Airway compression management in late-presenting absent pulmonary valve syndrome

Melnikova, Anastasia Martinez-Esteve; Sologashvili, Tornike; Beghetti, Maurice; Tissot, Cécile; Kalangos, Afksendiyos; Corbelli, Regula; Aggoun, Yacine; Didier, Dominique; Myers, Patrick Olivier

doi:10.1017/s1047951113002151

Cited by 4 publications

(3 citation statements)

References 11 publications

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“…This is probably because the decision to provide mechanical relief in these patients was related to their PA size, which is also a marker of the severity of intrinsic airway disease. We believe that PA plasty or the LeCompte manoeuvre is essential to treat proximal airway compression as described in the literature [12,27] but may not effectively relieve distal bronchi compression.…”

Section: Discussionmentioning

confidence: 93%

Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome

Karaca-Altintas

Laux

Gouton

et al. 2019

European Journal of Cardio-Thoracic Surgery

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OBJECTIVES Absent pulmonary valve syndrome is a rare congenital heart disease with severe airway compression due to dilatation of the pulmonary arteries (PAs). We investigated risk factors for death and prolonged mechanical ventilation (>7 days) and a threshold PA size for these outcomes. METHODS This retrospective 2-centre cohort study included 68 patients with complete repair between January 1996 and December 2015. RESULTS Median age at repair was 3.9 months (1.3–8.7 months), and median weight was 5 kg (4–7 kg). The mortality rate before hospital discharge was 12%, and the mortality rate at last follow-up was 19%. In multivariable analysis, risk factors for death were higher Nakata index [hazard ratio (HR) 1.001, 95% confidence interval (CI) 1.001–1.002; P < 0.001] and lower SpO2 (HR 1.06, 95% CI 1.02–1.09; P = 0.002). The accuracy of the Nakata index to predict death was excellent (area under the curve at 6 months: 0.92; P = 0.010). A Nakata index above 1500 mm2/m2 predicted mortality at 6 months with a sensitivity of 98% and a specificity of 82%. Twenty-five patients (37%) had prolonged mechanical ventilation. The only multivariable risk factor for prolonged ventilation was lower weight at repair (odds ratio 2.9, 95% CI 1.3–6.7; P = 0.008). Neither PA plasty nor the LeCompte manoeuvre had a protective effect on mortality or prolonged ventilation. A Nakata index above 1500 mm2/m2 remained a risk factor for mortality (P = 0.022) in patients who had a PA plasty or the LeCompte manoeuvre. CONCLUSIONS In patients with absent pulmonary valve syndrome, the Nakata index predicts mortality with a cut-off of 1500 mm2/m2. Lower weight at repair is the only multivariable risk factor for prolonged ventilation. Neither PA plasty nor the LeCompte manoeuvre had a protective effect on these outcomes.

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Section: Discussionmentioning

confidence: 93%

Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome

Karaca-Altintas

Laux

Gouton

et al. 2019

European Journal of Cardio-Thoracic Surgery

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“…Similarly, bronchoscopy may be important for treatment decisions in children with absent pulmonary valve syndrome. 34 Both papers underscore the importance of early cooperation of the pediatric bronchoscopist with other subspecialties in the treatment of complex disease.…”

Section: Flexible Bronchoscopy: Therapeutic Applicationsmentioning

confidence: 99%

“…This paper reported that in one of seven cases the observed bronchial compression during test angioplasty was so severe that it was decided not to place a pulmonary artery stent. Similarly, bronchoscopy may be important for treatment decisions in children with absent pulmonary valve syndrome . Both papers underscore the importance of early cooperation of the pediatric bronchoscopist with other subspecialties in the treatment of complex disease.…”

Section: Diagnostic Testing/endoscopymentioning

confidence: 99%

Pediatric pulmonology year in review 2015: Part 3

et al. 2016

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Our journal covers a broad range of research and scholarly topics related to children's respiratory disorders. For updated perspectives on the rapidly expanding knowledge in our field, we will summarize the past year's publications in our major topic areas, as well as selected publications in these areas from the core clinical journal literature outside our own pages. The current review covers articles on neonatal lung disease, pulmonary physiology, and respiratory infection.Word count: 71 3

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Diagnostic imaging for absent pulmonary valve syndrome: an update with an emphasis on cardiothoracic computed tomography

Goo

2022

Pediatr Radiol

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Airway compression management in late-presenting absent pulmonary valve syndrome

Cited by 4 publications

References 11 publications

Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome

Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome

Pediatric pulmonology year in review 2015: Part 3

Diagnostic imaging for absent pulmonary valve syndrome: an update with an emphasis on cardiothoracic computed tomography

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