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Congenital Heart Disease
Single Ventricles—Norwood Operation to prevent further stress on sutures and development of post-
The Norwood procedure is undertaken for patients with hypoplastic left coarctectomy syndrome.
Routine use of vasoactive medications and
heart syndrome and typically includes arch reconstruction, atrial positive inotropes via continuous intravenous infusion is essential. This
septectomy, and BT or Sano shunt placement.
These patients can be care should include appropriate doses of analgesics and sedative
extremely labile post-operatively, and particular attention should be paid medications in the immediate post-operative period. Finally, post-operative
to balancing the Qp:Qs (pulmonary:systemic blood flow ratio). Low pulmonary toilet is vital to prevent atelectasis and pneumonia.
cardiac output is a common postoperative concern and can be due to
AV valve regurgitation, ventricular dysfunction, or unbalanced Qp:Qs.
When to Obtain Further Imaging
Manifestations of low cardiac output include necrotizing enterocolitis Chest radiography should be obtained routinely for any change in
and liver or kidney ischemia.
respiratory status post-operatively. Most symptomatic effusions, pleural or
pericardial, should be visible on radiography. Follow-up echocardiography
Glenn Operation can be obtained for questions of valve function, or conduit gradients,
The second-stage surgery for most patients with single ventricle pericardial effusions, or to assess residual shunts. Cardiac catheterization
physiology (including hypoplastic left heart syndrome) is the Glenn or is not routinely performed unless coronary insufficiency, residual shunts,
bidirectional cavopulmonary anastomosis. This operation diverts all or fistulous connections require evaluation. CT or MRI can be performed if
superior vena cava return to the pulmonary arteries.
Common surgical needed to assess aortic arch repairs, aortic grafts, pulmonary artery
complications can include phrenic nerve injury, superior vena cava architecture, or right ventricular function.
pathway obstruction (with resultant superior vena cava syndrome), and
transient sinus node dysfunction.
With recent advances in intra- and post-operative care for patients with
Fontan Operation congenital heart disease, survival rates continue to improve. Today more
The final stage for patients with single ventricle physiology involves children and adults have successful surgical procedures. Post-operative
creation of a pathway for inferior vena cava venous return to the monitoring and follow-up is essential for the prevention of serious
pulmonary arteries, bypassing the right ventricle. This is most commonly morbidity and mortality. Physicians caring for post-operative patients
performed using a tunneled pathway of material, either through the with congenital heart disease require a unique understanding of lesion-
right atrium (lateral tunnel pathway) or via an extra-cardiac conduit specific complications, as well as the baseline physiology and surgical
Arrhythmias are common, including atrial flutter and JET.
techniques involved. n
Since ventricular contraction does not assist in propelling pulmonary
flow, post-Fontan patients are extremely sensitive to volume depletion,
Jonathan N Johnson, MD, is a second-year Pediatric
anemia, and arrhythmia. They are highly preload-dependent and often
Cardiology Fellow at the Mayo Clinic in Rochester. He is a
develop hemodynamic compromise in response to these stressors.
member of the American Academy of Pediatrics (AAP) and
the American Heart Association (AHA), and serves on the
Intensive Care Management
Board of Directors for the Minnesota Chapter of the (AAP).
The role of post-operative pain control and sedation is often under-
appreciated, especially in neonates. Prevention of hemodynamic changes
in shunting, particularly right-to-left shunts, can in turn prevent
hypercyanotic episodes and hypoxemia in susceptible patients.
Frank Cetta, MD, is a Professor of Internal Medicine and
Pediatrics and Chair of the Division of Pediatric Cardiology
Ventricular tachycardia or SVT can be induced by tachycardia responses to
at the Mayo Clinic in Rochester. He is a Fellow of the
painful stimuli. Cardiac output is often at its lowest point the first night
American College of Cardiology (ACC) and the American
following surgery and any interventions that can be undertaken to prevent
Society of Echocardiography (ASE) and a member of the
American Heart Association (AHA). He sees both adult and
further uncontrolled and unexpected drops in cardiac output are crucial,
pediatric patients with congenital heart disease.
particularly in patients with single ventricle physiology.
After repair of
coarctation of the aorta, patients need aggressive control of hypertension
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