Management of Moderate Functional Tricuspid Valve Regurgitation at the Time of Pulmonary Valve Replacement: Is Concomitant Tricuspid Valve Repair Necessary?

Congenital heart defects with a component of pulmonary stenosis are often palliated in childhood by disrupting the pulmonary valve. Patients often undergo subsequent pulmonary valve replacement (PVR) to protect the heart from the consequences of pulmonary regurgitation. In the presence of associated moderate functional tricuspid valve (TV) regurgitation, it is unclear what factors contribute to persistent TV regurgitation following PVR. In particular, it is unknown whether PVR alone will reduce the right ventricular (RV) size and restore TV function or whether concomitant TV annuloplasty is required as well. Thirty-five patients were analyzed. Each patient underwent initial palliation of congenital pulmonary stenosis or tetralogy of Fallot, underwent subsequent PVR between 2002 and 2008, and had at least moderate TV regurgitation at the time of valve replacement. Serial echocardiograms were analyzed. Pulmonary and TV regurgitation, along with RV dilation and dysfunction, were scored (0, none; 1, mild; 2, moderate; 3, severe). RV volume and area were also calculated. Potential risk factors for persistent postoperative TV regurgitation were evaluated. One month following PVR, there was a significant reduction in pulmonary valve regurgitation (mean, 3 vs. 0.39; P < 0.0001) and TV regurgitation (mean, 2.33 vs. 1.3; P < 0.0001). There were also significant reductions in RV dilation, volume, and area. There were no significant further improvements in any of the parameters at 1 and 3 years. There was no difference in the degree of TV regurgitation postoperatively between those patients who underwent PVR alone and those who underwent concomitant tricuspid annuloplasty (mean, 1.29 vs. 1.31; P = 0.81). We conclude that following PVR, improvement in TV regurgitation and RV size occurs primarily in the first postoperative month. TV function improved to a similar degree with or without annuloplasty.     

Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation

While exercise stress testing (EST) is an important tool, little is known about its use for determining ischemia in patients with hypoplastic left heart syndrome (HLHS) and Fontan palliation. We sought to determine the frequency of ST segment changes during EST in HLHS patients after Fontan and examine results of further testing performed in response to ST changes. A single-center chart review of HLHS patients post-Fontan from January 1995 to December 2012 was performed. Data collected included demographics, indications for EST, resting electrocardiogram findings, EST and echocardiogram results and outcomes. ESTs were evaluated for ST segment changes concerning for ischemia. Results of additional testing performed based on concerning EST findings were collected. Twenty-seven patients underwent 64 ESTs (mean 2.4 ESTs/patient). Median age at first EST was 9.6 years (range 6.2–16.4). EST was concerning for ischemia in 13 patients (48 %) on 25 (39 %) ESTs. Based on EST results, two patients had stress sestamibi testing, two underwent coronary angiography, and one had both. No reversible perfusion defects or coronary artery obstructions were demonstrated. No patient who underwent EST has died. ST segment depression was not associated with ventricular dysfunction prior to EST or at the end of follow-up (p > 0.05). In patients with HLHS post-Fontan palliation, ST segment depression on EST is common. In patients who underwent further testing, no evidence of ischemia or coronary abnormalities was found. Additional testing may not be necessary in all patients.     

Norwood procedure for hypoplastic left heart syndrome: BT shunt or RV‐PA conduit?

BACKGROUND: The Norwood procedure is the first stage palliative procedure for hypoplastic left heart syndrome (HLHS). Traditionally the pulmonary circulation has been supplied via a modified Blalock Taussig (BT) shunt but a recent modification, adopted in some UK centres, substitutes a conduit between right ventricle and pulmonary arteries (RV-PA conduit). It is argued that this will result in a more favourable balance between pulmonary and systemic circulations. AIM: To compare the early postoperative haemodynamic profile between patients undergoing a BT shunt or an RV-PA conduit. METHODS: Retrospective review in a tertiary referral PICU of 51 children with HLHS undergoing the Norwood procedure with either a BT shunt (Group 1; n = 23) or an RV-PA conduit (Group 2; n = 28). Data items were extracted at 10 set time points in the initial 96 h, postoperatively. RESULTS: Diastolic BP was significantly lower in Group 1 (p<0.001) with a trend towards a higher systolic BP and no difference in mean BP. No between-group differences were found in markers of pulmonary blood flow (PaO2, PaCO2, PaO2/FiO2 ratio), or in markers of systemic blood flow (blood lactate, oxygen extraction ratio), or in estimated ratio of pulmonary:systemic blood flow (Qp:Qs). Despite lower diastolic blood pressure in Group 1 renal and hepatic function did not differ over five post-operative days between groups. CONCLUSIONS: With the exception of a higher diastolic blood pressure in the RV-PA conduit group, we found no difference in the early haemodynamic profile between patients undergoing an RV-PA conduit or a BT shunt.     

An Angiographic Predictor of Pulmonary Artery Stenosis After the Norwood-Sano Operation for Hypoplastic Left Heart Syndrome

The objective of this study was to determine angiographic predictors of future pulmonary artery stenosis (PS) in patients with hypoplastic left heart syndrome (HLHS) at the time of pre-stage 2 cardiac catheterization (PS2C). The Sano modification of the Norwood operation (NSO) for HLHS includes placement of a right ventricle-to-pulmonary artery (RV-PA) conduit. Branch PS is a recognized complication. Data from patients with HLHS who underwent NSO from 2005 to 2009 and who underwent PS2C were reviewed retrospectively. Nakata and McGoon indices were calculated in the traditional fashion, and modified Nakata and McGoon indices were calculated using the narrowest branch PA diameters. Thirty-three patients underwent NSO and 28 patients underwent PS2C. Mean follow-up was 35.8?±?7.5?months. Ten (36?%) patients had significant left branch PS, with two requiring balloon angioplasty and eight requiring stent placement, a median of 15.2?months after PS2C (interquartile range 1.2, 32.8). The modified Nakata index was predictive of future intervention for left PS (receiver operating characteristic curve area under the curve 0.811), with a cut-off of 135?mm²/m² and a sensitivity of 100?% and specificity of 72.2?%. A modified Nakata index <135?mm²/m² at PS2C predicts future need for intervention on left-branch PS in patients with HLHS after the NSO. Surgical pulmonary arterioplasty at the time of stage 2 surgical palliation may obviate the need for future interventions.     

Outcomes of the Modified Norwood Procedure: Hypoplastic Left Heart Syndrome Versus Other Single-Ventricle Malformations

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p < 0.001). The follow-up data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignificant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan–Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant difference). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ significantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post-Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan completion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of functional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.     

Hypoplastic left heart syndrome in PAGOD syndrome

Chromosomal abnormalities as well as non‐cardiac anomalies have been identified as independent risk factors for surgical morbidity and mortality in Fontan palliation. The combination of malformations consisting of pulmonary hypoplasia, agonadism (sex reversal), omphalocele, and diaphragmatic defect is compatible with pulmonary artery and lung hypoplasia, agonadism, omphalocele, and diaphragmatic defect (PAGOD). Most cases have been associated with cardiac disease, particularly hypoplastic left heart syndrome (HLHS) that is potentially destined for Fontan palliation. Reported herein is the case of a Japanese female infant diagnosed with PAGOD syndrome along with HLHS (mitral atresia and aortic atresia), in whom intractable respiratory failure manifested as bilateral eventration of the diaphragm and presumed right lung hypoplasia. These characteristic pulmonary lesions associated with the syndrome precluded use of the Fontan pathway.     

功能性单心室的外科治疗及近期疗效

目的 总结功能性单心室的外科治疗经验.方法 回顾性分析我科2003年12月至2010年7月59例功能性单心室患儿,对其手术时机,手术方式及手术效果进行分析,并用Logistic回归分析Glenn手术的高危因素.结果 死亡8例(病死率13.56%),其中4例为Glenn术后死于低心排和多器官功能衰竭,2例为Banding术后死于低心排,1例为TCPC术后死于多器官功能衰竭,1例为B-T分流术后死于低心排.Glenn术后的主要并发症为上腔静脉梗阻综合症和低心排,Fontan术后的主要并发症是房性心律失常和胸腔积液.Logistic回归分析得出房室瓣反流是Glenn术后患儿死亡的高危因素.结论 功能性单心室心内畸形复杂,需根据肺血流的情况及早进行姑息手术,选择合适的术式.改良Fontan是进行生理矫正的理想术式,保留肺动脉前向血流的Glenn手术是保证患儿顺利进行Fontan术的有效方法,但需根据肺动脉狭窄的程度进行处理,避免上腔静脉综合症的发生.

Abstract：

Objective To retrospectively analyze the outcome of surgical treatment of patients with functional single ventricle. Methods Betveen December 2003 and July 2010, 59 patients with functional single ventricle underwent corrective surgeries, and were followed up at this center. Their median age was 3. 0 months old at pulmonary artery band surgery (PAB), 13. 5 months at bidirectional Glenn procedure (BDG), and 11 years old at Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, age at surgery, and type of the surgical procedure were retrospectively analyzed. Logistic regression analysis was also performed to determine the risk factors associated with Glenn. Results Duration of mechanical ventilation was 30. 4 ± 19. 7 hours. Length of stay in ICU was 4. 3 ± 1.6 days. Length of hospital stay was 34. 8 ± 15. 9 d.SpC2 increased from 75 ± 6. 97% to 88 ± 6. 28% after operation. Exercise tolerance was also improved remarkably. Eight deaths were noted after surgery (13. 56%). The leading complications after Glenn surgery were superior vena cava syndrome and low cardiac output syndrome. Complications after Fontan surgery were atrial tachycardia and pleural effusions. Logistic regression analysis suggested atrioventricular valve regurgitation was an important risk factor for the death of the patients had Glenn surgery. Conclusions The clinical outcome of surgical treatment of patients with functional single ventricle is satisfied. The surgical strategy should be made according to anatomic abnormality of the functional single ventricle and pulmonary circulation. Modified Fontan operations can become an alternative therapy for patients who are not suitable for double ventricle correction. Glenn with antegrade pulmonary blood flow may be a temporary palliation prior to Fontan surgery, but the pulmonary blood flow should be controlled to avoid the occurrence of superior vena cava syndrome.     

The Lateral Tunnel Fontan Procedure for Hypoplastic Left Heart Syndrome: Results of 100 Consecutive Patients

The Fontan procedure for hypoplastic left heart syndrome (HLHS) is well established. Multiple surgical techniques including extracardiac conduits and autologous tissue connections have been developed. We reviewed the results of 100 consecutive patients undergoing the lateral tunnel modification of the Fontan procedure at the University of Michigan. A cross-sectional retrospective study was performed for 100 consecutive patients identified in the University of Michigan Congenital Heart Surgery database with the diagnosis of HLHS. All patients had undergone a lateral tunnel Fontan procedure between June 2000 and August 2004. The medical record was reviewed to assess patient, procedural, and morphologic determinants of outcome. Hospital survival was 97% and intermediate-term survival was 96% with a median follow-up time of 34 months. Preoperative mean pulmonary artery pressure, right ventricular end diastolic pressure, aortic cross-clamp time, and tricuspid valve regurgitation were not associated with late right ventricular function or survival. Three patients required takedown of the lateral tunnel Fontan in the early postoperative period. A positive association was found between protein-losing enteropathy and prolonged (>2 weeks) postoperative pleural drainage (p = 0.035). No patient required cardiac transplantation or late intervention on the Fontan pathway. At the time of follow-up, 100% of patients were New York Heart Association class I or II and 90% were in normal sinus rhythm. The lateral tunnel Fontan procedure for HLHS can be performed with acceptable early and intermediate-term risk. There was a low prevalence of late rhythm disturbances and other complications. Protein-losing enteropathy and prolonged pleural drainage were associated.     

心外管道全腔肺分流手术的临床应用

目的 探讨心外管道全腔肺分流手术在小儿复杂青紫型先天性心脏病中的应用价值.方法 1998年1月至2008年10月48例患儿接受了心外管道全腔肺分流手术,其中男37例,女11例;年龄3.1～14.5岁,平均(5.6±3.0)岁;体重13～56 kg,平均(21.1±10.3)kg;三尖瓣闭锁23例,单心室20例,右心室双出口(远离大动脉型)2例,室间隔完整的肺动脉闭锁2例,法乐四联症伴完全性房室间隔缺损1例;术前SPO270%～93%,平均(80.2±6.1)%.38例患儿在行心外管道全腔肺分流手术前接受多次手术,包括改良BT分流5例次,肺动脉环缩4例次,双向腔肺分流手术36例次.12例患儿在同一次手术中完成全腔肺分流手术.结果 术后早期死亡3例;乳糜胸7例,气胸2例,胸腔金葡菌感染1例,短暂的交界性心动过速2例,均治愈;术后胸腔引流放置3.5～48 d.随访1个月至12年,晚期死亡2例,其余35例心功能恢复至Ⅰ级,6例心功能Ⅱ级,2例心功能Ⅲ级,随访心电图均为窦性心律.统计分析Ⅰ期Fontan和分期Fontan在术后机械通气时间、监护时间、引流时间,病死率上无统计学差异.结论 心外管道全腔肺分流手术术后心律失常的发生率低,开窗术有助于围术期恢复,Ⅰ期Fontan在部分患儿中是可行的.     

Tricuspid Annular Plane Systolic Excursion Does Not Correlate With Right Ventricular Ejection Fraction in Patients With Hypoplastic Left Heart Syndrome After Fontan Palliation

Tricuspid annular plane systolic excursion (TAPSE) reflects longitudinal myocardial shortening, the main component of right ventricular (RV) contraction in normal hearts. To date, TAPSE has not been extensively studied in patients with hypoplastic left heart syndrome (HLHS) and systemic RVs after Fontan palliation. This retrospective study investigated HLHS patients after Fontan with cardiac magnetic resonance (CMR) performed between 1 January 2010 and 1 August 2012 and transthoracic echocardiogram (TTE) performed within 6 months of CMR. The maximal apical displacement of the lateral tricuspid valve annulus was measured on CMR (using four-chamber cine images) and on TTE (using two-dimensional apical views). To create TTE–TAPSE z-scores, published reference data were used. Intra- and interobserver variability was tested with analysis of variance. Inter-technique agreement of TTE and CMR was tested with Bland–Altman analysis. In this study, 30 CMRs and TTEs from 29 patients were analyzed. The age at CMR was 14.1 ± 7.1 years, performed 11.9 ± 7.8 years after Fontan. For CMR–TAPSE, the intraclass correlation coefficients for inter- and intraobserver variability were 0.89 and 0.91, respectively. The TAPSE measurements were 0.57 ± 0.2 cm on CMR and 0.70 ± 0.2 cm on TTE (TTE–TAPSE z score, ?8.7 ± 1.0). The mean difference in TAPSE between CMR and TTE was ?0.13 cm [95 % confidence interval (CI) ?0.21 to ?0.05], with 95 % limits of agreement (?0.55 to 0.29 cm). The study showed no association between CMR–TAPSE and RVEF (R = 0.08; p = 0.67). In patients with HLHS after Fontan, TAPSE is reproducible on CMR and TTE, with good agreement between the two imaging methods. Diminished TAPSE suggests impaired longitudinal shortening in the systemic RV. However, TAPSE is not a surrogate for RVEF in this study population.     

Assessment of Myocardial Function in Pediatric Patients with Operated Tetralogy of Fallot: Preliminary Results with 2D Strain Echocardiography

The global myocardial function in patients after repair of tetralogy of Fallot (TOF) can be assessed by cardiovascular magnetic resonance (CMR) and measurement of B-type natriuretic peptides. Two-dimensional echocardiography-derived strain and strain rate (2D strain) facilitate the assessment of regional myocardial function. We evaluated myocardial function in 16 children with residual severe pulmonary valve regurgitation and right ventricular (RV) volume overload after TOF repair before, 1 month after, and 6 months after pulmonary valve replacement (PVR). In 2D strain echocardiography preoperatively, the longitudinal systolic RV strain was reduced (p < 0.05). One month after PVR, longitudinal systolic RV strain decreased further (p < 0.05), while systolic and early diastolic radial left ventricular strain and strain rate increased (each p < 0.05), followed by a return toward preoperative values after 6 months. Six months after PVR, preoperatively elevated RV end-diastolic volume (p < 0.01) assessed by CMR and N-terminal pro-B-type natriuretic peptide (p < 0.05) decreased. In conclusion, the impairment of the regional myocardial after TOF repair and transient changes after PVR can be subtly analyzed by 2D strain echocardiography in addition to the established assessment of myocardial function with CMR and measurement of B-type natriuretic peptides.     

无创伤肺血管阻力估测法在二期Fontan术术前肺血管评价中的应用

目的 采用无创伤肺血管阻力评价方法,观察二期Fontan术术前肺血管阻力(PVR)估计值与手术预后的关系.方法 选择33例施行二期Fontan手术的患儿,根据术前血红蛋白(HGB)、红细胞比容(HCT)、经皮氧饱和度(SpO2)计算PVR估计值.按PVR估计值＜2.4 wood、2.4～3.2wood和＞3.2 wood单位分成低危、高危和极高危3组,观察术后严重低心排和多脏器功能衰竭的发生率;同时按手术后是否出现严重低心排和多脏器功能衰竭分成2组,未出现者为甲组,出现者为乙组,观察2组间术前HGB、HCT、SpO2和PVR估计值的差异.结果 根据PVR估计值分成的低危、高危和极高危3组患儿,术后严重低心排和多脏器功能衰竭的平均发生率分别为10.0%、32.4%和63.6%,差异有统计学意义(P＜0.01);根据手术后是否出现严重低心排和多脏器功能衰竭的两组患儿,术前HGB、HCT、SpO2和PVR均有显著差异,其中,PVR(HGB)甲组为(2.53±0.56),乙组为(3.24±0.58),PVR(HCT)甲组为(2.59±0.58),乙组为(3.21±0.79),PVR(SpO2)甲组为(2.22±0.55),乙组为(2.93±0.58),差异均有统计学意义(t=3.25、2.52、3.33,P＜0.01或0.05).结论 无创伤方法计算的PVR估计值与手术后的多脏器功能不全的发生概率相吻合,故可作为评价二期Fontan手术指征和估计预后的方法.     

Fate of Ventricular and Valve Performance Following Early Bidirectional Glenn Procedure After Norwood Operation Controlled for Hypoplastic Left Heart Syndome Anatomic Subtype

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS–MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS–MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7–4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS–MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.     

Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part II - Cyanotic Heart Defects

The purpose of this review/editorial is to discuss how and when to treat the most common cyanotic congenital heart defects (CHDs); the discussion of acyanotic heart defects was presented in a previous editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. While some patients with acyanotic CHD may not require surgical or transcatheter intervention because of spontaneous resolution of the defect or mildness of the defect, the majority of cyanotic CHD will require intervention, mostly surgical. Total surgical correction is the treatment of choice for tetralogy of Fallot patients although some patients may need to be palliated initially by performing a modified Blalock-Taussig shunt. For transposition of the great arteries, arterial switch (Jatene) procedure is the treatment of choice, although Rastelli procedure is required for patients who have associated ventricular septal defect (VSD) and pulmonary stenosis (PS). Some of these babies may require Prostaglandin E₁ infusion and/or balloon atrial septostomy prior to corrective surgery. In tricuspid atresia patients, most babies require palliation at presentation either with a modified Blalock-Taussig shunt or pulmonary artery banding followed later by staged Fontan (bidirectional Glenn followed later by extracardiac conduit Fontan conversion usually with fenestration). Truncus arteriosus babies are treated by closure of VSD along with right ventricle to pulmonary artery conduit; palliative banding of the pulmonary artery is no longer recommended. Total anomalous pulmonary venous connection babies require anastomosis of the common pulmonary vein with the left atrium at presentation. Other defects should also be addressed by staged correction or complete repair depending upon the anatomy/physiology. Feasibility, safety and effectiveness of treatment of cyanotic CHD with currently available medical, transcatheter and surgical methods are well established and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.     

Severe Pulmonary Regurgitation Late After Total Repair of Tetralogy of Fallot: Surgical Considerations

Background After total repair of tetralogy of Fallot (TOF-R) with transannular patching (TAP), severe pulmonary regurgitation (PR) is reported to develop in up to 30% of patients at a follow-up of 20 years, and 10–15% or more need pulmonary valve replacement (PVR). In this study, time-related progression of PR and right ventricular (RV) dilatation, and functional recovery of the RV after PVR are analyzed, and the possible causes of PR and timing of PVR are discussed.Methods Eighteen patients, who late after TOF-R with TAP underwent PVR for severe PR, were chosen for the study. NYHA class, QRS duration, RV dilatation index (RVDI = RVEDD/LVEDD), and RV–distal pulmonary artery (PA) peak systolic gradient were reviewed and retrospectively analyzed.Results TOF-R was performed at a mean age of 5.1 ± 3.9 years (range: 0.6–12.8 years); the mean time interval from TOF-R to PR grade 3 onset was 11.8 ± 7.0 years (range: 3.3–27.4 years), and from TOF-R to PVR was 18.5 ± 7.8 years (range: 8.7–37.1 years).At PVR, 11 patients were in NYHA class II–III, all patients had severe PR (grade 3/3) and severe RV enlargement, 4 patients had ventricular arrhythmias, 7 patients significant distal pulmonary artery stenosis, and 2 patients small nonrelevant residual VSD. The mean preoperative RVDI (normal: 0.5) was 0.99 ± 0.14 (range: 0.75–1.3), the mean QRS duration 170 ± 24 ms (140–220 ms), and the mean RV–distal PA peak systolic pressure gradient 33.3 ± 19.0 mmHg (range: 10–60 mmHg). Patients aged at TOF-R> 5 years had considerably longer redo-free intervals than their younger counterparts: mean 23.1 years (range 8.7–37.1 years) vs 14.8 years (range: 9.3–21.2 years), respectively. The redo-free intervals and the duration of severe PR correlated inversely with the RV-PA gradient.At a mean follow-up of 1.3 years (2 weeks–5 years), the mean RVDI decreased from 0.99 ± 0.14 to 0.69 ± 0.15, the mean validity class improved from 2.5 to 1.1. One patient died.Conclusions After TOF-R with TAP, the progression of PR has very individual dynamics, resulting in extremely varying redo-free intervals. Concomitant pulmonary stenosis seems to exaggerate progression of PR. PVR results in effective reduction of diastolic dimensions of severely dilated RV and in improvement of validity class. Referred PVR in no-risk cases seems to be justified.Presented at the 32nd Annual Meeting of the GSTCS, Leipzig, Germany, 24 February 2003.     

Outcomes of Hypoplastic Left Heart Syndrome in Low-Birth-Weight Patients

The objective of this study was to assess outcomes of hypoplastic left heart syndrome (HLHS) patients weighing ≤2.5 kg throughout staged palliation. We performed a single-center retrospective review. Abstracted data included gestational age, birth weight, presence of noncardiac anomalies, and survival through Fontan. Fifty-two patients met inclusion criteria, with a median birth weight of 2.14 kg and gestational age of 36 weeks. Five patients received comfort care only. Of 47 patients who underwent initial surgical palliation, 51% survived to initial hospital discharge. Birth weight and gestational age (GA) were similar between survivors and nonsurvivors. Compared with survivors, risk factors for death prior to initial hospital discharge were as follows: small for GA (P = 0.005), noncardiac anomalies (P = 0.04), need for post-perative extracorporeal membrane oxygenation (P = 0.0004), and conversion from initial palliation to Sano shunt (n = 5, no survivors). Operative survival following Stage 2 palliation was 91% (21/23) and 94% after Fontan (17/18). Overall survival for palliated patients from birth through Fontan was 36%. Low-birth-weight neonates with HLHS have poor overall survival through the Fontan operation, with highest mortality following Stage 1 palliation. Being small for GA and the presence of noncardiac anomalies are important preoperative risk factors for early mortality.     

Lessons Learned from the Development of a New Hybrid Strategy for the Management of Hypoplastic Left Heart Syndrome

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.     

Lessons Learned from the Development of a New Hybrid Strategy for the Management of Hypoplastic Left Heart Syndrome

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.     

Tricuspid atresia with imperforate valve: Angiographic findings and surgical implications in two cases with AV concordance and normally related great arteries

Summary The angiographic and intraoperative findings are described in two children with imperforate tricuspid valve with atrioventricular and ventriculoarterial concordance who underwent a successful Fontan operation. It is important from a surgical point of view to distinguish this rare variant of tricuspid atresia from the more classic form with absent atrioventricular connection. The characteristic feature of hearts with imperforate tricuspid valve is the presence of a normally formed right ventricle which, in the presence of ventriculo-arterial concordance, can be used to support the pulmonary circulation. When the imperforate valve is large, it may be possible to remove it, and restore atrioventricular continuity. Alternatively, an atrioventricular type of Fontan repair using a valved conduit should be considered as the treatment of choice in the presence of a suitable pulmonary arterial tree and pulmonary valve.