We present an extremely rare case of an infant with subtotal anomalous caval drainage into the left atrium and duct-dependent pulmonary circulation. Surgical correction is the only permanent treatment option. This case demonstrates that ductal stenting as a bridge procedure in small affected infants is a good option until the patient achieves a more optimal weight.
A severely cyanotic neonate was admitted to our hospital immediately after birth. Saturation in room air was <50%. She responded well to intravenous infusion of prostaglandin. Angiogram revealed a very rare congenital heart disease consisting of anomalous drainage of the inferior and left superior vena cava into the left atrium, while a right superior vena cava was normally connected to the right atrium. Right atrium and right ventricle, supplied only by a rather small right superior vena cava, were almost hypoplastic and could not maintain adequate pulmonary circulation, which was duct dependent.
Van Praagh et al1 suggest that this malformation could be due to the lack of formation of the common wall that normally separates the pulmonary veins and the venae cavae. There was not, however, coexistent anomalous drainage of the pulmonary veins.
Ductal stenting was decided as a palliative intervention instead of surgically performing an aortopulmonary shunt, with a view to a future surgical correction of the congenital heart disease at an older age with less risk. At that point, the type of surgical repair (univentricular with cavopulmonary shunts or biventricular with total correction of the anomaly) will depend on the size of the right ventricle.
At the age of 2 months, with a body weight of 3.9 kg, the infant was transferred from the neonatal care unit to the catheterization laboratory for attempted ductal stenting. Antimicrobial treatment with teicoplanin 10 mg/kg/24 hr and piperacillin-tazobactam 300 mg/kg/24 hr was delivered 1 day before and after the procedure.
Under general anesthesia, the right femoral artery and vein were percutaneously cannulated. Heparin 50 U/kg was administered after vascular access was obtained. Arch aortography demonstrated a 16 mm long and tortuous patent duct. The duct was accessed from the femoral artery using a 4 Fr Cobra catheter (Cook Medical) and a 0.014 ̋ straight, 320 cm-long guidewire. The wire was stabilized in the right pulmonary artery. Over the wire, a 4 x 20 mm sinus-SuperFlex DS self-expandable stent (Optimed) was advanced, introduced, and deployed in the duct. Total fluoroscopy time was 8 minutes.
The infant improved and prostaglandin was discontinued immediately after stenting. Intravenous heparin 50 U/kg was administered four times daily for 24 hours, with aspirin 5 mg/kg/day afterward. Transdermal saturation was constantly 95%.
Anomalous drainage of the inferior vena cava to the left atrium is a very rare cardiac malformation;2,3 it is usually part of a more complex congenital heart disease. Subtotal venous drainage without visceral heterotaxy is extremely rare or probably unique.4 Surgical correction is the only treatment. Ductal stenting as a bridge procedure in small affected infants5 is a good option.6
1. Van Praagh S, Carrera ME, Sanders SP, et al. Sinus venosus defects: unroofing of the right pulmonary veins — anatomic and echocardiographic findings and surgical treatment. Am Heart J. 1994;128(2):365-379.
2. Genoni M, Jenni R, Vogt PR. Drainage of the inferior vena cava to the left atrium. Ann Thorc Surg. 1999;67(2):543-545.
3. ShiinaY, Slavik S, Uemura H, et al. The inferior caval vein draining into the left atrial cavity – a rare case. Images Paediatr Cardiol. 2011;13(4):1-5.
4. Yildirim A, Kosger P, Ozdemir G, et al. Total anomalous systemic venous drainage with heterotaxia syndrome: a rare case. Case Rep Cardiol. Epub 2014 Aug 12.
5. Alwi M. Stenting the ductus arteriosus: case selection, technique and possible complications. Ann Pediatr Cardiol. 2008;1(1):38-45.
6. Bentham JR, Zava NK, Harrison WJ, et al. Duct stenting versus modified Blalock-Taussig shunt in neonates with duct-dependent pulmonary blood flow. Circulation. 2018;137(6):581-588.