Congenital anomalies of the heart
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Congenital Anomalies of the heart. DR 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

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  • Discuss the congenital anomalies related to the heart development.

  • Congenital Heart Defect

  • AcyanoticCyanotic

    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 septaldefect aortic valve stenosis single ventricle pulmonary atresia

  • AV canal coarctation of aortatruncusarteriosustricuspid atresia

  • patent ductusarteriosustotal anomalous pulm. total anomalous pulm.

    return w/o obstructionreturn w/ obstruction

  • Atrial Septal defects:

  • Septum PrimumDefect: Patent foramen ovale

    Defect in the interatrial septum

    Due to absence of the septum

    Primum (Patent foramen ovale)

  • Septum Secondum Defect:

    Defect in the interatrial septum

    Due to absence of the septum

    Secondum(Patent foramen


  • Complete absence of the interatrial septum (Core TriloculareBiventriculare):

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

  • Patent osteumprimum:

  • 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.

  • 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 cushions 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 septaldefect

    3- Abnormalities in the leaflets of the valves guarding the A-V canal.

Tetralogy of Fallot:

Four co-occurring heart defects:

• Pulmonary stenosis

• Ventricular septal defect

• Overriding aorta (dextroposition)

• Right ventricular hypertrophy

  • Eisenmenger'ssyndrome:

    Characteristics of Eisenmenger's syndrome summarized as:

  • persistent truncusarteriosus

  • 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 ltventr. Hypertrophy.

  • Congenital Aortic valve atresia:

  • Under developed lt ventricle

  • Narrow ascending aorta

  • Patent ductusarteriosus 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 to

    take a spiraling course

    • Fatal without PDA, ASD, & VSD

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.

  • Single ventricle:

  • EctopiaCordis:

  • 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 Situsinversus