Risk Prediction in Male Adolescents With Congenital Long QT Syndrome: Implications for Sex-Specific Risk Stratification in Potassium Channel-Mediated Long QT Syndrome.

BACKGROUND: Sex-specific risk management may improve outcomes in congenital long QT syndrome (LQTS). We recently developed a prediction score for cardiac events (CEs) and life-threatening events (LTEs) in postadolescent women with LQTS. In the present study, we aimed to develop personalized risk estimates for the burden of CEs and LTEs in male adolescents with potassium channel-mediated LQTS. METHODS AND RESULTS: The prognostic model was derived from the LQTS Registry headquartered in Rochester, NY, comprising 611 LQT1 or LQT2 male adolescents from age 10 through 20 years, using the following variables: genotype/mutation location, QTc-specific thresholds, history of syncope, and ß-blocker therapy. Anderson-Gill modeling was performed for the end point of CE burden (total number of syncope, aborted cardiac arrest, and appropriate defibrillator shocks). The applicability of the CE prediction model was tested for the end point of the first LTE (excluding syncope and adding sudden cardiac death) using Cox modeling. A total of 270 CEs occurred during follow-up. The genotype-phenotype risk prediction model identified low-, intermediate-, and high-risk groups, comprising 74%, 14%, and 12% of the study population, respectively. Compared with the low-risk group, high-risk male subjects experienced a pronounced 5.2-fold increased risk of recurrent CEs (P<0.001), whereas intermediate-risk patients had a 2.1-fold (P=0.004) increased risk . At age 20 years, the low-, intermediate-, and high-risk adolescent male patients had on average 0.3, 0.6, and 1.4 CEs per person, respectively. Corresponding 10-year adjusted probabilities for a first LTE were 2%, 6%, and 8%. CONCLUSIONS: Personalized genotype-phenotype risk estimates can be used to guide sex-specific management in male adolescents with potassium channel-mediated LQTS.

Cannula-Associated Deep Vein Thrombosis After Venovenous Extracorporeal Membrane Oxygenation in Patients with and Without Systemic Anticoagulation.

OBJECTIVE: To identify and compare the rates of cannula-associated deep vein thrombosis (CaDVT) in patients on venovenous extracorporeal membrane oxygenation (VV-ECMO) who receive systemic anticoagulation (AC) and those who do not receive AC. DESIGN: Retrospective observational study. SETTING: Tertiary academic medical center. PARTICIPANTS: Consecutive patients who successfully have been decannulated from VV-ECMO for treatment of refractory acute respiratory distress syndrome between 2017 and 2022. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: After decannulation of ECMO, a duplex sonograph was performed on the cannulation sites to determine the incidence and characteristics of cannula-related thrombosis. Thrombosis was classified as occlusive or nearly occlusive. Ninety-four of 161 patients were weaned from VV-ECMO. Nineteen patients who were placed on VV-ECMO due to COVID-19 were excluded. Twenty-seven of 52 patients (52%) who did not receive AC were identified to have thrombus. Twelve of 23 patients (52%) who received AC were identified to have thrombus. Patients who received AC required more blood products during the ECMO run and required longer support on VV-ECMO. CONCLUSION: This study showed a high incidence of cannula-related venous thrombosis after VV-ECMO decannulation. Surprisingly, the incidence of CaDVT in anticoagulation-free patients was the same as in patients requiring anticoagulation. Anticoagulated patients required longer support on VV-ECMO and required more transfusions. Routine post-decannulation screening for DVT is recommended due to the high incidence of CaDVT.

Less Invasive Left Ventricular Assist Device Implantation Is Safe and Feasible in Patients With Smaller Body Surface Area.

OBJECTIVE: Smaller body surface area (BSA) frequently precludes patients from left ventricular assist device (LVAD) therapy. We sought to investigate the clinical outcomes in patients with small BSA undergoing less invasive LVAD implantation. METHODS: We conducted a retrospective review of 216 patients implanted with HeartMate 3 LVAD (Abbott, Chicago, IL) via less invasive surgery at our institution. Patients were dichotomized based on their preimplant BSA for comparison between small BSA (≤1.8 m2) and normal/large BSA (>1.8 m2). We analyzed patient perioperative characteristics and outcomes. RESULTS: In our study, small BSA was found in 32 patients (14.8%), while 184 patients (85.2%) had normal/large BSA. Women were more prevalent in the small BSA group (50.0% vs 13.0%, P < 0.001). Preoperative and intraoperative data showed comparable results. Major complications and hospital length of stay did not differ by BSA group. Patients with smaller BSA had significantly decreased pump parameters at discharge, including LVAD flow (4.11 ± 0.49 vs 4.60 ± 0.54 L/min, P < 0.001) and pump speed (5,200 vs 5,400 rpm, P < 0.001). Survival to discharge and within 6 months after implantation were similar between the groups. CONCLUSIONS: Our study results suggest that less invasive HeartMate 3 implantation can be safely performed and demonstrates equivalent outcomes in patients with smaller body habitus. Randomized trials are required to confirm our data.

Left atrial appendage exclusion with less invasive left ventricular assist device implantation.

OBJECTIVES: The objective of this single-center, pilot, prospective, and historical control study is to evaluate safety and feasibility outcomes associated with left atrial appendage exclusion (LAAE) concomitant with left ventricular assist device (LVAD) implantation via less invasive surgery (LIS) as a stroke prevention strategy. METHODS: A predefined number of 30 eligible subjects scheduled for LIS LVAD with LAAE were enrolled in the prospective arm between January 2020 and February 2021. Eligible retrospective LIS LVAD patients without LAAE were propensity-matched in a 1:1 ratio with the prospective arm subjects. The primary study objectives were to evaluate the safety, feasibility, and efficacy of the LAAE concomitant with LIS LVAD. RESULTS: Preoperative characteristics of patients in the Non-LAAE and LAAE groups were similar. LAAE was successfully excluded in all prospective patients (100%). Primary safety endpoints of chest tube output within the first 24 postoperative hours, Reoperation for bleeding within 48 h, and index hospitalization mortality demonstrated comparable safety of LAAE versus Non-LAAE with LIS LVAD. Cox proportional hazard regression demonstrated that LAAE with LIS LVAD was associated with 37% and 49% reduction in the risk of stroke and disabling stroke, respectively (p > .05). CONCLUSION: Results from our pilot study demonstrated the safety and feasibility of LAAE concomitant with LIS LVAD as a stroke prevention strategy. This is the first prospective study describing LAAE performed concomitantly to less invasive LVAD implantation. The efficacy of LAAE in long-term stroke prevention needs to be confirmed in future prospective randomized clinical trials.

Impact of surgical approach for left ventricular assist device implantation on postoperative invasive hemodynamics and right ventricular failure.

BACKGROUND: Right ventricular failure (RVF) remains one of the major causes of morbidity and mortality after left ventricular assist device (LVAD) implantation. We sought to compare immediate postoperative invasive hemodynamics and the risk of RVF following two different surgical approaches: less invasive surgery (LIS) versus full sternotomy (FS). METHODS: The study population comprised all 231 patients who underwent implantation of a HeartMate 3 (Abbott) LVAD at our institution from 2015 to 2020, utilizing an LIS (n = 161; 70%) versus FS (n = 70; 30%) surgical approach. Outcomes included postoperative invasive hemodynamic parameters, vasoactive-inotropic score (VIS), RVF during index hospitalization, and 6-month mortality. RESULTS: Baseline clinical characteristics of the two groups were similar. Multivariate analysis showed that LIS, compared with FS, was associated with the improved cardiac index (CI) at the sixth postoperative hour (p = .036) and similar CI at 24 h, maintained by lower VIS at both timepoints (p = .002). The LIS versus FS approach was also associated with a three-fold lower incidence of in-hospital severe RVF (8.7% vs. 28.6%, p < .001) and need for RVAD support (5.0% vs. 17.1%, p = .003), and with 68% reduction in the risk of 6-month mortality after LVAD implantation (Hazard ratio, 0.32; CI, 0.13-0.78; p = .012). CONCLUSION: Our findings suggest that LIS, compared with FS, is associated with a more favorable hemodynamic profile, as indicated by similar hemodynamic parameters maintained by lower vasoactive-inotropic support during the acute postoperative period. These findings were followed by a reduction in the risk of severe RVF and 6-month mortality in the LIS group.

Sex hormones and repolarization dynamics during the menstrual cycle in women with congenital long QT syndrome.

BACKGROUND: Women with congenital long QT syndrome (LQTS) experience increased cardiac event risk after the onset of adolescence, perhaps stemming from the known modulating effects of sex hormones on the cardiac potassium channels. OBJECTIVE: We hypothesized that the effect of sex hormones on cardiac ion channel function may modify electrocardiographic (ECG) parameters associated with the propensity for ventricular tachyarrhythmias during the menstrual cycle in women with LQTS. METHODS: We prospectively enrolled 65 women with congenital LQTS (type 1 LQTS [LQT1], n = 24 [36.9%]; type 2 LQTS [LQT2], n = 20 [30.8%]) and unaffected female relatives (n = 21 [32.3%]). Patients underwent three 7-day ECG recordings during their menstrual cycles. Simultaneous saliva testing of sex hormone levels was conducted on the first day of each 7-day ECG recording cycle. RESULTS: The mean age was 35 ± 8 years, without a significant difference among the groups. In women with LQT2, linear mixed effects models showed significant inverse correlations of the corrected QT interval with progesterone levels (P < .001) and with the progesterone to estradiol ratio (P < .001). Inverse relationships of the R-R interval with estradiol levels (P = .003) and of the T-wave duration with testosterone levels (P = .014) were also observed in women with LQT2. In contrast, no significant associations were observed between ECG parameters and sex hormone levels in women with LQT1 or unaffected relatives. CONCLUSION: This is the first study to prospectively assess correlations between repolarization dynamics and sex hormone levels during the menstrual cycle in women with congenital LQTS. Our findings show genotype-specific unique corrected QT dynamics during the menstrual cycle that may affect the propensity for ventricular tachyarrhythmia in women with LQTS, particularly women with LQT2.

Cause of In-Hospital Death After Weaning from Venoarterial-Extracorporeal Membrane Oxygenation.

PURPOSE: A survival gap between weaning from venoarterial-extracorporeal membrane oxygenation (VA-ECMO) and the hospital discharge has been consistently reported. The aim of this study is to investigate the clinical features of patients who underwent successful VA-ECMO decannulation at our institution and to identify the major contributors responsible for adverse outcomes. METHODS: We retrospectively reviewed all patients supported with VA-ECMO in our institution between January 2013 and June 2020. Only patients that survived VA-ECMO and underwent successful decannulation were included and dichotomized based on survival to hospital discharge: non-survivors versus survivors. The primary study outcome was the cause of death after successful VA-ECMO decannulation. RESULTS: Of the 262 adult patients who underwent VA-ECMO decannulation, 72 (27.5%) patients did not survive to hospital discharge. Non-survivors were older (62 vs. 54 years, p < 0.001) and suffering from many pre-existing comorbidities. Pneumonia and sepsis were the most frequent infectious complication and almost twice as likely in non-survivors. Major causes of death were: cardiovascular (31.9%), infections (25.0%) and neurological (20.8%). The survival curve demonstrated that 51.4% of our patients died within 8 days after decannulation. Multivariate analysis identified older age, central venous cannulation, pulmonary bleeding and infection, dialysis after VA-ECMO, sepsis, and ischemic stroke (OR = 7.86, 95% CI: 2.76-2.43, p < 0.001) as factors significantly predisposing to patients' death. CONCLUSION: In our study, one-third of patients decannulated off VA-ECMO did not survive to hospital discharge due to end-stage heart failure, infections or neurological injury. The first 8 post-decannulation days were recognized as a critical period where thorough strategies to prevent acquired infections and cautious support of end-organ function should be warranted. Future large-scale trials are needed to confirm our results.

Study results suggest less invasive HeartMate 3 implantation is a safe and effective approach for obese patients.

BACKGROUND: Historically, obesity was considered a relative contraindication to left ventricular assist device (LVAD) implantation with less invasive surgery (LIS). The present study aimed to compare the outcomes of obese patients who underwent LVAD implantation through LIS with those who received full sternotomy (FS) implantation. METHODS: We retrospectively reviewed all patients implanted with HeartMate 3 LVAD in our institution between September 2015 and June 2020. Obese patients (BMI ≥ 30 kg/m2) were included and dichotomized based on surgical approach into the FS or LIS cohort. RESULTS: Of 231 implanted patients, 107 (46%) were obese and included in the study. FS was performed in 26 (24%) patients and LIS approach in 81 (76%) patients. Preoperative patient characteristics were similar between the cohorts. Postoperatively, patients in LIS cohort had less bleeding (p = 0.029), fewer transfusions (p = 0.042), shorter duration of inotropic support (p = 0.049), and decreased incidence of severe RV failure (11.1% vs 30.8%, p = 0.028). Survival to discharge for the obese population was 87.5% overall and did not differ based on an approach (91.4% LIS vs 76.9% FS, p = 0.079). More LIS patients were discharged home (60.0% vs 82.4%, p = 0.041) rather than to rehabilitation center. CONCLUSION: Our results showed that the LIS approach in obese patients is associated with fewer postoperative complications and a trend towards better short-term survival. These results suggest that less invasive LVAD implantation is a safe and effective approach for obese patients. Future prospective randomized trials are required to substantiate these results.

Enhanced Recovery After Surgery in Patients Implanted with Left Ventricular Assist Device.

INTRODUCTION: We sought to develop and implement a comprehensive enhanced recovery after surgery (ERAS) protocol for patients implanted with a left ventricular assist device (LVAD). METHODS AND RESULTS: In this article, we describe our approach to the development and phased implementation of the protocol. Additionally, we reviewed prospectively collected data for patients who underwent LVAD implantation at our institution from February 2019 to August 2020. To compare early outcomes in our patients before and after protocol implementation, we dichotomized patients into two 6-month cohorts (the pre-ERAS and ERAS cohorts) separated from each other by 6 months to allow for staff adoption of the protocol. Of the 115 LVAD implants, 38 patients were implanted in the pre-ERAS period and 46 patients in the ERAS period. Preoperatively, the patients` characteristics were similar between the cohorts. Postoperatively, we observed a decrease in bleeding (chest tube output of 1006 vs 647.5 mL, P < .001) and blood transfusions (fresh frozen plasma 31.6% vs 6.7%, P = .04; platelets 42.1% vs 8.7%, P = .001). Opioid prescription at discharge were 5-fold lower with the ERAS approach (P < .01). Furthermore, the number of patients discharged to a rehabilitation facility decreased significantly (20.0% vs 2.4%, P = .02). The index hospitalization length of stay and survival were similar between the groups. CONCLUSIONS: ERAS for patients undergoing LVAD implantation is a novel, evidence-based, interdisciplinary approach to care with multiple potential benefits. In this article, we describe the details of the protocol and early positive changes in clinical outcomes. Further studies are needed to evaluate benefits of an ERAS protocol in an LVAD population.Lay Summary: Enhanced recovery after surgery (ERAS) is the implementation of standardized clinical pathways that ensures the use of best practices and decreased variation in perioperative care. Multidisciplinary teams work together on ERAS, thereby enhancing communication among health care silos. ERAS has been used for more than 30 years by other surgical services and has been shown to lead to a decreased length of stay, fewer complications, lower mortality, fewer readmissions, greater job satisfaction, and lower costs. Our goal was to translate these benefits to the perioperative care of complex patients with a left ventricular assist device. Early results suggest that this goal is possible; we have observed a decrease in transfusions, discharge on opioids, and discharge to a rehabilitation facility.

Complete sternal-sparing left ventricular assist device implantation is associated with improved postoperative mobility.

Left ventricular assist device (LVAD) implantation via a complete sternal-sparing (CSS) technique is gaining interest due to several potential benefits. We hypothesized that the CSS approach for HeartMate 3 (HM3) LVAD implantation improves postoperative mobility and physical independence compared to full sternotomy (FS). We retrospectively reviewed patients who were implanted with a commercial HM3 at our institution from September 2017 to August 2018. The Activity Measure for Post-Acute Care short forms and Functional Independence Measure scores were used to assess the patient's physical limitations postoperatively. A total of 43 patients were included in the study: 27 (63%) CSS patients and 16 (37%) FS patients. At postoperative day 3, the CSS cohort demonstrated improved mobility based on Activity Measure for Post-Acute Care scores compared to the FS group; 40% of the CSS cohort versus 67% of the FS cohort remained 100% impaired. The CSS cohort also demonstrated greater postoperative independence in the Functional Independence Measure sit-to-stand metric with 78% of the CSS cohort achieving modified or complete independence by postoperative day 15 compared to only 21% of the FS patients. These early data suggest that the CSS approach for HM3 LVAD implantation improves postoperative mobility and functional independence compared to FS.

Long-term renal function after venoarterial extracorporeal membrane oxygenation.

BACKGROUND: The utilization of venoarterial extracorporeal membrane oxygenation (VA-ECMO) as a life-supporting therapy has increased exponentially over the last decade. As more patients receive and survive ECMO, there are a number of unanswered clinical questions about their long-term prognosis and organ function including the need for long-term dialysis. METHODS: We aimed to utilize over 208 patient-years of follow-up data from our large institutional cohort of VA-ECMO patients to determine the incidence of requiring VA-ECMO support on the need for renal replacement therapy after discharge (LT-dialysis). This retrospective review included all adult VA-ECMO patients at our institution from January 2014 to October 2018 (N = 283). RESULTS: Out of the 99 (35%) survivors, 88 (89%) did not require LT-dialysis of any duration after discharge from the index hospitalization. Patients who required VA-ECMO for decompensated cardiogenic shock were more likely to need LT-dialysis (p = .034), and those who required renal replacement therapy during VA-ECMO (N = 27) also had a higher incidence of LT-dialysis (33%). CONCLUSION: Overall, these data suggest there is a low incidence of long-term dialysis dependence among survivors of VA-ECMO support. Worries about the potential long-term detrimental effect of VA-ECMO should not preclude patients from receiving this life-saving support.

Transcatheter aortic valve replacement in left ventricular assist device patient-overcoming the complications with transapical approach and circulatory arrest.

We present the patient with severe aortic insufficiency (AI) 5 years post left ventricular assist device (LVAD) implantation. His management was complicated with unsuccessful percutaneous aortic valve closure attempt, transcatheter aortic valve replacement (TAVR) implantation with a severe paravalvular leak, eventual valve dislodgment into the left ventricle (LV), and LVAD inflow cannula occlusion. We utilized a mini-thoracotomy approach to successfully retrieve the dislodged valve through the LV apex while deploying a valve-in-valve TAVR under direct visualization and deep hypothermic cardiac arrest. This case can serve as an example of the serious pitfalls and potential resolution strategies when treating LVAD-associated AI.

Minimally invasive off-pump surgical pulmonary embolectomy for improved patient-centred care.

We present a complicated case of massive pulmonary embolism occurring 11 weeks after a craniotomy in a patient with multiple high-risk comorbidities. The patient underwent successful pulmonary artery surgical embolectomy via left mini-thoracotomy incision on peripheral venoarterial extracorporeal membrane oxygenation support. For this patient, avoiding a sternotomy allowed for greatly decreased postoperative morbidity and the use of venoarterial extracorporeal membrane oxygenation allowed for the avoidance of intraoperative anticoagulation. This case demonstrates the feasibility of off-pump surgical pulmonary embolectomy via left mini-thoracotomy as a treatment strategy for appropriate patients to improve patient-centred care.

Surgical Pulmonary Embolectomy With No Systemic Anticoagulation for Patient With Recent Stroke.

We present the successful use of surgical embolectomy (SE) without systemic anticoagulation to treat a complicated case of pulmonary embolism. The patient presented with an embolic cerebrovascular accident and subsequently developed a massive pulmonary embolism. Because of the risk of hemorrhagic transformation, the decision was made to proceed with emergent SE on venoarterial extracorporeal membrane oxygenation support without anticoagulation. The surgery was performed without complication. The potential to perform SE without anticoagulation could potentially decrease the incidence of surgical bleeding and make SE a therapeutic option for patients with contraindications to anticoagulation. Further research is needed to substantiate the efficacy of this treatment strategy.