A Rare Case of a Right Atrial Paraganglioma in an Individual with the SHDB Mutation.

Paragangliomas are extra-adrenal chromaffin cell tumors. A small percentage of these tumors can be found in the thoracic cavity and, when in the heart, are typically in the left atrium. In this case report, we discuss the case of an individual with a history of several paragangliomas with the SHDB mutation who was found to have two cardiac paragangliomas in the right atrium.

Extended post-ex vivo lung perfusion cold preservation predicts primary graft dysfunction and mortality: Results from a multicentric study.

BACKGROUND: Ex vivo lung perfusion (EVLP) allows for a reassessment of lung grafts initially deemed unsuitable for transplantation, increasing the available donor pool; however, this requires a pre- and post-EVLP period of cold ischemic time (CIT). Paucity of data exists on how the sequence of cold normothermic-cold preservations affect outcomes. METHODS: A total of 110 patients were retrospectively analyzed. Duration of 3 preservation phases was measured: cold pre-EVLP, EVLP, and cold post-EVLP. The donor and recipient clinical data were collected. Primary graft dysfunction (PGD) and survival were monitored. Risk of mortality or PGD was calculated using Cox proportional hazards and logistic regression models to adjust for baseline characteristics. RESULTS: Using the highest quartile, patients were stratified into extended vs non-extended pre-EVLP (<264 vs ≥264 minutes) and post-EVLP (<287 vs ≥287 minutes) CIT. The rates of 1-year mortality (8.4% vs 29.6%, p = 0.013), PGD 2-3 (20.5% vs 52%, p = 0.002), and PGD 3 (8.4% vs 29.6%, p = 0.005) at 72 hours were increased in the extended post-EVLP CIT group. After adjusting for baseline risk factors, the extended group remained an independent predictor of PGD ≥2 (odd ratio: 6.18, 95% CI: 1.88-20.3, p = 0.003) and PGD 3 (odd ratio: 20.4, 95% CI: 2.56-161.9, p = 0.004) at 72 hours and 1-year mortality (hazard ratio: 17.9, 95% CI: 3.36-95.3, p = 0.001). Cold pre-EVLP was not a significant predictor of primary outcomes. CONCLUSIONS: Extended cold post-EVLP preservation is associated with a risk for PGD and 1-year mortality. Pre-EVLP CIT does not increase mortality or high-grade PGD. These findings from a multicenter trial should caution on the implementation of extended cold preservation after EVLP.

Incidence of Atrioventricular Block After Valve Replacement in Carcinoid Heart Disease.

BACKGROUND: Carcinoid heart disease (CaHD) is a rare condition that has a high impact on the morbidity and mortality of its patients. Once heart failure symptoms develop in the patient with CaHD, cardiac valve surgery is often the only effective treatment. Although atrioventricular block (AVB) is a known postoperative complication of the valve surgery, the incidence of AVB in this population has not been well described. METHODS: Comprehensive records were collected retrospectively on consecutive patients with CaHD who underwent a valve surgery at a tertiary medical center from January 2001 to December 2015. We excluded patients with pre-existing permanent pacemaker (PPM). RESULTS: Nineteen consecutive patients were included in this study and 18 of them underwent at least dual valve (tricuspid and pulmonary valve) replacement surgery. Our 30-day post-surgical mortality was 0%. During the 6-month observation period following the surgery, 31.5% (n = 6) required PPM implantation due to complete AVB. There was no statistical difference in baseline characteristics and electrocardiographic and echocardiographic parameters between the patients who did or did not require PPM placement. CONCLUSIONS: Our study revealed that almost one-third of CaHD patients who underwent a valve replacement surgery developed AVB requiring PPM implantation. Due to high incidence of PPM requirement, we believe that prophylactic placement of an epicardial lead during the valve surgery can be helpful in these patients to reduce serious complication from placement of pacemaker lead on a later date through a prosthetic valve.

Positive Impact of an Aortic Center Designation.

BACKGROUND: In January 2015, we created a multidisciplinary Aortic Center with the collaboration of Vascular Surgery, Cardiac Surgery, Interventional Radiology, Anesthesia and Hospital Administration. We report the initial success of creating a Comprehensive Aortic Center. METHODS: All aortic procedures performed from January 1, 2015 until December 31, 2016 were entered into a prospectively collected database and compared with available data for 2014. Primary outcomes included the number of all aortic related procedures, transfer acceptance rate, transfer time, and proportion of elective/emergent referrals. RESULTS: The Aortic Center included 5 vascular surgeons, 2 cardiac surgeons, and 2 interventional radiologists. Workflow processes were implemented to streamline patient transfers as well as physician and operating room notification. Total aortic volume increased significantly from 162 to 261 patients. This reflected an overall 59% (P = 0.0167) increase in all aorta-related procedures. We had a 65% overall increase in transfer requests with 156% increase in acceptance of referrals and 136% drop in transfer denials (P < 0.0001). Emergent abdominal aortic cases accounted for 17% (n = 45) of our total aortic volume in 2015. The average transfer time from request to arrival decreased from 515 to 352 min, although this change was not statistically significant. We did see a significant increase in the use of air-transfers for aortic patients (P = 0.0041). Factorial analysis showed that time for transfer was affected only by air-transfer use, regardless of the year the patient was transferred. Transfer volume and volume of aortic related procedures remained stable in 2016. CONCLUSIONS: Designation as a comprehensive Aortic Center with implementation of strategic workflow systems and a culture of "no refusal of transfers" resulted in a significant increase in aortic volume for both emergent and elective aortic cases. Case volumes increased for all specialties involved in the center. Improvements in transfer center and emergency medical services communication demonstrated a trend toward more efficient transfer times. These increases and improvements were sustainable for 2 years after this designation.

Surgical Thoracic Transplant Training: Super Fellowship-Is It Super?

OBJECTIVE: The quality of training provided to thoracic transplant fellows is a critical step in the care of complex patients undergoing transplant. The training varies since it is not an accreditation council for graduate medical education accredited fellowship. METHOD: A total of 104 heart or lung transplant program directors throughout the United States were sent a survey of 24 questions focusing on key aspects of training, fellowship training content and thoracic transplant job satisfaction. Out of the 104 programs surveyed 45 surveys (43%) were returned. RESULTS: In total, 26 programs offering a transplant fellowship were included in the survey. Among these programs 69% currently have fellows of which 56% are American Board of Thoracic Surgery board eligible. According to the United Network for Organ Sharing (UNOS) requirements, 46% of the programs do not meet the requirements to be qualified as a primary heart transplant surgeon. A total of 23% of lung transplant programs also perform less than the UNOS minimum requirements. Only 24% have extra-surgical curriculum. Out of the participating programs, only 38% of fellows secured a job in a hospital setting for performing transplants. An astounding 77% of replies site an unpredictable work schedule as the main reason that makes thoracic transplant a less than favorable profession among new graduates. Long hours were also a complaint of 69% of graduates who agreed that their personal life is affected by excessive work hours. CONCLUSION: Annually, almost half of all thoracic transplant programs perform fewer than the UNOS requirements to be a primary thoracic surgeon. This results in a majority of transplant fellows not finding a suitable transplant career. The current and future needs for highly qualified thoracic transplant surgeons will not be met through our existing training mechanisms.

Implantation of Short-Term and Long-Term Right Ventricular Assist Devices.

The last decade has seen considerable growth in the use of left ventricular assist devices (LVAD), in end-phase heart failure treatment. The indications, contraindications and implantation techniques are well-defined. However, information about mechanical support for right ventricular failure is lacking. The aim of this communication is to present alternative techniques for implantation of short- and longterm right ventricular assist devices. Implanting the device in the right atrium has certain advantages when compared with the right ventricle. It is an easier surgical technique that preserves the tricuspid valve and it can potentially reduce the risk of pump thrombosis.

Implantation of short-term and long-term right ventricular assist devices

Abstract The last decade has seen considerable growth in the use of left ventricular assist devices (LVAD), in end-phase heart failure treatment. The indications, contraindications and implantation techniques are well-defined. However, information about mechanical support for right ventricular failure is lacking. The aim of this communication is to present alternative techniques for implantation of short- and longterm right ventricular assist devices. Implanting the device in the right atrium has certain advantages when compared with the right ventricle. It is an easier surgical technique that preserves the tricuspid valve and it can potentially reduce the risk of pump thrombosis.

Pulmonary tuberculous and tuberculous pericardial tamponade post lung transplant.

Pulmonary tuberculosis (TB) associated with tuberculous pericarditis after lung transplantation is a very rare entity. Here we report a unique case of cardiac tamponade due to tuberculous pericarditis, in a patient 4 months after single right lung transplantation, the patient underwent a subxyphoid pericardial window. The pericardial effusion analysis showed exudate effusion with high level of lymphocyte concentration. Both patient and donor didn't have history of TB or TB Exposure.

Ex vivo lung perfusion review of a revolutionary technology.

Donor lung shortage has been the main reason to the increasing number of patients waiting for lung transplant. Ex vivo lung perfusion (EVLP) is widely expanding technology to assess and prepare the lungs who are considered marginal for transplantation. the outcomes are encouraging and comparable to the lungs transplanted according to the standard criteria. in this article, we will discuss the history of development, the techniques and protocols of ex vivo, and the logics and rationales for ex vivo use.

Hemocompatibility-Related Outcomes in the MOMENTUM 3 Trial at 6 Months: A Randomized Controlled Study of a Fully Magnetically Levitated Pump in Advanced Heart Failure.

BACKGROUND: The HeartMate 3 (HM3) Left Ventricular Assist System (LVAS) (Abbott) is a centrifugal, fully magnetically levitated, continuous-flow blood pump engineered to enhance hemocompatibility and reduce shear stress on blood components. The MOMENTUM 3 trial (Multicenter Study of MagLev Technology in Patients Undergoing Mechanical Circulatory Support Therapy With HeartMate 3) compares the HM3 LVAS with the HeartMate II (HMII) LVAS (Abbott) in advanced heart failure refractory to medical management, irrespective of therapeutic intention (bridge to transplant versus destination therapy). This investigation reported its primary outcome in the short-term cohort (n=294; 6-month follow-up), demonstrating superiority of the HM3 for the trial primary end point (survival free of a disabling stroke or reoperation to replace the pump for malfunction), driven by a reduced need for reoperations. The aim of this analysis was to evaluate the aggregate of hemocompatibility-related clinical adverse events (HRAEs) between the 2 LVAS. METHODS: We conducted a secondary end point evaluation of HRAE (survival free of any nonsurgical bleeding, thromboembolic event, pump thrombosis, or neurological event) in the short-term cohort (as-treated cohort n=289) at 6 months. The net burden of HRAE was also assessed by using a previously described hemocompatibility score, which uses 4 escalating tiers of hierarchal severity to derive a total score for events encountered during the entire follow-up experience for each patient. RESULTS: In 289 patients in the as-treated group (151 the HM3 and 138 the HMII), survival free of any HRAE was achieved in 69% of the HM3 group and in 55% of the HMII group (hazard ratio, 0.62; confidence interval, 0.42-0.91; P=0.012). Using the hemocompatibility score, the HM3 group demonstrated less pump thrombosis requiring reoperation (0 versus 36 points, P<0.001) or medically managed pump thrombosis (0 versus 5 points, P=0.02), and fewer nondisabling strokes (6 versus 24 points, P=0.026) than the control HMII LVAS. The net hemocompatibility score in the HM3 in comparison with the HMII patients was 101 (0.67±1.50 points/patient) versus 137 (0.99±1.79 points/patient) (odds ratio, 0.64; confidence interval, 0.39-1.03; P=0.065). CONCLUSIONS: In this secondary analysis of the MOMENTUM 3 trial, the HM3 LVAS demonstrated greater freedom from HRAEs in comparison with the HMII LVAS at 6 months. CLINICAL TRIAL REGISTRATION: URL: http://clinicaltrials.gov. Unique identifier: NCT02224755.

Surgical Treatment of an Isolated Metastatic Myocardial Neuroendocrine Tumor.

We describe a patient diagnosed with a neuroendocrine tumor of the small intestine metastatic to the heart who underwent successful cardiac metastasectomy. The tumor was located on the right ventricle free wall, obstructing the right ventricular outflow tract. There was no valvular involvement.

Plasma Free Hemoglobin Is an Independent Predictor of Mortality among Patients on Extracorporeal Membrane Oxygenation Support.

BACKGROUND: Hemolysis is common in all extracorporeal circuits as evident by the elevated plasma free hemoglobin (PFHb) level. We investigated whether increased hemolysis during extracorporeal membrane oxygenation (ECMO) is an independent mortality predictor. METHODS: We performed a retrospective observational study of consecutive subjects who received ECMO at a tertiary care facility from 2007-2013 to investigate independent predictors of in-hospital mortality. We examined variables related to patient demographics, comorbidities, markers of hemolysis, ECMO characteristics, transfusion requirements, and complications. 24-hour PFHb > 50 mg/dL was used as a marker of severe hemolysis. RESULTS: 154 patients received ECMO for cardiac (n = 115) or pulmonary (n = 39) indications. Patients' mean age was 51 years and 75.3% were males. Compared to nonsurvivors, survivors had lower pre-ECMO lactic acid (p = 0.026), lower 24-hour lactic acid (p = 0.023), shorter ECMO duration (P = 0.01), fewer RBC transfusions on ECMO (p = 0.008) and lower level of PFHb 24-hours post ECMO implantation (p = 0.029). 24-hour PFHb > 50 mg/dL occurred in 3.9 % versus 15.5% of survivors and nonsurvivors, respectively, p = 0.002. A Cox proportional hazard analysis identified PFHb > 50 mg/dL 24-hours post ECMO as an independent predictor of mortality (OR= 3.4, 95% confidence interval: 1.3 - 8.8, p = 0.011). CONCLUSION: PFHb > 50 mg/dL checked 24-hour post ECMO implantation is a useful tool to predict mortality. We propose the routine checking of PFHb 24-hours after ECMO initiation for early identification and treatment of the cause of hemolysis.

Incidence and Risk Factor Analysis for Gastrointestinal Bleeding and Pump Thrombosis in Left Ventricular Assist Device Recipients.

Left ventricular assist devices (LVADs) are associated with hemostatic complications. We describe the incidence and risk factors for gastrointestinal bleeding (GIB) and pump thrombosis (PT) to optimize patient selection/management. An IRB-approved retrospective review of first LVAD implants between October 1, 2011 and September 30, 2013 at a single center was conducted. Endpoints included epidemiological and risk factor analyses for GIB and PT. Descriptive statistics, chi-squared, and t-tests were used. Sixty-four patients received continuous-flow LVADs. The 12-month incidence of GIB and PT was 23.4% and 12.5%. Time to first GIB was 72.6 days (9-160). The 1-, 3-, and 6-month rate of PT was 1.6%, 6.25%, and 12.5%, respectively. All PT required pump exchange. Females (50% vs. 16%, P = 0.026) and patients without antiplatelet therapy (12.5% vs. 50%, P = 0.046) were at increased risk of PT. No pre-implant comorbidities were associated with PT. Infection was not identified as a risk factor in our cohort (25% vs. 51.8%, P = 0.156). Mean INR preceding event was not different from nonevent patients (2.1 vs. 2.24, P = 0.24). Regarding biomarkers preceding event, elevated plasma free hemoglobin (pfHg) did not reach significance (75% vs. 58%, P = 0.383) while lactate dehydrogenase was elevated significantly (744 vs. 298, P < 0.001). Receiver operating characteristic (ROC) analysis demonstrated that an LDH of >500 was highly sensitive and specific for PT. No pre-implant factors were associated with GIB. Post-implant risk factors for GIB included infection (80% vs. 38.8%, P = 0.005) and infrequent elevations in pfHg (13.3% vs. 63.3%, P < 0.001). Increased pump speed as a GIB risk factor was confirmed (HeartMate II 9560 rpm vs. 9490 rpm, P < 0.001; HeartWare 2949 rpm vs. 2710 rpm, P < 0.001). Anticoagulation/antiplatelet therapy did not affect GIB: mean INR preceding event was not different from nonevent patients (2.21 vs. 2.27, P = 0.67) and antiplatelet use was not different (46.7% vs. 46.9%, P = 0.985). LVADs are associated with early hemostatic-related morbidity. Few pre-implantation risk factors were elucidated; however, post-implantation factors including antiplatelet therapy, infection, and pump speed were identified.

Improvement in blood glucose control in patients with diabetes after implantation of left ventricular assist devices.

Some reports suggest that the course of diabetes mellitus (DM) in heart failure (HF) may improve after implantation of left ventricular assist devices (LVADs). The objective of our study was to explore longitudinal changes in glycosylated hemoglobin (HbA1C) in patients with diabetes mellitus with advanced HF post-LVAD implantation. We retrospectively reviewed the records of all patients who received LVADs at our institution between 2002 and 2012 and selected those who 1) survived posthospital discharge and 2) had DM. We collected data on HbA1C before and after implantation of LVADs, daily doses of insulin, and antidiabetic drugs. Comparisons were done using Student's t-test. A total of 50 patients met the inclusion criteria. HbA1C was 7.6 ± 1.6 before LVAD, 5.7 ± 0.9 within 3 months after the LVAD implant (p = 0.0001), 6.1 ± 1.0 (p = 0.004 in comparison with pre-LVAD level) in 3-6 months after the implant, 6.3 ± 1.0 (p = 0.01) in 6-9 months, and 5.3 ± 0.1 in 9-12 months (p = 0.002). There were no significant changes in body mass index. Favorable changes in clinical course of diabetes in patients with HF occur after the implantation of LVADs, persist for at least 1 year after the implant, and are likely associated with improved hemodynamics and metabolism after normalization of cardiac output.

Centrimag right ventricular support after tricuspid valvectomy for endocarditis.

Recurrent endocarditis of a bioprosthetic tricuspid valve is a challenging condition, which sometimes requires complete valvectomy. We report a case in which a right ventricular assist device, a Thoratec Centrimag Blood Pump (Thoratec Corp., Pleasanton, CA), was successfully used for temporary hemodynamic support to protect the right ventricle until the new tricuspid bioprosthesis could be safely implanted.

Reconstruction of pulmonary venous conduit with CorMatrix in lung transplant.

Anomalous pulmonary vein anatomy is infrequently encountered during reconstructive lung surgery, especially lung transplantation. Complications of pulmonary venous anastomosis carry high morbidity and mortality. We report a case of anomalous pulmonary vein reconstruction with a decellularized porcine small intestinal submucosa-derived extracellular matrix during bilateral lung transplantation in an 18-year-old woman with cystic fibrosis.

Clinical observations and outcome of ventricular tachycardia ablation in patients with left ventricular assist devices.

BACKGROUND: Ablation of ventricular tachycardia (VT) in patients with left ventricular assist devices (LVAD) is challenging and not well documented. This report describes our experience with endocardial VT ablation in six patients with an LVAD. METHODS: We retrospectively reviewed the clinical records of LVAD patients who underwent an ablation procedure for refractory VT. RESULTS: A total of eight ablation procedures were performed in six patients who, during the last 2 weeks before the ablation procedure, received a total of 101 appropriate shocks for VT. A closed aortic valve (n = 2) or aortic atheroma (n = 1) required a transseptal catheterization in three of six patients. The apical LVAD cannula served as a VT substrate in two of six patients. VT was eliminated in four patients and markedly reduced in two others. The latter two patients experienced a total of only four implantable cardioverter defibrillator (ICD) shocks during a follow-up of 130 and 493 days. Intravenous antiarrhythmic medications used in five of six patients before ablation were discontinued in all. The ablation procedures permitted hospital discharge in four of six patients. Five patients died during follow-up (228 ± 207 days after the procedure). The cause of death was unrelated to cardiac arrhythmias. One patient is still alive 1,205 days after the procedure. CONCLUSION: Ablation of VT in LVAD patients is feasible and can result in a markedly decreased VT burden with a reduction of ICD shocks. The subsequent discontinuation of intravenous antiarrhythmic medications may facilitate hospital discharge.

Orthotopic heart transplantation in patients with persistent left superior vena cava and agenesis of left brachiocephalic vein.

Persistent left superior vena cava (PLSVC) is an infrequent abnormality. Anatomical variations can be unexpectedly identified during cardiac procedures. Modifications of surgical techniques become a must during these operations, especially during orthotopic heart transplantation. We present two cases of patients with PLSVC and discuss the anatomy, embryology, diagnosis, and modifications in transplant techniques.

Ablation of left ventricular tachycardia via transeptal approach and crossing of a mechanical mitral valve prosthesis.

This report describes the clinical course of a patient with left ventricular assist device (LVAD) and refractory ventricular tachycardia (VT) who underwent successful left ventricular (LV) mapping and ablation complicated by the presence of a bioprosthetic aortic and a mechanical mitral valve. LV catheterization was achieved by crossing the mechanical valve. The patient remained hemodynamically stable during the procedure most likely as a result of LVAD support. There were no complications. A recurrence of monomorphic VT 2 months later required a second VT ablation procedure using the same transseptal-transmitral approach. The patient has since been free of implantable cardioverter defibrillator shocks for 2 months since the second procedure.

Elevated B-type natriuretic peptide without volume overload in a left ventricular assist device patient with a subdural hematoma.

We report a case of elevated B-type natriuretic peptide (BNP) without volume overload in a left ventricular assist device patient with a subdural hematoma and cerebral salt wasting syndrome suggesting a noncardiac etiology for BNP elevation.