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CONGENITAL HEART DISEASE

  1. Pulmonic Stenosis-
    1. Essentials of Dx
      1. Asymptomatic unless lesion is at least moderately severe.
      2. Severe cases may present with right-sided heart failure.
      3. High-pitched systolic ejection murmur maximal in the second left interspace with radiation to the left shoulder.
      4. P2 delayed and soft or absent.
      5. Ejection click often present and decreases with inspiration—the only right heart sound that decreases with inspiration; all others increase.
      6. Echocardiography/Doppler is diagnostic.
      7. Patients with peak pulmonic valve gradients greater than 60 mm Hg or a mean of 40 mm Hg by echocardiography/Doppler should undergo intervention regardless of symptoms. Otherwise, operate for symptoms.
      8. A dysplastic pulmonary valve usually requires surgical treatment, while a domed pulmonary valve stenosis usually can be treated with balloon valvuloplasty.
      9. RV outflow tract obstruction due to conduit stenosis, homograft stenosis and some prior bioprosthetic valve dysfunction may be treated with a percutaneous pulmonary valve replacement.
    2. Fun pictures for our edification

 

    1. Signs/Symptoms-
      1. Mild cases = asymptomatic; Moderate to Severe = symptoms of dyspnea on exertion, syncope, chest pain, and eventually RV failure.
      2. Palpable parasternal lift due to right ventricular hypertrophy (RVH)
      3. Loud, harsh systolic murmur and occasionally a prominent thrill are present in the left second and third interspaces parasternally.
      4. Murmur radiates toward the left shoulder (d/t the flow pattern) and increases with inspiration
      5. Loud ejection click CAN precede the murmur
      6. Right S4 gallop may be heard in the right subclavicular area
    2. Diagnostics Tests
      1. EKG–right axis deviation or peaked P waves give evidence of right atrial overload
      2. CXR–may show prominent RV or RA
      3. Echo–Test of choice
    3. Treatment
      1. mild pulmonic stenosis may be asymptomatic early in life and progressively get worse with age–important to monitor.  It can cause right sided heart failure in ages 20-30s.  
      2. percutaneous balloon valvuloplasty–treatment of choice
        1. indications–peak pulmonary valve gradient is greater than 60mmHg or mean gradient greater than 40 mmHg
  1. Coarctation of the Aorta
    1. Essentials of Dx
      1. usual presentation is systemic hypertension
      2. echocardiography/doppler is diagnostic: gradient over 20 mmHg may be significant due to collaterals around the coarctation
      3. associated bicuspid aortic valve (in 50-80% of patients)
      4. systolic pressure is higher in upper extremities than lower extremities; diastolic is similar
      5. pulse in femoral artery delayed compared to brachial artery
    2. symptoms and signs
      1. if cardiac failure does not occur in infancy, there are no symptoms until the hypertension produces LV failure or cerebral hemorrhage occurs
      2. strong arterial pulsations seen in the neck and suprasternal notch
      3. hypertension is present in the arms, but the pressure is normal in the legs and difference exaggerated by exercise
      4. femoral pulsations are weak and delayed compared to brachial or radial pulse.
      5. in severe cases–a continuous murmur heard superiorly and midline in the back or over the left anterior chest
    3. Diagnostic Studies
      1. ECG–shows LV hypertrophy
      2. CXR–”scalloping” of the ribs due to enlarged collateral intercostal arteries
      3. Echo–is diagnostic
      4. MRI and CT
    4. Treatment
      1. endovascular stenting is treatment of choice
      2. self expanding and balloon expandable covered stent
      3. surgical resection (end to end anastomosis)
        1. if untreated patients die of hypertension, rupture of aorta, infective endarteritis, cerebral hemorrhage before the age of 50
  2. Atrial Septal Defect and Patent Foramen Ovale
    1. Essentials of Dx
      1. often asymptomatic and discovered on routine physical examination
      2. RV lift; S2 widely split and fixed
      3. echocardiography/doppler is diagnostic
      4. All atrial septal defects should be closed by a percutaneous device or by surgery if there is any evidence of an RV volume overload regardless of symptoms
      5. A patent foramen ovale, present in 25% of the population, rarely can lead to paradoxic emboli.  Suspicion should be highest in patients who have cryptogenic stroke before age 55 years.
    2. symptoms and signs

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