PRENATAL DIAGNOSIS

Prenat Diagn 2005; 25: 796–806.

Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/pd.1269

REVIEW

Acardiac anomaly: current issues in prenatal assessment and

treatment
Amy E. Wong and Waldo Sepulveda*
Fetal Medicine Center, Department of Obstetrics and Gynecology, Clinica Las Condes, Santiago, Chile

Acardiac anomaly is a rare condition affecting monochorionic multiple pregnancies. We review this condition
with emphasis on its prenatal diagnostic features and treatment options. Due to the parasitic hemodynamic
dependence of the acardiac twin on the pump twin, it is important to monitor the pump twin for signs of
decompensation and, if indicated, intervene by interrupting vascular supply to the acardiac twin. The goal of
treatment is to maximize the pump-twin’s chance of survival. To assist with the decision of when to treat,
we suggest a new classification system based on prognostic factors, specifically the size and growth of the
acardiac twin and the cardiovascular condition of the pump twin. When the acardiac twin is small and no
signs of cardiovascular impairment in the pump twin are present, we suggest serial ultrasound surveillance
to detect any worsening of the condition. In cases with a large acardiac twin or rapid growth of the acardiac
mass, we recommend prompt intervention. Once treatment is indicated, the intrafetal approach to interrupt the
vascular supply to the acardiac twin appears to be superior to cord occlusion techniques as it is simpler, safer
and more effective. The first line of treatment, if available, should be ultrasound-guided laser coagulation or
radiofrequency ablation of the intrafetal vessels. Copyright  2005 John Wiley & Sons, Ltd.
KEY WORDS: acardiac anomaly; acardiac twin; TRAP sequence; monochorionic twins; twin pregnancy; prenatal
diagnosis; fetal therapy

INTRODUCTION (Seckin et al., 2003) or significant shrinkage of the

Acardiac anomaly, one of the most severe human mal-
formations, is a rare complication unique to monochori-
onic multiple pregnancies. In this condition, the primary
malformation is the lack of a well-defined cardiac struc-
ture in one twin (the acardiac twin), which is kept
alive by its structurally normal co-twin (the pump twin)
through a superficial artery-to-artery placental anastomo-
sis. Arterial blood flows in a retrograde fashion from the
pump twin toward, rather than away from, the affected
twin, which is why acardiac anomaly is also referred to
as the twin reversed arterial perfusion (TRAP) sequence
(Van Allen et al., 1983). The acardiac twin thereby acts
as a parasite that is hemodynamically dependent upon
the pump twin and, therefore, its continuous growth
threatens the survival of the pump twin by stealing blood
as well as acting as a space-occupying mass. Based on
two large series reporting on the natural history of the
condition, perinatal mortality rates of the co-twin range
from 35 to 55% (Moore et al., 1990; Healey, 1994).
Of note, no study has addressed the issue of short- and
long-term morbidity of the surviving pump twins.
Acardiac anomaly occurs in approximately 1 in 35 000
pregnancies and in 1% of monochorionic twins (James,
1977; Napolitani and Schreiber, 1960). It has also been
described in triplet, quadruplet and quintuplet preg-
nancies (Napolitani and Schreiber, 1960; James, 1977;
Benirschke and Kaufmann, 1995). Due to misdiagnosis
*Correspondence to: Professor Waldo Sepulveda, Fetal Medicine
Center, Clinica Las Condes, Casilla 208, Santiago 20, Chile.
E-mail: fetalmed@yahoo.com
acardiac twin by the time of delivery, it is likely that
many cases go unnoticed and the incidence of this condi-
tion may be, in fact, higher. According to one patholog-
ical series of 30 cases, one-third have a monochorionic-
monoamniotic placenta and two-thirds are associated
with a monochorionic-diamniotic placenta (Benirschke
and Kaufmann, 1995).
In the past two decades, concurrent with technological
advancements in diagnostic and therapeutic capabilities
in the field of obstetrics, numerous reports have been
published to describe acardiac anomaly in different
clinical settings, possible prognostic factors that may
influence its management, and techniques and outcomes
of various modes of treatment. The aim of this article
is to review the current knowledge of the condition,
focusing on its prenatal assessment, management options
and available invasive treatments.
PATHOLOGY
As a rule, the development of all organ systems is
affected in acardiac anomaly. Consequently, the struc-
ture of the acardiac fetus is extremely variable. Some
acardiac twins possess well-differentiated organ struc-
tures, while others lack any recognizable anatomy
(Figure 1). Size can vary from small teratoma-like
masses to fetuses more than double the size of its co-twin
(Sato et al., 1983). The heart may be completely absent
(holoacardia) or be in a primitive state of development
(pseudoacardia). Most commonly, acardiac twins are
acephalic with absent upper extremities. A central trunk
is usually present with a well-developed or rudimentary

Copyright  2005 John Wiley & Sons, Ltd.

 ACARDIAC ANOMALY
Figure 1—Acardiac twin (acardius acephalus)
spine. Structures that are frequently absent include the
heart (less than 20% of fetuses have identifiable car-
diac tissue), head, upper limbs, pancreas, lungs, liver
and small intestines. A two-vessel cord is found in more
than two-thirds of cases (Healey, 1994). Due to the lack
of communication between the lymphatic and vascular
systems, the acardiac twin frequently develops severe
subcutaneous edema and cystic hygromas, which can
significantly increase the size of the fetus and distort the
already abnormal anatomy.
The placenta in pregnancies complicated by acardiac
anomaly has not been studied in great detail. However,
all placentas have a similar pattern consisting of the
presence of two anastomoses, one artery-to-artery and
one vein-to-vein, connecting the circulation between
the acardiac and the pump twins. In most cases, these
anastomoses are on the placental surface and allow flow
between the acardiac twin’s umbilical cord and the major
arterial and venous branches of the cord of the pump
twin. Occasionally, the cord of the acardiac twin inserts
into the cord of the pump twin, in which case the
umbilical arteries and veins of the twins are directly
anastomosed and covered by Wharton’s jelly (Figure 2).
As a result of this vascular connection, acardiac twins
always lack functional placental tissue.
PATHOPHYSIOLOGY
The etiology and pathophysiology underlying acardiac
anomaly are not completely understood. The two main
Copyright  2005 John Wiley & Sons, Ltd.
797
Figure 2—Direct connection between the umbilical cord of the
acardiac twin and the umbilical cord of the pump twin
theories attempting to explain the origin of acardiac
anomaly are:
1. Abnormal placental vasculature leading to circulatory
reversal with subsequent alteration in cardiac devel-
opment;
2. Abnormal cardiac embryogenesis occurring as a
primary event.
A major contribution to the knowledge of this com-
plex anomaly was made by Van Allen et al. (1983), who
first developed the TRAP sequence hypothesis. Accord-
ing to this theory, which is now accepted as an accurate
description of the pathophysiology, arterial blood flows
in a retrograde nature in the acardiac twin’s umbilical
artery via a single artery-to-artery anastomosis. Con-
sequently, poorly oxygenated and nutrient-poor blood
bypasses the placenta, enters the circulation of the acar-
diac twin and passively follows a course through the iliac
arteries at subnormal pulse pressures to preferentially
perfuse the caudal rather than the cephalad structures.
The blood flow then returns to the pump-twin’s circula-
tion via a single vein-to-vein anastomosis. This circula-
tory event explains why the lower limbs of an acardiac
twin are better formed than its more cephalad struc-
tures, as the latter receives more severely deoxygenated
blood, resulting in abnormal development and atrophy
of the heart and dependent organs. However, the TRAP
sequence only describes the final stage of the process
that results in continuous perfusion and growth of an
acardiac fetus, failing to explain the mechanism leading
to the persistence of the single artery-to-artery anasto-
mosis and lack of placentation. In addition, this theory
neither explains the development of a pseudoacardiac
twin nor why some acardiac twins have a three-vessel
cord.
Alternative etiologic theories have postulated that the
primary event is a disturbance in cardiac embryogenesis
that secondarily causes retrograde flow in the affected
twin (Severn and Holyoke, 1973), for instance, due to
chromosome abnormality (Benirschke and Kaufmann,
1995) or environmental factors (Wilson, 1972). Cytoge-
netic analyses have failed to demonstrate any consistent
Prenat Diagn 2005; 25: 796–806.
798 A. E. WONG AND W. SEPULVEDA
chromosomal abnormality (Blaicher et al., 2000). More-
over, molecular studies have demonstrated that acardiac
anomaly only occurs in monozygotic identical twins,
thus ruling out the possibility of discordant aneuploidy
or fertilization of the polar body as the etiology (Fisk
et al., 1996). According to the hypotheses that suggest
that aberrant cardiac development is the primary event,
the acardiac twin only ‘survives’ due to the anasto-
moses that form secondarily after fetal demise. However,
demonstration of fetal heartbeats in early gestation, with
subsequent reversal of umbilical blood flow and disap-
pearance of cardiac activity, suggests that the primary
defect is the anastomosis allowing retrograde flow, not
failure of cardiac development (Coulam and Wright,
2000).
Of note, the artery-to-artery anastomosis that is inte-
gral to the TRAP sequence may not be present in all
acardiac twins. Shih et al. (1999) reported two cases
of artery-to-vein anastomoses between the acardiac and
the pump twin, confirmed upon pathological examina-
tion. In their study of five acardiac cases, they described
three different Doppler waveforms: the first, occurring
in the absence of a primitive heart regardless of the type
of vascular connection (pump-in pattern); the second,
occurring in the presence of an artery-to-artery anasto-
mosis when the acardiac twin possesses a primitive heart
(collision-summation pattern); and the third, occurring in
the presence of an artery-to-vein anastomosis when the
acardiac twin possesses a primitive heart (twin-pulse pat-
tern). In the cases with artery-to-vein anastomoses, blood
flow was dorsocranial and circulated upward to the trunk
rather than downward to the lower limbs, perhaps fol-
lowing the course of the primitive cardinal vein, yet the
upper limbs remained more deformed than the lower
limbs. This discordance between theory and observation
suggests that retrograde flow of poorly oxygenated blood
may not be the only cause of acardiac anomaly.
Regardless of its etiology, it is irrefutable that the
parasitic acardiac twin threatens the wellbeing of its co-
twin by a vascular-stealing phenomenon. There are at
least three mechanisms by which it can increase perina-
tal mortality of the pump twin. First, continued growth
of the acardiac twin, which occasionally becomes larger
than the pump twin, can significantly increase intrauter-
ine volume and lead to perinatal complications, par-
ticularly preterm delivery. Second, the systemic shunt
produced by the acardiac twin can increase the hemo-
dynamic demands of the pump twin, thereby leading
to congestive heart failure and polyhydramnios. Third,
deoxygenated blood from the pump twin is further
deoxygenated when it perfuses the acardiac mass. This
‘double-used’ blood is then circulated back to the pump
twin through a vein-to-vein anastomosis, thus reducing
the oxygen level in the blood flowing to the pump twin
and causing chronic hypoxia and growth restriction, as
well as demanding a higher cardiac output and increas-
ing likelihood of cardiac failure.
The primary contributors leading to perinatal death
of the pump twin are congestive heart failure and
preterm delivery due to polyhydramnios or mass effect
of the acardiac twin. Whereas twin pregnancies deliver
at a mean gestational age of 36.6 weeks (Loos et al.,
Copyright  2005 John Wiley & Sons, Ltd.
2001), the mean gestational age at delivery of acardiac
twin cases is 31.1 weeks (Healey, 1994). In addition,
intrauterine growth restriction and hydrops may further
complicate the well-being of the pump twin (Moore
et al., 1990; Healey, 1994). In cases of monoamniotic
placentation, the cord of the pump twin can become
entangled with the acardiac twin’s cord (Chang et al.,
1996). The risk of these complications may be higher in
the third trimester when the rate of fetal growth is the
greatest.
CLASSIFICATION
The simplest classification system of acardiac twins dif-
ferentiates between two types: pseudoacardius, where
the twin has evidence of a rudimentary cardiac structure,
and holoacardius, where no such structure is present. A
more detailed classification, also based on the morphol-
ogy of the acardiac twin, separates cases into four groups
as follows (Napolitani and Schreiber, 1960):
1. Acardius acephalus, where the fetus has a well-
developed pelvis and lower limbs, but no head,
usually no thoracic organs and often no arms. This is
the most common form.
2. Acardius anceps, where the fetus has a well-
developed body and extremities, but only a partially
formed head and face. As the most developed form,
it is likely that many of the other types begin as
acardius anceps and evolve into one of the less well-
differentiated forms due to poor oxygen and nutrient
supply.
3. Acardius acormus, where only the head of the fetus
is developed. This form is very rare.
4. Acardius amorphus, where the fetus is a shapeless
mass of tissue containing no recognizable organs, but
has some form of axial structure. This is the least
differentiated form.
Although this system has been widely used in the past,
it has no correlation with pregnancy outcome and offers
no implications for management. This classification sys-
tem was developed at a time when no prenatal diagnosis
was available and is, therefore, based on postpartum
findings. With modern ultrasound equipment, it is now
possible to classify acardiac twins prenatally according
to findings at diagnosis or at follow-up. We therefore
suggest that a more practical classification system based
on the size of the acardiac twin and its impact on the
condition of the pump twin is needed (see below).
PRENATAL DIAGNOSTIC FEATURES
Because the perinatal mortality rate of the pump twin
may be as high as 55% (Moore et al., 1990), it is impor-
tant to diagnose acardiac anomaly as early as possible
so preventive measures can be taken to improve the
chance of survival. Lehr and DiRe (1978) were the first
to describe the in utero diagnosis of an acardiac twin
Prenat Diagn 2005; 25: 796–806.
 ACARDIAC ANOMALY 799

using ultrasound. A decade later, Pretorius et al. (1988)

described the application of color Doppler ultrasound in
the diagnosis and first documented the reversed arte-
rial perfusion pattern in vivo. The implementation of
transvaginal Doppler ultrasound in the 1990s allowed
earlier diagnosis of acardiac anomaly (Langlotz et al.,
1991; Shalev et al., 1992; Zucchini et al., 1993). Most
recently, three-dimensional ultrasound has been used to
confirm the diagnosis and to establish the extent of fetal
malformations more precisely (Bonilla-Musoles et al.,
2001).
The prenatal diagnosis of acardiac twins should be
suspected when a grossly malformed fetus is seen in the
setting of a monochorionic twin pregnancy (Sepulveda
(a)
et al., 1993; Sebire et al., 2003). Features visualized on
ultrasound that point to the diagnosis include biometric
discordance between the twins, the absence of identi-
fiable cardiac pulsation in one twin, poor definition of
the head, trunk and upper extremities, deformed lower
extremities, marked and diffuse subcutaneous edema and
abnormal cystic areas in the upper part of the body
of the affected twin (Figure 3). The presence of car-
diac motion does not exclude the diagnosis, as it may
result from a rudimentary heart or transmitted pulsa-
tion from the cardiac function of the pump twin. The
demonstration of the circulation of blood in a paradox-
ical direction with arterial blood flowing toward, rather
than away, from the acardiac twin, and in a caudal-to-
cranial direction in the abdominal aorta, establishes the
diagnosis. This retrograde flow has been documented by (b)
color Doppler ultrasound (Pretorius et al., 1988; Ben-
son et al., 1989; Sherer et al., 1989; Crade et al., 1992; Figure 4—(a,b) Color Doppler ultrasound in acardiac anomaly shows
blood flow signals within the mass
Fouron et al., 1994), as well as postmortem placen-
tal and fetal angiography and umbilical cord blood-gas
analyses (Donnenfeld et al., 1991). However, the sim- anomaly. The diagnosis of a teratoma from the placental
ple documentation of blood flow using color or power surface or the umbilical cord must also be ruled out
Doppler ultrasound within a mass lacking cardiac pulsa- (Benirschke and Kaufmann, 1995). Two ultrasound
tion is pathognomonic of acardiac anomaly (Figure 4). criteria can be used to differentiate between a teratoma
The main differential diagnosis of acardiac anomaly and an acardiac twin prenatally. First, an acardiac twin
is the single intrauterine death of one grossly abnormal always has a separate, though rudimentary, umbilical
monochorionic twin. However, the continued growth of cord; and second, the twin shows some evidence of
the ‘presumed dead’ twin on subsequent scans should body organization, whereas the tissues in teratomas are
point the examiner toward the diagnosis of acardiac completely disorganized. Postpartum, the fact that an
acardiac twin is always covered by normal skin can be
used as an additional criterion for diagnosis.

PRENATAL PROGNOSTIC FEATURES

Attempts to identify factors that predict pump fetus

outcome originally focused on the structure of the
acardiac twin. Healey (1994) reviewed 184 cases and
described factors associated with a high risk of perinatal
mortality, including acardius anceps and the presence
of ears, larynx, trachea, pancreas, renal tissue and small
intestine. While the nature of the relationship between
these factors and pump-twin outcome is not intuitive,
(a) (b)
the relative size of the acardiac twin compared to its
Figure 3—Conventional two-dimensional ultrasound findings in acar- co-twin plays a more obvious role. Accordingly, Moore
diac anomaly. (a) The structurally normal ‘pump’ twin and (b) the et al. (1990) developed the twin-weight ratio, the weight
acardiac twin of the acardiac twin expressed as a percentage of the

Copyright  2005 John Wiley & Sons, Ltd. Prenat Diagn 2005; 25: 796–806.
800 A. E. WONG AND W. SEPULVEDA
pump-twin weight, as a prognostic factor. They reported
that if the ratio is greater than 70%, the incidence
of preterm delivery was 90%, polyhydramnios 40%
and pump-twin congestive heart failure 30%, compared
with rates of 75, 30 and 10%, respectively, when the
ratio was less than 70%. Their data further showed
significantly better outcomes when the twin-weight ratio
was less than 50%, in which cases the preterm delivery
occurred in only 35% of cases, polyhydramnios in 18%
of cases and congestive heart failure in none of the
cases. However, their results were based on postpartum
measurements and therefore are only of limited value
prenatally at the time of prenatal diagnosis, as the size
of the acardiac mass in utero at the time of diagnosis
may differ significantly from the size at delivery. For
example, autocessation or reduction of blood flow to the
acardiac mass frequently occurs, leading to its shrinkage
and a much lower postpartum weight. Implementation of
the twin-weight ratio prenatally is also limited because
estimation of fetal weight of the acardiac twin due to its
amorphous structure may be highly inaccurate.
The capability of color Doppler ultrasound to mea-
sure and analyze the hemodynamics of both the acardiac
and the pump twins has allowed more recent attempts
to identify prognostic factors. An increased resistance
to perfusion was demonstrated by Sherer et al. (1989),
who described a case in which the umbilical arteries
of the acardiac and pump twins had markedly different
impedance indices. They reported that a small difference
in the systolic-to-diastolic ratio was associated with a
poor outcome for the pump twin. Similarly, Brassard
et al. (1999) analyzed the ratio of the umbilical artery
pulsatility index (PI) values between the acardiac and
pump twins in nine cases. Their results indicated that
a low PI, representing a high diastolic velocity, in the
acardiac twin compared with the PI of the pump twin
correlated with poor prognosis because it reflected sub-
stantial flow into the acardiac twin. Most recently, Dashe
et al. (2001) noted that pump-twin outcomes varied by
differences in resistance index (RI) between the twins.
The difference in RIs between twins may approximate
the flow of blood pumped to the acardiac twin, with
a small RI difference indicating a small difference in
flow. This suggests a large artery-to-artery anastomosis
Table 1—Proposed classification of acardiac anomaly
Acardiac: pump-twin Signs of pump-twin’s
Type AC ratio compromisea
Ia <50% Absent
Ib <50% Present
IIa ≥50% Absent
IIb ≥50% Present
AC, abdominal circumference.
a Defined as physical changes visualized on two-dimensional ultrasound (moderate-to-severe polyhydramnios, cardiomegaly or pericardial
effusion) or abnormal Doppler signals (tricuspid regurgitation, reverse flow in the ductus venosus, pulsation in the umbilical vein or high
middle cerebral artery peak velocity).
Copyright  2005 John Wiley & Sons, Ltd.
and a large volume of flow from the pump twin to the
acardiac twin, placing the pump twin at greater risk of
developing cardiac failure. Indeed, in their three cases
with poor outcomes, the differences in RI were <0.05,
whereas the three cases with favorable outcomes were
associated with calculated or hypothetical RI differences
of >0.20.
Although more studies need to be performed before
determining with certainty the prognostic utility of these
specific Doppler indices, we fully agree that the size
of the acardiac twin and the cardiovascular status of
the pump twin are the most important factors to predict
pump-twin outcome and guide management. In addition
to the size of the acardiac twin at diagnosis, we consider
the change in size of the acardiac twin based on serial
ultrasound scans as another important prognostic factor.
This is because a growing acardiac mass, particularly if
rapid, signifies a continuous increase in cardiac demand
of the pump twin. On the other hand, a decrease in size
likely represents a reduction of blood flow supply to the
acardiac twin that may result in spontaneous resolution
of the condition.
To assess the impact of the acardiac twin on the
cardiovascular status of the pump twin, two modali-
ties should be used. First, two-dimensional ultrasound
can be used to look for physical signs of early car-
diovascular deterioration of the pump twin, specifically
polyhydramnios, cardiomegaly and pericardial effusion.
Second, color Doppler ultrasound should be employed
to look for tricuspid regurgitation, reverse flow in the
ductus venosus, pulsation in the umbilical vein and high
peak velocity of middle cerebral artery flow secondary
to fetal anemia. The presence of any one of these ultra-
sound features predicts a poor prognosis.
We propose a new classification system that groups
acardiac anomalies according to acardiac twin size and
the cardiovascular impact on the pump twin on prenatal
ultrasound, thereby providing critical information of the
severity of the case and necessity of prenatal treatment
(Table 1). To most accurately estimate the size of the
acardiac twin prenatally, we suggest using abdominal
circumference (as acardiac masses almost always have
a central trunk), which correlates with growth of both the
length and girth of a fetus. In contrast, the only formula
Management
Reclassify within two weeks based on follow-up scan.
Consider treatment if no change in stage but increase in
absolute size or persistence of moderate or significant
vascularity of acardiac twin
Reclassify within two weeks based on follow-up scan.
Prompt treatment if increase in absolute size or persistence of
moderate or significant vascularity of acardiac twin
Prompt intervention
Emergency intervention
Prenat Diagn 2005; 25: 796–806.
 ACARDIAC ANOMALY
currently available is solely based on fetal length and
does not take the girth of the mass into account (Moore
et al., 1990). Another advantage of using the abdominal
circumference is that when comparing the sizes of the
acardiac and pump twins, a ratio based on the single
parameter of abdominal circumference can be calculated,
rather than a ratio that compares the length of one fetus
to multiple and different parameters of the other fetus.
According to our classification system, acardiac
anomalies are divided into two types. Small or medium-
sized acardiac twins (abdominal circumference ratio less
than 50%) are classified as Type I, and acardiac twins
that are large (abdominal circumference ratio greater
than or equal to 50%) are classified as Type II. Cases are
then further divided based on the absence or presence
of signs of pump-twin cardiac insufficiency. If signs are
absent, cases are classified into subtype ‘a’, and if signs
are present, into subtype ‘b’. Small or medium-sized
acardiac twins that do not affect the cardiovascular sta-
tus of the pump twin (Type Ia) do not pose an imminent
danger to the pump twin and should be managed con-
servatively with ultrasound supervision according to the
clinical situation. If a small or medium-sized acardiac
twin has mildly compromised the cardiovascular func-
tion of its co-twin (Type Ib), close surveillance with
frequent scans is appropriate to monitor whether the
impact will worsen, therefore warranting treatment, or
resolve. If the compromise becomes moderate or severe,
invasive treatment should be considered. An acardiac
twin that is large (abdominal circumference ratio greater
than or equal to 50%) may not impose large cardiac
demands from its co-twin (Type IIa) if its size may be
primarily due to subcutaneous edema or hydrops, but
it still poses a significant threat of preterm labor with
advancing gestation. In this situation, therefore, prompt
intervention is appropriate. If a large acardiac twin has
caused signs of pump-twin cardiac insufficiency (Type
IIb), urgent intervention is warranted.
During follow-up surveillance of Type I acardiac
twins, two factors require close attention to determine
if and when treatment is appropriate. First, the absolute
size of the acardiac twin should be followed. If the abso-
lute size of the mass increases (even if the pump-twin
weight ratio decreases or does not change), interven-
tion should be considered depending on the rate of the
growth of the mass. Second, vascularity of the acar-
diac mass should be assessed to predict whether the size
of mass will increase or decrease. Based on Doppler
measurements of umbilical artery impedance and other
indicators of amount of flow to the acardiac twin, vascu-
larity can be subjectively classified as minimal, moderate
or significant. Moderate or significant vascularity imply
continuing growth of the acardiac mass and therefore
intervention is appropriate. The value of this proposed
classification has yet to be validated in a prospective
series.
TREATMENT
The goal of treating acardiac anomaly is to maximize
the likelihood of term delivery and survival of the pump
Copyright  2005 John Wiley & Sons, Ltd.
801
twin in a safe and effective manner. Some authors advo-
cate early prophylactic treatment of all acardiac twins at
about 16 weeks if reversed blood flow is demonstrated
(Jolly et al., 2001). Treatment at this early stage would
also avoid the difficulty in stopping blood flow that is
experienced in cases with larger and sometimes hydropic
acardiac twins. On the other hand, Sullivan et al. (2003)
advocate that the best initial treatment is expectant man-
agement, based on their reported survival rate of 90%.
As cessation of blood flow to the acardiac fetus may
occur spontaneously, or the size of the acardiac twin may
remain small and not pose any complication, they argue
for a conservative approach to avoid morbidity associ-
ated with aggressive intervention. However, 4 of the 10
fetuses reported in their series were associated with a
very small acardiac twin with a postpartum acardiac-to-
pump twin-weight ratio less than 3% (Type I according
to our classification). Also of note, the three cases that
did experience complications, such as intrauterine fetal
demise or admission of the infant to the neonatal inten-
sive care unit, were associated with large acardiac twins
(Type II according to our classification). Although the
authors did not provide estimates of the weights of the
acardiac twins in utero based on ultrasound, it is likely
that the vast majority of acardiac masses in their series
were small even at the time of diagnosis. Conversely,
in another series of five cases, managed expectantly and
with birth weight twin-ratios ranging from 81 to 171%
(Type II according to our classification), only one pump
twin survived (Sogaard et al., 1999). Therefore, we sug-
gest that expectant management is only appropriate if no
poor prognostic factors such as a large acardiac twin are
present. Treatment is indicated if serial ultrasound exam-
inations reveal the presence of adverse factors, since
prenatal treatment is important not only to prevent peri-
natal death of the pump twin but also to avoid significant
preterm delivery.
Numerous prenatal interventions to ablate blood flow
to the acardiac twin have been described (Arias et al.,
1998; Tan and Sepulveda, 2003), but due to the rarity of
the condition and the heterogeneity of its presentation,
no single technique has been shown to be unequivocally
optimal (Sepulveda and Sebire, 2004). Initial efforts to
treat acardiac anomaly focused on the alleviation of
symptoms. Inotropic agents such as digoxin were used to
treat congestive heart failure in the pump twin (Simp-
son et al., 1983). Indomethacin has also been used to
alleviate polyhydramnios as well as to prevent prema-
ture labor (Ash et al., 1990). However, these medical
treatments should be discouraged not only because they
are not efficacious, but also because their use delays the
need for definitive treatment. Similarly, amniodrainage
can be performed to alleviate polyhydramnios to relieve
maternal discomfort and prolong pregnancy (Platt et al.,
1983), but as it does not correct the underlying vascu-
lar defect, should only be used as an adjunct to a more
aggressive procedure aimed to definitively treat the con-
dition.
At the other extreme of the interventional spectrum,
hysterectomy with selective delivery of the acardiac twin
(sectio parva) has been performed. Robie et al. (1989)
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802 A. E. WONG AND W. SEPULVEDA
performed the first reported successful invasive inter-
vention by delivering a 710-g acardiac twin at 22 weeks
by sectio parva with delivery of a 2130-g healthy pump
twin at 33 weeks. Due to this favorable outcome, this
extremely aggressive intervention was subsequently car-
ried out by other groups, with seven cases reported
between 1989 and 1992 (Robie et al., 1989; Fries et al.,
1992; Ginsberg et al., 1992). Six of the seven (86%)
pump twins survived, although two (29%) experienced
preterm premature rupture of membranes and three
(43%) pump twins were delivered before 30 weeks.
The procedure also involved significant maternal risks,
including pulmonary edema due to aggressive tocolytic
therapy and abruptio placenta secondary to the proce-
dure itself. Given the invasiveness of sectio parva and
its associated high rate of maternal complications, there
is no reason to favor this approach over less invasive
techniques which are now available. Similarly, the tech-
nique of exteriorizing and ligating the umbilical cord
through a hysterotomy incision after ultrasound-guided
grasping of the cord (Foley et al., 1995) should not be
the method of choice given the many minimally invasive
techniques that have been developed.
Nowadays, there is consensus that when intervention
is required, minimally invasive techniques are associ-
ated with the best outcome for the pump twin and
pose the fewest maternal risks. These techniques can
be divided into ultrasound-guided and fetoscopy-guided
treatments (Table 2). Furthermore, if ultrasound guid-
ance is selected, ablation can be performed by targeting
the acardiac’s umbilical cord or, alternatively, its main
intra-abdominal vessels.
Cord occlusion techniques
The publication of Van Allen et al. (1983) describing
the TRAP sequence spurred attempts to treat the condi-
tion by interrupting the vascular supply to the acardiac
twin. Platt et al. (1983) were the first to suggest that
umbilical cord occlusion could be a plausible interven-
tion as a definitive treatment. Although this approach
Table 2—Minimally invasive treatment modalities
for vascular occlusion of acardiac twins
Cord occlusion
Ultrasound guided Coil
Alcohol-suture material
Absolute alcohol
Ligation
Monopolar diathermy
Bipolar diathermy
Fetoscopy guided Ligation
Laser
Intrafetal ablation
Ultrasound guided Absolute alcohol
Monopolar diathermy
Laser
Radiofrequency
Fetoscopy guided Currently not available
Copyright  2005 John Wiley & Sons, Ltd.
is theoretically a simple treatment, perinatologists have
encountered difficulties performing it successfully.
Cord occlusion can be achieved using either ultra-
sound or fetoscopy. Hamada et al. (1989) reported in
the Japanese literature the first successful case of percu-
taneous cord occlusion in an acardiac twin. Using ultra-
sound guidance, they introduced a thrombogenic steel
coil into the umbilical artery of the acardiac twin. How-
ever, this technique went largely unnoticed until Porreco
et al. (1991) described another successful case using
a similar technique in the English literature. Alcohol-
soaked suture material (Holzgreve et al., 1994) and other
thrombogenic agents, such as glucose, fibrin and enbu-
crilate gel, have also been used to achieve vascular
occlusion (Tan and Sepulveda, 2003). Electrical energy
has also been used to interrupt cord blood flow; both
monopolar (Rodeck et al., 1998; Holmes et al., 2001)
and bipolar (Deprest et al., 2000; Nicolini et al., 2001)
thermocoagulation techniques have been successfully
performed under ultrasound guidance. A multicenter
series of selective termination of monochorionic twins
using bipolar thermocoagulation that included five cases
of acardiac anomaly showed promising results with clin-
ical success in four cases (Deprest et al., 2000). Bipolar
coagulation has the theoretical advantage that the elec-
trical current does not travel through the umbilical cord,
placenta, the body of the co-twin or even the metal-
lic needle itself since the current passes only between
the two blades of the forceps (Deprest et al., 2000).
However, one potential disadvantage of this technique
includes the use of instruments with larger diameter than
needle-based procedures and, therefore, may be more
likely to cause preterm rupture of membranes. In addi-
tion, this technique has limited use in edematous cords
and requires disruption of the dividing membrane if the
access is through the amniotic sac of the pump twin.
Techniques employing fetoscopy can be used to ligate
the umbilical cord or to obliterate cord vessel flow with
laser energy. Fetoscopic-guided ligation of the umbilical
cord was first attempted by McCurdy et al. (1993). In
this case, the cord of the pump twin was accidentally
severed, but subsequent attempts have been successful
(Quintero et al., 1994, 1996; Willcourt et al., 1995).
Nevertheless, the overall experience with 16 pregnancies
in which fetoscopic-guided umbilical cord ligation was
performed has yielded a perinatal mortality rate of 38%
and severe preterm delivery rate, defined as <32 weeks,
of 70% (Tan and Sepulveda, 2003).
To date, all seven reported cases of fetoscopic-guided
coagulation of umbilical vessels using a neodymium:
yttrium-aluminum-garnet (Nd : YAG) laser have resulted
in live-born pump twins (Ville et al., 1994; Hecher
et al., 1997; Arias et al., 1998; Quintero et al., 2002).
However, procedures performed on pregnancies after
24 weeks of gestation failed to interrupt blood flow
to the acardiac fetus and were followed by severe
preterm delivery (Ville et al., 1994). On the other hand,
procedures performed before 24 weeks of gestation
successfully achieved cessation of blood flow and were
associated with term deliveries (Arias et al., 1998).
This suggests that advancing gestational age may be
associated with a hydropic cord or large umbilical
Prenat Diagn 2005; 25: 796–806.
 ACARDIAC ANOMALY 803

vessels that are more difficult to coagulate, and that

laser coagulation should only be performed at or before
24 weeks of gestation when it is associated with a
better prognosis (Ville et al., 1994; Arias et al., 1998).
Accordingly, a review by Arias et al. (1998) of 22
acardiac anomaly cases treated invasively concluded that
fetoscopic-guided laser coagulation is the best treatment
for acardiac anomaly if performed at or before 24 weeks,
and endoscopic- or ultrasound-guided cord ligation is
the best treatment after 24 weeks. Since the publication
of this review, however, new occlusive techniques
involving the fetal abdomen, which are less invasive
than fetoscopic procedures that target the umbilical cord,
have been developed (see below).
Using fetoscopy to perform cord ligation or laser
coagulation has its intrinsic disadvantages. Endoscopic
techniques are associated with a failure rate of about
10%, as well as increased risk of postoperative rupture of
membranes, intra-amniotic infection and bleeding of the
normal twin (Deprest et al., 1996; Quintero et al., 1996).
Cord ligation using endoscopy requires the insertion of
at least two 12-gauge trocars into the amniotic cavity
and the use of general anesthesia (Quintero et al., 1994).
Similarly, laser coagulation under endoscopy requires
the passage of larger instruments into the amniotic cav-
ity, is also usually performed under general anesthesia
and is a lengthy procedure (Arias et al., 1998; Quin-
tero et al., 2002). In addition, the identification of and
access to the umbilical cord is usually difficult, par-
ticularly in cases with anterior placentas, occasionally Figure 5—Pseudomonoamnionicity and entanglement of the umbilical
requiring additional procedures such as amnioinfusion cords as a complication of disruption of the dividing membrane during
attempted fetoscopic ligation of the umbilical cord in an acardiac twin.
and amniotomy to gain access to the acardiac twin’s The pump twin died as a result of cord entanglement
umbilical cord. This results in prolongation of operative
time and a more invasive procedure, together with the
potential risk of pseudomonoamnionicity due to disrup- present in hydropic acardiac fetuses and can cause dif-
tion of the intertwin membrane and entanglement of the ficulties in achieving cord occlusion by laser or bipolar
cord (Figure 5). Furthermore, fetoscopy is only available coagulation.
in a few centers in the world since it requires expensive In contrast, an intrafetal approach targets the abdom-
equipment and skilled operators. In contrast, ultrasound- inal aorta or pelvic vessels of the acardiac twin, which
guided needling techniques can be performed in facili- are easily identified regardless of cord condition, pla-
ties with the resources to perform fetal blood sampling, cental location, amniotic fluid volume and position of
requires simple instruments and low-cost materials, and the acardiac twin. The use of color Doppler ultra-
is less traumatic to the uterus and membranes (Tan and sound has greatly facilitated this method by allowing
Sepulveda, 2003). the clear visualization of the acardiac twin’s feeding
vessel and its main intra-abdominal branches. Intrafe-
tal ablation has been achieved with variety of tech-
Intrafetal techniques niques, including alcohol chemosclerosis (Sepulveda
et al., 1995, 2000, 2004a; Tongsong et al., 2002; Por-
reco, 2004; Gul et al., 2005), monopolar thermoco-
One of the main disadvantages of targeting the acardiac agulation (Rodeck et al., 1998; Holmes et al., 2001;
twin’s umbilical cord is the technical difficulty presented Chao et al., 2002), ultrasound-guided Nd : YAG laser
by its anatomy. The cord is often located near that of the (Jolly et al., 2001; Soothill et al., 2002; Sepulveda et al.,
pump twin, and cord occlusion procedures can inadver- 2004b) and radiofrequency (Tsao et al., 2002). A recent
tently damage the pump twin’s cord. Furthermore, the systematic review of the literature (Tan and Sepulveda,
close proximity of the umbilical artery and vein within 2003) of 71 cases using minimally invasive techniques
the acardiac twin’s cord poses a challenge when identify- (40 cases using cord occlusion in a funicular approach
ing the artery, which is crucial to avoid the intravascular and 31 cases using an intrafetal approach), found that
transfer of any ablative material into the circulation of intrafetal ablation, compared to cord occlusion, was
the pump twin. In addition, the umbilical cord of the associated with later median gestational age at delivery
acardiac twin is often short, thin and structurally abnor- (37 vs 32 weeks; p = 0.04), lower technical failure rate
mal, making it vulnerable to cord accidents secondary (13 vs 35%; p = 0.03), lower rate of premature delivery
to rupture or bleeding. Edematous cords may also be or rupture of membranes before 32 weeks (23 vs 58%;

Copyright  2005 John Wiley & Sons, Ltd. Prenat Diagn 2005; 25: 796–806.
804 A. E. WONG AND W. SEPULVEDA
p = 0.003) and higher rate of clinical success defined as
survival of the pump twin after achievement of complete
cessation of blood flow in the acardiac twin and deliv-
ery after 32 weeks (77 vs 50%; p = 0.02). This suggests
that intrafetal ablation is easier, less invasive, has a
higher rate of clinical success compared to ultrasound-
and fetoscopy-guided cord occlusion procedures, and
should be the treatment of choice for acardiac anomaly.
Alcohol chemosclerosis
The first attempt at an intrafetal approach to treat
acardiac anomaly was described in the 1980s, when the
pulsatile tissue within the acardiac twin was repeatedly
pierced to produce tamponade (Seeds et al., 1987).
However, this attempt failed. Using absolute alcohol as
the sclerosing agent, Sepulveda et al. (1995) reported
the first case of intrafetal ablation, along with the
technique and rationale for targeting the intra-abdominal
vessels rather than the umbilical cord. Several other
cases have since been reported (Sepulveda et al., 2000,
2004a; Tongsong et al., 2002; Porreco, 2004). Indeed,
alcohol embolization has the potential of being the most
widely available technique for intrafetal ablation, as
it can be performed as an outpatient procedure with
minimal equipment including a spinal needle through
which absolute alcohol is injected. However, its main
disadvantages are that it may cause transient occlusion
and intrauterine demise of the pump twin from the
transfer of the sclerosant into its circulation. In a recent
multicenter series of eight cases using a standardized
protocol of intrafetal alcohol chemosclerosis, three pump
twins died as a result of the procedure, presumably
due to acute thrombosis of the umbilical vessels of the
pump twins secondary to inadvertent vascular migration
of the alcohol (Sepulveda et al., 2004a). In another
report, massive injection of alcohol failed to obliterate
blood flow into the acardiac twin (Ozenren et al., 2004),
probably due to extravascular injection of the alcohol.
Nevertheless, as safer alternatives are now available,
soluble sclerosants such as alcohol should, in our
current opinion, only be used in centers where more
sophisticated treatments are not available.
Monopolar diathermy
Another approach to coagulation of the intra-abdominal
vessels of the acardiac twin is monopolar diathermy.
This technique uses an 18-gauge needle, a 1-mm diam-
eter wire electrode insulated with polytetrafluroethylene
along its length except for 3 mm at the tip, a ground pad
and a standard monopolar diathermy generator (Rodeck
et al., 1998). Alternatively, a 1.5-mm monopolar needle
electrode, commonly used for ovarian drilling in poly-
cystic ovarian syndrome, can be introduced through an
18-gauge trocar (Chao et al., 2002). Monopolar coag-
ulation can be performed in early pregnancy as an
outpatient procedure under local anesthesia. Although
the original monopolar device is purpose-made and still
not commercially available, a technique using a simple
wire as an alternative was recently described (Sepulveda
et al., 2003). The major disadvantage of this technique
Copyright  2005 John Wiley & Sons, Ltd.
Figure 6—Thermal injury along the track of the needle as a
complication of intrafetal monopolar coagulation
is the risk of thermal injury along the path of the needle
which can potentially damage the uterus and membranes
(Figure 6).
Laser coagulation
Interstitial laser can also be applied intrafetally using
an 18-gauge needle and a standard Nd : YAG laser fiber
(Jolly et al., 2001; Sepulveda et al., 2004b; Soothill
et al., 2002). With ultrasound guidance, the needle is
advanced into the abdomen or pelvis of the acardiac twin
close to the target vessel, and the laser fiber is introduced
through the needle until its tip protrudes slightly beyond
the needle tip. The laser energy can then be applied in
pulses, with increases in power if necessary, until the
vascular area is hyperechogenic indicating occlusion of
the blood vessels. Unlike monopolar diathermy, there
is no risk of inadvertent cautery burns that can pose a
risk to the surrounding tissues as the energy is applied
only at the tip of the laser fiber. However, the primary
disadvantage of this method is that few fetal medicine
units have access to such facilities due to the expense
of, and the infrequent need for, the technique.
Radiofrequency ablation
The most recently developed technique of radiofre-
quency ablation (Tsao et al., 2002) uses a 3-mm 14-
gauge needle specifically designed for this purpose and
a radiofrequency generator, which is expensive and
not widely available. This method uses radiofrequency
energy that is progressively increased until impedance
of the device indicates satisfactory coagulation or when
color Doppler ultrasound confirms obliteration of blood
flow to the acardiac fetus. All 13 of the reported cases
using this technique were technically successful, and
only one infant did not survive due to complications of
prematurity. Because the energy from the radiofrequency
device is applied only to the tip after deployment into
tissue, it avoids injury to the co-twin and surrounding
tissues. Although the device has a large diameter and
Prenat Diagn 2005; 25: 796–806.
 ACARDIAC ANOMALY
therefore has the theoretical risk of increasing the rate
of rupture of membranes, no postoperative rupture of
membranes occurred in this reported series. Based on
this single series, radiofrequency ablation appears to be
a relatively safe and effective technique for the treatment
of acardiac anomaly.
CONCLUSION
Once acardiac anomaly is diagnosed, both the acardiac
and pump twins should be assessed to classify the
pregnancy according to prognostic factors based on
the size and growth of the acardiac twin and the
cardiovascular condition of the pump twin. In cases
where the acardiac twin is small and when there are no
signs of cardiovascular impairment in the pump twin,
serial ultrasound surveillance is important to detect any
worsening of the condition, which may suggest the need
for intervention to optimize the pump-twin’s chance
of survival. It is our recommendation that all cases
associated with large acardiac twins or those showing
rapid growth of the acardiac mass should be treated.
Once treatment is indicated, the intrafetal approach
to interrupt the vascular supply to the acardiac twin
appears to be superior to cord occlusion techniques
as it is simpler, safer and more effective. The first
line of treatment, if available, should be ultrasound-
guided laser coagulation or radiofrequency ablation of
the intrafetal vessels. If these methods are not available,
however, chemosclerosis of the intrafetal arterial vessel
with alcohol should be attempted.
ACKNOWLEDGMENTS
We are grateful to Dr O. Atobe, Dr E. Corral, Dr P.
Espinola and Dr A. Espinoza, who kindly provided
some of the pictures used in this review. This work was
supported by the Sociedad Profesional de Medicina Fetal
‘Fetalmed’ Limitada and a grant from the Direccion
Academica, Clinica Las Condes, Chile.
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