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Received: 5 July 2019    Accepted: 13 August 2019

DOI: 10.1111/echo.14470

Absence of the fetal right pulmonary artery complicated with

coarctation of the aorta: Prenatal and postnatal diagnosis

Yu Wang MD1,2  | Jiaqi Zhang MD1,2  | Wei Feng MD1,2 | You Wu MD1,2 |

Li Yang MD1,2 | Jiabao Yin MD1,2

1
Department of Ultrasound, Xiangyang
No.1 People’s Hospital, Hubei University of Abstract
Medicine, Xiangyang, China Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular
2
Xiangyang Key Laboratory of Maternal‐
malformation that can present as an isolated lesion or may be associated with other
Fetal Medicine in Fetal Heart Disease,
Xiangyang, China congenital heart malformations. Several studies have reported UAPA after birth. To
our knowledge, the absence of the right pulmonary artery in the fetus has not been
Correspondence
Yu Wang and Jiabao Yin, Department reported. Here, we report a rare case of fetal right pulmonary artery absence with
of Ultrasound, Xiangyang No.1 People’s
aortic coarctation, which was confirmed by postpartum ultrasound and computed
Hospital Affiliated to Hubei University of
Medicine, Xiangyang Key Laboratory of tomography angiography (CTA). Our case demonstrates that fetal echocardiography,
Maternal‐Fetal Medicine in Fetal Heart
especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary
Disease, No 15, Jiefang Avenue, Xiangyang,
Hubei, 441000, China. artery malformations.
Emails: 287383672@qq.com (YW) and
jiabaoyin-xf1227@163.com (JY) KEYWORDS
Funding information absence of right pulmonary artery, coarctation of the aorta, fetus
This work was supported by the Key Project
in new profession and new technology
of Xiangyang No.1 People's Hospital,
NO.2019412.06.

A 33‐year‐old pregnant woman (gravida 1, para 0) was referred to
our maternal–fetal medical center at 27 + 4 weeks for suspected
heart defects. The cell‐free fetal DNA test suggested a low risk of
aneuploidy.
Fetal echocardiography showed the absence of the right pulmo‐
nary artery in the three‐vessel view (Figure 1A). Prenatal ultrasound
showed a left aortic arch. The left pulmonary artery originated from
the main pulmonary artery. A major aortopulmonary collateral artery
(MAPCA) was formed. Coarctation of the aortic arch was detected
in the isthmus with an internal diameter of approximately 0.16 cm
(Figure 1B–F). Postpartum ultrasound showed that the left pulmo‐
nary artery originated from the autonomous pulmonary artery, but
no right pulmonary artery was found (Figure 2B,C). The internal di‐
ameter of the isthmus of the aortic arch was 0.5 cm, and the aortic
arch velocity was also increased (Figure 2D–F). Postpartum CTA and
3D reconstruction confirmed the prenatal diagnosis of the absence
of the right pulmonary artery associated with mild coarctation of the
aorta. The distal branch of the right lung was supplied by the bron‐
chial artery and MAPCA (Figure 3A–F).
Unilateral absence of the pulmonary artery (UAPA) refers to a rare
congenital malformation in which the main pulmonary artery is un‐
branched with either the left or the right pulmonary artery being ab‐
sent. The pulmonary blood supply of the affected side mainly depends
on the bronchial artery, ascending aorta and descending aorta, which
are often accompanied by other intracardiac malformations or can
be solitary.1 The reported prevalence of adult UAPA is 1/200 000. 2
Observation of the left and right pulmonary arteries under three‐ves‐
sel view is the key to diagnosing UAPA. Considering that infants born
with congenital UAPA may be asymptomatic in the early stages of
development,3 prenatal diagnosis is of vital importance.

Wang, Zhang, and Feng are co‐first authors.

Echocardiography. 2019;36:1787–1789. © 2019 Wiley Periodicals, Inc. |  1787

wileyonlinelibrary.com/journal/echo  
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1788       WANG et al.

F I G U R E 1   Prenatal echocardiographic diagnosis of the absence of the right pulmonary artery associated with mild coarctation of the
aorta. A, Three‐vessel view showed that the left pulmonary artery originated from the main pulmonary artery, and the right pulmonary
artery was not found. B, Prenatal ultrasound showed that the fetus had a left aortic arch. C, Left ventricular outflow tract view revealed a
collateral arterial vessel from the aorta and the formation of a major aortopulmonary collateral artery (MAPCA), with mild coarctation of
the aorta. D, CDFI showed the collateral artery entering the right lung. E, 3D HD‐flow showed the blood flow of MAPCA. F, The isthmus of
the aortic arch was narrow with an internal diameter of approximately 0.16 cm. AO = aorta; DAO = descending aorta; LPA = left pulmonary
artery; PA = pulmonary artery; SP = spine; SVC = superior vena cava; T = trachea

F I G U R E 2   Postpartum echocardiographic diagnosis of the absence of the right pulmonary artery with mild coarctation of the aorta.
A, The short‐axis view of the great artery showed that the left pulmonary artery originated from the main pulmonary artery, and the right
pulmonary artery was not found. B, No blood flow of the right pulmonary artery was detected in the CDFI. C,D, Mild coarctation of the
aorta with an internal diameter of approximately 0.5 cm, and the blood velocity is increased. There are continuous blood flow signals
scattered below the arch (white arrow). It is suspected that there is bronchial artery compensatory participation in the right lung blood
supply. E,F, The velocity of blood flow in the initial segment of the descending aorta increased, with a front velocity of 2.6 m/s and a
pressure difference of 27 mm Hg. After 1 mo, echocardiography was reexamined. The velocity in the initial segment of the descending aorta
decreased slightly, with a front velocity of 1.9 m/s and a pressure difference of 14 mm Hg. ARCH = aortic arch; LPA = left pulmonary artery;
MPA = main pulmonary artery
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F I G U R E 3   Computed tomography angiography (CTA) with 3D reconstruction confirmed the absence of the right pulmonary artery
associated with mild coarctation of the aorta. A, CTA showed that the width of the ascending aorta was approximately 7.3 mm. The
aortic arch narrowed slightly from the opening of the brachiocephalic trunk to the isthmus. The width of the aorta at the opening of the
brachiocephalic trunk, left common carotid artery and left subclavian artery was approximately 6.1, 4.1, and 3.9 mm, respectively. The width
of the descending aorta at the distal end of the stenosis was approximately 5.9 mm. B, CTA showed the right bronchial artery and MAPCA.
C, CT showed that the right collateral artery had entered the right lung. D,E, 3D reconstruction showed the right bronchial artery and
MAPCA. F, 3D reconstruction revealed coarctation of the aorta (yellow arrow)

C O N FL I C T O F I N T E R E S T S 2. Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied

manifestation of pulmonary artery agenesis in adulthood. Chest.
The authors declare no conflict of interests. 1995;108(3):670–676.
3. Seedat F, Kalla IS, Feldman C. Unilateral absent pulmonary artery in
an adult ‐ a diagnostic and therapeutic challenge. Respir Med Case Rep.
ORCID 2017;22:238–242.

Yu Wang  https://orcid.org/0000-0001-6282-9840

Jiaqi Zhang  https://orcid.org/0000-0003-2234-1730
REFERENCES
1. Li X, Mu Z, Li XU, Weng Z. Prenatal diagnosis of anomalous origin of
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How to cite this article: Wang Y, Zhang J, Feng W, Wu Y,
Yang L, Yin J. Absence of the fetal right pulmonary artery
complicated with coarctation of the aorta: Prenatal and
postnatal diagnosis. Echocardiography. 2019;36:1787–1789.
https​://doi.org/10.1111/echo.14470​