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Anatomy Atlases: Illustrated Encyclopedia of Human Anatomic Variation: Opus II: Cardiovascular System: Arteries: Heart

Illustrated Encyclopedia of Human Anatomic Variation: Opus II: Cardiovascular System: Arteries


Ronald A. Bergman, PhD
Adel K. Afifi, MD, MS
Ryosuke Miyauchi, MD

Peer Review Status: Internally Peer Reviewed

Congenital cardiac anomalies are seen in 0.6% of live births and in 2.0% of stillbirths.

Common cardiac anomalies are: a) atresia or stenosis of the pulmonary trunk or aorta, b) defective closure of septal openings, c) defective closure of the ductus arteriosus, d) reverse rotation of the heart and, e) mirror imaging. Situs inversus are examples of d and e.

Incomplete closure of the foramen ovale, "probe patency," is common (~25% of adult hearts) and may be regarded as a normal variant rather than an abnormality. The foramen is closed physiologically but patent anatomically. In one study of 2648 subjects, probe patency was found in 23.1%. There is virtually no sex difference (25.5% occurrence in females and 21.4% in male subjects). Schaefer, et al. (1915), reported the foramen ovale to be patent in 24.2% of adult males and 29% of females; in children under one year it was patent in 94%, and between one and five years in 52%. The most common arrangement is an oblique slit, from a pinhead size to 1 cm. in diameter, under the greatest convexity of the anulus. This slit may be blind, even being 2 cm. in length or it may open into the left atrium . There may be a blind slit on the left side and none on the right side; less commonly there are two blind slits. Nañagas (1921) found three types of foramina in a study of 107 subjects: circular (the smallest type) (53.4%), Ovale (27.6%) and triangular (the largest type) (20%).

A foramen may exist in the atrial septum above and behind the fossa ovalis.and may co-exist with a persistent foramen ovale. Deficiencies in the septum below and in front of the fossa ovalis are more common, but are still rare. These represent persistences of the foramen primum. The foramen lies in front of the anterior limb of the anulus, its anterior boundry being formed by the upper parts of the aortic cusp of the mitral valve and the anterior and septal cusps of the tricuspid valve. The foramen is interatrial. It is usually present when the right pulmonary veins open into the superior vena cava.

The fossa ovalis varies in size, but seldom exceeds 3.5 cm. in its maximum diameter.

The coronary sinus may be doubled.

The coronary sinus may be absent or closed or drain into the left atrium (via a left superior vena cava, veing of Marhall). See coronary sinus below.

A heart with a more or less bifid apex and three-chambered hearts in adult subjects have been reported.

Cor triatrium, a heart with a doubled left atrium, has been reported.

The heart valves may be composed of accessory cusps which vary in size. The posterior (inferior) cusp may be divided in the right atrioventricular valve, and the bicuspid valve of the left ventricle may have an anterior or posterior cusp or both.

The aortic and pulmonary valves may present with two cusps in front and one behind. The aortic valve may be composed of two to four cusps rather than the usual three. The mitral valve may be doubled.

The (right) superior vena cava may be joined to the left atrium. Other anomalous vascular connections of the heart will be found in subsequent sections.

The heart normally weighs from 280 to 389 grams in males and 250 to 384 in females although these values are bound to change in our modern society.

Heterotopic renal tissue has been reported in the heart.

The pericardium may be partially or completely absent (frequency 2/13,000 necropsies or in about 0.015%). A cases of agenesis was first described by Columbus in 1559.

The incisura apicis cordis may be so deep that the apex appears doubled or bifid; this occured in three of 106 cases (Théremin) and in another study, 4 of 631 cases (Abbot). The incisura is the junction point of the anterior and posterior longitudinal grooves that end in a small depression at the right of the apex.of the heart.

The diameter of the the superior vena cava varies from 18 to 22 mm., and the inferior vena cava is larger and varies between 27 to 36 mm. at their junction with the right atrium.

The orifice of the inferior vena cava is partially covered by a semilunar fold, the Eustachian valve. The valve in the adult heart is varying in height and in a very few instances exceeds 1 cm. Very often it is perforated and it may present appearing as a network or a delicate trellis.

The circular opening of the coronary sinus is flanked laterally by the Thebesian valve, or the valve of the coronary sinus, which is very variable in size and shape. Most commonly it is smaller than the coronary opening and is semilunar in shape. Very frequently it is perforated or in the form of a network but may also appear as a few delicate strands attached to the lateral surface of the Eustachian valve and bridging across the the sub-Eustachian diverticulum.

The valve of the coronary sinus has been seen doubled. The opening of the coronary sinus is occasionally on the bottom of a small fossa.

The level of the orifices of the coronary arteries in the sinuses of Valsalva varies in both vertical and horizontal directions. Most commonly they lie at the level of the free edge of the cusp, but they may be above this level and it has been found to be below it in some instances. While most commonly at the center of the sinus they are most often found nearer its anterior margin.

The two coronary arteries have been seen arising from a common trunk or both arise from one sinus of Valsalva.

Not infrequently the number of coronary arteries is increase to three and in a few instances four have been found. The supplementary vessels are generally small and may be overlooked but Symmers found 40 in 100 hearts examined. They arise from the aorta near the main coronary trunk but appear to have acquired an independent origin. They occur on both sides, but are more frequent on the right side.

In very rare instances an additional coronary artery has been found arising from the pulmonary trunk.

Congenital Variations of the Heart with the Maximum Recorded Life Span based on 1000 cases in the Bibliography of M.E. Abbott. (Bauer, D. deF. and E.C. Astbury. (1944) Am. Heart J. 27:688-732. (slightly modified and updated by present authors).

Disease or Condition

No. Cases

Maximum Life Span Recorded

1. Anomalies of Pericardium

a. Pericardial defects


75 yrs.

b. Pericardial diverticulum


52 yrs.

2. Displacements of the Heart

a. Ectopis Cordis


15 mo

b. Isolated dextrocardia

i. Without inversion chambers


49 yrs.

ii. With inversion chambers


33 yrs.

c. Dextrocardia with situs inversus


58 yrs.

d. Incomplete heterotaxy


6.5 yrs.

e. Dextroposition cordis


41 yrs.

3. Anomalies of the Heart as a Whole

a. Bifid apex


Adult (40 yrs)

b. Diverticulum


7 mo.

c. Primary congenital hypertrophy


14 mo.

d. Congenital rhabdomyoma


35 yrs.

e. Congenital heart block


20 mo.

4. Anomalous Septa or Chordae

a. Anomalous septa

i. In left auricle


48 yrs.

ii. In right auricle


33 yrs.

iii. In vertricles


39 yrs.

b. Anomalous Chordae

i. In left auricle


84 yrs.

ii. In right auricle


69 yrs.

iii. In ventricles


76 yrs.

iv. In aorta


76 yrs.

5. Defects of Interauricular Septum

a. Patent foramen ovale


62 yrs.

b. Defects auricular septum above


64 yrs.

c. Defects auricular septum below


46 yrs.

d. Multiple defects, auricular septum


52 yrs.

e. Premature closure foramen ovale


32 yrs.

6. Defects of Interventricular Septum

a. At base without dextroposition


44 yrs.

b. Defects elsewhere or multiple


79 yrs.

c. Aneurysms of pars membranacea


60 yrs.

7. Complete Defects of Cardiac Septa

a. Cor trioculare biventriculare


31 yrs. (39, 56 yrs.)

b. Cor trioculare biatriatum


24 yrs. (35, 56 yrs.)

c. Cor bioculare


8.5 mo.

d. Incomplete double heart


4.75 mo.

8. Defects in Aortic Septum

a. Persistent truncus (complete defect)


25 yrs.

b. Communication between aorta and pulmonary artery


48 yrs.

c. Congenital aneurysm of right aortic sinus


41 yrs.

9. Transposition of Arterial Trunks

a. Dextroposition of aorta

i. Aorta from left ventricle, ventricular septum entire


39 yrs.

ii. Aorta from both ventricles


48 yrs.

iii. Aorta from right ventricle


13 mo.

iv. Aorta from right ventricle, double conus



b. Complete transposition

i. Closed ventricular septum


11 yrs.

ii. Defect of ventricular septum


4 yrs.

c. Partial transposition


20 yrs.

d. Corrected transposition


24 yrs.

10. Pulmonary Stenosus

a. With closed septa


45 yrs.

b. With patent foramen ovale, closed ventricular septum


57 yrs.

c. With closed foramen ovale, defect ventricular septum


60 yrs.

d. With patent foramen ovale and defect ventricular septum


28 yrs.

11. Pulmonary Atresia

a. With closed ventricular septum


20 yrs.

b. With closed foramen ovale, defect ventricular septum


29 yrs.

c. With patent foramen ovale, defect ventricular septum


6 yrs.

12. Pulmonary Insufficiency or Dilatation

a. Valvular insufficiency


64 yrs.

b. Congenital dilatation pulmonary artery


59 yrs.

13. Aortic Stenosis and Atresia

a. Subaortic stenosis


58 yrs.

b. Aortic stenosis


24 yrs.

c. Aortic atresia


25 days.

14. Anomalies of Semilunar Cusps

a. Supernumerary cusps

i. Of pulmonary valve



ii. Of aortic valve


41 yrs.

b. Reduced number

i. Bicuspid pulmonary valve


20 yrs.

ii Bicuspid aortic valve


68 yrs.

15. Tricuspid and Mitral Stenoses

a. Tricuspid stenosis


28 yrs.

b. Tricuspid atresia


56 yrs.

c. Mitral stenosis


27 yrs.

d. Mitral atresia


3.5 yrs.

16. Anomalies of Auriculoventricular Cusps

a. Double orifices

i Of tricuspid orifice


43 yrs.

ii Of mitral orifice


71 yrs.

b. Insufficiency or defect

i Of tricuspid valve


20 yrs.

ii Of mitral valve


14 yrs.

17. Patent Ductus Arteriosus

a. Simple patency


66 yrs.

b.So-called aneurysm


40 yrs.

18. Coarctation of the Aorta

a Adult type.


70 yrs.

b Infantile type


9 mo.

c. Pulmonary artery forms descending aorta


5.5 yrs.

19. Hypoplasia of Aortic Arch


20 yrs.

20. Anomalies of the Aortic Arch

a. Double aortic arch


87 yrs.

b. Right aortic arch


61 yrs.

c. Right subclavian from descending aorta


44 yrs.

d. Left subclavian from ductus arteriosus or pulmonary artery


5 yrs.

e. Common brachiocephalic trunk


38 yrs.

21. Anomalous Coronary Arteries

a. Origin from pulmonary artery


61 yrs.

b. Right coronary into coronary sinus


54 yrs.

c. Aneurysm right coronary


46 yrs.

22. Anomalous Pulmonary Arteries


65 yrs.

23. Anomalies of Great Veins

a. Of systemic vein (persistent left superior vena cava)


61 yrs

b. Of pulmonary veins


86 yrs.

24. Congenital Arteriovenous Aneurysm


35 yrs.

Note of Interest: According to Hyrtl, the name valve was first used for the heart valves by Benedictus; the Greeks named them hymenes, while Vesalius named them membranae.

Galen compared the arterial valves to the letter C in shape, while Heister introduced the name semilunar for them. The Greeks named the atrio-ventricular valves triglochines, and Galen trisulcas, because of their resemblance to a three-pointed spear head. Vesalius suggested the name mitral valve for the left atrio-ventricular valve because of its resemblance to a Bishop's mitre (hat). From Schaefer, et al., Quain's Anatomy, 1915.

Image 403


Image 253, Image 256, Image 405

Mitral Valves

Image 114, Image 161, See Image 405, Image 440,

Ductus Arteriosus

Image 103


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