Received: 6 May 2018 Revised: 19 December 2018 Accepted: 21 January 2019

DOI: 10.1002/jcu.22700

CASE REPORT

Criss-cross heart with double-outlet right ventricle,

subpulmonary ventricular septal defect, and bicuspid
pulmonary valve
Sihua Ren MD1 | Chunyan Ma MD, PhD2 | Songbai Li MD, PhD1

1
Department of Radiology, The First Affiliated
Hospital of China Medical University, Abstract
Shenyang, China Criss-cross heart (CCH) is an extremely rare complex congenital heart malformation. It accounts for
2
Department of Cardiovascular Ultrasound, less than 0.1% of congenital heart diseases. Here, we describe a unique case of CCH with double-
The First Affiliated Hospital of China Medical
outlet right ventricle, huge subpulmonary ventricular septal defect, bicuspid pulmonary valve, and
University, Shenyang, China
right-hand aortic arch. The anatomic features were observed with echocardiography, and the diag-
Correspondence
Songbai Li, Department of Radiology, The First nosis was confirmed at surgery. Many variations of CCH have been described. The present case
Affiliated Hospital of China Medical University, expands the spectrum of this entity and may provide new insight into this complex anatomy.
No.115 Nanjing Street, Heping District,
Shenyang 110001, China.
KEYWORDS
Email: songbaili01@163.com
aortic arch, bicuspid pulmonary valve, congenital cardiac defects, criss-cross heart,
echocardiography

1 | I N T RO D UC T I O N previous 3 days. The first child of nonconsanguineous parents, he was

Criss-cross heart (CCH) is characterized by distorted atrioventricular
connections and crossed systemic and pulmonary venous streams at
the atrioventricular level, without mixing.1 It is found in less than 0.1%
of all congenital heart diseases.2 The diagnosis and management of
this pathology, given the many forms that it can take, can be highly
challenging even for an experienced pediatric cardiologist and cardio-
vascular surgeon. The first case was reported by Lev and Rowlatt in
1961.3,4 The term “criss-cross heart” first appeared in the literature in
a manuscript published in 1974.5 CCH was found to be associated
with a wide range of abnormal cardiac anatomic features and hemody-
namic findings.6 CCH with well-developed ventricles, double-outlet
right ventricle (DORV), subpulmonary ventricular septal defect (VSD),
abnormal pulmonary valve, straddling of the morphologic mitral valve,
right-sided aortic arch, and patent ductus arteriosus, as in the present
case, is an extremely rare congenital cardiovascular malformation.
2 | CASE REPORT
A four-month-old male infant, weighing 5.5 kg, was referred to our
hospital because of worsening cyanosis of the lips, acrocyanosis for
1 week, and fever above 38 C, as well as a productive cough for the
born by vaginal delivery with an Apgar score of 9 at 1 minute and a
birth weight of 3 kg. He manifested dyspnea, feeding difficulty, and
cyanosis from birth. There was no family medical history of congenital
heart disease. Physical examination revealed that his heart and respi-
ratory rates at rest were 138/min and 38/min, respectively. His blood
pressure was 97/51 mmHg. Percutaneous oxygen saturation was
13% without oxygen and 30% to 60% with the administration of low-
flow-rate oxygen by nasal catheter. Cardiac auscultation revealed that
the cardiac sounds were powerful and regular, with a grade 4/6 sys-
tolic murmur best heard at the apical region of the heart. Electrocardi-
ography showed a normal sinus rhythm. There was evidence of
respiratory distress (the “three-concave” sign was positive). Chest aus-
cultation yielded rough breath sounds and rales. The chest X-ray
revealed an enlarged levocardia (cardiothoracic ratio 0.6) with scat-
tered patchy pulmonary shadows. Visceral palpation showed the liver,
soft in texture, to be 2 cm below the rib cage. The cytomegalovirus
IgM antibody was positive. The results of karyotyping were normal.
Two-dimensional transthoracic echocardiography (2D TTE) using
an EPIQ7C sonographic system (Philips, Andover, MA) with a pediatric
S8-3 probe revealed situs solitus. The atria were situated normally,
with normal veno-atrial connections and atrioventricular concordance.
However, by rotating the probe, the ventricular spatial relationship

J Clin Ultrasound. 2019;1–4. wileyonlinelibrary.com/journal/jcu © 2019 Wiley Periodicals, Inc. 1

2 REN ET AL.
was found to be abnormal. Subcostal coronal sections demonstrated
that the left-sided left atrium and the right-sided morphologically left
ventricle were connected and that the right-sided right atrium was
connected to the left-sided morphologically right ventricle (Figure 1A).
The left ventricle was in posteroinferior position, whereas the right
ventricle was anterosuperior. This made it look as though each atrium
was ejecting into the contralateral ventricle. The atrioventricular axes
were nearly orthogonal to each other instead of parallel, and the atrio-
ventricular valves were seen to cross each other. Furthermore, the
ventricular septum was parallel rather than orthogonal to the dia-
phragmatic muscle plane (Figure 1B, Supporting Information Video
S1). The ventricles were twisted clockwise along their long axes when
viewed from the apical position, whereas the base of the heart
remained fixed. Bidimensional color Doppler TTE demonstrated the
anomalous ventriculoarterial connection. The two great arteries origi-
nated entirely from the morphologic right ventricle (Figure 1C, Sup-
porting Information Videos S2 and S3). A large subpulmonary VSD
about 11 mm in diameter was also revealed (Figure 1D and Support-
ing Information S1), with low-velocity right-to-left shunting
(Supporting Information Figure S2). Mitral tendinous straddling
through the VSD connected it to the wall of the right ventricle
(Supporting Information Video S4). The pulmonary trunk was located
to the right and posterior to the aorta (Figure 1E). Subpulmonary coni
were absent; therefore, the pulmonary artery was directly connected
to the tricuspid valve. However, subaortic coni were present. The pul-
monary valve showed unusual bicuspid structure, and both thickening
and adhesion, with severe stenosis, were present (Figure 1F, Support-
ing Information Video S5). The anteroposterior diameter of the pulmo-
nary valve orifice was about 4 mm, and the cross-valve flow velocity
accelerated to about 4 m/s. The internal diameter of the main pulmo-
nary artery's trunk was 15 mm, and the diameter of the aortic trunk
was 14 mm. The aortic valve still had a three-leaf structure. The thick-
ness of the right ventricular wall was 4 mm. Color Doppler echocardi-
ography also revealed a patent ductus arteriosus with a small amount
of left-to-right shunting (Figure 1G). The aortic arch was right-sided.
This infant had well-developed left and right ventricles. The left ven-
tricle was slightly smaller than the right, and the anteroposterior diam-
eters were 21 and 22 mm, respectively. Left ventricular systolic
function was normal. There was no pericardial effusion.
These echocardiographic findings together with the other clinical
data led to the following diagnoses: (1) congenital heart disease (CCH
with well-developed ventricles, DORV, subpulmonary VSD, bicuspid
pulmonary valve malformation with severe valvular stenosis, strad-
dling of the morphologic mitral valve, right-sided aortic arch, and pat-
ent ductus arteriosus), (2) acute severe bronchopneumonia, (3) mild
heart failure, and (4) cytomegalovirus infection.
The infant was transferred to the pediatric intensive care unit in a
critical condition. Rescue was needed several times in a period of
10 days until he was in stable condition. Cardiac operation was under-
taken later. During surgery, interventricular septum closure by patch,
arterial switch for connecting the left ventricle to the aorta in postop-
erative heart, pulmonary valve stenosis correction, and ligation of pat-
ent ductus arteriosus were performed. The patient was followed-up
every 6 months and remained stable. He is currently 2-years-old and
doing well.
3 | DI SCU SSION
CCH is considered to be an extremely rare congenital cardiac
abnormality. One proposed mechanism suggests that the spatial
arrangement of the ventricles is altered at the embryonic developmen-
tal stage owing to clockwise or counterclockwise rotation of the ventri-
cles along the cardiac long axis, so that the ambilateral atrioventricular
structures are not parallel but angulated by nearly 90 1,2
(Figure 1H).
The rotation of ventricles in CCH can be either clockwise or counter-
clockwise.7,8 If the ventricles are rotated clockwise, the atrioventricular
connections tend toward concordance; complete transposition of the
great arteries (TGA) and DORV may be combined. If the ventricles are
rotated counterclockwise, the atrioventricular connections tend toward
discordance; corrected TGA and DORV may be combined. This posi-
tional anomaly can coexist with a horizontal displacement of the ven-
tricular mass, placing the ventricles in supero-inferior position. Medical
imaging has shown that one can suspect CCH on the basis of the fol-
1
lowing characteristics: inability to obtain a four-chamber view at any
2
angle; a spatial atrioventricular connection with the interventricular
septum in a spiraling orientation; 3 the two ventricular inlets directed in
a supero-inferior and crossing position. To image the other atrioventric-
ular valves and ventricles, the probe must be tilted and a careful scan
(postero-inferior to antero-superior) implemented in order to visualize
the twisted spatial relationship of the atrioventricular valves, which are
not in parallel but cross each other.
In our patient, there was atrioventricular concordance with an
abnormal ventricular arterial junction. In CCH, changes in cardiac con-
duction bundles may vary. A review of the literature does not reveal
any isolated cases of CCH. VSD in CCH is common,9 but the present
case, with a subpulmonary valve VSD, is rare. In addition, CCH is usu-
ally associated with subpulmonary valvular stenosis or pulmonary ste-
nosis. However, to the best of our knowledge, the present case, in
which echocardiography clearly showed a bicuspid pulmonary valve
malformation in CCH, is the first to be reported in the literature. Fur-
ther, CCH often incorporates abnormalities of the great arteries, the
most common being transposition of great arteries with a double-
cone structure under two large arterial valves. There was no conical
structure under the pulmonary valves in this infant, and we found no
previous report of a pulmonary valve directly linked with the tricuspid
valve in a patient with CCH in the available literature.
In conclusion, CCH is a rare congenital cardiac malformation that
can be diagnosed by an expert echocardiographer to determine the
relationships of cardiac chambers and associated other cardiac anoma-
lies. Surgical options vary according to exact sequential segmental
analysis and associated abnormalities. Echocardiography is a good
method of diagnosis and should be used in conjunction with MRI
when available. The differential diagnoses for CCH include single ven-
tricle, common atrioventricular valve, isolated supero-inferior ventri-
cles, an L-transposed aorta with pulmonary hypoplasia, etc. Through
echocardiography, true CCH should be suspected when the parallel
arrangement of the atrioventricular valves and ventricular inlets can-
not be showed in any single view and the two atrioventricular valves
cannot be visualized clearly simultaneously on parasternal, apical, or
subcostal four-chamber views. Color Doppler imaging can provide
REN ET AL. 3

FIGURE 1 Criss-cross heart with double-outlet right ventricle, subpulmonary ventricular septal defect, and bicuspid pulmonary valve in an infant.
(A) Subcostal sections demonstrate that the left-sided left atrium connects to right-sided left ventricle through mitral valve (left arrow traces the route from
left atrium to left ventricle); the right-sided right atrium connects to left-sided right ventricle through tricuspid valve (right arrow traces the route from right
atrium to right ventricle). The atria and ventricles are of normal size. (B) Left ventricle postero-inferiorly and right ventricle antero-superiorly located. The
ventricular septum is parallel to the diaphragm. (C) Color Doppler parasternal views show the aorta arising from the morphological right ventricle (left); the
pulmonary artery also arises from the right ventricle, and bifurcation and turbulence flow in the pulmonary artery can be seen (right). (D) Parasternal short-
axis view of the pulmonary valve reveals that the closure line appears as a line, and there is a large defect of interventricular septum (arrow) under the
pulmonary valve. (E) Color Doppler showing that the pulmonary trunk is located at the right of the aorta. (F) Parasternal short-axis view of pulmonary valve
showing the bicuspid valve open and close during systole and diastole (arrows). The valve was thickened, with adhesion. (G) Color Doppler parasternal view
demonstrates a patent ductus arteriosus with a small amount of left-to-right shunting (arrow). (H) Embryonic development schematic of criss-cross heart.
Atria are situated normally. With right loop of ventricles and clockwise rotation of ventricles along the cardiac long axis, the left-sided left atrium connects
to right inferior morphologically left ventricle, and the right-sided right atrium connects to the left superior morphologically right ventricle, resulting in
atrioventricular connections concordance. With left loop of ventricles and counterclockwise rotation of the ventricles rotate, the left-sided left atrium
connects to right superior morphologically right ventricle, and the right-sided right atrium connects to left inferior morphologically left ventricle, resulting in
atrioventricular connections discordance. Abbreviation: AO, aorta; CCH, criss-cross heart; IVS, interventricular septum; LA, left atrium; L.I., left inferior; L.S.,
left superior; LV, left ventricle; PA, pulmonary artery; PV, pulmonary valve; RA, right atrium; R.I., right inferior; R.S., right superior; RV, right ventricle
4 REN ET AL.
critical information to assess the atrioventricular connection, visualize
the direction of intracardiac blood flows, and identify the crossover of
the inflow streams. Isolated CCH with adequate four-chamber devel-
opment may require only palliative surgery after birth. Successful
intracardiac repair of CCH like this case with straddling valve has
rarely been reported. CCH should be kept in mind in order to recog-
nize it on medical imaging. Given the rarity of this cardiac malforma-
tion, the specimen-like quality of visual image interpretation and vivid
schematic diagrams are very helpful not only for clinical diagnosis but
also for didactic purposes.
CONF LICT OF IN TE RE ST
None.
ORCID
Songbai Li https://orcid.org/0000-0002-5302-4976
RE FE R ENC E S
1. Yang YL, Wang XF, Cheng TO, et al. Echocardiographic characteristics
of the criss-cross heart. Int J Cardiol. 2010;140:133-137.
2. Duncan WJ, Wong KK, Freedom RM. A criss-cross heart with twisted
atrioventricular connections, "perfect streaming," and double discor-
dance. Pediatr Cardiol. 2006;27:604-607.
3. Lev M, Rowlatt UF. The pathologic anatomy of mixed levocardia. A
review of thirteen cases of atrial or ventricular inversion with or with-
out corrected transposition. Am J Cardiol. 1961;8:216.
4. Yadav S, Haranahalli PE, Shukla M, et al. Criss-cross heart with atrial
inversion and juxtaposed atrial appendages: an echocardiographic
study. Turk Kardiyol Dern Ars. 2013;41:564.
5. Anderson RH, Shinebourne EA, Gerlis LM. Criss-cross atrioventricular
relationships producing paradoxical atrioventricular concordance or dis-
cordance. Their significance to nomenclature of congenital heart dis-
ease. Circulation. 1974;50:176-180.
6. Shirakawa A, Kaji T, Hayabuchi Y, et al. Prenatal three-dimensional color
Doppler imaging showing crossover of the inflow streams of two ventri-
cles in a case of criss-cross heart. J Echocardiogr. 2017;15:191–193.
7. Hluchy J, Prull MW, Brandts B. Radiofrequency ablation of a left lateral
atrioventricular accessory pathway in a 13-year-old boy with a criss-
cross heart guided by nonfluoroscopic imaging. J Electrocardiol. 2014;
47:311-315.
8. Kasar T, Ayyildiz P, Turkvatan A, et al. Criss-cross heart with dextrocar-
dia and transposition of the great arteries: a rare pathology. Turk Kar-
diyol Dern Ars. 2016;44:91.
9. Martinez QE, Rodriguez GF, Agredo MJ. Criss cross heart in a congeni-
tally corrected transposition of the great arteries. Int J Cardiol. 2008;
130:e81-e82.
SUPPOR TI NG I NFORMATION
Additional supporting information may be found online in the Sup-
porting Information section at the end of the article.
How to cite this article: Ren S, Ma C, Li S. Criss-cross heart
with double-outlet right ventricle, subpulmonary ventricular
septal defect, and bicuspid pulmonary valve. J Clin Ultrasound.
2019;1–4. https://doi.org/10.1002/jcu.22700