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Congenital Heart Disease
Figure 1: Echocardiograph of a Three-year-old with a
syndrome. White blood cell counts and other markers of inflammation
Large Pericardial Effusion
(erythrocyte sedimentation rate, C-reactive protein) will be elevated.
patient for whom the pericardial sac is opened is at risk for post-
pericardiotomy syndrome, but it appears to occur more frequently in older
children and adolescents rather than in neonates.
diuretics and anti-inflammatory medications including salicylates, non-
steroidal anti-inflammatory drugs (NSAIDs), or steroids in extreme cases.
If pericardiocentesis is needed to drain a large pericardial effusion, a
pericardial drain should remain in place for at least two to three days. If
drainage is persistent, the effusion may need to be addressed surgically,
particularly in the case of pericardial hemorrhage.
If the fluid is not
free-flowing, an extensive decortication procedure may be needed.
Chylothorax refers to a collection of chyle in the pleural cavity. This
is most commonly caused by damage to the thoracic duct or other
major lymphatic vessels in the chest during surgery, and is most
common in the neonatal–infantile age group.
Chylous effusions can
be particularly frequent following the Glenn and Fontan procedures.
The effusion seen in chest tube drainage is often milky-colored in
appearance (if the patient has received enteric feedings) due to the
high triglyceride content. Treatment consists of removal of free fatty
Figure 2: Chest Radiograph of a Two-week-old
acids from the diet, either by using parenteral nutrition or by
Following Norwood Procedure—Note the Elevated
providing oral nutrition with a low free fatty acid formula.
treatments for persistent effusions can include intravenous infusion
of octreotide or surgical pleurodesis.
Diaphragmatic paralysis can occur with any manipulation of the
phrenic nerve, but is a particular risk after manipulation of the branch
pulmonary arteries, superior vena cava, or aorta reconstruction.
Diagnosis is often made several days post-operatively by an elevated
hemidiaphragm noted on chest radiography (see Figure 2), as well as
by difficulty with ventilation (or inability to tolerate extubation) in
the absence of other pulmonary disease. If suspected but not evident
on chest radiography, chest fluoroscopy, or ultrasound scanning can
be used to examine diaphragm mobility.
Paralysis is usually
transient, but if a patient is continually unable to be extubated due to
persistent diaphragmatic paralysis, a surgical plication of the
diaphragm can be performed.
Injury to the recurrent laryngeal nerve is common during aortic arch
surgery, including repair of coarctation, interrupted arch, the Norwood
procedure, and patent ductus arteriosus ligation. Recurrent laryngeal
nerve injury can cause transient vocal cord paralysis.
cause stridor and difficulty in extubation and changes in voice
post-operatively, and can increase the risk of aspiration. Rarely, but
dangerously, if both vocal cords are paralyzed significant airway
obstruction can occur.
The diagnosis can be made by laryngoscopy.
Treatment ranges from simple observation to surgery, depending on
presentation and effect on respiration.
continued chest tube drainage, as they can delay and complicate
recovery from cardiac surgery. Supplemental procedures including Post-operative cyanosis despite adequate ventilation is a dilemma in
pleurodesis can be performed if the pleural effusions are persistent and the ‘repaired’ patient with congenital heart disease. In all patients a
healthy index of suspicion needs to be maintained regarding the surgical
procedure and accuracy of preoperative diagnosis, particularly when
New-onset pericardial effusion (see Figure 1) presenting weeks after post-operative clinical status and hemodynamics are not improving in
surgery can be a sign of the immune-mediated post-pericardiotomy the expected manner.
One potential cause of post-operative cyanosis
108 US CARDIOLOGY