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Total-Anomalous-Pulmonary-Venous-Connection: Management and Outcome,. Mohammad Asim Khan and Tariq
Waqar
Original Article
Total-Anomalous-Pulmonary-VenousConnection: Management and Outcome,
Experience from Children’s Hospital, &
Ittefaq Hospital, Lahore, Pakistan
Objective; To determine factors contributing to mortality among patients of TAPVC
undergoing surgical repair at Children Hospital, Lahore.
Study Design: Descriptive case series.
Place and Duration of the Study: September 2004 to October 2009
Materials and Methods: All patients of either gender who had TAPVC and unde went
surgical repair during study period were included.
Results: In the last 4.5 years, 44 patients (29 males & 15 females) underwent repair for
total anomalous pulmonary venous connection. Ages ranged from 2 day to 9 years (median
6 & mean 17 months) and 26 of them were less than 1 year. Weight ranged from 2.4 to 20 kg
(median 5 kg). The anomalous connection was supracardiac in 21 (48%), cardiac in 10
(23%), infracardiac in 5 (11%) and mixed in 8 (18%) patients. Fifteen (34%) patients had
obstructed drainage and 21 (48%) had moderate or severe pulmonary arterial hypertension.
15 patients (34%) had to be operated upon on an emergency basis. Mortality was more in
obstructed drainage patients (40%),compared with patients without obstruction (6%).The
major causes of early death were weight < 10%ile (OR 1.1; 95% CI: 0.1-6.5, p-0.009),
obstruction (OR 9.8; 95% CI: 1.6-60, p-0.006) and sepsis (OR 23.3; 95% CI: 3 – 177, p-0.002).
Follow-up ranged from 1 to 45 months (median 24 months). There was one late death due to
late pulmonary vein stenosis and was re-operated upon and died.
Conclusions: In a developing country like Pakistan, mortality continues to be high in
infants with total anomalous pulmonary venous connection. Weight < 10 percentile,
obstruction, severe pulmonary arterial hypertension and post operative sepsis appears to
be the most important predictor of operative mortality.
Keywords: Total-anomalous, Pulmonary-venous connection, Surgical repair, Mortality.
Introduction
Total anomalous pulmonary venous connection
(TAPVC) is a rare clinical entity and represents less
than 2% of all congenital heart diseases. It is a group of
anomalies where pulmonary veins connect directly to
the systemic veins. The resulting clinical picture
depends upon the shunting between left and right heart
as well as degree of obstruction to the pulmonary
venous drainage.1 The anomaly is divided into four
anatomical categories namely supra-cardiac (45%)
cardiac (25%) infra-cardiac (25%) and mixed (5%)2.
Treatment is surgical correction, which in cases of
obstruction becomes a true emergency.1 Over time
there has been a steady improvement in surgical results
due to improvements in surgical technique and perioperative management of pulmonary hypertension.3
Ann. Pak. Inst. Med. Sci. 2012; 8(3): 196-199
Mohammad Asim Khan*
Tariq Waqar *
*Assistant Professor
Pediatric Cardiac Surgery
The Children’s Hospital &
Institute of Child Health, Lahore
Address for Correspondence
Dr. Mohammad Asim Khan
Pediatric Cardiac Surgery
The Children’s Hospital & Institute
of Child Health, Lahore, Email:
[email protected]
Presently the surgical mortality is less than 5% at
specialized centers in developed world.4 Mortality is
associated with the severity of obstruction in pulmonary
venous drainage5, the age at presentation (older the age
less favorable the outcome), the presence of pulmonary
hypertension and associated cardiac anomalies, and in
developing countries malnutrition and sepsis are
associated with increased mortality and morbidity.
A retrospective analysis of the demographic,
morphological and clinical profiles of patients who under
went operative repair for total anomalous pulmonary
venous connection, was undertaken to determine the
factors contributing to mortality in our set up.
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Total-Anomalous-Pulmonary-Venous-Connection: Management and Outcome,. Mohammad Asim Khan and Tariq
Waqar
Materials and Methods
All patients diagnosed as having TAPVC who were
operated with complete repair whether emergency or
elective are included in this review.
All pre-operative, operative and postoperative data was
collected prospectively and analyzed using SPSS
version 15. Categorical variables were expressed as
percentage frequencies and continuous variables as
mean ± standard deviation or as median. Morbidity and
mortality expressed as percentage.
All the patients were diagnosed on chocardiography to
have TAPVC. After diagnosis the patients were
resuscitated if required which included correction of
acidosis, oxygenation and sepsis. Surgery was
performed as a priority in patients with an element of
obstruction to the pulmonary venous drainage.
Surgical strategy included median sternotomy and
initiation of cardiopulmonary bypass. For supracardiac
lesions, the patients were cooled down to a moderate
hypothermia (lowest range was 280 C). While cooling the
posterior pericardium was dissected to visualize the
pulmonary veins and the communicating vertical vein.
The aorta was cross clamped and antegrade cold
crystalloid cardioplegia was given. Our surgical strategy
is trans-atrial approach for repair of these lesions. The
transverse right atriotomy was performed crossing the
plane of inter atrial septum at right angles, the incision
was continued through the foramen ovale onto the
posterior wall of the left atrium, extending into left atrial
appendage to increase the size of anastomosis. A
corresponding incision was made in the common
venous confluence and anastomosis fashioned between
the left atrium and the venous chamber. Additionally left
atrial enlargement was done by closing interatrial
septum with autologous fresh pericardium, leaving a
small size ~4 mm fenestration as a rescue in pulmonary
hypertension crisis. Vertical vein was ligated in all the
patients after initiation of bypass.
For intra-cardiac defects involving the coronary sinus,
atrium was opened in a conventional vertical plane. The
coronary sinus was unroofed and connected to the
foramen ovale. The resultant defect in the atrial septum
was then closed using autologous fresh pericardium so
that coronary sinus along with the pulmonary veins
would drain into the left atrium.
For infra-cardiac TAPVC, surgical approach was to
perform the anastomosis between the common venous
confluence and the left atrium by retracting the apex of
the heart anteriorly and upwards. This technique
required brief periods of low flow to facilitate a blood
less field during the anastomosis. A combination of
techniques was used for mixed lesions.
Cardiopulmonary bypass was terminated after rewarming, de-airing and a period of reperfusion.
Epicardial pacing wires were attached to the atrium and
Ann. Pak. Inst. Med. Sci. 2012; 8(3): 196-199
ventricle. In neonates we inserted LA pressure
monitoring lines, whereas in older patients direct
pulmonary pressures were monitored. We have a low
threshold for keeping the sternum open after surgery if
hemodynamic instability or uncontrolled bleeding is
present. Echo was done immediately after surgery in the
CICU, then on the first post op day, after chest closure
and before discharge from the Hospital.
Results
The demographic and clinical profile of the patients is
shown in table I.
Table I : Demographic and Clinical Profile of the
patients (n=44)
Variable
No./Range
frequency/
Gender
mean/
median
Male
29
65.90%
Female
15
35.10%
Age
2 days-9 yrs 6 months
Weight (Kgs)
2.4-20
5
Mode of presentation
Urgent
15
34%
Elective
29
66%
Presenting complaint
Cyanosis
20
46%
Low Cardiac output
10
22%
PHT crisis
6
14%
Congestive heart failure 5
11%
Asymptomatic
3
7%
Obstructed lesion
Type of lesion
15
34%
Supra-cardiac
22
50%
Cardiac
9
20%
Infra Cardiac
5
12%
Mixed
8
18%
Ten patients had established pre-operative sepsis. The
mean cardiopulmonary bypass time was 90.54±17
minutes and mean aortic cross clamp time was
40.15±15.91 minutes. Total correction was done in all
the patients. For mixed lesions a combination of the
above mentioned techniques were used.
8 patients were shifted to the ICU with open chest. The
mean duration of keeping the chest open was 1.82
days. Ionotropic support was required in all the patients
and all those who were discharged received treatment
for congestive heart failure. Postoperative ICU stay was
3.31±2.1 days (median 3 days), the range being 2-6
days. The frequency of prolonged ventilation (more than
72 hrs) was 23%, 5 patients (11.4%) acquired post-
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Total-Anomalous-Pulmonary-Venous-Connection: Management and Outcome,. Mohammad Asim Khan and Tariq
Waqar
operative sepsis, 8 (18.18%) had pulmonary
hypertensive crisis. Overall mortality was 18%. It was
was much higher in those with obstructed pulmonary
venous drainage (40%), compared with patients without
obstruction (6%).The major causes of early death were
weight < 10%ile (OR 1.1; 95% CI: 0.1-6.5, p-0.009),
obstruction (OR 9.8; 95% CI: 1.6-60, p-0.006),
pulmonary hypertension (OR 4.5; 96% CI:1.9-9.5, p0.004) and sepsis (OR 23.3; 95% CI: 3 – 177, p-0.002).
Follow-up ranged from 1 to 45 months (median 24
months). One patient presented with delayed pulmonary
vein stenosis and was re-operated but could not survive
thereafter.
Discussion
In this small case series we have reviewed our results
for complete repair of Total anomalous pulmonary
venous connection. TAPVC is a rare congenital heart
defect 1,2 left untreated 80% of the patients die in first
year of life.6 However it is not unusual to find an
occasional patient surviving into adulthood especially in
a developing country where antenatal screening and
routine checkup is done infrequently. The factors
favoring survival into adulthood are a large ASD and
non obstructed drainage through a short route. 7
The treatment is surgical correction; the operative
results have been improving over the past decades, in a
single center experience from 1946 to 2005 the authors
have shown that mortality was about 40% in 1970s
however it has declined to about <5% in recent years3.
Important factors responsible for improved surgical
outcome were development of hypothermic circulatory
arrest and improved anastomosis techniques as well as
early recognition and adequate resuscitation.
Historically the risk factors for operative mortality were
young age, anatomical type, pulmonary artery
hypertension, presence of obstruction to the venous
drainage, metabolic acidosis and urgency of operation 8.
However In recent series presence or absence of
obstruction at any site in the course of the draining vein
has been correlated with adverse out come 5 technique
of sutureless repair has been shown to improve the
outcome in patients having obstruction (stenosis) of the
pulmonary veins.9 10 Pulmonary hypertension resulting
from recurrent venous obstruction after initial surgical
repair has been reported to be 5-15%. 11 This has
shown to affect the long term outcome. Mortality of
reoperation for obstruction of pulmonary venous
drainage is up to 50%.12 Similarly the operative mortality
is high if the condition is associated with a single
ventricle physiology.13
Post operatively pulmonary artery hypertension is
responsible for mortality and in part is attributed to small
size of the left atrium as well as pulmonary vascular
disease. Various methods to counter the crises are
keeping a patent foramen ovale, valved closure of ASD,
Ann. Pak. Inst. Med. Sci. 2012; 8(3): 196-199
and keeping the vertical vein patent.14, 15 In our study we
kept a small patent foramen ovale as a counter in
patients with preoperative obstruction or high pulmonary
arterial pressures.
Traditionally the improvement in surgical results were
achieved with deep hypothermic circulatory arrest to
facilitate the anastomosis but in our experience the
technique of biatrial cannulation and trans-atrial repair
along with moderate hypothermia produced clear
surgical field and facilitated unhindered construction of
the anastomosis, hence offsetting the requirement of
hypothermic circulatory arrest. These findings are
supported by other contemporary series.3, 12
In patients with an evidence of obstruction or excessive
postoperative bleeding chest was kept open after
surgery with delayed primary closure performed
whenever feasible. Literature review has shown that
keeping the chest wound open after selective
procedures is helpful in offsetting the effects of tissue
temponade resulting from increased tissue edema as
well as bleeding related problems. Samir et al16
reporting a case series on infants who underwent open
heart surgery found delayed sternal closure as life
saving in infants and identified age less than 7 days,
prolonged bypass and cross clamp time as well as
diagnosis of Interrupted Aortic Arch and Total
anomalous pulmonary venous drainage as factors
associated with it .
In contrast to the western reports4, we had a high
operative mortality (18%), especially in obstructed group
(40%). Another study from India7 also reported very
high operative mortality (33.3%). In our experience,
obstruction, weight < 10%ile; sepsis and pulmonary
hypertension were the most important predictors for
operative mortality. When taken together, patients with
these risk factors represent a group of very sick infants
and require emergency surgery.
In another study reported from India 73 patients were
operated between January 1987 to October 1992;
overall operative mortality was 23.35 %; pulmonary
hypertensive crises being responsible for more than
60% of deaths.4 Risk factors for mortality were emergent
nature of the operation and weight less than 25th
percentile and it was concluded that operative mortality
is high in a developing setup.17
Other limitation is absence of long term follow up to
know the development of late obstruction due to short
time span of the study however one of the patient in
post operative follow up has shown signs of late left
lower pulmonary venous obstruction. He was reoperated abroad and died.
Conclusion
In a developing country like Pakistan, mortality
continues to be high in infants with total anomalous
pulmonary venous connection. Weight < 10 percentile,
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Total-Anomalous-Pulmonary-Venous-Connection: Management and Outcome,. Mohammad Asim Khan and Tariq
Waqar
obstruction, severe pulmonary arterial hypertension and
post operative sepsis appears to be the most important
predictor of operative mortality.
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