Reversible Catecholamine Induced Cardiomyopathy

Background: Pheochromocytomas and paragangliomas (PPGL) are rare neuro-endocrine tumors associated with a myriad of poor outcomes as a result of long-term exposure to catecholamines. Although paragangliomas are less commonly associated with increased catecholamine production than adrenal pheochromocytomas, there have been a few reports of catecholamine-induced cardiomyopathy in patients diagnosed with PPGL. We report a case of a PPGL associated with hypercoagulability and cardiomyopathy. Clinical Case: 42-year-old man with uncontrolled hypertension presented to the emergency department with abdominal pain. On CT imaging, he was found to have hepatic lesions, aortocaval lymph node concerning for metastatic disease, left renal infarct, and a left ventricular thrombus. Soon after his admission, he developed acute ataxia, gaze palsies and left hemiparalysis. CTA of the head showed a basilar artery thrombus [FJ1] which was treated with emergent thrombectomy. In addition patient had absent distal pulse of the right foot[FJ2], and found to have thrombus of the popliteal artery, which was treated with thrombectomy. Further workup with abdominal MRI showed retroperitoneal mass[FJ3] and multiple hepatic lesions concerning for metastatic extra-adrenal neuroendocrine tumor. Plasma normetanephrine was 4.5 nmol/L (ULN 0.89), plasma metanephrine 0.3 nmol/L (ULN 0.49) Chromogranin A was 387 ng/ml (ULN 160). Ga-68 DOTATE scan was consistent with an extra adrenal paraganglioma with less prominent radiotracer activity in hepatic lesion concerning for dedifferentiated metastatic disease. In addition, echocardiogram showed reduced LV ejection fraction of 24% with global hypokinesis, and confirmed the LV thrombus. Cardiac MRI showed infiltrative nonischemic cardiomyopathy and mild dilation of left ventricle, as well as patchy delayed enhancement in the basal and inferoseptal walls suggestive of myocarditis. Treatment included rivaroxaban[FJ4], lisinopril, doxazosin, furosemide, and carvedilol. Several months after discharge, his EF improved to 48%. Hepatic lesions concerning for dediffertiated tumor vs unrelated malignancy was biopsied[FJ5] and consistent with neuroendocrine tumor. Future plan for his PPGL include revaluation for resection of retroperitoneal mass or DOTA Lutathera therapy. Conclusions: This case highlights a young man who was incidentally found to have metastatic paraganglioma with catecholamine-induced cardiomyopathy. The patient was asymptomatic until he developed significant heart failure. Cardiomyopathy in this setting is thought to be secondary to uncontrolled hypertension, as well as sympathetic overdrive from overstimulation of norepinephrine. We present the case to highlight the management challenges in a patient with PPGL with significant cardiovascular compromise and limited therapeutic options.

Management of supracondylar fractures of the humerus in children

Supracondylar fractures of the humerus are the most frequent fractures of the paediatric elbow, with a peak incidence at the ages of five to eight years. Extension-type fractures represent 97% to 99% of cases. Posteromedial displacement of the distal fragment is the most frequent; however, the radial and median nerves are equally affected. Flexion-type fractures are more commonly associated with ulnar nerve injuries. Concomitant upper-limb fractures should always be excluded. To manage the vascular status, distal pulse and hand perfusion should be monitored. Compartment syndrome should always be borne in mind, especially when skin puckering, severe ecchymosis/swelling, vascular alterations or concomitant forearm fractures are present. Gartland’s classification shows high intra- and inter-observer reliability. Type I is treated with casting. Surgical treatment is the standard for almost all displaced fractures. Type IV fractures can only be diagnosed intra-operatively. Closed reduction and percutaneous pinning is the gold standard surgical treatment. Open reduction via the anterior approach is indicated for open fractures, absence of the distal vascular flow for > 10 to 15 minutes after closed reduction, and failed closed reduction. Lateral entry pins provide stable fixation, avoiding the risk of iatrogenic ulnar nerve injury. About 10% to 20% of displaced supracondylar fractures present with alterations in vascular status. In most cases, fracture reduction restores perfusion. Neural injuries occur in 6.5% to 19% of cases involving displaced fractures. Most of them are neurapraxias and it is not routinely indicated to explore the nerve surgically.Cite this article: EFORT Open Rev 2018;3:526-540. DOI: 10.1302/2058-5241.3.170049

Respond to the letter on ‘Interobserver reliability of the ankle brachial index, toe–brachial index and distal pulse palpation in patients with diabetes: a methodological issue’

We respond to the letter of Nadery and Shahsavari regarding our paper entitled ‘Interobserver reliability of the ankle brachial index, toe -brachial index and distal pulse palpation in patients with diabetes. In this letter, we concluded that despite some limitations, the kappa coefficient is an informative measure of agreement in most circumstances that we can use in this type of clinical research.

Interobserver reliability of the ankle–brachial index, toe–brachial index and distal pulse palpation in patients with diabetes

Objective: We conducted a prospective pilot study in patients with diabetes to analyse the interobserver reliability of the ankle–brachial index, toe–brachial index and distal pulse palpation depending on the training of the professional involved. Materials and Methods: The ankle–brachial index, toe–brachial index and distal pulses were assessed by three clinicians with different levels of experience on the same day. Measurements were supervised and recorded by a fourth clinician. Results: Twenty-one patients (42 ft) were included in this study. We observed moderate agreement between clinicians in the palpation of posterior tibial arteries (K = 0.45, p < 0.001) and low agreement in dorsalis pedis arteries (K = 0.33, p < 0.001). The measurement of ankle–brachial index had moderate agreement between clinicians in patients with medial arterial calcification (K = 0.43, p < 0.001) and low agreement in patients with normal ankle–brachial index (K = 0.4, p < 0.001). The measurement of toe–brachial index had moderate agreement between clinicians in patients with a normal toe–brachial index (K = 0.4, p < 0.001) and in patients with medial arterial calcification (K = 0.60, p < 0.001). Conclusion: Palpation of distal pulses, ankle–brachial index and toe–brachial index determination in patients with diabetes are not highly reproducible and reliable between clinicians with different levels of experience under routine conditions.

Abstract 3028: Muscle Microdialysis to Confirm Subischemic Injury in a Clinical Trial of Remote Ischemic Preconditioning

Introduction: Transient exposure to sublethal ischemia-reperfusion injury of one tissue confers a protective effect to remote organs, referred to as remote ischemic preconditioning (RIPC). Confirmation that the desired effect of sublethal ischemia is occurring in the tissues used to induce RIPC requires an objective measure before this technique can be used consistently in the clinical practice. Methods: Three to four RIPC sessions were conducted during non-consecutive days in patients with aneurysmal SAH aged 18-80, within days 4-12 of aneurysm rupture. Sessions consisted of 4 cycles of 5-minutes of lower extremity blood pressure cuff inflation to 30 mmHg above the systolic blood pressure, followed by 5 minutes of reperfusion. The absence of dorsalis pedis pulse was confirmed by Doppler evaluation and, if pulse signals were detected, the cuff was inflated until they disappeared. During periods of reperfusion, distal pulse recovery was confirmed. To evaluate limb sublethal ischemic injury, patients were monitored with a microdialysis probe inserted into the mass of the gastrocnemius muscle on the preconditioning limb. Glucose, lactate, pyruvate, lactate/pyruvate ratio, and glycerol levels were compared before and immediately after the RIPC sessions. Results: Twenty-one RIPC sessions were performed in 6 patients (50% male, mean age 48.5, Fisher 4, H&H 2-4). The procedure was well tolerated and there were no systemic changes in HR, BP or CVP. An average follow up of 25 days of daily examination after the last RIPC session demonstrated no complications associated with the RIPC procedure or the microdialysis probe implantation. Muscle microdialysis during the RIPC sessions showed a significant increase in L/P ratio (21.2 to 26.8, p=0.001) and lactate (3.0 to 3.9mmol/L, p=0.002), indicating muscle ischemia. There was no significant variation in glycerol (234 to 204μ g/L, p=0.43), indicating no permanent cell damage. Conclusion: The RIPC protocol used in this study is safe, well tolerated, and induces transient metabolic changes in skeletal muscles comparable with sublethal ischemia. Muscle microdialysis can be used safely as a confirmatory tool in the induction of RIPC.