Initial Pulmonary Artery Banding Research Articles

Bidirectional glenn surgery without palliative pulmonary artery banding in univentricular heart with unrestricted pulmonary flow. Retrospective multicenter experience

BackgroundAlthough pulmonary artery banding (PAB) has been generally acknowledged as an initial palliative treatment for patients having single ventricle (SV) physiology and unrestrictive pulmonary blood flow (UPBF), it may result in unfavorable outcomes. Performing bidirectional Glenn (BDG) surgery without initial PAB in some selected cases may avoid the complications associated with PAB and reduce the number of operative procedures for these patients. This research aimed to assess the outcome of BDG surgery performed directly without doing initial PAB in patients with SV-UPBF.MethodsThis Multicenter retrospective cohort includes all patients with SV-UPBF who had BDG surgery. Patients were separated into two groups. Patients in Group 1 included patients who survived till they received BDG (20 Patients) after initial PAB (28 patients), whereas patients in Group 2 got direct BDG surgery without first performing PAB (16 patients). Cardiac catheterization was done for all patients before BDG surgery. Patients with indexed pulmonary vascular resistance (PVRi) ≥ 5 WU.m2 at baseline or > 3 WU.m2 after vasoreactivity testing were excluded.ResultsCompared with patients who had direct BDG surgery, PAB patients had a higher cumulative mortality rate (32% vs. 0%, P = 0.016), with eight deaths after PAB and one mortality after BDG. There were no statistically significant differences between the patient groups who underwent BDG surgery regarding pulmonary vascular resistance, pulmonary artery pressure, postoperative usage of sildenafil or nitric oxide, intensive care unit stay, or hospital stay after BDG surgery. However, the cumulative durations in the intensive care unit (ICU) and hospital were more prolonged in patients with BDG after PAB (P = 0.003, P = 0.001respectively).ConclusionDirect BDG surgery without the first PAB is related to improved survival and shorter hospital stays in some selected SV-UPBF patients.

Abstract 10158: Initial Bilateral Pulmonary Artery Banding for Infants With Critical Left Heart Obstruction: A Multi-Institutional Analysis of Outcomes

Introduction: The hybrid procedure (bilateral PA bands [PAB] ± ductal stent) is a contemporary management option for infants with critical left heart obstruction. We sought to determine patient/procedural characteristics, subsequent outcomes and associated factors after PAB in a multi-institutional study. Methods: From 2005-2019, 214 of 1236 infants from 24 Congenital Heart Surgeons’ Society participating institutions underwent PAB. Median follow-up was 7 years. Parametric hazard modeling with competing risk methodology was performed to determine risk and associated factors for end-states of biventricular repair (BVR), Fontan, transplantation (Tx) or death without an end-state. Results: PAB was performed at a median age of 7 days & median weight of 3.1 kg. Intention of PAB varied - institutional preference (21%), non-cardiac comorbidities (20%), BVR consideration (12%), etc. Ductal stent was placed in 69% of patients (74% same day as PAB) and atrial septal interventions occurred in 61% (24% same day as PAB). Preoperative comorbidities (e.g. prematurity, genetic syndromes, neurological diagnoses, etc.) were present in 70%. At 5 years, 9% had reached BVR, 36% Fontan and 12% Tx (21/26 Tx immediately after PAB), with 35% having died and 8% alive without an end-state (Figure). Significant factors associated with BVR were presence of VSD and ductal stent; with Tx: earlier era, underlying aortic atresia, older age and greater weight at PAB, and absence of ductal stent; with death: low birth weight, ≥ moderate tricuspid valve regurgitation before PAB, & older age at PAB. Conclusions: Heterogeneity is prevalent in patient/procedural characteristics and subsequent procedures and outcomes after PAB, possibly influenced by institutional preference and different intended management pathways. There is important mortality and <50% of patients achieve a Fontan or BVR. Preoperative factors influence the use of this approach and thus are an important influence on outcomes.

Hypoplastic Left Ventricle: Left Ventricular Recruitment With Hybrid Approach.

Decision-making for biventricular repair (BVR) or univentricular palliation is challenging in neonates with hypoplastic left heart complex (HLHC). Hybrid strategy can be used successfully to achieve BVR in some of these patients. Between June 1998 and January 2022, 342 patients with a diagnosis of HLHS/variants, ductal-dependent lower body circulation with two ventricles, or HLHC with borderline left ventricle (LV) underwent initial bilateral pulmonary artery banding and ductal stenting in our institution. Among these 342 patients, 224 patients were defined as HLHS/variants and underwent univentricular palliation. 118 patients were determined to have borderline LV and hypoplastic left ventricular structures (HLHC, n = 48) or ductal-dependent lower body circulation with two ventricles (n = 70) considered suitable for BVR. 48 patients had multilevel obstructions including both aortic and mitral valve hypoplasia, aortic arch hypoplasia, and borderline LV. These were considered to have HLHC. These 48 HLHC patients are the subject of this report. Neonatal hybrid palliation at a median age of seven days was performed in 48 patients and 46 patients underwent BVR at a median age of 156 days. In 46 patients who underwent BVR, hospital mortality was not observed. The median follow-up was 66 months, with no late mortality. Heart transplant-free survival at 5, 10, and 15 years was 95.7%. 12 patients (26%) required reoperation and 9 (19.5%) required catheter reinterventions. Hybrid palliation may increase the chances for biventricular circulation in patients with borderline LV and small left-sided structures. Giessen hybrid approach as a left ventricular recruitment strategy achieves excellent early and long-term results.

Abstract 10996: Predictors of Surgical Reintervention Before Bidirectional Cavopulmonary Shunt in Children with Single Ventricle Physiology Who Underwent Main Pulmonary Artery Banding

Introduction: Unplanned intervention before stage 2 palliation (S2P) in patients with single ventricle (SV) lesions with dual unobstructed outflows palliated with pulmonary artery banding (PAB) is common. The factors contributing to intervention and its association with successfully undergoing S2P are unclear. Hypothesis: Early intervention is more common when PAB is placed at a younger age and with a lower initial PAB pressure gradient. Methods: Retrospective single center study of patients with SV lesions undergoing PAB between Jan 2000 and Dec 2020. Association with reintervention and successful S2P was modeled using exploratory cause-specific hazard regression. A multivariable model was developed adjusting for clinical and statistically relevant predictors. The cumulative proportion of patients undergoing intervention were summarized using a competing risk model. Results: A total of 77 patients - 53% female, 86% term and 12% syndromic - underwent PAB at median (IQR) 47 (24-66) days and 3.73 (3.2-4.5) kg. Within 18 months of PAB, 60 (78%) reached S2P, 9 (12%) died, 1 (1%) transplanted and 7 (9%) were alive without S2P. Intervention occurred in 18 (23%) prior to S2P (10/18) or death (4/18); 16 (21 %) occurred within 6 months of PAB. Intervention included PAB adjustment (n=7), conversion to DKS/BTT shunt (n=5), balloon atrial septostomy (n=4) and addition of a BTT shunt (n=2). Repeat intervention was required in 4 patients. Intervention was associated with genetic syndrome (p= 0.03) and lower weight at PAB (p=0.02) but not with age at PAB, pre/postoperative oxygen saturation, PAB length or gradient. Presence of genetic syndrome was the only risk factor associated with not reaching S2P (p=0.02). Conclusions: Intervention is common following PAB and associated with presence of genetic disorder or smaller patient size. Children with genetic syndrome aiming to undergo S2P are at higher risk for intervention and death and may benefit from alternate treatment strategy.

Contemporary patterns of the management of truncus arteriosus (primary versus staged repair): outcomes from the Japanese National Cardiovascular Database.

Although primary repair in early infancy has for decades been the prevalent strategy for management of truncus arteriosus (TA), recent concerns about the levels of morbidity and mortality have led to consideration of a staged surgical approach. Our goal was to describe recent patterns of management, to characterize patients who underwent primary or staged repair and to evaluate risk factors associated with operative mortality in a contemporary multicentre cohort. In the Japanese Cardiovascular Surgery Database, we identified all cases of TA undergoing an initial surgical procedure from 2008 to 2018. Operative mortality was defined as death within 30 days of an operation or in-hospital death regardless of the length of hospital stay. The hospital volume was defined by the average volume of TA repairs per year. The total number of patients undergoing initial surgery for TA was 286. Sixty-eight (24%, 68/286) underwent primary repair (primary repair group). The remaining 218 (76%, 218/286) underwent initial bilateral pulmonary artery banding as part of a planned staged approach (staged repair group). One hundred sixty-two patients out of 218 initially banded patients underwent the repair of TA during this study period. Concomitant diagnoses in the entire cohort included interrupted aortic arch repair in 36 patients and truncal valve regurgitation in 32. No centres handling an average of ≥2 truncus cases/year of the repair of TA were identified in this cohort. A total of 30% (85/286) of the cases were performed at centres that handled an average of ≥1 and <2 cases/year. The remaining 70% were at centres with <1 case/year. Overall, 37 patients (12.9%; 37/286) died. The operative mortality rates in the primary and staged repair groups were similar: that for the primary repair group was 16.2% (11/68) versus 11.9% for the staged repair group (26/218; P = 0.41). With multivariable logistic regression analysis, the factors most strongly associated with operative mortality were preoperative heart failure requiring catecholamine support (odds ratio, 4.18; 95% confidence interval 1.96-8.96) and the repeat bilateral pulmonary artery banding (odds ratio, 3.89; 95% confidence interval 1.08-14.07). The staged repair of TA has emerged as the preferred option for surgical timing at most of the centres participating in the Japanese Cardiovascular Surgery Database. The management outcomes of the patients with TA were favourable, even for the patients at low-volume centres.

Outcomes of Primary Bidirectional Glenn in Children with Single Ventricle Physiology and Increased Pulmonary Blood Flow.

We reported our experience in managing patients with single ventricle (SV) physiology and increased pulmonary blood flow (PBF), aiming to assess if it is feasible to proceed with primary Bidirectional Glenn (BDG) without a prior operation to limit PBF. This is a retrospective study with 51 consecutive patients who underwent BDG operation as a primary operation or a second stage prior to the definitive Fontan operation at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia between 2010 and 2018. Patients were categorized into two groups based on their PBF prior to the operation: Patients who had SV physiology and increased PBF (seven patients) vs. patients with SV physiology and restricted PBF (44 patients). The median age for the increased PBF group was 9.9 months [interquartile range (IQR): 2-16.9 months], and the median age for the restricted PBF group was 15.3 months (IQR: 6.7-42.6 months). Although the length of hospital stay was longer in patients with increased PBF (P = 0.039), we couldn't find a statistically significant difference in early mortality, duration of mechanical ventilation, length of pleural drainage, and length of intensive care unit (ICU) stay between the groups. In our experience, we found that primary BDG could be done safely for patients having SV physiology and increased PBF with acceptable short-term outcomes. It might further reduce the morbidity and mortality for those patients by avoiding the risk of initial pulmonary artery banding or aortopulmonary shunts.

Early repair of complete atrioventricular septal defect has better survival than staged repair after pulmonary artery banding: A propensity score–matched study

ObjectivesComplete atrioventricular septal defect (cAVSD) repair is usually performed between 3 and 6 months of age. However, some children present with early heart failure requiring intervention. It is unclear whether primary complete repair or initial pulmonary artery banding (PAB) provides better outcomes. MethodsAll patients (n = 194) who underwent surgery for cAVSD younger than 3 months of age between 1990 and 2019 were included. Propensity score matching was performed on risk factors for mortality. ResultsPrimary complete repair was performed in 77.8% (151/194), whereas PAB was performed in 22.2% (43/194). Children who had PAB were younger (P < .01), had lower weight (P < .001), and less trisomy 21 (P = .04). Interstage mortality for PAB was 18.6% (8/43), whereas early mortality for primary repair was 3.3% (5/151). Survival at 20 years was 92.0% (95% confidence interval [CI], 85.6%-95.7%) for primary repair and 63.2% (95% CI, 42.5%-78.1%) for PAB (P < .001). There was no difference in left atrioventricular valve (LAVV) reoperation rates (P = .94). Propensity score matching produced 2 well-matched groups. Survival at 20 years was 94.2% (95% CI, 85.1%-98.8%) for primary repair, and 58.4% (95% CI, 33.5%-76.7%) for PAB (P = .001). There was no difference in LAVV reoperation rates (P = .71). Neonatal repair was achieved with no early deaths and 100% survival at 10 years. ConclusionsIn children younger than 3 months of age, complete repair of cAVSD is associated with better survival than PAB. Both strategies have similar rates of LAVV reoperation. Neonatal repair of cAVSD can be achieved with excellent results. Primary repair of cAVSD should be the preferred strategy in children younger than 3 months of age.

Early- and midterm outcomes of pulmonary artery band as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(Q(R))), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model. Results: There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(Q(R))) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death. Conclusions: The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.

Bilateral Pulmonary Artery Banding before Norwood Procedure: Survival of High-Risk Patients

This study reports midterm results of high-risk patients with hypoplastic left ventricle treated with initial bilateral pulmonary artery banding (PAB) before secondary Norwood procedure (NP). Retrospective study of 17 patients admitted between July 2012 and February 2017 who underwent this treatment strategy because diagnosis or clinical status was associated with high risk for NP. Survival was compared with that of patients who underwent primary NP. Mean Aristotle comprehensive complexity score for NP would have been 19.7 ± 2.6. Risk factors included obstructed pulmonary venous return (n = 9), body weight < 2.5 kg (n = 7), total anomalous pulmonary venous connection (n = 3), and necrotizing enterocolitis (n = 1). Ten patients had a score ≥ 19.5. Early survival after PAB was 82.4% (14/17). NP was performed in 14 patients after improvement of clinical condition at a median age of 56 days and a weight ≥2,500 g. There was no 30-day mortality, but one interstage death. One patient died later after Glenn operation. One-year survival after primary PAB followed by NP was 70.6 ± 11.1%. During the same period, 35 patients with overall lower risk factors underwent primary NP; early postoperative survival and 1-year survival were 88.6 ± 5.4% and 68.6 ± 7.8%, respectively. There was no significant difference in survival between the two groups (p = 0.83) despite higher risk in the secondary Norwood group (p <0.0001). PAB before NP in high-risk patients constituted salvage management. Primary PAB provided enough time for stabilization and control of most risk factors. It allowed midterm survival equivalent to the survival after primary NP in lower risk neonates.

LONG TERM OUTCOMES IN DOUBLE INLET LEFT VENTRICLE AND TRICUSPID ATRESIA WITH TRANSPOSED GREAT VESSELS

In patients with tricuspid atresia and transposed great vessels (TA-TGA) and double inlet left ventricle (DILV), the presence of systemic outflow tract obstruction and aortic arch obstruction has led to varied surgical approaches including Norwood palliation or initial pulmonary artery banding with

2.5 kg以下の新生児・乳児における体外循環非使用肺動脈絞扼術の転帰と問題点

Background: Congenital heart defects have been an important cause for death among low birth weight (LBW) infants. Corrective surgery is not always a favorable solution for these fragile babies, and surgeons may choose palliative surgery, which is technically demanding. We reviewed our 10 years of experience and the outcome of initial pulmonary artery banding (PAB) without cardiopulmonary bypass (CPB) in LBW infants. Patients and methods: Eleven infants (age, 6~78 days; body weight, 1.1~2.5 kg) underwent initial PAB without CPB (9 cases of main PAB and 2 cases of branch PAB). Results: One patient died from a non-cardiac event. Nine patients successfully underwent the second-stage operation, at a mean weight of 4.6 kg, and eight completed the nal stage. One patient experienced ventricular volume overload a er main PAB. e mean Pp/Ps (pulmonary systemic blood pressure ratio) was 0.54 and the circumference of the band for the main PAB was body weight (kg)+18.2 mm, which was a little tighter than Trusler’s rule. e circumference for the branch PAB was body weight (kg)+8.0 mm. Of the patients who weighed less than 2 kg, all ve gained body weight, and four of them successfully reached the nal stage. Conclusion: PAB in LBW infants resulted in fair clinical outcomes with acceptable morbidity and mortality. However, there is a need for speci c guidance, based on accumulated experience, for the procedure and management.

Outcomes of Double Inlet Left Ventricle and Similar Morphologies: A Single Center Comparison of Initial Pulmonary Artery Banding Versus a Norwood-Type Reconstruction

Patients with double inlet left ventricle (DILV)/transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. The aim of this study is to evaluate the outcomes of initial pulmonary artery banding (PAB) compared with the Norwood-type reconstruction in neonates. A retrospective study of children with DILV and similar morphologies presenting between 1982 and 2012. The outcomes of initial PAB (n=17) are compared with a Norwood-type reconstruction (n=20). The two groups were similar with respect to age, gender, weight, noncardiac syndromes, and Fontan completion. Mean follow-up was longer in PAB patients (13.3 ± 9.8 years) versus Norwood (4.5 ± 3.0 years, p=0.001). Survival was 75% at eight years in the Norwood group versus 71% in the PAB group (p=0.76). Mortality in the Norwood group was higher before 2002 (p=0.06). The age of patients who underwent a bidirectional Glenn shunt was significantly higher for PAB group (PAB, 9.1 ± 1.4 months vs. Norwood, 6.1 ± 1.6 months; p<0.001). Freedom from any type of reintervention (systemic outflow obstruction or coarctation) was similar (Norwood, 83% vs. PAB, 71%; p=0.62). Freedom from heart block with a pacemaker insertion was significantly better for Norwood patients (Norwood, 89% vs. PAB, 41%; p=0.001). The Norwood-type reconstruction provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining an unobstructed systemic outflow tract and avoiding the risk of heart block. Survival does not differ depending on the type of procedure. Patients with PAB show comparable satisfactory early and long-term results, with an increasing reoperation risk and heart block remaining a major concern.

Invited Commentary

The long-term outcome of single ventricle physiology remains pretty dismal without surgical interventions. The current management ultimately favors toward the path of the Fontan operation, and these patients could be managed with relatively good palliation. However, the management between the times of clinical diagnosis and the optimal achievement of the Fontan operation remains much more debatable. It is well documented that the second-stage bidirectional cavopulmonary shunt has optimized the preparation for the ultimate Fontan operation. The goal of the interim surgical therapy for single ventricle is to establish unobstructed systemic circulation and competent atrioventricular and semilunar valves that serve to preserve the systolic and diastolic ventricular function crucial for the Fontan physiology. Equally important is the avoidance of excessive pulmonary blood flow and obstructive vasculature that may compromise the long-term Fontan outcome. Although many alternative surgical procedures have been described, the two main contenders are the early Norwood or Damus-Kaye-Stansel procedure vs pulmonary artery banding (PAB), particularly in the presence of concomitant systemic obstruction, depending on the comfort and results of various procedures between different centers [1Tchervenkov C.I. Shum-Tim D. Béland M.J. Jutras L. Platt R. Single ventricle with systemic obstruction in early life: comparison of initial pulmonary artery banding versus the Norwood operation.Eur J Cardiothorac Surg. 2001; 19: 671-677Crossref PubMed Scopus (30) Google Scholar]. Traditional PAB has the advantage of avoiding a complex operation, cardiopulmonary bypass, and systemic-pulmonary shunt early during the neonatal period, but it has been related to increased reintervention and poor preparation for the Fontan candidate due to the presence of systemic obstruction, as suggested by the group 1 patients in this study [2Kajihara N. Asou T. Takeda Y. et al.Pulmonary artery banding for functionally single ventricles: impact of tighter banding in staged Fontan era.Ann Thorac Surg. 2010; 89: 174-180Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar]. This may arguably compromise the Fontan operation if not addressed aggressively. Over the decades, the experience of many pediatric centers and surgical intensive care teams has gained comfort and good results with neonatal and infant complex operations. Although some are more comfortable with the Norwood type of operation, others are more comfortable with neonatal PAB and bidirectional cavopulmonary shunt early in infancy. Kajihara and colleagues [2Kajihara N. Asou T. Takeda Y. et al.Pulmonary artery banding for functionally single ventricles: impact of tighter banding in staged Fontan era.Ann Thorac Surg. 2010; 89: 174-180Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar] demonstrated this point very well and were able to gain excellent results with the later approach. Subsequently, with better preservation of the requirement for the optimal Fontan as described, they demonstrated that their early aggressive tighter PAB strategy could prevent volume overload, and if followed with right heart bypass early enough, may prevent the occurrence of systemic obstruction. However, one caution with their article is that the two groups of patients were managed in two different eras of pediatric cardiac surgery and may not be extrapolated in other centers that do not have the same expertise and philosophic approach to the same cardiac anomalies. Pulmonary Artery Banding for Functionally Single Ventricles: Impact of Tighter Banding in Staged Fontan EraThe Annals of Thoracic SurgeryVol. 89Issue 1PreviewIn this study, we assessed our surgical strategy, tighter pulmonary artery banding (PAB) during the neonatal period, as an initial step followed by early application of bidirectional cavopulmonary shunts (BCPS) in infancy, to treat functionally single ventricles with unobstructed pulmonary blood flow. Full-Text PDF

Left Ventricular Retraining and Anatomic Correction in Teenage Patient With Congenitally Corrected Transposition of the Great Arteries

Left ventricular (LV) retraining followed by anatomical repair would be a superior alternative in patients with congenitally corrected transposition (ccTGA) having a deconditioned morphologically left ventricle (MLV); however, LV retraining in older children is a challenging task. A retraining process of the MLV in a teenage patient with ccTGA is reported here. Cardiac catheterization at 7 years of age revealed low pressure of the MLV (33/4 mm Hg) and a LV to right ventricular pressure ratio (LVp/RVp ratio) of 0.32. The first pulmonary artery banding (PAB) was performed at 10 years of age. Although the LVp/RVp ratio reached 0.68, there was no evidence of adequate LV hypertrophy. The second PAB was performed 2 years after the initial PAB, resulting in an increase in the LVp/RVp ratio to 0.93 and an adequate LV hypertrophy. The double switch procedure was successfully performed at 13 years of age. Although the ejection fraction of the MLV mildly decreased, the patient has been doing well during a follow-up period of 4 years. The MLV in the teenage patient with ccTGA was successfully trained using a retraining strategy and has sustained systemic circulation after anatomical repair.

Successful pregnancy after an arterial switch procedure for complete transposition of the great arteries

later expanded the populationthat could benefit from this by introducing a two-stage vari-ant comprising initial pulmonary artery banding to conditionthe left ventricle. The first early survivors of this arterialswitch procedure are now reaching child- bearing age andwe report the first patient undergoing a successful (live birth)pregnancy.The patient, born in 1974, had complete TGA and aventricular septal defect. After atrial balloon septostomy andpulmonary artery banding in 1975, she underwent an arterialswitch procedure according to Jatene in 1978. During theoperation, the coronary arteries and part of their surround-ing sinus Valsalva (aortic) wall tissue were excised and re-implanted in the proximal part of the pulmonary artery.The proximal pulmonary artery was then connected to thedistal part of the transected ascending aorta. The ventricularseptal defect was closed with a Dacron graft and the atrialseptostomy with a direct suture. Sinus Valsalva tissue utilisedfor coronary artery re-implantation was replaced by twopatches of pericardium. A 16-mm Dacron conduit was placedbetween the distal pulmonary artery and proximal aorta tocomplete the anatomical repair. The pulmonary artery con-duit was replaced due to stenosis in 1985 and 1990, on thelatter occasion with a pulmonary homograft.In 1995, she conceived. Assessment before this had shownthat her exercise tolerance was good (NYHA functionalclass I) even though she smoked more than 20 cigarettesper day and continued to do so throughout the pregnancy.Pre-conceptional physical examination showed an averageheart rate of 75 bpm, blood pressure of 115/70 mmHg. Therewere no signs of (congestive) heart failure. Her last recordedelectrocardiogram prior to pregnancy demonstrated a normalsinus rhythm with a complete right bundle branch block(QRS 160), but without signs of myocardial ischaemia. Dur-ing the first and second trimester, she experienced no com-plaints and exercise tolerance was comparable to otherpregnant women. She used no medication. Physical examina-tions [e.g. blood pressure (105/65 mmHg) and heart rate (77bpm)] stayed well within acceptable limits. No changes in theperformed 12 lead electrocardiograms were reportedthroughout the pregnancy. All three transthoracic echocar-diograms (before, at 28 weeks and after pregnancy) showedminor regurgitation of the pulmonary homograft and a minoraortic valve stenosis (25 mmHg). No changes in ventricularand valvular function were detected. During pregnancy, theaortic sinus diameter, however, increased by 5–33 mm at 28weeks of gestation, which returned back to the baseline valueof 27 mm within 1.5 years after delivery. No cardiac compli-cations occurred during the patient’s gestation.At 30 weeks of gestation, the patient was admitted to hos-pital to assess mild vaginal bleeding for which no cause wasdetected. Fetal ultrasound revealed intrauterine growthrestriction but no structural fetal defects. Close monitoringof fetal growth was performed and at 38 + 5 weeks labour wasinduced to guarantee fetal wellbeing. Fetal distress developedduring labour and she was delivered by caesarean sectionunder general anaesthesia with endocarditis prophylaxis. Thebaby was male, small for his gestational age (1930 g, <2.5th

Atrioventricular Canal Defects.

Atrioventricular (AV) canal defects should be diagnosed by means of echocardiography. Infants with complete AV canal defects should undergo intracardiac surgical repair before pulmonary vascular obstructive disease develops, preferably before 6 months of age. Repair of partial AV canal defects or primum atrial septal defects can be postponed until 18 to 36 months of age if the patient does not have pulmonary hypertension or growth failure. The one- and two-patch techniques can both provide excellent results with a low mortality rate in repair of complete AV canal defects. Repair of the cleft mitral valve at the time of initial surgery is advised to preserve long-term mitral valve function. Tetralogy of Fallot with AV canal defect can also be repaired but at an older age and with a greater incidence of reoperation. Unbalanced AV canal defects associated with a hypoplastic ventricle may necessitate initial pulmonary artery banding and a subsequent decision about the suitability of two-ventricle repair. If ventricular septation is unsuitable, a single-ventricle path is chosen. Bidi-rectional cavopulmonary anastomosis and, eventually, the Fontan procedure are used.

Staged surgical approach to neonates with aortic obstruction and single-ventricle physiology

Background. The surgical management of neonatal systemic outflow obstruction and complex single ventricle pathology is variable. Methods. In 15 neonates (12 boys and 3 girls) with complex forms of single-ventricle pathology and aortic coarctation or interruption, an initial strategy of banding the pulmonary artery and repair of the obstruction from a left thoracotomy was undertaken. Results. The median age at operation was 6 days (range 2 to 33 days) and the median weight was 3.3 kg (range 2 to 4.6 kg). There were no early deaths and one late death after the initial surgical palliation. Of the 14 survivors, 8 have undergone a bidirectional cavopulmonary anastomosis. The median age for bidirectional Glenn was 9.75 months (range 3.5 to 26 months). Seven infants have required Damus-Kaye-Stansel reconstruction for subaortic obstruction (one early death). The median age of the Damus-Kaye-Stansel procedure was 4 months (range 3 weeks to 9 months). Thirteen of 15 patients (87%) are alive and 6 have proceeded to a Fontan operation (median follow-up 68 months). A single failing Fontan required takedown to bidirectional Glenn and central shunt. Conclusions. Our experience suggests that this high-risk subgroup of neonates with aortic obstruction and single-ventricle pathophysiology is safely managed by initial pulmonary artery banding palliation and repair of aortic obstruction. This strategy, careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later bidirectional Glenn and Fontan procedures.

Systemic obstruction in univentricular hearts: Surgical options for neonates

The surgical management for bridging patients with univentricular heart and systemic obstruction to a Fontan procedure remains controversial. Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood flow referred for surgical palliation were seen with systemic obstruction. Twenty-six were neonates with coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 other patients, subaortic stenosis developed after initial pulmonary artery banding. Four different palliative procedures were performed: coarctation repair with pulmonary artery banding (group I, n = 15); Norwood or Damus-Kaye-Stansel or arterial switch operation (group II, n = 9); coarctation repair with pulmonary artery banding and bulboventricular foramen enlargement (group III, n = 2); and orthotopic heart transplantation with coarctation repair (group IV, n = 1). The mortality rate was 34.3% (n = 12) for all patients, 53.3% in group I, 33.3% in group II (p = 0.003 versus group I), and 50% in group III. Nine patients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel operation in 5, arterial switch operation in 3, and bulboventricular foramen enlargement in 1. Three had a concomitant or subsequent Fontan procedure and 2, a bidirectional Glenn procedure. In group II, 1 patient underwent a subsequent Fontan procedure and another, a bidirectional Glenn anastomosis. Six of the 8 patients with subaortic stenosis after initial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enlargement (n = 2), or creation of an aortopulmonary window (n = 1). Three had a concomitant Fontan procedure and 2, a bidirectional Glenn procedure. Actuarial 4-year survival was 65.5% +/- 8.4% (70% confidence limits) for all patients; it was 40% +/- 13.3% in group I and 66.6% +/- 16.3% in group II (p < 0.05). Initial management of patients with univentricular heart and systemic obstruction by Norwood-like procedures provides a better outcome. Success of the Fontan operation relies on the ability to provide timely relief of subaortic stenosis.

Surgical aortico-pulmonary anastomosis for a complex aortic obstruction with single ventricle

Management of the neonate and infant with single ventricle and obstruction to systemic blood flow remains controversial and carries significant risk. Options include a neonatal pulmonary artery banding procedure or a pulmonary artery (PA)-to-aortic anastomosis and systemic-to-pulmonary artery shunt. A series of 162 patients with tricuspid atresia or other forms of single ventricle presented between 1985 and 1991, and in 71, there was unrestricted pulmonary blood flow. Of these, 41 had initial pulmonary artery banding with 3 deaths, whereas 5 of 29 patients undergoing PA-to-aortic anastomosis died early. However, when the patients were analyzed by whether aortic arch obstruction was also present, 66.7% ( 4 6 ) of patients managed with PA banding plus arch repair eventually died, whereas only 40% ( 10 25 ) died at some point after PA-aortic anastomosis plus arch repair. Continued experience and follow-up are necessary to improve the outcome of patients with single ventricle and systemic blood flow obstruction.

Complete repair of atrioventricular septal defect

We report our experience with 103 consecutive children who underwent repair of complete atrioventricular septal defect between 1971 and 1990. Ninety-one patients were less than 18 months old (mean age, 6.2 months; mean weight, 5.8 kg) and were repaired using deep hypothermia and circulatory arrest. There were 15 perioperative deaths. Twelve patients were older (mean age, 40.2 months; mean weight, 18.9 kg) and were repaired using moderate hypothermia and cardiopulmonary bypass. There were two perioperative deaths. Repairs were performed with the single-patch technique. Four younger patients required repeat repair to control residual mitral regurgitation. Two of the older children required late reoperation to replace one or both atrioventricular valves. Three younger children underwent pulmonary artery banding initially; 1 died after complete repair. Three older children underwent initial pulmonary artery banding; 2 died at definitive repair, and the survivor required pulmonary artery reconstruction, which was repeated subsequently. Since 1977 our policy has been to perform primary definitive repair whenever possible. Two patients died late from unrelated causes. At the most recent follow-up the majority of patients had no or minimal symptoms. We continue to advocate primary definitive repair whenever possible using the single-patch technique in symptomatic patients with complete atrioventricular septal defect.