 days before death. Five patients died of cardiac complications, including one with sudden cardiac death. PH is an unusual complication of amyloidosis. Patients develop PH late in the disease process and do not have a worse prognosis compared to other patients with cardiac amyloidosis. PH is a marker of advanced amyloidosis.

Section snippets

Materials and Methods

We performed a computerized search of all patient records at the Mayo Clinic having both PH and amyloidosis listed as major diagnoses. The study was approved by the institutional review board in accordance with federal regulations. Our search covered the period from January 1, 1980, to December 31, 1999. Amyloidosis was diagnosed by tissue biopsy. The diagnosis of PH was based on either transthoracic echocardiography (TTE) data and/or measurements during right-sided heart catheterization or

Results

Our search generated a list of 18 patients with both PH and amyloidosis. Of these, 13 patients were excluded. Eleven patients had echocardiographic evidence of severe cardiac amyloidosis, 1 patient had severe valvular heart disease (ie, aortic stenosis and grade 4 mitral regurgitation), and another patient had solitary bronchial amyloidosis. The characteristics of our remaining five patients are summarized in Table 1. One patient already has been reported.¹⁶ Testing to rule out pulmonary

Discussion

Amyloid deposition can be a systemic or focal process. The deposition of fibrils in the extracellular matrix leads to organ dysfunction, though the exact mechanisms for this are not understood. AL and AA tend to be the results of systemic processes with deposition of fibrils in the kidney, heart, liver, GI tract, nervous system and respiratory tract. This explains why amyloidosis can present as different “organ syndromes” requiring a high index of suspicion.¹

An increase in pulmonary vascular

Acknowledgment

We thank William D. Edwards, MD, Division of Anatomic Pathology, Mayo Clinic Rochester, for the photomicrograph.

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L’amylose thoracique est une pathologie rare et grave. Son association à une hypertension artérielle pulmonaire (HTAP) ne fait habituellement qu’en assombrir le pronostic. Nous rapportons le cas d’un patient âgé de 40 ans tabagique hospitalisé pour des douleurs thoraciques, abdominales et une détresse respiratoire aiguë. L’échographie cardiaque a objectivé un épanchement péricardique circonférentiel ainsi qu’une pression artérielle pulmonaire systolique (PAPS) à 80 mmHg. L’imagerie thoracique a montré un processus tissulaire développé dans la gaine du péricarde (60 × 45 mm) engainant l’aorte ascendante et infiltrant le tronc de l’artère pulmonaire et sa branche droite. L’étude anatomopathologique et immunohistochimique de ce processus a révélé une amylose AL. Le patient n’avait pas de signes d’amylose extrathoracique. L’électrophorèse et l’immunoélectrophorèse sanguine et urinaire ainsi que le myélogramme et la biopsie ostéomédullaire étaient normaux. Le patient a été mis sous anticoagulant oral devant l’HTAP. Un protocole de traitement par thalidomide et dexaméthasone a été entrepris. L’évolution a été marquée par la régression totale des signes cliniques, la diminution nette du processus amyloïde à l’imagerie et la normalisation de la PAPS avec un recul actuel de trois ans.
Isolated thoracic involvement in amyloidosis is a rare and serious condition. Its association with pulmonary arterial hypertension (PAH) usually weakens the prognosis. We report the case of a 40-year-old man with a smoking history, hospitalized for chest pain, abdominal pain and acute respiratory distress. The cardiac ultrasound revealed a circumferential pericardial effusion as well as a pulmonary artery systolic pressure (PAPS) at 80 mmHg. Chest imaging (computed tomography scan and magnetic resonance imaging) showed a tissue process developed in the pericardial sheath (60 × 45 mm) sheathing the ascending aorta and infiltrating the trunk of the pulmonary artery and its right branch. Anatomopathological and immunohistochemical study of the process revealed AL amyloidosis. Note that the patient had no signs of extrathoracic amyloidosis. Blood and urine electrophoresis and immunoelectrophoresis as well as bone marrow mylogram and biopsy were normal. The patient was put on oral anticoagulant as he presented with PAH. A therapeutic protocol with thalidomide and dexamethasone has been initiated. The course of the disease was marked by total regression of the clinical signs, a marked decrease in the amyloid process on imaging and a normalization of the PAPS; our follow-up being three years.
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Citation Excerpt :
Pulmonary amyloid can be subdivided into nodular, laryngeal/tracheobronchial, and diffuse interstitial. Only the third type represents a manifestation of systemic AL amyloidosis.196,197 The nodular form of amyloid presents as solitary pulmonary nodules or multiple nodules.
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Chest

Selected ReportsPulmonary Hypertension in Patients With Amyloidosis

Section snippets

Materials and Methods

Results

Discussion

Acknowledgment

Hematol Oncol Clin North Am

Mayo Clin Proc

Int J Cardiol

Am J Med

Mayo Clin Proc

Am J Med

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Cardiovasc Pathol

Am J Cardiol

Selected Reports
Pulmonary Hypertension in Patients With Amyloidosis