Catecholamine-induced cardiomyopathy in pheochromocytoma/paraganglioma (PPGL) is a potential fatal cardiovascular complication caused by excessive secretion of catecholamines by PPGL, leading to structural changes and functional abnormalities in the heart. According to the morphology and function of the heart, it is clinically divided into three types: dilated cardiomyopathy, Takotsubo cardiomyopathy, and hypertrophic cardiomyopathy. The treatment of catecholamine-induced cardiomyopathy in PPGL requires attention to drug selection, application of life support equipment, and perioperative management. Most patients with cardiac dysfunction can effectively improve after tumor resection. This article mainly reviews the diagnosis and treatment of catecholamine-induced cardiomyopathy in PPGL.
We tried to explore the value of contrast echocardiography (CEcho) on evaluating hypertrophic cardiomyopathy (HCM) with the inferior wall hypertrophy. A total of 114 patients with HCM were investigated. All the patients received CEcho and routine echocardiography (Echo), and 45 of them received cardiac magnetic resonance (CMR) and 47 of them received Holter. The frequency and percentage of inferior wall hypertrophy were analyzed in HCM patients, as well as the structure and function. The results showed that: (1) Inferior wall hypertrophy was detected in 55 patients (48%) by Echo, while 68 patients (60%) by CEcho. (2) There was no significant difference between CMR and CEcho in the measurement of inferior wall at end-diastole and end-systole. Thickness of inferior wall by CEcho tended to be higher than CMR. However, the inferior wall thickness measured by Echo was obviously lower than that by CMR (P < 0.05) and CEcho ( P < 0.05). (3) Bland-Altman plot suggested good consistency between CEcho and CMR in measuring inferior wall thickness. 95% CI of mean differences in inferior wall thickness between CEcho and CMR were smaller in HCM patients as compared with that between Echo and CMR. Unary linear regression analysis showed good degree of fitting between CEcho and CMR. (4) Holter showed that HCM patients with inferior wall hypertrophy were likely to have higher incidence of premature ventricular complexes (PVC) ≥ 500/24 h. We demonstrate that CEcho is rather sensitive in detecting inferior wall hypertrophy. Echo may underestimate the inferior wall thickness. The risk of ventricular premature beats may increase in HCM patients with inferior hypertrophy.
Glycogen storage-related cardiomyopathy refers to a group of rare hereditary disorders caused by genetic defects, characterized by aberrant glycogen accumulation in the myocardium. It includes glycogen storage diseases that can affect the myocardium, PRKAG2 cardiac syndrome, and Danon disease. Cardiac manifestations are predominantly characterized by a hypertrophic cardiomyopathy phenotype. The diagnostic workflow is initiated with cues from distinctive clinical presentations and family history, while genetic testing serves as the core diagnostic modality. Most of these conditions are managed with empirical symptomatic therapies. Currently, adeno-associated virus based gene therapy agents have entered clinical trials, with preliminary findings demonstrating favorable safety and efficacy profiles, thus holding promise as disease-specific therapeutic strategies. This article elaborates on the recent progress in the diagnosis and management of glycogen storage-related cardiomyopathy, along with the challenges confronted in this field, aiming to provide a reference for the clinical practice of such diseases.
