Advertisement

Prenatally Diagnosed Congenital Ventricular Outpouchings: An Institutional Experience and Review of the Literature

  • Jacqueline M. ShuplockEmail author
  • Ann Kavanaugh-McHugh
  • David Parra
Original Article

Abstract

Congenital ventricular outpouchings (CVOs) are rare congenital heart defects with limited data regarding prognosis and outcomes. We aimed to describe the characteristics, outcomes and factors associated with morbidity and mortality of prenatally diagnosed CVOs using our institutional experience and a review of published cases. A total of 86 cases of prenatally diagnosed CVOs were identified, including 3 from our institution and 83 cases identified from a review of the literature. Fetal and postnatal outcomes were analyzed for each case. Pericardial effusions (44%) and ventricular dysfunction (17%) were the most common associated findings. Excluding cases that resulted in pregnancy termination, mortality was 17%, with the majority (11/13) occurring in the prenatal period. Factors associated with mortality included an outpouching located on the left ventricle, a diagnosis of hydrops fetalis, the presence of a pericardial effusion, and an earlier gestational age at diagnosis. Of those that survived to delivery, 57% remained asymptomatic without the need for intervention, and the outpouching regressed or resolved in an additional 15%. Prenatally diagnosed congenital ventricular outpouchings are a dynamic form of congenital heart disease with a high fetal mortality rate. The outcomes associated with the outpouchings appear to be the most variable in the prenatal period and the first year after birth. Serial prenatal and postnatal evaluations should be performed to evaluate for a change in the characteristics of the outpouching.

Keywords

Congenital heart defect Fetal echocardiography Pediatric cardiology Prenatal diagnosis 

Notes

Funding

This study received no funding.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflicts of interest to report.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors.

Supplementary material

246_2019_2252_MOESM1_ESM.pdf (219 kb)
Supplementary file1 (PDF 219 kb)

References

  1. 1.
    Goncalves L, Sims J, Philippe J (1992) Aneurysm, left ventricle. TheFetus.net. https://sonoworld.com/fetus/page.aspx?id=33. Accessed 17 Dec 1992
  2. 2.
    Williams J, Collardey K, Treadwell M, Owens S (2009) Prenatally diagnosed right ventricular outpouchings: a case series and review of the literature. Pediatr Cardiol 30(6):840–845PubMedCrossRefGoogle Scholar
  3. 3.
    Prefumo F, Bhide A, Thilaganathan B, Carvalho J (2005) Fetal congenital cardiac diverticulum with pericardial effusion: two cases with different presentations in the first trimester of pregnancy. Ultrasound Obstet Gynecol. 25(4):405–408PubMedCrossRefGoogle Scholar
  4. 4.
    Marijon E, Ou P, Fermont L, Concordet S, Le Bidois J, Sidi D, Bonnet D (2006) Diagnosis and outcome in congenital ventricular diverticulum and aneurysm. J Thorac Cardiovasc Surg 131(2):433–437PubMedCrossRefGoogle Scholar
  5. 5.
    Ohlow M, Bruneli M, Lauer B (2014) Characteristics and outcome of primary congenital left ventricular aneurysm and diverticulum: analysis of cases from the literature. Prenat Diagn 34(9):893–899PubMedCrossRefGoogle Scholar
  6. 6.
    Awad S, Patel A, Polimenakos A, Braun R, Abdulla R (2009) Left ventricular accessory chamber: a case report and review of the literature. Pediatr Cardiol 30(7):1022–1025PubMedCrossRefGoogle Scholar
  7. 7.
    Evans W, Madrid A, Castillo W, Rollins R, Berthoty D, Starnes V, Wiencek R, Ciccolo M, Acherman R (2009) All cardiac right ventricular outpouches are not created equal. Pediatr Cardiol 30(7):954–957PubMedCrossRefGoogle Scholar
  8. 8.
    Perlitz Y, Mukary M, Lorber A, Ben-Ami M (2009) Prenatal diagnosis of fetal cardiac right ventricular diverticulum disappearing at three months of age: a case report and literature review. Fetal Diagn Ther 25(1):44–46PubMedCrossRefGoogle Scholar
  9. 9.
    Wax J, Moran A, Pinette MG, Reyes A, Cartin A, Blackstone J (2007) Prenatal sonographic diagnosis of fetal right ventricular diverticulum. J Ultrasound Med 26(2):267–270PubMedCrossRefGoogle Scholar
  10. 10.
    Malakan Rad E, Awad S, Hijazi Z (2014) Congenital left ventricular outpouchings: a systematic review of 839 cases and introduction of a novel classification after two centuries. Congenit Heart Dis 9(6):498–511PubMedCrossRefGoogle Scholar
  11. 11.
    McAuliffe F, Hornberger L, Johnson J, Chitayat D, Ryan G (2005) Cardiac diverticulum with pericardial effusion: report of two new cases treated by in-utero pericardiocentesis and a review of the literature. Ultrasound Obstet Gynecol 25(4):401–404PubMedCrossRefGoogle Scholar
  12. 12.
    Abi-Nader K, David A, Yates R, Pandya P (2009) Successful outcome after prenatal treatment of a cardiac diverticulum with massive pericardial effusion. Fetal Diagn Ther. 25(1):148–152PubMedCrossRefGoogle Scholar
  13. 13.
    Barberato M, Barberato S, Binotto C, Cavalcanti M, Passos A, Miyague N (2009) Prenatal diagnosis of left ventricular aneurysm and diverticulum. Arg Bras Cardiol 93(2):e36–38CrossRefGoogle Scholar
  14. 14.
    Carrard C, Massardier J, Pangaud N, Champion F (2010) Fetal right ventricular diverticulum with pericardial effusion: report of a new case treated by in utero pericardiocentesis. Pediatr Cardiol 31(6):891–893PubMedCrossRefGoogle Scholar
  15. 15.
    Demir F, Ozbarlas N, Gocen U, Buyukkurt S (2015) Prenatal diagnosis of giant left ventricular diverticulum: case report. Echocardiography 32(2):395–397PubMedCrossRefGoogle Scholar
  16. 16.
    Nam K, Kwon J, Son G, Cho N, Park Y, Kim Y (2010) Prenatally diagnosed left ventricular diverticulum with thoracoabdominal wall defect: a case and review of the literature. J Perinatol 30(11):760–762PubMedCrossRefGoogle Scholar
  17. 17.
    Grethel E, Hornberger L, Farmer D (2007) Prenatal and postnatal management of a patient with pentalogy of Cantrell and left ventricular aneurysm: a case report and literature review. Fetal Diagn Ther 22(4):269–273PubMedCrossRefGoogle Scholar
  18. 18.
    Matias A, Fredouille C, Nesmann C, Azancot A (1999) Prenatal diagnosis of left ventricular aneurysm: a report of three cases and a review. Cardiol Young 9(2):175–184PubMedCrossRefGoogle Scholar
  19. 19.
    McElhinney D, Silverman N (1999) Left ventricular aneurysm in the fetus: a diagnosis with a mixed prognosis. Cardiol Young 9(2):123–126PubMedCrossRefGoogle Scholar
  20. 20.
    Bernasconi A, Delezoide A, Menez F, Vuillard E, Oury J, Azancot A (2004) Prenatal rupture of a left ventricular diverticulum: a case report and review of the literature. Prenat Diagn 24(7):504–507PubMedCrossRefGoogle Scholar
  21. 21.
    Oloron P, Ibarra C, Galdeano Miranda J (2011) Right ventricular outpouching associated with a ventricular septal defect: case report. Pediatr Cardiol 32(8):1269–1270PubMedCrossRefGoogle Scholar
  22. 22.
    Del Rio M, Martinez J, Bennasar M, Palacio M, Figueras F, Puerto B, Mortera C, Cararach V (2005) Prenatal diagnosis of a right ventricular diverticulum complicated by pericardial effusion in the first trimester. Ultrasound Obstet Gynecol 25(4):409–411PubMedCrossRefGoogle Scholar
  23. 23.
    Jacobson R, Perez A, Meyer R, Miodovnik M, Siddigi T (1991) Prenatal diagnosis of fetal left ventricular aneurysm: a case report and review. Obstet Gynecol 78(3 Pt 2):525–528PubMedGoogle Scholar
  24. 24.
    Sharma J, Oforl-Amando G, Marboe C, Quaegebeur J (2002) Congenital left ventricular aneurysm with pericardial effusion: prenatal diagnosis, surgical management and follow-up. Pediatr Cardiol 23(4):458–461PubMedCrossRefGoogle Scholar
  25. 25.
    Seo D, Won H, Ko J, Jhang W (2011) Modified damus-kaye-stansel/dor procedure for a newborn with severe left ventricular aneurysm. Korean Circ J 41(8):494–496PubMedPubMedCentralCrossRefGoogle Scholar
  26. 26.
    Hirose A, Maeno Y, Suda K, Fusazaki N, Kado H, Matsuishi T (2013) Serial hemodynamic assessment using Doppler echocardiography in a fetus with left ventricular aneurysm presented as fetal hydrops. J Perinatol 33(6):486–489PubMedCrossRefGoogle Scholar
  27. 27.
    Davidson A, Witeman V, Gaynor J (2006) Images in cardiovascular medicine fetal cardiac diverticulum. Circulation 113(4):e56PubMedCrossRefGoogle Scholar
  28. 28.
    Conway J, Hancock Friesen C, Thompson D, Warren A (2008) Fetal diagnosis of an “extra cardiac chamber”. Pediatr Cardiol 29(1):188–190PubMedCrossRefGoogle Scholar
  29. 29.
    Erek E, Odemis E, Tanidir I (2013) Right ventricular diverticulum and associated cyst. Pediatr Cardiol 34(8):2093–2095PubMedCrossRefGoogle Scholar
  30. 30.
    El Kady D, Gersovich E, Moon-Grady A, Towner D, McGahan J, Rhee-Morris L, Naderi S (2005) Congenital cardiac left ventricular aneurysm with pericardial effusion: early prenatal diagnosis and intervention. J Ultrasound Med 24(7):1011–1015PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Johnson J, Ryan G, Toi A, Smallhorn J (1996) Prenatal diagnosis of a fetal ventricular diverticulum associated with pericardial effusion: successful outcome following pericardiocentesis. Prenat Diagn 16(10):954–957PubMedCrossRefPubMedCentralGoogle Scholar
  32. 32.
    Killen S, Mouledoux J, Kavanaugh-McHugh A (2014) Pediatric prenatal diagnosis of congenital heart disease. Curr Opin Pediatr 26(5):536–545PubMedCrossRefPubMedCentralGoogle Scholar
  33. 33.
    Carles D, Maugey-Laulom B, Habboud H, Alberti E, Weichhold W, Leger F (1995) Early prenatal diagnosis of ventricular diverticulum complicated by serous pericardial effusion. Prenat Diagn 15(8):778–780PubMedCrossRefPubMedCentralGoogle Scholar
  34. 34.
    Cesko I, Hajdu J, Csapo Z, Toth T, Sipos B, Papp Z (1998) Fetal hydropericardium associated with left ventricular diverticulum. Prenat Diagn 18(7):721–724PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Sepulveda W, Drysdale K, Kyle P, McNeal A, Moore I (1996) Congenital left ventricular aneurysm causing hydrops fetalis: prenatal diagnosis with color Doppler ultrasonography. J Ultrasound Med 15(4):327–331PubMedCrossRefPubMedCentralGoogle Scholar
  36. 36.
    Papagiannis J, Van Praagh R, Schwint O, D’Orsogna L, Qureshi F, Reynolds J, Kallfelz C, Nozar J (2001) Congenital left ventricular aneurysm: clinical imaging, pathologic and surgical findings in seven new cases. Am Heart J 141(3):491–499PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    Cavalle-Garrido T, Cloutier A, Harder J, Boutin C, Smallhorn J (1997) Evolution of fetal ventricular aneurysms and diverticula of the heart: an echocardiographic study. Am J Perinatol 14(7):393–400PubMedCrossRefPubMedCentralGoogle Scholar
  38. 38.
    Sherman S, Leenhouts K, Utter G, Litaker M, Lawson P (1996) Prenatal diagnosis of left ventricular aneurysm in the late second trimester: a case report. Ultrasound Obstet Gynecol 7(6):456–457PubMedCrossRefPubMedCentralGoogle Scholar
  39. 39.
    Chaubal N, Dighe M, Shah M, Chaubal J, Raghavan J (2004) Congenital left ventricular aneurysm: prenatal sonographic diagnosis. J Ultrasound Med 23(1):125–128PubMedCrossRefPubMedCentralGoogle Scholar
  40. 40.
    Jowett V, Miller O (2011) Prenatal diagnosis of left ventricular aneurysm in association with interruption of the aortic arch. Cardiol Young 21(1):113–115PubMedCrossRefGoogle Scholar
  41. 41.
    Vernon M, Rutledge J, Kemma M (2014) In utero evolution of an apical left ventricular aneurysm. Circulation 130(22):1982–1983PubMedCrossRefGoogle Scholar
  42. 42.
    Patel C, Judge N, Muise K, Levine M (1996) Prenatal myocardial infarction suspected by fetal echocardiography. J Am Soc Echocardiogr 9(5):721–723PubMedCrossRefGoogle Scholar
  43. 43.
    Weichert J, Chiriac A, Axt-Fliedner R (2010) Fetal diagnosis of left ventricular aneurysm of the free wall and the interventricular septum: report of two cases and review of the literature. J Matern Fetal Neonatal Med 23(12):1510–1515PubMedCrossRefGoogle Scholar
  44. 44.
    Kitchiner D, Leung M, Arnold R (1990) Isolated congenital left ventricular diverticulum: echocardiographic features in a fetus. Am Heart J 119(6):1435–1437PubMedCrossRefGoogle Scholar
  45. 45.
    Brachlow A, Sable C, Smith S, Slack M, Martin G (2002) Fetal diagnosis and postnatal follow-up of an asymptomatic congenital left ventricular diverticulum. Pediatr Cardiol 23(6):658–660PubMedCrossRefGoogle Scholar
  46. 46.
    Pradhan M, Dalal A, Kapoor A, Kumar S, Manisha A (2008) Fetal left ventricular diverticulum presenting as dysrhythmia: diagnosis and management. Fetal Diagn Ther 23(1):10–14PubMedCrossRefGoogle Scholar
  47. 47.
    Paoletti D, Robertson M (2012) Prenatal diagnosis of a left ventricular diverticulum. Am J Ultrasound Med 15(3):112–114CrossRefGoogle Scholar
  48. 48.
    Peters C, Wacker-Gussmann A, Strasburger J, Cuneo B, Gotteiner N, Gulecyuz M, Wakai R (2015) Electrophysiologic features of fetal ventricular aneurysms and diverticula. Prenat Diagn 35(2):129–136PubMedCrossRefGoogle Scholar
  49. 49.
    Gembruch U, Steil E, Redel D, Hansmann M (1990) Prenatal diagnosis of a left ventricular aneurysm. Prenat Diagn 10(3):203–209PubMedCrossRefGoogle Scholar
  50. 50.
    Hornberger L, Dalvi B, Benacerraf B (1994) Prenatal sonographic detection of cardiac aneurysms and diverticula. J Ultrasound Med 13(12):967–970PubMedCrossRefGoogle Scholar
  51. 51.
    Pipitone S, Sperandeo V, Mongiovi M, Roberto G, Centineo G (2002) Prenatal diagnosis of ventricular aneurysm: a report of two cases and a review. Prenat Diagn 22(2):131–136PubMedCrossRefGoogle Scholar
  52. 52.
    Chiang Y, Yang C, Shih J, Lee C (2006) Prenatal diagnosis of congenital left ventricular aneurysm by four-dimensional ultrasonography with spatio-temporal image correlation (STIC). Ultrasound Obstet Gynecol 28(3):345–347PubMedCrossRefGoogle Scholar
  53. 53.
    Balakumar K (2009) Prenatal diagnosis of left ventricular aneurysm. Indian J Radiol Imaging 19(1):84–86PubMedPubMedCentralCrossRefGoogle Scholar
  54. 54.
    Fujita Y, Hidaka N, Yumoto Y, Morihana E, Fukushima K, Wake N (2012) Measurement of the fetal isovolumetric contraction time in the fetus with a left ventricular aneurysm. J Obstet Gynaecol Res 38(3):586–588PubMedCrossRefGoogle Scholar
  55. 55.
    Gardiner H, Wimalasundera R, Pasquini L, Wawryk S, Ho S (2005) Images in cardiovascular medicine. Pericardiocentesis at 14 weeks: effective treatment of pericardial effusion complicating right ventricular diverticulum. Circulation 112(9):e120PubMedCrossRefGoogle Scholar
  56. 56.
    Koshiishi T, Osada H, Hata A, Furugen Y, Murakoshi T, Mitsuhashi N (2007) Prenatal rupture of right ventricular diverticulum: a case report and review of the literature. Prenat Diagn 27(12):1154–1157PubMedCrossRefGoogle Scholar
  57. 57.
    John J, Bricker J, Fenrich A, Vick G, El-Said H, Ayres N, Bezold L (2002) Images in cardiovascular medicine. Fetal diagnosis of right ventricular aneurysm associated with supraventricular tachycardia with left bundle-branch block aberrancy. Circulation 106(1):141–142PubMedCrossRefGoogle Scholar
  58. 58.
    McCaffrey F (2002) Around PediHeart: aneurysm of the right ventricular apex in a 24-week fetus. Pediatr Cardiol 23(1):8PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Jacqueline M. Shuplock
    • 1
    Email author
  • Ann Kavanaugh-McHugh
    • 1
  • David Parra
    • 1
  1. 1.Thomas P. Graham Jr. Division of Pediatric CardiologyMonroe Carell Jr. Children’s Hospital at VanderbiltNashvilleUSA

Personalised recommendations