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Other Acyanotic Heart Defects

In these heart defects, there's a Left-to-right shunt that forms. In instances where the pulmonary pressure becomes greater than the systemic pressure, the shunt switches to right-to-left, causing the patient to become cyanotic. This is called Eisenmenger Syndrome.

Ventricular Septal Defect (VSD)

This forms a Left-to-right shunt, leading to R-sided overload and right-sided heart failure. It's commonly associated with Down’s syndrome and Turner’s syndrome.

“Illustration of a ventricular septum defect” © Centers for Disease Control and Prevention CC0 1.0 (https://commons.wikimedia.org/wiki/File:Truncus_arteriosus.jpg)

Eisenmenger syndrome can occur here as the pulmonary pressure increases so much that the shunt turns into a Right-to-left shunt, in which the baby will become Cyanotic.


It's often asymptomatic if small, but if it's large, it presents with:

  • SOB on exertion e.g. breastfeeding

  • Increased WOB

  • Poor feeding

  • Failure to thrive

  • Heart failure if heart decompensates in undetected cases


O/E - Pan-systolic murmur


N.B. For the murmur, it's similar to mitral regurgitation in which there's turbulent blood flow through the septal defect during systole.


Management:

  • Often closes spontaneously

  • Surgical repair – Transvenous catheter closure or open-heart surgery


Atrial Septal Defect (ASD)

The types of ASD are:

  • Patent foramen ovale (PFO)

  • Ostium secundum – Septum secundum fails to close

  • Ostium primum – Septum primum fails to close, and tends to lead to an AV valve defect

“Atrial septal defect, next to a normal heart” © Centers for Disease Control and Prevention CC0 1.0 (https://commons.wikimedia.org/wiki/File:Truncus_arteriosus.jpg)

It presents the same as a VSD.

O/E - Ejection-systolic murmur with a fixed, split, second heart sound (aortic and pulmonary valves close at slightly different times)


N.B. A split heart sound occurs as blood flows from the LA → RA, therefore increasing the volume of blood the RV has to eject before the pulmonary valve can close.


Management:

  • Surgical repair - Transvenous catheter closure or open-heart surgery

  • Anticoagulation in adults


Complications:

  • Eisenmenger Syndrome

  • Stroke – Embolism passes from R to L side, and up to brain

  • AF

  • Pulmonary HTN and Right-sided HF


Aortic Coarctation

This is where there's a narrowing of the aortic arch, usually just before the DA. It's often associated with Turner’s syndrome (5%).

“Coarctation of the Aorta” © Centers for Disease Control and Prevention CC0 1.0 (https://commons.wikimedia.org/wiki/File:Truncus_arteriosus.jpg)

In neonates, a weak femoral pulse is often the only presenting feature. As the baby grows, they'll begin to present with:

  • Radial-radial/femoral delay

    • If the narrowing is before the left subclavian artery, there's radial-radial delay, but if it's after this branch, there'll be radial-femoral delay.

  • Poor feeding


In these patients, it's very important to check the BP in both arms to assess for that radial-radial delay.


O/E - Systolic murmur


Over time, the pt may develop LVH, HF, and an underdeveloped left arm and legs due to a reduced blood flow.


Management:

  • Echo for monitoring

  • Neonates in a critical condition are given Prostaglandins to keep the DA patent

  • Surgical repair




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