Management of hypoplastic left heart syndrome in the 1990s

We used the discharge database of the University Hospital Consortium to determine the management and early outcome of neonates with hypoplastic left heart syndrome admitted to member institutions from 1990 to 1999. Of the 2,264 patients, 1,203 underwent a Norwood procedure, with 42% mortality. Cardiac transplantation was performed in 72, with 38% mortality, and 217 (10%) were discharged without any surgical procedure. The proportion of patients managed by the Norwood procedure increased from 43% during the first half of the decade to 59% in the second half, with corresponding decreases in the proportion managed by transplantation or nonintervention. A mortality rate of < or =40% was achieved in all 5 institutions performing >50 Norwood procedures, and by 9 of 40 institutions performing <50. Performance of a Norwood procedure has become the most frequent management for neonates with hypoplastic left heart syndrome. Lower operative mortality rates are generally, but not exclusively, achieved by institutions with high surgical volume.     

Hybrid palliation in hypoplastic left heart syndrome

PURPOSE OF REVIEW: Despite progressive improvement in surgical results, hypoplastic left heart syndrome remains one of the congenital heart abnormalities with the greatest morbidity and mortality. Hybrid approaches to management, combining surgical and interventional catheterization procedures, have been introduced to minimize exposure to cardiopulmonary bypass, and improve outcomes for these high-risk infants. RECENT FINDINGS: First-stage palliation of hypoplastic left heart syndrome has been performed as a hybrid procedure combining surgical pulmonary artery banding with catheterization stenting of the ductus arteriosus and balloon atrial septostomy, especially in high-risk patients. Additionally, several centers have performed second-stage palliation - bidirectional Glenn or hemi-Fontan procedures - in a manner that allows the subsequent 'Fontan' procedure to be completed in the catheterization laboratory with a covered stent. SUMMARY: These innovative procedures offer the potential of an alternative management strategy for hypoplastic left heart syndrome. They have been applied to a very limited number of patients and long-term results are not available. Their role in management of hypoplastic left heart syndrome remains to be defined, especially as results of conventional surgical management continue to improve.     

Hypoplastic left heart syndrome: is echocardiography accurate enough to guide surgical palliation?

Two-dimensional echocardiography can diagnose hypoplastic left heart syndrome. However, with the advent of the possibility of palliative open heart surgery, complete anatomic diagnosis is necessary. The anatomic findings of 15 neonates with hypoplastic left heart syndrome (age 1 to 10 days, mean 4.1) who had two-dimensional Doppler echocardiographic studies were compared with the results obtained by angiography (6 cases), surgery (11 cases) and autopsy (8 cases). Complete two-dimensional echocardiographic examination of the aortic arch, pulmonary and systemic venous return, atrial septum, ductus arteriosus and proximal coronary arteries was possible in all 15 neonates and correctly diagnosed hypoplastic left heart syndrome in each. Anatomic two-dimensional echocardiographic assessment was accurate in 13 (86%) of the 15 neonates and there were no false positive results. Undiagnosed associated abnormalities were hypoplasia of a left pulmonary artery in one patient and left superior vena cava in another. Accurate quantitation of the size of the tricuspid valve anulus, ascending aorta, pulmonary anulus and right and left pulmonary arteries was possible. Doppler examination was performed in seven patients and confirmed retrograde aortic arch flow and right to left systolic shunting in the patent ductus arteriosus. In selected neonates, surgical palliation can be attempted without angiography.     

Resuscitation and Perioperative Management of the High‐risk Single Ventricle Patient: First‐stage Palliation

Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. Nonetheless, these infants remain at a high risk of morbidity and mortality, and an appreciation of single ventricle physiology is fundamental to the care of these high‐risk patients. Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first‐stage clinical management strategies are reviewed.     

Neonatal critical valvar aortic stenosis. A comparison of surgical and balloon dilation therapy

Balloon aortic valvotomy (BAV) is an alternative to surgical valvotomy in infants and children. We compared BAV in 16 consecutive neonates (1985-1988) to surgical valvotomy in a prior group of 16 consecutive neonates (1978-1984). Both groups were comparable in terms of age, weight, hemodynamic data, left ventricular size, and associated lesions. There were six early and one late deaths after surgery. Five out of six neonates requiring a second operation died. Left ventricular size (measured in 13 neonates) had some influence on survival after surgery: three of three with small or hypoplastic left ventricles and three of 10 with normal-sized left ventricles died. After BAV, there were three early deaths, two patients who underwent stage I palliation of hypoplastic left heart syndrome, and two late deaths. As with surgical valvotomy, left ventricular size seemed to influence survival after BAV: five of six with small or hypoplastic left ventricles died or underwent stage I palliation for hypoplastic left heart syndrome and two of nine with normal-sized left ventricles died. At follow-up (26 +/- 17 months) in six patients in the surgical group, the peak systolic ejection gradient (PSEG) was 52.2 +/- 23 mm Hg and left ventricular end-diastolic pressure (LVEDP) 18.2 +/- 5.2 mm Hg. Aortic regurgitation was mild in five and moderate in the sixth patient. At follow-up (17.6 +/- 7.8 months) in nine patients in the balloon dilation group, the PSEG was 45.6 +/- 11 mm Hg in five patients at catheterization and 43.8 +/- 22.9 mm Hg in four patients by echocardiography-Doppler. Aortic regurgitation was mild in three and absent in the other six patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome

OBJECTIVE: Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. METHODS: We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. RESULTS: The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. CONCLUSION: Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.     

Fetal and Neonatal Imaging and Strategy of Primary Neonatal Heart Transplantation in Hypoplastic Left Heart with Ebstein's Anomaly

We present the anatomic constellation of mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome, Ebstein's anomaly, and partial anomalous pulmonary venous return, an exceeding rare congenital heart defect. Prenatal echocardiography led to concern about the capacity of the right ventricle to increase cardiac output with lung expansion and pulmonary arterial runoff at birth, prompting the precaution of extracorporeal membrane oxygenator standby at delivery. Stage I palliation was not attempted, and control of pulmonary arterial blood flow was achieved with pulmonary artery banding, allowing sufficient ongoing hemodynamic stability. Orthotopic cardiac transplantation, repair of hypoplastic aortic arch, and primary sutureless repair of left pulmonary veins was performed, using dual‐site arterial cannulation and continuous mild hypothermic cardiopulmonary bypass. We discuss how this unique echocardiographic anatomy influenced the surgical decision and point out how it guided therapy toward a strategy of primary transplantation rather than standard staged surgical palliation.     

Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome

Objective. Multiple surgical approaches to the initial palliation of patients with hypoplastic left heart syndrome (HLHS) have been advocated throughout the years. We sought to examine what procedure, if any, is recommended for HLHS management in regard to physician preference, anatomical variations, and concomitant medical issues. Patients and Methods. A Web‐based survey of pediatric cardiologist subscribed to PediHeart was conducted. Outcome Measures. The types of palliation recommended (Norwood palliation, Sano modification, hybrid palliation, primary cardiac transplantation, or hospice care) for patients with HLHS with anatomic or comorbid variants were queried. Counseling provided by the physicians to families was also documented as regards survival and outcomes. Results. Two hundred physicians (21% female) who averaged 12.3 years removed from training responded to the survey. US East Coast and Midwest respondents were more likely to recommend Norwood palliation (54% and 60%, respectively) and the US South and West respondents preferred Sano modification (73% and 82%, respectively). Norwood or Sano palliation was recommended over hospice care, hybrid palliation, or cardiac transplant for patients with an intact atrial septum (P < 0.05), moderate to severe tricuspid regurgitation (P < 0.05), or low birth weight defined as less than 2 kg (P < 0.05). Hospice was preferred in low‐birth‐weight infants over hybrid palliation or cardiac transplantation (P < 0.05). Hospice was recommended over any other palliation for premature infants (less than 30 weeks gestation), chromosomal abnormalities, or end‐organ dysfunction (P < 0.05). Conclusions. This survey demonstrates that different palliative options are primarily recommended by caretakers based upon institutional location and patient characteristics. Prospective comparative trials may force a rethinking of this approach over time.     

Palliation via Hybrid Procedure of a 1.4‐kg Patient with a Hypoplastic Left Heart

Despite improvements in survival of patients with hypoplastic left heart syndrome (HLHS) with various palliative procedures, certain risk factors, such as weight less than 2.5 kg, continue to predict increased mortality. We report the palliation of a patient with HLHS weighing 1.4 kg via a hybrid procedure consisting of banding of the pulmonary arteries bilaterally, stenting the ductus arteriosus, and balloon atrial septostomy. We propose that this may be another alternative for palliation in this high‐risk patient group.     

Freedom from Neoaortic Insufficiency: A Comparison of Classic Norwood and Norwood–Sano Procedures

Objectives. To investigate the incidence of neoaortic insufficiency in patients with hypoplastic left heart syndrome treated with the Norwood–Sano palliation and to compare it with that occurring after the classic Norwood procedure. Design, Setting, Patients, Interventions. This was a retrospective review of all echocardiograms of patients diagnosed with hypoplastic left heart syndrome (concomitant presence of left ventricular and aortic and mitral severe hypoplasia or atresia) who underwent staged palliation of the Norwood or Norwood–Sano type at a single academic institution between September 1999 and February 2005 and who survived a minimum of 3 months. Outcome Measures. Neoaortic insufficiency was categorized as absent or mild <1 mm jet width, moderate 1–3 mm jet width, or severe >3 mm jet width. The patients were grouped according to initial palliation, that is, classic Norwood and Norwood–Sano operation. Results. Fifty‐nine consecutive patients (median age of 20 months with a range from 3 to 66 months) satisfied inclusion criteria. Neoaortic insufficiency was absent or mild in 55 of 59 (93.22%) of the patients. There were 4 cases of significant neoaortic insufficiency at late follow‐up: 2 moderate following the classic Norwood and 1 moderate and 1 severe following the Norwood–Sano procedure, one of whom required valve replacement. Conclusions. In this series of patients with hypoplastic left heart syndrome, the Sano modification was not associated with an increased incidence of significant neoaortic insufficiency. When present, moderate/severe neoaortic insufficiency appeared late after initial palliation and was associated with recurrent ascending aortic or aortic arch pathology in every case.     

Achieving a balance in the current approach to the surgical treatment of hypoplastic left heart syndrome

In the modern era, it is possible to achieve programmatic balance in the approach to the treatment of hypoplastic left heart syndrome by offering staged palliation, transplantation, and in suitable cases, biventricular repair. Strategies for optimal selection should continue to improve and evolve. Overall, the current hope for survival, and excellent quality of life, for patients with hypoplastic left heart syndrome and other forms of functionally univentricular heart is better than ever before. Pioneering efforts allowed what we achieve today. Our challenge is to continue to improve the surgical and medical care of these patients towards normalization of survival and quality of life for future generations. These goals will be achieved by capitalizing on current advances in the field, and continuing to "push the envelope", with research into new advances.     

Pediatric Cardiac Care Consortium: An Instrument for Evidence-Based Clinical Decision Support

Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease.     

Variation in Preoperative and Intraoperative Care for First‐stage Palliation of Single‐ventricle Heart Disease: A Report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative

Background and Methods. As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first‐stage palliation of single‐ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. Results. From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9–5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first‐stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. Conclusions. In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single‐ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long‐term outcomes.     

Heart surgery under cardiopulmonary bypass during the neonatal period remains a high-risk procedure

Congenital Heart disease with a poor prognosis has to be operated early but with an acceptable surgical risk and a good chance of survival. The aim of this study was to analyse the indications, the operative mortality and medium-term survival of neonates undergoing cardiac surgery under cardiopulmonary bypass from 1991 to 1998. Three hundred and twenty nine operations were programmed in 326 neonates, 18% (329/1805) of all open heart surgical procedures. Anatomical detransposition of the great arteries was the commonest operation (N = 226). Correction was complete (biventricular) in 97% of cases (317/326). Twenty-eight neonates died. The operative mortality was 8.5%, much higher than that of children of 3 months or over (1.5%; p < 0.0001). There were 8 late deaths, all in the first postoperative year. The overall medium-term survival rate was 88.9 +/- 1.7%. It was 100% after correction of truncus arteriosus without interruption of the aortic arch; 94.2 +/- 1.5% after anatomical detransposition; 85.7 +/- 9.4% after commissurotomy of aortic stenosis; 79.2 +/- 8.3% for all the complex forms of interruption of the aortic arch; 75 +/- 9.7% for total anomalous pulmonary venous drainage and 42.9 +/- 18.7% after the Norwood palliative procedure of hypoplastic left heart syndrome. The authors conclude that early cardiac surgery saves the large majority of neonates suffering from complex congenital cardiac disease with poor prognosis. Survival stabilises one year after the operation. Other techniques or treatments are necessary to lower present surgical risk (8.5%) to that of surgery under cardiopulmonary bypass of children over 3 months of age (1.5%).     

Fetal hydrops in a newborn with hypoplastic left heart syndrome: tricuspid valve "stopper"

Fetal hydrops in a newborn infant with hypoplastic left heart syndrome led to the discovery of tricuspid stenosis and insufficiency from an unusual malformation of the right venous valve of the embryonic sinus venosus. This unfortunate combination of lesions precluded surgical palliation for the hypoplastic left heart.     

Recurrent Protein‐losing Enteropathy and Tricuspid Valve Insufficiency in a Transplanted Heart: A Causal Relationship?

This case report describes a toddler who developed a protein‐losing enteropathy (PLE) 4 years after orthotopic heart transplantation (OHT). He was born with a hypoplastic left heart syndrome for which he underwent a successful Norwood procedure, a Hemi–Fontan palliation, and a Fontan palliation at 18 months of age. Fifteen months following the Fontan operation, he developed a PLE and Fontan failure requiring OHT. Four years after OHT, he developed a severe tricuspid regurgitation and a PLE. His PLE improved after tricuspid valve replacement. It is now 2 years since his tricuspid valve replacement and he remains clinically free of ascites and peripheral edema with a normal serum albumin level. His prosthetic tricuspid valve is functioning normally.     

老年心脏瓣膜病变的外科治疗体会

目的 总结老年心脏瓣膜病变的外科治疗经验.方法 回顾性分析160例老年心脏瓣膜病变外科治疗患者的临床资料.行二尖瓣置换（MVR）65例,主动脉瓣置换（AVR）45例,双瓣置换50例;同期行三尖瓣成形术108例,冠状动脉搭桥术19例,左房折叠术4例.结果 手术早期死亡6例,死亡率为3.9%.随访140例,随访时间6个月至8年,死亡2例,其余138例术后随诊复查人工瓣膜功能良好,左心室射血分数（LVEF）提高,心功能均提高1～2级,无瓣周漏、人工瓣膜心内膜炎及心脏血栓形成,没有因瓣膜原因而再次手术者.结论 术前充分调整心功能,选择适当的手术时机,加强术中心肌保护,不断改进和提高手术技巧,加强围术期管理,是提高老年心脏瓣膜病变患者手术成功率的关键因素.     

Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect

A severely restrictive atrial septal defect (ASD) in neonates with hypoplastic left heart syndrome (HLHS) results in pulmonary venous hypertension, pulmonary edema, and intractable hypoxia. Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheterization at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography. Three underwent blade septostomy with 2 procedure-related deaths, and 1 had balloon atrial septostomy (BAS); all 4 died before surgical intervention. Fifteen underwent Brockenbrough atrial septoplasty with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a stent placed across the atrial septum after transatrial needle puncture. In the 16 patients treated with septoplasty or stent, oxygen saturation increased from 50 +/- 4% to 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting transplantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation. Eight of 14 (57%) survived to hospital discharge. Six of 7 (86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in profound cyanosis demands urgent intervention. BAS is frequently unsuccessful and blade septostomy has high mortality. Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, allowing stabilization before reconstructive surgery or while awaiting transplant.     

Morphologic determinants of coronary blood flow in the hypoplastic left heart syndrome

The diameters of the ascending aorta, coronary ostia, and proximal coronary arteries were measured in autopsy specimens from 51 infants with hypoplastic left heart syndrome and 18 normal infant heart specimens. Standard cross sections were prepared from 35 of the hypoplastic left heart syndrome hearts, and histologic indices of myocardial viability and fibrosis were determined. The diameters of the coronary arteries and ostia in hypoplastic left heart syndrome were not different from the values in control specimens. Little fibrosis was found but extensive myocardial necrosis was present. The degree of fibrosis and necrosis did not correlate significantly with any of the arterial or ostial diameters. The extent of myocardial preservation in the hypoplastic left heart syndrome, which may substantially limit the success of surgical palliation, cannot be predicted from these morphologic measurements. The observed myocardial damage appeared to be acquired postnatally, implying that early vigorous treatment of these infants may be necessary to preserve myocardial function.     

Biventricular repair in neonates with hypoplasia of the left ventricle]

BACKGROUND: Whether to perform uni or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal left ventricle and multi level left ventricle obstructions (hypoplastic left heart syndrome class III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. HYPOTHESIS: Increased afterload and multi level left ventricle obstruction is constant. We assumed that restoration of normal loading conditions by relief of left ventricle obstructions promotes its growth, provided that part of the cardiac output was pre operatively supported by the left ventricle, whatever the echocardiographic indices. METHODS: Twenty one ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multi level left ventricle obstruction. Pre operative echocardiographic assessment showed: mean end diastolic left ventricular volume of 13.3 +/- 3.5 mL/m2 and mean Rhodes score of -1.43 +/- 0.9. Surgery consisted in relief of left ventricle outflow tract obstruction by coarctation repair in 21 associated to atrial septal defect closure in 2, aortic commissurotomy in 1 and ascending aorta enlargement in 1. RESULTS: There were 3 early and 3 late deaths. There was no predictive risk factor for failure. Growth of the left heart was demonstrated in most patients. At hospital discharge the end diastolic left ventricular volume was 19.4 +/- 3.12 mL/m2 (p = 0.0001) and the Rhodes score was -0.38 +/- 1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were: 68.5% and 40.75%, respectively. CONCLUSION: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal left ventricle provided that all anatomical causes of left ventricle obstruction can be relieved. Secondary growth of the left heart then occurs, however the reoperation rate is not low.