Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals

Mainwaring, Richard D.; Patrick, William L.; Roth, Stephen J.; Kamra, Komal; Wise‐Faberowski, Lisa; Palmon, Michal; Hanley, Frank L.

doi:10.1016/j.jtcvs.2018.03.153

Cited by 61 publications

(78 citation statements)

References 31 publications

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“…Depending on the institution, the approach can be variable: do nothing, a staged and/or palliative approach, or unifocalization with complete repair (VSD closure and right ventricle‐to‐pulmonary artery conduit). The latter procedure has been performed in over 500 patients at our institution over the past 17 years . We will focus on our management approach to these patients, unifocalization with complete repair.…”

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

“…Although the variability in the anatomy and nonuniformity of perfusion to the various lung segments makes it difficult to discuss the anatomy in simple standardized terms, the physiology is more straightforward . Most patients present in early infancy with a relatively balanced ratio of pulmonary to systemic blood flow (Qp: Qs).…”

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

“…Once surgical candidacy is determined, the preference is to perform as much of the surgery as possible prior to CPB in an effort to minimize the amount of bleeding and reduce the duration of CPB. Because the objective is full unifocalization, CPB times are long, averaging around 250 minutes, with an overall case duration of approximately 930 minutes …”

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

“…We have anesthetized over 500 patients at our institution, many of whom have undergone a single‐stage complete repair with VSD closure. Vigilance in anesthetic management is a key component to the outcomes of these patients and to maintain a low perioperative mortality rate (1.7%) …”

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

“…The latter procedure has been performed in over 500 patients at our institution over the past 17 years. 26,30 We will focus on our management approach to these patients, unifocalization with complete repair.…”

Section: Major Aortopulmonary Collaterals (Mapcas)mentioning

confidence: 99%

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Tetralogy of Fallot: Everything you wanted to know but were afraid to ask

Wise‐Faberowski

Asija

McElhinney

2019

Pediatric Anesthesia

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Summary Tetralogy of Fallot (TOF) has four anatomic features: right ventricular hypertrophy (RVH), ventriculoseptal defect (VSD), overriding aorta and right ventricular outflow tract obstruction (RVOT) with an occurrence of 3.9 /10,000 births. The pathophysiologic effects in TOF are largely determined by the degree of RVOT and not the VSD. Intra‐operative anesthetic management is also dependent on the degree of RVOT obstruction and influenced by the extent of surgical RVOT repair.

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Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

Section: Tetralogy Of Fallot (Tof) With Major Aortopulmonary Collatermentioning

confidence: 99%

Section: Major Aortopulmonary Collaterals (Mapcas)mentioning

confidence: 99%

See 3 more Smart Citations

Tetralogy of Fallot: Everything you wanted to know but were afraid to ask

Wise‐Faberowski

Asija

McElhinney

2019

Pediatric Anesthesia

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Pulmonary artery and lung parenchymal growth following early versus delayed stent interventions in a swine pulmonary artery stenosis model

Pewowaruk

Hermsen

Johnson

et al. 2020

Cathet Cardio Intervent

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Objectives: Compare lung parenchymal and pulmonary artery (PA) growth and hemodynamics following early and delayed PA stent interventions for treatment of unilateral branch PA stenosis (PAS) in swine. Background: How the pulmonary circulation remodels in response to different durations of hypoperfusion and how much growth and function can be recovered with catheter directed interventions at differing time periods of lung development is not understood. Methods: A total of 18 swine were assigned to four groups: Sham (n = 4), untreated left PAS (LPAS) (n = 4), early intervention (EI) (n = 5), and delayed intervention (DI) (n = 5). EI had left pulmonary artery (LPA) stenting at 5 weeks (6 kg) with redilation at 10 weeks. DI had stenting at 10 weeks. All underwent right heart catheterization, computed tomography, magnetic resonance imaging, and histology at 20 weeks (55 kg). Results: EI decreased the extent of histologic changes in the left lung as DI had marked alveolar septal and bronchovascular abnormalities (p = .05 and p < .05 vs. sham) that were less prevalent in EI. EI also increased left lung volumes and alveolar counts compared to DI. EI and DI equally restored LPA pulsatility, R heart pressures, and distal LPA growth. EI and DI improved, but did not normalize LPA stenosis diameter (LPA/DAo ratio: Sham 1.27 ± 0.11 mm/mm, DI 0.88 ± 0.10 mm/mm, EI 1.01 ± 0.09 mm/mm) and pulmonary blood flow distributions (LPA-flow%: Sham 52 ± 5%, LPAS 7 ± 2%, DI 44 ± 3%, EI 40 ± 2%). Conclusion: In this surgically created PAS model, EI was associated with improved lung parenchymal development compared to DI. Longer durations of L lung hypoperfusion did not detrimentally affect PA growth and R heart hemodynamics. Functional and anatomical discrepancies persist despite successful stent interventions that warrant additional investigation.

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