High-Risk Anatomic Subsets in Hypoplastic Left Heart Syndrome

Geoffrion, Tracy R.; Fuller, Stephanie

doi:10.1177/21501351221111390

Cited by 3 publications

(1 citation statement)

References 47 publications

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“…Furthermore, many of the SV patients, who would have otherwise undergone the highest risk neonatal cardiac surgical procedure -the Norwood Stage 1 palliation [18][19][20][21][22][23] -had anatomical features associated with adverse outcomes, such as IAS or highly restrictive PFO [24][25][26][27] , LV to coronary fistulae 28,29 , preoperative systemic AVVR, and SV dysfunction 18,29,30 . For these high-risk patients, PFR-based palliation provided a feasible and less invasive strategy to bridge the patients to a later surgery or transplant, once hemodynamic stability had been achieved, prematurity-related comorbidities had improved, and weight had been significantly optimized.…”

Section: Discussionmentioning

confidence: 99%

Transcatheter palliation with pulmonary artery flow restrictors in neonates with congenital heart disease: feasibility, outcomes, and comparison with a historical hybrid Stage 1 cohort

Sperotto

Lang

Nathan

et al. 2023

Preprint

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Background: Neonates with complex congenital heart disease (CHD) and pulmonary overcirculation have been historically treated surgically. However, sub-cohorts of patients may benefit from less invasive procedures. Transcatheter palliation with pulmonary flow restrictors (PFRs) may represent a compelling alternative, but data are limited. Methods: We present our experience of palliation with PFRs in neonates with CHD and pulmonary overcirculation, including procedural feasibility, technical details, and patient-level outcomes. We then compared our sub-cohort of high-risk single ventricle (SV) neonates palliated with PFRs with a historical cohort of high-risk SV neonates palliated with a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and all-cause mortality risk at 6 months. Results: From 2021 to 2023, 17 patients (median age 4 days, interquartile range [IQR] 2-8); median weight 2.51 kilograms [IQR 2.09-3.26]) underwent a PFR procedure; 15 (88%) had SV physiology; 15 (88%) were considered high-risk surgical candidates. All the procedures were technically successful. At a median follow-up of 5.3 months (IQR 1.9-9.6), 13 patients (76%) were either successfully bridged to surgery (n=10, 59%) or are awaiting surgery (n=3, 17%). Patients underwent the target surgery after a median of 2.6 months (IQR 1.2-3.4) from the PFR procedure (median weight 4.6 kilograms [IQR 3.2-5.4]). Their pulmonary arteries were found to have grown adequately for age. All PFR devices were easily removed without the need for arterioplasty. The all-cause mortality rate before target surgery was 24% (n=4). Compared to a historical cohort of high-risk SV neonates palliated with a hybrid Stage 1 (n=23), after adjustment for main confounding (age, weight, presence of intact atrial septum or severely restrictive patent foramen ovale, and presence of left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted Hazard Ratio=0.30 [95% CI 0.10-0.93]). Conclusions: Transcatheter PFR palliation in high-risk neonates with CHD is feasible, safe, and may represent an effective alternative strategy to bridge such high-risk neonates to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.

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Section: Discussionmentioning

confidence: 99%

Transcatheter palliation with pulmonary artery flow restrictors in neonates with congenital heart disease: feasibility, outcomes, and comparison with a historical hybrid Stage 1 cohort

Sperotto

Lang

Nathan

et al. 2023

Preprint

View full text Add to dashboard Cite

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Systemic and Pulmonary Venous Abnormalities

Choy,

Adebo

2024

Board Review Atlas of Pediatric Cardiology

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Deferred Norwood in the setting of airway compression in double-inlet left ventricle with dextro-transposition of the great arteries

Ruiz-Avila,

Lahiri,

Zaidi

et al. 2024

Annals of Pediatric Cardiology

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A 4.1 kg male neonate with a diagnosis of double-inlet left ventricle with dextro-transposition of the great arteries was intubated shortly after birth due to respiratory insufficiency. The initial management consisted of a successful Stage I hybrid procedure. Persistent respiratory insufficiency led to cross-sectional imaging and bronchoscopy that demonstrated severe airway compression from a dilated main pulmonary artery. A Norwood procedure with Blalock–Thomas–Taussig shunt was performed at 1 month of age to relieve the airway obstruction. The patient was discharged home on room air at 2 months of age. This case highlights a unique single-ventricle anatomic variant with airway compression, which was successfully managed with deferred Norwood palliation.

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High-Risk Anatomic Subsets in Hypoplastic Left Heart Syndrome

Cited by 3 publications

References 47 publications

Transcatheter palliation with pulmonary artery flow restrictors in neonates with congenital heart disease: feasibility, outcomes, and comparison with a historical hybrid Stage 1 cohort

Transcatheter palliation with pulmonary artery flow restrictors in neonates with congenital heart disease: feasibility, outcomes, and comparison with a historical hybrid Stage 1 cohort

Systemic and Pulmonary Venous Abnormalities

Deferred Norwood in the setting of airway compression in double-inlet left ventricle with dextro-transposition of the great arteries

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