A 54-year-old man presented with extrinsic bronchial asthma and was diagnosed 4 years ago by transthoracic echo (TTE) of obstructive non-obstructive cardiomyopathy with mild mitral regurgitation seated.
She performed an active life and was in functional class I.
He came to the emergency room for presenting a picture of precordial pain after performing an effort with subsequent onset of episodes.
A first electrocardiogram (ECG) showed regular tachycardia with wide QRS at 210 bpm, which did not respond to treatment with adenosine or vagal maneuvers.
Electrical cardioversion was performed with 100 J resulting in ventricular fibrillation (VF) reversed with two 360 J shocks. Treatment with intravenous amiodarone was initiated but sinus rhythm was stopped shortly after 40 minutes.
Magnesium sulfate was administered empirically and the patient was admitted to the Coronary Unit.
Physical examination revealed a systemic blood pressure of 130/70 mmHg.
A systolic murmur in apex and edge was auscultated and showed no signs of pulmonary congestion.
The ECG showed sinus dysfunction, wide QRS with morphology of complete left bundle branch block and normal QT interval.
In the laboratory analysis at 30 hours of evolution of symptoms highlighted a total CPK of 2,657 U/l and troponin I of 2.21 ng/ml.
Sepsis was normal.
The echocardiogram performed on admission with a portable device showed a highly dilated and globally hypokinetic left ventricle (LV), with very hypertrophic myocardium, with trabeculations.
As part of the study protocol for ventricular tachycardia (VT), with clinical suspicion of cardiomyopathy, a hemodynamic study was performed.
Left ventriculography showed a ventricle with an ejection fraction (EF) of 20%, with generalized contractility defects and an image of significant trabeculation suggesting the diagnosis of non-compaction spongiform cardiomyopathy (NCD).
No angiographically significant mitral regurgitation was detected.
In the right catheterization, the pressures were: right atrium (RA) 10 mmHg, right ventricle (RV) 70/4 mmHg, pulmonary artery (PA) 70/15 mmHg, pulmonary capillary wedge (PECP) 15 mmHg.
Cardiac arrest 2 min/m2; end-diastolic pressure LV (LVDP) 30 mmHg; inverted mitral gradient (LVPT > CPET).
Coronary angiography showed normal epicardial coronary arteries.
With the suspected diagnosis of NCC the patient underwent a second echocardiography by specialized personnel.
2D echocardiography confirmed the presence of marked LV trabeculation, located in the apex and middle segments of the lateral and inferior wall, with a telesystolic ratio of 2.5 between the thick inner layer of myocardium (NC).
Color Doppler showed blood flow in communication between the deep intertrabecular recesses and the ventricular cavity.
Mitral diastolic flow showed a restrictive pattern.
The LV morphology was calculated as systolic pressure 55 mm and gradient in telediagram 70 mm, left atrium 47 mm and right ventricle 30 mm. A mitral regurgitation flow was assessed as moderate mitral regurgitation 65 mm.
Structural valve anomalies were not detected.
Contrast-enhanced echocardiography was not considered necessary given the good quality of the two-dimensional images.
With these findings, the diagnosis of NCC was made according to Jenni's criteria, due to the absence of compaction of the left ventricular myocardium without other associated cardiac anomalies2,3.
Although cardiac magnetic resonance was interesting, it could not be performed because this technique was not available in our hospital4,5.
