Pediatric Interventions

Hypoplastic Left Heart Syndrome With IntactAtrial Septum: Levoatriocardinal Vein Stent

Placement as a Bridge to Surgery

Michael S. Vance,* MD

Infants with hypoplastic left heart syndrome, an intact atrial septum and pulmonaryvenous hypertension, are critically ill and have a poor prognosis. This case describesrelief of severe pulmonary edema in such a patient by stent placement in a stenoticlevoatriocardinal vein, with subsequent successful surgical palliation. Cathet CardiovascIntervent 2002;57:85–87. © 2002 Wiley-Liss, Inc.

Key words: hypoplastic left heart syndrome; levoatriocardinal vein; stent

INTRODUCTION

In typical hypoplastic left heart syndrome (HLHS),there is atresia of both the mitral and aortic valves.Pulmonary venous blood entering the left atrium requiresan interatrial communication to exit the heart. An intactatrial septum (IAS) is a rare finding in HLHS, occurringin only 1% of pathology specimens [1]. Infants withHLHS and IAS frequently have unusual left atrial de-compressive pathways that allow egress of blood fromthe left atrium to systemic veins. The most commondecompressive pathway is the levoatriocardinal vein;however, it is often obstructed [1,2]. Infants with HLHS,IAS, and obstructed left atrial decompressive pathwayshave inadequate left atrial drainage and develop severepulmonary edema and cyanosis.

Pediatric cardiologists have struggled to open the atrialseptum in HLHS with IAS and obstructed left atrialdecompressive pathways. We present a case in whicheffective left atrial decompression was achieved by stent-ing an obstructed levoatriocardinal vein.

CASE REPORT

A 3.2 kg term male infant was born by elective repeatcaesarian section with Apgar scores of 8 at 1 min and 9at 5 min. The patient developed cyanosis and respiratorydistress on day 1 of life requiring intubation. A chestX-ray demonstrated severe pulmonary edema (Fig. 1).Echocardiography demonstrated HLHS (mitral atresia/aortic atresia) with an intact atrial septum. The echocar-diogram also demonstrated a vertical vein draining to the

superior vena cava (SVC); it was unclear whether thevertical vein originated from the left atrium or was ananomalous pulmonary venous connection. After startingthe patient on prostaglandin E1, the patient was taken tothe catheterization laboratory with the goal of decom-pressing his left atrium. Angiography demonstrated thevertical vein to be a levoatriocardinal vein decompress-ing the left atrium to the SVC. The levoatriocardinal veinwas compressed to a diameter of 1–2 mm as it coursedbetween the left main-stem bronchus and the arch of thepatent ductus arteriosus (Fig. 2). A 6 Fr guide catheterwas advanced from the right femoral vein to thelevoatriocardinal vein and across the stenosis, throughwhich a Multi-Link Ultra coronary stent was delivered.The stent was dilated to 5.5 mm to conform with thediameter of the levoatriocardinal vein at its junction withthe SVC. Follow-up angiography demonstrated the pre-viously stenotic area to be widely patent (Fig. 3). Therewas no compression of the left main-stem bronchus or

Division of Pediatric Cardiology, Children’s Hospital of theKing’s Daughters, Norfolk, Virginia

*Correspondence to: Dr. Michael S. Vance, Pediatric Cardiology,Children’s Hospital of the King’s Daughters, 601 Children’s Lane,Norfolk, VA 23507. E-mail: vancems@chkd.com

Received 18 January 2002; Revision accepted 9 April 2002

DOI 10.1002/ccd.10273Published online in Wiley InterScience (www.interscience.wiley.com).

Catheterization and Cardiovascular Interventions 57:85–87 (2002)

© 2002 Wiley-Liss, Inc.

the patent ductus arteriosus by the stent. Fluoroscopytime was 32 min.

Repeat chest X-rays demonstrated resolution of thepatient’s pulmonary edema (Fig. 4). In the 48 hr follow-ing stent placement, ventilator inspiratory pressure wasreduced from 40 cm H2O to 17 cm H2O, ventilator ratewas reduced from 60 to 18 breaths per minute, andinspired O2 was reduced from 100% to 21%. The patientunderwent successful Norwood I at 7 days of age, withremoval of the stent at surgery, and was discharged homeat day 22.

DISCUSSION

Infants with HLHS and severe obstruction to pulmo-nary venous return are critically ill, with pulmonaryedema and cyanosis. Obstructed pulmonary venous re-turn secondary to a restrictive or intact atrial septum hasbeen identified as predictive of poor surgical outcome ininfants with HLHS [3,4]. In the largest series lookingspecifically at infants with HLHS and IAS, Rychik et al.[1] reported attempting primary Norwood repair in sixpatients with obstructed decompressive pathways, withno survivors.

Given the poor surgical options, pediatric cardiologistshave attempted to decompress the left atrium in thecatheterization laboratory. Creating an interatrial com-munication by blade septectomy is difficult and has beenassociated with significant mortality [1,5]. While Atz etal. [5] have reported success with Brockenbrough transa-trial puncture and serial balloon dilations of the newASD, Kuhn et al. [6] have reported significant mortalitywith this approach.

The initial intent of the catheterization was to open theatrial septum by Brockenbrough puncture. After demon-strating the presence of a stenotic levoatriocardinal vein,we were presented with the additional options of tryingto dilate or stent the vessel to achieve left atrial decom-pression. Angiography demonstrated that the stenosis

Fig. 1. Chest X-ray at 6 hr of age.

Fig. 2. AP projection, digital subtraction angiogram prior tostent placement. A 4 Fr catheter has been advanced from thefemoral vein to a right pulmonary vein. LA, left atrium.

Fig. 3. AP projection, digital subtraction angiogram followingstent placement.

86 Vance

was secondary to compression of the levoatriocardinalvein between the left main-stem bronchus and patentductus arteriosus. Stenting the vessel seemed more likelyto relieve stenosis caused by external compression. Thestentotic levoatriocardinal vein was easily accessible andproved amenable to stenting, with excellent relief of leftatrial hypertension as demonstrated by the prompt reso-lution of pulmonary edema and clinical stabilization.This allowed the patient to undergo nonemergent sur-gery, which was successful.

In conclusion, stenting an obstructed levoatriocardinalvein in the setting of HLHS and IAS was effective in thepresent case and may offer a reasonable alternative toBrockenbrough puncture in this difficult population ofpatients.

REFERENCES

1. Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL. Thehypoplastic left heart syndrome with intact atrial septum: atrialmorphology, pulmonary vascular histopathology and outcome.J Am Coll Cardiol 1999;34:554–560.

2. Seliem MA, Chin AJ, Norwood WI. Patterns of pulmonary venousconnection/drainage in hypoplastic left heart syndrome: diagnosticrole of doppler color flow mapping and surgical implications. J AmColl Cardiol 1992;19:135–141.

3. Bove EL, Lloyd TR. Staged reconstruction for hypoplastic leftheart syndrome. Contemporary results. Ann Surg 1996;224:386–394.

4. Daebritz SH, Nollert GD, Zurakowski D, Khalil PN, Lang P, delNido PJ, Mayer JE, Jonas RA. Results of Norwood stage I opera-tion: comparison of hypoplastic left heart syndrome with othermalformations. J Thorac Cardiovasc Surg 2000;119:358–367.

5. Atz AM, Feinstein JA, Perry SB, Wessel DL. Preoperative man-agement of pulmonary venous hypertension in hypoplastic leftheart syndrome with restrictive atrial septal defect. Am J Cardiol1999;83:1224–1228.

6. Kuhn MA, Larsen RL, Mulla NF, Johnston JK, Chinnock RE,Bailey LL. Outcome of infants with hypoplastic left heart syndromewho undergo atrial septostomy before heart transplantation. Am JCardiol 2000;85:124–127.

Fig. 4. Chest X-ray 12 hr following stent placement.

Levoatriocardinal Vein Stent 87