Usefulness of PREDIC3T Case Type Risk Category in the CRISP Registry.

Procedural risk in Congenital Cardiac Catheterization (PREDIC3T) was recently reported as the contemporary procedure-type risk metric by the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. The usefulness of this metric has not been evaluated elsewhere. The CRISP registry of Congenital Cardiovascular Interventional Study Consortium (CCISC) data set was analyzed. The study period was 14 years (2009 to 2022). The primary outcome was significant adverse event (SAE). Cases were assigned to the 6 PREDIC3T risk categories. Univariate and multivariable logistic regression models were used to evaluate the association between PREDIC3T and the primary outcome. The model discriminative performance was evaluated by the c-statistic. In a total of 64,419 enrolled cases, PREDIC3T case types were assigned in 59,822 cases (93%). The frequency for PREDIC3T category was 0 = 7,494 (12.5%), 1 = 16,932 (28.3%), 2 = 17,023 (28.5%), 3 = 9,885 (16.5%), 4 = 4,403 (7.4%), and 5 = 4,085 (6.8%). SAE was observed in 2,474 cases (4.1%). The SAE rates for category were 0 = 1.0%, 1 = 2.3%, 2 = 4.0%, 3 = 6.2%, 4 = 8.2%, and 5 = 9.0%. In a multivariable model, PREDIC3T case type risk category (odds ratios for category: 0 = 0.49, 1 = 1.00, 2 = 1.40, 3 = 2.06, 4 = 2.79, and 5 = 3.15; p <0.001) were significantly associated with SAE (c-statistic of 0.707) after adjusting for age, preprocedural inotropic support and systemic illness, low systemic saturation, high pulmonary vascular resistance, and the use of general anesthesia. The PREDIC3T case type risk category was associated with the risk of SAE in the CRISP registry data set and appeared to be a useful procedural risk classification tool.

3-year follow-up of a prospective, multicenter study of the Amplatzer Piccolo™ Occluder for transcatheter patent ductus arteriosus closure in children ≥ 700 grams.

OBJECTIVE: This study describes 3-year follow-up of 200 infants weighing ≥ 700 grams who underwent transcatheter patent ductus arteriosus (PDA) closure with the Amplatzer Piccolo™ Occluder. STUDY DESIGN: Between June 2017 and February 2019, 200 children were enrolled in this U.S. study (NCT03055858). PDA closure, survival, and device- or procedure-related events were evaluated. A total of 156 of the available 182 patients (86%) completed the study. RESULTS: The implant success rate was 95.5% (191/200). At 3 years, PDA closure was observed in 100% (33/33) of patients. Survival was >95% with 9 reported deaths. No deaths were adjudicated as device- or procedure-related. Notable events included aortic obstruction (2) requiring stent placement and tricuspid regurgitation (5), for which no interventions were required. CONCLUSIONS: This follow-up study demonstrates high rates of PDA closure, low serious complication rates, and survival > 95% at 3 years. The Amplatzer Piccolo™ Occluder is a safe and effective therapy for PDA treatment in premature infants. CLINICALTRIALS: gov identifier: NCT0305585.

Combination Chemotherapy in Severe Pulmonary Vein Stenosis-A Case Series.

Pulmonary vein stenosis results from a proliferative process that leads to the progressive obstruction of venous return to the left atrium. It is often resistant to catheterization and surgical based interventions and is frequently fatal when encountered in its severe form. Here, we describe three patients with severe, primary pulmonary vein stenosis that was progressing despite aggressive conventional management strategies. All three patients were initiated on combination chemotherapy with imatinib and sirolimus, drugs which have been previously shown to independently have potential benefit against PVS. Soon after the initiation of these therapies, all three patients experienced a stabilization of their disease process and clinical improvement. All three patients remain alive, with tolerable side effects from the medications. Although early in our experience and with only a small number of patients, combination chemotherapy with imatinib and sirolimus shows promise and merits further investigation as a therapeutic option for this aggressive disease.

In-Silico and In-Vitro Analysis of the Novel Hybrid Comprehensive Stage II Operation for Single Ventricle Circulation.

Single ventricle (SV) anomalies account for one-fourth of all congenital heart disease cases. The existing palliative treatment for this anomaly achieves a survival rate of only 50%. To reduce the trauma associated with surgical management, the hybrid comprehensive stage II (HCSII) operation was designed as an alternative for a select subset of SV patients with the adequate antegrade aortic flow. This study aims to provide better insight into the hemodynamics of HCSII patients utilizing a multiscale Computational Fluid Dynamics (CFD) model and a mock flow loop (MFL). Both 3D-0D loosely coupled CFD and MFL models have been tuned to match baseline hemodynamic parameters obtained from patient-specific catheterization data. The hemodynamic findings from clinical data closely match the in-vitro and in-silico measurements and show a strong correlation (r = 0.9). The geometrical modification applied to the models had little effect on the oxygen delivery. Similarly, the particle residence time study reveals that particles injected in the main pulmonary artery (MPA) have successfully ejected within one cardiac cycle, and no pathological flows were observed.

Incidence, Predictors, and Outcomes of Cardiac Perforation During Pediatric Cardiac Catheterization: a Retrospective Observational Study from the Congenital Cardiac Interventional Study Consortium (CCISC).

INTRODUCTION: Cardiac perforation is a rare life-threatening complication of cardiac catheterization. There is very little published literature detailing risk factors for cardiac perforation and outcomes from this complication in children. MATERIALS AND METHODS: This was a retrospective study analyzing the cardiac catheterization case registry of the Congenital Cardiovascular Interventional Study Consortium. Children aged < 18 years were included during the study period of 9 years (January 2009-December 2017). The primary outcome measures were incidence of cardiac perforation, risk factors for and outcomes of patients who experience cardiac perforation during cardiac catheterization. RESULTS: Cardiac perforation occurred in 50 patients from a total of 36,986 (0.14%). Cardiac perforation was more likely to occur in younger, smaller patients undergoing urgent/emergent and interventional procedures (p < 0.01). Cardiac peroration risk was significantly different across diagnostic and procedure categories (p < 0.01). Higher CRISP score (Area Under Curve [AUC] = 0.87), lower age, and procedure category (radiofrequency perforation of pulmonary valve, AUC = 0.84) were independent predictors of cardiac perforation. Cardiac perforation was associated with a significantly higher rate of mortality (14%), further emergency procedure (42%), ECMO (14%), and cardiac arrest (6%), p < 0.01. CONCLUSIONS: Cardiac perforation during cardiac catheterization is a life-threatening complication with a range of associated secondary complications. Higher CRISP score, lower age, and radiofrequency perforation of pulmonary valve are independent predictors.

Clinical update on the hybrid comprehensive stage II operation.

Objective: We previously described the hybrid comprehensive stage II operation as an alternate surgical procedure for a subset of patients with single ventricle congenital heart disease with adequate native ascending aortic outflow. Here we provide a clinical update on the 4 patients who have undergone this procedure. Methods: After undergoing a hybrid approach to the stage I Norwood palliation, the hybrid comprehensive stage II procedure was performed with an incision to the main pulmonary artery (PA), dilation of the ductal stent, creation of a stented baffle between the branch PAs, and a bidirectional Glenn connection. With this approach, dissection of the distal arch and creation of a Damus-Kaye-Stansel anastomosis was avoided. A standard Fontan procedure was planned after the usual period of growth. Results: The first patient, who had trisomy 21 and elevated PA pressures, died postoperatively due to left PA thrombosis. The subsequent 3 patients survived the procedure and remain clinically well. All have required catheterizations for reintervention on their stented intrapulmonary baffles and ductal arches, and all have undergone successful completion of their Fontan procedures. Conclusions: The hybrid comprehensive stage II is a feasible, less complex alternative to the conventional comprehensive stage II operation in a subset of patients with single ventricle physiology. Early postoperative anticoagulation therapy to avoid PA thrombosis is recommended, and restenting of the ductal arch is anticipated. Although the long-term consequences of separate outflow tracts supplying the upper and lower body is unknown, the 3 surviving patients with this circulation are doing well with their Fontan circulation at midterm follow-up.

Computational fluid dynamics investigation of the novel hybrid comprehensive stage II operation.

Background: The hybrid comprehensive stage 2 (HCS2) procedure is a novel palliative operation applicable to a select subset of single ventricle patients with adequate native antegrade aortic flow to the upper body. Flow to the descending aorta, through the pulmonary outlet and ductal arch, is influenced by a stented intrapulmonary baffle connecting the branch pulmonary arteries. We used computational fluid dynamics (CFD) to elucidate the hemodynamic characteristics of this reconstruction. Methods: We used multiscale CFD analysis of a synthetic, patient-derived HCS2 anatomic configuration with unsteady laminar flow conditions and a non-Newtonian blood model to quantify the resultant hemodynamics. The 3-dimensional CFD model was coupled to a 0-dimensional lumped parameter model of the peripheral circulation to determine the required boundary conditions. Results: For the specific anatomy studied, the intrapulmonary baffle did not obstruct flow from the pulmonary trunk to ductal arch as long as the distance between the anterior pulmonary artery wall and baffle wall exceeded ∼7 mm. Vortex shedding off of the baffle wall did not develop, because of the short distance to the ductal arch. The stented baffle experienced significantly uneven "inward" loading from the systemic side. Pulmonary outlet flow separation distal to the baffle produced a low-speed recirculation region. Conclusions: Hemodynamic patterns in this complex anatomy are generally favorable. Low flow recirculation could be mitigated by preoperative shape optimization. Calculated inward stresses on the pulmonary baffle can be used in the future to study baffle stent deformation, which is expected to be small.

Amplatzer Piccolo Occluder clinical trial for percutaneous closure of the patent ductus arteriosus in patients ≥700 grams.

OBJECTIVES: Characterize the safety and effectiveness of the Amplatzer Piccolo Occluder for patent ductus arteriosus (PDA) closure. BACKGROUND: The presence of a hemodynamically significant PDA has been associated with an increased risk of morbidity and mortality in children born premature. METHODS: This was a single arm, prospective, multicenter, non-randomized study to evaluate the Amplatzer Piccolo Occluder to treat PDA in patients ≥700 g. From June 2017 to February 2019, 200 patients were enrolled at nine centers, with 100 patients weighing ≤2 kg. Primary effectiveness endpoint was the rate of PDA closure at 6-month follow-up. Primary safety endpoint was the rate of major complications through 6 months. Secondary endpoint was rate of significant pulmonary or aortic obstruction through 6 months' follow-up. RESULTS: The implant success rate was 95.5% (191/200) overall and 99% in patients ≤2 kg (99/100). The primary effectiveness endpoint was achieved in 99.4% of implanted patients. Four patients experienced a primary safety endpoint event (2 transfusions, 1 hemolysis, and 1 aortic obstruction). There were no branch pulmonary artery obstructions. Five patients, all ≤2 kg, were noted to have worsening of tricuspid regurgitation (TR) after the procedure. None of the TR incidences manifested clinically. The Amplatzer Piccolo Occluder received FDA approval in January 2019 and became the first device approved for PDA closure in patients ≥700 g. CONCLUSIONS: This study supports the safety and effectiveness of the Amplatzer Piccolo Occluder, particularly in patients between 700 g and 2 kg where there is currently a significant unmet need in the United States. ClinicalTrials.gov identifier: NCT03055858.

A Model for Assessment of Catheterization Risk in Adults With Congenital Heart Disease.

The purpose of this study was to define the risk for adults with congenital heart disease who underwent cardiac catheterization and to propose a precatheterization risk scoring system. Data were prospectively collected using a multicenter registry of the Congenital Cardiovascular Interventional Study Consortium. The occurrence of serious adverse events (SAE) was correlated with 12 predefined variables. Catheterization RISk in Adult patients (CRISA) score was derived using multivariate logistic regression with backward elimination model selection method. The CRISA score was compared with the American Society of Anesthesiology score and a consensus-derived, 20-point risk score based on their ability to predict SAE. From June 2008 to September 2017, 300 adjudicated SAE's occurred in 7317 catheterization procedures (overall SAE rate 4.1%) performed in adults over 18 years of age at 27 contributing centers. Nine of the 12 tested variables were ultimately included in the CRISA score. CRISA score positively correlated with risk of SAE, and was superior to American Society of Anesthesiology and the 20-point risk score in predicting SAE. Minimal (CRISA score 0 to 2), low (3 to 7), moderate (8 to 10) and high (≥11) risk categories were identified, corresponding to 0.5%, 3.2%, 7.9%, and 16.7% risk of SAE, respectfully. In conclusion, the CRISA score reliably predicts risk of SAE in adults with congenital heart disease who underwent cardiac catheterization and may be useful for preprocedural risk assessment.

Validation and refinement of the catheterization RISk score for pediatrics (CRISP score): An analysis from the congenital cardiac interventional study consortium.

OBJECTIVES: To externally validate the CRISP score, and determine if refinements might improve clinical utility. BACKGROUND: The CRISP score estimates risk of serious adverse events (SAEs) for pediatric catheterization. METHODS: Pediatric (age < 18) procedures reported to the Congenital Cardiovascular Interventional Study Consortium registry from 05/08 to 09/17 (n = 29,830, 27 centers) were divided into a development dataset of 14,784 earlier procedures, and a validation dataset of 15,046 more recent procedures. The development dataset was used to refit the original CRISP model, and to develop a revised(r) CRISP score, consisting of entirely pre-procedurally collected data. The validation dataset was then used to compare model fit and risk prediction between CRISP, rCRISP and two existing risk scores using Akaike's (AIC), Schwarz's (BIC) Bayes Information Criteria, -log Likelihood (N2LL), area under the receiver operator curve and chi-square goodness-of-fit statistic (across 5 risk categories). RESULTS: Overall 4.31% of patients experienced at least one SAE with frequency increasing from 1.08% in CRISP category 1 to 27.34% in category 5. Both CRISP and rCRISP (entirely pre-procedural) predicted risk of SAEs well, with observed to predicted ratios ranging from 0.71 to 1.18 across the 5 risk categories. Compared to the original CRISP score, rCRISP demonstrated less optimal model fit (higher AIC, BIC, and N2LL) but similar risk prediction (C-statistic = 0.71 vs. 0.70; chi-squared statistic = 6.77 vs. 6.85). CONCLUSION: The CRISP score accurately predicts procedural risk. With minor modifications, the revised version (rCRISP) performed well with arguably greater clinical utility as an entirely preprocedural risk model.

An unusual case of coronary artery compression that did not preclude successful transcatheter pulmonary valve placement.

During transcatheter pulmonary valve placement, coronary compression observed during simultaneous right ventricular outflow tract angioplasty and coronary angiography typically contraindicates valve implantation. We present a unique patient with tetralogy of Fallot who underwent successful transcatheter Melody valve placement despite coronary compression observed during right ventricular outflow tract balloon angioplasty.

Risk Factors for an Elevated Ventricular End-Diastolic Pressure Prior to the Fontan Operation.

Systemic ventricular end-diastolic pressure (SVEDP) is an important determinant of pulmonary artery pressure in those with a Fontan circulation. Predictors of an elevated SVEDP have been incompletely identified in this population. All who underwent the Fontan operation at our center between 1/2009 and 12/2013 were retrospectively identified. SVEDP at the pre-Fontan catheterization and other patient variables were extracted. We identified 61 patients. Pre-Fontan SVEDP was positively associated with systemic ventricular systolic pressure (ß = 0.4, p = 0.004), aortic systolic pressure (ß = 0.3, p = 0.007), aortic mean pressure (ß = 0.3, p = 0.02), and decreased ventricular systolic function (p = 0.03). Compared to those with pre-Fontan SVEDP ≤ 7 mmHg, patients with SVEDP > 7 mmHg had higher average ventricular systolic pressure (85.0 ± 7.5 vs. 78.7 ± 8.3 mmHg, p = 0.003), higher average descending aorta mean pressure (62.4 ± 4.9 vs. 58.6 ± 8.1 mmHg, p = 0.03), and a higher incidence of decreased ventricular systolic function (36 vs. 15%, p = 0.07). For those with a systemic right ventricle, the SVEDP decreased significantly from the pre-Stage 2 to pre-Fontan measurements (8.7 ± 2.6 vs. 7.3 ± 2.0 mmHg, p = 0.02), but not for those with a systemic left ventricle (7.8 ± 2.0 vs. 7.2 ± 1.8 mmHg, p = 0.3). At pre-Fontan catheterization, decreased ventricular systolic function and markers of systemic afterload were positively associated with the SVEDP. SVEDP decreased significantly after Stage 2 for those with a systemic right ventricle, but not for those with a systemic left ventricle; the systemic right ventricle may be particularly vulnerable to pre-Stage 2 volume loading.

Modeling Major Adverse Outcomes of Pediatric and Adult Patients With Congenital Heart Disease Undergoing Cardiac Catheterization: Observations From the NCDR IMPACT Registry (National Cardiovascular Data Registry Improving Pediatric and Adult Congenital Treatment).

BACKGROUND: Risk standardization for adverse events after congenital cardiac catheterization is needed to equitably compare patient outcomes among different hospitals as a foundation for quality improvement. The goal of this project was to develop a risk-standardization methodology to adjust for patient characteristics when comparing major adverse outcomes in the NCDR's (National Cardiovascular Data Registry) IMPACT Registry (Improving Pediatric and Adult Congenital Treatment). METHODS: Between January 2011 and March 2014, 39 725 consecutive patients within IMPACT undergoing cardiac catheterization were identified. Given the heterogeneity of interventional procedures for congenital heart disease, new procedure-type risk categories were derived with empirical data and expert opinion, as were markers of hemodynamic vulnerability. A multivariable hierarchical logistic regression model to identify patient and procedural characteristics predictive of a major adverse event or death after cardiac catheterization was derived in 70% of the cohort and validated in the remaining 30%. RESULTS: The rate of major adverse event or death was 7.1% and 7.2% in the derivation and validation cohorts, respectively. Six procedure-type risk categories and 6 independent indicators of hemodynamic vulnerability were identified. The final risk adjustment model included procedure-type risk category, number of hemodynamic vulnerability indicators, renal insufficiency, single-ventricle physiology, and coagulation disorder. The model had good discrimination, with a C-statistic of 0.76 and 0.75 in the derivation and validation cohorts, respectively. Model calibration in the validation cohort was excellent, with a slope of 0.97 (standard error, 0.04; P value [for difference from 1] =0.53) and an intercept of 0.007 (standard error, 0.12; P value [for difference from 0] =0.95). CONCLUSIONS: The creation of a validated risk-standardization model for adverse outcomes after congenital cardiac catheterization can support reporting of risk-adjusted outcomes in the IMPACT Registry as a foundation for quality improvement.

Prediction of adverse events after catheter-based procedures in adolescents and adults with congenital heart disease in the IMPACT registry.

AIMS: We sought to identify factors associated with major adverse events (MAE) after cardiac catheterization in adolescents and adults with congenital heart disease (CHD), and create the first model to individualize risk discussions in this growing population. METHODS AND RESULTS: Improving Pediatric and Adult Congenital Treatment (IMPACT), a National Cardiovascular Data Registry, contains congenital catheterization data from over 87 hospitals in the United States. Demographics, pre-procedure, and procedural variables were collected for patients over age 10. Multivariable logistic regression was used to identify significant predictors of MAE, a composite of death, urgent surgery or procedure due to a catheterization complication, transfusion, embolic stroke, tamponade, extracorporeal membrane oxygenation or ventricular assist device placement, and device embolization, malposition or thrombosis requiring surgical intervention. A risk score was built based on the effect sizes of each predictor and validated in a split sample. A MAE occurred in 686 (2.5%) of the 27 293 index procedures meeting inclusion criteria. The independent multivariate predictors of MAE were older age, pre-procedural anticoagulation use, renal disease, lower haemoglobin, lower oxygen saturation, non-elective procedure, higher index procedure risk and having had no prior cardiac procedures. Being underweight or overweight had borderline significance and was added to the model. The C-statistic for the model was robust at 0.787 in the derivation and 0.773 in the validation cohort. CONCLUSION: The factors predicting adverse events after cardiac catheterization in adolescents and adults with CHD are different than in the general population. Validation of this model in other national or multi-institutional datasets is the next step.

Superior Vena Cava Banding to Facilitate Unilateral Bidirectional Glenn Operation in Patients With Single Ventricle Heart Disease and Bilateral Superior Caval Veins.

Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.

Fontan Fenestration Closure Prior to Posterior Spinal Fusion in Patients With Single-Ventricle Heart Disease.

STUDY DESIGN: Case series. OBJECTIVE: To describe transcatheter closure of the Fontan fenestration prior to posterior spinal fusion in two children to prevent paradoxical venous air embolism during the operation. SUMMARY OF BACKGROUND DATA: Scoliosis is common among patients with single-ventricle congenital heart disease who have undergone Fontan operation and spinal surgery can offer physiologic benefits. Venous air embolism is a rare, but important reported complication during spinal surgery performed in the prone position. Patients with Fontan circulation can have significant right to left shunting via a patent Fontan fenestration that can increase the risk of paradoxical systemic embolization of any entrained venous air. METHODS: We retrospectively reviewed the charts of two patients with single-ventricle congenital heart disease who had undergone fenestrated Fontan operation and underwent transcatheter fenestration closure prior to spinal fusion. RESULTS: Two patients with Fontan circulation underwent successful transcatheter fenestration closure with Amplatzer Ductal Occluder II devices. Five to 6 months after closure, both underwent uncomplicated posterior spinal fusion. CONCLUSION: Transcatheter closure of the Fontan fenestration prior to spinal fusion in two with Fontan circulation and scoliosis is a rare, but important indication for fenestration closure that warrants emphasis. LEVEL OF EVIDENCE: N/A.

SCAI/CCAS/SPA expert consensus statement for anesthesia and sedation practice: Recommendations for patients undergoing diagnostic and therapeutic procedures in the pediatric and congenital cardiac catheterization laboratory.

Current practice of sedation and anesthesia for patients undergoing pediatric congenital cardiac catheterization laboratory (PCCCL) procedures is known to vary among institutions, a multi-society expert panel with representatives from the Congenital Heart Disease Council of the Society for Cardiovascular Angiography and Interventions (SCAI), the Society for Pediatric Anesthesia (SPA) and the Congenital Cardiac Anesthesia Society (CCAS) was convened to evaluate the types of sedation and personnel necessary for procedures performed in the PCCCL. The goal of this panel was to provide practitioners and institutions performing these procedures with guidance consistent with national standards and to provide clinicians and institutions with consensus-based recommendations and the supporting references to encourage their application in quality improvement programs. Recommendations can neither encompass all clinical circumstances nor replace the judgment of individual clinicians in the management of each patient. The science of medicine is rooted in evidence, and the art of medicine is based on the application of this evidence to the individual patient. This expert consensus statement has adhered to these principles for optimal management of patients requiring sedation and anesthesia. What follows are recommendations for patient monitoring in the PCCCL regardless of whether minimal or no sedation is being used or general anesthesia is being provided by an anesthesiologist. © 2016 Wiley Periodicals Inc.

SCAI/CCAS/SPA Expert Consensus Statement for Anesthesia and Sedation Practice: Recommendations for Patients Undergoing Diagnostic and Therapeutic Procedures in the Pediatric and Congenital Cardiac Catheterization Laboratory.

Current practice of sedation and anesthesia for patients undergoing pediatric and congenital cardiac catheterization laboratory (PCCCL) procedures is known to vary among institutions, a multi-society expert panel with representatives from the Congenital Heart Disease Council of the Society for Cardiovascular Angiography and Interventions, the Society for Pediatric Anesthesia and the Congenital Cardiac Anesthesia Society was convened to evaluate the types of sedation and personnel necessary for procedures performed in the PCCCL. The goal of this panel was to provide practitioners and institutions performing these procedures with guidance consistent with national standards and to provide clinicians and institutions with consensus-based recommendations and the supporting references to encourage their application in quality improvement programs. Recommendations can neither encompass all clinical circumstances nor replace the judgment of individual clinicians in the management of each patient. The science of medicine is rooted in evidence, and the art of medicine is based on the application of this evidence to the individual patient. This expert consensus statement has adhered to these principles for optimal management of patients requiring sedation and anesthesia. What follows are recommendations for patient monitoring in the PCCCL regardless of whether minimal or no sedation is being used or general anesthesia is being provided by an anesthesiologist.

Hybrid Pulmonary Artery Stenting at the Bidirectional Glenn or Fontan Operation in Patients With Single Ventricle Congenital Heart Disease.

BACKGROUND: Stenosis of the retroaortic pulmonary artery is common in patients with single ventricle heart disease. Intraoperative hybrid stenting at the bidirectional Glenn or Fontan operation can treat this lesion and avoid a complex surgical arterioplasty. METHODS: Patients who underwent intraoperative stent implantation to the retroaortic pulmonary artery during the bidirectional Glenn or Fontan operation at our center between January 2005 and July 2014 were retrospectively identified. RESULTS: Thirteen patients were included with a median weight of 8 kg (5.6-14.4 kg) and age of 6 months (4 months-3.8 years). All had undergone Norwood operation, and eight (62%) had hypoplastic left heart syndrome. Eight (62%) underwent stent placement during bidirectional Glenn and five (38%) during Fontan operation. Ten patients had one stent placed, and three had two overlapping stents. The median diameter of the stenotic vessel was 3 mm (2.0-5.5 mm) and diameter of the balloon used for stent expansion was 7 mm (5-10 mm). Two complications occurred including pulmonary hemorrhage from presumed wire perforation and left main stem bronchus compression requiring stent removal. No patient required stent intervention in the postoperative period, and all were discharged from the hospital. At a median follow-up of 1.3 years (2 months-7.1 years), six patients underwent interval dilation to account for somatic growth. CONCLUSIONS: Hybrid stenting of the retroaortic pulmonary artery at the bidirectional Glenn or Fontan operation is an effective treatment of pulmonary artery stenosis and prevents the need for a complex surgical arterioplasty.

Transcatheter Patent Ductus Arteriosus Occlusion in Small Infants.

BACKGROUND: Transcatheter patent ductus arteriosus (PDA) occlusion is feasible in small infants and may improve lung function in symptomatic patients. We aimed to describe transcatheter PDA closure in small infants including predictors of technical success and rate of complication and to identify factors associated with improved respiratory status after closure. METHODS: All patients in the NICU at our center who were referred for transcatheter PDA occlusion between 1/2010 and 11/2014 were retrospectively identified. Relevant details were extracted. Additionally, a modification of the respiratory severity score (RSS) (FiO2 × mean airway pressure) was used to characterize degree of pulmonary support before and at intervals after catheterization. RESULTS: Twenty patients were identified with median age of 96 days (13-247) and weight of 3.1 kg (1.7-4.7). The PDA was type F morphology in 14 (70%) patients. The PDA was successfully occluded in 16 (80%) patients. Ratio of minimum PDA diameter/length was >0.5 in all unsuccessful attempts and <0.4 in all successful cases (P = .01). Of the 16 cases of occlusion, Amplatzer Vascular Plug II was used in 15 (94%). No deaths or pulse loss occurred. Five (25%) patients required blood transfusion and transfusion was associated with lower hemoglobin (P = .049), lower weight (P = .008), and lower aortic pressure (P = .04). Excluding 1 patient with significant congenital heart disease, the RSS improved at 3 days in 9 (60%) patients and at 7 days in 12 (80%) compared with preintervention value. Patient factors were not associated with improved RSS at 3 or 7 days. CONCLUSIONS: In our cohort of symptomatic infants, transcatheter PDA occlusion was successful in most and a ratio of minimum PDA diameter/length of <0.4 was predictive of technical success. Using a surrogate for pulmonary support, the majority of patients were on less support 7 days after closure.