Staged approach for the management of atrial septal defect in the presence of a small left ventricle and suprasystemic pulmonary pressure

A 29-year-old woman presented with a symptomatic large atrial septal defect, a small left ventricle with a modest left atrium, mild to moderate nonrheumatic mitral valve incompetence, an apex-forming very large right ventricle and suprasystemic pulmonary artery pressure. Following one year of preprocedural drug treatment to lower pulmonary hypertension, she underwent percutaneous closure of the atrial septal defect with a customized fenestrated device that enabled gradual adaptation to the occlusion of the interatrial communication. This special case demonstrates the benefits of using a fenestrated occluder device in patients with pulmonary hypertension and components of left ventricular diastolic dysfunction, which is considered to be high risk and not amenable to therapeutic intervention. is a safe and effective alternative to surgery (1). Complete occlusion of the defect is generally the desired procedural outcome. However, there are clinical and morphological circumstances in which acute complete occlusion may worsen hemodynamics because this atrial communication serves as a 'pop-off' valve (2). Such situations have been previously described in the literature, and include children and adults with pulmonary arterial hypertension as well as closure of an ASD in elderly patients (3). In these settings, the use of a fenestrated occluder device was demonstrated to be beneficial (3). We present a case involving a young woman with an interesting cardiac morphology of a small left ventricle and suprasystemic pulmonary artery pressure. A staged approach, comprised of medical therapy followed by fenestrated device implantation, enabled gradual occlusion of the interatrial communication, leading to clinical and hemodynamic improvement. CASe pReSenTATion A previously healthy 29-year-old woman, who was diagnosed with an ASD when she was 14 years of age, became breathless on minor physical activity during pregnancy and remained decapacitated six months after spontaneous vaginal delivery of a healthy baby. She was referred to the authors as a tourist after several well-known cardiac and cardiothoracic services abroad elected not to intervene because of her unfavourable condition. On echocardiography, her heart morphology revealed a very small left ventricle (LV) -left ventricular end diastolic diameter of only 26 mm ( With regard to the possible etiology of increased PAp, there were no clinical or laboratory features of pulmonary embolism or a systemic illness such as Sjögren syndrome or lupus erythematosus, and the thrombophilia workup was negative. The patient was started on a combined drug therapy of bosentan 125 mg twice/day, sildenanfil 20 mg three times/day, bisoprolol 2.5 mg/day and furosemide 20 mg/day. The addition of bosentan aimed to decrease pulmonary resistance, thus decreasing the right-to-left shunt caused by the tricuspid valve jet through the ASD. Marked clinical and symptomatic improvements were documented soon thereafter and she returned home. Being a tourist, the patient could return for a follow-up visit only one year after the initial evaluation. Following one year of treatment, her O 2 saturation on room air increased from 88% to 90% to 98%, and her subjective limited walking capability improved. Doppler interrogation of tricuspid valve regurgitation revealed a pressure gradient of 60 mmHg. After informed consent was obtained, a hemodynamic study was performed in the catheterization laboratory. The measured PAp was 78/28 mmHg (mean 44 mmHg); the pulmonary capillary wedge pressure was 12/7 mmHg (mean 10 mmHg); and the Qp:Qs ratio was 3:1. The interatrial septum measured 50 mm. The ASD stretched diameter was 24 mm, with a small inferior fenestration revealed by transesophageal echocardiography. Although the LV was small, its systolic function was good. There was a large, apex-forming RV with good function. A sizing balloon was inflated through the ASD, resulting in an increase in mean pulmonary capillary wedge pressure from 10 mmHg to 12 mmHg

Perivesical abscesses caused by Staphylococcus aureus in two children

AbstractWe present two children with abscesses located adjacent to the urinary bladder without a demonstration of intestinal pathology. The abscesses were caused by Staphylococcus aureus and were successfully treated with computerized tomography-guided drainage and antimicrobials. We would like to stress that not every abdominal abscess is secondary to bowel disease or perforation. Therefore, the organisms cultured may differ from the classic mixed gut flora. Hence, especially if there is no evidence of intestinal disease and the location is not typical for intestinal pathology (e.g., perivesical), S. aureus must be considered a potential etiologic factor

Expert panel diagnosis demonstrated high reproducibility as reference standard in infectious diseases

Objective: If a gold standard is lacking in a diagnostic test accuracy study, expert diagnosis is frequently used as reference standard. However, interobserver and intraobserver agreements are imperfect. The aim of this study was to quantify the reproducibility of a panel diagnosis for pediatric infectious diseases. Study Design and Setting: Pediatricians from six countries adjudicated a diagnosis (i.e., bacterial infection, viral infection, or indeterminate)for febrile children. Diagnosis was reached when the majority of panel members came to the same diagnosis, leaving others inconclusive. We evaluated intraobserver and intrapanel agreement with 6 weeks and 3 years’ time intervals. We calculated the proportion of inconclusive diagnosis for a three-, five-, and seven-expert panel. Results: For both time intervals (i.e., 6 weeks and 3 years), intrapanel agreement was higher (kappa 0.88, 95%CI: 0.81-0.94 and 0.80, 95%CI: NA)compared to intraobserver agreement (kappa 0.77, 95%CI: 0.71-0.83 and 0.65, 95%CI: 0.52-0.78). After expanding the three-expert panel to five or seven experts, the proportion of inconclusive diagnoses (11%)remained the same. Conclusion: A panel consisting of three experts provides more reproducible diagnoses than an individual expert in children with lower respiratory tract infection or fever without source. Increasing the size of a panel beyond three experts has no major advantage for diagnosis reproducibility