Outcomes of Primary Bidirectional Glenn in Children with Single Ventricle Physiology and Increased Pulmonary Blood Flow

Abstract

Background: We reported our experience in managing patients with single ventricle (SV) physiology and increased pulmonary blood flow (PBF), aiming to assess if it is feasible to proceed with primary Bidirectional Glenn (BDG) without a prior operation to limit PBF. Materials and methods: This is a retrospective study with 51 consecutive patients who underwent BDG operation as a primary operation or a second stage prior to the definitive Fontan operation at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia between 2010 and 2018. Patients were categorized into two groups based on their PBF prior to the operation: Patients who had SV physiology and increased PBF (seven patients) vs. patients with SV physiology and restricted PBF (44 patients). Results: The median age for the increased PBF group was 9.9 months [interquartile range (IQR): 2-16.9 months], and the median age for the restricted PBF group was 15.3 months (IQR: 6.7-42.6 months). Although the length of hospital stay was longer in patients with increased PBF (P = 0.039), we couldn't find a statistically significant difference in early mortality, duration of mechanical ventilation, length of pleural drainage, and length of intensive care unit (ICU) stay between the groups. Conclusion: In our experience, we found that primary BDG could be done safely for patients having SV physiology and increased PBF with acceptable short-term outcomes. It might further reduce the morbidity and mortality for those patients by avoiding the risk of initial pulmonary artery banding or aortopulmonary shunts.

Outcomes of bidirectional Glenn surgery done without prior cardiac catheterization

“…The main drawback of pre-BDG catheterization is the risk of radiation exposure, the complications related to the catheterization procedure, and the need for hospital admission. In clinical practice, and in addition to previously published reports, very rarely were patients excluded from further Glenn surgery based on cardiac catheterization data [2][3][4][5]. In the last five years, we noticed that none of the patients with univentricular heart physiology and restrictive pulmonary flow that underwent pre-BDG catheterization were excluded from further surgery.…”

“…In this cohort, all patients with physiological univentricular hearts and restrictive pulmonary blood flow that underwent palliative BDG surgery were retrospectively included in the study. We considered patients to have restrictive pulmonary blood flow if the pressure gradient (PG) was > 45 mmHg during echocardiography across the following structures: pulmonary valve, subpulmonic infundibulum, pulmonary artery band, branch pulmonary arteries, Sano shunt after Norwood surgery, Modified Blalock-Taussig-Thomas shunt (MBTTS), patent ductus arteriosus (PDA) stent, or ventricular septal defect if pulmonary artery arises from the rudimentary ventricle (as in cases with tricuspid atresia with normally related vessels) [3].…”

Bidirectional glenn surgery without palliative pulmonary artery banding in univentricular heart with unrestricted pulmonary flow. Retrospective multicenter experience

“…As reported in the literature, a low PVRi (≤ 3 WU.m2) is one of the characteristics linked with favorable outcomes following BDG surgery [ 2 , 11 ]. In this cohort, 11/16 patients in group 2 got direct BDG without initial PAB between the ages of 2 to 4 months.…”

“…As the PVR is likely at its minimal value at this age, this age may be optimal for performing direct BDG. It may be risky to perform direct BDG after six months or before two months of life because the PVRi may exceed the safe values (≤ 3 WU.m2) [ 2 , 11 , 12 ]. In this cohort, 3/16 patients in group 2 underwent direct BDG at 10–11 months and three patients before the age of 2 months; fortunately, all these patients had a PVRi ≤ 5 WU.m2 on room air and < 3 following vasoreactivity testing.…”

“…Some reports even recommended using an aortopulmonary shunt plus surgical interruption of the main pulmonary artery as a first-stage palliative approach, which would offer a more reliable source of PBF and the protection of the pulmonary vascular bed from overflow [ 6 – 8 ]. In patients with aorta arising from the rudimentary ventricle, PAB can result in progressive ventricular hypertrophy, causing more restriction of the interventricular communication with time; these patients may require Damus Kay Stansel (DKS)operation or enlargement of the ventricular septal defect (VSD) at the time of BDG [ 3 , 4 , 6 , 9 – 11 ]. BDG surgery should not be done until the PVR reaches its lowest level.…”