CASE REPORT

Direct Operating Room Triage of Neonates With TotalAnomalous Pulmonary Venous Connection

Jason Aguirre • Constantine Mavroudis •

Marshall Jacobs • Robert Stewart

Received: 1 June 2012 / Accepted: 25 June 2012

� Springer Science+Business Media, LLC 2012

Abstract Total anomalous pulmonary venous connection

with obstruction constitutes a surgical emergency. Medical

therapy is palliative and unlikely to result in significant or

sustained physiologic improvement. Two cases demon-

strate the successful use of a novel management scheme in

which patients are admitted directly to the operating room

for diagnosis confirmation and treatment, obviating the

need for time-consuming preoperative assessment in an

intensive care unit before surgery.

Keywords Congenital heart disease � Neonate �Preoperative care � Pulmonary veins

Introduction

Total anomalous pulmonary venous connection (TAPVC)

is diagnosed when findings show that all pulmonary veins

attach anomalously to systemic veins, the right atrium, or

the coronary sinus, representing 1.5–2 % of congenital

heart disease [3]. The presentation varies depending on the

site or sites of anomalous venous connection and the

degree of obstruction of the pulmonary venous return.

Neonates with significant obstruction generally present as

critically ill and require timely surgical intervention [4].

Management strategies vary among institutions. Most

critically ill patients with TAPVC receive care in an

intensive care unit (ICU) before surgery in hopes that

pharmacologic management, mechanical ventilatory sup-

port, or mechanical cardiorespiratory support with extra-

corporeal membrane oxygenation (ECMO) may improve

the clinical condition and the chances of a satisfactory

surgical outcome [4].

We believe that confidence in the diagnosis made at the

referring hospital and the critical physiologic condition can

be grounds for an alternative management scheme: direct

operating room triage (DORT).

We describe the course of two critically ill neonates

with obstructed TAPVC who were transferred to our

institution and admitted directly to the operating room

without preoperative assessment or stabilization in the

ICU.

Direct admission to the operating room facilitated rapid

execution of conventional cardiopulmonary bypass just

before or immediately after the diagnosis was confirmed by

echocardiography. Reliance on a thorough evaluation at the

referring institution saved precious time by ‘‘bypassing’’

the customary preoperative ICU phase. The duration of

circulatory and respiratory embarrassment and poor oxygen

delivery to vital end organs was minimized.

Case 1

A 4.1-kg male neonate was the product of an uncompli-

cated pregnancy and delivery. At several hours of age, the

patient exhibited sweating with feeds and respiratory dis-

tress. He was transferred to the neonatal ICU of the

J. Aguirre

Case Western Reserve University School of Medicine,

10900 Euclid Avenue, Cleveland, OH 44106, USA

C. Mavroudis (&)

Congenital Heart Institute, Florida Hospital for Children,

2501 North Orange Avenue, Suite 540, Orlando, FL 32804, USA

e-mail: Constantine.Mavroudis.MD@flhosp.org

M. Jacobs � R. Stewart

Department of Pediatric and Congenital Heart Surgery,

Cleveland Clinic, 9500 Euclid Avenue, Cleveland,

OH 44195, USA

123

Pediatr Cardiol

DOI 10.1007/s00246-012-0426-1

referring institution. Echocardiography suggested obstruc-

ted infracardiac TAPVC to a hepatic vein, atrial septal

defect, patent ductus arteriosus, and right ventricular dila-

tion. The boy was ventilated [arterial pH, 7.27, arterial

pressure of carbon dioxide (PaCO2), 39 mmHg, arterial

pressure of oxygen (PaO2), 44 mmHg], but metabolic

acidosis persisted despite medical therapy.

At 30 h of life, the boy was transferred to our institution

receiving prostaglandin and dopamine infusions. Direct

admission to the operating room had been anticipated and

planned. An operating room echocardiography confirmed

TAPVC, infradiaphragmatic type, obstructed.

Repair involved division of the vertical vein, anasto-

mosis of pulmonary vein confluence to the left atrium,

closure of the atrial septal defect, and ligation of the patent

ductus arteriosus. The bypass time was 1 h and 40 min.

The cross-clamp time was 34 min, and deep hypothermic

circulatory arrest was 26 min. An echocardiogram showed

satisfactory repair. The patient was discharged home in

good condition 2 weeks postoperatively.

Case 2

A 1-day-old female with a diagnosis of obstructed TAPVC

was transferred via helicopter. She was hypoxic from birth

and had been treated initially for presumptive pulmonary

hypertension (persistent fetal circulation). An echocardi-

ography at the referring facility showed most of the pul-

monary venous flow draining below the diaphragm with a

dilated portal system. One small pulmonary vein may have

had a supracardiac connection. No pulmonary veins were

seen entering the left atrium.

The girl was in critical condition, with a pH ranging

from 6.9 to 7.1 and a PaCO2 in the 70s–90s despite efforts

to ventilate. Direct operating room triage was anticipated

and planned. The PaO2 at arrival was 9 mmHg, and the

base excess was -13.

Transesophageal echocardiography was performed in

the operating room before bypass was initiated. A conflu-

ence of pulmonary veins was visualized behind the left

atrium, with no pulmonary veins entering the left atrium.

The confluence could be followed into a large channel in

the liver. Atrial communication appeared unrestrictive,

with right-to-left shunting and no other cardiac abnormal-

ities. Biventricular function was adequate.

Inspection confirmed subdiaphragmatic draining of all

pulmonary veins. Repair was accomplished as described

earlier. The bypass time was 1 h and 45 min, and the cross-

clamp time was 26 min.

Postoperatively, the girl was admitted to the ICU intu-

bated, with nitric oxide added to the ventilating gases. She

recovered well and went home 2 weeks postoperatively.

Discussion

Direct admission to the operating room of two critically ill

neonates with an echocardiographic diagnosis of obstructed

TAPVC provided the opportunity to confirm the diagnosis

expeditiously without delaying rapid surgical intervention.

Although it has been suggested that magnetic resonance

angiography or computed tomography angiography may

delineate the abnormal anatomy of TAPVC more precisely

than echocardiography, the value of routinely performing

such additional diagnostic studies can be questioned [8].

Cardiac catheterization can worsen existing pulmonary

edema in TAPVC patients [9]. For the most critical

patients, the time and mobilization required to accomplish

additional imaging studies may contribute to the overall

risk. Transthoracic echocardiography by a skilled examiner

often can be sufficient for making the decision to admit a

critically ill infant directly to the operating room if

obstructed TAPVC is observed.

In some instances, preoperative stabilization with

ECMO may improve outcomes for critically ill neonates

with congenital heart disease by providing time for car-

diopulmonary recovery and recovery of end organ func-

tion, allowing the patient to have surgery under more

favorable physiologic conditions [1, 3, 5]. Acidosis,

hypoxia, and pulmonary congestion are preoperative risk

factors commonly present in critically ill neonates with

obstructed TAPVC, which may increase perioperative

morbidity or mortality [5].

The patient in case 2 likely would have been managed

with ECMO support before surgical repair at some insti-

tutions. Direct operating room triage provides the option to

use ECMO cannulation in the operating room. We believe,

however, that rapid confirmation of the diagnosis, followed

immediately by establishment of conventional cardiopul-

monary bypass and surgical repair is a logical alternative.

Data from the Society of Thoracic Surgeons Congenital

Heart Surgery Database (2002–2010) show that 60 of 1,523

patients who underwent surgical repair of TAPVC had

preoperative mechanical circulatory support [7]. The

median age at the operation of patients with preoperative

support (4.5 days) was similar to that of patients without

such support (4 days). Hospital survival was 52 % for the

patients with preoperative support compared with 85 % for

those without the support. The median postoperative hos-

pital length of stay was longer for the cohort receiving

preoperative support (26 days) than for those not needing

preoperative support (14 days) [7]. The high mortality rate

among those with preoperative mechanical support justifies

evaluation of alternative strategies.

Systems of triage have been developed in various health

care settings. These valuable tools classify patients meeting

certain presenting criteria to determine the course of

Pediatr Cardiol

123

treatment likely to yield the most favorable outcomes.

Triage protocols that include direct transport of patients to

the operating room, particularly trauma victims, are

described in the literature [6]. We suggest that these sys-

tems can serve as a model for analogous use with critically

ill children suspected of having obstructed TAPVC.

Brown et al. [2] suggested that among congenital heart

disease patients referred for ECMO support, the degree of

preoperative critical illness is the most important predictor

of survival. These authors recommend the use of scoring

methods that consider physiologic variables known to be of

value in predicting the outcome for pediatric patients who

undergo surgical correction of their cardiac abnormality

[2].

We believe that an algorithm could be created for

patients with suspected obstructed TAPVC using objective

physiologic measures (vital signs, inotrope score, arterial

blood gases, serum lactate, chest X-ray findings) that can

be helpful in determining which patients may benefit most

from a DORT strategy.

Preliminary experience with the two reported patients

supports exploration of a triage strategy of DORT for

critically ill neonates who have a provisional diagnosis of

TAPVC with obstruction. Multi-institutional collaboration

would help to define an algorithm for selection of those

most likely to benefit.

Acknowledgments Jason Aguirre is funded by NIH Grant No. 5

T35 HL 82544-5.

References

1. Bautista-Hernandez V, Thiagarajan RR, Fynn-Thompson F et al

(2009) Preoperative extracorporeal membrane oxygenation as a

bridge to cardiac surgery in children with congenital heart disease.

Ann Thorac Surg 88:1306–1311

2. Brown KL, Miles F, Sullivan ID et al (2005) Outcome in neonates

with congenital heart disease referred for respiratory extracorpo-

real membrane oxygenation. Acta Paediatr 94:1280–1284

3. Emmel M, Sreeram N (2004) Total anomalous pulmonary vein

connection: diagnosis, management, and outcome. Curr Treat

Options Cardiovasc Med 6:423–429

4. Kanter KR (2006) Surgical repair of total anomalous pulmonary

venous connection. Semin Thorac Cardiovasc Surg Pediatr Card

Surg Annu 40–44

5. Kyser JP, Bengur AR, Siwik ES (2006) Preoperative palliation of

newborn obstructed total anomalous pulmonary venous connection

by endovascular stent placement. Catheter Cardiovasc Interv

67:473–476

6. Manka M, Moscati R, Raghavendran K, Priya A (2010) Sono-

graphic scoring for operating room triage in trauma. West J Emerg

Med 11:138–143

7. Society of Thoracic Surgeons (2011) http://www.sts.org/sites/defa

ult/files/documents/STSCONG-Spring2011.pdf. Accessed 8 July

2011

8. Ucar T, Fitoz S, Tutar E, Atalay S, Uysalel A (2008) Diagnostic

tools in the preoperative evaluation of children with anomalous

pulmonary venous connections. Int J Cardiovasc Imaging 24:

229–235

9. van Son JA, Hambsch J, Kinzel P, Haas GS, Mohr FW (2000)

Urgency of operation in infracardiac total anomalous pulmonary

venous connection. Ann Thorac Surg 70:128–130

Pediatr Cardiol

123