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Case report

Rare case of adult survival in infracardiac non-obstructivetotal anomalous pulmonary venous connection

Mahipat Raj Soni a,*, Deepak Bohara a, Anup Taksande b, S. Kesavan b, Ajay Mahajan c,Pratap Nathani d

aResident, Department of Cardiology, Lokmanya Tilak Municipal Medical College, Sion, Mumbai 400022, IndiabAssistant Professor, Department of Cardiology, Lokmanya Tilak Municipal Medical College, Sion, Mumbai 400022, IndiacProfessor and Unit Head, Department of Cardiology, Lokmanya Tilak Municipal Medical College, Sion, Mumbai 400022, IndiadProfessor and Head, Department of Cardiology, Lokmanya Tilak Municipal Medical College, Sion, Mumbai 400022, India

a r t i c l e i n f o

Article history:

Received 15 July 2012

Accepted 6 September 2012

Available online 28 September 2012

Keywords:

Infracardiac TAPVC

Pulmonary venous obstruction

Inferior vena cava

* Corresponding author. Department of CardiTel.: þ91 9223587736.

E-mail address: mahipat_soni@yahoo.co.1561-8811/$ e see front matter Copyright ªhttp://dx.doi.org/10.1016/j.jicc.2012.09.008

a b s t r a c t

Adult survival in patients with uncorrected non-obstructive infracardiac total anomalous

pulmonary venous drainage is very rare. We report 20 year female referred for evaluation

of murmur diagnosed to have non-obstructive pulmonary venous drainage to the supra-

diaphragmatic inferior vena cava. To best of our knowledge, there has been no reported

instance of an adult survival in non-obstructed infracardiac TAPVC.

Copyright ª 2012, Indian College of Cardiology. All rights reserved.

1. Key message 2. Introduction

Patients with uncorrected infracardiac total anomalous

pulmonary venous connection (TAPVC) rarely survive

beyond early infancy. This is because of the pulmonary

venous obstruction that is almost invariably present.1,2 The

good prognostic factor allowing survival to adulthood may

be the drainage site being the inferior vena cava with no

obstruction, when compared to the portal vein drainage in

which hepatic sinusoids produce severe obstruction. Adult

patient with an atrial septal defect and cyanosis it is

prudent to exclude a TAPVC. Helical CT or MR angiography

are good diagnostic modalities for use in the preopera-

tive evaluation of total anomalous pulmonary venous

connection.

ology, Lokmanya Tilak Mu

in (M.R. Soni).2012, Indian College of C

This case is unique because to best of our knowledge, adult

survival of non-obstructive infracardiac TAPVC draining to

inferior vena cava has not been reported in literature. Impor-

tant factors which are responsible for survival in un-operated

patients with TAPVC beyond infancy and childhood are

a largeASDandshort route typedrainageveinwithout stenosis

and a near normal pulmonary vascular resistance.

3. Case report

Twenty years old female with no significant past history

referred for evaluation of murmur. On examination, she had

nicipal Medical College and Hospital, Sion, Mumbai 400022, India.

ardiology. All rights reserved.

Table 1 e Hemodynamics and oximetry parameters.

Pressures (mmHg) SPO2

Superior vena cava 69%

Infra renal IVC 68%

Supra renal IVC 70%

Proximal IVC 95%

Right atrium a8 v6 mean 4 89%

Right ventricle 40,7 90%

Pulmonary artery 42,18 mean 30 91%

Left ventricle 116,10 90%

Aorta 118,74 89%

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minimal cyanosis, no clubbing, and jugular venous pressure

was normal. Cardiac auscultation revealed a normal first

heart sound, wide spilt second sound, and a grade 3/6 ejection

systolic murmur along the left sternal border. Systemic arte-

rial saturation was 89%. Routine blood tests and biochemical

tests were within normal limits. An electrocardiogram

showed sinus rhythm and incomplete right bundle-branch

block. Chest X-ray revealed cardiomegaly with cardiotho-

racic ratio of 65%. Echocardiography revealed a dilated right

atrium and right ventricle, mild tricuspid regurgitation and

mild pulmonary arterial hypertension. A large ostium secun-

dum atrial septal defect with right-to-left shunt was present.

In the apical 4-chamber view venous confluence seen behind

left atrium. In subcostal examination venous confluence

appeared to be draining into a dilated proximal inferior vena

cava (Fig. 1, Videos 1 and 2).

Supplementary data related to this article can be found at

http://dx.doi.org/10.1016/j.jicc.2012.09.008.

The patient underwent cardiac catheterization, hemody-

namic and oximetry data are summarized in Table 1. Right

atrial, right ventricular, pulmonary arterial and systemic

arterial oxygen saturations were almost similar. Basal

systemic arterial saturation was 89%, saturation in proximal

IVC was 95%, Qp 9.8 L/minute, Qs 4.98 L/minute, Qp/Qs 1.96,

Pulmonary vascular resistance was 2 woods units, Systemic

vascular resistance was 17.2 woods unit. Selective cannula-

tion of all four pulmonary veins done through venous

confluence (Fig. 2). Pulmonary artery angiograms, in levo-

phase demonstrated the upper and lower pulmonary veins of

each side were seen to draining single common chamber,

however further drainage could not be delineated well. A 64-

slice helical contrast computed tomographic scan and three-

dimensional reconstruction done, volume-rendered image

clearly showed that the all four pulmonary veins made

a confluence which in turn, was seen joining the supra-

diaphragmatic part of inferior vena cava (Fig. 3) and same

finding confirmed with sequential reconstructed multiplanar

coronal image (Fig. 4). T1 weighted MRI high-resolution

sequential images showed same finding (Fig. 5).

Fig. 1 e Echo showing large ASD

4. Discussion

Total anomalous pulmonary venous connection is a rare

congenital anomaly, corresponding with approximately 2% of

all congenital heart defects.3 The commonly used Darling

classification for TAPVC consists of supracardiac, cardiac,

infracardiac, and mixed types. In infradiaphragmatic total

anomalous pulmonary venous connection, the pulmonary

veins coalesce to form a common chamber which is usually

situated directly behind the left atrium. From this common

chamber a single common pulmonary venous channel

descends through the diaphragm, most commonly through

the oesophageal hiatus and drains into either the portal vein,

ductus venosus, left hepatic vein, or rarely the inferior vena

cava, as in our case. Pulmonary venous obstruction is

common in TAPVC, and is especially serious in infracardiac

and mixed type. The mortality is as great as 90% if pulmonary

veins drain into the inferior vena cava or hepatic veins. The

natural history of TAPVC is unfavorable per se, with 50%

mortality in the first 3 months of life and a median survival of

2months, so thatmost infants will die of heart failure without

a surgical intervention.4

Duff et al studied twenty-eight cases of infradiaphragmatic

total anomalous pulmonary venous return, 17 were isolated

infradiaphragmatic total anomalous pulmonary venous

return, and 11 were with additional complex lesion.5

& common venous chamber.

Fig. 2 e Angiogram showing selective cannulation of all 4 pulmonary veins.

j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 2 ( 2 0 1 2 ) 1 6 0e1 6 4162

Anomalous site of connection was to the portal vein in 19

cases (68%), to the inferior vena cava in 4 (14%), the ductus

venosus in 2 (7%), to the left hepatic vein in 2 (7%), and

unknown in one. A patent foramen ovale was present in 60%

of cases and was frequently associated with a small left

atrium and left ventricle. The average age of the patients was

at the time of surgery was 20 days, with a range of 6 dayse8

weeks except one patient who was 10 years of age at the time

of diagnosis and surgical correction who had non-obstructed

TAPVC.6 Two cases of supradiaphragmatic TAPVC draining in

to IVC in children have been reported as similar to our case.7

Fig. 3 e CT with 3D rendered image shows common

chamber draining to IVC.

There is limited literature on the presentation and natural

history of unrepaired TAPVC beyond childhood and most of

the literature on the subject comprises isolated case reports.

However, pulmonary venous obstruction is encountered

frequently in patients with infracardiac TAPVC and for this

reason they usually develop severe pulmonary vascular

disease early in life and do not progress to adulthood.

Important factors which are responsible for survival in un-

operated patients with TAPVC beyond infancy and child-

hood are a large ASD and short route type drainage vein

without stenosis and a near normal pulmonary vascular

resistance.

In patients with the non-obstructive form, pulmonary

artery pressure is not always raised, being related to the

pulmonary flow and the response of the pulmonary vascular

bed. Though severe proliferative intimal changes in the small

pulmonary arteries have been described in infants with non-

obstructive total anomalous pulmonary venous return.8

Associated complex cardiac lesions have been noted in

20e30% of patients with total anomalous pulmonary venous

return.9 Splenic anomalies, especially asplenia, are common

in total anomalous pulmonary venous return when this is

associated with complex cardiac lesions.

The diagnosis of TAPVC in the adult can be easilymissed as

these patients are easily misdiagnosed as having a large

secundum atrial septal defect if the TAPVC is not suspected.

Assessment of operability can be difficult in these patients

because the systemic oxygen saturation may not be a true

guide to operability in the presence of a right-to-left shunt

through the ASD. However, an oxygen saturation of less than

80% in an unobstructed TAPVC should prompt cardiac cath-

eterization and a fixed PVRI of more than 8 U with no

Fig. 4 e Coronal multiplanar CT image show venous confluence draining to IVC.

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demonstrable fall on administration of 100% oxygen or

inhaled nitric oxide should probably contraindicate a repair.

Echocardiography is regarded as an initial screening and

diagnostic modality in patients with TAPVC. This technique

may not provide optimal definition of all four pulmonary

veins. In addition, it has a small field of view and is not always

available in certain planes because of the limitation of sonic

windows. CT angiography correctly depicts the drainage site

of the common pulmonary vein, stenosis of the vertical vein

and the course of the atypical vessel into the systemic vein in

case of vertical vein stenosis. 3D rendering and the multi-

planar images provide good anatomical details prior to

surgery. Cardiovascular magnetic resonance (CMR) imaging

plays an essential role in the evaluation and follow-up of adult

Fig. 5 e T1 weighted multiplanar sequentia

congenital heart disease (ACHD), providing safe, high-

resolution imaging of some of the most complex anatomies

encountered. Unlimited by acoustic windows and capable of

tissue characterization, CMR is devoid of ionizing radiation, do

not require sedation in adult patients, it provides superior

three-dimensional spatial resolution to transthoracic echo-

cardiography and superior temporal resolution to computed

tomography.10 Gadolinium-enhanced MR angiography (MRA)

is an extremely valuable tool in the evaluation of pulmonary

venous structures. This non-ECG-gated 3D spoiled GRE

imaging technique allows rapid dynamic imaging of thoracic

vascular structures, including during arterial and venous

(pulmonary and systemic) vascular phases. These images are

also typically reformatted in multiple planes as well as three-

l images show venous drainage to IVC.

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dimensionally reconstructed as either maximum-intensity

projection (MIP) or volume-rendered images.

In conclusion, we described a rare case of uncorrected non-

obstructive infracardiac TAPVC draining to IVC surviving up to

adulthood. This prolonged survival can be explained by the

large atrial septal defect and the absence of obstruction to

pulmonary venous return and with no associated cardiac

malformations. CT or CMR is a good additional diagnostic

modality for use in the preoperative evaluation of total

anomalous pulmonary venous connections.

Conflicts of interest

All authors have none to declare.

r e f e r e n c e s

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3. Bharati S, Lev M. Congenital anomalies of the pulmonaryveins. Cardiovasc Clin. 1973;5:23e41.

4. Vicente WV, Dias-da-Silva PS, Vicente Lde M, et al. Surgicalcorrection of total anomalous pulmonary venous drainage inan adult. Arq Bras Cardiol. 2006;87:e172ee175.

5. Duff DF, Nihill MR, McNamara DG. Infracardiac totalanomalous pulmonary venous return. Review of clinical andpathological findings and results of operation in 28 cases. BrHeart J. 1977;39:619e626.

6. Duff DF, Nihill NR, Vargo TA, Cooley DA. Infradiaphragmatictotal anomalous pulmonary venous return: diagnosis andsurgical repair in a 10-year-old child. Br Heart J.1975;75(37):1093e1096.

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