Cardiogenic Shock in Non-Ischemic Cardiomyopathy: Dynamic Mechanical Circulatory Support and Pathophysiology Illustration.

Nonischemic cardiomyopathy (NICM) is a significant cause of cardiogenic shock (CS). We present a case of a 56-year-old previously healthy man who arrived with vague abdominal symptoms, over 2 weeks. Subsequently, the patient's condition rapidly deteriorated over 12 hours, leading to cardiogenic shock categorized as Society for Cardiovascular Angiography and Interventions (SCAI) stage D. Echocardiography and right heart catheterization confirmed multiorgan failure secondary to severe cardiac dysfunction. Mechanical circulatory support was initiated using an Impella CP device 20 hours after admission due to ongoing deterioration. Considering refractory cardiogenic shock and within 24 hours, the patient received combined veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and Impella CP support (ECPElla). With gradual improvement in the patient's clinical status and organ function, successful weaning from VA ECMO to Impella 5.5 was achieved. Ultimately, the patient underwent a successful orthotopic heart and kidney transplantation, marking a significant milestone in his recovery. The case underscores the importance of promptly identifying and responding to cardiogenic shock through invasive hemodynamic assessment. Collaborative decision-making involving a multidisciplinary team played a crucial role in the initiation, escalation, and eventual weaning of mechanical circulatory support, culminating in the successful bridging to a dual organ transplantation for this patient with CS secondary to NICM.

Decoding Postinfarction Left Ventricular Pseudoaneurysm.

Recognizing true from pseudo left ventricular aneurysm after myocardial infarction is paramount to guide clinical management and determine need for surgical urgency. We discuss a case of a postinfarction pseudoaneurysm that poses unique anatomic challenges and may hold a secret "DaVinci code" beyond current diagnostic criteria. (Level of Difficulty: Advanced.).

Sudden cardiac death in the young: A consensus statement on recommended practices for cardiac examination by pathologists from the Society for Cardiovascular Pathology.

Sudden cardiac death is, by definition, an unexpected, untimely death caused by a cardiac condition in a person with known or unknown heart disease. This major international public health problem accounts for approximately 15-20% of all deaths. Typically more common in older adults with acquired heart disease, SCD also can occur in the young where the cause is more likely to be a genetically transmitted process. As these inherited disease processes can affect multiple family members, it is critical that these deaths are appropriately and thoroughly investigated. Across the United States, SCD cases in those less than 40 years of age will often fall under medical examiner/coroner jurisdiction resulting in scene investigation, review of available medical records and a complete autopsy including toxicological and histological studies. To date, there have not been consistent or uniform guidelines for cardiac examination in these cases. In addition, many medical examiner/coroner offices are understaffed and/or underfunded, both of which may hamper specialized examinations or studies (e.g., molecular testing). Use of such guidelines by pathologists in cases of SCD in decedents aged 1-39 years of age could result in life-saving medical intervention for other family members. These recommendations also may provide support for underfunded offices to argue for the significance of this specialized testing. As cardiac examinations in the setting of SCD in the young fall under ME/C jurisdiction, this consensus paper has been developed with members of the Society of Cardiovascular Pathology working with cardiovascular pathology-trained, practicing forensic pathologists.

Outflow graft foreign body reaction and thrombosis in HeartMate 3 left ventricular assist device.

Outflow cannula occlusion is an infrequent complication occurring among recipients of continuous flow left ventricular assist devices. Hereby, we present a case of a 66-year-old man with a HeartMate 3 left ventricular assist device that presented 18 months after implantation and during his admission, evidence of poor left ventricular unloading and forward flow uncovered intrinsic and extrinsic outflow cannula obstruction. The patient's course was complicated by cardiogenic shock, diffuse alveolar hemorrhage and multiorgan failure. Post-mortem examination suggested foreign body reaction as a potential contributor of outflow graft obstruction at the level of the bend relief.

Congenitally corrected transposition of the great arteries.

Congenitally corrected transposition of the great arteries (cc-TGA, also known as L-transposition) is a rare condition that represents approximately 0.5% of congenital heart disease. It is characterized by atrioventricular and ventriculoarterial discordance. Patients with cc-TGA are at risk for cardiac arrhythmias due to abnormalities of the conduction system. We present an autopsy case of a 49-year-old man with cc-TGA who died unexpectedly, likely as a result of a fatal arrhythmia. This case illustrates the cardiac pathology characteristic of cc-TGA and describes how to grossly identify inversion of the atrioventricular valves.

The Postmortem Features of Mucormycosis.

Mucormycosis is a rare and severe invasive fungal infection caused by ubiquitous fungi of the order Mucorales. Infection often occurs in immunocompromised hosts and includes cutaneous, pulmonary, gastrointestinal, rhinocerebral, and disseminated forms of disease. Although the clinical characteristics of mucormycosis are well established, infection can be difficult to diagnose antemortem, resulting in frequent postmortem diagnoses. Despite this, the gross appearance of mucormycosis at autopsy has not been well described. In the present report we illustrate the gross and histologic findings in four autopsy cases of mucormycosis, including one case of pulmonary disease and three cases of disseminated mucormycosis with cerebral, pulmonary, hepatic, renal, and gastrointestinal involvement. In all cases autopsy examination demonstrated characteristic hemorrhagic infarcts with a targetoid appearance in the affected organs. These findings are secondary to fungal angioinvasion with subsequent thrombosis and tissue necrosis. Mucormycosis should be suspected at autopsy when these characteristic infarcts are identified within the proper clinical context, and a high suspicion for atypical infections should be maintained postmortem in immunosuppressed patients.

3,4-Methylenedioxymethamphetamine (MDMA, Ecstasy) Intoxication in a Young Adult with Giant Coronary Aneurysms from Kawasaki Disease.

Kawasaki disease is an acute vasculitis of unknown etiology that usually occurs in children less than five years old. Coronary artery aneurysm due to arteritis is the most common cardiac complication. Chronic aneurysms are associated with an increased risk of developing luminal thrombosis and ischemic myocardial injury. We present a case of an 18-year-old male with a history of Kawasaki disease complicated by chronic giant aneurysms of the right and left coronary arteries. Serial echocardiographic studies and treadmill electrocardiogram stress tests as recent as 1.5 years before death revealed excellent cardiac function by clinical criteria. The decedent sustained a witnessed collapse after ingesting 3,4-methylenedioxymethamphetamine (MDMA) and ethanol. He was pronounced dead in the emergency department after unsuccessful resuscitative efforts. Autopsy revealed large aneurysms of the three main epicardial coronary arteries with extensive foci of severe stenosis by intimal fibrosis and organizing thrombus. Microscopic examination revealed multifocal severe myocyte hypertrophy. There were remote microinfarcts in the anterior and posterior aspects of the left ventricle and a recent, healing microinfarct in the posterior aspect of the left ventricle. Toxicology examination of postmortem femoral blood revealed MDMA, ethanol, and amiodarone. This case illustrates the residual, lasting effects of cardiac disease due to Kawasaki disease and a potential complication in the setting of use of an illicit stimulant, MDMA, an amphetamine derivative that produces sympathetic activation and cardiovascular effects including tachycardia, vasoconstriction, dysrhythmias, and coronary artery spasm. Kawasaki disease-related abnormalities of the heart likely resulted in a lower threshold for developing a fatal cardiac dysrhythmia under the circumstance of stimulant use.

Pathology Milestones: Assessing Clinical Competency by Committee.

All Accreditation Council for Graduate Medical Education accredited pathology residency training programs are now required to evaluate residents using the new Pathology Milestones assessment tool. Similar to implementation of the 6 Accreditation Council for Graduate Medical Education competencies a decade ago, there have been challenges in implementation of the new milestones for many residency programs. The pathology department at the University of Iowa has implemented a process that divides the labor of the task in rating residents while also maintaining consistency in the process. The process is described in detail, and some initial trends in milestone evaluation are described and discussed. Our experience indicates that thoughtful implementation of the Pathology Milestones can provide programs with valuable information that can inform curricular changes.

Four postmortem case reports with quantitative detection of the synthetic cannabinoid, 5F-PB-22.

In January 2014, the US government temporarily designated 5F-PB-22, along with three other synthetic cannabinoids (AB-FUBINACA, ADB-PINACA and PB-22), into Schedule I. Over the course of a 4-month time period (July-October 2013), our laboratory quantitatively identified 5F-PB-22 in specimens obtained from four postmortem cases. We describe the four cases, to include pertinent autopsy findings and decedent histories, together with quantitative results for 5F-PB-22 determined in postmortem blood and antemortem serum. Samples were prepared via a liquid-liquid extraction at pH 10.2 into hexane : ethyl acetate. Instrumental analysis was achieved with liquid chromatography coupled with electrospray ionization tandem mass spectrometry operating in multiple reaction monitoring mode. Two ion transitions were monitored for the analyte of interest, and one ion transition was monitored for the internal standard. The observed concentration range of 5F-PB-22 is 1.1-1.5 ng/mL for three postmortem blood specimens and one antemortem serum specimen. Three of the decedents experienced abrupt, sudden death; however, one decedent expired after a rapidly deteriorating hospital course.

Epstein-Barr virus-associated diffuse large B-cell lymphoma arising on cardiac prostheses.

Primary cardiac lymphoma is extremely rare and lymphoma arising in association with prosthetic valves has been described in only 3 case reports. We describe 3 patients with diffuse large B-cell lymphoma (DLBCL) involving prosthetic heart valves and a synthetic tube graft. All 3 specimens showed shallow layering of acellular fibrinous debris over the prosthetic or synthetic materials, with tumor lymphocytes present at the luminal surface. There were frequent mitoses and abundant karyorrhectic debris. All demonstrated a nongerminal center B-cell phenotype. All 3 cases were positive for Epstein-Barr virus, but there was no staining for human herpes virus 8. There was no other evidence of distant disease at the time of diagnosis and no recurrence or dissemination occurred after surgical removal of the prosthesis, though follow-up was limited. On the basis of 2008 World Health Organization diagnostic criteria, we believe these cases should be classified as DLBCL associated with chronic inflammation. However, unlike the characteristically poor prognosis reported in this entity, we hypothesize that the disease resectability in these cardiac sites, in many cases, may allow for a better prognosis than DLBCL with chronic inflammation at other less resectable sites.