Predictors of Inadequate Mixing in Transposition of the Great Arteries — a Critical Neonatal Condition

Muntean, Iolanda; Toma, Daniela; Togănel, Rodica

doi:10.1515/jce-2017-0024

Abstract

Background: Simple transposition of the great arteries (TGA) is a cyanotic critical congenital heart defect representing a neonatal cardiovascular emergency, which requires surgery early after birth. In case of inadequate mixing between systemic and pulmonary circulations, creating an unrestrictive interatrial communication is mandatory. The aim of the present study was to identify the most important echocardiographic parameters that can predict the need for balloon atrial septostomy (BSA) in TGA neonates.

Material and methods: We retrospectively reviewed the echocardiographic recordings of newborns with TGA referred to our emergency room during a 6-year period. We measured the following echocardiographic parameters: atrial septal defect (ASD) size, interatrial septum (IAS) length, peak/mean inter-atrial pressure gradient, transverse diameter of the left atrium (LA), and transverse diameter of the right atrium (RA). Also, the ratio between ASD/IAS, the ratio between LA/RA, the ratio between mitral/tricuspid annulus, the ASD peak gradient/ASD diameter and the ASD diameter/(LA:RA ratio) were calculated.

Results: There were 37 neonates with simple TGA presented to the emergency room, split into two groups: those with BAS (n = 21) and those without BAS (n = 16). Besides significant differences between the two groups in ASD size, peak/mean interatrial pressure gradient, and LA:RA ratio, we found that ASD peak gradient/ASD diameter was significantly higher, but ASD diameter/(LA:RA ratio) was significantly lower in the group that required BAS compared with the group without BAS. Multivariate analysis showed that ASD diameter/(LA:RA ratio) was an independent predictor of septostomy requirement, with a cut-off value of 2.58.

Conclusion: ASD diameter/(LA/RA ratio) is a useful echocardiographic parameter that can provide supplementary information regarding inadequate mixing and the need for BAS in neonates with TGA.

References

1. Samanek M. Congenital heart malformations: prevalence, severity, survival, and quality of life. Cardiol Young. 2000;10:179-185.10.1017/S104795110000908210824896
Search in Google Scholar Back to article
2. Blue GM, Kirk EP, Giannoulatou E, et al. Targeted next-generation sequencing identifies pathogenic variants in familial congenital heart disease. J Am Coll Cardiol. 2014;64:2498-506. doi: 10.1016/j.jacc.2014.09.048.10.1016/j.jacc.2014.09.04825500235
Open DOI Search in Google Scholar Back to article
3. Fahed AC, Gelb BD, Seidman JG, Seidman CE. Genetics of Congenital Heart Disease. The glass half empty. Circ Res. 2013;112:707-720. doi: 10.1161/CIRCRESAHA.112.300853.10.1161/CIRCRESAHA.112.300853382769123410880
Open DOI Search in Google Scholar Back to article
4. Kuehl K, Loffredo C, lammer EJ, Iovannisci DM, Shaw GM. Association of Congenital Cardiovascular Malformations With 33 Single Nucleotide Polymorphisms of Selected Cardiovascular Disease-Related Genes. Birth Defects Res A Clin Mol Teratol. 2010;88:101-110. doi: 10.1002/bdra.20630.10.1002/bdra.20630285741119764075
Open DOI Search in Google Scholar Back to article
5. Pierpoint ME, Basson CT, Benson DW Jr, et al. Genetic Basis for Congenital Heart Defects: Current Knowledge. A scientific Statement From the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: Endorsed by the American Academy of Pediatrics. Circulation. 2007;115:3015-3038. doi: 10.1161/CIRCULATIONAHA.106.183056.10.1161/CIRCULATIONAHA.106.18305617519398
Open DOI Search in Google Scholar Back to article
6. Togănel R, Muntean I, Duicu C, Făgărăşan A, Gozar L, Bănescu C. The role of eNOS and AGT gene polymorphisms in secondary pulmonary arterial hypertension in Romanian children with congenital heart disease. Rev Romana Med Lab. 2013;21:267-274. doi: 10.2478/rrlm-2013-0031.10.2478/rrlm-2013-0031
Open DOI Search in Google Scholar Back to article
7. Muntean I, Togănel R, Benedek T. Genetics of Congenital Heart Disease: Past and Present. Biochem Genet. 2017;55:105-123. doi: 10.1007/s10528-016-9780-7.10.1007/s10528-016-9780-727807680
Open DOI Search in Google Scholar Back to article
8. Unolt M, Putotto C, Silvestri LM, et al. Transposition of great arteries: new insights into the pathogenesis. Front Pediatr. 2013;1:11. doi: 10.3389/fped.2013.00011.10.3389/fped.2013.00011386088824400257
Search in Google Scholar Back to article
9. De Luca A, Sarkozy A, Consoli F, et al. Familial transposition of the great arteries caused by multiple mutations in laterality genes. Heart. 2010;96:673-677. doi: 10.1136/hrt.2009.181685.10.1136/hrt.2009.18168519933292
Open DOI Search in Google Scholar Back to article
10. Villafane J, Lantin-Hermoso MR, Bhatt AB, et al. D-Transposition of the great arteries: Hot topics in the current era of the arterial switch operation. J Am Coll Cardiol. 2014;64:498-511. doi: 10.1016/j.jacc.2014.06.1150.10.1016/j.jacc.2014.06.1150434009425082585
Search in Google Scholar Back to article
11. Sarris GE, Balmer C, Bonou P, et al. Clinical guidelines for the management of patients with transposition of the great arteries with intact ventricular septum. Cardiol Young. 2017;27:530-569. doi:10.1017/S1047951117000014.10.1017/S104795111700001428249633
Open DOI Search in Google Scholar Back to article
12. Al-Ata JA. Balloon atrial septostomy in patient with dextrotransposition of the great arteries with insufficient mixing: 18 years of experience. JKAU: Med. Sci. 2010;17:73-89. doi: 10.4197/Med.17-4.6.10.4197/Med.17-4.6
Open DOI Search in Google Scholar Back to article
13. Feltes TF, Bacha E, Beekman RH, et al. Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease A Scientific Statement From the American Heart Association. Circulation. 2011;123:2607-2652. doi: 10.1161/CIR.0b013e31821b1f10.10.1161/CIR.0b013e31821b1f1021536996
Open DOI Search in Google Scholar Back to article
14. Mukherjee D, Lindsay M, Zhang Y, et al. Analysis of 8681 neonates with transposition of the great arteries: outcomes with and without Rashkind balloon atrial septostomy. Cardiol Young. 2010;20:373-380. doi: 10.1017/S1047951110000296.10.1017/S104795111000029620470448
Open DOI Search in Google Scholar Back to article
15. Peterson C, Ailes E, Riehle-Colarusso T, et al. Late detection of critical congenital heart disease among US infants. Estimation of the potential impact of proposed universal screening using pulse oxymetry. JAMA Pedaitrics. 2014;168:361-370. doi: 10.1001/jamapediatrics.2013.4779.10.1001/jamapediatrics.2013.4779447037724493342
Open DOI Search in Google Scholar Back to article
16. Jawin V, Ang H-L, Omar A, Thong M-K. Beyond Critical Congenital Heart Disease: Newborn Screening Using Pulse Oximetry for Neonatal Sepsis and Respiratory Diseases in a Middle-Income Country. PLoS ONE 2015;10:e0137580. doi: 10.1371/journal.pone.0137580.10.1371/journal.pone.0137580456706926360420
Search in Google Scholar Back to article
17. Knowlles R, Griebsch I, Dezateux C, Brown J, Bull C, Wren C. Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis. Health Technol Assess. 2005;9:1-152.10.3310/hta944016297355
Search in Google Scholar Back to article
18. Eckersley L, Sadler L, Parry E, Finucane K, Gentles TL. Timing of diagnosis affects mortality in critical congenital heart disease. Arch Dis Child. 2016;101:516-520. doi: 10.1136/archdischild-2014-307691.10.1136/archdischild-2014-30769126130379
Open DOI Search in Google Scholar Back to article
19. Mahle WT, Gonzalez JH, Kreeger J, Marx G, Duldani G, Silverman NH. Echocardiography of transposition of the great arteries. Cardiol Young. 2012;22:664-670. doi: 10.1017/S1047951112001503.10.1017/S104795111200150323331585
Open DOI Search in Google Scholar Back to article
20. Lopez L, Colan SD, Frommelt PC, et al. Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the pediatric measurements writing group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr. 2010;23:465-495. doi: 10.1016/j.echo.2010.03.019.10.1016/j.echo.2010.03.01920451803
Open DOI Search in Google Scholar Back to article
21. Alwi M. Management algorithm in pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv. 2006;67:679-686. doi: 10.1002/ccd.20672.10.1002/ccd.2067216572430
Open DOI Search in Google Scholar Back to article
22. Dodge-Khatami A, Ott S, Di Bernardo S, Berger F. Carotidsubclavian artery index: new echocardiographic index to detect coarctation in neonates and infants. Ann Thorac Surg. 2005;80:1652-1621. doi: 10.1016/j.athoracsur.2005.04.041.10.1016/j.athoracsur.2005.04.04116242433
Open DOI Search in Google Scholar Back to article
23. Amoozgar H, Salaminia S, Amirghofran AA, et al. Evaluation of cardiac and valvular function after arterial switch operation: a midterm follow-up. Int Cardiovasc Res J. 2013;7:83-89.
Search in Google Scholar Back to article
24. Suteu CC, Muntean I, Benedek T, Togănel R. Giant dissecting ventricular septal haematoma associated with critical congenital heart disease. Interact Cardiovasc Thorac Surg. 2016;23:837-838. doi: 10.1093/icvts/ivw223.10.1093/icvts/ivw22327365006
Open DOI Search in Google Scholar Back to article
25. Peng DM, Punn R, Maeda K, Selamet Tierney ES. Diagnosing Neonatal Aortic coarctation in the setting of Patent Ductus Arteriosus. Ann Thorac Surg. 2016;101:1005-1010. doi: 10.1016/j.athoracsur.2015.09.050.10.1016/j.athoracsur.2015.09.05026652138
Open DOI Search in Google Scholar Back to article
26. Mivelaz Y, Di Bernardo S, Meijboom EJ, Sekarski N. Validation of two echocardiographic indexes to improve the diagnosis of complex coarctations. Eur J Cardiothorac Surg. 2008;34:1051-1056. doi: 10.1016/j.ejcts.2008.07.036.10.1016/j.ejcts.2008.07.03618824366
Open DOI Search in Google Scholar Back to article
27. Lu CW, Wang JK, Chang CI, et al. Noninvasive diagnosis of aortic coarctation in neonates with patent ductus arteriosus. J Pediatr. 2006;148:217-221. doi: 10.1016/j.jpeds.2005.09.036.10.1016/j.jpeds.2005.09.03616492432
Open DOI Search in Google Scholar Back to article
28. Familiari A, Morlando M, Khalil AA, Sven-Erik S, et al. Risk factor for coarctation of the aorta on prenatal ultrasound: a systemic review and meta-analysis. Circulation. 2017;136:24. doi: 10.1161/CIRCULATIONAHA.116.024068.10.1161/CIRCULATIONAHA.116.02406828034902
Open DOI Search in Google Scholar Back to article
29. Mărginean C, Mărginean CO, Muntean I, Togănel R, Voidăzan S, Gozar L. The role of ventricular disproportion, aortic and ductal isthmus ultrasound measurements for the diagnosis of fetal aortic coarctation, in the third trimester of pregnancy. Med Ultrason. 2015;17:475-481. doi: 10.11152/mu.2013.2066.174.rvd.10.11152/mu.2013.2066.174.rvd26649342
Open DOI Search in Google Scholar Back to article
30. Daubeney PE, Sharland GK, Cook AC, Keeton BR, Anderson RH, Webber SA. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Circulation. 1998;98:562-566.10.1161/01.CIR.98.6.5629714114
Open DOI Search in Google Scholar Back to article
31. Peterson RE, Levi DS, Williams RJ, Lai WW, Sklansky MS, Drant S. Echocardiographic predictors of outcome in fetuses with pulmonary atresia with intact ventricular septum. J Am Soc Echocardiogr. 2006;19:1393-1400. doi: 10.1016/j.echo.2006.05.021l.10.1016/j.echo.2006.05.021l
Open DOI Search in Google Scholar Back to article
32. Salvin JW, McElhinney DB, Colan SD, et al. Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum. Pediatrics. 2006;118:e415-e420. doi: 10.1542/peds.2006-0428.10.1542/peds.2006-0428
Open DOI Search in Google Scholar Back to article
33. Graziano JN, Heidelberger KP, Ensing GJ, Gomez CA, Ludomirsky A. The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome. Pediatr Cardiol. 2002;23:146-151.10.1007/s00246-001-0038-7
Open DOI Search in Google Scholar Back to article
34. Canter CE, Moorehead S, Huddleston CB, Spray TL. Restrictive atrial septal communication as a determinant of outcome of cardiac transplantation for hypoplastic left heart syndrome. Circulation. 1993;88:II456-II460.
Search in Google Scholar Back to article
35. Dash PK, Satpathy M. Atrial septal defect (secundum type). In: Sathpathy M, Mishra BR. Clinical Diagnosis of Congenital heart disease. Jaypee/The Health Sciences Publisher/New Delhi, 2015; p. 76-84.
Search in Google Scholar Back to article
36. Kuhn MA, Larsen RL, Mulla NF, Jofnston JK, Chinnock RE, Bailey LL. Outcome of infants with hypoplastic left heart syndrome who undergo atrial septostomy before heart transplantation. Am J Cardiol. 2000;85:124-127.10.1016/S0002-9149(99)00624-4
Search in Google Scholar Back to article

Predictors of Inadequate Mixing in Transposition of the Great Arteries — a Critical Neonatal Condition

Abstract

Paradigm

My account