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BackgroundAmyloidosis is a systemic disorder of abnormal protein folding and deposition resulting in a range of symptoms including neuropathy, heart failure, renal disease, and dermatologic findings. The two most common types of amyloidosis that affect the heart are transthyretin (ATTR) amyloidosis and light chain (AL) amyloidosis, which vary in clinical presentation. Skin findings such as periorbital purpura are considered more specific for AL amyloidosis. However, there are rare cases of ATTR amyloidosis causing the same dermatologic findings.Case SummaryA 69-year-old female presented for evaluation of amyloidosis after cardiac imaging done at the time of a recent atrial fibrillation ablation showed signs of infiltrative disease. On examination, she had periorbital purpura which she reportedly had for years without receiving a diagnosis, as well as macroglossia with teeth indentation. These exam findings, in addition to her transthoracic echocardiogram showing apical sparing, are typically considered characteristic of AL amyloidosis. Subsequent workup revealed the presence of hereditary ATTR (hATTR) amyloidosis with a heterozygous pathogenic variant in the TTR gene producing the p.Thr80Ala mutation.ConclusionSpontaneous periorbital purpura is thought to be pathognomonic for AL amyloidosis. However, we describe a case of hereditary ATTR amyloidosis with the Thr80Ala TTR genetic variant presenting initially with periorbital purpura, the first case documented in the literature to our knowledge.

In October 2018, the Organ Procurement and Transplantation Network (OPTN) revised the donor heart allocation system in an attempt to prioritize those patients with highest clinical urgency, reduce waitlist morality, and improve geographic equity in organ allocation. Our goal was to review the changes in the heart allocation policy and its impact on transplant characteristics and outcomes. After the new 2018 donor heart allocation system became effective, there has been a trend toward increased use of temporary mechanical circulatory support. Also, initial reports suggested reduced post-transplant survival, although the initial analysis was limited by short follow-up and small sample size. Recent reports however illustrate survival outcomes similar to those of the previous allocation system. The new donor heart allocation policy has been associated with a change in management strategies for bridging patients to transplantation, with increased utilization of temporary mechanical circulatory support, with still uncertain effects on post-transplant survival.

Introduction: Implantable cardioverter defibrillator (ICD) implantation in patients with cardiac amyloidosis is controversial, with no clear guidelines for clinical decision-making.. The role of ICD implantation in hereditary Transthyretin Amyloid Cardiomyopathy (hATTR-CM) is unclear. We sought to establish the burden of ventricular arrhythmias and the outcomes of ICD implantation in a single center cohort of hATTR-CM patients. Methods: A total of 69 patients with a confirmed diagnosis of hATTR-CM from genetic testing, and technetium pyrophosphate (PYP) scanning, or endomyocardial biopsy underwent retrospective chart review for demographic, clinical, and arrhythmia data. Results: Seventy-four percent of the cohort was male, with a mean age at diagnosis of 68 (SD=18 years). Sixty-five patients (94.2%) patients were African-American; all of whom carried the Valine 122 Isoleucine mutation. Most had systolic heart failure (New York Heart Association Staging II [18, 26%] and III [40, 58.8%]); 37 (54%) patients had an LVEF ≤ 35%. Thirty-six (52.2%) patients had documented episodes of non-sustained ventricular tachycardia (NSVT), three (4.3%) with ventricular tachycardia (VT), and one (1.5%) with ventricular fibrillation (VF). A total of 15 (21.7%) patients had ICDs placed for prevention of sudden cardiac death in the setting of low LVEF (EF Conclusions: In a cohort of patients with hATTR-CM, we observed a high incidence of NSVT, yet only 41% of patients with severe systolic heart failure had ICDs implanted. A high rate of successful defibrillation and no inappropriate ICD shocks were noted, suggesting that ICDs should be strongly considered, and may be underutilized, in patients with systolic heart failure and/or arrhythmias in the setting of hATTR-CM.

Background The Impella (ABIOMED, Danvers, MA) is a temporary left ventricular support device, intended for short-term cardiac support for percutaneous procedures and cardiac shock. The Impella device is available in several sizes capable of producing different flows. It is FDA approved for up to 6 days of support. The device can provide significantly higher blood flow than an intra-aortic balloon pump and can be implanted percutaneously. We sought to describe potential complications and outcomes associated with extended Impella use for cardiogenic shock. Methods We retrospectively evaluated patients who underwent implantation of either the Impella CP (Impella 3.5) or Impella 5.0 from September 1, 2017 to September 1, 2019 at our institution. Hemolysis was defined as plasma free hemoglobin level > 50 mg/dL, a lactate dehydrogenase (LDH) level > 500 units/L, and bilirubin increase > 1 mg/dL. Results 58 patients underwent percutaneous insertion of an Impella device, with the indication of cardiogenic shock in 32 patients (55%). In these 32 patients, only one had an Impella 2.5 device, 21 had an Impella CP (66%), and 10 (31%) had an Impella 5.0. The average age at time of implantation was 55 ± 17 years, and 25 patients were male (78%). Ejection fraction was 17 ± 11.7%. The average duration of Impella support was 5.5 days (1, 16 days). Complications included hematoma at the time of insertion (3 patients), leg ischemia (2 patients), improper positioning (7 patients), acute renal failure (28 patients), and hemolysis (20 patients). Hemolysis developed in 12 patients (57%) with the Impella CP and in 8 patients (80%) with the Impella 5.0, with an average LDH of 1452 units/L. 8 patients were transitioned to veno-arterial (VA) extracorporeal membrane oxygenation (ECMO), with the Impella utilized for left ventricular venting. 9 patients (28%) eventually underwent durable left ventricular assist device (LVAD) implantation on average 7 ± 6.1 days after Impella, and two patients underwent heart transplantation, both 9 days following Impella insertion. 23 patients (72%) did not survive past the initial hospitalization. Conclusion The Impella is a percutaneous temporary left ventricular support device used to provide additional cardiac output in patients with cardiogenic shock. Serious complications can occur with extended support, including hemolysis, acute renal failure, and high mortality. Of our 28 patients who received an Impella, 11 were able to be successfully bridged to either LVAD or heart transplantation with the Impella.

Introduction Aortic insufficiency (AI) following left ventricular assist device (LVAD) placement is a serious, well established complication. Transcatheter aortic valve replacement (TAVR) has been successfully used in LVAD patients developing AI. However, femoral/iliac arterial access required for TAVR is not always possible due to arterial calcifications and small arterial size. Transcaval approach is a novel alternative percutaneous catheter route from the IVC, through the retroperitoneal space, into the abdominal aorta. Transcaval TAVR in LVAD patients with severe AI has not been described. CASE Patient is a 28 year old female with history of postpartum STEMI secondary to spontaneous LAD/LCX dissection requiring CABG with subsequent cardiogenic shock requiring emergent LVAD (HeartMate 3) placement. One month following LVAD implantation, she presents with intractable nausea and vomiting. Physical exam illustrates a cachectic female who was hypotensive, tachycardic, with cool extremities. Laboratory evaluation reveals elevated lactate, transaminases and creatinine. Abdominal CTA shows a prior dissection in the infrarenal abdominal aorta, occlusion of the right external iliac artery and stenosis in the left external iliac artery. On echocardiography, there is severe, continuous, aortic regurgitation increased in severity compared to baseline. Right heart catheterization illustrates right atrial pressure 18 mmHg, pulmonary artery pressure 53/25 mmHg, pulmonary wedge pressure 24 mmHg, and cardiac index 1.3 L/min/m2. Patient is referred for urgent TAVR via transcaval access due to her stenotic and small sized iliac arteries. A pigtail was inserted in the left femoral artery and advanced to the abdominal aorta and another pigtail was inserted in the right femoral vein and advanced to the IVC to align with the aorta one. From the IVC, an astato wire was crossed into the aorta using wats to electrify the wire. A Sapien 3 valve was deployed. The caval aortic fistula was closed with a covered aortic stent. Final angiography showed complete occlusion of the fistula. Post intervention, patient experienced immediate improvement with resolution of cardiogenic shock. At three months, she remained asymptomatic and echocardiogram showed a well seated valve with no AI. DISCUSSION We present an urgent transcaval TAVR performed for a LVAD patient presenting with cardiogenic shock due to AI. Transcaval TAVR is a promising intervention that can be successfully used in LVAD patients developing AI with challenging iliofemoral arterial access.

Purpose Stereotactic body radiation therapy (SBRT) combined with electrocardiographic mapping has emerged as a potential noninvasive ablation technique for the treatment of refractory ventricular tachycardia (VT). The role of SBRT in patients with a left ventricular assist device (LVAD) is unknown. Methods Retrospective chart review was performed on all heart failure (HF) patients who underwent SBRT from January 1, 2018 to September 1, 2019. In this case series, we describe two patients who underwent SBRT after LVAD implantation. Results Of eleven heart failure patients who underwent SBRT, two had LVADs. The first was a 51 year old male with nonischemic cardiomyopathy and ventricular tachycardia refractory to medical therapy and stellate ganglion block. Given his intractable VT and cardiogenic shock, he underwent HeartMate 3 LVAD implantation as bridge-to-transplant (BTT). After LVAD implantation, he continued to have refractory VT despite subsequent VT ablations. CT cardiac mapping identified a 4 mm arrhythmic scar along the lateral wall of the right ventricle, and SBRT was performed with a single fraction of 25 Gy. Two weeks after SBRT, he experienced recurrent VT and eventually underwent orthotopic heart transplantation (OHT) four months after VT recurrence. The second patient was a 66 year old male with a history of nonischemic cardiomyopathy status post HeartWare LVAD as BTT who presented with recurrent VT three years following LVAD implantation. He was referred for SBRT after failure of antiarrhythmic drug (AAD) therapy and VT ablation. 4D-CT simulation defined a myocardial scar in the left ventricular outflow tract, and SBRT was performed with a single fraction of 25 Gy. His ventricular arrhythmias returned after four months, and additional AAD were utilized to suppress the arrhythmias until he underwent OHT two months later. The frequency of hospitalizations for VT did decrease following SBRT (Pt 1: 6 pre-SBRT vs. 3 post-SBRT, Pt 2: 4 pre-SBRT vs. 2 post-SBRT), but did not reach statistical significance (p= 0.12). Conclusion LVAD patients are prone to ventricular arrhythmias, and noninvasive SBRT may serve as an alternative therapy if AAD and traditional VT ablation have been unsuccessful, potentially keeping ventricular arrhythmias quiescent while awaiting heart transplantation. Further research is needed in this patient population.

Brain injury during the last trimester to the first 1-4 years in humans is now thought to trigger an array of intellectual and emotional problems later in life, including disorders such as schizophrenia. In adult schizophrenic brains, there is a specific loss of neurons that co-express glutamic acid decarboxylase-parvalbumin (GAD67-PV). Loss of this phenotype is thought to occur in mature animals previously exposed to N-methyl-D: -aspartate receptor (NMDAR) antagonists during late gestation or at postnatal day 7 (P7). However, in similarly treated animals, we have previously shown that GAD67 and PV are unaltered in the first 24 h. To more precisely define when changes in these markers first occur, we exposed rat pups (P7 or P6-P10) to the NMDAR antagonist MK801 and at P11 co-stained brain sections for GAD67 or PV. In the cingulate cortex, we found evidence for a reduction in PV (GAD67 levels were very low to undetectable). In contrast, in the somatosensory cortex, we found that expression of GAD67 was reduced, but PV remained stable. Further, repeated but not single doses of MK801 were necessary to see such changes. Thus, depending on the region, NMDAR antagonism appears to influence expression of PV or GAD67, but not both. These observations could not have been predicted by previous studies and raise important questions as to how the GAD67-PV phenotype is lost once animals reach maturity. More importantly, such differential effects may be of great clinical importance, given that cognitive deficits are seen in children exposed to anesthetics that act by blocking the NMDAR.

N-methyl-D-aspartate receptor blockade promotes apoptosis at postnatal day 7 (P7) and is linked to loss of glutamic acid decarboxylase 67 (GAD67) expression in older animals. To more fully appreciate this relationship we must first understand how GAD67 is regulated postnatally. Thus, the brains of P7, P14 and P21 rats were examined for expression of GAD67 protein and we found that levels of this GABAergic marker increased steadily with age, such that by P21 there was as much as a 6-fold increase compared to P7 animals and a 1.5- to 2-fold increase compared to P14 animals, depending on the region sampled. At P7, GAD67 was almost exclusively detected in puncta, with very few cell bodies displaying this marker. In contrast, at P14 and especially P21, both puncta and cell bodies were robustly labeled. Our data indicate that adult-like expression of GAD67 emerges quite late in the postnatal period.

Blockade of the N-methyl-D-aspartate receptor (NMDAR) in postnatal day 7 (P7) rats can promote rapid and robust induction of the pro-apoptotic marker activated caspase-3 (AC3) and loss of the GABAergic marker GAD67 at P56. Thus, we hypothesized that NMDAR blockade-induced AC3 occurs in GAD67 positive cells at P7. To test this idea, we injected P7 rat pups with vehicle or MK801 and after 8 hours (peak of AC3 induction) we examined brain sections for both AC3 and GAD67. Compared to vehicle, MK801 profoundly induced AC3 in all brain regions examined but co-expression of GAD67 in the same cells was not observed. However, in brain regions where punctate (synaptic) GAD67 was abundant (for example, layer IV of the somatosensory cortex), AC3 was robust. These data suggest that whereas somatic expression of AC3 and GAD67 may be non-overlapping, areas that exhibit punctate GAD67 (and are high in synaptic turnover) may be more vulnerable to MK801 exposure.

James Ballard, director at the Boston Medical Library, tracked questions he received at the reference desk in 1927 to recognize the trend of queries and to record the information for future use. He presented a paper on reference services that listed sixty of his reference questions at the Thirtieth Annual Meeting of the Medical Library Association (MLA) in 1927. During a two-month period in 2001, the authors examined Ballard's questions by attempting to answer them with print sources from the 1920s and with the Internet. The searchers answered 85% of the questions with the Internet and 80% with 1920s reference sources. The authors compared Internet and 1920s print resources for practical use. When answering the questions with 1920s resources, the searchers rediscovered a time in health sciences libraries when there was no Ulrich's Periodicals Directory, no standardized subject headings, and no comprehensive listings of available books. Yet, the authors found many of the 1920s reference materials to be quite useful and often multifunctional. The authors recorded observations regarding the impact of automation on answering reference questions. Even though the Internet has changed the outward appearance of reference services, many things remain the same.