This condition is considered an acyanotic congenital heart defect (CHD). As such, certain general rules apply:

Get a detailed medical history – understand all you can about what defect the patient has and how severe the symptoms are. Get surgical history, daily medications, hemodynamic status, and cardiac and lung function.

Reduce left-to-right shunt if present – Increases in SVR will worsen left-to-right shunting. Sudden increases or decreases in pulmonary vascular resistance or SVR will also be tolerated poorly. Volatile anesthetics, propofol, etomidate, and barbiturates all decrease SVR – so use cautiously.

Limit stress – or anything that would stimulate sympathetic response. Opioids are often used to reduce/eliminate sympathetic responses to pain, laryngoscopy, etc. Use a slow/cautious induction.

Maintain MAP and SVR – Arterial lines and/or central lines are ideally employed to keep tight controls. These patients will not have optimal cardiac reserve.

Avoid shunt reversal – Airway obstruction, hypoventilation, hypoxia, and pulmonary hypertension create greater pressures on the right side of the heart and can reverse a left-to-right shunt (making it a cyanotic shunt). This is otherwise called Eisenmenger Syndrome.

Debubble – avoid any bubbles in venous lines. These can lead to a paradoxical embolus.

Cardiac bypass – complex congenital defects sometimes require this. Be aware that this may result in hemodilution!

Endocarditis prophylaxis – for 6 months post-surgical repair of the cardiac defect.

This condition is the narrowing of the aorta close to the ductus arteriosus insertion site. This leads to creater upper extremity perfusion and a decreased lower extremity perfusion

This CHD frequently occurs with other heart defects. A bicuspid valve is present in 50% of these patients, and 30-60% will also have a ventricular septal defect.

Signs and symptoms – may include metabolic acidosis, systemic hypoperfusion, systolic murmur, left ventricular hypertrophy (LVH), and a high systolic blood pressure.

Treatment typically includes dilation using a balloon angioplasty and/or surgical repair

More common in males than females.