Heart Lesions, Congenital Increased Pulmonary Flow

• • 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

Epidemiology

Ruptured Sinus of Valsalva

• • More common in Marfan syndrome or other autoimmune diseases

Symptoms and Signs

Aortopulmonary Window

• • Findings similar to patent ductus arteriosus (PDA)

  • Early heart failure and pulmonary hypertension

Ruptured Sinus of Valsalva

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

  • Rapid heart failure develops in most patients

Left Ventricular-Right Atrial Shunt

• • Heart failure in infancy or late childhood

  • Murmur not diagnostic

Coronary Arterial Fistula

• • Many asymptomatic

  • Myocardial ischemia or heart failure

  • Continuous murmur over heart

Aortopulmonary Window

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

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

Ruptured Sinus of Valsalva

• • 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%)

Left Ventricular-Right Atrial Shunt

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

  • Uncommon, size of shunt variable

Coronary Arterial Fistula

• • 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

Ruptured Sinus of Valsalva

• • Echocardiography and catheterization needed for precise anatomic diagnosis

Left Ventricular-Right Atrial Shunt

• • At catheterization: Oxygen saturation increased in RA

  • RA opacifies with injection into LV

Coronary Arterial Fistula

• • Angiogram required to determine number and location of fistula

Aortopulmonary Window

• • Surgical repair with patch closure

Ruptured Sinus of Valsalva

• • Early operation warranted

Left Ventricular-Right Atrial Shunt

• • Closed primarily on bypass

Coronary Arterial Fistula

• • Ligate fistulous connections without interrupting coronary artery

Surgery