Novel application of the Diabolo technique to restrict pulmonary blood flow in a single ventricle patient with a right ventricle to pulmonary artery conduit.

Balancing pulmonary and systemic circulations in single ventricle patients with a conduit after Stage 1 palliation is challenging. A transcatheter intervention for excessive pulmonary blood flow would provide benefit. We report a case of a critically ill single ventricle patient with symptoms of excessive pulmonary blood flow after Stage 1 despite maximal medical therapy. The patient underwent percutaneous intraluminal downsizing of the right ventricle to pulmonary artery conduit using a novel application of the Diabolo-covered stent technique, with subsequent clinical improvement. A second catheterization was performed during the interstage period with successful dilation of the stent to achieve appropriate saturations. The Diabolo technique can be successfully employed in this population to restrict pulmonary blood flow and has the advantage of being adjusted during placement and in subsequent interventions.

Critical left main coronary artery stenosis presenting as cardiac arrest in coarctation of the aorta patient.

Congenital coronary artery stenosis coexisting with aortic coarctation in nonsyndromic patients has not previously been reported. This report describes a nonsyndromic aortic coarctation patient who experienced intraoperative cardiac arrest due to a previously undiagnosed critical left main coronary artery stenosis. The patient was successfully resuscitated, underwent patch coronary ostioplasty, and was discharged home. He remains well for four months following repair.

Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain.

BACKGROUND: Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter stent placement in a residual ductus arteriosus (PDA) is one potentially effective option in critically ill infants and young children with PAH. We sought to assess recovery of RV function by echocardiographic strain in infants and young children following PDA stenting for acute PAH. METHODS: Retrospective review of patients < 2 years old who underwent PDA stenting for acute PAH. Clinical data were abstracted from the electronic medical record. RV strain (both total and free wall components) was assessed from echocardiographic images at baseline and 3, 6, and 12 months post-intervention, as well as at last echocardiogram. RESULTS: Nine patients underwent attempted ductal stenting for PAH. The median age at intervention was 38 days and median weight 3.7 kg. One-third (3of 9) of patients had PAH associated with a congenital diaphragmatic hernia. PDA stents were successfully deployed in eight patients. Mean RV total strain was - 14.9 ± 5.6% at baseline and improved to - 23.8 ± 2.2% at 6 months post-procedure (p < 0.001). Mean free wall RV strain was - 19.5 ± 5.4% at baseline and improved to - 27.7 ± 4.1% at 6 months (p = 0.002). Five patients survived to discharge, and four patients survived 1 year post-discharge. CONCLUSION: PDA stenting for severe, acute PAH can improve RV function as assessed by strain echocardiography. The quantitative improvement is more prominent in the first 6 months post-procedure and stabilizes thereafter.

Predictors of Serious Adverse Events and High-Level Cardiorespiratory Support in Patients Undergoing Transcatheter Pulmonary Vein Interventions.

Patients with pulmonary vein stenosis (PVS) often require frequent transcatheter pulmonary vein (PV) interventions for management of restenosis. Predictors of serious adverse events (AEs) and need for high-level cardiorespiratory support (mechanical ventilation, vasoactive support, and/or extracorporeal membrane oxygenation) 48 h after transcatheter PV interventions have not been reported. This is a single-center retrospective cohort analysis of patients with PVS who underwent transcatheter PV interventions from 3/1/2014 to 12/31/2021. Univariate and multivariable analyses were performed using generalized estimating equations to account for within-patient correlation. 240 patients underwent 841 catheterizations involving PV interventions (median 2 catheterizations per patient [1,3]). At least one serious AE was reported in 100 (12%) cases, the most common of which were pulmonary hemorrhage (n = 20) and arrhythmia (n = 17). There were 14 severe/catastrophic AEs (1.7% of cases) including three strokes and one patient death. On multivariable analysis, age less than 6 months, low systemic arterial saturation (< 95% in patients with biventricular [BiV] physiology, < 78% in single ventricle [SV] physiology), and severely elevated mean PA pressure (≥ 45 mmHg in BiV, ≥ 17 mmHg in SV) were associated with SAEs. Age less than 1 year, hospitalization prior to catheterization, and moderate-severe RV dysfunction were associated with high-level support after catheterization. Serious AEs during transcatheter PV interventions in patients with PVS are common, although major events such as stroke or death are uncommon. Younger patients and those with abnormal hemodynamics are more likely to experience serious AEs and require high-level cardiorespiratory support after catheterization.

Safety and Short-Term Outcomes for Infants < 2.5 kg Undergoing PDA Device Closure: A C3PO Registry Study.

To evaluate short-term procedural outcomes and safety for infants < 2.5 kg who underwent catheterization with intended patent ductus arteriosus (PDA) device closure in a multi-center registry, as performance of this procedure becomes widespread. A multi-center retrospective review was performed using data from the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. Data were collected for all intended cases of PDA closure in infants < 2.5 kg from April 2019 to December 2020 at 13 participating sites. Successful device closure was defined as device placement at the conclusion of the catheterization. Procedural outcomes and adverse events (AE) were described, and associations between patient characteristics, procedural outcomes and AEs were analyzed. During the study period, 300 cases were performed with a median weight of 1.0 kg (range 0.7-2.4). Successful device closure was achieved in 98.7% of cases with a 1.7% incidence of level 4/5 AEs, including one periprocedural mortality. Neither failed device placement nor adverse events were significantly associated with patient age, weight or institutional volume. Higher incidence of adverse events associated with patients who had non-cardiac problems (p = 0.017) and cases with multiple devices attempted (p = 0.064). Transcatheter PDA closure in small infants can be performed with excellent short-term outcomes and safety across institutions with variable case volume.

Hemodynamic and anatomic changes after fetal aortic valvuloplasty are associated with procedural success and postnatal biventricular circulation.

BACKGROUND: There are minimal data characterizing the trajectory of left heart growth and hemodynamics following fetal aortic valvuloplasty (FAV). METHODS: This retrospective study included patients who underwent FAV between 2000 and 2019, with echocardiograms performed pre-FAV, immediately post-FAV, and in late gestation. RESULTS: Of 118 fetuses undergoing FAV, 106 (90%) underwent technically successful FAV, of which 55 (52%) had biventricular circulation. Technically successful FAV was associated with improved aortic valve growth (p < 0.001), sustained antegrade aortic arch (AoA) flow (p = 0.02), improved mitral valve (MV) inflow pattern (p = 0.002), and favorable patent foramen ovale (PFO) flow pattern (p = 0.004) from pre-FAV to late gestation. Compared to patients with univentricular outcome, patients with biventricular outcome had less decrement in size of the left ventricle (LV) (p < 0.001) and aortic valve (p = 0.005), as well as more physiologic PFO flow (p < 0.001) and antegrade AoA flow (p < 0.001) from pre-FAV to late gestation. In multivariable analysis, echocardiographic predictors of biventricular outcome were less decline in LV end diastolic dimension (p < 0.001), improved PFO flow (p = 0.004), and sustained antegrade AoA flow (p = 0.002) from pre-FAV to late gestation. CONCLUSION: Stabilization of left heart growth and improved hemodynamics following successful FAV through late gestation are associated with postnatal biventricular circulation.

Anesthesia in Children With Pulmonary Hypertension: Clinically Significant Serious Adverse Events Associated With Cardiac Catheterization and Noncardiac Procedures.

OBJECTIVES: To determine the incidence of clinically significant serious adverse events in a contemporary population of pediatric patients with pulmonary hypertension who require anesthesia and identify factors associated with adverse outcomes. DESIGN: A retrospective, cross-sectional study. SETTING: A single-center quaternary-care freestanding children's hospital in the northeastern United States. PARTICIPANTS: Pediatric patients with pulmonary hypertension based on hemodynamic criteria on cardiac catheterization during a 3-year period from 2015 to 2018. INTERVENTIONS: Anesthesia care for cardiac catheterization, noncardiac surgery, and diagnostic imaging. MEASUREMENTS AND MAIN RESULTS: Two hundred forty-nine children underwent 862 procedures, 592 for cardiac catheterization and 278 for noncardiac surgery and diagnostic imaging. The median age was 1.6 years, and the weight was 9.5 lbs. On index catheterization, median pulmonary artery pressure was 36 mmHg, and the pulmonary vascular resistance was 5.1 indexed Wood units. Ten percent of anesthetics were performed with a natural airway, and 80% used volatile anesthetics. Serious adverse events occurred in 26% of procedures (confidence interval [CI], 22%-30%). The rate of periprocedural cardiac arrest was 8 per 1,000 anesthetic administrations. In multivariate analysis, younger age (adjusted odds ratio [aOR], 1.4 per year; CI, 1.1-1.9; p = 0.01), location in the catheterization laboratory (aOR, 5.1; CI, 1.7-16; p = 0.004), and longer procedure duration (aOR, 1.3 per 30 minutes; CI, 1.1-1.4; p = 0.001) were associated with serious adverse events. Patients with a tracheostomy in place were less likely to experience an adverse event (aOR, 0.1; CI, 0.04-0.5; p = 0.001). The primary anesthetic technique was not associated with adverse events. Interventional cardiac catheterization was associated with an increased incidence of adverse events compared with diagnostic catheterization (42% v 21%; OR, 2.23; CI, 1.5-3.3; p < 0.001). CONCLUSIONS: Serious adverse events were common in this cohort. Careful planning to minimize anesthesia time in young children with pulmonary hypertension should be undertaken, and these factors considered in designing risk mitigation strategies.

Preliminary findings on the use of intravascular ultrasound in the assessment of pediatric pulmonary vein stenosis.

OBJECTIVE: Determine the feasibility of performing intravascular ultrasound (IVUS) in pediatric pulmonary vein stenosis (PVS) and investigate whether IVUS can delineate the mechanism of PVS. BACKGROUND: The use of IVUS in pediatric patients with PVS has not been reported. METHODS: Retrospective, single center, cohort analysis of all patients who underwent IVUS of pulmonary veins from August 2016 to December 2019. RESULTS: IVUS was performed on 81 pulmonary veins in 50 pediatric patients (median age = 1.7 years [0.9, 3.1], median weight = 8.6 kg [7.3, 11.8]). All veins accepted the IVUS catheter (.014 or .018), with adequate visualization in 88% (71/81) of imaged veins, and improvement in visualization in the more recent period (23/24; 96%). Veins were categorized as having presumed intimal thickening (PIT) with luminal narrowing (n = 36), ostial narrowing without PIT (n = 14), distortion/compression (n = 6), normal (n = 2), and stent with (n = 9) or without in-stent stenosis (n = 4). In veins with at least 6 months of follow up, (re)intervention occurred more commonly in veins with PIT (14/19; 74%) versus veins without PIT (3/13; 23%; p = 0.01). There were no IVUS related adverse events. CONCLUSION: IVUS can be used safely in pediatric patients and can reliably demonstrate vein lumen and wall architecture. With further refinement, IVUS has the potential to differentiate intimal neo-proliferation from other mechanisms of obstruction. The exact role of IVUS in the assessment of pediatric PVS is yet to be determined.

Late gestation predictors of a postnatal biventricular circulation after fetal aortic valvuloplasty.

OBJECTIVES: Fetal aortic valvuloplasty (FAV) for severe aortic stenosis (AS) has shown promise in averting progression to hypoplastic left heart syndrome. After FAV, predicting which fetuses will achieve a biventricular (BiV) circulation after birth remains challenging. Identifying predictors of postnatal circulation on late gestation echocardiography will improve parental counseling. METHODS: Liveborn patients who underwent FAV and had late gestation echocardiography available were included (2000-2017, n = 96). Multivariable logistic regression and classification and regression tree analysis were utilized to identify independent predictors of BiV circulation. RESULTS: Among 96 fetuses, 50 (52.1%) had BiV circulation at the time of neonatal discharge. In multivariable analysis, independent predictors of biventricular circulation included left ventricular (LV) long axis z-score (OR 3.2, 95% CI 1.8-5.7, p < 0.001), LV ejection fraction (OR 1.3, 95% CI 1.0-1.8, p = 0.023), anterograde aortic arch flow (OR 5.0, 95% CI 1.2-20.4, p = 0.024), and bidirectional or right-to-left foramen ovale flow (OR 4.6, 95% CI 1.4-15.8, p = 0.015). CONCLUSION: Several anatomic and physiologic parameters in late gestation were found to be independent predictors of BiV circulation after FAV. Identifying these predictors adds to our understanding of LV growth and hemodynamics after FAV and may improve parental counseling.

The snared wire technique for Sapien valve implantation in the pulmonary position.

OBJECTIVES: Description of the snared wire technique (SWT) to facilitate the delivery of the Sapien valve in pulmonary position, and comparison with standard delivery technique. BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Sapien delivery system has proven to be challenging. Therefore, alternative strategies for facilitating its delivery in this position are needed. METHODS: Retrospective analysis of patients who underwent TPVR with or without the new SWT. The SWT was chosen as an elective strategy when the anatomy was judged to be challenging for TPVR (planned SWT) or as a rescue strategy when a standard delivery failed (rescue SWT). RESULTS: From February 2018 to January 2020, 84 patients underwent TPVR with a Sapien S3 valve using either a standard delivery (n = 63, 75%) or a SWT (n = 21, 25%). Fifteen patients underwent a planned SWT, six patients underwent a rescue SWT after failure of a standard delivery. All planned SWT cases were successful and, compared to the standard delivery group, no significant differences were found in terms of time to valve-deployment, fluoroscopy time, procedure time, or frequency of complications. Rescue SWT cases had longer fluoroscopy time (p = .05), longer time to valve-deployment (p = .0001), and higher frequency of complications (p = .002) including tricuspid valve injury (p = .0004), but allowed the operator to successfully implant the valve into the desired location. CONCLUSIONS: Even in the most challenging anatomies, the SWT represents a feasible and effective alternative strategy for TPVR with the Sapien valve that should be considered when other techniques have failed.

Systemic Sirolimus to Prevent In-Stent Stenosis in Pediatric Pulmonary Vein Stenosis.

Evaluate the efficacy of systemic sirolimus (rapamycin) in preventing in-stent stenosis (ISS) in pediatric intraluminal pulmonary vein stenosis (PVS). Report the adverse events related to sirolimus therapy. There is a high incidence of ISS following stent implantation in PVS. The use of sirolimus in preventing ISS has not been reported. Retrospective review of all patients who received sirolimus (8 week course) for treatment of ISS for PVS between January 2013 and June 2018. Forty stents (37 bare metal, 3 drug-eluting) in 20 patients were treated with sirolimus; 20 at the time of implantation (primary prevention [1P]) and 20 following documented ISS requiring transcatheter reintervention (secondary prevention [2P]). Treated patients were young (median 2 y/o [0.7-5.7]) and most had PVS associated with congenital heart disease (75%, 15/20; 4/15 with TAPVC). In the 1P group, 85% (17/20) of stents were without significant (< 50%) ISS at median of 102 days (range 56-527); the growth rate of ISS in this group was 7.5 ± 7.1%/month. In the 2P group, most stents had a slower growth rate of ISS after sirolimus therapy compared to pre-treatment (median 3.7 [- 0.2 to 13.1] vs. 10.4 [1.3 to 19.5] %/month; p < 0.001). One patient developed pneumonia on drug while concurrently taking another immunosuppressive agent. No other serious adverse events were related to sirolimus therapy. Systemic sirolimus slows the growth rate of ISS following stent implantation in PVS compared to pre-treatment rates and was administered safely in a small number of pediatric patients with complex heart disease.

Prognostic risk factors for complications associated with tibiotalocalcaneal arthrodesis with a nail.

BACKGROUND: Tibiotalocalcaneal (TTC) arthrodesis with a nail can be an effective salvage procedure for several foot and ankle pathologies, but has a relatively high complication rate. The purpose of this study is to investigate risk factors associated with complications after TTC arthrodesis with a nail. METHODS: Clinical and radiographic outcomes for 82 patients from 2012 to 2016 who underwent TTC arthrodesis with a nail were retrospectivelyevaluated to determine if patient or surgeon specific variables offered prognostic value in predicting negative outcomes. RESULTS: Diabetes, diabetic neuropathy, high (>2) American Society of Anesthesiologists (ASA) classification, and Charcot neuroarthropathy all were predictive of developing a nonunion in either the subtalar ortibiotalar joints (p<0.05). Diabetic neuropathy was predictive ofreoperation, and along with HbA1C >7.5 was also predictive of hardwarefailure. The odds ratio (OR) for diabetic neuropathy was 2.99 (p<0.05)for nonunion in the tibiotalar or subtalar joints, 3.46 (p<0.05) for reoperation,and 4.11 (p<0.05) for hardware failure. High ASAclassification had an odds ratio of 3.93 (p<0.05) for nonunion in the tibiotalar or subtalar joints as well. Diabetes had an odds ratio of 2.57 (p<0.05) for nonunion. CONCLUSIONS: Patients with diabetic neuropathy, Charcot neuroarthropathy, elevated HbA1C, and ASA classification >2 demonstrated a higher complication rate in patients undergoing TTC arthrodesis with a nail.

Endometrial recognition of pregnancy occurs in the grey short-tailed opossum ( Monodelphis domestica).

In human pregnancy, recognition of an embryo within the uterus is essential to support the fetus through gestation. In most marsupials, such as the opossums, pregnancy is shorter than the oestrous cycle and the steroid hormone profile during pregnancy and oestrous cycle are indistinguishable. For these reasons, it was assumed that recognition of pregnancy, as a trait, evolved in the eutherian (placental) stem lineage and independently in wallabies and kangaroos. To investigate whether uterine recognition of pregnancy occurs in species with pregnancy shorter than the oestrous cycle, we examined reproduction in the short-tailed opossum ( Monodelphis domestica), a marsupial with a plesiomorphic mode of pregnancy. We examined the morphological and gene expression changes in the uterus of females in the non-pregnant oestrous cycle and compared these to pregnancy. We found that the presence of an embryo did not alter some aspects of uterine development but increased glandular activity. Transcriptionally, we saw big differences between the uterus of pregnant and cycling animals. These differences included an upregulation of genes involved in transport, inflammation and metabolic-activity in response to the presence of a fetus. Furthermore, transcriptional differences reflected protein level differences in transporter abundance. Our results suggest that while the uterus exhibits programmed changes after ovulation, its transcriptional landscape during pregnancy responds to the presence of a fetus and upregulates genes that may be essential for fetal support. These results are consistent with endometrial recognition of pregnancy occurring in the opossum. While the effects on maternal physiology appear to differ, recognition of pregnancy has now been observed in eutherian mammals, as well as, Australian and American marsupials.

Intraluminal Pulmonary Vein Stenosis in Children: A "New" Lesion.

Pulmonary vein stenosis (PVS) is a rare disorder that leads to progressive narrowing of the extrapulmonary veins. PVS has been reported in both children and adults and in its worse iteration leads to pulmonary hypertension, right ventricular failure, and death. Multiple etiologies of PVS have been described in children and adults. This review will focus on intraluminal PVS in children. Intraluminal PVS has an estimated incidence ranging from 0.0017% to 0.03%. It is associated with conditions such as prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, Smith-Lemli-Opitz syndrome, and Down syndrome. Cardiac catheterization and pulmonary vein angiography are the gold standard for diagnosis and anatomic delineation. Other imaging modalities including magnetic resonance imaging, chest tomography, and transesophageal echocardiography are increasingly being used. Mortality of PVS in children is approximately 50%. Predictors of mortality include involvement of ≥3 pulmonary veins, bilateral pulmonary vein involvement, onset of PVS in infancy, elevated pulmonary artery pressure or systolic pulmonary artery-to-aortic pressure ratio, right ventricular dysfunction, restenosis after surgery, distal/upstream disease, and disease progression to previously uninvolved pulmonary veins. Treatment includes catheter-based pulmonary vein dilations with or without stenting, surgical interventions, medical therapy, and in some instances, lung transplantation. Cardiac catheterization for PVS involves a comprehensive hemodynamic and anatomic assessment of the pulmonary veins as well as therapeutic transcatheter interventions. Several surgical strategies have been used. Sutureless repair is currently most commonly used, but patch venoplasty, endarterectomy, ostial resection, and reimplantation are used in select circumstances as well. Medical therapies such as imatinib mesylate and bevacizumab are increasingly being used in an effort to suppress the myofibroblastic proliferation seen in PVS patients. Lung transplantation has been used as an alternative treatment strategy for end-stage, refractory PVS. Nonetheless, despite the different innovative approaches used, morbidity and mortality remain high. At present, the preferred treatment strategy is frequent reassessment of disease progression to guide use of catheter-based and surgical interventions in conjunction with medical therapy.

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

OBJECTIVES: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort. DESIGN: Multicenter retrospective study. SETTING: Eleven pediatric hospitals within the United States. PATIENTS: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years. CONCLUSIONS: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Radiation Risk Categories in Cardiac Catheterization for Congenital Heart Disease: A Tool to Aid in the Evaluation of Radiation Outcomes.

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included. Using expert consensus, 40 unique procedure types were defined by diagnostic characteristics or the intervention(s) performed, and dose area product (DAP) per kilogram of body weight (µGy × m2/kg) was summarized. Using empiric and consensus methods, three radiation risk categories were created. A total of 11,735 cases were included for analysis. Thirteen (n = 7918) procedure types with median DAP/kg < 100 were categorized in the low radiation exposure category (median DAP/kg 39). The medium exposure category (n = 1807) consisted of 16 procedure types with median DAP/kg values ranging 100 to < 200 (overall median DAP/kg 131). Finally, the high radiation exposure category (n = 1073) consisted of 11 procedure types with median DAP/kg ≥ 200 (overall median DAP/kg of 231). The radiation exposure risk categories created in this multi-center dataset are a critical step towards the development of a robust risk adjustment methodology for radiation exposure in catheterization for congenital heart disease.

Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis.

OBJECTIVE: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS. STUDY DESIGN: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014. Drug therapy was discontinued at 48 weeks, or after 24 weeks of stabilization, whichever occurred later. RESULTS: Among 48 enrolled patients, 5 had isolated PVS, 26 congenital heart disease, 5 lung disease, and 12 both. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease (P = .03), a higher percentage of eligible drug doses received (P = .03), and was not associated with age, diagnosis, disease laterality, or number of veins involved. Survival to 72 weeks was 77% (37 of 48). Adverse events were common (n = 1489 total), but only 16 were definitely related to drug treatment, none of which were serious. CONCLUSION: Survival to 72 weeks was 77% in a referral population with multivessel intraluminal PVS undergoing multimodal treatment, including antiproliferative tyrosine kinase blockade. Toxicity specific to tyrosine kinase blockade was minimal.

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.

Transcatheter Pulmonary Valve Replacement and Acute Increase in Diastolic Pressure are Associated with Increases in Both Systolic and Diastolic Pulmonary Artery Dimensions.

Stable positioning of a transcatheter pulmonary valve (TPV) in native outflow tracts depends on a clear understanding of underlying anatomy and outflow tract dimensions. We hypothesized that restoration of pulmonary competence may acutely alter these dimensions. A retrospective single-center review of consecutive patients after TPV placement from 2007 to 2014 was performed. Patients with less than moderate pulmonary regurgitation were excluded. We reviewed acute catheterization data on 46 patients, most with tetralogy of Fallot (70%). Baseline and post-implant (7.5 ± 3 min post-deployment) measurements of central pulmonary arteries (PAs) were determined angiographically. The right PA diameter increased (20 ± 4-24 ± 6 mm systole*, 16 ± 4-21 ± 6 mm diastole*), as did the left PA (20 ± 6-24 ± 8 mm systole*, 16 ± 5-21 ± 7 mm diastole*). PA pressures increased from averages of 29.3/10.6 (17) to 29.8/15.1 (21) mmHg. We noted that pre-implant systolic PA diameter correlated with diastolic PA diameter post-implant (r = 0.9). On follow-up catheterization in seven patients [median 3 years; (1-8)], combined central PA diameter decreased an average of 20% (systole: 20% ± 12, diastole: 18% ± 11) as compared to post-implant measurements. Acute pulmonary valve competence in patients with at least moderate pulmonary regurgitation results in an immediate increase in PA diameter (20% systole and 30% diastole). The cause of this diameter change is unclear. This acute change may have implications for device and patient selection (*p < 0.001).