Pediatric lung transplantation for COVID-19: Unique clinical and psychosocial barriers.

BACKGROUND: SARS-CoV-2 infection in the age group of 0-17 years contributes to approximately 22% of all laboratory-confirmed SARS-CoV-2 infections. Fortunately, this age group has a lower death rate (0.5 per 100 000) that accounts for only 4% of the total deaths due to COVID-19. Despite the low mortality rate in the pediatric population, children of minority groups represented 78% of the deaths highlighting the existing disparities in access to health care. METHODS: With the emergence of the more contagious COVID-19 variants and the relatively slow pace of vaccination among the pediatric population, it is possible to see more cases of significant lung injury and potential for transplantation for the younger age group. RESULTS: To our knowledge, our patient is the youngest to have undergone lung transplantation for SARS-CoV-2. CONCLUSION: The case presented unique challenges, particularly in relation to timing for listing and psychosocial support for parents who were his decision makers.

Pulmonary Arteriovenous Malformations in Non-hereditary Hemorrhagic Telangiectasia Patients: An 18-Year Retrospective Study.

PURPOSE: Pulmonary arteriovenous malformations (PAVMs) are most commonly associated with hereditary hemorrhagic telangiectasia (HHT). Patients with PAVMs can present with serious complications including stroke, transient ischemic attack (TIA), and brain abscess. PAVMs are rare in non-HHT patients and little is known about this patient population. The aim of this retrospective study is to better understand clinical presentation and outcomes of PAVMs occurring exclusively in non-HHT patients. METHODS: Non-HHT patients with PAVMs at the Mayo Clinic-Rochester between 01/01/2000 and 12/31/2018 were reviewed. Patients with Curacao score > 1 were excluded. Demographics, imaging characteristics, neurological complications, and follow-up imaging were analyzed. RESULTS: Seventy-seven patients with PAVMs were identified. The mean age at diagnosis was 48.2 ± 18.3 years with female preponderance (59.7%). The majority of PAVMs had lower lobe predominance (66.7%) and were simple and single in 75.3% and 89.6% of cases, respectively. Most patients were asymptomatic (46.8%) with dyspnea being the most common symptom (28.6%). Neurologic complications occurred in 19.5% of patients. The majority of PAVMs were idiopathic (61%). Thirty patients (39%) had one or more possible risk factors including previous thoracic surgery (23.4%), congenital heart disease (19.5%), and chest trauma (10.4%). Embolization was performed in 37 (48.1%) patients and only 4 (5.2%) underwent surgical resection. CONCLUSIONS: Non-HHT PAVMs occur more commonly in females, are most commonly simple and single, and have lower lobe predominance and a high rate of neurologic complications. Potential predisposing risk factors were identified in about 40% of the cases. Clinicians should be aware of the risk of PAVM development in patients with history of chest trauma, congenital heart disease, lung infection/abscess, and thoracic surgery.

Relationship between portopulmonary hypertension and splenectomy: Mayo Clinic experience and review of published works.

AIM: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: This is a retrospective analysis of patients diagnosed with portopulmonary hypertension between 1 January 1988 and 30 June 2015 at Mayo Clinic (Rochester, MN, USA). We compared age, sex, right ventricle systolic pressure by echocardiography, and right heart catheterization measurements/calculations among subgroups of portopulmonary hypertension patients with splenectomy and/or autoimmune liver disease (autoimmune hepatitis/primary biliary cirrhosis/primary sclerosing cholangitis). RESULTS: The cohort consisted of 141 patients, of whom 8 (6%) had a history of splenectomy prior to the development of portopulmonary hypertension. Twenty-seven (19%) portopulmonary hypertension patients had autoimmune liver disease, and 5 of 8 (62.5%) splenectomized portopulmonary hypertension patients had autoimmune liver disease. No significant difference was noted in right heart catheterization measurements/calculations between splenectomized and non-splenectomized portopulmonary hypertension patients. Right ventricle systolic pressure by echocardiography was significantly higher in those splenectomized. CONCLUSIONS: Prior history of splenectomy in portopulmonary hypertension was 6% in this cohort. The combination of autoimmune liver disease and splenectomy in portopulmonary hypertension was not uncommon. History of splenectomy in patients with portal hypertension and/or autoimmune liver disease may have clinical implications.

Microscopic polyangiitis: Atypical presentation with extensive small bowel necrosis, diffuse alveolar hemorrhage, and renal failure.

Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes. Further investigations after transfer to our facility determined that the patient was actually suffering from a severe exacerbation of previously diagnosed microscopic polyangiitis. He quickly developed diffuse alveolar hemorrhage (DAH) necessitating intubation and acute kidney injury (AKI) requiring dialysis. He subsequently developed mesenteric ischemia and bowel necrosis resulting in emergent laparotomy and extensive small bowel resection. Physicians need to be aware that microscopic polyangiitis can very rarely present with severe involvement of the abdominal viscera and mesenteric vessels. Severe disease necessitates the use of high dose IV steroids, rituximab or cyclophosphamide, and plasma exchange (PLEX).

Primary spontaneous pneumothorax due to high bleb burden.

Primary spontaneous pneumothorax (PSP) occurs most frequently in young, tall, lean, male smokers without any known underlying lung disease. It is an important diagnosis to make promptly in order to prevent progression to obstructive shock. We present a case report of a young, male, former-smoker and polysubstance abuser with no prior lung history that developed acute dyspnea at rest and was found to have a large right pneumothorax on chest x-ray. A pig-tail catheter was utilized, but his course was complicated by a recurrent tension pneumothorax. Chest computed tomography (CT) revealed a significant bleb burden and the patient underwent a lung wedge resection with mechanical pleurodesis. What made our case unique were the chest x-rays revealing blebs that could have predicted the patient's recurrent pneumothorax as well as the multiple potential risk factors for developing blebs and a pneumothorax. All patients with recurrent PSPs and those with higher risk initial PSPs (e.g. blebs) should undergo preventive therapy with pleurodesis. We hope that clinicians can benefit from utilizing these representative chest x-ray images showing blebs.

Highly sensitive cardiac troponin assays: a comprehensive review of their clinical utility.

Cardiovascular disease is a major source of morbidity and remains the leading cause of death in the United States. To evaluate patients with suspected cardiovascular disease, several laboratory markers of cardiac injury are available including creatinine kinase, creatinine kinase isoenzyme MB, myoglobin, and cardiac troponins (cTn) I and T. cTns have become the preferred marker of cardiac damage because they are highly sensitive and are almost completely cardiac specific. Recently, highly sensitive cTn (hs-cTn) assays were developed and have been used across the world to more quickly and accurately diagnose myocardial infarction. In the United States, these assays have not been U.S. Food and Drug Association approved for clinical practice and therefore are only being used for research purposes. Advancements have taken place over the years in diagnosing and treating cardiovascular disease, but predicting who will suffer future cardiovascular events still remains elusive. A comprehensive review of the literature was conducted to discover how new hs-cTn are being utilized throughout other parts of the world and what potential future use they may serve here in the United States. Major subject points discussed in this article include the prevalence of detectable troponins in the general population, the use of hs-cTn assays in diagnosing a myocardial infarction, determining prognosis in patients who have experienced an acute coronary syndrome and in patients with congestive heart failure, predicting future risk of cardiovascular events in patients with coronary heart disease, and its potential role as a screening tool in primary prevention. The implementation of hs-cTn testing in the United States may be very beneficial to patients; however, more research will need to be done to fully elucidate the potential use of hs-cTn in clinical practice.

Pulmonary embolus diagnosed by endobronchial ultrasound.

Endobronchial ultrasound (EBUS) imaging is commonly used to evaluate and aid in biopsy of mediastinal lymph nodes. Pulmonary arteries are readily viewable with this type of imaging modality. We present a case report of a pulmonary embolism (PE) diagnosed by EBUS. Our patient had no smoking history and presented with respiratory and constitutional symptoms, urinary retention, and leg weakness suspicious for malignancy with metastasis to spine. Chest computed tomography (CT) was suggestive of lung carcinoma and specifically showed no PE. EBUS with TBNA was requested for tissue diagnosis. A mobile filling defect consistent with a PE was observed and reported to primary team. Follow-up chest CT showed an acute PE which confirmed the diagnosis originally made by EBUS. Bronchoscopists should be aware of potential to diagnose a PE while performing EBUS. Additionally, there may be a role in using EBUS specifically to diagnose a PE in the right patient population.