Combined Norwood and cavopulmonary shunt as the first palliation in late presenters with hypoplastic left heart syndrome and single-ventricle lesions

Published:November 29, 2021DOI:



      A primary cavopulmonary shunt as a component of the initial Norwood palliation could be an option in patients with hypoplastic left heart syndrome and single-ventricle lesions. We present our initial experience with this approach in carefully selected patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance.


      The study included 16 patients; the mean age was 137.9 ± 84.2 days. All patients underwent a Norwood palliation consisting of atrial septectomy, Damus-Kaye-Stansel connection, and arch augmentation in addition to the cavopulmonary shunt as the initial palliation.


      The mean preoperative pulmonary to systemic blood flow (Qp/Qs) ratio on room air (n = 9) and with 100% oxygen (n = 8) was 5.3 ± 3.2 and 8.6 ± 4.3, respectively. The mean pulmonary vascular resistance on room air (n = 10) and 100% oxygen (n = 9) was 4.8 ± 3.1 and 1.7 ± 0.97 WU/m2, respectively. Delayed chest closure was needed in 12 patients, and 6 patients required postoperative inhaled nitric oxide. One patient underwent takedown of the cavopulmonary shunt and construction of the right ventricle to pulmonary artery conduit after 1 month. The mean intensive care unit stay was 18.9 ± 15.4 days. There were 2 in-hospital deaths (48 hours and 8 days after surgery) and 2 postdischarge deaths (6 months and 2 years after hospital discharge). Seven patients have undergone the Fontan completion successfully, and 5 patients await further surgery.


      First-stage Norwood palliation with cavopulmonary shunt for patients with hypoplastic left heart syndrome or single-ventricle lesions is feasible in late presenters with low pulmonary vascular resistance.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      AVSD (atrioventricular septal defect), CPS (cavopulmonary shunt), HLHS (hypoplastic left heart syndrome), ICU (intensive care unit), MA (mitral atresia), PA (pulmonary artery), PVR (pulmonary vascular resistance), Qp (pulmonary blood flow), Qs (systemic blood flow), RV (right ventricle)
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      Linked Article

      • Commentary: The right procedure for the right patient
        The Journal of Thoracic and Cardiovascular SurgeryVol. 163Issue 5
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          The Achilles' heel of single-ventricle palliation is the inherently unstable interstage circulation following both the Norwood procedure with systemic-to-pulmonary artery shunt or stage I hybrid palliation. This is reflected in the high mortality reported in patients with hypoplastic left heart syndrome before completion of a cavopulmonary shunt and the subsequent flattening of survival curves after stage II palliation.1 Primary construction of a cavopulmonary anastomosis is impeded by high pulmonary vascular resistance (PVR) in neonates and young infants.
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      • Commentary: Successful pairing of the Norwood and bidirectional Glenn in select older infants
        The Journal of Thoracic and Cardiovascular SurgeryVol. 163Issue 5
        • Preview
          In the stepwise palliation of single ventricle disease with systemic obstruction, the Norwood operation is typically performed in the first week of life and followed at 3 to 6 months by superior cavopulmonary connection. The optimal timing of the staged operations has been extensively studied and has shaped current practice.1,2 Nevertheless, there may be the rare occasion when a patient presents unusually late and needs a Norwood operation. This scenario was recently described in a large series from Saudi Arabia in which patients underwent delayed Norwood surgery.
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