CASE REPORT
71-year-old male with progressive dyspnoea
and peripheral oedema: a case of cardiac amyloidosis C. Cable, J. Hodges, T. Tak
We present the case of a 71-year-old male with progressive dyspnoea. On physical examination there was evidence of congestive heart failure. The complete blood count was normal and twelve-lead ECG showed low voltage in the standard leads. Chest x-ray showed cardiomegaly, pulmonary congestion and left pleural effusion. Twodimensional echocardiography and Doppler ultrasound studies revealed biatrial enlargement and a restrictive diastolic filling pattern. Serum protein electrophoresis was abnormal showing a monodonal elevation of IgG. Amyloid heart disease was suspected. Fat pad biopsy showed findings consistent with amyloid heart disease. Melphalan and prednisone were initiated to help slow the progression of disease. (Neth HeartJ 2001;9:182-4.)
Key words: cardiac amyloidosis, melphalan, prednisone A 71 -year-old male was referred for evaluation of persistent fatigue and dyspnoea. He had an excellent exercise tolerance until developing pneumonia four months prior to presentation. His fever, chills and copious sputum cleared after several courses of antibiotics; however, he continued to have severe shortness of breath, non-productive cough, and malaise. The patient acknowledged anorexia with a ten pound weight loss and intermittent ankle swelling. He denied chest pain, orthopnoea, or paroxysmal nocturnal dyspnoea. His past medical history was significant for hypercholesterolaemia. There was no hisC. Cable. T. Tak. Division of Cardiology. J. Hodges. Community Internal Medicine Scott & White Memorial Hospital and Clinic Scott, Division of Cardiology, 2401 South 31st Street, Temple, Texas 76508, USA. Address for correspondence: T. Tak. E-mail: [email protected]
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tory of hypertension, diabetes mellitus, or tobacco abuse. His family history was negative for coronary artery disease and his medications included antibiotics and NSAIDs for degenerative joint disease. On physical examination, the patient was normotensive with a regular pulse. The jugular venous pressure was estimated at 13 cm H20 and Kussmaul's sign was present. Decreased breath sounds and dullness to percussion were appreciated over the left lower lung fields. On cardiac examination there were no significant murmurs. The pulmonary component of S2 was accentuated and audible at the apex. An S3 was present. There was no pericardial rub. There was mild enlargement of the liver. He had 2+ ankle oedema bilaterally. The complete blood count was normal. The electrocardiogram showed low voltage in the limb leads and right ventricular conduction delay (figure 1). A chest x-ray demonstrated mild cardiac enlargement with an increase in pulmonary vascularity. A large left pleural effusion was noted. The pleural fluid was found to be a transudate with negative cytology for malignancy. The patient's dyspnoea did not improve after thoracentesis. Echocardiography revealed an ejection fraction of0.50 and no significant valvular disease. Mild to moderate biventricular hypertrophy was present. Doppler studies demonstrated elevated right heart pressures with an estimated right ventricular systolic pressure (RVSP) of 83 mmHg. The E to A ratio of transmitral flow was >2; the deceleration time (DT) of E wave was
71-year-old male with progressive dyspnoea
and peripheral oedema: a case of cardiac amyloidosis C. Cable, J. Hodges, T. Tak
We present the case of a 71-year-old male with progressive dyspnoea. On physical examination there was evidence of congestive heart failure. The complete blood count was normal and twelve-lead ECG showed low voltage in the standard leads. Chest x-ray showed cardiomegaly, pulmonary congestion and left pleural effusion. Twodimensional echocardiography and Doppler ultrasound studies revealed biatrial enlargement and a restrictive diastolic filling pattern. Serum protein electrophoresis was abnormal showing a monodonal elevation of IgG. Amyloid heart disease was suspected. Fat pad biopsy showed findings consistent with amyloid heart disease. Melphalan and prednisone were initiated to help slow the progression of disease. (Neth HeartJ 2001;9:182-4.)
Key words: cardiac amyloidosis, melphalan, prednisone A 71 -year-old male was referred for evaluation of persistent fatigue and dyspnoea. He had an excellent exercise tolerance until developing pneumonia four months prior to presentation. His fever, chills and copious sputum cleared after several courses of antibiotics; however, he continued to have severe shortness of breath, non-productive cough, and malaise. The patient acknowledged anorexia with a ten pound weight loss and intermittent ankle swelling. He denied chest pain, orthopnoea, or paroxysmal nocturnal dyspnoea. His past medical history was significant for hypercholesterolaemia. There was no hisC. Cable. T. Tak. Division of Cardiology. J. Hodges. Community Internal Medicine Scott & White Memorial Hospital and Clinic Scott, Division of Cardiology, 2401 South 31st Street, Temple, Texas 76508, USA. Address for correspondence: T. Tak. E-mail: [email protected]
182
tory of hypertension, diabetes mellitus, or tobacco abuse. His family history was negative for coronary artery disease and his medications included antibiotics and NSAIDs for degenerative joint disease. On physical examination, the patient was normotensive with a regular pulse. The jugular venous pressure was estimated at 13 cm H20 and Kussmaul's sign was present. Decreased breath sounds and dullness to percussion were appreciated over the left lower lung fields. On cardiac examination there were no significant murmurs. The pulmonary component of S2 was accentuated and audible at the apex. An S3 was present. There was no pericardial rub. There was mild enlargement of the liver. He had 2+ ankle oedema bilaterally. The complete blood count was normal. The electrocardiogram showed low voltage in the limb leads and right ventricular conduction delay (figure 1). A chest x-ray demonstrated mild cardiac enlargement with an increase in pulmonary vascularity. A large left pleural effusion was noted. The pleural fluid was found to be a transudate with negative cytology for malignancy. The patient's dyspnoea did not improve after thoracentesis. Echocardiography revealed an ejection fraction of0.50 and no significant valvular disease. Mild to moderate biventricular hypertrophy was present. Doppler studies demonstrated elevated right heart pressures with an estimated right ventricular systolic pressure (RVSP) of 83 mmHg. The E to A ratio of transmitral flow was >2; the deceleration time (DT) of E wave was
