Prevalence of cardiac valvar abnormalities in children and young people with autosomal dominant polycystic kidney disease.

BACKGROUND: Valvar abnormalities in children and adults with autosomal dominant polycystic kidney disease (ADPKD) have previously been reported as a frequent occurrence. Mitral valve prolapse (MVP), in particular, has been reported in almost one-third of adult patients and nearly 12% of children with ADPKD. Our objective in this study was to establish the prevalence of valvar abnormalities in a large, contemporary series of children and young people (CYP) with ADPKD. METHODS: A retrospective, single centre, cross-sectional analysis of the echocardiograms performed on all consecutive children seen in a dedicated paediatric ADPKD clinic. Full anatomical and functional echocardiograms were performed and analysed for valvar abnormalities. RESULTS: The echocardiograms of 102 CYP with ADPKD (range 0.25-18 years, mean age 10.3 years, SD ± 5.3 years) were analysed. One (0.98%), 3-year-old boy, had MVP. There was no associated mitral regurgitation. Evaluating variations in normal valvar anatomy, 9 (8.8%) patients, aged 7.1 to 18 years, had minor bowing ± visual elongation of either the anterior or posterior leaflet of the mitral valve, none of which fell within the criteria of true MVP. Three (1.9%) patients, 2 boys and 1 girl aged between 7 and 14 years, had trivial or mild aortic regurgitation. No patients had echocardiographic evidence of tricuspid valve prolapse (TVP). CONCLUSION: In this contemporary cohort of CYP with ADPKD, the incidence of MVP and other valvar lesions is significantly lower than previously reported. A higher resolution version of the Graphical abstract is available as Supplementary information.

Multimodality cardiac evaluation in children and young adults with multisystem inflammation associated with COVID-19.

AIMS: Following the peak of the UK COVID-19 epidemic, a new multisystem inflammatory condition with significant cardiovascular effects emerged in young people. We utilized multimodality imaging to provide a detailed sequential description of the cardiac involvement. METHODS AND RESULTS: Twenty consecutive patients (mean age 10.6 ± 3.8 years) presenting to our institution underwent serial echocardiographic evaluation on admission (median day 5 of illness), the day coinciding with worst cardiac function (median day 7), and the day of discharge (median day 15). We performed cardiac computed tomography (CT) to assess coronary anatomy (median day 15) and cardiac magnetic resonance imaging (CMR) to assess dysfunction (median day 20). On admission, almost all patients displayed abnormal strain and tissue Doppler indices. Three-dimensional (3D) echocardiographic ejection fraction (EF) was <55% in half of the patients. Valvular regurgitation (75%) and small pericardial effusions (10%) were detected. Serial echocardiography demonstrated that the mean 3D EF deteriorated (54.7 ± 8.3% vs. 46.4 ± 8.6%, P = 0.017) before improving at discharge (P = 0.008). Left main coronary artery (LMCA) dimensions were significantly larger at discharge than at admission (Z score -0.11 ± 0.87 vs. 0.78 ± 1.23, P = 0.007). CT showed uniform coronary artery dilatation commonly affecting the LMCA (9/12). CMR detected abnormal strain in all patients with global dysfunction (EF <55%) in 35%, myocardial oedema in 50%, and subendocardial infarct in 5% (1/20) patients. CONCLUSIONS: Pancarditis with cardiac dysfunction is common and associated with myocardial oedema. Patients require close monitoring due to coronary artery dilatation and the risk of thrombotic myocardial infarction.

Echocardiographic Versus Angiographic Assessment of Patent Arterial Duct in Percutaneous Closure: Towards X-ray Free Duct Occlusion?

Device selection and procedural guidance for percutaneous ductal closure strongly rely upon angiographic and echocardiographic imaging. Current literature recognises 2D echocardiography as an essential tool for diagnosis and assessment but does not define a consistent methodology to optimise ductal measurement. There is little research comparing echocardiography with gold standard angiography for ductal measurement. Proving 2D echocardiographic ductal measurement to be equivalent to angiography could pave the way for its use as the primary modality in image guidance for percutaneous closure of the ductus. This was a retrospective study of 100 consecutive paediatric patients who underwent percutaneous ductal closure. Echocardiographic images were studied to determine ductal (a) morphology (b) dimensions (length, aortic ampulla, pulmonary end, minimum diameter) (c) size of device that would be appropriate for closure. These data were compared to corresponding measurements generated by angiographic images. Inter and intra-observer ratings were calculated to assess levels of agreement. There were significant differences between the imaging methods in classifying the morphological sub-type and ductal measurements (p < 0.005), except for length which was not found to be significantly different between modalities. Prediction of device selection from angiographic images showed excellent agreement (weighted k = 0.81). Predictions based on echocardiographic images showed a poor level of agreement (weighted k = 0.14). We found poor correlation between echocardiography and angiography for measurement, morphological assessment and device selection. Based on our findings, percutaneous arterial duct occlusion without angiographic guidance in this age group cannot be advocated.

A Novel Fusarium Species Causes a Canker Disease of the Critically Endangered Conifer, Torreya taxifolia.

A canker disease of Florida torreya (Torreya taxifolia) has been implicated in the decline of this critically endangered species in its native range of northern Florida and southeastern Georgia. In surveys of eight Florida torreya sites, cankers were present on all dead trees and 71 to 100% of living trees, suggesting that a fungal pathogen might be the causal agent. To identify the causal agent, nuclear ribosomal internal transcribed spacer region (ITS rDNA) sequences were determined for 115 fungi isolated from cankers on 46 symptomatic trees sampled at three sites in northern Florida. BLASTn searches of the GenBank nucleotide database, using the ITS rDNA sequences as the query, indicated that a novel Fusarium species designated Fsp-1 might be the etiological agent. Molecular phylogenetic analyses of partial translation elongation factor 1-alpha (EF-1) and RNA polymerase second largest subunit (RPB2) gene sequences indicate that Fsp-1 represents a novel species representing one of the earliest divergences within the Gibberella clade of Fusarium. Results of pathogenicity experiments established that the four isolates of Fsp-1 tested could induce canker symptoms on cultivated Florida torreya in a growth chamber. Koch's postulates were completed by the recovery and identification of Fsp-1 from cankers of the inoculated plants.

Laparoscopic Myomectomy.

Laparoscopic myomectomy (LM) is an increasingly accepted method of treatment for symptomatic uterine leiomyomas. It affords patients a minimally invasive surgery compared to the more traditional abdominal approach. Studies have shown the benefits of decreased blood loss, shorter hospital stay and recovery period, as well as decreased postoperative pain and fever in comparison to abdominal myomectomy (AM). Even myomas larger than 5 cm can be laparoscopically resected safely in the hands of an experienced surgeon. To date, studies indicate a decreased adhesion rate with the laparoscopic approach compared to the abdominal procedure. The use of adhesion barriers may decrease this rate further. In infertile women with myomas, pregnancy and live-birth rates improve following LM, and appear to be comparable with those rates achieved following AM. The data regarding the risk of uterine rupture in pregnancy following LM is limited. However, small studies show safe outcomes when patients are managed with the same degree of caution one might employ with post-abdominal myomectomy patients. In performing LM, a precise and diligent technique should be exercised to ensure a secure, multilayer closure.

Effects of fibrin sealant on single-layer uterine incision closure in the New Zealand white rabbit.

OBJECTIVE: To determine if the addition of fibrin sealant to incision closure in a rabbit uterine horn myomectomy model affects adhesion formation or strength of incision closure. DESIGN: Prospective randomized controlled trial. SETTING: Academic research center. ANIMAL(S): New Zealand white female rabbits. INTERVENTION(S): A pilot study revealed that the time interval for maximal uterine incision healing was eight weeks. Thirty New Zealand white rabbits underwent a 1-cm standardized myotomy incision on both uterine horns. A single interrupted stitch of 3-0 polygalactin suture was placed to reapproximate each incision. Fibrin sealant was then applied to one of each rabbit's horns (randomized). After eight weeks, the rabbits were killed, and the strength of myotomy closure was determined by measurement of maximal burst pressure for each uterine horn. Adhesion presence was recorded. MAIN OUTCOME MEASURE(S): Uterine burst pressure, adhesion presence. RESULT(S): The mean burst pressure was 267.8 (+/-75.8) mm Hg in the suture only group and 247.8 (+/-92.3) mm Hg in the suture and fibrin sealant group. There was no statistical difference in the presence of adhesions. CONCLUSION(S): Fibrin sealant did not strengthen myotomy repair nor did it reduce postoperative adhesion formation. There is no apparent advantage to fibrin sealant in this myomectomy model.