Beta 2-microglobulin: case report of a rare cause of cardiac amyloidosis.

Background: Cardiac amyloidosis is caused by the deposition of misfolded proteins in the myocardium. The majority of cases of cardiac amyloidosis is caused by misfolded transthyretin or light chain proteins. In this case report, we discuss a case of a rare form of cardiac amyloidosis related to beta 2-microglobulin (B2M) in a patient not on dialysis. Case summary: A 63-year-old man was referred for workup of possible cardiac amyloidosis. Serum and urine immunofixation electrophoresis demonstrated no monoclonal bands, and the serum kappa/lambda light chain ratio was normal, excluding light chain amyloidosis. Bone scintigraphy imaging showed diffuse radiotracer uptake in the myocardium, and genetic testing of the Transthyretin gene was negative for variants. This workup was consistent with wild-type transthyretin cardiac amyloidosis. The patient, however, later underwent endomyocardial biopsy due to factors inconsistent with this diagnosis, including a young age of presentation and a strong family history of cardiac amyloidosis despite no variants in the Transthyretin gene. This showed B2M-type amyloidosis, and genetic testing of the B2M gene showed a heterozygous Pro32Leu (p. P52L) mutation. The patient underwent heart transplantation with normal graft function 2 years post transplant. Discussion: While contemporary advancements allow for the non-invasive diagnosis of transthyretin cardiac amyloidosis with positive bone scintigraphy and negative monoclonal protein screen, clinicians should be aware of rarer forms of amyloidosis where endomyocardial biopsy is required to make the diagnosis.

Systematic Review of Malpractice Litigation in the Diagnosis and Treatment of Acute Stroke.

Background and Purpose- The emergency management of stroke is complex and highly time-sensitive. Recent landmark trials demonstrating the strong benefit of thrombectomy have led to rapid change in stroke management. This article reviews a large number of medical malpractice lawsuits related to the emergency management of stroke to characterize factors involved in these lawsuits. Methods- Three large legal databases were used to search for jury verdicts and settlements in cases related to the acute care of stroke patients in the United States. Search terms included "stroke" and "medical malpractice." Cases were screened to include only cases in which the allegation involved negligence in the acute care of a patient suffering a stroke. Results- We found 246 medical malpractice cases related to the acute management of ischemic stroke and 26 related to intracranial hemorrhage. Seventy-one cases specifically alleged a failure to treat with tPA (tissue-type plasminogen activator) and 7 cases alleged a failure to treat, or to timely treat, with thrombectomy. Overall there were 151 cases (56%) which ended with no payout, 74 cases (27%) were settled out of court, and 47 cases (17%) went to court and resulted in a verdict for the plaintiff. The average payout in settlements was $1 802 693, and the average payout in plaintiff verdicts was $9 705 099. Conclusions- Malpractice litigation is a risk in acute stroke care and can lead to significant financial consequences. The majority of malpractice lawsuits related to the emergency management of stroke allege a failure to diagnose and failure to treat. Allegations of a failure to treat acute ischemic stroke with tPA were frequently found and are common in lawsuits. Allegations of a failure to treat a large vessel occlusion with thrombectomy were less frequently found. Given recent changes in practice guidelines and the demonstrated strong treatment effect of thrombectomy, it is likely that such litigation will increase in the coming years.

An analysis of malpractice litigation in the surgical management of carotid artery disease.

OBJECTIVE: Carotid artery disease is a common illness that can pose a significant risk if left untreated. Treatment via carotid endarterectomy (CEA) or carotid artery stenting (CAS) can also lead to complications. Given the risk of adverse events related to treating, or failing to treat, carotid artery disease, this is a possible area for litigation. The aim of this review is to provide an overview of the medicolegal factors involved in treating patients suffering carotid artery disease and to compare litigation related to CEA and CAS. METHODS: Three large legal databases were used to search for jury verdicts and settlements in cases related to untreated carotid artery disease, CEA, and CAS. Search terms included "endarterectomy," "medical malpractice," "carotid," "stenosis," "stenting," "stent," and combinations of those words. Three types of cases were considered relevant: 1) cases in which the primary allegation was negligence performing a CEA or perioperative care (CEA-related cases); 2) cases in which the primary allegation was negligence performing a CAS or perioperative care (CAS-related cases); and 3) cases in which the plaintiff alleged that a CEA or CAS should have been performed (failure-to-treat [FTT] cases). RESULTS: One hundred fifty-four CEA-related cases, 3 CAS-related cases, and 67 FTT cases were identified. Cases resulted in 133 verdicts for the defense (59%), 64 settlements (29%), and 27 plaintiff verdicts (12%). The average payout in cases that were settled outside of court was $1,097,430 and the average payout in cases that went to trial and resulted in a plaintiff verdict was $2,438,253. Common allegations included a failure to diagnose and treat carotid artery disease in a timely manner, treating with inappropriate indications, procedural error, negligent postprocedural management, and lack of informed consent. Allegations of a failure to timely treat known carotid artery disease were likely to lead to a payout (60% of cases involved a payout). Allegations of procedural error, specifically where the resultant injury was nerve injury, were relatively less likely to lead to a payout (28% of cases involved a payout). CONCLUSIONS: Both diagnosing and treating carotid artery disease has serious medicolegal implications and risks. In cases resulting in a plaintiff verdict, the payouts were significantly higher than cases resolved outside the courtroom. Knowledge of common allegations in diagnosing and treating carotid artery disease as well as performing CEA and CAS may benefit neurosurgeons. The lack of CAS-related litigation suggests these procedures may entail a lower risk of litigation compared to CEA, even accounting for the difference in the frequency of both procedures.