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Vol. 12/Nr 3-4(45-46)/2018: 3-5
Z pogranicZa kardiologii
3Pracę otrzymano: 2.11.2018 Zaakceptowano do druku: 13.11.2018 “Copyright by Medical Education”
IntroductIon
Light-chain deposition disease (LCDD) is characterized by deposition of immunoglobulin light chains (LCs) in multiple organs. The most common manifestation is kidney involvement, including proteinuria and renal fail-ure [1]. Heart involvement is one of the extra-renal man-ifestations [2]. Described cardiac manman-ifestations are i.e. atrial fibrillation and restrictive cardiomyopathy [3, 4]. We present a case in which paroxysmal atrial fibrillation (PAF) and previous diagnosis of hypertrophic cardio- myopathy (HCM) conduced to diagnosis of LCDD.
case report
52-year-old Caucasian man was admitted to our Cardi-ology Department with yet another episode of PAF, he-modynamically unstable, with rapid ventricular rhythm. The history revealed several episodes of PAF, treated with electric cardioversion 39 times so far, and twice with radiofrequency ablation (RFA), ineffectively. At admission he underwent fortieth electric cardioversion. Apart from supraventricular arrhythmia the patient had also a history of HCM without left ventricular outflow tract obstruction and with ventricular arrhythmia. Due
to this condition, he underwent cardioverter-defibrilla-tor implantation a few years earlier, as a primary preven-tion of sudden cardiac death. Patient’s mother was also reported to have HCM.
Considering previous medical history, a Maze procedure was proposed. As a candidate for Maze surgery, the pa-tient underwent several diagnostic procedures. Echo-cardiography, an important one among them, showed: left atrial chamber enlargement, normal sizes of other chambers, ventricular septum thickness of 2 cm and the rest of left ventricular wall moderately hypertrophied. As the echocardiography’s results were not fully specif-ic of HCM (more typspecif-ical of restrspecif-ictive cardiomyopathy than HCM) and considering the family history of HCM, further investigations were planned. Despite already es-tablished diagnosis of HCM, we wanted to exclude other causes of heart muscle hypertrophy i.e. amyloidosis, that would change patient prognosis and influence further treatment choices. Blood samples for free light chains were obtained. Results revealed light chains lambda of 15,4 and light chains kappa of 23,2 (slightly elevated). Lambda to kappa ratio (L/K) was within normal range.
an episode of paroxysmal atrial fibrillation
with unexpected ending
Nieoczekiwane rozpoznanie ustalone u chorego z napadem migotania przedsionków
Maria Różańska, MD
1, Martyna Zaleska, MD
1, Piotr Suwalski, MD, PhD
2,
Dariusz A. Kosior, MD, PhD, FESC, FACC
1, 3, Olga Możeńska, MD, PhD
11 Department of Cardiology and Hypertension, Central Clinical Hospital of the Ministry of Interior and Administration, Warsaw, Poland 2 Department of Cardiac Surgery, Central Clinical Hospital of the Ministry of Interior and Administration, Warsaw, Poland 3 Department of Applied Physiology, Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland
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Vol. 12/Nr 3-4(45-46)/2018: 3-5 M. Różańska, M. Zaleska, P. Suwalski, D.A. Kosior, O. Możeńska
An episode of paroxysmal atrial fibrillation with unexpected ending
That spoke in favor of cardiac amyloidosis instead of HCM diagnosis.
Couple of weeks later patient underwent uneventful Maze procedure, during which endomyocardial left ven-tricle biopsy was obtained. The histopathological exam-ination did not reveal signs of amyloid depositions (no staining with Congo red), what resulted in final diagno-sis of observation towards LCDD.
dIscussIon
Atrial fibrillation is the most common sustained cardiac arrhythmia in clinical practice [5], often associated with underlying heart disease [6]. Hypertrophic cardiomyo-pathy is diagnosed on the basis of otherwise unexplained hypertrophied, nondilated left ventricle. A septal wall thickness ≥ 1,5 cm has been commonly used to diagnose HCM [7]. Restrictive cardiomyopathy is characterized by nondilated ventricles with impaired ventricular fill-ing [8]. The echocardiographic findfill-ings in our patient were ambiguous, as the septal thickness of 2 cm would support the diagnosis of HCM. However, the left atrial enlargement was more specific of restrictive cardiomyo-pathy. Although typically absent in restrictive cardiomy-opathy, left ventricular hypertrophy may be triggered by infiltrative diseases like amyloidosis or LCDD [9]. Kid-ney involvement usually dominates the clinical course of LCDD, though LCs may be deposited in other internal organs, including liver and heart. The clinical manifesta-tions depend on which tissues are involved and the con-sequent organ dysfunction. The clinical picture is
there-fore extremely heterogeneous [10]. Tissue deposits in LCDD are usually composed of kappa LCs [1], resulting in low L/K. In this case, the normal L/K increases sus-picion of cardiac amyloidosis. However the deposits do not stain with Congo red, which is the pathognomonic feature of amyloidosis [8].
conclusIon
In conclusion, this case suggests that PAF connect-ed with restrictive cardiomyopathy may be relatconnect-ed to LCDD. This disease should be taken into consideration in patients with suspicion of restrictive cardiomyopa-thy and cardiac arrhythmias resistant to RFA. Especially the mild to moderate left ventricular hypertrophy may be the sign of infiltrative disease of the heart rather than HCM, as shown in this case.
correspondence
olga Możeńska, Md, phd Department of Cardiology and Hypertension, Central Clinical Hospital of the Ministry of Interior and Administration 02-507 Warsaw, Wołoska 137 phone: (+48 22) 508-16-70 fax: (+48 22) 508-16-80 e-mail: olgamozenska@gmail.com Authors’ contributions: Różańska M.: 30%; Zaleska M.: 20%; Suwalski P.: 15%; Kosior D.A.: 5%; Możeńska O.: 30%. Conflict of interests: None. Financial support: None. Ethics: The contents presented in this paper are compatible with the rules the Declaration of Helsinki, EU directives and standardized requirements for medical journals.
abstract
We present a case of 52-year-old male with restrictive cardiomyopathy as well as with history of paroxysmal atrial fibrillation in whom detailed diagnostic process led to suspicion of light-chain deposition disease as a reason for left ventricular hypertrophy.
Key words: light-chain deposition disease, restrictive cardiomyopathy, Maze procedure
streszczenIe
Prezentujemy przypadek 52-letniego pacjenta z kardiomiopatią restrykcyjną oraz z wywiadem napadowego migo-tania przedsionków, u którego w toku szczegółowej diagnostyki wysunięto podejrzenie choroby łańcuchów lekkich jako potencjalnej przyczyny przerostu mięśnia lewej komory.
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M. Różańska, M. Zaleska, P. Suwalski, D.A. Kosior, O. Możeńska An episode of paroxysmal atrial fibrillation with unexpected ending
references:
1. Pozzi C, D’Amico M, Fogazzi GB et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis 2003; 42(6): 1154-1163.
2. Buxbaum JN, Chuba JV, Hellman GC et al. Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis: clinical features, immunopathology, and molecular analysis. Ann Intern Med 1990; 112(6): 455-464. 3. Fabbian F, Stabellini N, Sartori S et al. Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report. J Med Case Rep 2007; 1:
187.
4. Koopman P, Van Dorpe J, Maes B, Dujardin K. Light chain deposition disease as a rare cause of restrictive cardiomyopathy. Acta Cardiol 2009; 64(6): 821-824.
5. Chugh SS, Blackshear JL, Shen WK et al. Epidemiology and natural history of atrial fibrillation: clinical implications. J Am Coll Cardiol 2001; 37(2): 371- -378.
6. Benjamin EJ, Wolf PA, D’Agostino RB et al. Impact of atrial fibrillation on the risk of death: the Framingham Heart Study. Circulation 1998; 98(10): 946- -952.
7. Williams LK, Frenneaux MP, Steeds RP. Echocardiography in hypertrophic cardiomyopathy diagnosis, prognosis, and role in management. Eur J Echo-cardiogr 2009; 10(8): iii9-14.
8. Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med 1997; 336(4): 267-276.
9. Seward JB, Casaclang-Verzosa G. Infiltrative cardiovascular diseases: cardiomyopathies that look alike. J Am Coll Cardiol 2010; 55(17): 1769-1779. 10. Pozzi C, Locatelli F. The patient with insidious chronic renal failure and the patient with the nephrotic syndrome – two manifestations of a protean and
not so rare disease. Nephrol Dial Transplant 1996; 11(9): 1876-1880.
