Severe COVID-19 acute respiratory distress syndrome in an adult with single-ventricle physiology: a case report.

BACKGROUND: COVID-19 can induce acute respiratory distress syndrome (ARDS). In patients with congenital heart disease, established treatment strategies are often limited due to their unique cardiovascular anatomy and passive pulmonary perfusion. CASE PRESENTATION: We report the first case of an adult with single-ventricle physiology and bidirectional cavopulmonary shunt who suffered from severe COVID-19 ARDS. Treatment strategies were successfully adopted, and pulmonary vascular resistance was reduced, both medically and through prone positioning, leading to a favorable outcome. CONCLUSION: ARDS treatment strategies including ventilatory settings, prone positioning therapy and cannulation techniques for extracorporeal oxygenation must be adopted carefully considering the passive venous return in patients with single-ventricle physiology.

The Clinical Course of COVID-19 Pneumonia in a 19-Year-Old Man on Intravenous Immunoglobulin Replacement Therapy for X-Linked Agammaglobulinemia.

BACKGROUND Since the emergence of coronavirus disease 2019 (COVID-19), patients with the illness have presented with considerable variation in severity. Some infected individuals present mild or no symptoms, while others present severe illness with some fatal outcomes. Multiple lines of management have been suggested for critically ill patients, such as intravenous immunoglobulin (IVIG) and steroids. IVIG is the main treatment for patients with X-linked agammaglobulinemia. Multiple studies have reported that these patients have excellent outcomes when they contract COVID-19. This report describes the clinical course of COVID-19 pneumonia due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in a 19-year-old man on IVIG replacement therapy for X-linked agammaglobulinemia (XLA). CASE REPORT A patient with XLA receiving a monthly dose of IVIG and having bronchiectasis managed by prophylactic azithromycin presented with fever, shortness of breath, productive cough, and diarrhea. He was admitted to our hospital with SARS-CoV-2 infection. His treatment course for COVID-19 was uncomplicated and had excellent results. He completed a 10-day course of piperacillin/tazobactam and his symptoms resolved 3 days after admission, without complications, oxygen supplementation, or intensive care unit admission. CONCLUSIONS Patients with XLA have weakened immunity and therefore may present with an infection as a first symptom. This report describes the mild course of COVID-19 pneumonia in an immunologically vulnerable patient with XLA who presented with SARS-CoV-2 infection while undergoing IVIG replacement therapy. Currently, IVIG is one of many supportive immune therapies undergoing clinical evaluation in patients with severe COVID-19.

Two X-linked agammaglobulinemia patients develop pneumonia as COVID-19 manifestation but recover.

BACKGROUND: The recent SARS-CoV-2 pandemic, which has recently affected Italy since February 21, constitutes a threat to normal subjects, as the coronavirus disease-19 (COVID-19) can manifest with a broad spectrum of clinical phenotypes ranging from asymptomatic cases to pneumonia or even death. There is evidence that older age and several comorbidities can affect the risk to develop severe pneumonia and possibly the need of mechanic ventilation in subjects infected with SARS-CoV-2. Therefore, we evaluated the outcome of SARS-CoV-2 infection in patients with inborn errors of immunity (IEI) such as X-linked agammaglobulinemia (XLA). METHODS: When the SARS-CoV-2 epidemic has reached Italy, we have activated a surveillance protocol of patients with IEI, to perform SARS-CoV-2 search by nasopharyngeal swab in patients presenting with symptoms that could be a manifestation of COVID-19, such as fever, cough, diarrhea, or vomiting. RESULTS: We describe two patients with X-linked agammaglobulinemia (XLA) aged 34 and 26 years with complete absence of B cells from peripheral blood who developed COVID-19, as diagnosed by SARS-CoV-2 detection by nasopharyngeal swab, while receiving immunoglobulin infusions. Both patients developed interstitial pneumonia characterized by fever, cough, and anorexia and associated with elevation of CRP and ferritin, but have never required oxygen ventilation or intensive care. CONCLUSION: Our report suggests that XLA patients might present with high risk to develop pneumonia after SARS-CoV-2 infection, but can recover from infection, suggesting that B-cell response might be important, but is not strictly required to overcome the disease. However, there is a need for larger observational studies to extend these conclusions to other patients with similar genetic immune defects.