Rapid bilateral pulmonary artery banding: A developmentally based proposal for the management of neonates with hypoplastic left heart

“We cannot solve our problems with the same thinking we used when we created them.”–Albert Einstein“For things to reveal themselves to us, we need to be ready to abandon our views about them.”–Thich Nhat HahnCentral MessageRapid bPAB within 48 hours of birth will mitigate the reduction of systemic oxygen delivery due to circulatory maldistribution that occurs immediately during the transitional circulation in HLHS. Rapid bPAB within 48 hours of birth will mitigate the reduction of systemic oxygen delivery due to circulatory maldistribution that occurs immediately during the transitional circulation in HLHS. Since the description of hypoplastic left heart syndrome (HLHS) by Noonan and Nadas in 1958, and the seminal publication of surgical intervention by Norwood, Lang, and Hansen in 1982,1Norwood W.I. Lang P. Hansen D.D. 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The Fontan procedure in Australia: a population-based study.J Thorac Cardiovasc Surg. 2007; 134: 1353-1354Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar Finally, although neonatal heart transplantation remains a viable surgical option, limited donor availability renders this strategy as a primary mode of surgical therapy impractical for the majority of patients. In the context of these remarkable improvements in the past 40+ years, several management controversies currently exist and are subject to considerable debate and disagreement among practitioners caring for these neonates—both between and within cardiac programs. Most importantly, these include type of initial procedure (hybrid vs Norwood); optimal timing of the Norwood operation; practices to maximize systemic oxygen delivery, both before and after surgery; and the routine use of delayed sternal closure, among others. Many of these controversies have evolved due to the unstable physiology present, particularly during the 2 most vulnerable periods for these babies—immediately after birth and immediately after surgery. It must be emphasized that any comparison of surgical mortality rates is confounded by the risk profile of the patients who undergo the various surgical options. With this important caveat in mind, the cumulative mortality rate for the Fontan pathway—the classic Norwood operation during the neonatal period, combining early (∼10%),23Mascio C.E. Irons M.L. Ittenbach R.F. Gaynor J.W. Fuller S.M. Kaplinski M. et al.Thirty years and 1663 consecutive Norwood procedures: has survival plateaued?.J Thorac Cardiovasc Surg. 2019; 158: 220-229Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar interstage (∼10%),2Ghanayem N.S. Allen K.R. Tabbutt S. Atz A.M. Clabby M.L. Cooper D.S. Interstage mortality after the Norwood procedure: results of the multicenter single ventricle reconstruction trial.J Thorac Cardiovasc Surg. 2012; 144: 896-906Abstract Full Text Full Text PDF PubMed Scopus (257) Google Scholar SCPC/bidirectional Glenn, and Fontan (1%-6% and 1%-3%) have resulted in longer-term survival rates that have plateaued around 60%.3Newburger J.W. Sleeper L.A. Gaynor J.W. Hollenbeck-Pringle D. Frommelt P.C. Li J.S. et al.Transplant-free survival and interventions at 6 years in the SVR trial.Circulation. 2018; 137: 2246-2253Crossref PubMed Scopus (140) Google Scholar,24Mayer J.E. Hill K. Jacobs J.P. Overman D.M. Kumar S.R. The society of Thoracic Surgeons Congenital Heart Surgery Database: 2020 update on outcomes and research.Ann Thorac Surg. 2020; 110: 1809-1818Abstract Full Text Full Text PDF PubMed Google Scholar,25Iyengar A.J. Winlaw D.S. Galati J.C. Wheaton G.R. Gentles T.L. 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The society of Thoracic Surgeons Congenital Heart Surgery Database: 2021 Update on outcomes and Research.Ann Thorac Surg. 2021; 112: 1753-1762Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar7/1/16-6/30/20PC4∗PC4 specific definitions: newer PC4 sites contributed data for less than the specified date range; Norwood procedure and hybrid stage I included if index operation; cardiac arrest, ECMO, renal replacement therapy, neurologic injury, vocal cord injury all postoperative; renal replacement therapy only for acute kidney injury not prophylaxis; vocal cord injury before February 1, 2019, only diagnosed while still in cardiac intensive care unit, diagnosis is endoscopic; hypoxic ischemic encephalopathy included with stroke.,E3Pediatric Cardiac Critical Care Consortium. Improving outcomes and quality through collaboration.https://pc4quality.org/Date accessed: September 28, 2022Google Scholar1/18-12/21NPCQIC†NPCQIC data combines Norwood and hybrid data, and excludes anyone who transitioned to biventricular physiology, or who was deemed not a candidate for Comprehensive Stage II palliation. Mortality is Norwood-specific.,E4Luna A.O. Kuhnell P. Wooton S. Handler S.S. Wright G. Hammel J. et al.Factors associated with inability to discharge after stage 1 palliation for single ventricle heart disease: an analysis of the national Pediatric Cardiology Quality Improvement Collaborative Database.Pediatr Cardiol. 2022; 43: 1298-1310Crossref PubMed Scopus (0) Google ScholarSVR trialE5Tabbutt S. Ghanayem N. Ravishankar C. Sleeper L.A. Cooper D.S. 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Laryngopharyngeal dysfunction after the Norwood procedure.J Thorac Cardiovasc Surg. 2005; 130: 1293-1301Abstract Full Text Full Text PDF PubMed Scopus (127) Google ScholarSTSPC4E3Pediatric Cardiac Critical Care Consortium. Improving outcomes and quality through collaboration.https://pc4quality.org/Date accessed: September 28, 2022Google Scholar1/18-12/21NPCQIC∗PC4 specific definitions: newer PC4 sites contributed data for less than the specified date range; Norwood procedure and hybrid stage I included if index operation; cardiac arrest, ECMO, renal replacement therapy, neurologic injury, vocal cord injury all postoperative; renal replacement therapy only for acute kidney injury not prophylaxis; vocal cord injury before February 1, 2019, only diagnosed while still in cardiac intensive care unit, diagnosis is endoscopic; hypoxic ischemic encephalopathy included with stroke.,‡Mortality is hybrid-specific.,E4Luna A.O. Kuhnell P. Wooton S. Handler S.S. Wright G. 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Sakamoto K. et al.Long-term results of bilateral pulmonary artery banding versus primary Norwood procedure.Pediatr Cardiol. 2018; 39: 111-119Crossref PubMed Scopus (15) Google ScholarHospital mortality (%)12 (0-100)10.54.71626151.8-13.2Unplanned reintervention (%)Surgical 16%Catheterization 5.4Surgical 15%Catheterization 16Surgical Range 3-40Catheterization Range 6-20Surgical 22Catheterization 6.5Surgical 15%Catheterization 16Cardiac arrest (%)1415182315ECMO (%)1710101910Renal replacement therapy (%)3.03.65.63.6Neurological injury (%)7.96.97.90 Seizure (%)1115 Stroke (%)6.69.0Vocal cord injury (%)219-341420Nasogastric or gastrostomy tube at discharge (%)61682861Tracheostomy (%)2.23.4Postoperative hospital length of stay (d)Mean 59Median 47Note: 9.4% in hospital until SCPCMedian 60STS, Society of Thoracic Surgeons; PC4, Pediatric Cardiac Critical Care Consortium; NPCQIC, National Pediatric Cardiology Quality Improvement Collaborative; SVR, single ventricle reconstruction; ECMO, extracorporeal membrane oxygenation; SCPC, superior cavopulmonary connection.∗ PC4 specific definitions: newer PC4 sites contributed data for less than the specified date range; Norwood procedure and hybrid stage I included if index operation; cardiac arrest, ECMO, renal replacement therapy, neurologic injury, vocal cord injury all postoperative; renal replacement therapy only for acute kidney injury not prophylaxis; vocal cord injury before February 1, 2019, only diagnosed while still in cardiac intensive care unit, diagnosis is endoscopic; hypoxic ischemic encephalopathy included with stroke.† NPCQIC data combines Norwood and hybrid data, and excludes anyone who transitioned to biventricular physiology, or who was deemed not a candidate for Comprehensive Stage II palliation. Mortality is Norwood-specific.‡ Mortality is hybrid-specific. Open table in a new tab STS, Society of Thoracic Surgeons; PC4, Pediatric Cardiac Critical Care Consortium; NPCQIC, National Pediatric Cardiology Quality Improvement Collaborative; SVR, single ventricle reconstruction; ECMO, extracorporeal membrane oxygenation; SCPC, superior cavopulmonary connection. In addition to among the highest surgical mortality rates in congenital cardiac surgery, most of these patients typically have long hospitalizations, with the median length of hospitalization between >7-8 weeks,E17Michielon G. DiSalvo G. Fraisse A. Carvalho J.S. Krupickova S. Slavik Z. et al.In-hospital interstage improves interstage survival after the Norwood stage 1 operation.Eur J Cardio Thorac Surg. 2020; 57: 1113-1121Crossref PubMed Scopus (0) Google Scholar, E18Spigel Z.A. Kalustian A. Ghanayem N. Imamura M. Adachi I. 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Taylor A.L. Sillau S.H. Mitchell M.B. Rausch C.M. Restrictive lung function in pediatric patients with structural congenital heart disease.J Thorac Cardiovasc Surg. 2014; 148: 207-211Abstract Full Text Full Text PDF PubMed Google Scholar Considering the high mortality and morbidity subsequent to surgical intervention for HLHS, the need for a fundamental change in the management of these patients should be considered, utilizing the components of all 3 currently used approaches, with a specific emphasis on minimizing morbidity as well as improving short-term survival. Some patient-related risk factors for mortality and short- and long-term morbidity are nonmodifiable, such as low weight, prematurity, additional congenital anomalies, and genetic syndromes. However, there are 2 particularly high-risk periods for a neonate with HLHS where a change in strategy is modifiable and may improve outcome: during the transitional circulation and during the early postoperative period. The currently utilized care strategies during these time frames vary considerably across care teams, and lend themselves to both standardization and patient-specific application. Thus, given the suboptimal short- and long-term results, we are proposing a fundamental change in strategy to minimize morbidity and mortality during these two time frames, utilizing currently available techniques: rapid bilateral PAB (bPAB) during the first 24 to 48 hours of life with continued use of Prostaglandin, and abandoning the Norwood procedure in the neonatal period whenever possible. This approach is proposed, in part, due to a better understanding of the vulnerability during the transitional circulation, coupled with an in-depth understanding of the developmental biology of the heart as well as all other organ systems in the neonate. After birth, following the fall in pulmonary vascular resistance (PVR) and the closure of the ductus arteriosus, the afterload of the right ventricle (RV) decreases significantly, the RV dominance regresses rapidly in the first 48 hours of life, and RV mass is expected to stabilize around age 3 to 4 months.E40Joyce J.J. Dickson P.I. Qi N. Noble J.E. Raj J. Baylen B.G. Normal right and left ventricular mass development during early infancy.Am J Cardiol. 2004; 93: 797-801Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar, E41Anversa P. Olivetti G. Loud A.V. Morphometric study of early postnatal development in the left and right ventricular myocardium of the rat. I. 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