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Objectives: This study primarily aimed to compare the mechanical ventilation durations between pulmonary valve-sparing and transannular patch repair techniques in the surgical correction of Tetralogy of Fallot. Secondary objectives included comparison of demographic characteristics, cardiopulmonary bypass parameters, postoperative vasoactive inotrope requirements, incidence of cardiac conduction abnormalities, echocardiographic findings, intensive care unit and hospitalisation durations, reoperations rates, morbidity and mortality between the 2 approaches. Methods: This retrospective cohort study was conducted at the Royal Hospital, Muscat, Oman, between January 2016 and December 2019. This study included 102 paediatric patients who underwent complete surgical correction of Tetralogy of Fallot over 3 years, either by a pulmonary valve-sparing technique (Group 1, n = 43) or by transannular patch repair (Group 2, n = 59). Data for both primary and secondary outcomes were extracted from hospital records. Results: Mechanical ventilation duration was significantly shorter in Group 1 (P = 0.039). Patients in Group 1 were generally older, with shorter cardiopulmonary bypass and aortic clamp times, lower inotrope scores, and shorter chest tube retention, intensive care unit and hospitalisation periods. Junctional ectopic tachycardia and severe pulmonary regurgitation were significantly more common in Group 2, while right ventricular outflow tract peak pressure gradients were higher in Group 1. Multivariate analysis identified patient weight as the only independent predictor of mechanical ventilation duration. Conclusion: Pulmonary valve preservation was associated with better early outcomes, including reduced mechanical ventilation duration, lower vasoactive inotrope scores, decreased postoperative arrhythmias and shorter hospital stay. [ABSTRACT FROM AUTHOR]

OBJECTIVES The impact of residual pulmonary stenosis (rPS) or right ventricular (RV) outflow tract obstruction on prognosis after surgical pulmonary valve insertion) in repaired tetralogy of Fallot patients with pulmonary regurgitation (PR) remains controversial. rPS assessment is partially dependent on RV contractility. We investigated the impact of rPS according to RV ejection fraction (RVEF). METHODS In this multicentre, retrospective study, 117 repaired tetralogy of Fallot patients who underwent surgical pulmonary valve insertion for more than moderate PR between 2003 and 2021 were examined. Regarding rPS, the threshold for PR with rPS (PSR) and PR was 25 mmHg. For RVEF, the threshold for preserved RVEF (pEF) and reduced RVEF (rEF) was 40%. The patients were divided into 4 groups: patients with PR and pEF (PR-pEF) (n  = 48), those with PR and rEF (PR-rEF) (n  = 44), those with PSR and pEF (PSR-pEF) (n  = 16), and those with PSR and rEF (PSR-rEF) (n  = 9). Clinical parameters, postoperative adverse event rates and their associations were studied. RESULTS The 5-year freedom from adverse cardiovascular events was the highest in the PSR-pEF and the lowest in the PSR-rEF groups. The PSR-rEF group had the highest RV end-diastolic pressure (RVEDP) (12 ± 2.2 mmHg) (P = 0.006). From multivariable analysis, RVEDP was associated with postoperative adverse events (P = 0.016). RVEDP > 8 mmHg was associated with a lower freedom from adverse events. CONCLUSIONS The freedom from adverse events was the lowest in the PSR-rEF group, with the highest RVEDP, suggesting RV systolic and diastolic dysfunction. Reduced RVEF may mask the intrinsic degree of residual stenosis, delay surgical pulmonary valve insertion timing and increase adverse events. [ABSTRACT FROM AUTHOR]

A common feature of congenital heart disease is the presence of right ventricular outflow tract (RVOT) obstruction that can range from mild to severe and can lead to atresia of the pulmonary valve, in extreme conditions. RVOT abnormalities can frequently be corrected surgically or via interventional means. However, most of these patients will ultimately develop pulmonary valve insufficiency and eventual right ventricular dilation, which will require a pulmonary valve replacement at some point in their life to mitigate the detrimental effects of pulmonary valve regurgitation (PVR) on the right ventricle (RV). The evolution from the studies done by Philip Bonhoeffer to implant a pulmonary valve via transcatheter means, have provided a bedrock for transcatheter pulmonary valve replacement (TPVR). Yet, several areas of unmet need for a demographic of patients still exist. Here, we discuss the clinical unmet needs in children under 20 Kg and expand the use of hybrid and other TPVR approaches along with the current indications and contraindications for pulmonary valve replacement. The constraints and limitations from commercially available pulmonary valves will be discussed from a clinical standpoint. Finally, we explore the use of hybrid and periventricular delivery of transcatheter pulmonary valves in younger patients.

Current indications for pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) rely on cardiovascular magnetic resonance (CMR) image-based indices but are inconsistently applied, lead to mixed outcomes, and remain debated. This study aimed to test the hypothesis that specific markers of biventricular shape may discriminate differences between rTOF patients who did and did not require subsequent PVR better than standard imaging indices. In this cross-sectional retrospective study, biventricular shape models were customized to CMR images from 84 rTOF patients. A statistical atlas of end-diastolic shape was constructed using principal component analysis. Multivariate regression was used to quantify shape mode and imaging index associations with subsequent intervention status (PVR, n = 48 vs. No-PVR, n = 36), while accounting for confounders. Clustering analysis was used to test the ability of the most significant shape modes and imaging indices to discriminate PVR status as evaluated by a Matthews correlation coefficient (MCC). Geometric strain analysis was also conducted to assess shape mode associations with systolic function. PVR status correlated significantly with shape modes associated with right ventricular (RV) apical dilation and left ventricular (LV) dilation (p

BACKGROUND: Maladaptive remodelling mechanisms occur in patients with repaired tetralogy of Fallot (rToF) resulting in a cycle of metabolic and structural changes. Biventricular shape analysis may indicate mechanisms associated with adverse events independent of pulmonary regurgitant volume index (PRVI). We aimed to determine novel remodelling patterns associated with adverse events in patients with rToF using shape and function analysis. METHODS: Biventricular shape and function were studied in 192 patients with rToF (median time from TOF repair to baseline evaluation 13.5 years). Linear discriminant analysis (LDA) and principal component analysis (PCA) were used to identify shape differences between patients with and without adverse events. Adverse events included death, arrhythmias, and cardiac arrest with median follow-up of 10 years. RESULTS: LDA and PCA showed that shape characteristics pertaining to adverse events included a more circular left ventricle (LV) (decreased eccentricity), dilated (increased sphericity) LV base, increased right ventricular (RV) apical sphericity, and decreased RV basal sphericity. Multivariate LDA showed that the optimal discriminative model included only RV apical ejection fraction and one PCA mode associated with a more circular and dilated LV base (AUC = 0.77). PRVI did not add value, and shape changes associated with increased PRVI were not predictive of adverse outcomes. CONCLUSION: Pathological remodelling patterns in patients with rToF are significantly associated with adverse events, independent of PRVI. Mechanisms related to incident events include LV basal dilation with a reduced RV apical ejection fraction.

Guidelines for management of Melody transcatheter pulmonary valve (TPV) infective endocarditis (IE) are lacking. We aimed to identify factors associated with surgical valve removal versus antimicrobial therapy in Melody TPV IE. Multicenter retrospective analysis of all patients receiving Melody TPV from 10/2010 to 3/2019 was performed to identify cases of IE. Surgical explants versus non-surgical cases were compared. Of the 663 Melody TPV implants, there were 66 cases of IE in 59 patients (59/663, 8.8%). 39/66 (59%) were treated with IV antimicrobials and 27/66(41%) underwent valve explantation. 26/59 patients (44%) were treated medically without explantation or recurrence with average follow-up time of 3.5 years (range:1-9). 32% of Streptococcus cases, 53% of MSSA, and all MRSA cases were explanted. 2 of the 4 deaths had MSSA. CART analysis demonstrated two important parameters associated with explantation: a peak echo gradient ≥ 47 mmHg at IE diagnosis(OR 10.6, p  24 mmHg compared to baseline (OR 6.7, p = 0.01). Rates of explantation varied by institution (27 to 64%). In our multicenter experience, 44% of patients with Melody IE were successfully medically treated without valve explantation or recurrence. The degree of valve stenosis at time of IE diagnosis was strongly associated with explantation. Rates of explantation varied significantly among the institutions.

This case series presents two young patients with severe pulmonary valve regurgitation and pectus excavatum. Both patients underwent surgical repair of tetralogy of Fallot and pulmonary valvulotomy, respectively, during infancy and remained under close cardiological monitoring thereafter. After the diagnosis of severe pulmonary regurgitation was confirmed, both were referred to our center for pulmonary valve replacement. Minimally invasive pulmonary valve replacement was performed through a left anterior minithoracotomy. The swift recovery and return to daily activities observed in the presented cases suggest that minimally invasive pulmonary valve replacement through a left anterior minithoracotomy could indeed be considered the procedure of choice for patients with pectus excavatum. [ABSTRACT FROM AUTHOR]

BACKGROUND: Relationships between right ventricular (RV) and left ventricular (LV) shape and function may be useful in determining optimal timing for pulmonary valve replacement in patients with repaired tetralogy of Fallot (rTOF). However, these are multivariate and difficult to quantify. We aimed to quantify variations in biventricular shape associated with pulmonary regurgitant volume (PRV) in rTOF using a biventricular atlas. METHODS: In this cross-sectional retrospective study, a biventricular shape model was customized to cardiovascular magnetic resonance (CMR) images from 88 rTOF patients (median age 16, inter-quartile range 11.8-24.3 years). Morphometric scores quantifying biventricular shape at end-diastole and end-systole were computed using principal component analysis. Multivariate linear regression was used to quantify biventricular shape associations with PRV, corrected for age, sex, height, and weight. Regional associations were confirmed by univariate correlations with distances and angles computed from the models, as well as global systolic strains computed from changes in arc length from end-diastole to end-systole. RESULTS: PRV was significantly associated with 5 biventricular morphometric scores, independent of covariates, and accounted for 12.3% of total shape variation (p

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(1) Background: Cardiac electrotherapy is developing quickly, which implies that it will face a higher number of complications, with cardiac device-related infective endocarditis (CDRIE) being the most frequent, but not the only one. (2) Methods: This is a retrospective case study followed by a literature review, which presents a patient with a rare but dangerous complication of electrotherapy, which could have been prevented if modern technology had been used. (3) Results: A 34-year-old female was admitted with suspicion of CDRIE based on an unclear echocardiographic presentation. However, with no signs of infection, that diagnosis was not confirmed, though an endocardial implantable cardioverter-defibrillator (ICD) lead was found folded into the pulmonary trunk. The final treatment included transvenous lead extraction (TLE) and subcutaneous ICD (S-ICD) implantation. (4) Conclusions: With the increasing number of implantations of cardiac electronic devices and their consequences, a high index of suspicion among clinicians is required. The entity of the clinical picture must be thoroughly considered, and various diagnostic tools should be applied. Lead dislocation into the pulmonary trunk is an extremely rare complication. Our findings align with the available literature data, where asymptomatic cases are usually effectively treated with TLE. Modern technologies, such as S-ICD, can effectively prevent lead-related problems and are indicated in young patients necessitating long-term ICD therapy. [ABSTRACT FROM AUTHOR]

BACKGROUND: Varying rates of nonsustained ventricular tachycardia (NSVT) have been reported early after transcatheter pulmonary valve replacement (TPVR) with the Harmony valve, but data regarding rhythm outcomes beyond hospital discharge are limited. This study aims to characterize ventricular arrhythmias after Harmony TPVR from implant through mid-term follow-up. METHODS: Ventricular arrhythmia data from postimplant telemetry and follow-up extended rhythm monitoring (ERM) were analyzed after Harmony TPVR. RESULTS: Fifty-four patients with tetralogy of Fallot (n=39), valvar pulmonary stenosis (n=10), or pulmonary atresia with intact ventricular septum (n=5) were studied; 22% had prior NSVT and 24% were on prior rhythm medication. On postimplant telemetry, 27 patients (50%) had NSVT, including 1 who had torsade de pointes, but most had <5 episodes. Pre-TPVR NSVT or rhythm medications, diagnosis other than tetralogy, and substantial device contact with the myocardium were associated with more frequent NSVT on telemetry. Ten patients (19%) were started on a new antiarrhythmic medication. On discharge ERM, 37% of patients had NSVT, most with <5 episodes and only 13% with NSVT beyond 5 days post-discharge. On follow-up ERM, 14% of patients had a single episode of NSVT and 1 had 5 episodes. During follow-up, antiarrhythmic medications were discontinued in 8 of 10 patients and no patients died or had sustained ventricular tachycardia. CONCLUSIONS: NSVT and ventricular ectopy were common early after TPVR but were infrequent in most cases and diminished rapidly after discharge. The incidence of NSVT on follow-up ERM was similar to preimplant incidence. Few patients had antiarrhythmic medications initiated, and most were discontinued on follow-up. There were no major arrhythmic events after discharge. [ABSTRACT FROM AUTHOR]

Background: Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR).Methods: We performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed.Results: After a median follow-up of 38.6 (30.9-49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m2 for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m2 for RV end-systolic volume indexed(sensitivity 80%, specificity 56%).Conclusions: Previous RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate. [ABSTRACT FROM AUTHOR]

Abstract Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Background Tricuspid regurgitation (TR) is a common finding in adults with congenital heart disease referred for pulmonary valve replacement (PVR). However, indications for combined valve surgery remain controversial. This study aimed to evaluate early results of concomitant tricuspid valve intervention (TVI) at the time of PVR. Methods and Results Observational studies comparing TVI+PVR and isolated PVR were identified by a systematic search of published research. Random‐effects meta‐analysis was performed, comparing outcomes between the 2 groups. Six studies involving 749 patients (TVI+PVR, 278 patients; PVR, 471 patients) met the eligibility criteria. In the pooled analysis, both TVI+PVR and PVR reduced TR grade, pulmonary regurgitation grade, right ventricular end‐diastolic volume, and right ventricular end‐systolic volumes. TVI+PVR, but not PVR, was associated with a decrease in tricuspid valve annulus size (mean difference, −6.43 mm, 95% CI, −10.59 to −2.27; P=0.010). Furthermore, TVI+PVR was associated with a larger reduction in TR grade compared with PVR (mean difference, −0.40; 95% CI, −0.75 to −0.05; P=0.031). No evidence could be established for an effect of either treatment on right ventricular ejection fraction or echocardiographic assessment of right ventricular dilatation and dysfunction. There was no evidence for a difference in hospital mortality or reoperation for TR. Conclusions While both strategies are effective in reducing TR and right ventricular volumes, routine TVI+PVR can reduce TR grade to a larger extent than isolated PVR. Further studies are needed to identify the subgroups of patients who might benefit most from combined valve surgery.

Transcatheter Pulmonary Valve Implantation has become the treatment of choice in most congenital heart disease patients with degeneration and dysfunction of a previous Right ventricular outflow tract repair. It is less invasive, with a high procedural success rate. Efficacy and safety have been demonstrated for the Medtronic Melody® valve (Medtronic Inc., MN, USA) and Edwards SAPIEN™ valve (Edwards Lifesciences) in pulmonary position. We report the first successful percutaneous pulmonary valve replacement with an Indian made valve Meril Myval™ in a symptomatic patient who had severe Right ventricle-pulmonary artery conduit dysfunction post Tetralogy of Fallot intracardiac repair.

BackgroundTetralogy of Fallot (TOF) is one of the most common types of congenital heart disease and requires prompt surgical correction. Post-correction pulmonary insufficiency (PI) often ensues in adulthood. At times, the PI is accompanied by residual pulmonary stenosis (PS). Little is known regarding right ventricular (RV) function in the setting of combined PS and PI.PurposeTo compare cardiac magnetic resonance (CMR) parameters for the assessment of RV function between patients with combined pulmonary stenosis and pulmonary insufficiency (PSPI) and isolated PI following surgical repair of TOF.Material and methodsRetrospective review of patients with comparable corrected TOF and similar PI was performed. Seventeen patients (median age, 24 years; range, 10-52 years) had combined PSPI and 30 patients (median age, 30 years; range, 6-70 years) had isolated PI. Cine magnetic resonance (MR) images (Philips Medical Systems, Best, The Netherlands) in the short-axis plane were used to calculate end-systolic, end-diastolic, and stroke volumes (RVESV, RVEDV, RVSV) and to measure RV wall thickness. Velocity-encoded cine MR images were used to measure pulmonary regurgitation fraction (PRF) by calculating the ratio of backward flow and total forward flow, obtained from the main pulmonary flow analysis. Peak pressure gradient across the pulmonary valve was obtained from spectral Doppler echocardiography.ResultsRVEF was 51 ± 8% in the PSPI patients and 39 ± 11%, in the patients with isolated PI (P = 0.001). Additionally, RV wall thickness was 5.2 ± 0.8 mm in the PSPI patients compared to 2.6 ± 0.9 mm in the isolated PI patients (P = 0.001). RVESVi and RVEDVi were significantly lower (P < 0.05) in patients with combined PSPI (60 ± 21 mL/m(2), 121 ± 35 mL/m(2), respectively) compared to the patients with isolated PI (95 ± 48 mL/m(2), 152 ± 61 mL/m(2), respectively).ConclusionRV function is preserved in patients with PSPI when compared to patients with PI following surgical repair of TOF.

ObjectivesWe assessed the hypothesis that there is an improvement in clinical and physiologic parameters of cardiopulmonary exercise testing (CPET) after implantation of a transcatheter pulmonary valve (TPV).BackgroundTranscatheter pulmonary valve provides a new tool for treating conduit stenosis and regurgitation in patients with right ventricle (RV) to pulmonary artery conduit dysfunction.MethodsPatients who underwent a TPV placement between January 2007 and January 2010 (N = 150) were investigated with a standardized CPET protocol before and at 6 months after TPV placement. Cardiopulmonary exercise testing was performed on a mechanically braked cycle ergometer with respiratory gas exchange analysis.ResultsSix months post TPV, small but statistically significant improvements were observed in the maximum workload (65.0% ± 18.8% to 68.3% ± 20.3% predicted, P < .001) and the ratio of minute ventilation to CO(2) production at the anaerobic threshold (30.8 ± 4.7 to 29.1 ± 4.1, P < .001). There was no significant change in peak oxygen consumption (VO(2)). Patients with pre-TPV hemodynamics consistent with RV dysfunction and patients with a lower pre-TPV peak VO(2) tended to have the greatest improvement in peak VO(2). The correlation between TPV-related improvements in peak VO(2) and baseline clinical variables were weak, however, and these variables could not be used to reliably identify patients likely to have improved peak VO(2) after TPV.ConclusionIn patients with RV to pulmonary artery conduit dysfunction, TPV is associated with modest improvement in exercise capacity and gas exchange efficiency during exercise.

Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups – polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up. [ABSTRACT FROM AUTHOR]

Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients. [ABSTRACT FROM AUTHOR]