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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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.

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IPCCC Term EXTRATHORACIC HEART

IPCCC Code 02.01.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which the heart is at least partially outside of the thorax.

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This is an anterior view of the chest wall in a stillborn fetus at 18 weeks gestation with exteriorization of the heart. The heart is exteriorized because of the deficiency of the surrounding thoraco-abdominal wall. The cardiac apex is to the right, the left atrium is markedly dilated and the persistent left superior caval vein is easily visualized through the thin thoracic membrane. Internal examination revealed tetralogy of Fallot ,with a small right ventricle that did not extend to the apex of the heart. Note the anterior interventricular coronary artery marked with yellow arrows.

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IPCCC Term CONGENITAL TRICUSPID REGURGITATION

IPCCC Code 06.01.25

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac finding in which there is backward flow through the tricuspid valve.

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In Image #1, the right atrium and ventricle are opened in clam-shell fashion and viewed anteriorly in anatomic position. The right atrioventricular junction is guarded by a dysplstic and regurgitant tricuspid valve. All of the leaflets are redundant with a dilated, ballooned appearance in some areas. The tendinous cords are elongated. In image #2, the superior lateral view of the tricuspid valve inlet demonstrates a balloon-like anterior leaflet. The tricuspid valve does not occlude properly the atrioventricular orifice.

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IPCCC Term INTERATRIAL COMMUNICATION THROUGH CORONARY SINUS ORIFICE

IPCCC Code 05.05.03

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is a communication between the left atrium and the coronary sinus allowing interatrial communication through the coronary sinus ostium.

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Image #1 is viewed anteriorly demonstrating the septal surfaces of an opened right atrium and ventricle with a large coronary sinus orifice, acting as an interatrial communication. The oval foramen was restrictive with premature closure. Image #2 is a posterior view of the left side of the same heart. The left atrium and ventricle are hypoplastic and the pulmonary veins are normally connected. The typical horseshoe attachment of the flap valve within the left atrium is marked with red dots. There is a large interatrial communication at the coronary sinus. In this case the interatrial communication is not associated with a persistent left superior caval vein.

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IPCCC Term Divided left atrium (cor triatriatum sinister)

IPCCC Code 05.02.01

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is a partition that divides the left atrium into a posterior chamber that receives some or all of the pulmonary veins and an anterior chamber that communicates with the left atrial appendage and atrioventricular junction (usually the mitral valve).

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The left panel shows a posterior view of an opened left atrial chamber and the pulmonary veins connecting to a posterior chamber. The yellow dashed arrow illustrates the path of the pulmonary venous drainage from the posterior chamber to the left atrial vestibule. The opened mitral valve guards the left atrioventricular junction. In the right panel this postero-superior view shows the partition dividing the posterior left atrial chamber from the atrial vestibule. The pulmonary veins drain into the atrial vestibule via a small opening (yellow dots) in the floor of the partition that divides the left atrium.

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