a-large-animal-model-for-pulmonary-hypertension-right-ventricular

A Large Animal Model for Pulmonary Hypertension and Right Ventricular Failure: Left Pulmonary Artery Ligation and Progressive Main Pulmonary Artery Banding in Sheep

Decompensated right ventricular failure (RVF) in pulmonary hypertension (PH) is fatal, with limited medical treatment options. Developing and testing ...

Vanderbilt University Medical Center

A triage cardiology program, in which cardiologists provide consultation to the Emergency Department (ED), may safely reduce admissions. For patients with chest pain, the HEART Pathway may obviate the need for cardiology involvement, unless there is a difference between ED and cardiology assessments. Therefore, in a cohort concurrently evaluated by both specialties, we analyzed discordance between ED and cardiology HEART scores.

Documentation of HEART score discordance between emergency physician and cardiologist evaluations of ED patients with chest pain.

Left-sided inferior vena cava (IVC) is a rare congenital venous anomaly. We describe a case of open repair of a nonruptured abdominal aortic aneurysm (AAA) in a patient with left-sided inferior vena cava requiring IVC transection and reconstruction with interposition graft.

Abdominal Aortic Aneurysm Repair Requiring Left-Sided Inferior Vena Cava Interposition.

Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We describe the characteristics and outcomes of patients undergoing LT for LCH. All patients undergoing a first LT for LCH in the United States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987-2018). The Kaplan-Meier curve method and log-rank tests evaluated post-LT survival. A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. A total of 60 LCH LT recipients were identified in the SRTR, and 55 patients (91.7%) were children with median total bilirubin levels at LT of 5.8 mg/dL (interquartile range [IQR], 2.7-12.9). A total of 49 patients (81.7%) underwent deceased donor LT (DDLT). The 1-year, 3-year, and 5-year patient survival rates were 86.6%, 82.4%, and 82.4%, respectively. The systematic review yielded 26 articles reporting on 50 patients. Of the patients, 41 were children (82.0%), 90.0% had multisystem LCH, and most patients underwent DDLT (91.9%; n = 34/37). Pre-LT chemotherapy was administered in 74.0% and steroids in 71.7% (n = 33/46) of the patients, and a recurrence of LCH to the liver was reported in 8.0% of the patients. Of the 50 patients, 11 (22.0%) died during a median follow-up of 25.2 months (IQR, 9.0-51.6), and the 1-year patient survival rate was 79.4%. LT can be considered as a feasible life-saving option for the management of liver failure secondary to LCH in well-selected patients.

Liver Transplantation for Langerhans Cell Histiocytosis: A US Population-Based Analysis and Systematic Review of the Literature.

Hepatoblastoma is the most common type of liver cancer in children. Refined therapeutic approaches combining risk-adapted chemotherapy along with complete tumor resection has led to improved survival. We aimed to evaluate the current state of management and outcomes for hepatoblastoma in the United States.

Management of hepatoblastoma in the United States: Can we do better?

Decompensated right ventricular failure (RVF) in patients with pulmonary hypertension (PH) is fatal, with limited treatment options. Novel mechanical circulatory support systems have therapeutic potential for RVF, but the development of these devices requires a large animal disease model that replicates the pathophysiology observed in humans. We previously reported an effective disease model of PH in sheep through ligation of the left pulmonary artery (PA) and progressive occlusion of the main PA. Herein, we report a case of acute decompensation with this model of chronic RVF. Gradual PA banding raised the RV pressure (maximum RV systolic/mean pressure = 95 mmHg/56 mmHg). Clinical findings and laboratory serum parameters suggested appropriate physiologic compensation for 7 weeks. However, mixed venous saturation declined precipitously on week 7, and creatinine increased markedly on week 9. By the 10th week, the animal developed dependent, subcutaneous edema. Subsequently, the animal expired during the induction of general anesthesia. Post-mortem evaluation revealed several liters of pleural effusion and ascites, RV dilatation, eccentric RV hypertrophy, and myocardial fibrosis. The presented case supports this model's relevance to the human pathophysiology of RVF secondary to PH and its value in the development of novel devices, therapeutics, and interventions.

Progression Toward Decompensated Right Ventricular Failure in the Ovine Pulmonary Hypertension Model.

Liver transplantation (LT) for children with urea cycle disorders (UCD) is capable of correcting the enzymatic defect and preventing progressive neurologic injury. We describe the characteristics and outcomes of pediatric LT recipients with UCD.

Article	Year
Documentation of HEART score discordance between emergency physician and cardiologist evaluations of ED patients with chest pain. The American journal of emergency medicine\| PubMed ID: 27745728	2017
Abdominal Aortic Aneurysm Repair Requiring Left-Sided Inferior Vena Cava Interposition. Annals of vascular surgery\| PubMed ID: 32439518	2020
Liver Transplantation for Langerhans Cell Histiocytosis: A US Population-Based Analysis and Systematic Review of the Literature. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society\| PubMed ID: 33484600	2021
Management of hepatoblastoma in the United States: Can we do better? Surgery\| PubMed ID: 33526266	2021
Progression Toward Decompensated Right Ventricular Failure in the Ovine Pulmonary Hypertension Model. ASAIO journal (American Society for Artificial Internal Organs : 1992)\| PubMed ID: 33883506	2021
Liver Transplantation in Children with Urea Cycle Disorders: The Importance of Minimizing Waiting Time. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society\| PubMed ID: 34058057	2021

W. Kelly Wu

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