The Science Journal of the American Association for Respiratory Care

2004 OPEN FORUM Abstracts

PEDIATRIC POST-OPERATIVE MANAGEMENT WITH FLOLAN AND NITRIC OXIDE IN A PATIENT WITH CONGENITAL HEART DISEASE (CHD) AND AGENESIS OF THE RIGHT LUNG

Douglas E. Petsinger BS, RRT, Susan Roark RRT, Jeryl Huckaby RRT, Angel Cuadrado MD, CICU Children’s Healthcare of Atlanta

INTRODUCTION: A 4.5 mo. 5.0 Kg female diagnosed with Total Anomalous Pulmonary Venous Connection (TAPVR) to the Coronary Sinus and Right Lung Agenesis (absent right lung and corresponding architecture) that was being managed medically by her primary Cardiologist. Respiratory distress developed requiring intubation and mechanical ventilation. The patient was transferred to the Sibley Heart Center’s Cardiac Intensive Care Unit (CICU) of Children’s Healthcare of Atlanta. Cardiac Catheterization revealed a QP/QS of 2:1 with R>L shunting through an ASD and significant Pulmonary Artery Hypertension (PAH). Despite maximal medical management including inhaled Nitric Oxide (iNO) with Conventional Mechanical Ventilation (CMV) the patient was not progressing. A surgical option was offered to the patient’s parents.

CASE SUMMARY: The Coronary Sinus was unroofed to allow drainage of the Pulmonary Veins to the Left Atrium, the ASD was sutured closed, and a small Left SVC was ligated. The mediastinum was left open to reduce sequella from post-operative edema. A Pulmonary Artery Catheter along with a Left Atrial Catheter were used to assess hemodynamics. The patient remained on iNO at 20 PPM. Despite maximal mechanical ventilation, pharmacologic paralyzation, and optimal sedative-analgesic management the patient demonstrated frequent bouts of Suprasystemic Pulmonary Hypertension that required frequent interventions. A small window of patient stability occurred, the mediastinum was closed on post-operative day four and the iNO was slowly weaned off. The patient hemodynamically deteriorated requiring reinstituting iNO along with starting FLOLAN and titrating to maximum dosage. The Pulmonary Artery Pressures were noted to be less than1/2 systemic with both iNO and FLOLAN. On post-operative day eight the hemodynamics and resultant acid-base balance were optimal, the patient was extubated. Initially, the patient was on a 2.5 LPM 97% O2 NC with 20 PPM iNO. The patient’s hemodynamics and SpO2 suffered, requiring mask CPAP of +20 cmH2O at 97% O2 and 20PPM iNO with positive results. The patient was then placed on naso-pharyngeal CPAP (NPCPAP), allowing the patient’s hemodynamics to be stabilized. Over the next 48 hours, the CPAP and iNO could be weaned off. The patient was discharged home on FLOLAN.

DISCUSSION: The utilization of non-invasive ventilation as a bridge to unassisted ventilation is a strategy that is becoming standard of care for many different populations. Titrating iNO to achieve stabilization of pulmonary hemodynamics is successful in many patients in the short term, but may not be enough. The addition of FLOLAN, the patient’s pulmonary hypertension stabilized, allowing the weaning of both ventilatory support and iNO. Home use of iNO is fraught with both legal and technical issues. Although the use of FLOLAN requires the need for central IV access, its use can result in the patient to be discharged sooner.

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