(III-5A.2.o) Accidentally discover cardiomiopathy in patient with initial acute coronary syndrom Noncompaction Cardiomiopathy Trąbka-Zawicki A. MD, Rubiś P. MD, PhD, Assoc. Prof. Kostkiewicz M. MD, PhD, Miszalski-Jamka T. MD, PhD, Tomala I. MD, Assoc. Prof. Lelakowski J. MD, PhD, Prof. Żmudka K. MD, PhD

Keywords: cardiomyopathy, initial acute coronary syndrome, Noncompaction Cardiomiopathy

Background
Isolated noncompaction cardiomyopathy is a rarely occurring, congenital anomaly that belongs to the group of cardiomyopathies. The incidence of this pathology in the general population is estimated at 0.014-1.3%. It is characterized by the presence of numerous trabeculae on the myocardial wall of both ventricles and deep intertrabecular recesses resulting in thickened myocardium.

Case presentation
We present an accidentally discovered isolated left ventricular noncompaction in the 46-year-old patient with the preliminary diagnosis of an acute coronary syndrome. Levels of cardiac necrosis markers were elevated on admission. Two days before hospitalization he suffered shock while welding at work. Past history revealed two episodes of syncope without loss of consciousness. Detailed interview revealed that working environment contained significant concentrations of toxic compounds of zinc and lead. Echocardiograms revealed enlargement of the left ventricle (LV) with decreased global contractility and ejection fraction of 20% and asynchronous contraction of the LV due to left bundle branch block (LBBB). The thickening of the left ventricular wall with numerous prominent trabeculations and deep recesses was found. Enhancement of blood in the LV was also noticeable. Coronary angiography revealed intramyocardial muscular bridging of the left anterior descending branch (LAD) narrowing the vessel at systole to 60-70%. No signs of significant atherosclerosis were detected.
Cardiac magnetic resonance (CMR) was performed, which confirmed noncompacted cardiomiopathy. In Holter monitoring two episodes of nonsustained ventricular tachycardia were observed. A suspicion was formulated regarding a possible impact of the working environment and several electric shocks, he had encountered in the past, on the failure of the heart. A cardiac resynchronization device was implanted.
In a control study, the patient did not complain of anginal pain and exertional dyspnea, and showed good exercise tolerance.

References
1. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Maron BJ, Towbin JA, Thiene G et al; Circulation. 2006;113(14):1807.

Experts’ conclusions:
The experts recommended genetic screening of his close relatives. Optimization of the CTR-D settings. Regular follow-up with echocardiography. The impact of the harmful working conditions and the possibility of damage to the heart muscle after repeated shocks while welding at work considered as a coincidence. Also referred to the controversy over the use of anticoagulants, which in this case is unfounded. Subsequently right heart catheterization should be considered and heart transplantation as an ultimate treatment option.

Accidentally discover cardiomiopathy in patient with initial acute coronary syndrom Noncompaction Cardiomiopathy – flash presentation

Authors:
Trąbka-Zawicki A. MD1, Rubiś P. MD, PhD4, Assoc. Prof. Kostkiewicz M. MD, PhD2, Miszalski-Jamka T. MD, PhD2, Tomala I. MD3, Assoc. Prof. Lelakowski J. MD, PhD3, Prof. Żmudka K. MD, PhD1

Experts:
Assoc. Prof. Rudziński A. MD, PhD5, Kordon Z. MD, PhD6, Assoc. Prof. Gackowski A. MD, PhD7

1 Cardiovascular Intervention Center with the Unit of Interventional Cardiology
2 Center for Diagnosis, Prevention and Telemedicine with the Day-Care Unit, John Paul II Hospital, Krakow, Poland
3 Department of Electrocardiology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
4 Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
5 University Children Hospital of Krakow, Poland
6 Department of Pediatric Cardiology, Polish-American Children’s Hospital, Krakow, Poland
7 Department of Coronary Heart Disease, John Paul II Hospital, Krakow, Poland
 

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This entry was posted in 2. Metabolic disorders/genetic syndromes and LVNC, 5. Unclassified cardiomyopathies, A. Left ventricular noncompaction, Przypadki kliniczne, III. Rare diseases of the heart (cardiomyopathies). Bookmark the permalink.

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