Systemic Venous Anomalies and Partial Heterotaxia With Normal Heart

Abstract

A case of infrahepatic interruption of inferior vena cava with hemiazygos continuation into a persistent left superior vena cava and partial visceral heterotaxia in association with an otherwise normal heart is presented. The peculiarity of this rare lesion without cardiac malformation and the possible explanation for it are discussed. The clinical significance of systemic venous anomalies is discussed.The association of complex cardio¬ vascular anomalies with partial heterotaxia,13 and with anomalies of the systemic venous connection,46 has been well documented. We found a case with both incomplete visceral in¬ version (partial heterotaxia) and sys¬ temic venous anomalies without any associated cardiac malformation. The purposes of this paper are to present the details of this case, to outline the possible explanation for this associa¬ tion, and to discuss briefly the clinical applications of knowledge of anom¬ alies of the systemic venous connec¬ tions.A 5-year-old white girl was admitted to Good Samaritan Hospital in April 1969 for cardiac catheterization. A heart murmur had been noticed in her infancy. No history of cyanosis or dyspnea was present. She had been a healthy child with normal growth and development. Because of a uri¬ nary tract infection at the age of 4 years, an intravenous pyelogram was obtained, at which time residual barium in the appen¬ dix was noticed (from a previous cardiac series with barium swallow) on the left side of the abdomen (Fig 1, top).

Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium.

“…Nuclear angiography would also be helpful in delineating these anomalies.23 Though cardiac catheterisation and angiography remain the most useful procedures to confirm the diagnosis, they are not without hazard; use of these anomalous venous channels for catheterisation has been reported to produce paroxysmal supraventricular tachycardia, occasional angina, shock, and cardiac arrest. 25 In an attempt to explain the origin of the right superior vena cava to left atrium, persistence of the cephalic portion of the right valve of the sinus venosus22 and leftward and cephalic displacement of the right horn of sinus venosus2' 23 have been postulated. Misplaced interatrial septum as a cause for this anomaly was mentioned2' 22 but was excluded on the basis that the orifice of the inferior vena cava and coronary sinus would also be located on the left atrial side of the septum if this occurred.2' 22 More than normal leftward or cephalic displacement of the sinus venosus postulated by Kirsh and associates2' may not produce this anomaly unless associated with a shift of the embryological interatrial septae (septum spurium, primum, and secundum) to the right of (or caudal to) the common cardial vein.…”

Clinical Case Reports 1971-1974

“…Discussion: The emptying of most of the systemic venous return into the right atrium may have caused the diastolic murmur and the systolic murmur and was thought to be a functional murmur. In this case with partial heterotaxia and systemic venous anomalies, the heart was otherwise normal, which is unusual and prompted us to document the case because of prior reported association of complex heart disease with systemic venous anomalies and partial inversion of the abdominal viscera, as quoted in our paper [6]. The normal position of the atria may have prevented the development of a complex CHD, despite the systemic venous anomalies [6].…”

“…In this case with partial heterotaxia and systemic venous anomalies, the heart was otherwise normal, which is unusual and prompted us to document the case because of prior reported association of complex heart disease with systemic venous anomalies and partial inversion of the abdominal viscera, as quoted in our paper [6]. The normal position of the atria may have prevented the development of a complex CHD, despite the systemic venous anomalies [6]. The possible association of the described partial heterotaxia and systemic venous anomalies with asplenia/polysplenia syndromes was discussed [6].…”

Evolution of Pediatric Cardiology over the Last 50 Years - Part III

“…cardiac catheterization and selective cine-angiography were discussed. These are: Correlating pulmonary venous wedge pressures with pulmonary arterial pressures (Figure 11 and Figure 12) during cardiac catheterization [55], advocacy of femoral venous route for cardiac catheterization in pediatric patients infants [56], report of systemic venous anomalies and partial heterotaxia in a child with normal heart [57], examining the concepts with regard to pressure and energy in cardiac chambers both in terms of pressure gradient in the absence of obstruction/stenosis [58] and lack of pressure gradient in the presence of multiple obstructions in series [59], status of cardiac catheterization in children in the mid-1970s [60], demonstration of growth of hypoplastic ri- [43], echocardiographic follow-up results of buttoned device occlusion of atrial septal defect [44], review of ultrasound studies [45][46][47], collaborative echo-Doppler studies [48][49][50], echocardiographic evaluation of the results of balloon pulmonary valvuloplasty [51], editorials on echo topics, aneurysm of the ventricular septum producing pulmonary outflow tract obstruction in the morphologic left ventricle in corrected transposition of the great arteries [52][53][54] and echo descriptions of CHDs.…”