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PH-CV17

Congenital heart disease

How heart defects follow from a septum, shunt or outflow division that failed in development, why pressures after birth decide which way blood crosses, and how shunts, cyanotic lesions and obstructions cause their signs.

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  • 6 steps · 10 questions
  • 3D normal anatomy

By the end, you can

  • Relate each common defect to the step of heart development or the fetal shunt that failed.
  • Explain how the fall in pulmonary resistance at birth sets the direction and timing of shunts.
  • Explain how a large left-to-right shunt causes heart failure and can progress to Eisenmenger syndrome.
  • Explain the cyanosis of tetralogy of Fallot and transposition, and the effects of coarctation and valve stenosis.
  • Recognise the classic signs, classifications and tests of congenital heart disease, and explain how each main treatment acts on the mechanism.

What it is

Congenital heart disease is a structural abnormality of the heart or great vessels present at birth, arising when a step of heart development fails or a fetal shunt does not close. Defects are grouped by their effect: left-to-right shunts and obstructive lesions, which do not cause cyanosis at first, and right-to-left lesions, which do.

4 types to tell apart
Left-to-right shunts
Atrial and ventricular septal defects, patent ductus arteriosus and atrioventricular septal defect: oxygenated blood recirculates through the lungs, a volume load.
Right-to-left (cyanotic) lesions
Tetralogy of Fallot and transposition of the great arteries, among others: oxygen-poor blood reaches the body without passing through the lungs.
Obstructive lesions
Coarctation of the aorta and aortic and pulmonary stenosis: a narrowing loads the ventricle behind it with pressure.
Eisenmenger syndrome
The late reversal of a large left-to-right shunt once the pulmonary arteries have remodelled, turning an acyanotic defect into a cyanotic one.

US spelling: centre → center.

Normal function

How one tube becomes four chambers and two arteries

Between about weeks 4 and 8 the looped heart tube is divided from inside while it keeps beating. In the atria a thin grows down towards swellings in the canal between atria and ventricles. The gap below its edge, the , closes as it meets them, but holes open in its upper part (the ). A thicker then grows down on its right and stops short, leaving the foramen ovale, with the septum primum as a one-way flap.

The swellings, the , fuse to divide the canal into the tricuspid and mitral openings and to build the centre of the heart. Below, a rises from the floor of the ventricles; the last gap above it is closed by the thin , made from the cushions and the ridges of the outflow tract.

The single outflow tract is divided by ridges that fuse into a twisting aorticopulmonary septum, so the leaves the right ventricle and the the left, wound round each other. Before birth the carries most of the right ventricle’s output into the aorta past the fluid-filled lungs. Each defect in this lesson is one of these steps left incomplete or done wrongly.

Point to it on the model