Development of heart

Nutrition of the Embryo at Various Stages

of Development
• 1st week: before implantation, deutoplasm (accumulated cytoplasmic
nutrients) of oocyte supplies nutrition
• 2nd week: breakdown products of endometrium (due to implantation)
nourish the embryo by simple diffusion.
• After 3rd week: Maternal blood nourishes embryo through uteroplacental
circulation.
• Increasing nutritional demand of growing embryo initiates development of
cardiovascular system in the third week of intrauterine life.
Establishment of Cardiogenic Area (field)
∙ During 3rd week, cardiac progenitor cells
develop just lateral to the primitive streak.
• Primary heart field/area: These cells
migrate through primitive streak cranially
and form horseshoe-shaped
primitive/primary heart field in the
splanchnopleuric mesoderm by end of
the 3rd week
∙ On formation of head fold, primary heart
field come to lie on dorsal side of
pericardial sac.
Establishment of Cardiogenic Area (field)
• Secondary heart field: On day
20–21, ventral to the pharynx, the
cells of splanchnopleuric
mesoderm form secondary heart
field.
• Most of the heart develops from
primary heart field.
• Secondary heart field forms part
of the right ventricle and outflow
tracts of both the ventricles.
Establishment of Cardiogenic Area (field)
• Formation of heart tube:
Endoderm of primitive pharynx
induces vasculogenesis
(formation of blood cells and
vessels) in the primary heart field.
• Small vessels join to form two
(right and left) endothelial heart
tubes that give rise to the
endocardium
Establishment of Cardiogenic Area (field)
• Splanchnopleuric mesoderm that lies between
heart tube and pericardial cavity forms a
myoepicardial mantle.
• The myoepicardial mantle condenses to form
• Myocardium (cardiac muscles)
• Epicardium (visceral layer of pericardium)

• Somatopleuric mesoderm – forms parietal

layer of pericardium.
• Primitive heart starts beating on the 22nd day
• Blood begins to circulate within the embryo by
24th day
Heart tubes • Formation of single heart
tube: In the third week,
two heart tubes fuse and
form a single heart tube.
∙ Ends of the heart tube
remain bifurcated.
∙ Its cranial end is called
arterial end, whereas
caudal end is called
venous end.
Heart tubes • Dilatations of heart tube:
from cranial to caudal
end:
• 1. Bulbus cordis
• Truncus arteriosus
• Conus cordis
• Bulbar part
• 2. Primitive ventricle
• 3. Primitive atrium
• 4. Sinus venosus
Heart tubes
Ends of heart tube • Arterial end or truncus arteriosus
• Shows right and left limbs
• Continuous with corresponding
dorsal aorta through 1st pair of
pharyngeal arteries
• Soon 6 pairs of pharyngeal arch
arteries connect truncus
arteriosus with dorsal aorta
• All pharyngeal arch arteries run
on either side of foregut
(primitive pharynx)
Venous end of heart tube • Unfused part of sinus
venosus (venous end of
heart tube) forms two horns
(right and left)
• Each horn receives three
veins (from lateral to
medial):
1. Common cardinal vein
from the body wall
2. Umbilical vein from the
placenta
3. Vitelline vein from the yolk
sac.
Acquisition of External Features of Adult Heart

∙ Initially, heart tube is placed longitudinally in pericardial cavity

∙ Heart tube is suspended from dorsal wall of pericardial cavity by a fold of
pericardium called dorsal mesocardium
Acquisition of External Features of Adult Heart

∙ Formation of bulboventricular loop: Bulbus cordis and primitive ventricle grow ventrally
and form bulboventricular loop (U-shaped)
∙ Formation of transverse sinus: Mesocardium connecting bulboventricular loop
disappear to form a gap, that later called transverse sinus
Acquisition of External Features of Adult Heart

∙ Bulbus cordis and primitive ventricle are separated by bulboventricular sulcus that
later disappears and bulbus cordis and ventricle fuse to form a single chamber
Acquisition of External Features of Adult Heart

∙ Bulbus cordis and

primitive ventricle are
separated by
bulboventricular sulcus
that later disappears and
bulbus cordis and
ventricle fuse to form a
single chamber
Acquisition of External Features of Adult Heart

∙ Formation of auricles:
Primitive atrium lies dorsal
to (behind) the truncus
arteriosus. On expansion,
primitive atrium project
forward on either side of
truncus arteriosus as
auricles
 Fate of Heart Tube

Components of heart Fate or derivative

tube
Truncus arteriosus ∙ Ascending aorta
∙ Pulmonary trunk
Bulbus cordis ∙ Conus arteriosus (smooth part of right ventricle)
∙ Aortic vestibule (smooth part of left ventricle)
Primitive ventricle ∙ Trabeculated part of right and left ventricles
Primitive atrium ∙ Trabeculated part of right and left atrium
Sinus venosus ∙ Sinus venarum (smooth part of right atrium)
∙ Coronary sinus
∙ Oblique vein of atrium
Sinus venosus Sinus venosus.

∙ Caudal end of heart tube

∙ Represents venous end of developing heart
∙ Has body, right and left horns
∙ Each horn receives blood from various body
parts as follows:
1. Vitelline vein from yolk sac
2. Umbilical vein from placenta
3. Common cardinal vein (duct of Cuvier) from body
wall

∙ Sinoatrial orifice: Communication between

sinus venosus and primitive atrium
Sinus venosus Changes in left horn
∙ At the level of sinoatrial orifice,
sickle-shaped sino‑atrial fold
develops.
∙ This fold separates left horn from
the primitive atrium, and thus left
horn becomes just a tributary of
the right horn.
Sinus venosus Fate of sino-atrial orifice
∙ Initially, wide oval-shaped,
transversely oriented sino-atrial
orifice lies in the centre.
∙ On formation of sinoatrial fold, size
of the orifice reduces and it
becomes a narrow slit.
∙ Right margins of sinoatrial orifice is
called right venous value, whereas
left margin is called left venous
valve.
Sinus venosus Fate of sino-atrial orifice
∙ Cranial fusion of these valves forms a fold
called septum spurium, whereas caudal
fusion form sinus septum
Sinus venosus Fate of tributaries of sinus
venous
• Right common cardinal vein →
part of superior vena cava
• Right vitelline vein → terminal
part of inferior vena cava
• Left horn of sinus venosus and
left common cardinal vein →
coronary sinus
Sinus venosus Fate of tributaries of sinus
venous
• Left common cardinal vein →
oblique vein of left atrium
• Cephalic part of right posterior
cardinal vein → arch of azygos
vein
• Right umbilical vein and left
vitelline vein → obliterated by 5th
week
• Left common cardinal vein →
obliterated by the 10th week
Sinus venosus Fate of tributaries of
sinus venous
• Left umbilical vein →
proximal part disappears
Distal part drains into inferior
vena cava through ductus
venosus in fetal life and forms
ligamentum teres afterbirth
Changes in Atrioventricular Canal
• AV canal: Communication between
primitive atrium and primitive ventricle
• Two AV cushions: appear as
thickening of subendocardial
mesenchymal cells in ventral and
dorsal wall of AV canal.
• Cardiac jelly forms around the heart
tube during early development form
endocardial cushion and myocardium
Changes in Atrioventricular Canal
Septum intermedium
• AV cushions grow and fuse
to form septum intermedium
• Divide the AV canal into right
and left halves
Development of Interatrial Septum
development of interatrial septum.
Septum primum
• At the end of fourth week, a
septum primum starts developing
from roof of primitive atrium on
left side of septum spurium and
sinoatrial opening
Development of Interatrial Septum
Foramen (ostium) primum
• Septum primum grows toward AV
cushions (septum intermedium) and
becomes sickle-shaped septum.
• A small gap between the growing
septum primum and septum
intermedium is called foramen
primum
Development of Interatrial Septum
Foramen secundum
• Finally, septum primum fuses with AV
cushions that closes foramen primum.
• Simultaneously, a small gap as
foramen secundum appears in the
septum primum
Development of Interatrial Septum
Septum secundum
• A crescent-shaped septum secundum
starts growing from roof of the primitive
atrium between septum spurium and
septum primum

Human Embryology/Yogesh Sontakke/2nd/CBS Publishers

Development of Interatrial Septum
Foramen ovale
• The septum secundum grows toward
septum intermedium and overlaps
foramen secundum
• Overlapping septum secundum
converts foramen secundum into an
oblique passage called foramen ovale

Human Embryology/Yogesh Sontakke/2nd/CBS Publishers

Function of Foramen Ovale
• Foramen ovale is converted into a valve
by opposition of thick-flap of septum
secundum over thin mobile flap of septum
primum
• This valve allows transmission of blood
from right atrium to left atrium, but blood
cannot re-enter the right atrium.
• Thus, foramen ovale allows blood to
bypass pulmonary circulation and shunts
most of the blood from right atrium to left
atrium instead of right ventricle.

Human Embryology/Yogesh Sontakke/2nd/CBS Publishers

Fate of Foramen Ovale
After birth:
• Immediately after birth, pulmonary
circulation begins and volume of blood
returning to the left atrium increases. It
increases pressure in the left atrium and
produces physiological closure of
foramen ovale (later it closes
anatomically).
• After birth, foramen ovale obliterates.
• After birth, in the heart, floor of fossa
ovalis represents septum primum,
whereas the lower free margin of septum
secundum forms the annulus ovalis.
Development of
interatrial septum
Formation of Sinus Venarum
• The right and left horns of sinus venosus
are absorbed into right atrium and results
in segregation of openings of superior
vena cava, inferior vena cava and
coronary sinus from each other
• Superior and inferior limbic bands
(muscular bands) divide right venous
valve into three zones.
Formation of Sinus Venarum

• Right venous valve forms the following structures:

• Crista terminalis
• Valve of inferior vena cava
• Valve of coronary sinus
• Left venous valve fuses with interatrial septum.
• Absorbed part of sinus venosus forms sinus venarum (posterior smooth part of the
right atrium).
Absorption of Pulmonary Veins into Left Atrium

• Dorsal wall of left atrium outpouches to form a primordial pulmonary vein

• Single pulmonary vein divides into two and later into four pulmonary veins
• Part of pulmonary veins get absorbed into dorsal wall of left atrium and openings of
all four pulmonary veins get separated from each other
• Absorbed portion of pulmonary veins forms smooth posterior part of left atrium.
 Development of Right Atrium
Summary (examination guide)
1. Rough trabeculated part of right atrium and right auricle from right half of
primitive atrium.
2. Smooth part of right atrium (sinus venarum) from sinus venosus.
3. Crista terminalis, valve of inferior vena cava, and valve of coronary sinus
develop from right venous valve.
4. A small area of most ventral smooth part develops from right half of
atrioventricular canal.
Development of Left atrium

Summary (examination guide)

1. Anterior rough part of left atrium and left auricle develop from
left half of primitive atrium.
2. Posterior smooth part (between openings of pulmonary veins)
develops from absorption of pulmonary veins.
3. Ventral smooth part develops from left half of atrioventricular
canal.
DEVELOPMENT OF VENTRICLES

A. Rough (inflow) parts: From

primitive ventricles

B. Smooth (outflow) parts

(infundibulum of right ventricle
and aortic vestibule of left
ventricle): From conus cordis
or middle one-third of the
bulbus cordis
Bulbus Cordis bulbus cordis.
• Dilatation of arterial end of
developing heart tube
• Three parts:
• Proximal one-third: fuses with primitive
ventricle to form a bulboventricular
chamber. Proximal one-third later form
the trabeculated part of right ventricle.
• Middle one-third: conical-shaped;
hence, called conus cordis. It forms
outflow parts of both ventricles.
• Distal one-third: It is called truncus
arteriosus – forms pulmonary trunk and
ascending aorta.
FORMATION OF INTERVENTRICULAR SEPTUM
development of interventricular septum.
Muscular part
• A muscular interventricular septum grows from
the floor of bulboventricular cavity and divides
ventricles into two halves.
• It grows toward septum intermedium (AV
cushion) and fuses partially with it.
• Right and left ventricles communicate with
each other through an interventricular foramen
that lies cranial to the muscular interventricular
foramen
FORMATION OF INTERVENTRICULAR SEPTUM
Bulbar part
• From conus cordis, the right and left
bulbar ridges develop and later fuse to
form a bulbar part of the septum.
• Bulbar part grows caudally toward
muscular part of interventricular septum
or conus septum.
• The right and left ventricles
communicate with each other through
the interventricular foramen that lies
between muscular and bulbar parts.
FORMATION OF INTERVENTRICULAR SEPTUM
Membranous part
• By 8th week, the gap of interventricular
foramen is filled by a tissue that proliferates
from right side of AV cushions and right and
left bulbar ridges. It forms a membranous part.
• Presence of interventricular foramen is
essential until separation of bulbus cordis and
truncus arteriosus.
Human Embryology/Yogesh Sontakke/2e/CBS Publishers
Formation of Aorticopulmonary Septum

• In the truncus arteriosus, a spiral septum called aorticopulmonary septum appears

• This septum divides truncus arteriosus (aorticus) into aorta and pulmonary trunk.
Formation of Aorticopulmonary Septum
Steps
• 2 Truncal ridges: appear in
truncus arteriosus that grow and
fuse to form the spiral septum
• Fusion with bulbar septum:
Spiral septum lies in same
plane as that of bulbar septum
and grows to fuse with bulbar
septum. Here, aorta lies behind
the pulmonary trunk
Formation of Aorticopulmonary Septum
Steps
Spiralling of septum:
• Spiral septum separates
aorta and pulmonary trunk in
a spiral course
• Aorta that lies behind the
pulmonary trunk in the lower
part, comes to lie on the
right side and finally anterior
to the pulmonary trunk
Clinical integration
Transposition of great vessels
• Transposition of ascending aorta and pulmonary trunk
• May occur due to reverse spiral attachment of aortic-pulmonary septum.
• In this condition, aorta raises from the right ventricle, whereas pulmonary trunk
from the left ventricle.
• Incidence: 4.8 in 10,000 births.
Persistent (Common) Truncus Arteriosus
• Failure to develop a spiral septum results in common outflow tract for the right
and left ventricles
• Always associated with ventricular septal defect because of failure of contribution
of bulbar ridges to the ventricular septum
Atrial septal defects (ASD)

Atrial septal defects.

• Congenital anomalies that involve defective formation of interatrial septum resulting in an abnormal
communication between right and left atria
Atrial septal defects (ASD)

• Incidence: 6.4 in 10,000 births.

• More common in females (2:1) than in males.
• Cause: Mutation of NKX2.5
Atrial septal defects (ASD): Types

Ostium secundum defects

• Failure of septum secundum to cover septum primum
• May be due to short septum secundum or more absorption of septum primum
• Results in formation of large foramen ovale that does not close after birth
• One of the commonest congenital heart diseases.
Atrial septal defects (ASD): Types

Septum primum defect

• Failure of septum primum to close foramen primum results in septum primum
defect.
Atrial septal defects (ASD): Types

Endocardial cushion defect

• Failure of fusion of septum primum with endocardial cushions (septum
intermedium) results in persistent foramen primum.
Atrial septal defects (ASD): Types

Common atrium/cor triloculare biventriculare

• It is a rare condition with a failure of development of the interatrial septum.
Tetralogy of Fallot/TOF Fallot tetralogy.

TOF is a congenital heart defect.

∙ Components:
1. Pulmonary stenosis
2. Right ventricular or hypertrophy
3. Ventricle septal defect (VSD)
4. Overriding of aorta
Tetralogy of Fallot/TOF Incidence
∙ Most common congenital cyanotic
heart disease
∙ Incidence: 9.6 in 10,000 births
∙ Most common cyanotic heart
disease
∙ Accounts for 6%–10% of all CHDs.
Cause
∙ Mostly unknown
∙ Associates with maternal
phenylketonuria
∙ Responsible genes: VAG1, NKX2-5
Tetralogy of Fallot/TOF
Diagnosis
1. Echocardiography
2. X-ray Chest: Coeur en sabot (boot-like) appearance of a heart.
3. Pathophysiology
Pulmonary stenosis causes concentric right ventricular hypertrophy without cardiac enlargement
↓
Increase in right ventricular pressure
↓
Blood shunts from right ventricle to left ventricle through VSD
↓
Ejection of mixed blood to aorta → cyanosis
↓
Overriding of aorta (disposition)
Probe patency of Foramen Ovale
• Just after birth, foramen ovale closes physiologically by
forced opposition of septum primum on septum
secundum owing to
✔increased blood return to left atrium from lungs.
✔ increased pressure in left atrium.
• Anatomical fusion also takes place soon.
• In about 20% cases, anatomical fusion does not occur
and a small probe can be passed through the foramen
ovale.
 • Rare congenital positional
anomaly of heart.
Ectopia Cordis
• Heart lies exposed on
anterior thoracic wall.
• Cause: Non-union of
sternal halves.
• Treatment: Surgical
repositioning of heart is
not very successful due to
high post-partum mortality
(deaths).
Development of Valves of Heart

Atrioventricular valves
∙ Proliferation of subendocardial mesenchyme forms subendocardial cushions
around atrioventricular canals.
∙ Excavation of these cushions forms cusps of AV valves.
∙ Free margins of these cusps get connected by thin chordae tendinae with
papillary muscles of ventricular wall.
Development of Valves of Heart

Atrioventricular valves
∙ On the right side, there are three cushions - anterior, posterior and septal, whereas on
the left there are two cushions – anterior and posterior. Hence, on right, there is a
tricuspid valve and on left, bicuspid (mitral) valve.
Development of Valves of Heart
Pulmonary and Aortic Valves

∙ In trucus arteriosus, two endocardial cushions appear (right and left). Soon two more (anterior and
posterior) cushions also appear
∙ On separation of the pulmonary trunk from aorta by a spiral septum, right and left cushions divide
into two parts.
∙ Excavation of these cushions form cusps of aortic and pulmonary valves.
∙ Aorta and pulmonary trunk undergo spiral rotation and finally, valves show the following cusps:
- Aortic valve: one anterior and two posterior
- Pulmonary valve: one posterior and two anterior
Development of Conducting System of
Heart
1. SA node: SA node develops during the fifth week of IUL. After
incorporation of sinus venosus into right atrium, SA node comes to lie
near opening of SVC.
2. AV node and bundle of His: AV node and bundle of His are derived from
interatrial septum near opening of the coronary sinus. It develops from
dorsal endocardial cushion of AV canal in 6th week of IUL.
3. Purkinje fibres: Fibres from bundle of His form right and left bundle
branches that get distributed as Purkinje fibres.
Ventricular septal defects (VSD)
∙ VSD is the most common congenital anomaly of the heart.
∙ VSDs are more common in males than in females.
∙ VSD commonly involve the membranous part of interventricular septum.
∙ Incidence isolated VSD is 12 in 10,000 births.
• Embryological basis
Failure of fusion of light and left bulbar ridges with AV cushions
↓
Communication between ventricles
↓
Shunting of blood from left ventricles to right ventricle