Difference between revisions of "Tetralogy of Fallot/Pulmonary Atresia/Major Aortopulmonary Collateral Arteries"
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== Related surgical procedures<!-- List and briefly describe any procedures which may be performed specifically to treat this comorbidity or its sequelae. If none, this section may be removed. --> == | == Related surgical procedures<!-- List and briefly describe any procedures which may be performed specifically to treat this comorbidity or its sequelae. If none, this section may be removed. --> == | ||
Cardiac catheterization lab procedures after surgical repairs: branch PA stenosis ballooning, Melody or Harmony valves | |||
== Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> == | == Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> == | ||
Revision as of 13:31, 1 June 2023
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| Anesthetic management |
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Major aortopulmonary collateral arteries (MAPCAs) are present in 20-25% of patients with Tetralogy of Fallot plus pulmonary atresia.
Anesthetic implications
Preoperative optimization
Intraoperative management
Postoperative management
Related surgical procedures
Cardiac catheterization lab procedures after surgical repairs: branch PA stenosis ballooning, Melody or Harmony valves
Pathophysiology
Signs and symptoms
Diagnosis
Neonatal evaluation:
Estimate overall pulmonary blood flow, based on systemic oxygen saturation: high pulmonary blood flow >90%, low pulmonary blood flow < 75%, balanced pulmonary blood flow 75-90%. In most cases, newborns are within 75-90% systemic oxygen saturation and there no clinical need for neonatal intervention*.
*Two variants of pulmonary blood supply merit neonatal intervention: 1) unilateral ductus arteriosus (or anomalous PA from ascending aorta) with MAPCAs to other lung. 2) centrally confluent native PAs that arborize to all lung segments, with all dual supply MAPCAs
If systemic oxygen saturation <75% or > 90% (regardless of anatomy of pulmonary blood supply), then neonatal surgery is performed
Pre-surgical work-up:
Intracardiac anatomy determined by echocardiography.
Computed tomography or angiography used to determine the anatomy of the pulmonary blood supply including presence of central pulmonary arteries and MAPCA supply (single vs dual).
Treatment
Medication
Surgery
If stable, elective repair at around 4-7 months old.
Single-staged complete repair: complete unifocalization and intracardiac repair (VSD closure and conduit from right ventricle to unifocalized pulmonary arteries)
Palliation: AP window or unifocalization to a shunt (systemic to PA)
Note that unifocalization refers to incorporation of MAPCAs and PAs into a single central pulmonary artery system, additionally with augmentation/reconstruction of stenotic or hypoplastic branches.
A flow study can be conducted intra-operatively to assess for candidacy for intracardiac repair. Patients with a flow study with low PA pressures (<= 25 mmHg) at physiologic flows will have a intracardiac repair performed. Patients with a flow study with high PA pressures (> 25 mmHg) at slightly supraphysiologic levels of flow will have a systemic-to-PA shunt placed.