Outcomes in children with hypoplastic left heart syndrome undergoing open fundoplication

Background/Purpose

Some institutions recommend early fundoplication in patients with hypoplastic left heart syndrome (HLHS) with signs of gastroesophageal reflux disease because of the risk of reflux-related cardiac events. However, their cardiac physiology may impose high perioperative morbidity and mortality. Therefore, we reviewed our experience with fundoplication in this population to allow for assessment of the risk-benefit ratio.

Methods

A retrospective review of patients with a diagnosis of HLHS who underwent a fundoplication from January 1990 to July 7, 2009, was performed. All patients underwent open fundoplication between first and second stages of cardiac repair.

Results

Thirty-nine patients were identified. There were 3 intraoperative complications: hemodynamic instability (n = 2) and a pulmonary hypertensive crisis requiring extracorporeal membrane oxygenation and termination of the procedure (n = 1). There were 27 postoperative complications in 16 patients. There were 2 deaths (4%) within 30 days, and there were 9 deaths (23%) in patients between their first and second stage of cardiac repair during the study period.

Conclusions

Noncardiac surgical procedures in patients palliated for HLHS have a high morbidity and mortality. We recommend that routine fundoplication in this population should only be performed under prospective protocols until the relative risk of operation vs risk of reflux is delineated.     

Anesthetic management of tracheoesophageal fistula repair in a newborn with hypoplastic left heart syndrome

We present a case of a newborn with hypoplastic left heart syndrome (HLHS) and tracheoesophageal fistula (TEF). The anesthesia management for the repair of the TEF is presented and the management of the unique pathophysiology of the HLHS circulation is discussed.     

Outcomes after laparoscopic surgery in neonates with hypoplastic heart left heart syndrome

Background/Purpose

Laparoscopy has advanced the care of children for a variety of pediatric surgical diseases. However, complication rates for laparoscopic interventions in neonates with hypoplastic left heart syndrome (HLHS) have not been well described. The purpose of this study is to present the largest reported series of laparoscopic surgery performed in patients with HLHS.

Methods

We conducted a single-institution, retrospective chart review for all neonates with HLHS who underwent a laparoscopic procedure from September 2002 to March 2005. Data regarding patient characteristics, intraoperative monitoring, previous cardiac surgery, perioperative complications, and postoperative mortality were assessed.

Results

Twelve patients with HLHS underwent a total of 13 operations during the study period (8 combined Nissen fundoplication and gastrostomy tubes, 3 isolated gastrostomy tubes, 1 Ladd procedure, and 1 combined Nissen fundoplication and gastrocutaneous fistula closure). All cases were completed laparoscopically. Patients had undergone palliative cardiac surgery but were not completely corrected; therefore, they were cyanotic. Perioperative complications were observed in 6 patients (3 gastrostomy tube site infections, 1 small bowel obstruction, 1 postoperative sepsis, and 1 urinary tract infection). There was no mortality in this series.

Conclusions

From this experience, it appears that laparoscopy can be performed safely and with satisfactory outcomes in patients with HLHS. However, a multidisciplinary approach, including the availability of a skilled and experienced cardiac anesthesia team, is believed to be critical to optimize outcomes in these critically ill children.     

Preservation of an aberrant left hepatic artery during laparoscopic nissen fundoplication.

OBJECTIVE: Laparoscopic Nissen fundoplication is performed in neonates and children for significant gastroesophageal reflux. An aberrant left hepatic artery encountered during laparoscopic Nissen fundoplication makes dissection around the esophageal hiatus more difficult if the artery is not transected. Although some suggest division of the aberrant left hepatic artery, this is associated with risk of significant hepatic injury from ischemia. We routinely preserve the aberrant left hepatic artery and sought to determine (1) the incidence of aberrant left hepatic artery and (2) the results following preservation of the aberrant left hepatic artery. METHODS: Between January 2000 and October 2002, 195 laparoscopic Nissen fundoplications were performed. We documented intraoperative findings of each procedure, and reviewed postoperative radiographic studies and clinic visits. RESULTS: In 30 patients (15%), an aberrant left hepatic artery was identified. All dissections were performed laparoscopically with the Nissen fundoplication positioned cephalad to the aberrant left hepatic artery. Postoperatively, 2 patients (6%) have had evidence of wrap failure. The remainder of the patients has had normal radiographic studies or no clinical evidence of reflux during clinic visits. CONCLUSION: During laparoscopic Nissen fundoplication in neonates and children, an aberrant left hepatic artery may be encountered in approximately 15% of patients. When an aberrant left hepatic artery is identified, it should be preserved to avoid the potential risk of hepatic ischemic injury.     

Anesthetic management of the hybrid stage 1 procedure for hypoplastic left heart syndrome (HLHS)

Introduction: Despite advances in the surgical and perioperative management of patients with hypoplastic left heart syndrome (HLHS), outcomes for this high‐risk group of patients remains suboptimal. The hybrid approach [bilateral pulmonary artery (PA) banding, ductal stenting, balloon atrial septostomy], is an emerging alternative therapy for the management of HLHS, which defers the risks of a major surgical repair until the infants are older. This article will describe our experience providing the anesthetic management of patients undergoing the hybrid procedure. Methods: After Institutional Review Board approval, we retrospectively reviewed the records of 77 patients who underwent the hybrid procedure as neonates between July 2002 and August 2008. We reviewed both the anesthetic and intensive care records. Results: The hybrid procedure was performed in 77 patients (31 female and 46 male). The average age of the patients was 11.8 days with an average weight of 2.98 kg. Fentanyl was used for analgesia at an average dose of 5.7 mcg·kg^?1. The average increase in the systolic blood pressure after placement of the right and left PA bands was 11.3 mmHg. The average drop in the systemic saturation after placement of the bands was 7%, with an average postband and stent SaO₂ of 82%. Twenty‐one patients received blood transfusion (27.3%) at an average dose of 43.5 ml (14.5 ml·kg^?1). Forty patients received albumin during the case (51.9%) at an average dose of 23.2 ml (7.7 ml·kg^?1). Seventeen patients arrived at the hybrid suite already intubated, and no attempt was made to extubate these patients at the end of the case. Thirty‐six patients were extubated at the end of the procedure, and a total of 64.9% of patients were extubated within the first 24 h postoperatively. Patients had notably stable hemodynamics throughout the first 24 h in the intensive care unit. Discussion: Patients undergoing the hybrid procedure have relatively stable intraoperative and early postoperative hemodynamics. The procedure is performed without cardiopulmonary bypass (CPB) and with minimal narcotic and anesthetic exposure. Patients typically do not require blood transfusions or inotropic support and are extubated at either the end of the procedure or within 24 h of ICU admission. In our experience, the anesthetic management of patients undergoing the hybrid procedure is straightforward and requires relatively few interventions when compared to traditional neonatal surgical repairs. Deferring the risks of anesthesia, CPB, hypothermic circulatory arrest, and prolonged postoperative sedation may yield developmental advantages to patients born with HLHS.     

Anesthetic management of palliative surgery for hypoplastic left heart syndrome--a case report]

The first patient with hypoplastic left heart syndrome who was treated successfully by palliative surgery at our hospital is reported. Soon after birth, the female infant showed tachypnea and cyanosis, and was transferred to our institution under a presumptive diagnosis of HLHS. Although cardiologists confirmed the diagnosis by two-dimensional echocardiography, the surgery was postponed for one month because it was possible to keep the ductus arteriosus open without PGE1, and the patient showed no deterioration. Fortunately, an abnormal vessel connecting the left atrium with the superior vena cava relieved severe pulmonary venous congestion by diverting the blood flow. During the pre-CPB period, frequent adjustment of the oxygen concentration and ventilator setting was required in order to keep the blood gas values optimal compared with the values before surgery. After CPB, adequate blood pressure using catecholamines and hyperventilation with 100% oxygen was necessary to increase the pulmonary blood flow and to decrease the pulmonary vascular resistance. It is concluded that preservation of the balance between PVR and SVR during the perioperative period, and adequate systemic arterial pressure after CPB are crucial. Furthermore, constant and intense observation is mandatory to facilitate immediate treatment even after surgery in case of systemic hypoperfusion due to excessive pulmonary blood flow.     

Preoperative use of selective COX-II inhibitors for pain management in laparoscopic nissen fundoplication

Background This randomized, double-blind, prospective, placebo controlled study was planned to determine the effectiveness of selective COX-II inhibitors used preoperatively to alleviate pain after Nissen fundoplication surgery. Methods For this study, 60 patients were allocated to four groups at random: group C (celecoxib, 200 mg by mouth), group R (rofecoxib, 50 mg by mouth), group P (placebo, pill), or group D (diclophenac sodium, 75 mg intramuscularly). Postoperative abdominal and shoulder pain experienced by the patient at rest, with motion, and with coughing were assessed. Side effects and postoperative analgesic requirement (tramadol, intramuscular) also were recorded. Results The median tramadol requirement in the 1st h and total tramadol requirement at the 24th h were higher in group P than in the other study groups (p < 0.0l). The pain scores in the first postoperative hour were higher in group P (p < 0.05). Conclusions The preoperative use of celecoxib, rofecoxib, or diclophenac in laparoscopic Nissen fundoplication surgery decreases pain intensity and tramadol requirement in the first postoperative hour and has a trarnadol sparing effect in the first 24 h. Presented at the European Society of Anaesthesia Annual Meeting 5–8 June 2004, Lisbon Portugal     

Internal hernia as a complication of laparoscopic nissen fundoplication

A rare case of strangulated small bowel through a defect in the falciform ligament is presented to emphasize the possibility of complication after the creation of a window in the falciform ligament during laparoscopic surgery.     

Experience with operations for hypoplastic left heart syndrome

Aortic atresia is a form of congenital cardiac disease complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. Occasionally, neonates with severe aortic stenosis have associated severe hypoplasia of the ascending aorta and left ventricle. These two defects constitute the most prevalent forms of so-called hypoplastic left heart syndrome. At present, this lesion is universally fatal with no established surgical management. This report describes our experience with the evolution and evaluation of staged surgical management of infants with hypoplastic left heart syndrome.     

Surgical treatment of hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) was previously uniformally fatal within the first month of life. The development of the Norwood stage I operation has afforded new hope to the families of newborn infants with this defect. Recent, modification of the Norwood procedure has improved the surgical results. The one-year survival rate after the Norwood procedure is from 50% to 70% in major institutions. The results of second palliation (hemi-Fontan operation or bidirectional Glenn operation) are nearly acceptable. Recently, fetal echocardiography has allowed early diagnosis of HLHS, and after a prenatal diagnosis of HLHS, couples may be offered termination of the pregnancy. It is necessary to improve the results of the Norwood stage I operation, to save more fetuses and neonates with HLHS.     

Population pharmacokinetics of milrinone in neonates with hypoplastic left heart syndrome undergoing stage I reconstruction

We performed a blinded, randomized pharmacokinetic study of milrinone in 16 neonates with hypoplastic left heart undergoing stage I reconstruction to determine the impact of cardiopulmonary bypass and modified ultrafiltration on drug disposition and to define the drug exposure during a continuous IV infusion of drug postoperatively. Neonates received an initial dose of either a 100 or 250 microg/kg of milrinone into the cardiopulmonary bypass circuit at the start of rewarming. Postoperatively, milrinone was infused to clinical needs. A mixed-effect modeling approach was used to characterize milrinone pharmacokinetics during cardiopulmonary bypass, modified ultrafiltration, and postoperatively using the NONMEM algorithm. All patients in this study demonstrated a modified ultrafiltration concentrating effect that occurred despite a modified ultrafiltration drug clearance of 3.3 mL x kg(-1) x min(-1). The infants in this study demonstrated an impaired renal clearance during the immediate postoperative period. A constant infusion of 0.5 microg x kg(-1) x min(-1) resulted in drug accumulation during the initial 12 h of drug administration. Postoperatively, milrinone clearance was significantly impaired (0.4 mL x kg(-1) x min(-1)), improved by the 12th postoperative hour, and approached steady-state clearance (2.6 mL x kg(-1) x min(-1)) by postoperative day 4. In the postoperative setting of markedly impaired renal function, an infusion rate of 0.2 microg x kg(-1) x min(-1) should be considered.     

Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome

Despite improved early results with the Norwood procedure (stage one palliation), patients remain with at-risk anatomy and interstage mortality continues to be a limitation of staged single ventricle palliation. Retrospective analyses have implicated residual or recurrent anatomic lesions as well as intercurrent illness as causes of interstage mortality. We hypothesized that potentially life-threatening anatomic lesions and illnesses would be manifest before serious physiologic impact by alteration in arterial saturation, failure to gain weight or in the case of dehydration, acute weight loss. As a result, we developed a home monitoring program of daily weights and oxygen saturations to earlier identify those patients at increased risk for interstage death. Frequent monitoring of these physiologic variables between stage one and two palliation identified life-threatening anatomic lesions and illness and permitted timely intervention that ultimately improved survival. All 36 survivors of the stage one palliation discharged from the hospital and entered into the home monitoring program survived the interstage period.     

Noncardiac surgery in children with hypoplastic left heart syndrome

Background/Purpose: Hospital mortality rate among children with hypoplastic left heart syndrome (HLHS) after cardiac repair is well documented, but comparable data after noncardiac, surgical procedures are unknown. The authors hypothesized an increasing number of noncardiac procedures were being performed on children with HLHS, less than 2 years of age, from 1988 to 1997, and that these procedures were associated with a substantial mortality rate. Methods: A retrospective review of hospital discharge data for 2,457 children less than 2 years of age with HLHS for 1988 through 1997 was performed. The authors examined the outcomes of HLHS children undergoing only noncardiac surgical procedures during their hospital stay. Differences in hospital mortality rates between 1988 through 1992 versus 1993 through 1997 were assessed using the [Chi ]² square statistic. Results: Nineteen percent of the 147 children with HLHS undergoing noncardiac, surgical procedures died (95% CI, 13% to 25%). Comparing the 2 study periods, there was no significant change in outcome among HLHS children undergoing noncardiac, surgical procedures (78% v. 83%; P [gt ] .1). There was no significant difference in the percentage of hospital discharges with noncardiac, surgical procedures performed per year. Conclusions: Although children with HLHS were not undergoing an increase in the number of noncardiac surgical procedures performed annually, even minor surgical procedures were associated with considerable mortality. Outcomes after noncardiac surgery in high-risk children with congenital heart disease warrant further investigation. J Pediatr Surg 37:1399-1403.     

Effect of laparoscopic failure on outcome of laparoscopic nissen fundoplication: independent review

PURPOSE: This study was undertaken to determine whether conversion or early reoperation contributed significantly to the eventual outcome of laparoscopic Nissen fundoplication. PATIENTS AND METHODS: An independent surgeon, blinded to the operative events, administered two general and one system-specific quality of life tools to the first 100 consecutive patients booked for laparoscopic fundoplication in a community hospital, where the open conversion rate was 4 patients/surgeon and the early reoperation rate 1.5 patients/surgeon for the first 20 operations. Patients were also asked about need for medication, dysphagia, satisfaction (analog scale), and whether, if given it to do over, they would opt for surgery again. RESULTS: Of the original 100 patients, 40 were studied an average of 5 years after surgery: 26 completed laparoscopically and 14 with laparoscopic failure (13 conversions and 1 early reoperation). Patient characteristics in the two groups were similar, except that there were more older patients with more fixed intrathoracic hiatus hernias in the failure group. Among the parameters examined, no statistically significant differences could be detected between laparoscopic and converted patients. CONCLUSIONS: General and system-specific quality of life, digestive symptoms, need for medication, patient satisfaction, and willingness to have surgery over again are not altered by conversion or reoperation. Thus, surgeons who have adequate laparoscopic skills, experience with open fundoplication, and training in laparoscopic Nissen fundoplication should feel free to add it to their repertoires provided the likelihood of conversion and reoperation secondary to inexperience is understood by the patient.     

Surgical treatment for hypoplastic left heart syndrome

Once considered a uniformly fatal condition, the outlook for newborns with hypoplastic left heart syndrome has been dramatically improved with either a protocol of staged reconstruction or cardiac transplantation. Currently, a significant shortage of suitable donor hearts restricts the applicability of transplantation for most newborns. At the University of Michigan, we have adopted a policy of staged reconstruction for all patients with hypoplastic left heart syndrome, reserving transplantation only for those unsuitable for reconstructive techniques. Between January 1990 and September 1998, 303 patients underwent the Norwood operation for classic hypoplastic left heart syndrome with an overall hospital survival of 76%. Among patients considered at standard risk, survival was significantly higher (86%) than that for those patients with important risk factors (42%), p=0.0001. Adverse survival was most strongly associated with significant associated noncardiac congenital conditions (p=0.008) and severe preoperative obstruction to pulmonary venous return (p=0.03). Survival following second stage reconstruction with a hemi-Fontan or bidirectional Glenn procedure was 98%. The Fontan procedure has been completed in 117 of these patients with a hospital survival rate of 91%. Survival after the Fontan procedure improved significantly when the second stage of the reconstruction was completed with a hemi-Fontan procedure compared to a bidirectional Glenn, 98% vs 81%, p<.05. Among the patients considered at standard risk, actuarial survival was 70% at 5 years. The largest decrease in survival occurred in the first month of life and late deaths affected primarily those patients in the high risk group. Neurodevelopmental outcome studies demonstrated normal verbal and performance scores in the majority of patients. Among centers utilizing a protocol of transplantation, donor organ shortages have resulted in a mortality of approximately 25% while awaiting transplantation with 5 year survival rates for those actually receiving organs essentially equal to those for staged reconstruction. Staged reconstruction and transplantation have significantly improved the intermediate-term outlook for patients with hypoplastic left heart syndrome. Factors addressing improvements in early first stage survival following the Norwood would be expected to add significantly to an overall improved late outcome. Outcome following cardiac transplantation is limited by donor availability in addition to the late complications of infection, rejection, graft atherosclerosis, and lymphoproliferative disease.