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.

Development of pediatric multidisciplinary extracorporeal membrane oxygenation simulations: A novel educational program to enhance team communication and emergency preparedness.

INTRODUCTION: Pediatric extracorporeal membrane oxygenation (ECMO) is a high risk, low-volume technology. Infrequency of this technology and associated complications may translate to unfamiliarity of identification and management of potentially life-threatening events, which may require knowledge and procedural skills to be performed quickly. Providers involved in managing ECMO must be able to promptly identify and initiate management for such events, particularly when surgical colleagues are not readily available. METHODS: A multidisciplinary ECMO simulation program was implemented in a tertiary children's hospital. Over 18 months, a prospective, observational study was conducted evaluating simulations involving circuit and patient emergencies, teamwork and communication behaviors and technical skills. An on-line survey was sent to participants following sessions to evaluate post-simulation confidence, lessons learned and potential barriers to implementation of necessary skills and behaviors. RESULTS: Ten simulation sessions occurred during implementation. Mean participants per session was 7 (range: 5-11). Eight Pediatric Cardiac Intensive Care Unit attendings, four Advance Practice Nurses, 54 pediatric intensive care unit registered nurses, and 55 pediatric respiratory therapists attended. Tasks with highest self-reported increase in confidence were related to (1) diagnosis (tension pneumothorax, oxygenator failure, and ventricular tachycardia), (2) fluid administration and (3) early and efficient mobilization for ECPR, with less reported confidence increase with technical skills More than 90% of participants provided a task or behavior they would implement if a specific emergency was encountered in real-life following simulation training. Real-life application occurred following simulations with participants reporting direct impact of training on their ability to perform the skill efficiently and correctly. CONCLUSIONS: Implementation of ECMO multidisciplinary simulations provides structured opportunities for the team to learn and practice ECMO skills together in scenarios they may encounter without surgical presence. Ensuring competency of providers through implementation of such a program may improve patient safety through enhanced team communication, knowledge, and hands-on experience.

Aortic Valvuloplasty or Rootplasty for Aortic Regurgitation.

At present, aortic valvuloplasty is considered an effective procedure for treatment of aortic regurgitation in pediatric patients. It has encouraging mid- and long-term results. The improved outcome is primarily related to better understanding of the functional anatomy of the normal valve and the different factors that alter it. It is also related to the realization that outcome after valvuloplasty is dependent on comprehensive repair of all of the involved components of the aortic root. Refinement in preoperative diagnosis has helped identify these abnormal components and focus the surgical approach on the needed reconstruction. Although the technical aspects of valvuloplasty are well defined, suboptimal long-term results still occur in some cases because the patch material used for valve repair can become fibrotic or calcified. This review summarizes the surgical approach to and the management of the different abnormal root components in pediatric patients with significant aortic valve regurgitation.

Surgical Correction of Aberrant Right Coronary Anomalies Stranding an Aortic Commissure with and Without Unroofing.

The technique for successful surgical correction of an anomalous origin of the right coronary artery from the opposite aortic cusp with an aberrant course between the aorta and pulmonary artery is illustrated in a symptomatic 62-year-old woman. The intramural course of the right coronary artery traversed the tip of the commissure between the anterior and posterior leaflets, and its repair entailed unroofing of the intramural segment from inside the aortic intima. This technique required resuspension of the overlying commissure to maintain optimal aortic valve leaflet coaptation and prevent aortic insufficiency. Modifications of this technique have been utilized by us whenever the intramural segment traversed behind the commissure. In these cases, partial or subtotal unroofing of the intramural segment was performed to preserve the integrity of the intima behind the overlying commissure. More recently, we have performed the surgical correction by probing the intramural segment within the aortic wall to its most anterior location and then performing a wide anterior unroofing in the aortic intima, and marsupializing the aortic and coronary intima to avoid dissection or intimal flap development. We favor utilizing these techniques of anatomic correction of the anomalous coronary to other techniques involving coronary artery bypass grafting of the anomalous coronary, especially in adult patients, as unroofing provides more lasting results.

Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Coronary artery anatomy represents a challenging and, often, determining predictor of outcome in an arterial switch operation (ASO). Impact of specific coronary artery variants, such as single, intramural and inverted, on time-related events following ASO, is, yet, to be determined. We sought to compare early and late outcomes within the group of nonstandard coronary artery variants. Patients who underwent ASO from January 1995 to October 2010 were reviewed. Patients with coronary artery variants other than L1Cx1R2 ("standard" by Leiden classification) were included. Patients with single, intramural and inverted coronary artery variants incorporated in group A. All other nonstandard coronary variants incorporated in group B. Demographics, perioperative variables, early and late outcomes were assessed. Of the 123 ASO, 24 patients (19.5%) with nonstandard coronary variant were studied. Thirteen were in group A and 11 in group B. There were two early deaths (1 in group A and 1 in group B) (p > 0.05). There is one death early after hospital discharge (group A). Mean follow-up was 59.4 ± 55.1 months. There was no structural coronary artery failure after hospital discharge following ASO. Freedom from any reintervention at 8 years was (78.3 ± 9.6%) (p 0.55) with no late neo-aortic or mitral valve intervention. ASO with single, intramural or inverted coronary artery course carries no added longitudinal risk for structural or flow impairment within the group of nonstandard coronary artery variants. There is an early hazard period with no late survival attrition. Aortic arch repair as part of staged strategy prior to ASO might influence early and late outcome.

Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery of an Infant.

A female presented 2 weeks after birth with an unbalanced atrioventricular canal, double outlet right ventricle, mild pulmonary stenosis, and patent ductus arteriosus that eventually caused pulmonary over circulation. After pulmonary artery banding, she experienced myocardial ischemia, suggesting interference with coronary blood flow by the band that had been placed on the main pulmonary trunk. The band was removed and revised to bilateral branch pulmonary artery banding, and cardiac function improved. An anomalous left coronary artery from the underside of the right pulmonary artery was identified. Eight weeks later, the patient underwent coronary transfer and reimplantation of the left coronary artery into the aorta followed by main pulmonary artery banding. She subsequently underwent bidirectional Glenn.

Repair of Superior Sinus Venosus Atrial Septal Defect Using a Modified Two-Patch Technique.

BACKGROUND: Surgical repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection is usually performed with either a two-patch technique or the Warden procedure. However, both techniques can lead to superior vena cava or pulmonary vein stenosis, as well as sinus node dysfunction. To prevent these complications, we have utilized a modified two-patch technique. METHODS: Between January 2000 and December 2015, 44 patients underwent modified two-patch repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection at our institution. The technique utilizes the right atrial appendage to patch the superior vena cava and avoid superior vena cava obstruction. Patients' data were collected from reviewing hospital charts and clinic records. RESULTS: Mean age was 10.3 years (range, 0.68 to 61 years). There was no hospital mortality. Six patients (13.6%) had sinus node dysfunction after surgery. All of them recovered their normal sinus node function at a mean follow-up period of 45.7 months. No superior vena cava or pulmonary vein stenosis was observed at long-term follow-up. CONCLUSIONS: A modified two-patch technique is a valid alternative to other methods of superior sinus venosus atrial septal defect and partial anomalous pulmonary venous connection repair, and prevents late systemic/pulmonary venous pathway obstruction while maintaining sinus rhythm.

Efficacy of Nitric Oxide Administration in Attenuating Ischemia/Reperfusion Injury During Neonatal Cardiopulmonary Bypass.

OBJECTIVE: Nitric oxide (NO) plays several protective roles in ischemia/reperfusion (I/R) injury. Neonates undergoing the Norwood procedure are subject to develop I/R injury due to the immaturity of their organs and the potential need to interrupt or decrease systemic flow during surgery. We hypothesized that NO administration during cardiopulmonary bypass (CPB) ameliorates the I/R and could help the postoperative recovery after the Norwood procedure. METHODS: Twenty-four neonates who underwent a Norwood procedure were enrolled in a prospective randomized blinded controlled trial to receive NO (12 patients) or placebo (12 patients) into the oxygenator of the CPB circuit during the Norwood procedure. Markers of I/R injury were collected at baseline (T0), after weaning from CPB before modified ultrafiltration (T1), after modified ultrafiltration (T2), and at 12 hours (T3) and 24 hours (T4) after surgery, and they were compared between both groups, as well as other postoperative clinical variables. RESULTS: There was no difference in age, weight, anatomical diagnosis, CPB, and aortic cross-clamp time between both groups. Troponin levels were lower in the study group at T1 (0.62 ± 58 ng/mL vs 0.87 ± 0.58 ng/mL, P = .31) and became significantly lower at T2 (0.36 ± 0.32 ng/mL vs 0.97 ± 0.48 ng/mL, P = .009).There were no significant differences between both groups for all other markers. Despite a lower troponin level, there was no difference in inotropic scores or ventricular function between both groups. Time to start diuresis, time to sternal closure and extubation, and intensive care unit and hospital stay were not different between both groups. CONCLUSION: Systemic administration of NO during the Norwood procedure has myocardial protective effects (lower Troponin levels) but we observed no effect on postoperative recovery. Larger sample size may be needed to show clinical differences.

Surgical repair of anomalous coronary arteries arising from the opposite sinus of Valsalva in infants and children.

BACKGROUND: Unroofing of anomalous coronary artery originating from the opposite sinus of Valsalva has become the procedure of choice for this congenital lesion, with surgery performed in children as young as two years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication whether surgical repair should be done in this age group. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants. METHODS: Between April 2002 and February 2007, eight patients underwent surgical repair of anomalous coronary artery arising from the opposite sinus of Valsalva and coursing between the aorta and pulmonary artery. Patients' age varied from two months to 28 years with a mean of 11.7 +/- 11.1 years. SURGICAL TECHNIQUE: Surgical repair involved unroofing the intramural segment of the anomalous coronary artery using cardiopulmonary bypass. RESULTS: Two patients were younger than one year (Group A), and six patients were older than one year (group B). The mean intensive care unit stay was 2.5 +/- 0.7 days for Group A and 2.8 +/- 1.9 for Group B. The mean hospital stay was 4 +/- 1.4 days for Group A and 4.3 +/- 2.4 days for Group B. There was no mortality and no complications. The mean follow-up period is 14 +/- 15.7 months with a range of one to 39 months. At the time of the last follow-up, all patients were asymptomatic in New York Heart Association class I and follow-up echocardiography on six of eight patients showed wide open coronary ostium. CONCLUSION: Unroofing the anomalous coronary artery arising from the opposite sinus of valsalva can be done in infants with minimal morbidity and mortality. Longer follow-up is needed to assess long-term results.

Surgical Management of Neonatal Ebstein's Anomaly Associated With Circular Shunt.

Ebstein's anomaly is a rare congenital cardiac malformation that may present in the neonatal period with life-threatening physiologic derangement, especially when it is associated with circular shunt. Urgent surgical intervention is critical for survival because of hemodynamic compromise. We review our experience with surgical management of neonates with Ebstein's anomaly associated with circular shunt and review the literature for similar reported cases. The underlying pathophysiology and different options of treatment are discussed.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.

OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. RESULTS: The initial procedure was pulmonary artery banding in 9 patients, systemic-to-pulmonary shunt in 2 and Damus-Kaye-Stansel/Norwood procedure in 2. Median follow-up was 69 months (interquartile range 25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome ( P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left ( P > 0.05). CONCLUSIONS: A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.

Relationship of Normal Aortic Valve Cusp Dimensions: A Tool to Optimize Cusp Reconstruction Valvuloplasty.

Outcomes of surgical aortic valvuloplasty hinge primarily on optimal leaflet design. Imprecise valve reconstruction can result in insufficiency or stenosis. Predicting postrepair leaflet parameters from pre- or intraoperative readily measurable valve dimensions could result in improved reconstruction. This study analyzes the relationships between different parameters of the normal aortic valve to establish a method of deriving the optimal dimensions of the reconstructed leaflet following valvuloplasty. Morphologic analysis of the normal aortic valve was performed on 50 autopsy specimens. The following parameters were measured: circumference at ventriculoaortic junction, intercommissural circumferential distance (IC), commissural height, leaflet free-margin length (L1-length of the free margin of each leaflet), cusp attachment length (L2-cusp attachment length at the ventriculoaortic junction), and leaflet height (A-ie, the vertical or cephalocaudal distance along the convex surface of the leaflet from the midpoint of the leaflet free margin to the midpoint of the cusp attachment). Univariate linear regression analysis was used to test the interdependence of leaflet dimensions. The leaflet free-margin length L1 correlated strongly with the corresponding IC: r = 0.74, 0.81, and 0.79 for noncoronary, right coronary, and left coronary leaflets, respectively; P = 0.000. Leaflet height A and cusp attachment length L2 also correlated well with the corresponding IC: A-IC, r = 0.56, 0.74, and 0.66; and L2-IC, r = 0.78, 0.85, and 0.80 for noncoronary, right coronary, and left coronary cusps respectively; P = 0.000. Parameters required to construct an aortic valve leaflet can be reliably derived from the IC, which can be easily measured perioperatively. Using this as a basis for leaflet reconstruction might optimize the leaflet graft design process, compensate for pathologic variations in aortic annulus size and commissural height, and could result in better coaptation of the valve leaflets.

Systematic approach to aortic valvuloplasty in children and young adults.

Valve repair in children and young adults is an attractive alternative to replacement or Ross procedure. In the past, the various valvuloplasty techniques have addressed isolated valve pathology. Recently, however, a holistic approach involving repair of all aortic root structures has evolved and promised to be an excellent alternative to valve replacement. It provides good long-term results and avoids all the drawback of prosthetic valve insertion in this age group. Development of ideal patch material needed for valve repair and better understanding of the anatomy and function of the aortic root may further improve the short- and long-term outcomes of aortic valvuloplasty.

Proximal sano anastomosis aneurysm due to degeneration of bovine pericardial patch.

We report a 5-year-old girl with hypoplastic left heart syndrome who originally underwent a Norwood procedure with a Sano shunt. Subsequent procedures included a bidirectional Glenn and extracardiac Fontan with a Gore-Tex graft (W.L Gore and Associates, Flagstaff, AZ). The patient was found to have a heart murmur on a follow-up visit 3 years later. Echocardiography and computed tomography imaging showed an aneurysm of the proximal Sano anastomosis. The patient underwent successful surgical repair.

Selective tricuspidization and aortic cusp extension valvuloplasty: outcome analysis in infants and children.

BACKGROUND: Encouraging early outcomes of emerging aortic cusp extension valvuloplasty techniques have redirected attention to nonreplacement strategies in the management of younger patients with aortic insufficiency or aortic stenosis. Outcome analysis after aortic cusp extension valvuloplasty in infants and children was undertaken. METHODS: From July 1987 to December 2008, 78 patients younger than 10 years of age underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and selective use of tricuspidization. Sixteen (20.5%) patients were younger than 1 year of age. Twenty-seven had bicuspid aortic valve, 34, congenital aortic valve stenosis, and 17, congenital or acquired aortic insufficiency or aortic stenosis. Forty-two patients had balloon valvuloplasty or surgical valvotomy before aortic cusp extension valvuloplasty. Median follow-up was 12.4 years (range, 0.1 to 21.6 years). Freedom from aortic valve replacement (AVR) and determinants of outcome were analyzed. RESULTS: There were no early or late deaths. During the follow-up period, 23 patients (29.5%) had Ross operation and 8 patients (10.2%) had other AVR. The z values of left ventricular end-diastolic dimension, aortic annulus, aortic sinus diameter, sinotubular junction diameter, and left ventricular wall thickness before AVR were 3.8 +/- 2.95, 2.1 +/- 1.15, 4.2 +/- 1.22, 1.78 +/- 1.24, and 2.92 +/- 1.31, respectively. Actuarial freedom from AVR at 1, 5, and 10 years was 97.3 +/- 2.0%, 71.3 +/- 5.8%, and 55.6 +/- 6.9%, respectively. CONCLUSIONS: Aortic cusp extension valvuloplasty with tricuspidization allows left ventricular reverse remodeling with satisfactory long-term durability and freedom from AVR. Used selectively, it represents a reliable and effective approach in infants and children with congenital or acquired abnormal aortic valve.

Aortic cusp extension valvuloplasty with or without tricuspidization in children and adolescents: long-term results and freedom from aortic valve replacement.

OBJECTIVE: Aortic cusp extension valvuloplasty is increasingly used in the management of children and adolescents with aortic stenosis or regurgitation. The durability of this approach and the freedom from valve replacement are not well defined. A study was undertaken to investigate outcomes. METHODS: From July 1987 to November 2008, 142 patients aged less than 19 years underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and tricuspidization (when needed). Three patients with truncus arteriosus and severe truncal valve insufficiency were excluded. From the available follow-up data of 139 patients, 50 had bicuspid aortic valves, 40 had congenital aortic valve stenosis, 41 had combined congenital aortic valve stenosis/insufficiency, and 8 had other diagnoses. Median follow-up was 14.4 years (0.1-21.4). Long-term mortality and freedom from aortic valve replacement were studied. RESULTS: There were no early, intermediate, or late deaths. Z-values of left ventricular end-diastolic dimension, aortic annulus, aortic sinus diameter, and sinotubular junction diameter before aortic valve replacement were 4.2 +/- 3.11, 2.3 +/- 1.25, 4.4 +/- 1.23, and 1.84 +/- 1.28, respectively. During the follow-up period, 64 patients underwent aortic valve reinterventions. The Ross procedure was performed in 32 of 139 patients (23%) undergoing aortic cusp extension valvuloplasty. Other aortic valve replacements were undertaken after 16 aortic cusp extension valvuloplasties (11.5%). Freedom from a second aortic cusp extension valvuloplasty or aortic valve replacement at 18 years was 82.1% +/- 4.2% and 60.0% +/- 7.2%, respectively. CONCLUSION: Aortic cusp extension valvuloplasty is a safe and effective surgical option with excellent survival and good long-term outcomes in children and adolescents. The procedure provides acceptable durability and satisfactory freedom from aortic valve replacement.

Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation.

BACKGROUND: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. METHODS: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. RESULTS: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). CONCLUSIONS: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.

Alternative approach to the surgical management of congenital tracheal stenosis.

Congenital tracheal stenosis is a serious disease associated with high operative mortality and morbidity, especially in the newborn period. Its surgical management involves either sliding tracheoplasty, which is associated with extensive dissection and recurrent stenosis, or with pericardial patch tracheoplasty that fails to address recoiling of the stenotic complete ring and collapse of the patch into the tracheal lumen. We report a modification of the latter technique, in which a half ring external stenting device is used to keep the rings from recoiling.

Severe truncal valve stenosis: diagnosis and management.

Severe truncal valve stenosis, due to dysplastic valve, may be found in a small number of patients with truncus arteriosus. Its presence complicates the surgical management of this anomaly due to lack of optimal therapeutic options and associated ventricular dysfunction. We report a case of truncal root replacement using a homograft for the management of such condition, and review the literature related to its management and outcome.