eISSN: 2449-6731
ISSN: 2449-6723
Prenatal Cardiology
Current issue Archive About the journal Editorial board Abstracting and indexing Contact Instructions for authors Ethical standards and procedures
Case report

Prenatal diagnosis of criss-cross heart with congenitally corrected transposition of the great arteries – detection and fetal echocardiography monitoring with one-year postnatal follow-up

Julia Murlewska
Joanna Kućmierz
Sławomir Witkowski
1, 2
Agnieszka Żalińska
Piotr Grzelak
Maria Respondek-Liberska
1, 4

Department of Prenatal Cardiology, Polish Mother’s Memorial Hospital, Research Institute in Lodz, Poland
Medical Faculty, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Poland
Department of Radiology, Polish Mother’s Memorial Hospital, Research Institute in Lodz, Poland
Department of Diagnoses and Prevention of Fetal Malformations, Medical University of Lodz, Poland
Prenat Cardio 2022
Online publish date: 2022/12/30
Article file
- Prenatal diagnosis.pdf  [0.41 MB]
Get citation
JabRef, Mendeley
Papers, Reference Manager, RefWorks, Zotero
PlumX metrics:

Case report

This was a first pregnancy of a healthy woman. The fetus had routine ultrasound examinations performed at the 6th, 12th, and 20th weeks of gestation, which were determined as ‘normal’. However, at the 27th week of gestation a ventricular septal defect was detected, which resulted in a referral to our centre for targeted echocardiography.
At the 37th week of gestation, the heart area/cardio-thoracic area (HA/CA) ratio was normal. Moreover, levocardia and situs solitus were observed both on the abdominal and atrial level. Both atria were found on their correct sides; however, it was impossible to visualize a tricuspid and mitral valve opening in the same plane. Two ventricles were present, but the morphologically left ventricle was found on the right side, and the hypoplastic morphological right ventricle was located on the left side.
Sequential analysis off-line in slow motion confirmed that blood flows were not parallel within the fetal heart – the blood from the right atrium was directed to the left ventricle, whereas the blood from the left atria was directed to the right ventricle. The position of the hypoplastic right ventricle was superior – above to the left ventricle, which was inferior, and this was clearly confirmed by postnatal computed tomography (CT) angiography (Figure 1). So, there was a superior-inferior relationship between the ventricles. The aorta emerged from the morphological hypoplastic right ventricle and was parallel to the wide pulmonary trunk, which means that there was ventriculo-arterial discordance. The 2 arterial vessels in superior mediastinum had normal blood flow according to Doppler measurements. During subsequent four echo-sonographic exams (Table 1), there were normal umbilical Doppler blood flow parameters and normal heart rate up to the 38th week of gestation. Three weeks before delivery, the size of the pulmonary trunk was 13 mm (Z score +2.47) [1]. The development of the aorta stopped at the same time, and its maximum size was 7 mm (Z score –0.43). The aorta/main pulmonary artery (AO/MPA) ratio was 0.54, which suggested coarctation of the aorta (CoA). There was normal right-left blood flow through the foramen ovale, and the size of the ventricular septal defect (VSD) did not increase. In each fetal exam, the cardio-vascular profile score (CVPS) was 10, so a vaginal delivery was planned in our tertiary centre (prenatal cardiology, obstetrics, paediatric cardiology, and cardiac surgery departments). At the 40th week of gestation a spontaneous delivery occurred, and a male baby was born with a weight of 3000 g and an Apgar score of 10. The Prostin infusion was initiated just after birth to maintain the arterial duct patency. Postnatal echocardiography, chest X-rays, and volume-rendered CT angiography (Figures 1-4) confirmed the prenatal findings and also made it possible to confirm aortic coarctation type A. The aortic arch reconstruction surgery with pulmonary trunk banding was performed on the 8th day of postnatal life, and the child was asymptomatic during his first year of life (our postnatal follow-up).


Criss-cross heart (CCH) combined with corrected transposition of the great arteries (cc-TGA) is a cardiac malformation that is very difficult to diagnose both prenatally and postnatally because it is a rare and complex cardiac malformation. In the past, such a malformation was described only in autopsy examinations [2] or was detected by accident in adults [3-5]. In the years 2008-2020 progress in ultrasound technology and experience in fetal echocardiography enabled the diagnosis of CCH or cc-TGA prenatally [6-8]. However, the combination of criss-cross heart and corrected TGA was found only in individual cases (Table 2) [9, 10].
What is unique in our report is not only the diagnosis of abnormal heart anatomy, but also fetal heart monitoring by echocardiography. Besides the diagnosis, fetal echocardiography enabled safe monitoring and preparation of the pregnant woman and medical staff for vaginal delivery at term in the tertiary centre for obstetrics and cardiology. Further cardiological work-up is planned for our patient, but his medical history as well as other cases presented in the literature (Table 2) confirm that despite an abnormal fetal heart looping in early embryogenesis, it does not cause congestive heart failure both prenatally and postnatally and is not related to an abnormal karyotype. These data have crucial value for prenatal consultation purposes for parents-to-be as well as obstetricians, perinatologists, paediatric cardiologists, and adult cardiologists.


The cardiac surgery was performed by Prof. Jacek Moll and his team from the Paediatric Cardiac Surgery Department of the Polish Mother’s Memorial Hospital Research Institute. We also would like to thank our colleges from the Obstetrics Department, Neonatology Department, Paediatric Cardiology Department, and Intensive Care Department for their cooperation and appreciation of our input in their daily work.

Conflict of interest

The authors declare no conflict of interest.


1. Schneider C, McCrindle BW, Carvalho JS, et al. Development of Z‐scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 2005; 26: 599-605.
2. Symons JC, Shinebourne EA, Joseph MC, et al. Criss-cross heart with congenitally corrected transposition: report of a case with d-transposed aorta and ventricular preexcitation. Eur J Cardiol 1977; 5: 493-505.
3. Morelli PJ, Kimball TR, Witt SA, et al. Echocardiographic considerations in demonstrating complex anatomy of criss-cross atrioventricular valves and discordant atrioventricular and ventriculo-arterial relations. J Am Soc Echocardiogr 1996; 9: 727-729.
4. Martínez Quintana E, Rodríguez González F, Agredo Muñoz J. Criss cross heart in a congenitally corrected transposition of the great arteries. Int J Cardiol 2008; 130: e81-e82.
5. Zeigler S, Mainwaaring RD, Punn R, et al. Double-switch repair of corrected transposition in association with criss-cross atrial morphology. Ann Thorac Surg 2016; 101: 1208-1210.
6. Ngeh NN, Api O, Iasci A, et al. Criss-cross heart: report of three cases with double inlet ventricles diagnosed in utero. Ultrasound Obstet Gynecol 2008; 31: 461-465.
7. Ayabakan C, Binnetoğlu K, Sarısoy Ö, et al. Crisscross heart diagnosed in utero. Congenit Heart Dis 2013; 8: E153-E156.
8. Li S, Luo G, Norwitz ER, et al. Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases. J Perinatol 2013; 33: 98-102.
9. Ravi P, Fruitman D, Mills L, et al. Prenatal diagnosis of the criss-cross heart. Am J Cardiol 2017; 119: 916-922.
10. Vorisek CN, Kurkevych A, Kuhn V, et al. Prenatal diagnosis and postnatal outcome of eight cases with criss-cross heart – a multicenter case series. Ultraschall Med Dec 2022; 43: e90-e97.
This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0). License (http://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Quick links
© 2023 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.