Abstract
We report a 62-year-old man with a past medical history of dyslipidemia, paranoid schizophrenia and permanent atrial fibrillation. A ATTE performed at his district hospital revealed rheumatic mitral valve disease with double lesion: severe regurgitation and mild stenosis, plus moderate tricuspid regurgitation and a mean PAP of 32mm Hg. Cardiac catheterization showed no abnormalities of the coronary arteries.
He was transferred to our hospital and scheduled for mitral valve replacement and tricuspid ring valvuloplasty. Preoperative transesophageal echocardiography showed an abnormal subvalvular mitral apparatus, with false tendons and multiple papillary muscles, resembling a hammock mitral valve. Most cordae tendinae arose from a single dominant papillary muscle at a posterior medial region, which provoke severe mitral regurgitation due to coaptation defect and mild subvalvular mitral stenosis. It could also be appreciated hypertrabeculation in the lateral medial, basal and apical segments. This suggested no-compaction cardiomyopathy associated with hammock mitral valve. Left ventricular systolic function was preserved. No evidence of rheumatic mitral valve disease was found in transesophageal echocardiographic study performed at our hospital.
On the 30th April 2019 he underwent mechanic mitral valve replacement (Bicarbon 29mm) and tricuspid ring valvuloplasty (Edwards Physio 32mm) surgery. Once the patient was weaned from cardiopulmonary bypass, severe left ventricle systolic dysfunction ensued, predominantly localized in the anterior, inferior septal, inferior lateral basal and medial segments. Apical segments had preserved mobility An adrenalin infusion prior weaning from CBP was initiated. Preserved mobility of the mitral prosthesis discs was observed. The patient developed cardiogenic shock in spite of high doses of dobutamin and adrenaline infused. IACB was implanted with 1:1 assistance. The patient was transfered to the hemodynamic room in order to rule out coronary complications. Cardiac catheterization showed no significant angiographic lesions.
During the first postoperative hours, the patient was stabilized allowing progressive lowering of the drugs (adrenaline, dobutamine). TTE showed normally functioning prosthetic mitral valve and preserved left ventricle systolic function. An MRI was performed demostrating no-compaction cardiomyopathy
Conclusion
This case report describes a rare presentation of simultaneous ocurrence of hammock mitral valve and no-compaction cardiomyopathy.
Perioperative left ventricle dysfunction in no-compaction cardiomyopathy is related to subendocardial ischemia caused during extracorporeal circulation in the multiple prominent ventricular trabeculations with deep intertrabecular recesses corresponding to non-compacted myocardium .This must be taken account in those patients with no-compaction cardiomyopathy scheduled for cardiac surgery in order to take preventive measures.
Abstract 89 Figure. non - compacted myocardium
89 Simultaneous ocurrence of hammock mitral valve and no-compaction cardiomyopathy