CASE REPORT

Total Anomalous Pulmonary Venous Drainage Complicatedby Tracheoesophageal Fistula

Toru Okamura • Mitsugi Nagashima •

Fumiaki Shikata • Takashi Higaki •

Eiichi Yamamoto • Masaaki Ohta • Hidemi Takata

Received: 7 March 2011 / Accepted: 17 May 2011 / Published online: 9 June 2011

� Springer Science+Business Media, LLC 2011

Abstract We provided emergency treatment to a 1-day-

old neonate (1600 g) with tracheoesophageal fistula (gross

classification, type C) and total anomalous pulmonary

venous drainage (infracardiac type) complicated by pul-

monary venous obstruction. Emergency surgery was

required because the tracheoesophageal fistula would have

caused respiratory failure. Here we report the perioperative

management techniques we used, including the surgical

strategy.

Keywords Total anomalous pulmonary venous

drainage � Tracheoesophageal fistula � Esophageal atresia

Total anomalous pulmonary venous drainage (TAPVD) is

an uncommon cardiac anomaly observed in 1.5–3.0% of

cases of congenital heart disease (CHD) [2]. In addition,

there have been only a few reports of its association with

esophageal atresia (EA) [1, 3, 7]. We present the case of a

patient with tracheoesophageal fistula (TEF) (esophageal

atresia, gross type C) and TAPVD complicated by pul-

monary venous obstruction (PVO).

Case Report

A female neonate weighing 1600 g was born after a

cesarean section at 38 weeks’ gestation. She was diagnosed

with Goldenhar’s syndrome. The 5-min Apgar score was 5,

and she presented with cyanosis and respiratory failure.

Echocardiogram showed TAPVD, and chest roentgeno-

gram showed coil-up sign and pulmonary congestion. She

was admitted to our hospital for medical treatment.

Esophagus atresia (gross type C) was diagnosed on the

basis of the coil-up sign and stomach gas. Chest roent-

genogram taken on admission also showed severe pul-

monary congestion and dextrocardia. Echocardiogram

showed infracardiac-type TAPVD (Fig. 1a, b).

An urgent surgery involving two-stage repair was planned

for the TAPVD (infracardiac type) with PVO and EA (gross

type C). TAPVD repair was performed after gastrostomy and

insertion of a peritoneal dialysis catheter, which were per-

formed to facilitate postoperative medical treatment and

ventilation. Standard cardiopulmonary bypass (CPB) was

performed, wherein the CPB was established at the

ascending aorta and bicaval cannulations. After ligation

of the patent ductus arteriosus, blood temperature was

decreased to 20�C for dissection of the vertical vein. The

vertical vein and common pulmonary chamber were dis-

sected by carefully retracting the tiny heart. Cardioplegia

was infused through the ascending aorta after cross-clamp-

ing it, and the right atrium was opened. The point where the

diaphragm was pierced was ligated with 6-0 Prolene suture.

The vertical vein was divided and filleted proximally to the

level of the superior pulmonary veins. The left atrium was

opened obliquely as a suture line of the vertical vein through

the posterior approach. Continuous anastomosis of the left

atrium and vertical vein was performed by using 7-0 Prolene

suture under low-flow bypass (10–20 ml kg-1 min-1). The

T. Okamura (&) � M. Nagashima � F. Shikata

Department of Cardiovascular Surgery, School of Medicine,

Ehime University, Shitsukawa, Toon city, Ehime 791-0295,

Japan

e-mail: tokamura@hotmail.co.jp

T. Higaki � E. Yamamoto � M. Ohta � H. Takata

Department of Pediatrics, School of Medicine,

Ehime University, Shitsukawa, Toon city,

Ehime 791-0295, Japan

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Pediatr Cardiol (2011) 32:983–985

DOI 10.1007/s00246-011-0011-z

atrial septal defect was closed directly, and the right atrium

was closed. The heart was de-aired, and the patient was re-

warmed and weaned off the CPB uneventfully. The total

ischemic time was 43 minutes, and the CPB time was 123

min. After surgery, the patient, whose sternum still remained

open, was transferred to the neonatal intensive care unit.

Because of the preoperative PVO, our patient took a longer

time to recover from pulmonary hypertension; she was

administered midazolam, fentanyl, and rocuronium bromide

to ensure continuous sedation. The sternum was closed

uneventfully 5 days after surgery. Respiratory care was

maintained to prevent ventilation failure due to TEF. A

gastrostomy tube was inserted to continuously draw out the

gastric air during positive ventilation to prevent abdominal

dilatation. Three-dimensional computed tomography was

performed to evaluate the TEF (Fig. 2a–c). Four weeks after

the cardiac surgery, right thoracotomy was performed to

correct the EA and TEF. The fistula was divided, and direct

esophageal anastomosis was performed. Respiratory care

was carefully performed after surgery because bronchoma-

lacia was suspected on the basis of the surgical findings. The

patient’s hemodynamic status was stable; however, respi-

ration was unstable because of the bronchomalacia. Our

patient gradually recovered in the neonatal intensive care

unit. She was weaned off the ventilator 2 months after right

thoracotomy. She is currently in good health, and her weight

has increased to 3800 g.

Discussion

EA occurs in approximately 1/3000–4500 live births [1].

The incidence of EA, which ranges between 13.2 and 41%,

is comparable with most other CHDs that have been

reported [3, 5, 7]. Spitz et al. [8] previously proposed a

prognostic classification based on body weight ([1500

or \1500 g) and the presence or absence of major CHD

(MCHD). According to this classification, EA patients were

divided into three groups: group I (weight [ 1500 g and

without MCHD), group II (weight \ 1500 g or with

MCHD), and group III (weight \ 1500 g and with MCHD).

The mortality rate has been reported to be \10% in group

I, \50% in group II, and approximately 80% in group III

[3, 5, 7]. Recently, Lopez et al., who worked with Spitz et al.,

reanalyzed the outcomes of EA surgery. They reported a

decrease in the mortality rate of groups II and III and an

improvement in the diagnosis of congenital anomalies in the

fetus and their corresponding prenatal and postnatal man-

agement in recent decades [6]. Encinas et al. showed that

accurate diagnosis of CHD before EA repair had no signif-

icant influence on the results, except in one case in which the

patient’s hemodynamics were unstable [4].

In our case, the patient’s body weight was 1600 g;

however, she had MCHD, which needed immediate repair

for the PVO. Moreover, dextrocardia was also observed.

According to Spitz’s classification, our patient belonged to

group II, and the case was severe enough to warrant

medical treatment. There are only a few reports on such

complications, and we could not find any case of a patient

who presented with PVO immediately after birth.

We believed that it would be difficult for our patient to

receive aggressive respiratory and hemodynamic treatment

after one-stage surgery; therefore, two-stage surgery, con-

sisting of initial TAPVD repair and then EA repair, was

performed. Gastrostomy was essential after the first surgery

to continuously draw the gastric air out during positive

Fig. 1 a Chest roentgenogram.

The black arrow indicates the

coil-up sign, and the whitearrows indicate gastric air.

b Two-dimensional

echocardiogram. The

pulmonary venous chamber is

not connected to the left atrium

(LA)

984 Pediatr Cardiol (2011) 32:983–985

123

ventilation to prevent abdominal dilatation due to air

leakage through the TEF. Continuous air drainage after

gastrostomy was effective for respiratory management

after heart surgery. The gastrostomy also proved to be

useful for tube feeding after EA repair. We performed the

surgeries carefully and therefore did not encounter pul-

monary and esophageal complications, aspiration pneu-

monia, anastomotic leakage, or wound infection. Accurate

and precise surgical management, in addition to integrated

treatment, were essential for success.

Conclusion

We successfully treated a neonate (1600 g) with TEF

(gross classification, type C) and TAPVD (infracardiac

type) complicated by PVO by way of two-stage surgery.

However, this is a severe and complex complication, and

rapid medical treatment is needed in such cases. Such

patients do not always survive; therefore, prenatal diag-

nosis is important.

References

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Fig. 2 a, b CT scans of the

frontal sections of the

esophagus. The black arrowindicates the esophageal fistula,

and the white arrow indicates

the end of the esophagus with

air and the esophageal tube.

c Three-dimensional CT scan of

the esophagus. The white arrowindicates the end of the

esophagus, and the black arrowindicates the tracheal fistula

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