Until very recently, examination of the heart was left to the autopsy pathologist.
Now, with sophisticated monitoring and instruments, biopsies can be performed
upon the living patient. Common reasons for a biopsy include the evaluation
of cardiomyopathies, infections, and status of transplanted hearts. These
biopsies may be analyzed at both the light and electron microscopic level.
Cardiac pathologists who specialize in diagnosing heart biopsies usually reside
in major academic or tertiary referral centers.
| CLINICAL VARIANT |
CHARACTERIZATION |
| CHIARI NETWORK |
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Chiari's network: normal anatomic variant or risk factor for arterial
embolic events?
Schneider B, Hofmann T, Justen MH, Meinertz T.
II. Medizinische Abteilung, Allgemeines Krankenhaus St. Georg,
Hamburg, Germany.
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J Am Coll Cardiol 1995 Jul;26(1):203-10 Abstract quote
OBJECTIVES. This study was performed to assess the prevalence of Chiari's
network in patients undergoing transesophageal echocardiography and
to determine whether this anomaly is associated with other cardiac lesions
or is characterized by typical clinical findings.
BACKGROUND. Chiari's network is a congenital remnant of the right
valve of the sinus venosus. It has been found in 1.3% to 4% of autopsy
studies and is believed to be of little clinical consequence.
METHODS. Video recordings of 1,436 consecutive adult patients evaluated
by transesophageal echocardiography over a 30-month period were reviewed
for the presence of Chiari's network. Echocardiographic contrast studies
had been performed in all patients with Chiari's network and were compared
with those of 160 consecutive patients without a Chiari net, serving
as a control group.
RESULTS. Chiari's network was present in 29 of 1,436 patients (prevalence
2%). A frequently associated finding was a patent foramen ovale in 24
(83%) of the 29 patients with Chiari's network versus 44 (28%) of 160
control patients (p < 0.001). Intense right-to-left shunting occurred
significantly more often in patients with Chiari's network than in control
patients (16 [55%] of 29 patients vs. 19 [12%] of 160 control patients,
p < 0.001). Another frequent association was an atrial septal aneurysm
in 7 (24%) of 29 patients. The indication for transesophageal echocardiography
was a suspected cardiac source of arterial embolism in 24 (83%) of 29
patients with a Chiari net, 13 of whom (54%) had recurrent embolic events.
Chiari's network was significantly more common in patients with unexplained
arterial embolism than in patients evaluated for other indications (24
[4.6%] of 522 patients vs. 5 [0.5%] of 914 patients, p < 0.001).
Potential causes for arterial embolism were present in 9 of the 24 patients
with a Chiari net and embolic events (atrial septal aneurysm in 7, cerebrovascular
lesion in 2). In 15 (62%) of 24 patients only a patent foramen ovale
could be identified. Three patients had deep venous thrombosis and pulmonary
embolism at the time of arterial embolism; none had a thrombus detected
within the network.
CONCLUSIONS. In patients undergoing transesophageal echocardiography,
the prevalence of Chiari's network was 2%, which is consistent with
autopsy studies. By maintaining an embryonic right atrial flow pattern
into adult life and directing the blood from the inferior vena cava
preferentially toward the interatrial septum, Chiari's network may favor
persistence of a patent foramen ovale and formation of an atrial septal
aneurysm and facilitate paradoxic embolism. |
| CYSTIC TUMOR OF THE ATRIOVENTRICULAR NODE |
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Cystic tumor of the atrioventricular node of the heart appears to be the heart equivalent of the solid cell nests (ultimobranchial rests) of the thyroid.
Cameselle-Teijeiro J, Abdulkader I, Soares P, Alfonsin-Barreiro N, Moldes-Boullosa J, Sobrinho-Simoes M.
Department of Pathology, Clinical University Hospital, University of Santiago de Compostela, Santiago de Compostela, Spain.
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| Am J Clin Pathol. 2005 Mar;123(3):369-75. Abstract quote |
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We studied a series of 10 solid cell nests (SCNs) of the thyroid and a case of cystic tumor of the atrioventricular node (CTAVN) of the heart and reviewed the literature. The CTAVN and SCNs appeared as cystic and/or solid (squamoid) structures mainly composed of polygonal or oval cells (main cells) admixed with occasional clear cells (neuroendocrine and C cells).
Main cells were immunoreactive for simple and stratified epithelial-type cytokeratins, epithelial membrane antigen, carcinoembryonic antigen, carbohydrate antigen 19.9, p63, bcl-2, and galectin-3. Neuroendocrine (and C) cells were positive for simple-type cytokeratins, carcinoembryonic antigen, calcitonin, chromogranin, synaptophysin, and thyroid transcription factor-1.
Our data support the hypothesis that the CTAVN of the heart and the SCNs of the thyroid are identical structures that represent the same lesional process. The assumption that CTAVN is a ultimobranchial heterotopia fits with the known role of cardiac neural crest cells in cardiovascular development.
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| MALIGNANT TUMORS |
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Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956
to 1986. Bear PA, Moodie DS.
Department of Cardiology, Cleveland Clinic Foundation 44106.
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Chest 1987 Nov;92(5):860-2 Abstract quote
Malignant primary cardiac tumors have uniformly been associated with
poor long-term survival.
We reviewed data on 11 patients with such tumors seen between 1956
and 1986. There were five men and six women, with a mean age at presentation
of 44 years. Nine (82 percent) of 11 patients presented initially with
respiratory symptoms, and 45 percent (five) presented with chest pain.
No patient was asymptomatic on initial presentation. Forty-five percent
(five) of the patients had elevated jugular venous pressure or a systolic
murmur. Cardiomegaly was present in eight (73 percent) of 11 patients.
Nine of ten patients had a hemoglobin level less than 12 g/100 ml, and
30 percent (3/10) presented with thrombocytosis. Seventy percent (eight)
of the patients underwent surgical biopsy, and in 30 percent (three),
surgical excision of the tumor was attempted. Angiosarcoma was the most
frequent type of tumor (four patients), followed by malignant fibrous
histiocytoma (three patients), mesothelioma (two patients), rhabdomyosarcoma
(one patient), and primary lymphoma (one patient). Long-term follow-up
data reveals that ten (91 percent) of 11 patients had died, with a mean
duration from diagnosis until death of 9.7 months.
Newer pathologic techniques for identification of sarcomas, aggressive
surgical resection, and advanced chemotherapy for certain subgroups
may contribute to better survival for patients with these tumors in
the future. In addition, cardiac transplantation may play an important
role in the future for those patients with localized disease. |
| TRANSPLANTATION |
|
Cause of Death and Sudden Cardiac Death After Heart Transplantation
An Autopsy Study
Russell T. Alexander, MD, and Charles Steenbergen, MD, PhD
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Am J Clin Pathol 2003;119:740-748 Abstract quote
Postmortem findings in 39 patients following cardiac transplantation
are presented. Causes of death were right-sided heart failure after
transplantation (6 [15%]), infection (5 [13%]), multisystem organ failure
(4 [10%]), complications of noncardiac surgery (3 [8%]), acute rejection
(3 [8%]), malignant neoplasm (3 [8%]), graft vascular disease (3 [8%]),
preservation procurement injury (3 [8%]), cardiac arrhythmia (2 [5%]),
other (4 [10%]), and unclear (3 [8%]). Seven patients in medically stable
condition died after a sudden cardiac arrest, and these constituted
27% (7/26) of deaths more than 1 month after transplant.
The 7 sudden cardiac arrests were due to graft vascular disease (2
[29%]), acute rejection (2 [29%]), cardiac arrhythmias (2 [29%]), and
hyperkalemia during an exacerbation of acute renal failure (1 [14%]).
In 3 of the 5 patients who died of sudden cardiac arrest not due to
acute rejection, hemodialysis and plasmapheresis were triggers of the
event.
Pulmonary hypertensive arteriopathy was associated with early death
and right-sided heart failure, and 6 of 8 patients with these changes
died perioperatively or postoperatively. |
