Faculty Bibliography

Background Arrhythmogenic cardiomyopathy (ACM) can mimic inflammatory processes. We present a complex patient with scleroderma (Sc)-dermatomyositis overlap syndrome (Sc-DM) and cardiac disease. Case A 57-year-old woman with family history of Sc presented with progressive weakness, dyspnea, edema, and Raynaud's (1A). Troponin was 1.6 ng/mL and CRP was 13.2 mg/L. EKGs revealed sinus rhythm with RBBB and AV sequential pacing with multifocal PVCs (1B-C). CT chest showed bibasilar fibrosis (1D). Echocardiography revealed biventricular dysfunction. Cardiac catheterization showed non-obstructive coronaries and a cardiac index of 1.8 L/min/m2. Cardiac MRI had diffuse biventricular subendocardial late gadolinium enhancement (1E). Electromyography revealed proximal myopathy. Rheumatologic workup was consistent with seronegative Sc-DM. Decision-making She was treated with steroids, mycophenolate, IV immunoglobulins, diuretics, and inotropes. Her course was complicated by recurrent VT cardiac arrests, prompting escalation to VA-ECMO. She underwent cardiac transplant on day 9 of ECMO. Pathology revealed biventricular fibrofatty replacement consistent with ACM (1F-G), patchy fibrosis of the pericardium, and mitral valve with thickened and fused chordae suggestive of inflammatory changes from Sc (1H-I). Conclusion This case highlights an atypical presentation of ACM in a patient with Sc-DM and the multidisciplinary approach necessary for proper diagnosis and management. [Figure presented]
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The approach to monitoring anticoagulation in adult patients receiving heparin on extracorporeal membrane oxygenation (ECMO) support is controversial. The objective of this study was to compare the correlation between anti-Xa and aPTT with heparin dose and to describe their association with clinical events in adult ECMO patients. We conducted a retrospective single-center study of 34 adult ECMO patients whose heparin was monitored by anti-Xa or aPTT. The heparin dose-to-assay correlation coefficient was 0.106 for aPTT and 0.414 for anti-Xa (p < 0.001). Major thrombotic and hemorrhagic events occurred in 14.7% and 26.5% of patients, respectively. The median anti-Xa in patients who experienced a major thrombotic event was 0.09 (0.06-0.25) IU/mL compared with 0.36 (0.26-0.44) IU/mL in patients who did not (p = 0.031), whereas the median aPTT did not differ between these groups. The maximum aPTT in patients who experienced a major bleed was 96.9 (76.0-200) seconds compared with 63.5 (44.4-98.6) seconds in patients who did not (p = 0.049), whereas the maximum anti-Xa did not differ between these groups. Monitoring both anti-Xa and aPTT may be warranted to safely provide understanding of pure heparin activity as well as underlying bleeding diatheses in adult ECMO patients.

BACKGROUND:Several studies have described improved survival with double lung transplantation (DLT) compared to single lung transplantation (SLT) in pulmonary fibrosis. To avoid the innate selection bias of including patients exclusively listed for SLT or DLT, this study analyzed those deemed appropriate for either procedure at time of listing. METHODS:All consecutive adult lung transplants for idiopathic pulmonary fibrosis (IPF) provided by the Scientific Registry of Transplant Recipients were retrospectively reviewed (2007-2017). Isolated lobar transplants (N=11), or patients listed only for SLT (N=1834) or DLT (N=2372) were excluded. Group stratification was based on the ultimate procedure (SLT vs DLT). Group propensity matching was performed based on 24 recipient/donor characteristics. Recipient demographics, donor demographics, and outcomes were compared between groups. RESULTS:During the study period, 45% (974/2179) and 55% (1205/2179) of patients ultimately received SLT and DLT, respectively. After propensity matching, 466 matched patients remained in each group. SLT patients were less likely to require prolonged (>48 hours) ventilator support than DLT patients. There was also a trend towards reduced rates of post-transplant renal failure and hospital length of stay in SLT recipients. Whether analyzed by time of listing or time of transplant, survival was similar between groups. CONCLUSIONS:In recipients concurrently listed for SLT and DLT, overall survival was similar regardless of the eventual procedure. These data suggests that the previously purported survival advantage for DLT may purely represent selection bias, and should not preclude the use of SLT in appropriately-selected IPF patients.

Over the past several decades, the operation of choice for end-stage lung disease secondary to severe pulmonary hypertension (PH) has shifted from heart-lung transplantation (HLT) to bilateral lung transplantation (BLT). This change has maintained excellent long-term outcomes and is appropriate for the majority of patients presenting with end-stage disease in need of transplantation. However, a distinct subset of patients with severe PH have an excessive early mortality within 90 days of transplantation. Based on the different causes of this early mortality compared to BLT recipients with other indications, right heart failure and refractory primary graft dysfunction (PGD) appear to play a significant role. It is therefore critical to identify this subset of patient during their evaluation for transplant. This distinction would allow specific patient referral for HLT, which may mitigate those causes of early mortality. Similarly, there is a subgroup of BLT recipients for severe PH that fail to recover right ventricular function, with suboptimal long-term functional status that is independent of early survival. Identification and referral for HLT of these patients may also be important. In this manuscript, we describe our institutional approach and consideration for the risks of early mortality from right heart failure and PGD, as well failure of right ventricular recovery long-term. The described evaluation is used to ascertain those patients with severe PH who may benefit from a HLT over BLT.

A morbidly obese middle aged woman in her 40's presented to another hospital with methicillin resistant staphylococcus aureus pneumonia and subsequently developed severe acute respiratory distress syndrome. Her oxygenation demonstrated no improvement with low tidal volume ventilation, paralysis, or prostagladin therapy. She was unable to be manually proned secondary to her habitus. She was subsequently transferred to our facility, where she was initiated on VV-ECMO. Maximal flow through the ECMO circuit was inadequate for oxygenation given significant systemic shunt through her native lungs. In order to optimize lung protective ventilation and treat ARDS, we used an automated kinetic system (Rotoprone Therapy System) to prone the patient. To our knowledge, this is the first description in the literature of using an automated proning system with an ECMO circuit in place. This report describes the technique we used to safely perform axial rotations for two days with fewer providers required than manual proning.

OBJECTIVES/OBJECTIVE:Patients with low left ventricular ejection fraction (LVEF) undergoing high-risk coronary artery bypass grafting (CABG) are at increased risk for postcardiotomy cardiogenic shock. This report describes planned concomitant microaxial temporary mechanical support (MA-TMS) device placement as a viable bridge-to-recovery strategy for high-risk patients receiving surgical revascularization. METHODS:A retrospective review was performed for all patients from October 2017 to May 2019 with low LVEF (<30%), New York Heart Association Class III or IV symptoms, and myocardial viability who underwent CABG with prophylactic MA-TMS support at a single institution (n = 13). RESULTS:Mean patient age was 64.8 years, and 12 patients (92%) were male. Eight patients (62%) presented with acute coronary syndrome. Mean predicted risk of mortality was 4.6%, ranging from 0.6% to 15.6%. An average of 3.4 grafts were performed per patient. Greater than 60% of patients were extubated within 48 hours and out-of-bed within 72 hours, and the average duration of MA-TMS was 5.7 days. Mean postoperative length of stay was 16.7 days. There were no postoperative myocardial infarctions or deaths. CONCLUSIONS:Prophylactic MA-TMS may allow safe and effective surgical revascularization for patients with severe left ventricular dysfunction who may otherwise be offered a durable ventricular assist device.

The left ventricular outflow tract (LVOT) velocity time integral (VTI) is an easily measured echocardiographic stroke volume index analog. Low values predict adverse outcomes in left ventricular failure. We postulate the left ventricular VTI may be a signal of right ventricular dysfunction in acute pulmonary embolism, and therefore a predictor of poor outcomes. We retrospectively reviewed echocardiograms on all Pulmonary Embolism Response Team activations at our institution at the time of pulmonary embolism diagnosis. Low LVOT VTI was defined as ⩽ 15 cm. We examined two composite outcomes: (1) in-hospital death or cardiac arrest; and (2) shock or need for primary reperfusion therapies. Sixty-one of 188 patients (32%) had a LVOT VTI of ⩽ 15 cm. Low VTI was associated with in-hospital death or cardiac arrest (odds ratio (OR) 6, 95% CI 2, 17.9; p = 0.0014) and shock or need for reperfusion (OR 23.3, 95% CI 6.6, 82.1; p < 0.0001). In a multivariable model, LVOT VTI ⩽ 15 remained significant for death or cardiac arrest (OR 3.48, 95% CI 1.02, 11.9; p = 0.047) and for shock or need for reperfusion (OR 8.12, 95% CI 1.62, 40.66; p = 0.011). Among intermediate-high-risk patients, low VTI was the only variable associated with the composite outcome of death, cardiac arrest, shock, or need for reperfusion (OR 14, 95% CI 1.7, 118.4; p = 0.015). LVOT VTI is associated with adverse short-term outcomes in acute pulmonary embolism. The VTI may help risk stratify patients with intermediate-high-risk pulmonary embolism.

BACKGROUND:The national opioid epidemic has expanded the donor pool for heart transplantation, but concerns remain regarding infectious risk and allograft function. This study compared donor and recipient characteristics, outcomes, and reasons for organ discard between overdose-death donors (ODDs) and donors with all other mechanism of death. METHODS:Data on adult cardiac transplants from 2010 to 2017 were provided by the Scientific Registry of Transplant Recipients. Cardiac allografts used in multiple organ transplantations were excluded. Recipient and donor characteristics and organ discard were analyzed with regard to ODDs. Kaplan-Meier curves and log-rank tests described mortality survival. RESULTS:A total of 1,710 of 15,904 (10.8%) cardiac transplantations were from ODDs, approximately a 10-fold increase from 2000 (1.2%). ODDs were more frequently older than 40 years of age (87.2% vs 70.1%; p < 0.001), had higher rates of substance abuse, were more likely hepatitis C positive (1.3% vs 0.2%; p < 0.001), and less frequently required inotropic support at the time of procurement (38.4% vs 44.8%; p < 0.001). Overall survival was not different between the groups (p = 0.066). Discarded ODD allografts were more likely to be hepatitis C positive (30.8% vs 5.3%; p < 0.001) and to be identified as conveying increased risk by the Public Health Services (63.3% vs 13.2%; p < 0.001), but they were less likely to be discarded because of a diseased organ state (28.2% vs 36.1%; p < 0.001). CONCLUSIONS:Rates of ODDs have increased corresponding with the worsening opioid epidemic. Even though ODDs have higher rates of hepatitis C, cardiac allograft quality indices are favorable, and recipient outcomes are similar when compared with non-ODDs, a finding indicating that greater use of this donor pool may be appropriate.