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Presented by: Ivana Kholová, Tampere, Finland
A 56-year-old female presented with collapses. At hospital, total block was diagnosed on ECG. She had no previous diseases, nor medications. Laboratory tests were normal. Heart ultrasound was normal. No family history of heart diseases. Cardiac biopsy was performed. |
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| Slide 1 (AB-PAS) | Slide 2 (Masson Trichrome) |
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| Diagnosis & Comments [0] |
| Handout: Download |
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Presented by: Patrick Bruneval, Paris, France
A 46 year-old Caucasian woman had a diagnosis of systemic lupus erythematosus in 1987, presenting arthritis, dermatological symptoms, and glomerulonephritis (class V, WHO classification) that led to end stage renal failure.
At the age of 72 years, while she was on dialysis for 3 years, she was admitted in renal transplant care unit for renal transplantation. Cardiac screening before transplantation demonstrated a left anterior fascicular block with right bundle branch block, an anteroseptal ST elevation at ECG. The coronarography did not demonstrate any abnormality. Transesophageal echography showed a preserved left ventricular systolic function (LVEF 55%) with a moderate diastolic dysfunction, a concentric left ventricular hypertrophy (16 mm) and slightly elevated right-sided filling pressures. A cardiovascular magnetic resonance evidenced a diffuse subepicardial late gadolinium enhancement consistent with a myocarditis or an infiltrative cardiomyopathy. The patient had no biological inflammatory syndrome, no outburst of her disease and anti-nuclear factors remained inconspicuous. Three months later (September 2011) the patient had a double renal transplant. Renal function was consecutively improved but a chronic renal dysfunction subsided with a serum creatinin at 149 µmol/l. The patient then progressively developed a dyspnea related to a class-III NYHA cardiac failure.
As the heart failure worsened, she was referred in September 2012 for endomyocardial biopsy. The ECG showed a first degree atrioventricular block. Two weeks later she died from cardiogenic shock. An autopsy was performed and confirmed cardiomegaly at 590 g, with mild dilatation and symmetric hypertrophy of the left ventricle (2cm). The epicardial coronary arteries were free of significant lesions.
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Presented by: Rosa Henriques de Gouveia, Coimbra & Lisbon, Portugal
“C4d immunostaining in EMB case”
A 49 year-old male with a congenital cardiopathy – Ebstein’s Disease –, diagnosed during infancy, was asymptomatic until his thirties. In 1998, he first complained of fatigue and palpitations. In 2003 and despite medical therapy, he was submitted to implantation of goretex ring at the tricuspid valve and to surgical cryoablation due to class III-IV NYHA cardiac insufficiency and atrial fibrillation. Afterwards, a pacemaker was implanted. In February 2012, he was submitted to heart transplant, without complications, leaving the hospital in March 2012. Myocardial biopsies have been performed to monitor rejection status. The images / slides presented are from a November 2012 biopsy.
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Presented by: Aryan Vink, Utrecht, Netherlands
Myocardial biopsy of a patient with dilated cardiomyopathy and congenital cataract
The patient is 39 year old woman that presented with complaints of fatigue and nausea during exercise. She was diagnosed with dilated cardiomyopathy and a preventive Implantable Cardioverter Defibrillator was implanted.
She had a history of congenital posterior polar cataract. Family history: her father, brother and uncle died at young age due to dilated cardiomyopathy (father at 29 years, brother at 23 years). She has 3 children: both her sons also have congenital cataract, but no signs of cardiomyopathy. Her daughter has no diseases.
Four years later, at age 43, she presented with forward failure and panischemia on the electrocardiogram due to right and left ventricular failure. A biventricular extracorporeal circulatory assist device (Centrimag) was implanted. Two months later the right ventricular function was normalized. After weaning of the right ventricular assist device, the extracorporeal left ventricular assist device (LVAD) was replaced by an internal LVAD (HeartMate II). The cardiac muscle that was removed at the apex during this procedure was submitted for histopathological examination.
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Presented by: Professor Mary Sheppard, London, United Kingdom
A previously healthy 54-year-old man presented with dyspnoea and syncope. The admission ECG showed anterolateral T-wave inversion, and the troponin-I concentration was raised (0·20 μg/L). He was treated for an acute coronary syndrome. Echocardiography showed concentric left ventricular hypertrophy with inferoposterior hypokinesis, and mild systolic dysfunction.
The right ventricle (RV) was slightly dilated with mild systolic dysfunction. Coronary angiography showed mild atheroma; he was managed medically. He presented again to his local hospital the day after discharge, with further syncope and dyspnoea. Repeat echocardiography showed progressive cardiac dysfunction with moderate RV dilatation and severe RV systolic dysfunction. He
was transferred to our centre.Cardiac MRI showed several diffuse cardiac masses involving all four cardiac chambers and encasing the coronary arteries and ascending aorta;
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| MRI | Endomyocardial biopsy | Endomyocardial biopsy | Cellular infiltrate | Cellular infiltrate | CD45 | CD20 |
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| CD79A |
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| Diagnosis & Comments [0] |
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Presented by: Patrick J Gallagher MD, Southampton SO16 6YD, United Kingdom
A 58 year old female was transferred from a District Hospital to a Regional Neurological Centre. She had a short history of headache and confusion. There was a small frontal intracerebral bleed. A variety of investigations were performed. These included a cerebral biopsy. |
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| Slide 1 | Slide 2 | Arterial wall | Grocott | Arterial wall 2 | Arterial wall and adventitia | Artery wall |
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| Cerebral inflammation 1 | Cerebral inflammation 2 | Cerebral inflammation 3 |
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Presented by: Timo Paavonen, Tampere, Finland
A 58-year old man contacted his local health care centre complaining cardiac arrhythmia (extrasystoles). He was admitted to the university hospital due to an aneurysm in ascending aorta and in the aortic arch. Elective reconstructive operation, repair with a graft, was performed. |
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| HE | cd138 | cd20 | cd3 | evg | igg4 | CD138 media |
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| IgG4 media | CD 138 adventitia | IgG4 adventitia | Elastin |
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Presented by: Stephen Preston, Southampton, United Kingdom
Case history
A 77-year-old man admitted to hospital, unwell, with worsening jaundice, pain in the limbs and neck and hypotensive. He appeared septic and was started on antibiotics. Blood cultures were taken which grew Staphylococcus aureus. He was intubated, ventilated and given inotropic treatment along with antibiotics tailored to the cultured organism. Despite this, he failed to improve and died. He had a history of liver disease of unknown aetiology, prostate cancer diagnosed two years earlier, and retroperitoneal fibrosis.
Macroscopic examination
There was jaundice. There was increased fibrous tissue around the proximal aorta and the aortic wall was thickened with an “onion skin” appearance. There was some fibrous tissue around the abdominal aorta but no aneurysm or stenosis. The fibrosis extended around the kidneys and ureters, but without hydronephrosis. The heart weighed 400g. The left ventricular myocardium contained an old healed infarct in its posterior wall. The valves showed no evidence of endocarditis. The liver was congested with a vaguely nodular appearance. The right occipital lobe of the brain showed a small volume of subarachnoid blood. |
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| Thoracic aorta (H&E) | Thoracic aorta (EVG) | Retroperitoneum (H&E) | Retroperitoneum 2 (H&E) | Liver low power (H&E) | Liver (H&E) | Liver (orcein elastin) |
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Presented by: Professor Mary Sheppard, London, United Kingdom
50 year old male with a history of asthma, chest infection and intermittent eosinophilia. Admitted to hospital with chest symptoms dating from 2 weeks prior, died on the same day despite treatment with antibiotics and steroids. Postmortem revealed a large (700g) dilated heart and a paricardial effusion. |
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| Slide 1 | Slide 2 | Slide 3 | Slide 4 | A2 EVG Vasculitis | A2 HE Vasulitis | Macro CA aneurysm |
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| A1 EVG fibrosis | A1 HE close fibrosis | A1 HE fibrosis | A2 HE inflamation myo 10x | A2 HE inflamation myo 13x 2 | A2 HE intima inflamation | A2 HE intima inflamation 20x |
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| A3 HE fibrosis | A4 HE close intramural coronary vasculitis | A4 HE intramural coronary vasculitis |
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Presented by: Patrick Bruneval, Paris, France
A 24-year-old woman was referred to the hospital for bilateral claudication of lower limbs. She had asthenia, unspecific digestive symptoms, and mild fever for 2 years. At examination she presented an aortic thrill and a painful abdomen. Her blood pressure was normal (115/70 mmHg). All her peripheral pulses were present, but she described a typical lower limb claudication at effort. Biological tests showed an inflammatory syndrome: inflammatory anemia at 9.2 g/dL, CRP at 127 mg/dL, ESR at 75 mm/60 min, whereas all immunologic and infectious tests were negative. A thoracoabdominal CT-scan showed a thickening of the aorta wall (5 mm) with an infrarenal aortic dissection extending to the common iliac arteries yelding a bilateral iliac 40% stenosis. The thoracic aorta and the supraaortic trunks were normal.
The patient received 6 months of steroids initially associated with methotrexate leading to complete resolution of the inflammatory syndrome, but the clinical symptoms persisted and the bilateral iliac stenosis worsened to 60%. The false lumen of the dissection was not occluded. Therefore surgical treatment of the dissection was indicated. Through a midline laparotomy, the abdominal aorta was exposed: it was thickened. The patient had an aortofemoral bypass (end-to-end proximal anastomosis and end-to-side distal anastomosis on both common iliac arteries) with good immediate and mid-term (6 months) results. The resected part of the aorta was sent to pathology laboratory. |
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| Slide 1 - HE | Slide 1 | Whole section of abdominal aorta H&E x2 | External media adventitia elastic stain x2.5 | Media H&E x40 | Adventitia H&E x10 |
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