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Background: Pulse wave velocity (PWV) in the pulmonary arteries (PA) is a marker of vascular stiffening. Currently, only phase-contrast (PC) MRI-based options exist to measure PA-PWV., Purpose: To test feasibility, repeatability, and correlation to clinical data of Phase-Resolved Functional Lung (PREFUL) MRI-based calculation of PA-PWV., Study Type: Retrospective., Subjects: 79 (26 female) healthy subjects (age range 19-78), 58 (24 female) patients with chronic obstructive pulmonary disease (COPD, age range 40-77), 60 (33 female) patients with suspected pulmonary hypertension (PH, age range 28-85)., Sequence: 2D spoiled gradient echo, 1.5T., Assessment: PA-PWV was measured from PREFUL-derived cardiac cycles based on the determination of temporal and spatial distance between lung vasculature voxels using a simplified (sPWV) method and a more comprehensive (cPWV) method including more elaborate distance calculation. For 135 individuals, PC MRI-based PWV (PWV-QA) was measured., Statistical Tests: Intraclass-correlation-coefficient (ICC) and coefficient of variation (CoV) were used to test repeatability. Nonparametric tests were used to compare cohorts. Correlation of sPWV/cPWV, PWV-QA, forced expiratory volume in 1 sec (FEV 1 ) %predicted, residual volume (RV) %predicted, age, and right heart catheterization (RHC) data were tested. Significance level α = 0.05 was used., Results: sPWV and cPWV showed no significant differences between repeated measurements (P-range 0.10-0.92). CoV was generally lower than 15%. COPD and PH patients had significantly higher sPWV and cPWV than healthy subjects. Significant correlation was found between sPWV or cPWV and FEV 1 %pred. (R = -0.36 and R = -0.44), but not with RHC (P-range -0.11 - 0.91) or age (P-range 0.23-0.89). Correlation to RV%pred. was significant for cPWV (R = 0.42) but not for sPWV (R = 0.34, P = 0.055). For all cohorts, sPWV and cPWV were significantly correlated with PWV-QA (R = -0.41 and R = 0.48)., Data Conclusion: PREFUL-derived PWV is feasible and repeatable. PWV is increased in COPD and PH patients and correlates to airway obstruction and hyperinflation., Level of Evidence: 3 TECHNICAL EFFICACY: Stage 2., (© 2024 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Neonates with congenital heart disease (CHD) and ductal-dependent pulmonary blood flow (DD-PBF) require early intervention. Historically, this intervention was most often a surgical systemic-to-pulmonary shunt (SPS; e.g., Blalock-Thomas-Taussig shunt). However, over the past two decades an alternative to SPS has emerged in the form of transcatheter ductal artery stenting (DAS). While many reports have indicated safety and durability of the DAS approach, few studies compare outcomes between DAS and SPS. The reports that do exist are comprised primarily of small-cohort single-center reviews. Two multicenter retrospective studies suggest that DAS is associated with similar or superior survival compared to SPS. These studies offer the best evidence to-date, and yet both have important limitations. The authors describe herein the rationale and design of the COMPASS (COmparison of Methods for Pulmonary blood flow Augmentation: Shunt vs. Stent) Trial (NCT05268094, IDE G210212). The COMPASS Trial aims to randomize 236 neonates with DD-PBF to either DAS or SPS across approximately 27 pediatric centers in North America. The goal of this trial is to compare important clinical outcomes between DAS and SPS over the first year of life in a cohort of neonates balanced by randomization to assess whether one method of palliation demonstrates therapeutic superiority., (© 2024 The Author(s). Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Background: Pulmonary artery involvement (PAI) is not rare in Takayasu arteritis (TA). Persistently elevated pulmonary arterial pressure in TA-PAI patients leads to pulmonary hypertension (PH), and eventually cardiac death. Thus, the early detection of right ventricular dysfunction before the onset of PH is important., Purpose: To explore the potential of right ventricular global peak longitudinal and circumferential strain (RVGLS and RVGCS, respectively) in detecting right ventricular myocardial damage in TA-PAI patients without PH., Study Type: Retrospective., Population: One hundred and six TA patients (39.6 ± 13.9 years), of whom 52 were non-PAI and 54 were PAI patients (36 without PH and 18 with PH), along with 58 sex- and age-matched healthy volunteers (HVs) (36.7 ± 13.2 years). The involved arteries were validated by aorta magnetic resonance (MR) angiography and pulmonary artery computed tomography angiography., Field Strength/sequence: 3 T/Cine imaging sequence with a steady-state free precession readout., Assessment: Cardiac MRI-derived parameters measured by two radiologists independently were compared among HVs, and TA patients with and without PAI. In addition, these indices were further compared among HVs, and TA-PAI patients with and without PH., Statistical Tests: Student's t test, one-way ANOVA analysis, Pearson and Spearman correlation analysis, and reproducibility analysis. A P-value of <0.05 was considered statistically significant., Results: Although the TA-PAI patients without PH had a similar RV ejection fraction (RVEF) with HV (P = 0.348), RVGLS (non-PH 20.6 ± 3.7% vs. HV 24.0 ± 3.1%) was significantly lower and RVGCS (non-PH 14.8 ± 3.9% vs. HV 13.0 ± 2.7%) higher. The TA-PAI patients with PH had significantly poorer RVGLS (PH 13.5 ± 3.8% vs. non-PH 20.6 ± 3.7%) and RVGCS (PH 10.9 ± 3.2% vs. non-PH 14.8 ± 3.9%) than those without PH., Data Conclusion: Right ventricular dysfunction was detected in the TA-PAI patients without PH. MR-feature tracking may be an effective method for detecting early cardiac damage in the TA-PAI patients without PH., Level of Evidence: 3 TECHNICAL EFFICACY: Stage 3., (© 2023 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Background: The Alterra adaptive prestent is a novel self-expanding device designed to provide a landing zone for the 29 mm SAPIEN 3 valve to treat pulmonary regurgitation in patients with a right ventricular outflow tract that is too large for a balloon expandable valve alone. The mechanism of fixation for the Alterra prestent is radial force from the self-expanding stent frame, combined with a unique set of flared "tines" that protrude from both ends of the stent., Aims, Methods, and Results: In this report, we describe 6 patients who underwent uncomplicated transcatheter pulmonary valve replacement with an Alterra adaptive prestent and SAPIEN 3 valve and had surveillance chest computed tomography (CT) scans performed 1 day to 21 months after implant. In each patient, the CT scan demonstrated extravascular extension of a portion of the Alterra prestent, without clinical sequelae, but with extension into the ascending aorta in 1 patient and contact with the ascending aorta, left pulmonary vein, or left atrial appendage in 3 others., Conclusions: Surveillance CT imaging shows that the Alterra prestent can perforate the pulmonary artery and/or right ventricle. Although no sequelae were seen in these patients, prestent perforation has the potential to be clinically important. Implanters should be aware of this finding and its potential implications. As experience with the Alterra prestent grows, it will be important to further define the risk factors, incidence, and implications of this phenomenon., (© 2024 Wiley Periodicals LLC.)

We describe the safe and effective percutaneous pulmonary thrombectomy in an 18-year-old female with a Fontan circulation using the FlowTriever® device (Inari Medical®, Irvine, US). Aspiration thrombectomy of both pulmonary arteries was performed using 24 and 16 F FlowTriever® catheters retrieving large amounts of thrombus material resulting in near total angiographic recanalization., (© 2024 Wiley Periodicals LLC.)

Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery., (© 2024 The Author(s). Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Background: Stent implantation has become standard of care in older children and adults for treatment of branch pulmonary artery stenosis (BPAS) and coarctation aorta (CoAo). There are no stents approved or available for infants that have the potential to be dilated to adult diameters. The Minima stent was designed to fulfill this unmet need., Methods: Multicenter, prospective, nonrandomized early feasibility study evaluating safety and effectiveness of the Minima stent for treatment of BPAS and CoAo. Primary endpoints included: (1) successful deployment across lesion, (2) stenosis relief defined by an increase in angiographic diameter of >50% and (3) freedom from stent explant, embolization or migration at 30 days and 6 months., Results: Between 2/2022 and 5/2022, 10 pts underwent Minima stent implantation with a median age and weight of 9 months (4-43 months) and 7.6 kg (5.1-16.9 kg). Procedural success and predefined stenosis relief was achieved in all cases (CoAo [n = 4], BPAS [n = 6]). Adverse events occurred in 3 pts: transient diminished lower extremity pulse (n = 2), distal stent on-balloon displacement successfully managed in the catheterization suite (n = 1). There were no deaths or major adverse events. All patients were free from stent explant and migration at 30 days and 6 months with no evidence for significant restenosis at latest follow-up., Conclusions: Implantation of the Renata Minima stent was safe and effective for the treatment of BPAS and CoAo in this small cohort of infants and young children during early follow-up. Based on these early results, an expanded study with longer follow-up is warranted., (© 2024 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Background: Exercise-induced cardiac remodeling (CR) and myocardial fibrosis (MF) can increase cardiovascular risk in athletes. Early detection of pulmonary arterial hemodynamics parameters among athletes may be beneficial in optimizing the frequency of clinical follow-ups., Purpose: To analyze the hemodynamics of pulmonary arteries and its relationship with CR and MF in athletes using four-dimensional (4D) flow MRI., Study Type: Prospective., Population: One hundred twenty-one athletes (median age, 24 years; mean exercise per week 10 hours, for mean of 5 years) and twenty-one sedentary healthy controls (median age, 25 years; exercise per week <3 hours, irregular pattern)., Field Strength/sequence: True fast imaging with steady state free precession, time-resolved 3D Cartesian phase-contrast, and phase sensitive inversion recovery late gadolinium enhancement sequences at 3.0 T., Assessment: CR was defined as any cardiac parameters exceeding the 99th percentile upper reference limits, encompassing ventricular function, bi-atrium and bi-ventricle diameters, and ventricular wall thickness. MF was visually evaluated by three independent radiologists. 4D flow parameters were assessed in the main, right, and left pulmonary arteries (MPA, RPA, and LPA, respectively) and compared between different groups. Four machine learning (ML) models were developed to differentiate between athletes with and without CR and/or MF., Statistical Tests: Univariate analysis was used to compare groups. Area under the receiver operating characteristic curve (AUC) was used to assess the performance of the ML models., Results: Athletes had significantly higher WSSmax in the MPA, RPA, and LPA than controls. Athletes with CR and/or MF (N = 30) had significantly lower RPmax from MPA to RPA than those without (N = 91). Among the ML models, the gradient boosting machine model had the highest performance, with an AUC of 0.90., Conclusion: The pulmonary arterial hemodynamics parameters could differentiate CR and/or MF in athletes, which may be potential to assist in optimizing frequency of follow-up., Evidence Level: 1 TECHNICAL EFFICACY: Stage 2., (© 2023 International Society for Magnetic Resonance in Medicine.)

Background: Unilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear., Aims: To assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA-ASO., Methods: In this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging. RV pressures, RV afterload (arterial elastance [Ea]), PA compliance, RV contractility (end-systolic elastance [Ees]), RV-to-PA (RV-PA) coupling (Ees/Ea), and RV diastolic stiffness (end-diastolic elastance [Eed]) were analyzed and compared to normal values from the literature., Results: In all TGA patients (mean age 12 ± 3 years), RV afterload (Ea) and RV pressures were increased whereas PA compliance was reduced. RV contractility (Ees) was decreased resulting in RV-PA uncoupling. RV diastolic stiffness (Eed) was increased. CMR-derived RV volumes, mass, and ejection fraction were preserved., Conclusion: Unilateral PA stenosis results in an increased RV afterload in TGA patients after ASO. RV remodeling and function remain within normal limits when analyzed by CMR but RV pressure-volume loop analysis shows impaired RV diastolic stiffness and RV contractility leading to RV-PA uncoupling., (© 2024 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Objective: To evaluate the impact of increased pulmonary artery systolic pressure (PASP) on outcomes after transcatheter aortic valve replacement (TAVR)., Methods: A total of 242 patients who underwent TAVR were retrospectively reviewed. Transthoracic echocardiography estimated PASP. The cohorts were divided into three groups according to the numerical change of PASP; Increased (post-TAVR PASP at 1 month minus pre-TAVR PASP, ≥ + 5 mmHg; n = 52), No change (-5 to +5 mmHg; n = 86) and Decreased (≤ -5 mmHg; n = 104). Patient demographics and clinical outcomes until 1 year were evaluated. Logistic regression model was used for multivariate risk analysis., Results: At 1 year, the Increased group showed higher mortality (21 ± 6%) than the No change group (5 ± 2%) (hazard ratio [HR]: 4.8, 95% confidence interval [CI]: 1.7-13.5; p < .01) and the Decreased group (8 ± 3%) (HR: 2.8, 95% CI: 1.1-6.7; p = .02). Rehospitalization rate for valve-related or heart failure was also higher in the Increased group (21 ± 6%) than the No change group (10 ± 3%) (HR: 2.4, 95% CI: 1.1-6.0; p = .04). Predictors of PASP deterioration were hypertension (odds ratio [OR]: 3.9, 95% CI: 1.1-13.8; p = .04) and left ventricular end-diastolic diameter >50 mm (OR: 2.2, 95% CI: 1.1-4.6; p = .04), and the increased PASP remained an independent predictor of 1-year all-cause mortality (HR; 2.7, 95% CI: 1.0-6.8; p = .04)., Conclusions: Regardless of the baseline PASP, patients with increased PASP at 1 month after successful TAVR were at higher risk of mortality and rehospitalization within 1 year. Strict medical management should be considered for patients who showed dilated left ventricle preoperatively., (© 2020 Wiley Periodicals, Inc.)

Objective: Abnormal pulmonary vasculature directly affects the development and progression of congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension (PH). Though overarching structural and cellular changes in CDH-affected pulmonary arteries have been documented, the precise role of the extracellular matrix (ECM) in the pulmonary artery (PA) pathophysiology remains undefined. Here, we quantify the structural, compositional, and mechanical CDH-induced changes in the main and distal PA ECM and investigate the efficacy of mesenchymal stem cell-derived extracellular vesicles (MSC-EVs) as a therapy to ameliorate pathological vascular ECM changes., Methods: Pregnant Sprague-Dawley rodents were administered nitrofen to induce CDH-affected pulmonary vasculature in the offspring. A portion of CDH-affected pups was treated with intravenous infusion of MSC-EVs (1 × 10 10 /mL) upon birth. A suite of histological, mechanical, and transmission electron microscopic analyses were utilized to characterize the PA ECM., Results: The CDH model main PA presented significantly altered characteristics-including greater vessel thickness, greater lysyl oxidase (LOX) expression, and a relatively lower ultimate tensile strength of 13.6 MPa compared to control tissue (25.1 MPa), suggesting that CDH incurs ECM structural disorganization. MSC-EV treatment demonstrated the potential to reverse CDH-related changes, particularly through rapid inhibition of ECM remodeling enzymes (LOX and MMP-9). Additionally, MSC-EV treatment bolstered structural aspects of the PA ECM and mitigated pathological disorganization as exhibited by increased medial wall thickness and stiffness that, while not significantly altered, trends away from CDH-affected tissue., Conclusions: These data demonstrate notable ECM remodeling in the CDH pulmonary vasculature, along with the capacity of MSC-EVs to attenuate pathological ECM remodeling, identifying MSC-EVs as a potentially efficacious therapeutic for CDH-associated pulmonary hypertension., (© 2020 Wiley Periodicals LLC.)

Objectives: Aim of this study is to elucidate the impact of pulmonary hypertension on patients treated with a transapical aortic valve replacement., Background: In patients with aortic stenosis (AS) the coexistence of pulmonary hypertension (PH) is associated with increased peri-operative risk for surgical aortic valve replacement. For transcatheter aortic valve replacement (TAVR), it is unknown whether transapical TAVR (TA-TAVR) is associated with increased peri-interventional risk in PH patients., Methods: We performed a single center analysis in 189 patients with severe AS with (AS + PH) or without PH (AS - PH) undergoing TA-TAVR. PH was defined by mean pulmonary artery pressure ≥25 mmHg assessed by right heart catheterization (exclusion of 64 patients due to missing results). As the primary endpoint a combination of 30-day mortality or cardiopulmonary resuscitation (CPR) was analyzed., Results: Seventy three patients (58.4%) had PH. Increased peri-interventional risk in AS + PH patients was reflected by an increased rate of the primary endpoint in comparison to AS - PH patients (24.7 vs. 3.8%; p = .002). A higher proportion of acute kidney injury (34.2 vs. 15.7%; p = .025) was found in AS + PH patients while AS - PH patients showed a higher rate of bleeding in comparison AS + PH patients (18.5 vs. 6.8% p = .050)., Conclusion: Patients with AS + PH treated by TA-TAVR are at increased peri-interventional risk for severe complications in comparison to AS - PH patients. Therefore, the identification of preventive therapeutic strategies is needed., Classifications: TAVR, transapical, pulmonary hypertension, aortic stenosis., (© 2019 Wiley Periodicals, Inc.)

Aims: Cardiac surgery has improved life expectancy of patients with congenital heart diseases (CHDs). Exercise capacity is an important determinant of survival in patients with CHDs. There is a lack of studies focusing on the role of resting respiratory performance in reducing exercise tolerance in these patients., Objectives: To determine the prevalence and severity of respiratory functional impairment in different types of corrected/palliated CHDs, and its impact on an exercise test., Materials and Methods: Retrospective single-center study involving 168 corrected/palliated patients with CHD and 52 controls. Patients CHD were divided into subgroups according to the presence of native pulmonary blood flow or total cavopulmonary connection (TCPC). All subjects performed complete pulmonary function tests and gas diffusion; patients with CHD also performed cardiopulmonary exercise test (CPX)., Results: Mean values of lung volumes were within the normal range in all CHD groups. Comparing to controls, patients with the reduced pulmonary flow and with TCPC had the highest reduction in lung volumes. CPX was reduced in all groups, most severely in TCPC, and it was correlated to decreased dynamic volumes in all CHD groups except in TCPC. Younger age at intervention and number of surgical operations negatively affected lung volumes., Conclusions: Respiratory function is within the normal range in our patients with different CHDs at rest but altered in all CHDs during exercise when cardiorespiratory balance is likely to be inadequate. Comparing the different groups, patients with reduced pulmonary flow and TCPC are the most impaired., (© 2020 Wiley Periodicals, Inc.)

Objective: This article aims to describe the feasibility of a novel application of the Diabolo stent technique for percutaneous downsizing of systemic-to-pulmonary artery shunts in patients with pulmonary overcirculation., Background: Pulmonary overcirculation can lead to systemic hypoperfusion, impaired systemic oxygen delivery, and hemodynamic instability especially in patients with single-ventricle physiology. Different medical and surgical strategies to treat pulmonary overcirculation have been reported so far, but no percutaneous approach amenable to further adjustment over time has been described., Methods: Retrospective review of patients who underwent percutaneous intraluminal reduction of a systemic-to-pulmonary artery shunt with a covered stent in a Diabolo "hour-glass" configuration., Results: From July 2018 to February 4, 2019, consecutive patients underwent trans-catheter downsizing of a systemic-to-pulmonary artery shunt. The median age was 3.3 years (range 0.7-5.7 years). All patients had symptomatic pulmonary overflow despite maximal medical therapy. All patients underwent successful implantation of a covered stent in a Diabolo "hour-glass" configuration without any complications. Successful reduction in pulmonary overcirculation was noted in all patients with a decrease of mean pulmonary artery pressure from a median of 29 mmHg (range 25-32 mmHg) to a median of 21 mmHg (range 14-27 mmHg)., Conclusions: Percutaneous intraluminal downsizing of systemic-to-pulmonary artery shunts with a covered stent in a Diabolo configuration is safe and feasible. With this technique, the pulmonary blood flow can be adjusted in a physiologic closed-chest condition and further regulated over time., (© 2019 Wiley Periodicals, Inc.)

Objective: We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF)., Background: Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population., Methods: We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated., Results: CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval., Conclusions: Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics., (© 2019 Wiley Periodicals, Inc.)

Ischemic polymorphic ventricular ectopy was documented during exercise testing in a 65-year-old Caucasian male patient. Coronary angiogram revealed four coronary to pulmonary artery fistulas (CPAFs) originating from the right and left coronary artery, leading to myocardial ischemia due to steal phenomenon. The three dominant fistulas were coiled percutaneously, while one small fistula was left untreated. During follow-up, no significant residual ventricular arrhythmia was detected., (© 2019 Wiley Periodicals, Inc.)

Objectives: The aims of this study were to (1) evaluate risk factors of complications of balloon pulmonary angioplasty (BPA) and (2) assess the mechanism of the complications., Background: BPA represents a promising treatment option in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the complication ratio differs across reports, and the causes remain controversial., Methods: All complications found by angiography and non-contrasted computerized tomography (CT) immediately after BPA were recorded. New emergences of a ground-glass pattern or consolidation in CT after BPA that were not recognized on CT images before BPA were counted as pulmonary bleeding. Lesion morphologies were classified into four types (web, ring, abrupt narrowing and occlusive lesions) according to selective pulmonary angiography., Results: Thirty patients consented to analysis of the BPA-related images, and 879 lesions (112 sessions) were evaluated. One hundred and twenty-two (99.2%) of 123 complications were confirmed to be associated with BPA procedures at the local area. In the multivariate analysis, occlusive lesions were the sole independent predictor of procedure-related complications (adjusted odds ratio 5.83, 95%CI [1.94-17.47], p = 0.002). Hemodynamic parameters were not predictors of complications. CT images after BPA presented the predictive value for the occurrence of hemoptysis., Conclusions: Almost all complications were attributed to operators' procedures. Lesion morphology was the sole predictor of BPA-related complications, while hemodynamic parameters were not associated with the frequency of complications. CT scan images after BPA were useful to identify bleeding complications and to predict hemoptysis., (© 2019 Wiley Periodicals, Inc.)

Objective: To describe a subset of atrial septal defect (ASD) with severe pulmonary hypertension (PHT) that is suitable for closure., Background: As per American Heart Association/American College of Cardiology guidelines, ASD with elevated pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >2/3rd systemic is considered as a contraindication for closure., Methods: Patients with anatomically large ASD measuring >25 mm and a high probability of reversible pulmonary vascular disease were subjected to fenestrated device closure, despite severe PHT and elevated PVR. They were discharged on pulmonary vasodilators and were followed for 39.5 ± 8.5 months. Four patients had repeat cardiac catheterization., Results: Six patients underwent successful device closure of large ASD with severe PHT. Their basal Qp:Qs was 2.8 ± 0.3:1 while the systolic PAP and the PVR index (PVRI) were 102.6 ± 11.5 mm Hg and 9.6 ± 1.6 Wu m 2 , respectively. Post 100% oxygen inhalation, the Qp:Qs increased to 3.5 ± 0.3:1, systolic PAP remained 103.5 ± 7.6 mm Hg while the PVRI dropped to 5.4 ± 1.1 Wu m 2 . The postballoon occlusion systolic PAP decreased to 86.6 ± 8.8 mm Hg. At the last follow-up, their pulmonary artery systolic pressure by tricuspid regurgitation (TR) jet decreased from 105.6 ± 12.6 mm Hg to 45 ± 7.0 mm Hg. During follow-up cardiac catheterization (n = 4), the systolic PAP and PVRI were 55.7 ± 9.2 mm Hg and 3.2 ± 0.4, respectively., Conclusions: Patients with anatomically big defect and a large left to right shunt at baseline with a high probability of reversible PVR benefit with ASD closure and pulmonary vasodilators, despite significantly elevated PAP and PVRI., (© 2018 Wiley Periodicals, Inc.)

Severe pulmonary regurgitation (PR) most commonly occurs as a sequelae of treatment of pulmonic stenosis or Tetralogy of Fallot with fewer cases of primary pulmonic valvular regurgitation. The amount of PR is influenced by valvular integrity, right ventricular (RV) size, and RV diastolic pressures. In chronic severe PR, the RV remodels to accommodate the regurgitant flow and RV stroke volume increases to maintain effective forward blood flow. Hemodynamic changes include a widened pulmonary artery (PA) pulse pressure and low PA diastolic pressures. As the amount of regurgitation increases, RV end diastolic pressure becomes elevated and systemic cardiac output is reduced, especially with exercise. "Ventricularization" of the PA pressure tracing, in which the contour of the PA pressure is similar to the contour of the RV pressure, is a specific but not sensitive finding in severe PR. © 2015 Wiley Periodicals, Inc., (© 2015 Wiley Periodicals, Inc.)

Background: Aortopulmonary collateral flow is considered to have significant impact on the outcome of patients with single ventricle circulation and total cavopulmonary connection (TCPC). There is little information on collateral flow during exercise., Purpose: To quantify aortopulmonary collateral flow at rest and during continuous submaximal exercise in clinical patients doing well with TCPC., Study Type: Prospective, case controlled., Population: Thirteen patients with TCPC (17 (11-37) years) and 13 age and sex-matched healthy controls (18 (11-38) years)., Field Strength: 1.5T; free breathing; phase sensitive gradient echo sequence., Assessment: Blood flow in the ascending and descending aorta and superior vena cava were measured at rest and during continuous submaximal physical exercise in patients and controls. Systemic blood flow (Q s ) was assumed to be represented by the sum of flow in the superior caval vein (Q svc ) and the descending aorta (Q AoD ) at the diaphragm level. Aortopulmonary collateral flow (Q coll ) was calculated by subtracting Q s from flow in the ascending aorta (Q AoA )., Statistics: Mann-Whitney U-test and Wilcoxon test for comparison between groups and between rest and exercise., Results: Absolute collateral flow in TCPC patients at rest was 0.4 l/min/m 2 (-0.1-1.2), corresponding to 14% (-2-42) of Q s . Collateral flow did not change during exercise (difference -0.01 (-0.7-1.0) l/min/m 2 , P = 0.97). TCPC patients had significantly lower Q s at rest (2.5 (1.6-4.1) vs. 3.5 (2.6-4.8) l/min/m 2 , P = 0.001) and during submaximal exercise (3.2 (2.0-6.0) vs. 4.8 (3.3-6.9) l/min/m 2 , P = 0.001), compared to healthy controls. The increase in Q s with exercise was also significantly lower in patients than in healthy controls (median 0.6 vs. 1.2 l/min/m 2 , P < 0.02)., Data Conclusion: Clinical patients doing well with TCPC have significant aortopulmonary collateral flow at rest (14% of Q s ) compared to healthy controls, which does not change during submaximal exercise., Level of Evidence: 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:1509-1516., (© 2017 International Society for Magnetic Resonance in Medicine.)

Neonatal pulmonary hypertension (PHN) is a lethal progressive disease that occurs in prenatal circulatory transition. Mechanical wall strain caused by cardiac pulsation integrates with hypoxia to generate rapidly progressive myocyte cytoskeleton disassembly and failure to exert force generation. The physiological responses to such an interaction have not been investigated. The persistent phenotype does not respond to traditional vasodilator therapy; hence, there is a need for new treatment strategies to improve the morbidity and mortality outcomes. We reviewed the current research methods, models, and markers of persistent PHN relevant to oxidative and nitrosative stress as well as cell fate commitment, with an emphasis on apoptosis and proliferation. We surveyed potential investigations into the role of senescence in neonatal PHN cell fate decision programming during vasodilator treatment and suggested putative drug targets to improve clinical outcomes. We identified important signaling intermediates of senescence and cell cycle entry regulation in hypertensive pulmonary arterial tissues., (© 2017 Wiley Periodicals, Inc.)

Objectives: To evaluate the association between markers of precapillary pulmonary hypertension (PH) and survival in transcatheter aortic valve replacement (TAVR)., Background: The importance of precapillary PH has been sparsely investigated in patients undergoing TAVR. It may prove an important risk factor for poor outcomes., Methods: We identified patients at our institution undergoing TAVR with a baseline right heart catheterization (RHC) demonstrating PH. We evaluated the association between markers of precapillary PH and survival including the diastolic pulmonary gradient (DPG), transpulmonary gradient (TPG), and pulmonary vascular resistance (PVR). A multivariable analysis was performed using Cox Proportional Hazards Models, adjusting for age, gender, body mass index, and pulmonary artery systolic pressure (PASP) on echocardiography., Results: We identified 133 patients with PH on RHC. Of these 111 had low DPG and 22 had high DPG. All 3 markers of precapillary PH were associated with worse survival post TAVR, with OR of 2.1 (95% CI 1.1-3.9, P = 0.02), 3.4 (95% CI 1.8-6.4, P < 0.001) and 2.5 (95% CI 1.4-4.5, P = 0.003) for high DPG, TPG, and PVR, respectively. On multivariable analysis, both TPG and PVR remained predictors of worse survival, with OR of 3.4 (95% CI 1.7-6.9, P = 0.001) and 2.5 (95% CI 1.4-4.5, P = 0.003). Echocardiographic PASP and DPG were not predictive of survival., Conclusions: In patients undergoing TAVR, parameters of precapillary PH are associated with lower survival, and provide incremental prognostication over echocardiographic PASP. RHC should continue to play an important role in risk stratification prior to TAVR. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Objectives: A novel Occlutech atrial flow regulator (AFR) implantation gives an atrial septal predefined predictable fenestration., Background: Atrial septostomy relieves syncope in pulmonary arterial hypertension (PAH) by improving left heart filling, cardiac output and systemic oxygen transport despite hypoxia. Symptoms recur when small fenestrations close spontaneously., Methods: AFR was implanted after informed consent in patients with severe PAH presenting with syncope and right heart failure. Symptoms, hemodynamics, echocardiographic parameters, brain natriuretic peptide (BNP) levels and device patency were serially documented., Results: Twelve patients aged 28.3 ± 8.5 years with severe PAH underwent AFR implantation. All procedures were successful without any major complications. All patients had relief of syncope and 6-min walk distance improved significantly from 377.3 ± 33.2 to 423 ± 31.32 m. The cardiac index (2.36 ± 0.52 to 2.89 ± 0.56 L/min/m 2 ) and systemic oxygen transport (367.5 ± 75.5 to 428.0 ± 67.1 ml/min/m 2 ) also showed a significant improvement. Inferior caval vein congestion and pericardial effusion reduced due to improvement in heart failure, but other echocardiographic parameters of right ventricular function did not show significant change. The reduction in BNP levels too did not reach statistical significance. The device was patent in all patients at a median follow-up of 189 days (range 10-296 days) resulting in a significant reduction of oxygen saturations from 98 ± 0.18 to 85.26 ± 2.86% after exercise., Conclusions: AFR implantation was feasible and safe in all patients with PAH. There was a significant improvement of symptoms, six-minute walk distance, cardiac index and systemic oxygen transport. The device maintained patency in short-term follow-up and the resultant hypoxia was tolerated well., (© 2017 Wiley Periodicals, Inc.)

Purpose: To compare the quantification of pulmonary stroke volume (SV) by phase contrast magnetic resonance (PC-MR) in the main pulmonary artery (MPA) to the sum of SVs in both peripheral pulmonary arteries (PPA) in different right ventricular (RV) outflow pathologies., Materials and Methods: Pulmonary SV was determined by PC-MR in the MPA and the PPA in healthy individuals (H, n = 54), patients after correction for tetralogy of Fallot with significant pulmonary regurgitation and without pulmonary or RV outflow tract stenosis (PR, n = 50), and in patients with RV outflow tract or pulmonary valve stenosis (PS, n = 50). Resulting SVs were compared to aortic SV in the ascending aorta., Results: Mean age was similar between the groups: H 28 ± 17 vs. PR 24 ± 11 vs. PS 22 ± 10 years. Bland-Altman analyses revealed in all groups a relatively small systemic (bias) but large random error (limits of agreement) for pulmonary SV determined in the MPA as compared to summed SVs in the PPA. The largest limits of agreement were present in PS patients: H: MPA 3.9% (-11, + 19) vs. PPA 0.4% (-15, + 15); PR: MPA 5.2% (-25, + 36) vs. PPA 0.6% (-24, + 26); PS: MPA 5% (-36; + 46), PPA -0.03% (-34, + 35)., Conclusion: The accuracy of PC-MR in the MPA is reasonable; however, a large random error (precision) is observed that is most pronounced in PS patients. This potential error should be taken into consideration when interpreting MPA flow measurements., Level of Evidence: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2017;46:1839-1845., (© 2017 International Society for Magnetic Resonance in Medicine.)

Objectives: To describe our 20-year experience with intraoperative pulmonary artery (PA) stent placement and evaluate long-term patient outcomes, specifically the need and risk factors for reintervention., Background: Intraoperative PA stent placement is an alternative to surgical patch arterioplasty and percutaneous angioplasty or stent placement to treat branch PA stenosis., Methods: We performed a retrospective review of all intraoperative PA stents placed at our institution from 1994-2013. Patient and stent characteristics and outcome data were collected. Risk factors associated with reintervention were identified using univariate cox regression analysis., Results: Eighty-one PA stents were placed in 68 patients. The procedural complication rate was 4.4%. During a median follow-up period of 6 years (interquartile range [IQR] 0.9-12.7), 30 patients (44%) underwent reintervention on the stented PA with a median time to first reintervention of 2.6 years (IQR 0.7-4.4 years). The first reintervention was surgical in 30% and catheter-based in 70%. Risk factors for reintervention included age < 18 months (Hazard ratio [HR] 2.97, P = 0.005) and body surface area < 0.47 m 2 (HR 3.20, P = 0.003) at the time of stent implantation, and the presence of multiple aortopulmonary collaterals in patients with tetralogy of Fallot (HR 4.61, P = 0.003)., Conclusions: Intraoperative PA stent implantation is a safe and effective alternative to percutaneous stent implantation and offers several advantages, including the ability to implant adult-size stents in small patients while avoiding injury to peripheral vessels, to position stents to facilitate future percutaneous stent redilation, and to access the PAs directly, which eliminates radiation exposure. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Objective: To determine the short- and medium-term outcomes of ductal stenting (DS) in patients with functionally univentricular hearts (FUHs) and ductal-dependent pulmonary blood flow., Background: Several studies have evaluated the outcomes of DS in a limited number of patients with FUHs. Nonetheless, there is still no consensus regarding the indications for this procedure, and no appropriate patient selection criteria have been devised., Methods: From 2005 to 2015, cardiac catheterization for DS was performed in 68 patients with FUHs. Of these patients, 49 had single source pulmonary blood flow from ductus arteriosus. Procedural and follow-up data were evaluated., Results: The median weight of the patients was 3.6 kg (2.3-6.8 kg), and the median age was 26 days (3 days-8 months). The technical success rate of the procedure was 95% (65 of 68 patients). Mean oxygen saturation increased from 70% ± 7.6% to 87% ± 4.6% (P < 0.0001). Among patients in whom DS was successful, 55 (84.6%) were bridged to a Glenn procedure after a median of 9.1 months (6.4-14 months), and 41 (63%) were successfully bridged to a Glenn procedure without additional interventions. Of the 16 patients with preexisting mild pulmonary artery stenosis, five required a surgical shunt due to progressive branch pulmonary artery stenosis, while nine were bridged to a Glenn operation without shunt placement. Five (7.3%) patients died, including patients who were sent to surgery., Conclusion: DS is a reasonable and effective alternative to surgical shunt placement as a first-stage palliative procedure in patients with FUHs. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Purpose: To develop an estimate of pulmonary vascular resistance (PVR) using blood flow measurements from 3D velocity-encoded phase contract magnetic resonance imaging (here termed 4D MRI)., Materials and Methods: In all, 17 patients with pulmonary hypertension (PH) and five controls underwent right heart catheterization (RHC), 4D and 2D Cine MRI (1.5T) within 24 hours. MRI was used to compute maximum spatial peak systolic vorticity in the main pulmonary artery (MPA) and right pulmonary artery (RPA), cardiac output, and relative area change in the MPA. These parameters were combined in a four-parameter multivariate linear regression model to arrive at an estimate of PVR. Agreement between model predicted and measured PVR was also evaluated using Bland-Altman plots. Finally, model accuracy was tested by randomly withholding a patient from regression analysis and using them to validate the multivariate equation., Results: A decrease in vorticity in the MPA and RPA were correlated with an increase in PVR (MPA: R(2) = 0.54, P < 0.05; RPA: R(2) = 0.75, P < 0.05). Expanding on this finding, we identified a multivariate regression equation that accurately estimates PVR (R(2) = 0.94, P < 0.05) across severe PH and normotensive populations. Bland-Altman plots showed 95% of the differences between predicted and measured PVR to lie within 1.49 Wood units. Model accuracy testing revealed a prediction error of ∼20%., Conclusion: A multivariate model that includes MPA relative area change and flow characteristics, measured using 4D and 2D Cine MRI, offers a promising technique for noninvasively estimating PVR in PH patients. J. MAGN. RESON. IMAGING 2016;44:914-922., (© 2016 International Society for Magnetic Resonance in Medicine.)

Hemoptysis in the children is a rare but serious diagnosis and is even more uncommon in infancy. Mortality is reported and depends on associated illnesses, underlying etiology, and amount of bleeding. A 4-month-old patient presented with significant hemoptysis. Flexible bronchoscopy with differential lavage confirmed the presence and site of origin of hemoptysis. She was managed with cardiac catheterization for embolization of an aorto-pulmonary collateral vessel with immediate complete resolution and no further recurrences. This highlights the importance of cardiac catheterization to detect collateral vessels as a cause for hemoptysis at this age and its successful resolution following embolization. Pediatr Pulmonol. 2016; 51:E31-E33. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Background: Left pulmonary artery stenosis and hypoplasia is a well-recognized complication following surgical palliation of hypoplastic left heart syndrome. These lesions produce increased after load in a circulation in series so need to be effectively treated., Methods: Between 2000 and 2011, 86 patients after surgical palliation for hypoplastic left heart syndrome had left pulmonary artery stents implanted. Median age at implantation was 4.7(1.3-15.2) years and median weight was 16.4(9.3-55.2) kg. Uncovered peripheral vascular stents were implanted (median diameter 10(8-15) mm). This is a retrospective review of the incidence of in-stent restenosis over the medium to long term., Results: During primary stenting procedures, there were 2/88(2.3%) major complications of stent migration with no stroke or mortality. Follow-up was for a median period of 4.1(0.5-13.4) years. Follow-up catheter procedures were performed after a median time of 2.3(0.02-9.6) years in 59 patients (68.6%). 55/59(93.2%) had at most mild restenosis (≤10% loss of stent lumen) and 47/59(79.6%) had no evidence of any restenosis at all caused by neointimal in-growth. Freedom from reintervention was 77% at 5 years including stent dilation to compensate for somatic growth. Freedom from reintervention for restenosis was 93% at 5 years. Restenosis was successfully treated with standard balloon angioplasty or restenting. There was only 1/94 (1.1%) major complication in the follow-up catheterizations of stent embolization with successful transcatheter retrieval., Conclusions: Stenting of the left pulmonary artery after Norwood/Fontan palliation is safe and effective. Stents can be redilated to match somatic growth. The incidence of neointimal proliferation is extremely low and can be addressed by balloon dilation or stent implantation. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Objectives: The aims of this study are (1) to evaluate the safety and feasibility of using optical frequency domain imaging (OFDI) during balloon pulmonary angioplasty (BPA) procedures, (2) to assess the correlations between the vessel area (VA) and luminal area (LA) obtained by OFDI and intravascular ultrasound (IVUS), and (3) to compare inter- and intra-observer variability among measurements taken from OFDI and IVUS images., Background: The BPA in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is an evolving procedure., Methods: Twenty-three consecutive attempts of pair of OFDI and IVUS during BPA were evaluated. All complications that occurred during-BPA and up to 48 hr post-BPA were recorded. Using side branches as landmarks, 48 pairs of regions were chosen to compare measurements of VA and LA., Results: OFDI images can be obtained without any procedurally related complications. Although the VA and LA measurements obtained by OFDI were smaller than those obtained by IVUS, high correlations were found (VA: r = 0.78, P < 0.0001 and LA: r = 0.75, P < 0.0001). Less inter- and intra-observer variability was found when using measurements taken from OFDI versus IVUS images., Conclusions: OFDI during BPA was safe and feasible. The reproducibility of OFDI imaging was excellent and offered a favorable addition to the BPA procedures. © 2016 The Authors Catheterization and Cardiovascular Interventions Published by Wiley Periodicals, Inc., (© 2016 The Authors Catheterization and Cardiovascular Interventions Published by Wiley Periodicals, Inc.)

Introduction: A 24-year-old female presented for percutaneous pulmonary valve placement. She was born with tetralogy of Fallot and had initial palliation with a Blalock-Taussig shunt followed by complete repair at age of 4 years including placement of a homograft conduit in the right ventricle to pulmonary artery position. She had developed severe obstruction in the conduit., Procedure: Angiography showed a heavily calcified conduit with moderate insufficiency. During pre-dilation, a total of six balloons ruptured due to heavy conduit calcification prior to reaching desired inflation diameter. Subsequently, double balloon technique was attempted using two 9-mm Conquest balloons. One of the conquest balloons was then replaced by 16-mm Atlas balloon and conduit dilation was performed. At full inflation, the Conquest balloon ruptured. The deflated ruptured Conquest balloon was kept in the conduit and the Atlas balloon was exchanged for 18 and then 20-mm Atlas balloons and both were used to dilate the conduit. The deflated ruptured Conquest balloon protected the Atlas balloons and conduit angioplasty proceeded successfully without further rupture of any balloon. Subsequently, four stents were placed in the conduit followed by delivery of Melody Valve using a 20-mm Ensemble system with excellent results., Conclusion: This case illustrates a novel method of using the body of a ruptured balloon to protect subsequent balloons from rupture due to heavy conduit calcification. This method requires the presence of two venous access lines but might save time, effort, and cost from repeated balloon ruptures., (© 2015 Wiley Periodicals, Inc.)

The aim of catheter intervention for vascular stenosis is the restoration of lumen area and optimization of distal blood flow. In pediatric practice, this has traditionally been a compromise between less effective balloon angioplasty and bare metal stent insertion with its attendant limitations of size. Bioabsorbable stents offer short-term relief of stenosis, radial support of the healing lesion, return of endothelial function and crucially, in children, the potential for long-term growth. Initial experience, in pediatric practice, with metal-based bioabsorbable stents was relatively disappointing with frequent restenosis secondary to early reabsorption. Design modifications resulting in polymer-based, drug eluting, bioabsorbable vascular scaffolds (BVS) have reportedly overcome some of these faults. We describe the first reported use of a drug eluting BVS in three patients with: (1) A newborn with severe right pulmonary artery (RPA) stensosis post repair of type two common arterial trunk. (2) A child with pulmonary atresia/ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), and (3) An infant with severe left pulmonary artery (LPA) stenosis in the setting of an LPA sling., (© 2015 Wiley Periodicals, Inc.)

Objectives: We report our experience with a transcatheter technique to bypass the lung and to thus improve single-ventricle preload and reduce venous congestion in Fontan patients., Background: In the absence of a dedicated power source to serve the pulmonary circulation and a significantly elevated transpulmonary pressure gradient, fenestration of the Fontan circulation is an option to improve hemodynamics in patients by relieving excessive systemic venous pressure., Methods and Results: From 2005 to 2011, 22 transcatheter fenestrations were performed without any major complications in 19 patients (median age 3.2 years, interquartile range (IQR) 2.7-3.7 years)) with failing Fontan circulation and exceeding systemic venous pressure. In 16 patients, the procedure was performed for acute postoperative failure 1-24 days after surgery. After perforation of the conduit and atrial wall by a Brockenbrough needle and gradual balloon dilation, premounted stents were expanded to create a diabolo configuration with flaring stent edges, leaving a slight but definitive central waist. The procedure resulted in regression of pleural effusions and a significant decrease in systemic venous pressure. Clinical improvement was observed in 16 of the 19 treated patients. Follow-up demonstrated sustained fenestration in 85% of treated patients for at least 24 months., Conclusion: Transcatheter creation of a Fontan fenestration is a safe approach despite the anatomic gap between the extracardiac conduit cavity and the atrial wall. Stent implantation allows defining the diameter of the fenestration, reduces spontaneous occlusion, and ensures sustained clinical improvement., (© 2015 Wiley Periodicals, Inc.)

Early failure of the Fontan circulation is rare in the current era but remains associated with a high mortality rate. Surgical Fontan takedown has evolved as one of the strategies to stabilize the circulation, improve survival, and allow for a future attempt at Fontan completion. We have completed Transcatheter fontan takedown in three patients with extracardiac conduits 0.8-6 months following their Fontan operations. Superior vena cava flow was redirected into only the pulmonary arteries by occluding the conduit with a vascular plug between the pulmonary arteries and fenestration and unrestrictive inferior vena cava flow was redirected into only the atrium by stenting and enlarging the fenestration. There were no procedure related complications. All patients had resolution of large-volume chylous pleural effusions. One patient had resolution of protein-losing enteropathy, two patients had improvement of plastic bronchitis. Two of three patients remain alive at latest follow-up (4-24 months). This early experience suggests that Transcatheter fontan takedown is technically feasible and may be an alternative to surgical takedown in select patients with early failure of the Fontan circulation., (© 2015 Wiley Periodicals, Inc.)

Balloon angioplasty and stent placement in close proximity to the bifurcation of the branch pulmonary arteries can be challenging. Multiple approaches have been previously described, though none of these approaches both treats bilateral proximal branch pulmonary artery stenosis and provides an anchor for a transcatheter pulmonary valve replacement. We report a novel approach that involves serial stent placement and balloon dilation through the struts of the stent in each pulmonary artery, along with balloon expansion of the proximal portion of the stents to the diameter of the main pulmonary artery. In the two cases we describe, this strategy resulted in significant relief of branch pulmonary artery obstruction without compromising the anatomy of the main pulmonary artery segment. This technique can be an effective way to alleviate stenoses of the bilateral proximal branch pulmonary arteries and provides a landing zone for a future transcatheter pulmonary valve., (© 2015 Wiley Periodicals, Inc.)

Objectives: To evaluate early results, learning-curve changes, and mid-term outcome of arterial duct (AD) stenting in congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC) in a high-volume, tertiary referral center., Background: In spite of wide acceptance as cost-effective alternative to surgical palliation, AD stenting indications, results and mid-term outcome still largely depend on small series, experiences in particular subsets of patients or multicenter series with different approaches and interventional philosophy., Methods: Between April 2003 and December 2013, 119 patients underwent AD stenting as lower-risk palliation of CHD-DPC at our Institution. Procedural and mid-term follow-up data of these patients are reported., Results: The procedure was successfully completed in 93.3% of cases, with a complication rate and in-hospital mortality of 17.6% and 3.6%, respectively. No patient underwent rescue surgical shunt but elective Blalock-Taussig shunt was needed in 15 patients (13.5%). Over time, favorable trends toward higher feasibility and efficacy in complex ductal anatomy as well as lower procedural risk were recorded. Presurgical cardiac catheterization (n = 36) showed significant and balanced pulmonary artery (PA) growth (Nakata Index +113 ± 101%; left PA z-score +87 ± 52%; right PA z-score +97 ± 53%, P < 0.001 for all comparisons), without significant changes of left-to-right PA diameter ratio. PA growth was significantly better in patients with severely hypoplastic PAs at the time of duct stabilization (Nakata Index increase 194 ± 115 vs. 75 ± 61%, P < 0.001)., Conclusions: AD stenting is feasible and effective at low-risk in a high percentage of patients with CHD-DPC, promoting significant and balanced PA growth mainly in patients with hypoplastic main PAs at duct stabilization., (© 2015 Wiley Periodicals, Inc.)

Objectives: We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS)., Background: Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection., Methods: A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed., Results: Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P < 0.05). Post discharge, there were seven reinterventions in the DS group and two reinterventions in the SPS group., Conclusions: DS may provide a safe and effective alternative to SPS in selected patients with PAIVS., (© 2015 Wiley Periodicals, Inc.)