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Evaluation and Management of Fetal Cardiac Function and Heart Failure

  • Pediatric Congenital Heart Disease (G Singh, Section Editor)
  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Opinion statement

Two-dimensional (2D) and Doppler echocardiography has become both an essential component in the assessment of anatomic anomalies of the fetal heart and an important tool in the assessment of fetal cardiac function. Various techniques for assessing fetal cardiovascular function exist and can be used to characterize systolic and diastolic ventricular function and estimate fetal cardiac output. These techniques can be utilized to assess fetuses with cardiovascular compromise due to a number of cardiac and non-cardiac conditions, including twin-twin transfusion syndrome, high-cardiac output lesions such as fetal tumors and vascular malformations, and primary fetal cardiomyopathy. As many of these conditions can now be treated by a variety of fetal therapeutic techniques, it is now important to be able to understand and identify fetal cardiac dysfunction. Characterization of fetal cardiac dysfunction now has the potential to provide clinicians with the means to better select patients for intervention, predict outcome, and perform follow-up following fetal interventional procedures.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Lange LW, Sahn DJ, Allen HD, Goldberg SJ, Anderson C, Giles H. Qualitative real-time cross-sectional echocardiographic imaging of the human fetus during the second half of pregnancy. Circulation. 1980;62(4):799–806.

    Article  CAS  PubMed  Google Scholar 

  2. Sahn DJ, Lange LW, Allen HD, Goldberg SJ, Anderson C, Giles H, et al. Quantitative real-time cross-sectional echocardiography in the developing normal human fetus and newborn. Circulation. 1980;62(3):588–97.

    Article  CAS  PubMed  Google Scholar 

  3. Friedman WF. The intrinsic physiologic properties of the developing heart. Prog Cardiovasc Dis. 1972;15(1):87–111.

    Article  CAS  PubMed  Google Scholar 

  4. Nakanishi T, Jarmakani JM. Developmental changes in myocardial mechanical function and subcellular organelles. Am J Physiol. 1984;246(4 Pt 2):H615–25.

    CAS  PubMed  Google Scholar 

  5. Hofstaetter C, Hansmann M, Eik-Nes SH, Huhta JC, Luther SL. A cardiovascular profile score in the surveillance of fetal hydrops. J Matern Fetal Neonatal Med. 2006;19(7):407–13.

    Article  PubMed  Google Scholar 

  6. Goldinfeld M, Weiner E, Peleg D, Shalev E, Ben-Ami M. Evaluation of fetal cardiac contractility by two-dimensional ultrasonography. Prenat Diagn. 2004;24(10):799–803.

    Article  PubMed  Google Scholar 

  7. Harada K, Rice MJ, Shiota T, McDonald RW, Reller MD, Sahn DJ. Two-dimensional echocardiographic evaluation of ventricular systolic function in human fetuses with ductal constriction. Ultrasound Obstet Gynecol. 1997;10(4):247–53.

    Article  CAS  PubMed  Google Scholar 

  8. Tei C, Ling LH, Hodge DO, Bailey KR, Oh JK, Rodeheffer RJ, et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function—a study in normals and dilated cardiomyopathy. J Cardiol. 1995;26(6):357–66.

    CAS  PubMed  Google Scholar 

  9. Eidem BW, Edwards JM, Cetta F. Quantitative assessment of fetal ventricular function: establishing normal values of the myocardial performance index in the fetus. Echocardiography. 2001;18(1):9–13.

    Article  CAS  PubMed  Google Scholar 

  10. Mori Y, Rice MJ, McDonald RW, Reller MD, Wanitkun S, Harada K, et al. Evaluation of systolic and diastolic ventricular performance of the right ventricle in fetuses with ductal constriction using the Doppler Tei index. Am J Cardiol. 2001;88(10):1173–8.

    Article  CAS  PubMed  Google Scholar 

  11. Friedman D, Buyon J, Kim M, Glickstein JS. Fetal cardiac function assessed by Doppler myocardial performance index (Tei Index). Ultrasound Obstet Gynecol. 2003;21(1):33–6.

    Article  CAS  PubMed  Google Scholar 

  12. Barrea C, Alkazaleh F, Ryan G, McCrindle BW, Roberts A, Bigras JL, et al. Prenatal cardiovascular manifestations in the twin-to-twin transfusion syndrome recipients and the impact of therapeutic amnioreduction. Am J Obstet Gynecol. 2005;192(3):892–902.

    Article  PubMed  Google Scholar 

  13. Michelfelder E, Gottliebson W, Border W, Kinsel M, Polzin W, Livingston J, et al. Early manifestations and spectrum of recipient twin cardiomyopathy in twin-twin transfusion syndrome: relation to Quintero stage. Ultrasound Obstet Gynecol. 2007;30(7):965–71.

    Article  CAS  PubMed  Google Scholar 

  14. Aoki M, Harada K, Ogawa M, Tanaka T. Quantitative assessment of right ventricular function using doppler tissue imaging in fetuses with and without heart failure. J Am Soc Echocardiogr. 2004;17(1):28–35.

    Article  PubMed  Google Scholar 

  15. Huhta JC. Guidelines for the evaluation of heart failure in the fetus with or without hydrops. Pediatr Cardiol. 2004;25(3):274–86.

    Article  CAS  PubMed  Google Scholar 

  16. Pedra SR, Smallhorn JF, Ryan G, Chitayat D, Taylor GP, Khan R, et al. Fetal cardiomyopathies: pathogenic mechanisms, hemodynamic findings, and clinical outcome. Circulation. 2002;106(5):585–91.

    Article  PubMed  Google Scholar 

  17. Schmidt KG, Birk E, Silverman NH, Scagnelli SA. Echocardiographic evaluation of dilated cardiomyopathy in the human fetus. Am J Cardiol. 1989;63(9):599–605.

    Article  CAS  PubMed  Google Scholar 

  18. Simpson LL, Marx GR, Elkadry EA, D’Alton ME. Cardiac dysfunction in twin-twin transfusion syndrome: a prospective, longitudinal study. Obstet Gynecol. 1998;92(4 Pt 1):557–62.

    Article  CAS  PubMed  Google Scholar 

  19. Zosmer N, Bajoria R, Weiner E, Rigby M, Vaughan J, Fisk NM. Clinical and echographic features of in utero cardiac dysfunction in the recipient twin in twin-twin transfusion syndrome. Br Heart J. 1994;72(1):74–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Carceller-Blanchard AM, Fouron JC. Determinants of the Doppler flow velocity profile through the mitral valve of the human fetus. Br Heart J. 1993;70(5):457–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Kaufman TM, Horton JW, White DJ, Mahony L. Age-related changes in myocardial relaxation and sarcoplasmic reticulum function. Am J Physiol. 1990;259(2 Pt 2):H309–16.

    CAS  PubMed  Google Scholar 

  22. Harada K, Rice MJ, Shiota T, Ishii M, McDonald RW, Reller MD, et al. Gestational age- and growth-related alterations in fetal right and left ventricular diastolic filling patterns. Am J Cardiol. 1997;79(2):173–7.

    Article  CAS  PubMed  Google Scholar 

  23. Romero T, Covell J, Friedman WF. A comparison of pressure-volume relations of the fetal, newborn, and adult heart. Am J Physiol. 1972;222(5):1285–90.

    CAS  PubMed  Google Scholar 

  24. Hecher K, Campbell S, Doyle P, Harrington K, Nicolaides K. Assessment of fetal compromise by Doppler ultrasound investigation of the fetal circulation. Arterial, intracardiac, and venous blood flow velocity studies. Circulation. 1995;91(1):129–38.

    Article  CAS  PubMed  Google Scholar 

  25. Rizzo G, Capponi A, Arduini D, Romanini C. Ductus venosus velocity waveforms in appropriate and small for gestational age fetuses. Early Hum Dev. 1994;39(1):15–26.

    Article  CAS  PubMed  Google Scholar 

  26. Tulzer G, Gudmundsson S, Wood DC, Cohen AW, Weiner S, Huhta JC. Doppler in non-immune hydrops fetalis. Ultrasound Obstet Gynecol. 1994;4(4):279–83.

    Article  CAS  PubMed  Google Scholar 

  27. Axt-Fliedner R, Wiegank U, Fetsch C, Friedrich M, Krapp M, Georg T, et al. Reference values of fetal ductus venosus, inferior vena cava and hepatic vein blood flow velocities and waveform indices during the second and third trimester of pregnancy. Arch Gynecol Obstet. 2004;270(1):46–55.

    Article  CAS  PubMed  Google Scholar 

  28. Divanovic A, Cnota J, Ittenbach R, Tan X, Border W, Crombleholme T, et al. Characterization of diastolic dysfunction in twin-twin transfusion syndrome: association between Doppler findings and ventricular hypertrophy. J Am Soc Echocardiogr. 2011;24(8):834–40.

    Article  PubMed  Google Scholar 

  29. Berg C, Kremer C, Geipel A, Kohl T, Germer U, Gembruch U. Ductus venosus blood flow alterations in fetuses with obstructive lesions of the right heart. Ultrasound Obstet Gynecol. 2006;28(2):137–42.

    Article  CAS  PubMed  Google Scholar 

  30. Gudmundsson S, Huhta JC, Wood DC, Tulzer G, Cohen AW, Weiner S. Venous Doppler ultrasonography in the fetus with nonimmune hydrops. Am J Obstet Gynecol. 1991;164(1 Pt 1):33–7.

    Article  CAS  PubMed  Google Scholar 

  31. Tsyvian P, Malkin K, Artemieva O, Blyakhman F, Wladimiroff JW. Cardiac ventricular performance in the appropriate- for-gestational age and small-for-gestational age fetus: relation to regional cardiac non-uniformity and peripheral resistance. Ultrasound Obstet Gynecol. 2002;20(1):35–41.

    Article  CAS  PubMed  Google Scholar 

  32. Wong SF, Chan FY, Cincotta RB, McIntyre HD, Oats JJ. Cardiac function in fetuses of poorly-controlled pre-gestational diabetic pregnancies—a pilot study. Gynecol Obstet Invest. 2003;56(2):113–6.

    Article  CAS  PubMed  Google Scholar 

  33. Moon-Grady AJ, Rand L, Gallardo S, Gosnell K, Lee H, Feldstein VA. Diastolic cardiac pathology and clinical twin-twin transfusion syndrome in monochorionic/diamniotic twins. Am J Obstet Gynecol. 2011;205(3):279 e1- e11.

  34. Byrne FA, Lee H, Kipps AK, Brook MM, Moon-Grady AJ. Echocardiographic risk stratification of fetuses with sacrococcygeal teratoma and twin-reversed arterial perfusion. Fetal Diagn Ther. 2011;30(4):280–8.

    Article  PubMed  Google Scholar 

  35. Kinsel-Ziter ML, Cnota JF, Crombleholme TM, Michelfelder EC. Twin-reversed arterial perfusion sequence: pre- and postoperative cardiovascular findings in the ‘pump’ twin. Ultrasound Obstet Gynecol. 2009;34(5):550–5.

    Article  CAS  PubMed  Google Scholar 

  36. Statile CJ, Cnota JF, Gomien S, Divanovic A, Crombleholme T, Michelfelder E. Estimated cardiac output and cardiovascular profile score in fetuses with high cardiac output lesions. Ultrasound Obstet Gynecol. 2013;41(1):54–8. This study examines the important topic of elevated cardiac output and its relationship to fetal cardiovascular status in the fetus with high-output lesions. It also provides evidence of the utility of the Cardiovascular Profile Score as a tool to describe fetal cardiovascular status.

    Article  CAS  PubMed  Google Scholar 

  37. Mielke G, Benda N. Cardiac output and central distribution of blood flow in the human fetus. Circulation. 2001;103(12):1662–8.

    Article  CAS  PubMed  Google Scholar 

  38. Shah AD, Border WL, Crombleholme TM, Michelfelder EC. Initial fetal cardiovascular profile score predicts recipient twin outcome in twin-twin transfusion syndrome. J Am Soc Echocardiogr. 2008;21(10):1105–8.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Patel D, Cuneo B, Viesca R, Rassanan J, Leshko J, Huhta J. Digoxin for the treatment of fetal congestive heart failure with sinus rhythm assessed by cardiovascular profile score. J Matern Fetal Neonatal Med. 2008;21(7):477–82.

    Article  CAS  PubMed  Google Scholar 

  40. Bahtiyar MO, Emery SP, Dashe JS, Wilkins-Haug LE, Johnson A, Paek BW, et al. The North American Fetal Therapy Network consensus statement: prenatal surveillance of uncomplicated monochorionic gestations. Obstet Gynecol. 2015;125(1):118–23.

    Article  PubMed  Google Scholar 

  41. Johnson A. Diagnosis and management of twin-twin transfusion syndrome. Clin Obstet Gynecol. 2015;58(3):611–31.

    Article  PubMed  Google Scholar 

  42. Duryea EL, Happe SK, McIntire DD, Dashe JS. The natural history of twin-twin transfusion syndrome stratified by Quintero stage. J Matern Fetal Neonatal Med. 2016;1–5.

  43. Quintero RA, Morales WJ, Allen MH, Bornick PW, Johnson PK, Kruger M. Staging of twin-twin transfusion syndrome. J Perinatol. 1999;19(8 Pt 1):550–5.

    Article  CAS  PubMed  Google Scholar 

  44. Raboisson MJ, Fouron JC, Lamoureux J, Leduc L, Grignon A, Proulx F, et al. Early intertwin differences in myocardial performance during the twin-to-twin transfusion syndrome. Circulation. 2004;110(19):3043–8.

    Article  CAS  PubMed  Google Scholar 

  45. Lewi L, Jani J, Blickstein I, Huber A, Gucciardo L, Van Mieghem T, et al. The outcome of monochorionic diamniotic twin gestations in the era of invasive fetal therapy: a prospective cohort study. Am J Obstet Gynecol. 2008;199(5):514 e1-8.

  46. Society for Maternal-Fetal M, Simpson LL. Twin-twin transfusion syndrome. Am J Obstet Gynecol. 2013;208(1):3–18.

    Article  Google Scholar 

  47. Bahtiyar MO, Dulay AT, Weeks BP, Friedman AH, Copel JA. Prevalence of congenital heart defects in monochorionic/diamniotic twin gestations: a systematic literature review. J Ultrasound Med. 2007;26(11):1491–8.

    PubMed  Google Scholar 

  48. Michelfelder E, Tan X, Cnota J, Divanovic A, Statile C, Lim FY, et al. Prevalence, spectrum, and outcome of right ventricular outflow tract abnormalities in twin-twin transfusion syndrome: a large single-center experience. Congenit Heart Dis. 2015;10(3):209–18.

    Article  PubMed  Google Scholar 

  49. Moon-Grady AJ, Rand L, Lemley B, Gosnell K, Hornberger LK, Lee H. Effect of selective fetoscopic laser photocoagulation therapy for twin-twin transfusion syndrome on pulmonary valve pathology in recipient twins. Ultrasound Obstet Gynecol. 2011;37(1):27–33.

    Article  CAS  PubMed  Google Scholar 

  50. Stagnati V, Chalouhi GE, Essaoui M, Giuseppi A, Stirnemann JJ, Le Bidois J, et al. Pulmonary stenosis in complicated monochorionic twin pregnancies: prevalence, management and outcome. Prenat Diagn. 2015;35(11):1085–92.

    Article  CAS  PubMed  Google Scholar 

  51. O’Donoghue K, Cartwright E, Galea P, Fisk NM. Stage I twin-twin transfusion syndrome: rates of progression and regression in relation to outcome. Ultrasound Obstet Gynecol. 2007;30(7):958–64.

    Article  PubMed  Google Scholar 

  52. Dickinson JE, Evans SF. Obstetric and perinatal outcomes from the Australian and New Zealand twin-twin transfusion syndrome registry. Am J Obstet Gynecol. 2000;182(3):706–12.

    Article  CAS  PubMed  Google Scholar 

  53. Mari G, Roberts A, Detti L, Kovanci E, Stefos T, Bahado-Singh RO, et al. Perinatal morbidity and mortality rates in severe twin-twin transfusion syndrome: results of the International Amnioreduction Registry. Am J Obstet Gynecol. 2001;185(3):708–15.

    Article  CAS  PubMed  Google Scholar 

  54. De Lia JE, Cruikshank DP, Keye Jr WR. Fetoscopic neodymium:YAG laser occlusion of placental vessels in severe twin-twin transfusion syndrome. Obstet Gynecol. 1990;75(6):1046–53.

    PubMed  Google Scholar 

  55. Behrendt N, Galan HL. Twin-twin transfusion and laser therapy. Curr Opin Obstet Gynecol. 2016;28(2):79–85.

    Article  PubMed  Google Scholar 

  56. Senat MV, Deprest J, Boulvain M, Paupe A, Winer N, Ville Y. Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. N Engl J Med. 2004;351(2):136–44.

    Article  CAS  PubMed  Google Scholar 

  57. Rossi AC, D’Addario V. Laser therapy and serial amnioreduction as treatment for twin-twin transfusion syndrome: a metaanalysis and review of literature. Am J Obstet Gynecol. 2008;198(2):147–52.

    Article  PubMed  Google Scholar 

  58. Crombleholme TM, Shera D, Lee H, Johnson M, D’Alton M, Porter F, et al. A prospective, randomized, multicenter trial of amnioreduction vs selective fetoscopic laser photocoagulation for the treatment of severe twin-twin transfusion syndrome. Am J Obstet Gynecol. 2007;197(4):396 e1-9.

  59. Roberts D, Gates S, Kilby M, Neilson JP. Interventions for twin-twin transfusion syndrome: a Cochrane review. Ultrasound Obstet Gynecol. 2008;31(6):701–11.

    Article  CAS  PubMed  Google Scholar 

  60. Ruano R, Duarte S, Zugaib M. Percutaneous laser ablation of sacrococcygeal teratoma in a hydropic fetus with severe heart failure—too late for a surgical procedure? Fetal Diagn Ther. 2009;25(1):26–30.

    Article  PubMed  Google Scholar 

  61. Russell RT, Carlin A, Ashworth M, Welch CR. Diffuse placental chorioangiomatosis and fetal hydrops. Fetal Diagn Ther. 2007;22(3):183–5.

    Article  CAS  PubMed  Google Scholar 

  62. Wilson RD, Hedrick H, Flake AW, Johnson MP, Bebbington MW, Mann S, et al. Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome. Fetal Diagn Ther. 2009;25(1):15–20.

    Article  PubMed  Google Scholar 

  63. Iacovella C, Chandrasekaran N, Khalil A, Bhide A, Papageorghiou A, Thilaganathan B. Fetal and placental vascular tumors: persistent fetal hyperdynamic status predisposes to poorer long-term neurodevelopmental outcome. Ultrasound Obstet Gynecol. 2014;43(6):658–61.

    Article  CAS  PubMed  Google Scholar 

  64. Van Mieghem T, Al-Ibrahim A, Deprest J, Lewi L, Langer JC, Baud D, et al. Minimally invasive therapy for fetal sacrococcygeal teratoma: case series and systematic review of the literature. Ultrasound Obstet Gynecol. 2014;43(6):611–9.

    Article  PubMed  Google Scholar 

  65. Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD, et al. Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome. J Pediatr Surg. 2004;39(3):430-8; discussion -8.

  66. Van Allen MI, Smith DW, Shepard TH. Twin reversed arterial perfusion (TRAP) sequence: a study of 14 twin pregnancies with acardius. Semin Perinatol. 1983;7(4):285–93.

    PubMed  Google Scholar 

  67. Moore TR, Gale S, Benirschke K. Perinatal outcome of forty-nine pregnancies complicated by acardiac twinning. Am J Obstet Gynecol. 1990;163(3):907–12.

    Article  CAS  PubMed  Google Scholar 

  68. Livingston JC, Lim FY, Polzin W, Mason J, Crombleholme TM. Intrafetal radiofrequency ablation for twin reversed arterial perfusion (TRAP): a single-center experience. Am J Obstet Gynecol. 2007;197(4):399 e1-3.

  69. Rand L, Lee H. Complicated monochorionic twin pregnancies: updates in fetal diagnosis and treatment. Clin Perinatol. 2009;36(2):417–30. x-xi.

    Article  PubMed  Google Scholar 

  70. Zanardini C, Papageorghiou A, Bhide A, Thilaganathan B. Giant placental chorioangioma: natural history and pregnancy outcome. Ultrasound Obstet Gynecol. 2010;35(3):332–6.

    Article  CAS  PubMed  Google Scholar 

  71. Eliasson H, Sonesson SE, Sharland G, Granath F, Simpson JM, Carvalho JS, et al. Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients. Circulation. 2011;124(18):1919–26.

    Article  PubMed  Google Scholar 

  72. Izmirly PM, Saxena A, Kim MY, Wang D, Sahl SK, Llanos C, et al. Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus. Circulation. 2011;124(18):1927–35.

    Article  PubMed  PubMed Central  Google Scholar 

  73. Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation. 2004;110(12):1542–8.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Sibley Heart Center Cardiology, Children’s Healthcare of Atlanta, Emory University School of Medicine, 2835 Brandywine Road, Suite 300, Atlanta, GA, 30341, USA

    Erik Michelfelder MD

  2. The Heart Institute, Cincinnati Children’s Hospital Medical Center, MLC 2003 Burnet Avenue, Cincinnati, OH, 45229, USA

    Catherine Allen MD & Lindsay Urbinelli MD

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Michelfelder, E., Allen, C. & Urbinelli, L. Evaluation and Management of Fetal Cardiac Function and Heart Failure. Curr Treat Options Cardio Med 18, 55 (2016). https://doi.org/10.1007/s11936-016-0477-3

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