Impact of pulmonary stenosis on right ventricular global longitudinal strain in repaired tetralogy of Fallot patients post transcatheter pulmonary valve replacement.

BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Impact of Right Ventricular Surface Area-to-Volume Ratio on Ventricular Remodeling After Pulmonary Valve Replacement.

Optimal reverse remodeling of the right ventricle (RV), a sentinel goal of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot, is not fully predicted by volume-based pre-PVR parameters. Our objectives were to characterize novel geometric RV parameters in patients receiving PVR and in controls, and to identify associations between these parameters and chamber remodeling post-PVR. Secondary analysis was performed on cardiac magnetic resonance (CMR) data from 60 patients enrolled in a randomized trial of PVR with and without surgical RV remodeling. 20 healthy age-matched subjects served as controls. The primary outcome was optimal post-PVR RV remodeling (end-diastolic volume index (EDVi) ≤ 114 ml/m2 and ejection fraction (EF) ≥ 48%) vs. suboptimal remodeling (EDVi ≥ 120 ml/m2 and EF ≤ 45%). RV geometry was markedly different at baseline in PVR patients compared with controls, with lower systolic surface area-to-volume ratio (SAVR) (1.16 ± 0.26 vs.1.44 ± 0.21 cm2/mL, p < 0.001) and lower systolic circumferential curvature (0.87 ± 0.27 vs. 1.07 ± 0.30 cm- 1, p = 0.007) but similar longitudinal curvature. In the PVR cohort, higher systolic SAVR was associated with higher RVEF both pre- and post-PVR (p < 0.001). Among PVR patients, 15 had optimal and 19 had suboptimal remodeling post-PVR. Multivariable modeling showed that among the geometric parameters, higher systolic SAVR (OR 1.68 per 0.1 cm2/mL increase; p = 0.049) and shorter systolic RV long-axis length (OR 0.92 per 0.1 cm increase; p = 0.035) were independently associated with optimal remodeling. Compared with controls, PVR patients have lower SAVR and lower circumferential but not longitudinal curvature. Higher pre-PVR systolic SAVR is associated with optimal remodeling post-PVR.

Hand-sewn expanded polytetrafluoroethylene valved conduit for right ventricular outflow tract reconstruction. / 手工缝制的膨体聚四氟乙烯带瓣管道在右室流出道重建中的应用.

OBJECTIVES: Due to the lack of large-sized pulmonary valved conduit products in clinical practice, hand-sewn expanded polytetrafluoroethylene (ePTFE) valved conduit has been used for right ventricular outflow tract (RVOT) reconstruction in many heart centers around the world. This study aims to summarize the early results of the ePTFE valved conduit and the sewing technology of the conduit in combination with the latest progress, and to provide a reference for the application of ePTFE valved conduit. METHODS: A total of 21 patients using ePTFE valved conduit for RVOT reconstruction in the Second Xiangya Hospital, Central South University from October 2018 to October 2020 were prospectively enrolled in this study. The age at the implantation of the conduit was 4.3 to 43.8 (median 15.1) years old, with weight of (38.9±4.1) kg. In this cohort, 14 patients underwent re-reconstruction of RVOT, including 12 patients with pulmonary regurgitation at 6.3 to 31.0 (median 13.8) years after tetralogy of Fallot (TOF) repair, and 2 patients with failed bovine jugular vein conduit (BJVC). Seven patients underwent Ross operations. Among them, 3 were for aortic valve stenosis, 2 were for aortic regurgitation, and 2 were for both stenosis and regurgitation. The ePTFE valved conduits were standard hand-sewn during the surgery. The 3 leaflets were equal in size with arc-shaped lower edge of the valve sinus. The free edge of the valve leaflets was straight with the length of about 1 mm longer than the diameter. The height of the valve sinus was 4/5 of the diameter. The junction of the valve leaflet was 3/4 of the height of the sinus. The designed leaflets were then continuous non-penetrating sutured into the inner surface of Gore-Tex vessel to make a valved conduit. Valved conduits with diameter of 18, 20, and 22 mm were used in 2, 9, and 10 cases, respectively. The surgical results, postoperative recovery time, and serious complications were summarized, and the changes of postoperative cardiac function status and hemodynamic status of the conduits were investigated. RESULTS: During the implantation of ePTFE valved conduit for RVOT reconstruction, 2 patients underwent mechanical mitral valve replacement with Ross operation, 2 patients with pulmonary regurgitation with repaired TOF underwent left and right pulmonary artery angioplasty, and 1 patient with failed BJVC underwent tricuspid valvuloplasty. The cardiopulmonary bypassing time for patients underwent re-reconstruction of RVOT was (130.9±16.9) min, with aorta clamping for 1 patient to repair the residual defect of the ventricular septum. The cardiopulmonary bypassing and aorta clamping time for Ross operation were (242.7±20.6) min and (145.6±10.5) min, respectively. The duration of postoperative ventilator assistance, intensive care unit stay, and hospital stay were 3.5 h to 7.7 d (median 17.1 h),11.2 h to 29.5 d (median 1.9 d), and 6.0 to 56.0 (median 13.0) d, respectively. All patients survived after discharge from hospital. The follow-up rate after discharge was 100% with median time at 15.0 (13.0 to 39.0) months. No death happened during the follow-up. One patient underwent stent implantation due to right coronary stenosis 2 months after Ross operation. One patient underwent balloon dilation due to right pulmonary artery ostium stenosis 1 year after re-reconstruction of RVOT. The cardiac function of all patients recovered to NYHA class I 6 months after operation. The peak pressure gradient across the valve measured by transthoracic echocardiography before discharge was (9.4±2.6) mmHg (1 mmHg=0.133 kPa), and (18.3±6.1) mmHg at the last follow-up. There was no significant increase in the gradient during the follow-up (P=0.134). No patient suffered from mild or more pulmonary regurgitation. CONCLUSIONS: Hand-sewn ePTFE valved conduit is feasible for RVOT reconstruction. It is a promising material for RVOT reconstruction which can effectively meet clinical need. In our experience, the ePTFE valved conduit is simple to manufacture with satisfactory early outcomes.In the application of ePTFE valved conduit, attention should be paid to implantation indications and postoperative anticoagulation management, especially to the preparation details of the valved conduit, to obtain better function and durability of the conduit after implantation.

Usefulness of Right Ventricular Free Wall Strain Obtained with Two-Dimensional Speckle-Tracking Echocardiography in Patients with Repaired Tetralogy of Fallot and Pulmonary Regurgitation.

Right ventricular (RV) dysfunction caused by chronic pulmonary regurgitation (PR) is a major determinant of clinical outcome in adults with repaired tetralogy of Fallot (rTOF). However, the accurate assessment of RV function by conventional echocardiography remains challenging. This study tested the feasibility and usefulness of RV free-wall (RVFW) strain obtained by two-dimensional (2D) speckle-tracking echocardiography (STE) in evaluation of RV function in adults with rTOF by comparing cardiac magnetic resonance (CMR) imaging. We enrolled 22 consecutive patients (male/female, 8/14; mean age, 25.0 years) with rTOF who underwent transthoracic echocardiography at Tohoku University Hospital from July 2016 to June 2019. We measured RVFW strain by STE and compared them with 22 hemodynamically normal subjects (NOR) (male/female, 9/13; mean age, 32.0 years). The correlation between RV strain and CMR-derived RV ejection fraction (RVEF) or PR fraction (PRF) were also evaluated. All rTOF patients had more than moderate PR but were near asymptomatic. RVFW longitudinal strain (RVFW-LS) was significantly decreased in the rTOF group compared with that in the NOR group (-19.6 vs. -24.7, P < 0.01). In the rTOF group, RVFW-LS correlated with PRF (r = 0.44, P < 0.05), whereas RVFW circumferential strain at the mid-ventricular level correlated with RVEF (r = 0.57, P < 0.01). Intra-observer variability of RVFW strain was acceptable. These results indicate that RV systolic function and PR severity in rTOF could be assessed by RVFW strain measured by 2D STE. This method is feasible and can be used as a complement to CMR imaging.

A New Solution for Stenting Large Right Ventricular Outflow Tracts Before Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Prestenting right ventricular outflow tracts (RVOTs) before transcatheter pulmonary valve replacement (TPVR) is essential. Optimus-XXL (AndraTec GmbH, Koblenz, Germany) is a new extra-large, balloon-expandable cobalt-chrome stent with promising technologies. METHODS: From June 2020 to November 2020, 15 patients with congenital heart disease, dysfunctional RVOTs and target TPVR diameter ≥ 23 mm received Optimus-XXL stents before proceeding to TPVR using the SAPIEN valve (Edwards Lifesciences, Irvine, CA). Standard safety and outcomes were prospectively assessed. RESULTS: Patients' median age and weight were 25.8 years (range: 10.5-63.1 years) and 58 kg (range: 43.8-101 kg), respectively. Underlying diagnosis was tetralogy of Fallot (66.7%), and RVOTs were patched (80%). Fifteen bare-metal stents were implanted using femoral (n = 14) and jugular approaches (n = 1). One conduit rupture was immediately controlled with a covered Optimus-XXL. Median stent length was 43 mm (range: 33-57 mm), and median target expansion diameter was 28 mm (range: 23-30 mm). Two procedural incidents occurred during stent delivery and were percutaneously treated. Stent stability was documented during TPVRs immediately performed in 14 patients. Median stent shortening was 13.7%, and median percentage of intended stent expansion was 95.9%. There was no stent fracture on the short-term follow-up (median: 4.5 months). CONCLUSIONS: We report the first implantations of Optimus-XXL stents in dysfunctional RVOTs with excellent preliminary results. Optimus-XXL should be considered as a valuable adjunct in the armamentarium for routine and complex TPVR procedures.

Isolated pulmonary valve endocarditis with pulmonary annular abscess in a patient of Noonan syndrome.

Right-sided endocarditis is a rare entity, with various series reporting an incidence of 5%-10%. Pulmonary valve (PV) is not only the least commonly involved valve in infective endocarditis (IE), with an incidence of 1.5%-2%, but 'isolated' pulmonic valve endocarditis (PVE) without tricuspid valve involvement is even rarer with limited published data. We report a middle-aged man with Noonan syndrome and a dysplastic PV with severe pulmonary stenosis. He presented with a large isolated mobile PV vegetation with moderate pulmonary regurgitation (PR). Initially, he was managed conservatively, but due to persistent fever, pulmonary regurgitation and evidence of pulmonary annular abscess extending into the right ventricular outflow tract, he required surgical intervention. Considering the low incidence of isolated PVE, it poses a challenge for physicians in prompt diagnosis and timely management of the infection.

Impaired right and left ventricular function and relaxation induced by pulmonary regurgitation are not reversed by tardive antifibrosis treatment.

Pulmonary regurgitation (PR) after repair of tetralogy of Fallot (rTOF) is associated with progressive right (RV) and left (LV) ventricular dysfunction and fibrosis. However, angiotensin II receptor blockade therapy has shown mixed and often disappointing results. The aim of this study was to serially assess changes in biventricular remodeling, dysfunction, and interactions in a rat model of isolated severe PR and to study the effects of angiotensin II receptor blockade. PR was induced in Sprague-Dawley rats by leaflet laceration. Shams (n = 6) were compared with PR (n = 5) and PR + losartan treatment (n = 6). In the treatment group, oral losartan (50 mg·kg-1·day-1) was started 6 wk after PR induction and continued for 6 wk until the terminal experiment. In all groups, serial echocardiography was performed every 2 wk until the terminal experiment where biventricular myocardium was harvested and analyzed for fibrosis. PR and PR + losartan rats experienced early progressive RV dilatation by 2 wk which then stabilized. RV systolic dysfunction occurred from 4 wk after insult and gradually progressed. In PR rats, RV dilatation caused diastolic LV compression and impaired relaxation. PR rats developed increased RV fibrosis compared with shams. Although losartan decreased RV fibrosis, RV dilatation and dysfunction were not improved. This suggests that RV dilatation is an early consequence of PR and affects LV relaxation. RV dysfunction may progress independent of further remodeling. Reduced RV fibrosis was not associated with improved RV function and may not be a viable therapeutic target in rTOF with predominant RV volume loading.NEW & NOTEWORTHY The time-course of RV dilatation and the mechanisms of biventricular dysfunction caused by PR have not been well characterized and the effect of losartan in volume-overloaded RV remains controversial. Our findings suggest that severe PR induces early onset of RV dilatation and dysfunction with little progression after the first 4 wk. The RV dilatation distorts LV geometry with associated impaired LV relaxation. Losartan reduced RV fibrosis but did not reverse RV dilatation and dysfunction.

Predictors of low exercise cardiac output in patients with severe pulmonic regurgitation.

BACKGROUND AND OBJECTIVES: Chronic pulmonic regurgitation (PR) following repair of congenital heart disease (CHD) impairs right ventricular function that impacts peak exercise cardiac index (pCI). We aimed to estimate in a non-invasive way pCI and peak oxygen consumption (pVO2) and to evaluate predictors of low pCI in patients with significant residual pulmonic regurgitation after CHD repair. METHOD: We included 82 patients (median age 19 years (range 10-54 years)) with residual pulmonic regurgitation fraction >40%. All underwent cardiac MRI and cardiopulmonary testing with measurement of pCI by thoracic impedancemetry. Low pCI was defined <7 L/min/m2. RESULTS: Low pCI was found in 18/82 patients. Peak indexed stroke volume (pSVi) tended to compensate chronotropic insufficiency only in patients with normal pCI (r=-0.31, p=0.01). Below 20 years of age, only 5/45 patients had low pCI but near-normal (≥6.5 L/min/m2). pVO2 (mL/kg/min) was correlated with pCI (r=0.58, p=0.0002) only in patients aged >20 years. Left ventricular stroke volume in MRI correlated with pSVi only in the group of patients with low pCI (r=0.54, p=0.02). No MRI measurements predicted low pCI. In multivariable analysis, only age predicted a low pCI (OR=1.082, 95% CI 1.035 to 1.131, p=0.001) with continuous increase of risk with age. CONCLUSIONS: In patients with severe PR, pVO2 is a partial reflection of pCI. Risk of low pCI increases with age. No resting MRI measurement predicts low haemodynamic response to exercise. Probably more suitable to detect ventricular dysfunction, pCI measurement could be an additional parameter to take into account when considering pulmonic valve replacement.

Severe Cardiopulmonary Disease in a Parturient With Noonan Syndrome.

Noonan syndrome is a relatively common genetic disorder and the second most common cause of congenital heart disease after trisomy 21. The spectrum of cardiac anomalies in Noonan syndrome typically involves pulmonary valve stenosis occasionally in conjunction with hypertrophic cardiomyopathy. Mitral valve involvement is a rare finding in Noonan syndrome and is most commonly associated with either mitral valve prolapse or abnormal valvular insertion causing left ventricular outflow tract obstruction. Patients with Noonan syndrome typically have preserved fertility and, given the success of cardiac surgery and medical management of heart failure in this population, are beginning to present more commonly as parturients in adulthood. Maternal physiologic changes during pregnancy introduce an added complexity to hemodynamic management and anesthetic considerations during labor and delivery. In this article, we present a case of a patient with Noonan syndrome with severe mitral stenosis, pulmonary valve insufficiency, and severe restrictive and obstructive pulmonary disease who presented preterm for delivery due to increased dyspnea at rest. Here we review the pathophysiology behind Noonan syndrome and peripartum management strategies in a patient with severe combined cardiac and pulmonary disease.

"The Heart Sound Quintet": A Case Report of Right-Sided Heart Failure Due to Free Pulmonary Regurgitation Long After Intracardiac Repair of Tetralogy of Fallot.

BACKGROUND Intracardiac repair of tetralogy of Fallot (TOF) is generally performed during childhood. However, the majority of patients develop the sequelae long after surgical repair, which results in significant right ventricular (RV) dilation, RV myocardial dysfunction, and, ultimately, in right-sided heart failure. CASE REPORT A 52-year-old man was referred to our institution for the evaluation of sudden-onset ventricular tachycardia. His medical history included RV outflow tract reconstruction at 5 years of age. Auscultation revealed a harsh diastolic regurgitant murmur, widely split first heart sound (S1), and a single second heart sound (S2), indicating a severely dilated RV due to severe pulmonary regurgitation (PR) and the presence of a non-functioning pulmonary valve. Moreover, the right-sided third heart sound (S3) and fourth heart sound (S4) were present, consistent with elevated RV filling pressure and the presence of a non-compliant RV. Eventually, the aforementioned "heart sound quintet" was confirmed using multimodal evaluation as right-sided heart failure with a concomitant severely dilated RV because of complete regression of the pulmonary valve and resultant free PR. CONCLUSIONS We encountered a case with a "heart sound quintet" that was composed of a widely split S1, single S2 with a harsh diastolic regurgitant murmur, and right-sided S3 and S4 The logical interpretation of the findings from physical examination will contribute to understanding the pathophysiology and aid clinical decision-making.

Total pulmonary artery replacement with an Avalus-Gelweave conduit in a patient with giant pulmonary artery aneurysm with pulmonary regurgitation.

BACKGROUND AND AIMS: Pulmonary artery aneurysm is a rare disease. A 59-year-old Chinese female was diagnosed with idiopathic pulmonary aneurysm with pulmonary regurgitation. She had a past medical history of hemoptysis and systemic lupus erythematosus. METHODS: She underwent a successful total pulmonary artery and valve replacement with an Avalus-Gelweave conduit. RESULTS: The postoperative echocardiogram showed a 7 mm Hg peak gradient across the prosthetic valve. The patient's postoperative recovery was uncomplicated. CONCLUSIONS: A bioprosthetic aortic valve can be used in a pulmonary position to achieve a good gradient and avoid long term anticoagulation therapy.

Concomitant operation for pulmonary artery aneurysm and pulmonary valve regurgitation.

A 48-year-old man with a pulmonary artery aneurysm was referred to our hospital. Enhanced computed tomography revealed an aneurysm extending from the main trunk to the bilateral pulmonary branch (maximum diameter 6.4 cm) of the artery. Echocardiography revealed moderate pulmonary valve (PV) regurgitation with right ventricle dilatation. Surgery was indicated because of the pulmonary aneurysm and dyspnea on exertion due to moderate PV regurgitation. Intra-operatively, two cusps were found to be normal in shape, whereas a third left-facing cusp was thick and resembled a small ridge. Therefore, we created one neo-cusp with autologous pericardium using a custom-made template and sutured it along a new, predetermined annulus. We then replaced the pulmonary aneurysm with a T-shaped artificial graft. Postoperative echocardiography showed satisfactory movement of the neo-cusp without pulmonary regurgitation and reduced right ventricular size.

Surgical correction of a pulmonary artery aneurysm with severe pulmonary regurgitation with a valve-sparing technique.

Pulmonary artery aneurysms are rare but are associated with a significant risk of rupture and dissection. Moreover, pulmonary valve regurgitation and/or stenosis often coexist. In this study, we present a case of a pulmonary artery aneurysm with severe pulmonary regurgitation in a patient with pulmonary hypertension treated with aneurysm resection and pulmonary valve repair.

Postoperative and short-term atrial tachyarrhythmia burdens after transcatheter vs surgical pulmonary valve replacement among congenital heart disease patients.

OBJECTIVE: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC-) or surgical (S-) pulmonary valve replacement (PVR). DESIGN/SETTING: This was a retrospective observational study of patients who underwent PVR from 2010 to 2016 at UCLA Medical Center. PATIENTS: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded. OUTCOME MEASURES: The primary outcome was a time-to-event analysis of sustained AT. Sustained ATs were defined as focal AT, intra-atrial reentrant tachycardia/atrial flutter, or AF lasting at least 30 seconds or terminating with cardioversion or antitachycardia pacing. RESULTS: Two hundred ninety-seven patients (TC-PVR, n = 168 and S-PVR, n = 129) were included. During a median follow-up of 1.2 years, nine events occurred in TC-PVR group (5%) vs 23 events in S-PVR group (18%). In the propensity adjusted models, the following factors were associated with significant risk of AT after PVR: history of AT, age at valve implantation, severe right atrial enlargement, and S-PVR. In the secondary analysis, TC-PVR was associated with lower adjusted risk of AT events in the postoperative epoch (first 30 days), adjusted IRR 0.31 (0.14-0.97), P = .03, but similar risk in the short-term epoch, adjusted IRR 0.64 (0.14-2.94), P = .57. CONCLUSION: There was an increased risk of AT in the first 30 days following S-PVR compared to TC-PVR. Additional factors associated with risk of AT events after PVR were a history of AT, age at valve implantation, and severe right atrial enlargement.

Real-time video kinematic evaluation of the in situ beating right ventricle after pulmonary valve replacement in patients with tetralogy of Fallot: a pilot study.

OBJECTIVES: The timing for pulmonary valve replacement (PVR) after tetralogy of Fallot repair is controversial, due to limitations in estimating right ventricular dysfunction and recovery. Intraoperative imaging could add prognostic information, but transoesophageal echocardiography is unsuitable for exploring right heart function. Right ventricular function after PVR was investigated in real time using a novel video-based contactless kinematic evaluation technology (Vi.Ki.E.), which calculates cardiac fatigue and energy consumption. METHODS: Six consecutive patients undergoing PVR at 13.8 ± 2.6 years (range 6.9-19.8) after the repair of tetralogy of Fallot were enrolled. Mean right ventricular end-diastolic and end-systolic volume at magnetic resonance imaging were 115.6 ± 16.2 ml/m2 and 61.5 ± 14.6 ml/m2, respectively. Vi.Ki.E. uses a fast-resolution camera placed 45 cm above the open chest, recording cardiac kinematics before and after PVR. An algorithm defines cardiac parameters, such as energy, fatigue, maximum contraction velocity and tissue displacement. RESULTS: There were no perioperative complications, with patients discharged in satisfactory clinical conditions after 7 ± 2 days (range 5-9). Vi.Ki.E. parameters describing right ventricular dysfunction decreased significantly after surgery: energy consumption by 45% [271 125 ± 9422 (mm/s)2 vs 149 202 ± 11 980 (mm/s)2, P = 0.0001], cardiac fatigue by 12% (292 671 ± 29 369 mm/s2 vs 258 755 ± 42 750 mm/s2, P = 0.01), contraction velocity by 54% (3412 ± 749 mm/s vs 1579 ± 400 mm/s, P = 0.0007) and displacement by 23% (27 ± 4 mm vs 21 ± 4 mm, P = 0.01). Patients undergoing PVR at lower end-diastolic volumes, had greater functional recovery of Vi.Ki.E. parameters. CONCLUSIONS: Intraoperative Vi.Ki.E shows immediate recovery of right ventricular mechanics after PVR with less cardiac fatigue and energy consumption, providing novel insights that may have a prognostic relevance for functional recovery.

The standing of percutaneous pulmonary valve implantation compared to surgery in a non-preselected cohort with dysfunctional right ventricular outflow tract - Reasons for failure and contraindications.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) numbers are rising but are still minor compared to surgery due to several contraindications. We sought to analyze the impact of PPVI compared to standard surgery in an unselected cohort with dysfunctional right ventricular outflow tract (RVOT). Reasons for PPVI failure and possible contraindications were explored. METHODS: Between 2010 and 2015 all consecutive patients who underwent surgery or intervention for a dysfunctional RVOT were investigated. RESULTS: A total of 382 cases was identified - 246 patients underwent successful valve insertion: 166 surgeries (166/246=67.4%) with 55/166 homografts (33.1%), 106 Contegra® grafts (63.8%), 5 Hancock valves (3.0%). Overall, 70/246 patients presented a priori with an enlarged RVOT>28mm (28.5%) not appropriate for PPVI and 14/246 (5.7%) had additional defects necessitating surgery. Some 31/246 patients had surgery for initial repair of congenital defects or were too small (<20kg) for PPVI (12.6%). 80 underwent successful PPVI (32.5% of 246 valves implanted) [51 Edwards Sapien® valves (63.7%), 29 Melody valves (36.3%)]. The RVOT was too large for PPVI in 22/246 patients (8.9%). A total 20/246 patients (8.1%) showed coronary compression after balloon interrogation. In 4/246 patients PPVI was not possible due to technical issues (1.4%). CONCLUSION: PPVI could be performed successfully in 80/382 patients (20.9%). An enlarged RVOT, small patient size, and coronary compression were the major obstacles for interventional management. Future developments for larger RVOTs and smaller body weight may expand the indication for PPVI.

Bidirectional Glenn for residual outflow obstruction in Tetralogy of Fallot.

BACKGROUND: Residual right ventricular outflow obstruction during Tetralogy of Fallot repair necessitates peri-operative revision often requiring trans-annular patch with its negative sequels. Bidirectional Glenn shunt in this setting reduces trans-pulmonary gradient to avoid revision. METHODS: Bidirectional Glenn shunt was added during Tetralogy repair in patients with significant residual obstruction. A total of 53 patients between January, 2011 and June, 2018 were included. Final follow-up was conducted in July, 2018. RESULTS: Mean age at operation was 5.63±3.1 years. Right to left ventricular pressure ratio reduced significantly (0.91±0.09 versus 0.68±0.05; p<0.001) after bidirectional Glenn, avoiding revision in all cases. Glenn pressures at ICU admission decreased significantly by the time of ICU discharge (16.7±3.02 versus 13.5±2.19; p<0.001). Pleural drainage ≥ 7 days was seen in 14 (26.4%) patients. No side effects related to bidirectional Glenn-like facial swelling or veno-venous collaterals were noted. Mortality was 3.7%. Discharge echocardiography showed a mean trans-pulmonary gradient of 32.11±5.62 mmHg that decreased significantly to 25.64±5 (p<0.001) at the time of follow-up. Pulmonary insufficiency was none to mild in 45 (88.2%) and moderate in 6 (11.8%). Mean follow-up was 36.12±25.15 months (range 0.5-90). There was no interim intervention or death. At follow-up, all the patients were in NYHA functional class 1 with no increase in severity of pulmonary insufficiency. CONCLUSION: Supplementary bidirectional Glenn shunt significantly reduced residual right ventricular outflow obstruction during Tetralogy of Fallot repair avoiding revision with satisfactory early and mid-term results.

Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ±â€¯6.4% vs. 48.5 ±â€¯7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 ±â€¯17.7% vs. 75.6 ±â€¯20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ±â€¯3.9 vs.-25.7 ±â€¯4.4, P < .001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.

Late Neo-Aortic Valve Regurgitation Long After Arterial Switch Operation.

BACKGROUND: The purpose of this study is to assess risk factors of neo-aortic regurgitation (AR) after an arterial switch operation and outcomes of neo-aortic valve surgery. METHODS: This is a retrospective study of 469 hospital survivors after arterial switch operation from 1982 to 2016. Preoperative diagnoses included 285 transpositions of the great arteries with intact septum, 140 transpositions with ventricular septal defect, and 44 double-outlet right ventricles. More than mild preoperative pulmonary regurgitation was found in 15 patients. Median age at the operation was 1.0 months (range, 0.1-81.5). Concomitant relief of left ventricular outflow tract obstruction was performed in 20 patients. Seventy-seven patients had more than mild neo-AR at hospital discharge. RESULTS: More than moderate neo-AR was found in 41 patients (8.6%) at a median follow-up of 19.0 years (range, 0.1-35.2). Multivariate analysis identified more than mild preoperative pulmonary regurgitation, concomitant relief of left ventricular outflow tract obstruction, and more than mild neo-AR at hospital discharge as risk factors for late neo-AR. Seventeen patients (3.6%) underwent neo-aortic valve surgeries, including 11 aortic valve replacements, 3 aortic valve plasties, 2 Konno procedures, and 1 aortic root replacement, with median age at the neo-aortic valve surgery of 16.5 years (range, 6.2-27.3). There were 2 late deaths and 5 reoperations to the neo-aortic valve in 4 patients. CONCLUSIONS: Concomitant relief of left ventricular outflow tract obstruction and preoperative more than mild pulmonary regurgitation increase the risk of neo-AR after the arterial switch operation. Reoperation for neo-AR can be done safely.