Otto N. Krogmann, MD

IPCCC Term	ISOMERISM OF LEFT ATRIAL APPENDAGES
IPCCC Code	01.03.03
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which both atrial appendages have the morphology of a left atrial appendage.

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
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiovascular malformation in which the heart is at least partially outside of the thorax.

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	DYSPLASIA OF THE MITRAL VALVE
IPCCCode	06.02.03
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation that includes any structural abnormality of the mitral valvar leaflet(s), commonly consisting of leaflet thickening and restricted mobility.

In Image #1, this postero-superior view of the left atrioventricular junction demonstrates a stenotic, thickened, dysplastic mitral valve. The inferior horn of the flap valve is marked with red dots. In Image #2, the left atrioventricular junction is opened and viewed posteriorly, demonstrating a thickened, dysplastic mitral valve. All of the leaflets are supported by thickened, fused tendinous cords.

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IPCCC Term	CONGENITAL TRICUSPID REGURGITATION
IPCCCode	06.01.25
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac finding in which there is backward flow through the tricuspid valve.

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	OVERRIDING TRICUSPID VALVE
IPCCCode	06.01.05
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which the tricuspid valve annulus lies in part above both the right and left ventricles.

Image #1 the right atrium and ventricle are opened in clam-shell fashion and are viewed anteriorly. The right atrioventricular junction is shown with a perimembranous ventricular septal defect beneath the septal leaflet of the tricuspid valve (red dots). The defect essentially divides the septal leaflet with the portion adjacent to the hinge point of the valve overriding the ventricular septum. Image #2 is a posterior view of the left ventriculo-arterial junction and demonstrates the overriding tricuspid valve with a portion of the tricuspid valve annulus lying in part above the left ventricle. Note that there are no tendinous cords supporting the tricuspid valve within the left ventricle.

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IPCCC Term	INTERATRIAL COMMUNICATION THROUGH CORONARY SINUS ORIFICE
IPCCCode	05.05.03
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	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.

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	Supravalvar or intravalvar mitral ring
IPCCCode	05.02.02
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which a ridge of tissue is attached or integral to the atrial side of the mitral valvar leaflet(s).

Left Panel: This postero-superior view of the left atrioventricular junction shows a prominent, supravalvar ring (yellow dots) associated with the atrial aspect of the mitral valve. This ring is above the annulus or hinge point of the valve. Right Panel: The left atrioventricular junction is opened and viewed posteriorly, demonstrating a thickened mitral valve with thickened fused tendinous cords. Within the funnel of the mitral valve there is a prominent intravalvar ring (yellow dots).

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IPCCC Term	Divided left atrium (cor triatriatum sinister)
IPCCCode	05.02.01
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	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).

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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IPCCC Term	Congenital supravalvar aortic stenosis
IPCCCode	09.16.18
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiovascular malformation with narrowing of the aorta at the level of the sinotubular junction which may extend into the ascending aorta.

Image # 1 is an anterior superior view of the aortic root as it exits the ventricular mass there is marked dilation of the sinuses of Valsalva secondary to a fibrous membrane present internally at the level of the sinotubular junction (red dots).
In image # 2 the left ventricular outflow tract and the aortic root shows subvalvar and supravalvar aortic stenosis in this patient noted to have Shone’s syndrome. The subvalvar stenosis is caused by a prominent fibrous ridge that is circumferential and extends over the aorta to mitral fibrous continuity. At the sinotubular junction (red dots) there is a fibrous shelf accounting for the distal valvar stenosis. Although this is the lesion that is commonly defined as being “supravalvar”, it is in fact a valvar lesion since the “commissures”, or the peripheral attachments of the leaflets, are part of the valvar mechanism. The sinuses of Valsalva are markedly dilated (not seen in this image) with the right and non-adjacent leaflets tethered to the fibrous shelf.

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IPCCC Term	Muscular outlet ventricular septal defect with posteriorly malaligned outlet septum
IPCCCode	07.11.16
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the outlet of the right ventricle, with exclusively muscular borders when viewed from the right ventricle, and in which the muscular outlet septum is malaligned in postero-caudal fashion relative to the apical part of the muscular septum such that there is obstruction to the left ventricular outflow tract.

07-11-16 Muscular outlet ventricular septal defect with posteriorly malaligned outlet septum

This heart has been sectioned along the long axis and is viewed from the left posterior lateral aspect. A ventricular septal defect (interventricular communication) opens to the outlet of the right ventricle, with exclusively muscular borders. The muscular outlet septum is malaligned in postero-caudal fashion relative to the apical part of the muscular septum causing obstruction to the left ventricular outflow tract.

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IPCCC Term	Muscular outlet ventricular septal defect
IPCCCode	07.11.14
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there is a ventricular septal defect with exclusively muscular borders that opens to the outlet of the right ventricle, and in which the muscular outlet septum is aligned with the apical part of the muscular septum.

07-11-14 Muscular outlet ventricular septal defect

In this anterior view, the free wall of the right ventricle has been lifted away to view the septal surface. There is a ventricular septal defect (interventricular communication) that extends into the outlet component with no septal malalignment. In this case the defect is muscular with the postero-caudal arm (yellow dots) of the septal band extending to the ventriculo-infundibular fold, effectively separating the atrioventricular valve from the arterial valve.

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IPCCC Term	Outlet ventricular septal defect
IPCCCode	07.12.00
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the outlet of the right ventricle.

07-12-00 Outlet ventricular septal defect

In this anterior view, the free wall of the right ventricle has been lifted away from the septal surface demonstrating a perimembranous ventricular septal defect (interventricular communication). The defect extends into the outlet component of the right ventricle without malalignment of the outlet septum. The yellow dots mark the fibrous continuity between the tricuspid and aortic valves, rendering this defect perimembranous.

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IPCCC Term	Ventricular septal defect: Hemodynamically insignificant
IPCCCode	07.15.02
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there is one or more small, clinically insignificant ventricular septal defect(s) in the absence of flow-related cardiac chamber dilation or abnormal elevation of pulmonary arterial pressure.

07-15-02 Ventricular septal defect: Hemodynamically insignificant

This heart is viewed from posterior to anterior and has been sectioned to demonstrate all four chambers and the septal structures. The image shows the anterior half of the heart with a small, slit-like, mid septal, muscular ventricular septal defect (interventricular communication). This defect was clinically insignificant and was an incidental finding at autopsy.

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IPCCC Term	Muscular trabecular ventricular septal defect
IPCCCode	07.11.01
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there is a ventricular septal defect within the apical component of the muscular ventricular septum.

07-11-01 Muscular trabecular ventricular septal defect

The free wall of this hypertrophied right ventricle has been lifted rightward to expose the outlet component and is viewed anteriorly. There is a muscular ventricular septal defect (interventricular communication) that opens to the anterior part of the right ventricle, and is antero-superior (or cephalad) to the body of the septomarginal trabeculation, or septal band.

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IPCCC Term	Muscular trabecular ventricular septal defect: Midseptal
IPCCCode	07.11.04
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the right ventricle within the central component of the muscular ventricular septum.

07-11-04 Muscular trabecular ventricular septal defect: Midseptal

The right ventricle is viewed in anterior, anatomic position and has been opened in clam-shell fashion to demonstrate a muscular ventricular septal defect (interventricular communication) in the apical or trabecular component of the interventricular septum.

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IPCCC Term	Left heart obstruction at multiple sites (including Shone syndrome)
IPCCCode	01.01.33
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which there are deep, endothelial lined, blind-ended intramyocardial tunnels communicating with the left ventricular chamber. These occur in the setting of aortic atresia or severe left ventricular outflow tract obstruction, intact ventricular septum and a patent mitral valve.

01-01-33 Left heart obstruction at multiple sites (including Shone syndrome)

This heart has been sectioned along the long axis and is viewed from the left posterior lateral aspect. The left side of the heart demonstrates the features of Shone’s syndrome. There is marked endocardial fibroelastosis of the left atrium with a supravalvar mitral ring (red dots). The mitral valve is extremely stenotic and thickened with fused tendinous cords. There is a parachute mitral valve with a solitary papillary muscle. There is subvalvar aortic stenosis caused by a prominent fibrous ridge that was circumferential along with a prominent, subaortic, septal bulge. At the sinutubular junction there is a supravalvar ridge accounting for the supravalvar stenosis. The aortic valve is bicuspid.

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IPCCC Term	Left ventricular outflow tract obstruction due to atrioventricular valve
IPCCCode	07.08.09
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which the flow through the left ventricular outflow tract is blocked or impeded due to abnormal position or attachments of tricuspid, mitral, or common atrioventricular valvar tissue.

07-09-08 Left ventricular outflow tract obstruction due to atrioventricular valve

In this posterior view, the left ventricle has been opened with the free wall lifted away from the septal surface. The flow through the left ventricular outflow tract, proximal to the aortic valve, is impeded by a tag of tricuspid valvar tissue that extends through the perimembranous ventricular septal defect.

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IPCCC Term	Congenital left ventricular outflow tract obstruction
IPCCCode	07.09.28
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac condition in which the flow through the left ventricular outflow tract (proximal to the valve[s] guarding the outflow from the left ventricle) is blocked or impeded.

07.09.28-Congenital left ventricular outflow tract obstruction

This heart was sectioned along the long axis of the heart and is viewed from left posterior lateral aspect. The left side of the heart demonstrates subaortic stenosis secondary to asymmetric septal hypertrophy in this patient with hypertrophic cardiomyopathy.

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IPCCC Term	Atrioventricular septal defect with ventricular imbalance: dominant left ventricle, hypoplastic right ventricle
IPCCCode	06.07.06
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with the left ventricle significantly larger than the right.

This heart has been sectioned to demonstrate the short axis of the ventricular mass. It is photographed to show the view from the apex looking towards the base. There is an atrioventricular septal defect with a common atrioventricular junction. The common atrioventricular valve is committed predominately to the left ventricle, resulting on left ventricular dominance. The papillary muscles (black stars) are close together within the left ventricle, which shows excessive trabeculations. The zone of apposition between the superior and inferior bridging leaflets is marked with the yellow lines. The right ventricular cavity is hypoplastic as is the right component of the common atrioventricular valve.

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IPCCC Term	CONGENITAL GIANT RIGHT ATRIUM
IPCCCode	05.01.12
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation in which the right atrium is severely dilated. This is an isolated finding not secondary to abnormalities of the tricuspid valve or right ventricle.

Image # 1 demonstrates he anterior anatomic view of a grossly dilated right atrium. Image # 2 shows the heart an anterior view of the heart with an opened right atrium, demonstrating marked dilation of the appendage and atrial chamber. The oval foramen is probe patent without appreciable dilation of the flap valve. There are concordant atrioventricular and ventriculo-arterial connections without abnormalities of the atrioventricular or arterial valves.

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IPCCC Term	ATRIOVENTRICULAR SEPTAL DEFECT AND TETRALOGY OF FALLOT (ATRIOVENTRICULAR CANAL AND TETRALOGY OF FALLOT)
IPCCCode	01.01.20
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation with both an atrioventricular septal defect (atrioventricular canal defect) and tetralogy of Fallot.

01-01-20-atrioventricular-septal-defect-and-tetralogy-of-fallot-atrioventricular-canal-and-tetralogy-of-fallot

This heart is viewed in an anterior anatomic position and has been sectioned to illustrate the left and right ventricular outflow tracts and common atrioventricular valve. The specimen is photographed from the front, simulating the subcostal long-axis echocardiographic plane. There is antero-cephalad deviation of the outlet septum producing severe subpulmonary infundibular and valvar stenosis. The aortic valve overrides the interventricular septum in this heart with tetralogy of Fallot. There is also an atrioventricular septal defect with a common atrioventricular junction and valve.

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IPCCC Term	ATRIOVENTRICULAR SEPTAL DEFECT WITH VENTRICULAR IMBALANCE: DOMINANT RIGHT VENTRICLE, HYPOPLASTIC LEFT VENTRICLE
IPCCCode	06.07.05
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation that is a form of atrioventricular septal defect (atrioventricular canal defect) with the right ventricle significantly larger than the left.

06-07-05-atrioventricular-septal-defect-with-ventricular-imbalance

This heart has been sectioned to demonstrate the short axis of the ventricular mass. It is photographed to show the view from the apex looking towards the base. There is an atrioventricular septal defect with a common atrioventricular junction. The common atrioventricular valve is committed predominately to the right ventricle, producing right ventricular dominance. The left ventricle is hypoplastic, with excessive trabeculations and closely adjacent papillary muscles. The superior and inferior portions of the deficient muscular ventricular septum are marked with the black stars.

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IPCCC Term	ATRIOVENTRICULAR SEPTAL DEFECT WITH VENTRICULAR IMBALANCE: DOMINANT LEFT VENTRICLE, HYPOPLASTIC RIGHT VENTRICLE
IPCCCode	06.07.06
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with the left ventricle significantly larger than the right.

This heart has been sectioned to demonstrate the short axis of the ventricular mass. It is photographed to show the view from the apex looking towards the base. There is an atrioventricular septal defect with a common atrioventricular junction. The common atrioventricular valve is committed predominately to the left ventricle, resulting on left ventricular dominance. The papillary muscles (black stars) are close together within the left ventricle, which shows excessive trabeculations. The zone of apposition between the superior and inferior bridging leaflets is marked with the yellow lines. The right ventricular cavity is hypoplastic as is the right component of the common atrioventricular valve.

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IPCCC Term	AORTIC ROOT DIRECTLY LEFTWARD TO PULMONARY ROOT (SIDE-BY-SIDE)
IPCCCode	02.06.05
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly to the left of the pulmonary root or its remnant.

02-06-05-aortic-root-directly-leftward-to-pulmonary-root-side-by-side

This anterior view demonstrates the heart in anatomic position. The cardiac apex, not shown in this image, is directed to the right. The aortic root is positioned side by side and directly leftward of the pulmonary root.

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IPCCC Term	AORTIC ROOT DIRECTLY RIGHTWARD TO PULMONARY ROOT (SIDE-BY-SIDE)
IPCCCode	02.06.01
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly to the right of the pulmonary root or its remnant.

A portion of the anterior, right ventricular free wall has been removed to demonstrate the relationships of the arterial trunks. The aortic root is positioned directly to the right of the pulmonary trunk in this heart with ventriculo-arterial connection of double outlet right ventricle. The arterial trunks are side by side and both are supported by a complete, subarterial muscular infundibulum. Note the right ventricular hypertrophy and the restrictive ventricular septal defect (interventricular communication).

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IPCCC Term	PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION OF SCIMITAR TYPE
IPCCCode	01.01.16
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiovascular malformation with partial anomalous pulmonary venous connection in which some of the pulmonary veins (usually the right pulmonary veins) connect anomalously to the inferior caval vein (inferior vena cava) or to the right atrium at the insertion of the inferior vena cava.

This heart/lung block is obtained from a patient with Scimitar syndrome. Image # 1 shows the opened right atrium viewed from the front and slightly to the right. A probe has been placed in the solitary pulmonary vein draining the right lung, which opens anomalously into the inferior caval vein immediately above the diaphragm. Although not viewed in this image, the left pulmonary veins drained in the usual fashion to the left atrium.

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IPCCC Term	MESOCARDIA
IPCCCode	02.01.04
ICD-11 Code	PENDING
EACTS-STS Code	PENDING
EACTS-STS Term	PENDING
IPCCC Definition	A congenital cardiovascular malformation in which the heart is central or midline within the thorax.

The anterior portion of the sternum and ribs are removed to demonstrate the in situ position of the heart which lies in the midline with its apex pointing down. The yellow dots mark the limits of the diaphragmatic aspect of the pericardium.

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