Transcatheter Aortic Valve Implantation In A Patient With Systemic Senile Transthyretin Type Amyloidosis

Abstract

Trancatheter aortic valve implantation with CoreValve or Edwards-Sapien prosthesis is becoming the method of choice for treating severe, symptomatic aortic stenosis in surgical high risk patients. CoreValve implantation may worsen atrioventricular conduction with possible development of atrioventricular block. However, in patients with infiltrative cardiomyopathy, such as amyloidosis, conduction disturbances can be the result of the underlying disease alone. 88-yr old female patient with severe, symptomatic aortic stenosis, after was scheduled for transcatheter aortic valve implantation. The procedure itself was successfully performed however, after the procedure transient complete atrioventricular block developed and a temporary pacemaker electrode was inserted. One day after the procedure the atrioventricular block resolved into her basic heart rhythm which was chronic atrial fibrillation with slow ventricular response. Due to development of systemic signs of infection of unknown origin the temporary electrode was removed and implantation of permanent VVI-type pacemaker was indicated. While waiting for implantation the patient died of sudden cardiac death. Results of autopsy showed a CoreValve in the proper aortic valve position without blocking the ostia of coronary arteries, mildly enlarged and moderately thickened left and right ventricles on macroscopic level. Histopathological examination showed that the patient had an underlying systemic senile amyloidosis of the transthyretin type with predominant involvement of the heart. Our patient, who has undergone a transcatheter aortic valve implantation due to severe, symptomatic aortic stenosis, after two balloon valvuloplasties, was also suffering from systemic senile amyloidosis of transthyretin type. This was discovered only postmortem on autopsy. Transcatheter aortic valve implantation may cause iatrogenic transient or permanent conduction disturbances with possible development of atrioventricular block and present the need for transient or permanent pacemaker implantation. However, her underlying infiltrative cardiomyopathy, could also lead to the same conduction system abnormalities. It is hard to conclude whether conduction disturbances in this case that lead to sudden cardiac death were entirely iatrogenic or entirely due to amyloidosis. In terms of complete atrioventricular block developing just after the procedure and ending in sudden cardiac death, conduction disturbances may have been due to her underlying infiltrative cardiomyopathy and exacerbated by the CoreValve implantation.