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Chapter 1

Table 1.12 Common surgical procedures for congenital heart disease

Procedure Description Result

Modified Blalock- Brachiocephalic artery to pulmonary artery anastomosis; Increases pulmonary blood flow
Taussig palliative procedure
Fontan Anastomosis or conduit between IVC and pulmonary artery; Increases pulmonary blood flow
palliative procedure
Bidirectional SVC to pulmonary artery anastomosis; palliative procedure Increases pulmonary blood flow
Atrial switch Transection and reimplantation of aorta and pulmonary artery Creates normal relationship between the ventricles and
onto the correct ventricles; corrective procedure the great vessels in transposition
Mustard Arterial switch with intra-arterial pericardial baffle; corrective Reestablishes correct flow sequence to pulmonary
procedure artery and aorta in D-transposition
Rastelli Valved conduit from RV to pulmonary artery; corrective procedure Increases pulmonary blood flow
Ross Pulmonary autograph to aorta; corrective procedure Correction of aortic stenosis
Senning Atrial switch with atrial wall baffle; corrective procedure Reestablishes correct flow sequence to pulmonary
artery and aorta in transposition

Adapted from Segar, D. S. Common surgical procedures for congenital heart disease. ACC Current Journal Review 1996; 5: 46.

morphologic RV systolic failure as the patient reaches adulthood chronic thromboembolic disease. Overall maternal mortality is
or is exposed to the increased CO demands of pregnancy. A very high (approximately 30“50%) and has not improved signifi-
morphologic RV is not designed to pump chronically against cantly over the years despite improvements in medical care and
multidisciplinary management.197
SVR and will begin to fail over the years or under physiologic
high-output states (e.g. exercise or pregnancy). Symptoms of pulmonary hypertension are nonspecific and
The hallmarks of management post-fontan repair are the main- often difficult to differentiate from normal pregnancy symptoms.
tenance of univentricular systolic function and the abatement of The most frequent symptom is progressive dyspnea in addition to
fatigue, chest pain, peripheral edema, and syncope.198 Cardiac
pulmonary arterial pressure elevation. Since pulmonary CO is
passively driven by the gradient between the CVP and PAP, it is catheterization is the gold standard for assessment of pulmonary
necessary to keep PVR as low as possible. Factors that contribute hypertension. Echocardiography provides good information of
to PAP elevation (see Table 1.9) warrant close attention. Embolic the underlying cardiac defect, myocardial function, and PAP.
material entering the pulmonary arterial tree could significantly However, echocardiography may overestimate PAP in pregnant
patients with suspected pulmonary hypertension.199
impede pulmonary blood flow since there is no ventricular pump
to force blood past the obstruction. A small subset of post-Fontan
patients will have a ˜˜fenestrated™™ modification, which means
Eisenmenger syndrome
that an intentional potential right-to-left ˜˜pop-off™™ hole has been
created to decompress high central venous pressures. The ˜˜pop- This is the final end-stage condition where bidirectional or right-to-
off™™ effect will preserve systemic CO and minimize the adverse left shunting between the systemic and pulmonary circulations
effects of caval congestion at the expense of oxygen desaturation. occurs as a result of increased pulmonary pressures that approach
Pregnancy post-Fontan operation is usually well tolerated systemic pressures. Eisenmenger syndrome (ES) may develop in
although there is a 2% risk of death.193 Favorable anesthetic longstanding CHD lesions with large left-to-right shunts and high
management of pregnant women who have previously under- pulmonary blood flow (e.g. large ASD, VSD, PDA, or large surgical
gone Fontan repairs has been reported.194,195 Successful manage- systemic-pulmonary anastomoses following palliation or definitive
ment of a patient with Fontan physiology mandates a thorough repairs of CHD). The pulmonary hypertension is secondary to struc-
understanding of the hemodynamic consequences of this proce- tural changes in the pulmonary vasculature that eventually results
dure and the alterations during pregnancy.196 in shunt reversal when PAP finally exceeds systemic pressure.
The maternal mortality in patients with ES is very high, ranging
from 23“40%.200,201,202 Women with pulmonary hypertension or
Pulmonary hypertension and Eisenmenger ES should not become pregnant and therapeutic terminations
syndrome should be offered if patients present early in their gestation.
Pulmonary hypertension is defined as a mean PAP >25 mmHg at
rest (>30 mmHg during exercise and pregnancy). Pulmonary hyper-
Management principles
tension secondary to cardiac disease may result from longstanding
 Monitoring: Continuous ECG and pulse oximetery with inva-
high pulmonary blood flow due to systemic-to-pulmonary shunts,
sive arterial monitoring should always be used.197 Pulmonary
venous hypertension from cardiac or valvular dysfunction, or

1 Cardiovascular and respiratory disorders

artery catheterization is controversial and the risk of the pro- should be omitted because of potential epinephrine-induced
cedure should be balanced with the benefit of the additional tachycardia. In patients undergoing a trial of labor, an assisted
hemodynamic data that will be obtained. delivery will minimize expulsive efforts.
 It is crucial to maintain the delicate balance between systemic The choice between a vaginal birth and C/S will depend on the
and pulmonary pressures to avoid worsening right-to-left patient, obstetric, and institutional factors (see Table 1.8).
shunting. Maintain SVR and avoid maneuvers that increase Cesarean section has been associated with higher mortality in
patients with ES;200 however, this statistic may reflect C/S in
PVR (see Table 1.9).
 Consider agents to improve pulmonary hypertension, for patients with cardiac decompensation. Traditionally, GA has
been used in patients with pulmonary hypertension Æ ES. A
example inhaled nitric oxide and inhaled or i.v. prostaglandins
review of the literature by Martin et al.207 reveals that the surgical
(e.g. esoprostenol, iloprost). Intravenous esoprostenol has
been used successfully to manage a woman with ASD and ES procedure and disease, rather than the anesthetic technique, had
in late pregnancy.203 Inhaled nitric oxide transiently improved the greatest impact on outcome. There is no evidence to suggest
oxygenation and PAP in a woman with ES in labor and post- any anesthetic technique is superior for C/S; however, a trend
partum.204 L-arginine and sildenafil have been used to treat a towards a lower mortality rate is associated with regional anesthe-
sia.207 There are a number of reports describing the successful use
woman with severe pulmonary hypertension and ES during
of epidural anesthesia for C/S.208,209,210 Continuous spinal
pregnancy, delivery, and postpartum with significant improve-
ment in the mother™s condition.205 Chronic medications (e.g. anesthesia has also been used for C/S in a woman with ES.211
calcium channel blockers) that the patient receives for pulmon- The stated advantages of this technique are titratable anesthesia,
ary hypertension and cardiac function should be continued. less chance of failed or incomplete anesthesia, and less local anes-
 Maximize RV function by maintaining appropriate preload thetic use. A single-shot spinal technique is not recommended
and minimizing PVR increases (see Table 1.9). Increases in RV because of the critical importance of maintaining SVR and CO.
pressure may worsen tricuspid regurgitation and cause the General anesthesia has a number of disadvantages, for example,
interventricular septum to shift to the left, resulting in a reduc- induction agents with myocardial depressive effects (see
tion in LV function and CO. Table 1.1), and positive pressure ventilation, which may decrease
 Provide adequate oxygenation during labor, delivery, and post- venous return and increase ventilation/perfusion mismatch.
partum. This may reduce hypoxic pulmonary vasoconstriction Potential advantages of GA include facilitating the use of intra-
and improve PAP. Oxygen may not improve saturation if sig- operative TEE, and administration of inhaled or nebulized pul-
nificant right-to-left shunting exists. monary vasodilators. Transesophageal echocardiography is
 Avoid sedatives that may decrease respiratory drive, increase valuable for patients at risk from hemodynamic disturbance.
PaCO2, and therefore increase PVR (see Table 1.9). Transthoracic echocardiography can be of value in monitoring
awake patients with regional anesthesia.212
 Patients are at higher risk for thromboembolic events com-
pared to healthy parturients. A ˜˜cardiac™™ anesthetic utilizing induction agent with minimal
 Use air filters if there is significant right-to-left shunting. negative inotropic and SVR effects (e.g. etomidate) and an opioid-
However, air filters increase i.v. line resistance and reduce the based technique to reduce intubation and surgical response is
ability to resuscitate the patient in the case of peripartum preferable. Neonatal respiratory depression must be anticipated
hemorrhage. if high doses of opioids are used. Clinicians should be aware that a
 Vasoactive agents with predominantly chronotropic and ino- slow cardiac induction of GA may lead to pulmonary aspiration.
tropic effects (e.g. ephedrine) or low doses of alpha-agonists Nitrous oxide can increase PVR and should be avoided if possible.
(phenylephrine) are preferable when treating hypotension, Contraction of the uterus and relief of inferior vena cava (IVC)
since high doses of alpha-agonists may increase PVR. obstruction immediately following delivery can result in hyper-
 Uterotonic agents: hypotension and decreases in SVR may occur volemia and cardiac decompensation. Careful use of vasodilators
with bolus administration of oxytocin. An infusion of the lowest with continuous monitoring may be useful at this time. Cardiac
possible dose necessary to maintain uterine tone should be used decompensation and death are most likely to occur during the
postpartum period.207 Patients should receive several days of
to minimize these cardiovascular effects. Oxytocin has been
used for induction of labor without adverse cardiovascular dis- intensive care and remain in hospital for at least one to two
weeks postpartum.213
turbances. Prostaglandin F2-alpha can increase PVR and is rela-
tively contraindicated.66 Similarly, methylergonovine should be
used cautiously in these patients as it also has the potential to
produce severe systemic hypertension.
Obstetric anesthesiologists should not be dogmatic about the
choice of anesthetic for parturients with heart disease. Due to
Anesthetic options
the variation and rarity of cardiac diseases in pregnancy there is
Successful use of epidural analgesia has been reported for labor no good evidence based on randomized controlled studies to
and delivery analgesia206 and good analgesia is important to guide our practice. An understanding of the hemodynamic
minimize cardiovascular disturbances. Opioids should be added changes associated with pregnancy and the functional impair-
to minimize local anesthetic concentrations. A routine test dose ment of the structural cardiac lesion in question is most

Chapter 1

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1 Cardiovascular and respiratory disorders

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