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.
Similar content being viewed by others
References
Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82:507–513
Almeida A, Pinto F (2013) Non-compaction cardiomyopathy. Heart 99:1535–1542
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 46:101–105
Oechslin E, Jenni R (2011) Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart 32:1446–1455
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 103:1256–1263
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):1601–1611
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 117:2893–2901
Borges AC, Kivelitz D, Baumann G (2003) Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 89:e21
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):101–105
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):2477–2481
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):685–688
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):247–253
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):419–423
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):2215–2220
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):477–479
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):161–164
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):2672–2678
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):1295–1299
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):364–366
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):362–367
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):28–32
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):177–185
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):187–192
Towbin JA, Lorts A, Jefferies JL (2015) Left ventricular non-compaction cardiomyopathy. Lancet 386(9995):813–825
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):726–733
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):276–281
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 28:317–318
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):1307–1312
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.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
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.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Rodriguez-Fanjul, J., Tubio-Gómez, S., Carretero Bellón, J.M. et al. Neonatal Non-compacted Cardiomyopathy: Predictors of Poor Outcome. Pediatr Cardiol 41, 175–180 (2020). https://doi.org/10.1007/s00246-019-02241-w
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-019-02241-w