Case of the Week #600
(1) Australia, Melbourne, Monash Ultrasound For Women; (2) Pediatric & Fetal cardiologist
A 37-year-old G2P1 woman presents with no family history of structural congenital heart disease. Both pregnancies were in vitro fertilization, with this pregnancy a result of frozen embryo transfer. The first child had an epiglottic cyst and spent two weeks in the intensive care unit after birth. The following images were obtained at 12 weeks, 4 days. Noninvasive prenatal testing was low risk and nuchal translucency was 1.5 mm.
![](/site/cache/public/images/new-co/new-content-media-104/fieldList/file_path/95726__852__639__down__95.jpg)
![](/site/cache/public/images/new-co/new-content-media-105/fieldList/file_path/95727__872__655__down__95.jpg)
Repeat ultrasound exam was obtained approximately 1 week later.
View the Answer Hide the Answer
Answer
We present a case of D-transposition of the great arteries with ventricular septal defect diagnosed in early pregnancy.
Our prenatal images identified:
- The pulmonary artery with bifurcation into the left and right branches (Image 1)
- 3 vessel view demonstrating that the aorta arises from the right ventricle (Video 1,2)
- Balanced ventricles with unobstructed outflow tracts and aorta arising from the right ventricle. Normal caliber of the left-sided aortic arch (Video 3).
- Sagittal plane through aortic arch shows parallel course of the great vessels (Image 2, Video 6,7)
- High muscular perimembranous ventricular septal defect (video 4, 5)
- Normal ductus arteriosus with antegrade flow and confluent branch pulmonary arteries
- Mitral and tricuspid valvular function was normal with no atrioventricular valve regurgitation
- There was normal looking foramen ovale with right-to-left shunt
- There was normal systematic and pulmonary venous connections
- No markers of aneuploidy or extra-cardiac malformation was seen.
![Ao: Aorta; PA pulmonary artery](/site/cache/public/images/new-co/new-content-media-184/fieldList/file_path/94363__1074__644__down__95.jpg)
![Ao: Aorta](/site/cache/public/images/new-co/new-content-media-185/fieldList/file_path/94364__1440__864__down__95.jpg)
![LV: left ventricle; RV: right ventricle; VSD: ventricular septal defect](/site/cache/public/images/new-co/new-content-media-186/fieldList/file_path/94365__1438__862__down__95.jpg)
![Ao: Aorta](/site/cache/public/images/new-co/new-content-media-187/fieldList/file_path/94366__1371__823__down__95.jpg)
![Ao: Aorta](/site/cache/public/images/new-co/new-content-media-188/fieldList/file_path/94367__1349__810__down__95.jpg)
![Ao: Aorta; PA: Pulmonary artery; RV: Right ventricle](/site/cache/public/images/new-co/new-content-media-189/fieldList/file_path/94368__1433__860__down__95.jpg)
Discussion
Transposition of the great arteries is a common cardiac malformation with atrioventricular concordance (normal connection between atria and ventricles) and ventriculoarterial discordance (connection of the great vessels is switched). In our case, both great arteries display a parallel course, with the aorta anterior and to the right of the pulmonary artery (hence the term D-TGA “dexter”). D-transposition of the great arteries (D-TGA) is relatively frequent cardiac anomaly occurring in 5 to 7% of congenital cardiac malformations, with an incidence 0.315 cases per 1000 live births and 2:1 male preponderance.
Ventricular septal defect and pulmonary stenosis are common associations with D-TGA and may be present either alone or in combination in up to 30-40% cases. Associated extracardiac malformations as well as chromosomal abnormalities are rare with simple transposition of the great arteries.
On prenatal ultrasound, the four chamber view is typically normal, except for an associated ventricular septal defect. The aorta is noted to arise from the right ventricle in an anterior and parallel course to the pulmonary artery. Instead of a 3-vessel trachea view, a single large vessel (the transverse aortic arch) with a superior vena cava to its right is identified (I–sign). On longitudinal views, the aortic arch is seen arising from the anterior right ventricle, giving rise to head and neck vessels and assuming a “hockey stick” orientation as it curves posteriorly. The pulmonary artery assumes the “candy cane” orientation. Before inclusion of the outflow tracts to international fetal cardiac screening guidelines, the detection rates reported only 3 to 17 % of simple transposition of the great arteries. In one series of more than 3000 fetuses undergoing detailed screening for anomalies at 11 to 14 weeks, D-TGA is rarely diagnosed early in gestation [2]. The 3-vessel trachea view with a single great vessel may be helpful in early gestation. Additionally, an enlarged nuchal translucency in the setting of normal fetal chromosomes as well as an abnormal cardiac axis can be associated with conotruncal anomalies, including TGA.
Usually, babies with transposition of the great arteries with ventricular septal defect need to be to started on prostaglandin E1 infusion to maintain patency of the ductus arteriosus after birth. Some babies may require urgent balloon atrial septostomy to enlarge the foramen ovale if postnatal mixing is inadequate. Delivery at a tertiary center where neonatal intensive care facilities are available and capable of performing urgent septostomy is preferred. Surgical repair with an arterial switch and coronary translocation is normally performed at 1 week of age. If the ventricular septal defect allows good mixing within the heart, surgical correction can sometimes be deferred for 4-6 weeks, allowing babies to establish normal feeding and growth. The reported surgical mortality is approximately 1%. There is a higher incidence of learning difficulties and behavioral problems in children with transposition of the great vessels. The risks are greatest for babies born prematurely or with other associated extracardiac abnormalities. In the long term, a small number of patients require further surgery for the branch pulmonary arteries, which are placed over the top of the aorta as a part of surgery and are sometimes narrowed. Adult patients may require valvular repair or replacement due to neoaortic valve regurgitation as the original pulmonary root dilatates over many years.
Transposition of the great arteries can be a difficult diagnosis, but early identification allows more time for patient counselling, as well as planning pregnancy and delivery options.
References
1. Abuhamad A, Chaoui R. A practical guide to fetal echocardiography: normal and abnormal hearts, 3rd edition. Lippincott Williams & Wilkins, 2015. p447-466.
2. Becker R, Wegner RD. Detailed screening for fetal anomalies and cardiac defects at the 11–13-week scan. Ultrasound Obstet Gynecol. 2006 Jun;27(6):613-8.
Discussion Board
Winners
![Guest Profile Pic](/images/web/profile-default.png?v=2)
Guest United States
![Dr. Hitanshu Bhatt Profile Pic](/site/cache/public/images/dr.-hi/dr.-hitanshu-bhatt/fieldList/profile_image/99499__400__400__fillAndCrop__95.jpg)
Dr. Hitanshu Bhatt India Physician
![Dianna Heidinger Profile Pic](/site/cache/public/images/dianna/dianna-heidinger/fieldList/profile_image/100646__400__400__fillAndCrop__95.jpg)
Dianna Heidinger United States Sonographer
![Yulia Sologub Profile Pic](/images/web/profile-default.png?v=2)
Yulia Sologub Russian Federation Physician
![Javier Cortejoso Profile Pic](/site/cache/public/images/javier/javier-cortejoso/fieldList/profile_image/100172__400__400__fillAndCrop__95.jpg)
Javier Cortejoso Spain Physician
![Pawel Swietlicki Profile Pic](/images/web/profile-default.png?v=2)
Pawel Swietlicki Poland Physician
![Umber Agarwal Profile Pic](/images/web/profile-default.png?v=2)
Umber Agarwal United Kingdom Maternal Fetal Medicine
![Igor Yarchuk Profile Pic](/site/cache/public/images/igor-y/igor-yarchuk/fieldList/profile_image/99880__400__400__fillAndCrop__95.jpg)
Igor Yarchuk United States Sonographer
![Larysa Gazarova Profile Pic](/images/web/profile-default.png?v=2)
Larysa Gazarova United States Physician
![belen garrido Profile Pic](/images/web/profile-default.png?v=2)
belen garrido Spain Physician
![Andrii Averianov Profile Pic](/site/cache/public/images/andrii/andrii-averianov/fieldList/profile_image/100662__400__400__fillAndCrop__95.jpg)
Andrii Averianov Ukraine Physician
![Ana Ferrero Profile Pic](/site/cache/public/images/ana-fe/ana-ferrero/fieldList/profile_image/100174__400__400__fillAndCrop__95.jpg)
Ana Ferrero Spain Physician
![Alexandr Krasnov Profile Pic](/site/cache/public/images/aexand/aexandr-krasnov/fieldList/profile_image/100171__400__400__fillAndCrop__95.jpg)
Alexandr Krasnov Ukraine Physician
Mayank Chowdhury India Physician
![Vladimir Lemaire Profile Pic](/site/cache/public/images/vladim/vladimir-lemaire/fieldList/profile_image/100663__400__400__fillAndCrop__95.jpeg)
Vladimir Lemaire United States Physician
![Shilpen Gondalia Profile Pic](/site/cache/public/images/shilpe/shilpen-gondalia/fieldList/profile_image/100568__400__400__fillAndCrop__95.jpg)
Shilpen Gondalia India Physician
![Boujemaa Oueslati Profile Pic](/site/cache/public/images/boujem/boujemaa-oueslati/fieldList/profile_image/100410__400__400__fillAndCrop__95.jpg)
Boujemaa Oueslati Tunisia Physician
![Tatiana Koipish Profile Pic](/site/cache/public/images/tatyan/tatyana-koipish/fieldList/profile_image/99662__400__400__fillAndCrop__95.png)
Tatiana Koipish Belarus Physician
![CHARLES SARGOUNAME Profile Pic](/site/cache/public/images/charle/charles-sargouname/fieldList/profile_image/100471__400__400__fillAndCrop__95.jpg)
CHARLES SARGOUNAME India Physician
![Aysegul Ozel Profile Pic](/site/cache/public/images/aysegu/aysegul-ozel/fieldList/profile_image/100479__400__400__fillAndCrop__95.png)
Aysegul Ozel Turkey Physician
![Philippe Deblieck Profile Pic](/site/cache/public/images/philip/philippe-deblieck/fieldList/profile_image/96473__400__400__fillAndCrop__95.jpg)
Philippe Deblieck Germany Physician
![Kimberly Delaney Profile Pic](/site/cache/public/images/kimber/kimberly-delaney/fieldList/profile_image/100356__400__400__fillAndCrop__95.png)
Kimberly Delaney United States Sonographer
![Marianovella Narcisi Profile Pic](/site/cache/public/images/marian/marianovella-narcisi/fieldList/profile_image/99409__400__400__fillAndCrop__95.jpg)
Marianovella Narcisi Italy Physician
![Javier Ayala Profile Pic](/images/web/profile-default.png?v=2)
Javier Ayala Spain Physician
![Rebecca Evans Profile Pic](/site/cache/public/images/rebecc/rebecca-evans/fieldList/profile_image/97791__400__400__fillAndCrop__95.jpg)
Rebecca Evans Australia Sonographer
![Victoria Giang Profile Pic](/images/web/profile-default.png?v=2)
Victoria Giang Viet Nam Physician
![Muradiye Yıldırım Profile Pic](/site/cache/public/images/muradi/muradiye-yıldırım/fieldList/profile_image/99663__400__400__fillAndCrop__95.jpeg)
Muradiye Yıldırım Turkey Physician
![Ta Son Vo Profile Pic](/site/cache/public/images/ta-son/ta-son-vo/fieldList/profile_image/97539__400__400__fillAndCrop__95.jpg)
Ta Son Vo Viet Nam Physician
![PATRICIA MUÑOZ Profile Pic](/images/web/profile-default.png?v=2)
PATRICIA MUÑOZ United States Physician
![ALBANA CEREKJA Profile Pic](/site/cache/public/images/albana/albana-cerekja/fieldList/profile_image/100199__400__400__fillAndCrop__95.png)
ALBANA CEREKJA Italy Physician
![Akmaral Medetbekova Profile Pic](/site/cache/public/images/akmara/akmaral-medetbekova/fieldList/profile_image/98060__400__400__fillAndCrop__95.jpeg)
Akmaral Medetbekova Kazakhstan Physician
![Eti Zetounie Profile Pic](/images/web/profile-default.png?v=2)
Eti Zetounie Israel Sonographer
![Murat Cagan Profile Pic](/site/cache/public/images/murat-/murat-cagan/fieldList/profile_image/100194__400__400__fillAndCrop__95.jpg)
Murat Cagan Turkey Physician
![Sonio Sonio Profile Pic](/site/cache/public/images/sonio-/sonio-sonio/fieldList/profile_image/99470__400__400__fillAndCrop__95.jpg)
Sonio Sonio France AI
![Charlotte Conturie Profile Pic](/site/cache/public/images/charlo/charlotte-conturie/fieldList/profile_image/96155__400__400__fillAndCrop__95.jpg)
Charlotte Conturie United States Physician
![gholamreza azizi Profile Pic](/images/web/profile-default.png?v=2)
gholamreza azizi Iran, Islamic Republic of Physician
![Ionut Valcea Profile Pic](/site/cache/public/images/ionut-/ionut-valcea/fieldList/profile_image/100524__400__400__fillAndCrop__95.jpeg)
Ionut Valcea Romania Physician
![aastha mehra Profile Pic](/images/web/profile-default.png?v=2)
aastha mehra India Physician
![Viralkumar Madhu Profile Pic](/images/web/profile-default.png?v=2)
Viralkumar Madhu India Sonographer
![Martina Vagaská Profile Pic](/site/cache/public/images/martin/martina-vagaská/fieldList/profile_image/100044__400__400__fillAndCrop__95.jpg)
Martina Vagaská Slovakia Physician
![Anette Beverdam Profile Pic](/images/web/profile-default.png?v=2)
Anette Beverdam Netherlands Sonographer
![Grzegorz Rak Profile Pic](/images/web/profile-default.png?v=2)
Grzegorz Rak Poland Physician
![Annette Reuss Profile Pic](/images/web/profile-default.png?v=2)
Annette Reuss Germany Physician
![Jana Furlan Profile Pic](/images/web/profile-default.png?v=2)
Jana Furlan New Zealand Sonographer
![shruti Agarwal Profile Pic](/site/cache/public/images/shruti/shruti-agarwal/fieldList/profile_image/100406__400__400__fillAndCrop__95.jpg)
shruti Agarwal India Physician
![Mai Phương Profile Pic](/site/cache/public/images/mai-ph/mai-phương/fieldList/profile_image/98187__400__400__fillAndCrop__95.jpeg)
Mai Phương Viet Nam Physician
![shay kevorkian Profile Pic](/images/web/profile-default.png?v=2)
shay kevorkian Israel Physician
![SAVITA SHIRODKAR Profile Pic](/images/web/profile-default.png?v=2)
SAVITA SHIRODKAR India Physician
![Nawar Hatoum Profile Pic](/images/web/profile-default.png?v=2)
Nawar Hatoum United States Physician
![Andrea Stoop-Berends Profile Pic](/site/cache/public/images/andrea/andrea-stoop-berends/fieldList/profile_image/97888__400__400__fillAndCrop__95.jpeg)
Andrea Stoop-Berends Netherlands Sonographer
![Ismail Guzelmansur Profile Pic](/site/cache/public/images/ismail/ismail-guzelmansur/fieldList/profile_image/100201__400__400__fillAndCrop__95.jpg)
Ismail Guzelmansur Turkey Physician
![Fatemeh Shakki Katouli Profile Pic](/images/web/profile-default.png?v=2)
Fatemeh Shakki Katouli Iran, Islamic Republic of Physician
![Luciana Gimenez Profile Pic](/images/web/profile-default.png?v=2)
Luciana Gimenez Spain Physician
![Amy Scheible Profile Pic](/site/cache/public/images/amy-sc/amy-scheible/fieldList/profile_image/99095__400__400__fillAndCrop__95.jpg)
Amy Scheible United States Sonographer
![Flora Mirzoeva Profile Pic](/images/web/profile-default.png?v=2)
Flora Mirzoeva United States Sonographer
![Rupal Sasani Profile Pic](/images/web/profile-default.png?v=2)
Rupal Sasani India Physician
![Lashell Jones Profile Pic](/images/web/profile-default.png?v=2)
Lashell Jones United States Sonographer
![SHILPA KISHORE Profile Pic](/images/web/profile-default.png?v=2)
SHILPA KISHORE India Physician
![Eylem Eşsizoğlu Profile Pic](/site/cache/public/images/eylem-/eylem-eşsizoğlu/fieldList/profile_image/100378__400__400__fillAndCrop__95.jpg)
Eylem Eşsizoğlu Turkey Physician
![Süreyya Sarıdaş Demir Profile Pic](/images/web/profile-default.png?v=2)
Süreyya Sarıdaş Demir Turkey Physician
![Petra Barboríková Profile Pic](/site/cache/public/images/petra-/petra-barboríková/fieldList/profile_image/100362__400__400__fillAndCrop__95.jpg)
Petra Barboríková Slovakia Physician
![Carmen de Luis Rodríguez Profile Pic](/site/cache/public/images/carmen/carmen-de-luis-rodríguez/fieldList/profile_image/99187__400__400__fillAndCrop__95.jpeg)
Carmen de Luis Rodríguez Spain Physician
![Rajnikant Vasava Profile Pic](/images/web/profile-default.png?v=2)
Rajnikant Vasava India Physician
![Denys Saitarly Profile Pic](/site/cache/public/images/denys-/denys-saitarly/fieldList/profile_image/100661__400__400__fillAndCrop__95.jpg)
Denys Saitarly Israel Physician
![Le Tien Dung Profile Pic](/site/cache/public/images/dung-l/dung-le/fieldList/profile_image/100360__400__400__fillAndCrop__95.jpg)
Le Tien Dung Viet Nam Physician
![Tetiana Ishchenko Profile Pic](/site/cache/public/images/tetian/tetiana-ishchenko/fieldList/profile_image/100620__400__400__fillAndCrop__95.jpeg)
Tetiana Ishchenko Ukraine Physician
![Juliana Moren Profile Pic](/images/web/profile-default.png?v=2)
Juliana Moren Argentina Physician
![Jose Pablo Ling Garcia Profile Pic](/images/web/profile-default.png?v=2)
Jose Pablo Ling Garcia Mexico Physician
![Vipalee Trivedi Profile Pic](/images/web/profile-default.png?v=2)
Vipalee Trivedi India Consult gynecologist
![Gaurav Sharma Profile Pic](/site/cache/public/images/gaurav/gaurav-sharma/fieldList/profile_image/99518__400__400__fillAndCrop__95.jpg)
Gaurav Sharma India Physician
![María Victoria Peral Parrado Profile Pic](/images/web/profile-default.png?v=2)
María Victoria Peral Parrado Spain Physician
![Eduardo Namura Di Thomaz Profile Pic](/images/web/profile-default.png?v=2)
Eduardo Namura Di Thomaz Brazil Physician
![Le Duc Profile Pic](/site/cache/public/images/dinh-d/dinh-duc/fieldList/profile_image/99780__400__400__fillAndCrop__95.jpeg)
Le Duc Viet Nam Physician
![Huỳnh Trí Trương Profile Pic](/images/web/profile-default.png?v=2)
Huỳnh Trí Trương Viet Nam Physician
![Paul Timmons Profile Pic](/images/web/profile-default.png?v=2)
Paul Timmons United Kingdom Physician
![Erika Zanzarelli Profile Pic](/site/cache/public/images/erika-/erika-zanzarelli/fieldList/profile_image/99684__400__400__fillAndCrop__95.jpg)
Erika Zanzarelli Italy Physician