CARDIOLOGY II

APPROACH TO CONGENITAL HEART DISEASE

1. CLINICAL QUESTIONS TO CONSIDER:
   • Cyanosis or Acyanosis
   • Murmur (optional)
   • Patient's age

   1. Cyanosis

      1. Cyanosis suggests right to left shunting (i.e., >5 gms deoxygenated hemoglobin sent systemically).

         Think "5 T's":

         1. Total anomalous pulmonary venous return (TAPVR).

         2. Tetralogy of Fallot (T. of F.).

         3. Truncus arteriosus.

         4. Tricuspid atresia.

         5. Transposition of the great vessels (TGV).

      2. Acyanosis - nonspecific.

   2. Murmur

      1. Often nonspecific - false positives and false negatives.

   3. Patient's Age

      1. Onset of symptoms at birth.

         1. With congestive heart failure signs consider hypoplastic left heart lesions.

         2. Cyanosis - variable onset (5 T's).

      2. Onset of symptoms at approximately six weeks.

         1. Time required to reduce pulmonary vascular resistance to adult levels.

         2. Think left to right shunt.

            1. Ventricular septal defect (VSD).

            2. Atrial septal defect (ASD).

            3. Patent ductus arteriosus (PDA).

2. RADIOGRAPHIC APPROACH

   A. Heart Size and Shape

   1. Enlarged, normal, small (rare).

   2. Specific chamber enlargement - obliques may be helpful.

   3. Characteristic configurations.

      1. "Coer en sabot" - right ventricular enlargement (tetralogy of Fallot).

      2. "Egg on a string" - anterior/posterior relationship of great vessels in transposition complexes.

      3. "Snowman, figure 8" - anomalous drainage chamber in superior mediastinum of total anomalous pulmonary venous return.

   B. Pulmonary Vasculature

   1. Increased - active (left to right shunt) or passive (CHF).

   2. Normal.

   3. Decreased - suggests blood not reaching the lungs.

CASE 1 G1, G2

15 year old acyanotic male with long history of upper extremity hypertension.

1. Observe the aortic knob region.
2. What is causing the inferior indentations of the posterior ribs?

CASE 2 G3, G4

15 year old acyanotic male with systolic murmur heard across upper chest.

1. Note ascending aorta, especially its size.
2. Hint: The patient does have a bicuspid aortic valve.

CASE 3 G5, G6

8 year old acyanotic male with systolic ejection murmur heard best along the upper left sternal border.

1. What is the soft tissue mass adjacent to the carina on the left?

CASE 4 G7, G8

1 year old acyanotic female with a loud murmur along the lower left sternal border.

1. Note the heart size.
2. Is the pulmonary vascularity normal?

HINT: The patient was asymptomatic at birth.

CASE 5 G9

A 1 week old cyystolic murmur.

1. Note heart size.
2. Remember the patient is cyanotic (5 T's).

CASE 6 G10

A 2 week old cyanotic female born in "winter".

1. Note the superior mediastinum.
2. Note the pulmonary vasculature.

CASE 7 G11, G12

A 9 year old acyanotic male with murmur detected on routine physical exam for school athletic program.

1. Note the right heart border. What chamber lies here?
2. Is the pulmonary vascularity normal?
3. What is the size of the left atrium (lateral view)?

CASE 8 G13, G14

A 5 month old cyanotic female with a heart murmur.

1. Is the heart size normal?
2. Where is the cardiac apex?
3. What about the pulmonary outflow tract and vascularity?

CASE 9 G15, G16

A 3 day old cyanotic male with EKG showing right ventricular hypertrophy.

1. Note the heart and mediastinal configuration.
2. What about the pulmonary vascularity?