Outcome of Norwood operation for hypoplastic left heart syndrome

Rai, Vivek; Mroczek, Tomasz; Szypulski, Aleksander; Pac, Agnieszka; Gładki, Marcin; Dudyńska, Mirosława; Skalski, Janusz

doi:10.1007/s12055-017-0603-1

Cited by 6 publications

(3 citation statements)

References 33 publications

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“…The Norwood operation is a STAT category 5 and is considered one of the most, complex and technically challenging surgeries in the field of congenital heart surgery. 19,20 Although our sample size is larger than prior reports, we were not able to assess risk factors and their association with development of different outcomes (ie, new-morbidity and unfavorable outcomes). This calls for the need to conduct multicenter studies in this very unique patient population to identify modifiable risk factors that can help guide clinicians with clinical decision making and help improve functional outcomes.…”

Section: Discussionmentioning

confidence: 96%

Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study

Beshish,

Amedi,

Harriott

et al. 2024

ASAIO Journal

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Patients requiring extracorporeal life support (ECLS) post-Norwood operation constitute an extremely high-risk group. We retrospectively described short-term outcomes, functional status, and assessed risk factors for requiring ECLS post-Norwood operation between January 2010 and December 2020 in a high-volume center. During the study period, 269 patients underwent a Norwood procedure of which 65 (24%) required ECLS. Of the 65 patients, 27 (41.5%) survived to hospital discharge. Mean functional status scale (FSS) score at discharge increased from 6.0 on admission to 8.48 (p < 0.0001). This change was primary in feeding (p < 0.0001) and respiratory domains (p = 0.017). Seven survivors (26%) developed new morbidity, and two (7%) developed unfavorable functional outcomes. In the regression analysis, we showed that patients with moderate—severe univentricular dysfunction on pre-Norwood transthoracic echocardiogram (odds ratio [OR] = 6.97), modified Blalock Taussig Thomas (m-BTT) shunt as source of pulmonary blood flow (OR = 2.65), moderate—severe atrioventricular valve regurgitation on transesophageal echocardiogram (OR = 8.50), longer cardiopulmonary bypass time (OR = 1.16), longer circulatory arrest time (OR = 1.20), and delayed sternal closure (OR = 3.86), had higher odds of requiring ECLS (p < 0.05). Careful identification of these risk factors is imperative to improve the care of this high-risk cohort and improve overall outcomes.

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Section: Discussionmentioning

confidence: 96%

Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study

Beshish,

Amedi,

Harriott

et al. 2024

ASAIO Journal

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“…Table 1 includes a list of reports describing 1-year mortality after the Norwood procedure (9,(45)(46)(47)(48)(49)(50)(51)(52)(53)(54)(55)(56)(57). Scanning Table 1 provides some insight into post-surgical outcomes for HLHS patients.…”

Section: Hlhs Outcomesmentioning

confidence: 99%

Current state of the art in hypoplastic left heart syndrome

Birla

Brimmer²,

Short³

et al. 2022

Front. Cardiovasc. Med.

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Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.

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“… McGuirk et al 2006 [ 52 ] Norwood procedure - > bidirectional Glenn procedure (stage II) - > a modified Fontan procedure (stage III) Andrews et al [ 53 ] stage I (the Norwood operation) - > stage II (the hemi-Fontan operation - > stage III (the Fontan operation - > cardiac transplantation Rogers et al 2012 [ 54 ] stage I (mBTS) - > stage II (BDG) - > stage III extracardiac conduit (ECC) Fontan - > cardiac transplantation or stage I (RV-PA)- > stage II (the hemi-Fontan operation - > stage III (the Fontan operation - > cardiac transplantation Malec et al 2017 [ 55 ] stage I (mBTS) - > stage II (BDG) - > stage III extracardiac conduit (ECC) Fontan - > cardiac transplantation or stage I (RV-PA)- > stage II (the hemi-Fontan operation - > stage III (the Fontan operation - > cardiac transplantation Rai et al 2018. [ 56 ] stage I (RV-PA)- > stage II (the hemi-Fontan operation - > stage III (the Fontan operation - > cardiac transplantation BDG bidirectional Glenn procedure, mBTS modifed right Blalock-Taussig shunt, ECC extracardiac conduit, IALT intra-atrial lateral tunnels, HF hemi-Fontan, RV-PA the right ventricle-to-pulmonary artery shunt …”

Section: Third Stage Of Treatment—fontan Proceduresmentioning

confidence: 99%

Hypoplastic left heart syndrome [HLHS]: treatment options in present era

Rai

Gładki

Dudyńska

et al. 2018

Indian J Thorac Cardiovasc Surg

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Hypoplastic left heart syndrome (HLHS) is the most severe form of congenital heart defect (CHD). The first successful intervention for it was undertaken by Norwood in 1983. Since then, there have been much development in the pre, intra, and postoperative treatment option in staged palliative surgical procedures. Early diagnostic management, prenatal interventions, innovative diagnostic methods, constantly modified surgical techniques, and hybridization contribute to a significant progress in treatment options. This will allow for defining an optimal strategy of improving survival and quality of life in HLHS patients. The development of intervention cardiology makes possible the stepwise treatment of the defect with one operation only. The first and third stage may be done by hybrid or interventional methods, then only the second stage of treatment needs to be done surgically. The world experience and all the available literature says that the 1st-stage procedure could be done now safely either directly or with a bridge to Norwood followed by the stage 2 with a Glen or Hemi-Fontan and followed by a Fontan down the lane surgically.

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Outcome of Norwood operation for hypoplastic left heart syndrome

Cited by 6 publications

References 33 publications

Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study

Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study

Current state of the art in hypoplastic left heart syndrome

Hypoplastic left heart syndrome [HLHS]: treatment options in present era

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