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Congenital Heart Diseases
With Right-to-Left Shunts
Jason Z. Qu, MD
There are 6 common types of congenital heart diseases with right-toleft lesions: tetralogy of Fallot (TOF), transposition of the great arteries,
truncus arteriosus, tricuspid atresia, total anomalous pulmonary venous
return, and pulmonary atresia with intact ventricular septum.
Tetralogy of Fallot
Tetralogy of Fallot is the most common intrinsic cyanotic congenital
heart disease, which accounts for 5% to 10% of all congenital heart disease
and has a prevalence of about 1 out of 2,000 live births.1
Anatomy and Pathophysiology
Tetralogy of Fallot comprises a constellation of cardiac findings that
share the following common anatomic abnormalities (Fig. 1): a large
malaligned ventricular septal defect (VSD), overriding of the aorta over
the septal defect, right ventricular outflow obstruction, and right ventricular hypertrophy. The anatomic spectrum of TOF is diverse and includes other variants such as pulmonary stenosis (TOF-PS), pulmonary
atresia (TOF-PA), or absent pulmonary valve (TOF-APV).
The pathophysiology of TOF depends, primarily, on the degree of
right ventricular outflow tract obstruction (RVOTO) and, secondarily, on
the status of the systemic vascular resistance relative to the degree of right
ventricular obstruction. The patients can be fully saturated or severely
cyanotic depending on the ratio of pulmonary to systemic blood flow.
In a Tetralogy spell (tet spell), or hypoxic spell, the infants experience
episodes of severe hypoxemia and acidemia. The responsible factors may
be increase in RVOTO and increase in oxygen requirement, decrease in
peripheral vascular resistance, contraction of the right ventricular infundibulum, closure of the ductus arteriosus, or development of physiologic anemia.2–4
59
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Figure 1. The typical anomaly of tetralogy of Fallot: right ventricular outflow obstruction
(pulmonary valvular and infundibular obstruction shown in the picture), ventricular septal defect,
right ventricular hypertrophy, and dextroposition of the aorta. AO, aorta; LA, left atrium; LV, left
ventricule; RA, right atrium; RV, right ventricle; PA, pulmonary artery.
Erythrocytosis (increased blood cell mass) is the usual response of
patients with TOF to hypoxia. Persistent hypoxemia results in increased
blood erythropoietin, which stimulates bone marrow production of
erythroid cells.
Clinical Presentations
Depending on the severity of RVOTO, the patients may present from
no cyanosis to severe cyanosis. The tet spells frequently occur after crying.
The child may appear normal between spells. Frequent and prolonged
spells lead to cerebral injury. Hypoxia-induced erythrocytosis may result in
cerebral venous thrombosis and further compromises neurologic function. Bacterial endocarditis and brain abscesses are not uncommon in this
group of infants.
Laboratory Studies
Polycythemia is a common finding. The electrocardiogram shows right
ventricular hypertrophy with or without right bundle branch block. A
boot-shaped cardiac contour is very characteristic, and the pulmonary vascularity is diminished on chest x-ray. Echocardiography and color Doppler
analysis provide data regarding hemodynamic characteristics as well as
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Right-to-Left Congenital Cardiac Lesions n 61
morphologic features of the anomaly. Cardiac catheterization and angiocardiography can provide additional important information such as major
aortopulmonary collateral arteries (MAPCAs) or abnormal coronary
arteries that cannot be imaged adequately with ultrasound.
Surgical Management
The goals of surgical management are to improve pulmonary blood
flow and to correct pathologic anatomy. Blalock and Taussig initiated
a procedure of connecting the right side subclavian artery to the right
pulmonary artery by an end-to-side anastomosis.5 This procedure was later
modified by interposing a tubular graft of Teflon or Gore-Tex between
the subclavian and pulmonary arteries to preserve more blood flow to the
arm. Total correction for TOF consists of closing the ventricular septal
defect and relieving the right ventricular outflow obstruction.6 With
refinements of surgical technique and perioperative management in
recent years, early correction of tetralogy has been widely recommended
and excellent results are now achievable in neonates and infants.7,8 Early
correction can prevent or decrease complications (cerebral hypoxia,
embolus, abscess, and myocardial hypertrophy) and avoid the complications of palliative procedures.
Transposition of the Great Arteries
Transposition of the great arteries (TGA) is a congenital heart lesion
characterized by ventriculoarterial discordance. Transposition of the great
arteries accounts for 5% to 7% of all congenital cardiac malformations.9
Anatomy and Pathophysiology
In D-transposition of the great vessels, the aorta arises from the right
ventricle (Fig. 2), and its route is anterior and rightward. The pulmonary
artery originates from the left ventricle, and its route is posterior and
leftward.
Unlike in the normal heart, where the systemic and pulmonary circulations are in series, in TGA, the circulations are parallel. The deoxygenated blood returns to the right atrium and is pumped into the
systemic circulation by the right ventricle via the aorta, whereas the oxygenated blood returns to the left atrium and is pumped into the pulmonary
circulation by the left ventricle via the pulmonary artery. Therefore, there
must be adequate mixing between the pulmonary and systemic circulation
for survival. The mixing can occur at the levels of atrium (ASD), ventricle
(VSD), or great vessels.
L-transposition of the great arteries, also called corrected transposition, is less frequent. In this malformation, the arterial ventricle (anatomic
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Figure 2. Transposition of great arteries (D-transposition) with patent foramen ovale, patent
ductus arteriosus, and intact interventricular septum. The atria and ventricles are concordant.
The ventricles and arteries are discordant with the aorta arising from right ventricle and the
pulmonary artery from the left ventricle. AO, aorta; LA, left atrium; LV, left ventricle; RA, right
atrium; RV, right ventricle; PA, pulmonary artery.
right ventricle) is displaced leftward and posteriorly and the venous
ventricle (anatomic left ventricle) is displaced rightward and anteriorly.
The right atrium empties blood into the anatomic left ventricle through
the mitral valve, and the left atrium empties into the anatomic right
ventricle via the tricuspid valve. The pulmonary artery originates from the
venous ventricle, and the aorta originates from the arterial ventricle. About
half of the patients with L-TGA are dextrocardiac. Patients with L-TGA lead
normal lives if there are no other cardiac defects.
Clinical Presentations
In D-TGA, moderate to severe cyanosis is present after birth, and the
infant is tachypneic. A systolic (regurgitant) murmur of VSD may be
audible in less cyanotic infants with associated VSD. Congestive heart
failure (CHF) develops in the first weeks of life. Without surgical intervention, death occurs in 90% of patients before they reach 6 months of
age.10,11 Progressive hypoxia and acidosis result in death unless the
mixing of the systemic and pulmonary blood improves. Many infants with
TGA and a large VSD develop moderate pulmonary vascular obstructive disease by 3 to 4 months of age. Thus, early surgical procedures are
recommended.
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Right-to-Left Congenital Cardiac Lesions n 63
Laboratory Studies
Severe arterial hypoxemia with or without acidosis is present.
Electrocardiography shows rightward QRS axis. Biventricular hypertrophy may be present in infants with large VSD, PDA, or pulmonary
vascular obstructive disease. An egg-shaped cardiac silhouette with
a narrow, superior mediastinum is characteristic. Echocardiography
usually provides all the anatomic and functional information needed
for the management of infants with D-TGA.11 Catheterization provides
the delineated specifics such as great artery origin and coronary artery
anomalies.
Surgical Management
Palliative procedures include pulmonary artery banding and atrial
septostomy. Pulmonary artery banding is aimed to decrease pulmonary
blood flow to control CHF and minimize pulmonary vascular obstructive disease. It is also performed in the neonatal period to prepare the LV
for future definitive procedures. Atrial septostomy can be achieved by
balloon dilation (Rashkind procedure)12 or excision of the posterior
aspect of the atrial septum via a right thoracotomy (Blalock-Hannon
operation).13
Intra-atrial physiological repairs (atrial switch) include the Mustard
and Senning procedures, which result in physiologic correction of TGA.
The Mustard operation redirects the pulmonary venous return to the
right ventricle and the systemic venous return to the left ventricle by
using a pericardial or prosthetic baffle.14 The Senning operation is a
modification of the Mustard procedure.15 It creates the same discordant
atrioventricular connections using the right atrial wall and interatrial
septum instead of pericardium or prosthetic material.
The Rastelli procedure is a method of anatomically correcting TGA
with VSD and RVOTO.16 In this operation, the VSD is closed with an
intraventricular patch-tunnel so that LV is directed to the aorta. The
pulmonary artery is transected and ligated distal to the pulmonary valve.
A right ventriculostomy is performed, and a prosthetic conduit or an aortic
homograft is connected between the RV and PA.
The Jatene (arterial switch) operation is the anatomic correction of
TGA by switching the great arteries and retransplanting the coronary
arteries.17,18 In this operation, the aorta and pulmonary artery are transected distal to their respective valves. The coronary arteries are explanted
from the aortic root and then reimplanted into the proximal pulmonary
artery (neoaorta). The great arteries are switched with the distal aorta,
reanastomosed to the old proximal pulmonary artery, and the distal
pulmonary artery brought anterior to be reanastomosed to the old
proximal aorta.
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Truncus Arteriosus
Truncus arteriosus accounts for 1% to 2% of congenital heart disease.19
There is a single semilunar valve and annulus with a common outflow
trunk arising from the ventricles. Truncus arteriosus may be associated with
DiGeorge syndrome.20
Anatomy and Pathophysiology
A large and malaligned VSD is also part of the complex. There are 4
types of truncus arteriosus (Fig. 3). Because of the unrestricted VSD and
Figure 3. The common types of truncus arteriosis (TA). In all types, TA arises from the base of the
ventricles and straddles a VSD. In type I (A), a main pulmonary artery (PA) arises from the posterior
left side of the TA and bifurcates into right (RPA) and left (LPA) pulmonary arteries. In type II
(B) and III (C), there is no main PA, and the RPA and LPA arise separately from the posterior or
lateral sides of the TA. In type IV (D), there is no pulmonary artery or its branches. Blood supplies to
the lungs are derived entirely from major aortopulmonary collateral arteries (MAPCAs). AO, aorta;
LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; PA, pulmonary artery.
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Right-to-Left Congenital Cardiac Lesions n 65
the origin of the pulmonary arteries and the aorta from a single trunk,
blood flow to the lungs and the systemic circulation is determined by
the relative resistances in these circulations. In the infant with truncus
arteriosus type I, II, and III who has no stenosis in the pulmonary arteries,
the decreased pulmonary vascular resistance after birth will result in an
increase in pulmonary blood flow, which will maintain the arterial oxygen
saturation between 75% to 80%. The systemic arterial oxygen saturation
may reach levels of 85% to 90% due to the increase in the pulmonary to
systemic blood flow ratio. If the LV volume overload happens rapidly, the
patient will develop cardiac failure within the first week of life. Ninety
percent of patients die of heart failure during early infancy if left untreated.21 Truncal valve insufficiency or stenosis, pulmonary artery stenosis,
and aortic arch anomalies may also be present with truncus arteriosus.22
Clinical Presentations
The newborn with truncus arteriosus exhibits mild cyanosis right after
birth. Cyanosis then deceases as pulmonary blood flow increases (type I
and II), but the signs of heart failure become significant if the pulmonary
blood flow is too large. Patients may have moderate to severe cyanosis if
there is stenosis of pulmonary ostia or pulmonary arteries (type III).
Laboratory Studies
Chest x-ray reveals cardiomegaly, and the pulmonary vascular markings
are increased within a week after birth. The electrocardiogram (ECG) can
show the changes of left or right ventricular hypertrophy. Echocardiography can usually give diagnostic information in patients with truncus
arteriosus. Cardiac catheterization and angiography can reveal the precise
origin of pulmonary arteries, severity of obstruction of pulmonary ostia or
pulmonary arteries, and coronary anomalies.
Surgical Management
The palliative procedure for patients with truncus arteriosus is PA
banding. Surgical repair can be accomplished with a relatively low
mortality, even in small infants.23,24 The essentials of the surgical repair
are to close VSD, divide the truncus using the truncal valve as the aortic
valve, and place a conduit with or without a valve from the RV to the PA.
Tricuspid Atresia
Accounting for 1% to 3% of congenital heart defects, tricuspid atresia
is a congenital heart lesion characterized by agenesis of the tricuspid
valve.25
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Anatomy and Pathophysiology
Tricuspid atresia is commonly classified into 3 types.25 Type I tricuspid
atresia is not associated with transposition of the great arteries. Type II
is associated with D-transposition, and type III consists of L-transposition
with tricuspid atresia. All patients with tricuspid atresia will need an ASD
with right-to-left shunt to survive. More than 70% of patients with tricuspid
atresia have pulmonary atresia or pulmonary or subpulmonary stenosis. An
additional downstream shunt (PDA, bronchial collaterals) is necessary for
survival if pulmonary atresia exists.
Clinical Presentations
Patients often show cyanosis on the first day of life. Squatting may be
noted in older children. Physical examination will reveal cyanosis as well as
clubbing of fingers and toes in children older than 1 year. A systolic thrill
and loud systolic murmur along the sternal border can be appreciated if
a VSD present. A systolic murmur of pulmonary stenosis and a machinery
murmur of PDA may be heard. Symptoms and signs of heart failure may
appear when the disease progresses into late stage.
Laboratory Studies
Arterial hypoxemia and polycythemia (hematocrit >56%) are revealed
on blood tests. Electrocardiogram findings include LVH, LV strain pattern,
and ST and T wave changes. Chest x-ray shows an elevated apex, left atrial
enlargement, and absence of right ventricle. Echocardiography is often
diagnostic for tricuspid atresia.
Surgical Management
Prostaglandin E1 should be used to maintain ductal patency after the
diagnosis is confirmed. A balloon atrial septostomy or atrial septectomy
may be urgently needed for patients with CHF and a large pressure
gradient between the 2 atria.
The most common palliative shunt performed early in tricuspid atresia
is a modified Blalock-Taussig. A bidirectional Glenn shunt can provide
pulmonary perfusion by anastomosing the upper end of divided superior
vena cava (SVC) to the side of the right pulmonary artery. Both of these
procedures allow adaptation to a Fontan correction at a later stage. The
Fontan operation is based upon the realization that venous (or atrial)
pressure alone is adequate to drive blood through the pulmonary circulation.26 By connecting SVC to right pulmonary artery and right atrium
to left pulmonary artery, the Fontan operation successfully separates the
pulmonary from systemic circulation. The blood returning from the SVC
now perfuses the right pulmonary artery, and blood from the inferior
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Right-to-Left Congenital Cardiac Lesions n 67
vena cava (IVC) passes through the right atrium and then into the left
pulmonary artery. The Fontan procedure has been modified into many
variations such as anastomosing RA to the main PA, connecting RA to
RV via homograft, and constructing a tunnel between the IVC and pulmonary artery.27
Total Anomalous Pulmonary Venous Return
Total anomalous pulmonary venous return (TAPVR) accounts for 1%
of all congenital heart lesions. The pulmonary veins connect to the
systemic venous system instead of to the left atrium.
Anatomy and Pathophysiology
Total anomalous pulmonary venous return may be classified into
4 types28: supracardiac (45%), cardiac (25%), infracardiac (25%), and
mixed (5%). In the supracardiac type, the common pulmonary vein confluence drains into the right atrium via left innominate vein or into the
SVC. In the cardiac configuration, the common pulmonary vein connects
to the coronary sinus or directly with the right atrium. In the infracardiac
(subdiaphragmatic) pattern, the common pulmonary vein drains through
diaphragm into the portal vein, ductus venosus, hepatic vein, or IVC. The
mixed type is a combination of the other types.
Clinical Presentations
Clinical manifestations differ, depending on the degree of pulmonary
vein obstruction. Neonates with obstructed TAPVR present with profound
cyanosis, severe respiratory distress, and failure to thrive. The infant without pulmonary venous obstruction will have mild cyanosis, CHF, frequent
pulmonary infection, and growth retardation.
Laboratory Studies
Right ventricle hypertrophy (RVH) is common on the EKG. X-ray
studies reveal normal or slightly enlarged heart silhouette and pulmonary
edema. Two-dimensional echocardiography delineates the anatomic connection of the pulmonary veins and the type of TAPVR.
Surgical Management
Corrective surgery is necessary for all patients with TAPVR. Balloon
atrial septostomy may be done if immediate surgery cannot be performed.
All procedures are performed under cardiopulmonary bypass and are
intended to redirect the pulmonary venous return to the LA.
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Pulmonary Atresia With Intact Ventricular Septum
Pulmonary atresia with intact ventricular septum (PAIVS) accounts for
less than 1% of all congenital heart defects. The prevalence of this disorder
is 4.1 per 100, 000 live births.29
Anatomy and Pathophysiology
In the usual form of pulmonary atresia and intact ventricular septum,
the pulmonary valve is atretic with a diaphragmlike membrane. The
infundibulum is also atretic in some infants. Because there is no direct
communication between the RV cavity and the PA, the pulmonary blood
flow is usually entirely dependent on a PDA. The systemic venous return to
the RA must flow to the LA via an ASD or a PFO. Systemic and pulmonary
venous returns mix in the LA and go to the LV to supply the body and
lungs. If the PDA closes after birth, the pulmonary blood flow is significantly decreased, and the infant becomes severely cyanotic. Tricuspid
valve regurgitation is common due to the high pressure in the RV. The high
RV pressure is also decompressed retrograde through the dilated coronary
sinus and its microcirculation into the left or right coronary artery. If the
proximal coronary artery obstruction is present, coronary circulation is
perfused by desaturated blood causing myocardial ischemia.
Clinical Presentations
Cyanosis is usually apparent within hours of birth because of functional
and anatomic closure of the PDA. Severe tachypnea, dyspnea, and hypoxia
are seen in distressed infants. A tricuspid regurgitation murmur may be
audible. Progressive hypoxia and metabolic acidosis indicate the poor
prognosis.
Laboratory Studies
The ECG commonly shows sinus rhythm with LVH and right atrial
enlargement. The ST-T abnormalities consistent with subendocardial
ischemia are frequently seen. The chest x-ray may show an enlarged heart
and reduced pulmonary vascular markings. Echocardiography reveals an
atretic pulmonary valve with no blood flow through it, and a right-to-left
interatrial shunt by color Doppler imaging. Cardiac catheterization and
angiocardiography are required in infants with severe hypoplastic right
ventricle and anticipated ventriculocoronary artery connections.
Surgical Management
Pharmacologic preservation of the PDA with prostaglandin E1
infusion should begin as soon as the diagnosis is suspected or confirmed.
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Right-to-Left Congenital Cardiac Lesions n 69
A balloon atrial septostomy may be performed to improve the right-to-left
atrial shunt if RV sinusoids are confirmed.
The goals of surgical procedures are to restore ‘‘normal’’ biventricular
circulation and pulmonary blood flow. The initial operation is directed
toward establishing pulmonary blood flow with modified Blalock-Taussig
shunt and promoting normal RV development by relieving RVOTO with
a pulmonary valvotomy or transannular path. Patients who have severe
tricuspid regurgitation, right ventricle-dependent coronary circulation, or
do not have appreciable right ventricular development at follow-up should
be tracked to a Fontan procedure or cardiac transplantation.
Anesthesia Management for Right-to-Left
Congenital Heart Disease
Preoperative assessment and preparation have been discussed in detail
in the other part of this issue.
Prior to induction of anesthesia, routine monitors should be applied
including ECG, pulse oximetry, capnogram, and noninvasive blood pressure measurement. Warm ambient temperature and a quiet induction
room can help peripheral circulation and facilitate intravenous (IV)
access, which sometimes can be very challenging in younger infants. It is
essential that all intravenous fluids be free of air bubbles.
In children who come to the operating suite without an IV line in
place, anesthesia is usually induced with sevoflurane by mask. Ketamine
intramuscular (IM) injection is an alternative for infants without an IV
line. Although, theoretically, a right-to-left shunt may slightly delay the
induction time with inhaled anesthetics, the minor delay is of little
practical concern in the clinical setting.
In patients with existing intravenous access, IV induction of anesthesia
is performed with propofol (1–2 mg/kg) or ketamine (1–2 mg/kg) and
fentanyl (2 ^g/kg). Endotracheal intubation is facilitated with nondepolarizing muscle relaxant, such as pancuronium (0.1 mg/kg), vecuronium (0.1 mg/kg), or cisatracurium (0.2 mg/kg). Because of potential
contraction of the PDA, succinylcholine is preferably avoided.
In patients with right-to-left shunt, a decrease in SVR and/or increase
PVR may further increase shunt. It is therefore crucial to provide adequate
ventilation with oxygen and maintain systemic vascular resistance. Continuous monitoring of the oxygen saturation, blood pressure, ECG, and
end-tidal capnography will help achieve these goals.
Blood pressure cuff and radial artery cannulation should be avoided
on the side where a Blalock-Taussig shunt will be performed, as flow will be
diverted from the subclavian to the pulmonary artery. Placement of arterial
and central venous pressure lines is performed after successful tracheal
intubation. Body temperature should be monitored at 2 different sites
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(rectal and tympanic/esophageal/nasopharyngeal) for children who need
CPB. Urine output is quantified. Left atrial pressure monitoring is useful in
sick children or patients needing complex repairs.
Intraoperative transesophageal echocardiography is being used more
frequently to provide instant morphologic and hemodynamic information, especially during the early phase after CPB.
Anesthesia is usually maintained with IV narcotic (fentanyl 50–
100 ^/kg), benzodiazepine (midazolam 50–100 ^/kg), and isoflurane.
Patients should remain paralyzed throughout the case with nondepolarizing muscle relaxants (pancuronium or cisatracurium). Arterial blood
gases, serum calcium and potassium, and hematocrit should be frequently
monitored. Heparin is usually injected into the right atrium by the surgeon or through the central venous line before cannulation. Protamine
is administered at the conclusion of extracorporeal circulation. In patients with poor ventricular function and prolonged CPB time, dopamine
(3–10 ^g/kg/min) and milrinone (0.5 ^g/kg/min after 50 ^g/kg load)
are commonly used.
Perioperative fluid and ventilation management are very important in
patients with right-to-left shunt lesions. Because these children have higher
hematocrits to maintain oxygen delivery, packed red blood cells should be
transfused to keep the hematocrit optimized. Some patients may benefit
from the addition of slight positive end expiratory pressure because they
have increased pulmonary blood flow (truncus arteriosus, TAPVC). However, in patients with decreased pulmonary blood flow (TOF, pulmonary
atresia with intact ventricular septum, tricuspid atresia) and post-Fontan
procedure, one should avoid using excessive positive airway pressure or
positive end expiratory pressure during ventilation.
Patients who have had a complicated repair, long CPB time, or unstable
cardiopulmonary status are usually left intubated and mechanically ventilated postoperatively. Once the patient is awake, stable, rewarmed to a
normal body temperature, and without metabolic imbalance, the endotracheal tube can be removed.
In summary, understanding the general principles and pathophysiology of congenital heart lesions is essential for the management of a child
with CHD for any type of surgery. Taking care of this group of patients in
the operating room can be very challenging. The anesthesiologist should
be able to predict the effects of various anesthetics and ventilation techniques on the hemodynamic stability of the patients.
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