CA-32
When Two Wrongs Don't Make a Right; Anesthetic Management for Congenital Diaphragmatic Hernia (CDH) in a Neonate with Hypoplastic Left Heart Syndrome (HLHS) - A Case Report.
Safi A, Deer J
Ann & Robert Lurie Children's Hospital of Chicago, Chicago, IL, USA
Introduction: Hypoplastic left heart syndrome (HLHS) is a prevalent congenital cardiac anomaly with high mortality. The focus is on maintaining adequate pulmonary-to-systemic circulation ratio while keeping a low inspiratory fraction of oxygen (FiO2) (1). Congenital diaphragmatic hernia (CDH) results in various manifestations of pulmonary hypertension and are treated with a high FiO2 (1). Given competing cardiorespiratory goals, the management of combined HLHS and CDH can present challenges. We present to you a case of CDH repair in a neonate with HLHS.
Case: A 13 day old 2.9 kg female neonate born at 37 weeks was transferred to our institution. Medical abnormalities included CDH, coarctation of aorta, double outlet right ventricle complicated by HLHS, syndactyly of fingers and choanal atresia. Echocardiogram showed preserved right ventricular systolic function and severe pulmonary hypertension. The patient was intubated and mechanically ventilated from outside hospital. Patient remained on prostaglandin infusion to maintaining potency of the ductus arteriosis. Preoperative discussion with various teams agreed upon an open repair versus a laparoscopic approach. Anesthetic maintenance was achieved with infusions of midazolam, fentanyl and dexmedetomidine with intermittent bolus doses of neuromuscular paralytic agents. To minimize hemodynamic perturbations, a total intravenous anesthetic was utilized with avoidance of volatile anesthetic agents. The CDH repair was successful without any catastrophic intra operative events, despite the diaphragmatic defect encompassing almost the entire right heme-diaphragm. No vasopressors or NO was utilized during the case, but were available. At the end of the procedure, the patient was transferred smoothy back to the CCU.
Discussion: The isolated presence of congenital cardiac anomalies presents challenges for the pediatric anesthesiologist in the perioperative setting. The presence of CDH with congenital heart disease presents a unique challenge with an inherent high risk for mortality. Although HLHS occurs in 7.5 percent of infants with congenital heart anomaly, the incidence of associated extra- cardiac abnormalities is low in patients with HLHS (1). In an analysis of 122 cases of HLHS, no patient was found to have associated gastrointestinal disease(2). Furthermore, though CDH is sometimes associated with heart anomalies, its association with HLHS is rare (2). Minimal literature or reference support exists for managing this unique constellation of co-morbidities. Balancing analgesia, amnesia, and muscle relaxation while ensuring adequate pulmonary-to-systemic circulation was the primary anesthetic goal. The surgical outcome was successful and the patient remains critically ill due to her co-morbidities, but stable and awaiting cardiac palliation.
References: 1) Masaft Ntshimura, M.D.; Akihiro Taniguchi, M.D.; Hideaki lmanaka, M.D .; and Nobuyuki Taenaka, M.D. Hypoplastic Left Heart Syndrome Associated with Congenital Right- Sided Diaphragmatic Hernia and Omphalocele .Chest 1992; 101-I ;263-64
2) Kashani IA, Kimmons H, Valdes-Cruz LM, Swensson RE, Sahn DJ, Bloor C. Congenital right-sided diaphragmatic hernia and hypoplastic left heart syndrome. Am Heart J 1985; 109:177-78
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