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Review Question - QID 105564

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QID 105564 (Type "105564" in App Search)
A one-day-old cyanotic infant with a grade 4/6 systolic murmur heard at the left, lower sternal border (LLSB) has the EKG shown. What is the most likely diagnosis?
  • A

Coarctation of the aorta

0%

0/10

Tricuspid valve atresia

30%

3/10

Tetralogy of Fallot

40%

4/10

Mitral stenosis

0%

0/10

Ventricular septal defect

20%

2/10

  • A

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The EKG shown demonstrates some typical findings noted in infants with tricuspid valve atresia (TVA): tall P-waves (due to right atrial enlargement), left-axis deviation (due to hypertrophy of the left ventricle), and small/absent R-waves in precordial leads V1–V3 (due to a hypoplastic right ventricle).

In individuals with TVA, there is vital right-to-left flow of the entire systemic venous return across the foramen ovale because there is no direct communication between the right atrium and right ventricle. As a result, the left ventricle, which receives all the systemic venous return, becomes the dominant ventricle. There has to be a source of pulmonary blood flow, commonly a ventricular septal defect (95%), which results in a systolic murmur at the LLSB. Blood flow through the VSD is left-to-right. If there is no VSD, a patent ductus arteriosus is usually the source of pulmonary blood flow.

Saenz et al. note that congenital heart defects can be classified broadly into two main categories: acyanotic and cyanotic. The congenital heart defects that are cyanotic can be remembered by the fact that each begins with the letter 'T': tetralogy of fallot, transposition of the great vessels, truncus arteriosus, tricuspid valve atresia, and total anomalous pulmonary venous return.

Epstein notes that the most common classification of TVA is based on the associated anatomic conditions, mainly the anatomy of the great arteries (see the classification in illustration E).

Image A shows a characteristic TVA EKG.

Illustration A shows an echocardiogram of TVA.
Illustration B shows normal fetal circulation.
Illustration C shows fetal circulation in TVA.
Illustration D shows EKG axis findings in infants with cyanotic heart disease.
Illustration E shows the most common TVA classification.

Incorrect answers:
Answers 1, 4, & 5: Coarctation of the aorta, mitral stenosis, and VSDs are not congenital cyanotic heart defects.
Answer 3: Tetralogy of Fallot is a cyanotic lesion, however, it is characterized by right axis deviation on EKG due to right ventricular hypertrophy.

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