R i g h t Ve n t r i c u l a r Fa i l u re

and Congenital Heart

Disease
Payton Kendsersky, MDa,*, Cary Ward, MDb

KEYWORDS
 Right ventricle  Congenital heart disease  Tetralogy  Transposition

KEY POINTS
 The overall mortality of ACHD patients with heart failure is significantly higher than patients with
ACHD without heart failure.
 The significance of right ventricular physiology in the development of heart failure in ACHD patients
is increasingly recognized.
 Congenital malformations, palliations, residual defects, and their resultant physiology impact the
RV through both pressure and volume loading.

INTRODUCTION and neurohormonal coupling that exists between

the 2 ventricles has subsequently provided insight
Survivorship into adulthood of patients with into the altered biventricular mechanics of CHD.4
congenital heart disease (CHD) lesions has been Congenital malformations, palliations, residual de-
due in large part to improvement in prenatal detec- fects, and their resultant physiology impact the
tion, novel surgeries and procedural interventions, RV, and this relationship influences morbidity and
and the development of specialized adult CHD mortality. LV mechanics and ventricular–
(ACHD) care.1 As patients survive further into ventricular interactions are adversely affected in
adulthood, the long-term complications of both the pressure loaded and volume loaded
congenital and repaired physiology have been RVs—isolated volume overloading being associ-
more clearly elucidated. A major source of health ated with decreased LV ejection fraction, and
care use in ACHD patients is a result of the treat- pressure overloading being associated with pre-
ment for heart failure.2 The overall mortality of pa- served LV ejection fraction.5 For this discussion,
tients with ACHD with heart failure stands at we therefore categorize right-sided congenital
around 4%, which is significantly higher than pa- heart defects into RV pressure and volume over-
tients with ACHD without heart failure, even after loading lesions, focusing on atrial septal defects,
controlling for age and comorbidities.2 Historically, Ebstein anomaly, tetralogy of Fallot (TOF), trans-
the contribution of left ventricle (LV) dysfunction position of the great vessels, and single RV
has received more attention, owing in part to a bet- physiology.
ter understanding of its morphology and contribu-
tion to normal hemodynamics, resulting in the right
ventricular (RV) moniker “the forgotten ventricle.”3 RIGHT VENTRICULAR VOLUME OVERLOAD
However, it is now well-understood that the LV and
There are several congenital lesions that influence
RV, which share a pericardium, septum, and
cardiac function by overloading the RV with vol-
myocardial fibers, cannot be considered singu-
ume, including atrial septal defects, atrialization
cardiology.theclinics.com

larly. Growing understanding of the mechanical

of the RV (Ebstein anomaly), and corrected TOF.

a b
Duke University Medical Center, Box 2819, Durham, NC 27710, USA; Division of Cardiovascular Medicine,
Duke University Medical Center, Box 2819, Durham, NC 27710, USA
* Corresponding author.
E-mail address: [email protected]

Cardiol Clin 38 (2020) 239–242

https://doi.org/10.1016/j.ccl.2020.02.002
0733-8651/20/Ó 2020 Elsevier Inc. All rights reserved.
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240 Kendsersky & Ward

The RV is able to tolerate an excess of volume for a arrhythmias.12 Studies by Kodaira and col-
long time, mainly owing to the compliance of a leagues12 and Takaya and colleagues13 have
thin-walled RV, a more distensible right atrium as also demonstrated that in patients with moderate
compared with the left atrium, and a roomier or severe tricuspid regurgitation in the setting of
tricuspid valve as compared with the left-sided an unrepaired ASD, tricuspid regurgitation
mitral valve.4 Despite this adaptation of the right severity, and heart failure symptoms were
heart to volume overloading, it is well-understood improved for the vast majority of patients who un-
that long-standing volume overload of the RV derwent percutaneous closure.
leads to adverse ventricular interdependence and
LV dysfunction resulting from myofibril rearrange- Ebstein Anomaly
ment, decreased myofiber preload, and altered
Ebstein anomaly has a wide range of clinical pre-
chamber geometry.6
sentations, but is uniformly characterized by ab-
normalities of the RV and tricuspid valve. The
Atrial Septal Defects anterior leaflet of the tricuspid valve is attached
normally to the annulus, whereas varying portions
An atrial septal defect is a communication be-
of the posterior and septal leaflets are displaced
tween the atria resulting from a deficiency of tissue
downward attaching to the RV wall below the
in the septum and can be subdivided according to
annulus.6,13 In other words, there is an atrialization
anatomic location. The 3 most common types of
of the RV and varying degrees of tricuspid regurgi-
atrial septal defects (ASDs) include ostium secun-
tation. Often there is an associated atrial commu-
dum, ostium primum, and sinus venosus. Of these
nication in the form of a patent foramen ovale. For
3 defects, ostium secundum is the most common
patients surviving into adulthood with an Ebstein
type, accounting for about 70% of all cases.7–9
anomaly, mortality is largely due to heart failure
Ostium secundum defects classically involve the
and arrhythmias.13 In adulthood, a combination
fossa ovalis and can be associated with anoma-
of severe tricuspid regurgitation, severe right
lous pulmonary venous return to the right atrium.
atrium dilation, and reduced effective RV size
Defects of the septum inferior to the fossa ovalis
can result in inadequate antegrade flow and pro-
include ostium primum defects, which occur
gression to RV failure. In addition to the impact
immediately adjacent to the tricuspid and mitral
of volume overload, the natural history of RV failure
valves in a portion of the atrioventricular canal.
in Ebstein anomaly also owes to increased
The least commonly occurring sinus venosus de-
arrhythmia burden, as RV cardiomyocytes pre-
fects, around 6% of cases, straddle an otherwise
serve ventricular specificity despite anatomic atri-
intact septum, but represent a biatrial connection
alization.14 One study confirmed an association
of the superior vena cava.7 Common to all sub-
between myocardial fibrosis and supraventricular
types of ASD is volume overloading of the RV, right
arrhythmias in this population and suggests that
atrium, and pulmonary vasculature. In a small per-
chronic RV volume overload contributes to the
centage of cases of unrepaired ASD, Eisenmenger
development of said fibrosis.15
physiology can develop, characterized by reversal
of the intracardiac shunt, cyanosis, and RV hyper-
Tetralogy of Fallot
trophy and failure. RV failure can also develop in
unrepaired ASDs later in life, even in the absence Characterized by a large ventricular septal defect
of Eisenmenger syndrome, as the left-to-right with overriding aorta, obstruction of pulmonary
shunt magnitude increases with progressive LV blood flow, and RV hypertrophy, TOF is among
stiffness associated with aging.10 In this case, it the most common cyanotic congenital cardiac de-
is both volume and pressure loading that begets fects.6 Among the first lesions to be corrected sur-
right heart failure. ASDs do not close spontane- gically, TOF represents a model not only of the
ously, but surgical or percutaneous closure of impact of abnormal physiology of the defect, but
the ASD results in varying degrees of reversal of also of repair physiology. Classically, infundibular
the increased mortality and morbidities (ie, heart pulmonary stenosis owing to hypertrophy and de-
failure, arrhythmia, and thromboembolic events) viation of the infundibular septum, as well as mal-
associated with unrepaired defects. Current formation and stenosis of the pulmonary valve
guidelines recommend closure of an ASD in pa- result in obstruction of pulmonary blood flow.4
tients with RV enlargement, regardless of symp- Currently, severe pulmonary regurgitation (PR) is
toms.11 Patients who do not undergo ASD the most common residual lesion seen in adult-
closure have worse long-term outcomes, including hood after repair; significant pulmonary annular
decreased functional capacity, greater degrees of hypoplasia requires a transannular patch, which
pulmonary arterial hypertension, and more atrial disrupts the valve architecture.16 Although the

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 RV Failure and CHD 241

surgical approach to the repair of TOF has shifted Transposition of the Great Arteries
recently to a transatrial–transpulmonary approach,
Discordant ventriculoarterial connection underly-
more than 60% of TOF repairs still use transannu-
ing D-transposition of the great arteries results in
lar patch enlargement according to the Society for
displaced aorta and pulmonary artery, so that the
Thoracic Surgery database.17 Over time, the
morphologic RV supports the systemic circulation
resulting severe PR precipitates RV dilation and
and the morphologic LV supports the pulmonary
tricuspid regurgitation, likely in combination with
circulation. These parallel circuits are incompatible
a component of tricuspid valve trauma. Electrical
with life and require atrial or arterial switch opera-
remodeling of the heart after TOF repair also im-
tions.20 The long-term outcomes of the newer
pacts the synchronicity and function of the RVs
arterial switch operations remain to be elucidated,
and LVs.14
but the complications of the older atrial switch op-
In a 1993 review of patients who had under-
erations, the Mustard and Senning procedures,
gone repair of TOF from 1955 to 1960, 22 late
include RV dysfunction in adult survivors of the
deaths were discovered: 10 of these were due
correction.21 The RV coupled to the high-
to sudden cardiac death and 3 of heart failure.
pressure systemic circulation, leading to an
Since then, significant advances in the care of pa-
increased risk of RV dilatation and failure.22 In
tients with repaired TOF have revealed the con-
these patients, there may also be a contribution
nections between PR, RV dilation, longer QRS
of myocardial perfusion defects and RV fibrosis.4
duration, and ventricular arrhythmias.18 There is
The role of medical therapy in a patient with sys-
now recognition that it is important to correct sig-
temic RV dysfunction related to an atrial switch
nificant PR before the RV dilatation and dysfunc-
procedure is unclear. Currently, there is not suffi-
tion becomes too advanced. The 2018 American
cient evidence to recommend the use of
College of Cardiology/American Heart Associa-
angiotensin-converting enzyme inhibitors, beta-
tion Adult Congenital Heart Disease guidelines
blockers, or aldosterone inhibitors in this popula-
recommend regular surveillance of the RV by car-
tion. Although patients with a systemic RV are at
diac MRI to evaluate for size and RV fibrosis.11
risk for ventricular arrhythmias, there is also not
Pulmonary valve replacement is suggested for
enough evidence to recommend primary preven-
patients with severe PR and symptoms, as well
tion implantable cardioverter-defibrillators in this
as for patients with severe PR and mild to moder-
population.11
ate RV dysfunction and/or severe RV dilation,
even in the absence of symptoms.11 The option
Single Right Ventricle Physiology
of a transcatheter pulmonary valve is also avail-
able and thousands have been implanted since The most common of the functional single
they became available in 2000.19 However, the ventricle palliations is the Fontan procedure, which
transcatheter pulmonary valves have specific reroutes systemic venous return directly to the pul-
anatomic requirements that limit their use in a monary artery, eliminating the need for a functional
native RV outflow tract, including a uniform diam- pulmonary ventricle. This palliation comes at the
eter from the RV outflow tract to the pulmonary expense of a chronic increase in the central
artery bifurcation and adequate pulmonary artery venous pressure. Many of the long-term problems
length. They are most commonly used in dysfunc- associated with Fontan physiology are a result of
tional RV to pulmonary artery conduits or as a chronically increased central venous pressure,
second procedure in a preexisting but dysfunc- including liver disease, coagulation factor abnor-
tional bioprosthetic pulmonary valve.19 malities and resultant thromboembolic events,
and protein-losing enteropathy. In instances of hy-
RIGHT VENTRICULAR PRESSURE OVERLOAD poplasia or total absence of the LV, the Fontan
palliation also results in a morphologic RV sup-
Surgical palliation of several different forms of porting systemic circulation.16,23 The high resis-
CHD, including D-transposition of the great ar- tance systemic circulation pressure overloads
teries and hypoplastic left heart syndrome requires the RV, leading to higher likelihood of RV dilation,
reassignment of the RV from its role in the low- tricuspid regurgitation, and RV failure in these pa-
resistance pulmonary circuit to supporting the tients over time as compared with patients with a
systemic circulation. The same is true for the phys- systemic LV. As in Ebstein anomaly, in a cohort
iology of L-transposition of the great arteries or of late Fontan survivors, myocardial fibrosis was
congenitally corrected transposition of the great associated with arrhythmia and adverse ventricu-
arteries. These systemic RV systems represent in- lar mechanics.22 The medical management of pa-
stances of RV pressure overload, leading to RV tients with a single ventricle is the same regardless
dysfunction and failure. of the morphology of that ventricle.11

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242 Kendsersky & Ward

DISCLOSURE 12. Kodaira M, Kawamura A, Okamoto K, et al. Compar-

ison of clinical outcomes after transcatheter vs. Mini-
The authors have nothing to disclose. mally invasive cardiac surgery closure for atrial
septal defect. Circ J 2017;81(4):543–51.
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