Limited 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).
SOCIETY OF CRITICAL CARE MEDICINE 32ND CRITICAL CARE CONGRESS SAN ANTONIO, TEXAS, USA JANUARY 28-FEBRUARY 2, 2003: ORAL/SANDWICH PRESENTATIONS: Poster Presentation: Basic Science: Sepsis Signal Transduction: PDF Only
Central MessageA multidisciplinary planned approach is critical to successfully manage hypoplastic left heart syndrome with an intact atrial septum.See Article page 51. A multidisciplinary planned approach is critical to successfully manage hypoplastic left heart syndrome with an intact atrial septum. See Article page 51. Over the past few decades, major advances in the form of surgical technique, catheter-based interventions, and critical care management have resulted in improved outcomes with hypoplastic left heart syndrome (HLHS) palliation. Despite this progress, the subset of HLHS that presents with a highly restrictive atrial septum (RAS) or intact atrial septum (IAS) continues to present a challenge. Both an IAS and RAS will produce left atrial and pulmonary venous hypertension. This is thought to lead to abnormal lung development, with reduction in lung perfusion characterized by congenital pulmonary lymphangiectasis and muscularization of pulmonary veins. This has been noted to occur as early as 23 weeks of gestation with histologic changes such as hypercapillarization and widened inter-airspace mesenchyme noted at fetal autopsy.1Goltz D. Lunkenheimer J.M. Abedini M. Herberg U. Berg C. Gembruch U. et al.Left ventricular obstruction with restrictive inter-atrial communication leads to retardation in fetal lung maturation.Prenat Diagn. 2015; 35: 463-470Crossref PubMed Scopus (15) Google Scholar Whether these changes are reversible with left atrial decompression, either prenatally by fetal intervention or postnatally, is unknown. Currently, the 1-year survival in the HLHS population with RAS/IAS is low, with survival between 35% and 50% reported in recent case series.2Gellis L. Drogosz M. Lu M. Sleeper L.A. Cheng H. Allan C. et al.Echocardiographic predictors of neonatal illness severity in fetuses with critical left heart obstruction with intact or restrictive atrial septum.Prenat Diagn. 2018; 38: 788-794Crossref Scopus (6) Google Scholar,3Tanem J. Rudd N. Rauscher J. Scott A. Frommelt M.A. Hill G.D. Survival after Norwood procedure in high-risk patients.Ann Thorac Surg. 2020; 109: 828-833Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar In this issue of the JTCVS Open, Sood and colleagues4Sood V. Zampi J.D. Romano J.C. Hypoplastic left heart syndrome with an intact atrial septum.J Thorac Cardiovasc Surg Open. 2020; 1: 51-56Scopus (4) Google Scholar present their institutional strategy for the management of HLHS/IAS. Their multidisciplinary approach involves cesarean delivery, airway and line establishment, and transfer to the cardiac catheterization suite within 30 minutes of delivery. Sternotomy and per-atrial balloon septostomy or atrial septal stenting is performed, followed by bilateral pulmonary artery bands and, in the majority of cases, a ductal stent (HS1P). The authors are to be congratulated on organizing a large group of experts and developing a reproducible approach to this critically ill, high-risk group of neonates. The management plan emphasizes, importantly, efficiency with times of 30 minutes from delivery to the catheterization suite and 5 minutes to per-atrial access. The outcomes on 15 neonates with HLHS/IAS briefly presented are notable, with 10 being discharged home after HS1P and 7 undergoing successful Norwood. Although the authors present a comprehensive plan, there are a few particulars that would be helpful in providing the complete picture. With regard to the atrial septal intervention, details regarding time to adequate septostomy, failure rate, and indication for surgical conversion would be helpful in understanding the limitations of this strategy. In addition, information about the reasons for mortality and failing to progress to the next stage may provide insight into the challenges these neonates may face secondary to pulmonary venous disease or unfavorable anatomy. We suspect that these specifics will be clarified in a future publication reporting on the outcomes of their strategy. In closing, we are in full agreement with the authors in the need for institutions to have a multidisciplinary strategy to optimize the outcomes of high-risk neonates with HLHS/IAS. Our strategy has been to have elective cesarean delivery within the women's center inside the children's hospital. The newborn is resuscitated, prostaglandins are initiated, and the patient is transported immediately to the cardiac operating room, where the surgical team is standing by. Cardiac anesthesia intubates and places venous and arterial lines expeditiously. A surgical atrial septectomy is done followed by bilateral pulmonary banding. We favor tight bilateral pulmonary artery banding (1.8-2.2 mm), using supplemental oxygen if needed, as even mild pulmonary overcirculation is poorly tolerated in these patients.5Barker G.M. Forbess J.M. Guleserian K.J. Nugent A.W. Optimization of preoperative status in hypoplastic left heart syndrome with intact atrial septum by left atrial decompression and bilateral pulmonary artery bands.Pediatr Cardiol. 2014; 35: 479-484Crossref PubMed Scopus (12) Google Scholar,6He D. Sinha P. Jonas R.A. Pulmonary overcirculation following left atrial decompression in hypoplastic left heart syndrome with intact/restrictive atrial septum.J Card Surg. 2012; 27: 758-759Crossref Scopus (3) Google Scholar We have kept these patients on prostaglandins for ductal patency and delayed the Norwood operation for at least 4 to 6 weeks to achieve a period of lung recovery. This period is geared toward nutrition, lowering respiratory support, and optimizing pulmonary function with diuresis and dual pulmonary hypertensive medications. This strategy has been used in 4 neonates with HLHS/IAS, with all undergoing Norwood followed by bidirectional cavopulmonary shunt (1 mortality after Glenn). How these high-risk babies will fare long term remains to be seen, but developing an institutional plan is undoubtedly the first step toward success. Hypoplastic left heart syndrome with an intact atrial septumJTCVS OpenVol. 1PreviewFeature Editor's Note—Newborns with hypoplastic left ventricle and intact atrial septum are among the most challenging subgroups of patients. Despite the fact that most patients are now diagnosed antenatally by fetal echocardiography, the mortality in these patients remains high and the long-term prognosis is dismal. Herein, Dr Romano and colleagues present a team-based hybrid approach developed by the University of Michigan multidisciplinary group. One can only hope that this admirable, labor-intense collaborative effort by fetal and interventional cardiologists, pediatric cardiothoracic surgeons, pediatric intensivists, and palliative care and social work specialists will overcome the severity of the underlying condition and improve the outcomes of these patients. Full-Text PDF Open Access
Cardiac surgery employing cardiopulmonary bypass exposes infants to a high risk of morbidity and mortality. The objective of this study was to assess the utility of clinical and laboratory variables to predict the development of low cardiac output syndrome, a frequent complication following cardiac surgery in infants. We performed a prospective observational study in the pediatric cardiovascular ICU in an academic children’s hospital. Thirty-one patients with congenital heart disease were included. Serum levels of nucleosomes and a panel of 20 cytokines were measured at six time points in the perioperative period. Cardiopulmonary bypass patients were characterized by increased levels of interleukin-10, -6, and -1α upon admission to the ICU compared to non-bypass cardiac patients. Patients developing low cardiac output syndrome endured longer aortic cross-clamp time and required greater inotropic support at 12 h postoperatively compared to bypass patients not developing the condition. Higher preoperative interleukin-10 levels and 24 h postoperative interleukin-8 levels were associated with low cardiac output syndrome. Receiver operating characteristic curve analysis demonstrated a moderate capability of aortic cross-clamp duration to predict low cardiac output syndrome but not IL-8. In conclusion, low cardiac output syndrome was best predicted in our patient population by the surgical metric of aortic cross-clamp duration.
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.
Narrowing of the retroaortic space after repair of common arterial trunk (CAT) with interrupted aortic arch (IAA) is a well-known issue. We present a newborn with CAT, IAA, and functionally univentricular heart (tricuspid atresia) who underwent repair of CAT, IAA, and left ventricle-to-pulmonary artery (LV-to-PA) conduit placement with the Lecompte maneuver. The patient suffered from left bronchus compression postoperatively, which was relieved by tracheobronchial release.
