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Year : 2018  |  Volume : 2  |  Issue : 2  |  Page : 130-131

Unusual form of partial pulmonary venous connection

Department of Cardiovascular Surgery, Faculty of Medicine, Ege University, Bornova, Izmir, Turkey

Date of Web Publication6-Sep-2018

Correspondence Address:
Dr. Osman Nuri Tuncer
Department of Cardiovascular Surgery, Faculty of Medicine, Ege University, Kazimdirik Mahallesi, Üniversite Cd. No: 9, 35100 Bornova, İzmir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_24_18

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Success in congenital heart surgery has increased with improvement in surgical technique, anesthesia, and perioperative care. Transthoracic echocardiography (TTE) is regarded as an initial screening and diagnostic method in patients, but it may be limited by lack of an adequate acoustic window and more importantly suboptimal depiction of the extracardiac vasculature. Computed tomography angiography is an important alternative because of its wide availability; short acquisition time can also provide useful information about all anatomical structures. In this article, we present a case of bilateral partial anomalous pulmonary venous connection in which TTE is inadequate to show the left vertical vein.

Keywords: Congenital heart disease, partial pulmonary venous connection, transthoracic echocardiography

How to cite this article:
Tuncer ON, Ayik MF, Bilen &, Atay Y. Unusual form of partial pulmonary venous connection. J Indian Acad Echocardiogr Cardiovasc Imaging 2018;2:130-1

How to cite this URL:
Tuncer ON, Ayik MF, Bilen &, Atay Y. Unusual form of partial pulmonary venous connection. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2018 [cited 2021 Dec 5];2:130-1. Available from: https://www.jiaecho.org/text.asp?2018/2/2/130/240636

  Introduction Top

Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital cardiovascular disorder and characterized by drainage of one or several pulmonary veins to the systemic venous system. The most common form of PAPVC is pulmonary veins from the right upper lobe to the superior vena cava (SVC) in association with sinus venosus atrial septal defect (ASD). PAPVC of the right pulmonary veins is approximately ten times more common than the left pulmonary veins. Bilateral PAPVC is extremely unusual.[1] The following report describes a case of bilateral PAPVC in a patient who underwent surgical closure of ASD and correction of an unusual PAPVC.

  Clinical Presentation Top

A 3-years-old boy was referred for evaluation of a systolic murmur which is noticed during routine physical examination. Transthoracic echocardiography (TTE) revealed the 88211;9 mm sinus venosus ASD and a gradient of 40 mmHg across pulmonary valve. Right cardiac chambers determined as dilated. Pulmonary venous drainage could not be evaluated by TTE. Computed tomography angiography (angio CT) of thorax performed to patient with a suspicion of being PAPVC. Multislice angio-CT showed ASD and also four pulmonary veins. Right upper and middle pulmonary lobe veins were draining into the SVC and left upper pulmonary lobe veins were draining to innominate vein through a vertical vein [Figure 1].
Figure 1: Computed tomography angiographic scene: SVC: Superior vena cava, R-SPv: Right superior pulmonary vein, L-SPv. Left superior pulmonary vein

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Patient underwent a surgical correction. Standard median sternotomy was performed. After meticulous dissection of the SVC, upper right-sided pulmonary veins were seen draining to SVC. Left vertical vein was dissected carefully and looped [Figure 2]a. Aortabicaval cannulation (selective high SVC cannulation) was performed, and cardiopulmonary bypass started. After aortic cross-clamping and antegrade blood cardioplegia administration, left vertical vein was divided from the innominate vein and opened longitudinally. Left atrial appendage was prepared and sutured to the left vertical vein with an end-to-side anastomosis [Figure 2]b. A right atriotomy was performed. There was a sinus venosus ASD which has a smaller diameter than the pulmonary vein orifice.
Figure 2: (a) Dissected and looped left vertical vein. (b) Anastomosis of left vertical vein and left atrial appendage

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The atrial septal flap for the pulmonary venous channel was created by cut-back procedure of the defect along the septal attachment to the atrial wall. Fresh autologous pericardium is used to suture the margin of the sinus venosus defect while re-routing right upper pulmonary veins into left atrium. Right atriotomy closed primarily, and the operation was complete. Postoperative low-molecular- weight heparin therapy was administered for 2 weeks, and anti-aggregate therapy was continued alone. After an uneventful recovery, the patient was discharged on the 5th postoperative day.

Predischarge transthoracic echocardiogram showed no cardiac pathologies.

  Discussion Top

The prevalence of PAPVC is about 0.4%8211;0.7%[2] and usually occurs in the right lung, with only involving 10% left sided. Bilateral PAPVC is extremely rare. Alsoufi et al. found right-sided, left-sided, and bilateral anomalous pulmonary veins in 91%, 7%, and 2% of the patients.[3] Most patients with PAPVC are asymptomatic and usually incidentally detected. The natural history dictates that if significant left-to-right shunt exists, patients may develop irreversible pulmonary hypertension, pulmonary vascular obstructive disease, or right heart failure.

Currently, the diagnosis of PAPVC is established with TTE or transesophageal echocardiography (TEE) in most of the cases. The diagnosis of the left-sided anomalous pulmonary venous connection can be missed by TTE or TEE. Cases were reported that diagnosis was missed, and another cardiac catheterization performed days or years after the initial surgical correction and revealed an other PAPVC on the left side.[4] Angio-CT is an alternative diagnostic approach in these patients if TEE is insufficient. Angio-CT has 100% sensitivity for diagnosing PAPVC in patients with sinus venosus ASD, 100% specificity, 100% positive predictive value, and 100% negative predictive value.[5]

In our case, TTE was inadequate to show bilateral PAPVC. Appropriate surgical treatment applied to the patient with complete diagnosis by CT-angio.

Magnetic resonance angiography should be a better modality (as well as avoiding the use of ionizing radiation). The duration of the application is long, and the need for sedation, especially in young children limit its widespread use.

  Conclusion Top

TTE can evaluate all four pulmonary veins in most of the cases. If TTE does not show drainage of all four pulmonary veins clearly, especially in grown-up children or children with complex congenital defect or with poor echo window, CT-angio is highly useful due to its high resolution for extracardiac structures.

This case highlights the weakness of TTE to detect PAPVC. In our opinion, proper surgical planning can be achieved with TTE and completed by angio-CT and should be kept in mind that the PAPVC may be bilateral.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

ElBardissi AW, Dearani JA, Suri RM, Danielson GK. Left-sided partial anomalous pulmonary venous connections. Ann Thorac Surg 2008;85:1007-14.  Back to cited text no. 1
Broy C, Bennett S. Partial anomalous pulmonary venous return. Mil Med 2008;173:523-4.  Back to cited text no. 2
Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG, et al. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg 2007;84:2020-6.  Back to cited text no. 3
Aguilar Jiménez JM, Torres EG, Arlati F, Comas Állas JV. Unnoticed bilateral partial anomalous pulmonary vein connection. J Thorac Cardiovasc Surg 2015;149:e40-2.  Back to cited text no. 4
Amat F, Le Bret E, Sigal-Cinqualbre A, Coblence M, Lambert V, Rohnean A, et al. Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children. Interact Cardiovasc Thorac Surg 2011;12:179-82.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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