Authors

• Vlatka Rešković Lukšić — University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-4721-3236
• Marija Mance — University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-1542-2890
• Zvonimir Ostojić — University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-1762-9270
• Dejan Došen — University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-2641-4768
• Ivan Bitunjac — General Hospital “Dr. Josip Benčević” Slavonski Brod, Slavonski Brod, Croatia — ORCID: 0000-0002-4396-6628
• Kristina Gašparović — University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-1191-4831
• Jadranka Šeparović Hanževački — University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-3437-6407

Abstract

**Aim**: To investigate prevalence of arrythmias and thromboembolic events, as well as long term outcomes among patients diagnosed and treated of non-compaction cardiomyopathy (NCC) according to current recommendations in University Hospital Centre (UHC) Zagreb. **Patients and Methods**: A single center retrospective study was conducted. Patients newly diagnosed with NCC in UHC Zagreb during period 2009-2018 were analyzed. The diagnosis was confirmed by both echocardiography and cardiovascular magnetic resonance. Hospital database and charts were used for clinical data, echocardiography data was obtained from digital database using EchoPac. Patients were followed-up clinically and by the means of echocardiography. **Results**: 32 patients (pts), 18 men (53.25%) were included. At the time of diagnosis (baseline), mean age was 47.7±15.4 years, majority of pts (84.38% of pts, N=27) were in functional NYHA class ≥2, with mean NT-proBNP values of 3870±6619 ng/L. Echocardiography revealed reduced left ventricular systolic function; baseline ejection fraction (EF) was 27.52±11.94%. Patients were discharged with heart failure therapy: beta-blockers (30 pts, 93.75%), angiotensin-converting enzyme inhibitors or angiotensin receptor blockers (29 pts, 90.63%), angiotensin receptor–neprilysin inhibitor (1 pts, 3.13%), mineralocorticoid receptor antagonists (28 pts, 84.38%); and 26 pts (81.25%) required symptomatic diuretic use. Cardiac resynchronization therapy with defibrillator was implanted in 11 pts (34.38%) and implantable cardioverter defibrillator (ICD) in 16 pts (50%). At baseline, 9 pts (28.13%) were already receiving anticoagulation due to previous thromboembolic events (**Table 1**). At discharge, 17 pts (53.13%) were anticoagulated (warfarin in 14 pts, 82.35%, novel direct oral anticoagulants in 3 pts, 17.65%). Mean follow-up period was 3.42±3.31 years. At the end of follow up period, functional improvement was observed: 21.88% (N=7) pts were in NYHA ≥2 status (p<0.05), with manifest, but statistically nonsignificant reduction of NT-proBNP levels (1260±2266 ng/L, p=0.063). Control echocardiography revealed significant improvement in EF (40.24±11.39%, p<0.001). There were no new thromboembolic events. While arrythmias were common at the time of diagnosis (**Table 1**), there was only one ICD activation during follow-up. None of the pts have died, 1 patient received heart transplant and 2 were implanted with left ventricular assist device. ### TABLE 1: Number of patients diagnosed with thromboembolic events and arrythmias at the time of diagnosis and at the end of the follow-up period. | Number of patients: | **At diagnosis** | **At follow up** | | --- | --- | --- | | **THROMBOEMBOLIC EVENTS** | | | | Left ventricular thrombus | 3 (9.38%) | 0 (0) | | Stroke/TIA | 4 (12.50%) | 0 (0) | | Other embolus | 1 (3.13%) | 0 (0) | | **ARRYTHMIAS** | | | | Atrial fibrillation | 3 (9.38%) | 7 (21.88%) | | nsVT | 15 (46.88%) | 8 (25.00%) | | Sustained VT | 1 (3.13%) | 4 (12.50%) | | ICD activation | 0 (0%) | 1 (6.25%) | [†] TIA = transient ischemic attack; nsVT = non-sustained ventricular tachycardia; VT = ventricular tachycardia; ICD = implantable cardioverter defibrillator. **Conclusion**: Optimal medical treatment in patients with NCC (1) corresponds with good long-term outcomes, functional improvement, and low risk of recurrent thromboembolic events or malignant arrythmias.

Keywords

non-compaction cardiomyopathy, arrythmia, thromboembolic event, prognosis

DOI

Literature

1. Carrilho-Ferreira P, Almeida AG, Pinto FJ. Non-compaction cardiomyopathy: prevalence, prognosis, pathoetiology, genetics, and risk of cardioembolism. Curr Heart Fail Rep. 2014 Dec;11(4):393–403. https://doi.org/10.1007/s11897-014-0227-3