Idiopathic Capillary Leak Syndrome (Clarkson's Disease): First Reported Case in Oman.

Idiopathic capillary leak syndrome, also known as Clarkson's Disease, is a rare cause of hypovolemic shock that physicians should be aware of. It is characterized by a state of hypovolemia with features of widespread fluid third spacing and poses diagnostic and therapeutic challenges. Here, we present a challenging case of a 36-year-old woman who experienced recurrent episodes of widespread edema and hypovolemic shock symptoms suggestive of capillary leak syndrome. The resuscitative and therapeutic measures employed in managing this disease are described in this case report.

Evaluating the Impact of Pulmonary Arterial Hypertension Therapies on Pericardial Effusion and Patient Survival.

Objectives: To evaluate the impact of Pulmonary Arterial Hypertension (PAH) therapies on the incidence of pericardial effusion and its prognostic implications for patient survival. Methods: This retrospective cohort study included 60 patients diagnosed with PAH at a high-volume tertiary care center, treated with intravenous or subcutaneous prostanoids. Data were collected from 2015 to 2019, including echocardiographic assessments, right heart catheterization, World Health Organization functional class evaluations, six-minute walk distance tests, and biomarkers such as brain natriuretic peptide and N-terminal prohormone of brain natriuretic peptide. Follow-up was conducted at least 90 days post-treatment initiation. Results: Pericardial effusion was observed in 31.7% of patients before therapy. Patients with moderate to large effusions had a significantly higher mortality risk (HR = 1.92; 95% CI 1.1-44.78; p =0.0044), while small effusions appeared protective (HR = 0.27; 95% CI 0.15-0.48; p =0.006). Survival rates declined from 89% at one year to 71% at three years post-therapy, with effusion presence correlating with more severe PAH manifestations. Conclusions: Initial pericardial effusion severity is a critical predictor of mortality in PAH patients. Early assessment and stratified management of pericardial effusion are essential for optimizing therapeutic outcomes in PAH management. Future research should explore targeted interventions for managing pericardial effusion to improve patient prognosis.

Iron Deficiency in Pulmonary Hypertension.

Pulmonary hypertension (PH) is a complex cardiovascular condition that is characterized by elevated pulmonary arterial pressure, which leads to significant morbidity and mortality. Among the various factors that influence the pathophysiology and progression of PH, iron deficiency has become a critical, yet often overlooked, element. In this review, the prevalence, implications, and therapeutic potential of addressing iron deficiency in patients with PH are elucidated.Iron deficiency, which is prevalent in a significant proportion of patients with PH, has been associated with worsened clinical outcomes, including diminished exercise capacity, impaired oxygen transport and utilization, and compromised right ventricular function. The pathophysiological linkages between iron deficiency and PH are multifaceted and involve alterations in oxygen sensing, endothelial function, and metabolic disturbances.In this review, the evidence from recent clinical trials and studies that assess the impact of iron supplementation, both oral and intravenous, on PH outcomes is critically analyzed. Although some studies suggest improvements in exercise capacity and hemodynamic parameters following iron repletion, the responses appear variable and are not universally beneficial. This review highlights the complexities of iron metabolism in PH and the challenges in effectively diagnosing and treating iron deficiency in this patient population.Furthermore, the potential mechanisms through which iron supplementation might influence pulmonary vascular and right ventricular function, emphasizing the need for personalized treatment approaches are discussed. In this review, the importance of recognizing iron deficiency in the management of patients with PH is highlighted, and further research is warranted to establish comprehensive, evidence-based guidelines for iron supplementation in this unique patient cohort. The ultimate goal of this review is to improve clinical outcomes and quality of life for patients suffering from this debilitating condition.

The Road to Heart Transplant in a Patient With Cardiomyopathy, Shone Complex, and Severe Pulmonary Hypertension.

Our case report details the journey of a 16-year-old male patient with Shone complex and advanced heart failure. We highlight the pivotal role of the HeartWare Ventricular Assist Device (Medtronic) implantation in mitigating severe pulmonary hypertension, thereby facilitating his eligibility for a heart transplant. We discuss the subsequent management of post-transplant pulmonary hypertension and right ventricular dysfunction using targeted pulmonary vasodilators and inotropic support, underscoring the intricacies of postoperative care in pediatric heart transplant patients. This case emphasizes our observation of the critical role that left ventricular assist devices play in redefining transplant candidacy and the necessity for complex, ongoing management in pediatric heart transplant scenarios.

Impact of tricuspid regurgitation severity on mortality in pulmonary hypertension patients: A comprehensive analysis.

BACKGROUND: Pulmonary hypertension (PH) presents as a complex cardiovascular condition with a highly variable prognosis. Secondary tricuspid regurgitation (TR), a frequent comorbidity in PH, has an uncertain impact on patient outcomes. This study investigates the relationship between the severity of TR and mortality in patients with PH. METHODS: A retrospective analysis of 110 PH patients who underwent right-heart catheterization was conducted. TR severity was categorized echocardiographically as mild, moderate, or severe. Both univariate and multivariate Cox regression analyses were used to assess predictors of mortality. RESULTS: The median age of the cohort was 60 years, predominantly female (65 %). Severe TR was present in 36 % of patients. Over a 20-month median follow-up, mortality was notably higher in patients with severe TR (30 %) compared to those with milder forms (15 %, p = 0.04). Multivariate analysis confirmed severe TR, WHO functional class III/IV, and right ventricular stroke work index as independent predictors of mortality. CONCLUSION: Severe TR significantly predicts increased mortality in PH patients, underscoring its importance in patient management. These findings advocate for early detection and comprehensive management of TR, integrating its assessment into routine PH care to potentially enhance patient outcomes.

Inappropriate Hospital Stay of Patients Admitted Under Care of General Medicine Units: A retrospective study.

Objectives: This study aimed to assess the incidence of inappropriate hospital stay and to identify the reasons behind inappropriate hospitalisation. Methods: This retrospective cohort study included patients admitted in the General Internal Medicine Unit, Sultan Qaboos University Hospital, Muscat, Oman, from January to June 2020. The average length of hospital stay for all included patients was calculated. The appropriateness evaluation protocol technique was used to examine admissions that exceeded the average length of hospital stay; subsequently, the reasons for the inappropriate hospital stay were identified. Results: There were 855 admissions during the study period. In this cohort, 53.1% were male and the median age was 64 years (interquartile range [IQR]: 44-75 years). There was a total of 6,785.4 hospitalisation days and the average length of hospital stay was five days (IQR: 3-9 days). A total of 31.8% of admissions (n = 272) and 9.9% of hospitalisation days (n = 674 days) were classified as inappropriate. Delay in complementary tests (29.0%) and unavailability of extra hospital resources (21.7%) were identified as the most common reasons associated with inappropriate hospital stays. Old age was associated with increase in inappropriate hospital stay. Conclusion: A significant proportion of hospitalisation days were inappropriate due to hospital-related factors. Therefore, auditing hospital services and investing in home-based care are among the top strategies that are likely to improve early discharge and minimise inappropriate hospital bed occupancy.

Myocarditis, Pulmonary Hemorrhage, and Extensive Myositis with Rhabdomyolysis 12 Days After First Dose of Pfizer-BioNTech BNT162b2 mRNA COVID-19 Vaccine: A Case Report.

BACKGROUND The COVID-19 pandemic is a current global crisis, and there are hundreds of millions of individuals being vaccinated worldwide. At present, there have been few reports of COVID-19 vaccine-induced autoimmune processes manifested as myositis, thrombocytopenia, and myocarditis. CASE REPORT A 37-year-old man presented to the Emergency Department (ED) with a 3-day history of back pain and a 1-day history of left upper limb swelling with paresthesia and shortness of breath, 12-days after receiving the first dose of Pfizer/BioNTech BNT162b2 mRNA COVID-19 vaccine. He was diagnosed with severe myositis complicated with rhabdomyolysis and non-oliguric acute kidney injury, thrombocytopenia, myocarditis with pulmonary edema, and pulmonary hemorrhage. Screens for potential toxic, infectious, paraneoplastic, and autoimmune disorders were unremarkable. The patient was treated with a 5-day course of intravenous methylprednisolone and intravenous immunoglobulin, with a good response. He was hospitalized for 16 days and discharged home on a tapering dose of oral prednisolone for 6 weeks. CONCLUSIONS The case describes a possible link between Pfizer/BioNTech BNT162b2 mRNA COVID-19 vaccine and immune-mediated myocarditis, pulmonary vasculitis, myositis, and thrombocytopenia. However, further data are required to confirm such an association.