First-stage Palliation Research Articles

The Norwood procedure for hypoplastic left heart syndrome: results of the King Chulalongkorn Memorial Hospital

Background: The Norwood procedure is often performed to treat hypoplastic left heart syndrome (HLHS). Only Blalock-Taussig (BT) shunt is used with the Norwood procedure in our institute. Objective: To analyze the first-stage palliation for HLHS. Patients and methods: The Norwood procedure with right modified BT shunt was performed in 26 patients with HLHS between August 1996 and November 2008. The first four patients were performed using only autologous great vessel tissue (group 1), and the other 22 patients were operated with the Norwood procedure using a homograft for arch reconstruction (group 2). Results: The hospital mortality was 50.0 % in group 1 and 18.2% in group 2. The overall hospital mortality was 23.1%. Four out of twenty survivors (20%) had the modified Fontan procedure. Conclusion: Only a limited number of pediatric cardiac centers offered surgical treatment of the hypoplastic left heart syndrome. The survival rate in our study was in acceptable range despite the limited resource.

1067 MR assessment of long term pulmonary arterial morphology following surgical ligation of systemic to pulmonary arterial shunts: visualising a potential risk

Introduction Formation of a Blalock-Taussig (BT) shunt to establish a secure source of pulmonary blood flow is the first stage of palliation for a significant proportion of children born with congenital heart disease. With further palliative or corrective surgery, ligation rather than surgical division of a previously-formed BT shunt has been the preferred surgical strategy. We hypothesise that ligation rather than division of a shunt carries a risk of iatrogenic vascular constraint with respect to the pulmonary arteries, producing distortion with somatic growth.

Evolving Strategies and Improving Outcomes of Norwood Stage One Reconstruction: A Decade Experience of a Single Institution in Taiwan

The aim of this study was to define the improvement in short-term outcome and risk factors of Norwood stage one reconstruction for hypoplastic left heart syndrome (HLHS) in Taiwan, after implementing new perioperative management strategies. Data were retrieved from a retrospective chart review of patients with HLHS treated between July 1997 and July 2007. Since we implemented new perioperative strategies in 2004, we divided our patients into two groups, early era (1997-2003) and late era (2004-2007), and compared the outcome. We enrolled 48 patients. In the early era group (n=28), the diagnosis was confirmed by cardiac catheterization and controlled ventilation was used to manipulate the balance between systemic and pulmonary blood flow. The survival rate was only 17.9% (5/28). Surgery was performed at 15.2+/-10.7 days, which was significantly later than in the late era group (4.6+/-4.0 days, n=20). A lower preoperative shock and more prenatal diagnoses were recorded for the late era group. RV-PA conduit was used in 17 patients in the late era group of which 12 (70.6%) survived to be discharged from hospital. The risk factor was significant TR (tricuspid regurgitation). With our contemporary perioperative management and change in surgical strategy, survival after first-stage palliation has improved. We believe that our HLHS experience is valuable for low volume centers and also for Asian cohorts.

The Palliative Arterial Switch Procedure for Single Ventricles: Are These Patients Suitable Fontan Candidates?

The traditional first stage of palliation for functional single-ventricle lesions with transposition of the great vessels and systemic outflow tract obstruction has been either the Norwood or Sano procedure or the Damus-Kaye-Stansel procedure. There is limited literature on the use of an arterial switch procedure as the initial staged palliation for functional single ventricles in this setting. This study is an examination of our institutional experience with performing the palliative switch procedure with examination of suitability for Fontan completion and midterm outcome in these patients. This is a retrospective review of our institutional experience from October 1991 through August 2006 on single-ventricle patients with transposition of the great vessels and systemic outflow tract obstruction who underwent a palliative arterial switch procedure. Nine patients underwent an initial palliative switch procedure. Six of 9 patients underwent completion of palliation with the Fontan procedure and are alive and well. One patient is well and is awaiting the next stage of palliation (78%). There was 1 early operative death (11%) and 1 late death (11%). There was 1 case of recoarctation (11%). The palliative switch procedure appears a reasonable surgical option for patients with functional single-ventricle lesions, transposition of the great vessels, and systemic outflow tract obstruction. Patients who have undergone a palliative switch procedure are suitable candidates for completion of palliation with the Fontan procedure.

Right atrial isomerism with infracardiac total anomalous pulmonary venous connection complicated by hiatal hernia

A 31-day-old girl with right atrial isomerism, a single atrium, a single right ventricle, pulmonary atresia, patent ductus arteriosus, coarctation of the pulmonary artery, infracardiac total anomalous pulmonary venous connection, and a large hiatal hernia was referred to our institution. We successfully performed a first-stage open palliation comprising the creation of a systemic-to-pulmonary arterial shunt, pulmonary artery angioplasty, and correction of the total anomalous pulmonary venous connection. During cardiopulmonary bypass, the hiatal hernia was also repaired through the median sternotomy itself without another laparotomy. The postoperative course was uneventful, and we avoided the potential complications of pulmonary venous obstruction and enteral feeding. She underwent a bidirectional Glenn operation and Fontan completion at 5 and 19 months of age, respectively.

Stent Dilatation of a Right Ventricle to Pulmonary Artery Conduit in a Postoperative Patient with Hypoplastic Left Heart Syndrome

A 10-day-old child with hypoplastic left heart syndrome (HLHS) underwent first-stage palliation for HLHS, Norwood procedure with a Sano modification, i.e., placement of a right ventricular to pulmonary artery (RV-PA) conduit. The patient developed progressively worsening systemic oxygen desaturation in the immediate postoperative period. Stenosis of the proximal RV-PA conduit was diagnosed by echocardiography. In the catheterization laboratory stent placement in the conduit was performed. This resulted in increased systemic oxygen saturation. The patient was eventually discharged from the hospital with adequate oxygen saturations.

Modification of stage II procedure after hybrid palliation (bilateral pulmonary artery banding and ductal stenting) for hypoplastic left-sided heart syndrome: Modified arch reconstruction with retained stented ductus patch

Bilateral pulmonary artery banding and ductal stenting can be used as a “hybrid” alternative to Norwoodbased management for the palliation of neonates with single ventricle physiology. 1 By using the hybrid strategy, first-stage palliation defers cardiopulmonary bypass and arch reconstruction to a second-stage procedure at 4 to 6 months of age. At the second stage, distal aortic arch reconstruction is more difficult than a neonatal Norwood reconstruction because the ductal stent often extends into the descending aorta. The requirement for resection of all ductal tissue is based on observations that ductus tissue is friable, does not suture well, and will contract after cessation of prostaglandins. After 4 to 6 months, however, stented ductal tissue is robust and handles sutures well. Consequently, the necessity of removing all stented ductal tissue can be questioned. This report describes a simplified hybrid second-stage procedure that uses stented ductal tissue to reconstruct the aortic arch.

Invited commentary

Invited commentary

Effect of Shunt Type on Echocardiographic Indices After Initial Palliations for Hypoplastic Left Heart Syndrome: Blalock-Taussig Shunt Versus Right Ventricle-Pulmonary Artery Conduit

The study objective was to compare echocardiographic indices in infants with hypoplastic left heart syndrome (HLHS) randomized to different surgical pathways. Initial surgical palliation for HLHS has evolved to two strategies that vary by source of pulmonary blood flow: a modified Blalock-Taussig (BT) shunt or a right ventricle to pulmonary artery (RV-PA) conduit. Seventeen patients were randomized to either a BT shunt or RV-PA as their first-stage palliation, and 15 survived through bidirectional Glenn (BDG) (seven BT shunts/eight RV-PAs). Echocardiography was performed pre-stage 1 palliation (S1P), early post-S1P, pre-BDG, and post-BDG. Echocardiographic measurements included indices of right ventricle function and shape, right ventricle myocardial performance index, neoaortic Doppler flow patterns and cardiac output, Doppler inflow and tissue imaging, and conduit/neoaortic regurgitant fraction. There were no significant differences in right ventricle size, shape, or estimates of systolic and diastolic function between groups. Patterns of changes in neoaortic Doppler flow in the RV-PA conduit group showed a decrease in all indices of systemic flow early after initial palliation, but these measures consistently increase later post-S1P and post-BDG. In contrast, the BT shunt group increases these same indices post-S1P and pre-BDG with a decrease after shunt removal. Right ventricle size and function do not appear significantly affected by surgical type of initial palliation for HLHS, and this correlates with early outcome. Echocardiographic Doppler findings correlate well with the expected physiologies of the different shunts as these infants progress through initial palliative strategies.

Hybrid palliation in hypoplastic left heart syndrome

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. 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. 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.

First-stage angioplasty for a single ventricle with pulmonary artery coarctation

We report first-stage palliation consisting of pulmonary artery angioplasty and a systemic-to-pulmonary shunt using a minimized cardiopulmonary bypass technique performed with modified ultrafiltration for two patients with a single ventricle, pulmonary atresia, patent ductus arteriosus, and pulmonary coarctation during early infancy. Postoperative early extubation (15 and 18 h) and bilateral pulmonary artery growth before the second stage (Nakata indexes, in mm2/m2: right 94, left 53; and right 209, left 70) were experienced. Less-invasive pulmonary artery continuity reconstruction at an early stage is advantageous for low-weight patients with a single ventricle and pulmonary coarctation.

Clinical Implications of Major Aortopulmonary Collateral Arteries in Patients With Right Isomerism

The presence of major aortopulmonary collateral arteries (MAPCA) in patients with right isomerism may alter the prognosis, which is still unclear. From 1994 to 2003, 138 out of 155 patients (89%) with right isomerism had pulmonary stenosis or pulmonary atresia and constituted the study population. Two groups of patients were identified, one with MAPCA and the other without. The MAPCA originating from descending thoracic aorta or aortic arch was noted in 9 patients (6.5%), with a median number of MAPCA being 3 (range, 2 to 4). Patients with MAPCA commonly had congestive heart failure (8/9, 89%) and frequent lower airway infection (6/9, 67%). In comparison with patients with MAPCA and without, the intrapericardial pulmonary artery was more frequently absent (2 of 9 vs 1 of 129, p < 0.001), and the pulmonary arterial size was smaller (McGoon ratio <1.5, 8 of 9 vs 57 of 129, p = 0.009) in the MAPCA group. The mean pulmonary arterial pressure measured was often higher than the suggested limit for Fontan circulation. Major aortopulmonary collateral arteries were addressed at a median of 6 months old (2 to 19 months old) in 5 patients. Although surgical mortality was not different for first-stage palliation and Glenn shunt in both groups, total cavopulmonary connection could only be performed in one patient with MAPCA with poor results. However, the 5-year survival of patients with and without MAPCA was not statistically different (74.1% vs 55.2%, p = 0.19). The presence of MAPCA was uncommon but still noted in patients with right isomerism. It may cause heart failure and a less favorable pulmonary vasculature for a complete Fontan-type operation.

Three-dimensional Tricuspid Annular Function Provides Insight into the Mechanisms of Tricuspid Valve Regurgitation in Classic Hypoplastic Left Heart Syndrome

Tricuspid regurgitation (TR) has a negative impact on outcome in hypoplastic left heart syndrome (HLHS). There is a paucity of data assessing the mechanisms of TR. Lateral forces from the left ventricle play an important role in normal tricuspid valve (TV) function. This study evaluates the role of real-time 3-dimensional echocardiography in the assessment of the TV annulus and subvalvular apparatus in HLHS. In all, 31 real-time 3-dimensional echocardiographic studies from 26 patients (1 day-17 years old) with classic HLHS were included. In all, 7 studies were before and 8 were after first-stage palliation; 8 were postcavopulmonary shunt and 8 were post-Fontan. Only patients with severe left ventricular attenuation were included. Studies were subdivided into two groups: mild TR (N = 20) and severe TR (STR) (N = 11). Data from 17 control subjects were used for comparison. TV annular area, bending angle (saddle shape), and position of anterior papillary muscle were analyzed. All annular segments moved in equally during systole in mild TR, whereas septal-lateral diameter contraction was significantly diminished in STR. TV annular area was larger in HLHS (mild TR, 1265 +/- 325; STR, 1767 +/- 450 mm2/m2) than in control subjects (762 +/- 85) (P < .001). However, area change was reduced only in STR (12.4 +/- 4.6% vs control subjects 20.4 +/- 8.3%, P < .05). Annular shape was flat during systole in HLHS. Anterior papillary muscle was laterally displaced in STR. TV annular dynamics were different in patients with HLHS as a result of lack of interaction form left ventricle. Annular function, especially in septal-lateral direction, was further impaired in STR.

Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome

Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome

Risk-Stratified Approach to Hybrid Transcatheter–Surgical Palliation of Hypoplastic Left Heart Syndrome

We prospectively employed a risk-stratified approach to first-stage palliation of hypoplastic left heart syndrome. High-risk features included severe tricuspid insufficiency, severe right ventricular dysfunction, a severely restrictive or intact atrial septum, an ascending aortic diameter < or = 2 mm, late presentation, weight < 2 kg, or significant extracardiac issues, Infants without high-risk features underwent a Norwood procedure (with Sano modification), whereas infants with high-risk features underwent a hybrid procedure consisting of bilateral pulmonary artery banding, ductal stenting, and atrial septostomy or a Norwood/Sano. Operative survival for 10 infants without high-risk features undergoing a Norwood/Sano procedure was 90%. Operative survival for 5 infants with high-risk features undergoing hybrid palliation was 100%, compared to 29% in 7 infants with high-risk features undergoing the Norwood/Sano procedure. Although only short-term data are available, this hybrid palliative procedure may have a role for infants with hypoplastic left heart syndrome and high-risk features.

Right Ventricle-Pulmonary Artery Shunt in First-Stage Palliation of Hypoplastic Left Heart Syndrome

This article reviews our experience using a prosthetic conduit between the right ventricle (RV) and the pulmonary artery (PA), in lieu of the more traditional aortopulmonary shunt, for infants undergoing surgical palliation of hypoplastic left heart syndrome. Thirty-three consecutive infants underwent Norwood procedure between February 1998 and November 2003, using an RV-PA conduit. There were 31 hospital survivors (94%) and 25 patients have undergone a stage II bidirectional Glenn anastomosis with an additional two deaths. Nine patients have undergone completion Fontan. This technique provides reproducible results, simplifies postoperative management, and improves outcome, especially for low volume programs.

Simplified Antegrade Cerebral Perfusion and Myocardial Protection during Stage I Norwood Procedure

Several important modifications have been introduced in the intraoperative management of neonates with hypoplastic left heart syndrome during first-stage palliation. Among these, utilization of selective antegrade cerebral perfusion and interposition of a conduit between the right ventricle and pulmonary artery are currently favored by many centers. We briefly describe our current approach to the modified stage I Norwood procedure.

Outcome of Right Ventricle–to–Pulmonary Artery Shunt in First-Stage Palliation of Hypoplastic Left Heart Syndrome: A Multi-Institutional Study

Background To evaluate the developing surgical technique of a modified Norwood procedure using a right ventricle–to–pulmonary artery shunt, we analyzed data obtained from 73 infants who underwent first-stage palliation for hypoplastic left heart syndrome between 1998 to 2002 at three centers in Japan. Methods Procedures performed included an aortopulmonary neoaortic reconstruction and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 6 patients, 5 mm in 41, and 6 mm in 26. Continuous cerebral perfusion was used in all patients and an additional descending aortic perfusion was used in 39. Postoperative management was basically the same as that for infants undergoing other types of operations. Results There were 61 hospital survivors (84%), including 5 of 6 patients weighing less than 2 kg, with 8 late deaths. Risk factors for hospital mortality include preoperative treatment without ventilatory support and surgeon's experience (first 10 cases). Three patients underwent a primary Fontan operation at 5, 9, and 10 months of age, with one late death. Forty-one patients underwent the bidirectional Glenn shunt after a mean interval of 6.9 months, and 19 of them completed the Fontan operation at median age of 2.1 years. Overall survivals were 65% at 1 year and 63% at 2 years. Conclusions Improved survival for patients after first-stage palliation of hypoplastic left heart syndrome is reproducible for many centers by an application of the modified Norwood procedure with the right ventricle–to–pulmonary artery shunt.

Use of a saphenous venous homograft for constructing a conduit from the right ventricle to the pulmonary arteries in the Norwood procedure

The use of a conduit of polytetrafluoroethylene placed between the right ventricle and the pulmonary arteries as source of pulmonary arterial supply during the first stage of palliation for the hypoplastic left heart syndrome has facilitated post-operative management and resulted in decreased mortality. We describe here the use of a cryopreserved saphenous vein inserted in reversed direction to create the connection between the right ventricle and the pulmonary arteries in a neonate with low birth weight undergoing the modified Norwood procedure.

Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome

This article reviews our experience using a prosthetic conduit between the right ventricle (RV) and the pulmonary artery (PA), in lieu of the more traditional aortopulmonary shunt, for infants undergoing surgical palliation of hypoplastic left heart syndrome. Thirty-three consecutive infants underwent Norwood procedure between February 1998 and November 2003, using an RV-PA conduit. There were 31 hospital survivors (94%) and 25 patients have undergone a stage II bidirectional Glenn anastomosis with an additional two deaths. Nine patients have undergone completion Fontan. This technique provides reproducible results, simplifies postoperative management, and improves outcome, especially for “low volume” programs.