Alpha gal syndrome, a relative not absolute contraindication to the use of bovine pericardium to close an intracardiac septal defect: a case report.

BACKGROUND: Alpha-gal syndrome is an allergic condition in which individuals develop an immune-mediated hypersensitivity response when consuming red meat and its derived products. Its diagnosis is important in individuals undergoing cardiac surgery, as patients frequently require large doses of unfractionated heparin or the insertion of surgical implants, both of which are porcine or bovine in origin. There are currently no guidelines for heparin administration in alpha-gal patients, with even less knowledge regarding the long-term clinical implications of these patients after receiving bioprosthetic valve replacements or other prostheses. CASE PRESENTATION: We present the case of a 31-year-old male who underwent cardiac surgery in the setting of alpha-gal syndrome for a large atrial septal defect (ASD) and mitral valve prolapse (MVP). The patient continues to do well one year after undergoing a mitral valve repair, tricuspid valve repair and an ASD closure using bovine pericardium. He sustained no adverse reaction to the use of heparin products or the presence of a bovine pericardial patch. This rare case was managed by a multidisciplinary team consisting of cardiothoracic surgery, cardiac anesthesiology, and allergy/immunology that led to an optimal outcome despite the patient's pertinent allergic history. CONCLUSIONS: This case highlights that the use of bovine pericardium and porcine heparin to close septal defects in patients with milder forms of alpha-gal allergy can be considered if other options are not available. Further studies are warranted to investigate the long-term outcomes of such potential alpha-gal containing prostheses and heparin exposure and establish the optimal decision making algorithm and prophylactic regimen.

Respiratory extracorporeal membrane oxygenation : From rescue therapy to standard tool for treatment of acute respiratory distress syndrome?

BACKGROUND: The use of extracorporeal membrane oxygenation (ECMO) for patients with acute respiratory distress syndrome (ARDS) has increased substantially. With modern trials supporting its efficacy, ECMO has become an important tool in the management of severe ARDS. OBJECTIVES: The objectives of this paper are to discuss ECMO physiology and configurations used for patients with ARDS, review evidence supporting the use of ECMO for ARDS, and discuss aspects of management during ECMO. CONCLUSION: Current evidence supports the use of ECMO, combined with an ultra-lung-protective approach to mechanical ventilation, in patients with ARDS who have refractory hypoxemia or hypercapnia with severe respiratory acidosis. Furthermore, data suggest that center volume and experience are important factors in the care of patients receiving ECMO. The use of extracorporeal technologies in expanded patient populations and the optimal management of patients during ECMO remain areas of investigation. This article is freely available.

Double jeopardy: Successful management of a traumatic ascending aortic pseudoaneurysm with concurrent subdural hematoma.

We present the case of a 59-year-old male who sustained an ascending aortic injury and a subdural hematoma after a head on collision. After undergoing emergent craniotomy for evacuation of the subdural hematoma, he was maintained with strict blood pressure control. Once able to be safely anticoagulated, he underwent replacement of the ascending aorta. This exceedingly rare case was managed by a multidisciplinary team approach that led to an optimal outcome given his disastrous multi-traumatic injuries.

Interventions and neonatal outcomes of fetuses with hypoplastic left heart syndrome and congenital diaphragmatic hernia: a systematic review.

OBJECTIVE: To evaluate the current interventions and outcomes in neonates born with hypoplastic left heart syndrome (HLHS) and congenital diaphragmatic hernias (CDHs). METHODS: A comprehensive search of the literature using relevant search words was conducted from the date of each database inception to August 2018 to identify interventions and outcomes in patients with CDH and HLHS. Conference papers, review articles, case reports, and animal studies were not eligible for this review. Articles were also excluded if left heart hypoplasia, rather than HLHS, was studied. Neither language nor sample size was used for exclusion. A preliminary screening of article titles was performed to rule out irrelevant articles and article types by a single reviewer. A second screening circle was performed by two independent reviewers to assess remaining abstracts. Data were presented as a systematic review and were descriptively summarized. RESULTS: Of the 162 retrieved articles, five studies were eligible for inclusion. The total number of neonates included in this systematic review was 5063. One hundred and eighteen (2.3%) presented specifically with CDH and HLHS. Overall, a total of 62 of the 118 patients underwent a surgical procedure (51%) with 36 (59%) surviving to discharge. CONCLUSIONS: Available data suggest current surgical interventions still result in a high degree of mortality. Age at which surgery occurred, pulmonary function and the use of extra-corporeal membrane oxygenation (ECMO) are potential variables that may determine and improve outcomes in these patients in the future. However, long-term outcomes and analyses of more variables are needed to determine effective treatment for this rare but often fatal comorbid congenital diagnosis.

Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect.

BACKGROUND: Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echocardiography for right ventricular (RV) cardiac output assessment in patients with left-to-right shunt. The purpose of the study was to determine the correlation between Doppler-derived and Fick-derived RV cardiac index (CI), and the interobserver correlation in Doppler-derived RV CI assessment. METHODS: Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004-2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle outflow tract diameter)2  × right ventricular outflow tract (RVOT) time velocity integral × heart rate]/body surface area. RESULTS: A total of 128 patients (age 52 ± 17 years; female 88 [69%]) met the inclusion criteria. There was a modest correlation between Doppler-derived and Fick-derived RV CI (r = .57, P < .001), and the mean difference between Doppler-derived and Fick-derived RV CI was -.3 (95% confidence interval of agreement, -.8 to +.9) L/min/m2 . There was also a modest correlation between Doppler-derived RV CI from observer #1 and observer #2 (r = .62, P < .001), and the mean difference between Doppler-derived RV CI from observer #1 and observer #2 was -.2 (95% confidence interval of agreement, -.9 to +.6). CONCLUSIONS: The current study demonstrated a modest correlation between Doppler-derived and Fick-derived RV cardiac output, and a modest interobserver correlation in Doppler-derived RV cardiac output assessment. Further studies are required to validate these results and to explore other potential applications such as in patients with chronic pulmonary regurgitation.

Redo mitral valve surgery following prior mitral valve repair.

BACKGROUND: The optimal treatment strategy following a failed mitral valve repair remains unclear. This study evaluated early and long-term outcomes of redo mitral valve repair (MVr) and replacement (MVR) after prior mitral valve repair. METHODS: Patients undergoing redo mitral valve surgery after prior mitral valve repair at a single institution between 2002 and 2014 were reviewed. Primary outcomes included operative mortality (30-day or in-hospital mortality) and long-term freedom from mitral valve reoperation and death. Secondary outcomes included postoperative complications. RESULTS: 305 patients underwent redo MVr (n = 48) or MVR (n = 257) after prior mitral valve repair. Concomitant procedures included tricuspid valve repair or replacement (23%), aortic valve replacement (6%), and coronary artery bypass grafting (4%), with no differences between cohorts. 18% were performed via right mini-thoracotomy (24% MVr vs 18% MVR, P = 0.31). Unadjusted and risk-adjusted operative mortality were lower with MVr (0% vs 8%, P = 0.04). Rates of postoperative complications were similar except for blood product transfusion (35% MVr vs 59% MVR, P = 0.003) and prolonged mechanical ventilation (8% MVr vs 29% MVR, P = 0.003). Long-term freedom from mortality was comparable: 96% MVr versus 86% MVR at 1 year and 78% MVr versus 68% MVR at 5 years (P = 0.29). CONCLUSIONS: When technically feasible, mitral valve re-repair can be safely performed with outcomes comparable to MVR.

A Novel Mouse Model to Study Image-Guided, Radiation-Induced Intestinal Injury and Preclinical Screening of Radioprotectors.

Radiation is an important treatment modality for gastrointestinal tumors, but intestinal injury is a common side effect. Here we describe a physiologically relevant model for studying the molecular determinants of radiation-induced intestinal damage and testing novel radioprotectors. The model employs a radiopaque marker implanted into the surface of the mouse jejunum, serving as a fiducial marker for precise radiation targeting. Mice were imaged with Cone-Beam CT (CBCT) and irradiated (IR) to the marked area using the Small Animal Radiation Research Platform (SARRP). IR-induced damage was acute but reversible and largely restricted to the area of the marker, leaving the surrounding tissues intact. Although whole gut irradiation with these doses caused lethal GI syndrome, focal (5 mm) radiation of the intestine did not cause any weight loss or lethality. However, fibrosis and collagen deposition 4 months post-IR indicated chronic intestinal damage. A separate cohort of mice was treated daily with curcumin, a clinically tested radioprotector, prior to and post-IR. Curcumin-treated mice showed significant decreases in both local and systemic inflammatory cytokine levels and in fibrosis, suggesting it is an effective radioprotector of the intestine. Our results indicate that this model, which emulates clinically relevant intestinal radiation-induced injury, can be used to assess radioprotectors prior to testing in the clinic. Cancer Res; 77(4); 908-17. ©2016 AACR.

Local and sustained miRNA delivery from an injectable hydrogel promotes cardiomyocyte proliferation and functional regeneration after ischemic injury.

MicroRNA-based therapies that target cardiomyocyte proliferation have great potential for the treatment of myocardial infarction (MI). In previous work, we showed that the miR-302/367 cluster regulates cardiomyocyte proliferation in the prenatal and postnatal heart. Here, we describe the development and application of an injectable hyaluronic acid (HA) hydrogel for the local and sustained delivery of miR-302 mimics to the heart. We show that the miR-302 mimics released in vitro promoted cardiomyocyte proliferation over one week, and that a single injection of the hydrogel in the mouse heart led to local and sustained cardiomyocyte proliferation for two weeks. After MI, gel/miR-302 injection caused local clonal proliferation and increased cardiomyocyte numbers in the border zone of a Confetti mouse model. Gel/miR-302 further decreased cardiac end-diastolic (39%) and end-systolic (50%) volumes, and improved ejection fraction (32%) and fractional shortening (64%) four weeks after MI and injection, compared to controls. Our findings suggest that biomaterial-based miRNA delivery systems can lead to improved outcomes in cardiac regeneration.