Summary
Pheochromocytoma/paraganglioma (PPGL) are neuroendocrine tumors that can secrete catecholamines. The authors describe a challenging case who presented as stress cardiomyopathy and myocardial infarction (MI). A 76-year-old man, with a medical history of Parkinson’s disease, type 2 diabetes mellitus, hypertension, dyslipidaemia and a previous inferior MI in 2001, presented to the emergency department due to chest pain, headaches and vomiting. He also reported worsening blood glucose levels and increasing constipation over the preceding weeks. BP was 185/89 mmHg (no other relevant findings). EKG had ST segment depression in leads V2-V6, T troponin was 600 ng/L (<14) and the echocardiogram showed left ventricular hypokinesia with mildly compromised systolic function. Nevertheless, he rapidly progressed to severe biventricular dysfunction. Coronary angiogram showed a 90% anterior descendent coronary artery occlusion (already present in 2001), which was treated with angioplasty/stenting. In the following days, a very labile BP profile and unexplained sinus tachycardia episodes were observed. Because of sustained severe constipation, the patient underwent an abdominal CT that revealed a retroperitoneal, heterogeneous, hypervascular mass on the right (62 × 35 mm), most likely a paraganglioma. Urinary metanephrines were increased several fold. 68Ga-DOTANOC PET-CT scan showed increased uptake in the abdominal mass (no evidence of disease elsewhere). He was started on a calcium-channel blocker and alpha blockade and underwent surgery with no major complications. Eight months after surgery, the patient has no evidence of disease. Genetic testing was negative for known germline mutations. This was a challenging diagnosis, but it was essential for adequate cardiovascular stabilization and to reduce further morbidity.
Learning points:
PPGL frequently produces catecholamines and can manifest with several cardiovascular syndromes, including stress cardiomyopathy and myocardial infarction.
Even in the presence of coronary artery disease (CAD), PPGL should be suspected if signs or symptoms attributed to catecholamine excess are present (in this case, high blood pressure, worsening hyperglycaemia and constipation).
Establishing the correct diagnosis is important for adequate treatment choice.
Inodilators and mechanical support might be preferable options (if available) for cardiovascular stabilization prior to alpha blockade and surgery.
Laboratory interference should be suspected irrespective of metanephrine levels, especially in the context of treated Parkinson’s disease.
Chronic kidney disease is highly prevalent in patients with diabetes mellitus. There are many specific aspects in the treatment of diabetes that have to be taken into account when there is concomitant nephropathy. All antihyperglycemic drugs can be used in earlier stages of chronic kidney disease. With worsening nephropathy, most will require dose adjustments (some eventually suspension) and increased monitoring of adverse events and kidney function. New treatment options that are safe and effective are now available for more advanced stages of disease. Moreover, findings from large clinical trials suggest that some drugs, namely GLP-1 receptor agonists and SGLT2 inhibitors, might potentially have kidney and cardiovascular protective effects, although clinical significance and putative mechanisms are not yet fully understood. The aim of this review is to provide an updated overview of relevant data to guide clinical practice in the use of antihyperglycemic agents in chronic kidney disease patients, including older drugs and also the most recently available treatment options.
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