|
Cardiovascular Pathology
Monday, February 13, 2006 - 7:30 PM
Regency VI
Cardiovascular Pathology:
Advances in Diagnosis and Elucidation of Disease Mechanisms
|
Moderator:
Jeffrey Saffitz
Beth Israel Deaconess Medical Center
Boston, MA
Disclosure: The speakers have indicated they have nothing to disclose.
|
Clinical histories are printed below.
Click on the case numbers for text and references of each case.
Click on each slide thumbnail image for an enlarged view
Submitted by:
Gayle L. Winters
Brigham and Women's Hospital
Harvard Medical School, Boston, MA
A 51 year-old female nurse presented with a three-week history of fatigue and dyspnea on exertion. The symptoms were progressive and led to a significant limitation in her activities of daily living, such that by the day of admission she was unable to climb a single flight of stairs. She also reported occasional burning chest discomfort that did not radiate and had no clear association with exertion, as well as some brief palpitations. Her past medical history (including risk factors for coronary artery disease) was entirely negative and she took no regular medications. She did not smoke or use illicit drugs, consumed alcohol only occasionally, and denied any high-risk sexual behaviors. She had no history of travel outside the US and there were no notable occupational or environmental exposures.
On physical examination, she appeared well. She was afebrile with a blood pressure of 88/60 mm Hg, pulse 90 beats per minute, and respiratory rate 20 breaths per minute. Cardiac examination revealed a regular gallop rhythm with audible left-sided third and fourth heart sounds. Admitting laboratory studies were non-contributory. ECG revealed left axis deviation, poor R wave progression, 2-3 mm ST segment elevations in leads V2, V3, and 2-3 mm ST segment depressions in leads V5 and V6. Emergent left-sided cardiac catheterization revealed a right dominant circulation with clean coronary vessels except for a 20-30% stenosis in the left anterior descending artery. There was global hypokinesis. Transthoracic echocardiography showed a mildly enlarged left ventricle with global hypokinesis and a left ventricular ejection fraction of 15-20%.
The day of admission, the patient became severely hypotensive and developed sustained monomorphic ventricular tachycardia requiring direct-current cardioversion. Urgent right heart catheterization with endomyocardial biopsy was performed (Slide A). She remained hemodynamically unstable and required placement of an intra-aortic balloon pump. Urgent transplant evaluation was undertaken. Two days after her biopsy a Thoratec BiVAD was inserted for cardiogenic shock and recurrent ventricular tachyarrhythmias. Her condition stabilized and she awaited transplantation in the hospital. Approximately two months after her initial presentation, she underwent heart transplantation and her explanted heart became available for examination (Slide B).
Case 1 - Figure 1 -
(Endomyocardial Biopsy): Low power view of four endomyocardial biopsy samples. There is extensive and diffuse replacement of myocardium by an inflammatory process.
|
Case 1 - Figure 2 -
(Endomyocardial Biopsy): Intermediate power of extensive inflammatory infiltrate containing giant cells. There is extensive myocyte damage.
|
Case 1 - Figure 3 -
(Endomyocardial Biopsy): High power view showing polymorphous nature of infiltrate, including lymphocytes, histiocytes, plasma cells and eosinophils.
|
Case 1 - Figure 4 -
(Endomyocardial Biopsy): High power of multinucleated giant cells.
|
Case 1 - Figure 5 -
(LVAD Core): Intermediate power of diffuse mixed inflammatory infiltrate with multinucleated giant cells and extensive myocyte damage.
|
Case 1 - Figure 6 -
(LVAD Core): High power of mixed inflammatory infiltrate with multinucleated giant cells and myocyte damage.
|
Case 1 - Figure 7 -
(Explanted Heart): Four-chamber cut of 310 gm explanted heart with LVAD cannula in left ventricular apex. There is biventricular dilation and a partially organized thrombus in the right atrium. The mitral valve is mildly myxomatous and there is no fusion (post-LVAD) of the aortic valve cusps. The coronary arteries contained only minimal (nonocclusive) atherosclerosis.
|
Case 1 - Figure 8 -
(Explanted Heart): There is mild mottling of the interventricular septal myocardium (left). Histology of the myocardium (H&E and Trichrome) reveals extensive fibrosis in a non-coronary distribution (right, top and bottom).
|
Case 1 - Figure 9 -
(Explanted Heart): Scattered giant cells are present within areas of fibrosis.
|
Case 1 - Figure 10 -
(Explanted Heart): There are multiple foci of residual active inflammation consisting of a mixed infiltrate with giant cells and myocyte damage.
|
Submitted by:
Mary Sheppard
Royal Brompton Heart Hospital
London, U.K.
- DOB: 09-02-1949.
- Patient aged 53 originally presented from a family screening program. His mother presented with hypertrophic cardiomyopathy (aged 65 years) and he was subsequently also found to have the condition. At the time his only cardiac symptom was that of palpitations. Echocardiography demonstrated concentric left ventricular hypertrophy (maximal wall thickness 20mm). He was also found to have asthma and was treated with inhalers and intermittent courses of oral steroids. He progressed over 5 years to heart failure and presented to hospital with severe chest pain and difficulty breathing. He suffered cardiac arrest shortly after admission from which he was not able to be resuscitated.
AUTOPSY - The weight of the heart is 879g. On examination, there is left ventricular hypertrophy particularly noted in the anteroseptal and posteroseptal wall of the left ventricle. The thickness of the postero-septal wall is 21mm whereas the anterior and posterior appear to be 16mm. The lateral free wall of the left ventricle appears of normal thickness at 15mm. There is hypertrophy of the trabeculae which appear quite prominent in the left ventricle.
- In the postero-lateral wall of the left ventricle there is a slightly sunken area in the inner one third of the subendocardium. Elsewhere, the muscle appears prominent with some swirling but no other macroscopic areas of fibrosis.
- The right ventricle appears normal in the anterior and lateral wall with overlying epicardial fat, but there is hypertrophy of the posterior wall which is approximately 6mm.
- There is nodular calcification of the aortic valve leaflets which extends onto the anterior mitral leaflet with calcification extending onto the chordae of the mitral valve. The aortic leaflets are slightly thickened but there is no significant stenosis of the valve leaflets which move freely. There is extensive nodular calcification of the anterior leaflet of the mitral valve which involves both the annulus and the body of the mitral leaflet. At the edge of the anterior leaflet, there is slight ballooning and thickening between the chordae. The tricuspid valve also appears slightly thickened and ballooned between the chordae. Pulmonary valve appears macroscopically normal. There are no congenital defects seen in the heart. The coronary arteries appear normal with no narrowing macroscopically.
Case 2 - Figure 1 -
Shows a transverse short axis view of the heart with left ventricular hypertrophy. Note slight thinning of the posterolateral wall. There is also mild right ventricular hypertrophy in the posterobasal area.
|
Case 2 - Figure 2 -
Mitral valve shows extensive annular calcification with mild thickening of valve leaflets
|
Case 2 - Figure 3 -
Aortic valve and anterior leaflet of mitral valve shows extensive annular calcification extending onto the aortic valve. Note the mild thickening of the aortic valve leaflets.
|
Case 2 - Figure 4 -
Tricuspid valve shows thickening and ballooning between the chordae.
|
Case 2 - Figure 5 -
Transverse sections of the coronary arteries showing eccentric intimal proliferation and vacuolization of medial smooth muscle cells. Haematoxylin and Eosin stain.
|
Case 2 - Figure 6 -
Sections of the myocardium showing myocyte hypertrophy, vacuolization of the cytoplasm and interstitial fibrosis. Haematoxylin and Eosin stain.
|
Case 2 - Figure 7 -
Sections of the myocardium showing myocyte hypertrophy, vacuolization of the cytoplasm and interstitial fibrosis. Note also the foamy macrophages surrounding degenerating myocytes . Haematoxylin and Eosin stain.
|
Case 2 - Figure 8 -
Electron microscopic section of myocyte showing the typical lamellar bodies within the cytoplasm.
|
Case 2 - Figure 9 -
Shows fibrosis ( red stain) replacing myocardium and surrounding individual myocytes (yellow). Picro Sirius red staining.
|
Case 2 - Figure 10 -
Shows mitral valve with diffuse thickening and calcification . Haematoxylin and Eosin stain.
|
Case 2 - Figure 11 -
Shows aorta with vacuoles within the smooth muscle cells highlighted by the van Gieson Trichrome staining.
|
Case 2 - Figure 12 -
Shows aorta with vacuoles within the smooth muscle cells (Haematoxylin and Eosin stain)
|
Submitted by:
Bruce McManus
University of British Columbia
Vancouver, BC, Canada
A 54-year-old woman presented with a three day history of nausea, vomiting, and fever. After resuscitation from asystolic arrest, the patient had an ejection fraction of about 15%. Coronary angiograms were unremarkable. The patient underwent endomyocardial biopsy in anticipation of the need for heart transplantation. The heart tissue was abnormal. However, following a certain therapy the patient's status markedly improved.
Case 3 - Figure 1 -
Hematoxylin and eosin stained slide of the endomyocardial biopsy showing the general architecture of the tissue and the extent of the interstitial and endocardial inflammatory infiltrate. (X10)
|
Case 3 - Figure 2 -
Higher magnification of the same area as in Figure 1 showing a percolating, largely mononuclear cell infiltrate with some distortion of architecture and focal myocyte damage. (X40)
|
Case 3 - Figure 3 -
An image of the same biopsy piece showing how the infiltrate involves the endocardium. Focal fibrosis is noted. (X40)
|
Case 3 - Figure 4 -
Hematoxylin and eosin stained slide of a second biopsy piece showing an area of infiltration by mature adipocytes. Inflammation is noted nearby. (X10)
|
Case 3 - Figure 5 -
A second view of the second biopsy piece shows the extent of neighboring myocarditis. (X10)
|
Case 3 - Figure 6 -
Higher magnification of the same area shows features similar to those in the other biopsy piece, emphasizing architectural disruption, numerous macrophages, some lymphocytes and occasional eosinophils. (X40)
|
Case 3 - Figure 7 -
A higher power of the second piece showing similar features as in Figure 6. (X40)
|
Case 3 - Figure 8 -
A higher magnification of the second piece showing the infiltrate and occasional apoptotic bodies. (X40)
|
Case 3 - Figure 9 -
Full view of the biopsy showing the inflammatory process and fatty accumulation. (X10)
|
Case 3 - Figure 10 -
A fourth piece of the biopsy showing prominent endocardial inflammation including many lymphocytes. Occasional eosinophils are
|
Submitted by:
James Stone
Massachusetts General Hospital
Boston, MA
The patient is a 37 year-old woman who presented with dyspnea on exertion, fatigue, orthopnea, and ankle edema. Chest X-ray revealed mild cardiac enlargement. An echocardiogram revealed biatrial enlargement, moderate left ventricular hypertrophy with mild global dysfunction, mild mitral regurgitation, moderate tricuspid regurgitation, moderate pulmonary hypertension, and a left ventricular ejection fraction of 45%. Coronary angiography revealed no significant coronary artery disease. A chest CT scan showed no evidence of pulmonary emboli or pericardial thickening. A right ventricular endomyocardial biopsy was performed, and a specific diagnosis rendered. The patient subsequently underwent cardiac allograft transplantation.
|
|
|
|
