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.
