Right Ventricle-to-pulmonary Artery Research Articles

Aspirin Responsiveness in a Cohort of Pediatric Patients with Right Ventricle to Pulmonary Artery Conduits and Transcatheter Valve Replacement Systems.

The aim of this study was to determine the rate of aspirin responsiveness in a cohort of pediatric patients with in situ xenograft valved right ventricle to pulmonary artery (RV-PA) conduits and/or transcatheter valve replacements (TVR). Aspirin is routinely prescribed to these patients. Optimizing anti-platelet therapy could promote valve longevity and reduce the risk of infective endocarditis in this at-risk group. This was a prospective, observational study. Patients were recruited from both ward and outpatient settings. Patients were eligible if under 18 years and taking aspirin. Non-response to aspirin was defined as >20% platelet aggregation using light transmission platelet aggregometry (LTA) and <50% platelet inhibition by thromboelastography with platelet mapping (TEGPM). Participants were invited to provide a confirmatory sample in cases of aspirin resistance and dose adjustments were made. Thirty patients participated. Median age was 9 years (2 months to 18 years). The majority (93%) had complex right ventricular outflow tract pathology. 13 (43%) had an RV-PA conduit and 24 (80%) had a TVR, with valve situated in conduit in 7 (23%) cases. Rate of aspirin non-response on initial testing was 23% (n=7/30) with median LTA 74.55% (60-76%) and TEG 13.25% (0-44%) in non-responders. Non-responders were more likely to be under 1 year. Two patients required dose increases and one patient non-adherence to dose was identified. Four patients on repeat testing were responsive to aspirin by laboratory tests. The rate of aspirin non-response on laboratory testing in this cohort of patients was 23% and resulted in therapeutic intervention in 10%.

Right ventricle to pulmonary artery conduit: a comparison of long-term graft-related events between bovine jugular vein conduit, aortic homograft, and porcine-valved conduits.

The optimal conduit for right ventricle to pulmonary artery (RV-PA) reconstruction does not exist. Reinterventions are common due to conduit stenosis and endocarditis. Tailoring conduit choice according to patients' characteristics could improve the outcomes. The study aimed to compare graft-related events (infective endocarditis, transcatheter pulmonary valve replacement (PVR), transcatheter conduit dilatation, surgical conduit replacement, and transcatheter pulmonary branch intervention for RV-PA reconstruction using bovine jugular vein, aortic homograft, and porcine-valved conduits. In a retrospective cohort study, 155 patients with 193 procedures that were done in King Faisal Specialist Hospital and Research Center-Jeddah (KFSHRC JED) for implanting RV-PA conduits from 1999 to 2021 were included. The patients were grouped according to the type of conduit into 3 groups; Group 1 (n=153) received bovine jugular vein (BJVs) grafts, Group 2 (n=29) received aortic homografts, Group 3 (n=11) received porcine-valved conduits. Factors associated with graft-related events were evaluated using stepwise Cox regression analysis. Patients with BJVs were significantly younger than those with porcine-valved conduits 3 (P=0.009). The weight of BJVs patients was significantly lower than homografts (P=0.002) and porcine-valved conduits patients 3 (P<0.001). The conduit size was as expected significantly lower in BJVs patients than patients who received porcine-valved conduits (P<0.001) and patients who received aortic homografts (P<0.001). There was no difference between Group 2 and 3 (P=0.084). Operative mortality occurred in 13 patients: 12 (7.84%) with BJV conduit and 1 (9.09%) with porcine valved conduit (P=0.351). Male gender [odds ratio (OR): 10.04; 95% confidence interval (CI): 1.28-78.86; P=0.028] and smaller conduit size (OR: 0.78; 95% CI: 0.61-0.99; P=0.048) were associated with increased operative mortality. Freedom from graft-related events at 5 and 10 years was 67% and 52% in BJVs patients, 74% and 36% in patients who received aortic homografts, and 53% in patients who received porcine-valved conduits. Factors associated with increased graft-related events were male gender (HR: 1.58; 95% CI: 1.004-2.50, P=0.048) and younger age (HR: 0.995; 95% CI: 0.991-0.999, P=0.041). RV-PA reconstruction was associated with low mortality, unrelated to the conduit type. Reinterventions for graft-related events were common. The durability and graft-related events might be comparable among BJV grafts, aortic homografts, and porcine-valved conduits. Factors associated with increased graft-related events in this study were male gender and younger age.

Outcomes after Biventricular Repair Using a Conduit between the Right Ventricle and Pulmonary Artery in Infancy.

This study investigated the outcomes of biventricular repair using right ventricle to pulmonary artery (RV-PA) conduit placement in patients aged <1 year. Patients aged <1 year who underwent biventricular repair using an RV-PA conduit between 2011 and 2020 were included in this study. The outcomes of interest were death from any cause, conduit reintervention, and conduit dysfunction (peak velocity of ≥3.5 m/sec or moderate or severe regurgitation). In total, 141 patients were enrolled. The median age at initial conduit implantation was 6 months. The median conduit diameter z-score was 1.3. The overall 5-year survival rate was 89.6%. In the multivariable analysis, younger age (p=0.006) and longer cardiopulmonary bypass time (p=0.001) were risk factors for overall mortality. During follow-up, 61 patients required conduit reintervention, and conduit dysfunction occurred in 68 patients. The 5-year freedom from conduit reintervention and dysfunction rates were 52.9% and 45.9%, respectively. In the multivariable analysis, a smaller conduit z-score (p<0.001) was a shared risk factor for both conduit reintervention and dysfunction. Analysis of variance demonstrated a nonlinear relationship between the conduit z-score and conduit reintervention or dysfunction. The hazard ratio was lowest in patients with a conduit z-score of 1.3 for reintervention and a conduit z-score of 1.4 for dysfunction. RV-PA conduit placement can be safely performed in infants. A significant number of patients required conduit reintervention and had conduit dysfunction. A slightly oversized conduit with a z-score of 1.3 may reduce the risk of conduit reintervention or dysfunction.

20-Year follow-up and comparison of valved conduits used for right ventricular outflow tract reconstruction: single-centre, propensity score match analysis.

Surgical repair of complex congenital heart defects with hypoplasia or atresia of the right ventricular outflow tract (RVOT) may require pulmonary valve implantation or replacement during the primary repair or reoperation. The purpose of this study is to evaluate the outcomes of cryopreserved homografts, bovine jugular vein conduits and decellularized Matrix P Plus N conduits in patients undergoing RVOT reconstruction at a single centre. Retrospective, single-centre review of 173 patients with 199 conduits undergoing right ventricle-to-pulmonary artery reconstruction with valved conduit from 2002 to 2022. A total of 199 conduits were implanted in 173 patients (62.8% male), with a mean age of 8.97 ± 8.5 years. The following 3 types of conduits were used: homografts 129 (64.8%), bovine jugular vein conduits 45 (22.7%) and Matrix P Plus N 25 (12.5%). During the mean follow-up duration of 8.6 ± 5.8 years, there were 20 deaths, 35 conduit reoperations and 44 catheter reinterventions. Overall survival, reoperation-free and catheter reintervention-free survival at 20 years were 83%, 67.8% and 65.6%, respectively. Multivariable Cox analysis identified younger patient age, smaller conduit size, low patient weight and primary diagnosis of common arterial trunk as risk factors for reoperation and catheter reintervention. Long-term outcomes of reconstruction of the RVOT using homografts, bovine jugular vein and Matrix P Plus N conduits were acceptable. The reoperation rate for conduit dysfunction did not differ significantly among groups. Over time, the need for conduit replacement was higher in smaller conduits and in patients with common arterial trunk diagnosis.

Clinical Value of a Novel Three-Dimensional Echocardiography–Derived Index of Right Ventricle–Pulmonary Artery Coupling in Tricuspid Regurgitation

Echocardiographic surrogates of right ventricle-to-pulmonary artery (RV-PA) coupling have been reported to be associated with outcomes in patients with secondary tricuspid regurgitation (STR). However, pulmonary artery systolic pressure (PASP) is difficult to estimate using echocardiography in patients with severe STR. The aim of the present study was to evaluate the predictive power of a surrogate of RV-PA coupling obtained using right ventricular (RV) volumes measured on three-dimensional echocardiography. One hundred eight patients (mean age, 73±13years; 61% women) with moderate or severe STR were included. At a median follow-up of 24months (interquartile range, 2-48months), 72 patients (40%) had reached the composite end point of death of any cause and heart failure hospitalization. RV-PA coupling was computed as the ratio between RV forward stroke volume (SV) (i.e., RV SV-regurgitant volume) and RV end-systolic volume (ESV). RV forward SV/ESV was significantly more related to the composite end point than RV ejection fraction (area under the curve, 0.85 [95% CI, 0.78-0.93] vs 0.73 [95% CI, 0.64-0.83], respectively; P=.03). A value of 0.40 was found to best correlate with outcome. On multivariate Cox regression, RV forward SV/ESV, tricuspid annular plane systolic excursion/PASP, and RV free wall longitudinal strain/PASP were all independently associated with the occurrence of the composite end point when added to a group of parameters including STR severity (severe vs moderate), atrial fibrillation, pulmonary arterial hypertension, right atrial volume, RV end-diastolic volume, and RV free wall longitudinal strain. RV forward SV/ESV<0.40 (HR, 3.36; 95% CI, 1.49-7.56; P<.01) carried higher related risk than RV free wall longitudinal strain/PASP<-0.42%/mm Hg (HR, 3.1; 95% CI, 1.26-7.84; P=.01) and tricuspid annular plane systolic excursion/PASP<0.36mm/mm Hg (HR, 2.69; 95% CI, 1.29-5.58; P=.01). RV ejection fraction did not correlate independently with prognosis when added to the same group of variables. RV forward SV/ESV is associated with the risk for death and heart failure hospitalization in patients with STR.

Right ventricular to pulmonary artery coupling and outcome in patients with cardiac amyloidosis.

To investigate the prognostic value of the right ventricle-to-pulmonary artery (RV-PA) coupling in patients with either transthyretin (ATTR) or immunoglobulin light-chain (AL) cardiac amyloidosis (CA). Overall, 283 patients with CA from 3 Italian high-volume centres were included (median age 76 years; 63% males; 53% with ATTR-CA, 47% with AL-CA). The RV-PA coupling was evaluated by using the tricuspid annular plane systolic excursion/pulmonary artery systolic pressure (TAPSE/PASP) ratio. The median value of TAPSE/PASP was 0.45 (0.33-0.63) mm/mmHg. Patients with a TAPSE/PASP ratio <0.45 were older, had lower systolic blood pressure, more severe symptoms, higher cardiac troponin and N-terminal pro-B-type natriuretic peptide levels, greater left ventricular (LV) thickness, and worse LV systolic and diastolic function. A TAPSE/PASP ratio <0.45 was independently associated with a higher risk of all-cause death or heart failure (HF) hospitalization [hazard ratio (HR) 1.98, 95% confidence interval (CI) 1.32-2.96; P = 0.001] and all-cause death (HR 2.18, 95% CI 1.31-3.62; P = 0.003). The TAPSE/PASP ratio reclassified the risk of both endpoints [net reclassification index 0.46 (95% CI 0.18-0.74) P = 0.001 and 0.49 (0.22-0.77) P < 0.001, respectively], while TAPSE or PASP alone did not (all P > 0.05). The prognostic impact of the TAPSE/PASP ratio was significant both in AL-CA patients (HR for the composite endpoint 2.47, 95% CI 1.58-3.85; P < 0.001) and in ATTR-CA (HR 1.81, 95% CI 1.11-2.95; P = 0.017). The receiver operating characteristic curve showed that the optimal cut-off for predicting prognosis was 0.47 mm/mmHg. In patients with CA, RV-PA coupling predicted the risk of mortality or HF hospitalization. The TAPSE/PASP ratio was more effective than TAPSE or PASP in predicting prognosis.

A 30-Year Survival Analysis of 232 Patients (Using National Death Index) of Right Ventricle to Pulmonary Artery Conduit Insertion in Adults With Congenital Heart Disease

This study aimed to assess the survival of patients with congenital heart disease who underwent right ventricle to pulmonary artery (RV-to-PA) conduit insertion.

Outcomes of Surgical Repair for Truncus Arteriosus: A 30-Year Single-Center Experience.

We investigated the long-term outcomes of truncus arteriosus repair at a single institution with a 30-year study period. Patients who underwent repair of truncus arteriosus between 1993 and 2022 were reviewed retrospectively. Factors associated with early mortality, overall attrition, and reintervention were identified using appropriate statistical methods. In total, 42 patients were enrolled in this study. The median age and weight at repair were 26 days and 3.5 kg, respectively. Thirty patients (71.4%) underwent 1-stage repair. There were 8 early deaths (19%). In the univariable analysis, undergoing surgery before 2011 was associated with early mortality (p=0.031). The overall survival rate at 10 years was 73.8%. In the multivariable analysis, significant truncal valve (TrV) dysfunction (p=0.010), longer cardiopulmonary bypass time (p=0.018), and the earlier era of surgery (p=0.004) were identified as risk factors for overall mortality. During follow-up, 47 reinterventions were required in 27 patients (64.3%). The freedom from all-cause reintervention rate at 10 years was 23.6%. In the multivariable analysis, associated arch obstruction (p<0.001) and significant TrV dysfunction (p=0.011) were identified as risk factors for all-cause reintervention. Arch obstruction (p=0.027) and a number of TrV cusps other than 3 (p=0.014) were identified as risk factors for right ventricle to pulmonary artery (RV-PA) reintervention, and significant TrV dysfunction was identified as a risk factor for TrV reintervention (p=0.002). Despite recent improvements in survival outcomes after repair of truncus arteriosus, RV-PA or TrV reinterventions were required in a significant number of patients during follow-up.

The prognostic role of the echocardiographic tricuspid annular plane systolic excursion/systolic pulmonary arterial pressure (TAPSE/sPAP) ratio and its relationship with NT-proANP plasma level in systemic sclerosis.

The tricuspid annular plane systolic excursion/systolic pulmonary arterial pressure (TAPSE/sPAP) ratio is an echocardiographic estimation of the right ventricle to pulmonary artery (RV/PA) coupling, with a validated prognostic role in different clinical settings. Systemic sclerosis (SSc) patients without evident cardiovascular involvement frequently display subtle RV impairment. The amino-terminal atrial natriuretic peptide (NT-proANP) plasma level relates to SSc disease progression and mortality. We aimed to assess the prognostic value of the TAPSE/sPAP ratio and its relationship with NT-proANP plasma level in SSc patients without overt cardiovascular involvement. We retrospectively analysed 70 SSc consecutive patients, with no clinical evidence of cardiovascular involvement or pulmonary hypertension (PH), and 30 healthy controls (HC) in a retrospective, single-centre study. All SSc patients underwent recurrent clinical and echocardiographic assessments and NT-proANP plasma level was assessed at baseline. SSc-related cardiovascular events and deaths were extracted during a 6-year follow-up. The complete work-up for the diagnosis, treatment and management of PH performed along the 6 years of follow-up referred to the 2015 European Society of Cardiology guidelines. Systemic sclerosis patients showed lower TAPSE/sPAP ratio at baseline compared to HC [SSc median value = 0.71 mm/mmHg, (IQR 0.62-0.88) vs. HC median value = 1.00 mm/mmHg, (IQR 0.96-1.05); p < 0.001]. Multivariable Cox analysis revealed TAPSE/sPAP ratio as an independent predictor for SSc-related cardiovascular events [HR = 3.436 (95% CI 1.577-7.448); p = 0.002] and mortality [HR = 3.653 (95% CI 1.712-8.892); p = 0.014]. The value of TAPSE/sPAP ratio < 0.7 mm/mmHg was identified as an optimal cut-off for predicting adverse outcomes (p < 0.001) by receiver operating characteristic (ROC) analyses. NT-proANP level significantly related to TAPSE/sPAP ratio (r = 0.52, p < 0.001). TAPSE/sPAP ratio combined with NT-proANP showed an overall significant prognostic role in this SSc population, confirmed by Kaplan-Meier analysis (Log rank p < 0.001). The TAPSE/sPAP ratio, as an index of RV/PA coupling, is an affordable predictor of cardiovascular events and mortality in SSc and, combined with NT-proANP level, may improve the clinical phenotyping and prognostic stratification of SSc patients.

Abstract 13000: The Effect of Flow Dynamics on the Neotissue Formation of 3D Printed Tissue Engineered Vascular Graft

Introduction: Complex vascular reconstruction such as that of the right ventricle to pulmonary artery (RVPA) and the aortic arch remains challenging due to the various 3D geometry of each patient. Tissue-engineered vascular grafts (TEVG) possess not only excellent antithrombotic and anti-infective properties but also the ability to grow as the patient matures. 3D printed patient-specific TEVG can maintain optimal hemodynamics with the growth of the patient and may improve surgical outcomes. We applied our unique 3D printing technology to create patient-specific TEVG for RVPA and the aortic arch. Methods: We acquired MRI and 4-dimensional flow data for the native anatomy of pigs (n=8) to design a patient-specific TEVG of the pulmonary artery (n=5) and the aortic arch (n=3) 4 weeks prior to surgery. The optimal shape of the branched vascular graft (n=8) was designed using a computer-aided design informed by computational flow dynamics analysis. We manufactured and implanted the grafts into the RVPA or aortic arch in the porcine model. The grafts were explanted after surgery at 8 - 12 weeks for evaluation. Pre- and post-op implant flow dynamics data in MRI and histology were analyzed. Results: The local neointimal thickness of TEVG in histology and the corresponding area of wall shear stress in 4D MRI were measured. Low wall shear stress, an aspect of MRI flow analysis, demonstrated a significant correlation with decreased neointimal thickness through histology (R2=0.65, P=0.016), which suggested optimal shape and design is important for appropriate neotissue formation. Conclusions: Our 3D printed custom designed TEVG demonstrated optimal anatomical fit maintaining ideal flow dynamics can attenuate neointimal hyperplasia and appropriate neovessel formation.

Journey Toward Improved Long-Term Outcomes After Norwood-Sano Procedure: Focus on the Aortic Arch Reconstruction.

The disadvantage of right ventricle-to-pulmonary artery (RV-PA) shunt is the need for more unplanned interventions to address stenosis in the shunt or branch pulmonary arteries, as compared to the modified Blalock-Taussig shunt group. Ring-enforced RV-PA PTFE conduit and dunk technique minimized these complications and right ventricle (RV) damage. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation; therefore, most surgeons prefer to use homograft, autologous pericardium, or bovine pericardium to reconstruct the neoaorta. Artificial materials decrease the elastic properties, increase wall stiffness, and decrease the distensibility of the aorta; and as a result, RV function gradually deteriorates. This inelastic reconstructed aorta may be one of the reasons why long-term outcomes after the Fontan procedure are worse in hypoplastic left heart syndrome (HLHS) patients, in comparison to non-HLHS. Reconstruction of the neoaorta without any patch materials, or at least techniques that largely minimize the use of non-autologous materials, will offer a further refinement of our ability to optimize ventriculoarterial coupling and thereby long-term RV function.

Prognostic value of right ventricle to pulmonary artery coupling in transcatheter aortic valve implantation recipients

To investigate the impact of the right ventricle to pulmonary artery (RV-PA) coupling on the outcome of patients undergoing transcatheter aortic valve intervention (TAVI), and to describe changes in right ventricular function, pulmonary hypertension, and their ratio after TAVI. Three hundred and seventy-seven patients from the Italian ClinicalService Project, who underwent TAVI between February 2011 and August 2020, were included. Tricuspid annular plane systolic excursion/pulmonary artery systolic pressure (TAPSE/PASP) ratio was retrospectively calculated as a surrogate of RV-PA coupling. The population was stratified according to TAPSE/PASP using a cut-off of 0.36 mm/mmHg derived from a receiving operating characteristic (ROC) curve. The primary end point was 6-month all-cause death. Compared with patients with a TAPSE/PASP ratio ≥0.36 mm/mmHg (81%), those with TAPSE/PASP ratio <0.36 mm/mmHg (19%) had more comorbidities and were more symptomatic.Moreover, they were more likely to receive general anesthesia and an old generation device. Device success was similar between the two groups. A TAPSE/PASP ratio <0.36 mm/mmHg was associated with a higher risk of all-cause death at 6-months (17.3% versus 5.3%; adjusted HR 2.66; P = 0.041). The prognostic impact of the TAPSE/PASP ratio was stronger than the impact of TAPSE and PASP as separate parameters and was independent of the surgical risk score. Both TAPSE, PASP, and their ratio improved from baseline to 1 month and 6 months after TAVI. A TAPSE/PASP ratio <0.36 mm/mmHg is strongly associated with an increased risk of mortality after TAVI. Providing a left ventricle unloading, TAVI is associated with improvement of both TAPSE, PASP, and their ratio.

Right Ventricle to Pulmonary Artery Coupling Predicts the Risk Stratification in Patients With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.

BackgroundPulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc). PAH has high mortality, and risk assessment is critical for proper management. Whether the right ventricle to pulmonary artery (RV-PA) coupling accurately assesses risk status and predicts prognosis in patients with SSc-associated PAH has not been investigated.MethodsBetween March 2010 and July 2018, 60 consecutive patients with SSc-associated PAH diagnosed by right heart catheterization were enrolled prospectively, and the mean follow-up period was 52.9 ± 27.0 months. The RV-PA coupling was assessed by the ratio of tricuspid annular plane systolic excursion (TAPSE) and pulmonary artery systolic pressure (PASP) which was obtained by transthoracic echocardiography. The simplified risk stratification strategy was applied to assess the risk level of participants, and the endpoint was a composite of all-cause death and clinical worsening.ResultsThe receiver operating characteristic (ROC) curve of the ability to determine high-risk patients identified the optimal cut-off value of the TAPSE/PASP ratio as 0.194 mm/mmHg, and the ratio appeared to be a reliable indicator in the stratification of patients with high risk (area under the curve = 0.878, ROC P-value = 0.003), which showed the highest positive likelihood ratio (LR) (5.4) and the lowest negative LR (0) among a series of echocardiographic parameters. The TAPSE/PASP ratio was an independent predictive factor (HR = 0.01, 95% CI: 0.00–0.77, P = 0.037) for the composite endpoint, and patients with a TAPSE/PASP ratio >0.194 had a better overall survival for both the composite endpoint (log-rank χ2 = 5.961, P = 0.015) and all-cause mortality (log-rank χ2 = 8.004, P = 0.005) compared to the patients with a TAPSE/PASP ≤ 0.194.ConclusionRV-PA coupling assessed by the TAPSE/PASP ratio provides added value as a straightforward and non-invasive approach for predicting risk stratification of patients with SSc-associated PAH. Meanwhile, a lower TAPSE/PASP ratio identified a subgroup with worse prognosis.

Covered Stents in the Management of Aortic Coarctation and Right Ventricular Outflow Tract Obstruction.

To review the use of covered stents in the treatment of coarctation of the aorta (CoA) and right ventricle to pulmonary artery (RV-PA) conduit obstruction. The only commercially available covered stent approved for treatment of CoA and dysfunctional RV-PA conduits is the covered Cheatham-Platinum stent (CCPS). Early outcomes have demonstrated its safety and have suggested its efficacy in treating or preventing aortic wall injury (AWI) or conduit disruption. A recent study of CCPS use for CoA reported a progressive risk of stent fracture over time and a risk of AWI despite the purported protection that the CCPS provides. The use of other covered stents has been reported, but large, systematic studies are lacking. CCPS use may reduce but does not eliminate the risk of conduit disruption or AWI. Structural limitations of the CCPS may predispose it to stent fracture. Access to a broad range of covered stents continues to be an unmet need in the field of congenital interventional cardiology.

Systemic Venous Hypertension and Low Output Are Prevalent at Catheterization in Adults with Pulmonary Atresia and Intact Ventricular Septum Regardless of Repair Strategy

Patients with pulmonary atresia and intact ventricular septum (PA-IVS) require intervention early in life, and most survive to a definitive procedure of either Fontan circulation or right ventricle to pulmonary artery (RV-PA) repair. It remains unknown how surgical strategy impacts hemodynamics and comorbidities in adults. Retrospective analysis of adults (age ≥18 years) with PA-IVS undergoing hemodynamic catheterization at Mayo Clinic, MN between January 2000 through January 2020 was performed. 14 patients in the RV-PA group (71% biventricular, 29% 1.5 ventricle repair) and 19 post-Fontan patients [9 lateral tunnel (48%), 6 atriopulmonary (32%), and 4 extracardiac (21%)] were identified. Median age was 29 (21, 34) years. There were no differences in demographics and laboratory data (including MELD-XI) between groups. All patients assessed for liver disease had evidence of hepatic congestion or cirrhosis (14 in the Fontan group and 4 in the RV-PA group). Invasive hemodynamics were comparable between groups with the Fontan and RV-PA groups having similar systemic venous pressure (15.7±4.4 vs. 14.3±6.2, p = .44) and cardiac output (2.2±0.6 vs. 2.0±0.4 L/min/m2, p = .23). There was no difference in transplant-free survival (p = .92; 5-year transplant-free survival RV-PA 84%, Fontan 80%). Hemodynamic derangements, namely elevated systemic venous pressure and low cardiac output, are prevalent in patients with PA-IVS undergoing cardiac catheterization regardless of surgical strategy.

Postoperative Renal Failure, Shunt Type, and Mortality After Norwood Palliation

BackgroundThe Single Ventricle Reconstruction Trial demonstrated increased risk of death or heart transplant 1 year after Norwood among subjects randomly assigned to modified Blalock-Taussig shunts compared with right ventricle to pulmonary artery (RV-PA) shunts. We used the Single Ventricle Reconstruction Trial public use database to evaluate incidence and risk factors for postoperative renal failure and relationships between renal failure, shunt type, and outcomes after Norwood. MethodsPostoperative renal failure was defined a priori as a threefold rise in creatinine from baseline, or dialysis use, within 7 days of Norwood. We used multivariate logistic regression to evaluate risk factors for postoperative renal failure and Cox hazard regression to determine the association between postoperative renal failure and 1 year after Norwood mortality. ResultsOverall, postoperative renal failure occurred in 8.4% (46 of 544) patients with risk factors including receipt of a modified Blalock-Taussig shunt (adjusted odds ratio [aOR] 3.3, P = .02), low center volume (aOR 2.7, P = .005), two or more preoperative complications (aOR 4.0, P < .001), low birth weight (aOR 3.2, P = .002), postoperative heart block (aOR 8.5, P = .001), and delayed sternal closure (aOR 5.3, P = .026). Renal failure was an independent risk factor for 1-year mortality (aHR 1.9, P = .019). Assessing interaction by shunt type, mortality risk associated with renal failure was greatest in the RV-PA shunt group (aHR 3.3 vs RV-PA shunt without renal failure, P = .001), but was also increased in the modified Blalock-Taussig shunt group (aHR 1.9, P = .03). ConclusionsPostoperative renal failure is common after Norwood and is independently associated with mortality. Although renal failure is more common after modified Blalock-Taussig shunt, the highest mortality risk with renal failure occurs after RV-PA shunt.

Long-term outcomes of right ventricle-to-pulmonary artery conduit insertion in adults with congenital heart disease: survival analysis by National Death Index.

The aim of this study was to investigate the long-term outcomes following right ventricle-to-pulmonary artery (RV-to-PA) conduit insertion of Medtronic Freestyle® porcine valve (MFV) or pulmonary allograft valve (PAV) in adult patients with congenital heart disease. Retrospective medical record review of consecutive RV-to-PA conduit insertion, using either PAV or MFV from 1991 to 2017. Perioperative data and clinic reports were collected. Cause and date of death were obtained from the Australian National Death Index to obtain survival function. In total, 232 patients (median age 31.5 years, interquartile range 25-41 years) underwent RV-to-PA conduit insertion (PAV = 84 and MFV = 148) and were eligible for inclusion [63.8% tetralogy of Fallot (TOF); 11.6% congenital pulmonary stenosis (PS); 24.6% other diagnoses]. The overall median follow-up time was 9.1 years (interquartile range 5.3-12.6 years). The mean gradient was 11.8 ± 7.1 mmHg in PAV and 16.6 ± 9.6 mmHg in MFV patients. Congenital PS patients had 100% survival at 20 years, TOF patients at 5, 10, 15 and 20 years had 99%, 97%, 96% and 96% survival, respectively. Patients with other primary diagnoses at 5, 10, 15 and 20 years had 93%, 91%, 87% and 87% respectively. Freedom from reintervention did not differ significantly at 5 and 10 years between pulmonary allograft (98.6%, 98.6%) and Freestyle® porcine bioprosthesis (97.5%, 93%). Both valves perform equally well with regard to patients' freedom from reoperation, although transvalvular gradient was higher for Freestyle® patients. Congenital PS and TOF patients had better survival than patients with other primary diagnoses.

Outcomes Following Heterotopic Placement of Right Ventricle to Pulmonary Artery Conduits

We sought to evaluate the outcomes following right ventricle to pulmonary artery (RV-PA) conduit placement in pediatric patients, excluding those with a RV-PA conduit for the Ross procedure which is associated with improved conduit durability, partly related to its orthotopic position. Outcomes for 119 patients who underwent RV-PA conduit placement at a single institution from January 2004 to December 2016 were reviewed. Primary outcome measures were reintervention-free survival (RFS) and overall survival. Survival analyses were performed using the Kaplan-Meier method, and risk factors associated with reintervention were evaluated. The median age at the time of conduit placement was 6 months (interquartile range, IQR: 1-14), and the median length of follow-up was 63 months (range: 0-156). During follow-up, 39 patients required conduit-related reintervention, while 6 patients died perioperatively with an overall survival of 90% at 10 years. Among the remaining 113 patients, the RFS at one, five, and ten years was 91% (84%-95%), 72% (60%-80%), and 33% (16%-50%), respectively. The median time to conduit replacement in the series was 43.5 months (IQR: 19.3-76.2). The use of a pulmonary homograft was associated with improved RFS (P = .03), and this was particularly pronounced in comparison with aortic homografts in neonates. Infection was the indication for replacement in only one patient. The majority of the conduits placed during the neonatal period required conduit replacement before the age of five years. Endocarditis was not a common indication for replacement. In neonates and infants, we prefer pulmonary homografts for most indications.

Early multicenter experience of a new balloon expandable MyVal transcatheter heart valve in dysfunctional stenosed right ventricular outflow tract conduits.

Background :Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI.Methods :Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed.Results :Seven patients aged 17–60 years (median 26 years) had stenosed RV-PA conduits implanted 5–17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50–137 mmHg) to 12.7 ± 6.4 mmHg (5–20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10–22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events.Conclusions :Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes.

A Comparative Analysis of Nontraditional Right Ventricle to Pulmonary Artery Conduits: Maybe We Should Judge the Book by Its Cover?

In 1964, Rastelli and colleagues1 inserted a nonvalved pericardial tube as the first right ventricle to pulmonary artery (RV-PA) conduit. As is common with technical advances in any subspecialty, the evolution of how best to reconstruct the right ventricular outflow tract or which conduit is best suited to create the RV-PA connection remains ill-defined. Freedom from reintervention has historically been the defining quantitative metric of success. The ideal option for the conduit would have the characteristics of long-term patency and valve function, availability in a wide range of sizes, good handling, growth potential, low cost, low infectious potential, and no need for anticoagulation.