Name : Abbas
Family :Arjmand Shabestari
Affiliation : Radiology Department, Modarres Hospital, SBMU
Academic Degree: Associate Professor of Radiology
Resident : Ramin Pourghorban
Gender : Female
Age : Neonate
Neonate with congenital heart disease
The RV and RA are enlarged while the LV and LA are remarkably reduced in size and their appearance implies hypoplastic left heart syndrome. Patent ductus arteriosus (PDA) and a relatively large ASD are observed; nevertheless, no VSD could be found. The PDA measures 1mm, in caliber. The ascending aorta is smaller than normal. There is a post-ductal coarctation of aorta just beyond the PDA, which has led to its caliber reduction. In addition, there is double SVC; the right one enters the RA and the left one enters the dilated coronary sinus. The main pulmonary artery is remarkably enlarged; whereas, the pulmonary veins have normally entered the small LA and their number is normal. The innominate, left common carotid and left subclavian arteries have ordinarily arisen from aortic arch.
Discussion (Related Text)
Hypoplastic left heart syndrome (HLHS) is a complex combination of cardiac malformations that probably results from multiple developmental errors in the early stages of cardiogenesis. In HLHS, pulmonary venous blood enters the left atrium, but atrial systole cannot push blood across the atretic or stenotic mitral valve into the hypoplastic left ventricle. Consequently, left atrial blood is shunted across an interatrial communication. The interatrial defect can range from a highly restrictive pinhole-sized foramen ovale to a wide, nonrestrictive atrial septal defect. Associated cardiac malformations include pre- and postductal coarctation of the aorta, patent ductus arteriosus, patent foramen ovale, dilated pulmonary artery, ventricular septal defect, dilatation of the right atrium, enlarged right ventricle, and various forms of double-outlet right ventricle. Fibrotic thickening of the endocardium (endocardial fibroelastosis) can occur in either side of the heart and contribute to poor cardiac function. Additional complicating factors include right ventricular failure and tricuspid valve or common atrioventricular valve regurgitation. Anomalous systemic venous return can occur in congenital heart disease (CHD). Left superior vena cava (SVC) occurs more commonly in persons with CHD than in the general population. Most patients with CHD with a left SVC also have a right SVC, and a bridging vein may connect these structures. Although most left SVCs drain to a dilated coronary sinus and eventually the right atrium, in rare instances they drain directly to the left atrium and cause hypoxemia. CT can show the existence of a left SVC, its route of drainage, and the presence or absence of a bridging vein, all important findings in the surgical management of CHD.
Hypoplastic Left Heart Syndrome, ASD, PDA, Post-Ductal Coarctation, Double SVC
1. Bardo DME, Frankel DG, Applegate KE, Murphy DJ, Saneto RP. Hypoplastic Left Heart Syndrome. RadioGraphics 2001; 21:705-717.
2. Dillman JR, Hernandez RJ. Role of CT in the Evaluation of Congenital Cardiovascular Disease in Children. AJR 2009; 192:1219-1231.