Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes

Murphy, Maureen; Bellsham‐Revell, Hannah; Morgan, Gareth J.; Krasemann, Thomas; Rosenthal, Éric; Qureshi, Shakeel A.; Salih, Caner; Austin, Conal; Anderson, David

doi:10.1016/j.athoracsur.2015.06.098

Cited by 41 publications

(32 citation statements)

References 23 publications

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“…Prepathway procedures such as stabilisation procedures for neonatal HLHS including bilateral pulmonary artery banding17 and enlargement of a restrictive atrial septum18 or the aortic valve or arch in neonates with hypoplastic left ventricle where subsequent events indicate biventricular strategy was unsuccessful 19…”

Section: Methodsmentioning

confidence: 99%

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015

Rogers

Pagel

Sullivan

et al. 2018

Heart

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ObjectiveTo describe the long term outcomes, treatment pathways and risk factors for patients diagnosed with Hypoplastic Left Heart Syndrome (HLHS) in England and Wales. MethodsThe UKs national audit database captures every procedure undertaken for congenital heart disease and updated life status for patients resident in England and Wales. HLHS patients born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code. ResultsThere were 976 patients with HLHS. Of these, 9.6% had a pre-pathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing pre-pathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Pre-pathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off pathway procedure after their primary procedure, most frequently stenting or dilation of residual or re-coarctation and most commonly occurring between Stage 1 and 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5-63.7) and 56.3% (53.0-59.5) respectively. Patients with an antenatal diagnosis (multivariable hazard ratio (MHR) 1.63 (95% CI 1.12-2.38)), low weight (<2.5kg) (MHR 1.49 (1.05-2.11)) or the presence of an acquired comorbidity ) were less likely to survive. ConclusionTreatment pathways amongst HLHS patients are complex and variable. It is essential that the long term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement. 3 KEY MESSAGES

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Section: Methodsmentioning

confidence: 99%

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015

Rogers

Pagel

Sullivan

et al. 2018

Heart

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“…The survival advantage can be explained by the reduced invasiveness of the hybrid procedure. The survival benefit in the 30 days postprocedure did not extend into the mid-term and longterm follow-up, however, as reported in other studies [16]. Interestingly, when selecting premature infants and patients whose birth weight was less than 2.6 kg, midterm survival was significantly higher.…”

Section: Commentmentioning

confidence: 46%

“…Also, longer vessels may require placement of multiple stents to ensure coverage of all ductal tissue. In addition, there is potential for retrograde arch obstruction after ductal stenting, specifically in the presence of HLHS with aortic atresia, which has been noted in multiple studies [13,16,19]. Therefore, neonates with echocardiographic evidence of preprocedure retroaortic arch obstruction are not typically considered hybrid candidates.…”

Section: Commentmentioning

confidence: 99%

Hybrid Strategy for Neonates With Ductal-Dependent Systemic Circulation at High Risk for Norwood

Nwankwo

Morell

Trucco

et al. 2018

The Annals of Thoracic Surgery

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“…Over time, multiple centers worldwide have established Hybrid programs for high-risk cases [33]. In Japan [34] and London [35], the Hybrid procedure is used in some critical newborns as a palliative step for bridging newborns to a delayed Norwood procedure beyond the neonatal period with or without ductal stenting.…”

Section: The Hybrid Approachmentioning

confidence: 99%

“…We recommend against manipulations such as transpulmonary ductal stenting or placement of a reverse MBTS, which may result in blood loss, circulatory instability, or need for inotropic infusions. Concurrent use of additional interventions at this time have been associated with reported mortality rates between 10 to 25% [32, 35]. Conversely, the surgical approach in Giessen is simply focused on bPAB, the surgical procedure mortality has been less than 1% [29].…”

Section: The Hybrid Approachmentioning

confidence: 99%

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

2016

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Universally fatal only four decades ago, the progress in the three-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. While the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low risk operations, the stage I Norwood procedure remains one of the highest risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals in excess of 90% for the Norwood. This traditional three-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomized 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years post-randomization. In addition to modifications to the Norwood procedure, the “hybrid procedure,” a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a “comprehensive” stage II, that combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to not only improve short-term survival, but potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also burden of disease, neurodevelopmental outcomes, psychosocial development and quality of life.

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Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes

Cited by 41 publications

References 23 publications

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015

Hybrid Strategy for Neonates With Ductal-Dependent Systemic Circulation at High Risk for Norwood

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

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