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Ann Thorac Surg 2003;75:277-279
© 2003 The Society of Thoracic Surgeons

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Case report

Bilateral pulmonary artery banding for resuscitation in hypoplastic left heart syndrome

^a Division of Pediatric Cardiovascular Surgery, Section of Cardiac Surgery, C. S. Mott Children’s Hospital, University of Michigan School of Medicine, Ann Arbor, Michigan, USA

Accepted for publication August 14, 2002.

* Address reprint requests to Dr Ishizaka, Pediatric Cardiovascular Surgery, F7830 C. S. Mott Children’s Hospital, 1500 East Medical Center Dr, Ann Arbor, MI 48109, USA
e-mail: tishizaka{at}aol.com

	Abstract

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We report a case of a hypoplastic left heart syndrome with a nearly intact atrial septum and an obstructed anomalous pulmonary to systemic venous connection. Surgical atrial septectomy followed by bilateral pulmonary artery banding provided an optimal condition for the Norwood operation.

	Introduction

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Despite continued improvements in the results of first-stage palliation for hypoplastic left heart syndrome (HLHS), pulmonary venous obstruction is associated with a significantly higher risk of mortality [1]. This report describes the successful management of a neonate with HLHS associated with a severely restrictive atrial septum and an obstructed anomalous pulmonary to systemic venous connection between the left atrium and the innominate vein.

A 3.4-kg male infant prenatally diagnosed with HLHS was delivered after 37 weeks’ gestation. Prostaglandin E1 infusion was begun and metabolic status corrected. Chest roentgenogram after intubation at 6 hours of age showed dextrocardia with mild cardiomegaly and significant pulmonary venous congestion (Fig 1A). The arterial blood gas under controlled ventilation with an FiO₂ of 30% showed paO₂ of 27 mm Hg and pCO₂ of 40 mm Hg. Despite an adequate arterial blood gas the patient was thought to be a poor candidate for proceeding directly to a Norwood procedure owing to the severe pulmonary congestion on the roentgenogram. Two-dimensional echocardiography revealed situs solitus and dextrocardia with mitral and aortic atresia. An extremely restrictive high atrial defect was detected only by color Doppler as a left to right shunt flow of 1 mm width. Two right and one left pulmonary vein were seen joining a small confluence, which then joined a small left atrium. A large vertical vein from the left atrium joined a dilated innominate vein. At its entrance to the innominate vein an obstruction with a mean gradient of 10 mm Hg was observed (Fig 2).

View larger version (61K): [in this window] [in a new window]   Fig 1. (A) Chest roentgenogram taken 6 hours after birth shows dextroposition of the heart and marked pulmonary venous congestion. (B) After septectomy significant improvement of pulmonary venous congestion was noted but reticular shadows due to pulmonary overcirculation as well as marked edema of the chest wall were seen. (C) Seven days after bilateral pulmonary artery banding, chest roentgenogram demonstrates improvement in both lung fields and chest wall edema.

View larger version (85K): [in this window] [in a new window]   Fig 2. Doppler echocardiography demonstrates the anomalous vein from the left atrium (LA) to the innominate vein and a severe stenosis at its entrance to the innominate vein.

View larger version (42K): [in this window] [in a new window]   Fig 3. Schematic representation of the heart. (LA = left atrium.)

Because the patient had pulmonary edema on chest roentgenogram and poor end-organ perfusion despite good ventricular function and no tricuspid valve regurgitation on echocardiography, we believed there was excessive pulmonary blood flow. On day 5 of life he underwent bilateral pulmonary artery banding as a lifesaving procedure. The diameters of the right and left pulmonary arteries were measured at 6 mm and 4 mm, respectively. A 4-mm dilator on the right and a 3-mm dilator on the left were placed adjacent to each branch and heavy silk ligatures were tied down over both the dilators and pulmonary arteries. After the removal of the dilators the right pulmonary artery was cinched down to 3.5 mm in diameter by placing an additional 6-0 Prolene (Ethicon, Somerville, NJ) suture through the silk band. That resulted in an increase in the systolic blood pressure of 15 to 20 mm Hg. The effectiveness of this procedure was dramatic. The patient was easily weaned from inotropic support and began making urine within 12 hours. Chest roentgenogram on the seventh postoperative day after banding showed significant improvement in pulmonary congestion and chest wall edema (Fig 1C). On day 14 of life a Norwood operation with a 3.5-mm Gore-Tex (W.L. Gore & Assoc, Naperville, IL) shunt was performed. The patient was weaned from cardiopulmonary bypass without difficulty and remained hemodynamically stable on mild inotropic support. The chest was left open to avoid compression of the heart and was uneventfully closed 9 days later. The patient was extubated after 16 days and discharged in good condition. He remains well and is awaiting a hemi-Fontan operation.

	Comment

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Patients with HLHS and intact or nearly intact atrial septum deteriorate rapidly after birth owing to severe pulmonary venous obstruction. Variations of the Norwood procedure, including the use of a larger shunt [2], have failed to overcome the severe acute postoperative hypoxemia. Rychik and colleagues [3] reported in their series of patients with HLHS and intact atrial septum that 6 of 18 survived the Norwood procedure. However, none of the 7 patients who had an obstructed decompression pathway, such as the patient in this report, survived initial intervention. We selected surgical septectomy as an initial procedure because the atrial septum was too thick for balloon atrial septostomy and the left atrium too small for safe blade atrial septostomy. In addition the patient underwent initial septectomy alone to allow resolution of the pulmonary edema before undergoing the Norwood procedure. The open septectomy resulted in adequate decompression of the left atrium but intractable pulmonary overcirculation rapidly followed [4].

The small size of the neonatal branch pulmonary arteries and precarious balance of the pulmonary and systemic vascular resistances present a challenge to achieving an optimal outcome for bilateral pulmonary artery banding. It has been previously reported in patients with truncus arteriosus that the constriction of each pulmonary artery to one third of its diameter resulted in normal distal pulmonary artery pressure [5]. We selected a more conservative estimate, adjusting each band to gain a moderate (10 to 15 mm Hg) increase in systemic pressure. This was accomplished with a reduction of the diameter of approximately 30%. The effectiveness of the procedure in our patient suggests that it is applicable to other patients who have intractable pulmonary overcirculation and also are at prohibitive risk for a Norwood procedure.

Edwards and associates [6] first described an anomalous pulmonary to systemic venous connection between the LA and a systemic vein as a "levoatriocardinal vein." The anomalous vein observed in our case is a different entity than that described by Edwards because the left upper pulmonary veins connected to this vein [7]. In addition the hemiazygos-like connection in our case, as is seen with the left-sided superior vena cava, has not been previously described among the reports of levoatriocardinal veins [8]. The recognition of such a connection at operation is important because persistence of a collateral systemic vein may become a potential source of cyanosis after the completion of a cavopulmonary operation.

Although the patient is now doing well and awaiting the next stage of surgical reconstuction close followup remains mandatory and the long-term outcome is uncertain. Even after the hemi-Fontan or Fontan operation further deterioration has been reported in this patient population due to a pulmonary vasculopathy caused by the in utero pulmonary venous obstruction [3].

	References

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1. Bove E.L., Lloyd T.R. Staged reconstruction for hypoplastic left heart syndrome—contemporary results. Ann Surg 1996;224:387-395.[Medline]
2. Mosca R.S., Bove E.L., Crowley D.C., Sandhu S.K., Schork M.A., Kulik T.J. Hemodynamic characteristics of neonates following first stage palliation for hypoplastic left heart syndrome. Circulation 1995;92(suppl 2):267-271.[Abstract/Free Full Text]
3. Rychik J., Rome J.J., Collins M.H., DeCampli W.M., Spray T.L. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulonary vascular histopathology and outcome. J Am Coll Cardiol 1999;34:554-560.[Abstract/Free Full Text]
4. Canter C.E., Moorhead S., Huddleston C.B., Spray T.L. Restrictive atrial septal communication as a determinant of outcome of cardiac transplantation for hypoplastic left heart syndrome. Circulation 1993;88:II456-460.
5. Singh A.K., DeLeval M.R., Pincott J.R., Stark J. Pulmonary artery banding for truncus arteriosus in the first year of life. Circulation 1976;54(suppl 3):17-19.
6. Edwards J.E., DuShane J.W. Thoracic venous anomalies. Arch Pathol 1950;49:517-537.
7. Pinto C.A.M., Ho S.Y., Redington A., Shinebourne E.A., Anderson R.H. Morphological features of the levoatriocardinal vein. Pediatr Pathol 1993;13:751-761.[Medline]
8. Bernstein H.S., Moore P., Stanger P., Silverman N.H. The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-sytemic connection. J Am Coll Cardiol 1995;26:995-1001.[Abstract]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Toru Ishizaka Richard G. Ohye Takaaki Suzuki Eric J. Devaney Edward L. Bove Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ishizaka, T. Articles by Bove, E. L. Search for Related Content PubMed PubMed Citation Articles by Ishizaka, T. Articles by Bove, E. L. Related Collections Congenital - acyanotic