117H Congenital Rubella with mild PS and PDA

  • 60 y.o. female
    • PMH of
      • HTN, HLD, T2DM,
      • Congenital Rubella,
        • moderate supravalvular pulmonic stenosis,
        • PAD,
      • 12 yeaRS AGO
        • spect
          • Abnormal myocardial perfusion SPECT scan, with reversible ischemia
            of the anterior septal wall and at the apical cap.
            2. Global left ventricular systolic function was abnormal with global
            hypokinesis and with post-stress LVEF of 28%.
            3. Regional left ventricular systolic function was abnormal with
            septal and inferior wall hypokinesis.
        • MRI
          • 1. The LV is moderately enlarged with moderately depressed systolic
            function, LVEF: 37 %.
            2. The RV is normal in size with normal systolic function, RVEF: 62 %.
            3. No anatomic obstruction or flow acceleration was seen in the RVOT
            or proximal PA (right PA was well seen, but left PA was not well
            seen).
            4. Flow acceleration and turbulence were seen at the level of the
            pulmonic valve, suggesting that there is valvular pulmonic stenosis.
        • Non ischemic cardiomyopathy
          • EF 20-25% ( 2/2023)
          • s/p CRTD placement,
            • Cath from 3 years ago
              • Normal coronary arteries
                Congenital heart disease: Mild supravalvular stenosis (peak to peak
                < 20 mm Hg)
                Hemodynamics – PCWP & LVEDP Top-Normal
        • CKD IIIb,
        • hypothyroidism,
        • asthma,
        • nontoxic nodular goiter
          • s/p total thyroidectomy,
      • Cath
        • Congenital heart disease: Mild pulmonic valve stenosis, peak-peak
          gradient 10 mmHg
          Congestive Heart Failure
          Hemodynamics – Elevated Pulmonary Vascular Resistance Moderate (PVR
          402)
    • Dyspnea and orthopnea
      • BNP elevated at 3500
  • The cardiac abnormality most frequently found in rubella syndrome is a
    • combination of branch pulmonary artery stenosis and
    • patent ductus arteriosus,
      • isolated branch pulmonary artery stenosis is twice as common as isolated patent ductus arteriosus [
        • multiple stenoses of the pulmonary arteries.
        • involvement of the bifurcation of the pulmonary trunk
        • membrane situated immediately above the pulmonary valve
    • wide variety of cardiac malformations
      • ventricular and atrial septal defects,
      • stenosis of the pulmonary and aortic valves,
      • Fallot’s tetralogy,
      • coarctation of the aorta,
      • tricuspid atresia and
      • transposition of the great vessels. Localised pulmonary arterial stenosis may appear in one of three main forms.
  • Neuro
    • small head circumference/microcephaly
    • periventricular calcifications
    • white matter hypodensity
    • ventriculomegaly
  • Links and References