Letter to the Editor Deficiency of creatine kinase in a ST-segment elevation myocardial infarction patient with Kartagener syndrome Takehiro Hashikata a , Taiki Tojo a , Sayaka Namba a , Lisa Kitasato a , Takuya Hashimoto a , Ryo Kameda a , Takao Shimohama a , Minako Yamaoka-Tojo a,b , Fumio Takada c , Junya Ako a, ⁎ a Department of Cardiovascular Medicine, Kitasato University School of Medicine, Sagamihara, Japan b Department of Rehabilitation, Kitasato University School of Allied Health Sciences, Sagamihara, Japan c Department of Medical Genetics, Kitasato University Graduate School of Medical Sciences, Sagamihara, Japan article info Article history: Received 4 December 2014 Accepted 25 December 2014 Available online 27 December 2014 Keywords: Creatine kinase Kartagener syndrome Primary ciliary dyskinesia 1. Case presentation A 75 year old male with a history of a diagnosis of Kartagener syn- drome (situs inversus, chronic bronchitis, and rhinitis) was referred for the treatment of inferior ST-segment elevation myocardial infarction (STEMI) (Fig. 1, Panels A, B). He received percutaneous coronary inter- vention (PCI) for the culplit lesion in the middle left coronary artery with an everolimus-eluting stent. Computed tomography (CT) showed internal organs in reverse position (Fig. 2, Panel A). Noncontrast CT showed peribronchial thickening, mucus plugging and bronchiectasis (Fig. 2, Panel B). Laboratory date on arrival showed the elevation of car- diac enzyme except for serum creatine kinase (CK) activity. CK activity on arrival indicated only 7 IU/L. After PCI procedure, CK levels showed no elevation throughout hospitalization. Peak CK level was only 8 IU and CK-MB level was below detection limit. The amount of the CK- MB, which was measured by the chemiluminescent enzyme immunoas- say (CLIA) method, was extremely low as well. The change in the levels of cardiac enzymes except for CK revealed a typical course of myocardial infarction (MI) (Fig. 1, Panel C). Peak troponin T reached 0.491 ng/ml after the procedure. The cardiovascular magnetic resonance (CMR) demonstrated late gadolinium enhancement and high intensity area in T2 in the myocardium at risk (Fig. 1, Panel D). Cardiac scintigraphy with technetium-99m tetrofosmin in rest, performed 7 days after proce- dure, was compatible with inferior MI. The clinical course was unevent- ful. He had no family history of situs inversus, abnormal function of respiratory system or d\eficiency of CK, and he had 2 daughters. Dysfunction of inferior ventricular wall motion, which had existed on admission, improved almost normally within 2 weeks. There was no complication during his clinical course. 2. Discussion We experienced a case of STEMI patient with Kartagener syndrome. Although clinical symptoms, electrocardiogram, and laboratory findings were compatible with STEMI, the CK levels remained almost undetect- able range. The patient was presumably complicated with congenital CK deficiency as well. CK consists of CK-muscle (CK-M) and -brain (CK-B). Three isoen- zymes, CK-MM, -MB and -BB, are formed by hybridization of CK-M and -B subunits [1]. Increased CK activity accompanied by the appear- ance of CK-MB isoenzyme is useful for the diagnosis of MI. The primary role of CK is to catalyze the reversible transfer of a high-energy phos- phoryl group between adenosine triphosphate and phosphocreatine. The functional and physical coupling of certain members of the CK iso- enzyme family to the sites of energy production and utilization have underscored the integrated properties of this important enzyme system in excitable tissue, particularly in muscle cells [2]. There have been two case reports of patient with deficiency of CK up to date, including a fam- ily cluster case [3,4]. Nahrendorf et al. [5] reported that CK knockout mice show significant left ventricular (LV) dilatation and marked LV hy- pertrophy, however, CK-deficient hearts did not develop adverse left ventricular remodeling post-MI. In the present case, patient had no LV hypertrophy on admission and no significant remodeling after MI. We speculated that the myocardium of this patient might have some com- pensatory pathway or other enzyme with a similar effect. Kartagener syndrome is a primary ciliary dyskinesia (PCD) with situs inversus. PCD is an autosomal recessive disease characterized by abnor- mal ciliary function, accompanied with chronic sinusitis and bronchiec- tasis. The pulmonary dysfunction in PCD is considered a result from recurrent airway infections resulting in scarring with airway remodel- ing. The respiratory cilia are highly complex structures composed of International Journal of Cardiology 182 (2015) 31–33 ⁎ Corresponding author at: Department of Cardiovascular Medicine, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan. E-mail address: jako@kitasato-u.ac.jp (J. Ako). http://dx.doi.org/10.1016/j.ijcard.2014.12.100 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved. Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard