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Common Congenital Heart Lesions - page 99 / 126

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99 / 126

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  • Cardiac Cath Æ reserved for pts for whom diagnosis is uncertain or if cardiac surgery is planned

    • o

      Major feature is for those with obstruction Æ finding a pressure gradient w/in outflow portion of LV

TREATMENT (again, we don’t need this for objectives, but in case you want it…)

  • Beta blockers is standard therapy

  • Ca channel antagonists

  • Anti-arrhythmics

  • Avoid strenuous exercise

  • Antibiotic prophylaxis to ward off infective endocarditis

  • Myomectomy (surgical removal of parts of hypertrophied muscle mass)

  • Genetic counseling

Restrictive Cardiomyopathy (RCM)

  • Less common than DCM and HCM

  • Abnormally rigid (but not necessarily thickened) ventricles with impaired diastolic filling but usual systolic fxn

  • Causes:

    • o

      Fibrosis or scarring of endomyocardium

      • o

        Infiltration of the myocardium by an abnormal substance, like amyloid

PATHOPHYS

  • Rigid myocardium

    • o

      Incr diastolic ventricular pressure Æ venous congestion Æ JVD, hepatomegaly and ascites, peripheral edema

      • o

        Decr ventricular filling Æ decreased CO Æ weakness and fatigue

PRESENTATION

  • Signs of both L and R sided heart failure (as stated just above)

PHYSICAL EXAM

  • Signs of CHF Æ pulmonary rales, JVD, ascites

  • Kussmaul’s sign Æ similar to constrictive pericarditis Æ JVD worsens with inspiration

b/c RV can’t accommodate the incr venous return

TESTS and IMAGING

  • Chest x-ray Æ normal sized heart w/ signs of pulmonary congestion

  • ECG Æ nonspecific ST and T wave abnormalities; conduction disturbances such as AV block or BBB

  • Transvenous endomyocardial biopsy, CT and MRI Æ very useful to differentiate RCM from constrictive pericarditis (RCM will not show a thickened pericardium)

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