Right-Sided
Aortic Arch, Kommerell
Diverticulum withAberrant Left Subclavian
Artery: Diagnosed byPrenatal Ultrasound andPostnatalCardiovascular
Casting
Jiaqi Zhang, MD* , Yu
Wang,
MD*, ,
Wei Feng, MD* , Ziyi Si, MD, Jinzhi Xu, MD, He Zeng, MD, Qiaoyue
He, MD, Ya Liu, MD
Department of Ultrasound, Xiangyang No.1 People’s Hospital, Hubei
University of Medicine
* Drs Zhang, Wang and Feng contributed equally to this work
Correspondence to: Dr Yu Wang, Department of Ultrasound,
Xiangyang No.1 People’s Hospital, Hubei University of Medicine, No 15,
Jiefang Avenue, Xiangyang, Hubei, 441000, China, e-mail:
287383672@qq.com.
CONFLICT OF INTERESTS: The authors declare no conflict of
interests.
KEY WORDS: Kommerell diverticulum, aberrant left subclavian
artery, fetal echocardiography, cardiovascular casting
Funding information :
This work was supported by Science and Technology Research Project of
Education Department of Hubei Province (B2019112).
Abstract : Kommerell diverticulum (KD) is a rare cardiovascular
malformation, which is usually diagnosed after birth. To our knowledge,
there is no report of prenatal diagnosis of KD so far. Here we present a
case of RAA, KD with ALSA prenatally. We confirmed the prenatal
diagnosis with a new modified cardiovascular casting technique, which
perfectly demonstrated the cardiovascular malformations of the fetus.
Our case demonstrates that the modified cardiovascular casting technique
can distinctly delineated correlations and spatial structure of heart
and cardiac vessels, and this new approach of casting combined with
clinical diagnosis may have a role in postnatal diagnosis and clinical
teaching.
.
A 31-year- old primigravida was referred to our maternal–fetal medical
center at a
gestational week
of 23+3 weeks for suspected heart
defects. The cell-free fetal DNA
test suggested a low risk of aneuploidy.
Prenatal echocardiography revealed
RAA with right descending aorta (RDA), left posterior ductus arteriosus
(LPDA), forming a “U-shaped” vascular ring. ALSA originating from
Kommerell diverticulum (KD) was also seen
(Figure 1, A, B). 3D HD-Flow and
glass body confirmed the “U-shaped” vascular ring and enlarged KD
(Figure 1, C, D). Left ventricular outflow tract section showed a VSD
with the aortic riding across ventricular septum
(Figure 2).
The fetus died in the uterus due
to umbilical cord knot at the gestational age of 26 +4 weeks. The
induction gave a male fetus with a weight of 724 g. The family voluntary
donation of specimen to our maternal and fetal medical center and a
written informed consent for this study was provided. The fetal specimen
was later made into a cardiovascular cast by following five specific
steps. First, the abdominal wall was incised. The umbilical vein was
separated and intubated with a small tube. The umbilical arteries were
separated with the left side cut off. Heparin (5–10 mL) and acetone
(30–60 mL) were injected successively via the umbilical vein to
flush out residual blood clots in the cardiovascular system. Second, the
specimen was perfused with butadiene-styrene plastic (ABS) gel (30–60
mL) at appropriate pressure and constant speed for approximately 10 h.
Third, after 24 hours, the ABS gel-filled specimen was soaked in 30%
hydrochloric acid. Fourth, after 2 weeks, the specimen was removed
carefully from hydrochloric acid and rinsed thoroughly until there was
no residual tissue adhesion on the cardiovascular cast. Finally, the
fetal cardiovascular cast was marked, displayed and assessed. The
subsequent anatomy and
cardiovascular cast confirmed the prenatal diagnosis (Figure 3, Figure
4).
Kommerell diverticulum is a rare
vascular malformation characterized by bulbous configuration of the
origin of an aberrant left subclavian artery in the setting of a RAA. It
is usually detected by accident in asymptomatic patient and rarely
diagnosed prenatally. The reported prevalence of KD is 0.05% of the
population.1 KD with right aortic arch is a rare
congenital anatomical anomaly whose clinical course has not been fully
determined. Complications may occur depending on the anatomical
distribution of the local structures, the size and extent of the
aneurysm. Greater fragility this vascular alteration and more extensive
lesions are associated with occurrence with more adverse consequences
such as dissections, thromboses and ruptures.2Echocardiography is the preferred method for diagnosing KD, it can be
used not only as an imaging method for diagnosis, but also as an imaging
method for outpatient follow-up. Moreover, it is safe, convenient and
inexpensive.3 Cast models can visually display the
origin, course, dimension and relative spatial position of the great
vessels, and may become an important supplement to prenatal
echocardiography.4 In our case, echocardiography and
cardiovascular casting have a complementary relationship and provide a
comprehensively evaluation of inside and outside of heart, which is of
great importance in diagnosing and teaching of complex fetal cardiac
malformations.