Mixed connective tissue disease (MCTD) was initially described as a chronic immune-mediated disease with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis. We conducted a cross-sectional study to describe the clinical and immunological profile of patients with MCTD and to compare the four diagnostic criteria, namely, Sharp, Kasukawa, Alarcón-Segovia, and Khan criteria. A total of 291 patients who were admitted from June 2007 to June 2017 and fulfilled the inclusion criteria were included in the study. A clinical diagnosis of MCTD was made in 111 patients, of whom 103 (92.8%) were women. The mean age at presentation was 39.3 years (). The most common organ systems that were involved were musculoskeletal system (95.5%), skin and mucosa (78.4%), and the gastrointestinal and hepatobiliary systems (56%). The maximum sensitivity was for the Kasukawa criteria with a sensitivity of 77.5% (95% CI 68.4-84.6) and specificity of 92.2% (95% CI 87-95.5). The Kahn criteria and Alarcón-Segovia criteria had the maximum specificity; the Alarcón-Segovia criteria had a sensitivity of 69.4% (95% CI 59.8-77.6) and a specificity of 99.4% (95% CI 96.5-99.9), while the Kahn criteria had a sensitivity of 52.3% (95% CI 42.6-61.7) and a specificity of 99.4% (95% CI 96.5-99.9). The sensitivity and specificity of Sharp criteria were 57.7% (95% CI 47.9-66.87) and 90% (95% CI 84.4-93.8), respectively.
18 June 2024 in Journal of the American Heart Association
Background: Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5% to 10% of all atrial septal defects. Although surgical closure is the standard treatment for SVASD, data on outcomes have been confined to small cohorts. Thus, we conducted a systematic review of the outcomes of SVASD repair. Methods and Results: The primary outcome was death. Secondary outcomes encompassed atrial fibrillation, sinus node dysfunction, pacemaker insertion, cerebrovascular accident, reoperation, residual septal defect, superior vena cava obstruction, and reimplanted pulmonary vein obstruction. Pooled incidences of outcomes were calculated using a random‐effects model. Forty studies involving 1320 patients who underwent SVASD repair were included. The majority were male patients (55.4%), with 88.0% presenting with associated anomalous pulmonary venous connection. The weighted mean age was 18.6±12.5 years, and the overall weighted mean follow‐up period was 8.6±10.4 years. The in‐hospital mortality rate was 0.24%, with a 30‐day mortality rate of 0.5% reported in 780 patients. Incidences of atrial fibrillation, sinus node dysfunction, pacemaker insertion, and cerebrovascular accident over the long‐term follow‐up were 3.3% (2.18%–4.93%), 6.5% (5.09%–8.2%), 2.23% (1.34%–3.57%), and 2.03% (0.89%–2.46%) respectively. Reoperation occurred in 1.36% (0.68%–2.42%) of surgeries, residual septal defect in 1.34% (0.69%–2.42%), superior vena cava obstruction in 1.76% (1.02%–2.9%), and reimplanted pulmonary vein obstruction in 1.4% (0.7%–2.49%). Conclusions: This is the first comprehensive analysis of outcomes following surgical repair of SVASD. The findings affirm the safety and effectiveness of surgery, establishing a reference point for evaluating emerging transcatheter therapies. Safety and efficacy profiles comparable to surgical repair are essential for widespread adoption of transcatheter treatments.
27 June 2024 in The Journal of Heart and Lung Transplantation
Although the most frequent presentation of the novel Coronavirus disease-2019 (COVID-19) is a respiratory syndrome, cardiac involvement is being increasingly recognized. One such entity is takotsubo cardiomyopathy. We sought to review the various cases of takotsubo cardiomyopathy reported during the COVID-19 pandemic and consolidate the information available on its clinical features, evaluation and treatment. We performed a PubMed search using the MeSH terms “Takotsubo Cardiomyopathy” or “Stress Cardiomyopathy” and “COVID-19”, and identified 16 case reports, two case series, and one retrospective cohort study. There was a total of 24 reported patients with COVID-19 infection, who developed takotsubo cardiomyopathy, and two patients without COVID-19 who developed takotsubo cardiomyopathy due to the emotional stress associated with the global pandemic. The mean age of the patients was 67.19 years (SD 15.83) and 16(59.3%) were women. Chest pain was reported in only ten patients (38.46 %) and ST-elevation was seen in 11 patients (42.3%). While most patients had typical takotsubo cardiomyopathy, four patients had inverted(reverse) takotsubo cardiomyopathy, two had bi-ventricular involvement, one had median takotsubo and another had global takotsubo with apical sparing variant. Most patients had a positive outcome with complete or near-complete reversal of cardiac dysfunction at the time of discharge. Five deaths (19.23%) were reported. Takotsubo cardiomyopathy is a rare, but increasingly reported reversible cardiomyopathy that can be seen in patients with COVID-19 infection and the diagnosis must be actively sought for in these patients.
We conducted a retrospective observational study to describe the clinical profile and outcomes of patients admitted with a diagnosis of dengue fever in a tertiary hospital in South India. A total of 159 patients admitted from April 2014 to October 2018 were included in the study. Vomiting (70.4%), myalgia (60.4%), headache (42.1%), abdominal pain (38.4%), bleeding (38%), and rash (37.1%) were the most common symptoms at presentation. The mean duration of hospital stay was 4.9 days (SD ± 2.4), and the median cost was INR 19,708 ($285) (IQR INR 12,968–32,056 ($188–$305)). Major bleeding was associated with elevated SGOT and SGPT, severe dengue, and secondary dengue. Mortality was associated with elderly age; elevated total leukocyte count, serum bilirubin, serum creatinine, SGOT, and SGPT; and high SOFA score. In view of these observations, we recommend stratifying patients according to the WHO classification of dengue and avoiding the use of thrombocytopenia as a single marker of the severity of the illness.