Journal of Rare Cardiovascular Diseases

We present a case of a 65-year old woman, who was admitted to hospital due to recurrent pulmonary oedema. Patient’s history included chronic obstructive pulmonary disease. Echocardiography revealed a concentric hypertrophy of the left ventricle walls without the narrowing of the outflow tract, accompanied by the mild/moderate mitral stenosis, preserved systolic and mildly impaired diastolic function of the left ventricle. Those comorbidities were not severe but their coexistence contributed to rapid heart failure progression. During another admission for pulmonary oedema the patient had cardiac arrest in the mechanism of the pulseless electrical activity, which triggered the diagnostics of amyloidosis. Right heart catheterization was performed showing an unusually high wedge pressure. Histological analysis confirmed the diagnosis of amyloid light-chain (AL) amyloidosis, so the patient was scheduled for the first course of chemotherapy. Unfortunately, shortly after discharge, the patient developed pulmonary oedema and died before an admission to the hospital.

This case shows the adverse course of the disease, fast progression of unspecific symptoms that may attribute to multiple comorbidities. JRCD 2015; 2 (5): 156–160

amyloidosis; heart failure; pulmonary oedema; chronic obstructive pulmonary disease

Prochorec-Sobieszek M, Bilińska ZT, Wagner T. Amyloidoza serca. Współczesne poglądy. Kardiol Pol 2004; 61: 63-66.

Falk RH, Dubrey SW. Amyloid heart disease. Prog Cardiovasc Dis. 2010;52:347–361.

Banypersad SM, Moon JC, Whelan C, et al. Updates in cardiac amyloidosis: a review. J Am Heart Assoc. 2012 Apr;1(2):e000364. doi: 10.1161/JAHA.111.000364. Epub 2012 Apr 24.

Bellavia D, Pellikka PA, Abraham TP, et al. Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard two-dimensional and Doppler echocardiography. Am J Cardiol. 2008; 101: 1039–1045.

Tsang W, Lang RM. Echocardiographic evaluation of cardiac amyloid. Curr Cardiol Rep. 2010; 12: 272–276.

Velazquez-Cecena JL, Lubell DL, Nagajothi N, et al. Syncope from dynamic left ventricular outflow tract obstruction simulating hypertrophic cardiomyopathy in a patient with primary AL-type amyloid heart disease. Tex Heart Inst J. 2009; 36: 50–54.

Dinwoodey DL, Skinner M, Maron MS, et al. Light-chain amyloidosis with echocardiographic features of hypertrophic cardiomyopathy. Am J Cardiol. 2008; 101: 674–676.

Burgess M, Mogulkoc N, Wright-Thomas R, et al. Comparison of echocardiographic markers of right ventricular function in determining prognosis in chronic pulmonary disease. J Am Soc Echocardiogr. 2002; 15: 633-639.

Alpert SA. The effect of right ventricular dysfunction on left ventricular form and function. Chest 2001; 119: 1632-1633.

Suchoń E, Tracz W, Podolec P, et al. Ocena czynności lewej komory u chorych na przewlekłą obturacyjną chorobę płuc. Pol Arch Med Wewn. 2007; 117: 86-90.

d’Avila A1, Rabischoffsky A, Saad E. Images in cardiovascular medicine. Reverse Bernheim phenomenon as a cause of exercise-induced syncope. Circulation. 2006; 114: e618-20.

Schena M, Clini E, Errera D, et al. Echo-Doppler evaluation of left ventricular impairment in chronic cor pulmonale. Chest 1996; 109: 1446-1451.

Chaouat A, Bugnet AS, Kadaoui N. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2005; 172: 189-194.

Weitzenblum E, Chaouat A. Right ventricular function in COPD. Can it be assessed reliably by the measurements of right ventricular ejection fraction? Chest 1998; 113: 567-568.

Matthay R, Berger H, Davis R, et al. Right and left ventricular exercise performance in chronic obstructive pulmonary disease: Radionuclide assessment. Ann Int Med. 1980; 93: 234-239.

Kumar SK, Gertz MA, Lacy MQ, et al. Recent improvements in survival in primary systemic amyloidosis and the importance of an early mortality risk score. Mayo Clin Proc. 2011; 86: 12-8. doi: 10.4065/mcp.2010.0480.

Salpeter SS, Ormiston T, Salpeter E, et al. Cardioselective beta-blockers for chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2002;(2): CD003566.

. Brooks TW, Creekmore FM, Young DC, et al. Rates of hospitalizations and emergency department visits in patients with asthma and chronic obstructive pulmonary disease taking beta-blockers. Pharmacotherapy. 2007; 27: 684-690.

Shimojima Y, Matsuda M, Ishii W, et al. High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement. Intern Med 2005; 44: 484-489.

Dubrey S, Burke M, Hawkins P, et al. Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant 2004; 23: 1142-1153.

Alloni A, Pellegrini C, Ragni T, et al. Heart transplantation in patients with amyloidosis: single-center experience. Transplant Proc 2004; 36: 643-644.

Rubinow A, Skinner M, Cohen AS. Digoxin sensitivity in amyloid cardiomyopathy. Circulation. 1981; 63: 1285–1288.

Pollak A, Falk RH. Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis. Chest. 1993; 104: 618–620.

Maron BJ, Spirito P, Shen WK, et al. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA. 2007; 298: 405-412.

Kristen AV, Dengler TJ, Hegenbart U, et al. Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death. Heart Rhythm. 2008; 5: 235–240.

Bellavia D, Pellikka PA, Abraham TP, et al. ‘Hypersynchronisation’ by tissue velocity imaging in patients with cardiac amyloidosis. Heart. 2009; 95: 234–240.