Advanced Thoracic Anatomy

Session 2: Development of the heart and congenital cardiac diseases

Anatomical terms reminder:

Dorsal – embryological back
Ventral – embryological front
Cranial – towards head
Caudal – towards “tail”

The heart first develops as a two pair of endothelial tubes (derived from the mesoderm), these form at
day 19 post fertilisation. They grow and swell until they fuse to become a single primitive heart tube.

The primitive heart tube develops in the pericardial cavity and divides in to 5 regions

 Sinus venosum (notice that before the heart folds the sinus venosus is inferior/caudal, after the
folding of the heart tube it forms part of the atria, sitting more superior).
 Atrium
 Ventricle
 Bulbus cordis
 Truncus arteriosus
Cardiac looping

The next step in the development of the heart is cardiac looping. Firstly, the heart tube elongates and as
the tube gets longer it begins to fold.

The direction that the primitive heart folds is of high importance (see Manner et al 2009). For a normal
heart to develop, the tube must loop and fold in a counterclockwise direction (see “development of
heart” image, day 23 and 24 - look at grey arrows). Firstly, a U-shape is formed as a fold appears
between the bulbus cordis and the ventricle with the base of the U pointing rightwards. The tube then
develops an s-shape as the atrium and sinus venosus moves dorsally and cranially.

Formation of Cardiac Septa

Currently we have a heart that has developed as a single long tube. This needs to be divided in to 4
chambers. This is achieved by the formation of the interatrial septum, interventricular septum and a
septum between the atria and ventricles (in which the tricuspid and mitral valve will develop). These
develop simultaneously, but we will discuss each separately to help understanding.
Dividing the atria and ventricles: Boundary tissue between the primitive single atrial cavity and single
ventricle grows out as dorsal and ventral endocardial cushions. The endocardial cushions meet in the
midline, dividing the common atrioventricular orifice into a right (where the tricuspid valve will form)
and left (mitral) orifice. The valves form by further growth and remodeling of the endocardial cushions.

Dividing the ventricles: An interventricular septum develops from the apex up towards the endocardial
cushions.

Dividing the atria: In the atrium a partition, the septum primum, grows down to fuse with the
endocardial cushions. Before fusion is complete a hole appears in the upper part of the septum primum
which is called the foramen secundum.

A second incomplete membrane, the septum secundum, then develops on the right of the septum
primum but is never complete. It has a free lower edge which extends low enough to overlap with the
foramen secundum.

The two overlapping defects in the septa form the valve-like foramen ovale. Blood can flow straight
from the right to the left side of the heart in the fetus.

At birth, where there is an increased blood flow through the lungs and a rise in left atrial pressure, the
septum primum is pushed across to close the foramen ovale.

The septum primum and septum secundum usually fuse, obliterating the foramen ovale and leaving a
small residual dimple (the fossa ovalis).
The sinus venosus joins the atria, becoming the two vena cava on the right and the four pulmonary veins
on the left.

Formation of outflow tract

There is still one area that requires explanation. We have discussed how the heart is divided in to 4
chambers, however we have not discussed how the truncus arteriosus divides to become the aorta and
pulmonary trunk.

Mesenchymal cells in the bulbus cordis begin to proliferate to create 2 ridges that are opposite each
other. These are situated above the developing interventricular septum is. These ridges continue up into
the truncus arteriosus. Over time they grow together to create a septum that divides the truncus
arteriosus in to the future aorta and pulmonary trunk.

However, remember how the aorta arches over the pulmonary trunk in the adult heart? This
configuration is created by the septum forming a 180 degree spiral as seen in the figure above.

How do the 5 areas of the primitive heart relate to the structures found in the adult heart?
Fetal Circulation

We are now going to use our knowledge of development of the heart and use it to help understand
some congenital cardiac diseases. Firstly, let us have a look at blood flow in fetal circulation compared to
after birth:

Fetal circulation: Oxygenated blood from the placenta goes into the right atrium. Some of the blood will
travel through the foramen ovale to the left atrium. This means much less blood will go into the right
ventricle to be pumped to the lungs via the pulmonary trunk. There is also a second opportunity for
blood to avoid going to the lungs, it can travel from the pulmonary trunk into the aorta through a
connection between the two called the ductus arteriosus.

After birth: The lungs expand reducing resistance in the vasculature, meaning that more blood flows in
to the lungs. Increased pressure in the left atrium closes the valve-like foramen ovale. The ductus
arteriosus also closes.

Congenital heart disease

We can divide congenital heart disease into cyanotic and acyanotic:
VSD = Ventricular septal defect

ASD = atrial septal defect

PDA = Patent ductus arteriosus

In acyanotic congenital heart disease, oxygenated blood on the left side of the heart flows back in to the
right side of the heart. The blood “skips the body”, putting more strain on the right side of the heart. It is
important to understand that in ASDs and VSDs, blood moves from the left side to the right side of
heart, as the left side is higher pressure than the right.

In cyanotic congenital heart disease, deoxygenated blood skips the lungs and is recirculated around the
body. In other words, there is a right to left shunt. See the figure above for examples.

Further reading:

As mentioned in the lecture, these articles are at a significantly higher level than our tutorial, so they
make tough reading!

 Clin Anat. 2009 Jan;22(1):21-35. doi: 10.1002/ca.20652. The anatomy of cardiac looping: a step
towards the understanding of the morphogenesis of several forms of congenital cardiac
malformations. Männer J1.

 Orphanet J Rare Dis. 2009. Tetralogy of Fallot. Frederique Bailliard 1 and Robert H
Anderson1,2

 American heart journal, 1979. The normal anatomy of the atrial septum in the human heart. LJ
Sweeney, GC Rosenquist

 Diseases of the Chest. March 1964, Pages 251-261. The Pathologic Anatomy of Tetralogy of
Fallot and Its Variations. MAURICELEVM.D., F.C.C.P.FRIEDRICH A.O.ECKNERM.D.