Comparison of 10-year graft failure rates after induction with basiliximab or anti-thymocyte globulin in pediatric heart transplant recipients-The influence of race.

BACKGROUND/OBJECTIVE: The impact of induction therapy in pediatric heart transplantation has been uncertain. Given the risk of poor outcomes in black pediatric heart transplant recipients, we evaluated the effect on graft survival of ATG and BAS induction in black and non-black pediatric recipients. METHODS: This was a retrospective analysis of pediatric candidates (aged ≤18 years) who underwent heart transplantation from 2000 to 2016 identified from the UNOS database. Primary outcome was 10-year graft survival. RESULTS: This study included 654 patients receiving BAS, 2385 patients receiving ATG, and 2425 receiving no induction. Ten-year survival was similar for the following groups: non-black BAS (57%), non-black ATG (66%), and black ATG (51%). The black BAS group had a 10-year graft survival of 39% which was inferior on pairwise comparison to the other groups (all P values < 0.001). On multivariate analysis, ATG was associated with decreased risk of graft failure when compared to no induction (HR 0.86, 95% CI 0.76-0.97, P = 0.011) and had an association approaching statistical significance when compared to BAS induction (0.84, 0.7-1.01, P = 0.069). This association was seen in black recipients in whom ATG was strongly associated with decreased risk of graft failure when compared to either no induction (0.65, 0.5-0.83, P = 0.001) or BAS (0.64, 0.46-0.89, P = 0.008) but was not seen in non-black recipients. CONCLUSIONS: Black pediatric heart transplant recipients who received ATG induction had an improved long-term graft survival compared to those who received BAS induction or no induction.

Obesity class does not further stratify outcome in overweight and obese pediatric patients after heart transplantation.

The effect of obesity stratification on pediatric heart transplant outcomes is unknown. The UNOS database was queried for patients ≥2-<18 years listed for heart transplant and stratified by BMI: normal (BMI>5%-≤85 percentile), overweight (BMI=86%-95 percentile), class 1 (BMI=100%-120% of 95 percentile), class 2 (BMI=121%-140% of 95 percentile), and class 3 obesity (BMI>140% of 95 percentile). A total of 5056 individuals were listed for transplant, with 71% normal, 13% overweight, 10% class 1, 4% class 2, and 2% class 3 obesity. Waitlist survival was not different between groups. Post-transplant survival was decreased in overweight and combined obese groups vs normal, with no further difference between overweight and obese classes. Overweight and obese patients had higher listing status and were more likely to have ventilator, inotrope, and mechanical circulatory support at listing. After transplant, there was an association of overweight-obese patients with diabetes and rejection requiring hospitalization. Stricter definition of normal weight reveals overweight-obese status was an independent risk factor for poorer post-transplant survival, without further effect by stratification of weight class. However, because there is no difference in waitlist survival, this study does not allow the selection of absolute weight-based criteria regarding transplant listing and suggests the need to look further for modifiable risk factors post-transplant.

Identifiable Risk Factors and Miscalculations During Listing for Pediatric Heart Transplantation.

The objective of this study is to describe identifiable risk factors, complications, and pitfalls while listing pediatric patients for heart transplantation, which is the standard of care for end-stage heart disease in children. Since the introduction of cyclosporine in the 1980s, the management in pediatric heart transplantation has shown consistent improvement, mainly because of technological advances and the integration of multidisciplinary teams in the field. However, the complexity of this patient population makes medical providers vulnerable to complications as a result of undesirable mistakes. Transplant survival is impacted negatively when mistakes from health-care providers compound the high-risk status of the patient. The identification of multiple risk factors and undesirable miscalculations may help transplant teams make decisions before allocating organs, intervene or minimize morbidity, and provide the best quality of life to recipients.

Allosensitization does not alter post-transplant outcomes in pediatric patients bridged to transplant with a ventricular assist device.

Patients supported with a VAD are at increased risk for sensitization. We aimed to determine risk factors for sensitization as well as the impact of sensitization on post-transplant outcomes. The UNOS database (January 2004-June 2014) was used to identify patients (≤18 yrs) supported with a durable VAD. Rates and degree of sensitization in the VAD cohort were calculated. Post-transplant survival was determined comparing outcomes of sensitized vs. non-sensitized patients. There were 3097 patients included in the study; 19% (n = 579) were bridged with a VAD. Of these, 41.8% were sensitized vs. 29.9% of the patients who were not bridged with a VAD (p < 0.001). VAD was an independent predictor of sensitization (OR 2.05 [1.63-2.57]; p < 0.001). There was no difference in sensitization based on device type (continuous vs. pulsatile flow, p = 0.990). Post-transplant survival rates between the sensitized and non-sensitized VAD patients were not different, including patients with a PRA >50% and VAD patients with a positive DSC (p = 0.280 and 0.160, respectively). In conclusion, pediatric VAD patients are more likely to be sensitized, but there was no difference in sensitization based on device type. In addition, sensitization does not appear to impact outcomes.

The Impact of Concomitant Left Ventricular Non-compaction with Congenital Heart Disease on Perioperative Outcomes.

Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculations and inter-trabecular recesses which may occur in association with congenital heart disease (CHD). To date, few studies have been performed to assess whether the concomitant diagnosis of LVNC affects the outcomes of CHD surgery. A retrospective review of patients with LVNC with CHD (LVNC-CHD), 0-5 years of age, was conducted. Patients with CHD without LVNC (CHD-only) and 0-5 years of age with similar diagnosis distribution were selected for comparison. Perioperative data, including CHD diagnosis, operative course, and postoperative complications were collected and compared between groups. LVNC-CHD was diagnosed in 26 children. Of the 26 with LVNC-CHD, 20 underwent surgery and these patients were compared with 276 CHD-only controls. Median total length of stay in the hospital was 12.5 days (IQR 5.5-63 days) in LVNC-CHD compared to 5 days (IQR 3-10 days) in CHD-only (p < 0.005). Postoperative death, cardiac arrest, or need for ECMO or transplantation occurred in 6/20 (30 %) of the LVNC-CHD patients compared to 3/276 (1 %) of the CHD-only group (p < 0.0001). LVNC-CHD patients had significantly longer hospital length of stay and higher perioperative complications compared to CHD-only patients without myocardial abnormalities. Pediatric cardiac care teams should be cognizant of the possibility of the increased perioperative risk associated with concomitant LVNC. Future prospective studies are warranted.

Transthoracic Echocardiography in the Preoperative Assessment of Newborn Coarctation: Limiting Risks Associated with Advanced Imaging Techniques.

A newborn male child with prenatally identified aortic arch hypoplasia presented to our facility for cardiac management. He had been started on prostaglandins at the delivery facility and was subsequently placed on a high-flow nasal cannula due to associated apnea. On the day of life three, the patient underwent cardiac computed tomography scan for delineation of his anatomy. The patient remained intubated after his imaging study in anticipation of surgical intervention, which took place at the age of 5 days. The patient required a peritoneal dialysis catheter placement 2 days after his procedure due to oliguria. He progressed into renal failure requiring continuous renal replacement therapy. This patient was subsequently discussed at our departmental morbidity and mortality conference. The short time frame between contrast administration for the computed tomography and surgical intervention was thought to have contributed to his renal failure. We discussed the adequacy of transverse aortic arch imaging by echocardiogram and the utility of advanced imaging in the fragile neonatal period. This discussion resulted in our department asking, "Is transthoracic echocardiography accurate when diagnosing and characterizing aortic coarctation at our institution? Are advanced imaging studies necessary in instances of simple coarctation?"

Racial disparity exists in the utilization and post-transplant survival benefit of ventricular assist device support in children.

PURPOSE: Children of minority race and ethnicity experience inferior outcomes postheart transplantation (HTx). Studies have associated ventricular assist device (VAD) bridge-to-transplant (BTT) with similar-to-superior post-transplant-survival (PTS) compared to no mechanical circulatory support. It is unclear whether racial and ethnic discrepancies exist in VAD utilization and outcomes. METHODS: The United Network for Organ Sharing (UNOS) database was used to identify 6,121 children (<18 years) listed for HTx between 2006 and 2021: black (B-22% of cohort), Hispanic (H-21%), and white (W-57%). VAD utilization, outcomes, and PTS were compared between race/ethnicity groups. Multivariable Cox proportional analyses were used to study the association of race and ethnicity on PTS with VAD BTT, using backward selection for covariates. RESULTS: Black children were most ill at listing, with greater proportions of UNOS status 1A/1 (p < 0.001 vs H & W), severe functional limitation (p < 0.001 vs H & W), and greater inotrope requirements (p < 0.05 vs H). Non-white children had higher proportions of public insurance. VAD utilization at listing was: B-11%, H-8%, W-8% (p = 0.001 for B vs H & W). VAD at transplant was: B-24%, H-21%, W-19% (p = 0.001 for B vs H). At transplant, all VAD patients had comparable clinical status (functional limitation, renal/hepatic dysfunction, inotropes, mechanical ventilation; all p > 0.05 between groups). Following VAD, hospital outcomes and one-year PTS were equivalent but long-term PTS was significantly worse among non-whites-(p < 0.01 for W vs B & H). On multivariable analysis, black race independently predicted mortality (hazard ratio 1.67 [95% confidence interval 1.22-2.28]) while white race was protective (0.54 [0.40-0.74]). CONCLUSIONS: Pediatric VAD use is, seemingly, equitable; the most ill patients receive the most VADs. Despite similar pretransplant and early post-transplant benefits, non-white children experience inferior overall PTS after VAD BTT.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Widening our Focus: Characterizing Socioeconomic and Racial Disparities in Congenital Heart Disease.

BACKGROUND: Socioeconomic and racial (SER) disparities among patients with congenital heart disease (CHD) may limit access to high-quality care. We characterized the national SER landscape and its relationship to early outcomes and identified interactions among determinants mitigating adverse outcome. METHODS: The Pediatric Health Information System (PHIS) database was queried for patients (age <26 years) with CHD between 2016 and 2018. International Classification of Diseases, 10th Revision, codes were mapped to diagnostic categories for complexity adjustment. Correlational and hierarchical regression analyses identified risk factors and characterized interactions. RESULTS: We identified 166,599 unique admissions from 52 hospitals, with 58,395 having interventions. Median age was 0 years (interquartile range [IQR], 4 years). Race/ethnicity was predominantly White (59%), Hispanic (20%), and Black (16%). Median neighborhood household income (NHI) was $41,082 and varied among hospitals. Patient NHI had a parabolic relationship with mortality, with both higher and lower values having increased risk. Black patients had significantly higher death, and this relationship was potentiated by lower NHI and complexity. Hospital length of stay was longer among Black neonates (median, 51 days; IQR, 93 days) compared with neonates of other ethnic groups (median, 32 days; IQR, 71 days; P < .0001. Care pathways, including permanent feeding tubes, were also more prevalent among Black neonates (17.8%) compared with White neonates (15%; P = .02). CONCLUSIONS: Interactions among SER disparities modify CHD outcomes. Specific hospitals have more SER fragile patients but may have developed care pathways that prolong length of stay to mitigate risk among Black neonates. Adverse outcomes among SER-disadvantaged patients are magnified in complex CHD, suggesting tangible benefits to targeted resource allocation and population health initiatives.

A mapping algorithm for International Classification of Diseases 10th Revision codes for congenital heart surgery benchmark procedures.

BACKGROUND: Administrative billing data are critical to many initiatives in congenital heart surgery. Mapping algorithms for International Classification of Disease, 10th Revision diagnosis and procedure codes to clinical registry procedure definitions will allow identification of surgical cases and account for patient and procedural factors within administrative data. Our objectives were to develop mapping logic to crosswalk International Classification of Disease, 10th Revision procedure codes to 10 Society of Thoracic Surgeons Congenital Heart Surgery Database benchmark and beta-test the algorithm. METHODS: Patients undergoing Society of Thoracic Surgeons Congenital Heart Surgery Database benchmark procedures from 2015 to 2019 were identified and served as the gold standard. Cases were linked on direct identifiers to cases from the Pediatric Health Information System Database. Two independent teams developed International Classification of Disease, 10th Revision-based algorithms for cases capture. Algorithms were compared and iteratively refined to optimize sensitivity and specificity. Operative mortalities for cases identified in the administrative versus registry data were compared. RESULTS: Overall sensitivity was 91% and specificity was 99% for capture of benchmark operations using International Classification of Diseases 10th Revision codes. Sensitivity was more than 90% in identifying 6 of the 10 individual benchmark procedures and more than 98% sensitive in identifying Fontan, Glenn, and arterial switch with ventricular septal defect procedures. Specificity was more than 98% for all benchmark operations. There were no statistical differences in operative mortality between cases identified in the administrative versus the registry data. CONCLUSIONS: Novel mapping algorithm for International Classification of Disease, 10th Revision procedure codes enables identification of congenital heart benchmark procedures within administrative billing data. This crosswalk facilitates population-based congenital heart surgical research and quality assessment.

Slide Tracheoplasty for Repair of Complex Tracheoesophageal Fistulas.

OBJECTIVES/HYPOTHESIS: Repair of large, recurrent, and complex tracheoesophageal fistulas (TEFs) is challenging and numerous different surgical approaches exist. These various techniques each carry a set of risks and possible complications such as fistula recurrence, tracheal stenosis or pouches, esophageal stenosis, and recurrent laryngeal nerve injury. Slide tracheoplasty is a reconstructive technique successfully used in many different airway pathologies, including TEF repair. This study examines the success, limits, and complications related to slide tracheoplasty for repair of complex TEFs. STUDY DESIGN: Retrospective chart review. METHODS: Patients undergoing TEF repair using a cervical or thoracic approach slide tracheoplasty, at a single institution, between July 2008 and December 2019 were retrospectively reviewed. Demographic data, comorbidities, TEF etiology and surgical history, slide tracheoplasty details and outcomes, and postoperative complication data were examined using descriptive statistics. RESULTS: Twenty-six patients underwent 27 slide tracheoplasties for TEF (20 cervical approaches, 7 thoracic approaches) with a mean age of 5.2 years (IQR 0.7-7.6) at time of surgery. The most common TEF etiologies included congenital (n = 13), tracheostomy tube erosion (n = 5), and button battery ingestion (n = 4). Fistulas ranged in size from <0.5 mm to 4 cm and 59% had previous endoscopic or open repairs. There were two TEF recurrences (7.4%), one of which was successfully revised and the other which was treated with stent placement. Postoperative complications included dehiscence (3.7%), unilateral vocal fold paralysis (3.7%), and mild tracheal stenosis (18.5%). CONCLUSIONS: Slide tracheoplasty is an effective surgical technique for treating complex congenital and acquired TEFs with lower rates of complications when compared to other techniques. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1542-1547, 2022.

Risk Stratification for Congenital Heart Surgery for ICD-10 Administrative Data (RACHS-2).

BACKGROUND: As the cardiac community strives to improve outcomes, accurate methods of risk stratification are imperative. Since adoption of International Classification of Disease-10th Revision (ICD-10) in 2015, there is no published method for congenital heart surgery risk stratification for administrative data. OBJECTIVES: This study sought to develop an empirically derived, publicly available Risk Stratification for Congenital Heart Surgery (RACHS-2) tool for ICD-10 administrative data. METHODS: The RACHS-2 stratification system was iteratively and empirically refined in a training dataset of Pediatric Health Information Systems claims to optimize sensitivity and specificity compared with corresponding locally held Society of Thoracic Surgeons-Congenital Heart Surgery (STS-CHS) clinical registry data. The tool was validated in a second administrative data source: New York State Medicaid claims. Logistic regression was used to compare the ability of RACHS-2 in administrative data to predict operative mortality vs STAT Mortality Categories in registry data. RESULTS: The RACHS-2 system captured 99.6% of total congenital heart surgery registry cases, with 1.0% false positives. RACHS-2 predicted operative mortality in both training and validation administrative datasets similarly to STAT Mortality Categories in registry data. C-statistics for models for operative mortality in training and validation administrative datasets-adjusted for RACHS-2-were 0.76 and 0.84 (95% CI: 0.72-0.80 and 0.80-0.89); C-statistics for models for operative mortality-adjusted for STAT Mortality Categories-in corresponding clinical registry data were 0.75 and 0.84 (95% CI: 0.71-0.79 and 0.79-0.89). CONCLUSIONS: RACHS-2 is a risk stratification system for pediatric cardiac surgery for ICD-10 administrative data, validated in 2 administrative-registry-linked datasets. Statistical code is publicly available upon request.

The use of mechanical assist devices in the pediatric population.

The last two decades have witnessed an expansion in the devices, support strategies, and outcomes for pediatric patients who require mechanical circulatory support. The use of large registries that house data on these devices and the development of shared learning networks have provided clinicians with the ability to critically assess outcomes for emerging and existing technology. The purpose of this review is to provide the reader with perspective on the most contemporary devices utilized for pediatric mechanical circulatory support. It will examine existing support strategies and the most contemporary outcomes regarding these devices including those in high risk patients.

Risk of Pediatric Cardiac Surgery Increased in Patients Undergoing Tracheal Surgery During the Same Hospitalization.

BACKGROUND: The impact of complex tracheal surgery (TS) on outcomes of children undergoing cardiac surgery has rarely been investigated in large national databases and could impact anticipated outcomes in those patients. METHODS: The Pediatric Health Information System database was reviewed from January 1, 2005 to December 31, 2014, for pediatric (<18 years) patients undergoing select cardiac surgical procedures using International Classification of Disease-9 procedural coding. Patients were divided based upon having TS (excluding tracheostomy) during the same hospitalization. Patients with tracheal and cardiac surgeries (CHS + TS) were propensity matched in a 1 : 2 fashion to patients undergoing isolated cardiac surgeries without TS (CHS) based upon cardiac procedure(s), age at surgery, gender, and discharge year. Hospital mortality and other outcomes were compared between the 2 matched groups. RESULTS: The 283 CHS + TS patients were similar to the 566 CHS patients in gender, race, age, cardiac procedures, and presence of chromosomal abnormalities, all P > .05. Hospital mortality was 13.8% (n = 39) for CHS + TS and 5.8% (n = 33) for CHS patients with an unadjusted 2.58 hazard ratio of death (95% confidence interval: 1.59-4.20; P < .001). CHS + TS was also associated with a greater length of stay (63 days vs 12 days), a higher cost per hospitalization ($322 402 vs $80 273), and more readmissions (64.3% [n = 182] vs 41.8% [n = 243]), all P < .001. CONCLUSIONS: Patients undergoing cardiac and TS in the same hospitalization are at greater risk of in-hospital mortality than patients undergoing similar cardiac surgeries alone and incur higher resource utilization thereafter. The increased risk of mortality is currently underappreciated, but it is important to recognize when discussing expectations with families and providers.

Heart-Lung Transplant via an Eighth-Time Sternotomy.

We report a combined heart-lung transplantation following seven prior sternotomies in a patient born with a transitional atrioventricular septal defect. Previous surgeries to repair and replace the mitral valve led to pulmonary vein stenosis and pulmonary vascular disease. Eighth-time sternotomy and significant vascular adhesions led to a prolonged operation and to placing the heart-lung block anterior to the phrenic nerves. Despite this, the patient was ready for discharge after two weeks and continues to do well over nine months later. As more patients survive multiple cardiac palliations with some developing pulmonary vascular disease, heart-lung transplantation may become relevant again.

Tracheal surgery for airway anomalies associated with increased mortality in pediatric patients undergoing heart surgery: Society of Thoracic Surgeons Database analysis.

OBJECTIVES: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database. METHODS: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001). CONCLUSIONS: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.

Initial clinical experience with the HeartMate II ventricular assist system in a pediatric institution.

In many adult cardiac programs, intracorporeal mechanical circulatory support has become a routine treatment for end-stage cardiac failure. For the pediatric population, options are often limited by a small body habitus. Even when an adolescent's weight may suggest adequate space for device implant, most intracorporeal adult devices remain too large for adolescents. The Thoratec HeartMate II (HM II) (approved by the FDA in April of 2008) is a small, noiseless device that is easily operated and monitored. By having an uncomplicated operating system and small percutaneous drive line, the HM II provides an opportunity for these patients to aggressively rehabilitate to become a better transplant candidate and also provides the potential to be discharged home. The two youngest patients ever to utilize the HM II are also the first two cases of using the HM II at a freestanding pediatric hospital. A 12-year-old, 53 kg, girl with dilated cardiomyopathy was supported for 85 days before receiving her heart transplant. The second patient, a 13-year-old, 149 kg, Hispanic male suffering from morbid obesity and dilated cardiomyopathy, was supported for 128 days. The HM II allowed for rehabilitation and nutritional education, resulting in this patient losing 50 kg before heart transplant. Despite both of these patients' size, their thoracic cavities were that of a preadolescent and thus techniques were developed to avoid morbidities like chest wall abrasion and bleeding. Because of differences between adult and pediatric patients and institutions, these cases provided unique challenges. However, as pediatric device therapy is now maturing, pediatric programs such as Texas Children's Hospital have begun to develop strategies for mechanical support that factor in patient's size and need for long-term or temporary support, utilizing the growing number of devices (i.e., Jostra Rotoflow, Tandem Heart PTVA, Thoratec CentriMag, Berlin Heart EXCOR, etc.) that are now available to children.

Transthoracic echocardiography does not reliably predict involvement of the aortic valve in patients with a discrete subaortic shelf.

BACKGROUND: A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve. METHODS: A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings. RESULTS: A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement - 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively - than found at surgery - 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker. CONCLUSION: Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.