Compartilhar
Informação da revista
Vol. 39. Núm. 5.
Páginas 291-293 (Maio 2020)
Compartilhar
Compartilhar
Baixar PDF
Mais opções do artigo
Visitas
...
Vol. 39. Núm. 5.
Páginas 291-293 (Maio 2020)
Case report
DOI: 10.1016/j.repc.2017.12.006
Open Access
Levoatrial cardinal vein with left-to-right shunt in a neonate: Multi-imaging diagnosis
Veia levoatriocardinal com shunt esquerdo-direito num recém-nascido: uma abordagem multi-imagem
Visitas
...
Rui J. Cerqueiraa,b,
Autor para correspondência
ruicerqueira@med.up.pt

Corresponding author.
, Joana O. Mirandab,c, Alexandre Carneirod, Jorge Casanovaa,b
a Department of Cardiothoracic Surgery, São João Hospital Centre, Porto, Portugal
b Department of Surgery and Physiology, Faculty of Medicine, University of Porto, Porto, Portugal
c Department of Pediatric Cardiology, São João Hospital Centre, Porto, Portugal
d Department of Radiology, São João Hospital Centre, Porto, Portugal
Informação do artigo
Resume
Texto Completo
Bibliografia
Baixar PDF
Estatísticas
Figuras (2)
Material adicional (2)
Abstract

We present a rare case of a levoatrial cardinal vein identified during the work-up of a patient with coarctation of the aorta. Early diagnosis and repair in the neonatal period prevented future manifestations of left-to-right shunt and the need for reoperations, in contrast with the later-age presentation of this congenital anomaly. An integrative approach was crucial for prompt detection, intraoperative confirmation and complete one-stage repair.

Keywords:
Levoatrial cardinal vein
Neonate
Cardiac surgery
Resumo

Os autores apresentam um caso raro de diagnóstico de uma veia levoatriocardinal identificada na avaliação incial de um recém-nascido com coartação da aorta. O diagnóstico e tratamento no período neonatal permitiram evitar as manifestações de shunt esquerdo-direito tardias e a necessidade de reintervenção no futuro. A abordagem multi-imagem integrada foi essencial para o diagnóstico precoce desta malformação congénita, confirmação intraoperatória e reparação completa num único procedimento.

Palavras-chave:
Veia levoatriocardinal
Recém-nascido
Cirurgia cardíaca
Texto Completo
Introduction

Levoatrial cardinal vein is a rare congenital anomalous connection between the left atrium and the systemic venous system, classically described in association with severe left heart hypoplasia or obstruction, particularly mitral atresia and interrupted aortic arch.1 The levoatrial cardinal vein is usually regarded as a collateral pathway enabling pulmonary venous decompression and further attenuation of putative severe pulmonary venous hypertension in the context of a small, restrictive or absent atrial septal defect.2,3 This vessel has also been described in association with other more common congenital heart defects without left ventricular hypoplasia.4 As an isolated anomaly, levoatrial cardinal vein may be diagnosed in adulthood as a cause of left-to-right shunt and right heart overload.5,6

Case report

A two-day-old newborn, with birth weight of 3745 g, was admitted to the neonatal intensive care unit in cardiogenic shock. A diagnosis of ductal-dependent coarctation of the aorta was made and prostaglandin E1 was initiated. The echocardiographic examination also detected an anomalous left-sided vessel, with ascending flow, communicating with the brachiocephalic vein (Figure 1A and Video 1). At this time, the possibility of a vertical vein consistent with anomalous pulmonary venous drainage was considered, however the four pulmonary veins could be seen entering the left atrium, which excluded the differential diagnosis of a partial anomalous pulmonary venous connection. For further clarification, computed tomography (CT) angiography was performed, and three-dimensional CT reconstructions showed an anomalous vein connecting the left atrium to the brachiocephalic vein. Additionally, the left upper pulmonary vein appeared to be draining into the caudal portion of this vessel (Figure 1B-D and Video 2).

Figure 1.

Sequential imaging approach to definitive diagnosis of a levoatrial cardinal vein in a neonate: pre-operative assessment. (A) Visualization of an abnormal ascending flow in the left superior vena cava position during the work-up echocardiogram for suspected coarctation of the aorta; (B-D) computed tomography confirming an abnormal venous connection between the left atrium and brachiocephalic vein (blue arrows).

(0,26MB).

On the sixth day of life a left lateral thoracotomy was performed. After coarctation repair, the anterior aspect of the right pulmonary hilum was inspected. The suspected levoatrial cardinal vein was identified, isolated and cannulated and an intraoperative subcostal transthoracic echocardiogram was performed. The continuity of the levoatrial cardinal vein with the left atrium was confirmed by echocardiographic contrast injection (bubble test), which showed this was not an exclusive pathway for left superior pulmonary venous drainage (Figure 2A and B). Ligation of the levoatrial cardinal vein could then be safely performed above the connection of the pulmonary vein and below the brachiocephalic vein, redirecting the upper pulmonary venous return into the left atrium. The postoperative course was uneventful.

Figure 2.

Sequential imaging approach to definitive diagnosis of a levoatrial cardinal vein in a neonate: intraoperative assessment. (A) Visualization of the levoatrial cardinal vein by direct inspection during surgery (blue arrows); (B) confirmation of the continuity of the levoatrial cardinal vein with the left atrium by echocardiographic contrast injection.

(0,14MB).
Discussion

Levoatrial cardinal vein is a very rare congenital anomaly. It is an embryologic remnant of the fetal venous system and typically represents an escape drainage route of pulmonary venous blood in severe left-sided obstructive malformations. To our knowledge this is the first report of simultaneous correction in the neonatal period of a persistent levoatrial cardinal vein and severe extracardiac left-sided obstructive disease.

This abnormality may be easily missed in an echocardiographic assessment. The main differential diagnoses are a partial anomalous pulmonary vein connection, as the levoatrial cardinal vein can be confused with a vertical vein draining the left pulmonary veins, and persistent left superior vena cava. The latter can be differentiated on echocardiography by the direction of the flow, which is descending from the brachiocephalic vein into the coronary sinus in left superior vena cava and ascending in a levoatrial cardinal vein. The presence of four pathways of inflow into the left atrium on the echocardiogram excludes the differential diagnosis of a partial anomalous pulmonary venous connection. The normal anatomy of the four pulmonary veins can be further confirmed on CT angiography. Accurate differential diagnosis is essential for determination of the treatment strategy. This case report further illustrates the usefulness of intraoperative functional imaging as an adjuvant for diagnostic confirmation.

A levoatrial cardinal vein may be a source of persistent left-to-right shunt and cause clinical manifestations of chronic right heart volume overload later in life.

The multimodal imaging approach used in this case was of paramount importance for the correct diagnosis and prompt repair of this rare congenital anomaly in the early neonatal period. This straightforward procedure enabled us to eliminate the left-to-right shunt created by this pathway and to avoid future procedures. An integrative approach was crucial for prompt detection, intraoperative confirmation and complete one-stage repair.

Conflicts of interest

The authors have no conflicts of interest to declare.

Appendix A
Supplementary material

The following are the supplementary material to this article:

(0,41MB)
(0,98MB)

References
[1]
J.E. Edwards, J.W. DuShane.
Thoracic venous anomalies.
Mayo Clin Proceed, 50 (1950), pp. 599-600
[2]
H.S. Bernstein, P. Moore, P. Stanger, et al.
The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection.
J Am Coll Cardiol, 26 (1995), pp. 995-1001
[3]
C.A. Pinto, S.Y. Ho, A. Redington, et al.
Morphological features of the levoatriocardinal (or pulmonary-to-systemic collateral) vein.
Pediatr Pathol, 13 (1993), pp. 751-761
[4]
K. Fujiwara, Y. Naito, H. Komai, et al.
Tetralogy of Fallot with levoatrial cardinal vein.
Pediatr Cardiol, 20 (1999), pp. 136-138
[5]
P. Ciliberti, A.M. Taylor, R. Yates, et al.
Occlusion of persistent levoatrial cardinal vein without left heart hypoplasia utilizing an Amplatzer device.
Eur Heart J Cardiovasc Imaging, 14 (2013), pp. 857
[6]
T. Kaneda, M. Onoe, M. Matsuda, et al.
Patent levoatrial cardinal vein without left heart hypoplasia.
Ann Thorac Surg, 81 (2006), pp. 740-742
Copyright © 2020. Sociedade Portuguesa de Cardiologia
Idiomas
Revista Portuguesa de Cardiologia

Receba a nossa Newsletter

Opções de artigo
Ferramentas
Material Suplementar
en pt

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

en pt
Cookies policy Política de cookies
To improve our services and products, we use "cookies" (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here. Utilizamos cookies próprios e de terceiros para melhorar nossos serviços e mostrar publicidade relacionada às suas preferências, analisando seus hábitos de navegação. Se continuar a navegar, consideramos que aceita o seu uso. Você pode alterar a configuração ou obter mais informações aqui.