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  • • Congenital heart lesion that increases pulmonary artery (PA) blood flow

    • Results in the following:

    • -Left to right shunt

      -Lung infection

      -Pulmonary vascular congestion

      -PA hypertension

      -Right heart failure

      -Pulmonary vasoconstriction

      -Pulmonary vascular obstructive disease

    Eisenmenger syndrome: Increased pulmonary hypertension such that left to right shunt ceases and shunt becomes right to left, requiring heart-lung transplant

    • Inhaled nitric oxide, oxygen, or IV tolazoline reverses PA vasoconstriction

    • PA band is palliative and can reduce PA flow to alleviate RV failure and progression of pulmonary hypertension

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Epidemiology

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Ruptured Sinus of Valsalva

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  • • More common in Marfan syndrome or other autoimmune diseases

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Symptoms and Signs

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Aortopulmonary Window

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  • • Findings similar to patent ductus arteriosus (PDA)

    • Early heart failure and pulmonary hypertension

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Ruptured Sinus of Valsalva

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  • • Continuous, well localized, parasternal murmur with associated thrill

    • Rapid heart failure develops in most patients

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Left Ventricular-Right Atrial Shunt

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  • • Heart failure in infancy or late childhood

    • Murmur not diagnostic

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Coronary Arterial Fistula

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  • • Many asymptomatic

    • Myocardial ischemia or heart failure

    • Continuous murmur over heart

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Aortopulmonary Window

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  • • Connection between ascending aorta and main PA, rare anomaly

    • 50% associated anomalies (atrial septal defect, ventricular septal defect, interrupted aortic arch)

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Ruptured Sinus of Valsalva

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  • • Rupture of thin membranous tissue between aortic sinus of Valsalva and intracardiac chamber (immediate left to right shunt)

    • Rupture into RV (70%), into RA (20%)

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Left Ventricular-Right Atrial Shunt

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  • • Defect in membranous septum near annulus or septal leaflet of tricuspid valve

    • Uncommon, size of shunt variable

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Coronary Arterial Fistula

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  • • Fistula between RV and right coronary (60%) or left coronary (40%) artery

    • Produces left to right shunt

    • Involved coronary artery is dilated

    • Fistulous openings may be multiple

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Ruptured Sinus of Valsalva

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  • • Echocardiography and catheterization needed for precise anatomic diagnosis

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Left Ventricular-Right Atrial Shunt

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  • • At catheterization: Oxygen saturation increased in RA

    • RA opacifies with injection into LV

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Coronary Arterial Fistula

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  • • Angiogram required to determine number and location of fistula

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Aortopulmonary Window

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  • • Surgical repair with patch closure

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Ruptured Sinus of Valsalva

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  • • Early operation warranted

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Left Ventricular-Right Atrial Shunt

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  • • Closed primarily on bypass

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Coronary Arterial Fistula

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  • • Ligate fistulous connections without interrupting coronary artery

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Surgery

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Indications

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  • • Once diagnosed, all of these defects should be repaired

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