Catecholamine-induced cerebral vasospasm and multifocal infarctions in pheochromocytoma.
Endocrinology, diabetes & metabolism case reports
Palliative Care Opportunities Among Adults with Congenital Heart Disease - A Systematic Review.
Journal of pain and symptom management
We report the case of a 76-year-old male with a remote history of papillary thyroid cancer who developed severe paroxysmal headaches in the setting of episodic hypertension. Brain imaging revealed multiple lesions, initially of inconclusive etiology, but suspicious for metastatic foci. A search for the primary malignancy revealed an adrenal tumor, and biochemical testing confirmed the diagnosis of a norepinephrine-secreting pheochromocytoma. Serial imaging demonstrated multiple cerebral infarctions of varying ages, evidence of vessel narrowing and irregularities in the anterior and posterior circulations, and hypoperfusion in watershed areas. An exhaustive work-up for other etiologies of stroke including thromboembolic causes or vasculitis was unremarkable. There was resolution of symptoms, absence of new infarctions, and improvement in vessel caliber after adequate alpha-adrenergic receptor blockade for the management of pheochromocytoma. This clinicoradiologic constellation of findings suggested that the etiology of the multiple infarctions was reversible cerebral vasoconstriction syndrome (RCVS). Pheochromocytoma remains a poorly recognized cause of RCVS. Unexplained multifocal cerebral infarctions in the setting of severe hypertension should prompt the consideration of a vasoactive tumor as the driver of cerebrovascular dysfunction. A missed or delayed diagnosis has the potential for serious neurologic morbidity for an otherwise treatable condition.The constellation of multifocal watershed cerebral infarctions of uncertain etiology in a patient with malignant hypertension should trigger the consideration of undiagnosed catecholamine secreting tumors, such as pheochromocytomas and paragangliomas. Reversible cerebral vasoconstriction syndrome is a serious but reversible cerebrovascular manifestation of pheochromocytomas that may lead to strokes (ischemic and hemorrhagic), seizures, and cerebral edema. Alpha-adrenergic receptor blockade can reverse cerebral vasoconstriction and prevent further cerebral ischemia and infarctions. Early diagnosis of catecholamine secreting tumors has the potential for reducing neurologic morbidity and mortality in patients presenting with cerebrovascular complications.
View details for DOI 10.1530/EDM-20-0078
View details for PubMedID 32820130
CONTEXT: Little is known about advance care planning (ACP) and palliative care needs among adults with congenital heart disease (ACHD).OBJECTIVES: To identify and synthesize studies concerning palliative care among ACHD patients.METHODS: We searched five electronic databases (PubMed, Embase, SCOPUS, Web of Science, and CINAHL) using the keywords palliative care and congenital heart disease. Inclusion criteria were adults (age >18) with congenital heart disease and publications in English through March 3, 2019.RESULTS: Our search yielded 2872 studies, and after removal of duplicates, we screened 2319 abstracts and identified 7 for inclusion. Study findings were grouped into three domains: ACP, symptomatology, and End-of-Life care. Among the 5 cross-sectional studies, only 1-28% of ACHD patients recalled participating in ACP discussions with their doctors but 69-78% reported a strong interest and desire to participate in ACP. In one study, 46% (n=67) of patients had elevated anxiety symptoms (Hospital Anxiety and Depression Scale (HADS-A) ? 8) and 11% (n=15) had elevated depressive symptoms (HADS-A ? 8). ACHD patients who had a documented goals of care conversation prior to cardiac decompensation had a lower incidence of resuscitation and aggressive treatments at end-of-life (12% (n=3) vs 100% (n=12), p<0.001).CONCLUSION: While few ACHD patients complete advance directives, our findings support that many ACHD patients recognize the value of initiating end-of-life and goals of care conversations early on in the course of illness. Future studies investigating communication and implementation strategies of ACP as well as the symptom experience of patients with ACHD are needed.
View details for DOI 10.1016/j.jpainsymman.2019.07.025
View details for PubMedID 31404639