Surgical treatment for hypoplastic left heart syndrome: case reports.

A 58-day-old girl with hypoplastic left heart syndrome underwent bypass grafting from the pulmonary artery to the descending aorta, ligation of the ductus arteriosus, and banding of the main pulmonary artery distal to the graft. Anastomoses of the graft were performed by partial clamping of the arteries under moderate surface hypothermia. The patient tolerated the procedure well and was discharged from the intensive care unit on the eleventh postoperative day, but she died on the twentieth day of metabolic derangement. Our total experience with this anomaly includes three other infants, and a summary of these patients and applied surgical procedures are presented. Surgical and diagnostic considerations for this anomaly based on our experiences are discussed.     

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.     

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.     

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.     

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.     

Method of heart transplantation for treatment of hypoplastic left heart syndrome

Technical details of investigational orthotopic cardiac transplantation for management of hypoplastic left heart syndrome in a neonate are presented. Extracorporeal perfusion technique and need for extensive aortic arch reconstruction are emphasized. Although this experience was with a subhuman primate (baboon) donor, source of donor graft makes little difference with regards to the unique technical aspects of cardiac transplantation in a ductus-dependent newborn infant with a diminutive aortic arch.     

Phenoxybenzamine in the treatment of hypoplastic left heart syndrome: a core review

Perioperative management of neonates after the Norwood procedure is extremely complex. Limited reserve of the neonatal single ventricle and the parallel arrangement of the pulmonary and systemic circuits result in a tenuous balance between pulmonary and systemic blood flows. Precise manipulations of both pulmonary and systemic vascular resistance are necessary to prevent excessive pulmonary blood flow at the expense of systemic oxygen delivery. An emerging treatment strategy aimed at improving early mortality is the intraoperative administration of phenoxybenzamine, a profound systemic vasodilator. Maximum systemic vasodilation is thought to reduce afterload of the single ventricle and produce a more stable parallel circulation by ameliorating the postoperative fluctuations in systemic vascular resistance. Although this strategy has gained popularity at many centers, it is not without scrutiny. The following review provides an overview of the pharmacology of phenoxybenzamine, the surgical and physiologic implications of the Norwood procedure, and a discussion of the pros and cons of phenoxybenzamine administration.     

A proposed technique for treatment of hypoplastic left heart syndrome

The Hypoplastic Left Heart Syndrome includes a variety of anomalies, all characterized by a hypoplastic or absent left ventricle. A technique is described for producing a normal course of circulation in these cases. Its ultimate success will depend on the patients having a low pulmonary vascular resistence.     

Surgical treatment of coronary sinus orifice atresia with hypoplastic left heart syndrome after total cavo-pulmonary connection

Atresia of the coronary sinus orifice is rare. We describe the surgical treatment of coronary orifice atresia in an infant with a persistent left superior vena cava after total cavo-pulmonary connection for hypoplastic left heart syndrome. The diagnosis was made by cardiac catheterization after total cavo-pulmonary connection at 8 months of age. After surgery, cardiac performance deteriorated. At reoperation, the coronary sinus was fenestrated to the left atrium. The patient survived surgical treatment of coronary sinus ostial atresia unroofed to the left atrium, guiding the placement of the fenestration with a probe placed through the open cardiac end of left superior vena cava.     

Norwood procedure to hypoplastic left heart syndrome

Since March 1992, 25 neonates and small infants with HLHS have undergone a modified Norwood procedure. The mean age and weight at operation were 17 days (2 days-2 months) and 2.7 kg (1.6–3.3 kg). Isolated cerebral and/or myocardial perfusion (ICMP) with direct anastomosis of aorta and pulmonary artery was utilized since January 1995 to 16 patients. Under median sternotomy, PTFE graft (usually 3.0–3.5 mm) was anastomosed to the brachiocephalic artery and the arterial cannula was inserted to this PTFE graft. The left carotid and the left subclavian arteries were snared and a clamp was placed on the aortic arch just distal to the brachiocephalic artery. This allowed blood to enter the brain and the coronary arteries, keeping the brain perfused and the heart-beating. After reconstruction of distal aortic arch, a single dose of crystalloid cardioplesia was infused and the rest of the arch was reconstructed. There were 14 early deaths (56%) and 4 late deaths (16%). Bidirectional Glenn procedure was performed to 5 patients with 1 death. Three patients underwent modified Fontan procedure without mortality. Mean aortic cross clamp time was 24 min. and mean ICMP time was 32 min. There was no neurologic complications. In conclusion, isolated cerebral and/or myocardial perfusion may offer an advantage of protecting the brain and myocardium during arch repair in Norwood procedure.     

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.     

Norwood procedure to hypoplastic left heart syndrome]

Since March 1992, 25 neonates and small infants with HLHS have undergone a modified Norwood procedure. The mean age and weight at operation were 17 days (2 days-2 months) and 2.7 kg (1.6-3.3 kg). Isolated cerebral and/or myocardial perfusion (ICMP) with direct anastomosis of aorta and pulmonary artery was utilized since January 1995 to 16 patients. Under median sternotomy, PTFE graft (usually 3.0-3.5 mm) was anastomosed to the brachiocephalic artery and the arterial cannula was inserted to this PTFE graft. The left carotid and the left subclavian arteries were snared and a clamp was placed on the aortic arch just distal to the brachiocephalic artery. This allowed blood to enter the brain and the coronary arteries, keeping the brain perfused and the heart-beating. After reconstruction of distal aortic arch, a single dose of crystalloid cardioplesia was infused and the rest of the arch was reconstructed. There were 14 early deaths (56%) and 4 late deaths (16%). Bidirectional Glenn procedure was performed to 5 patients with 1 death. Three patients underwent modified Fontan procedure without mortality. Mean aortic cross clamp time was 24 min. and mean ICMP time was 32 min. There was no neurologic complications. In conclusion, isolated cerebral and/or myocardial perfusion may offer an advantage of protecting the brain and myocardium during arch in Norwood procedure.     

Fontan procedure for hypoplastic left heart syndrome.

Since 1985, 354 neonates have undergone palliative reconstruction for hypoplastic left heart syndrome with 109 early deaths and 12 late deaths. Of the survivors, before 1989, 77 patients underwent a subsequent modified Fontan operation, consisting of baffling the atrial septal defect to the tricuspid valve (initial 25 patients) or intraatrial baffling of the inferior vena cava to the pulmonary arteries and superior vena cava (52 patients). There were 17 early deaths and three late deaths. Major serous effusions developed in 42 patients (54%) after Fontan operation. Since 1989, a staged approach to Fontan's operation was undertaken in an effort to reduce the volume load of the right ventricle as early as possible, to minimize the impact of rapid changes in ventricular geometry and diastolic function that can accompany a primary Fontan operation, and to reduce effusive complications. Thus, at a mean age of 6 months, 121 patients have undergone closure of aortopulmonary shunt, augmentation of central pulmonary arteries, and association of the superior vena cava with the branch pulmonary arteries (hemi-Fontan procedure). Of these, 61 patients have already undergone completion of the Fontan procedure with six early deaths and three late deaths. Major serous effusions developed in 28 patients (46%) with the staged Fontan. For perspective, the contemporary experience since January 1991 consists of 58 neonates who have undergone initial palliation with 11 deaths (19%), 17 patients who have undergone the hemi-Fontan procedure with one death (6%), and 21 patients who have undergone completion of the Fontan operation with one death (5%).(ABSTRACT TRUNCATED AT 250 WORDS)