Pathologt of The Heart

THE HEART
MMUVWIMI
CONGENITAL HEART DISEASE (CHD)
Congenital heart disease is the abnormality of the heart present
from birth.
The incidence is higher in premature infants.
CAUSED OF CHD
Unknown in majority of cases.
Multifactorial inheritance involving genetic and environmental influences.
Other factors
Rubella infection to the mother during pregnancy,
Drugs taken by the mother
Heavy alcohol drinking by the mother
CLASSIFICATION
Shunts (left-to-right or right-to-left)
Defects causing obstructions to flow.
Complex anomalies involving combinations of shunts and obstructions are also often present.
MALPOSITIONS OF THE HEART
Dextrocardia is the condition when the apex of the heart points to the right side of the chest.
May be accompanied by situs inversus so that all other organs of the body are also transposed
SHUNTS (CYANOTIC CONGENITAL
HEART DISEASE)
A shunt may be:
Left-to-right side
Right-to-left side of the circulation.

LEFT-TO-RIGHT SHUNTS (ACYANOTIC OR
LATE CYANOTIC GROUP)
Shunting of blood from left-to right side of the heart
Cause volume overload on the right heart
Produces pulmonary hypertension and right ventricular hypertrophy.
The important conditions included in this category are described below:
VENTRICULAR SEPTAL DEFECT (VSD)
Involves a hole in the septum between ventricles
VSD is the most common congenital anomaly of the heart
The condition is recognised early in life.
The smaller defects often close spontaneously
Larger defects remain patent and produce significant effects.
ATRIAL SEPTAL DEFECT (ASD)
Involves a hole in the septum between Atria
The condition remains unnoticed in infancy and childhood till pulmonary hypertension is
induced causing late cyanotic heart disease and right-sided heart failure.
PATENT DUCTUS ARTERIOSUS (PDA)
The ductus arteriosus is a normal vascular connection between the aorta and the bifurcation of
the pulmonary artery.
Normally, the ductus closes functionally within the first or second day of life. Its persistence
after 3 months of age is considered abnormal.
The cause for patency of ductus arteriosus is not known but possibly it is due to continued
synthesis of PGE2 after birth
Occurance may be as a result of isolated defect
May also be associated with other anomalies like VSD, coarctation of aorta and pulmonary or
aortic stenosis.
RIGHT-TO-LEFT SHUNTS (CYANOTIC
GROUP)
In conditions where there is shunting of blood from right side to the left side of the heart,
There is entry of poorly-oxygenated blood into systemic circulation resulting in early cyanosis.
TETRALOGY OF FALLOT
Tetralogy of Fallot is the most common cyanotic congenital heart disease
The severity of the clinical manifestations is related to two factors:
extent of pulmonary stenosis
The size of VSD.
Accordingly, there are two forms of tetralogy:
cyanotic and
acyanotic
TRANSPOSITION OF GREAT ARTERIES
The term transposition is used for complex malformations as regards position of:
The aorta,
Pulmonary trunk,
Atrioventricular orifices
and the position of atria in relation to ventricles
PERSISTENT TRUNCUS ARTERIOSUS
Truncus arteriosus (PTA) is a rare anomaly.
In PTA, the arch that normally separates the aorta from the pulmonary artery fails to develop.
Results in a single large common vessel receiving blood from the right as well as left ventricle.
There is often an associated VSD.
There is left-to-right shunt and frequently early systemic cyanosis.
The prognosis is generally poor.
TRICUSPID ATRESIA AND STENOSIS
Tricuspid atresia and stenosis are rare anomalies.
There is often associated pulmonary stenosis or pulmonary atresia.
In tricuspid atresia, there is absence of tricuspid orifice and instead there is a dimple in the floor
of the right atrium.
In tricuspid stenosis, the tricuspid ring is small and the valve cusps are malformed.
In both the conditions, there is often an interatrial defect through which right-to-left shunt of
blood takes place.
Children are cyanotic since birth and live for a few weeks or months.
OBSTRUCTIONS
(OBSTRUCTIVE CONGENITAL HEART DISEASE)
Congenital obstruction to blood flow may result from:
Obstruction in the aorta due to narrowing (coarctation of aorta)
Obstruction to outflow from the left ventricle (aortic stenosis and atresia)
Obstruction to outflow from the right ventricle (pulmonary stenosis and atresia).
COARCTATION OF AORTA
The word ‘coarctation’ means contracted or compressed.
Coarctation of aorta is localized narrowing in any part of aorta
Constriction is more often just distal to ductus arteriosus (postductal or adult),
Or occasionally proximal to the ductus arteriosus (preductal or infantile type) in the region of
transverse aorta:
AORTIC STENOSIS AND ATRESIA
The most common congenital anomaly of the aorta is bicuspid aortic valve which does not have
much functional significance but predisposes it to calcification.
Congenital aortic atresia is rare and incompatible with survival.
Aortic stenosis may be acquired (e.g. in rheumatic heart disease, calcific aortic stenosis) or
congenital.
PULMONARY STENOSIS AND ATRESIA
Isolated pulmonary stenosis and atresia do not cause cyanosis and hence are included under
acyanotic heart diseases.
ISCHAEMIC HEART DISEASE
Ischaemic heart disease (IHD) is defined as:
Acute
Chronic
Cardiac disability arising from imbalance between the myocardial supply and demand for
oxygenated blood.
Since narrowing or obstruction of the coronary arterial system is the most common cause of
myocardial anoxia, the alternate term ‘coronary artery disease (CAD)’ is used synonymously with
IHD.
ETIOPATHOGENESIS OF IHD
IHD is caused by disease affecting the coronary arteries
the most prevalent being atherosclerosis accounting for more than 90% cases
EFFECTS OF MYOCARDIAL ISCHAEMIA
Development of lesions in the coronaries is not always accompanied by cardiac disease.
Depending upon the suddenness of onset, duration, degree, location and extent of the area affected by myocardial
ischaemia, the range of changes and clinical features may range from an asymptomatic state at one extreme to
immediate mortality at another:
Asymptomatic state
Angina pectoris (AP)
Acute myocardial infarction (MI)
Chronic ischaemic heart disease (CIHD)/Ischaemic cardiomyopathy/ Myocardial fibrosis
Sudden cardiac death
The term acute coronary syndromes include a triad of acute myocardial infarction, unstable angina and sudden
cardiac death.
HYPERTENSIVE HEART DISEASE (HHD)
Hypertensive heart disease or hypertensive cardiomyopathy is the disease of the heart resulting
from systemic hypertension of prolonged duration
Manifesting by left ventricular hypertrophy
Hypertension predisposes to atherosclerosis.
Most patients of hypertensive heart disease have advanced coronary atherosclerosis and may
develop progressive IHD.
PATHOGENESIS
Pathogenesis of left ventricular hypertrophy (LVH) which is most commonly caused by systemic
hypertension is described here.
Stimulus to LVH is pressure overload in systemic hypertension.
Both genetic and haemodynamic factors contribute to LVH.
Aggressive control of hypertension can regress the left ventricular.

VALVULAR DISEASES AND DEFORMITIES
Valvular diseases - congenital and acquired diseases which cause valvular deformities.
Many of them result in cardiac failure.
Rheumatic heart disease is the most common form of acquired valvular disease.
The valvular deformities may be of 2 types:
Stenosis
Insufficiency:
Stenosis is the term used for failure of a valve to open completely during diastole resulting in
obstruction to the forward flow of the blood.
Insufficiency or incompetence or regurgitation is the failure of a valve to close completely during
systole resulting in back flow or regurgitation of the blood.
Various acquired valvular diseases that may deform the heart valves are listed below:
1. RHD, the commonest cause
2. Infective endocarditis
3. Non-bacterial thrombotic endocarditis
4. Libman-Sacks endocarditis
5. Syphilitic valvulitis
6. Calcific aortic valve stenosis
7. Calcification of mitral annulus
8. Myxomatous degeneration (floppy valve syndrome)
9. Carcinoid heart disease.
CARDIOMYOPATHY
Disease of the heart muscle but the term was originally coined to restrict its usage to myocardial
disease of unknown cause.
The WHO definition of cardiomyopathy also excludes heart muscle diseases of known etiologies.
This controversy is resolved by classifying all cardiomyopathies into two broad groups:
A) Primary cardiomyopathy i.e. heart muscle disease with unknown underlying cause.
B) Secondary cardiomyopathy i.e. myocardial disease with known underlying cause.
Based on these principles, a classification of primary cardiomyopathy and its subtypes is
presented in
A. PRIMARY CARDIOMYOPATHY
This is a group of myocardial diseases of unknown cause. It is subdivided into the following 3
pathophysiologic categories
1. Idiopathic dilated (congestive) cardiomyopathy.
2. Idiopathic hypertrophic cardiomyopathy.
3. Idiopathic restrictive or obliterative or infiltrative cardiomyopathy.
Idiopathic Dilated (Congestive) Cardiomyopathy
This type of cardiomyopathy is characterised by gradually progressive cardiac failure along with
dilatation of all the four chambers of the heart.
The condition occurs more often in adults and the average survival from onset to death is less
than 5 years.
ETIOLOGY Though the etiology is unknown, a few hypotheses based on associations with the
following conditions have been proposed:
i) Possible association of viral myocarditis (especially coxsackievirus B) with dilated
cardiomyopathy, due to presence of viral nucleic acids in the myocardium, has been noted.
ii) Association with toxic damage from cobalt and chemotherapy with doxorubicin and other
anthracyclines is implicated in some cases.
iii) Inherited mutations have been implicated due to occurrence of disease in families.
iv) Chronic alcoholism has been found associated with dilated cardiomyopathy.
v) Peripartum association has been observed in some cases. Poorly-nourished women may
develop this form of cardiomyopathy within a month before or after delivery (peripartum
cardiomyopathy).
Idiopathic Hypertrophic Cardiomyopathy
This form of cardiomyopathy is known by various synonyms like asymmetrical hypertrophy,
hypertrophic subaortic stenosis and Teare’s disease.
The disease occurs more frequently between the age of 25 and 50 years.
It is often asymptomatic but becomes symptomatic due to heavy physical activity causing
dyspnoea, angina, congestive heart failure and even sudden death
Idiopathic Restrictive (Obliterative or Infiltrative) Cardiomyopathy
This form of cardiomyopathy is characterised by restriction in ventricular filling due to reduction
in the volume of the ventricles.
PERICARDIAL DISEASES
Diseases of the pericardium are usually secondary to, or associated with, other cardiac and
systemic diseases.
These are discussed under 2 headings:
I. Pericardial fluid accumulations
II. Pericarditis
PERICARDIAL FLUID ACCUMULATIONS
Accumulation of fluid in the pericardial sac may be watery or pure blood.
2 types:
Hydropericardium (pericardial effusion)
Haemopericardium
PERICARDITIS
Pericarditis is the inflammation of the pericardial layers and is generally secondary to diseases in
the heart or caused by systemic diseases.
Pericarditis is classified into acute and chronic types, each of which may have several etiologies.

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THE HEART

Defects causing obstructions to flow.

Right-to-left side of the circulation.

Stimulus to LVH is pressure overload in systemic hypertension.

Both genetic and haemodynamic factors contribute to LVH.

Aggressive control of hypertension can regress the left ventricular.

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