Abstract
Objective. To evaluate the accuracy of prenatal echocardiography in the differential diagnosis between common arterial trunk (CAT) and pulmonary atresia with ventricular septal defect (PA VSD) and to track the combination of these defects with chromosomal and extracardiac pathology.
Material and methods. We conducted a retrospective analysis of 88 fetuses with a single vessel that “rests” above the VSD and a poorly visualized right ventricular outflow tract without obvious direct flow. The database also revealed information on the type of blemish, the duration of pregnancy, the peculiarities of the structure of the trunk valve, the presence of other vascular, chromosomal and extracardiac anomalies, postnatal outcome. In difficult for visualization cases, four-dimensional (4D) echocardiography was used in the program STIC (spatiotemporal image correlation).
Results. The overall diagnostic accuracy was 84.1%. Chromosomal and extracardiac anomalies were detected in 15.9% of cases. In the PA VSD group, the open arterial duct and major aortopulmonary collateral arteries (MAPCAs) were visualized in 86.3 and 33.3% of cases, respectively. MAPCAs was significantly more common with the right aortic arch, in 58.3% of cases.
Conclusions. Prenatal differentiation of CAT and PA VSD can now be achieved in the main cases. If there is a suspicion of PA VSD, MAPCAs should be looked for, since they are, according to the world literature, associated with a higher risk of microdeletion 22q11.2 and complicate postpartum treatment.
References
1. Volpe P., Paladini D., Marasini M., Buonadonna A.L.,
Russo M.G., Caruso G. et al. Common arterial trunk in the
fetus: characteristics, associations, and outcome in a multicentre
series of 23 cases. Heart. 2003; 89: 1437–41.
2. Vesel S., Rollings S., Jones A., Callaghan N., Simpson J.,
Sharland G.K. Prenatally diagnosed pulmonary atresia with
ventricular septal defect: echocardiography, genetics, associated
anomalies and outcome. Heart. 2006; 92: 1501–5.
3. Galindo A., Mendoza A., Arbues J., Graсeras A., Escribano D.,
Nieto O.Conotruncal anomalies in fetal life: accuracy of diagnosis,
associated defects and outcome. Eur. J. Obstet.
Gynecol. Reprod. Biol. 2009; 146: 55–60.
4. Гасанова Р.М., Беспалова Е.Д., Сыпченко Е.В., Тюмене-
ва А.И., Бартагова М.Н. Пренатальная диагностика общего
артериального ствола при помощи трехмерной эхокардио-
графии. Вопросы практической педиатрии. 2014; 9 (1):
29–34.
5. Беспалова Е.Д., Суратова О.Г., Бокерия Е.Л., Бартаго-
ва М.Н., Гасанова Р.М., Тюменева А.И. Диагностика и лечение
кардиальной патологии у плода. М.: НЦССХ им. А.Н. Бакуле-
ва РАМН; 2015.
6. Khoshnood B., De Vigan C., Vodovar V., Goujard J.,
Lhomme A., Bonnet D. et al. Trends in prenatal diagnosis,
pregnancy termination, and perinatal mortality of newborns
with congenital heart disease in France, 1983–2000: a population-
based evaluation. Pediatrics. 2005; 115: 95–101.
7. Robinson H.P., Fleming J.E. A critical evaluation of sonar
'crown-rump length' measurements. Br. J. Obstet. Gynecol.
1975; 82: 702–10.
8. Mul T., Mongelli M., Gardosi J. A comparative analysis of second-
trimester ultrasound dating formulae in pregnancies conceived
with artificial reproductive techniques. Ultrasound
Obstet. Gynecol. 1996; 8: 397–402.
9. Yagel S., Cohen S.M., Achiron R. Examination of the fetal heart
by five short axis views: a proposed screening method for
comprehensive cardiac evaluation. Ultrasound Obstet.
Gynecol. 2001; 17: 367–9.
10. Allan L. Technique of fetal echocardiography. Pediatr. Cardiol.
2004; 25: 223–33.
11. International Society of Ultrasound in Obstetrics and
Gynecology. Cardiac screening examination of the fetus:
guidelines for performing the “basic” and “extended basic”
cardiac scan. Ultrasound Obstet. Gynecol. 2006; 27: 107–13.
12. Bennasar M., Martínez J.M., Gуmez O., Bartrons J.,
Olivella A., Puerto B. et al. Accuracy of four-dimensional spatiotemporal
image correlation echocardiography in the prenatal
diagnosis of congenital heart defects. Ultrasound Obstet.
Gynecol. 2010; 36: 458–64.
13. Van Praagh R. Truncusarteriosus: what is it really and how
should it be classified? Eur. J. Cardiothorac. Surg. 1987; 1:
65–70.
14. Sivanandam S., Glickstein J.S., Printz B.F., Allan L.D.,
Altmann K., Solowiejczyk D.E. et al. Prenatal diagnosis of
conotruncal malformations: diagnostic accuracy, outcome,
chromosomal abnormalities and extracardiac anomalies.
Am. J. Perinatol. 2006; 23: 241–5.
15. Duke C., Sharland G.K., Jones A.M., Simpson J.M.
Echocardiographic features and outcome of truncus arteriosus
diagnosed during fetal life. Am. J. Cardiol. 2001; 88:
1379–84.
16. Гасанова Р.М., Беспалова Е.Д., Сыпченко Е.В., Тюмене-
ва А.И., Бартагова М.Н. Применение новых режимов четы-
рехмерной эхокардиографии в диагностике атрезии легоч-
ной артерии с дефектом межжелудочковой перегородки
у плода. Вопросы практической педиатрии. 2014; 9 (4):
20–4.
17. Seale A.N., Ho S.Y., Shinebourne E.A., Carvalho J.S. Prenatal
identification of the pulmonary arterial supply in tetralogy of
Fallot with pulmonary atresia. Cardiol. Young. 2009; 19:
185–91.
18. Song S.W., Park H.K., Park Y.H., Cho B.K. Pulmonary atresia
with ventricular septal defects and major aortopulmonary collateral
arteries. 18-year clinical experience and angiographic
follow-up of major aortopulmonary collateral arteries. Circ. J.
2009; 73: 516–22.
19. Ким А.И., Хамитов Р.Р., Рогова Т.В., Григорьянц Т.Р. Этапная
хирургическая коррекция атрезии легочной артерии с де-
фектом межжелудочковой перегородки у детей первого го-
да жизни. Детские болезни сердца и сосудов. 2013; 4:
45–50.
20. Fouilloux V., Bonello B., Kammache I., Fraisse A., Macе L.,
Kreitmann B. Management of patients with pulmonary atresia,
ventricular septal defect, hypoplastic pulmonary arteries and
major aortopulmonary collaterals: focus on the strategy of
rehabilitation of native pulmonary arteries. Arch. Cardiovasc.
Dis. 2012; 105: 666–75.
21. Watanabe N., Mainwaring R.D., Reddy V.N., Palmon M.,
Hanley F.L. Early complete repair of pulmonary atresia with
ventricular septal defect and major aortopulmonary collaterals.
Ann. Thorac. Surg. 2014; 97: 909–15, discussion 914–5.
22. Чвамания А.А., Свободов А.А., Туманян М.Р., Докторо-
ва В.П., Матаева Т.В. Результаты унифокализации БАЛКА у
детей первого года жизни с атрезией легочной артерии в со-
четании с дефектом межжелудочковой перегородки. Бюл-
летень НЦССХ им. А.Н. Бакулева РАМН. 2017; 18 (S3): 7.
23. Chessa M., Butera G., Bonhoeffer P., Iserin L., Kachaner J.,
Lyonnet S. et al. Relation of genotype 22q11 deletion to phenotype
of pulmonary vessels in tetralogy of Fallot and pulmonary
atresia-ventricular septal defect. Heart. 1998; 79: 186–90.
24. Gotsch F., Romero R., Espinoza J., Kusanovic J.P., Erez O.,
Hassan S. et al. Prenatal diagnosis of truncus arteriosus using
multiplanar display in 4D ultrasonography. J. Matern. Fetal.
Neonatal. Med. 2010; 23: 297–307.
About the authors
- Gasanova Rena Mamedovna, Cand. Med. Sc., Researcher, Cardiologist, Ultrasonic Diagnostics Physician, orcid.org/0000-0003-3318-1074;
- Bespalova Elena Dmitrievna, Dr Med. Sc., Professor, Head of Department, orcid.org/0000-0002-6360-4245;
- Bockeria Ekaterina Leonidovna, Dr Med. Sc., Head of Department;
- Sypchenko Elena Vyacheslavovna, Cand. Med. Sc., Ultrasonic Diagnostics Physician, orcid.org/0000-0002-8809-7913;
- Tyumeneva Adel' Il'darovna, Cand. Med. Sc., Researcher, Cardiologist, Ultrasonic Diagnostics Physician, orcid.org/0000-0001-8044-3669;
- Bartagova Mariya Nikolaevna, Cand. Med. Sc., Ultrasonic Diagnostics Physician, orcid.org/0000-0002-6425-4048;
- Aleksandrova Svetlana Aleksandrovna, Cand. Med. Sc., Senior Researcher