Bilateral Pulmonary Artery Banding Research Articles

A case of truncus arteriosus with severe heart failure and pulmonary stenosis: bridge to transplant candidacy with surgical correction and a ventricular-assist device.

Ventricular-assist device therapy for small patients with congenital heart disease is challenging due to its complex anatomy and hemodynamics. We describe a 3-year-old patient with heart failure with truncus arteriosus in the palliative stage. The patient underwent palliative right ventricular outflow tract reconstruction following bilateral pulmonary artery banding. At 6months of age, the patient developed severe truncal valve regurgitation and left ventricular dysfunction. Emergent truncal valve replacement with a mechanical valve was performed, but left ventricular dysfunction persisted. At 3years of age, the patient developed acute progression of heart failure triggered by influenza infection. The patient was intubated and transferred to our center to determine the indication for heart transplantation. On the second day after admission, signs of multiorgan failure appeared. Emergent ventricular-assist device implantation for both ventricles was performed with truncal valve closure, ventricular septal defect closure, atrial septal defect closure, and re-right ventricular outflow tract reconstruction. The right ventricular-assist device was successfully removed on the seventh postoperative day. Due to the small pulmonary arteries, severe pulmonary stenosis persisted after ventricular-assist device implantation, but it gradually improved with multiple pulmonary angioplasties. The patient was registered in the Japanese organ transplant network and is awaiting a donor organ in a stable condition.

Bilateral pulmonary artery banding facilitates the systemic ventricular outflow tract growth for biventricular and univentricular repair candidates of complex arch anomaly.

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: - 4.1 ~ - 1.0) at pre-PAB to -2.8 (range: - 3.6 ~ - 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: - 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches.

Staged biventricular repair in a premature neonate with critical aortic stenosis, severe mitral regurgitation, and fetal hydrops: a case report

BackgroundThe surgical management of critical aortic stenosis, mitral regurgitation, and left ventricular dysfunction is a significant clinical challenge. Whether left ventricular function will recover to support systemic circulation after the relief of aortic stenosis is a concern. In this setting, surgical or balloon aortic valvotomy combined with bilateral pulmonary artery banding and atrial septectomy may allow time for left ventricular adaptation, while the systemic circulation is supported by the right ventricle through the ductus arteriosus. We describe the case of a premature neonate with critical aortic stenosis, severe mitral regurgitation, and fetal hydrops who successfully underwent staged biventricular repair after bilateral pulmonary artery banding, atrial septectomy, balloon aortic valvuloplasty, and stent implantation for ductus arteriosus.Case presentationA 29-year-old female was referred to our hospital at 25 weeks of gestation with fetal echocardiography findings of critical aortic stenosis, severely impaired left ventricular function, severe mitral regurgitation, and restrictive foramen ovale. At 33 weeks of gestational age, the baby was born via cesarean delivery. Prostaglandin E1 infusion was immediately initiated, and the neonate underwent emergecy bilateral pulmonary artery banding and atrial septectomy. On the second day, a balloon aortic valvuloplasty was performed. The neonate underwent stent implantation to open the ductus arteriosus and multiple-balloon aortic valvuloplasty. At 4 months of age, he underwent biventricular repair consisting of surgical aortic valvuloplasty, atrial septal defect closure, bilateral pulmonary artery debanding, and ductus arteriosus ligation. At 1 year of age, he underwent the Ross –Konno procedure. Six years after the operation, the patient’s general condition was stable, and the patient is doing well.ConclusionsStaged biventricular repair was successfully achieved in a premature neonate with fetal hydrops and critical aortic stenosis associated with severe mitral valve regurgitation and left ventricular dysfunction.

Successful ductal stent implantation for initial palliation in two patients with aortic atresia and interrupted aortic arch.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.

Staged Norwood operation for patients with hypoplastic left heart syndrome and cor triatriatum: a report of two cases

Two patients with hypoplastic left heart syndrome (HLHS) concomitant with cor triatriatum underwent the staged Norwood procedure following hybrid palliation. Cor triatriatum was diagnosed after birth in both cases. Case 1 with aortic stenosis and mitral atresia underwent bilateral pulmonary artery banding at 2 days of age. Subsequently, balloon atrial septostomy was conducted at 21 days of age due to hypoxia and increased intra-atrial pressure gradient. At 2 months of age, concomitant Norwood operation, cor triatriatum repair, and tricuspid valve repair were successfully performed. She has suffered from branch pulmonary artery hypoplasia since then; thus, Fontan operation was out of indication. Case 2 with aortic stenosis and mitral stenosis underwent bilateral pulmonary artery banding at 6 days of age. After that, the patient’s hemodynamic condition remained stable. The Norwood operation concomitant with cor triatriatum repair was performed at 2 months of age. However, during the procedure, massive pulmonary edema and hemorrhage occurred right after the initiation of cardiopulmonary bypass. She could not be weaned from cardiopulmonary bypass.

Results of a hybrid approach for high risk term newborn patients with interrupted aortic arch (IAA) with left ventricular outflow tract obstruction.

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated. Nine cases underwent hybrid approach during the study period. The mean age and weight at interventions were 7 days (3-16 days) and 3280 g (2700-4300 g). Six of the patients were diagnosed with type B IAA, 2 with type A, and one with type C. LVOTO was present in 7 patients. The success rate for the procedures was 100%. No patients died during the procedure or within the first 5 days after the procedure or from reasons related to the procedure. The median length of the hospital stay after stent placement was 28 days (22-35 days) for discharged patients. Three patients died in interstage period, and 6 patients underwent total corrective surgery after a median of 7 months (4-10 months). The average LVOT diameter was increased from 3.1 mm to 4.8 mm before total repair surgery. The hybrid approach should be kept in mind for treating high risk newborns with IAA with LVOTO and high-risk newborns who are not suitable for single stage total corrective surgery.

Abstract 17152: Use and Outcomes of the Medical Hybrid Procedure for Stage 1 Palliation in Infants With Hypoplastic Left Heart Syndrome and Variants

Background: Staged palliation of hypoplastic left heart syndrome (HLHS) and variants typically begins with the Norwood operation or the hybrid procedure. Hybrid palliation is used in 13% of patients nationally and is often reserved for high-risk patients. A stented hybrid (SH) consists of bilateral pulmonary artery bands (bPAB) and a ductal stent while a ‘medical hybrid’ (MH) consists of bPAB and prostaglandins. MH use and outcomes have not been well described. Hypothesis: MH is used in higher-risk patients and therefore has lower survival than other stage 1 strategies. Aims: Compare use and outcomes of MH, SH, and surgical stage 1 (SS1) using a multicenter database. Methods: Patients from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) database were categorized by initial intervention: MH, SH, or SS1. Other/unclear procedures were excluded. Results: The study included 2423 patients from 65 centers: 277 (11%) MH, 133 (5%) SH, 2013 (83%) SS1. MH had the lowest birth weight (BW) and gestational age (GA), most non-cardiac anomalies, and most preoperative risk factors (PRF). Most centers performed MH in a minority of cases, though use ranged from 0% to 82% at centers with ≥10 patients. Transplant-free survival at 1 year was 56% for MH, 66% for SH, and 81% for SS1 (p&lt;0.0001). Using multivariable logistic regression, predictors of MH vs SS1 were lower BW (p&lt;0.001), lower GA (p&lt;0.001), genetic syndrome (p=0.010), non-cardiac anomaly (p=0.031), and ≥4/12 PRF (p&lt;0.001). Predictors of MH vs SH were HLHS (p=0.044) and ≥4/12 PRF (p=0.045). Cox proportional hazards regression was used to compare risk-adjusted outcomes, excluding one center that performed &gt;50% SH: MH had higher risk of one-year mortality/transplant compared to SS1 (HR = 1.89, 95% CI 1.47-2.42) and no difference compared to SH (HR = 1.16, 95% CI 0.76-1.79). Conclusions: Survival after MH is similar to SH and worse than SS1. This may be due to patient risk factors not controlled for in this study.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction

BackgroundHybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued. ObjectivesThis study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation. MethodsFrom 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons’ Society’s critical left heart obstruction cohort underwent hybrid palliation across 24 institutions. Multivariable hazard modeling with competing risk methodology was performed to determine risk and factors associated with outcomes of biventricular repair, Fontan procedure, transplantation, or death. ResultsPreoperative comorbidities (eg, prematurity, low birth weight, genetic syndrome) were identified in 70% of infants (150 of 214). Median follow-up was 7 years, ranging up to 17 years. Overall 12-year survival was 55%. At 5 years after hybrid palliation, 9% had biventricular repair, 36% had Fontan procedure, 12% had transplantation, 35% died without surgical endpoints, and 8% were alive without an endpoint. Factors associated with transplantation were absence of ductal stent, older age, absent interatrial communication, smaller aortic root size, larger tricuspid valve area z-score, and larger left ventricular volume. Factors associated with death were low birth weight, concomitant genetic syndrome, cardiopulmonary bypass use during hybrid palliation, moderate to severe tricuspid valve regurgitation, and smaller ascending aortic size. ConclusionsMortality remains high after hybrid palliation for infants with critical left heart obstruction. Nonetheless, hybrid palliation may facilitate biventricular repair for some infants and for others may serve as stabilization for intended functional univentricular palliation or primary transplantation.

Long-term outcomes of hypoplastic left heart syndrome with analysis of the Norwood procedure in infants following bilateral pulmonary artery banding

ObjectiveTo investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure. MethodsThis retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23). Preoperative risk factors, surgical results, survival rates, Fontan candidacy, and long-term complications were compared. ResultsEarly survival rates after the Norwood procedure were 91.3% (21 of 23) in both groups. Late survival rates after the Norwood procedure were similar at the 10-year follow-up (group N, 76.3%; group I, 68.7%; P = .63). Fontan completion rates also were comparable in the 2 groups (group N, 77.8%; group I, 85.7%; P = .67). Group N showed a higher median pulmonary artery (PA) index before bidirectional cavopulmonary connection (group N, 177 [interquartile range (IQR), 147-243] mm2/m2; group I, 152 [IQR, 146-163] mm2/m2; P = .03); this trend continued until 5 years after Fontan completion (P = .01). Group N also had a lower rate of freedom from protein-losing enteropathy (PLE) at 9.0 years after the Fontan operation (90.0% vs 52.5% for group I; P = .04), although the incidences of other Fontan-associated events were not significantly different. ConclusionsFontan candidacy and survival rates were similar regardless of the timing of the Norwood procedure. Early performance of the Norwood procedure may lead to lower rates of late Fontan-associated events, such as PLE.

Bilateral pulmonary artery banding in higher risk neonates with hypoplastic left heart syndrome

ObjectivesLimited data on performing bilateral pulmonary artery banding (BPAB) before stage 1 Norwood procedure suggest that some patients may benefit through the postponement of the major cardiopulmonary bypass procedure. The objective of this study was to evaluate the effectiveness of BPAB in the surgical management of high-risk patients with hypoplastic left heart syndrome (HLHS). MethodsA retrospective review of all high-risk neonates with HLHS who underwent BPAB at our institution was performed. No patients, including those with intact or highly restrictive atrial septum (IAS), were excluded. ResultsBetween October 2015 and April 2021, 49 neonates with HLHS (including 6 with IAS) underwent BPAB, 40 of whom progressed to the Norwood procedure. Risk factors for not progressing to the Norwood procedure after BPAP include low birth weight (P = .043), the presence of multiple extracardiac anomalies (P = .005), and the presence of genetic disorders (P = .028). Operative mortality was 7.5% (3/40). IAS was associated with operative mortality (P = .022). ConclusionsThe strategy of BPAB prestage 1 Norwood procedure was successful in identifying at-risk patients and improving Norwood survival. Although not all patients will need this hybrid approach, a significant number can be expected to benefit from this tactic. These results support the need for a substantial hybrid strategy, in addition to a primary stage 1 Norwood surgical strategy, in the management of HLHS.

Norwood Operation with Right Ventricular-Pulmonary Artery Shunt Versus Comprehensive Stage II After Bilateral Pulmonary Artery Banding Palliation.

As a strategy for the primary Norwood operation, the right ventricular-pulmonary artery shunt is associated with satisfactory early outcome. However, use of this shunt after bilateral pulmonary artery banding remains controversial. This study compared the operative outcomes and late hemodynamics in patients who underwent the Norwood operation, preceded by bilateral pulmonary artery banding, with a right ventricular-pulmonary artery shunt or with bidirectional Glenn anastomosis (comprehensive stage II strategy).We retrospectively reviewed 38 patients who underwent the Norwood operation preceded by bilateral pulmonary artery banding between 2004 and 2017. Of these, 17 underwent the Norwood operation with a right ventricular-pulmonary artery shunt (Group S), whereas 21 underwent the comprehensive stage II strategy (Group G).5 years after the Norwood operation, 10 (60%) and 17 (81%) patients in Group S and Group G, respectively, underwent the Fontan procedure. Group S showed significantly lower pressure in the superior vena cava after bidirectional Glenn anastomosis than Group G (13 ± 2 mmHg vs. 18 ± 3 mmHg; p < 0.01), but pressures were similar after the Fontan procedure. The right ventricular end-diastolic volume at 1 year post-Fontan procedure was significantly higher in Group S than in Group G (142 ± 41% vs. 91 ± 28%; p < 0.01).In terms of early outcomes, the Norwood operation with a right ventricular-pulmonary artery shunt enabled low pressure in the superior vena cava, but in the long term, this shunt adversely influenced the right ventricular volume.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction

ObjectiveTo compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). MethodsFrom 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. ResultsCompared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). ConclusionsInfants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

Norwood operation versus comprehensive stage II after bilateral pulmonary artery banding palliation for infants with critical left heart obstruction

ObjectiveTo determine patient characteristics and outcomes after Norwood versus comprehensive stage II (COMPSII) for infants with critical left heart obstruction who had prior hybrid palliation (bilateral pulmonary artery banding ± ductal stent). MethodsFrom 23 Congenital Heart Surgeons' Society institutions (2005-2020), 138 infants underwent hybrid palliation followed by either Norwood (n = 73, 53%) or COMPSII (n = 65). Baseline characteristics were compared between Norwood and COMPSII groups. Parametric hazard model with competing risk methodology was used to determine risk and factors associated with outcomes of Fontan, transplantation, or death. ResultsInfants who underwent Norwood versus COMPSII had a higher prevalence of prematurity (26% vs 14%, P = .08), lower birth weight (median 2.8 vs 3.2 kg, P < .01) and less frequent ductal stenting (37% vs 99%; P < .01). Norwood was performed at a median age of 44 days and median weight of 3.5 kg, versus COMPSII at 162 days and 6.0 kg (both P < .01). Median follow-up was 6.5 years. At 5 years after Norwood and COMPSII, respectively; 50% versus 68% had Fontan (P = .16), 3% versus 5% had transplantation (P = .70), 40% versus 15% died (P = .10), and 7% versus 11% are alive without transition, respectively. For factors associated with either mortality or Fontan, only preoperative mechanical ventilation occurred more frequently in the Norwood group. ConclusionsHigher prevalence of prematurity, lower birth weight, and other patient-related characteristics in the Norwood versus COMPSII groups may influence differences in outcomes that were not statistically significant for this limited risk-adjusted cohort. The clinical decision regarding Norwood versus COMPSII after initial hybrid palliation remains challenging.

Neurodevelopmental and functional outcome in hypoplastic left heart syndrome after Hybrid procedure as stage I.

Patients with hypoplastic left heart syndrome (HLHS) undergoing staged palliation until Fontan procedure are at risk for impaired neurodevelopmental (ND) outcome. The Hybrid procedure with bilateral pulmonary artery banding, ductal stenting, and balloon atrioseptostomy may offer a less invasive stage I procedure compared to the Norwood stage I procedure avoiding early neonatal cardiopulmonary bypass (CPB) surgery. Despite altered fetal cerebral hemodynamics, the type of stage I procedure may be a covariate influencing ND outcome and functional outcome may also be altered due to postponing neonatal CPB surgery. Within this review, we analyzed ND outcome as well as functional outcome after Hybrid procedure as stage I procedure. The review analyzed original publications (OPs) published before March 15, 2022, identified by Cochrane, EMBASE, OVID, Scopus, and Web of science. An OP was included if short-to-long-term neurodevelopment outcome, brain development, somatic, and cardiac outcome in patients for HLHS and variants treated by Hybrid procedure were analyzed. In addition to database searches, we reviewed all references of the analyzed OP to obtain a comprehensive list of available studies. The author, year of publication, demographic characteristics of study population, study design (prospective or retrospective), study assessment, and main findings were summarized. Twenty-one OPs were included with data of patients with ND outcome and functional cardiac outcome. Overall, there is an impaired mid-term ND outcome in patients with Hybrid procedure as stage I for HLHS. Only slight differences between stage I procedures (Hybrid vs. Norwood) in two comparing studies have been determined affecting right ventricular remodeling, short- and mid-term ND outcome, reduced brain growth until two years of age, sufficient quality of life, and altered hemodynamics influencing brain volumes and cerebral perfusion pattern. Despite some minor differences regarding the mid-term follow-up in patients with HLHS comparing Hybrid vs. Norwood procedure, its impact on ND outcome seems rather low. This may be explained by the large number of covariates as well as the small study populations and the different selection criteria for patients undergoing Hybrid or Norwood procedure as stage I.

Tricuspid atresia and common arterial trunk: a rare form of CHD.

Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant's small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81.

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 &amp; 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, &amp; 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 &lt;50% of patients achieve a Fontan or BVR. Preoperative factors influence the use of this approach and thus are an important influence on outcomes.

Anesthetic management using high-flow nasal cannula therapy during cardiac catheter examination of a neonate with hypoplastic left heart syndrome

BackgroundSedation during cardiac catheter examination in neonates with complex congenital heart disease is challenging, as even the slightest change in the circulatory or respiratory status can lead to hemodynamic collapse. Here, we report a case wherein we achieved adequate sedation with a high-flow nasal cannula (HFNC) for catheter examination in a neonate with a congenital cardiac anomaly.Case presentationAn 11-day-old boy with hypoplastic left heart syndrome was scheduled for a cardiac catheter examination prior to the Norwood procedure. He underwent bilateral pulmonary artery banding (PAB) on day 1 and was receiving dobutamine, milrinone, alprostadil, and dexmedetomidine in addition to air and nitrogen insufflation via HFNC, which was applied following extubation on day 3 and nitrogen therapy on day 6 owing to persistent pulmonary overcirculation symptoms (tachypnea and low arterial blood pressure) despite bilateral PAB. A catheter examination was performed on day 11 with careful monitoring of expired carbon dioxide and observation of chest wall motion. Adequate sedation was provided with supplemental midazolam and fentanyl along with HFNC without tracheal intubation.ConclusionsThe findings from this case suggest that HFNC is a safe and effective tool for oxygenation during cardiac catheter examination under sedation in neonates.

Tailored strategy to match anatomy and physiology with intervention can improve outcomes of symptomatic neonates with Ebstein anomaly

ObjectiveNeonatal presentation of Ebstein anomaly (EA) represents the most severe form of this condition. Despite significant advances, operative mortality remains high and management decisions represent a formidable challenge. We used a strategy aimed to match anatomy and physiology with type and time of intervention to increase survival. MethodsWe performed a review of all patients with fetal or neonatal diagnosis of EA managed at a single center between 2007 and 2020. ResultsAmong 18 patients with EA, 8 underwent neonatal intervention. The most common indication included cyanosis and heart failure (8/8), end organ dysfunction (6/8), and maldistribution of cardiac output (6/8). Only 2/8 had antegrade pulmonary blood flow. Associated conditions included pulmonary regurgitation in 4/8, atrial tachyarrhythmia in 4, and a ventricular septal defect in 3. Three patients underwent initial stabilization with main pulmonary artery occlusion including bilateral pulmonary artery banding in 2. Five patients underwent biventricular repair with conversion to right ventricle exclusion in 2 cases. Three others underwent the Starnes procedure as initially planned. The median age at surgery was 10 days (range, 1-30) and median weight 2.6 kg (range, 1.9-4.0). The median duration of mechanical ventilation and intensive care unit stay were 9 days (range, 5-34) and 30 days (range, 15-100), respectively. Operative mortality was 1/8. At a median follow-up of 130 months (range, 5-146), there were no late deaths, and all survivors remain in functional class I and free of valvular reintervention. ConclusionsSymptomatic neonates with EA can be effectively managed with good outcomes. Preoperative stabilization and choice of management pathway on the basis of anatomy and physiology can help reduce morbidity and mortality.

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.

The Technique of Bilateral Pulmonary Artery Banding in High-Risk Patients With Hypoplastic Left Heart Syndrome

The management of high-risk neonates with hypoplastic left heart syndrome or its variants remains a tremendous surgical and medical challenge for our specialty. The hybrid strategy has been shown to improve outcomes in high-risk patients. This article illustrates the specific technical details of bilateral branch pulmonary artery banding for such high-risk patients.