alternative operations have been devised to create a direct connection between the aorta and the

anomalous coronary artery. Most commonly, this can be achieved by removing the origin of the left

coronary artery (along with a button of adjacent PA) and reimplanting the vessel directly into the side

of the aorta. Another approach involves creation of a side-to-side connection between the aorta and PA

with placement of an intrapulmonary baffle to direct flow from this connection to the anomalous left

coronary ostium. With improved coronary transfer techniques learned from the arterial switch

operation, direct reimplantation of the anomalous coronary artery is always feasible and preferred.

Survival following surgical repair of ALCAPA has improved over the years.146 Recent reports have

suggested an operative mortality of 6% or less.147,148 Ventricular function tends to normalize after

surgery.149 In most patients, mitral valve function also improves,147,149 but for patients with severe

mitral regurgitation, concurrent mitral valve repair may rarely be indicated.

Anomalous aortic origins of the coronary arteries are usually minor defects, but a potentially

dangerous abnormality exists when the left main coronary artery arises from the right coronary sinus

and passes between the PA and aorta. This defect has been associated with cardiac symptoms and

sudden death, as has the origin of the right coronary artery from the left coronary sinus (usually when

the right coronary is dominant).150,151 The etiology of ischemia in both defects is thought to be related

to the acute angle of origin and slit-like orifice of the anomalous vessel often with an intramural course

and the extrinsic compression created by the opposing walls of the aorta and PA. These defects usually

present in older patients. Symptomatic patients are treated surgically by unroofing the coronary ostia

into the aorta or by coronary artery bypass.

Myocardial bridging occurs when a segment of an epicardial coronary artery (usually the left anterior

descending) takes an intramyocardial course over a short segment. Although this is a common incidental

finding at cardiac catheterization, this defect has been associated in some cases with myocardial

ischemia. Treatment involves dividing the muscle bridge to free the coronary, coronary bypass beyond

the bridge, or transcatheter stenting.

Coronary aneurysms occur rarely, usually in conjunction with an inflammatory condition such as

Kawasaki syndrome, polyarteritis nodosa, Takayasu arteritis, or syphilis. Coronary aneurysms may

thrombose or lead to distal coronary stenosis or embolization. Rupture occurs uncommonly. Treatment

ranges from antiplatelet therapy to coronary bypass grafting.

UNIVENTRICULAR HEART

In the univentricular heart, only one ventricular chamber is connected to the atria. To be classified as a

ventricle, a chamber must receive at least half of an inlet valve. In the most common form of

univentricular heart, both the mitral and tricuspid valves connect to a morphologic LV (double-inlet

LV), which ejects blood through a hypoplastic outlet chamber and then, due to malposition of the great

arteries, to the aorta. The outlet chamber is not considered to be a ventricle, regardless of its size,

because it does not receive an inlet valve. Similarly, the intracardiac communication is not termed a

VSD, but a bulboventricular foramen. Univentricular hearts are frequently associated with malpositions

of the great vessels and varying degrees of obstruction to PBF. In double-inlet LV, the aorta is usually

anterior and to the left of the PA.

The presentation of infants with univentricular heart is variable, depending on the amount of PBF.152

When the pulmonary flow is excessive, cyanosis may be mild, and the dominant feature is CHF.

Associated PS decreases the PBF, and the degree of cyanosis is increased. Associated lesions may further

complicate the picture, such as coarctation, subaortic stenosis, or a restrictive ASD. Patients with

moderate PS may achieve a well-balanced circulation with acceptable systemic oxygenation and normal

PA pressure. These patients may be symptom-free well into adolescence. Most patients, however,

require intervention early in life to reduce excessive PBF or to increase it in the presence of significant

PS. Pulmonary vascular obstructive disease develops early when PBF is excessive. With the possible

exception of patients in whom the pulmonary and systemic blood flow is well balanced, the prognosis

for patients with unoperated univentricular hearts is poor, with more than half dying early of CHF or

dysrhythmias.

In the presence of excessive PBF and pulmonary hypertension, operation should be performed early in

life to control PBF and prevent the development of pulmonary vascular occlusive disease. Options

include PA banding or division of the main PA in conjunction with a controlled aortopulmonary shunt.

PA banding is a less complicated procedure; however, it is often difficult to adjust the pulmonary flow

accurately, and too proximal or too distal a band can lead to distortion of the pulmonary valve or

2353

branch pulmonary arteries, which further complicates later operations. Simple division of the PA with

placement of a modified Blalock–Taussig shunt can be undertaken in the presence of an adequate

bulboventricular foramen providing unobstructed flow to the aorta. This option avoids the problems

associated with a PA band, and more accurately controls the PBF. Another similar technique is division

of the main PA, side-to-side anastomosis of the proximal PA with the native aorta, and placement of a

modified Blalock–Taussig shunt (modified DKS procedure). It is not uncommon for the bulboventricular

foramen to decrease in size over time due to progressive ventricular hypertrophy. Maintaining

continuity from both the outlet chamber and the LV eliminates the possibility of subaortic obstruction,

which can occur when the systemic blood flow depends entirely on egress through a bulboventricular

foramen. In infants who are well balanced and can be safely managed medically until the age of 4 to 6

months, a hemi-Fontan or bidirectional Glenn, in which the superior vena caval flow is directed into the

pulmonary arteries, can be used to increase the effective PBF. This procedure maximizes pulmonary

flow without causing a volume overload to the single ventricle. It is most commonly used as part of an

interim stage to a complete atriopulmonary connection or Fontan procedure.

The ultimate goal of surgical correction in patients with a univentricular heart is the total diversion of

all vena caval blood directly into the pulmonary arteries. The Fontan procedure was first successfully

performed in a patient with tricuspid atresia, but has since evolved as an excellent way to establish

physiologic repair for patients with more complex forms of univentricular heart.153 The superior vena

caval blood returns directly via an end-to-side anastomosis with the PA (bidirectional Glenn) or through

a right atrial–PA connection (hemi-Fontan). The inferior vena caval flow is directed to the PA with an

intra-atrial baffle (lateral tunnel technique, Fig. 81-10) or an extracardiac conduit. All oxygenated

pulmonary venous flow empties into the ventricular chamber through the AV valves to be ejected to the

systemic circulation, while superior and inferior vena caval blood flows directly to the lungs to acquire

oxygen prior to returning to the heart. For the Fontan procedure to be performed with a low operative

mortality and an acceptable functional result, certain criteria must be met. Normal PA pressure (<20

mm Hg) and PVR (<2 Woods units/m2) are the most important prerequisites. Additionally, it is

essential that ventricular function and AV valve function be normal. Many of the criteria originally

proposed, including normal cardiac rhythm, right atrial hypertrophy, normal systemic venous return,

and age older than 4 years, are of little or no importance in the current era. Although the Fontan

procedure cannot be considered a truly corrective operation, it offers benefits that cannot be equaled by

any of the other palliative procedures. The major advantages include restoration of normal systemic

oxygen saturation and reduction of ventricular volume overload.

Figure 81-10. Total cavopulmonary connection for univentricular heart. The internal orifices of the superior and inferior venae

2354

cavae are connected in the right atrium with a patch cut of polytetrafluoroethylene. The superior vena cava is divided just above

its junction with the right atrium, and both ends are anastomosed to the right pulmonary artery. The main pulmonary artery is

ligated.

Figure 81-11. Steps in the Norwood procedure for hypoplastic left-heart syndrome. A: Cannulation for bypass. B: Division of

pulmonary artery and ductus arteriosus. C: Aortic arch opened from the origin to beyond the ductus. D: Pulmonary allograft used

to enlarge arch and connect to the ventricle. E: Completed repair.

2355

Ventricular septation procedures have also been successfully performed in patients with univentricular

hearts. The subset of patients with a double-inlet LV, anterior and leftward aorta, nonrestrictive outlet

foramen, and mild or no PS are best suited for septation. This anatomy allows placement of a relatively

direct and straight prosthetic patch in the ventricle that separates the pulmonary and systemic

circulations. The septation procedure has been associated with a relatively high morbidity, primarily

related to complete heart block, so that its overall effectiveness is reduced. Few centers have continued

to apply this procedure in carefully selected patients.

The current results for the Fontan are excellent, with hospital mortality ranging from 2% to

9%.154–158 The condition of survivors is generally good and most attain a functional status of New York

Heart Association class I or II. The long-term results have been reported with a 93% to 97% 5-year

survival and a 91% 10-year survival.156,157 Although long-term results are encouraging, late

complications may be seen. Continued surveillance for arrhythmias, CHF, protein-losing enteropathy,

and hepatic dysfunction remains important.

HYPOPLASTIC LEFT- HEART SYNDROME

Hypoplastic left-heart syndrome (HLHS) refers to a constellation of congenital cardiac anomalies

characterized by marked hypoplasia or absence of the LV and severe hypoplasia of the ascending aorta.

The systemic circulation is dependent on the right ventricle via a PDA and there is obligatory mixing of

pulmonary and systemic venous blood in the right atrium. There is associated aortic valve stenosis or

atresia, and mitral valve stenosis or atresia. The descending aorta is essentially a continuation of the

ductus arteriosus, and the ascending aorta and aortic arch are a diminutive branch from this vessel.

Initial management includes a prostaglandin infusion to maintain ductal patency and correction of

metabolic acidosis. The patient may require intubation and ventilator adjustment to reduce

supplemental oxygen and maintain a partial pressure of carbon dioxide (PCO2

) of about 40 mm Hg to

avoid excessive pulmonary flow.

Treatment options for this problem include cardiac transplantation and staged reconstructive surgery.

Transplantation for HLHS is generally performed with the same techniques that are standard for

transplantation in older children and adults. However, it is necessary to have a generous amount of

donor aortic arch that can be used to augment the hypoplastic recipient arch. Results of transplantation

in neonates have been excellent in centers with extensive experience in this area, and a 2-year survival

as high as 70% has been reported.159 In the context of improving results for staged reconstruction, risks

of immunosuppression, and limited donor availability, competing risk analysis favors staged repair, and

most centers pursue this option as primary therapy for HLHS.160 Transplantation is generally reserved

for very high-risk patients, such as those with depressed right ventricular function or severe tricuspid

regurgitation.

The first successful palliation of HLHS was reported by Norwood et al. on a series of infants operated

on between 1979 and 1981.161 This procedure has been technically refined over the years, but three

essential components remain: atrial septectomy, anastomosis of the proximal PA to the aorta with

homograft augmentation of the aortic arch, and aortopulmonary shunt or right ventricle–PA conduit

(Fig. 81-11). As described previously for the univentricular heart, subsequent reconstructive

management of HLHS includes an interim hemi-Fontan procedure or bidirectional Glenn anastomosis at

4 to 6 months of age, followed by a Fontan procedure at about 18 to 24 months.

Universally fatal only two decades ago, tremendous strides have been made in improving the

outcomes for patients with HLHS. Of the three stages, the highest-risk stage of the repair remains the

Norwood operation. During the 1990s, the hospital survival for the Norwood procedure across the

United States was approximately 40%.162 Currently, select centers have reported hospital survivals of

90% or greater.163–167 Reported survivals for the hemi-Fontan and Fontan procedures have also been

excellent at 98% for both operations.155,157,168

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