Authors

• Karolina Kalanj — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
• Ljiljana Banfić — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-4538-8980
• Majda Vrkić — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
• Kirhmajer — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-1340-1917
• Miroslav Krpan — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-0639-953X
• Krešimir Putarek — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-0361-5740
• Mislav Puljević — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-1477-2581
• Zoran Miovski — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-3850-8905

Keywords

left ventricular noncompaction, celiac disease, thromboembolic event

DOI

Full Text

**Introduction:** Left ventricular noncompaction is a rare cardiomiopathy morphologically characterized by left ventricular (LV) trabeculae, deep intertrabecular recesses and a thin layer of compacted epicardium. The etiology of the disorder is still not clear, and the disease can develop during the embrionic phase of heart development, between 5-8 weeks of pregnancy, or sporadically in adults mostly in athletes, patient with sickle cell anemia and during pregnancy. Clinical manifestations of the disease are: heart failure, heart arrhythmias (atrial and ventricular) and thromboembolic events (21-24%). Celiac disease is an autoimmune disorder that affects the intestinal mucosa causing inflammation, crypt hyperplasia and villous atrophy, which leads to the malnutrition of different nutrients. In addition to hematological and gastroenterological symptoms, celiac disease can affect the cardiovascular system causing dilated cardiomiopathy, ischemic heart disease, atrial fibrillation and thromboembolic events. Due to the significant incidence of disease of 1% in Europe and North America and the increasing incidence of cardiovascular disease, the effect of celiac disease on the cardiovascular system is the subject of much current research. (1-3) **Case report:** We present a patient (recreational athlete), 34 years old, admitted to University Hospital Center Zagreb, Croatia whom we diagnosed as having noncompaction LV and celiac disease. Due to the critical chronic ischemia of the left leg caused by a thromboembolic event (occlusion of femoral and popliteal artery) limb saving procedures were performed twice, but because of infection and eventual gangrene in the left foot, lower limb amputation was indicated. During the episode of care patient was diagnosed with noncompaction LV (MR imaging: NC/C 2.7 LV apical part) and celiac disease (EMA positive; tTG >200 H RU/ml, DNA analyses homozygous C677T and heterozygous A1298C). As both diseases, acting in concert can, by different pathogenesis, cause further deterioration of the heart function, condition management protocols must include a gluten free diet, regular cardiac function staging and adequate rehabilitation (use of prostheses depending on heart function).

Literature

1. Arbustini E, Weidemann F, Hall JL. Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases? J Am Coll Cardiol. 2014;64(17):1840–50. https://doi.org/10.1016/j.jacc.2014.08.030
2. Udeoji DU, Philip KJ, Morrissey RP, Phan A, Schwarz ER. Left ventricular noncompaction cardiomyopathy: updated review. Ther Adv Cardiovasc Dis. 2013;7(5):260–73. https://doi.org/10.1177/1753944713504639
3. Ikeda U, Minamisawa M, Koyama J. Isolated left ventricular noncompaction cardiomyopathy in adults. J Cardiol. 2015;65(2):91–7. https://doi.org/10.1016/j.jjcc.2014.10.005