SummaryThyrotoxicosis is associated with cardiac dysfunction; more commonly, left ventricular dysfunction. However, in recent years, there have been more cases reported on right ventricular dysfunction, often associated with pulmonary hypertension in patients with thyrotoxicosis. Three cases of thyrotoxicosis associated with right ventricular dysfunction were presented. A total of 25 other cases of thyrotoxicosis associated with right ventricular dysfunction published from 1994 to 2017 were reviewed along with the present 3 cases. The mean age was 45 years. Most (82%) of the cases were newly diagnosed thyrotoxicosis. There was a preponderance of female gender (71%) and Graves’ disease (86%) as the underlying aetiology. Common presenting features included dyspnoea, fatigue and ankle oedema. Atrial fibrillation was reported in 50% of the cases. The echocardiography for almost all cases revealed dilated right atrial and or ventricular chambers with elevated pulmonary artery pressure. The abnormal echocardiographic parameters were resolved in most cases after rendering the patients euthyroid. Right ventricular dysfunction and pulmonary hypertension are not well-recognized complications of thyrotoxicosis. They are life-threatening conditions that can be reversed with early recognition and treatment of thyrotoxicosis. Signs and symptoms of right ventricular dysfunction should be sought in all patients with newly diagnosed thyrotoxicosis, and prompt restoration of euthyroidism is warranted in affected patients before the development of overt right heart failure.Learning points:Thyrotoxicosis is associated with right ventricular dysfunction and pulmonary hypertension apart from left ventricular dysfunction described in typical thyrotoxic cardiomyopathy.Symptoms and signs of right ventricular dysfunction and pulmonary hypertension should be sought in all patients with newly diagnosed thyrotoxicosis.Thyrotoxicosis should be considered in all cases of right ventricular dysfunction or pulmonary hypertension not readily explained by other causes.Prompt restoration of euthyroidism is warranted in patients with thyrotoxicosis complicated by right ventricular dysfunction with or without pulmonary hypertension to allow timely resolution of the abnormal cardiac parameters before development of overt right heart failure.

Levothyroxine remains the standard therapy for patients with hypothyroidism worldwide. Levothyroxine allergy is rarely seen and alternative therapies are less efficacious and scarcely available. The use of liothyronine (LT3) monotherapy is less favoured due to its short half-life and unpredictable pharmacological profile. We report a 59-year-old male with a hypersensitivity reaction to levothyroxine who was successfully desensitized with oral levothyroxine within a day using a 14-step protocol (Table 1).

An adrenal mass can be a diagnostic challenge as it is not easy to differentiate the adrenal glands from other adrenal pseudotumours with only radio-imaging. We report a 28-year-old patient who was diagnosed radiologically as an adrenal cortical carcinoma after he presented with abdominal pain and fullness. Biochemically, he demonstrated secondary hyperaldosteronism. Intra-operatively there was a huge mass, inferior to a normal right adrenal, which was histopathologically proven to be a dedifferentiated liposarcoma.

Background: Cushing’s syndrome (CS) in a patient with VHL has been attributed to a number of possible causes; pancreatic NET and renal cell carcinoma. The precise location of ectopic ACTH aid enormously in the management of VHL. Clinical case: A 31-year-old woman with Type 2 diabetes and family history of VHL presented with florid features of CS in her second trimester of pregnancy. A diagnosis of ACTH dependent CS was made based on elevated 24h urinary cortisol (1122 and 2448nmol/24hr, n<150-800), midnight cortisol (1322nmol/L, n<220nmol/L) and a detectable ACTH level (18.8pg/mL, n<10pg/mL). She underwent emergency caesarean section due to pre-eclampsia at 28 week gestation. Post-delivery, her morning cortisol was 2823nmol/L (n:171-536nmol/L). MRI pituitary was reported as normal. CT abdomen showed an enlarged pancreas which was almost completely cystic and a right renal mass (3.7 X 2.6 X 4cm). Serum chromogranin A was elevated (530.2ng/mL, n:27-94). 24h urinary free metanephrine was normal. Selective ACTH sampling was done together with bilateral inferior petrosal sinus sampling to elicit source of ACTH. Increased gradient of ACTH level compared to the periphery (138.9pg/mL vs. samples from IVC:115.1 pg/mL, renal vein:100.2 pg/mL) was detected from the hepatic vein that drains the pancreas via the portal venous system. Ketoconazole and metyrapone were given to control the cortisol level close to 600nmol/L prior to surgery. Preoperatively, IV hydrocortisone 100mg was administered, with additional 50mg given every 4 hours intraoperatively. Total pancreatectomy and right nephrectomy were performed. Inotropic support was required five hours into the surgery. Hydrocortisone was tapered down to a maintenance dose of 10mg, 5mg and 2.5mg TDS over 7 days. A week after surgery, am cortisol was 103nmol/L suggesting successful removal of ectopic ACTH source. Histopathological examination identified a solid tumour (18X12X12 mm) at the pancreatic tail which stained positive to Chromogranin A, synaptophysin and weakly positive to CD56. The mitoses was 0-1/10hpf with a Ki67 index of 2%. ACTH staining was positive. The renal mass was a Grade 1 clear cell renal cell carcinoma with equivocal ACTH staining. Two months later, there was resolution of cushingoid features. DOTATE and FDG/PET scans along with close surveillance using serum Chromogranin A and 6 monthly abdominal imaging were planned. Conclusion: When managing VHL with CS, there is always a possibility of more than one source of ACTH production. In delineating the cause of CS the use of selective ACTH sampling may be considered where functional imaging (DOTATATE) is unavailable. Reference : (1) Tamura K, Nishimori I, Ito T, Yamasaki I, Igarashi H, Shuin T. Diagnosis and management of pancreatic neuroendocrine tumor in von hippel-lindau disease. World J Gastroenterol . 2010;16(36):4515-4518. doi:10.3748/wjg.v16.i36.4515