PAEDIATRIC CARDIOLOGY & INTERVENTIONS

Her whole body encrusted in sarcoptes scabiei, a 50-year-old woman lay in foetal position on a hospital bed in Cape Town (Richards 2021). It was 2012 and I, a medical anthropologist, was accompanying a consultant on a clinical teaching ward round. She diagnosed her condition as Norwegian Scabies but the fissures in her flesh marked her HIV status. In preparation for our medical humanities tutorial, I asked the students to find out why her neighbours had not helped her?
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Objectives:We sought to evaluate the safety and efficacy of Amplatzer septal occluder (ASO) to close very large pulmonary arteriovenous fistula (PAVF).We sought to evaluate the safety and efficacy of Amplatzer septal occluder (ASO) to close very large pulmonary arteriovenous fistula (PAVF).Background:Transcatheter coil embolization, the standard treatment for PAVF, has potential complications particularly in patients with very large fistulas. Several recently available devices have been tried effectively, however, they too have limitations.Transcatheter coil embolization, the standard treatment for PAVF, has potential complications particularly in patients with very large fistulas. Several recently available devices have been tried effectively, however, they too have limitations.Methods:During 2004, three patients (aged 17–56 years), diagnosed with large PAVF including one with pulmonary artery (PA) to left atrial (LA) fistula, had interventional closure prospectively using ASO. Following hemodynamic evaluation and angiographic localization of PAVF, the feeding artery (FA) was selectively cannulated with Amplatzer delivery sheath. ASO, with the right atrial (RA) disc diameter equal to or up to 4 mm larger than the maximum diameter of FA, was delivered through it in such a way that the left atrial disc assumed an oblong shape and the right atrial disc retained its designed flat configuration for better anchorage and thrombogenicity.During 2004, three patients (aged 17–56 years), diagnosed with large PAVF including one with pulmonary artery (PA) to left atrial (LA) fistula, had interventional closure prospectively using ASO. Following hemodynamic evaluation and angiographic localization of PAVF, the feeding artery (FA) was selectively cannulated with Amplatzer delivery sheath. ASO, with the right atrial (RA) disc diameter equal to or up to 4 mm larger than the maximum diameter of FA, was delivered through it in such a way that the left atrial disc assumed an oblong shape and the right atrial disc retained its designed flat configuration for better anchorage and thrombogenicity.Results:All patients had very large PAVF fed by a single FA (size 12–24 mm), which was closed completely, without complications, using ASO (size 7–16 mm; RA disc diameter 15–26 mm). Their arterial saturation rose from mean 72.3% to 97.3%. Follow up (1.5–3 years) showed disappearance of the radiological shadows and stable arterial saturations (mean 97.7%).All patients had very large PAVF fed by a single FA (size 12–24 mm), which was closed completely, without complications, using ASO (size 7–16 mm; RA disc diameter 15–26 mm). Their arterial saturation rose from mean 72.3% to 97.3%. Follow up (1.5–3 years) showed disappearance of the radiological shadows and stable arterial saturations (mean 97.7%).Conclusions:We conclude that, using the new criteria for device size selection and modified technique of implantation, very large PAVFs including PA to LA fistula can be closed safely, effectively and nonsurgically with ASO. © 2007 Wiley-Liss, Inc.We conclude that, using the new criteria for device size selection and modified technique of implantation, very large PAVFs including PA to LA fistula can be closed safely, effectively and nonsurgically with ASO. © 2007 Wiley-Liss, Inc.

Pre-axial upper limb skeletal deformities involving thumb and radial bones are commonly associated with congenital cardiac defects. The common deformities are hypoplasia or aplasia of thumbs and radial bones. Skeletal involvement may be unilateral or bilateral. Atrial septal defect of ostium secundum is the most commonly associated congenital cardiac defect. Other possible cardiac defects are ostium primum variety of atrial septal defect, ventricular septal defects, patent foramen ovale and electric cardiac conduction abnormalities. The incidence is 1 in 1,00,000 live births. This syndrome involves TBX5 gene defect on long arm of chromosome 12. The most common inheritance pattern is autosomal dominant variety. New mutations are common. Antenatal detection by midtrimester anomaly scan is possible. Recently, we detected three cases of Holt Oram Syndrome at our health care facility during 2016-2019. These cases were presented with full spectrum of skeletal and cardiac defects in one case, near total expression in one case and partial expression of physical features in another case. Thumb abnormalities and cardiac defects involving atrial septal defect were found in all cases. All cases underwent second trimester anomaly scans but missed antenatal detection and lost potential antenatal counselling with parents.