scholarly journals Airway obstruction in children with complex conotruncal cardiac anomalies

2021 ◽  
pp. 1-8
Author(s):  
Courtney E. Wein ◽  
Luisa Cervantes ◽  
Nao Sasaki
Keyword(s):  
Mortality Rate ◽  
Airway Obstruction ◽  
Aortic Arch ◽  
Clinical Data ◽  
Pulmonary Valve ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Vascular Rings ◽  
Cardiac Anomalies ◽  
Absent Pulmonary Valve Syndrome

Abstract The incidence of airway obstruction in patients with complex CHD other than vascular rings and absent pulmonary valve syndrome is unknown. We reviewed pre-operative CT and clinical data of children with conotruncal abnormalities to assess for airway obstruction. Airway obstruction was common (41% of patients), often moderate to severe, of diverse aetiology, and most commonly associated with a right aortic arch. Patients with airway obstruction showed a trend towards a higher mortality rate. Patients with complex conotruncal abnormalities should be assessed for airway obstruction as it may help predict the need for additional interventions and assist with prognostication.

scholarly journals Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

2014 ◽  
Vol 41 (6) ◽  
pp. 664-667 ◽  
Author(s):  
Muhammad Yasir Qureshi ◽  
Harold M. Burkhart ◽  
Paul Julsrud ◽  
Frank Cetta
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Subclavian Artery ◽  
Pulmonary Valve ◽  
Left Subclavian Artery ◽  
Ductus Arteriosus ◽  
Vascular Ring ◽  
Right Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

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

2019 ◽  
Vol 57 (1) ◽  
pp. 46-53
Author(s):  
Yasemin Karaca-Altintas ◽  
Daniela Laux ◽  
Marielle Gouton ◽  
Myriam Bensemlali ◽  
Régine Roussin ◽  
...  
Keyword(s):  
Risk Factors ◽  
Mechanical Ventilation ◽  
Risk Factor ◽  
Mortality Rate ◽  
Pulmonary Valve ◽  
Prolonged Mechanical Ventilation ◽  
Absent Pulmonary Valve ◽  
Lower Weight ◽  
Prolonged Ventilation ◽  
Absent Pulmonary Valve Syndrome

AbstractOBJECTIVESAbsent 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.METHODSThis retrospective 2-centre cohort study included 68 patients with complete repair between January 1996 and December 2015.RESULTSMedian 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.CONCLUSIONSIn 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.

Absent pulmonary valve syndrome with interrupted aortic arch

1998 ◽  
Vol 66 (1) ◽  
pp. 244-246 ◽  
Author(s):  
Carmelo Mignosa ◽  
Dirk G Wilson ◽  
Andrew Wood ◽  
C.Richard Kirk ◽  
Francesco Musumeci
Keyword(s):  
Aortic Arch ◽  
Pulmonary Valve ◽  
Interrupted Aortic Arch ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

Absent pulmonary valve syndrome: Operation in infants with airway obstruction

1992 ◽  
Vol 54 (6) ◽  
pp. 1116-1119 ◽  
Author(s):  
Kevin G. Watterson ◽  
Torsten K. Malm ◽  
Tom R. Karl ◽  
Roger B.B. Mee
Keyword(s):  
Airway Obstruction ◽  
Pulmonary Valve ◽  
Absent Pulmonary Valve ◽  
Absent Pulmonary Valve Syndrome

An unusual case of tetralogy of Fallot with an absent pulmonary valve associated with a retro-aortic innominate vein in a patient with a 16p12.2 microdeletion

2021 ◽  
pp. 1-2
Author(s):  
Niall Linnane ◽  
Andrew Green ◽  
Colin J. McMahon
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Unusual Case ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Innominate Vein ◽  
Absent Pulmonary Valve ◽  
Rare Association

Abstract 16p12.2 microdeletion has been associated with congenital heart defects and developmental delay. In this case, we describe the rare association between tetralogy of Fallot with an absent pulmonary valve a right-sided aortic arch and a retro-aortic innominate vein associated with a 16p12.2 microdeletion and epilepsy.

Sign in / Sign up

Export Citation Format

Share Document