This is a 33-year-old woman admitted for evaluation for a heart transplantation. She was admitted with functional class IV of NYHA. There was an initial suspicion of muscular dystrophy due to neuromuscular alterations.
Regrettably, I don’t have plenty of clinical data since the patient died and the family did not agree to an autopsy.
Transthoracic Echo; LV = 62/52; LA=53 mm, Mass 307 g, EF=31%, right chambers and pulmonary artery trunk without anomalies, the LV presented marked trabeculation in the lateral wall suggestive of Left Ventricular Noncompaction (LVNC) cardiomyopathy.
Mild mitral valve insufficiency.
Which is the ECG diagnosis?
Which is the clinical diagnosis: Muscular dystrophy? Noncompacted LV myocardium? Both?
Note: other family members died with similar symptoms (being young)
Trata-se de uma mulher de 33 anos que foi internada para avaliação de transplante cardíaco. Admitida com ICC classe funcional IV.Havia uma suspeita inicial de distrofia muscular devido alterações neuro musculares.
ECO transtorácico; VE=62/52,AE=53mm,Massa 307g,FE=31%,câmaras direitas e TAP sem anormalidades,VE apresentando acentuada trabeculação na parede lateral sugestiva de miocárdio não compactado.I. mitral leve.
Distrofia muscular? Mioc. VE não compactado? Ambas?
Observação: outros membros da família faleceram com quadro semelhante (jovens)