European Heart Journal Supplements (2014) 16 (Supplement B), B32B36

The Heart of the Matter

doi:10.1093/eurheartj/suu008

Evaluation of cardiovascular anomalies in conjoined

twins: a single-center experience from King
Abdulaziz Cardiac Center
Abdulsalam Alsayad1, Abdu AlKhatabi1, Mohammed H. Alghamdi1,2,
Abdullah Al Rabeeah3, Fahad Alhabshan1,4, and Riyadh M. Abu-Sulaiman1,4*
1
Division of Pediatric Cardiology, King Abdulaziz Cardiac Center, King Abdulaziz Medical City, Ministry of National
GuardHealth Affairs, PO BOX 22490, Riyadh 11426, Kingdom of Saudi Arabia
2
Pediatric and Fetal Echocardiography Laboratory, King Fahad Cardiac Center, King Saud University, Riyadh, Kingdom of
Saudi Arabia
3
Division of Pediatric Surgery, Department of Surgery, King Abdulaziz Medical City, Ministry of National Guard - Health
Affairs, Riyadh, Kingdom of Saudi Arabia

Downloaded from by guest on January 16, 2015

4
King Saud bin Abdulaziz University for Health Sciences, Riyadh, Kingdom of Saudi Arabia

KEYWORDS The aim of the study is to describe varieties of congenital heart disease (CHD) in different
Conjoined twins; types of conjoined twins (ConTw). This is a retrospective (from 1997 to 2014) analysis of
Congenital heart disease 42 sets of ConTw with different levels of body and/or organ fusion, referred to our center
for cardiac evaluation prior to separation. Conjoined twins were classied into Classes 1,
2, and 3 according to heart abnormalities and further subclasses of a, b, and c according
to minor or major heart disease. Of the 42 sets, there were 35 sets of twins (83.3%) who
were symmetrical including 3 craniopagus, 3 pygopagus, 14 thoracopagus, 11 omphalo-
pagus, 3 ischiopagus, and 1 parapagus. Seven sets of twins (16.7%) were asymmetrical
with a variable degree of thoraco-omphalo-ischiopagus fusion. Twenty-six of 40 sets
(65%) were female. Overall incidence rate of cardiac abnormalities was 76.2%. Major
cardiac abnormalities were common in thoracopagus twins (14 sets). Two sets (14%)
shared the pericardium, whereas three sets (21.5%) were fused at atrial level, two sets
(14%) at ventricle level, and seven sets (50%) had a severely malformed single heart
with fusion at both the atrial and ventricular level. Conjoined twins have a high incidence
of CHD. All symmetrical thoracopagus tetrapus twins had major cardiac abnormalities
precluding their separation and all of them did not survive. Incidence of major cardiac
malformations was less in the other types of ConTw.

Introduction of 3 : 1.4 Twins are classied according to the major site of

The incidence of conjoined twins (ConTw) is rare, ranging
from one in 50 000 to one in 200 000, which accounts for
1% of monochorionic twin pregnancies.1,2 About 60% of
these twins are stillborn or die shortly after birth.3 A higher
incidence was observed in girls with a female-to-male ratio
* Corresponding author. Tel: +966 1 8011111, Fax: +966 1 8016773.
Email: abusulaiman@mailzone.com
union. Thoracopagus (joined at the thorax) twins are the
most common type, accounting for 40% of cases, followed
by omphalopagus (joined at upper abdomen + lower
thorax), accounting for 32%. Other forms are: pygopagus
(joined at the sacrum and coccyx), ischiopagus (joined at
the lower abdomen below umbilicus down to the pelvis/peri-
neum), and craniopagus (joined at the cranial vault). The
term parapagus is used to describe a variable degree of
side-to-side fusion.2,5

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014.
For permissions please email: journals.permissions@oup.com
Evaluation of cardiovascular anomalies in conjoined twins B33

The overall incidence rate of congenital heart disease

10 (23.8%)

5 (11.9%)
(CHD) in ConTw is 66%.5 Thoracopagus twins carry the

42 (100%)
4 (9.5%)

2 (4.8%)
8 (19%)
13 (31%)
highest incidence rate of cardiac malformations reaching
Total
up to 94.4% for major CHD and 72.2% for cardiac defects
with single-ventricle physiology.6 The extent of cardiac
fusion and the severity of cardiac abnormalities play a
major role in the medical decision of surgical separation
and in long-term survival.
Thoraco-omphalo-ischiopagus (variable

We report the association of cardiovascular anomalies

in 42 sets of ConTw who have been referred over a period
of 17 years from 1997 to 2014 at our center for cardiac
evaluation with regard to their potential separation. To
Asymmetrical (7; 16.7%)

the best of our knowledge, this is the largest single-center

degrees of conjunction)

series of ConTw that describe the presence of CHD in this

1 (14.27%) challenging population.
1 (14.27%)
1 (14.27%)
7 (16.7%)
2 (28.6%)

2 (28.6%)

Methods
Between 1997 and 2014, 42 sets of ConTw with different levels
of fusion were referred to our institution to be assessed for
Parapagus

1 (2.35%)

the feasibility of surgical separation. Twenty-six of 40 sets

1 (100%)

(65%) were female. In two sets of ConTw, gender was not con-
rmed since diagnosis was made during fetal life. Thirty-ve
sets of twins (83.3%) were symmetrical (3 craniopagus, 3 pygo-
pagus, 14 thoracopagus, 11 omphalopagus, 3 ischiopagus, and
Ischiopagus

1 parapagus twins), whereas 7 sets of twins (16.7%) were asym-

3 (7.15%)

Downloaded from by guest on January 16, 2015

1 (33.3%)

2 (66.7%)

metrical with a variable degree of thoraco-omphalo-ischiopagus

fusion. Full cardiac evaluation was performed in all sets by com-
Ventral/lateral conjunction (29; 69%)

prehensive two-dimensional (2D) transthoracic echocardiography

modied as needed by the accessibility of subcostal and precordial
Omphalopagus

views.
3 (27.25%)
3 (27.25%)

11 (26.2%)

ConTw were classied according to the presence or absence of

2 (18.2%)
2 (18.2%)

1 (9.1%)

minor/major cardiac abnormalities. Class 1 indicates that both

twins had normal hearts. Class 2 means that both twins had abnor-
mal hearts, which was further subdivided into further subclasses 2a
if both had minor CHD, 2b if both twins had major CHD, and 2c if one
Table 1 Type of conjoined twins and classication of congenital heart disease

Thoracopagus

twin had minor while the other had major CHD. Class 3 indicates
13 (92.85%)

14 (33.3%)
1 (7.15%)

that one twin had a normal heart while the other had either
minor 3a or major 3b CHD. Major CHD was dened as any cardiac
abnormality that required medical or surgical intervention in the
rst year of life.
In nine sets of twins (21.4%), cardiac diagnosis was made by
Pygopagus

3 (7.15%)
Symmetrical (35; 83.3%)

fetal echocardiography. Most of them were thoracopaus (six

3 (100%)

Bold values indicate the total for both numbers and percentages.

sets), while three were found to be thoraco-omphalopagus. All

Conjoined twins (42)

Dorsal conjunction

of these nine sets of twins had major CHD in terms of a fused

heart chamber or single-ventricular anatomy. Transoesophageal
Craniopagus

echocardiography was performed in only one set. Three sets of

3 (7.15%)

twins required further cardiac imaging, including computed tom-

1 (33.3%)
2 (66.7%)
(6; 4.3%)

ography cardiac angiography (CTA) in two sets and cardiac mag-

netic resonance imaging (CMRI) in one set. This was necessary
Class 3 (one normal and one abnormal)

to assess the vascular anatomy that was not clear by 2D echocar-

diography.
One minor and one major (2c)
Class 2 (both are abnormal)

Results
Class 1 (both are normal)
Classication of CHD

Table 1 summarizes the classication used which can be

Both major (2b)
Both minor (2a)

summarized as follows: 10 sets were in Class 1 (23.8%), 25

sets in Class 2, and more precisely 8 in 2a, 13 in 2b, and 4
Major (3b)
Minor (3a)

in 2c.
Figure 1 shows the level of sharing/fusion in 14 sets of
Total

thoracopagus ConTw. All of them had major CHD. Two sets

(14%) shared the pericardium only, whereas 3 sets (21.5%)
B34 A. Alsayad et al.

Figure 1 Flowchart of our series of thoracopagus conjoined twins with classication of their congenital heart disease and fusion level of cardiac structures.

Downloaded from by guest on January 16, 2015

Figure 2 Single fused heart: two-dimensional and two-dimensional colour ow Doppler echocardiography images (AE) and schematic diagram (F ) showing
major cardiac abnormalities with fusion of both hearts at the atrial and ventricular level in one set of conjoined thoracopagus twins (Twin A and Twin B). There
are two atrial (a) and four ventricular masses (v). Each heart gives rise to aorta (Ao) and pulmonary artery (PA) to each twin.
Evaluation of cardiovascular anomalies in conjoined twins
Figure 3 Computed tomography angiography and three-dimensional reconstruction images from conjoined twins with thoraco-omphalopagus showing a
single fused heart at the atrial and ventricular level. Maximum intensity projection (MIP) images in the sagital (A) and axial (C) planes as well as three-dimen-
sional reconstruction (B) and (D) delineate clearly the vascular structure for both twins. There are two atrial (a) and four ventricular (v) masses. Each heart
gives rise to aorta (Ao) and pulmonary artery (PA) to each twin.
were fused at the atrial level, 2 sets (14%) at the ventricle
level, and 7 sets (50%) had a severely malformed single
heart with fusion at both the atrial and ventricular level
(Figures 2 and 3).
Major CHD was far more common in thoracopagus twins
(14/14 sets; 100%), compared with omphalopagus twins
(3/11 sets). No major cardiac abnormalities were detected
in the other forms of ConTw.
Discussion
Although the literature related to ConTw is extensive, only
few large series have been reported. Similar to previous
reports,2,6 our study conrmed that thoracopagus connec-
tion is the most common form of ConTw with a higher inci-
dence rate in female. Thoracopagus twins are associated
with a greater degree of cardiac fusion and carry a higher
risk of cardiac malformations compared with other forms
of connections.3
An accurate delineation of cardiac anatomy and the
degree of cardiac fusion is essential to determine the feasi-
bility of surgical separation. In our series, all sets of twins
who had fused hearts at atrial, ventricular, or both levels
were considered not to be candidates for surgical separ-
ation, and as expected none survived.
B35
Downloaded from by guest on January 16, 2015
Our experience has been accumulated through the years
during which the quality and availability of imaging modal-
ities has changed considerably. Although 2D echocardiog-
raphy performed by a skilled operator continues to be the
initial tool to assess cardiac fusion, anatomy, and function,
new imaging modalities such as CTA and CMRI are more ac-
curate with regard to the denition of fusion and to the
great vessels anatomy.7 MRI has an increasing role in multi-
system evaluation of ConTw before separation. It offers the
advantage to allow generation of three-dimensional (3D)
image reconstructions in any plane and orientation as
well as to provide information about intra-cardiac and
great vessels anatomy.
The support from the advances in imaging, multidiscip-
linary approach, and improvements in intensive care man-
agement has resulted in improvement in surgical
separation over time. In any case, separation should be
only performed in specialized tertiary centers familiar
with this kind of complex surgery.5,8
Acknowledgements
We thank Prof. Talat M. Yelbuz, MD, PhD, for his help during draft-
ing, critical reading, and revision of this manuscript.
Conict of interest: none declared.
B36
References
1. Lopes LM, Brizot ML, Schultz R, Liao AW, Krebs VL, Francisco RP, Zugaib M.
Twenty-ve years of fetal echocardiography in conjoined twins: lessons
learned. J Am Soc Echocardiogr 2013;26:530538.
2. Andrews RE, McMahon CJ, Yates RW, Cullen S, de Leval MR, Kiely EM,
Spitz L, Sullivan ID. Echocardiographic assessment of conjoined twins.
Heart 2006;92:382387.
3. Spitz L, Kiely EM. Experience in the management of conjoined twins. Br J
Surg 2002;89:11881192.
A. Alsayad et al.
4. Machin GA. Conjoined twins: implications for blastogenesis. Birth
Defects Orig Artic Ser 1993;29:141179.
5. McMahon CJ, Spencer R. Congenital heart defects in conjoined twins:
outcome after surgical separation of thoracopagus. Pediatr Cardiol
2006;27:112.
6. Thomas Collins R II, Weinberg PM, Gruber PJ, St John Sutton MG.
Conjoined hearts in thoracopagus twins. Pediatr Cardiol 2012;33:252257.
7. McHugh K, Kiely EM, Spitz L. Imaging of conjoined twins. Pediatr Radiol
2006;36:899910.
8. Al Rabeeah A. Conjoined twinspast, present, and future. J Pediatr Surg
2006;41:10001004.
Downloaded from by guest on January 16, 2015