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Neonatal Non-compacted Cardiomyopathy: Predictors of Poor Outcome

  • Javier Rodriguez-Fanjul
  • Sheila Tubio-Gómez
  • Juan Manuel Carretero BellónEmail author
  • Carles Bautista-Rodríguez
  • Joan Sánchez de Toledo
Original Article

Abstract

Non-compacted cardiomyopathy (NCM) is a heterogenous myocardial disorder. Although much has been published in recent years, little is known about NCM in the neonatal period. The objective of this study is to characterize the involvement of newborns affected with NCM and to identify risk factors associated with increased mortality. This is a retrospective study including all neonates diagnosed with NCM between 2006 and 2018. Diagnosis was based on echocardiographic findings. Data were collected regarding prenatal history, gestational age and weight at birth, gender, age at diagnosis, left or biventricular involvement and associated malformations, medical and surgical treatments, and evolution. Fourteen patients were included. The median follow-up duration was 34 months (range 1–87 months). The left ventricular apex and lateral wall were involved in all cases (100%). Thirteen patients (92.8%) had other associated heart malformations. Six patients (42.8%) died during the follow-up period. Patients who had biventricular involvement and poor ventricular function presented a higher risk of death. The main cause of death was ventricular dysfunction (5/6 [83.3%]). During follow-up, eight patients (57.1%) underwent surgery for their cardiac malformations, without higher mortality. NCM must be included in the differential diagnosis of neonatal cardiomyopathy. The higher mortality observed in our series is related not only to the high association with congenital heart disease, but also to a greater presence of early and severe left ventricular dysfunction. We did not find that patients who underwent surgery with cardiopulmonary bypass had worse outcomes.

Keywords

Non-compacted cardiomyopathy Newborn Mortality Left ventricular dysfunction 

Notes

Acknowledgements

The authors thank Mrs. Stephanie M. Hadley from the Vanderbilt University School of Medicine, Nashville, Tennessee, USA, for a thorough revision of the English language.

Funding

No funding was received for this work.

Compliance with Ethical Standards

Conflict of interest

Authors declare that they have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

References

  1. 1.
    Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82:507–513CrossRefGoogle Scholar
  2. 2.
    Almeida A, Pinto F (2013) Non-compaction cardiomyopathy. Heart 99:1535–1542CrossRefGoogle Scholar
  3. 3.
    Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, Watkins H, Neubauer S (2005) Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 46:101–105CrossRefGoogle Scholar
  4. 4.
    Oechslin E, Jenni R (2011) Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart 32:1446–1455CrossRefGoogle Scholar
  5. 5.
    Ichida F, Tsubata S, Bowles KR, Haneda N, Uese K, Miyawaki T, Dreyer WJ, Messina J, Li H, Bowles NE, Towbin JA (2001) Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation 103:1256–1263CrossRefGoogle Scholar
  6. 6.
    van Waning JI, Caliskan K, Michels M, Schinkel AFL, Hirsch A, Dalinghaus M, Hoedemaekers YM, Wessels MW, Jpam AS, Hofstra RNW, van Slegtenhorst MA, Majoor-Krakauer D (2019) Cardiac phenotypes, genetics, and risks in familiar noncompaction cardiomyopathy. J Am Coll Cardiol 73(13):1601–1611CrossRefGoogle Scholar
  7. 7.
    Klaassen S, Probst S, Oechslin E, Gerull B, Krings G, Schuler P, Greutmann M, Hürlimann D, Yegitbasi M, Pons L, Gramlich M, Drenckhahn JD, Heuser A, Berger F, Jenni R, Thierfelder L (2008) Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation 117:2893–2901CrossRefGoogle Scholar
  8. 8.
    Borges AC, Kivelitz D, Baumann G (2003) Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 89:e21CrossRefGoogle Scholar
  9. 9.
    Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH (2005) Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 46(1):101–105CrossRefGoogle Scholar
  10. 10.
    Stahli BE, Gebhard C, Biaggi P, Klassen S, Valsangiacomo BE, Attenhofer Jost CH, Jenni R, Tanner FC, Greutmann M (2013) Left ventricular non-compaction: prevalence in congenital heart disease. Int J Cardiol 167(6):2477–2481CrossRefGoogle Scholar
  11. 11.
    Cremer K, Lüdecke H-J, Ruhr F, Wieczorek D (2008) Left-ventricular non-compaction (LVNC): a clinical feature more often observed in terminal deletion 1p36 than previously expected. Eur J Med Genet 51(6):685–688CrossRefGoogle Scholar
  12. 12.
    Kanemoto N, Horigome H, Nakayama J, Ichida F, Xing Y, Buonadonna AL, Kanemoto K, Gentile M (2006) Interstitial 1q43-q43 deletion with left ventricular noncompaction myocardium. Eur J Med Genet 49(3):247–253CrossRefGoogle Scholar
  13. 13.
    Pauli RM, Scheib-Wixted S, Cripe L, Izumo S, Sekhon GS (1999) Ventricular noncompaction and distal chromosome 5q deletion. Am J Med Genet 85(4):419–423CrossRefGoogle Scholar
  14. 14.
    Blinder JJ, Martinez HR, Craigen WJ, Belmont J, Pignatelli RH, Jefferies JL (2011) Noncompaction of the left ventricular myocardium in a boy with a novel chromosome 8p231 deletion. Am J Med Genet A 155A(9):2215–2220CrossRefGoogle Scholar
  15. 15.
    McMahon CJ, Chang AC, Pignatelli RH, Miller-Hance WC, Eble BK, Towbin JA, Denfield SW (2005) Left ventricular noncompaction cardiomyopathy in association with trisomy 13. Pediatr Cardiol 26(4):477–479CrossRefGoogle Scholar
  16. 16.
    Beken S, Cevik A, Turan O, Hirfanoglu IM, Unal S, Altuntas N, Pektas TC, Tunaoglu S (2011) A neonatal case of left ventricular noncompaction associated with trisomy 18. Genet Couns 22(2):161–164PubMedGoogle Scholar
  17. 17.
    Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, Craigen WJ, Wu J, El Said H, Bezold LI, Clunie S, Fernbach S, Bowles NE, Towbin JA (2003) Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 108(21):2672–2678CrossRefGoogle Scholar
  18. 18.
    Madan S, Madan-Khetarpal S, Park SC, Surti U, Bailey AL, McConnell K, Tadros SS (2010) Left ventricular non-compaction on MRI in a patient with 22q112 distal deletion. Am J Med Genet A 152A(5):1295–1299CrossRefGoogle Scholar
  19. 19.
    Van Heerde M, Hruda J, Hazekamp MG (2003) Severe pulmonary hypertension secondary to a parachute-like mitral valve, with the left superior caval vein draining into the coronary sinus, in a girl with Turner’s syndrome. Cardiol Young 13(4):364–366CrossRefGoogle Scholar
  20. 20.
    Digilio MC, Bernardini L, Gagliardi MG, Versacci P, Baban A, Capolino R, Dentici ML, Roberti MC, Angioni A, Novelli A, Marino B, Dallapiccola B (2013) Syndromic non-compaction of the left ventricle: associated chromosomal anomalies. Clin Genet 84(4):362–367CrossRefGoogle Scholar
  21. 21.
    Tian Y, Yang Y, Zhou L, Luo F, Li Y, Fan P, Dong X, Liu Y, Cui J, Zhou X (2018) Left ventricular non-compaction: a cardiomyopathy with acceptable prognosis in children. Heart Lung Circ 27(1):28–32CrossRefGoogle Scholar
  22. 22.
    Habib G, Charron P, Eicher JC, Giorgi R, Donal E, Laperche T, Boulmier D, Pascal C, Logeart D, Jondeau G, Cohen-Solal A (2011) Isolated left ventricular non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail 13(2):177–185CrossRefGoogle Scholar
  23. 23.
    Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E, Papra E, Kiotsekoglou A, Tome MT, Pellerin D, Mckenna WJ, Elliot PM (2005) Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J 26(2):187–192CrossRefGoogle Scholar
  24. 24.
    Towbin JA, Lorts A, Jefferies JL (2015) Left ventricular non-compaction cardiomyopathy. Lancet 386(9995):813–825CrossRefGoogle Scholar
  25. 25.
    Aras D, Tufekcioglu O, Ergun K, Ozeke O, Yildiz A, Topalogu S, Deveci B, Sahin O, Kisacik HL, Korkmaz S (2006) Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail. 12(9):726–733CrossRefGoogle Scholar
  26. 26.
    Greutmann M, Mah ML, Silversides CK, Klaassen S, Attenhofer Jost CH, Jenni R, Oechslin EN (2012) Predictors of adverse outcome in adolescents and adults with isolated left ventricular noncompaction. Am J Cardiol 109(2):276–281CrossRefGoogle Scholar
  27. 27.
    McMahon C, Murchan E, Prendiville E, Burch M (2007) Long-term support with milrinone prior to cardiac transplantation in a neonate with left ventricular noncompaction cardiomyopathy. Pediatr Cardiol 28:317–318CrossRefGoogle Scholar
  28. 28.
    Ramachandran P, Woo JG, Ryan TD, Bryant R, Heydarian HC, Jefferies JL, Towbin JA, Lorts A (2016) The impact of concomitant left ventricular non-compaction with congenital heart disease on perioperative outcomes. Pediatr Cardiol 37(7):1307–1312CrossRefGoogle Scholar

Copyright information

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

Authors and Affiliations

  1. 1.Pediatric Intensive Care Unit, Pediatric DepartmentHospital Joan XXIIITarragonaSpain
  2. 2.Pediatric Cardiology Department, Hospital Sant Joan de Déu-ClínicUniversity of BarcelonaBarcelonaSpain
  3. 3.Pediatric Cardiology Unit, Pediatric DepartmentHospital Joan XXIIITarragonaSpain
  4. 4.Pediatric Cardiology DepartmentRoyal Brompton HospitalLondonUK
  5. 5.Critical Care Medicine DepartmentUniversity of PittsburghPittsburghUSA

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