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Year : 2018  |  Volume : 28  |  Issue : 4  |  Page : 239-241

Dual communication of confluence of total anomalous pulmonary venous connection: An interesting but a rare entity

1 Department of Pediatric Cardiology, BL Kapur Super Speciality Hospital, New Delhi, India
2 Fortis Escorts Heart Institute and Research Centre, New Delhi, India

Date of Web Publication24-Dec-2018

Correspondence Address:
Gaurav Agrawal
BL Kapur Super Speciality Hospital, New Delhi - 110 005
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcecho.jcecho_29_18

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Here, we describe the case of a 9-month-old male child who was diagnosed with a very unusual and rare variety of mixed total anomalous pulmonary venous connection.

Keywords: Computerized tomography, echocardiography, mixed total anomalous pulmonary venous connection

How to cite this article:
Agrawal G, Radhakrishnan S. Dual communication of confluence of total anomalous pulmonary venous connection: An interesting but a rare entity. J Cardiovasc Echography 2018;28:239-41

How to cite this URL:
Agrawal G, Radhakrishnan S. Dual communication of confluence of total anomalous pulmonary venous connection: An interesting but a rare entity. J Cardiovasc Echography [serial online] 2018 [cited 2021 Jan 21];28:239-41. Available from: https://www.jcecho.org/text.asp?2018/28/4/239/248408

  Introduction Top

A 9-month-old male child presented with tachypnea and desaturation. He was diagnosed with a very unusual but an interesting variety of mixed total anomalous pulmonary venous connection (TAPVC) in which right-sided pulmonary veins and left inferior pulmonary veins (LIPVs) were forming a confluence behind left atrium (LA). Confluence was connecting at two sites: on one end, it was connecting with coronary sinus (CS) and at the other end, it continued as a left-sided vertical vein in which left superior pulmonary vein (LSPV) was draining. The vertical vein was finally draining into right atrium (RA) through innominate vein and right-sided superior vena cava (RSVC).

  Case Report Top

A 9-month-old male child came with tachypnea and cyanosis since birth. On examination, heart rate was 140/min, respiratory rate was 48/min, and saturation was 75%. On auscultation, there was a wide split and fixed second heart sound with loud pulmonary component, Grade 3/6 ejection systolic murmur at left upper sternal border.

Transthoracic echocardiography (TTE) showed large ostium secundum atrial septal defect (ASD), shunting right to left, dilated RA and right ventricle, and severe pulmonary artery hypertension. Confluence was seen behind the LA which was formed by right superior pulmonary vein (RSPV) and right inferior pulmonary vein (RIPV) [Figure 1]. Confluence was communicating with CS on one end and continued as a left-sided vertical vein in which LSPV was draining on other end. Drainage of LIPV was unclear on TTE. The vertical vein was draining into RA through innominate vein and RSVC. The communication of confluence with CS, as well as with vertical vein, was obstructed with a mean pressure gradient of 11 and 5 mmHg, respectively.
Figure 1: Transthoracic echocardiography image in subcostal short-axis view showing drainage of right superior (right superior pulmonary vein) and inferior pulmonary veins (right inferior pulmonary veins) into confluence and drainage of confluence into coronary sinus and that is draining into right atrium

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Computerized tomographic (CT) angiographic image (including three-dimensional [3D] volume-rendered image) was done for defining the anatomy in detail, which confirmed the echocardiographic findings as well as showed drainage of LIPV into the confluence [Figure 2] and [Figure 3].
Figure 2: Computerized tomographic image showing drainage of left inferior pulmonary vein into confluence, large atrial septal defect, and confluence behind left atrium

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Figure 3: Three-dimensional volume-rendered computerized tomographic image in posterior view showing dual communication of confluence along with drainage of left superior pulmonary vein into vertical vein that is draining into superior vena cava

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  Discussion Top

TAPVC develops when the primordial pulmonary vein fails to unite with the plexus of veins surrounding the lung buds. TAPVC is a rare congenital anomaly, corresponding to approximately 2% of all congenital heart defects.[1] It consists of an abnormality of blood flow in which all four pulmonary veins drain into systemic veins or the RA with or without pulmonary venous obstruction.[2] Failure of the common pulmonary vein to connect with the pulmonary venous plexus leads to persistence of one or more earlier venous connections to the RSVC, to the left vertical vein/innominate vein, or to the umbilicovitelline vein/portal vein.[3]

In most cases of TAPVC, TTE is sufficient for diagnosis. Magnetic resonance imaging, CT angiography, and transesophageal echocardiography with 3D analysis are other imaging modalities that may provide an accurate description of pulmonary venous drainage.[4]

According to Darling's classification of this disorder, Type IV or mixed type, unlike the others, is characterized by the lack of confluence of the pulmonary veins into a vertical collecting vein.[5] Mixed variety is further surgically classified into two groups: “3 + 1” pattern, in which three veins drain at the cardiac level or in the vertical vein and the other in another site, and “2 + 2” pattern, in which the veins from each lung unite and then drain in different sites.[6] In most cases, the veins of one lung drain at the cardiac level and from the other at the supracardiac level.[7],[8]

There are very few case reports where RSPV and RIPV drained individually to the CS and then to the RA through an obstructed orifice; LSPV and LIPV formed a confluence and had dual drainage to CS and brachiocephalic vein through vertical vein.[9]

In the present article, we are reporting extremely unusual variety of mixed TAPVC in which confluence itself was communicating with dilated CS as well as also continuing as a vertical vein and both sites were obstructed, within which LSPV was draining. To the best of our knowledge, this variety of mixed TAPVC has not been reported in the literature.

The correct preoperative diagnosis and an accurate anatomic description are essential for planning surgical treatment of these patients. The goal of surgery is to redirect all pulmonary veins to the LA through wide and nonrestrictive connection. Careful intraoperative examination of the pulmonary venous drainage is mandatory for all patients. Precise technique for surgery adopted in an individual patient depends on the pattern of anatomic drainage, and an individualized surgical approach is recommended.

Mortality after TAPVC repair has decreased but remains highest in young patients and in those with cardiac connection type or pulmonary venous obstruction despite improved perioperative care.[10] Surgical mortality remains higher in repair of mixed form of TAPVC, especially in patients with more complex patterns of pulmonary venous connection. Patients with “3 + 1” variety have higher risk for surgery than a “2 + 2” pattern of mixed totally anomalous pulmonary venous connection.[9]

Our patient underwent successful surgical correction with anastomosis of confluence with LA and rerouting of LSPV to LA appendage. The patient did very well with improvement in tachypnea and disappearance of cyanosis. The patient was discharged after 10 days, and on follow-up his pulmonary artery pressure was normal.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Bharati S, Lev M. Congenital anomalies of the pulmonary veins. Cardiovasc Clin 1973;5:23-41.  Back to cited text no. 1
Emmanouilides GC, Gutgesell HP, Riemenschneider TA, Allen HD. Moss and Adams Heart Disease in Infants, Children and Adolescents: Including the Fetus and Young Adult. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 1995.  Back to cited text no. 2
Neill CA. Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics 1956;18:880-7.  Back to cited text no. 3
Choe YH, Lee HJ, Kim HS, Ko JK, Kim JE, Han JJ, et al. MRI of total anomalous pulmonary venous connections. J Comput Assist Tomogr 1994;18:243-9.  Back to cited text no. 4
Craig JM, Darling RC, Rothney WB. Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest 1957;6:44-64.  Back to cited text no. 5
Delius RE, de Leval MR, Elliott MJ, Stark J. Mixed total pulmonary venous drainage: Still a surgical challenge. J Thorac Cardiovasc Surg 1996;112:1581-8.  Back to cited text no. 6
Thompson LD, McElhinney DB, Reddy VM, Jue KL, Hanley FL. Infradiaphragmatic totally anomalous pulmonary venous return with two separate descending veins in association with right atrial isomerism. Ann Thorac Surg 2000;70:1400-2.  Back to cited text no. 7
Sarioğlu T, Kinoğlu B, Paker T, Sarioğlu A, Saltik L, Aytaç A, et al. A rare case of mixed type total anomalous pulmonary venous connection and its surgical treatment. Thorac Cardiovasc Surg 1997;45:152-4.  Back to cited text no. 8
Chowdhury UK, Airan B, Malhotra A, Bisoi AK, Saxena A, Kothari SS, et al. Mixed total anomalous pulmonary venous connection: Anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg 2008;135:106-16, 116.e1-5.  Back to cited text no. 9
Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Williams WG, Caldarone CA, et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation 2007;115:1591-8.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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