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Ann Thorac Surg 2007;84:1746-1748. doi:10.1016/j.athoracsur.2007.05.068
© 2007 The Society of Thoracic Surgeons
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Case Reports

Hypoplastic Left Heart Syndrome, Interrupted Inferior Vena Cava, Biliary Atresia

Michiaki Imamura, MD, PhDa,*, Umesh Dyamenahalli, MDb, Ritu Sachdeva, MDb, Evan R. Kokoska, MDc, Robert D.B. Jaquiss, MDa

a Division of Pediatric Cardiothoracic Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas
b Division of Pediatric Cardiology, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas
c Division of Pediatric General Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas

Accepted for publication May 29, 2007.

* Address correspondence to Dr Imamura, Division of Pediatric Cardiothoracic Surgery, Arkansas Children’s Hospital, 800 Marshall St, Slot 677, Little Rock, AR 72202 (Email: imamuramichiaki{at}uams.edu).


   Abstract
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 Abstract
Introduction
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A neonate was diagnosed with hypoplastic left heart syndrome, heterotaxy syndrome, and interrupted inferior vena cava soon after birth. At 6 days old she underwent the Norwood procedure using Sano modification. Postoperatively she had persistent direct hyperbilirubinemia and was diagnosed with biliary atresia for which she underwent a Kasai procedure at 29 days old. At 10 months she underwent the Kawashima procedure. She is now 20 months old and has been thriving without any jaundice. This case report illustrates that even in the presence of major multiple congenital anomalies, staged reconstruction for hypoplastic left heart syndrome can be successfully performed.


   Introduction
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 Abstract
 Introduction
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Surgical results for hypoplastic left heart syndrome (HLHS) are continuously improving with resulting higher survival rates [1–3]. Multiple noncardiac anomalies commonly associated with HLHS are risk factors for the Norwood procedure, which is used for palliation of HLHS [4]. The association of biliary atresia with HLHS is rare [1, 4]. In the presence of interrupted inferior vena cava (IVC), the second stage of the Norwood procedure requires a Kawashima procedure, which might require a longer time interval between stage I and stage II surgeries [5].

We report a case of successful results with the Norwood procedure in a newborn diagnosed with HLHS, interrupted IVC, and biliary atresia, which we believe has not been previously reported.

A 2.8 kg, full-term, female newborn had severe respiratory distress develop shortly after birth. Echocardiography confirmed HLHS with aortic atresia, mitral atresia, a very small ascending aorta (1.4 mm), unrestrictive atrial septal defect, and a dilated right ventricle with normal systolic function. In addition, there was an interruption of the IVC and azygous continuation to the right superior vena cava. She was managed with prostaglandin infusion. At 6 days of age, she underwent the modified Norwood procedure using right ventricle-to-pulmonary artery conduit (Sano modification). During surgery, only short circulatory arrest was used during cardioplegic solution injection, and regional cerebral perfusion was used during arch reconstruction. Because of interrupted IVC, we anticipated waiting longer to perform second-stage surgery; therefore, we used a larger 6-mm polytetrafluoroethylene tube for the right ventricle-to-pulmonary artery conduit. The patient had an arterial oxygen saturation level of 87% as well as mild congestive heart failure initially. She was treated with diuretics and afterload reduction therapy. She was extubated 7 days after surgery.

After the stage I Norwood procedure, the patient had persistent neonatal jaundice due to direct hyperbilirubinemia (Fig 1). Abdominal ultrasound indicated the absence of a gallbladder. In addition, diisopropyl iminodiacetic acid scintigraphy showed no tracer in the gastrointestinal tract even in the late phase, indicating the diagnosis of biliary atresia. The patient also had a paralyzed left vocal cord and significant gastroesophageal reflux. At 29 days of age she underwent a liver biopsy and a cholangiography followed by a Kasai procedure for biliary atresia. A Ladd’s procedure for malrotation and a Nissen fundoplication with gastostomy tube placement for severe gastroesophageal reflux were performed at the same time. During the surgery accessory spleens were noted. Three months after the Kasai procedure, serum bilirubin levels decreased to normal (Fig 1).


Figure 1
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  		Fig 1. Changes in total bilirubin (T-Bil) level after stage I Norwood procedure.

 
Due to the presence of interrupted IVC, we decided to perform the second-stage of the Norwood operation at approximately 10 months of age, anticipating that the patient would weigh approximately 8 kg at that time. She had increasing cyanosis at 5 months of age; however, oxygen saturation levels improved with supplemental oxygen. Consequently she was managed at home with oxygen by nasal cannula. Preoperative pulmonary angiography showed mild hypoplasia of the right pulmonary artery, normal size of the left pulmonary artery, and normal intrapulmonary architecture.

She underwent the Kawashima procedure [5] of total cavopulmonary shunt operation at 10 months of age and weighed 8.9 kg at the time of the surgery. Postoperative echocardiography showed laminar caval flow, normal right ventricular systolic function, and trace tricuspid valve regurgitation. Her postoperative oxygen saturations ranged between 85% and 90%. At her most recent follow-up at 20 months of age, she is doing well clinically with oxygen saturations at 87% on room air and no jaundice.


   Comment
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 Abstract
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 Comment
References
 
The short-term and long-term results for patients with HLHS have continued to improve due to modification of surgical and medical management [1, 2]. Hypoplastic left heart syndrome has been reported to accompany several chromosomal and extracardiac anomalies; however, heterotaxy syndrome has been reported only in 2.7% of cases [1]. Interrupted IVC is present mainly in patients with left isomerism, which is very rare in the patient with HLHS. Association of interrupted IVC and HLHS has not been reported even in the larger series of HLHS and the Norwood procedure [1, 2]. However, there is one case report with similar anomalies in which the patient did not survive [6]. In the review of 163 patients with left atrial isomerism, Gilljam and colleagues [7] noted biliary atresia in 11% of patients and coarctation of the aorta in 16%, with half the patients who had coarctation also had a severely hypoplastic subaortic ventricle. Thus one might infer an incidence of biliary atresia with HLHS to be about 1% to 2% in patients with left atrial isomerism. Gilljam and colleagues [7] also reported several independent factors associated with time-related death including aortic coarctation, single ventricle, biliary atresia, and other gastrointestinal malformations. All of these factors were present in our patient.

The second-stage surgery in HLHS is bidirectional cavopulmonary anastomosis. Recently, in selected patients we have been performing the second-stage Norwood procedure for younger and smaller patients [8]. However, in the presence of interrupted IVC, a Kawashima procedure is performed [5]. Therefore we performed the second stage surgery in our patient when she was 10 months of age and weighed 8.9 kg.

Becker and colleagues [4] reported a case of successful biliary atresia repair after modified Norwood surgery. Unlike our patient, their patient was not complicated by heterotaxy syndrome with interrupted IVC and malrotation.

On follow-up visits we will continue to assess the liver function in this patient. We will also be assessing the patient for evidence of pulmonary arteriovenous fistulae at which time we will consider performing the Fontan procedure. This should improve systemic saturations and more importantly prevent pulmonary arteriovenous fistulae. However, the Fontan procedure may cause liver dysfunction in this patient who underwent the Kasai procedure due to increased venous pressure. Fenestrating the Fontan circuit may minimize significant elevation of right-sided venous pressures, preserve liver function, and prevent development of pulmonary arteriovenous fistulae.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark III BJ. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution Circulation 2000;102(19 Suppl 3):III136-III141.
  2. Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients Circulation 2002;106(12 Suppl 1):I82-I89.
  3. Stasik CN, Goldberg CS, Bove EL, Devaney EJ, Ohye RG. Current outcomes and risk factors for the Norwood procedure J Thorac Cardiovasc Surg 2006;131:412-417.
  4. Becker DJ, Islam S, Geiger JD. Biliary atresia associated with hypoplastic left heart syndrome: a case report and review of the literature J Pediatr Surg 2004;39:1411-1413.
  5. Kawashima Y, Kitamura S, Matsuda H, Shimazaki Y, Nakano S, Hirose H. Total cavopulmonary shunt operation in complex cardiac anomalies: a new operation J Thorac Cardiovasc Surg 1984;87:74-81.
  6. Alves MV. Hypoplastic left heart syndrome in mirror-imaged arrangement Cardiol Young 2005;15:523-524.
  7. Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Outcomes of left atrial isomerism over a 28-year period at a single institution J Am Coll Cardiol 2000;36:908-916.
  8. Jaquiss RDB, Ghanayem NS, Hoffman GM, et al. Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality J Thorac Cardiovasc Surg 2004;127:982-989.



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