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Double Inlet Left Ventricle
161
vomiting, and pain, as well as arterial or venous blockage
due to spasm or thrombosis.
Blood vessel rupture, bradyarrhythmias, tachyarrhythmias,
and vascular blockage are all possible complications [9].
Treatment
Patients with a single ventricle should be admitted for tests
and surgery. Patients with a single ventricle are supposed to
be evaluated as an inpatient in an intensive care unit. Patients
with severe pulmonary outow tract or aortic arch obstruction should receive intravenous prostaglandin E1 immediately after birth. The initial arterial blood gas test is the best
indicator of the requirement for an arterial line and assisted
ventilation. Prior to the Fontan operation, patients’ resting
cardiac index ranges from 70 to 80 percent normally.
Additionally, ability to raise cardiac output is limited and
usually leads to a reduction in exercise capacity.
Surgical Procedures
There are three main procedures used to treat DILV which
are:
1. Blalock-Taussig shunt: This is the initial surgery that is
normally performed within the rst weeks of the life of
the baby, and it involves the use of a tiny tube to guide
blood ow to the lungs.
2. The Glenn treatment is done within the rst 6months of
the baby’s life, and surgeons direct blood ow from the
upper body to the pulmonary artery, allowing the blood to
collect oxygen from the lungs without passing through
the heart.
3. Fontan procedure: This is the third surgery, which is nor-
mally performed when the child is between the ages of
two and three. The doctors will now separate the circulation so that blood does not mingle. The workload on the
solitary ventricle is likewise reduced. Although this surgery does not restore the normal circulation of the blood
to the body, it only improves it.
Complications oftheFontan Procedure Include
• Intolerance of heavy exercise.
• Protein-losing enteropathy.
• Poor ventricular function.
• Mortality.
• Heart blocks or dysrhythmias.
• Hepatomegaly and/or cardiomegaly.
• Ascites also might develop or edema of the limbs.
• Thrombosis of systemic veins.
Additionally, other operations may be necessary while the
infant waits for the Fontan surgery, and the kid may require
anticoagulants digitalis, diuretics, and ACE inhibitors for the
remainder of his or her life or before and after surgery. In
extreme circumstances, as the patient reaches maturity, a
heart transplant may be necessary. It is critical to recognize
that the Fontan method is constantly changing. Over extended
follow-up periods, patients treated in this manner continue to
be at risk for an increased incidence of heart failure, cardiac
arrhythmias such as supraventricular arrhythmias, predominantly due to right atrial dilatation, and pulmonary
hypertension.
Aortic Arch Blockage or Stenosis
ofthePulmonary Outow Tract
If pulmonary stenosis exists, the severity of the condition
determines if a systemic-to-pulmonary artery shunt is
required following the ductal closure. In the presence of an
obstruction to the aortic arch, the commonest treatment is
mainly done to restore the ow of the unobstructed aortic
arch while restricting the pulmonary blood continue to ow.
The reason is that most of the patients with arch obstruction
have a bulboventricular foramen that is tiny, banding the pulmonary artery has given place to other means of reducing
pulmonary blood ow. Bulboventricular foramen diameter
has the tendency to shrink in overtime and might suddenly
shrink in caliber after unloading treatment for the volume by
pulmonary artery banding, even if it is not initially restricted
(also the Fontan operations and hemi-Fontan) [10].
Reconstruction by a Norwood type (the anastomosis
between the proximal pulmonary artery to the aorta) is now
preferred to eliminate the potential of hemodynamically substantial systemic outow tract stenosis. The reason is the
proximity between conduction system and the occurrence of
atrioventricular valve connection into the rim frequently,
making the bulboventricular foramen larger by muscle resection is dangerous.
The Physiologic Sensitivity ofInfants Treated
withaNorwood-Type Operation WasReduced
Although this is not always the case in single ventricle
patients, physiology of palliation of newborn after treatment
by Norwood-type had been generally noted to be frail relatively. This was found to be attributable to tiny aorta. Because
the coronary artery owing is primarily or could be completely dependent on retrograde aortic perfusion in the case
of aortic valve atresia, the coexisting customized Blalock-
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A. D. Al-Obaidi et al.
Taussig shunt creates “diastolic steal” incident that is considered extremely susceptible to pulmonary vascular resistance
changes. Actually, the mortality during the transition from
the rst stage to the second stage had found to be high for
decades, also in some facilities, patients are kept in the hospital for the whole interstate period.
Creating aCavopulmonary Circulatory System
Due to the acute volume unloading there is an association with
an acute upsurge in ventricular thickness of the wall, it is safer
to create a cavopulmonary circulation in phases over 1–2years.
The diastolic performance of the single ventricle is signicantly altered by this increase in wall thickness, which might
decrease cardiac output. The hemi-Fontan procedure improves
systemic blood ow (cardiac output) compared to the nonpenetrated “full Fontan” procedure since the cardiac output
reduces by a smaller amount. The less-than- complete Fontan
is now thought to offer the best blend of near-normal arterial
oxygen saturation and low exuberant complication rates. As a
result, even the “ultimate” step frequently leads to the formation of a fenestrated Fontan, where nearly all the vena cava
ow is directed to the pulmonary arteries. Whatever the structure dividing the pulmonary venous pathway from the systemic venous pathway, a single 4 mm diameter hole in or
numerous 2–3mm in diameter holes are drilled. Although the
latter form is characterized by nal spontaneous closure, some
patients develop protein- losing enteropathy and require placement of a catheter or surgery in order to establish a stable fenestration. Partial hepatic vein exclusion is a less-than-complete
Fontan option. When the bafe is inserted, left anterior hepatic
vein, one hepatic vein usually, could be expelled from the
venous systemic stream. Hepatic vein drains into the pulmonary venous system were ruled out. Unfortunately, most
patients who have had their hepatic veins partially removed
eventually develop collaterals from the right hepatic vein to
the left hepatic vein.
Transplantation oftheHeart
Those who have had the Fontan operation and have experienced major problems, as well as the patients who has hemodynamics make them poor candidates for the Fontan
procedure, may be candidates for cardiac transplantation.
Prevention
Due to the fact that the etiology of double inlet left ventricle
is unknown, there is no method to prevent it entirely.
However, you can begin preparing for a healthy pregnancy
even before you get pregnant by taking care of yourself.
Consider the following:
• Eat healthy types of foods and try to exercise regularly to
keep or to get a healthy weight.
• Visit your healthcare professional before getting pregnant
to nd out how healthy you are and to share your family’s
health issues and history.
• Stop smoking if you are a smoker. As well as the same
goes for drinking and/or using prescription or street drugs.
As those things could harm your baby.
• Take a 400-mcg of folic acid supplement in order to pre-
vent brain and spine defects.
Dierence inBetween theDouble Outlet RV
andtheDouble Inlet LV
Both (DORV) and (DILV) are congenital anomalies that
result in single ventricle formation. This concludes that the
heart, that ordinarily consists of 2 different chambers for
pumping (left and right ventricles), now consists of a single
functioning chamber.
DORV and DILV are both single ventricle abnormalities
that are related to hypoplastic left heart syndrome and tricuspid atresia. Additionally, pulmonary atresia and some types
of atrioventricular canal abnormalities may contribute to the
development of the diseases associated with a heart with a
single ventricular [11].
Generally, the RV which is the heart’s chamber lying on
the lower right, gets oxygen-depleted blood from the right
atrium on its way to other parts of the body. The RV delivers
blood to the lungs by the pulmonary artery. The left ventricle
is the heart’s chamber lying to the lower left which gets
blood rich in oxygen from the lungs given by the atrium on
the left. Left ventricle then pumps this blood under pressure
which is high and distributes it to the other parts of the body
through the aorta.
Great arteries (aorta and pulmonary artery) both of them
nd their exit through the right ventricle in DORV, and the
patients are often found to have associated (VSD) ventricular
septal defect. On the other hand, in people with DILV, the
great arteries are inverted and both atria drain to an abnormal
left ventricle as it is enlarged in its size.
The RV is typically reported in DILV to be small, and
both atrial septal defects (ASDs) and VSDs may be present
[12].
Double Inlet Left Ventricle
163
Multiple Choice Questions
1. The gene defect that mostly associated with single
ventricle.
A. Tbx5 misexpression in the ventricles.
B. GATA4 inactivation.
C. GATA3 inactivation.
D. Both A and B.
2. Patients who are LEAST cyanotic, are those with aortic
arch obstruction and a single ventricle because.
A. Pulmonary stenosis does not develop.
B. They usually develop pulmonary stenosis.
C. They never develop aortic stenosis.
D. Both A and C.
3. Most single-ventricle patients live.
A. For at least 10years.
B. For at least 20years.
C. 10–15years.
D. 5years only.
4. The most common of the multiple late complications
that can occur after various Fontan alterations.
A. Atrial tachyarrhythmias.
B. Thromboembolism.
C. Plastic bronchitis.
D. Malformations of pulmonary AV system.
5. Which of the following is FALSE according to thromboembolic complications in DILV?
A. Nearly 10% of those who survive the fenestrated
Fontan procedure have venous thrombosis,
B. Arterial thrombosis is common,
C. The pulmonary arteries and cerebral veins are exam-
ples of possible sites.
D. Altered hepatic syntheses of endogenous thrombo-
lytic pathway components have all been presented as
plausible etiologies.
6. According to the “DILV” with leftward and anterior
aorta (left-handed ventricular topology/ l-looped ventri-
cles) which one is false.
A. It’s the least common variant.
B. Severe overriding in addition to straddling of Lt-
sided AV valve leads to commitment of it to the left
ventricle.
C. VSD ensures a route of communication to the hypo-
plastic Lt-sided Rt ventricle.
D. Obstruction in the subaortic area can be associated
with VSD that is restrictive in type.
7. “DILV” with rightward and anterior aorta (right-handed
ventricular topology/D-looped) which one is true.
A. Left side hypoplastic subaortic incomplete/hypo-
plastic RV cavity.
B. Morphologic Lt ventricle which is right side.
C. VSD ensures a route of communication to hypoplas-
tic Rt-ventricle.
D. Obstruction in the subaortic area can be associated
with VSD that is restrictive in type.
8. In monitoring of the complication of protein-losing
enteropathy (PLE) after a Fontan operation we depend
on.
A. The detection of fecal alpha1-antitrypsin.
B. Lipoproteins lipase level.
C. Doppler analysis.
D. Holter Placement.
9. Regarding Glenn treatment which one is false.
A. Performed when the baby is roughly 6months old.
B. Surgeons route blood ow from the upper body to
the pulmonary artery.
C. Allowing the blood to collect oxygen from the lungs
without passing through the heart.
D. It’s the initial surgery.
10. The false statement regarding Fontan procedure.
A. Normally performed when the child is between the
ages of two and three.
B. Separate the circulation so that blood does not
mingle.
C. The workload on the solitary ventricle is likewise
reduced.
D. Although this surgery restores normal blood circula-
tion to the body.
Answers
1. D.
2. A.
3. B.
4. A.
5. B.
6. A.
7. C.
8. A.
9. D.
10. D.
References
1. Sanders SP. Hearts with functionally one ventricle. In: Lai WW,
Mertens LL, Cohen MS, Geva T, editors. Echocardiography in
pediatric and congenital heart disease: from fetus to adult. 2nd ed.
Boston, USA: Wiley Blackwell; 2016. p.509–40.
2. Earing MG, Hagler DJ, Edwards WD. Univentricular atrioventricular connection. In: Allen HD, Shaddy RE, Penny DJ, Feltes
TF, Cetta F, editors. Moss & Adams’ heart disease in infants, children, and adolescents, including the fetus and young adult. 9th ed.
Lippincott Williams & Wilkins; 2016.
3. Penny DJ, Anderson RH.Other forms of functionally univentricular hearts. In: Anderson RH, Baker EJ, Penny DJ, Redington AN,
Rigby ML, Wernovsky G, editors. Paediatric cardiology. 3rd ed.
London: Churchill Livingston; 2010.
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4. von Both I, Silvestri C, Erdemir T, etal. Foxh1 is essential for development of the anterior heart eld. Dev Cell. 2004;7(3):331–45.
5. Sanford LP, Ormsby I, Gittenberger-de Groot AC, etal. TGFbeta2
knockout mice have multiple developmental defects that are
non-overlapping with other TGFbeta knockout phenotypes.
Development. 1997;124(13):2659–70.
6. Gottlieb PD, Pierce SA, Sims RJ, et al. Bop encodes a musclerestricted protein containing MYND and SET domains and is
essential for cardiac differentiation and morphogenesis. Nat Genet.
2002;31(1):25–32.
7. Camenisch TD, Spicer AP, Brehm-Gibson T, et al. Disruption of
hyaluronan synthase-2 abrogates normal cardiac morphogenesis
and hyaluronan-mediated transformation of epithelium to mesenchyme. J Clin Invest. 2000;106(3):349–60.
8. Zeisberg EM, Ma Q, Juraszek AL, Moses K, Schwartz RJ, Izumo
S, etal. Morphogenesis of the right ventricle requires myocardial
expression of Gata4. J Clin Invest. 2005;115(6):1522–31.
9. Pizzuto M, Patel M, Romano J, etal. Similar interstage outcomes
for single ventricle infants palliated with an aortopulmonary shunt
compared to the Norwood procedure. World J Pediatr Congenit
Heart Surg. 2018;9(4):407–11.
10. Simsic JM, Phelps C, Kirchner K, et al. Interstage outcomes in
single ventricle patients undergoing hybrid stage 1 palliation.
Congenit Heart Dis. 2018;13(5):757–63.
11. Rydberg A, Teien DE, Krus P.Computer simulation of circulation
in patient with total cavo-pulmonary connection: inter-relationship
of cardiac and vascular pressure, ow, resistance and capacitance.
Med Biol Eng Comput. 1997;35(6):722–8.
12. Poterucha JT, Anavekar NS, Egbe AC, et al. Survival and outcomes of patients with unoperated single ventricle. Heart.
2016;102(3):216–22.
Double Outlet Right Ventricle
MaysSufyanAhmad
Abstract
Dual outlet right ventricle (DORV) is a cardiac abnormality involving the outow of the right ventricular system,
the great arteries both originate from the right ventricle.
Categorization of the group of abnormalities is based on
the location of the VSD in relation to the aortic and pulmonary arteries. The location of the VSD has a signicant
impact on the physiologic symptoms of DORV as well as
surgical concerns (subpulmonary, subaortic, committed,
and non-committed VSD types). A comprehensive history
of the patient’s illness and physical examination to assess
the precordium and respiratory system should be used to
identify clinically relevant heart abnormalities.
Echocardiography typically gives enough information for
a precise and appropriate diagnosis, as well as the information needed to design the surgical strategy. The pulmonary stenosis presence greatly inuences the patient’s
symptom prole and age at the time of clinical manifestation. Most of the patients were present earlier, in neonatal
period. ECG, x-ray, cardiac angiography, and MRI were
used but not standard or well-established diagnostic technique for this disease. CT scanning may be benecial in
determining coronary artery architecture in infants with
TOF type. Medical and surgical treatment depends on the
types of DORV and the associated anomalies. Palliative
surgery can be done until the denitive treatment is possible. The prognosis also depends on whether the patient
did a surgery or not, the type of intervention, and the
underlying anomaly.
Keywords
Double outlet · Right ventricle · Congenital heart
Pulmonary stenosis · Congestive heart failure
Taussig- Bing heart · Committed VSD · Non-committed
VSD · Bidirectional Glenn · Tetralogy of Fallot
M. S. Ahmad (*)
College of Medicine, University of Baghdad, Baghdad, Iraq
Introduction
The term “dual outlet right ventricle” (DORV) refers to a
diverse group of related cardiac abnormalities involving the
outow of the right ventricular system in which both great
arteries originate from the right ventricle. This anatomic
defect can range from tetralogy of Fallot (TOF) to full transposition of the great arteries (TGA).
The incidence of DORV is estimated to be 0.09 per 1000
live births in the United States. About 1–1.5% of all congenital cardiac disease is caused by DORV.There has been
no identication of a single causative agent or predicted
event.
The clinical picture might range from severe cyanosis to
fulminant congestive heart failure. DORV denition has
been a source of contention among congenital cardiac surgery specialists. In general, classifying the lesion as DORV
is appropriate from a surgical standpoint when more than
50% of both major arteries emerge from the right ventricle.
Typically, one artery and the majority of the other vessels
emerge from the right ventricle. Others specify that if the
brous continuity between the arterial and atrioventricular
valves is absent, then that is a characteristic feature of
DORV.Lev etal. (1972) revised this categorization by proposing that aortomitral brous discontinuity be needed.
Furthermore, Lev et al. started to categorize the group of
abnormalities based on the location of the VSD (its location
regarding the great vessels) [1].
The following are the occurrence rates of related cardiovascular anomalies:
• Pulmonary stenosis: 20–45% (most common with VSD
of subaortic type).
• Atrial septal defect: 20–25%.
• Patent ductus arteriosus: 15%.
• Atrioventricular canal: 9%.
• Subaortic stenosis: 5–33%.
• Aortic coarctation, hypoplasia, or interruption: 3–46%.
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
G. Tagarakis et al. (eds.), Clinical and Surgical Aspects of Congenital Heart Diseases,
https://doi.org/10.1007/978-3-031-23062-2_21
165
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M. S. Ahmad
• Mitral valve anomalies: 32%.
• And other non-cardiac associations: aneuploidy (trisomy
13,18) and tracheoesophageal stula.
Pathophysiology
A VSD is usually often linked with DORV.The location of
the VSD has a signicant impact on the physiologic symptoms of DORV as well as surgical concerns. Four major categories can be identied:
1. Double outlet right ventricle with subaortic VSD.
2. Double outlet right ventricle with subpulmonary VSD.
3. Double outlet right ventricle with doubly committed
VSD.
4. Double outlet right ventricle with noncommitted VSD.
According to the position of the great vessels:
1. Side by side great vessels position.
2. Right-sided position of great vessels.
3. Left-sided position of great vessels.
Noncommitted VSD Type
This type is located far away from the aortic and pulmonary
valves. The majority of patients with noncommitted VSD are
treated with single ventricular palliative methods.
Clinical Features
The clinical appearance and therapy of DORV are largely
determined by its type and the existence of concomitant cardiac abnormalities.
A comprehensive history of the patient’s illness and its
evolution and detailed physical examination to assess the
precordium should be used to identify clinically relevant
heart abnormal. Furthermore, pulmonary auscultation, as
well as peripheral cyanosis and capillary lling, should be
examined also.
The pulmonary stenosis presence greatly inuences the
patient’s symptom prole and age at the time of clinical
manifestation. Most of the patients were present earlier, in
the neonatal period. Cyanosis is evident in patients with signicant pulmonary stenosis, while congestive heart failure is
seen in patients with the massive pulmonary ow [3].
Subaortic VSD Type
It is the most frequent type of DORV. The degree of pulmonary stenosis determines the pathophysiology resulting in
cyanosis (TOF type). When there is no pulmonary stenosis,
the ow of blood increases, resulting in heart failure.
Subpulmonary VSD Type
The pulmonary artery gets oxygenated blood from the left
ventricle, whereas deoxygenated blood from the right ventricle ows to the aorta (TGA type). The Taussig-Bing anomaly is a classic case of a DORV with subpulmonary VSD.A
common connection is aortic arch hypoplasia. Associated
with reversed differential cyanosis.
Doubly Committed VSD Type
Both the aortic and pulmonary valves are connected to the
VSD because of the infundibular missing. The pulmonary
stenosis determines the clinical characteristics if present [2].
Diagnosis
Electrocardiographic (ECG) results are rarely diagnostic.
Right ventricular hypertrophy, right axis deviation, and, on
rare occasions, indications of left ventricular hypertrophy are
common ndings. Routine laboratory testing includes the
following: complete blood count (CBC), electrolyte levels,
renal prole, hepatic function, coagulation prole, and
assessment of nutritional status [2, 3].
Echocardiography
In neonates, echocardiography typically gives enough information for a precise and appropriate diagnosis, as well as the
information needed to design the surgical strategy. Sanders
and colleagues found that in 109 of 113 babies, conventional
transthoracic echocardiogram (TTE) was utilized to diagnose conotruncal malformation. The diagnosis of DORV was
conrmed with angiography in 11 of the 12 babies who had
previously received a diagnosis based on subxiphoid twodimensional echocardiography [4].
Double Outlet Right Ventricle
167
Cardiac Angiography
Heart catheterization, which was previously the gold standard, is now seldom used in the evaluation or preoperative
planning of this cardiac disease.
When warranted, angiography offers numerous advantages, including the following: Hemodynamic parameters
can be evaluated directly in an older child with a longstanding illness. The real anatomic variance can be described
in the context of probable aberrant coronary architecture.
When a Rastelli-type repair is considered, this knowledge
can inuence surgical strategy. Angiography can assist in
identifying the major pulmonary branches, the pulmonary
vascular tree, and the collateral arteries to the lungs in the
setting of pulmonary vascular abnormalities.
Magnetic Resonance Imaging (MRI)
MRI has been used before but, it is not currently a standard
or well-established diagnostic technique for this disease.
MRI can provide additional anatomic information, such as
the connection of the two ventricles.
Chest Radiography
Anteroposterior and lateral chest radiography results are
dependent on the degree of pulmonary (or subpulmonary) stenosis. The pulmonary parenchyma is largely oligemic in the
situation of severe stenosis, but in the setting of mild pulmonary stenosis (particularly with a Taussig-Bing heart), results
are likely to be consistent with congestive heart failure. In
either case, the chest picture demonstrates cardiomegaly.
Computed Tomography (CT) Scanning
Preoperative CT scanning may be benecial in determining coronary artery architecture in infants with TOF type. A study that
looked at the incidence and diagnostic accuracy of preoperative
cardiac CT scanning for identifying detailed coronary artery
anatomy in 318 children with TOF type discovered a 95% concordance between cardiac CT scanning and surgical ndings, as
well as a 96.9% diagnostic accuracy for cardiac CT scanning [5].
Management
Medical therapy of DORV is based on the abnormalities and
underlying physiology. Maintaining patent ductus arteriosus
is critical in the situation of insufcient pulmonary blood
ow. Prostaglandin E (alprostadil) infusions are the standard
of treatment until healing may occur. Having congestive
heart failure is contradictory; cautious diuresis, inotropic
support digoxin usage, and pulmonary blood ow management through intubation and blood gas manipulation may be
needed [5].
Indications forSurgery
Because (DORV) is a disease that does not resolve on its
own, the diagnosis alone is sufcient to warrant surgery.
Sakakibara et al. reported the rst successful biventricular
repair for this condition in 1967.
The clinical appearance and surgical technique required
for correction are determined by the relative anatomic abnormalities discovered. The accompanying VSD is generally big
and nonrestrictive.
Palliative procedures are often done solely in patients
who need short-term therapy, whereas noncardiac illness
(e.g., sepsis) is addressed when anatomic characteristics do
not allow for nal repair [6].
In an ideal world, DORV is a procedure that repairs both
ventricles, with the left ventricle linked to the aorta and the
right ventricle attached to the major pulmonary artery.
Palliative procedures differ depending on the subtype’s physiology. With excessive pulmonary ow, banding can be done
until denitive treatment. In case of insufcient blood ow,
commonly a Blalock-Taussig shunt (an aortopulmonary
shunt) can relieve symptoms until denitive treatment can be
done.
Bidirectional Glenn operation is used for univentricular
hearts or complicated congenital heart illness one of the
DORV. In a retrospective cohort (4years) study including
115 patients who underwent this surgery, the doctors determined that this procedure can enhance gas exchange and volume overload. Poor nutrition and late presentation increase
the morbidity post-operation.
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M. S. Ahmad
Contraindications fortheSurgery
Signicant left ventricular hypoplasia and severe atrioventricular valve overriding or straddling are absolute contraindications to double outlet right ventricle biventricular
surgery. Single ventricle palliation would be suggested in
individuals who are not candidates for biventricular surgery
[7].
Preoperative Assessment
Preoperative investigations should be used to correctly assess
surgically important characteristics such as pulmonary and
tricuspid valve separation and their diameter, the presence of
aortic coarctation, and anatomy of heart coronaries.
Signicance of pulmonary valve stenosis, VSD location, pulmonary stenosis, and size of major vessels are veryimportant
to be assessed prior to surgery.
Intraoperative Assessment
Repair ofDORV withSubaortic VSD Subtype
It is repaired usually by making an intraventricular tunnel
that shunts blood from the left ventricle through VSD to the
aorta. A patch (like polytetrauoroethylene) can be used.
Post cardiopulmonary bypass by cannulation and cardiac
arrest, the anatomy is examined by a right atriotomy. Through
the tricuspid valve, the VSD can be visualized and connection to the aorta is done. The VSD can be enlarged if needed.
This can be done superiorly and anteriorly, leading to the
excision of some pieces of the infundibular septum [8].
The VSD closure by interrupted or a continuous suture. If
the intraventricular tunnel bulges into the right ventricular
outow tract, the right ventriculotomy should be sealed by a
patch. Surgery of subaortic VSD and pulmonary stenosis
subtypes of RVO is done by the same procedure used in
TOF.Before pulmonary bypass, it is important to locate the
coronaries and plan for right ventriculotomy if indicated.
Doubly Committed VSD-Type Repair
This is a rare type; the repair is done in the same manner as
the subaortic VSD type method. The VSD mostly does not
block the left ventricular ow through an intraventricular
tunnel to the aorta. There may be a need for a conduit from
the right ventricle to the pulmonary artery if there is a right
ventricular obstruction or pulmonary stenosis.
Noncommitted VSD Repairs
The most difficult to repair and carry a high risk for the
patient. This abnormality is distinguished by a persistent subaortic conus and a double infundibulum. The
subaortic conus is larger than the typical right ventricular structure leading to aortic displacement normal position of the pulmonary artery and both of them are placed
nearby.
Pulmonary artery banding is required for severe subaortic blockage, restrictive VSD, or aortic arch obstruction.
Types with pulmonary stenosis should be managed conservatively or by modied Blalock-Taussig shunt which is a
systemic to pulmonary shunt. The hypoplastic left ventricle
and severe overriding of the atrioventricular valve are the
main limitations to doing a biventricular repair. The surgery
of choice when using combined atrial and ventricular methods is an intraventricular tunnel that links the VSD to the
aorta [10, 11].
Outcome andPrognosis
Brown etal. reported a 56% of 15-year survival rate in individuals with DORV from 1980 to 2000 (including those who
undergo the surgery and the patients that don’t do it). The
majority of non-complicated cases (95%) had a 15-year survival rate after surgery, whereas the Taussig-Bing abnormality had an 89% survival rate.
individuals with a double outlet right ventricle and a noncommitted VSD were at a greater risk of reoperation and
mortality [12].
Subpulmonary VSD Type Repair
This type can be associated with aortic coarctation, so coarctation repair with a pulmonary artery band is the preferred
method; however, repairing both defects can be done in one
stage operation. If the VSD is restricted, it should be expanded
to allow the blood ow from the left ventricle to the pulmonary artery. Other defects can be done concurrently [9].
Multiple Choice Questions
1. The most useful diagnostic method for dual outlet right
ventricle is:
A. ECG.
B. X-ray.
C. CT scan.
D. MRI.
E. Echocardiography.
Double Outlet Right Ventricle
169
2. Patent ductus arteriosus should be kept open in some
cases with pulmonary stenosis by administration of:
A. Indomethacin.
B. Alprostadil.
C. Mifepristone.
D. Aspirin.
E. Baclofen.
3. Contraindication for biventricular repair:
A. Left ventricular hypoplasia.
B. Coarctation of aorta.
C. Having ventricular septal defect.
D. Aneuploidy.
4. In noncommitted type of double outlet right ventricle, the
VSD location is:
A. Subaortic VSD.
B. Subpulmonary VSD.
C. VSD that located far away from major vessels.
D. VSD that is connected to both major vessels.
5. The most common type of DORV you can see in patients
is:
A. Subaortic VSD.
B. Subpulmonary VSD.
C. Committed VSD.
D. Noncommitted VSD.
6. Taussig-Bing anomaly is a type of:
A. Subaortic VSD.
B. Subpulmonary VSD.
C. Committed VSD.
D. Noncommitted VSD.
7. Reversed differential cyanosis seen in subpulmonary
VSD type in:
A. Toes of the feet more than hand ngers.
B. Fingers of the hand mostly.
C. Equally in toes and ngers.
D. There is no differential cyanosis in the subpulmonary
type.
Answers
1. E.
Explanation: Echocardiography typically gives
enough information for a precise and appropriate diagnosis, as well as the information needed to design the surgical strategy.
2. B.
Explanation: Alprostadil is a prostaglandin agonist
that is used to keep ductus arteriosus open.
Mifepristone is antiprogesterone and the other drugs
are non-steroidal anti-inammatory drugs that close the
duct.
3. A.
Explanation: Signicant left ventricular hypoplasia
and severe atrioventricular valve overriding or straddling
are absolute contraindications to double outlet right ventricle biventricular surgery. Single ventricle palliation
would be suggested in individuals who are not candidates
for biventricular surgery.
4. C.
Explanation: Located far away from the aortic and
pulmonary valves
5. A.
Explanation: Subaortic VSD.
6. B.
Explanation: Subpulmonary VSD.
7. B.
Explanation: Differential cyanosis is mostly seen in
hand ngers associated with clubbing usually.
References
1. Goo HW.Double outlet right ventricle: in-depth anatomic review
using three-dimensional cardiac CT data. Korean J Radiol.
2021;22(11):1894.
2. Yoo S-J, van Arsdell GS. 3D printing in surgical management of
double outlet right ventricle. Front Pediatr. 2018;5:289.
3. Kariya T, et al. Personalized perioperative multi-scale, multiphysics heart simulation of double outlet right ventricle. Ann
Biomed Eng. 2020;48(6):1740–50.
4. Yim D, etal. Essential modiers of double outlet right ventricle:
revisit with endocardial surface images and 3-dimensional print
models. Circ Cardiovasc Imaging. 2018;11(3):e006891.
5. Goo HW. Coronary artery anomalies on preoperative cardiac CT
in children with tetralogy of Fallot or Fallot type of double outlet
right ventricle: comparison with surgical ndings. Int J Cardiovasc
Imaging. 2018;34(12):1997–2009.
6. Wang Z, et al. A new ISL1 loss-of-function mutation predisposes to congenital double outlet right ventricle. Int Heart J.
2019;60(5):1113–22.
7. Pang K-J, et al. Echocardiographic classication and surgical
approaches to double-outlet right ventricle for great arteries arising almost exclusively from the right ventricle. Tex Heart Inst J.
2017;44(4):245–51.
8. Lu C-X, etal. A novel MEF2C loss-of-function mutation associated with congenital double outlet right ventricle. Pediatr Cardiol.
2018;39(4):794–804.
9. Ebadi A, et al. Double-outlet right ventricle revisited. J Thorac
Cardiovasc Surg. 2017;154(2):598–604.
10. Bhatla P, et al. Utility and scope of rapid prototyping in patients
with complex muscular ventricular septal defects or double-outlet
right ventricle: does it alter management decisions? Pediatr Cardiol.
2017;38(1):103–14.
11. Lo CW, etal. Reply to ‘double-outlet right ventricle is not hypoplastic left heart syndrome’. Nat Genet. 2019;51(2):198–9.
12. Kumar P, Bhatia M.Role of computed tomography in pre-and postoperative evaluation of a double-outlet right ventricle. J Cardiovasc
Imaging. 2021;29(3):205.
Ebstein’s Anomaly
Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/
AhmedDheyaaAl-Obaidi, SaraShihabAhmad,
AbeerMundherAli, andRawaaFadhilAl-Tofakchi
Abstract
Ebstein’s anomaly, which is described by Wilhelm
Ebstein, is one of the congenital heart diseases that are
seldom encountered with a 1 per 200,000 live birth prevalence. It entitles the presence of deformity in the right side
of the heart and the tricuspid valve and more specically
it is a downward displacement of septal and posterior leaflet into the right ventricle leading to enlarged atrium and
atrialized ventricle. Many heart abnormalities can be
associated with EA like ASD, conduction system abnormalities, PFO, pulmonary stenosis or atresia, and
VSD. This anomaly has a wide range of manifestation
depending on the severity of the lesion and it can be diagnosed by echocardiogram which is considered to be the
diagnostic test for this anomaly. Management could be by
observation in asymptomatic patient, medical therapy in
mild cases while surgical intervention may be required in
severe cases as well as valve replacement and heart
transplantation.
Keywords
Ebstein’s anomaly · Lithium therapy · MYH gene
Tricuspid valve · Atrialized right ventricle · Elevated JVP
Cyanosis · Starnes procedure · Valve replacement
Milrinone
Introduction
Ebstein’s abnormality (EA) is one of the congenital heart
diseases that are seldom encountered. It affects 1in every
two hundred thousand living-births and accounts for approximately 1% of all congenital heart disease occurrences.
A. D. Al-Obaidi · S. S. Ahmad · A. M. Ali · R. F. Al-Tofakchi (*)
College of Medicine, University of Baghdad, Baghdad, Iraq
e-mail: rawaafadhil24@gemail.com
Wilhelm Ebstein rst identied this condition in 1866in his
report “Concerning a very rare case of insufciency of the
tricuspid valve caused by a congenital malformation.” EA is
a type of CHD marked by Rt-side of the heart and tricuspid
valve (TR) abnormality. The inability of the leaets of the
TR to delaminate from the underlying cardiac-endocardium
causes distinct characteristics [1]. There are different levels
of downward displacement of the tricuspid leaets into the
Rt ventricle with the septal leaet being the most signicantly affected, followed by the posterior one; as a result, the
annular circumference is often quite large, and the Rt atrium
is quite enlarged; there is myopathic changes in the RV and
it is divided into two distinct areas, an “atrialized” area of
poor function, situated between the hinge point of the septal
leaet that is displaced apically and the true annulus, and the
functional area which is located below the hinge point. This
functional RV volume might be fairly small, its volume
mainly depends on how much the leaet is displaced. ASD,
persistent patency of foramen ovale, abnormalities in the
conductive system of the heart, VSD and stenosis or atresia
of the pulmonary valve are among the cardiac abnormalities
linked to EA [2].EA manifests clinically as a spectrum ranging from minor types that become apparent in adulthood to
severe forms that result in signicant newborn mortality. EA
can cause hydrops fetalis in the uterus, and cyanosis and dyspnea in children and adults, but the disease can manifest in a
variety of ways and can become clinically severe at any
moment during life. The diagnostic technique of choice for
denitively diagnosing Ebstein’s abnormality is echocardiography, which can also be used to characterize the degree
of valvular insufciency and the quality of the leaets [3].
Other modalities, including an electrocardiogram (ECG),
radiography of the chest, or prenatal ultrasonography, are
frequently used to detect heart abnormalities and it is the initial tests that lead to additional investigation. When possible,
valve repair is obviously preferred and it is more likely to
succeed if the anterior leaet is broad, sail-like, and relatively thin while thicker, muscularized anterior leaet is an
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
G. Tagarakis et al. (eds.), Clinical and Surgical Aspects of Congenital Heart Diseases,
https://doi.org/10.1007/978-3-031-23062-2_22
171
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