PRACA KAZUISTYCZNA

18-year-old man with cardiomyopathy resulting from isolated left ventricular non-compaction and atrial fibrillation diagnosed for the first time — difficult choices and therapeutic decisions

18-letni mężczyzna z kardiomiopatią z niescalenia mięśnia lewej komory oraz rozpoznanym po raz pierwszy migotaniem przedsionków — trudne wybory i decyzje terapeutyczne

Andrzej Jakub Sałacki, Andrzej Wysokiński

Chair and Department of Cardiology, Medical University of Lublin

Address for correspondence: prof. dr hab. n. med. Andrzej Wysokiński, Katedra i Klinika Kardiologii, Uniwersytet Medyczny w Lublinie, ul. Jaczewskiego 8, 20–954 Lublin, Poland, tel. +48 81 724 41 51, e-mail: a.wysokinski@umlub.pl

Introduction

Left ventricular non-compaction (LVNC) is a form of familial cardiomyopathy resulting from disrupted fusion of myocardium trabeculae carneae during intrauterine development [1]. The number of diagnosed instances of LVNC has increased significantly within the last twenty years [2]. According to the European Society of Cardiology, it belongs to the group of unclassified cardiomyopathies [3], whereas, according to the American Heart Association, it is a distinct genetic primary cardiomyopathy [4]. A characteristic trait of LVNC is the presence of non-compacted, in other words excessively trabeculate, left ventricular (LV) myocardium. Layers of non-compacted and compacted LV muscle jointly form a characteristic two-layer structure of myocardium [5]. Clinical signs depend on how disrupted the structure is therefore the description included both asymptomatic patients and symptomatic patients with disorders of LV systolic and diastolic functions, various forms of Cardiac arrhythmias or instances of embolism [6]. However, there have been no therapies specifically targeted at LVNC yet and the treatment should incorporate the standards applied in the case of Cardiovascular System disorders, arrhythmias, as well as eliminating the risk of sudden death or embolic complications.

Case report

An 18-year-old man was transferred from an emergency department to the Cardiac Intensive Care Unit (CICU) as a result of his first bout of atrial fibrillation (AF) lasting from morning (Fig. 1).

Figure 1. ECG taken upon admission

The patient in 2016 underwent ablation with the use of alternating electric current with radio frequency of the accessory pathway (inferior septal pathway) due to the WPW syndrome diagnosed in 2013. Moreover, he was diagnosed with cardiomyopathy resulting from LVNC.

Physical examination showed abnormal heart action 75–100/min, peripheral pulse deficit, dull and quiet heart sounds. The patient experienced strong heart palpitation. Following the recommendations after ablation, for three month he would take a 150 gm dose of aspirin on a regular basis. Laboratory tests showed the TSH of 0,285 mIU/L, standard (0.55–4.78) in the case of regular peripheral thyroid hormone levels — FT3 and FT4.

The TTE showed that LVEDd was 62 mm. Moreover, the patient had irregular LV muscularis increased LV trabeculation below papillary muscles, segmentally thinned LV wall in the apex area — a probable LV non-compaction center, heterogeneous echogenicity of interventricular septum and its thickened upper part (13 mm), EF ~55%, SF ~30%, 15 mm scope of mitral annular plane systolic excursion, LA ~35 mm.

Prior to his treatment, unfractionated heparin was administered in the dose of 5 thousand units by intravenous bolus. The authors have considerable experience of using antazoline in the case of paroxysmal atrial fibrillation, thus the therapy began with its intravenous administration in the dose of 100 mg. As it turned out to be ineffective, it was administered with potassium, magnesium and Xylocaine added to the multi-electrolyte fluid. The therapy has not produced any results therefore slow intravenous infusion of 150 mg of Amiodarone in 250 ml of 5% glucose was initiated. Unfortunately, it has not produced any therapeutic effects either. As a result, a decision was made to perform anterolateral electrical cardioversion with 150 J two-phase current under total intravenous anesthesia. The cardioversion produced the expected results by restoring sinus heart rhythm. Initiation of oral anticoagulant (OAC) therapy introduced after electrical cardioversion proved to be another problematic issue, especially at duty in CICU. Finally, the decision was made to continue using the ASA preparation.

Discussion

There is a need for an in-depth discussion due to the lack of clear recommendations and guidelines for introducing and using OAC in the case of patients diagnosed with LVNC and patients who due to their basic disease, such as LVNC, also come down with malignant arrhythmias like AF which sooner or later also require such therapy. In the reported case the TTE did not show any mitral valve defects. Based on the CHA2DS2VASc score, the risk of a stroke and thromboembolic complications was estimated at 0 points. Following the ESC guideline [7] for the year 2012 regarding actions to be taken in the case of AF, a 4-week OAC therapy should be introduced after electrical cardioversion if AF lasts under 48 hours; in the reported case it lasted for several hours.

Previous scientific reports advised to introduce anticoagulants in the case of this form of cardiomyopathy in treatment of all symptomatic patients. It was considered to be particularly important due to a 25-times bigger thromboembolic risk in the group of patients with LVNC [8]. Currently, a less restrictive view is prevailing – it supports the idea that OAC should be considered in the case of patients who have undergone embolic events and patients with other coexisting indications like AF, thrombus in LV or significant LV systolic dysfunction. OAC should not be used on a regular basis in the case of asymptomatic patients with LVNC [9]. Pignatelli et al. [10] have not observed any embolic complications in retrospective analysis of LVNC pediatric population where all patients were administered aspirin. The issue of applying OAC in the case of LVNC is still very controversial and has not been settled yet.

Conflict of interest

None declared.

References

1. 1. Borges A.C., Kivelitz D., Baumann G. Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 2003; 89: e21.
2. 2. Captur G., Nihoyannopoulos P. Left ventricular non-compaction: gene­tic heterogeneity, diagnosis and clinical course. Int. J. Cardiol. 2010; 140: 145–153.
3. 3. Elliott P., Andersson B., Arbustini E. et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur. Heart J. 2008; 29: 270–276.
4. 4. Maron B.J., Towbin J.A., Thiene G. et al. Contemporary definitions and classification of the cardiomyopathies. Circulation 2006; 113: 1807–1816.
5. 5. Miszalski-Jamka K., Głowacki J., Kalarus Z. Niescalenie mięśnia lewej komory — coraz częściej rozpoznawana kardiomiopatia. Kardiochir. Torakochir. Pol. 2012; 1: 106–113.
6. 6. Weiford B.C., Subbarao V.D., Mulhern K.M. Noncompaction of the ventricular myocardium. Circulation 2004; 109: 2965–2971.
7. 7. Wytyczne ESC dotyczące postępowania w migotaniu przedsionków na 2012 rok. Kardiol. Pol. 2012; 70 (Suppl IV): 197–234.
8. 8. Serwa-Stępień E., Barylski M., Banach M. i wsp. Niescalenie mięśnia lewej komory. Kardiol. Pol. 2006; 64: 1126–1131.
9. 9. Oechslin E.N., Attenhofer Jost C.H., Rojas J.R. et. al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J. Am. Coll. Cardiol. 2000; 36: 493–500.
10. 10. Ilercil A., Barack J., Malone M.A. et al. Association of noncompaction of left ventricular myocardium with Ebstein’s anomaly. Echocardiography 2006; 23: 432–433.