Checklists and safety in pediatric cardiac surgery.

In rebuilding Tulane's pediatric heart center after Hurricane Katrina, the use of checklists proved to be essential, not only in rebuilding inventory and systems, but the culture of continued debriefing around their use was seminal in establishing a culture of safety and trust between caregivers; safety that ultimately benefitted our patients.

Morphology of the patent ductus arteriosus does not preclude successful patent ductus arteriosus stent implantation in high-risk patients undergoing hybrid stage I palliation: recommendations to optimize ductal stent positioning.

OBJECTIVE: To describe patent ductus arteriosus (PDA) stenting regardless of ductal morphology in high risk patients with hypoplast physiology undergoing hybrid stage I palliation (PDA stenting and placement of bilateral pulmonary artery bands). BACKGROUND: Hybrid palliation is an accepted alternative for patients with hypoplastic left heart syndrome. Patients weighing less than 2.5 kg, history of prematurity, intracranial hemorrhage, and chromosomal abnormality belong to a high-risk group who otherwise might not be ideal candidates for traditional surgical repair (Norwood Operation). METHODS: Between May 2005 and February 2013, a series of 13 high-risk patients with hypoplast physiology with varying types of ductal morphology underwent PDA stenting as part of hybrid stage I palliation. RESULTS: Three major types of ductal morphology were identified: (1) short and semi horizontal, (2) long and semi horizontal, and (3) tortuous. All patients underwent successful PDA stenting. One patient developed proximal coarctation from inadequate coverage of the pulmonary end of the PDA and was successfully treated with a balloon expandable stent 69 days after the initial procedure. CONCLUSIONS: Mid- to long-term follow-up indicates that PDA stents remain widely patent regardless of ductal morphology until comprehensive stage II repair.

Right pulmonary artery thrombosis after hybrid stage 1 palliation: resolution after direct tissue plasminogen activator infusion.

A patient with unbalanced right ventricular dominant atrioventricular septal defect with ascending and transverse arch hypoplasia underwent hybrid stage 1 palliation. On postoperative day 7, he experienced irritability and acute cyanosis. Echocardiography demonstrated a thrombus occluding the right pulmonary artery band. Emergency cardiac catheterization confirmed thrombosis of the right pulmonary artery at the site of the band. Direct infusion of tissue plasminogen activator successfully lysed the thrombus.

Improvement of cardiopulmonary function after minimally invasive surgical repair of pectus excavatum (Nuss procedure) in children.

BACKGROUND: Severe pectus excavatum in children may result in cardiorespiratory functional impairment; therefore, we evaluated cardiopulmonary response to exercise before and after the Nuss procedure. METHODS: Twenty-four physically active pediatric patients aged 9-18 years with severe pectus excavatum (Haller index >3.25) were included in the study. Cardiopulmonary exercise testing using treadmill and modified Bruce protocol was performed before and after the Nuss procedure. RESULTS: Maximal oxygen uptake and oxygen pulse improved by 40.6% (32 ± 13-45 ± 10 ml/kg/min; P = 0.0001) and 44.4% (9 ± 4-13 ± 5 ml/beat; P = 0.03), respectively, after surgical correction of pectus excavatum by Nuss procedure. Significant improvement in maximum voluntary ventilation and minute ventilation after Nuss procedure was also noted. CONCLUSIONS: We found that, after repair of pectus excavatum by Nuss procedure, the exercise capacity as measured by maximal oxygen consumption improved significantly primarily due to increase in oxygen pulse, an indirect measurement of stroke volume.

Jugular venous valved conduit (Contegra) matches allograft performance in infant truncus arteriosus repair.

OBJECTIVE: Limited availability and durability of allograft conduits require that alternatives be considered. We compared bovine jugular venous valved (JVV) and allograft conduit performance in 107 infants who survived truncus arteriosus repair. METHODS: Children were prospectively recruited between 2003 and 2007 from 17 institutions. The median z-score for JVV (n=27, all 12 mm) was +2.1 (range +1.2 to +3.2) and allograft (n=80, 9-15mm) was +1.7 (range -0.4 to +3.6). Propensity-adjusted comparison of conduit survival was undertaken using parametric risk-hazard analysis and competing risks techniques. All available echocardiograms (n=745) were used to model deterioration of conduit function in regression equations adjusted for repeated measures. RESULTS: Overall conduit survival was 64+/-9% at 3 years. Conduit replacement was for conduit stenosis (n=16) and/or pulmonary artery stenosis (n=18) or regurgitation (n=1). The propensity-adjusted 3-year freedom from replacement for in-conduit stenosis was 96+/-4% for JVV and 69+/-8% for allograft (p=0.05). The risk of intervention or replacement for branch pulmonary artery stenosis was similar for JVV and allograft. Smaller conduit z-score predicted poor conduit performance (p<0.01) with best outcome between +1 and +3. Although JVV conduits were a uniform diameter, their z-score more consistently matched this ideal. JVV exhibited a non-significant trend towards slower progression of conduit regurgitation and peak right ventricular outflow tract (RVOT) gradient. In addition, catheter intervention was more successful at slowing subsequent gradient progression in children with JVV versus those with allograft (p<0.01). CONCLUSIONS: JVV does match allograft performance and may be advantageous. It is an appropriate first choice for repair of truncus arteriosus, and perhaps other small infants requiring RVOT reconstruction.

Cardiac complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the cardiac system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases.The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a near-exhaustive list of cardiac complications, including intraoperative complications and cardiopulmonary bypass-related complications. These cardiac complications are presented in the following subgroups: 1) Cardiac (general), 2) Cardiac--Metabolic, 3) Cardiac--Residual and Recurrent cardiac lesions, 4) Arrhythmia, 5) Cardiopulmonary bypass and mechanical circulatory support, and 6) Operative/Procedural. Within each subgroup, complications are presented in alphabetical order. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.

Transcatheter ablation of supraventricular tachycardia and closure of a large secundum ASD in a Jehovah's Witness.

This report describes successful radiofrequency ablation of typical atrioventricular (AV) node reentrant tachycardiafollowed by transcatheter closure of a large secundum atrial septal defect in a patient of the Jehovah's Witness faith. Both procedures were performed successfully during the same catheterization, without complication or need for blood transfusion.

Central sympathetic blockade ameliorates brain death-induced cardiotoxicity and associated changes in myocardial gene expression.

OBJECTIVE: Brain death results in cardiac injury and hemodynamic instability. After brain death, catecholamine levels surge in concert with increased expression of select myocardial gene products. Sympathetic blockade was used to investigate the effects of the adrenergic nervous system on myocardial gene expression in a rabbit model of brain death. METHODS: A balloon expansion model of brain death in rabbits (n = 42) was used with and without sympathetic blockade (xylazine, acetylpromazine, and ketamine). Sham-operated and naive rabbits served as control animals. Over 4 hours, mean arterial pressure, heart rate, electrocardiographic results, catecholamine levels, myocardial histology, and messenger RNA levels were assessed. RESULTS: Sympathetic blockade decreased basal catecholamine levels and blocked the catecholamine surge accompanying brain death. The typical hemodynamic instability, adverse electrocardiographic changes, and myocellular injury associated with brain death were all significantly decreased. Sympathetic blockade not only suppressed the previously reported increases in myocardial gene expression (cardiac and skeletal alpha-actin, egr-1, and heat shock protein 70) but also suppressed the expression of multiple other genes (alpha and beta myosin heavy chain, calcium ATPase [sarcoplasmic reticulum Ca(2+)-adenosine triphosphatase pump, SERCA-2a], phospholamban [ryanodine receptor], and c-jun). CONCLUSION: Central sympathetic blockade minimizes the hemodynamic instability associated with brain death and neutralizes the increased expression of multiple myocardial gene products associated with brain death.

Catheter closure of autologous pericardial extracardiac Fontan fenestration.

We describe two patients who required fenestration of an autologous pericardial extracardiac Fontan (APEF), short-medium term angiographic follow-up, and subsequent successful transcatheter closure of the fenestration.

Routine mechanical ventricular assist following the Norwood procedure--improved neurologic outcome and excellent hospital survival.

BACKGROUND: Although excellent survival following the Norwood procedure for palliation of hypoplastic left heart syndrome (HLHS) is being achieved by some, most centers, especially the ones with small surgical volume and limited experience, continue to struggle with initial results. Survivors often showed evidence of significant neurologic injury. The early postoperative care is labor-intensive as attempts are made to balance the systemic and pulmonary circulation for these infants. We report our experience with routine use of mechanical circulatory assist to support the increased cardiac output requirements present following Norwood procedure. METHODS: Eighteen consecutive infants undergoing Norwood operation for HLHS (Oregon Health & Science University [OHSU] 13; University of Louisville [UL] 5) were placed on a ventricular assist device (VAD) immediately following modified ultrafiltration in the operating room using the cardiopulmonary bypass (CPB) cannulas that were in the right atrium and the neoaorta. VAD flows were maintained at approximately 200 mL x kg(-1) x min(-1) and the patients were transported to the intensive care unit (ICU). Patients operated at OHSU also received neurodevelopmental testing before their Glenn procedure, approximately 4 to 6 months following their Norwood operation. RESULTS: All patients were stable on VAD support and no attempt was made to balance the systemic and pulmonary circulation. The ventilator was manipulated to achieve systemic Pa0(2) between 30 and 45 mm Hg and PaC0(2) between 35 and 45 mm Hg. Evidence of hypoperfusion (increasing lactates) was managed by increasing the VAD flow. Lactates normalized [< 2 mmol/L]) by 1.8 +/- 1.1 days following surgery. Average time of VAD support was 3.1 +/- 1.0 (range, 2 to 5 days) and average time until chest closure was 3.4 +/- 1.5 (range, 2 to 8 days). There were two cases of postoperative bleeding (11.1%) requiring reexploration and one case of mediastinitis (5.5%) in a patient who has now gone on to successful Glenn. Sixteen of the eighteen patients survived (hospital survival mean 89% with a 95% confidence interval of 63.9% to 98.1%; 12/13 OHSU [92.3%]; 4/5 UL [80%]). Neurodevelopmental testing using the Mullen Scales of Early Learning and the Vineland Adaptive Behavior Scale were normal for all infants tested. CONCLUSIONS: Routine postoperative use of VAD can support the increased cardiac output demands of infants following Norwood operation and results in a stable postoperative convalescence that does not require aggressive ventilator or inotrope manipulation. Although not a panacea, this strategy can simplify postoperative management, lead to excellent hospital survival, and possibly augment cerebral oxygen delivery, resulting in improved neurologic outcomes for this challenging group of patients.

Late superior vena cava perforation and aortic laceration after stenting to treat superior vena cava syndrome secondary to fibrosing mediastinitis.

We describe a case of late perforation of the superior vena cava and laceration of the ascending aorta after stent implantation for superior vena cava syndrome. The etiology of the late perforation is unclear, and could be secondary to either flaring of the trailing edge of the stent or chest trauma.

Left atrial thrombus formation on a CardioSeal Septal Occlusion device in a patient with elevated factor VIII: resolution with medical therapy.

We describe a case of thrombus formation on the left atrial disc of a CardioSeal Septal Occlusion device 14 days after transcatheter closure of a patent foramen ovale in a patient with a history of multiple cryptogenic strokes. Work-up to rule out the presence of a hypercoagulable state prior to device closure demonstrated an elevated factor VIII level. Medical therapy with heparin and aspirin was initiated, with subsequent clot resolution.

Contraction of the Amplatzer Vascular Plug I and II in pulmonary artery and systemic venous collateral vessels is safe and of no hemodynamic or vascular consequence in short- and mid-term follow-up.

BACKGROUND: The Amplatzer Vascular Plug (AVP) I and AVP II have been used successfully to occlude moderate-large venous collateral vessels (VC) and pulmonary arteriovenous malformations (PAVM) in children and adults with congenital heart disease. PURPOSE: To report our experience in 4 patients who underwent device occlusion of systemic venous collaterals (3 patients) and device closure of pulmonary arteriovenous malformation (1 patient) that developed contraction of the AVP. METHODS: The records of all patients who underwent device occlusion utilizing both AVP I and AVP II between November 2006 and January 2011 were retrospectively reviewed. All available follow-up chest x-rays were reviewed and compared with angiograms obtained post device occlusion. A device 30%-50% larger than the targeted vessel was utilized to occlude the vessel. RESULTS: Four patients were identified with a mean age of 21 years (range, 7 years and 2 months-52 years) and mean weight of 60.6 kg (range, 15.3-131.5 kg). Two patients received AVP I and 2 patients received AVP II. One patient who received the 12 mm AVP I showed moderate contraction. The 3 other patients who received AVP I (6 mm) and AVP II (10 mm, 12 mm) all demonstrated device contraction to the original shape of the device. Mean follow-up time of 24 months (range, 12-40 months) has shown no evidence of hemodynamic or vascular compromise. CONCLUSIONS: Short to mid-term follow-up indicate that contraction of AVP I and AVP II is safe with no evidence of hemodynamic or vascular compromise. Continued long-term follow-up is warranted.

Ventricular septal avulsion and ventricular septal defect after blunt trauma.

Traumatic ventricular septal defect is an uncommon complication of blunt chest trauma. We report a case of ventricular septal avulsion, associated with traumatic ventricular septal defect and the associated management concerns.

Ross and Yasui operations for complex biventricular repair in infants with critical left ventricular outflow tract obstruction.

OBJECTIVE: To define the outcomes following Ross and Yasui procedures for complex biventricular repair of critical left ventricular outflow tract obstruction (LVOTO). METHODS: Of 1217 neonates presenting with critical LVOTO enrolled in the Congenital Heart Surgeons Society studies (1994-2008), 52 underwent the Ross or Yasui procedure and their outcomes were investigated using univariate and multivariable parametric models. RESULTS: ROSS (N=39): The Ross procedure (median age 87 days) was rarely the primary intervention (5/39, 13%). A significant number of cases were performed to treat iatrogenic aortic regurgitation after other previous interventions (25/39, 64%). Co-existing functional morphological defects were also common: 72% had preoperative evidence of mitral dysfunction, moderate-to-severe left ventricular dysfunction or endocardial fibroelastosis. Emergency iatrogenic aortic regurgitation (P=0.005) and co-existing abnormalities (mitral stenosis, P=0.02; mitral regurgitation, P=0.05; LV dysfunction, P=0.03) were strong determinants of death. Severe postoperative ventricular dysfunction or need for extracorporeal membrane oxygenation (ECMO) conferred negligible survival. Younger age was associated with disproportionately worse late outcome (5-year survival 44+/-10% for neonates vs 76+/-8% for age >3 months, P=0.0013). However, mitral and left ventricular dysfunction and emergency presentation were significantly more common in the younger age groups. Infants less than 3 months of age without co-existing abnormalities had acceptable late survival ( approximately 75+/-20%). YASUI (N=13): Yasui repair (median age 22 days) was usually the primary intervention (nine of 13) but occasionally followed Norwood palliation (four of 13). None was an emergency. All had a ventricular septal defect. Survival was 69+/-13% at 10 years, which is not significantly different from other biventricular repair strategies in neonates. Aortic atresia was associated with better survival than stenosis (90+/-12% vs 30+/-14% at 3 years, P=0.039). None reverted to univentricular physiology later. CONCLUSIONS: Case selection is key for complex biventricular repair and the importance of appropriate case selection is exaggerated at young ages. All available options should be considered before pursuing the Ross operation in the presence of co-existing functional morphological abnormalities or emergent iatrogenic aortic regurgitation. However, both the Ross and Yasui operations in children (including neonates and young infants) with favourable functional morphology offer good survival, at least matching that of other biventricular repair strategies.

Persistent risk of subsequent procedures and mortality in patients after interrupted aortic arch repair: a Congenital Heart Surgeons' Society study.

OBJECTIVE: Multiple subsequent procedures directed at the arch and/or the left ventricular outflow tract are frequently required after interrupted aortic arch repair. We the investigated patterns and factors associated with these subsequent procedures and mortality. METHODS: We reviewed the data from 447 patients with interrupted aortic arch at 33 institutions enrolled from 1987 to 1997. We classified the subsequent procedures by type (catheter-based or surgical) and focus (arch, left ventricular outflow tract, and "other" cardiovascular lesions). We used competing risks and modulated renewal analysis to explore subsequent procedures. RESULTS: There were 158 subsequent arch and 100 left ventricular outflow tract procedures. Freedom from death at 21 years was 60% overall. The risk of additional subsequent arch procedures decreased after the first subsequent arch procedure in the acute phase, but did not significantly change in the chronic phase. The risk of additional subsequent left ventricular outflow tract procedures increased after the first subsequent left ventricular outflow tract procedure in the chronic phase. The risk factors for subsequent arch procedures and mortality, but not for subsequent outflow track procedures, were related in a complex way to previous procedures and their timing. CONCLUSIONS: Interrupted aortic arch is a chronic disease in which patients often undergo multiple subsequent procedures with persistent risk for additional intervention and mortality. The risk factors are related to the nature and timing of previous procedures and to the morphology and details of the index procedure. Interrupted aortic arch should be considered a chronic disorder.

Introducing the "teamlet": initiating a primary care innovation at san francisco general hospital.

CONTEXT: The 15-minute office visit to primary care clinicians cannot meet the health care needs of patients. Innovation is needed to address this limitation, but practice redesign is challenging in clinical settings. OBJECTIVE: Here we describe the implementation of a practice innovation, the teamlet model, in a San Francisco safety-net clinic. The teamlet consists of a clinician and "health coach" who expand the traditional medical visit into previsit, visit, postvisit, and between-visit care. DESIGN: Teamlet implementation is occurring in phases. Phase 1 is evaluated using plan-do-study-act improvement cycles and interviews with a few patients, clinicians, and coaches. Phase 2 is evaluated using a pre- and postevent questionnaire, focused interviews, and focus groups with patients, faculty, clinicians, and coaches. MAIN OUTCOME MEASURES: Phase 1: Plan-do-study-act cycles generate ideas to improve implementation. Phase 2 evaluation will query demographics, satisfaction, knowledge of self-management support, access, teamwork, and benefits/challenges of the teamlet model. Future research would measure objective clinical outcomes. RESULTS: Phase 1 of the teamlet project led to useful adaptations, with anecdotal evidence that patients and clinicians were satisfied overall with practice improvements. Logistic problems made implementation of the innovation challenging. Phase 2 is currently underway, with results expected in 2008. CONCLUSIONS: Primary care innovation requires multiple perspectives and constant revision. Traditional randomized controlled trials and quantitative evaluation designs are not appropriate for assessing practice-improvement pilot projects because projects must change and develop in their early stages. Despite numerous challenges, the teamlet practice redesign has the potential for improving on the traditional 15-minute physician's office visit.

A novel bioresorbable film reduces postoperative adhesions after infant cardiac surgery.

BACKGROUND: Adhesions encountered in reoperative cardiac surgery can prolong operating time and increase risk. This study was designed to evaluate the ability of a novel bioresorbable barrier film to reduce adhesions in infants. METHODS: A comparative, evaluator-masked, randomized, multicenter study design was used. Before chest closure, infants undergoing initial sternotomy for eventual staged palliative cardiac operations were randomized to barrier film placement (n = 54) or control (no treatment, n = 49) at 15 centers. At repeat sternotomy 2 to 13 months later, the extent and severity of adhesions at the investigational surgical site (ISS) were assessed. A four-grade adhesion severity scoring system was standardized as follows: none, mild (filmy, noncohesive, requiring blunt dissection), moderate (filmy, noncohesive, requiring sharp and blunt dissection), and severe (dense, cohesive, requiring extensive sharp dissection). RESULTS: There were significantly fewer patients with any severe adhesions (29.6% vs 71.4%, p < 0.0001), and a significantly lower percentage of the ISS had severe adhesion involvement (21.1 +/- 36.9% vs 49.5 +/- 42.7%, p = 0.0005) in the barrier group compared with the control group at the second sternotomy. Delayed chest closure (p = 0.0101), Norwood procedure (p = 0.0449), and cardiopulmonary bypass (p = 0.0001) were univariate risk factors for more severe adhesions. Multivariate analysis revealed only control group to be a significant risk factor for more severe adhesions (p = 0.003). There were no statistically significant differences in adverse events between the groups. No adverse events were definitely attributed to the study device. CONCLUSIONS: Use of a novel bioresorbable film was safe and effective in reducing the extent and severity of postoperative adhesions in infants undergoing repeat median sternotomy.