ABSTRACT
The mechanisms responsible for the development of apical aneurysms in cases of hypertrophic cardiomyopathy (HCM) are currently unclear but likely involve multiple factors. Here, we present a case of HCM with marked subendocardial fibrosis involving the apical and proximal portions of the left ventricle. A 71-year-old man with left ventricular hypertrophy presented with signs and symptoms of heart failure. The presence of asymmetrical left ventricular hypertrophy and bilateral, thickened ventricular walls with an apical aneurysm on transthoracic echocardiography suggested a diagnosis of HCM with ventricular dysfunction. No intraventricular pressure gradients with obstruction were identified. Late gadolinium enhancement (LGE) with cardiac magnetic resonance imaging and endomyocardial biopsies showed subendocardial fibrosis involving the apical aneurysm and proximal portion. Whereas LGE in a transmural pattern is commonly observed in HCM apical aneurysms, subendocardial LGE, as noted in the present case, is a relatively rare occurrence. Thus, the present case may provide unique insights into the adverse remodeling process and formation of apical aneurysms in cases of HCM.
ABSTRACT
The imaging features of chronic periaortitis resemble those of infected aneurysms. Two illustrative cases of chronic periaortitis, in which the etiologies were caused by IgG4-related disease, are presented. The first case involved a 68-year-old man who presented with vague discomfort in his lower abdomen. The second case was a 42-year-old man who presented with a fever of 38°C and persistent, vague chest discomfort. Both cases demonstrated an increased amount of connective tissue around the aorta in computed tomography images and low intensity in the T2-weighed sequence and high intensity in the diffusion-weighed sequence, suggesting the presence of inflammation, in the magnetic resonance imaging. Negative blood cultures, elevated IgG4 levels, and pathological findings confirmed the diagnosis as chronic periaortitis due to IgG4-related disease. This is a newly recognized syndrome of unknown etiology, characterized by a fibroinflammatory condition, tumefactive lesions, and a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells. Both cases were successfully treated with corticosteroids. Infected aneurysms need to be carefully differentiated from this syndrome in view of the similar imaging features.
ABSTRACT
We present a case of syncope caused by coronary artery spasm without chest pain leading to ventricular fibrillation despite of vasodilator therapy with a calcium channel blocker (CCB). A 68-year-old man presented with two episodes of syncope without chest pain. Ergonovine provocation test induced a diffuse spasm in the right coronary artery (RCA) and subsequently, ventricular fibrillation. Under the therapy with multiple vasodilators including two CCBs, a second ergonovine provocation induced a spasm of the proximal RCA resulting in complete obstruction. Owing to drug-resistant coronary spasm complicated by ventricular fibrillation, an implantable cardioverter defibrillator (ICD) was implanted. This case report highlights the occurrence of syncope caused by coronary artery spasm without chest pain that was refractory to single CCB therapy and needed ICD implantation. Therapy with multiple vasodilators, including two or more CCBs, along with ICD implantation may be required to treat such refractory coronary artery spasms leading to lethal arrhythmia.
