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Impact of Phrenic Nerve Palsy and Need for Diaphragm Plication Following Surgery for Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals
Christina L. Greene, *Richard D. Mainwaring, Douglas Sidell, Vamsi Yarlagadda, William L. Patrick, *Frank L. Hanley
stanford university school of medicine, stanford, CA

OBJECTIVES: Injury to the phrenic nerves may occur during surgery for Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries (PA/VSD/MAPCAs). These patients may develop respiratory failure and require diaphragm plication. The purpose of this study was to evaluate the impact of phrenic nerve palsy on recovery following surgery for PA/VSD/MAPCAs.
METHODS: This was a retrospective review of 24 patients who underwent surgery for PA/VSD/MAPCAs. Sixteen patients were undergoing their first surgical procedure, whereas eight were undergoing re-operations. All 24 patients had clinical evidence of a new phrenic nerve palsy post-operatively and subsequently underwent diaphragm plication.
The median interval from primary surgery to diagnosis of phrenic nerve palsy was 11 days, and the median interval from diagnosis to diaphragm plication was an additional 2 days. A cohort of matched "controls" were identified based on identical diagnosis and procedures but who did not sustain a phrenic nerve palsy.
RESULTS: Eighteen of the 24 patients (75%) had clinical improvement following diaphragm plication as evidenced by the ability to undergo successful extubation (5 2 days), transition out of the intensive care unit (32 16 days), and discharge from the hospital (42 19 days). In contrast, there were six patients (25%) who did not demonstrate improvement following diaphragm plication, as evidenced by intervals of 61 38, 106 45, and 108 46 days, respectively (p < 0.05 for all three comparisons). The six patients who failed to improve following diaphragm plication had a significantly greater number of co-morbidities compared to the 18 patients who demonstrated improvement (2.2 vs 0.6 per patient, p < 0.05). These co-morbidities are summarized in the Table below. Compared to the "control" group, patients who improved following diaphragm plication spent an additional 22 days in the hospital at a differential cost of $1,127,000 in total charges and $100,000 in direct costs. Patients who failed to improve following diaphragm plication spent an additional 90 days in the hospital at a cost of $4,515,000 in total charges and $402,000 in direct costs. CONCLUSIONS: The data demonstrate a bifurcation of clinical outcome in patients undergoing diaphragm plication following surgery for PA/VSD/MAPCAs. This bifurcation appears to be linked to the presence or absence of other co-morbidities. Phrenic nerve palsy was associated with prolongation of hospital stay and came at a very significant financial price.

Co-morbidities
Patients who failed to improvePatients who improved
DiGeorge syndromen = 3n = 5
Alagille syndromen = 1n = 1
ECMOn = 1n = 1
Tracheostomyn = 3n = 1
Bronchial obstructionn = 0n = 1
Bronchomalacian = 2n = 0
Vocal cord paralysisn = 1n = 1
RUL collapsen = 1n = 0
Respiratory coden = 1n = 0


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