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

OBJECTIVE: To 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). METHODS: From 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. RESULTS: Infants 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. CONCLUSIONS: Higher 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.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

BACKGROUND: Hybrid 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. OBJECTIVES: This study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation. METHODS: From 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. RESULTS: Preoperative 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. CONCLUSIONS: Mortality 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.

Preliminary Results With a Novel Expanded Polytetrafluoroethylene-based Pulmonary Valved Conduit.

BACKGROUND: A novel polymeric pulmonary valved conduit, resistant to calcification and structural valve deterioration, may provide a more durable therapy option for the pediatric population by preventing loss of right ventricular function and increasing freedom from valve-related reintervention and mortality. METHODS: This was a prospective, multicenter, single-arm study evaluating safety and performance of an investigational novel expanded polytetrafluoroethylene-based valve. Patients met study inclusion/exclusion criteria, had a signed informed consent, had pre- and postoperative evaluation via transthoracic echocardiography, and 6-month cardiac magnetic resonance imaging. RESULTS: Seventeen patients were enrolled from 3 sites. Median age was 12 years (range, 6-17 years) with 52.9% male. Body surface area ranged from 0.82 to 1.57 m2. There has been no mortality and 100% freedom from device related reinterventions. Baseline compared with 6-month cardiac magnetic resonance imaging (in 11 of 16 patients with available data) suggests favorable right ventricular remodeling (right ventricular end-diastolic volume, 123 ± 37 to 94 ± 25 mL/m2) with no significant change in ejection fraction. Through current follow-up, no patient has a right ventricular outflow tract gradient >20 mm Hg (mean, 11.2 ± 4.3 mm Hg). No evidence of worsening valvular insufficiency was observed throughout postoperative serial transthoracic echocardiogram evaluations. No pulmonary regurgitation above baseline (≤ mild) was observed. No patient developed endocarditis. No thrombus or calcification was identified. CONCLUSIONS: This preliminary evaluation of a novel expanded polytetrafluoroethylene-based valved conduit suggests promising valve function with no thromboembolic or infectious complications, no valve related reinterventions, no valve-related adverse events or unexpected findings, improved right ventricular volumes, and encouraging hemodynamic performance through current follow-up.

Necrotizing enterocolitis in neonates undergoing the hybrid approach to complex congenital heart disease.

OBJECTIVE: To investigate the prevalence of necrotizing enterocolitis (NEC) in neonates undergoing the Stage I hybrid procedure for palliation of complex congenital heart disease (CHD). Neonates undergoing the Norwood surgery for hypoplastic left-heart syndrome have the highest risk for NEC of all CHD patients. The hybrid procedure is another palliative option for hypoplastic left-heart syndrome, but NEC in neonates undergoing this procedure has not been reported. DESIGN: Retrospective chart review of 73 neonates who underwent the hybrid procedure for palliation of complex CHD. Demographic, perinatal, perioperative, clinical, and procedural data were collected. NEC was defined as modified Bell's Stage II and above. SETTING: The cardiothoracic and neonatal intensive care units in a large free-standing children's hospital. PATIENTS: All neonates who underwent the hybrid Stage I procedure for the palliation of complex CHD from April 2002 through April 2008. MEASUREMENTS AND MAIN RESULTS: Seventy-three neonates were reviewed and 11.0% (eight of 73) developed NEC. Of the patients with NEC, 37.5% (three of eight) died and two patients required abdominal surgery. Earlier gestational age (< 37 wks), lower maximum dose of prostaglandin infusion, and unexpected readmission to the intensive care unit were statistically associated with NEC (p = .009, 0.02, and 0.04, respectively). No other demographic, perinatal, perioperative, clinical, or procedural variables were associated with the development of NEC in this patient population, including enteral feeding regimens, umbilical artery catheters, inotrope use, and average oxygen saturation and diastolic blood pressure. CONCLUSIONS: The prevalence of NEC in patients undergoing the hybrid procedure is comparable to that reported for neonates undergoing the Norwood procedure. Earlier gestational age is a significant risk factor for NEC in patients who undergo the hybrid Stage I procedure. Multidisciplinary approaches to better understand abdominal complications and to develop feeding regimens in neonates undergoing the hybrid approach to complex CHD are needed to improve outcomes and decrease morbidities.

There's an App for That; Improving Communication during Pediatric Cardiothoracic Surgery.

INTRODUCTION: Waiting while a loved one is in surgery can be a very stressful time. Current processes for updating families vary from institution to institution. Providing timely and relevant updates, while important to the family, may strain a surgical team's operational system. In our initial experience with the Electronic Access for Surgical Events (EASE) application (app), we tested the extent to which its implementation improved communication with patient families. METHODS: We compared compliance data collected pre-EASE (December 2013 through September 2014) and post-EASE implementation (October 2014 until December 2015). RESULTS: Although the pre-EASE compliance rate for bi-hourly updates was 46% (118/255) of cases, post-EASE implementation achieved a compliance rate of 97% (171/176). A 2-sample test of proportions confirmed a significant improvement in compliance after the introduction of EASE technology (P < 0.001). Analysis of the 177 noncompliant cases in the pre-EASE period indicated that noncompliance occurred most frequently at the end of the case (97/177, 55%) when the patient remained in the operating room > 2 hours after the last update to the family. We also observed noncompliance at the beginning of the case (46/177, 26%), when the patient arrived in the operating room > 2 hours before the time of the first update. Family satisfaction scores that rated their experience during surgery as "Very Good" improved from 80% pre-EASE implementation to 97% postimplementation. We sustained this improvement for 1 year. CONCLUSIONS: A mobile technology app (EASE) improved both frequency and compliance with surgical updates to families, which resulted in a statistically significant increase in family satisfaction scores.

Changes in right ventricular function in neonates with hypoplastic left heart syndrome before and after the hybrid procedure.

AIMS: Decreased right ventricular function via deformation analysis has been noted in patients with hypoplastic left heart syndrome (HLHS) after the Norwood procedure. No data exist in HLHS patients undergoing the hybrid procedure. The goal of this study was to evaluate right ventricular functional changes in HLHS patients undergoing the hybrid procedure under steady-state conditions. METHODS AND RESULTS: Echocardiograms were prospectively obtained on patients with HLHS before and after the hybrid procedure. Fractional area change, tricuspid inflow velocities, tissue Doppler imaging (TDI), and deformation analysis were performed. Paired Wilcoxon's signed rank or Student's t-test was used for analysis. P < 0.05 was considered significant. Twenty HLHS patients were studied (10 males:10 females). Median age at the pre-hybrid echocardiogram was 3 (1-16) days, age at hybrid procedure was 5 (3-17) days, and age at post-hybrid echocardiogram was 10 (6-34). There were significant decreases in systolic function as measured by TDI and deformation analysis. There was no significant change in right ventricular fractional area change. Diastolic function was also noted to significantly decrease after the hybrid procedure. CONCLUSION: Systolic and diastolic functions decreased after the hybrid procedure despite the fact that patients avoided cardiopulmonary bypass. These results are comparable with previous reports in HLHS patients undergoing the Norwood procedure. Further studies are needed to determine if these echocardiographic changes have prognostic significance.

Cardiac transplantation after the Fontan or Glenn procedure.

OBJECTIVES: The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure. BACKGROUND: Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods. METHODS: The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors. RESULTS: Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 +/- 8.5 years) with a mean follow-up of 54 +/- 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died

Potential Molecular Mechanism of Retrograde Aortic Arch Stenosis in the Hybrid Approach to Hypoplastic Left Heart Syndrome.

BACKGROUND: The hybrid palliation for hypoplastic left heart syndrome has emerged as an alternative approach to the Norwood procedure. The development of patent ductus arteriosus (PDA) in-stent stenosis can cause retrograde aortic arch stenosis (RAAS), leading to significant morbidity. This study aimed to identify potential mechanisms of PDA in-stent stenosis contributing to RAAS. METHODS: Tissues from stented PDAs were collected from 17 patients undergoing comprehensive stage II repair between 2009 and 2014. Patients requiring RAAS intervention based on cardiology-surgery consensus were defined as RAAS(+) (n = 10), whereas patients without any RAAS intervention were defined as RAAS(-) (n = 7). Tissues were examined by quantitative polymerase chain reaction analysis for vascular smooth muscle cell (VSMC) differentiation and proliferation markers. RESULTS: Patient characteristics were hypoplastic left heart syndrome with aortic atresia in 6 and with aortic stenosis in 3; unbalanced atrioventricular canal in 3; double-inlet left ventricle/transposition of the great arteries in 3; and double-outlet right ventricle in 2. VSMC differentiation markers (ß-actin, SM22, and calponin) and signaling pathways for VSMC modulation (transforming growth factor-ß1, Notch, and platelet derived growth factor-BB) were significantly higher in the RAAS(+) than in RAAS(-) patients. The proliferation marker Ki67 was increased in RAAS(+) patients. Cell cycle markers were comparable in both groups. CONCLUSIONS: Increased VSMC differentiation and proliferation markers suggest a mechanism for inward neointima formation of the PDA in RAAS. The apparent lack of change in cell cycle markers is contrary to coronary artery in-stent stenosis, suggesting further targets should be examined. Combined primary in vitro PDA cell culture and proteomics can be strong tools to elucidate targets to reduce PDA in-stent stenosis for RAAS in the future.

Combined heart and lung transplantation for unresectable primary cardiac sarcoma.

OBJECTIVE: The prognosis for patients with primary cardiac sarcoma is poor. Median survival is less than 10 months, especially when complete surgical excision is not feasible. Removal of all cardiopulmonary structures involved by tumor followed by orthotopic allotransplantation has been proposed to improve long-term survival. METHODS: From 1996 through 1999, we performed combined heart and lung resection followed by en bloc heart and bilateral lung transplantation in 4 patients (2 men and 2 women): 2 with inoperable pulmonary arterial sarcoma and 2 with left atrial sarcoma extending into the pulmonary vein. RESULTS: Median age at diagnosis was 39 years (range 37-45 years). All 4 patients were given chemotherapy before transplantation: doxorubicin and ifosfamide in 2 cases, and doxorubicin, ifosfamide, mesna, and dacarbazine in 2 cases. There were no operative deaths. Median survival after transplantation was 31 months (range 5-49 months). All patients had tumor recurrence: local recurrence in the chest (n = 1) and distant metastases in the brain (n = 2) and abdomen (n = 1). One patient remains alive 49 months after disease progression with cerebral metastasis as the only site of recurrence treated with whole-brain irradiation, resection, and stereotactic radiosurgery. CONCLUSIONS: Combined heart and lung transplantation is a technically feasible treatment for highly selected patients with localized advanced primary cardiac sarcomas. The high incidence of metastatic disease, however, limits its utility.

Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it?

OBJECTIVE: Although the outcome of cardiac surgery in neonates with low birth weight (LBW) has improved, LBW remains a risk factor for surgical palliation. Few surgical series of LBW patients include those with hypoplastic left heart syndrome (HLHS). To identify variables associated with poor outcome in this group, we reviewed our experience with patients with HLHS and LBW who underwent Stage I Norwood palliation. METHODS: Between January 1998 and December 2000, 20 consecutive LBW (<2500 g) neonates with HLHS (n=13) or HLHS variant (n=7) underwent surgical palliation. Retrospective review of all patient data and analysis to identify risk factors was performed. RESULTS: Mean age at surgery was 5.1+/-4.6 days (range 1-17), mean weight was 1.98+/-0.44 kg (range 1.1-2.5), including nine patients under 2 kg. Ten patients were born at <35 weeks gestation. Anatomic diagnosis included HLHS in 13 patients (10 with aortic atresia), unbalanced atrioventricular canal defect in two, double outlet right ventricle in two and other variants in three. Mean ascending aortic size was 4.0+/-1.8 mm (range 1.5-8). Associated cardiac defects were present in three patients, and a genetic syndrome and/or congenital anomaly was present in four of them. Mean circulatory arrest time was 60+/-10 min. Extracorporeal support was used perioperatively in 10 patients. Early mortality was 9/20 (45%). At a mean follow up at 22+/-10 months (range 8-38), six patients underwent stage II, and are awaiting stage III; four patients have completed their Fontan. Anatomic variant, ascending aortic size, prematurity, age at surgery, weight, duration of circulatory arrest, cardiopulmonary bypass time and associated non-cardiac anomalies were not risk factors for poor outcome whereas restrictive pulmonary venous drainage and coronary artery anomalies were associated with decreased survival. CONCLUSION: LBW newborns with HLHS and physiologic variants have an increased early surgical risk but have acceptable intermediate survival rates for subsequent palliation including Fontan. LBW and prematurity should not be contraindications to early surgical palliation.

Anatomy of the aortic and pulmonary roots.

The use of the pulmonary autograft for aortic root replacement has renewed interest in the morphology of the arterial roots. In this article the basic construction of the roots, their anchorage, and their support from surrounding structures are reviewed. The arterial roots manifest a complex anatomy, with an intricate relation between the anulus and its adjacent structures, which span the transition from ventricle to the great vessel. The pulmonary root is anchored over its entire circumference to the thin myocardium of the free-standing pulmonary infundibulum. The anchorage of the aortic root is more extensive, being partly inserted into the thick left ventricular and septal myocardium and partly continuous with fibrous structures such as the membranous septum and the mitral valve. The pulmonary root is supported only by a slight collar of myocardium. The aortic root is better encased, supported by the more pronounced bulging of ventricular myocardium as well as the adjacent atrial myocardium and atrial septum. When the pulmonary autograft is used for aortic root replacement it will obtain maximum support from the surrounding tissues by implanting the autograft as proximal as possible. Copyright 1998 by W.B. Saunders Company

Helium-oxygen mixture to facilitate ventilation in patients with bronchiolitis obliterans syndrome after lung transplantation.

A combination of helium and oxygen (heliox) can facilitate gas exchange and limit peak inspiratory pressures through reduced resistance to gas flow and decreased turbulent flow. The combination of these gases has been used for a variety of upper and lower airway conditions, including patients who were spontaneously breathing, receiving noninvasive ventilation, as well as during mechanical ventilation. To date, there are no reports regarding the use of heliox in patients with bronchiolitis obliterans syndrome following lung transplantation. We report the use of such a combination of gases in 2 patients with bronchiolitis obliterans syndrome following lung transplantation as a supportive measure to facilitate ventilation during the initial treatment course for acute respiratory failure in the ICU. A heliox mixture was administered with noninvasive ventilation and with mechanical ventilation through the ventilator in a heart-lung transplant recipient and a lung transplant recipient, respectively.

Thrombotic risk of recombinant factor seven in pediatric cardiac surgery: a single institution experience.

BACKGROUND: Recombinant activated factor seven (rFVIIa) is increasingly being used as a hemostatic adjunct in pediatric cardiac surgery. We evaluated the thrombotic safety profile of rFVIIa in pediatric congenital heart disease (CHD) surgery. METHODS: This was a retrospective matched case-control study over six years at a single institution. Patients who received rFVIIa after CHD surgery were matched to controls based on age, diagnosis, and procedure. We compared thrombosis, hemorrhage, transfusions, length of stay, and repeat procedures between groups. RESULTS: Twenty-five patients received rFVIIa (mean dose: 70 mcg/kg); 50 controls were matched. There was no significant difference in the rate of thrombosis between patients who received rFVIIa and controls (8% vs 4%). After rFVIIa, there was a significant reduction in transfusion volume (median 77.1 mL/kg vs 14.6 mL/kg; p < 0.001) as well as a significant decrease in hemorrhagic chest tube output (8.3 +/- 1.6 mL/kg/hour vs 1.4 +/- 0.3 mL/kg/hour; mean +/- standard error of the mean; p < 0.001). No difference was seen in intensive care unit or hospital length of stay or mortality between patients receiving rFVIIa and controls. CONCLUSIONS: The rFVIIa therapy did not increase thrombotic complications when used as rescue therapy after CHD surgery but did appear to decrease bleeding complications in this small cohort.

The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome.

BACKGROUND: Before palliative stage 2 for hypoplastic left heart syndrome, the coronary and cerebral circulations are often dependent on retrograde perfusion by means of the aortic arch. Results of hybrid palliation with a focus on patients exhibiting retrograde aortic arch obstruction (RAAO) were analyzed. METHODS: From July 2002 to March 2008 66 consecutive hybrid procedures for hypoplastic left heart syndrome were performed. Patients requiring RAAO intervention based on cardiology-surgery consensus were defined as group 1 (n = 16), whereas all other hypoplastic left heart syndrome patients formed group 2 (n = 50). RESULTS: At birth there were no differences between groups in terms of demographics or cardiac function. Group 1 had more patients with aortic atresia (94% versus 58%; p = 0.01), and 69% of patients had initial echocardiographic comments regarding incipient RAAO versus 26% in group 2 (p = 0.007). The type of ductal stent, balloon versus self-expandable, did not influence the subsequent development of RAAO. Before RAAO intervention (mean age, 74 days), group 1 patients had significantly more tricuspid regurgitation. The main treatment for RAAO in group 1 was coronary stent insertion, with 3 patients having a reverse central shunt. At a mean follow-up of 611 days, group 1 had reduced survival interstage (56.3% versus 88%; p = 0.005) and overall (43.7% versus 70%; p = 0.03). CONCLUSIONS: Clinically important RAAO occurred in 24% of the hypoplastic left heart syndrome patients in this series. If RAAO is detected at birth or early interstage, a Norwood operation is now favored. Palliative interventional catheterization remains very important mid and late interstage for continuing the hybrid strategy toward comprehensive stage 2.

Palliation via hybrid procedure of a 1.4-kg patient with a hypoplastic left heart.

Despite improvements in survival of patients with hypoplastic left heart syndrome (HLHS) with various palliative procedures, certain risk factors, such as weight less than 2.5 kg, continue to predict increased mortality. We report the palliation of a patient with HLHS weighing 1.4 kg via a hybrid procedure consisting of banding of the pulmonary arteries bilaterally, stenting the ductus arteriosus, and balloon atrial septostomy. We propose that this may be another alternative for palliation in this high-risk patient group.