Congenital Anomalies of the heart

CONGENITAL ANOMALIES OF

THE HEARTDR RANIA GABR

OBJECTIVES Discuss the congenital anomalies related to the

heart development.

Congenital Heart Defect

Acyanotic Cyanotic

volume load pressure load ↑ pulmonary flow ↓ pulmonary flow

left-to-right shunts obstr. ventric. outflow

• atrial septal defect • pulmonary valve stenosis transpos. of gr. Vessels tetralogy of Fallot

• ventricular septal defect aortic valve stenosis single ventricle pulmonary atresia

• AV canal coarctation of aorta truncus arteriosus tricuspid atresia

• patent ductus arteriosus total anomalous pulm. total anomalous pulm.

return w/o obstruction return w/ obstruction

Atrial Septal defects:

Septum Primum Defect:Defect in the interatrial septumDue to absence of the septum Primum (Patent foramen ovale)

Septum Secondum Defect:Defect in the interatrial septumDue to absence of the septum Secondum (Patent foramenSecondum)

Complete absence of the interatrial septum (Core Triloculare Biventriculare):

There is complete absence of both septum primum and septum secondum. i.e. there is a common atrium.

Patent osteum primum:

The foramen primum may fail to close inspite of formation of the foramen secondum .

This will cause disturbance in the valvular mechanism of the interatrial septum.

Patent foramen ovale: Mentioned before

Premature closure of foramen ovale:Rarely , it is closed BEFORE birth.It results in marked enlargement of the Right atrium and ventricle.

Persistent atrioventricular canal:

The A-V cusions and the septum intermedium fail to develop.

The A-V canal remains divided into rt and lt parts.

There is usually: 1- Patent foramen primum 2- IV sepltal defect 3- Abnormalities in the

leaflets of the valves guarding the A-V canal.

Ventricular Septal defects:

Usually in the membranous part

Tetralogy of Fallot:

Four co-occurring heart defects:

• Pulmonary stenosis

• Ventricular septal defect

• Overriding aorta (dextroposition)

• Right ventricular hypertrophy

Eisenmenger's syndrome:

Characteristics of Eisenmenger's syndrome summarized as:

• persistent truncus arteriosus

• ventricular septal defect • left-right ventricular

shunt • right ventricle

hypertrophy

Congenital Aortic valve stenosis:

Occurs due to fusion of the cusps of the aortic valve .

Very narrow aortic orifice will cause Lt ventricular hypertrophy.

Regurge of blood from the aorta to the left ventricle will increase the lt ventr. Hypertrophy.

Congenital Aortic valve atresia:

Under developed lt ventricle

Narrow ascending aorta Patent ductus arteriosus

to carry the blood into the aorta

Pulmonary atresia The pulmonary artery is

underdeveloped, the right ventricle very small, and also sometimes the tricuspid valve. The condition is also sometimes referred to as hypoplastic right heart.

Transposition of the great arteries:

Most common cyanotic neonatal heart defect

• Failure of aorticopulmonary septum totake a spiraling course• Fatal without PDA, ASD, & VSD

Single ventricle:A univentricular heart may be best described as a defect that encompasses two complete atria that provide venous inflow into a dominant ventricle (either right or left) via a malformed AV connection.

Ectopia Cordis:

The heart is bulging outside the chest due to wide separation in the 2 parts of the sternum

Dextrocardia: The heart and great

vessels are reversed as in mirror image

It might be a separate condition or part of Situs inversus

Situs inversus

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