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Subpulmonary Stenosis Assessed in Midtrimester Fetuses With Tetralogy of Fallot: A Novel Method for Predicting Postnatal Clinical Outcome

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Abstract

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6–25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: −3.68 + (0.91 × prenatal PVZ) – (4.44 × SPS/DAO) – 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

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References

  1. Canfield M, Honein M, Yuskiv N, Xing J, Mai C, Collins J, Devine O, Petrini J, Ramadhani T, Hobbs C, Kirby R (2006) National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999–2001: birth defects research. Clin Mol Teratol 76:747–756

    Article  CAS  Google Scholar 

  2. Escribano D, Herraiz I, Granados M, Arbues J, Mendoza A, Galindo A (2011) Tetralogy of Fallot: prediction of outcome in the mid-second trimester of pregnancy. Prenat Diagn 31:1126–1133

    PubMed  CAS  Google Scholar 

  3. Gembruch U, Weinraub Z, Bald R, Redel DA, Knopfle G, Hansmann M (1990) Flow analysis in the pulmonary trunk in fetuses with tetralogy of Fallot by colour Doppler flow mapping; two case reports. Eur J Obstet Gynecol Reprod Biol 35:259–265

    Article  PubMed  CAS  Google Scholar 

  4. Gott VL (1990) C. Walton Lillehei and total correction of tetralogy of Fallot. Ann Thorac Surg 49:328–332

    Article  PubMed  CAS  Google Scholar 

  5. Guntheroth WG, Forster FK, Stevenson JG (2003) Cause of normal pulmonic velocity in fetal tetralogy of Fallot. Am J Cardiol 92:1485–1487

    Article  PubMed  Google Scholar 

  6. Hirji A, Bernasconi A, McCrindle BW, Dunn E, Gurofsky R, Manlhiot C, Miner SE, Kingdom JC, Jaeggi ET, Van Arsdell G, Nield LE (2010) Outcomes of prenatally diagnosed tetralogy of Fallot: implications for valve-sparing repair versus transannular patch. Can J Cardiol 26:e1–e6

    Article  PubMed  CAS  Google Scholar 

  7. Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900

    Article  PubMed  Google Scholar 

  8. Hornberger LK, Sanders SP, Sahn DJ, Rice MJ, Spevak PJ, Benacerraf BR, McDonald RW, Colan SD (1995) In utero pulmonary artery and aortic growth and potential for progression of pulmonary outflow tract obstruction in tetralogy of Fallot. J Am Coll Cardiol 25:739–745

    Article  PubMed  CAS  Google Scholar 

  9. Hove JR, Koster RW, Forouhar AS, Acevedo-Bolton G, Fraser SE, Gharib M (2003) Intracardiac fluid forces are an essential epigenetic factor for embryonic cardiogenesis. Nature 421:172–177

    Article  PubMed  CAS  Google Scholar 

  10. Lai WW, Geva T, Shirali GS, Frommelt PC, Humes RA, Brook MM, Pignatelli RH, Rychik J, Task Force of the Pediatric Council of the American Society of Echocardiography (2006) Guidelines and standards for performance of a pediatric echocardiogram: a report from the Task Force of the Pediatric Council of the American Society of Echocardiography. J Am Soc Echocardiogr 19:1413–1430

    Article  PubMed  Google Scholar 

  11. Lee W, Smith RS, Comstock CH, Kirk JS, Riggs T, Weinhouse E (1995) Tetralogy of Fallot: prenatal diagnosis and postnatal survival. Obstet Gynecol 86:583–588

    PubMed  CAS  Google Scholar 

  12. Parry AJ, McElhinney DB, Kung GC, Reddy VM, Brook MM, Hanley FL (2000) Elective primary repair of acyanotic tetralogy of Fallot in early infancy: overall outcome and impact on the pulmonary valve. J Am Coll Cardiol 36:2279–2283

    Article  PubMed  CAS  Google Scholar 

  13. Pepas LP, Savis A, Jones A, Sharland GK, Tulloh RM, Simpson JM (2003) An echocardiographic study of tetralogy of Fallot in the fetus and infant. Cardiol Young 13:240–247

    PubMed  Google Scholar 

  14. Rao V, Kadletz M, Hornberger LK, Freedom RM, Black MD (2000) Preservation of the pulmonary valve complex in tetralogy of Fallot: how small is too small? Ann Thorac Surg 69:176–179; discussion 179–180

    Article  PubMed  CAS  Google Scholar 

  15. Reddy VM, Liddicoat JR, McElhinney DB, Brook MM, Stanger P, Hanley FL (1995) Routine primary repair of tetralogy of Fallot in neonates and infants less than 3 months of age. Ann Thorac Surg 60:S592–S596

    Article  PubMed  CAS  Google Scholar 

  16. Rychik J, Ayres N, Cuneo B, Gotteiner N, Hornberger L, Spevak PJ, Van Der Veld M (2004) American Society of Echocardiography guidelines and standards for performance of the fetal echocardiogram. J Am Soc Echocardiogr 17:803–810

    Article  PubMed  Google Scholar 

  17. Schneider C, McCrindle BW, Carvalho JS, Hornberger LK, McCarthy KP, Daubeney PE (2005) Development of z scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 26:599–605

    Article  PubMed  CAS  Google Scholar 

  18. Stewart RD, Backer CL, Young L, Mavroudis C (2005) Tetralogy of Fallot: results of a pulmonary valve-sparing strategy. Ann Thorac Surg 80:1431–1438; discussion 1438–1439

    Article  PubMed  Google Scholar 

  19. Van Arsdell GS, Maharaj GS, Tom J, Rao VK, Coles JG, Freedom RM, Williams WG, McCrindle BW (2000) What is the optimal age for repair of tetralogy of Fallot? Circulation 102:III123–III129

    PubMed  Google Scholar 

  20. Zilberman MV, Khoury PR, Kimball RT (2005) Two-dimensional echocardiographic valve measurements in healthy children: gender-specific differences. Pediatr Cardiol 26:356–360

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

The authors are most grateful to data analyst, Jose E. Tabora, PhD, and James Godbold, PhD for their assistance in the statistical analysis of the data in this study.

Conflict of interest

None of the authors have any conflicts of interest, financial or otherwise, to disclose.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, Mount Sinai School of Medicine, Mount Sinai Medical Center, One Gustave L. Levy Place, Box 1201, New York, NY, 10029, USA

    Elena N. Kwon, Ira A. Parness, Shubhika Srivastava, James C. Nielsen & Miwa Geiger

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  1. Elena N. Kwon
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  2. Ira A. Parness
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  3. Shubhika Srivastava
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  4. James C. Nielsen
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  5. Miwa Geiger
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Correspondence to Miwa Geiger.

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Kwon, E.N., Parness, I.A., Srivastava, S. et al. Subpulmonary Stenosis Assessed in Midtrimester Fetuses With Tetralogy of Fallot: A Novel Method for Predicting Postnatal Clinical Outcome. Pediatr Cardiol 34, 1314–1320 (2013). https://doi.org/10.1007/s00246-013-0642-3

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  • DOI: https://doi.org/10.1007/s00246-013-0642-3

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