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.     

Edge-to-edge tricuspid valve repair in hypoplastic left heart syndrome

Tricuspid regurgitation (TR) is known to be a risk factor for mortality in the surgical management of patients with hypoplastic left heart syndrome (HLHS). Concomitant repair for TR should be considered when the TR is moderate to severe to achieve successful Fontan completion. The present case was a 20-month-old girl who was diagnosed with HLHS (mitral atresia and aortic atresia). She underwent a Norwood procedure as the first palliation followed by a Glenn procedure. After that, she gradually developed TR, which progressed to a severe state at the time of the Fontan procedure. An edge-to-edge tricuspid valve repair, in which the anterior and septal leaflets were sutured together, was performed simultaneously with the extracardiac Fontan procedure. Discharge echocardiography revealed that the degree of TR was less than mild. The technique is simple, not time-consuming, and may be an effective adjunct for successful completion of the Fontan procedure in these patients.     

Norwood procedure for hypoplastic left heart syndrome associated with valvular pulmonary stenosis

Association between hypoplastic left heart syndrome and valvular pulmonary stenosis is very rare. Severity of valvular pulmonary stenosis in this setting limits management options. Consequently, patients with this condition are considered poor candidates for Norwood stage one reconstruction. Herein, we describe a newborn with hypoplastic left heart syndrome and significantly dysplastic pulmonary valve who successfully underwent the Norwood procedure with neoaortic valve reconstruction. Therefore, the Norwood procedure with neoaortic valve reconstruction might be an option for this difficult condition.     

Orthotopic heart transplantation and concomitant aortic arch replacement in an adult Fontan patient with hypoplastic left heart syndrome

Heart failure and neoaortic dilation are common long-term complications in patients with hypoplastic left heart syndrome after single-ventricle palliation. Orthotopic heart transplantation and concomitant transverse aortic arch replacement can be performed safely and effectively. The addition of preoperative three-dimensional modelling can supplement operative planning, facilitate intraoperative execution, and optimize clinical outcomes.     

Right ventricle-to-pulmonary artery shunt and modified Blalock–Taussig shunt in preparation to hemi-Fontan procedure in children with hypoplastic left heart syndrome

Objective: The advantageous effect of right ventricle-to-pulmonary artery shunt (RV–PA) on the early postoperative hemodynamics in the Norwood procedure for hypoplastic left heart syndrome (HLHS) is well known. Numerous controversies still exist with respect to the late consequences of this new palliation method in preparation for the second stage procedure. Methods: Between September 1997 and September 2004, a consecutive series of 78 children with HLHS from a single institution underwent the hemi-Fontan procedure: Group 1 (n=27) after Blalock–Taussig shunt (BT), and Group 2 (n=51) after RV–PA. Hemodynamic, echocardiographic and clinical perioperative data were analyzed. Results: There were no significant differences in the age and operative weight (Group 1: 6.9±1.04 months, 6.22±0.99 kg; Group 2: 6.57±1.12 months, 6.36±0.86 kg). Children after RV–PA were characterized by a significantly higher preoperative hematocrit value (P=0.014), lower aortic and superior vena cava oxygen blood saturation (P<0.001, P=0.024), severe right ventricle hypertrophy more rarely diagnosed in echocardiography (P<0.004), lower Qp:Qs ratio (P=0.011), larger right (P=0.001) and left (P=0.006) pulmonary artery index and a shorter intensive care unit stay after the hemi-Fontan procedure (P=0.004). Conclusions: The Norwood procedure with the RV–PA shunt provides satisfactory late hemodynamics and improves the development of the pulmonary arteries. Children with hypoplastic left heart syndrome subjected to this new method of palliation are good candidates for the hemi-Fontan procedure.     

Improvement in right ventricular function by mitral valve closure in hypoplastic left heart syndrome

A male neonate presented with the aortic/mitral stenotic variant of hypoplastic left heart syndrome, wherein the suprasystemic left ventricular pressure and relatively large left ventricle had shifted the intraventricular septum. Despite bilateral pulmonary artery banding, the stroke volume was difficult to maintain owing to the compressed right ventricle, causing heart failure symptoms. Percutaneous balloon aortic valvuloplasty decreased the left ventricular pressure, restoring the right ventricular function. Norwood procedure with mitral valve closure after catheter intervention reduced the left ventricular size and improved the right ventricular function. This paper refers to the potential of mitral valve closure for hypoplastic left heart syndrome.     

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome

Objective. Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. Methods. Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. Results. Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 ± 2.6 mmHg, cardiac index of 3.6 ± 0.8 l/kg/min, and PA index of 194.0 ± 58.4 mm²/m²; there was no difference between the groups. Conclusion. The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity. Presented at the 59th Annual Scientific Meeting of the Japanese Association for Thoracic Surgery, held in Tokyo, Japan, October 1–4, 2006     

Norwood with right ventricle-to-pulmonary artery conduit is more effective than Norwood with Blalock–Taussig shunt for hypoplastic left heart syndrome: mathematic modeling of hemodynamics

Objective: The introduction of right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure for hypoplastic left heart syndrome resulted in a higher survival rate in many centers. A higher diastolic aortic pressure and a higher mean coronary perfusion pressure were suggested as the hemodynamic advantage of this source of pulmonary blood flow. The main objective of this study was the comparison of two models of Norwood physiology with different types of pulmonary blood flow sources and their hemodynamics. Method: Based on anatomic details obtained from echocardiographic assessment and angiographic studies, two three-dimensional computer models of post-Norwood physiology were developed. The finite-element method was applied for computational hemodynamic simulations. Norwood physiology with RV-PA 5-mm conduit and Blalock–Taussig shunt (BTS) 3.5-mm shunt were compared. Right ventricle work, wall stress, flow velocity, shear rate stress, energy loss and turbulence eddy dissipation were analyzed in both models. Results: The total work of the right ventricle after Norwood procedure with the 5-mm RV-PA conduit was lower in comparison to the 3.5-mm BTS while establishing an identical systemic blood flow. The Qp/Qs ratio was higher in the BTS group. Conclusions: Hemodynamic performance after Norwood with the RV-PA conduit is more effective than after Norwood with BTS. Computer simulations of complicated hemodynamics after the Norwood procedure could be helpful in establishing optimal post-Norwood physiology.     

Spinal anaesthesia in a child with hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS), a complex congenital heart disease, is the most common lethal cardiac defect in neonates. Its treatment includes cardiac transplantation and/or surgical palliation. Associated extracardiac congenital abnormalities are exceptional. We report the case of a neonate with HLHS and anorectal atresia who required urgent surgical management to relieve intestinal obstruction. The surgery was successfully performed under spinal anaesthesia.     

Hybridizing the hybrid: a “serendipitous bridge” to heart transplantation in a child with hypoplastic left heart syndrome

We report a unique experience with stage 1 palliation of hypoplastic left heart syndrome (HLHS) using the hybrid approach. The tortuous anatomy of the ductus arteriosus precluded stenting. Persistent ductal patency and ability to support the systemic circulation adequately after prolonged prostaglandin E1 (PGE1) infusion and its subsequent discontinuation presented us with a novel approach to hybrid palliation of hypoplastic left heart syndrome. Our experience offers a tempting approach, but it should not be considered a standard alternative management option to stage I hybrid palliation based on a single case report.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12055-020-01088-8.     

Bilateral pulmonary artery banding for hypoplastic left heart syndrome and related anomalies

Objective Bilateral pulmonary artery banding is considered an option for initial palliation in high-risk patients with hypoplastic left heart syndrome or related anomalies. However, there are potential interim morbidities, including ductal constriction, compromised growth of the ascending aorta, and pulmonary artery stenosis at the banding site. In addition, there are still controversies regarding the optimal timing and choice of the second-stage operation. Methods Between February 2003 and October 2005, twelve high-risk patients with hypoplastic left heart syndrome or related anomalies underwent bilateral pulmonary artery banding. An atrial septal defect was created simultaneously when the septum was intact or the defect was restrictive. After the procedure, intravenous prostaglandin E₁ was continuously administered. Results The median weight was 3.0 kg (range 1.8–3.6 kg), and the median age was 16 days (range 0–27 days). Atrial septum defect creation was performed simultaneously in two patients. Ten of the twelve patients were discharged from the hospital. One patient died of progressive multiple organ failure and another patient died of cerebral hemorrhage. Eight patients developed various degrees of ductal constriction and required dose adjustment of prostaglandin E₁. Two patients underwent ductal arteriosus stent placement: one was unsuccessful owing to the size mismatch between the stent and the ductus. The diameter of the ascending aorta remained unchanged until the time of the second-stage operation, except in two patients who had a sufficiently sized aorta at the time of pulmonary artery banding. Pulmonary arterial branch stenosis developed at the banding site in three patients. At the time of the second-stage operation, the Norwood procedure was performed in seven patients, biventricular repair in two, and Norwood plus Glenn procedure in one. Conclusion Bilateral pulmonary artery banding is an option to salvage high-risk neonates with hypoplastic left heart syndrome or related anomalies. However, it is associated with high interstage morbidity, and patients may benefi t from early conversion to the Norwood operation.     

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.     

Norwood-I in a hypoplastic left heart variant and right aortic arch

Even though preoperative diagnostics have improved significantly, intraoperative surprises may still occur especially in the case of complex congenital heart disease. An instance of such a complex congenital heart disease is a hypoplastic left heart syndrome with a right-sided aortic arch. In this case report, we present 1 patient with such a complex and unexpected anatomy, as well as a possible way to overcome the obstacles.     

Biliary atresia associated with hypoplastic left heart syndrome: a case report and review of the literature

Biliary atresia is a cholestatic disorder of infancy that is associated with other anatomic anomalies in approximately 20% of cases. These frequently are defects in situs determination and laterality, causing syndromes of heterotaxy and complex heart defects. The authors describe an infant with both biliary atresia and hypoplastic left heart syndrome (HLHS) in the absence of a laterality defect or evidence of any other morphologic defect. The presence of a hypoplastic left ventricle in a patient with biliary atresia has previously been reported only in the context of a heterotaxy syndrome. The coexistence of these 2 disorders raises etiologic considerations and represents a potential challenge for the surgical treatment of both conditions.     

Gastric fundoplication is effective in promoting weight gain in children with severe congenital heart defects

Aim

The aim of this study was to determine outcomes, including weight gain, morbidity, and mortality, of children with severe congenital heart disease who underwent fundoplication (FP) for gastroesophageal reflux disease.

Methods

An institutional review board-approved retrospective review was conducted on all children with congenital heart disease who underwent FP from 1999 to 2005. Preoperative age, weight, cardiac procedures, postoperative weight, and mortality were extracted from medical records. The Wilcoxon signed rank, Wilcoxon rank sum, and log-rank tests were used; P value less than .05 was significant. All procedures were performed with dedicated cardiac anesthesia personnel with recovery in a cardiac intensive care unit.

Results

Of 112 subjects identified, 37 (33%) had single ventricle (SV) physiology. The most frequent cardiac procedures performed were Norwood (33), pulmonary artery band (11), and systemic pulmonary artery shunt (11). A total of 104 laparoscopic FPs (with 2 conversions to open) and 8 open FPs were performed. The median preoperative age was 3 months, and weight percentile was 1.5%. From baseline, postoperative median weight percentiles increased to 4% at 3 months (P < .001) and to 20% at 5 years postoperatively (P = .004). Single ventricle physiology had no significant effect on outcomes. Postoperative mortality (≤30 days) was 4.5% (5/112); 5-year survival was 74% (83/112). Five-year survival of SV subjects (59%) was significantly lower (P = .03) than that of the other subjects (81%). No significant difference in survival was seen between SV subjects with FP and all SV patients seen at our center during the study period. Only one death was directly related to antireflux surgery (SV subject). There were 8 patients who had recurrent gastroesophageal reflux disease: 4 were treated with reoperation, and 4 were treated medically.

Conclusion

Weight gain in this high-risk population can be expected after antireflux surgery. Mortality is high because of intrinsic disease, especially in the SV population. Fundoplications performed with the assistance of dedicated pediatric cardiac anesthesia personnel followed by recovery in a cardiac intensive care unit is possible with acceptable postoperative morbidity and mortality.     

Modified Norwood operation for hypoplastic left heart syndrome

Background. We examined early results in infants with hypoplastic left heart syndrome undergoing the Norwood operation with perioperative use of inhaled nitric oxide and application of extracorporeal membrane oxygenation.

Methods. Medical records were reviewed retrospectively.

Results. Between April 1997 and March 2001, 50 infants underwent a modified Norwood operation for hypoplastic left heart syndrome. Mean age at operation was 7.5 ± 5.7 days, and mean weight was 3.1 ± 0.5 kg. Five infants had a delayed operation because of sepsis. The mean diameter of the ascending aorta by echocardiography was 3.6 ± 1.8 mm. Ductal cannulation was used to establish cardiopulmonary bypass in all patients. Mean circulatory arrest time was 39.4 ± 4.8 minutes. The size of the pulmonary-systemic shunt was 3.0 mm in 6 infants, 3.5 mm in 37, and 4.0 mm in 7. Infants with persistent hypoxia (partial pressure of oxygen < 30 mm Hg) received nitric oxide after they were weaned from cardiopulmonary bypass. Extracorporeal membrane oxygenation was initiated in 8 infants in the pediatric intensive care unit primarily for low cardiac output and in 8 in the operating room because of the inability to separate them from cardiopulmonary bypass. The 30-day mortality rate was 22% (11 of 50 patients), and the hospital mortality rate was 32% (16 of 50 patients). Mean follow-up was 17 months. Ten patients (20%) underwent stage-two repair, with one operative death. One survivor had a Fontan procedure, and 2 underwent heart transplantation, with one death.

Conclusions. Early application of extracorporeal membrane oxygenation for hemodynamic instability and selective use of nitric oxide for persistent hypoxia in the immediate postoperative period may improve survival of patients with hypoplastic left heart syndrome. Renal failure requiring hemofiltration during extracorporeal membrane oxygenation (p < 0.05) and cardiopulmonary arrest in the pediatric intensive care unit (p < 0.05) were predictors of hospital mortality.     

Anesthetic management of infants with palliated hypoplastic left heart syndrome undergoing laparoscopic nissen fundoplication

The safety of laparoscopic surgery in infants with single ventricle physiology has been a subject of controversy despite potential benefits over open surgery. We present the anesthetic management of five infants with palliated hypoplastic left heart syndrome that underwent laparoscopic Nissen fundoplication. After anesthetic induction and tracheal intubation, an intraarterial catheter was placed for hemodynamic monitoring. Insufflation pressure was limited to 12 mm Hg and was well tolerated by all patients. There were no intraoperative or postoperative complications. In patients with hypoplastic left heart syndrome, laparoscopic Nissen fundoplication can be safely performed with careful patient selection and close intraoperative monitoring.     

A combined stage 1 and 2 repair for hypoplastic left heart syndrome: anaesthetic considerations

Therapy of hypoplastic left heart syndrome (HLHS) consists of the staged Norwood procedure or cardiac transplantation. Stenting the ductus arteriosus and subsequent banding of the pulmonary arteries allows the combination of neoaortic reconstruction with the establishment of a bidirectional cavopulmonary connection (combined stage 1 and 2 procedure) in a later session. We report the anaesthetic management in eight infants ranging from 107 to 195 days undergoing a combined stage 1 and 2 procedure. Nonselective pulmonary vasodilators and nitric oxide were needed in all cases to improve oxygen saturation in the postbypass period. Phosphodiesterase inhibitors and epinephrine were required in all patients for inotropic support during and after weaning off cardiopulmonary bypass. The procedure was successful in seven patients. One patient died intraoperatively because of right heart failure. The physiological changes of this new surgical strategy for palliation of HLHS offers a challenge for the anaesthetist primarily in the early postbypass period.