S. Adil Husain

Total Anomalous Pulmonary Venous Connection: Factors Associated With Mortality and Recurrent Pulmonary Venous Obstruction

BACKGROUND Surgical repair of total anomalous pulmonary venous connection (TAPVC) is associated with high rates of mortality and need for reintervention. The purpose of this study was to identify variables associated with surgical mortality and, in particular, to define predictors of recurrent pulmonary venous obstruction. METHODS All patients who underwent surgical repair for TAPVC from 2005 to 2010 at a single institution were included in our analysis. Hospital course, operative data, and outpatient records were reviewed. RESULTS Fifty-one patients were available for review and all were included in the analysis. Anatomic TAPVC subtypes included supracardiac 26 (51%), intracardiac 10 (19.6%), infracardiac 9 (17.6%), and mixed 6 (11.8%). Pulmonary venous obstruction was present at initial operation in 13 (25.5%) patients. Median age at repair was 18 days and median weight was 3.6 kg. Single-ventricle physiology was present in 9 (17.6%), with a diagnosis of heterotaxy syndrome in 7 (13.7%). There were 5 (9.8%) operative and 2 late deaths. Recurrent pulmonary venous obstruction requiring reintervention was found in 8 (15.7%) patients with median time to reintervention of 220 days. Obstructed TAPVC was found to be associated with surgical mortality (p=0.01). Cardiopulmonary bypass (p=0.02) and aortic cross-clamp times (p=0.03) were found to be associated with increased risk for reintervention. Intraoperative transesophageal echocardiography findings of a mean confluence gradient 2 mm Hg or greater was found to be markedly associated with recurrent pulmonary venous obstruction requiring reintervention (p≤0.001). CONCLUSIONS Mortality after repair of TAPVC is highest in patients presenting with obstruction at time of repair. Longer cardiopulmonary bypass and cross-clamp times are associated with recurrent pulmonary venous obstruction requiring reintervention. The strongest association with need for reintervention was in patients with intraoperative transesophageal echocardiography Doppler evidence of pulmonary venous obstruction.

Anterior pericardial tracheoplasty for long-segment tracheal stenosis: Long-term outcomes

OBJECTIVE Although several techniques have been described for the treatment of tracheal stenosis, including slide tracheoplasty, tracheal autograft, rib grafting, and use of a pericardial patch, the optimal repair remains controversial because of a lack of long-term follow-up data. The purpose of this study is to examine the long-term results of anterior pericardial tracheoplasty. METHODS To assess the long-term outcomes of patients who underwent repair of tracheal stenosis with anterior pericardial tracheoplasty, we reviewed the case histories of 26 consecutive patients (1984-present). All but 5 had long-segment tracheal stenosis with more than 10 complete tracheal rings. Twenty-one had significant cardiac disease, and 10 had their cardiac lesions repaired at the time of their tracheoplasty. The median age was 6 months (range, 2 days-25 years). All patients underwent anterior pericardial tracheoplasty through a median sternotomy during normothermic cardiopulmonary bypass. We have previously described our tracheoplasty technique. An average of 14 tracheal rings (range, 5-22) was divided anteriorly, and a patch of fresh autologous pericardium was used to enlarge the trachea to 1.5 times the predicted diameter for age and weight. RESULTS There were 3 hospital deaths (at 1, 2, and 7 months, respectively) and 2 late deaths (at 2 and 13 years postoperatively, respectively). No deaths were related to airway obstruction. Two survivors required tracheostomy postoperatively, one after formation of granulation tissue and stenosis and the other after failure to wean from mechanical ventilation. All survivors remain asymptomatic, with minimal to no evidence of airway obstruction. Median follow-up is 11 years (range, 3 months-22 years). CONCLUSION Anterior pericardial tracheoplasty for tracheal stenosis provides excellent results in the majority of patients at long-term follow-up.

Sternal Wound Infections in Pediatric Congenital Cardiac Surgery: A Survey of Incidence and Preventative Practice

BACKGROUND Guidelines exist for prevention of sternal wound infections (SWI) in adults. There are no guidelines for pediatric patients and limited reports on SWI incidence. The purpose of this study was to determine the incidence of, and preventative practice regarding pediatric SWIs with a long-term aim to develop best practice guidelines. METHODS Eighty-nine congenital heart programs were sent a 31 question on-line survey regarding pediatric SWI. RESULTS Thirty eight (43%) of the 89 programs responded. They reported 8,774 pediatric congenital procedures with a mean SWI rate of 1.53% (range, 0 to 9.09). Mean yearly volume was 237 operations (range, 50 to 720). Neither program size nor delayed sternal closure was associated with increased incidence of SWI. Variations in preoperative measures, antibiotic regimens, and wound care did not statistically impact incidence of SWI. Programs with protocols to monitor and control blood glucose levels postoperatively had statistically lower infection rates (1.04 vs 2.35, p = 0.03), and those that sent mediastinal cultures at time of delayed sternal closure reported lower infection rates (1.34 vs 1.74, p = 0.051). CONCLUSIONS This report provides a multiinstitutional SWI incidence from pediatric programs of 1.53%. Despite variations in clinical practice between programs, this survey revealed two strategies resulting in reduced SWIs; protocol-based management of glucose levels and mediastinal wound cultures sent at time of closure. Pediatric programs do not consistently follow adult preventative guidelines. Multicenter randomized research is needed to formulate preventative guidelines to reduce the incidence of pediatric SWI.

Prevention of sternal wound infection in pediatric cardiac surgery: a protocolized approach.

Background: Sternal wound infections (SWIs) are a costly complication for children after cardiac surgery, increasing morbidity, mortality, and financial cost. There are no pediatric guidelines to reduce the incidence of SWI in this vulnerable population. Methods: A quality improvement, multidisciplinary team was formed, and a protocol to prevent SWI was developed. A prospective review of patients who underwent pediatric cardiac surgery was conducted over a two-year period to follow adherence to the protocol and incidence of SWI. The Centers for Disease Control definitions for surgical site infections were used to determine the depth and presence of infection. Results: Three hundred and eight children <18 years of age had sternotomies during the study period. There was a reduction in all SWI between the first and second years of the study (odds ratio [OR] = 0.35; confidence interval [CI] 95% 0.12-1.01; P = .059). Delayed sternal closure (DSC) was associated with increased risk of SWI (OR = 5.4; CI 95% 2.13-14.9; P ≤ .001). Institution of a protocol in patients with DSC was associated with decreased infections during the second year (first year: n = 7 (14%), second year: n = 2 (4%), P = .14). Conclusions: Institution of a protocol was associated with a decreased number of infections in children. A multicenter study of a bundled protocol approach to SWI prevention is needed. Children with DSC had a significantly higher risk of developing a wound infection. Initiating strategies to reduce SWI with a focus on children with DSC may result in improved overall infection rates.

Congenital Heart Operations Performed in the First Year of Life: Does Geographic Variation Exist?

BACKGROUND Geographic variations associated with surgical intervention for congenital heart disease are ill defined. This study uses a large clinical registry to assess frequency of surgical intervention for various infant congenital heart diseases overall and across US geographic regions. METHODS Patients younger than 1 year of age in the Society of Thoracic Surgeons Congenital Heart Surgery Database (January 2010 through June 2012) were included. Index operations were classified on the basis of seven major diagnostic groups and 10 specific diagnoses and were compared across geographic regions using a χ(2) test. Region was defined by patient residence. RESULTS The study included 23,379 patients (94 centers). Septal defects (26.2%) were the most frequently reported diagnostic group, and tetralogy of Fallot (10.6%) was the most frequent specific diagnosis. Significant geographic variation was noted for all seven major diagnostic groups. The proportion of patients undergoing surgery for septal defects varied from 23.9% to 30.2% (p = 0.001); pulmonary venous anomalies, 2.8% to 4.5% (p = 0.03); right heart lesions, 15.7% to 21.4% (p < 0.0001); left heart lesions, 22.7% to 30.4% (p = 0.0002); single-ventricle lesions, 7.3% to 11.4% (p < 0.0001); transposition of the great arteries and double-outlet right ventricle, 9.0% to 15.3% (p < 0.0001); and coronary artery anomalies, 0.4% to 1.4% (p = 0.04). Significant regional variation was also observed for 7 of the 10 specific diagnoses examined. CONCLUSIONS These data demonstrate significant variation in congenital heart disease diagnostic groups requiring surgery before 1 year of age across US geographic regions.

Cardiac inflammatory myofibroblastic tumor: a comprehensive review of the literature.

Although inflammatory myofibroblastic tumors (IMTs) can be found in virtually every major organ, cardiac origin is rare. After recently providing care to a child who presented with a significant myocardial infarction, interest in this rare tumor was piqued. We describe a comprehensive review of cardiac IMT, including information on nomenclature, epidemiology, clinical features, pathogenesis, gross/histological features, immunohistochemical profile, diagnosis, treatment, and prognosis. Fifty-seven cases were identified in the literature. Interestingly, our case represents the seventh case of coronary artery involvement reported. Moreover, it was found that an initial presentation of sudden death most commonly involves the coronary arteries.

Ventricular assist device implantation in the pediatric population: does pump size selection and associated hemodynamics impact outcomes?

The use of pediatric ventricular assist devices (VADs) continues to evolve with the availability of smaller blood pumps. We examine the correlation of implanting appropriate sized blood pumps with a lower incidence of VAD related complications (VADRC). A 7-year retrospective review was undertaken for all pediatric VAD patients. Optimal VAD hemodynamics were defined as cardiac index of 2.7 L/m2 and rate of 80 beats per minute (bpm) with complete fill/empty of the blood pump. Patient/blood pump size match, VAD rate and fill/empty ratios were calculated (optimum = 1.0) and then correlated with incidence of VADRC. The study included 22 patients, mean age 9.77 years (6 mo–18 yrs) and mean body surface area (BSA) of 1.14 m2 (0.14 m2–2.32 m2), who underwent VAD implantation. VADRC included death while on support (n = 5), bleeding requiring reoperation (n = 8), hemolysis (n = 2), neurologic events (n = 2), thrombus formation (n = 3), and infection (n = 3). Six patients were bridged to transplant without any VADRC. This subset of patients had a mean blood pump size match ratio of 0.98, VAD rate ratio of 0.92 and fill/empty ratio of 1.00. Patients with VADRC (n = 16) were found to have a mean blood pump size match ratio of 0.72, VAD rate ratio of 0.72 and fill/empty ratio of 0.78. We report a series of pediatric patients with wide ranging BSA receiving VAD implantation. Selection of appropriate sized blood pumps can be correlated with decreased VADRC.

Multicenter Quality Improvement Project to Prevent Sternal Wound Infections in Pediatric Cardiac Surgery Patients

Background: Children undergoing cardiac surgery are at risk for sternal wound infections (SWIs) leading to increased morbidity and mortality. Single-center quality improvement (QI) initiatives have demonstrated decreased infection rates utilizing a bundled approach. This multicenter project was designed to assess the efficacy of a protocolized approach to decrease SWI. Methods: Pediatric cardiac programs joined a collaborative effort to prevent SWI. Programs implemented the protocol, collected compliance data, and provided data points from local clinical registries using Society of Thoracic Surgery Congenital Heart Surgery Database harvest-compliant software or from other registries. Results: Nine programs prospectively collected compliance data on 4,198 children. Days between infections were extended from 68.2 days (range: 25-82) to 130 days (range: 43-412). Protocol compliance increased from 76.7% (first quarter) to 91.3% (final quarter). Ninety (1.9%) children developed an SWI preprotocol and 64 (1.5%) postprotocol, P = .18. The 657 (15%) delayed sternal closure patients had a 5% infection rate with 18 (5.7%) in year 1 and 14 (4.3%) in year 2 P = .43. Delayed sternal closure patients demonstrated a trend toward increased risk for SWI of 1.046 for each day the sternum remained open, P = .067. Children who received appropriately timed preop antibiotics developed less infections than those who did not, 1.9% versus 4.1%, P = .007. Conclusion: A multicenter QI project to reduce pediatric SWIs demonstrated an extension of days between infections and a decrease in SWIs. Patients who received preop antibiotics on time had lower SWI rates than those who did not.

Novel Annular and Subvalvular Enlargement in Congenital Mitral Valve Replacement

Reparative procedures are not always feasible in congenitally abnormal mitral valves. Mechanical prosthesis has been accepted as the choice for valve replacement in the pediatric population. This report describes a case of congenital mitral valve disease requiring mitral valve replacement. The infants mitral valve annulus was not amenable to placement of the smallest available mechanical prosthesis. The approach used here for annular and subvalvular enlargement facilitated implantation of a larger prosthesis for congenital mitral valve replacement. Five-year outcomes in a single patient may indicate broader applicability and avoidance of patient-prosthesis mismatch.

Surgical cardiac strangulation: Should we worry?

Carerras and associates from the University of British Columbia present an insightful article regarding cardiac strangulation (CS) after placement of epicardial pacing leads within the pediatric cardiac surgical population. The group defines CS as a product of somatic growth in pediatric patients for whom epicardial leads become adherent to the heart in a circumferential manner and lead to a compressive process, which can affect myocardial perfusion with the potential of arrest and death. The article serves several purposes as a knowledge gap exists with only 8 case reportswithin the literature describing this unusual phenomenon. As a result, it creates a platform for the authors to raise awareness regarding this complication, and in doing so, allows for congenital heart surgeons not only to reevaluate implantation techniques and strategies but also to begin the process of devising an accepted follow-up algorithm for patients after placement of epicardial pacing leads. The author group identifies 2 specific goals. First, the group attempts to provide the reader with a true incidence of CS with an overall belief that it is underreported. Second, the group suggests a clinical pathway to aid in the diagnosis and follow-up of patients at risk for CS. The authors directly contacted Medtronic Inc (Minneapolis, Minn) on May 25, 2013, to learn that there have been 100,900 epicardial leads implanted per this single manufacturer in the United States over a 30-year period (October 1981 to August 2012). With only 8 case reports in the literature, an incorrect assumption could be made that the incidence of CS is extremely low (0.016%). This is a common inaccuracy in our ability to best quantify complication incidences of certain surgical interventions for which our only data come from case reports. This group was concerned because of their personal experience with CS and a perception that the overall incidence was indeed higher. In particular, the group noted 2 cases of CS at their institution within a 2-year period with 1 patient being