Three-stage Palliation of Hypoplastic Left Heart Syndrome in the University HealthSystem Consortium

Objective. The objective of this study was to describe current management and short-term outcomes for patients with hypoplastic left heart syndrome representative of nationwide experience. Additionally, this study identifies risk factors associated with mortality in patients undergoing staged surgical palliation. Design. The University HealthSystem Consortium database was queried to identify all hospital admissions between 1998 and 2007 with a diagnosis of hypoplastic left heart syndrome. Procedure codes were used to determine surgical management, and outcomes were ascertained by discharge status (discharged, transferred, or expired). Results. Discharge data were present from 118 hospitals in the United States. First-stage surgical palliation was performed in 1949 neonates with 30% mortality. Mortality decreased from 43% in 1998 to 18% in 2007. Large institutional case volume and later era of surgery were associated with improved operative mortality in first-stage palliation. Primary cardiac transplantation was performed in 28 neonates, and rescue transplantation in 11, with 36% mortality. Second-stage palliation was performed in 1244 patients with 5.2% mortality, and third-stage palliation was performed in 1084 patients with 4.1% mortality. An additional 62 patients over 1 month of age at time of admission received cardiac transplantation with 15% mortality. Conclusions. First-stage palliative mortality for hypoplastic left heart syndrome fell dramatically over the past decade, while that for second- and third-stage procedures remained stable. The cumulative operative mortality for three-staged repair of hypoplastic left heart syndrome was 39% over the decade, but fell to 24% for procedures in 2007.     

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.     

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)     

Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery.

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.     

Fetal interventions for structural heart disease

Fetal cardiac intervention (FCI) offers the potential to alter in utero anatomy and physiology. For aortic stenosis with evolving hypoplastic left heart syndrome and pulmonary atresia with intact ventricular septum with evolving hypoplastic right heart syndrome, FCI may result in maintenance of a biventricular circulation, thus avoiding single‐ventricle palliation and its attendant complications. In the case of hypoplastic left heart syndrome with intact atrial septum, FCI may ameliorate in utero pathophysiology and portend a more favorable postnatal prognosis. In all cases, a detailed fetal echocardiographic assessment to identify the appropriate FCI candidate is essential. This article reviews the three aforementioned lesions for which FCI can be considered. The pathophysiology and rationale for intervention, echocardiographic assessment, patient selection criteria, and outcomes for each lesion will be reviewed. A primary focus will be the echocardiographic evaluation of each lesion.     

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.     

The management of hypoplastic left heart syndrome with a right aortic arch

The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs. We present here a patient with this combination who survived an initial Damus-Kay-Stansel procedure combined with placement of a conduit from the right ventricle to the pulmonary arteries, and who has subsequently undergone a successful bidirectional cavopulmonary anastomosis. We believe that the conduit placed from the right ventricle provides the most reliable source of flow of blood to the lungs at the time of initial palliation in this usual combination of cardiac lesions.     

A case of PAGOD syndrome with hypoplastic left heart syndrome

PAGOD syndrome is an extremely rare congenital malformation complex involving multiple organs. These include pulmonary artery and lung hypoplasia, diaphragm defects, omphalocele, sex reversal or ambiguous genitalia, and complex cardiac defects. Eight cases have been reported to date. We report a case of PAGOD syndrome that is manifested by right diaphragm eventration, horseshoe lung with right lung hypoplasia, hypoplastic left heart syndrome (mitral atresia, aortic atresia), scimitar syndrome, agonadism with ambiguous genitalia. A karyotype revealed normal 46-XY. This patient received a modified Norwood procedure for hypoplastic left heart syndrome as an initial palliation and bidirectional cavopulmonary anastomosis as a second stage of operation. The postoperative courses were uneventful. This patient is waiting for Fontan operation.     

Initial transcatheter palliation of hypoplastic left heart syndrome.

Initial percutaneous transcatheter palliation of hypoplastic left heart syndrome is now feasible. The primary procedures for palliation include stenting of the ductus arteriosus with a self expanding nitinol stent to secure an adequate systemic blood flow, placement of an internal pulmonary arterial band to protect the pulmonary vascular bed and to prevent pulmonary overcirculation, and widening of the interatrial communication by blade and balloon septostomy or static balloon dilation to decompress the left atrium. Anatomic variations of the ductus arteriosus have important implications for technical success with ductal stenting. Patients who have undergone complete transcatheter palliation with the internal pulmonary band appear to have less immediate morbidity at the time of transplant, with preserved integrity and growth of the branch pulmonary arteries at one year follow-up.     

Impact of placing a conduit from the right ventricle to the pulmonary arteries as the first stage of further palliation in the Norwood sequence for hypoplasia of the left heart

OBJECTIVE: We describe the experience from a single institution with the Norwood sequence of palliation for hypoplasia of the left heart, emphasizing complications related to placement of a conduit from the right ventricle to the pulmonary arteries and their management. METHODS: Between November, 2002 and January, 2006, we palliated 32 patients with hypoplastic left heart syndrome or its variants by placing a conduit from the right ventricle to the pulmonary arteries. We reviewed retrospectively the charts and angiograms from these patients. RESULTS: Hospital survival after construction of the conduit was 90.6%. There were 3 interstage deaths, of which 2 were likely due severe obstruction of the conduit. Stents were implanted into the proximal or medial portions of the conduits of 3 patients. Early revision of the distal anastomosis, and shortening the conduit, was performed early postoperatively in 2 patients. So far, 24 out 26 survivors of the first stage underwent a bi-directional cavopulmonary anastomosis after a mean interval of 4.3 plus or minus 1.4 months. Of these, 3 required a semi-urgent second stage of palliation because of worsening cyanosis, with one patient dying after the second stage. Completion of the Fontan circulation by insertion of an extracardiac conduit was performed in 8 patients at the mean age of 19.8 plus or minus 2.2 months. We were able to achieve biventricular repair in 1 patient, with aortic atresia, hypoplastic arch and ventricular septal defect, 4.3 months after the initial palliative procedure. Overall survival of the whole cohort of 32 patients was 78.9%, plus or minus 7.8%, at 5 months, and 74.3%, plus or minus 8.6%, up to 25 months. CONCLUSIONS: The introduction of the conduit placed from the right ventricle to the pulmonary arteries has led to an improved outcome in the complex entity of hypoplastic left heart syndrome and its variants. Stenosis of the conduit, nonetheless, may account for significant interstage morbidity, and often requires intervention or early installation of the second stage of palliation.     

Catheter Intervention for Congenital Heart Disease at Risk of Circulatory Failure

Percutaneous interventions complement other therapeutic modalities in the management of patients with heart failure secondary to congenital heart disease. The systemic left ventricle can fail when submitted to excessive afterload as seen with critical aortic stenosis or coarctation. Balloon dilation of the aortic valve is safe and effective in establishing adequate forward flow and improving myocardial function in neonates. A large left-to-right shunt caused by a ventricular septal defect or a patent ductus arteriosus can lead to left ventricular failure. In selected infants, device closure of these defects is a therapeutic option. The postoperative right ventricle burdened by chronic residual obstruction and volume overload is at risk of failure. Balloon dilation of the pulmonary valve, pulmonary artery angioplasty with or without stent implantation, and pulmonary conduit stenting are effective in minimizing afterload to the right ventricle. More recently, percutaneously implantable pulmonary valves have become an option, in selected patients, for full rehabilitation of dysfunctional pulmonary conduits. Patients with single ventricle physiology have a fragile physiology, justifying an aggressive catheter interventional approach to optimize hemodynamic characteristics. The hybrid approach to palliation of hypoplastic left heart syndrome has become a viable option in selected centres.     

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.     

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.     

Coarctation and related aortic arch anomalies in hypoplastic left heart syndrome

The aortic arches of 34 specimens with hypoplastic left heart syndrome were studied in order to establish the frequency, the nature and the clinical implications of aortic arch anomalies. A localized aortic coarctation was present in 23 specimens. The coarcation was located preductally in 2 and paraductally in 21 cases. The degree of obstruction caused by the coarctation varied considerably. In only 6 cases (1 preductal and 5 paraductal) was the obstruction judged to be severe. One of these cases had an additional aneurysm of the aortic wall proximal to the coarctation. An aberrant relation of the ductus arteriosus and the aortic arch was found in 2 specimens without localized coarctation. In the remaining 9 specimens the aortic arch appeared normal. The aortic arch anomalies were mostly present in specimens with a severely hypoplastic ascending aorta. Clinicians, when preparing infants with hypoplastic left heart syndrome for surgical palliation, should always suspect associated aortic arch anomalies, especially when there is severe hypoplasia of the ascending aorta. Coarctation of the aorta will require additional surgical treatment.     

Truncal or aortic valve stenosis in functionally single arterial trunk: A clinical,hemodynamic and pathologic study of six cases

Stenosis of the semilunar valve in the presence of a functionally single arterial trunk is uncommon. Three patients with truncus arteriosus, two with tetralogy of Fallot and pulmonary atresia and one with pulmonary atresia and intact septum were diagnosed as having stenosis of the truncal or aortic valve on the basis of clinical, echocardiographic, hemodynamic and angiocardiographic findings. Echocardiograms consistently showed multiple diastolic closure lines and abnormal semilunar valves in addition to the aortic override in five patients and hypoplastic right ventricle in the patient with pulmonary atresia and intact septum. Peak systolic gradients between the left ventricle and truncus (or aorta) at cardiac catheterization ranged from 20 to 47 mm Hg. Retrograde aortography confirmed a domed and stenotic semilunar valve. Cyanosis was progressive in the three patients with pulmonary atresia. Two patients had arterial anastomosis, and one had a right ventricle-pulmonary arterial graft in addition to aortic valvotomy. One of the three patients with truncus arteriosus underwent complete repair in addition to truncal valvotomy but he died in the postoperative period. The other two patients with truncus arteriosus died of intractable congestive cardiac failure before surgical intervention.It is suggested that the presence of semilunar valve stenosis in these patients adversely affects the prognosis. The myocardium is already jeopardized as a result of hypoxia in pulmonary atresia and left ventricular diastolic overload in patients with truncus arteriosus. The added burden of semilunar valve stenosis may further compromise the functional status of the myocardium.     

Criterions for selection of patients for, and results of, a new technique for construction of the modified Blalock-Taussig shunt

BACKGROUND: We describe alternative surgical techniques for construction of systemic-to-pulmonary arterial shunts, and propose criterions for their application in selected patients. PATIENTS AND METHODS: We constructed a variety of modified systemic-to-pulmonary arterial shunts, using polytetrafluoroethylene grafts, in 92 selected patients with cyanotic congenital heart disease and anomalies of the aortic arch and systemic veins. Their age ranged from 7 days to 3.6 years, with a mean of 7.08 months. We performed 88 operations through a thoracotomy. Of this cohort, 60 patients underwent a second-stage operation, with 15 receiving a superior cavopulmonary connection, 16 a total cavopulmonary connection, and 29 proceeding to biventricular repair after a mean interval of 15.6 months. We have 21 patients awaiting their second or final stage of palliation. RESULTS: There were five early (5.4%) and six late deaths (6.8%), two of which were related to construction of the shunts. At a mean follow-up of 45.29 months, the increase in diameter of pulmonary trunk and its right and left branches was uniform and significant (p value less than 0.001). Pulmonary arterial distortion requiring correction at the time of second-stage operation was observed in 5 patients (6.1%). Adequate overall palliation was achieved in 98% of the cohort at 8 months, 91% at 12 months, and 58% at 18 months. CONCLUSIONS: Patients with a right- or left-sided aortic arch and right-sided descending thoracic aorta, those with anomalies of systemic venous drainage masking the origin of great arterial branches, and those with disproportionately small subclavian arteries, constitute the ideal candidates for our suggested modification of the construction of a modified Blalock-Taussig shunt. The palliation provided by these shunts was satisfactory, with predictable growth of pulmonary arteries, insignificant distortion in the great majority, and easy take-down.     

Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome.

OBJECTIVE--To assess the feasibility of pulmonary artery banding, atrial septectomy or septostomy, and percutaneous stenting of the arterial duct in babies with the hypoplastic left heart syndrome. PATIENTS--Four infants with hypoplastic left heart syndrome. SETTING--Two supraregional paediatric cardiac centres. METHODS--Ductal patency was maintained initially with prostaglandin E. Banding of the proximal branch pulmonary arteries was performed through a median sternotomy and open atrial septectomy was performed if balloon septostomy was not. Stainless steel stents (Johnson & Johnson) mounted in a balloon catheter were implanted into the arterial duct under radiographic control and expanded to a diameter of approximately 8 mm, prostaglandin treatment was then stopped. RESULTS--All the patients survived the immediate postoperative period and maintenance of wide ductal patency was achieved in three of the four patients by stent implantation. Two weeks after the procedure two babies died of right ventricular failure and respiratory infection: some distal ductal constriction had occurred in one where the stent was not quite sufficiently distally placed. One child was discharged home 15 days after treatment and was well at follow up at age 16 weeks and one was stable but required diuretic therapy five weeks after the procedure. CONCLUSIONS--This new approach is technically feasible. At least in the short term it seems to offer hope of effective palliation for the hypoplastic left heart syndrome and it warrants further study.     

Neonatal cardiac transplantation for hypoplasia of the left heart diagnosed in utero]

The poor results of palliative surgery in the hypoplastic left heart syndrome has led Bailey to propose neonatal cardiac transplantation for this condition. The authors report the case of a neonate who underwent transplantation 2 days after birth for hypoplastic left heart with mitro-aortic atresia, diagnosed in utero. The child was prescribed triple immunosuppressive therapy (steroids, azathioprine, ciclosporine) and is now 24 months old. An episode of acute rejection during the first month was treated with a short intensive course of intravenous steroids. At the fourth month, the child underwent aortic angioplasty for an isthmic stenosis. Growth is retarded (- 2SD) and he has mild renal failure but psychomotor development has been normal. The number of neonatal cardiac transplantations remains limited by parental refusal and the lack of donor organs. Despite encouraging medium term results, questions remain as to the long-term viability of the graft and the patient's renal function.     

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.