Neonatal Non-compacted Cardiomyopathy: Predictors of Poor Outcome
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.
KeywordsNon-compacted cardiomyopathy Newborn Mortality Left ventricular dysfunction
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.
No funding was received for this work.
Compliance with Ethical Standards
Conflict of interest
Authors declare that they have no conflict of interest.
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 was obtained from all individual participants included in the study.
- 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.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
- 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
- 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
- 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