Search

Vein of Galen malformation (VGM) is a rare intracranial vascular malformation. High output heart failure is common in infancy and is characterized by dilatation of all the cardiac chambers. We report an unusual case of VGM in a 3-month-old infant without dilatation of the right-sided cardiac chambers. We then demonstrate importance of comprehensive evaluation in detecting rare coexistence of anomalous right superior caval vein connection to the left atrium.

Treatment options include off-label use of Sirolimus, pericardiocentesis, open fetal resection or postnatal surgery. After delivery at 36 weeks, titrated oral Sirolimus therapy was started from day 2 in view of LV inflow obstruction. Serial echocardiography revealed increase in size, appearance of smaller masses and large pericardial effusion (PE) for which percutaneous pericardiocentesis was done at 26 weeks. [Extracted from the article] Copyright of Ultrasound in Obstetrics & Gynecology is the property of John Wiley & Sons, Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

Objectives To review the success and technical aspects of pulmonary valve (PV) perforation using chronic total occlusion (CTO) hardware in patients with pulmonary atresia and intact ventricular septum (PA-IVS). Background Interventional therapy is possible in selected patients with PA-IVS. Among the various interventional options available, radiofrequency and laser assisted perforation may be more successful, but require expertise and may be substantially costly. Methods We describe the technique of mechanical catheter PV perforation using currently available coronary hardware meant for coronary CTO in nine cases with PA-IVS. After complete echocardiographic evaluation and informed parental consent was obtained, patients were electively intubated, mechanically ventilated, adequately heparinized and were placed on intravenous prostaglandin infusion. Basic steps involved were—localizing the atretic segment and accomplishing coaxial alignment of catheters using biplane fluoroscopy, crossing the atretic segment with the soft end of perforating guidewire, stabilizing the assembly and performing graded balloon dilatation with the balloon size never exceeding 130% of pulmonary annulus diameter. For crossing the atretic PV, a retrograde approach was used in one patient where the antegrade approach was not possible. Results The procedure was successful in 8/9 cases (89%). Valve opening was achieved in all eight patients with immediate fall in right ventricular (RV) systolic pressures. One neonate died following surgery after catheter induced RV perforation. All surviving cases were discharged from the hospital in good general condition with no evidence of heart failure and a room air oxygen saturation of >85%. No patient required an additional pulmonary irrigation procedure. Conclusion With appropriate patient and hardware selection, PV perforation using readily available coronary hardware is feasible in PA-IVS. © 2014 Wiley Periodicals, Inc.

Background and Aim: Follow-up studies on growth and histological recovery of children with celiac disease (CD) while on a gluten-free diet (GFD) are lacking from Asia. We therefore assessed the effects of this diet. Methods: Forty-two children with CD were enrolled. Weight and height were expressed as weight for height (WfH) and height standard deviation scores (HSDS), respectively. Twenty-five children had repeated duodenal biopsies after 1-2 years and 14 had a third biopsy after 3-7 years of GFD. Compliance was checked by regular interview and IgA antiendomysial antibody estimation (EMA). Results: At diagnosis (n = 25), mean HSDS was -3.3 ± 1.6 with 76% having a HSDS of

Arterial thrombosis is the most frequent major complication of percutaneous arterial catheterization in children. We prospectively studied the effect of randomized dosage of heparin, 50 IU/kg-group I and 100 IU/kg-group II, on the incidence of arterial thrombosis in 366 children and analysed the various factors which may influence the occurrence of this complication. The age of patients ranged from 17 d to 11 yr (mean age 39.5 +/- 40.9 mo) and mean weight was 11.2 +/- 7.8 kg (range 3 to 39 kg). The incidence of arterial thrombosis was 9.8% in group I and 9.3% in group II (P = NS). There was no statistical difference in precatheterization and procedure variables in the two groups and also in the group with absent pulse (n = 35) to the group with pulse present post cath (n = 331). There were 24.9% infants in our study and 14.3% of these had arterial thrombosis. The loss of pulse was more often seen with more number of attempts at arterial puncture (P < 0.001), absence of back bleed at the end of the procedure (P < 0.001), and increased duration of catheterization (P < 0.01). Use of larger sheath size in a given weight and body surface area of children increased incidence of arterial thrombosis. The administration of heparin 50 IU/kg was equally efficacious to heparin 100 IU/kg. Of the patients with arterial thrombosis, 23 responded with intravenous heparin and 12 needed streptokinase. There was no bleeding or haematoma. Thus our study shows that less attempt for arterial puncture, use of smaller sheath size, maintaining shortest procedure time and ensuring back bleed minimises incidence of arterial thrombosis post catheterization.

We describe an 11-year-old girl with tetralogy of Fallot and absent pulmonary valve, who on selective coronary angiography was found to have extrinsic compression of the left main coronary artery by the aneurysmally dilated pulmonary artery. This abnormality has not been reported previously. Cathet. Cardiovasc. Intervent. 46:438–440, 1999. © 1999 Wiley-Liss, Inc.

Thirty-five PVC formulations were compounded initially from which selected nontoxic formulations were subjected to studies in changes of mechanical, optical, migratory, viscosity, and toxicity properties due to gamma irradiation to 2.5 M rads. Degree of discoloration was found to vary from negligible to intense discoloration after irradiation on the selected samples. The nontoxic character of all samples was retained after irradiation. One formulation (R2) showed excellent radiation resistance and minimum discoloration. Effects of various additives on PVC radiation stability is also discussed.