Case Report
Isolated ventricular noncompaction: a case report
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* Corresponding author: João Bento joao.a.p.bento@gmail.com
1 Pulmonology Department, Hospital S João, Alameda Professor Hernâni Monteiro 4300-319, Porto, Portugal
2 Respiratory Intensive Care Unit, Pulmonology Department, Hospital Santa Maria, Avenida Professor Egas Moniz, Cidade Universitária, 1649-028 Lisboa, Portugal
3 Lisbon Medical Faculty, Avenida Professor Egas Moniz (Hospital Santa Maria), Cidade Universitária, 1649-028, Lisboa, Portugal
4 Cardiology Department, Hospital Santa Maria, Avenida Professor Egas Moniz, Cidade Universitária, 1649-028 Lisboa, Portugal
Cases Journal 2009, 2:9312 doi:10.1186/1757-1626-2-9312
Published: 11 December 2009Abstract
Isolated ventricular noncompaction is an extremely rare cardiomyopathy, not fully clarified.
It is characterized by persistent embryonic myocardium morphology without associated cardiac abnormalities.
Since first description in 1984, few clinical studies were done. Data in the literature are lacking and most reports consist on a few case studies.
Doppler ecocardiogram is considered the reference method for diagnosis.
Diagnosis remains difficult since there are similarities with other cardiac defects, clinical manifestations are non-specific and echocardiographic criteria are not universally accepted.
As a consequence diagnosis may be easily missed.
Moreover, clinical and echocardiographic features were just recently clarified.
Treatment is directed towards important clinical manifestations (heart failure, arrhythmias and embolic events).
We present a clinical case of severe cardio-respiratory failure in previously healthy and asymptomatic young male, which was the initial presentation of an isolated ventricular noncompaction.
A brief review of available literature is done concerning to this case study.