Scientific Online Resource System

Izvestia Journal of the Union of Scientists - Varna. Medicine and Ecology Series

Clinical case of a noncompaction cardiomyopathy with restrictive filling diastolic dysfunction in a 5-year-old girl

Kalina Ganeva, Petar Shivachev, Milena Avdzhiyska, Martin Boyadzhiev

Abstract

Noncompaction cardiomyopathy is a rare cardiomyopathy, which is characterized by trabeculations and deep intratrabecular recesses, forming a sponge-like appearance of the myocardium. Clinical variability is possible – from asymptomatic patients to congestive heart failure, tromboembolic complications, and even sudden cardiac death. Two-dimensional echocardiography is the preferred diagnostic tool, based on specific criteria, which are not yet definite. Discovery of specific genetic mutations support the diagnosis. The main therapeutic approach is symptomatic – treatment of congestive heart failure and anticoagulant medications.

We present a case of a 5-year-old girl, without a family history of chronic diseases, who was referred to our department because of a fever, cough and bilateral pneumonia. Upon admission she was with impaired general condition, with signs of respiratory and congestive heart failure. Echocardiography examination demonstrated noncompaction cardiomyopathy, mostly for the left ventricle, with leading diastolic left ventricular dysfunction with restrictive filling pattern, evaluated by tissue doppler, and pulmonary hypertension.

This clinical case demonstrates the rare combination of noncompaction cardiomyopathy with restrictive left ventricular filling pattern, clinically manifested by congestive heart failure and pulmonary hypertension.


Keywords

noncompaction cardiomyopathy, diastolic dysfunction, pediatric population

Full Text


References

Витлиянова К, Ташева Р, Негрева М, Клинична ехокардиография, София 2018:305-306

Bennett C, Freudenberger R; The current approach to diagnosis and management of left ventricular noncompaction cardiomyopathy: review of the literature; Cardiology Research and practice, Vol 2016, Article ID 5172308

Chin T.K, Perloff R et al; Isolated noncompaction of left ventricular myocardium. A study of eight cases, Circulation, vol. 82, no. 2, pp. 507–513, 1990

Haas N, Kleiderter U; Pediatric cardiology Symptoms-Diagnosis-Treatment; 2015:270-271

Jefferies J, Wilkinson J et al; Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the pediatric cardiomyopathy registry; J Card Fail.2015 Nov; 21(11):877-84.

Jenni R, Oechslin E et al.; Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy, Heart, vol. 86, no. 6, pp. 666–671, 2001

Kido K, Guglin M, Anticoagulation therapy in specific cardiomyopathies: isolate left ventricular noncompaction and peripartum cardiomyopathy; J Cardiovasc Pharmacol Ther. 2019 Jan; 24(1):31-36.

Malcic I, Jelusic M et al. Epidemiology of cardiomyopathies in children and adolescents: a retrospective study over the last 10 years; Cardiol Young. 2012; 12(3):253-9.

Maron BJ, Towbin JA et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006;113:1807–16

Nunez-Gil I, Feltes-Guzman G, Left ventricular noncompaction, e-journal of the ESC Council for Cardiology Practice, Vol.10, №31-26 Jun 2012.

Ritter M, Oechslin E et al, Isolated noncompaction of the myocardium in adults, Mayo Clin Proc 1997;72:26-3

Stöllberger C, Finsterer J, Left ventricular hypertrabeculation/noncompaction, Journal of the American Society of Echocardiography, vol. 17, no. 1, pp. 91–100, 2004.




DOI: http://dx.doi.org/10.14748/isuvsme.v24i2.6196

Refbacks

Font Size


|