Management of Anticoagulation and Antifibrinolytics in Catastrophic Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is an autoimmune disorder that presents with hypercoagulability and results in a lab artifact of prolonged PTT. The most severe form is catastrophic antiphospholipid antibody syndrome (CAPS), which manifests as rapidly progressing thromboses in multiple organ systems leading to multi-organ ischemia. The mainstay management CAPS is anticoagulation and systemic corticosteroids. Antifibrinolytic agents have previously been thought to be relatively contraindicated in CAPS due to the pro-thrombotic nature of the disease; the complex coagulation profile of CAPS can make it difficult to assess the risks and benefits of antifibrinolytic therapy. Also, should a patient with CAPS require cardiopulmonary bypass (CPB) for surgery, it poses a unique challenge in providing appropriate anticoagulation in the setting of prolonged ACT. We present a case of a 32-year-old postpartum female with CAPS requiring heart transplant who safely received intraoperative antifibrinolytic therapy and was successfully anticoagulated during CPB after perioperative plasmapheresis.

Effect of ascending aorta replacement on the long-term outcomes of bicuspid aortic valve repair.

OBJECTIVE: The study objective was to determine the effect of sinutubular junction stabilization on long-term outcomes of bicuspid aortic valve repair. METHODS: From January 1998 to January 2020, 419 patients underwent bicuspid aortic valve repair with ascending aorta replacement and 421 without (bicuspid aortic valve repair alone). Propensity score matching (97 pairs) was used to compare outcomes. RESULTS: Before matching, prevalence of severe aortic regurgitation at 10 years was 5.4% after bicuspid aortic valve repair + ascending aorta replacement and 10% after bicuspid aortic valve repair alone; aortic valve gradient was 20 mm Hg after bicuspid aortic valve repair + ascending aorta replacement and 19 mm Hg after bicuspid aortic valve repair alone. Ten-year freedom from reoperation overall was 79% after bicuspid aortic valve repair + ascending aorta replacement and 75% after bicuspid aortic valve repair alone; freedom from late aortic regurgitation was 93% after bicuspid aortic valve repair + ascending aorta replacement and 92% after bicuspid aortic valve repair alone; and freedom from aortic stenosis was 87% after bicuspid aortic valve repair + ascending aorta replacement and 93% after bicuspid aortic valve repair alone. Ten-year survival was 95% after bicuspid aortic valve repair + ascending aorta replacement and 96% after bicuspid aortic valve repair alone. After matching, prevalence of severe aortic regurgitation at 10 years was 11% after bicuspid aortic valve repair + ascending aorta replacement and 9.1% after bicuspid aortic valve repair alone (P = .33); aortic valve gradient was 16 mm Hg after bicuspid aortic valve repair + ascending aorta replacement and 25 mm Hg after bicuspid aortic valve repair alone (P < .0001). Ten-year freedom from reoperation was 85% after bicuspid aortic valve repair + ascending aorta replacement and 72% after bicuspid aortic valve repair alone (P = .08) overall. Ten-year freedom from reoperation for late aortic regurgitation was 88% after bicuspid aortic valve repair + ascending aorta replacement and 86% after bicuspid aortic valve repair alone (P = .65). Freedom from aortic stenosis was 97% after bicuspid aortic valve repair + ascending aorta replacement and 91% after bicuspid aortic valve repair alone (P = .03). Ten-year survival was 96% after bicuspid aortic valve repair + ascending aorta replacement and 96% after bicuspid aortic valve repair alone (P = .16). CONCLUSIONS: Bicuspid aortic valve repair with or without ascending aorta replacement is associated with good short- and long-term outcomes. Bicuspid aortic valve repair + ascending aorta replacement has a minimal effect on long-term repair durability. Sinutubular junction stabilization should not be performed for the sole purpose of long-term repair durability.

Adjunctive endovascular balloon fracture fenestration for chronic aortic dissection.

OBJECTIVE: Positive remodeling after thoracic endovascular aortic repair (TEVAR) for chronic thoracic aortic dissection is variable due to incomplete distal seal and retrograde false lumen perfusion. We assessed the outcomes of adjunctive balloon fracture fenestration (BFF) during TEVAR in patients with chronic aortic dissection complicated by negative remodeling. METHODS: From June 2013 to January 2016, 49 patients with chronic aortic dissection complicated by aneurysm due to negative remodeling underwent TEVAR with BFF. Contrast-enhanced computed tomography was performed before discharge, at 3 to 6 months, and annually. RESULTS: Intraoperatively, endovascular stent graft expansion was achieved in all patients. There was 1 hospital death due to visceral malperfusion related to acute-on-chronic dissection noted before planned BFF. There were no occurrences of paraplegia, 3 patients had stroke, and 3 had acute renal failure. Survival at 1 year was 91%. Late reintervention for incomplete false lumen exclusion was required in 16 patients and freedom from reintervention was 75% at 1 year. Thirty-six patients (73.5%) had complete false lumen thrombosis through the treated segment. True lumen area increased following TEVAR with BFF and continued to incrementally expand with subsequent aortic remodeling at 1-year follow-up. Thirteen patients had positive remodeling, defined as thrombosis of false lumen, ≥10% decrease in aortic dimension, and ≥10% increase in true lumen diameter. Patients with positive remodeling had an average decrease of 11 mm in maximal aortic diameter at final follow-up. CONCLUSIONS: BFF of chronic dissection membrane is a beneficial adjunct to TEVAR during short-term follow-up and may promote positive aortic remodeling and is worthy of further study.

Statin Therapy in Patients Undergoing Thoracic Aorta Replacement for Aortic Aneurysms.

BACKGROUND: Patients undergoing surgery for thoracic aortic aneurysms receive statin therapy out of proportion to cardiovascular comorbidity. We sought to determine the prevalence of statin use among patients presenting for thoracic aortic aneurysm surgery and investigate its effect on outcomes. METHODS: From January 1, 2005 to January 1, 2011, 1,839 consecutive patients underwent aortic replacement for degenerative thoracic aortic aneurysm at Cleveland Clinic. Of these, 771 (42%) were on statins preoperatively. Statin users (vs. nonstatin users) were older (65 ± 11 vs. 56 ± 16 years) and had more hypertension (78 vs. 59%). Propensity matching based on 56 preoperative variables other than lipid levels was used to compare outcomes among 570 matched patient pairs (74% of possible pairs). RESULTS: Propensity-matched statin and nonstatin users were aged 64 ± 11 years, 394 (69%) versus 387 (68%) were male, and 437 (77%) versus 442 (78%) had ascending aortic aneurysms, respectively. Overall, 25% of patients were followed for more than 8.2 years and 10% for more than 10 years. Perioperative outcomes were similar, including hospital mortality (11 [1.9%] vs. 5 [0.88%]) and stroke (22 [3.9%] vs. 13 [2.3%]), but 16 statin users (2.8%) versus 5 nonstatin users (0.88%) required temporary dialysis after surgery (p = 0.02). At 6 years, 3.7% of statin users versus 5.1% of nonstatin users (p[log-rank] = 0.5) underwent further aortic surgery, and at 10 years, mortality was 25% in both groups (p > 0.5). CONCLUSION: Patients presenting for thoracic aortic aneurysm surgery frequently receive unnecessary statins. Additionally, statin use was associated with more postoperative renal failure, but not less intermediate-term risk for aortic reintervention or all-cause mortality after surgery. Therefore, presence of a thoracic aortic aneurysm should not be considered an indication for statin therapy in the absence of well-established indications.

Impact of Endovascular False Lumen Embolization on Thoracic Aortic Remodeling in Chronic Dissection.

BACKGROUND: Retrograde false lumen (FL) perfusion after thoracic endovascular aortic repair (TEVAR) for chronic dissection is a mode of treatment failure. Thrombosis of the FL is associated with favorable reverse remodeling. Objectives are to describe FL embolization (FLE) strategy and assess aortic remodeling and survival. METHODS: From January 2009 to December 2017, 51 patients with chronic dissection underwent FLE, most after previous TEVAR. Devices included a combination of iliac plug (29 patients), coils (19 patients), or nitinol plug (3 patients). Computed tomography was performed before discharge, at 3 months, and annually (median follow-up 2 years [range, 1 month to 7 years]). RESULTS: After FLE, mean maximum aortic diameter decreased (64.2 ± 12 mm to 61.0 ± 13 mm; P = .03), true lumen diameter increased (24.7 ± 10 mm to 33.7 ± 8 mm; P < .001), and FL diameter decreased (36.7 ± 12 mm to 25.6 ± 15 mm, P < .001). For reverse remodeling, FL thrombosis with ≥10% decrease in diameter and ≥10% increase in true lumen diameter was achieved in 20 (39.2%; 16 primarily, 4 secondarily). Nine patients progressed after the first FLE: persistent FL flow with increase in aortic diameter and underwent repeat FLE with complete thrombosis (n = 4) or open thoracoabdominal completion (n = 5). A total of 26 patients had indeterminate response (FL thrombosis without change in maximum diameter), and none have required reoperation. Six patients had complete obliteration of the entire FL. At last follow-up, 42 (82%) patients were alive. Three deaths were related to aortic pathology. CONCLUSIONS: FLE is an important endovascular adjunct to TEVAR promoting reverse aortic remodeling in select patients with chronic aortic dissection and persistent retrograde FL perfusion.

Fibrous skeleton reconstruction for invasive aortic and mitral valve endocarditis.

Invasive endocarditis of the aortic and mitral valves with involvement of the intervalvular fibrosa is a particular surgical challenge. We describe a technique for aortic and mitral valve replacement with concomitant reconstruction of the intervalvular fibrosa, utilizing a folded bovine pericardial patch (Commando operation).

Mechanical circulatory support in pediatrics.

End-stage heart failure affects thousands of children yearly and mechanical circulatory support is used at many points in their care. Extracorporeal membrane oxygenation supports both the failing heart and lungs, which has led to its use as an adjunct to cardiopulmonary resuscitation as well as in post-operative cardiogenic shock. Continuous-flow ventricular assist devices (VAD) have replaced pulsatile-flow devices in adults and early studies have shown promising results in children. The Berlin paracorporeal pulsatile VAD recently gained U.S. Food and Drug Administration approval and remains the only VAD approved in pediatrics. Failing univentricular hearts and other congenitally corrected lesions are new areas for mechanical support. Finding novel uses, improving durability, and minimizing complications are areas of growth in pediatric mechanical circulatory support.

Prospective Validation of a Novel Vasoactive-Ventilation-Renal Score as a Predictor of Outcomes After Pediatric Cardiac Surgery.

BACKGROUND: We sought to further validate the novel vasoactive-ventilation-renal (VVR) score in a prospective study of a heterogeneous cohort of children undergoing cardiac surgery that includes patients with single-ventricle anatomy and residual mixing lesions. METHODS: We prospectively performed an observational study of all children less than 18 years of age who underwent surgery for congenital heart disease at our center from November 2013 to June 2014. We calculated VVR score as follows: vasoactive-inotrope score + ventilation index + (change in serum creatinine from baseline × 10). Admission, peak, and 48-hour measurements were recorded. Outcomes of interest were prolonged duration of mechanical ventilation and intensive care unit and hospital stays, represented by the upper 25% for all patients. Areas under the receiver-operating characteristic curves (AUC) were determined for all study timepoints and outcome variables. RESULTS: Ninety-two patients were analyzed; their median age was 0.65 (range, 3 days to 17.9 years), and 17 (18%) had single-ventricle anatomy. The VVR measurements outperformed vasoactive-inotrope scores in isolation at all timepoints, with higher AUC values for all outcomes. Of the three timepoints assessed, the 48-hour VVR score most consistently predicted poor outcome, especially with regard to prolonged duration of mechanical ventilation (AUC 0.980) and prolonged intensive care unit stay (AUC 0.919). CONCLUSIONS: In a heterogeneous population of children undergoing cardiac surgery, the 48-hour VVR score was a very strong predictor of outcomes, and outperformed the more traditional vasoactive-inotrope score. The VVR score, therefore, represents a novel and potentially powerful means of predicting clinical outcomes relatively early in the hospital course of these patients.

Use of a novel vasoactive-ventilation-renal score to predict outcomes after paediatric cardiac surgery.

OBJECTIVES: Prior studies have established peak postoperative lactate and the vasoactive-inotrope score (VIS) as modest predictors of outcome following paediatric cardiac surgery. We developed a novel vasoactive-ventilation-renal (VVR) score and aimed to determine if this index, which incorporates postoperative respiratory, cardiovascular and renal function, would more consistently predict outcome in this patient population. METHODS: We performed an Institutional Review Board-approved retrospective analysis of 222 infants at our institution less than 365 days old who underwent surgery for congenital heart disease at our centre from January 2009 to April 2013. The VVR score was calculated as follows: vasoactive-inotrope score + ventilation index + (change in serum creatinine from baseline × 10). For all patients, peak lactate and admission, peak, and 48 h VIS and VVR were recorded. RESULTS: For all outcome measures, areas under the curve for 48-h VVR were greater than its corresponding admission and peak values, VIS alone at all three time points and peak lactate. On multivariate regression, 48-h VVR was strongly associated with prolonged intubation [odds ratio (OR): 39.13, P <0.0001], significantly more so than 48-h VIS (odds ratio: 6.18, P <0.0001) and peak lactate (odds ratio: 2.52, P = 0.017). The 48-h VVR was also more significantly associated with prolonged use of vasoactive infusions, chest tube drainage and ICU and hospital stay when compared with VIS alone and peak lactate. CONCLUSIONS: The novel 48-h VVR was a robust predictor of outcome following paediatric cardiac surgery and outperformed the VIS and peak postoperative lactate.

Effect of corticosteroids on arginine vasopressin after pediatric cardiac surgery.

INTRODUCTION: Arginine vasopressin's (AVP) efficacy in the treatment of refractory hypotension is, in part, dependent upon preinfusion endogenous AVP concentration. Corticosteroids, also commonly used to treat refractory hypotension, have been shown to suppress endogenous AVP release. We aimed to determine if corticosteroids affect endogenous AVP concentrations in children recovering from cardiac surgery. MATERIALS AND METHODS: We reviewed the records of children who underwent cardiac surgery between January 2008 and January 2009 and had AVP concentrations available as part of a prior prospective study. Doses of hydrocortisone, methylprednisolone, and dexamethasone administered within the first 48 hours after cardiopulmonary bypass were quantitated. Multivariable linear regression was performed to determine if corticosteroids had a significant effect on 48-hour plasma AVP concentration. RESULTS: Sixty-nine children with plasma AVP concentrations available were reviewed, 34 (49%) of which received corticosteroids within 48 hours after cardiopulmonary bypass. On multivariable regression, greater number of corticosteroid doses but not cumulative corticosteroid dosage was significantly associated with low 48-hour AVP concentration (ß=-4.0; 95% confidence intervals, -6.5 to -1.4). CONCLUSIONS: Children who receive multiple doses of corticosteroids after cardiac surgery, regardless of potency, are likely to have low endogenous AVP concentrations. Children who remain unstable despite corticosteroids may respond favorably to exogenous AVP therapy.

Readmissions after diagnosis of surgical site infection following knee and hip arthroplasty.

CONTEXT: Surgical site infection (SSI) after total hip and knee arthroplasty is a common postoperative complication. We sought to determine readmission rates and costs for total hip and knee arthroplasty complicated by SSI. DESIGN: The Thomson Reuters MarketScan database was searched for patients who underwent knee or hip arthroplasty in 2007. From these data, patients who received a diagnosis of SSI and were readmitted after diagnosis were identified. SETTING: A population of 31 to 45 million individuals receiving insurance coverage. Patients who underwent knee or hip arthroplasty who experienced a hospitalization for SSI in the year after surgery were analyzed. OUTCOME MEASURES: Total readmission rates and costs per readmission at 30, 60, and 90 days and 1 year after diagnosis of SSI. RESULTS: Of the 76,289 case patients with hip or knee replacement in 2007, 1,026 (1.3%) had a hospitalization for SSI within the year after surgery. Among these patients, 310 (30.2%) were subsequently rehospitalized in the year after initial hospitalization specifically due to SSI-related issues. These rehospitalizations were associated with a mean hospital stay of [Formula: see text] days and a median cost of $20,001 (interquartile range [IQR], $14,057-$30,551). A total of 517 subjects had a subsequent "all-cause" hospitalization during the year after SSI. These rehospitalizations were associated with a mean hospital stay of [Formula: see text] days and a median cost of $19,870 (IQR, $13,913-$29,728). CONCLUSIONS: Readmissions during the year after SSI diagnosis accounted for 1,072 hospital admissions and cost over $25.5 million. These readmissions are costly and might be a future target for decreased reimbursement.