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How and when to suspect to non-compaction cardiomyopathy: A case series of 40 patients confirmed by cardiac MRI.

عنوان مقاله: How and when to suspect to non-compaction cardiomyopathy: A case series of 40 patients confirmed by cardiac MRI.
شناسه (COI) مقاله: HFMED06_003
منتشر شده در ششمین کنگره بین المللی نارسایی قلب ایران در سال 1397
مشخصات نویسندگان مقاله:

Zohreh Shoraka - MD.
Esmat Asaei - MD.; Adult cardiologist
Farhad Akhavein - MD.; Adult cardiologist, Cardiovascular MRI fellowship.Aria hospital, Mashhad University of Medical Sciences, Mashhad, Iran.Behsaz Teb medical imaging center, Mashhad, Iran

خلاصه مقاله:
Non-compaction cardiomyopathy (NC.CM), as an unclassified CM,has gained increasing recognition during the last 25 years. It can occur in isolation or in association with congenital heart defects (CHDs), genetic syndromes, and neuromuscular disorders. Its classical triad of complications are heart failure, arrhythmias, and systemic thromboembolic embolic (TE) events. Clinical aspects of NVM are not completely defined yet and despite increased awareness and improvements in echo and MRI in the past decade, there is still a significant delay in establishing the correct diagnosis due to little knowledge and absence of an international consensus on absolute diagnostic criteria, symptoms and prognosis. Objective: Providing an organized insight into the early detection of NC.CM based on spectrum of the initial presentations or symptoms before doing any other diagnostic approaches. Method and results: From Nov. 2016 to Nov. 2017, 40 patients were diagnosed NC.CM according to CMRI criteria as the gold standard imaging modality. There were 17 male, 23 female, the median age 43±14 (range 15-71). Initial presentation or symptom(s) was as: asymptomatic (6), palpitation (18), non-coronary chest pain (7), dyspnea or shortness of breath (10), repetitive syncope (1), embolic stroke (5; 2 pregnants), arrhythmia (13 ; 12 ventricular, 1supraventricular, 3 resting sinus tachycardia, 1 permanent sinus bradycardia), ECG abnormalities (18; 3 WPW, 17 ST-T alterations). Underlying conditions were: Muscle dystrophy (Emery Dreifuss)(1), congenital heart defect (1) , professional athletes (6), pregnancy (2), misdiagnosed dilated CM (10), misdiagnosed CAD according to ETT and SPECT with normal angiography(4), familial NC.CM (5), unexplained arrhythmia with poor response to medications and ablation (9), undiagnosed underlying pathology of heart failure (10). Conclusion: The study indicates that the clinical spectrum is wide and vary, and early recognition is essential. Physicians should be suspected to NC.CM if there are any of the following items, leading to detect it in non-symptomatic stages. Then, CMRI should be done to rule out or confirm NC.CM. 1) Clinical and echo findings related to systolic or diastolic dysfunction with no evidence of underlying CAD, VHD, CM, HTA. 2) Any non-specific and unexplained cardio-respiratory symptoms (breathlessness, shortness of breath, dyspnea, palpitation, arrhythmia, decreased physical capacity …in pregnancy, professional athletes, muscular dystrophy, family history of NC.CM and SCD).3) Non-coronary chest pain or discomfort in the absence of documented CAD. 4) CVA or TE events in patients with no specific risk factors, particularly in young people, pregnancy,5) Presence non-specific ECG changes particularly unexplained LBBB, repolarization abnormalities (beat-to-beat ST-T alterations), WPW,.. in apparently asymptomatic patients or with non-specific cardiac symptoms. 6) Any kind of arrhythmia (ventricular or supraventricular) with unknown origin particularly in those with poor response to anti-arrhythmic therapy. 7) Undiagnostic or equivocal echo for NC.CM. However, after a welldocumented echo, CMRI as a gold standard imaging is needed.

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