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Anatomic substrate for preexcitation in idiopathic myocardial hypertrophy with fibroelastosis of the left ventricle

Identifieur interne : 000256 ( Main/Corpus ); précédent : 000255; suivant : 000257

Anatomic substrate for preexcitation in idiopathic myocardial hypertrophy with fibroelastosis of the left ventricle

Auteurs : Saroja Bharati ; Boris Strasberg ; Michael Bilitch ; Habbouba Salibi ; William Mandel ; Kenneth M. Rosen ; Maurice Lev

Source :

RBID : ISTEX:7E61288A324AEF443F2B3EE4043042ABF7D59E68

Abstract

Serial section of the conduction system and both atrioventricular (A-V) rims was performed in three patients who died with known preexcitatlon and idiopathic myocardial hypertrophy with fibroelastosis proved at autopsy. All three patients had type B preexcitation and a leftward and inferior 20 ms vector, suggesting a right free wall anomalous pathway. Patient 1 had no arrhythmia, Patient 2 died suddenly and Patient 3 had recurrent paroxysmal supraventricular tachycardia. Electrophysiologic study in Patients 1 and 3 revealed a bidirectionally conducting anomalous pathway with short refractoriness. Patient 1 had no, and Patient 3 had easily inducible A-V reentrant paroxysmal supraventricular tachycardia. Electrophysiologic study in Patient 3 revealed an anterior right free wall or anteroseptal anomalous pathway, manifested by the shortest stimulus-delta interval with pacing of the right anterior atrium. No electrophysiologic studies were performed in Patient 2.In Patient 1 serial section revealed a large right free wall anomalous pathway with myocardial disarray on the ventricular side of the anomalous pathway. In Patient 2, there were two small anomalous pathways in the right free wall. In Patient 3, no anomalous pathway was found in the right free wall; however, the right atrium was connected to the infundibular septum anterior to the membranous septum. This anomalous pathway had continuity with an anterior A-V nodal-like structure.In summary, (1) all three patients anatomically had a right-sided anomalous pathway (as predicted on electrocardlography in all three and electrophyBiologically in Patients 1 and 3). (2) In Patient 2, myocardial disarray in the Kent bundle may have prevented paroxysmal supraventricular tachycardia. (3) In Patient 3, histologic study revealed an anterior septal anomalous pathway on the right side with an anterior A-V nodal-like structure. Because the anomalous pathway did not show any A-V nodal properties, the significance of this structure is not clear. (4) The relation of the right-sided anomalous pathway to the left-sided fibroelastosis is not known.

Url:
DOI: 10.1016/0002-9149(81)90571-3

Links to Exploration step

ISTEX:7E61288A324AEF443F2B3EE4043042ABF7D59E68

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<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:given-name>Boris</ce:given-name>
<ce:surname>Strasberg</ce:surname>
<ce:degrees>MD, FACC</ce:degrees>
<ce:cross-ref refid="AFF1">
<ce:sup loc="post">1</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF2">
<ce:sup loc="post">2</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF3">
<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:given-name>Michael</ce:given-name>
<ce:surname>Bilitch</ce:surname>
<ce:degrees>MD, FACC</ce:degrees>
<ce:cross-ref refid="AFF1">
<ce:sup loc="post">1</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF2">
<ce:sup loc="post">2</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF3">
<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:given-name>Habbouba</ce:given-name>
<ce:surname>Salibi</ce:surname>
<ce:degrees>MD, FACC</ce:degrees>
<ce:cross-ref refid="AFF1">
<ce:sup loc="post">1</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF2">
<ce:sup loc="post">2</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF3">
<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:given-name>William</ce:given-name>
<ce:surname>Mandel</ce:surname>
<ce:degrees>MD, FACC</ce:degrees>
<ce:cross-ref refid="AFF1">
<ce:sup loc="post">1</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF2">
<ce:sup loc="post">2</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF3">
<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:given-name>Kenneth M.</ce:given-name>
<ce:surname>Rosen</ce:surname>
<ce:degrees>MD, FACC</ce:degrees>
<ce:cross-ref refid="AFF1">
<ce:sup loc="post">1</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF2">
<ce:sup loc="post">2</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF3">
<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:given-name>Maurice</ce:given-name>
<ce:surname>Lev</ce:surname>
<ce:degrees>MD, FACC</ce:degrees>
<ce:cross-ref refid="AFF1">
<ce:sup loc="post">1</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF2">
<ce:sup loc="post">2</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF3">
<ce:sup loc="post">3</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF4">
<ce:sup loc="post">4</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF5">
<ce:sup loc="post">5</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF6">
<ce:sup loc="post">6</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF7">
<ce:sup loc="post">7a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="AFF8">
<ce:sup loc="post">7b</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="FN1">
<ce:sup loc="post"></ce:sup>
</ce:cross-ref>
</ce:author>
<ce:affiliation id="AFF1">
<ce:label>1</ce:label>
<ce:textfn>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF2">
<ce:label>2</ce:label>
<ce:textfn>From the Departments of Pathology of Northwestern University Medical School USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF3">
<ce:label>3</ce:label>
<ce:textfn>From Pritzker School of Medicine, University of Chicago USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF4">
<ce:label>4</ce:label>
<ce:textfn>From the Chicago Medical School, University of Health Sciences USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF5">
<ce:label>5</ce:label>
<ce:textfn>From the Section of Cardiology, Loyola University, Stritch School of Medicine USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF6">
<ce:label>6</ce:label>
<ce:textfn>From Department of Medicine and the Department of Pathology, Abraham Lincoln School of Medicine, University of Illinois USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF7">
<ce:label>7</ce:label>
<ce:textfn>From the Departments of Pediatrics, Medicine and Pathology, Rush Medical College, Chicago, Illinois USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="AFF8">
<ce:label>8</ce:label>
<ce:textfn>From Department of Internal Medicine, Division of Cardiology, University of Southern California School of Medicine, Los Angeles, California USA</ce:textfn>
</ce:affiliation>
<ce:correspondence id="COR1">
<ce:label></ce:label>
<ce:text>Address for reprints: Saroja Bharati, MD, Hektoen Institute for Medical Research, 637 South Wood Street, Chicago, Illinois 60612.</ce:text>
</ce:correspondence>
<ce:footnote id="FN1">
<ce:label></ce:label>
<ce:note-para>Career Investigator and Educator, Chicago Heart Association, Chicago, Illinois.</ce:note-para>
</ce:footnote>
</ce:author-group>
<ce:date-received day="22" month="12" year="1980"></ce:date-received>
<ce:date-revised day="25" month="2" year="1981"></ce:date-revised>
<ce:date-accepted day="3" month="3" year="1981"></ce:date-accepted>
<ce:abstract class="author">
<ce:section-title>Abstract</ce:section-title>
<ce:abstract-sec>
<ce:simple-para view="all" id="simple-para.0010">Serial section of the conduction system and both atrioventricular (A-V) rims was performed in three patients who died with known preexcitatlon and idiopathic myocardial hypertrophy with fibroelastosis proved at autopsy. All three patients had type B preexcitation and a leftward and inferior 20 ms vector, suggesting a right free wall anomalous pathway. Patient 1 had no arrhythmia, Patient 2 died suddenly and Patient 3 had recurrent paroxysmal supraventricular tachycardia. Electrophysiologic study in Patients 1 and 3 revealed a bidirectionally conducting anomalous pathway with short refractoriness. Patient 1 had no, and Patient 3 had easily inducible A-V reentrant paroxysmal supraventricular tachycardia. Electrophysiologic study in Patient 3 revealed an anterior right free wall or anteroseptal anomalous pathway, manifested by the shortest stimulus-delta interval with pacing of the right anterior atrium. No electrophysiologic studies were performed in Patient 2.</ce:simple-para>
<ce:simple-para view="all" id="simple-para.0015">In Patient 1 serial section revealed a large right free wall anomalous pathway with myocardial disarray on the ventricular side of the anomalous pathway. In Patient 2, there were two small anomalous pathways in the right free wall. In Patient 3, no anomalous pathway was found in the right free wall; however, the right atrium was connected to the infundibular septum anterior to the membranous septum. This anomalous pathway had continuity with an anterior A-V nodal-like structure.</ce:simple-para>
<ce:simple-para view="all" id="simple-para.0020">In summary, (1) all three patients anatomically had a right-sided anomalous pathway (as predicted on electrocardlography in all three and electrophyBiologically in Patients 1 and 3). (2) In Patient 2, myocardial disarray in the Kent bundle may have prevented paroxysmal supraventricular tachycardia. (3) In Patient 3, histologic study revealed an anterior septal anomalous pathway on the right side with an anterior A-V nodal-like structure. Because the anomalous pathway did not show any A-V nodal properties, the significance of this structure is not clear. (4) The relation of the right-sided anomalous pathway to the left-sided fibroelastosis is not known.</ce:simple-para>
</ce:abstract-sec>
</ce:abstract>
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<title>Anatomic substrate for preexcitation in idiopathic myocardial hypertrophy with fibroelastosis of the left ventricle</title>
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<title>Anatomic substrate for preexcitation in idiopathic myocardial hypertrophy with fibroelastosis of the left ventricle</title>
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<namePart type="given">Saroja</namePart>
<namePart type="family">Bharati</namePart>
<namePart type="termsOfAddress">MD, FACC</namePart>
<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
<description>Address for reprints: Saroja Bharati, MD, Hektoen Institute for Medical Research, 637 South Wood Street, Chicago, Illinois 60612.</description>
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<namePart type="termsOfAddress">MD, FACC</namePart>
<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
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<name type="personal">
<namePart type="given">Michael</namePart>
<namePart type="family">Bilitch</namePart>
<namePart type="termsOfAddress">MD, FACC</namePart>
<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Habbouba</namePart>
<namePart type="family">Salibi</namePart>
<namePart type="termsOfAddress">MD, FACC</namePart>
<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">William</namePart>
<namePart type="family">Mandel</namePart>
<namePart type="termsOfAddress">MD, FACC</namePart>
<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
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<name type="personal">
<namePart type="given">Kenneth M.</namePart>
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<namePart type="termsOfAddress">MD, FACC</namePart>
<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
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<affiliation>From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research USA</affiliation>
<description>Career Investigator and Educator, Chicago Heart Association, Chicago, Illinois.</description>
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<abstract lang="en">Serial section of the conduction system and both atrioventricular (A-V) rims was performed in three patients who died with known preexcitatlon and idiopathic myocardial hypertrophy with fibroelastosis proved at autopsy. All three patients had type B preexcitation and a leftward and inferior 20 ms vector, suggesting a right free wall anomalous pathway. Patient 1 had no arrhythmia, Patient 2 died suddenly and Patient 3 had recurrent paroxysmal supraventricular tachycardia. Electrophysiologic study in Patients 1 and 3 revealed a bidirectionally conducting anomalous pathway with short refractoriness. Patient 1 had no, and Patient 3 had easily inducible A-V reentrant paroxysmal supraventricular tachycardia. Electrophysiologic study in Patient 3 revealed an anterior right free wall or anteroseptal anomalous pathway, manifested by the shortest stimulus-delta interval with pacing of the right anterior atrium. No electrophysiologic studies were performed in Patient 2.In Patient 1 serial section revealed a large right free wall anomalous pathway with myocardial disarray on the ventricular side of the anomalous pathway. In Patient 2, there were two small anomalous pathways in the right free wall. In Patient 3, no anomalous pathway was found in the right free wall; however, the right atrium was connected to the infundibular septum anterior to the membranous septum. This anomalous pathway had continuity with an anterior A-V nodal-like structure.In summary, (1) all three patients anatomically had a right-sided anomalous pathway (as predicted on electrocardlography in all three and electrophyBiologically in Patients 1 and 3). (2) In Patient 2, myocardial disarray in the Kent bundle may have prevented paroxysmal supraventricular tachycardia. (3) In Patient 3, histologic study revealed an anterior septal anomalous pathway on the right side with an anterior A-V nodal-like structure. Because the anomalous pathway did not show any A-V nodal properties, the significance of this structure is not clear. (4) The relation of the right-sided anomalous pathway to the left-sided fibroelastosis is not known.</abstract>
<note>This study was supported by Grant HL 07605-18, HL 18794 and HL 23566 from the National Institutes of Health, National Heart, Lung, and Blood Institute, Bethesda, Maryland.</note>
<note type="content">Section title: Clinical study</note>
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