Hypoplastic Left Heart Syndrome

HLHS comprises a wide spectrum of cardiac malformations, including hypoplasia or atresia

of the aortic and mitral valves and hypoplasia of the LV and ascending aorta.100 HLHS has
a reported prevalence of 0.2 per 1000 live births and occurs twice as often in boys as in girls.
Left untreated, HLHS is invariably fatal and is responsible for 25% of early cardiac deaths in
neonates.However, the recent evolution of palliative surgical procedures has dramatically
improved the outlook for patients with HLHS, and an improved understanding of anatomic
and physiologic alterations has spurred advances in parallel arenas such as intrauterine
diagnosis and fetal intervention, echocardiographic imaging, and neonatal critical care.
Anatomy. As implied by its name, HLHS involves varying degrees of underdevelopment of
left-sided structures, including the LV and the aortic and mitral valves. Thus, HLHS can be
classified into four anatomic subtypes based on the valvular morphology: (a) aortic and mitral
stenosis; (b) aortic and mitral atresia; (c) aortic atresia and mitral stenosis; and (d) AS and
mitral atresia. Aortic atresia tends to be associated with more severe degrees of hypoplasia of
the ascending aorta than does AS. Even in cases without frank aortic atresia, however, the
aortic arch is generally hypoplastic and, in severe cases, may even be interrupted. There is an
associated coarctation shelf in 80% of patients with HLHS, and the ductus itself is usually
quite large, as is the main pulmonary artery.The segmental pulmonary arteries, however, are
small, secondary to reduced intrauterine pulmonary blood flow, which is itself a consequence
of the left-sided outflow obstruction. The left atrial cavity is generally smaller than normal
and is accentuated because of the leftward displacement of the septum primum.
There is almost always an interatrial communication via the foramen ovale, which can be
large, but more commonly restricts right-to-left flow. In rare cases, there is no atrial-level
communication, which can be lethal for these infants because there is no way for pulmonary
venous return to cross over to the RV.
Associated defects can occur with HLHS, and many of them have importance with respect to
operative repair. For example, if a VSD is present, the LV can retain its normal size
during development even in the presence of mitral atresia. This is because a right-to-left
shunt through the defect impels growth of the LV.This introduces the feasibility of
biventricular repair for this subset of patients. Although HLHS undoubtedly results from a
complex interplay of developmental errors in the early stages of cardiogenesis, many
investigators have hypothesized that the altered blood flow is responsible for the structural
underdevelopment that characterizes HLHS. In other words, if the stimulus for normal
development of the ascending aorta from the primordial aortic sac is high-pressure systemic
blood flow from the LV through the aortic valve, then an atretic or stenotic aortic valve,
which impedes flow and leads to only low-pressure diastolic retrograde flow via the ductus,
will change the developmental signals and result in hypoplasia of the downstream structures.
Normal growth and development of the LV and mitral valve can be secondarily affected,
resulting in hypoplasia or atresia of these structures.

and the distance between the tricuspid and pulmonary valve. Cardiac catheterization is rarely
necessary in neonates or infants, except to determine the degree of pulmonary hypertension
and to determine the effects of previous palliative procedures on the pulmonary arterial
anatomy.
Therapy. The goals of corrective surgery are to relieve pulmonary stenosis, to provide
separate and unobstructed outflow pathways from each ventricle to the correct great vessel,
and to achieve separation of the systemic and pulmonary circulations.
Double-Outlet Right Ventricle with
Noncommitted Ventricular Septal Defect
The repair of hearts with DORV and noncommitted VSD can be accomplished by
constructing an intraventricular tunnel connecting the VSD to the aorta, closing the
pulmonary artery, and placing a valved extracardiac conduit from the RV to the
pulmonary artery. In patients without pulmonary stenosis who have intractable congestive
failure, a pulmonary artery band can be placed in the first 6 months to control pulmonary
artery overcirculation and prevent the development of pulmonary
hypertension.
Infants with pulmonary stenosis can be managed with a systemic-to-pulmonary shunt
followed by biventricular repair as described by Belli and colleagues in 1999, or with a
modified Fontan.There is no consensus on the timing of repair, but recent literature suggests
that repair within the first 6 months is associated with better outcome. However, in cases
where an extracardiac valved conduit is necessary, it is better to delay definitive repair until
the child is 2 to 3 years of age, because this allows placement of a larger conduit and possibly
reduces the number of future obligatory conduit replacements.
Double-Outlet Right Ventricle with Subaortic or Doubly Committed Ventricular Septal
Defect Without Pulmonary Stenosis
This group of patients can be treated by creating an intracardiac baffle that directs blood from
the LV into the aorta. Enlargement of the VSD may be necessary to allow ample room for the
baffle; this should be done anterosuperiorly to avoid injury to the conduction system that
normally lies inferoposteriorly along the border of the VSD. In addition, other important
considerations in constructing the LV outflow tunnel include the prominence of the conal
septum, the attachments of the tricuspid valve to the conal septum, and the distance between
the tricuspid and pulmonary valves. In some instances, unfavorable anatomy may preclude
placement of an adequate intracardiac baffle, necessitating single ventricle repair.
Double-Outlet Right Ventricle with Subaortic or Doubly Committed Ventricular Septal
Defect with Pulmonary Stenosis
Repair of this defect is similar to the above except that concomitant RVOT reconstruction
must be performed in addition to the intracardiac tunnel. The RVOT augmentation can be
accomplished with the placement of a transannular patch or with placement of an
extracardiac valved conduit when an anomalous left anterior descending artery precludes use
of a patch.
Taussig-Bing Syndrome without Pulmonary Stenosis
These infants are best treated with a balloon septostomy during the neonatal period to
improve mixing, followed by VSD closure baffling LV egress to the pulmonary artery and an
arterial switch operation. The Kawashima procedure, in which an intraventricular tunnel is
used to baffle LV egress directly to the aorta, may alternatively be used when the aorta is
more posterior or when there is associated pulmonary stenosis.
Taussig-Bing Syndrome with Pulmonary Stenosis
This defect may be treated with a variety of techniques,depending on the specific anatomic
details and the expertise of the treatment team. A Rastelli-type repair, which involves
construction of an intraventricular tunnel through the existing VSD that connects the LV to
both great vessels, followed by division of the pulmonary artery at its origin and insertion of
a valved conduit from the RV to the distal pulmonary artery, can be performed.Alternatively,
a Yasui procedure, which involves baffling the VSD to the pulmonary artery and creation
of a DKS anastomosis between the pulmonary artery and the aorta with patch augmentation,
can be accomplished concomitant with placement of a RV-pulmonary artery conduit.
Results. The results of DORV repairs are generally favorable, especially for the tetralogy-
type DORV with subaortic VSD. However, more complex types of DORV, including
noncommitted VSD and Taussig-Bing type, still carry important morbidity and mortality.
Furthermore, repeated interventions for RVOT reconstruction or staged operations for
patients triaged to single-ventricle pathways pose late hazards for patients surviving initial
repair. A recent single-institution series evaluated 393 patients with DORV.138 The authors
found that the need for reintervention approached 37% at 15 years following repair. Arterial
switch operation, as opposed to Rastelli- type repair, was associated with an increased risk of
early postrepair mortality, but mitigated against the risk of late death. Patients with
hypoplastic left-sided structures and a nonsubaortic VSD may fare better with a single
ventricle repair.
Tetralogy of Fallot
Anatomy. The original description of tetralogy of Fallot (TOF) by Ettienne Louis Fallot,as
the name implies, included four abnormalities: a large perimembranous VSD adjacent to the
tricuspid valve; an overriding aorta; a variable degree of RVOT obstruction, which might
include hypoplasia and dysplasia of the pulmonary valve as well as obstruction at the
subvalvar and pulmonary artery level; and right ventricular hypertrophy.
More recently, the Van Praagh et al141 pointed out that TOF could be more correctly termed
monology of Fallot, since the four components are explained by the malposition of the
infundibular septum. When the infundibular septum is displaced anteriorly and leftward, the
RVOT is narrowed and its anterior displacement results in failure of fusion of the ventricular
septum between the arms of the trabeculo-septo-marginalis (Fig. 20-26).
The morphology of TOF is markedly heterogeneous and includes an absent pulmonary valve,
concomitant AV septal defects, and pulmonary atresia with major aortopulmonary collaterals.
The present discussion will focus only on the so-called classic presentation of TOF without
coexisting intracardiac defects.
Anomalous coronary artery patterns, related to either origin or distribution, have been
described in TOF. However, the most surgically important coronary anomaly occurs when
the left anterior descending artery arises as a branch of the right coronary artery. This occurs
in approximately 3% of cases of TOF and may preclude placement of a transannular patch, as
the left anterior descending coronary artery crosses the RVOT at varying distances from the
pulmonary valve annulus