Endovascular repair of severe aortic coarctation,transcatheter aortic valve replacement for severe aortic stenosis,and percutaneous coronary intervention in an elderly patient with long term follow-up

To the best of our knowledge, there have not been any reports of total transcatheter approach including stenting of severe coarctation of the aorta (CoA), transcatheter aortic valve replacement (TAVR) for concomitant severe aortic valve stenosis, and percutaneous coronary intervention (PCI) to treat significant coronary artery disease in a single patient. We report a 70-year-old female, who presented with uncontrolled hypertension and acute decompensated heart failure (ADHF) and was found to have severe CoA, severe bicuspid aortic valve (BAV) stenosis, and significant proximal left anterior descending (LAD) coronary artery disease. In a multidisciplinary heart team meeting, we decided to perform an endovascular repair of both cardiac and vascular pathologies using a two-stage approach due to the significant comorbidities; mainly uncontrolled hypertension, type 2 diabetes mellitus, chronic obstructive pulmonary disease, and severe calcifications of the ascending aorta. The procedures were successfully performed and the patient was asymptomatic 30?months later at follow-up and was without any significant gradients across the coarctation or the aortic valve.     

Cardiac involvement in nonketotic hyperglycinemia

Nonketotic hyperglycinemia is an autosomal recessive disorder of glycine metabolism characterized by the accumulation of glycine in the serum and cerebrospinal fluid with elevated cerebrospinal fluid to serum glycine ratio. The disease primarily affects the central nervous system, and has not been previously associated with myocardial involvement. In this article, the authors report an infant with nonketotic hyperglycinemia, who was found to have progressive left ventricular hypertrophy and dysfunction. His older sibling, who had a similar neurologic presentation, died of dilated cardiomyopathy as stated by the parents. The authors speculate that glycine may have a role in the development of cardiac dysfunction. The incidence of cardiac involvement may be under-diagnosed. They suggest the need for a cardiac evaluation in confirmed cases of nonketotic hyperglycinemia.     

Imaging of the uterovaginal anomalies

Objective

To assess the role of different imaging modalities including ultrasonography, hysterosalpingogram, and magnetic resonance imaging in detection of variable Müllerian anomalies. Preoperative proper diagnosis data about Müllerian anomalies necessary for clear indications of how and when to operate.

Patients and methods

A retrospective MRI study of 34 patients was done in the period from February 2008 to February 2010, their age ranging from 3 months to 38 years (mean 24 years), with uterovaginal anomalies. Ultrasonography was performed for all cases; HSG was performed in 16 cases before MRI imaging.

Results

MRI is the most reliable method for evaluating uterovaginal anomalies, particularly in pediatrics and virgins. MRI is an accurate examination for identification and categorization of MDAs and should be carried out prior to any surgery, in this study MRI allowed correct diagnosis of 34 uterine anomalies (accuracy 100%) whereas US was correct in 30 out of 34 cases (accuracy 88%). HSG had a limited role as cannot be preformed for virgins, and cannot identify non-communicating horns in unicornuate cases.

Conclusion

MRI is the examination of choice in uterovaginal anomalies. Endovaginal ultrasound cannot be preformed for children or females who have never had sexual intercourse. TAUS have not proved completely reliable in Müllerian duct anomalies.     

Echocardiographic surveillance for asymptomatic late-onset anthracycline cardiomyopathy in childhood cancer survivors

Background

The optimal frequency of echocardiographic surveillance in asymptomatic childhood cancer survivors exposed to anthracyclines has not been established. We evaluated the effectiveness of performing surveillance echocardiograms according to the Children's Oncology Group's (COG) Long‐Term Follow‐Up Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancers in survivors ≥1 year from concluding therapy.

Methods

We reviewed all children treated at our institution with anthracycline chemotherapy from 1995 to 2003. We assessed the frequency of abnormal echocardiograms according to risk groups defined in the COG guidelines, and evaluated the risk factors for an abnormal echocardiogram using Cox proportional hazards modeling.

Results

At least one echocardiogram was completed by 469/603 (77.8%) eligible survivors. Mean diagnosis age was 7.7 (SD = 4.6) years. Mean cumulative doxorubicin‐equivalent dose was 205 mg/m² (SD = 115). Survivors completed 1,013 echocardiograms (median = 2, range = 1–10) beyond 1 year after concluding therapy. Seventy‐nine (16.8%) survivors had an abnormal echocardiogram at a median of 2.9 years (range 0.01–9.8) from 1 year after concluding therapy. Anthracycline dose >300 mg/m² (hazard ratio [HR] 3.00; 95% CI 1.51–5.98), age 1–4 years at treatment (HR 1.89; 95% CI 1.08–3.31) and radiation to a field involving the heart (HR 1.73; 95% CI 1.08–2.76) predicted an increased risk of an abnormal echocardiogram; however, even survivors in the lower COG risk groups demonstrated abnormalities.

Conclusion

Periodic echocardiographic surveillance in childhood cancer survivors can yield abnormalities that require further evaluation. Abnormalities may become evident as early as 1 year after the conclusion of therapy and can impact even those survivors considered to be at low risk. Pediatr Blood Cancer 2011; 57: 467–472. © 2011 Wiley‐Liss, Inc.     

Preoperative intravenous dexamethasone combined with glossopharyngeal nerve block: role in pediatric postoperative analgesia following tonsillectomy

Tonsillectomy is one of the most frequently performed ambulatory surgical procedures in children (Litman et al. in Anesth Analg 78:478–481, 1994). Several techniques have been described for alleviation of pain (Ginstrom et al. in Acta Otolaryngol 125:972–975, 2005). the objective of this study determination of the postoperative analgesic efficacy of the pre-surgical intravenous administration of dexamethasone together with glossopharyngeal nerve block (GNB) in children undergoing tonsillectomy. Prospective double blind randomized control study using both pre-operative injection of 0.5 mg/kg dexamethasone iv and 3 ml of 0.5% bupivacaine local injection for bilateral glossopharyngeal nerve block. Patients in group B had significantly less visual analogue scale values, longer absolute analgesia time, lesser swallowing difficulty and they were discharged earlier from the hospital when compared to patients in both groups D and G. Using both pre-operative dexamethasone IV injection with GNB has reduced postoperative pain and morbidity to a great extent than using either alone.     

Intraoperative blood transfusion in pediatric patients undergoing renal transplant—Effect of renal graft size

In pediatric RT, donor allograft size often exceeds the expected recipient norms, especially in younger recipients. An “oversize” graft might not only present a technical‐ and space‐related challenge, but may possibly lead to increased demands in perioperative volume requirements due to the disparity between donor and recipient in renal blood flow. We evaluated transfusion practices at a single tertiary institution with special consideration of kidney graft size, hypothesizing that oversize graft kidneys might lead to a quantifiable increased need of blood transfusion in smaller recipients. Retrospective analysis of all patients who underwent pediatric RT from January 2004 to June 2014 at a tertiary pediatric centre was performed. Variables analyzed included patient age, weight, pre‐ and postoperative Hb concentration, graft size, EBL, amount of intraoperative blood transfusion, and preoperative use of erythropoietin. Based on graft size in relation to patient's age, a SMR and an OvR were identified. A subcohort of age‐matched pairs was used to allow for comparison between groups. We calculated the expected procedure‐ and transfusion‐induced changes in Hb and compared these changes to the observed difference in pre‐ vs postoperative Hb to assess the influence of graft size on transfusion requirements. RT was performed in 188 pediatric recipients during the study period. In the matched cohort, percentage of transfused patients during transplantation in the OvR group was more than double compared with SMR (89% vs 39%, P < .001); similarly, the median number of transfused PRBC units in OvR was 1, while the median of SMR did not receive transfusion (P < .001). The difference between expected (calculated) and observed change in Hb was significantly higher in OvR with a median of 1.9 g/dL compared with SMR with a median of 1.0 g/dL (P = .026). Correspondingly, the calculated median volume taken up by a regular size kidney was significantly higher with 213 mL compared with 313 mL (P = .031) taken up by an oversize graft kidney. Median estimated intraoperative blood loss was significantly higher in OvR than in SMR (6.9 mL/kg, vs 5.3 mL/kg, respectively; P = .04). Median postoperative Hb was similar among groups (10.4 g/dL vs 10.6 g/dL for SMR vs OvR, respectively). Transplantation of an oversized kidney in pediatric RT recipients is associated with a quantifiable higher need for blood transfusion. This may be caused by a higher intraoperative EBL and/or greater blood volume sequestered by the larger renal allograft and requires further evaluation.