Seguimiento Ecocardiográfico De Cambios Anatómicos Del Miocardio De Pacientes Pediátricos con Diagnóstico De Miocardiopatía No Compactada.  2017 – 2021: Echocardiographic Monitoring of Anatomical Changes in the Myocardium of Pediatric Patients with a Diagnosis of Non-Compaction Cardiomyopathy.  2017 – 2021

María  Loo-Valverde; Vivian Tuesta-García; Michael Zárate-Quispe; Consuelo  Luna-Muñoz; Lucy Correa-Lopez; Willer  Chanduví-Puicón

doi:10.25176/RFMH.v24i4.6688

Authors

María Loo-Valverde Instituto de Investigación de Ciencias Biomédicas
Vivian Tuesta-García Hospital Nacional Edgardo Rebagliati Martins
Michael Zárate-Quispe Hospital Nacional Edgardo Rebagliati Martins
Consuelo Luna-Muñoz Instituto de Investigación de Ciencias Biomédicas
Lucy Correa-Lopez Instituto de Investigación de Ciencias Biomédicas
Willer Chanduví-Puicón Instituto de Investigación de Ciencias Biomédicas

DOI:

https://doi.org/10.25176/RFMH.v24i4.6688

Keywords:

Non-compaction cardiomyopathy, Pediatrics, survival.

Abstract

Background:

Non-compaction cardiomyopathy (NCM) is known as the lack of maturation of the myocardium that is evidenced by prominent trabeculations and the presence of recesses in newborns and infants that can manifest clinically as heart failure.

The purpose of the research is to evaluate the anatomical development of pediatric patients diagnosed with NCM at an early age.

Clinical Case: Retrospective case series study of patients diagnosed with non-compaction cardiomyopathy during the first year of life, confirmed according to Jenni criteria, with echocardiographic follow-up every 6 months, from 2017 - 2021. The patients were diagnosed in the first months of life. life, 3 had associated heart disease, 3 had arrhythmias, 3 died and 1 abandoned the controls at 2 months of age. In the 3 patients who remained, it was evident that the ratio of non-compacted myocardium (NC) / compacted myocardium (C) only remained high in 1 patient. The ejection fraction (EF) improved in all patients, the number of trabeculae only decreased in one patient.

Conclusions: Anatomical changes in patients diagnosed with non-compaction cardiomyopathy can evolve to myocardial maturation and improvement in ventricular function.

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Author Biographies

María Loo-Valverde, Instituto de Investigación de Ciencias Biomédicas

Cardiólogo Pediatra Hospital Nacional Edgardo Rebagliati Martins

Vivian Tuesta-García, Hospital Nacional Edgardo Rebagliati Martins

Pediatric Cardiologist

Michael Zárate-Quispe, Hospital Nacional Edgardo Rebagliati Martins

Pediatric Cardiologist

References

Kádár K, Tóth A, Tóth L, Símor T. Csecsemo- és gyermekkori

noncompacted (embrionális) cardiomyopathia. Klinikai sajátosságok és

diagnosztikai lehetoségek [Noncompacted cardiomyopathy in infants and

children. Clinical findings and diagnostic techniques]. Orv Hetil. 2010 Apr

;151(16):659-64. https://doi.org/10.1556/OH.2010.28734.

Udeoji DU, Philip KJ, Morrissey RP, Phan A, Schwarz ER. Left ventricular

noncompaction cardiomyopathy: updated review. Ther Adv Cardiovasc

Dis. 2013 Oct;7(5):260-73. https://doi.org/10.1177/1753944713504639

Adabifirouzjaei F, Igata S, DeMaria AN. Hypertrabeculation; a phenotype

with Heterogeneous etiology. Prog Cardiovasc Dis. 2021 Sep-Oct; 68:60-

https://doi.org/10.1016/j.pcad.2021.07.007.

Zhang J, Wang Y, Feng W, Wu Y. Prenatal ultrasound diagnosis of fetal

isolated right ventricular noncompaction with pulmonary artery sling: A rare

case report. Echocardiography. 2019 Nov;36(11):2118-2121.

https://doi.org/10.1111/echo.14528

Mongiovì M, Fesslova V, Fazio G, Barbaro G, Pipitone S. Diagnosis and

prognosis of fetal cardiomyopathies: a review. Curr Pharm Des.

;16(26):2929-34. https://doi.org/10.2174/138161210793176428.

Lilje C, Rázek V, Joyce JJ, Rau T, Finckh BF, Weiss F, Habermann CR,

Rice JC, Weil J. Complications of non-compaction of the left ventricular

myocardium in a paediatric population: a prospective study. Eur Heart J.

Aug;27(15):1855-60. https://doi.org/10.1093/eurheartj/ehl112.

Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA.

Echocardiographic and pathoanatomical characteristics of isolated left

ventricular non-compaction: a step towards classification as a distinct

cardiomyopathy. Heart. 2001 Dec;86(6):666-71.

http://dx.doi.org/10.1136/heart.86.6.666

Kogaki S. Left Ventricular Noncompaction Develops Even in Late Fetal

Life -The Need for Fetus-Specific Diagnostic Criteria. Circ J. 2021 Dec

;86(1):106-108. https://doi.org/10.1253/circj.CJ-21-0730.

M. Passerini, G. Prado et al., 2019. Miocardiopatía no compactada.

Anuario (Fund. Dr. J. R. Villavicencio). 27:151-154.

http://www.villavicencio.org.ar/ALMACEN/archivos/publicaciones_0000000

pdf

Mallavarapu A, Taksande A. Dilated Cardiomyopathy in Children: Early

Detection and Treatment. Cureus. 2022 Nov 4;14(11):e31111. doi:

7759/cureus.31111.

Dhar R, Reardon W, McMahon CJ. Biventricular non-compaction

hypertrophic cardiomyopathy in association with congenital complete heart

block and type I mitochondrial complex deficiency. Cardiol Young. 2015

Jun;25(5):1019-21. DOI: https://doi.org/10.1017/S1047951114001279

Menon SC, O'Leary PW, Wright GB, Rios R, MacLellan-Tobert SG,

Cabalka AK. Fetal and neonatal presentation of noncompacted ventricular

myocardium: expanding the clinical spectrum. J Am Soc Echocardiogr.

Dec;20(12):1344-50. https://doi.org/10.1016/j.echo.2007.04.028.

Arunamata A, Punn R, Cuneo B, Bharati S, Silverman NH.

Echocardiographic diagnosis and prognosis of fetal left ventricular

noncompaction. J Am Soc Echocardiogr. 2012 Jan;25(1):112-20.

https://doi.org/10.1016/j.echo.2011.09.019

Echocardiographic Monitoring of Anatomical Changes in the Myocardium of Pediatric Patients with a Diagnosis of Non-Compaction Cardiomyopathy. 2017 – 2021