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Background: Children with congenital heart disease (CHD) are at risk for advanced heart failure (AHF). We sought to define the mortality and resource utilization in CHD-related AHF in children and young adults.Methods: All hospitalizations in the Pediatric Health Information System database involving patients ≤21 years old with a CHD diagnosis and heart failure requiring at least 7 days of continuous inotropic support between 2004 and 2015 were included. Hospitalizations including CHD surgery were excluded.Results: Of 465,482 CHD hospitalizations, AHF was present in 2,712 (0.6%) [58% infant, 55% male, 30% single ventricle]. AHF therapies frequently used included extracorporeal membrane oxygenation (ECMO) (15%) and cardiac transplant (16%). Ventricular assist device (VAD) support was rare (3%), although VAD use significantly increased from 2004 to 2015 (P < .0010). Hospital mortality in CHD with AHF was 26%, with higher mortality associated with single ventricle heart disease (OR 1.64, 95% CI 1.23-2.19; P = .0009), infancy (OR 1.71, 95% CI 1.17-2.5; P = .0057), non-white race (OR 1.28, 95% CI 1.04-1.59; p=0.0234), and chronic complex comorbidities (OR 1.76, 95% CI 1.34-2.30; P < .0001). Over the 11-year study period, despite the significant increase in CHD-related AHF hospitalizations (P < .0001), hospital mortality improved (P = .0011). Median hospital costs were $252,000, a 6-fold increase above those without AHF, and was primarily driven by hospital length of stay (P < .0001).Conclusion: AHF in children with CHD in uncommon but increasing and is associated with significant morbidity, mortality and resource utilization. Approximately 1 in 5 children do not survive to hospital discharge. Many risk factors for mortality may not be modifiable, and further study is needed to identify modifiable risk factors and improve care for this complex population. [ABSTRACT FROM AUTHOR]

Background: Infants with hypoplastic left heart syndrome (HLHS) are susceptible to pre-Norwood comorbidities (PCs) and complications. This study aimed to describe the effect of PCs on timing and survival of Norwood palliation (NP). Methods: A single-center, retrospective review of infants with HLHS who underwent initial NP between 2003 and 2010 was performed. PCs included intact atrial septum, ≥ moderate atrioventricular regurgitation (AVVR), no antenatal diagnosis, mitral stenosis/aortic atresia subtype, genetic abnormality, and prematurity. Complications included pre-NP mechanical ventilation, inotropic support, infection, arrhythmia, and end-organ injury. The primary outcome measure was survival after NP. Results: 113 patients were included with 78 (69%) patients having at least one PC and 61 (78%) of those patients having at least one complication. Patients with PCs underwent NP later than those without PCs (7 vs 6 days, P = .036) as well as when associated with a complication (8 vs 5 days, P < .001). Patients with PCs had similar post-Norwood hospital length of stay (P = .116) except when the PC occurred in conjunction with a complication (28 vs 21 days; P = .015). In-hospital mortality post-NP was 10% and interstage mortality was 15%. On multivariable analysis, ≥ moderate AVVR was associated with increased overall mortality (OR 2.8, 95% CI 1.3-6.2). Age at NP was not associated with mortality (P = .638). Conclusions: Although PCs are common in infants with HLHS, only ≥ moderate AVVR was associated with increased mortality in this single-center experience. Older age at NP was not a significant risk factor for interstage mortality. [Copyright &y& Elsevier]

OBJECTIVE: The purpose of this study was to describe patient characteristics and outcomes of heart failure (HF)-related intensive care unit (ICU) hospitalizations in children with cardiomyopathy (CM). METHODS AND RESULTS: A query of the Pediatric Health Information System database, a large administrative and billing database of 43 tertiary children's hospitals, was performed. A total of 17,309 HF-related ICU hospitalizations from 2005 to 2010 of 14,985 children <=18 years old were analyzed. Of those, 2,058 (12%) hospitalizations for CM-HF in 1,599 (11%) children were identified. Classification into CM subtypes was not possible owing to database limitations. The number of yearly CM-HF hospitalizations significantly increased during the study period (P = .036). Overall mortality was 11%, and cardiac transplantation occurred in 20% of hospitalizations. Mechanical circulatory support (MCS) was used in 261 (13%) of hospitalizations. Renal failure, MCS, respiratory failure, sepsis, and vasoactive medications were associated with mortality on multivariable analysis. Significant comorbidities associated with these hospitalizations included arrhythmias in 42%, renal failure in 13%, cerebrovascular disease in 6%, and hepatic impairment in 5%. CONCLUSIONS: HF-related ICU hospitalizations in children with cardiomyopathy are increasing. These children are at high risk for poor outcomes with an in-hospital mortality of 11%. [ABSTRACT FROM AUTHOR]

Objectives: Infective endocarditis is rare in children but potentially carries high mortality and morbidity. Few data exist regarding surgical therapy and the associated outcomes in children with infective endocarditis. The aim of the present study was to describe the characteristics and outcomes of children undergoing surgery for infective endocarditis. Methods: A retrospective review of all patients aged 21 years or younger diagnosed with definitive infective endocarditis at a single center from 1996 to 2010 was performed. Results: Of 76 identified patients with infective endocarditis (median age, 8.3 years; 73.9% boys), 46 patients (61%) required surgical intervention. Staphylococcus aureus was most commonly isolated (18 patients, 24%) followed by Streptococcus (17 patients, 22%). Common surgical indications included severe valvular insufficiency in 13 patients, septic embolization in 12, concomitant severe valvular insufficiency and ventricular dysfunction in 9, persistent vegetations in 9, and persistent bacteremia in 3. Although early surgery was performed within 7 days of diagnosis in 35 patients (76%), 25 (54%) underwent surgery within 3 days or less. The factors associated with surgery included the presence of ventricular dysfunction, left-sided vegetation, severe valvular insufficiency, septic embolization, and S aureus. Surgery within 3 days or less was associated with the presence of ventricular dysfunction and S aureus. Native valve repair was performed in 50% of patients with native-valve disease. Postoperatively, no septic embolization events occurred and recurrence was low (2%). The 1-, 5-, and 10-year survival was 98% ± 2%, 90% ± 8%, and 81% ± 11%, respectively. Conclusions: Children with infective endocarditis can undergo successful early surgical therapy with a low risk of septic embolization, recurrence, and operative mortality. [Copyright &y& Elsevier]

Objective: Few data are available on the frequency or importance of perioperative atrial tachycardia in infants. We hypothesized that atrial tachycardia in infants undergoing cardiac surgery is not rare and is associated with increased morbidity and mortality. Methods: From 2007 through 2010, 777 infants (median age, 1.8 months; interquartile range, 0.33–5.73) underwent cardiac surgery. Their medical records were reviewed for atrial tachycardia during the perioperative period. Results: Of the 777 patients, 64 (8.2%) developed atrial tachycardia. The independent risk factors for developing atrial tachycardia included surgical age 6 months or younger (odds ratio, 4.4; 95% confidence interval, 1.1–19.15), use of 3 or more inotropes (odds ratio, 2.9; 95% confidence interval, 1.4–6.2), and heterotaxy syndrome (odds ratio, 2.9; 95% confidence interval, 1.1–7.4). All-cause mortality in the atrial tachycardia group was increased (21.9% vs 7.2%, P < .001) during a median follow-up period of 14.6 months (interquartile range, 6.8–24.6), and atrial tachycardia was independently associated with decreased survival (hazard ratio, 1.9; 95% confidence interval, 1.1–3.8). Infants with perioperative atrial tachycardia had a longer hospital length of stay (32 vs 17 days, P < .001) and duration of inotrope use (10.5 vs 3.0 days, P < .001). A total of 57 patients received antiarrhythmic therapy, with propranolol the most common (n = 31). Among the survivors, 48 patients received outpatient antiarrhythmic therapy, which was successfully discontinued in 23 patients at a median duration of 14 months (interquartile range, 5.7–18.6) without recurrence. Conclusions: Atrial tachycardia is common in infants undergoing cardiac surgery and is independently associated with decreased survival. Among survivors, antiarrhythmic agents successfully controlled atrial tachycardia in most patients with a low recurrence risk after discontinuation. [Copyright &y& Elsevier]

Objectives: To describe the frequency, characteristics, and outcomes of heart failure-related emergency department (ED) visits in pediatric patients. We aimed to test the hypothesis that these visits are associated with higher admission rates, mortality, and resource utilization.Study Design: A retrospective analysis of the Nationwide Emergency Department Sample for 2010 of patients ≤18 years of age was performed to describe ED visits with and without heart failure. Cases were identified using International Classification of Disease, Ninth Revision, Clinical Modification codes and assessed for factors associated with admission, mortality, and resource utilization.Results: Among 28.6 million pediatric visits to the ED, there were 5971 (0.02%) heart failure-related cases. Heart failure-related ED patients were significantly more likely to be admitted (59.8% vs 4.01%; OR 35.3, 95% CI 31.5-39.7). Among heart failure-related visits, admission was more common in patients with congenital heart disease (OR 5.0, 95% CI 3.3-7.4) and in those with comorbidities including respiratory failure (OR 78.3, 95% CI 10.4-591) and renal failure (OR 7.9, 95% CI 1.7-36.3). Heart failure-related cases admitted to the hospital had a higher likelihood of death than nonheart failure-related cases (5.9% vs 0.32%, P < .001). Factors associated with mortality included respiratory failure (OR 4.5, 95% CI 2.2-9.2) and renal failure (OR 7.8, 95% CI 2.9-20.7). Heart failure-related ED visits were more expensive than nonheart failure-related ED visits ($1460 [IQR $861-2038] vs $778 [IQR $442-1375] [P < .01].) CONCLUSIONS: Heart failure-related visits represent a minority of pediatric ED visits but are associated with increased hospital admission and resource utilization. [ABSTRACT FROM AUTHOR]

Background: Childhood hypertension is increasingly common and likely contributes to the premature development of cardiovascular and renal disease. Early detection of hypertensive cardiac damage may allow for prompt therapy and reduce the risk of late morbidity and mortality. While left ventricular mass index (LVMI) has been used as a surrogate of hypertensive cardiac damage in children, its prognostic and diagnostic value is not completely understood in this population.Objective: To test the hypothesis that strain assessment using automated function imaging (AFI) detects cardiac changes in hypertensive children earlier than standard 2D echocardiographic techniques.METHODS: A cross-sectional study of hypertensive children < 19 years old between October 2010 and May 2012 was performed. Any patient who was newly diagnosed with hypertension and underwent echocardiographic examination including standard 2D, Doppler, and automated function imaging (AFI) modalities was included. Global peak longitudinal systolic strain (GLPSS) was measured using a 17 segment model of the left ventricle. All echocardiographic measures were compared to normative reference values for age.Results: Twenty four patients (mean age 14.6 ± 2.7 years, 67% male) were identified. Mean systolic and diastolic blood pressure index was 1.1 ± 0.1 and 0.9 ± 0.1, respectively. Mean LVMI did not significantly differ from 95th-percentile normative values (41.6 ± 11.2 g/m2.7 vs. 39.3 ± 1.5 g/m2.7, p = 0.328). Mean GLPSS was significantly lower than 5th-percentile normative values (-17.6 ± 2.5 vs. -19.5 ± 0.42, p = 0.001) and were decreased across all myocardial segments. Left atrial volume was significantly higher than reported normal values in children (27.6 ± 7.3 ml/m2 vs. 22.0 ± 6.0 ml/m2, p = 0.002). No patient exhibited abnormal shortening or ejection fraction, mitral E-wave to A-wave ratio, or lateral mitral E-wave to e’-wave ratio.Conclusions: A population of unselected, newly diagnosed hypertensive adolescents had atypically low GLPSS and increased left atrial volume. The use of GLPSS measured by AFI may be a more sensitive marker of early myocardial remodeling in hypertensive children and routine measurement of GLPSS should be considered. [ABSTRACT FROM AUTHOR]

SAR445088 is an anti-C1s humanized monoclonal antibody that inhibits activated C1s in the proximal portion of the classical complement system and has the potential to provide clinical benefit in the treatment of complement-mediated diseases. A phase I, first-in-human, double-blind, randomized, placebo-controlled, dose-escalation trial of single and multiple doses of SAR445088 was conducted in 93 healthy participants to evaluate the safety, tolerability, and pharmacokinetic (PK) and pharmacodynamic (PD) profiles. Single (intravenous [i.v.] and subcutaneous [s.c.]) ascending doses (SAD) and multiple (s.c.) ascending doses (MAD) of SAR445088 were well-tolerated. The PK of SAR445088 was characterized by slow absorption after the s.c. dose and a long half-life (mean terminal half-life [t 1/2 ] 8-15 weeks). Two PD assays were used to measure inhibition of the classical complement pathway (CP): Wieslab CP and complement mediated hemolytic capacity (CH50). The estimated half-maximal inhibitory concentration (IC 50 ) and 90% inhibitory concentration (IC 90 ) for the Wieslab CP assay were 96.4 and 458 μg/ml, respectively, and 16.6 and 57.0 μg/ml, respectively, for the CH50 assay. In summary, SAR445088 was well-tolerated and had favorable PK and PD profiles after SAD (i.v. or s.c.) and MAD (s.c.) in humans. These findings warrant further clinical investigations in patients with classical complement-mediated disorders., (© 2023 Sanofi. Clinical and Translational Science published by Wiley Periodicals LLC on behalf of American Society for Clinical Pharmacology and Therapeutics.)

Background: Paracorporeal continuous-flow ventricular assist devices (PCF VAD) are increasingly used in pediatrics, yet PCF VAD resource utilization has not been reported to date., Methods: Pediatric Interagency Registry for Mechanically Assisted Circulatory Support (PediMACS), a national registry of VADs in children, and Pediatric Health Information System (PHIS), an administrative database of children's hospitals, were merged to assess VAD implants from 19 centers between 2012 and 2016. Resource utilization, including hospital and intensive care unit length of stay (LOS), and costs are analyzed for PCF VAD, durable VAD (DVAD), and combined PCF-DVAD support., Results: Of 177 children (20% PCF VAD, 14% PCF-DVAD, 66% DVAD), those with PCF VAD or PCF-DVAD are younger (median age 4 [IQR 0-10] years and 3 [IQR 0-9] years, respectively) and more often have congenital heart disease (44%; 28%, respectively) compared to DVAD (11 [IQR 3-17] years; 14% CHD); p < 0.01 for both. Median post-VAD LOS is prolonged ranging from 43 (IQR 15-82) days in PCF VAD to 72 (IQR 55-107) days in PCF-DVAD, with significant hospitalization costs (PCF VAD $450,000 [IQR $210,000-$780,000]; PCF-DVAD $770,000 [IQR $510,000-$1,000,000]). After adjusting for patient-level factors, greater post-VAD hospital costs are associated with LOS, ECMO pre-VAD, greater chronic complex conditions, and major adverse events (p < 0.05 for all). VAD strategy and underlying cardiac disease are not associated with LOS or overall costs, although PCF VAD is associated with higher daily-level costs driven by increased pharmacy, laboratory, imaging, and clinical services costs., Conclusion: Pediatric PCF VAD resource utilization is staggeringly high with costs primarily driven by pre-implantation patient illness, hospital LOS, and clinical care costs., Competing Interests: Conflict of interest disclosure The PediMACS database is funded by a contract grant (HHSN268201100025C) from the National Heart, Lung, and Blood Institute. JWR has served as a consultant for Novartis, Abiomed, Bayer, Amgen. DLSM has served as a consultant for Abbott, Syncardia, and Berlin Heart. The other authors have no relevant disclosures to report., (Copyright © 2021 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: There is no nonparenteral medication for the rapid termination of paroxysmal supraventricular tachycardia., Objectives: The purpose of this study was to assess the efficacy and safety of etripamil nasal spray, a short-acting calcium-channel blocker, for the rapid termination of paroxysmal supraventricular tachycardia (SVT)., Methods: This phase 2 study was performed during electrophysiological testing in patients with previously documented SVT who were induced into SVT prior to undergoing a catheter ablation. Patients in sustained SVT for 5 min received either placebo or 1 of 4 doses of active compound. The primary endpoint was the SVT conversion rate within 15 min of study drug administration. Secondary endpoints included time to conversion and adverse events., Results: One hundred four patients were dosed. Conversion rates from SVT to sinus rhythm were between 65% and 95% in the etripamil nasal spray groups and 35% in the placebo group; the differences were statistically significant (Pearson chi-square test) in the 3 highest active compound dose groups versus placebo. In patients who converted, the median time to conversion with etripamil was <3 min. Adverse events were mostly related to the intranasal route of administration or local irritation. Reductions in blood pressure occurred predominantly in the highest etripamil dose., Conclusions: Etripamil nasal spray rapidly terminated induced SVT with a high conversion rate. The safety and efficacy results of this study provide guidance for etripamil dose selection for future studies involving self-administration of this new intranasal calcium-channel blocker in a real-world setting for the termination of SVT. (Efficacy and Safety of Intranasal MSP-2017 [Etripamil] for the Conversion of PSVT to Sinus Rhythm [NODE-1]; NCT02296190)., (Copyright © 2018 Milestone Pharmaceuticals Inc. Published by Elsevier Inc. All rights reserved.)

Background: Few data exist on resource utilization with pediatric ventricular assist devices (VADs). We tested the hypothesis that device type and adverse events are associated with increased resource utilization in pediatric patients supported with VADs., Methods and Results: The Pediatric Interagency Registry for Mechanically Assisted Circulatory Support, a national registry of VADs in patients <19 years old, and the Pediatric Health Information System, an administrative database, were merged. Univariate analysis was performed assessing the association of all factors with the total cost and length of stay first. Significant variables ( P <0.05) were subjected to multivariable analysis. The study included 142 patients from 19 centers with VAD implants from October 2012 to June 2016. The median age was 9 years (interquartile range [IQR] 2-15), 84 (59%) supported with a continuous-flow VAD. Overall median hospital costs were $750 000 (IQR $539 000 to $1 100 000) with a median hospital length of stay of 81 days (IQR 54-128). On multivariable analysis, device type and postoperative complications were not associated with resource utilization. Factors associated with increased costs included patient age, lower-volume VAD center, being intubated, being on extracorporeal membrane oxygenation, number of complex chronic medical conditions, and length of stay. Among continuous-flow VAD patients, discharge to home before transplant versus remaining hospitalized was associated with lower hospital costs (median $600 000 [IQR $400 000 to $820 000] versus median $680 000 [IQR $500 000 to $970 000], P =0.03)., Conclusion: VADs in pediatric patients are associated with high resource utilization. Increased resource utilization was associated with lower-volume VAD centers, disease severity at VAD implantation, and the presence of complex chronic medical conditions. Further study is needed to develop cost-effective strategies in this complex population., (© 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.)

Background: The purpose of this study was to describe the prevalence, characteristics, and outcomes in pediatric patients with chromosomal anomalies (CA) undergoing orthotopic heart transplantation (OHT)., Methods: A query of the database of the Pediatric Health Information System, a large administrative and billing database of 43 tertiary children's hospitals, was performed for the Years 2004 to 2016. Pediatric patients who received OHT were analyzed based on presence and type of CA. CA analyzed included: Down syndrome (DS); Turner syndrome (TS)/gonadal dysgenesis; conditions due to anomaly of unspecified chromosome; autosomal deletion; microdeletion; and autosomal anomaly. Healthcare-associated charge analysis during hospitalization for OHT and survival after OHT were assessed., Results: A total of 3,080 hospitalizations were identified in which OHTs were performed. Of these OHTs, 64 (2.1%) were performed in patients with a concomitant diagnosis of CA. The presence of CA did not confer a higher risk of in-hospital mortality after OHT (odds ratio 1.2 [0.5 to 3.2], p = 0.651). Differences in in-hospital mortality between different types of CA, including DS and TS, did not reach statistical significance. Survival at 1-year post-OHT was similar in patients with CA compared to those without CA (p = 0.248). Length of stay after OHT was longer in patients with CA: 76 (interquartile range [IQR] 76 to 142 days vs 49 [IQR 21 to 98] days) (p < 0.001), respectively. Overall adjusted hospital charges were significantly higher in the CA group: $1.2 million (IQR $740,000 to $2.2 million) vs $792,000 (IQR $425,000 to $1.5 million] (p < 0.001), respectively., Conclusions: CA is present in ~2% of pediatric patients undergoing OHT. The presence of CA was not associated with increased mortality in pediatric patients undergoing OHT. Limitations of this study include the small number of patients available for analysis and a likely highly selective cohort of patients with CA., (Copyright © 2018 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Aim: Perhexiline (PEX), being developed to treat hypertrophic cardiomyopathy, is toxic at levels above the therapeutic range. Plasma level monitoring is therefore essential. The absence of a UV-absorbing chromophore has in the past required quantitative analysis of PEX in plasma using lengthy derivatization methods, followed by HPLC and fluorescence detection. The routine and urgent analysis of a large number of patient plasma samples necessitates faster and reliable analytical methodology., Results: An LC-MS/MS method, using two novel internal standards, has been validated for the quantitative measurement of PEX and its major hydroxy metabolites in human plasma., Conclusion: The assay has been applied to therapeutic drug monitoring (TDM), where PEX and the ratio of the drug to cis-hydroxy perhexiline, were measured at designated intervals.

Background: Although the risk of thoracic aortic dissection and rupture (TAD) is well-known in bicuspid aortic valve (BAV), the risk of TAD in other congenital heart diseases (CHD), particularly conotruncal lesions like tetralogy of Fallot (TOF), truncus arteriosus, D-transposition of the great arteries (D-TGA), and double outlet right ventricle is currently unknown. The primary purpose of this study was to describe TAD in conotruncal CHD, and the secondary purpose was to explore whether an association exists between TAD and conotruncal CHD., Methods and Results: Using the Texas Inpatient Public Use Data File, an administrative database of all Texas hospitalizations, including >37.9 million hospitalizations from January 1999 through June 2012, 12,016 cases of TAD and 214 cases of TAD in CHD were identified. The most common lesions were BAV (42%), atrial septal defect (21%), aortic coarctation (7%), ventricular septal defect (6%), and patent ductus arteriosus (4%). Three patients with TOF, 2 with D-TGA, and 1 with truncus arteriosus were admitted with TAD. An exploratory case-control study in patients older than 1 year using multilevel logistic regression models to evaluate the association between CHD and TAD that controlled for known TAD risk factors demonstrated a significant association between TAD and BAV (OR 10, 95% CI 8.2-13) but not coarctation of the aorta or any conotruncal lesion., Conclusions: TAD in conotruncal CHD is exquisitely rare. In our hospitalized population, there was no increased occurrence of TAD in conotruncal CHD above what would be expected in the rest of the hospitalized population., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

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., (Copyright © 2010 Wiley-Liss, Inc.)

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., ((c) 2009 Wiley-Liss, Inc.)