Double Jeopardy: Truncus Arteriosus with Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection

Anil Kumar Singhi; Mrinal Bandhu Das; Soumya Kanti Mohapatra; Soumen Das; Anish Nath; Dipanjan Chatterjee

doi:10.4103/jiae.jiae_57_22

INTERESTING CASE REPORT

Year : 2023 | Volume : 7 | Issue : 2 | Page : 178-181

Double Jeopardy: Truncus Arteriosus with Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection

Anil Kumar Singhi¹, Mrinal Bandhu Das¹, Soumya Kanti Mohapatra¹, Soumen Das², Anish Nath¹, Dipanjan Chatterjee³
¹ Department of Pediatric and Congenital Heart Disease, Medica Super Specialty Hospital, Kolkata, West Bengal, India
² Department of Cardiovascular and Thoracic Surgery, Medica Super Specialty Hospital, Kolkata, West Bengal, India
³ Department of Radiodiagnosis, Medica Super Specialty Hospital, Kolkata, West Bengal, India

Date of Submission	24-Nov-2022
Date of Decision	13-Dec-2022
Date of Acceptance	20-Dec-2022
Date of Web Publication	08-Jun-2023

Correspondence Address:
Dr. Anil Kumar Singhi
Department of Pediatric and Congenital Heart Disease, Medica Super Specialty Hospital, Mukundapur, Kolkata - 700 099, West Bengal
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_57_22

Abstract

Truncus arteriosus (TA) is a congenital cyanotic heart disease with admixture physiology at the great arterial level. It can have associated anomalies of the pulmonary arteries, truncal valve narrowing, etc., Total pulmonary venous anomalous drainage associated with TA is extremely rare. We report a sick young infant who presented with type I TA and obstructed infracardiac total anomalous pulmonary venous return.

Keywords: Admixture physiology, pulmonary hypertension, total anomalous pulmonary venous connection, truncus arteriosus

How to cite this article:
Singhi AK, Das MB, Mohapatra SK, Das S, Nath A, Chatterjee D. Double Jeopardy: Truncus Arteriosus with Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection. J Indian Acad Echocardiogr Cardiovasc Imaging 2023;7:178-81

How to cite this URL:
Singhi AK, Das MB, Mohapatra SK, Das S, Nath A, Chatterjee D. Double Jeopardy: Truncus Arteriosus with Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2023 [cited 2023 Sep 27];7:178-81. Available from: https://jiaecho.org/text.asp?2023/7/2/178/378257

Introduction

Truncus arteriosus (TA) is a rare congenital anomaly seen in <3% of congenital heart disease. It can have associated congenital cardiac anomalies such as interrupted aortic arch, hypoplasia of pulmonary arteries, coronary artery anomalies, and truncal valve stenosis.^[1] Very rarely, total anomalous pulmonary venous connection (TAPVC) can be associated with TA. We report the coexistence of two rare congenital cardiac anomalies in a young baby.

Case Report

A 55-day-old baby weighing 3.3 kg presented with severe cyanosis and respiratory distress. The baby had an oxygen saturation of 55%. The heart rate was 165/min, the second heart sound was single, and there was a 2/6 ejection systolic murmur at the second left intercostal space. Chest X-ray showed a cardiothoracic ratio ~0.5 and diffuse bilateral ground glass haziness [Figure 1]. Electrocardiogram showed sinus tachycardia with right axis deviation. Echocardiogram revealed a rare cardiac anomaly: type I TA along with infracardiac TAPVC. The pulmonary veins appeared forming a common chamber and draining via the descending vertical vein to the hepatic venous system. The infracardiac TAPVC was obstructive. The small patent foramen ovale was shunting right to left. The right heart chambers were dilated with right ventricular dysfunction along with severe pulmonary hypertension. The branch pulmonary arteries were measuring ~5 mm and were arising close from the posterolateral aspect of the common trunk. The coronary artery was single from the right side of the common trunk [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f and [Video 1]. The patient was taken to the intensive care unit for stabilization. There was acidosis and hypoxia requiring mechanical ventilation. After initial stabilization, computed tomographic pulmonary angiogram (CTPA) was done to delineate the anatomy before surgery for the rare anomaly. The angiogram showed all pulmonary veins joining to form a common chamber behind the left atrium (LA) and draining into the hepatic system through the inferior vertical vein. The branch pulmonary arteries were seen originating from the posterolateral aspect of the trunk [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e, [Figure 3]f, [Figure 3]g and [Video 2]. The main pulmonary artery (PA) measured 9.3 mm (Z score: +1.02), and the right and left pulmonary arteries measured 6 mm (Z score: +1.65) and 7. 3 mm (Z score: +3.2), respectively. The plan of surgery was discussed in the combined surgical and cardiology meeting. The age and clinical status were felt not suitable for TAPVC repair along with truncal repair with right ventricle to PA conduit. The plan decided was TAPVC repair with bilateral PA banding if tolerated by the baby. On the surgical table, the anatomy was confirmed. The TA was type I, and the main PA was coming from the left posterior aspect of the common trunk and was immediately dividing into left and right pulmonary arteries. The right atrium and right ventricle were dilated. The surgery was done at 20°C temperature with low-flow technique with 7/0 Prolene suture (Ethicon US, LLC). Infracardiac TAPVC was dissected in the posterior mediastinum. The inferior vertical vein was tied and divided. The common chamber was dissected and sutured to the LA and left atrial appendage base [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. The oxygen saturation rose from 55% to nearly 80%. The surgical team was not comfortable with bilateral PA bands at the same time. Hence, the sternum was kept open for possible delayed bilateral PA banding. After initial stable hemodynamics, the baby had significant metabolic derangement and instability which led to the demise of the baby.

Figure 1: Chest X ray in the posteroanterior view showing cardiothoracic ratio ~ 0.5 with bilateral diffuse haziness

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Figure 2: Echocardiogram in the subcostal view (a-d) and the parasternal short-axis view (e and f) showing the anatomy. The patent foramen ovale is seen shunting right to left (white arrow, a and b). The inferior vertical vein (green arrow, c) and the origin of common trunk and the subtruncal ventricular septal defect (yellow arrow, d) are also seen. The branch pulmonary arteries are seen coming from the left and posterior aspect of the common trunk (red arrow, e). The single coronary artery is seen originating from the right sinus and immediately dividing (gray arrow, f)

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Figure 3: Computed tomographic pulmonary angiogram showing the inferior vertical vein in the frontal plane (a, green arrow), and the sub-truncal ventricular septal defect (orange arrow) and the origin of the pulmonary artery (red arrow) in the lateral view (b). Three dimensional reconstruction in various views (c-g) demonstrating the anomalous pulmonary venous connection and truncus arteriosus. The origin of the pulmonary artery from the common trunk is marked with the red arrow and the inferior vertical vein draining into the liver is marked with the green arrow

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Figure 4: Surgical photograph showing (a) looped aorta (yellow arrow) and pulmonary artery (sky blue notched arrow) arising from the common trunk. (b and c) The heart retracted superiorly and rightward (heart sign). The right upper pulmonary vein (white arrow) is seen forming the common pulmonary venous chamber (black arrow). The shining diaphragmatic surface is seen inferiorly (green star). The left pulmonary vein visualized after dissection (c, yellow arrow). (d) The vertical vein is seen looped towards the diaphragm (violet arrow)

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[Additional file 1]

Video 1: Echocardiogram in the subcostal view (a-d) and the parasternal short-axis view (e and f) showing the anatomy. The patent foramen ovale is seen shunting right to left (a and b). The inferior vertical vein (c) and the origin of common trunk and the subtruncal ventricular septal defect (d) are also seen. The branch pulmonary arteries are seen coming from the left and posterior aspect of the common trunk (e). The single coronary artery is seen originating from the right sinus and immediately dividing (f).

[Additional file 2]

Video 2: Computed tomographic pulmonary angiogram in the transverse (a) sagittal (b) and coronal (c) planes delineating the anatomy of this rare combination of infracardiac total anomalous pulmonary venous return and type I truncus arteriosus.

Discussion

TA represents a type of conotruncal anomaly. It falls in admixture physiology at the great arterial level. It can have a rare association in the form of arch anomalies or truncal valve abnormalities. The presence of total anomalous pulmonary venous abnormality in a patient with TA is an extremely rare anomaly with only limited case reports in the literature.^[2] Anomalous pulmonary venous return is attributed to the abnormality of embryonic pulmonary venous channel. TAPVC represents admixture physiology at the right atrial level. Two types of rare admixture physiologies in a given patient are extremely rare. Litovsky et al.^[2] reported six cases of TA with pulmonary venous anomalies. Out of this cohort of six cases, four had TAPVC with two supracardiac, one cardiac and only one infracardiac variant. The other two patients had partial anomalous pulmonary venous connections. Five out of the six patients eventually died with or without surgery. The combination of TA and TAPVC was reported only in two out of 180 specimens analyzed by Bharati et al.^[3] In those who present with the combination of TA and TAPVC, the infracardiac variant of TAPVC with obstruction is even rarer. They are the sickest subset like our index patient and have the worst prognosis. Our patient presented relatively late in a very sick state with severe hypoxia, acidosis, and distress. The ideal operation of TAPVC correction along with truncus repair with RV to PA conduit could not be planned because of nonavailability of conduit on an urgent basis for a sick baby with very low oxygen saturation. Even after successful surgery, the risk of pulmonary hypertensive crisis and postoperative pulmonary venous obstruction are the major determinants of the outcome. The presence of hypoplasia or narrowing of individual pulmonary veins, coronary artery abnormality, or mixed type of TAPVC adds to the unfavorable outcome.^[2],[3] Mixed surgical results have been reported in TA, interrupted arch, and single ventricle with associated TAPVC.^[4],[5] A scimitar-type mixed pulmonary venous return in a patient with TA in the neonatal period has been reported to have had successful surgical repair.^[6] Overall surgical outcome of this rarest combination is poor. Detailed and correct anatomical diagnosis is essential for proper treatment planning. A high degree of suspicion is required for diagnosing double types of admixture physiology lesions in the same patient. Echocardiography remains the baseline imaging modality. The focus of imaging should be on possible associated anomalies such as arch anomalies, truncal valve anatomy, and coronary artery, PA, or pulmonary venous hypoplasia. CTPA is a great addition for confirmation of the diagnosis and delineation of the PA and pulmonary venous anatomy, drainage site of the pulmonary veins, hypoplasia of the vessels, etc., Timely diagnosis and appropriate care can help to salvage the baby. Despite the best care, the final outcome may not be good for all the babies. A further detailed study and analysis of interventions in such cases is essential to shed light on appropriate management.

Conclusion

Multiple complex rare congenital cardiac abnormalities can very rarely coexist in a baby. The babies with TA and total anomalous pulmonary venous drainage can be very symptomatic at presentation. Detailed anatomical diagnosis with multimodality imaging such as echocardiogram and CTPA helps in appropriate diagnosis. Team approach in surgical and intensive care can help in managing this difficult substrate, but still, the outcome can be poor in these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Alamri RM, Dohain AM, Arafat AA, Elmahrouk AF, Ghunaim AH, Elassal AA, et al. Surgical repair for persistent truncus arteriosus in neonates and older children. J Cardiothorac Surg 2020;15:83.
2.	Litovsky SH, Ostfeld I, Bjornstad PG, Van Praagh R, Geva T. Truncus arteriosus with anomalous pulmonary venous connection. Am J Cardiol 1999;83:801-4, A10.
3.	Bharati S, McAllister HA Jr., Rosenquist GC, Miller RA, Tatooles CJ, Lev M. The surgical anatomy of truncus arteriosus communis. J Thorac Cardiovasc Surg 1974;67:501-10.
4.	Berdjis F, Wells WJ, Starnes VA. Truncus arteriosus with total anomalous pulmonary venous return and interrupted arch. Ann Thorac Surg 1996;61:220-2.
5.	Sinha M, Pandey NN, Bhambri K, Kumar S. Type A3 truncus arteriosus with infracardiac total anomalous pulmonary venous return and single ventricle physiology: A triad of tribulations. J Cardiovasc Comput Tomogr 2020;14:e137-8.
6.	Luciani GB, Hoxha S, Lucchese G, Rebonato M, Rossetti L, Faggian G, et al. Neonatal repair of truncus arteriosus with "scimitar-like" mixed total pulmonary venous return. Ann Thorac Surg 2014;97:e167-9.