Hypoplastic Left Heart Syndrome: Stage 1 palliation

Spectrum of disease



Operative options




2010, NEJM – SVR trial
Landmark NIH-funded trial of 555 newborn infants comparing outcomes following S1P with BTS vs Sano conduit.  Most of the following studies use data from this cohort of well-studied patients.

2010, Circulation – Enalapril does not change interstage survival or growth following S1P

2010, JTCVS – Preoperative risk factors

2011, Ped Card – Celiac artery flow better in Sano than BTS

2012, Circulation – 14 month SVR followup

2012, Circulation – Shunt type does not impact ventricular size at 14 month

2012, JACC – Current outcomes in HLHS

2012, JTCVS – Causes and timing of death in SVR trial

2012, JTCVS – Differences in postoeperative care following S1P

2012, JTCVS – Low center volume, open sternum affect survival to hospital discharge

2013, ASE – Retrograde flow in BTS and RVPAS does not affect clinical outcomes

2013, Circ CV Imaing – Shunt type does not affect RV echo parameters, volume unloading following Glenn

2013, Circulation – 18% incidence of recurrent arch obstruction following S1P

2013, JTCVS – Timing and outcomes of BDG in SVR trial

2014, J Peds – Feeding practices following S1P

2016, JAHA – Digoxin reduces interstage mortality

2016, JHLT – Transplant outcomes following S1P

2017, ASE – BDG decreases RV volume and mass equally


2012, Anesth Analg – Mathematical modeling of DO2 and QpQs

2000, ATS – SvO2 of 30% is anaerobic threshold

1998, Circulation – Theoretical DO2 calculations following S1P

2004, Circulation – Alpha blockade decreases postoperative cardiac arrest

2004, JTCVS – Low SVR improves DO2

Technical Aspects

1986, JTCVS – Early lessons learned S1P

1997, ATS – Coronary obstruction, Sano obstruction causes of death in S1P

2003 – Sano technical description

2012 – Description of the ‘dunk’ technique

2017, ATS – FVH improves PA growth

2017, Card Young – FVH improves PA growth

2017, JTCVS – RVPAS to right of aorta may decrease shunt revisions


Historical Papers

1983, NEJM – Norwood
This is the original description of the Norwood procedure performed at Boston Children’s Hospital.

2003, JTCVS – Sano
The original description of the Sano modification to the Norwood Procedure.

1975, Ann Thor Surg – Stansel
The original description (one of 3) of a proximal anastamosis between the ascending aorta and pulmonary artery, in this case used as a treatment for dTGA.  Damus, Kaye, and Stansel all submitted separate papers describing the same thing in 1975, so we credit all 3 of them.  Damus’ paper was rejected.  When he read Stansel’s accepted paper, he wrote this letter to the editor…

1975, Ann Thor Surg – Damus
Damus’ letter to the editor re: his rejected paper.

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