09.45.16 – Congenital coronary arterial fistula

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IPCCC Term CONGENITAL CORONARY ARTERIAL FISTULA

IPCCC Code 09.45.16

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with a cardiac chamber or with any segment of the pulmonary circulation.

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[su_carousel source=”media: 6762,6763″ limit=”27″ width=”600″ height=”460″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image # 1: This anterior superior view of an explanted heart with hypoplastic left heart demonstrates multiple, ectatic coronary arteries over the epicardial surface with several communicating arteries between the right and left coronary circulations. These cross the anterior subpulmonary aspect of the right ventricle. The atretic origin of the left main coronary artery is marked with the black star with a long atretic segment extending to the large ectatic portion that bifurcates into the anterior interventricular and circumflex (black arrows) branches. There was aortic valve atresia (not shown). Image #2: A wedge of tissue is removed from the posterior aspect of the same heart seen in Image #1, demonstrating severe mitral valve stenosis and a hypoplastic left ventricle with a large coronary artery to left ventricular fistula.

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09.45.22 – Coronary arterial fistula to the left ventricle

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IPCCC Term CORONARY ARTERIAL FISTULA TO THE LEFT VENTRICLE

IPCCC Code 09.45.22

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with the left ventricle.

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[su_carousel source=”media: 6762,6763″ limit=”27″ width=”600″ height=”460″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image # 1: This anterior superior view of an explanted heart with hypoplastic left heart demonstrates multiple, ectatic coronary arteries over the epicardial surface with several communicating arteries between the right and left coronary circulations. These cross the anterior subpulmonary aspect of the right ventricle. The atretic origin of the left main coronary artery is marked with the black star with a long atretic segment extending to the large ectatic portion that bifurcates into the anterior interventricular and circumflex (black arrows) branches. There was aortic valve atresia (not shown). Image #2: A wedge of tissue is removed from the posterior aspect of the same heart seen in Image #1, demonstrating severe mitral valve stenosis and a hypoplastic left ventricle with a large coronary artery to left ventricular fistula.

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09.42.00 – Anomalous aortic origin or course of coronary artery

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IPCCC Term ANOMALOUS AORTIC ORIGIN OR COURSE OF CORONARY ARTERY

IPCCC Code 09.42.00

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the origin and/or course of a coronary artery is abnormal.

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09-42-00 and 09-46-03 and 09-42-20 The left ventricular outflow tract is open, demonstrating the aortic root with the normal aorta to mitral valve fibrous continuity. The right anterior interventricular and circumflex coronary arteries all take origin from the aorta via separate coronary orifices. The aortic valve is bicuspid with the incomplete interleaflet triangle or raphe marked with the black arrow and the two normal interleaflet triangles marked with the black stars.

[/restab][restab title=”Echocardiogram”]Content for Echocardiogram[/restab][restab title=”Computerized Axial Tomography”]Content for Computerized Axial Tomography[/restab][restab title=”MRI”]MRI[/restab][restab title=”Angiography”]Content for Angiography[/restab][restab title=”Intraoperative Videos”]Content for Intraoperative Videos[/restab][restab title=”Other”]Other[/restab][/restabs]

09.31.35 – Vascular ring: right aortic arch and left arterial duct or ligament

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IPCCC Term VASCULAR RING: RIGHT AORTIC ARCH AND LEFT ARTERIAL DUCT OR LIGAMENT

IPCCC Code 09.31.35

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which continuity of the left aortic arch, pulmonary trunk and right arterial duct (or ligament) encircles the trachea and esophagus.

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09-30-04 and 09-28-15 and 09-31-35 This superior view of the arterial trunks in this heart with concordant ventriculo-arterial connections demonstrates a right aortic arch with mirror image branching of the brachiocephalic arteries and distal origin of the left subclavian artery. Note the left arterial extending from the left subclavian artery to the pulmonary trunk, encircling the trachea (yellow star) and esophagus (red star). The proximal part of the aberrant left subclavian artery represents the distal remnant of the left aortic arch and is typically referred to as the diverticulum of Kommerell.

[/restab][restab title=”Echocardiogram”]Content for Echocardiogram[/restab][restab title=”Computerized Axial Tomography”]Content for Computerized Axial Tomography[/restab][restab title=”MRI”][/restab][restab title=”Angiography”]Content for Angiography[/restab][restab title=”Intraoperative Videos”]Content for Intraoperative Videos[/restab][restab title=”Other”]Other[/restab][/restabs]

09.28.15 – Right aortic arch

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IPCCC Term RIGHT AORTIC ARCH

IPCCC Code 09.28.15

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation of the great vessels in which the aortic arch crosses to the right of the trachea.

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09-30-04 and 09-28-15 and 09-31-35 This superior view of the arterial trunks in this heart with concordant ventriculo-arterial connections demonstrates a right aortic arch with mirror image branching of the brachiocephalic arteries and distal origin of the left subclavian artery. Note the left arterial extending from the left subclavian artery to the pulmonary trunk, encircling the trachea (yellow star) and esophagus (red star). The proximal part of the aberrant left subclavian artery represents the distal remnant of the left aortic arch and is typically referred to as the diverticulum of Kommerell.

[/restab][restab title=”Echocardiogram”]Content for Echocardiogram[/restab][restab title=”Computerized Axial Tomography”]Content for Computerized Axial Tomography[/restab][restab title=”MRI”]MRI[/restab][restab title=”Angiography”]Content for Angiography[/restab][restab title=”Intraoperative Videos”]Content for Intraoperative Videos[/restab][restab title=”Other”]Other[/restab][/restabs]

09.30.04 – Aberrant origin of left subclavian artery

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IPCCC Term ABERRANT ORIGIN OF LEFT SUBCLAVIAN ARTERY

IPCCC Code 09.30.04

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the left subclavian artery arises distal to the right subclavian artery in the setting of a right arch.

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09-30-04 and 09-28-15 and 09-31-35 This superior view of the arterial trunks in this heart with concordant ventriculo-arterial connections demonstrates a right aortic arch with mirror image branching of the brachiocephalic arteries and distal origin of the left subclavian artery. Note the left arterial extending from the left subclavian artery to the pulmonary trunk, encircling the trachea (yellow star) and esophagus (red star). The proximal part of the aberrant left subclavian artery represents the distal remnant of the left aortic arch and is typically referred to as the diverticulum of Kommerell.

[/restab][restab title=”Echocardiogram”]Content for Echocardiogram[/restab][restab title=”Computerized Axial Tomography”]Content for Computerized Axial Tomography[/restab][restab title=”MRI”]MRI[/restab][restab title=”Angiography”]Content for Angiography[/restab][restab title=”Intraoperative Videos”]Content for Intraoperative Videos[/restab][restab title=”Other”]Other[/restab][/restabs]

06.01.09 – Straddling tricuspid valve

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IPCCC Term STRADDLING TRICUSPID VALVE

IPCCC Code 06.01.09

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which the tricuspid subvalvar apparatus has attachments within both ventricles.

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06-01-09 and 06-01-05 The heart has been cut in a plane to demonstrate all four chambers and the inferior half of the heart is shown. There are concordant atrioventricular connections with straddling and overriding of the tricuspid valve, with the greater part of the valve (double-headed red arrow) committed to the right ventricle. The lesser part (double-headed yellow arrow) marks the portion that overrides the interventricular septum. The tricuspid valve has support from tension apparatus within both the right and left ventricles; the portion straddling and overriding the septum supported by a papillary muscle (yellow star) within the left ventricle.

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01.03.01 – Atrial situs inversus

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IPCCC Term ATRIAL SITUS INVERSUS

IPCCC Code 01.03.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which the atrial morphologies and positions are the mirror image of normal.

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01-03-01 This heart with discordant atrioventricular and ventriculoarterial connections is viewed in anatomic position with a window cut in the anterior surface of the right-sided, morphologically right ventricle. The apex points to the right; there is pulmonary stenosis (not seen in this image) and mirror image arrangement of the atrial appendages.

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01.01.24 – Double outlet right ventricle with intact ventricular septum

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IPCCC Term DOUBLE OUTLET RIGHT VENTRICLE WITH INTACT VENTRICULAR SEPTUM

IPCCC Code 01.01.24

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation that is a variant of double outlet right ventricle that is associated with an intact ventricular septum.

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[su_carousel source=”media: 6230,6231″ limit=”27″ width=”460″ height=”600″ responsive=”no” items=”1″ title=”no” centered=”no” mousewheel=”no” autoplay=”0″] Image #1 shows an anterior view of a heart with double outlet right ventricle that has been sectioned along the long axis. The pulmonary trunk is dilated and there is aortic stenosis (red arrow – pulmonary outflow and yellow arrow – aortic outflow). A portion of the tricuspid valve is attached to the caudal limb of the septal band in the subaortic region. The interventricular septum is intact. Image #2 shows the heart viewed from the posterior aspect, with windows removed from the left atrium and ventricle. Both left chambers are hypoplastic with absence of the left atrioventricular connection (yellow bar). The views from the left confirm the integrity of the ventricular septum.

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01.03.03 – Isomerism of left atrial appendages

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IPCCC Term ISOMERISM OF LEFT ATRIAL APPENDAGES

IPCCC Code 01.03.03

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which both atrial appendages have the morphology of a left atrial appendage.

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01-03-03 This view of the base of the heart demonstrates bilateral morphologically left atrial appendages. The pectinate muscles are confined within the tubular appendages bilaterally and both have a narrow junction (double headed red arrows) with the body of the atriums. The vestibules are smooth circumferentially to the crux on both sides. Note the common atrioventricular junction and the persistent left superior caval vein draining to the coronary sinus.

[/restab][restab title=”Echocardiogram”]Content for Echocardiogram[/restab][restab title=”Computerized Axial Tomography”]Content for Computerized Axial Tomography[/restab][restab title=”MRI”]MRI[/restab][restab title=”Angiography”]Content for Angiography[/restab][restab title=”Intraoperative Videos”]Content for Intraoperative Videos[/restab][restab title=”Other”]Other[/restab][/restabs]