Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.

Management of the Adult with Arterial Switch.

Dextro-transposition of the great arteries (d-TGA) is a lethal congenital heart defect in which the great arteries-the pulmonary artery and aorta-are transposed to create ventriculoarterial discordance. Corrective surgical interventions have resulted in significant improvements in morbidity and mortality for this once-fatal congenital heart defect. The initial palliative surgery for d-TGA was the atrial switch operation, which provided physiological correction. The Mustard and Senning "atrial switch" procedures, in which an atrial baffle is created to produce a discordant atrioventricular connection on the existing discordant ventriculoarterial connection, showed preliminary success for the correction of d-TGA. However, follow-up evaluations demonstrated increasing complications from the right ventricle utilized as a systemic ventricle, resulting in progressive right ventricular dysfunction. Thus, the search continued for an anatomical correction of d-TGA to return the great arteries to their normal ventricular connections. The arterial switch operation (ASO), though attempted and theorized by many, was first successfully performed by Dr. Jatene and colleagues in 1975. For ASO, the distal main pulmonary artery and the distal ascending aorta are transected and then anastomosed to their respective ventricles with relocation of the coronary arteries to the neoaorta. The ASO has replaced the atrial switch operation since the 1980s and is now the standard surgical correction for d-TGA. As more patients who have undergone ASO are living into adulthood, late complications of this procedure have become more evident. The most common late postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency, and neopulmonic stenosis. Adults who have undergone ASO in childhood will need follow-up with surveillance imaging and evaluation of new symptoms or declining function to prevent and manage late postoperative complications. This review describes the management strategies for common late complications in patients who have undergone ASO.

Results of international assistance for a paediatric heart surgery programme in a single Ukrainian centre.

BACKGROUND: Surgery for CHD has been slow to develop in parts of the former Soviet Union. The impact of an 8-year surgical assistance programme between an emerging centre and a multi-disciplinary international team that comprised healthcare professionals from developed cardiac programmes is analysed and presented.Material and methodsThe international paediatric assistance programme included five main components - intermittent clinical visits to the site annually, medical education, biomedical engineering support, nurse empowerment, and team-based practice development. Data were analysed from visiting teams and local databases before and since commencement of assistance in 2007 (era A: 2000-2007; era B: 2008-2015). The following variables were compared between periods: annual case volume, operative mortality, case complexity based on Risk Adjustment for Congenital Heart Surgery (RACHS-1), and RACHS-adjusted standardised mortality ratio. RESULTS: A total of 154 RACHS-classifiable operations were performed during era A, with a mean annual case volume by local surgeons of 19.3 at 95% confidence interval 14.3-24.2, with an operative mortality of 4.6% and a standardised mortality ratio of 2.1. In era B, surgical volume increased to a mean of 103.1 annual cases (95% confidence interval 69.1-137.2, p<0.0001). There was a non-significant (p=0.84) increase in operative mortality (5.7%), but a decrease in standardised mortality ratio (1.2) owing to an increase in case complexity. In era B, the proportion of local surgeon-led surgeries during visits from the international team increased from 0% (0/27) in 2008 to 98% (58/59) in the final year of analysis. CONCLUSIONS: The model of assistance described in this report led to improved adjusted mortality, increased case volume, complexity, and independent operating skills.

No difference found in safety or efficacy of balloon atrial septostomy performed at the bedside versus the catheterisation laboratory.

BACKGROUND: Balloon atrial septostomy is performed in infants with dextro-transposition of the great arteries to improve oxygenation before surgery. It is performed in the catheterisation laboratory with fluoroscopy or at the bedside using echocardiography. It is unclear whether procedural safety and efficacy is superior in one location versus the other, although the bedside procedure may improve resource utilisation and present an opportunity for reducing cost. This study compares safety and efficacy of atrial septostomy performed at the patient's bedside versus the catheterisation laboratory. METHODS: Neonates with dextro-transposition of the great arteries who underwent balloon atrial septostomy from October, 2000 to January, 2014 were included. Medical and procedural records, echocardiograms, and catheterisation data were reviewed. Comparisons between the two procedural locations included patient demographics, pre- and post-procedure oxygen saturations, and outcomes. Complications reviewed included bleeding, arrhythmia, cardiac trauma, stroke, and death. Coronary artery evaluations were recorded. T-tests were used for continuous variables, and Fisher's exact tests were used for all categorical variables. Wilcoxon rank sum and analysis of covariance modelling were used for time variables and oxygen saturation, respectively. RESULTS: A total of 88 infants met the inclusion criteria. Among them, 53 underwent septostomy at the bedside and 35 underwent septostomy in the catheterisation laboratory. No safety or outcome benefit was identified between the two procedural locations. CONCLUSION: Septostomy performed at the bedside and in the catheterisation laboratory had similar outcomes and efficacy. Further, bedside septostomy has the advantage of no radiation exposure, and obviating risks with patient transfer from the ICU to the catheterisation laboratory.

Transcatheter closure of a sinus venosus atrial septal defect using 3D printing and image fusion guidance.

A 63-year-old man with cirrhosis, hepatocellular carcinoma, and coagulopathy was diagnosed with a sinus venosus atrial septal defect (ASD) and partial anomalous pulmonary venous return (PAPVR) of the right upper pulmonary vein (RUPV). Transcatheter repair by positioning a stent graft in the superior vena cava was planned. Based on three-dimensional (3D) reconstruction of gated cardiac CTA, a 28 mm × 7 cm Endurant II® aortic extension stent graft (Medtronic, MN) was chosen. A 3D model printed from the CTA was used to simulate device deployment, demonstrating successful exclusion of the sinus venosus ASD with return of the RUPV to the left atrium (LA). Post simulation, the 3D model was used for informed consent. The patient was then taken to the hybrid operating room. On-table cone beam CT was performed and registered with the CTA images. This enabled overlay of 3D regions of interest to live 2D fluoroscopy. The stent graft was then deployed using 3D regions of interest for guidance. Hemodynamics and angiography demonstrated successful exclusion of the sinus venosus ASD and unobstructed return of RUPV to the LA. This is the first report of comprehensive use of contemporary imaging for planning, simulation, patient consent, and procedural guidance for patient-centered complex structural intervention in repair of sinus venosus ASD with PAPVR. We propose this as a process model for continued innovation in structural interventions.

Intraoperative cone beam computed tomography-guided endovascular recanalization of an interrupted aorta.

A 42-year-old man sustained a stroke secondary to malignant hypertension and was found to have complete aortic interruption. We report a case of real-time image-guided endovascular repair to highlight the value of preprocedural planning and intraprocedural cone beam computed tomography. Two-dimensional fluoroscopy enhanced with three-dimensional landmarks from cone beam computed tomography was used to direct a Nykanen radiofrequency wire (Baylis Medical, Montreal, Quebec, Canada) through the interruption, avoiding critical adjacent structures. A covered Cheatham-Platinum stent (NuMED, Inc, Hopkinton, NY) was deployed successfully, recanalizing the thoracic aorta. The pressure gradient normalized, and the patient was discharged on postoperative day 1. At 10-month follow-up, the patient was on an antihypertensive regimen of two minimum-dose drugs.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). RESULTS: Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Hybrid Approach to Pulmonary Valve Replacement with Melody Prosthesis Following Pulmonary Banding.

We report the case of a 27-year-old Jehovah's Witness patient with severe pulmonary insufficiency and right ventricular dilatation 16 years after primary repair, who sought transcatheter therapy as a means to avoid surgery and the risk of blood product administration. A hybrid procedure involving pulmonary artery banding to a diameter amenable to fixation of a Melody valve (Medtronic Inc.) via trans-ventricular puncture was performed.

Balloon angioplasty of recoarctation of the neoaortic arch after the Norwood operation: factors affecting outcome and recurrence.

BACKGROUND: Balloon angioplasty (BA) is effective in relieving neoaorta recoarctation (reCoa) after the Norwood procedure. However, recurrence is not uncommon and risk factors for success and recurrence require further elucidation. We report the results of BA for reCoa following the Norwood procedure. We examine acute results and risk factors associated with success and recurrence after BA. METHODS: Patients who underwent BA between November 2000 and June 2010 were studied. Factors for immediate success and recurrence after BA were determined using logistic regression. Recurrence-free survival was evaluated using the Kaplan-Meier curve. RESULTS: Forty-seven angioplasties were performed in 39 patients. Mean age at catheterization was 5.0 ± 4.1 months. BA was successful in 40 angioplasties (85.1%). A higher preprocedure gradient across the coarctation (P = 0.04) and a higher ratio of balloon to descending aorta (P = 0.01) were associated with success. Six patients required redilation. Risk factors for recurrence included older age at Norwood (P = 0.02), younger age (P = 0.03), lower weight (P = 0.04) and smaller body surface area at balloon angioplasty (BA) (P = 0.04), and shorter duration between surgery and angioplasty (P = 0.03). Freedom from recurrence from the first catheterization was 82% after 6 months and 78% after 1 year. There were no neurologic sequelae or deaths. CONCLUSIONS: BA is effective acutely and long term with limited morbidity and mortality. Recurrence occurs usually within the first year. Delay in performing the initial angioplasty may be beneficial in reducing the risk of recurrence, but further study is needed.

Outcomes of interrupted aortic arch repair using the carotid artery turndown procedure.

OBJECTIVE: Interrupted aortic arch is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Multiple surgical modalities exist to address this defect. We evaluate the long-term outcome of interrupted aortic arch with the left carotid artery turndown technique from a single institution. METHODS: Patients with interrupted aortic arch who underwent the carotid turndown procedure were identified between September 1982 and March 2010. Medical and surgical records were reviewed. Mortality data were obtained from state death records. RESULTS: Forty-seven patients met inclusion criteria. Median follow-up was 6.2 years (5 days to 23.2 years). Twenty-one patients (45%) had genetic syndromes. There were 4 operative deaths (8.5%) and 9 late deaths (19%). One-year and 5-year survivals were 80.2% and 72.6%, respectively. Seventeen patients (36.2%) required reoperation or other interventions on the aortic arch. CONCLUSIONS: Left carotid artery turndown offers a favorable surgical outcome. It compares with end-to-end repair, while providing a tension-free anastomosis and avoiding neonatal circulatory arrest and cardiopulmonary bypass. Disadvantages include a 2-stage repair and a significant reintervention rate, particularly when compared with the aortic arch advancement technique. Nevertheless, the reduced exposure to circulatory arrest and bypass and avoidance of left bronchial obstruction are important considerations that may offset these limitations.

Mid-term follow up of perventricular device closure of muscular ventricular septal defects.

BACKGROUND: Surgical and transcatheter management of muscular ventricular septal defects (MVSD) have independent drawbacks. Hybrid procedures are becoming increasingly utilized to manage congenital cardiac lesions including MVSDs. This report describes the mid-term results of perventricular device closure of MVSDs at a single institution. METHODS: The cardiology database of patients who underwent attempted perventricular MVSD closure from a single institution was reviewed. RESULTS: Between January 2004 and December 2009, six patients underwent attempted perventricular MVSD closure in the operating room. Mean age was 9.8 ± 9.1 months; mean weight was 7.2 ± 3.7 kg. In five patients, closure was successful without use of bypass. In one patient, the device embolized to the left ventricle after release and patch closure of the MVSD was performed on cardiopulmonary bypass. The mean interval from the procedure to the most recent echocardiogram for the patients with successful perventricular closure was 39.8 ± 25.2 months. Three patients demonstrated no residual shunt at the last echocardiogram. Two patients had mild, hemodynamically insignificant shunting; one had a left ventricular pseudoaneurysm that was embolized during repeat catheterization. CONCLUSIONS: Perventricular closure of MVSDs is attractive because it overcomes the limitations of surgery and catheterization. Additionally, it spares the need for cardiopulmonary bypass and its comorbidities. In some instances, however, successful deployment of the device is not possible. Our mid-term results demonstrate overall success but identify possible complications that are not immediately identified in the short term.

The outcome of pulmonary artery stents following surgical manipulation.

BACKGROUND: Pulmonary artery (PA) stents are utilized to treat branch pulmonary stenosis (BPS). Often patients with PA stents undergo subsequent cardiac surgery for other indications, and the stents can be manipulated during the procedure. OBJECTIVE: The purpose of this study was to evaluate the outcome of branch PA stenoses following surgical manipulation of previously implanted PA stents and to determine factors associated with future reintervention. METHODS: Catheterization data, operative reports, and clinical summaries were reviewed on patients with PA stents placed between September 1989 and December 2006 undergoing subsequent cardiac surgery. Surgical manipulation was recorded as removed, trimmed, or longitudinally transected, and patched. Those that were not manipulated were defined as untouched. RESULTS: 459 patients had branch PA stents placed. About 54 patients, with 70 stents in branch PA's. subsequently had further cardiac surgery. The median age of stent placement was 7.5 (0.5-32.4) years with a median age of surgery of 12.7 (5.1-39.6) years. Surgical manipulation was performed in 23 (33%) PA's and 47 (66%) stents were untouched. Stent removal occurred in 11 (16%), with transecting longitudinally and patching in 5 (7%), and trimming in 7 (10%). Comparing the surgical manipulation and the untouched groups, there was no difference in median age of stent placement [7.2 (0.5-30.2) versus 7.6 (1.8-32.4) years, p=0.40], wt [21.0 (5.3-86.5) versus 24.7 (9.0-96.0) kg, p=0.42], or residual catheterization gradient across the stent [3 (0-59) versus 4 (0-50) mmHg, p=0.81]. Catheter reintervention (stent n=6 or balloon dilation n=14) on the previously stented PAs was similar between the surgically manipulated (median 7.5 years) and untouched groups (median 11.5 years) (p=0.31). In multivariate analysis, the factors associated with future catheter reintervention were having the stent transected longitudinally and patched (p=0.003) and a lower weight (p=0.006) at the time of stent placement. CONCLUSIONS: Surgical stent manipulation is often performed in patients who have PA stents. Surgical manipulation does not alter the need for future reintervention and catheter re-intervention may be more likely when the stents are transected longitudinally and patched.

Viral endomyocardial infection is an independent predictor and potentially treatable risk factor for graft loss and coronary vasculopathy in pediatric cardiac transplant recipients.

OBJECTIVES: This study sought to evaluate the outcome and prevalence of viral endomyocardial infection after cardiac transplantation. BACKGROUND: Viral myocardial infection causes heart failure, but its role after cardiac transplantation is unclear. We hypothesized that viral infection of the cardiac allograft reduces graft survival. METHODS: Between June 1999 and November 2004, 94 pediatric cardiac transplant patients were screened for the presence of viral genome in serial endomyocardial biopsies (EMBs) using polymerase chain reaction (PCR) assays. Graft loss, advanced transplant coronary artery disease (TCAD), and acute rejection (AR) were compared in the PCR-positive (n = 37) and PCR-negative (n = 57) groups, using time-dependent Kaplan-Meier and Cox regression analyses. From November 2002 to November 2004, intravenous immunoglobulin therapy (IVIG) was administered to patients with PCR-positive EMBs. The outcomes of the IVIG-treated, PCR-positive patients (n = 20) were compared with IVIG-untreated, PCR-positive patients (n = 17). RESULTS: Viral genomes were detected in EMBs from 37 (39%) patients; parvovirus B19, adenovirus, and Epstein-Barr virus (EBV) were the most common. The PCR-positive group (n = 37, 25% graft loss at 2.4 years) had decreased graft survival (p < 0.001) compared with the PCR-negative group (n = 57, 25% graft loss at 8.7 years) and developed advanced TCAD prematurely (p = 0.001). The number of AR episodes was similar in both groups. On multivariate analysis, presence of viral genome was an independent risk factor for graft loss (relative risk: 4.2, p = 0.015). The time to advanced TCAD after becoming PCR-positive was longer in the IVIG-treated patients (p = 0.03) with a trend toward improved graft survival (p = 0.06). CONCLUSIONS: Viral endomyocardial infection is an independent predictor of graft loss in pediatric cardiac transplant recipients. This effect appears to be mediated through premature development of advanced TCAD. IVIG therapy in this subgroup may improve survival and merits further investigation.

Viral epidemiologic shift in inflammatory heart disease: the increasing involvement of parvovirus B19 in the myocardium of pediatric cardiac transplant patients.

BACKGROUND: Detection of viral genome in rejecting cardiac transplant patients has been reported, with coxsackievirus and adenovirus causing premature graft failure. Recently, parvovirus B19 (PVB19) genome in myocardial samples has been increasingly reported, but its role in cardiac pathology and effect on transplant graft survival are unknown. The objectives of this study were to determine if changes in the viruses identified in the myocardium represent an epidemiologic shift in viral myocardial disease and whether PVB19 adversely affects transplant graft survival. METHODS: From September 2002 to December 2005, nested polymerase chain reaction was used to evaluate endomyocardial biopsy specimens for 99 children (aged 3 weeks-18 years) with heart transplants for the presence of viral genome. Cellular rejection was assessed by histology of specimens. Transplant coronary artery disease (TCAD) was diagnosed by coronary angiography or histopathology. RESULTS: Specimens from 700 biopsies were evaluated from 99 patients; 121 specimens had viral genome, with 100 (82.6%) positive for PVB19, 24 for Epstein-Barr virus (EBV; 7 positive for PVB19 and EBV), 3 for CMV, and 1 for adenovirus. Presence of PVB19 genome did not correlate with rejection score, nor did a higher viral copy number. Early development of advanced TCAD (p < 0.001) occurred in 20 children with persistent PVB19 infection (> 6 months). CONCLUSIONS: PVB19 is currently the predominant virus detected in heart transplant surveillance biopsy specimens, possibly representing an epidemiologic shift. Cellular rejection does not correlate with the presence or quantity of PVB19 genome in the myocardium, but children with chronic PVB19 infection have increased risk for earlier TCAD, supporting the hypothesis that PVB19 negatively affects graft survival.

Pulmonary artery stents: long-term follow-up.

OBJECTIVES: Determine the long-term outcomes of branch pulmonary artery (PA) stents. BACKGROUND: PA stents in congenital heart disease effectively relieve stenoses in the short-term. Published long-term data are limited. METHODS: Patients enrolled in an FDA IDE protocol from 1989-92 were included. Clinical follow-up and catheterization data were evaluated. Patients were included if >5 year follow-up data was available or if mortality occurred following the initial procedure. RESULTS: There were five deaths: four due to progression of their underlying heart disease, and one from a complication during a follow-up catheterization. Clinical data for 43 surviving patients demonstrated 39 patients (91%) are in NYHA class I or II. Seven patients underwent surgical intervention during the follow-up period (five RV-PA conduit, two Fontan revisions), but none addressed PA stenosis. Final repeat catheterizations were performed in 36 patients (55 stents) 7.2 +/- 4.3 years post stent insertion with 1.2 +/- 0.9 further procedures with stent dilations. In this subgroup, the minimum vessel diameter increased from 4.7 +/- 1.8 to 13.4 +/- 2.4 mm (P < 0.001), and the pressure gradient improved from 41 +/- 25 to 9 +/- 11 mm Hg (P < 0.001). Higher initial gradient and smaller balloons were associated with a final stent diameter of <14 mm (P = 0.030 and 0.046). Jailed vessels occurred in 49% of stents with abnormal angiographic flow in 18/55. Six repeat catheterizations resulted in complications, including the one procedural death. CONCLUSION: Stents implants for PA stenoses provide effective improvement in vessel caliber in the long-term. Although repeat interventions are necessary, this procedure reduces RV pressure and provides an important alternative to surgery for residual PA obstruction.

Myocardial pro-inflammatory cytokine expression and cellular rejection in pediatric heart transplant recipients.

BACKGROUND: Accumulating evidence suggests that immune-mediated injury is important in the development of rejection after heart transplantation. We hypothesized that pro-inflammatory cytokine expression would increase in biopsy samples that manifest cellular rejection and that this would correlate with the development and progression of transplant cellular rejection. METHODS: Children with heart transplants were prospectively enrolled from July 2004 to November 2005. Right ventricular endomyocardial biopsies were obtained during routine catheterization for rejection surveillance. Cellular rejection was graded using criteria established by the International Society for Heart and Lung Transplantation. RNA was extracted from biopsy samples and reverse transcription was used for complementary DNA synthesis. The cDNA product was evaluated by quantitative real-time polymerase chain reaction (PCR) to measure the following cytokines: interleukin (IL)-1beta, IL-6 and IL-18; tumor necrosis factor-alpha (TNF-alpha); and interferon-gamma (IFN-gamma). Normalized cytokine mRNA transcripts were correlated with cellular rejection scores and the presence of viral genome. RESULTS: Seventy-four children (mean age 9.6 +/- 5.5 years, range 0.2 to 20.5 years) were enrolled and 95 biopsies were obtained. None of the cytokines demonstrated a correlation with the cellular rejection score, even within individual patients for whom multiple, serial biopsy samples were studied. Eighteen biopsy samples were found to have parvovirus B19 genome present, but there was no correlation between cytokine levels and the presence of parvovirus. CONCLUSIONS: Cytokine transcripts in heart transplants do not correlate with cellular rejection. In addition, there is no correlation between cytokine transcripts and the presence of viral genome.

The efficacy of mitral valve surgery in children with dilated cardiomyopathy and severe mitral regurgitation.

Severe mitral regurgitation predicts poor outcomes in adults with left ventricular dysfunction. Frequently, adult patients now undergo initial mitral valve surgery instead of heart transplant. Pediatric data are limited. This study evaluates the efficacy of mitral valve surgery for severe mitral regurgitation in children with dilated cardiomyopathy. This is a single-institution experience in seven children (range, 0.5-10.9 years) with severe mitral regurgitation and dilated cardiomyopathy who underwent mitral valve surgery between January 1988 and February 2005, with follow-up to January 2006. Children with dilated cardiomyopathy had a depressed fractional shortening preoperatively (24.4% +/- 6.1%) that remained depressed (22.9% +/- 7.6%) 1.3 +/- 1.2 years after surgery (p = 0.50). Left ventricular end-diastolic (6.5 +/- 1.5 to 4.8 +/- 1.8 z-scores, p < 0.01) and end-systolic (6.8 +/- 1.5 to 5.5 +/- 2.1 z-scores, p < 0.05) dimensions improved. Hospitalization frequency had a median decrease of 6.0 hospitalizations per year (p < 0.02). Three patients were transplanted 0.2, 2.4, and 3.5 years after surgery. There was no perioperative mortality. Mitral valve surgery in children with dilated cardiomyopathy was performed safely and improved symptoms, stabilizing ventricular dysfunction in most patients. Mitral valve surgery should be considered prior to heart transplant in children with dilated cardiomyopathy and severe mitral regurgitation.

Effect of body mass index on outcome in pediatric heart transplant patients.

BACKGROUND: Obesity and cachexia are risk factors for adverse outcomes in adult transplant patients. However, little is known about the effects of body mass index (BMI) on outcomes in pediatric heart transplant patients. METHODS: Patients > 2 years of age undergoing heart transplantation from 1985 to 2004 at our institution were included in this study. BMI was assessed at the time of transplant and at 1 year post-transplant. Long-term outcomes were assessed by weight group. RESULTS: The cohort included 105 patients with a mean age at transplant of 9.6 +/- 5.3 years. The mean BMI percentile at the time of transplant was 39 +/- 34, with 22 (21%) patients underweight (< 5th percentile) and 8 (8%) patients overweight (> or = 95th percentile). Among patients surviving to 1 year (n = 92), the mean BMI percentile increased to 57 +/- 33 (p < 0.05). Overall graft survival was decreased in patients underweight at transplant, mean 6.7 years (95% confidence interval [CI] 3.6 to 9.9), vs normal weight patients, mean 10.6 years (95% CI 8.8 to 12.4) (p < 0.05). Patients overweight at transplant did not have decreased graft survival. Neither low nor high BMI at 1 year post-transplant was associated with adverse outcomes. On multivariate analysis, being underweight at transplant was an independent predictor of decreased graft survival (p = 0.03). CONCLUSIONS: Weight gain was nearly universal post-transplant with only 4% of patients underweight at 1 year. In the small number of patients overweight at transplant, graft survival was similar to normal-weight patients. Conversely, being underweight at transplant was an independent predictor of decreased graft survival.