Congenitally interrupted inferior vena cava without other features of the heterotaxy syndrome: report of five cases and characterization of a rare entity.

Congenital interruption of the inferior vena cava (IVC) due to absence of its hepatic segment is usually found in patients with polysplenia. The occurrence without other features of the heterotaxy syndrome is rare, and the frequency of associated additional vascular and cardiac anomalies in these patients is not well known. We reviewed the literature and present 5 of our own patients to characterize this entity. All but 1 of the 67 known cases had visceroatrial situs solitus. There was 1 patient with interruption of the IVC in complete situs inversus. The interrupted IVC typically continued via the azygos system into the superior vena cava, but there were 4 cases without a direct connection between the infrahepatic IVC and the azygos system. Additional venous abnormalities or cardiac malformations were present in 47.8% and 31.4%, respectively. In contrast to the classical heterotaxy syndrome, heart defects with interrupted IVC and normal situs did not involve inversion of cardiac segments. The diagnosis can readily be made by echocardiography or magnetic resonance imaging. Failure to recognize this anomaly can result in various problems during invasive medical or surgical procedures. In our own patients, interventional procedures failed, necessitating an operation in all 5 cases. Careful analysis of systemic venous drainage should be performed in all patients prior to cardiac catheterization, pacemaker implantation, or surgery, even if the visceroatrial situs is normal.

Normative angiographic data relating to the dimensions of the aorta and pulmonary trunk in children and adolescents.

BACKGROUND: Definition of normative data of the great arteries from neonatal to adult ages may aid in assessment of the growth of cardiovascular structures, thus guiding the timing and type of intervention in patients with congenital cardiac disease. METHODS: We calculated the cross-sectional areas of the arterial roots at the basal attachment of the valvar leaflets, the sinuses, and standardized distal sites using cineangiograms of 59 normal children and adolescents with mean age of 5.4 plus or minus 4.7 years and a range from 0.1 to 16 years, the children having a mean weight of 21.2 plus or minus 15.7 kilograms, with a range from 2.2 to 68 kilograms, and mean height of 108 plus or minus 35 centimetres, with a range from 43 to 184 centimetres. Values at each site were calculated averaging end-diastolic and end-systolic measurements, and indexed to body surface area. Results are expressed as the mean plus or minus the standard deviation. RESULTS: The diameter of the aortic root at the basal attachment of the leaflets was 249 plus or minus 26, the midpoint of the sinuses 379 plus or minus 59, the sinutubular junction 290 plus or minus 58, the isthmus 158 plus or minus 36, the postisthmic region 152 plus or minus 33, and the descending aorta at the level of diaphragm 130 plus or minus 18 millimetres squared per metre squared. The pulmonary root measured at the basal attachment of the leaflets was 253 plus or minus 28, the midpoint of the sinuses 352 plus or minus 58, the sinutubular junction 293 plus or minus 58, the right pulmonary artery 176 plus or minus 25, the left pulmonary artery 153 plus or minus 20, and sum of right and left pulmonary arteries 330 plus or minus 37 millimetres squared per metre squared. All indexes were consistent over a wide range for body surface areas. CONCLUSIONS: Definition of normative data of the great vessels may aid in the evaluation of congenital or acquired abnormalities, serving as guidelines for intervention during medical or surgical management and follow-up.