Transcatheter Intervention of Coronary Obstructions in Infants, Children, and Young Adults.

This study assesses the characteristics of coronary obstructions that underwent transcatheter intervention in a pediatric catheterization laboratory, the procedural details, and patient outcomes. Acute cardiac failure due to coronary obstructions in children is rare. The role of catheter based intervention is largely unreported. Single center retrospective review between January 2000 and December 2016. Thirty-three patients (median age 2y/o [0-38], weighing 9.6 kg [2.2-91]) underwent 39 transcatheter interventions on 39 lesions, mainly left main coronary (16/39; 39%) and right coronary (9/39; 23%) arteries. Most patients had congenital heart disease (29/33; 88%). Cath indications included ventricular dysfunction (17), cardiac arrest (7), failure to wean from cardiopulmonary bypass (5), and other (4). Almost half (18/39; 46%) were performed on ECMO support. Obstructions were post-surgical (16; 4 with coronary manipulation), thrombotic (13; 5 < 30 days from cardiac surgery), and miscellaneous lesions (10). Interventions included 25 bare metal stents implanted in 22 lesions in 17 patients (mainly post-surgical lesions; 3 at Damus-Kaye-Stansel anastomosis), nine balloon angioplasty only, four lytic therapy ± mechanical disruption of thrombus, and four technical failures. There were no procedure-related deaths. Most patients survived to discharge or transplant (24/33; 73%). Six patients who received stents had follow-up catheterization (median 15.5 months [1-106]); all were without restenosis. Most coronary obstructions intervened upon in a pediatric cath lab were on young, critically ill patients with congenital heart disease secondary to surgical manipulation/injury or thrombosis. Transcatheter intervention should be considered a potential treatment strategy in this population.

Targeted Increase in Pulmonary Blood Flow in a Bidirectional Glenn Circulation.

In patients with a Glenn shunt and unilateral pulmonary vascular abnormalities, supplemental pulmonary blood flow may improve antegrade flow in the target lung and may permit eventual Fontan completion, while mitigating the risks of uncontrolled additional flow to the contralateral lung. We attempted this by inserting a small aortopulmonary shunt into the affected pulmonary artery and banding the central pulmonary artery. We performed a retrospective review of all patients who underwent this procedure from 2000 to 2015. Comparisons of nonparametric variables were performed with the Wilcoxon signed rank test. Twenty patients were followed for a median of 36 months. Vascular abnormalities included recalcitrant unilateral pulmonary arterial (n = 8) or venous (n = 5) obstruction or hypoplasia, combined disease (n = 4), or abundant aortopulmonary collaterals (n = 3). Nine had retrograde flow from the affected lung. The most frequent complications were pleural effusions (n = 4) and shunt thrombosis (n = 4). Postoperatively, all patients demonstrated antegrade flow to the target lung at the earliest postoperative catheterization. Glenn pathway pressures and saturations were unchanged, whereas the shunted pulmonary arterial oxygen saturation and pressure increased by 4% (P = 0.04) and 6 mm Hg (P = 0.04), respectively. Three patients died; 9 achieved definitive palliation, 4 were deemed to have no improvement, and 4 continue to have a contralaterally shunted Glenn at this time. Targeted additional pulmonary blood flow may be useful for the palliation of single ventricle patients with abnormal pulmonary vasculature. However, morbidity and mortality are common in this high-risk population.

Tissue expander-stimulated lengthening of arteries for the treatment of midaortic syndrome in children.

BACKGROUND: Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta. Patients with disease refractory to medical management will usually require either endovascular therapy or surgery with use of prosthetic graft material for bypass or patch angioplasty. We report our early experience with a novel approach using a tissue expander (TE) to lengthen the normal native arteries in children with MAS, allowing primary aortic repair without the need for prosthetic graft material. METHODS: We conducted a retrospective review of patients with MAS undergoing the TE-stimulated lengthening of arteries (TESLA) procedure at our institution from 2010 to 2014. Data are presented as mean (range). RESULTS: Five patients aged 4.8 years (3-8 years) underwent the TESLA procedure. Stages of this procedure include the following: stage I, insertion of retroaortic TE; stage II, serial TE injections; and stage III, final repair with excision of aortic stenosis and primary end-to-end aortic anastomosis. Stage II was completed in 4 months (1-9 months) with 12 (7-20) TE injections. Goal lengthening was achieved in all patients. Stage III could not be completed in one patient because of extreme aortic inflammation, which precluded safe excision of the aortic stenosis and required use of a prosthetic bypass graft. The other four patients completed stage III with two (one to three) additional vessels also requiring reconstruction (renal or mesenteric arteries). At 3.2 years (1-6 years) of follow-up, all patients are doing well. CONCLUSIONS: The TESLA procedure allows surgical correction of MAS without the need for prosthetic grafts in young children who are still growing.

Concept of an expandable cardiac valve for surgical implantation in infants and children.

BACKGROUND: Options for cardiac valve replacement in children are limited to fixed-diameter prostheses that do not accommodate for somatic growth. An externally stented bovine jugular vein graft has been modified for surgical valve replacement in pediatric patients, with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. METHODS: Pediatric patients at a single institution who underwent surgical implantation of an expandable bovine jugular vein valve between 2010 and 2014 were reviewed retrospectively. Forty-two patients underwent implantation at median age of 10 months (range, 3 weeks to 5.8 years) in aortic, mitral, pulmonary, or tricuspid positions. Numerous techniques for valve modification and implantation were used. RESULTS: The valve was competent with low gradient acutely postoperatively in all patients. Eight patients experienced central or paravalvular deterioration, and 7 required reoperation for valve-related adverse outcomes. Twenty patients underwent at least one previous valve repair or replacement. Twenty patients underwent 32 episodes of catheter-based balloon expansion of the valve, exhibiting a significant decrease in median gradient from 12 mm Hg to 8 mm Hg (P < .001) with no significant increase in grade of regurgitation. At 12 months after implantation, Kaplan-Meier analysis indicated that 88% would be expected to be free from reoperation (95% confidence interval, 78%-98%). A total of 6 deaths occurred, 3 before discharge and 3 late. CONCLUSIONS: A surgically implanted externally reinforced bovine jugular vein demonstrates acceptable short-term function and is amenable to catheter-based enlargement as the child grows. Modification of valve design and implantation techniques are necessary to reduce perivalvular complications.

Stented bovine jugular vein graft (Melody valve) for surgical mitral valve replacement in infants and children.

OBJECTIVE: The options for mitral valve replacement in children with irreparable mitral valve disease have been limited to fixed-diameter prostheses that do not accommodate for somatic growth. We have modified an externally stented bovine jugular vein graft (Melody valve) for implantation in this cohort. Because it is not a fixed-diameter prosthesis, we hypothesized that the valve can be expanded in the catheterization laboratory as the child grows. METHODS: The medical records of patients who had undergone Melody valve implantation in the mitral or left atrioventricular valve position from 2010 to 2013 were reviewed. RESULTS: Eleven patients had undergone Melody valve implantation at a median age of 7 months (range, 2-28). The techniques of valve modification and implantation included stent shortening, adding a pericardial sewing cuff, intraoperative balloon expansion, and fixation of the distal stent to the inferior left ventricle wall. The valve was competent, with a low gradient acutely postoperatively in all patients. One patient died, and one required permanent pacemaker implantation. One patient developed valve dysfunction and required explantation. Two patients without a pericardial sewing cuff developed paravalvular leaks. One patient who had not undergone distal stent fixation developed left ventricular outflow tract obstruction. Three patients who had undergone subsequent catheter-based balloon expansion of the valve have continued to demonstrate acceptable valvular function. CONCLUSIONS: The Melody valve has demonstrated acceptable short-term function. Implantation techniques to prevent left ventricular outflow tract obstruction (suture fixation of the distal stent) and paravalvular leaks (the addition of a pericardial cuff) should be considered. The Melody valve can be percutaneously expanded as the child grows.

Outcomes after primary transcatheter therapy in infants and young children with severe bilateral peripheral pulmonary artery stenosis.

BACKGROUND: Angioplasty and stent implantation have become accepted therapies for isolated peripheral pulmonary stenosis, and have been shown to increase vessel diameter and reduce right ventricular (RV) pressure acutely in patients with pulmonary artery (PA) stenosis. The purpose of this study was to assess long-term outcomes after primary transcatheter therapy for peripheral pulmonary stenosis. METHODS AND RESULTS: We studied 69 patients who underwent primary transcatheter intervention for severe isolated peripheral pulmonary stenosis at ≤ 5 years of age. Genetic/syndromic diagnoses included Williams syndrome (n=23), non-Williams familial arteriopathy (n=12), and Alagille syndrome (n=3). At the initial PA intervention, median RV:aortic pressure ratio decreased from 1.00 to 0.88 (median decrease, 0.18; P<0.001). Patients with a higher preintervention RV:aortic pressure ratio had a greater reduction (P<0.001). During follow-up (median, 8.5 years), 10 patients died, 5 from complications of PA catheterization (all before 1998). Thirteen patients underwent surgical PA intervention, most within 1 year and along with repair of supravalvar aortic stenosis. Freedom from any PA reintervention was 38 ± 6% at 1 year and 22 ± 6% at 5 years. The median RV:aortic pressure ratio decreased from 1.0 at baseline to 0.53 at the most recent catheterization (P<0.001), and 82% of patients with available clinical follow-up were asymptomatic. CONCLUSIONS: Transcatheter therapy for infants with severe peripheral pulmonary stenosis has become safer, regardless of genetic condition. Coupled with reintervention and surgical relief in selected cases, RV:aortic pressure ratios decrease substantially and most patients are asymptomatic at late follow-up.

Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management.

BACKGROUND: Midaortic syndrome is often associated with refractory hypertension. The aim of our study was to better understand the short- and medium-term outcomes in this patient population utilizing a multidisciplinary management approach. METHODS: We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years. RESULTS: Fifty-three patients presented at a median age of 6.7 years (birth to 28.7 years). Thirty-five patients (66 %) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58 % at 1 year and 33 % at 5 years. Freedom from reintervention after a surgical procedure was longer: 83 % at 1 year and 72 % at 10 years. At the most recent follow-up, the majority of patients (69 %) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4-21.1) years. The median number of anti-hypertensive medications was 1 (0-5). CONCLUSIONS: A multidisciplinary management strategy which couples comprehensive medical management with catheter-based and surgical interventions can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome.

Bloodstream infections occurring in patients with percutaneously implanted bioprosthetic pulmonary valve: a single-center experience.

BACKGROUND: Percutaneous pulmonary valve implantation using a stent-based bioprosthetic valve provides an alternative to surgery in select patients. Systemic infections in Melody valve-implanted patients with and without identified valve involvement have been reported, yet the incidence is unknown, and risk factors remain unidentified. METHODS AND RESULTS: Between 2007 and 2012, a total of 147 consecutive patients with congenital heart disease underwent Melody percutaneous pulmonary valve implantation at our institution. Demographic and clinical variables were collected at baseline and at follow-up and analyzed as predictors. The occurrence of bloodstream infection (BSI), defined as a bacterial infection treated with ≥4 weeks of antibiotics, served as our primary outcome. The mean age at implantation for the study population was 21.5±11 years, and tetralogy of Fallot was the cardiac condition in 59%. During a median follow-up of 19 months, 14 patients experienced BSI (9.5%; 95% confidence interval, 5.3%-15%). Of these, 4 (2.7%) patients had Melody valve endocarditis. Two patients died during the event, neither of whom had known valve involvement. The median procedure to infection time was 15 months (range, 1-56). In univariate analysis, male sex, previous endocarditis, in situ stents in the right ventricular outflow tract, and presence of outflow tract irregularities at the implant site were associated with BSI occurrence. CONCLUSIONS: In this cohort, 9.5% of patients who underwent Melody percutaneous pulmonary valve implantation experienced subsequent BSI, occurring 1 to 56 months after implant, and 2.7% of patients had prosthetic endocarditis. Our findings suggest that patient and nonvalve anatomic factors may be associated with BSI after percutaneous pulmonary valve implantation.

Risk factors for profuse systemic-to-pulmonary artery collateral burden in hypoplastic left heart syndrome.

Risk factors for developing systemic-to-pulmonary artery collaterals (SPCs) in hypoplastic left heart syndrome (HLHS) are unknown. We performed a retrospective case-control study to identify risk factors for developing a profuse SPC burden in HLHS. Angiograms of 439 patients with HLHS (performed <2 years of age) were reviewed using a previously published angiographic grading scale to identify cases (profuse SPC burden, n = 20) and controls (no or minimal SPC burden, n = 35). In univariate analyses, profuse SPC burden was associated with mitral atresia and aortic atresia subtype (MA/AA) (65% vs 14%, p <0.0001), use of a Sano shunt (70% vs 37%, p = 0.03), longer log-transformed durations of intensive care unit stay (p = 0.02), hospital stay (p = 0.002), pleural drainage (p = 0.008) after stage 1 palliation, lower oxygen saturation at discharge after stage 1 palliation (82 ± 4 vs 85 ± 4%, p = 0.03), and a history of severe shunt obstruction (37% vs 11%, p = 0.04). In a multivariate logistic regression model, profuse SPC burden was associated with MA/AA subtype (odds ratio 6.6), Sano shunt type (odds ratio 8.6), and log-transformed duration of hospital stay after stage 1 (odds ratio 7.9, model p <0.0001, area under the curve 0.88). Nonassociated parameters included fetal aortic valve dilation, severe cyanotic episodes, number of days with open sternum or number of additional exploratory thoracotomies after stage 1 palliation, pulmonary vein stenosis, and restrictive atrial septal defect. In conclusion, in the present case-control study of patients with HLHS, the development of a profuse SPC burden was associated with MA/AA subtype, Sano shunt type, and longer duration of hospital stay after stage 1 palliation.

Annulus upsizing for mitral valve re-replacement in children.

BACKGROUND: Mitral valve replacement remains the last resort for treatment of congenital mitral valve disease. Enlarging the mitral annulus at the time of mitral valve replacement may allow implantation of a larger prosthesis in children. METHODS: All mitral valve replacement procedures from 1990 to 2012 were included, and mitral annulus enlargement techniques were analyzed. A control group of patients undergoing replacement of a previously implanted mechanical mitral valve prosthesis was compared with the cohort of patients who underwent annular enlarging procedures. Primary end points were increases in annulus size and Z score, and freedom from paced rhythm. RESULTS: Among 205 mitral valve replacement procedures, 16 (8%) included techniques to upsize the mitral annulus in 15 patients, all but 1 of whom had undergone prior mitral valve replacement. These patients were compared with a control cohort of 53 patients undergoing mitral valve re-replacement without annulus enlargement. The annulus was upsized by 18% ± 11% (compared with 16% ± 20% in controls, P = .5) using open balloon dilation in 4 patients, radial annular incisions in 5 patients, and patch augmentation of the aorto-mitral continuity in 7 patients. The mean valvar diameter Z score increased from -0.2 ± 1.3 to +1.1 ± 1.4 (P < .001), compared with an increase from -0.3 ± 1.2 to 0.9 ± 1.1 in controls (P < .001). During a mean follow-up of 6.5 ± 6.4 years, 4 of 12 patients required permanent pacing in the upsizing group (predominantly with patch augmentation), versus 5 of 53 patients in the control group. CONCLUSIONS: In children undergoing mitral valve replacement, various techniques can be used to upsize the mitral annulus and allow implantation of a larger prosthesis. There is a nontrivial risk of heart block with annulus upsizing, which deserves further study.

Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience.

BACKGROUND: Patients with severe pulmonary arterial hypertension (PAH) face significant morbidity and death as a consequence of progressive right heart failure. Surgical shunt placement between the left PA and descending aorta (Potts shunt) appears promising for PAH palliation in children; however, surgical mortality is likely to be unacceptably high in adults with PAH. METHODS: We describe a technique for transcatheter Potts shunt (TPS) creation by fluoroscopically guided retrograde needle perforation of the descending aorta at the site of apposition to the left PA to create a tract for deployment of a covered stent between these vessels. This covered stent-anchored by the vessel walls and surrounding tissue-serves as the shunt. RESULTS: TPS creation was considered in 7 patients and performed in 4. The procedure was technically successful in 3 patients; 1 patient died during the procedure as a result of uncontrolled hemothorax. One acute survivor, critically ill at the time of TPS creation, later died of comorbidities. The 2 mid-term survivors (follow-up of 10 and 4 months) are well at home, with symptomatic improvement and no late complications. The 3 candidate patients in whom the procedure was not performed died within 1 month of consideration, underscoring the tenuous nature of this population. CONCLUSIONS: TPS creation is feasible and may offer symptomatic relief to select patients with refractory PAH. Further study of this innovative approach is warranted.

Obstruction of the superior vena cava after neonatal extracorporeal membrane oxygenation: association with chylothorax and outcome of transcatheter treatment.

BACKGROUND: Obstruction of the superior vena cava is one of the potential complications of neonatal extracorporeal membrane oxygenation. Chylothorax is a known complication of surgery involving the thoracic cavity in children, and of extracorporeal membrane oxygenation. The aim of this study was to evaluate the association between chylothorax and superior vena cava obstruction after neonatal extracorporeal membrane oxygenation. METHODS AND RESULTS: Twenty-two patients diagnosed with superior vena cava obstruction at ≤ 6 months of age (median 1.8 months) after neonatal extracorporeal membrane oxygenation were compared with a randomly selected cohort of 44 neonatal extracorporeal membrane oxygenation patients without superior vena cava obstruction. Among patients with superior vena cava obstruction, 18 underwent extracorporeal membrane oxygenation for respiratory disease and four for cardiac insufficiency. Chylothorax was more prevalent among patients with superior vena cava obstruction than controls (odds ratio 9.4 [2.2-40], p = .01) and was associated with extension of obstruction into the left innominate vein. Patients with superior vena cava obstruction were supported by extracorporeal membrane oxygenation for a longer duration than controls. Nineteen patients with superior vena cava obstruction (86%) underwent transcatheter balloon angioplasty and/or stent implantation (median 7 days after diagnosis), which decreased the superior vena cava pressure and superior vena cava-to-right atrium pressure gradient and increased the superior vena cava diameter (all p < 0.001). There were no serious procedural adverse events. Six study patients died within 30 days of the diagnosis of superior vena cava obstruction (including three of nine with chylothorax), which did not differ from controls. During a median follow-up of 2.7 yrs, two additional patients died and nine underwent 14 superior vena cava reinterventions. CONCLUSIONS: Among neonates treated with extracorporeal membrane oxygenation, superior vena cava obstruction is associated with an increased risk of chylothorax. In neonates with chylothorax after extracorporeal membrane oxygenation, evaluation for superior vena cava obstruction may be warranted. Although mortality is high in this population, transcatheter treatment can relieve superior vena cava obstruction and facilitate symptomatic improvement.

Bare metal stenting for obstructed small diameter homograft conduits in the right ventricular outflow tract.

OBJECTIVE: Our aim was to assess acute hemodynamic changes with stent insertion, outcomes, and factors associated with increased longevity of stented small diameter homograft conduits. BACKGROUND: Right ventricle-to-pulmonary artery (RV-PA) homograft conduits are commonly used to palliate RV outflow tract obstruction. Bare metal stenting (BMS) and transcatheter pulmonary valve implantation have been shown to relieve obstructed larger diameter conduits and may delay surgical conduit reintervention. Less is known about BMS of small conduits. METHODS AND RESULTS: From 1992 to 2009, BMS was performed to relieve obstruction in 106 homograft conduits that were ≤12 mm at implant. The peak RV-PA gradient fell from 54.3 ± 17.4 mm Hg at baseline to 46.1 ± 15.2 mm Hg after balloon dilation alone and to 25.1 ± 11.4 mm Hg with stenting (all P < 0.001). Higher pre-BMS gradient and RV pressure were the only factors associated with higher post-BMS RV-PA gradient (≥30 mm Hg; both P < 0.001). There were no procedural deaths, two patients required surgical removal of embolized stents. At a median follow-up of 1.6 years, 83 conduits were replaced; freedom from conduit reoperation after BMS was 66% ± 5% at 1 year and 28% ± 5% at 3 years. Factors associated with shorter freedom from reoperation included implanted conduit diameter <10 mm (P = 0.009), higher post-stent RV-PA gradient (P = 0.026), and higher post-stent RV pressure (P < 0.01); only post-stent RV pressure remained significant on multivariable analysis (P < 0.001). CONCLUSION: BMS was acutely effective for the treatment of obstructed small diameter homograft conduits, with low morbidity. Prolongation of small diameter homograft conduit longevity with BMS may be useful in the lifetime management of conduit dysfunction in this patient population.

Modification of a stented bovine jugular vein conduit (melody valve) for surgical mitral valve replacement.

We report the use of a Melody valve as a surgical implant in the mitral position in 2 infants, one with severe mitral regurgitation and another with mitral stenosis, where other prostheses are not small enough to be implanted in the mitral position nor expandable as the child grows.

Cardiac medical conditions have become the leading cause of death in children with heart disease.

OBJECTIVE: Mortality among children with congenital and acquired heart disease has decreased significantly over the past decades. We sought to determine whether the underlying problems leading to death in these patients had changed over the past decade. METHODS: We reviewed medical records for 100 deaths of cardiac patients in 2004-2005 and 100 deaths in 1995-1996. Demographic, clinical, and procedural data as well as circumstances of death were collected. A consensus committee reviewed each case and sought to identify the condition leading to death. These conditions were classified as predominantly surgical or medical. RESULTS: General patient characteristics (age, gender, cardiac history, comorbidities, proportion of surgical patients) did not change significantly between the two time periods. However, in 1995-1996, 64% of deceased surgical patients had died within 30 days of surgery. This rate was nearly halved to only 38% by 2004-2005 (P= .003). Furthermore, the conditions leading to death changed significantly: 51% of patient deaths in 1995-1996 resulted from a surgical problem, 29% from a medical condition. This ratio was reversed in 2004-2005: Only 31% of patient deaths were due to a surgical problem, while 50% of deaths resulted from a medical condition (P= .005). The most common medical conditions resulting in death were pulmonary vein stenosis, pulmonary arterial hypertension, and primary myocardial failure. CONCLUSIONS: The proportion of deaths within 30 days of cardiac surgery decreased significantly over the past decade. While surgical causes accounted for the majority of these deaths in 1995-1996, most patient deaths in 2004-2005 resulted from cardiac medical causes.

Outcomes after stent implantation for the treatment of congenital and postoperative pulmonary vein stenosis in children.

BACKGROUND: Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid. METHODS AND RESULTS: We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases. CONCLUSIONS: Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

Effectiveness of screening for life-threatening chest pain in children.

OBJECTIVE: We sought to determine the incidence of sudden cardiac death among patients discharged from the cardiology clinic with presumed noncardiac chest pain (CP). METHODS: The records of children >6 years of age who presented to Children's Hospital Boston between January 1, 2000, and December 31, 2009, with a complaint of CP were reviewed for demographic features, clinical characteristics, resource utilization, and presumed diagnosis. Patients were searched for in the US National Death Index and Social Security Death Index. RESULTS: Data for a total of 3700 patients with CP (median age at evaluation: 13.4 years [range: 7-22.3 years]) were reviewed. The median follow-up period was 4.4 years (range: 0.5-10.4 years), for total of 17 886 patient-years of follow-up data. CP with exertion occurred in 1222 cases (33%), including 15 with associated syncope. A cardiac cause was determined in 37 cases; the remaining 3663 patients (99%) had CP of unknown (n = 1928), musculoskeletal (n = 1345), pulmonary (n = 242), gastrointestinal (n = 108), anxiety-related (n = 34), or drug-related (n = 4) origin. Emergency department visits for CP were documented for 670 patients (18%), and 263 patients (7%) had cardiology follow-up visits related to CP. There were 3 deaths, including 2 suicides and 1 spontaneous retroperitoneal hemorrhage. CONCLUSION: CP in children is a common complaint and rarely has a cardiac cause. Review of 1 decade of cardiology visits (nearly 18 000 patient years) revealed that no patient discharged from the clinic died as a result of a cardiac condition.

The evolving role of intraoperative balloon pulmonary valvuloplasty in valve-sparing repair of tetralogy of Fallot.

OBJECTIVE: The late morbidity of pulmonary regurgitation has intensified the interest in valve-sparing repair of tetralogy of Fallot. This study reviewed a single institution's experience with valve-sparing repair and investigated the role of intraoperative balloon valvuloplasty. METHODS: A retrospective chart review identified 238 patients who underwent complete primary repair of tetralogy of Fallot at less than 180 days of age. Patients were divided into 4 groups on the basis of the type of right ventricular outflow tract repair: transannular patch (n = 111), commissurotomy or standard rigid dilation (n = 71), intraoperative balloon pulmonary valvuloplasty (n = 32), or no valvar intervention (n = 24). RESULTS: Baseline demographic and anatomic factors differed among the 4 procedural groups with substantial overlap. Among 142 patients with pulmonary valve hypoplasia (z score, -2 to -4), 37% had valve-sparing repair. These patients had significant annular growth over time: z score increased 0.67 and 1.00 per year in the intraoperative balloon valvuloplasty (P < .001) and traditional valve-sparing (P < .001) groups, respectively. Rates of valve growth did not differ across groups, but z scores were 0.58 lower for the balloon valvuloplasty group across all time points (P = .001). Freedom from reintervention and surgery was shorter for the balloon valvuloplasty group than for the other groups (P < .001). CONCLUSIONS: Patients with tetralogy of Fallot and pulmonary valve hypoplasia who undergo valve-sparing repair with intraoperative balloon valvuloplasty have significant longitudinal annular growth, with normalization of annular size over time. Despite application in patients with more hypoplastic valves, balloon valvuloplasty resulted in similar valve growth and pulmonary regurgitation as traditional methods, but higher rates of reintervention. Although the precise role of this technique needs further refinement, it is likely to be most useful in patients with moderate pulmonary stenosis and moderate pulmonary valve dysplasia.