Does reduced cardiopulmonary exercise testing performance predict poorer quality of life in adult patients with Fontan physiology?

BACKGROUND: Cardiopulmonary exercise testing performance has been shown to be a predictor of morbidity, mortality, and quality of life in patients with Fontan physiology; however, the role of exercise performance along with other diagnostics is not fully understood. We evaluated the hypothesis that reduced exercise performance correlates with poorer quality of life in Fontan patients as they continue to age. METHODS: Chart review was performed on patients 12 years and older with Fontan who had completed cardiopulmonary exercise testing and age-appropriate quality of life surveys. Quality of life outcomes were analysed against exercise performance and other descriptive data. RESULTS: For the younger cohort (n = 22), exercise performance predicted quality of life with different measures across domains and had a stronger correlation than echocardiographic parameters. For the older cohort (n = 34), exercise performance did not predict quality of life. CONCLUSIONS: Objective exercise performance was a useful marker for general, physical, emotional, social, and school quality of life in a younger cohort but less helpful in older adults. This is perhaps due to older patients accommodating to their conditions over time. The role of exercise performance and objective data in predicting quality of life in patients with Fontan physiology is incompletely understood and additional prospective evaluation should be undertaken.

Varied Presentations and Comorbidities in Pediatric Autoimmune Pancreatitis.

Autoimmune pancreatitis (AIP) is a chronic inflammatory condition rarely reported in children. In 2018, to standardize the approach to AIP, INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) defined AIP, outlined the clinical course, and developed diagnostic and therapeutic recommendations. We performed a retrospective review of cases at our institution from January 1, 2016, to June 1, 2019, and compared their presentations with the INSPPIRE guidelines. Our patients showed variable laboratory, radiographic, and histologic findings, highlighting the difficulty in diagnosing AIP. Histologic samples were obtained in our patients due to diagnostic uncertainty, which ultimately confirmed the diagnosis. One patient was diagnosed with autoimmune hepatitis coexistent with AIP, which has not been previously described in the pediatric population. Exocrine and endocrine complications of AIP were also noted. In all cases, symptoms improved following treatment, and decompression of the common bile duct was seen on repeat imaging.

Long Term Consequences of the Fontan Procedure and How to Manage Them.

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.

Problem-Based Learning: Cervical Adenocarcinoma Three-Session Case for First-Year Medical Students-Patient Minnie Pauls.

INTRODUCTION: Cervical adenocarcinoma represents a critical health problem in many underserved regions of the world and parts of the U.S. This module provides learning opportunities in the areas of female anatomy, physiology, histology, and pathology. This includes diagnosis by ultrasound and CT/PET scan, detailed staging and treatment of the cancer by various criteria, and future prevention by vaccination and screening. METHODS: Authors include a fourth-year medical student and a seasoned PBL facilitator with a basic science interest in cancer. In this problem-based learning module (PBL), a group of first-year medical students review the material that is released online for each of three weekly 90-minute sessions. Key learning issues are identified, researched out-of-class, and discussed at the beginning of the subsequent session. A differential diagnosis is weighed and the module culminates with a concept map drawn by students to integrate all relevant aspects and mechanisms of the case. RESULTS: The module was implemented twice with a small group of seven students. Students learned to correlate relevant biochemical mechanisms, histological, and anatomical features with the clinical signs and symptoms, to diagnose and suggest treatment options. The module was well-liked, and revised for publication by rebalancing the material based on specific student feedback. DISCUSSION: The PBL small-group format provides a unique opportunity over both semesters for first-year medical students to study clinical cases in a student-directed fashion and develop professional skills at various levels. Potential pitfalls lie in the online format, as this requires clear rules on computer usage and data sharing.

Amnesia Associated with Bilateral Hippocampal and Bilateral Basal Ganglia Lesions in Anoxia with Stimulant Use.

We report a case of a 55-year-old man with ischemic lesions of the bilateral hippocampus and bilateral basal ganglia following a myocardial infarction during an episode of multiple drug use with subsequent anoxia requiring resuscitation. He presented for a neuropsychological evaluation with an anterograde amnesia for both explicit and procedural memory. There are two main points to this case, the unique aspects of the bilateral multifocal lesions and the functional, cognitive impact of these lesions. We hypothesize that his rare focal bilateral lesions of both the hippocampus and basal ganglia are a result of anoxia acting in synergy with his stimulant drug use (cocaine and/or 3,4-methylenedioxy-methamphetamine). Second, his unique lesions produced an explicit and implicit/procedural anterograde amnesia.