Rapid Review·Cardiovascular

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PATHOLOGY

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Congenital Heart Disease: Left-To-Right Shunts

P301

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cyanosis appears years later if uncorrected. Frequency: VSD > ASD > PDA.
an abnormal communication between the ventricles, most often through the membranous part of the septum.
  • Asymptomatic at birth, may declare itself weeks later or stay silent for life. Most smaller defects close on their own.
  • Larger untreated defects raise pulmonary blood flow and overload the LV, leading to Eisenmenger syndrome and heart failure.
  • Holosystolic, harsh murmur loudest at the tricuspid area; it may be inaudible right after birth, because high pulmonary vascular resistance limits the left-to-right flow.
  • Oxygen saturation steps up in the RV and pulmonary artery.
a defect in the interatrial septum, i.e. a patent foramen ovale that never fused. Symptoms range from none to heart failure.
  • Systolic ejection murmur with a wide, fixed split S2. The ejection murmur is early-peaking and pulmonic, from the relative pulmonary stenosis of the extra flow.
  • Ostium secundum defects are the most common and usually isolated; ostium primum is rarer and usually accompanies other anomalies.
  • Oxygen steps up in the RA, and onward through the RV and pulmonary artery.
  • Associated with Down syndrome, and can cause paradoxical emboli during transient shunt reversal, classically while lifting weights.
the endocardial cushions fail to fuse, leaving an ostium primum ASD, a VSD, and a single common AV valve. Strongly associated with Down syndrome. Auscultation gives AV valve regurgitation (holosystolic, best at the apex) and a mid-diastolic rumble from the increased pulmonary venous return.
normal in utero, and normally closes only after birth.
  • In the fetus the shunt is right to left, which is normal. After birth pulmonary vascular resistance falls and the shunt becomes left to right, driving progressive RVH and/or LVH and heart failure.
  • Continuous machine-like murmur, best at the left infraclavicular area and loudest at S2. Patency is maintained by prostaglandin synthesis and low oxygen tension.
  • Risk factors: congenital rubella and prematurity.
  • Uncorrected, it causes late differential cyanosis of the lower extremities only, because the duct delivers deoxygenated blood distal to the left subclavian.
VSD, ASD, or PDA.
causes irreversible pathologic remodeling of the pulmonary vasculature.
the age of onset depends on the size and severity of the original shunt.

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What congenital cardiac defect is associated with Williams syndrome?

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