Search

A dilated native right ventricular outflow tract (RVOT) presents unique challenges for transcatheter management using balloon-expandable valves. The Alterra Adaptive Prestent was designed to expand transcatheter therapy to treat patients with dilated RVOTs. The aim of this study was to report 2-year outcomes of the main cohort of the ALTERRA (Multicenter Study of Congenital Pulmonic Valve Dysfunction Studying the SAPIEN 3 THV With the Alterra Adaptive Prestent) pivotal trial using the prestent with transcatheter pulmonary valve replacement. The prestent device used with the 29 mm SAPIEN 3 transcatheter heart valve (THV) was evaluated for the management of patients with moderate or greater pulmonary valve regurgitation (PR). The primary endpoint was THV dysfunction at 6 months, defined as a nonhierarchical composite of RVOT/pulmonary valve reintervention, moderate or greater total PR on transthoracic echocardiography, and mean RVOT/pulmonary valve gradient 35 mm Hg or greater on transthoracic echocardiography. The primary endpoint and outcomes through 2 years are presented in this analysis. Of 97 patients screened, 60 underwent prestent and THV implantation. There was 1 staged procedure. No patients had THV dysfunction at 6 months. At 2 years, the majority of patients (92.5%) had mild or less PR, with no reports of coronary compression, stent fractures warranting reintervention, or endocarditis. Of the 21 patients (34.4%) who experienced early (days 0-1) arrhythmias, 12 had episodes of nonsustained ventricular tachycardia that resolved with medication. One patient underwent reintervention secondary to an iatrogenic RVOT obstruction; there were no deaths or explantations through 2 years. The Alterra prestent in combination with the SAPIEN 3 THV has excellent outcomes at 2 years, with no significant valve dysfunction in the main pivotal cohort. [Display omitted] [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. centers. The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes. [Display omitted] [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) has expanded and evolved since its initial commercial approval in the United States in 2010. This study sought to characterize real-world practice, including patient selection, procedural outcomes, complications, and off-label usage. Characteristics and outcomes for patients undergoing balloon-expandable TPVR were collected from the American College of Cardiology National Cardiovascular Data Registry IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry. Between April 2016 and March 2021, 4,513 TPVR procedures were performed in patients with a median age of 19 years, 57% with a Melody (Medtronic Inc) and 43% with a SAPIEN (Edwards Lifesciences) valve. Most implanting centers performed <10 cases annually. One-third of transcatheter pulmonary valve implants were into homograft conduits, one-third were into bioprosthetic valves (BPVs), 25% were in native or patched right ventricular outflow tracts (RVOTs), and 6% were into Contegra (Medtronic Inc) conduits. Over the course of the study period, SAPIEN valve use grew from ∼25% to 60%, in large part because of implants in patients with a native/patched RVOT. Acute success was achieved in 95% of patients (95.7% in homografts, 96.2% in BPVs, 94.2% in native RVOTs, and 95.4% in Contegra conduits). Major adverse events occurred in 2.4% of procedures, more commonly in patients with a homograft (2.9%) or native RVOT (3.4%) than a prior BPV (1.4%; P = 0.004). This study describes novel population data on the use and procedural outcomes of TPVR with balloon-expandable valves. Over time, there has been increasing use of TPVR to treat regurgitant native RVOT anatomy, with the SAPIEN valve more commonly used for this application. [Display omitted] [ABSTRACT FROM AUTHOR]

The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration–approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT). One-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients. Eligible patients had severe PR by echocardiography or PR fraction ≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n = 42) or TPV25 (n = 45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately. In the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 group and 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient >40 mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately. The Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability. [Display omitted] [ABSTRACT FROM AUTHOR]

Many 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. In 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. Pulmonary 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. [ABSTRACT FROM AUTHOR]

Double-chamber right ventricle repair surgery requires the excision of anomalous obstructive muscular or fibromuscular bundles in the right ventricular outflow tract. Because of the close proximity of key structures in the right ventricular outflow tract, the surgery is extremely challenging and requires precise resection. Underresection of the muscle bands can lead to significant residual gradients in the postoperative period, whereas overenthusiastic resection can cause iatrogenic injury to surrounding structures. Various techniques like Hegar sizing by the surgeons, direct chamber pressure measurement, transesophageal echocardiography, and epicardial echocardiography can guide the surgeons about the adequacy of repair. Transesophageal echocardiography is crucial at each step, as it can precisely determine the exact site of obstruction in the preoperative period. Postoperatively, it helps determine the adequacy of surgical repair and identification of inadvertent iatrogenic complications. [ABSTRACT FROM AUTHOR]

[Display omitted] [ABSTRACT FROM AUTHOR]

There is limited information about durability of large diameter porcine bioprostheses implanted for pulmonary valve replacement (PVR). We studied patients who underwent surgical PVR from 2002-2019 with a stented porcine bioprosthetic valve (BPV) with a labeled size ≥27 mm. The primary outcome was freedom from reintervention. During the study period, 203 patients underwent PVR using a porcine BPV ≥27 mm, 94% of whom received a Mosaic valve (Medtronic Inc., Minneapolis, MN). Twenty patients underwent reintervention from 3.4-12.0 years after PVR: 5 surgical and 15 transcatheter PVR procedures. The indication for reintervention was regurgitation in 13 patients, stenosis in 2, mixed disease in 4, and endocarditis in 1. Estimated freedom from reintervention was 97±1% at 5 years and 82±4% at 10 years, and freedom from prosthesis dysfunction (moderate or severe regurgitation and/or a maximum Doppler gradient ≥50 mm Hg) over time was 91±2% at 5 years and 74±4% at 10 years. Younger age and smaller true valve diameter were associated with shorter freedom from reintervention, but valve oversizing was not. The durability of large stented porcine bioprostheses in the pulmonary position is generally excellent, particularly in adolescents and adults, similar to various other types of BPV. In the current study, relative valve size was not associated with valve longevity, although the low event-rate in this population was a limiting factor. [ABSTRACT FROM AUTHOR]

Ventricular interdependence may account for altered ventricular mechanics in congenital heart disease. The present study aimed to identify differences in load-dependent right ventricular (RV)–left ventricular (LV) interactions in porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) by invasive admittance-derived hemodynamics in conjunction with noninvasive cardiovascular magnetic resonance (CMR). Seventeen pigs were used in the study (7 with PS, 7 with PI, and 3 controls). Progressive PS was created by tightening a Teflon tape around the pulmonary artery, and PI was created by excising 2 leaflets of the pulmonary valve. Admittance catheterization data were obtained for the RV and LV at 10 to 12 weeks after model creation, with the animal ventilated under temporary diaphragm paralysis. CMR was performed in all animals immediately prior to pressure–volume catheterization. In the PS group, RV contractility was increased, manifested by increased end-systolic elastance (mean difference, 1.29 mm Hg/mL; 95% confidence interval [CI], 0.57-2.00 mm Hg/mL). However, in the PI group, no significant changes were observed in RV systolic function despite significant changes in RV diastolic function. In the PS group, LV end-systolic volume was significantly lower compared with controls (mean difference, 25.1 mL; 95% CI, -40.5 to -90.7 mL), whereas in the PI group, the LV showed diastolic dysfunction, demonstrated by an elevated isovolumic relaxation constant and ventricular stiffness (mean difference, 0.03 mL−1; 95% CI, -0.02 to 0.09 mL−1). The LV exhibits systolic dysfunction and noncompliance with PI. PS is associated with preserved LV systolic function and evidence of some LV diastolic dysfunction. Interventricular interactions influence LV filling and likely account for differential effects of RV pressure and volume overload on LV function. Porcine models of pressure and volume overload. We developed and acquired pressure–volume loops from porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) from both the right and left ventricles. In addition to expected changes in contractility in compliance for each respective model, we observed mild diastolic dysfunction in both models, suggesting that interventricular interactions are important in understanding pathophysiology. [Display omitted] [ABSTRACT FROM AUTHOR]

Carcinoid disease is caused by neuroendocrine tumors, most often located in the gut, and leads in approximately 20% of cases to specific, severe heart disease, most prominently affecting right-sided valves. If cardiac disease occurs, it determines the patient's prognosis more than local growth of the tumor. Surgical treatment of carcinoid-induced valve disease has been found to improve survival in observational studies. Cardiac imaging is crucial for both diagnosis and management of carcinoid heart disease; in the past, imaging was accomplished largely by echocardiography, but more recently, imaging for carcinoid heart disease has increasingly become multimodal and warrants awareness of the particular diagnostic challenges of this disease. This paper reviews the pathophysiology and manifestations of carcinoid heart disease in light of the different imaging modalities. [Display omitted] • Carcinoid heart disease is a complication of metastatic neuroendocrine tumors of the gut affecting mainly the tricuspid and pulmonary valves and rarely also the mitral and aortic valves. • Adequate cardiac imaging is crucial for diagnosis and for the decision to intervene surgically, which has been shown to improve prognosis in severe carcinoid heart disease. • Carcinoid heart disease displays specific, well-recognized features which should be systematically sought in patients with known tumors or right-sided valvular heart disease. • Pulmonary valve involvement is frequent, tends to be underestimated by echocardiography, and may require cardiac magnetic resonance for complete evaluation. • Cardiac computed tomography and nuclear imaging can contribute to improved evaluation in some cases. [ABSTRACT FROM AUTHOR]

Background/purpose: Prognostic factors remain unclear in patients undergoing transcatheter implantation of Venus P-valve for their severe pulmonary regurgitation associated with native right ventricular (RV) outflow tract.Method: Between January 2017 and October 2018, we prospectively collected data of patient characteristics, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) before and 6-12 months after valve graft implantation.Results: Fifteen patients (male: 8, median age: 24.8 years) were enrolled. The procedure success rate was 100%. The median follow-up was 16.3 months without any dysfunction of the valve graft. The cohort demonstrated a significant improvement in cardiac index (from 3.3 to 3.9 L/min/m2) and increase of percentage of New York Heart Association functional class I (P < 10-3), reduction in RV end-diastolic volume index (P = 0.008), and reductions in NT-proBNP levels (from 78.9 to 45.8 pg/mL, P = 0.040). However, the peak oxygen consumption (VO2) dropped from 50.2% to 48.5% of the predicted value. Interestingly, we determined that patients with NT-proBNP levels below 70 pg/mL and left ventricular end-diastolic pressure (LVEDP) below 11 mmHg had a significantly higher chance of exhibiting improvement in peak VO2 compared with those without (3/4 vs 1/10, P = 0.041).Conclusion: In the small cohort with severe pulmonary regurgitation, implantation of a Venus P-valve led to promising reductions in RV volume. However, no definite improvement in cardiopulmonary exercise capacity or RV ejection fraction was achieved. Levels of NT-proBNP and LVEDP may be helpful for refining the indications of the Venus P-valve implantation. [ABSTRACT FROM AUTHOR]

Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO 2). All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p <.0001, r = 0.72). RV end-diastolic area > 21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m2. RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO 2. Among the Echo data only right atrial strain (RAS) correlated with peak VO 2. In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO 2 and should be added in their follow up. • In children with repaired Tetralogy of Fallot RV area by echocardiogram is a valid first line parameter to screen RV dilation as compared with CMR. • Right ventricle longitudinal systolic dysfunction coexists with a still preserved RV EF. • Flow-reversal in pulmonary branches identifies PR RF ≥ 35% at CMR. • The only parameter correlated to peak Vo2 max in children with r-TOF is right atrial strain [ABSTRACT FROM AUTHOR]

The aim of this study was to demonstrate the safety and functionality of the Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (THV) in patients with dysfunctional, dilated right ventricular outflow tract (RVOT) greater or equal to moderate pulmonary regurgitation (PR). Significant variations in the size and morphology of the RVOT affect the placement of transcatheter pulmonary valves. The Alterra Prestent internally reduces and reconfigures the RVOT, providing a stable landing zone for the 29-mm SAPIEN 3 THV. Eligible patients had moderate or greater PR, weighed >20 kg, and had RVOT diameter 27 to 38 mm and length >35 mm. The primary endpoint was device success, a 5-item composite: 1 Alterra Prestent deployed in the desired location, 1 SAPIEN 3 THV implanted in the desired location within the Prestent, right ventricular–to–pulmonary artery peak-to-peak gradient <35 mm Hg after THV implantation, less than moderate PR at discharge, and no explantation 24 h post-implantation. The secondary composite endpoint was freedom from THV dysfunction (RVOT/pulmonary valve (PV) reintervention, greater or equal to moderate total PR, mean RVOT/PV gradient ≥ 35 mm Hg at 30 days and 6 months. Descriptive statistics are reported. Enrolled patients (N = 15) had a median age and weight of 20 years and 61.7 kg, respectively; 93.3% were in New York Heart Association functional class I or II. Device success was 100%. No staged procedures were necessary. No THV dysfunction was reported to 6 months. No serious safety signals were reported. This early feasibility study demonstrated the safety and functionality of the Alterra Adaptive Prestent in patients with congenital RVOT dysfunction and moderate or greater PR. Durability and long-term outcome data are needed. [ABSTRACT FROM AUTHOR]

An increasing number of patients will require pulmonary valve replacement following repair of tetralogy of Fallot. Most of these patients have a history of surgical repair with a transannular patch (ie, pulmonary valve not preserved). Ten to twenty years after the initial repair, severe pulmonary regurgitation results in right ventricular dilation and dysfunction. There are multiple prosthetic valve options and techniques to use when performing pulmonary valve replacements. Herein, we describe our technique of bioprosthetic pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

Les pathologies de la voie droite sont historiquement les conséquences de la chirurgie correctrice d'une malformation cardiaque congénitale en période néonatale ou pédiatrique (Tétralogie de Fallot, intervention de Ross). Ces chirurgies réalisées dans l'enfance conduisent à l'apparition de dysfonctionnements de la voie droite (sténose, fuite) chez le grand enfant ou l'adulte jeune pour lesquels une nouvelle chirurgie serait à risque opératoire élevé. L'avènement des premières valves percutanées en 2000 a permis d'ouvrir la voie de la revalvulation pulmonaire. La procédure de revalvulation pulmonaire percutanée offre une alternative crédible à la chirurgie dans la population des patients porteurs d'une cardiopathie congénitale souvent multi opérés donc à risque opératoire élevé. Les deux valves actuellement sur le marché français (Melody®, SapienXT®) permettent de couvrir l'ensemble des indications exceptées les voies très larges qui restent éminemment chirurgicales. Les équipes qui pratiquent ces actes sur des sujets jeunes doivent bénéficier d'une expérience suffisante pour limiter le risque procédural. Sur cette base, plusieurs équipes positionnent désormais la revalvulation pulmonaire percutanée en priorité sur la revalvulation chirurgicale qui n'est retenue qu'en cas d'impossibilité du traitement percutané. Right ventricular outflow tract diseases are historically outcomes of surgical reconstruction for heart defects in neonates or children (Tetralogy of Fallot, Ross surgery). This kind of surgery performed during childhood lead often to dysfunctional right ventricular outflow tract (stenosis, leak) in older infants or young adults. In this case, reintervention on the right ventricular outflow tract would be associated with a high surgical risk. Development of the first percutaneous valves in the year 2000 paved the way for the pulmonary revalvulation. This percutaneous procedure has emerged as a credible alternative to the surgery for multioperated high risk patients with congenital cardiopathies. Two valves are currently available on the French market (Melody®, Sapien®); they cover all therapy indications, except the example of very wide outflow tracts which remain a surgical issue. Medical teams in charge of these young patients have to be enough trained in order to limit risks during the procedure. To this end, several teams promote the percutaneous pulmonary revalvulation over surgical revalvulation, this latter becoming limited only to cases in which percutaneous treatment is not feasible. [ABSTRACT FROM AUTHOR]

Only few studies have reported long-term outcome of the transatrial-transpulmonary approach in the current era of management of tetralogy of Fallot (ToF). We investigated 15-year outcome of correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated in the 21st century. All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.1 (interquartile range 3.0-10.9) years. Data regarding postoperative complications, reinterventions, development of atrial and ventricular arrhythmia, cardiac function, and survival were evaluated. Prior shunting was performed in 10 patients (6%). The transatrial-transpulmonary approach resulted in valve-sparing surgery in 57 patients (32%). Postoperative surgical complications included junctional ectopic tachycardia (N = 12, 7%), pericardial (N = 10, 6%) or pleural effusion (N = 7, 3%), chylothorax (N = 7, 4%), bleeding requiring reoperation (N = 4, 3%), and superficial wound infection (N = 1). Fifty-one patients underwent 68 reinterventions, mainly due to pulmonary restenosis (PS) (N = 57). ToF correction at age <2 months and double outlet or double-chambered right ventricle variants of the ToF spectrum were independent predictors for reintervention. Patients undergoing valve-sparing ToF correction had a significant longer PR-free survival than those with a transannular patch (8.5 [95% confidence interval 6.8-10.3] years vs 1.1 [95% confidence interval 0.8-1.5] years; P < 0.001). Overall mortality was 2.8%; mortality rates were higher in premature/dysmature newborns (0.7% vs 9.5%; P < 0.001). Although the 15-year outcome of the transatrial-transpulmonary approach in terms of postoperative complications and mortality rates is excellent, the high incidence of moderate and severe PR is worrisome. Valve-sparing surgery was associated with a substantially lower incidence of PR, yet was surgically not possible in the majority of patients. [ABSTRACT FROM AUTHOR]

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Patients with repaired tetralogy of Fallot (TOF) have not uncommonly fragmented QRS complexes (fQRS) on surface electrocardiogram. We suggested that the right heart hemodynamics might be correlated with both the number of leads showing fQRS (fQRS extent) and the QRS duration. A retrospective analysis of the magnetic resonance imaging (MRI) derived data of 30 patients following total correction of TOF was performed. The number of leads showing fragmentation (fQRS extent) as well as the QRS duration were assessed and tested for correlation with the right ventricular volumes and severity of pulmonary regurgitation (PR) as detected by MRI. Twenty-one patients had fQRS in at least 2 consecutive leads. fQRS correlated with larger right ventricular volumes and lower ejection fraction. fQRS in 5 leads was an accurate parameter in identifying an indexed right ventricular end diastolic volume >150 ml/m2 with a sensitivity of 87.5% and a specificity of 85.7%. Moreover, it was the most accurate parameter in detecting a PR fraction > 45%. To conclude, assessment of fQRS extent and duration is a simple, available, and an accurate parameter in identifying patients with significant right ventricular hemodynamic changes in repaired TOF patients. [ABSTRACT FROM AUTHOR]

Congenital heart defects that involve obstruction to the right ventricular outflow tract are common. Surgical repair involves early relief of right ventricular outflow tract obstruction, which typically results in pulmonary regurgitation and large irregularly shaped "native" right ventricular outflow tract. This type of anatomy represents the majority of patients who could potentially benefit from transcatheter pulmonary valve therapy. Currently approved balloon-expandable devices were not designed for this application and the unique anatomy of these patients presents tremendous challenges for designing a valve that is. This article explores those challenges and the newest self-expanding devices designed to treat this challenging population. [ABSTRACT FROM AUTHOR]

Abstract Background Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy. Methods and results A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs −0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S′ lateral [1.0 (2.0) cm/s vs −0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E′ lateral [0.57 (2.4) cm/s vs −3.3 (3.9) cm/s; p < 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E′ lateral [−0.7 (1.9) vs 1.5 (1.9); p < 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs −5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [−0.18 (0.42) vs 0.23 (0.48); p = 0.037]. Conclusion Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified. Highlights • Left ventricular (LV) function is a known predictor of mortality after tetralogy of Fallot repair. • We aimed to characterise LV reverse remodelling achievable with ramipril therapy. • 6-months' ramipril treatment limits progression of both diastolic and systolic LV function. [ABSTRACT FROM AUTHOR]

Abstract Objectives The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position. Background The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI). Methods From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included. Results The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p < 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%). Conclusions Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success. Graphical abstract [ABSTRACT FROM AUTHOR]

• Development of a 3D dynamic computational bi-ventricle model. • Effects of pulmonary regurgitation on cardiac motion and function. • Dilation of right ventricle and high myofibre stress agree with the literature. • Interaction of left ventricle and right ventricle and motion of the septum. Background and objective: Assessing the severity of pulmonary regurgitation (PR) and identifying optimal clinically relevant indicators for its treatment is crucial, yet standards for quantifying PR remain unclear in clinical practice. Computational modelling of the heart is in the process of providing valuable insights and information for cardiovascular physiology research. However, the advancements of finite element computational models have not been widely applied to simulate cardiac outputs in patients with PR. Furthermore, a computational model that incorporates both the left ventricle (LV) and right ventricle (RV) can be valuable in assessing the relationship between left and right ventricular morphometry and septal motion in PR patients. To enhance our understanding of the effect of PR on cardiac functions and mechanical behaviour, we developed a human bi-ventricle model to simulate five cases with varying degrees of PR severity. Methods: This bi-ventricle model was built using a patient-specific geometry and a widely used myofibre architecture. The myocardial material properties were described by a hyperelastic passive constitutive law and a modified time-varying elastance active tension model. To simulate realistic cardiac functions and the dysfunction of the pulmonary valve in PR disease cases, open-loop lumped parameter models representing systemic and pulmonary circulatory systems were designed. Results: In the baseline case, pressures in the aorta and main pulmonary artery and ejection fractions of both the LV and RV were within normal physiological ranges reported in the literature. The end-diastolic volume (EDV) of the RV under varying degrees of PR was comparable to the reported cardiac magnetic resonance imaging data. Moreover, RV dilation and interventricular septum motion from the baseline to the PR cases were clearly observed through the long-axis and short-axis views of the bi-ventricle geometry. The RV EDV in the severe PR case increased by 50.3% compared to the baseline case, while the LV EDV decreased by 18.1%. The motion of the interventricular septum was consistent with the literature. Furthermore, ejection fractions of both the LV and RV decreased as PR became severe, with LV ejection fraction decreasing from 60.5% at baseline to 56.3% in the severe case and RV ejection fraction decreasing from 51.8% to 46.8%. Additionally, the average myofibre stress of the RV wall at end-diastole significantly increased due to PR, from 2.7 ± 12.1 kPa at baseline to 10.9 ± 26.5 kPa in the severe case. The average myofibre stress of the LV wall at end-diastole increased from 3.7 ± 18.1 kPa to 4.3 ± 20.3 kPa. Conclusions: This study established a foundation for the computational modelling of PR. The simulated results showed that severe PR leads to reduced cardiac outputs in both the LV and RV, clearly observable septum motion, and a significant increase in the average myofibre stress in the RV wall. These findings demonstrate the potential of the model for further exploration of PR. [ABSTRACT FROM AUTHOR]

In our new echocardiographic method, pulmonary regurgitant velocity immediately before right atrial (RA) contraction is used to estimate pulmonary artery diastolic pressure (PADP) and mean PA wedge pressure (MPAWP). Our aim here was to compare the usefulness of this new method with that of the conventional method, which uses pulmonary regurgitant velocity at end diastole. We studied 55 consecutive patients who underwent echocardiography and right-sided heart catheterization. The pulmonary regurgitant velocities just before RA contraction and at end diastole were measured to obtain echocardiographic estimates of PADP (EPADPpreA and EPADPED, respectively) by adding the pressure gradients to the echocardiographically estimated RA pressure. Compared with EPADPED, EPADPpreA correlated better with PADP (r = 0.87) and MPAWP (r = 0.80), and direct fixed biases were detected for EPADPED but not for EPADPpreA. The area under the receiver operating characteristic curve distinguishing patients with MPAWP ≥18 mm Hg was greater for EPADPpreA (0.97) than for E/e' (0.94) and E/A (0.83). EPADPpreA is thus useful in estimating PADP and MPAWP in patients with heart disease. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease. After surgical repair, the excellent survival rate has led to long-term complications dominated by pulmonary regurgitation (PR). Our study aimed to identify echocardiographic criteria for assessment of right ventricular (RV) function and quantification of PR correlated with cardiac MRI indices. We conducted a descriptive study between June 2021 and March 2022 including patients followed for repaired ToF since 2016 or earlier. All patients were seen for clinical, electrocardiographic, and ultrasound evaluation. Cardiac MRI was performed with a maximum delay of 1 month from Doppler echocardiography. 38 patients were included, with a mean age at imaging of 18 ± 9 years. Our study showed that the echocardiographic parameters of PR quantification correlated with a pulmonary regurgitation fraction on MRI greater than 40% were: the PHT value (p = 0.005) and the ratio of PR jet width to pulmonary valve annulus diameter (p = 0.032). The only ultrasound parameter estimating RV systolic function correlated with RVEF on cardiac MRI was FAC, with p = 0.007. Quantitative assessment of RV parameters in patients followed for repaired ToF, as well as the assessment of the severity of PR, is feasible with a standardized ultrasound examination, with good correlation with cardiac MRI. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) is an important treatment option in repaired tetralogy of Fallot (TOF) and right ventricular outflow tract (RVOT) dysfunction. Indications for timing of TPVR are extrapolated from surgical pulmonary valve replacement guidelines, which are themselves controversial as published evidence is scarce and expert opinion therefore prevails. We review current indications for PVR following TOF repair, focusing on those for TPVR specifically, and discuss anatomical and functional considerations as these pertain to determination of candidacy for TPVR. Hemodynamic assessment surrounding PVR has focused on assessment of the right ventricle (RV) size and systolic function, with the goal of intervening in the asymptomatic patient prior to the development of irreversible RV deterioration and right heart failure. The impact of abnormal RV mechanics on the LV has been appreciated, with the assessment of LV function assuming higher priority in decision-making regarding possible PVR. In addition to the standard volumetric assessment, evaluation with indices of myocardial wall strain, tissue velocities, diastology, and ventricular response to exercise is emerging as tools with potential to further refine timing of PVR. We conclude that, at present, current evidence, although limited, supports a more aggressive approach in those who meet inclusion for TPVR in patients with repaired TOF and RVOT dysfunction guided by the discussed hemodynamic assessment, however, more importantly this review should lay the framework for future investigations regarding hemodynamic assessment of this population. [ABSTRACT FROM AUTHOR]

Background: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well understood. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: Forty-eight Cardiovascular Magnetic Resonance (CMR) studies in patients after TOF repair were analyzed. In addition to the routine blood flow and ventricular volume quantification cardiac angle between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured on an axial image. Statistical analysis was performed to compare flow parameters between RPA and LPA and to assess correlation among right ventricular volume, pulmonary blood flow parameters and lung area. Results: There was no difference between LPA and RPA diameters. The LPA showed significantly less total forward flow (2.49 ± 0.87 L/min/m² vs 2.86 ± 0.89 L/min/m²; p = 0.02), smaller net forward flow (1.40 ± 0.51 vs 1.89 ± 0. 60 mL/min/m²; p = <0.001), and greater regurgitant fraction (RF) (34 ± 10 % vs 43 ± 12 %; p = 0.001) than the RPA. There was no difference in regurgitant flow volume between RPA and LPA (p = 0.29). Indexed right ventricular end-diastolic volume (RVEDVi) correlated with LPA RF (R = 0.48, p < 0.001), but not with RPA RF (p = 0.09). Larger RVEDVi correlated with a more leftward cardiac axis (R = 0.46, p< 0.001) and with smaller left lung area (R = -0.58, p < 0.001). LPA RF, but not RPA RF, correlated inversely with left lung area (R = -0.34, p = 0.02). The follow-up CMRs in 20 patients showed a correlation of the rate of RV enlargement with the rates of LPA RF worsening (R = 0.50, p = 0.03), and of increasing left lung compression (R = -0.55, p = 0.012). Conclusion: An enlarged and levorotated heart is associated with left lung compression and impaired flow into the left lung. Keywords: Differential pulmonary blood flow, Pulmonary regurgitation, Tetralogy of Fallot, Right ventricular enlargement, Cardiovascular magnetic resonance [ABSTRACT FROM AUTHOR]

Due to recurrent right ventricular outflow tract (RVOT) dysfunction, patients with complex congenital heart disease of the RVOT traditionally require multiple surgical interventions during their lifetimes. Percutaneous pulmonary valve implantation (PPVI) has been developed as a nonsurgical alternative for the treatment of right ventricular to pulmonary artery stenosis or pulmonary regurgitation. PPVI has been shown to be a safe and effective procedure in patients with dysfunctional surgical RVOT conduits. In this population, PPVI has the potential to improve symptoms, functional capacity, and biventricular hemodynamics. However, limitations to the anatomical substrate and size of the RVOT currently restrict PPVI eligibility to less than one-quarter of patients with RVOT dysfunction. The current review discusses contemporary practices in PPVI, evidence supporting the procedure, and future technologies and developments in the field. [ABSTRACT FROM AUTHOR]

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The recovery of cardiopulmonary variables from peak exercise in patients with pulmonary stenosis (PS) or regurgitation (PR) is delayed, but the impact of treating PS or PR on exercise recovery kinetics is unknown. 43 patients (median age 14 years) with PS (n = 23) or PR (n = 20) after repair of congenital heart disease underwent successful percutaneous pulmonary valve implantation (PPVI). Cardiopulmonary exercise tests (CPET) were performed both before and within 1 month after PPVI. Apart from peak oxygen uptake (VO2), the constant decay of VO2, CO2 output (VCO2), minute ventilation (VE), and heart rate (HR) and oxygen pulse were calculated for the first minute of recovery as the first-degree slope of a single linear relation. PPVI led to a significant improvement in NYHA functional class in the PS and PR groups (p < 0.001 and p = 0.0015, respectively). On CPET, peak VO2 improved post-PPVI only in the PS (25.6 ± 6.2 vs. 27.8 ± 7.9 ml/kg/min; p = 0.01) but not PR group (29.0 ± 9.8 vs. 28.6 ± 8.9 ml/kg/min; p = 0.6). However, VO2 slope improved in the PS (0.40 ± 0.23 vs. 0.65 ± 0.27, p < 0.001) as well as in the PR group (0.56 ± 0.37 vs. 0.67 ± 0.37, p = 0.003) as did VCO2 slope (0.39 ± 0.2 vs. 0.55 ± 0.24, p = 0.002 and 0.42 ± 0.33 vs. 0.53 ± 0.35, p = 0.02: for the PS and PR groups, respectively). The VE and HR slopes did not change after PPVI. Despite the lack of improvement in exercise capacity in the PR group, treatment of PS and PR by PPVI induces significant and similar improvements in the ability of recovering from maximal exercise in the 2 groups. [ABSTRACT FROM AUTHOR]

Objectives This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial. Background TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years. The Melody TPV received U.S. Food and Drug Administration approval in 2010 as a Humanitarian Use Device. Methods Patients with dysfunctional RVOT conduits were entered in this prospective, nonrandomized study at 10 centers. The primary endpoint was acceptable hemodynamic function at 6 months post-implantation, defined as a composite of RVOT echocardiographic mean gradient ≤30 mm Hg, pulmonary regurgitation less than moderate as measured by echocardiography, and freedom from conduit reintervention and reoperation. Results Cardiac catheterization was performed in 120 patients for potential implantation of the Melody TPV; of these, 100 patients were implanted, with a 98.0% procedural success rate. There were no procedure-related deaths. Acceptable hemodynamic function at 6 months was achieved in 96.7% of patients with evaluable data (87.9% of the entire implanted cohort), with results maintained through 1 year. No patient had moderate or severe pulmonary regurgitation after implantation. No patient required catheter reintervention in the first year after implantation, and 2 patients required reoperation for conduit replacement. The rate of freedom from TPV dysfunction was 96.9% at 1 year. Conclusions This first prospective, real-world experience with the Melody TPV in the United States demonstrates continued high procedural success, excellent short-term TPV function, and low reintervention and reoperation rates at 1 year. (Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692 ) [ABSTRACT FROM AUTHOR]

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore “hot topics” to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging. [ABSTRACT FROM AUTHOR]

Abstract: Background: Free pulmonary regurgitation (PR) after surgical correction of Tetralogy of Fallot (ToF) with transannular patching can lead to irreversible right ventricular (RV) failure. However, the optimal timing of valve replacement is still debated. Methods and results: Thirty six pigs were included in the study. Twenty one pigs had a bare metal stent placed in the pulmonary annulus inducing free PR and 9 animals served as control. Six animals died prematurely due to procedural complications. The 21 animals were divided into 3 groups with differential duration of PR (1, 2, 3months, respectively) after which PPVR was performed. After 1month with competent valve the animals were euthanized. Cardiac magnetic resonance (CMR) and right heart catheterization were performed serially. Free PR led to severe dilation of the RV in all three groups compared to matched controls (p <0.001). Final RV volume after one month with competent pulmonary valve was modeled. Increase in RV volume from baseline to valve replacement (ΔRV) was the only predictor of RV recovery (p <0.001) and increases in ΔRV beyond 120mL/m2 were predictive of very low probability of recovery. A total of 5 animals did not recover. Conclusions: Recovery of right ventricular function after free PR by treatment with PPVR was successful in the majority of animals. Increases in RV volume during PR were the only predictor of non-recovery after PPVR and duration of PR did not in itself predict treatment success. [Copyright &y& Elsevier]

Abstract: Background: To compare the ventricular response to dobutamine stress between adult patients with chronic pulmonary regurgitation (PR) after repair of tetralogy of Fallot (r-TOF) and healthy volunteers using a staged dobutamine stress MR (DS-MR) protocol. Methods: Eighteen r-TOF patients (median age 31.9years, range 16.2–60.1) with severe PR and 10 healthy controls (median age 40.6years, range 23.9–51.8) completed staged DS-MR (baseline, 10 and 20μg/kg/min) with ventricular volumetry and pulmonary flow quantification. Comparative analysis involved 3-way ANOVA, t-test, regression analysis, and coefficient of variance. Results: All controls had significant increase of ejection fraction (EF) at each stress level for both ventricles (normal contractile reserve, all p<0.05). In r-TOF patients (RV-EDV 126±27ml/m2, RV-EF 55±7%, LV-EF 58±6%, PR-fraction 43±15%), low-dose DS-MR at 10μg/kg/min demonstrated normal biventricular contractile reserve as seen in volunteers. On increase from 10 to 20μg/kg/min a subgroup showed worsening ejection fraction (n=8, p<0.05), mainly due to lack of reduction or even increase of RV-ESV, while the remainder responded with further reduction of RV-ESV and RV-EDV (n=10, p<0.05) and a non-significant trend to increased EF. This different response could not be predicted at baseline. Conclusions: In r-TOF patients with chronic PR, DS-MR at 10μg/kg/min showed normal biventricular systolic response compared with controls. Increase to 20μg/kg/min provoked abnormal RV-ESV response in some r-TOF patients, suggesting presence of ventricular systolic dysfunction not evident at rest. [Copyright &y& Elsevier]

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Tetralogy of Fallot (TOF) repair and pulmonary valvotomy for pulmonary stenosis (PS) lead to progressive pulmonary insufficiency (PI), right ventricular enlargement and dysfunction. This study assessed whether pulmonary regurgitant fraction measured by cardiovascular magnetic resonance (CMR) could be reduced with inhaled nitric oxide (iNO). Methods: Patients with at least moderate PI by echocardiography undergoing clinically indicated CMR were prospectively enrolled. Patients with residual hemodynamic lesions were excluded. Ventricular volume and blood flow sequences were obtained at baseline and during administration of 40 ppm iNO. Results: Sixteen patients (11 with repaired TOF and 5 with repaired PS) completed the protocol with adequate data for analysis. The median age [range] was 35 [19-46] years, BMI was 26 ± 5 kg/m2 (mean ± SD), 50% were women and 75% were in NYHA class I. Right ventricular end diastolic volume index for the cohort was 157 ± 33 mL/m2, end systolic volume index was 93 ± 20 mL/m2 and right ventricular ejection fraction was 40 ± 6%. Baseline pulmonary regurgitant volume was 45 ± 25 mL/beat and regurgitant fraction was 35 ± 16%. During administration of iNO, regurgitant volume was reduced by an average of 6 ± 9% (p=0.01) and regurgitant fraction was reduced by an average of 5 ± 8% (p=0.02). No significant changes were observed in ventricular indices for either the left or right ventricle. Conclusion: iNO was successfully administered during CMR acquisition and appears to reduce regurgitant fraction in patients with at least moderate PI suggesting a potential role for selective pulmonary vasodilator therapy in these patients. [ABSTRACT FROM AUTHOR]

Objectives: The objectives of this study were to evaluate outcomes of pulmonary valve replacement (PVR) in patients with chronic pulmonary regurgitation (PR) and to better define the optimal timing of PVR. Background: Although PVR is effective in reducing right ventricular (RV) volume overload in patients with chronic PR, the optimal timing of PVR is not well defined. Methods: A total of 170 patients who underwent PVR between January 1998 and March 2011 for chronic PR were retrospectively analyzed. To define the optimal timing of PVR, pre-operative and post-operative cardiac magnetic resonance imaging (MRI) data (n = 67) were analyzed. Results: The median age at the time of PVR was 16.7 years. Follow-up completeness was 95%, and the median follow-up duration was 5.9 years. Overall and event-free survival at 10 years was 98% and 70%, respectively. Post-operative MRI showed significant reduction in RV volumes and significant improvement in biventricular function. Receiver-operating characteristic curve analysis revealed a cutoff value of 168 ml/m2 for non-normalization of RV end-diastolic volume index (EDVI) and 80 ml/m2 for RV end-systolic volume index (ESVI). Cutoff values for optimal outcome (normalized RV volumes and function) were 163 ml/m2 for RV EDVI and 80 ml/m2 for RV ESVI. Higher pre-operative RV ESVI was identified as a sole independent risk factor for suboptimal outcome. Conclusions: Midterm outcomes of PVR in patients with chronic PR were acceptable. PVR should be considered before RV EDVI exceeds 163 ml/m2 or RV ESVI exceeds 80 ml/m2, with more attention to RV ESVI. [ABSTRACT FROM AUTHOR]

Abstract: Background: Patients may suffer from right ventricular (RV) failure and malignant cardiac arrhythmias after late pulmonary valve replacement correcting pulmonary regurgitation (PR). But the underlying mechanisms of the refractory arrhythmias are not well understood. Methods: The aim of present study was to characterize the RV myocardium after percutaneous pulmonary valve implantation (PPVI) in a porcine model after severe PR for 3months. RV histology was evaluated with morphometric methods and RV function was assessed with electrophysiology, echocardiography, and biochemical measures: The results were compared with age-matched sham-operated animals. Results: At euthanasia, RV weight was increased compared to sham-animals, median 127g (115–137) vs. 71g (69.5–76.5), p=0.0007. RV myocyte diameters corrected for individual variation with the RV/LV ratio were enlarged, 1.06 (1.02–1.13) vs. 0.84 (0.80–0.91), p=0.0006. There were no excess collagen tissue (RV/LV ratio), p=0.77. Electrophysiological stimulation resulted in RV arrhythmia in 67% of the animals compared to 25% in the sham-operated animals, but this difference was not statistically significant, p=0.28. Echocardiography revealed geometrical dilation in end-systolic RV area, mean±SD, 11.8±4.9cm2 vs. 6.0±3.5cm2, p=0.05, and end-diastolic area, 23.3±10.4cm2 vs. 12.7±2.5cm2, p=0.08. RV anterior free wall thickness was not increased, 0.7±0.2cm vs. 0.7±0.1cm, p=0.66. Echocardiographic functional parameters and plasma natriuretic peptides were unchanged. Conclusions: The RV does not completely recover after three months of PR with persistent myocardial hypertrophy one month after PPVI. Future studies should address whether RV chamber and cellular hypertrophy, without fibrosis or interventional scar tissue, may be substrate for arrhythmia. [Copyright &y& Elsevier]

Abstract: Background: Optimal treatment for stable repaired tetralogy of Fallot (rTOF) patients with pulmonary regurgitation (PR) and related right ventricular (RV) dilatation, including timing of valve implantation, remains uncertain. We sought to study tolerability of the angiotensin-converting-enzyme (ACE) inhibitor ramipril and its effects on cardiovascular function in these patients. Methods: Clinically stable rTOF patients with moderate/severe PR were included. A double-blinded, placebo-controlled study of 6months of ramipril vs placebo was performed. All patients underwent cardiovascular magnetic resonance (CMR), echocardiography, neurohormonal analysis, and objective cardiopulmonary exercise testing at baseline and follow-up. Primary endpoint: The main aim was to detect changes in RV function (primary endpoint CMR-derived RV ejection fraction). Results: Seventy-two patients were enrolled and 64 qualified for the final analysis. There was no difference in the primary endpoint RV ejection fraction. RV long-axis shortening significantly improved in the ramipril group compared to placebo (RV: 2.3±3.8 vs 0.02±2.7mm; P=0.017) as did LV long-axis shortening (1.9±4.5 vs −0.2±3.7mm respectively; P=0.030). No clear differences were detected between ramipril and placebo for other measures. In a subgroup of patients with restrictive RV physiology, ramipril resulted in decrease in LV end-systolic volume index and increase in LVEF (−2.4±5.0 vs 2.7±3.6mL/m2; P=0.005, 2.5±5.0 vs −1.3±3.5%; P=0.03). Ramipril did not cause adverse events and was well tolerated. Conclusions: Ramipril is a well tolerated therapy, improves biventricular function in patients with rTOF and may have a particular role in patients with restrictive RV physiology. Larger, longer-term studies are needed to determine if ACE inhibitors can improve both ventricular remodelling and clinical outcomes. (ISRCTN: 97515585) [Copyright &y& Elsevier]

Abstract: Objectives: Assessment of the long term outcome of mechanical valve prosthesis at pulmonary position in a population of grown-up congenital heart disease patients from a tertiary referral center. Methods: From 1977 to 2007, 22 consecutive patients underwent a total of 25 pulmonary valve replacements with mechanical prosthesis. The most frequent underlying cardiac condition was tetralogy of Fallot (n =16, 64%) and the mean age at the time of pulmonary valve replacement was 32±11years (range 14–50years). Results: The postoperative mortality rate was 4% ( n = 1) with no late deaths documented after a mean follow-up of 7.6±7.6years (range 0.29–24years). No major bleeding episodes occurred. Three patients presented with valve thrombosis in the setting of long term anticoagulation withdrawal and required valve re-replacement. Two of these patients, both with poor right ventricular function and overt clinical signs of right heart failure at the time of valve re-replacement, experienced further episodes of thrombosis despite correct anticoagulation. All episodes resolved with thrombolysis. After addition of antiplatelet treatment in one case and anticoagulation self-control, in the other, no further thrombosis has been documented. Conclusions: Mechanical valve prosthesis may be an alternative to tissue valve prosthesis in patients with congenital heart disease requiring pulmonary valve replacement. Optimal anticoagulation is crucial and additional antiplatelet treatment should be considered. Our data also suggest that patients with severe right ventricular dysfunction and congestive heart failure might be at particular risk for valve thrombosis. [Copyright &y& Elsevier]

Abstract: Background: After tetralogy of Fallot (ToF) repair the right ventricle (RV) is commonly exposed to abnormal volume load resulting from pulmonary regurgitation (PR) leading to progressive RV dilatation. The objective of this study was to assess the relationship between RV volumes, especially the end systolic volume index (ESVi), and RV contractility in patients after ToF repair and significant PR and to determine whether RV dilatation reflects intrinsic RV dysfunction in these patients. Methods: Twenty-nine ToF patients were studied 11.6 (range: 1.9–30.1) years after repair with the pressure–volume conductance system. The patient cohort was divided into two groups according to the median ESVi (group 1: ESVi<34.7 ml/m2 ×1.18, n =14; group 2: ESVi≥34.7 ml/m2 ×1.18, n =15). Results: The slope of the end systolic pressure–volume relationship (end systolic elastance, Ees) was higher in group 1 compared to group 2 both at baseline and during dobutamine infusion (0.87±0.36 vs. 0.46±0.28 mm Hg/ml and 1.50±0.77 vs. 0.92±0.37 mm Hg/ml; P <0.005 and P =0.02, respectively). Overall, Ees at baseline correlated significantly with ESVi and also with the end diastolic volume index (r =−0.64, and P <0.001 for both). Receiver operating characteristic curve analysis revealed that ESVi was superior to RV ejection fraction (EF) in predicting an Ees in the lowest quartile of the study group (area under curve ESVi vs. EF: 0.84 (0.64–0.95) vs. 0.68 (0.47–0.85); P= 0.015). Conclusion: ESVi is a valid estimate of intrinsic RV function in repaired ToF patients with residual PR and in this respect seems superior to EF. These data underscore the importance of serial ventricular volume assessment in the follow-up of these patients. [Copyright &y& Elsevier]

Volumetric determinations by cardiac magnetic resonance imaging after tetralogy of Fallot repair may more accurately assess significant right ventricular dilation and pulmonary regurgitation to guide timing of pulmonary valve replacement. Recent guidelines by the American and European Societies of Echocardiography have summarized the clinical approach to valvular stenosis. They emphasize aortic stenosis given its high incidence and assessment confounders such as left ventricular function, aortic regurgitation, systemic hypertension, and mitral regurgitation. The applications of 3-dimensional echocardiography have reached transcatheter procedures such as atrial septal closure, mitral valve repair, and aortic valve replacement. It also provides detailed assessment of the mitral valve, cardiac chambers, and can guide pediatric aortic valve repair. The timing of surgery in mitral regurgitation remains controversial, especially when it is asymptomatic with normal left ventricular function. Recent data emphasize the outcome advantage of mitral valve repair in asymptomatic mitral regurgitation when the effective regurgitant orifice area is >40 mm2. Transesophageal echocardiography is an established gold standard in the assessment of endocarditis. Multislice computed tomographic imaging has facilitated simultaneous detailed assessment of the cardiac valves and coronary arteries. Recent comparison has shown that these 2 imaging modalities are equivalent and complementary. Tricuspid valve regurgitation associated with mitral disease is common and important. At the time of mitral surgery, moderate or greater tricuspid regurgitation should be corrected, preferably by rigid annuloplasty. Recent evidence also supports tricuspid annuloplasty for an annular diameter >35 mm regardless of regurgitation severity. Although repair is preferred, tricuspid replacement also has acceptable outcomes. [Copyright &y& Elsevier]

Objectives: This study sought to examine the prevalence and predictors of pulmonary regurgitation (PR) following balloon dilation (BD) for pulmonary stenosis (PS) and to investigate its impact on ventricular volume and function, and exercise tolerance. Background: Balloon pulmonary valvuloplasty relieves PS but can cause late PR. The sequelae of isolated PR are not well understood. Methods: Patients were at least 7 years of age and 5 years removed from BD, and had no other form of congenital heart disease or significant residual PS. Cardiac magnetic resonance imaging and exercise testing were performed prospectively to quantify PR fraction, ventricular volumes and function, and exercise capacity. Results: Forty-one patients underwent testing a median of 13.1 years after BD. The median PR fraction was 10%; 14 patients (34%) had PR fraction >15%; 7 (17%) had PR >30%. PR fraction was associated with age at dilation (ln-transformed, R = −0.47, p = 0.002) and balloon:annulus ratio (R = 0.57, p < 0.001). The mean right ventricular (RV) end-diastolic volume z-score was 1.8 ± 1.9; RV dilation (z-score ≥2) was present in 14/35 patients (40%). PR fraction correlated closely with indexed RV end-diastolic volume (R = 0.79, p < 0.001) and modestly with RV ejection fraction (R = 0.50, p < 0.001). Overall, peak oxygen consumption (Vo 2) (% predicted) was below average (92 ± 17%, p = 0.006). Patients with PR fraction >15% had significantly lower peak Vo 2 than those with less PR (85 ± 17% vs. 96 ± 16%, p = 0.03). Conclusions: Mild PR and RV dilation are common in the long term following BD. A PR fraction >15% is associated with lower peak Vo 2, suggesting that isolated PR and consequent RV dilation are related to impaired exercise cardiopulmonary function. [Copyright &y& Elsevier]

Abstract: The population of adult survivors with tetralogy of Fallot (TOF) is growing over the last decades due to improvements in perinatal management, intensive care and surgical techniques. Pulmonary regurgitation (PR) plays a crucial role in the long-term outcome of these patients. Although PR may be tolerated well for many years, eventually it may lead to right ventricular (RV) dilatation, RV dysfunction, exercise intolerance, arrhythmia and sudden cardiac death. Cardiovascular magnetic resonance (CMR) imaging is an important tool in the follow-up of patients after TOF repair, because biventricular volumes and function and PR volume and fraction can be measured with great accuracy and reproducibility. CMR imaging studies have identified risk factors for late adverse outcomes in patients after TOF repair. These include increased RV end-diastolic volume (EDV), reduced left ventricular ejection fraction and abnormal RV outflow tract function. Other applications of CMR include stress imaging, assessment of diastolic function and late gadolinium enhancement, which have provided additional insight in the function of the RV. Timing of pulmonary valve replacement (PVR) is controversial and should balance between the preservation of RV function and the need for subsequent PVR surgery, since the life-span of a homograft is limited. Based on CMR imaging studies, PVR will be considered if the RVEDV reaches a threshold of between 150 and 200ml/m2 in the presence of severe PR. However, timing of PVR should be based on multiple factors, other than RV size and PR fraction alone. [Copyright &y& Elsevier]

Abstract: We report a case of a patient with congenital aortic stenosis previously repaired using the Ross procedure, who presented to our unit for urgent caesarean delivery. Management was complicated by moderate residual cardiac disease and the presence of a suprapubic femoro-femoral crossover graft. Following application of five-lead electrocardiogram and invasive blood pressure monitoring, anaesthesia was induced via combined spinal-epidural with epidural volume extension. A high transverse surgical approach avoided the course of the vascular graft, while further precautions included the immediate availability of vascular surgeons and cell salvage. Our anaesthetic technique was tailored to minimise disruption to cardiovascular function, and in particular to limit regurgitant flow across the pulmonary valve. This case highlights the value of early identification of high-risk parturients and multidisciplinary involvement at delivery. Risk stratification in the patient with grown-up congenital heart disease is based upon timely evaluation of the underlying congenital pathology, surgical history and subsequent functional status. [Copyright &y& Elsevier]

Abstract: Purpose: To assess biventricular functional reserve (FR), NT-proBNP levels and exercise performance, in relation to right ventricular volume in patients with pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) at young age. Methods: In 53 TOF patients (maximum age at repair 2.0 years, interval since repair 15 (5) years) without residual lesions except PR, biventricular FR (derived from magnetic resonance imaging with dobutamine stress), NT-proBNP levels, maximal workload, and peak oxygen uptake were assessed. Results: Mean right ventricular end-diastolic volume was 140(38) ml/m2. Median pulmonary regurgitant fraction was 37% (range 0–57%). Biventricular systolic stress response was normal: mean (SD) ESV decreased (ΔRVESV −17(8) ml/m2, ΔLVESV −11(5)), SV increased (ΔRVSV +12(9) ml/m2, ΔLVSV +9(6)), FR was positive in all (RV-FR +11(5)%, LV-FR +13(6)). No serious adverse effects to dobutamine were encountered. NT-proBNP was increased in 2 patients. Median level was 10 pmol/L (range 2–42). NT-proBNP correlated with PR-percentage but not with right ventricular size. High-risk levels of NT-proBNP indicated a smaller RV-FR and a smaller decrease of biventricular ESV. Mean (SEM) VO2max was 96(3)%, mean Workloadmax 89(2)% of predicted. Conclusion: At mid to long term follow-up overall NT-proBNP levels are normal and biventricular functional reserve and exercise tolerance are well preserved in TOF repaired at young age, irrespective of RV volume. This questions the validity of isolated PR or RV volume criteria for pulmonary valve replacement in this group. Low-dose dobutamine stress testing is well tolerated and may be a useful additional tool for clinical decision making. [Copyright &y& Elsevier]

Abstract: Background: Pulmonary regurgitation is common after repair of tetralogy of Fallot, predisposing to right ventricular dilatation and potentially fatal arrhythmias. Magnetic resonance studies of such patients led us to hypothesize that the amount of regurgitation, in the absence of an effective valve, depends on pulmonary arterial compliance and on the location of resistance relative to the compliance. Methods and results: Using a pre-existing mathematical model representing the cardiovascular system, removal of the virtual pulmonary valve gave a triphasic pulmonary artery flow curve similar in shape to those recorded in patients with free regurgitation, with a regurgitant fraction of 30%. There was no reversal of flow at pulmonary capillary level, the regurgitant volume originating entirely from the compliance of the virtual pulmonary arteries and arterioles. Doubling their compliance increased regurgitation to 35%, whereas halving it decreased regurgitation to 23%. Doubling the total pulmonary vascular resistance by increasing arteriolar resistance increased regurgitation to 46%, whereas doubling it by simulating pulmonary annular stenosis proximal to the compliance limited regurgitation to 10%, but at the cost of a 32 mmHg peak systolic pressure drop. Conclusions: The model supported our hypotheses, indicating the relevance to pulmonary regurgitation of previously overlooked variables. The virtual pulmonary regurgitation was exacerbated by pulmonary artery compliance and by elevated resistance distal to it, but was limited by more proximal resistance. These relationships merit careful clinical investigation as they would have implications for the initial management, subsequent investigation and decisions on re-intervention in patients with pulmonary regurgitation. [Copyright &y& Elsevier]

This chapter describes the structure/function relationships of the right ventricle (RV), and shows how the geometry of the helical ventricular myocardial band model defines spatial geometry of the free wall and septum that underlie dynamic action. Myocardial fiber orientation is the keynote to performance in health and disease. The transverse geometry of the RV free wall allows constriction (bellows-type motion), whereas oblique septal fiber orientation and midline septal position is essential for ventricular twisting, the vital mechanism for RV ejection against increased pulmonary vascular resistance. Therefore, the septum is considered “the lion or motor of RV performance.” Distortion of such normal structure/function relationships underlies the pathophysiologic mechanisms of RV failure. Operative methods that restore normal myocardial fiber orientation are described to outline evolving surgical techniques for the surgical treatment of RV failure. [Copyright &y& Elsevier]