Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum

2017 ◽  
Vol 22 (3) ◽  
pp. 256-264 ◽  
Author(s):  
Stephen Gleich ◽  
Gregory J. Latham ◽  
Denise Joffe ◽  
Faith J. Ross
Keyword(s):  
Pulmonary Valve ◽  
Anesthetic Management ◽  
Treatment Options ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Disease Treatment ◽  
Obstructive Lesion ◽  
Anesthetic Considerations ◽  
Single Ventricle Palliation

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation. Cardiac transplantation is recommended in the most severe cases. This review will discuss the perioperative and anesthetic management of patients with PA/IVS and highlight the challenges in management.

Pulmonary atresia with intact septum: the use of Conquest Pro coronary guidewire for perforation of atretic valve and subsequent interventions

2012 ◽  
Vol 23 (2) ◽  
pp. 197-202 ◽  
Author(s):  
Mazeni Alwi ◽  
Rahmat R. Budi ◽  
Marhisham Che Mood ◽  
Ming C. Leong ◽  
Hasri Samion
Keyword(s):  
Patent Ductus Arteriosus ◽  
Balloon Dilatation ◽  
Pulmonary Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Total Occlusion ◽  
Patent Ductus

AbstractObjectiveTo determine the feasibility and safety of the Conquest Pro wire as an alternative to radiofrequency wire for perforation of atretic pulmonary valve and subsequent balloon dilatation and patent ductus arteriosus stenting in patients with pulmonary atresia with intact ventricular septum.BackgroundRadiofrequency valvotomy and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum in many institutions today.MethodsWe report eight consecutive patients in whom we used the Conquest Pro coronary guidewire, a stiff wire normally reserved for revascularisation of coronary lesions with chronic total occlusion, for perforation of atretic pulmonary valve and subsequent balloon dilatation, and stenting of the patent ductus arteriosus.ResultsPerforation of atretic pulmonary valve was successful in seven out of eight cases. Radiofrequency valvotomy was employed after failure of perforation by the Conquest Pro wire in one case where the right ventricular outflow tract was broad based and tapered towards the pulmonary valve, and was heavily trabeculated. Failure of the Conquest Pro wire to perforate the pulmonary valve plate was mainly attributed by the failure to engage the wire at the correct position.ConclusionThe Conquest Pro wire for perforation and subsequent interventions in the more straightforward cases of pulmonary atresia with intact ventricular septum is effective and safe, simplifying the entire procedure. However, the radiofrequency generator and wires remain essential tools in the paediatric interventional catheter laboratory.

OP-090 Treatment Options of Neonates with Pulmonary Atresia with Intact Ventricular Septum

2014 ◽  
Vol 113 (7) ◽  
pp. S22-S23
Author(s):  
S. Özgür ◽  
V. Doğan ◽  
Ö. Ceylan ◽  
İ. Ertuğrul ◽  
M. Koç ◽  
...  
Keyword(s):  
Treatment Options ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum

scholarly journals Type I and Ii Pulmonary Atresia With Intact Ventricular Septum in Infants-10-year Experience in Initial Surgery at One Center

2021 ◽  
Author(s):  
Hailong Song ◽  
Ziying Chen
Keyword(s):  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Type I ◽  
Intact Ventricular Septum ◽  
Icu Stay ◽  
Initial Surgery ◽  
Operation Rate ◽  
Pda Ligation ◽  
Length Of Icu Stay

Abstract Background: To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS). Methods: Patients with type I and II PA/IVS undergoing initial surgery were enrolled, including type I with systemic to pulmonary (BT) shunt+patent ductus arteriosus (PDA) ligation+mosaic surgery (surgical A), BT shunt+PDA ligation+pulmonary valve incision without extracorporeal circulation (surgical B), type II with BT shunt+PDA ligation+right ventricular outflow tract (RVOT) incision+transpulmonary annulus patch (TP) (surgical C), and BT shunt+PDA ligation+RVOT incision+TP+artificial pulmonary valve (surgical D). Mechanical ventilation time (MVT), length of ICU stay, mortality rate, tricuspid Z value (TZ), tricuspid regurgitation (TR), McGoon ratio, oxygen saturation (SpO2), pulmonary transvalvular pressure (PTP), pulmonary regurgitation (PR), survival rate and re-operation rate were compared between surgical A and B, and between surgical C and D.Results: After surgery, PR was greater by surgical A than by surgical B at 1 month (P<0.05); lower TZ and McGoon ratio and greater PR, PTP and TR at 3 months (P<0.05); lower SpO2 (P<0.05), greater PTP at 6 months (P<0.01); greater TR and PTP at 1 year (P<0.05). MVT and length of ICU stay were longer by surgical C than by surgical D (P<0.05). There was greater PR at discharge and 1 month (P<0.01); greater TR and PR, lower McGoon ratio and SpO2 at 3 months (P<0.05); lower TZ and PTP and greater PR and PTP at 6 months (P<0.05) and 1 year (P<0.01) respectively.Conclusion: Surgical B and D are superior to surgical A and C respectively.

scholarly journals Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum

2017 ◽  
Vol 10 (1) ◽  
pp. 5 ◽  
Author(s):  
NageswaraRao Koneti ◽  
Shweta Bakhru ◽  
Shilpa Marathe ◽  
Manish Saxena ◽  
Sudeep Verma ◽  
...  
Keyword(s):  
Chronic Total Occlusion ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Total Occlusion
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