Submitting/Presenting Author

Anmol GoyalFollow

Presenter Status

Fellow

Abstract Type

Case report

Primary Mentor

Maria Kiaffas

Start Date

10-5-2021 11:30 AM

End Date

10-5-2021 1:30 PM

Presentation Type

Poster Presentation

Description

Background: Tricuspid atresia comprises 3-4% of all congenital heart disease (CHD). Anatomic findings are no true connection between the right atrium (RA) and right ventricle (RV) with concomitant findings of a ventricular septal defect (VSD), right ventricular hypoplasia, abnormalities of right ventricular outflow tract, pulmonary valve and pulmonary arteries. Common associated lesions have been described including transposition of great vessels, hypoplasia of aortic arch, double outlet ventricle or a common arterial trunk. Total anomalous pulmonary venous connection (TAPVC) is relatively rare and we present a case in addition to the six previously described cases in literature.

Case: A 33-year-old woman was referred at 28 weeks gestation for fetal echocardiography due to suspected CHD. Fetal echocardiography revealed a hypoplastic, thick echogenic tricuspid valve annulus with trivial antegrade flow and insufficiency, moderately hypoplastic RV (infundibulum), moderately hypoplastic pulmonary valve, main and branch pulmonary arteries. A left superior vena cava (LSVC) was noted and there were concerns for anomalous pulmonary venous connection possibly to the coronary sinus (CoS). (Figure 1a). A scheduled repeat cesarean was performed at 38 weeks gestation resulting in the delivery of a female neonate weighing 2.87 Kg. Prostaglandins were initiated soon after delivery for maintaining adequate pulmonary blood flow. Postnatal echocardiogram confirmed the fetal diagnosis and defined the anomalous pulmonary venous connection to the LSVC close to its junction with the coronary sinus (Figure 1b). A CT angiogram was performed for better delineation of the pulmonary venous anatomy and size (1c). An initial palliation with a BT shunt was undertaken to ensure adequate pulmonary blood flow, coming off prostaglandins. Postoperatively, patient demonstrated signs of pulmonary over-circulation and she subsequently had main pulmonary artery banding for reduction of overall pulmonary blood flow. Better balance of her pulmonary and systemic circulation flow was achieved. The patient is clinically stable awaiting the second stage of repair given her single ventricle physiology. Planned surgical repair is a left bidirectional Glenn procedure and TAPVC repair.

Learning points: Prenatal diagnosis of tricuspid atresia with TAPVC is a very rare association but should be considered when there is failure to connect the pulmonary veins to the left atrium and when a left atrial to descending aorta gap is present on fetal echocardiogram. Diligent interrogation for a confluence or vertical vein should be undertaken in order to identify the abnormal connection. Use of multi-modality imaging postnatally compliments echocardiography and assists management decision making. Balance of pulmonary blood flow and unmasking potential presence of obstruction to pulmonary venous return are things to be considered and addressed postnatally. Repair of TAPVC can be deferred for the second stage repair of this single ventricle physiology when there is no clinical evidence of obstruction to the pulmonary venous flow.

MeSH Keywords

Tricuspid atresia; Total anomalous pulmonary venous connection; Prenatal diagnosis

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May 10th, 11:30 AM May 10th, 1:30 PM

Tricuspid atresia with totally anomalous pulmonary venous connection: An uncommon finding

Background: Tricuspid atresia comprises 3-4% of all congenital heart disease (CHD). Anatomic findings are no true connection between the right atrium (RA) and right ventricle (RV) with concomitant findings of a ventricular septal defect (VSD), right ventricular hypoplasia, abnormalities of right ventricular outflow tract, pulmonary valve and pulmonary arteries. Common associated lesions have been described including transposition of great vessels, hypoplasia of aortic arch, double outlet ventricle or a common arterial trunk. Total anomalous pulmonary venous connection (TAPVC) is relatively rare and we present a case in addition to the six previously described cases in literature.

Case: A 33-year-old woman was referred at 28 weeks gestation for fetal echocardiography due to suspected CHD. Fetal echocardiography revealed a hypoplastic, thick echogenic tricuspid valve annulus with trivial antegrade flow and insufficiency, moderately hypoplastic RV (infundibulum), moderately hypoplastic pulmonary valve, main and branch pulmonary arteries. A left superior vena cava (LSVC) was noted and there were concerns for anomalous pulmonary venous connection possibly to the coronary sinus (CoS). (Figure 1a). A scheduled repeat cesarean was performed at 38 weeks gestation resulting in the delivery of a female neonate weighing 2.87 Kg. Prostaglandins were initiated soon after delivery for maintaining adequate pulmonary blood flow. Postnatal echocardiogram confirmed the fetal diagnosis and defined the anomalous pulmonary venous connection to the LSVC close to its junction with the coronary sinus (Figure 1b). A CT angiogram was performed for better delineation of the pulmonary venous anatomy and size (1c). An initial palliation with a BT shunt was undertaken to ensure adequate pulmonary blood flow, coming off prostaglandins. Postoperatively, patient demonstrated signs of pulmonary over-circulation and she subsequently had main pulmonary artery banding for reduction of overall pulmonary blood flow. Better balance of her pulmonary and systemic circulation flow was achieved. The patient is clinically stable awaiting the second stage of repair given her single ventricle physiology. Planned surgical repair is a left bidirectional Glenn procedure and TAPVC repair.

Learning points: Prenatal diagnosis of tricuspid atresia with TAPVC is a very rare association but should be considered when there is failure to connect the pulmonary veins to the left atrium and when a left atrial to descending aorta gap is present on fetal echocardiogram. Diligent interrogation for a confluence or vertical vein should be undertaken in order to identify the abnormal connection. Use of multi-modality imaging postnatally compliments echocardiography and assists management decision making. Balance of pulmonary blood flow and unmasking potential presence of obstruction to pulmonary venous return are things to be considered and addressed postnatally. Repair of TAPVC can be deferred for the second stage repair of this single ventricle physiology when there is no clinical evidence of obstruction to the pulmonary venous flow.