Copyright © 2005, The European Society of Cardiology
Dynamic left ventricular outflow tract obstruction in senile cardiac amyloidosis
aDivision of Cardiovascular Diseases and Internal Medicine, Mayo Clinic College of Medicine, 200 First Street, SW, Rochester, MN 55905, USA
bClinic for Cardiovascular Diseases, University Hospital Centre Zagreb, Kispaticeva 12, 10000 Zagreb, Croatia maja.cikes{at}zg.htnet.hr
Received 31 May 2005; received in revised form 2 September 2005; accepted after revision 11 September 2005.
* Corresponding author. Tel.: +1 507 284 3684; fax: +1 507 266 9142. mookadam.farouk{at}mayo.edu
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Abstract |
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Dynamic left ventricular outflow tract (LVOT) obstruction is classically seen in hypertrophic obstructive cardiomyopathy (HOCM). This can also be seen in cardiac amyloidosis. We describe a rare case of senile systemic amyloidosis with dynamic LVOT obstruction and concomitant three vessel coronary artery disease presenting with clinical and echocardiographic findings similar to those seen in HOCM. We also highlight the importance of distinguishing the sub-types of amyloidosis so that the appropriate therapy can be offered to patients with cardiac involvement, including coronary artery bypass grafting and septal myotomy/myectomy to relieve LVOT obstruction in the more benign forms of cardiac amyloidosis.
Keywords: Hypertrophic obstructive cardiomyopathy; Left ventricular outflow tract obstruction; Coronary artery disease; Senile amyloidosis; Cardiac amyloidosis
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Case report |
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A 73-year-old Caucasian gentleman presents with a three-year history of presyncope and chest discomfort. He was considered New York Heart Association (NYHA) Class III with an effort tolerance of 50 yards.
Six months prior, an extensive work-up including chest X-ray (CXR), electrocardiogram (ECG), laboratory data, 24-h ambulatory Holter recording, and physical examination was completed. Subsequent ambulatory blood pressure monitoring, exercise stress test with nuclear tomographic imaging, and CT scan of the head were all normal. Coronary angiography revealed only moderate coronary atherosclerotic disease at multiple sites. None of the stenoses were considered hemodynamically significant and ventriculography revealed a normal ejection fraction with no regional wall motion abnormalities.
He subsequently underwent electrophysiologic assessment with poorly tolerated polymorphic ventricular tachycardia resulting in hemodynamic collapse and subsequent restoration of hemodynamic stability with urgent cardioversion. This finding was considered non-specific by the electrophysiologist.
On physical examination, he appeared younger than his stated age. Heart rate was 70 beats per minute and blood pressure was 160/80mmHg, neck veins were not distended; the precordial examination was unremarkable except for a forceful well localised apical impulse. A soft ejection systolic murmur at the upper left sternal border, grade 1/6 was appreciated; carotid upstroke was considered normal; no added cardiac sounds were noted; remainder of the physical examination was normal with no postural blood pressure drop.
Basic laboratory data were within normal limits. Twelve-lead ECG showed normal sinus rhythm with occasional premature supraventricular beats, left axis deviation, and minor non-specific ST–T changes with normal voltages. The CXR showed mild calcification of the aortic knuckle with normal heart size and clear lung fields. Adenosine stress test revealed reversible defects in the anterior and posterior circulation consistent with multivessel coronary artery disease. Two-dimensional echocardiogram revealed thickened left and right ventricular walls (the interventricular septum measured 18mm) with evidence of dynamic outflow tract obstruction considered mild in the right ventricular outflow tract (RVOT) (20mmHg gradient) and a gradient across the left ventricular outflow tract (LVOT) of 46mmHg at rest and 81mmHg post-Valsalva. Systolic anterior motion of the mitral leaflet was also present (Figs. 1–5
). LA was moderately enlarged (55mm). Degenerative changes of the aortic valve were noted. The diastolic parameters were consistent with a marked relaxation abnormality. There was no evidence of pericardial effusion.
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Coronary angiography confirmed severe three vessel coronary artery disease and septal biopsy confirmed cardiac amyloidosis with focal nodular and interstitial amyloid on sulfated LC and ALCIA in blue stain. Immunohistochemical stains were positive for serum amyloid protein and prealbumin. Stains for kappa and lambda light chain, serum amyloid associated protein, beta II microglobulin and albumin were negative. Findings were consistent with senile familial amyloidosis.
The patient was referred for coronary artery bypass grafting (CABG) which was completed along with an extended left ventricular septal myectomy. The resting gradient in the LVOT was 80mmHg confirmed on intraoperative transesophageal echocardiography as well as by direct intracardiac manometry. Pressure in the aorta and LV were measured as 80/40mmHg and 167/20mmHg, respectively. An extended left ventricular septal myectomy was completed with no residual LVOT gradient post-procedure.
The patient had an uncomplicated postoperative course, and at 5 years postoperative was doing well with complete resolution of his symptoms and was NYHA Class I. On a 6 year postoperative examination the patient was NYHA Class II with new onset atrial fibrillation, moderate evolving mitral regurgitation and moderate diastolic dysfunction.
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Discussion |
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Hypertrophic cardiomyopathy with dynamic LVOT obstruction has classic echocardiographic and clinical features. Mimickers of LVOT obstruction have recently been described in patients with acute myocardial infarction.1 LVOT obstruction has been described in secondary amyloidosis with multiple myeloma and type I Gaucher's disease.2,3 In a study of 204 surgical pathology specimens of subaortic septal myectomy associated with hypertrophic obstructive cardiomyopathy, amyloidosis was detected in three cases and was considered to be senile based on the patients' ages.4 In all the three of these cases, the finding of amyloidosis was unexpected, as opposed to the case we report of in which the suspicion was raised preoperatively. To our knowledge, neither a case report of senile cardiac amyloidosis causing dynamic LVOT obstruction has been described in the literature nor has there been a report of such a patient undergoing CABG and septal myotomy/myectomy successfully with excellent results at 5 year follow-up. It is known that transmitral flow indices consistent with restrictive physiology are strong predictors of cardiac death; therefore we attribute the good follow-up result to low grade diastolic dysfunction at presentation.5
It may be challenging to differentiate cardiac amyloid from hypertrophic cardiomyopathy on the basis of two-dimensional echocardiography features alone. Asymmetric increases in septal wall thickness, systolic anterior motion of the mitral valve, and dynamic LVOT obstruction have been previously described in patients with cardiac amyloidosis.6–8
It has been shown that patients with systemic senile amyloidosis (SSA) have a survival of 60 months compared to patients with primary amyloidosis (AL) of only 5.4 months.9 Senile cardiac amyloidosis is a heterogenous group of disorders that is not readily distinguished by routine microscopy or transmission electron microscopy.10 It is recommended that patients with suspected cardiac amyloid be considered for endomyocardial biopsy in the absence of evidence of amyloid deposition in other organs. In patients with negative urine and serum monoclonal electrophoretic bands, specific immunohistochemical staining for prealbumin (transthyretin) and immunoglobulin light chain should be undertaken to distinguish between SSA and immunoglobulin derived amyloidosis because of the vast differences in prognosis and hence therapies.
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References |
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- Haley J.H., Sinak L.J., Tajik A.J., Ommen S.R., Oh J.K. Dynamic left ventricular outflow tract obstruction in acute coronary syndromes. Mayo Clin Proc (1999) 74:901–906.[Abstract]
- Oh J.K., Tajik A.J., Edwards W.D., Bresnahan J.F., Kyle R.A. Dynamic left ventricular outflow tract obstruction in cardiac amyloidosis detected by continuous wave Doppler echocardiography. Am J Cardiol (1987) 59:1008–1010.[CrossRef][Web of Science][Medline]
- Hrebicek M., Zeman J., Musilova J., Hodanova K., Ghrenkema G.H. A case of type I Gaucher's disease with cardiopulmonary amyloidosis and autotriosidiase deficiency. Virchows Arch (1996) 429:305–309.[CrossRef][Web of Science][Medline]
- Lamke G.T., Allen R.D., Edwards W.D., Tazelaar H.D., Danielson G.K. Surgical pathology of subaortic septal myectomy associated with hypertrophic cardiomyopathy: a study of 204 cases (1996–2000). Cardiovasc Pathol (2003) 12:149–158.[CrossRef][Web of Science][Medline]
- Klein A.L., Hatle L.K., Taliercio C.P., Oh J.K., Kyle R.A., Gertz M.A., et al. Prognostic significance of Doppler measures of diastolic function in cardiac amyloidosis. A Doppler echocardiography study. Circulation (1991) 83:808–816.
[Abstract/Free Full Text] - Siqueira-Filho A.G., Cunha C.L.P., Tajik A.J., Seward J.B., Schattenberg T.T., Giuliani E.R. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation (1981) 63:188–196.
[Abstract/Free Full Text] - Griffiths B.E., Hughes P., Dowdle R., Stephens M.R. Cardiac amyloidosis with asymmetrical hypertrophy and deterioration after nifedipine. Thorax (1982) 37:711–712.
[Free Full Text] - Sedlis S.P., Saffitz J.E., Schwob V.S., Jaffe A.S. Cardiac amyloidosis simulating hypertrophic cardiomyopathy. Am J Cardiol (1984) 53:969–970.[Web of Science][Medline]
- Kyle R.A., Spittell P.C., Gertz N.A., Li C.Y., Edwards W.D., Olson L.J., et al. The pre-mortem recognition of systemic senile amyloidosis with cardiac involvement. Am J Med (1996) 171:395–400.
- Olson L.J., Gertz M.A., Edwards W.D., Li C.Y., Pellikka P.A., Holmes D.R., et al. Senile cardiac amyloidosis with myocardial dysfunction. N Engl J Med (1987) 317:738–742.[Abstract]
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