Left Main coronary angioplasty of a 9-year-old child with bioresorbable vascular scaffold [corrected].

Familial hypercholesterolemia is an autosomal dominant disorder due to mutations in the low-density lipoprotein receptor gene, characterized by skin and tendon xanthomas, xanthelasmas, and increased risk of premature coronary artery disease. Here, we report a case of 9-year-old girl who presented with angina and dyspnoea on exertion with xanthomas and an elevated serum cholesterol and triglyceride. She had severe stenosis of the left main coronary artery (LMCA) requiring angioplasty and placement of Bioresorbable vascular scaffold (BVS). This is the first case report in literature of the use of BVS for LMCA stenosis in a 9-year-old child. © 2017 Wiley Periodicals, Inc.

Clinical Application and Multidisciplinary Assessment of Three Dimensional Printing in Double Outlet Right Ventricle With Remote Ventricular Septal Defect.

BACKGROUND: Double outlet right ventricle (DORV) with two well-developed ventricles and with a remote ventricular septal defect (VSD) may present a therapeutic challenge. Echocardiographic imaging of such complex cases does not always provide all of the information required to decide on an operative approach (biventricular or univentricular) and to design an intracardiac baffle to direct left ventricular outflow through the VSD and to the aorta for biventricular repair. A three dimensional (3D) printed model of the heart based upon data derived from computed tomography (CT) or magnetic resonance imaging (MRI) may contribute to a more complete appreciation of the intracardiac anatomy. METHODS: From April to September 2015, six consecutive patients with DORV and remote VSD underwent CT/MRI scans. Data sets from these studies were used to generate life-size 3D models using a 3D printer. We compared the assessment of 3D printed heart model findings with information obtained from echocardiography, CT, or cardiac MRI and with details of the surgeon's intraoperative direct observations when available. Quantification of the information provided by the 3D model was achieved using a unique scale that was created for the purpose of this study. The accuracy and utility of information derived preoperatively from the models were assessed. RESULTS: Six data sets from six patients were analyzed. Five data sets could be successfully used to create sandstone models using 3D printing. The five patients ranged from 7 months to 11 years of age and weighed 6.7 to 26 kg. The spatial orientation of the heart in the thorax, the relationships of the great arteries and the semilunar valves, the size and location of the VSD were well appreciated in all models, as were the anticipated dimensions and orientation of a surgically planned interventricular baffle. Three of the five patients underwent successful biventricular repair. CONCLUSION: The 3D printed models scored higher than conventional imaging, with respect to most aspects of the surface spatial orientation and intracardiac anatomy. The models are a useful adjunct in preoperative assessment of complex DORV. The unique scale helps quantify the advantages and limitations of the 3D heart models.

Unusual cardiac associations with Tetralogy of Fallot-a descriptive study.

We present a large single-center series (>2200 cases) operated for Tetralogy of Fallot (TOF). We analyzed the incidence of associated unusual and uncommonly described cardiac lesions and their diagnostic and therapeutic implications in TOF patients. This retrospective study was conducted by reviewing records of patients operated for TOF at a large tertiary care pediatric cardiac centre. From 2002 to 2008, a total of 2235 cases of TOF were evaluated with echocardiography, cardiac catheterization, and/or cardiac computed tomography followed by cardiac surgery. Known and well-described associations were excluded from the study. Unusual associations were tabulated. Several unusual associations having an incidence >0.1% were detected. These included subaortic membrane (1%), pulmonary venous abnormalities (0.5%), small left ventricle (0.5%), interrupted inferior vena cava (0.5%), mitral valve abnormalities (0.4%), hemitruncus (0.4%), tricuspid valve abnormalities (0.4%), biventricular dysfunction (0.3%), retroaortic innominate vein (0.3%), bicuspid aortic valve (0.2%), and pericardial effusion (0.2%). This series describes unusual, not previously routinely reported cardiac lesions associated with TOF that may affect management and should be sought on preoperative evaluation. We demonstrate that more unusual associations do exist in not infrequent numbers, i.e., a TET is not just a TET.

Supracardiac anomalous pulmonary venous connection with unilateral pulmonary venous atresia: Diagnosis and management.

We report a case of a 6-day-old neonate referred to us for surgical correction of total anomalous pulmonary venous connection. Meticulous evaluation contributed to accurate diagnosis of associated unilateral pulmonary venous atresia. This unique association provides insights into the importance of evaluation of all pulmonary veins using various imaging tools.

Williams syndrome and Ebstein's anomaly: A rare association.

We report a rare case of Williams syndrome associated with Ebstein's anomaly of the tricuspid valve. To our knowledge, such an association has never been reported.

Vein of galen arteriovenous malformation: diagnostic clues from echocardiography.

We report a rare association of vein of Galen malformation in a young infant with associated sinus venosus atrial septal defect and aortic arch hypoplasia. This unique association of lesions provides insights into the embryological basis as well as offers potential echocardiographic clues for the diagnosis of the cerebral arterio-venous malformation.