Pulmonary Valve Replacement Research Articles

Sex Differences in Transcatheter Structural Heart Disease Interventions: How Much Do We Know?

The number of structural heart disease interventions has greatly increased in the past decade. Moreover, interest in the sex-specific outcomes of various cardiovascular conditions and procedures has increased. In this review, we discuss the sex differences in the clinical profiles and outcomes of patients undergoing the most commonly performed structural procedures: transcatheter aortic valve replacement, transcatheter edge to edge repair of the mitral and tricuspid valve, transcatheter pulmonary valve replacement, patent foramen ovale closure and left atrial appendage occlusion. We shed light on potential reasons for these differences and emphasize the importance of increasing the representation of women in randomized clinical trials, to understand these differences and support the application of these cutting-edge technologies.

Transcatheter pulmonary valve replacement for congenital heart defects

Transcatheter pulmonary valve replacement for congenital heart defects

Simultaneous Stenting With Edwards SAPIEN Transcatheter Pulmonary Valve Replacement.

Prestenting of the landing zone for transcatheter pulmonary valve replacement (TPVR) with a balloon-expandable valve can dilate a stenotic right ventricular outflow tract (RVOT), prevent paravalvar leak (PVL), and protect against conduit tear. Simultaneous stenting (SS) with the Melody valve has been described, but to our knowledge, SS with a SAPIEN valve has not been reported. We report our experience with this novel technique. A retrospective chart review of patients who underwent TPVR at Rady Children's hospital and UCSD Medical Center was performed. Patients were included if they had underwent SAPIEN TPVR with SS. Rationale for stent choice was a bare metal stent to relieve long-segment stenosis and covered stents to prevent PVL or to protect against conduit tear. A total of 17 cases were identified. The majority of RVOTs were transannular patches (n = 9, 56%), with a minimum diameter of 19.6 ± 5.2 mm, and the most common valve placed was an Edwards SAPIEN 26.0 mm (n = 10, 59%). All SAPIEN valves placed were of the S3 generation. The procedure was successful in all patients, with no conduit tears. Minor complications occurred in 3 patients (17.6%). Simultaneous stent deployment with a SAPIEN TPVR is an alternative 1-step technique for patients who require prestenting. SS simplifies the procedure, has low complication rates, and offers the benefits of a longer landing zone and decreased PVL.

Outcomes of Self-Expanding Transcatheter Pulmonary Valves: Extended Follow-Up of a Prospective Trial

<b>Background:</b> The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). <b>Methods:</b> A total of 38 patients with severe PR (mean age 24.2 ± 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scanning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. <b>Results:</b> All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed under-expanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3–8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8–94.7) and 83.5% (95%CI 73.8–93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. <b>Conclusion:</b> TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture.

20-year follow-up and comparison of homografts with bovine jugular vein conduits in children less than 2 years of age.

Repair of congenital heart defects involving right ventricular outflow tract (RVOT) may require pulmonary valve replacement at time of primary repair or reoperation. This study compares the outcomes of cryopreserved homografts with bovine jugular vein conduits (BJVC) in children < 2 years of age with RVOT reconstruction. Retrospective, single-center review of 70 conduits implanted in 63 patients undergoing right ventricle-to-pulmonary artery reconstruction with valved conduit from 2002 to 2022. A total of 70 conduits were implanted in 63 patients, with mean age of 4.5 ± 6.9 months (range 1 day ‒ 23.5 months).The following conduits were used; homografts 38 (54.2 %), BJVC 32 (45.8 %). During mean follow-up of 6.2 ± 5.6 years, there were 12 deaths, 24 conduit reoperations, and 25 catheter reinterventions. Overall survival, reoperation-free, and catheter reintervention-free survival at 15 years was 82.7 %, 31.2 %, and 25.7 %, respectively. Multivariate analysis revealed that low patient weight, age < 30 days at repair, ventilation time, and ICU length of stay were associated with increased risk of death. The performance of homografts and BJVC is comparable in patients ent between the two groups (Tab. 3, Fig. 3, Ref. 16).

Is the QRS duration useful for determining the optimal timing of pulmonary valve replacement after tetralogy of Fallot repair?

In pulmonary valve replacement (PVR) after tetralogy of Fallot (TOF) repair, the right ventricular end-diastolic and end-systolic volume index (RVEDVI and RVESVI) of cardiac magnetic resonance imaging (cMRI) are often used as indicators of the RV volume. We examined the utility of QRS duration, cardiothoracic ratio (CTR), and plasma brain natriuretic peptide (BNP) as indicators of the appropriate timing of cMRI to assess the RV volume and function before PVR. We assessed the correlation of QRS duration, CTR, and BNP with RVEDVI and RVESVI on cMRI in 26 patients after TOF repair. Fifteen underwent PVR (age, 45.2 ± 11.4years). Twelve underwent post-PVR cMRI. The RV volume change from before to after PVR was investigated. QRS duration, BNP, and CTR were positively correlated with RVEDVI and RVESVI after TOF repair. The post-PVR QRS duration was also positively correlated with post-PVR RVEDVI (p = 0.017) and RVESVI (p = 0.001). From before to after PVR, in 5 cases with QRS duration ≤ 160ms, the QRS duration decreased from 110.4 ± 28.9 to 101.8 ± 30.5ms (p = 0.063). Both RVEDVI and RVESVI decreased to the normal range in 4 of 5 cases. In contrast, in 7 cases with QRS duration > 160ms, the QRS duration decreased from 183.0 ± 17.4 to 160.3 ± 23.8ms (p = 0.013); however, RVESVI did not normalize in 6 of 7 cases. A prolonged QRS duration is a useful marker of RVEDVI and RVESVI enlargement after TOF repair. We recommend performing cMRI before the QRS duration reaches 160ms due to normalization of the RV volume after PVR.

966 ADVANCED ECHOCARDIOGRAPHIC AND CMR FEATURES OF PATIENTS WITH REPAIRED TETRALOGY OF FALLOT IN THE LONG-TERM FOLLOW-UP

Abstract Advanced echocardiographic and CMR features of patients with repaired Tetralogy of Fallot in the long-term follow-up. Anna Gozzi1, Jolanda Sabatino1, Elena Cuppini1, Irene Cattapan1, Alessia Basso1, Jennifer Fumanelli1, Angela Di Candia1, Martina Avesani1, Domenico Sirico1, Biagio Castaldi1, Elena Reffo1, Alessia Cerutti1, Roberta Biffanti1, Giovanni Di Salvo1 1Department for Women's and Children's Health, University Hospital of Padova, Pediatric and Congenital Cardiology Unit, Via Nicolo` Giustiniani, 2, 35128 Padova, Italy Background Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Children and, more frequently young adults, with repaired ToF develop late complications. Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in patients with repaired tetralogy of Fallot. The aim of this study was to describe and analyse advanced echocardiographic and CMR features of patients with repaired ToF in the long-term follow-up and to find non-invasive predictors of adverse prognosis. Methods This is a retrospective cohort study. Consecutive 200 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 19 ± 8. The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively. Right myocardial work index (RWI) was calculated as the area of the right ventricular pressure strain loops. From RWI, Right Constructive Work (RCW), Wasted Work (RWW) and Work Efficiency (RWE) were estimated. Patients were screened according the age at surgery (&amp;lt;12 months and &amp;gt;12 months) and the time since the repair (&amp;lt; 25 years and ≥ 25 years). A composite end-point of major adverse cardiovascular events (sudden cardiac death, sustained and non-sustained ventricular tachycardia, resuscitated sudden cardiac death, or syncope) was used. Results CPET values were not significantly different in patients with more than 25 years since the corrective surgery. Left ventricular ejection fraction measured either by CMR or echocardiography were slightly but significantly reduced in patients with more than 25 years since the corrective surgery. Patients with older age at surgery (&amp;gt;12 months) have significantly reduced V02max at CPET, larger RA and LA areas and larger RV basal diameter. During a median follow-up of 2.9 years, 1 patient died suddenly, 5 had documented ventricular tachycardia, and other 4 had syncope. On univariate analysis, echocardiographic left atrial area/BSA (P=0.044) and right ventricular longitudinal strain (P=0.010) were significantly related to the combined end point. Patients with reported VT at Holter ECG presented significantly reduced right ventricular function in terms of CMR-EF, echocardiographic FAC and RWI. Finally, TOF patients with borderline/impaired predicted VO2 max at CPET presented with significantly reduced RV freewall longitudinal strain and right ventricular myocardial work index. Conclusion Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF - operated patients could be excellent up to 25 years after the repair. However, patients with older age at surgery have relative reduced functional status compared with those undergoing early repair. The echocardiographic and CMR evaluation of RV function are usually related to worse prognosis, recurrency of VT and reduced functional status in patients with repaired TOF. Right ventricular myocardial work index is feasible in patients with repaired TOF; disadvantageous right ventricular work may be a more sensitive indicator of right ventricular impairment compared with standard echocardiographic parameters, and is able to predict exercise capacity.

Straightened Segmentation in 4D Cardiac CT: A Practical Method for Multiparametric Characterization of the Landing Zone for Transcatheter Pulmonary Valve Replacement

Cardiac computed tomography angiography (C-CTA) is crucial in assessing the right ventricular outflow tract (RVOT) prior to a transcatheter pulmonary valve replacement (TPVR), as an incorrect evaluation can make the procedure more challenging and can lead to device-related complications. This study aimed to evaluate the feasibility and accuracy of 4D straightened segmentation for a landing zone analysis over anatomical segmentation. Seven pre-operative CTAs and seven post-operative CTAs were used to measure the cross-sectional area, circumference, and diameters at five selected planes as the landing zone for TPVR and compared these to the 4D straightened model with the anatomical model. Furthermore, the right ventricular volume, stent volume, and 4D ellipticity index were calculated from the 4D straightened model. The 4D straightened segmentation had comparable accuracy and efficacy for the measurements at the landing zone. The cross-sectional area and the circumference varied greatly at the RVOT and the basal plane of the pulmonary valve compared with the other three planes of the 4D straightened models from the pre-operative CTAs; however, only the values at the RVOT were found to vary greatly from the post-operative CTAs. The 4D straightened model can provide accurate measurements and is thus a useful method for the periprocedural evaluation of TPVR.

Reversal of Right Ventricular Remodeling After Correction of Pulmonary Regurgitation in Tetralogy of Fallot

In the sheep model with pathophysiologic changes similar to patients with repaired TOF, severe PR leads to fibrotic changes in the RV. Pulmonary valve replacement reverses these fibrotic changes. Early valve replacement led to a quick RV recovery, and in time there was no difference in outcome between early and late valve replacement. These data support the benefit of valve replacement for RV function and suggest that there is a margin in the timing of the surgery. The fibrotic changes correlated well with the circulating biomarker PICP, which can have an added value in the clinical follow-up of patients with repaired TOF.

Valvular and aortic surgery in an adult patient late after repaired tetralogy of Fallot

BackgroundThe most common complication after tetralogy of Fallot repair is pulmonary valve regurgitation. However, concomitant surgery for tricuspid valve regurgitation and/or aortic dilatation is occasionally required.Case presentationA 56-year-old woman who underwent surgery for tetralogy of Fallot at age 29 years was diagnosed with progressive tricuspid valve regurgitation, hepatosplenomegaly, and thrombocytopenia. Moreover, computed tomography and transesophageal echocardiography revealed regurgitation in pulmonary and mitral valves and dilatation of the ascending aorta. One month after splenectomy for increasing platelet count, she underwent pulmonary valve replacement, tricuspid and mitral valve annuloplasty, and ascending aortic replacement. Postoperatively, the intensive care stay was complicated because of ventricular dysfunction and long-term ventilation. After 1 week, the patient was disconnected from the respirator, and she was transferred on the 55th postoperative day to another hospital for rehabilitation, without permanent disabilities.ConclusionWe herein reported a patient with repaired TOF who successfully underwent PVR, tricuspid and mitral valve annuloplasty, and ascending aortic replacement.

Transcatheter pulmonary valve replacement in congenital heart diseases.

Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non-valved conduit to connect the right ventricle (RV) to the pulmonary artery (PA) or just patch enlargement of the native RVOT. With time, this RV-PA conduit will degenerate with deterioration of function, either causing pulmonary stenosis or pulmonary regurgitation. This RVOT dysfunction may result in RV dilation, RV dysfunction, and eventual RV failure and arrhythmias. Multiple surgical pulmonary valve replacement (PVR) is often required throughout the patient's lifetime. Patients are subjected to increased risks with each additional cardiac operation. Transcatheter PVR (TPVR) has been developed over the past two decades as a valuable non-surgical alternative to restore the RVOT and RV function, and hence reduce patients' lifetime risks related to surgery. This article will discuss the long-term results of TPVR which are demonstrated to be comparable to surgical results and the latest development of large pulmonary valves which will allow TPVR to be performed on native or larger RVOT.

Ten Years of Percutaneous Pulmonary Valve Implantation in Australia and New Zealand

This study sought to investigate the characteristics, morbidity (including the rate of infective endocarditis and valve replacement) and mortality of individuals undergoing percutaneous pulmonary valve implantation in Australia and New Zealand since the procedure has been performed. The outcomes of percutaneous pulmonary valve implantation in Australia and New Zealand have not been evaluated. Recent international data, including patients from New Zealand, suggests the rate of infective endocarditis is not insignificant. A retrospective multi-site cohort study was undertaken via medical record review at the centres where percutaneous pulmonary valve implantation has been performed. All procedures performed from 2009-March 2018 were included. Individuals were identified from local institution databases. Data was collected and analysed including demographics, details at the time of intervention, haemodynamic outcome, post procedure morbidity and mortality. Multi-site ethics approval was obtained. One hundred and seventy-nine (179) patients attended the cardiac catheter laboratory for planned percutaneous pulmonary valve implantation. Of these patients, 172 underwent successful implantation. Tetralogy of Fallot and pulmonary atresia were the most common diagnoses. The median age at procedure was 19 years (range 3-60 yrs). There was a significant improvement in the acute haemodynamics in patients undergoing percutaneous pulmonary valve implantation for stenosis. Seven (7) patients (3.9%) experienced a major procedural/early post procedure complication (death, conversion to open procedure, cardiac arrest), including two deaths. The annualised rates of infective endocarditis and valve replacement were 4.6% and 3.8% respectively. There was one death related to infective endocarditis in follow-up. Percutaneous pulmonary valve replacement is a relatively safe method of rehabilitating the right ventricular outflow tract.

Right ventricular echocardiographic remodeling after pulmonary valve replacement in repaired Tetralogy of Fallot

BackgroundMany patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution. ResultsIn a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003–2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up. ConclusionsPulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery.

Current Practice in Carcinoid Heart Disease and Burgeoning Opportunities.

Cardiac surgery with tricuspid valve and potentially pulmonic valve replacement at an experienced center is currently the most effective strategy available for the treatment of carcinoid heart disease. Cardiac surgery for carcinoid heart disease requires a multidisciplinary team including cardiology, medical oncology, cardiothoracic anesthesia, and cardiac surgery. Without cardiac surgery, morbidity and mortality from carcinoid heart disease is high. Aggressive management of carcinoid before and after cardiac surgery is critical. Over time, though, circulating carcinoid hormones can lead to destruction of prosthetic valves as well, resulting in recurrent right heart failure. Percutaneous options for valve repair may be on the horizon for management of carcinoid heart disease.

Trans cather pulmonary valve replacement in carcinoid heart disease: a potential option in selected cases

Trans cather pulmonary valve replacement in carcinoid heart disease: a potential option in selected cases

Abstract 14737: Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia With Intact Ventricular Septum and Critical Pulmonary Stenosis

Background: Patients with pulmonary atresia with intact ventricular septum (PA/IVS) and critical pulmonary stenosis (CPS) managed to a biventricular circulation may eventually require pulmonary valve replacement (PVR) for pulmonary regurgitation (PR). Right ventricular (RV) remodeling after PVR has been well described in tetralogy of Fallot (TOF); we sought to investigate RV volumetric and functional changes in PA/IVS using CMR. Methods: A retrospective cohort of PA/IVS and CPS patients who underwent PVR at Boston Children’s Hospital from 1995-2021 with CMR before and after PVR was matched 1:3 with TOF patients by age at PVR. Patients with initial intervention &gt;2 weeks of age or 1.5 ventricle circulation were excluded. Median regression modeling accounting for matching was performed with reduction in indexed right ventricular end diastolic volume (RVEDVi) post PVR as the primary outcome, adjusting for covariates including tricuspid regurgitation (TR) and PVR valve size. Results: Twenty PA/IVS or CPS patients (cases) were matched with 60 TOF (controls), with median age at PVR 14 years; at median follow-up 8 years post PVR, 95% were NYHA Class I. Pre-PVR RVEDVi was similar between groups (165 vs 167 ml/m 2 , p=0.7), although cases had higher RV EF (51.4 vs 48.6%, p=0.03), TR fraction (23 vs 9%, p=0.008), and a trend toward lower RV mass; PR fraction, LV volumes and EF were similar. Pre-PVR RV free wall and LV longitudinal strain were similar, although LV circumferential strain was worse in cases (-15.6 vs -17.1, p=0.01). At median 2 years after PVR, RVEDVi was similarly reduced from 166 to 118 ml/m 2 (p=0.73), although cases had higher RV EF (52.3% vs 46.9%, p=0.007) with less reduction in indexed RV mass (Δ4.5 vs 9.6 g/m 2 , p=0.02); LV volumes and EF were similar. Post PVR, RV and LV longitudinal strain remained unchanged both within and between groups; LV circumferential strain was similar post PVR and remained lower in cases vs controls. Conclusion: Patients with PA/IVS after PVR demonstrate similar RV remodeling to TOF patients, with lesser reduction in RV mass and comparatively better post RV EF. CMR strain imaging found no significant pre-post differences in RV or LV systolic parameters. Further investigation is needed to evaluate for changes in diastolic parameters.

Abstract 13633: Differences in Right and Left Ventricular Remodeling After Tetralogy of Fallot Repair in Patients With and Without Pulmonary Valve Replacement

Background: Pulmonary valve replacement (PVR) is required to address residual lesions after tetralogy of Fallot repair (rTOF). We wished to determine the progression of ventricular dilation after rTOF, using cardiac magnetic resonance (CMR) data in children, to determine age related RV and LV volume differences in patients who did or did not require PVR. Methods: Single center, retrospective longitudinal study, including 214 patients with rTOF who had primary repair before 24 months and at least one CMR performed from 2000-2020. During follow-up, 142 patients (66.3%) had PVR at median age 12 years (IQR 9-15.5). Linear mixed effect modeling was used to evaluate time-related changes in ventricular volumes by log-transformed age at CMR adjusted for sex and regurgitant lesion status. Patients were grouped as per need for PVR during follow-up. Results: A total of 323 CMR from 201 patients performed before PVR were analyzed. The median age at first CMR was 9.4 years (IQR 5.9-12.3): 126 (62.7%) patients had one CMR, 34 (16.9%) had 2 and 41 (20.4%) had 3 or more. Prior to PVR there was steeper time-related RV dilation compared to non-PVR requiring patients: RVEDVi = 19.4 (CI 15.1 - 23.7) vs 2.8 (CI -2.5 - 8.2) ml/m 2 /log2year, p&amp;lt0.001; RVESVi = 11.9 (8.9 - 14.8) vs 0.8 (CI -2.9 - 4.4) ml/m 2 /log2year, p&amp;lt0.001. A time-related increase in LV volume also occurred in the PVR group compared to non-PVR: LVEDVi = 7.2 (CI 4.7 - 9.7) vs 1.5 (CI -1.6 - 4.6) ml/m 2 /log2year, p=0.005; LVESVi = 3.2 (CI 1.9 - 4.6) vs -0.4 (CI -2.1 - 1.3) ml/m 2 /log2year, p=0.001) (Figure 1). The factors associated with PVR were presence of regurgitant residual lesion, more prolonged QRS duration and transannular patch as surgical technique for TOF repair. Conclusion: Early and significantly increased progression of RV dilation and LV dilation are identifiable by CMR in patients destined to require PVR following rTOF. Early CMR surveillance may be indicated to permit early identification of progressive remodeling.

Abstract 14051: QRS Duration After Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot: Prediction of Ventricular Arrhythmias and Correlation With Right Ventricular Size

Background: Tetralogy of Fallot (ToF) patients face complications such as heart failure and ventricular arrhythmias (VA) after early repair which may be mitigated by pulmonary valve replacement (PVR). Prior studies have shown significant decreases in QRS duration and right ventricular (RV) size following PVR. We aimed to determine whether QRS duration reduction independently identifies patients at lower risk of VA and/or correlates with a decrease of RV size on cardiac magnetic resonance imaging (CMR) with long-term follow-up. Methods: We retrospectively identified patients ≥18 years old with repaired ToF who underwent surgical or transcatheter PVR, respectively, at our tertiary care center. Demographics, imaging, and ECGs (pre-PVR, 30 days to 1-year post-PVR, and closest to follow up CMR) were collected. Patients with ventricularly paced rhythm were excluded. The composite primary outcome was defined as sustained VT, ICD shock for sustained VT or inducible VT on EP study. Results: During the study period, 85 patients were included (median follow-up 3.6 years; median age 34 years; 51% females, 68% surgical PVR and median LVEF 56%). The primary outcome was observed in 8 (9.6%) patients. QRS duration decreased by 5 ms (pre-PVR mean 154±28 ms to 149±28ms post-PVR; p=0.0001). Increased age at PVR (OR 1.1 per year), QRS≥180ms post-PVR, no reduction in QRS after PVR (ΔQRS ≤ 0ms), and a history of VT were significantly associated with the composite outcome. (Table) The change in QRS was linearly correlated with the change in RVEDVi (R = 0.66; p=0.002). Conclusion: In conclusion, adults with repaired ToF experience a reduction in QRS duration post-PVR. This change correlates with the change in the RV size post-operatively. A QRS ≥180ms post-PVR, no reduction in QRS, increased age at repair, and a history of VT independently predict the occurrence of VT post-PVR. Patients with these risk factors may warrant closer monitoring and/or ICD consideration.

Abstract 14967: Rate of Growth of the Right Ventricular Outflow Tract in Repaired Tetralogy of Fallot: A Cardiovascular Magnetic Resonance Imaging Study

Introduction: Patients with repaired tetralogy of Fallot (rTOF) often require pulmonary valve replacement (PVR). Transcatheter PVR is a less invasive alternative to surgery but many right ventricular outflow tracts (RVOTs) are too large. Understanding the rate of RVOT growth may help optimize timing of referral. This study examined the growth of the RVOT over time in rTOF. Methods: We retrospectively analyzed serial cardiac MRI (CMR) data from 121 patients with TOF (median 3 MRIs per patient; range 2 to 10) to calculate the rate of increase in perimeter-derived diameters of the proximal infundibulum, middle RVOT, and distal RVOT at the point of PA bifurcation (Figure 1). The median age at first CMR was 14.7 years (range 3.3, 43.6 years) with a mean interval between first and last CMR of 8 years ± 3 years. Results: All parameters increased over time. The proximal RVOT increased at a rate of 4.1 mm per 10 years (p &lt; 0.001); the mid and distal RVOT both increased at a rate of 3.1 mm per 10 years (p &lt; 0.001) (Figure 2). The RVOT was larger on average in patients with a higher RV end diastolic volume (RVEDV) at first MRI and in patients with a higher pulmonary regurgitation (PR) fraction at first MRI. Patients with greater increases in RV EDV (Spearman correlation 0.48, p &lt; 0.001) or PR fraction (Spearman correlation 0.27, p = 0.003) over time had greater RVOT growth. Conclusions: This largest-to-date serial CMR study of patients with rTOF detailed the rate of growth of the RVOT over time. More rapid growth was associated with higher PR fraction and larger RVEDV. This data may be useful in the design and clinical use of transcatheter pulmonary valves.

Long-term outcome of repaired tetralogy of Fallot: Survival, tachyarrhythmia, and impact of pulmonary valve replacement.

Pulmonary valve replacement (PVR) is recommended for severe pulmonary regurgitation in repaired tetralogy of Fallot (rTOF). The purpose of this study was to investigate the event rate and effectiveness of PVR. A retrospective study of tetralogy of Fallot patients who survived total repair from 1970 to 2020 was conducted. We identified 1744 rTOF patients; 86.6% with classic rTOF, 11.5% with pulmonary atresia, 0.8% with endocardial cushion defect, and 1.1% with absent pulmonary valve. Annual risks of tachyarrhythmia/sudden cardiac arrest (SCA) increased to 0.295% and 1.338% in patients aged 10-30 and 30-60 years, respectively, without sex predominance. PVR (223 surgical and 39 percutaneous) event rate was 34.7% ± 2.1% by 30 years after repair (annual risk: 1.57% between 10 and 30 years after repair). The second PVR rate was 9.9% ± 4.1% by 20 years after the first PVR. Tachyarrhythmia/SCA risk was higher in PVR patients than in No PVR patients and was reduced in PVR patients without tachyarrhythmia/SCA before PVR. However, survival in patients with ventricular tachyarrhythmia/SCA still was better after PVR. At PVR, 13% of patients had tachyarrhythmia/SCA, which was the major predictor of events after PVR. Before PVR, although the ventricular tachyarrhythmia/SCA risks included QRS duration >160 ms and New York Heart Association functional class III or IV, supraventricular tachyarrhythmia was associated with PVR age ≥28 years and N-terminal pro-brain natriuretic peptide >450 pg/mL. Tachyarrhythmia/SCA occurrence and the need for PVR increased with age during young adulthood. PVR reduced subsequent arrhythmias only in those patients without arrhythmias before PVR.