PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Long-term follow-up after endovascular treatment of aortic coarctation with bare and covered Cheatham platinum stents.

BACKGROUND: Endovascular treatment of aortic coarctation (CoA) in children and adults frequently requires stent implantation. The aim of this study was to analyze long-term results after CoA treatment with bare and covered Cheatham-PlatinumTM (CP) stents in our institution and to derive recommendations for the differential use of these stent types. METHODS: In this retrospective single institution study, 212 patients received endovascular CoA treatment with bare (n = 71) and covered (n = 141) CP stents between September 1999 and July 2021, respectively. The indications for treatment were native CoA in 110/212 patients (51.9%) and re-coarctation after primary surgical or interventional treatment in 102/212 patients (48.1%). Median patient age at endovascular CoA treatment was 18.8 years [IQR 11.9; 35.8]. Long-term follow-up was available in 158/212 patients (74.5%) with a median follow-up of 7.3 years [IQR 4.3; 12.6]. RESULTS: Procedural success was achieved in 187/212 (88.2%) patients. Survival rate was 98.1% after 5, and 95.6% after 10 and 15 years, respectively. The probability of freedom from re-intervention was 93.0% after 5, 82.3% after 10 and 77.8% after 15 years, respectively. Freedom from re-interventions (44/158, 27.8%) did not differ between patients who received bare or covered CP stents (p = 0.715). Multivariable risk factor analysis identified previous CoA surgery (HR: 2.0, 95% confidence interval (CI): 1.1-3,9, p = 0.029), postdilatation (HR: 2,9, 95% CI: 1.1-6.3, p = 0.028) and age at intervention (HR: 0.96, 95% CI: 0.94-0.99, p = 0.002) as independent risk factors for re-intervention. Peri-procedural complications occurred in 15/212 (7.1%) patients (dissection/thrombosis of vascular access vessel: n = 9; bleeding: n = 1; stent dislocation: n = 2; aortic dissection/aortic wall rupture: n = 3). Long-term complications were observed in 36 patients and included stent fracture (n = 19), aneurysm formation (n = 14), endoleak (n = 1) and subclavian artery stenosis (n = 2). Peri-procedural and long-term complications did not differ between patients who received CoA treatment with bare or covered CP stents (all p > 0.05). CONCLUSION: Endovascular treatment of CoA using bare or covered CP stents can be performed safely and effectively with excellent long-term results. Survival, re-intervention and complication rate did not significantly differ between both stent types. However, individual stent selection is advisable with regard to CoA morphology and severity as well as patient age.

Nationwide Registry-Based Analysis of Infective Endocarditis Risk After Pulmonary Valve Replacement.

Background Infective endocarditis (IE) after pulmonary valve replacements in congenital heart disease is a significant concern. This study aimed to identify specific long-term risk factors for IE after percutaneous pulmonary valve implantation or surgical pulmonary valve replacement. Methods and Results All patients with congenital heart disease from the National Register for Congenital Heart Defects with at least 1 pulmonary valve replacement before January 2018 were included. A total of 1170 patients (56.3% men, median age at study inclusion 12 [interquartile range {Q1-Q3} 5-20 years]) received 1598 pulmonary valve replacements. IE occurred in 4.8% of patients during a follow-up of total 9397 patient-years (median 10 [Q1-Q3, 6-10] years per patient). After homograft implantation 7 of 558 (1.3%) patients developed IE, after heterograft implantation 31 of 723 (4.3%) patients, and after Melody valve implantation 18 of 241 (7.5%) patients. Edwards Sapien and mechanical valves were used less frequently and remained without IE. The incidence of IE in heterografts excluding Contegra valves was 7 of 278 (2.5%), whereas the incidence of IE in Contegra valves was 24 of 445 (5.4%). The risk of IE was not increased compared with homografts if Contegra valves were excluded from the heterografts (hazard ratio [HR], 2.60; P=0.075). The risk of IE was increased for bovine jugular vein valves, Contegra valves (HR, 6.72; P<0.001), and Melody valves (HR, 5.49; P<0.001), but did not differ between Melody valves and Contegra valves (HR, 1.01; P=0.978). Conclusions Bovine jugular vein valves have the highest risk of IE, irrespective of the mode of deployment, either surgical or percutaneous.

Long-term experience using CNI-free immunosuppression in selected paediatric heart transplant recipients.

BACKGROUND: CNI-free immunosuppression with conversion to mTORi-based immunosuppression has been demonstrated to reduce CNI-toxicity and to exhibit anti-proliferative properties. However, the experience of CNI-free immunosuppression in paediatric heart transplantation is limited. METHODS: A retrospective analysis was conducted of 129 paediatric heart transplants performed between 1997 and 2015. Fifteen patients with clinically indicated conversion from CNI-based to CNI-free immunosuppression were identified. Survival data, rejection episodes, renal function, post-transplantation lymphoproliferative disorder and CAV, including examination with OCT were analysed. RESULTS: Immunosuppression conversion was successful in all patients. Fourteen of 15 patients (93%) are currently living with good graft function. Median post-transplant survival was 15 years (range, 5-23 years), and median follow-up since conversion was 6 years (range, 1-11 years). Mild (grade 1R) ACR was present in three patients after discontinuation of CNIs. The recovery of renal function with a significant increase in eGFR was observed at 1 and 3 years after conversion. No patient had angiographic signs of macroscopic CAV according to the current ISHLT classification; however, OCT showed the signs of angiographically silent CAV in all patients. CAV did not progress in any patient, implying CAV was stabilised by mTORi-based CNI-free immunosuppression. CONCLUSIONS: CNI-free immunosuppression based on mTORis is a safe and appropriate strategy for maintenance therapy in selected paediatric patients, significantly improves renal function and stabilises CAV. OCT revealed early development of angiographically silent CAV.

Persisting and reoccurring cyanosis after Fontan operation is associated with increased late mortality.

OBJECTIVES: The Fontan operation aims at reducing cyanosis and cardiac volume overload in patients with complex univentricular heart malformations. However, persisting or reoccurring cyanosis is frequently observed. We sought to systematically determine the prevalence and clinical consequences of persisting and secondary cyanosis after Fontan operation. METHODS: A total of 331 Fontan patients, operated between 1984 and 2016 with a median postoperative follow-up of 7.9 (interquartile range 2.6-15.8) years, were studied retrospectively. Cyanosis was defined as transcutaneous oxygen saturation ≤93% at rest measured by pulse oximetry. Prevalence of cyanosis was analysed at 3 different time points (t1 = post-Fontan operation, t2 = post-Fontan cardiac catheterization, t3 = last follow-up) and the association of cyanosis with mortality was examined. RESULTS: Prevalence of cyanosis was 50% at t1 and 39% at t3. Fenestration was patent in 71% and 33% of all cyanotic patients at t1 and t3, respectively. In patients with clinical indication for catheterization (t2; n = 178/331), prevalence of cyanosis was 72%. At t2, patent fenestration (33%), veno-venous collaterals (24%) or both (32%) were present. Thirty-six (11%) patients died during follow-up. In a time-varying multivariable Cox regression analysis, cyanosis was the strongest predictor for late mortality (P < 0.001, hazard ratio 12.2, 95% confidence interval 3.7-40.5). CONCLUSIONS: Prevalence of cyanosis was considerable during long-term follow-up after Fontan operation and-as a surrogate parameter for unfavourable Fontan haemodynamics-is associated with increased late mortality. Accordingly, particular attention should be directed towards the persistence or reoccurrence of cyanosis during follow-up since it may indicate haemodynamic attrition and development of Fontan failure.

Pulmonary artery augmentation using decellularized equine pericardium (Matrix Patch™): initial single-centre experience.

OBJECTIVES: The aim of this study was to report our initial experience when using Matrix Patch™ a cell-free equine-derived pericardium for the augmentation of branch pulmonary arteries (PAs) in children. METHODS: Between September 2016 and September 2019, Matrix Patch was used for the augmentation of branch PAs in 96 patients and implanted in 147 separate locations. The median age at implantation was 3.2 years (interquartile range: 0.9-8.4), and 33% of patients were infants. The patch was used mainly in redo surgeries (89.6%). Intra-procedural feasibility and reinterventions were analysed. Primary end points were death or patch-related reoperation/stent implantation. Explanted patches were stained for recellularization/calcification, or to reveal proliferation/inflammation. RESULTS: A total of 81 patients, who received patches in 119 separate locations, were followed within a median of 20 months (interquartile range: 10.2-30.2). Patients with early reoperation/stent implantation were excluded from follow-up. No patch-related death was noted. Survival at last follow-up was 88% (95% CI: 78.8-93.7%). Overall probability of freedom from reoperation/stent implantation per location, 12 and 24 months after initial surgery was 85.8% (95% CI: 76.2-91.7%) and 78.7 (95% CI: 65.9-87.2%), respectively. At 20 months, superficial proliferation with discrete macrophage activity was seen in explants; however, no signs of calcification are observed. CONCLUSIONS: The initial experience with the Matrix Patch in PAs showed comparable results to other xenogeneic patch materials. Long-term follow-up data are needed to prove the desired durability of the patch in different locations.

Results of aortic valve repair using decellularized bovine pericardium in congenital surgery.

OBJECTIVES: The search for an optimal patch material for aortic valve reconstruction (AVR) is an ongoing challenge. In this study, we report our experience of AVR using decellularized bovine pericardial patch material in congenital heart surgery. METHODS: Data of 40 consecutive patients who underwent AVR using the CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) between February 2014 and August 2016 were retrospectively reviewed. The median age of the patients at operation was 9 (2-34) years, and 18 patients were younger than 7 years. Twenty-six patients initially presented with aortic valve insufficiency (AI) and 14 with stenosis. Clinical and echocardiographic data were available until August 2017 for a median postoperative follow-up (FU) of 22 (6-42) months. RESULTS: Nine of 40 (23%) patients experienced an event during FU (death: n = 1, 2.5%; reoperation: n = 8, 20%). Overall, the probability of freedom from reoperation or death was 97 ± 3%, 76 ± 9% and 57 ± 12% at 12, 24 and 36 months of FU, respectively. Reason for reoperation was stenosis in 3 (37.5%) patients, insufficiency in 4 (50%) patients and 1 (12.5%) patient was diagnosed with aortic valve endocarditis. Of the remaining 31 patients, 2 patients are scheduled for reoperation (aortic valve stenosis: n = 1 and AI: n = 1) and 9 patients exhibit worsening of aortic valve function with moderate AI. Freedom from developing combined end point [death/reoperation/moderate degree of aortic valve dysfunction (aortic valve stenosis, AI)] after AVR was 92 ± 5%, 55 ± 9% and 28 ± 9% at 12, 24 and 36 months, respectively. CONCLUSIONS: AVR using decellularized bovine pericardial patch material in patients with congenital aortic valve disease show unsatisfactory results within the first 3 years of FU.

Presence of reduced regional left ventricular function even in the absence of left ventricular wall scar tissue in the long term after repair of an anomalous left coronary artery from the pulmonary artery.

BACKGROUND: We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery. METHODS: A total of 20 patients aged 12.8±7.4 years were assessed 10 (0.5-17) years after the repair of an anomalous origin of the left coronary artery from the pulmonary artery; of them, 10 (50%) patients showed left ventricular wall scar tissue on current cardiac MRI. Left ventricular regional function was assessed by two-dimensional speckle-tracking echocardiography in 10 patients with scar tissue and 10 patients without scar tissue and in 10 age-matched controls. RESULTS: In patients with scar tissue, MRI-derived left ventricular ejection fraction was significantly reduced compared with that in patients without scar tissue (51 versus 61%, p<0.05), and echocardiography-derived longitudinal strain was significantly reduced in five of six left ventricular areas compared with that in healthy controls (average relative reduction, 46%; p<0.05). In patients without scar tissue, longitudinal strain was significantly reduced in two of six left ventricular areas (average relative reduction, 23%; p<0.05) and circumferential strain was reduced in one of six left ventricular areas (relative reduction, 56%; p<0.05) compared with that in healthy controls. CONCLUSIONS: Regional left ventricular function is reduced even in patients without left ventricular wall scar tissue late after successful repair of an anomalous origin of the left coronary artery from the pulmonary artery. This highlights the need for meticulous lifelong follow-up in all patients with a repaired anomalous origin of the left coronary artery from the pulmonary artery.

Systemic right ventricular morphology in the early postoperative course after extracardiac Fontan operation: is there still a need for special care?

Objectives: We aimed to compare early postoperative outcome after extracardiac (EC) Fontan operation between patients with right (RV) or left (LV) systemic ventricles. Methods: In total, 173 consecutive patients (median age 4 years, median weight 14 kg) underwent EC Fontan between 1995 and 2013. Pre- and intraoperative data as well as detailed postoperative haemodynamic variables were compared between patients with LV [ n = 109 (63%)] and RV [ n = 64 (37%)]. Results: : RV patients showed significantly lower mean arterial (median 55 vs 59 mmHg, P = 0.04), higher atrial (median 8 vs 6 mmHg, P = 0.03) and comparable pulmonary pressure (median 14 vs 14 mmHg, P = 0.7) as well as lower mean systemic perfusion pressure (median 39 vs 43 mmHg, P = 0.03) on Day 0 after EC Fontan. They suffered from longer intubation time (median 18 vs 12 h, P = 0.008), higher incidence of ascites (46% vs 28%, P = 0.04) and need for dialysis (21% vs 4%, P = 0.003). Prolonged inotropic support (25% vs 8%, P = 0.02) and pharmacological treatment to reduce pulmonary vascular resistance (71% vs 53%, P = 0.002) were more often used in RV patients and they showed more often supraventricular tachyarrhythmia (27% vs 5%, P < 0.001) and a longer intensive care unit-stay (median 4 vs 3 days, P = 0.03). However, early mortality, need for Fontan takedown, use of mechanical circulatory support, pleural effusions and hospital stay were not significantly different between both groups. Conclusions: Patients with systemic RV demonstrate higher morbidity in the early postoperative course compared with patients with systemic LV anatomy and require intensified postoperative management to avoid postoperative Fontan failure.

Use of cardiovascular magnetic resonance imaging for TAVR assessment in patients with bioprosthetic aortic valves: comparison with computed tomography.

PURPOSE: Transcatheter aortic valve replacement (TAVR) has been successfully used to treat patients with failing aortic bioprostheses. Computed tomography (CT) is the usual method of pre-procedural imaging for TAVR in the native position; however, the optimal modality for valve-in-valve procedures has not been established. CT can assess intracardiac anatomy and is superior to cardiovascular magnetic resonance (CMR) in the assessment of coronary artery disease. However, CMR can provide superior haemodynamic information, does not carry the risk of ionising radiation, and may be performed without contrast in patients with renal insufficiency. In this study, we compared CT and CMR for the evaluation of TAVR in a small cohort of patients with existing aortic bioprostheses. MATERIALS AND METHODS: 21 patients with aortic bioprostheses were prospectively evaluated by CT and CMR, as pre-assessment for TAVR; agreement between measurements of aortic geometries was assessed. RESULTS: 16/21 patients had aortic bioprostheses constructed with a metal ring, and 5/21 patients had a metal strut construction. Patients with metal struts had significant metal-artefact on CMR, which compromised image quality in this region. There was good agreement between CT and CMR measurements of aortic geometry. The mean difference (d) in annulus area-derived diameter was 0.5mm (95% limits of agreement [L.A] 4.2mm). There was good agreement between modalities for the cross-sectional area of the sinuses of valsalva (d 0.5 cm(2), L.A 1.4 cm(2)), sinotubular junction (d 0.9 cm(2), L.A 1.5 cm(2)), and ascending aorta (d 0.6 cm(2), L.A 1.4 cm(2)). In patients without metal struts, the left coronary artery height d was 0.7 mm and L.A 2.8mm. CONCLUSIONS: Our analysis shows that CMR and CT measurements of aortic geometry show good agreement, including measurement of annulus size and coronary artery location, and thus provide the necessary anatomical information for valve-in-valve TAVR planning. However, in patients with metal strut aortic valve constructions, CT should be performed due to the presence of limiting metal artefacts on CMR. CMR may be considered as an appropriate alternative to CT in patients in whom iodinated contrast agents are contraindicated or where additional haemodynamic assessment with phase-contrast CMR is required.

Functional outcomes after the Ross (pulmonary autograft) procedure assessed with magnetic resonance imaging and cardiopulmonary exercise testing.

OBJECTIVE: To assess autograft, homograft and ventricular function, as well as exercise capacity, in adult patients who have undergone the Ross procedure. SETTING: Single centre paediatric and adult congenital heart disease unit. Patients 45 subjects (24.6 years, range 16.9-52.2 years) who underwent the Ross procedure between 1994 and 2006 (8.1 years after the Ross operation, range 2.0-14.0 years). Interventions Cardiovascular magnetic resonance imaging, echocardiography and cardiopulmonary exercise testing. MAIN OUTCOME MEASURES: Autograft and homograft stenosis, and regurgitation. Autograft size. Biventricular function, scar volume and exercise capacity. RESULTS: Mean autograft regurgitation was 6.8%+/-8.3% (trivial regurgitation) and diameter was 40.0+/-7.0 mm. Mean homograft velocity was 2.4+/-0.6 m/s (mild-moderate stenosis) and regurgitation was 6.1%+/-8.3% (trivial regurgitation). Biventricular systolic function was normal (LV EF 63.1+/-6.4% and RV EF 60.1%+/-7.6%). In 38% of cases there was evidence of LV scar, mostly noted within the inter-ventricular septum. The mean exercise capacity achieved was 87%+/-22% of predicted. There was no correlation between exercise capacity and ventricular function or scar. CONCLUSION: This study demonstrates minor autograft and homograft dysfunction in the majority of patients after the Ross procedure, associated with good ventricular function and exercise capacity. In addition, minor scar was present in a third of patients with no functional consequences.

Effective transcatheter valve implantation after pulmonary homograft failure: a new perspective on the Ross operation.

OBJECTIVE: The Ross procedure offers good autograft function and low reoperation rates for the neoaortic valve; however, the rate of conduit dysfunction in the right ventricular outflow tract remains a concern. This study assessed percutaneous pulmonary valve implantation in this setting. METHODS: We retrospectively analyzed outcomes of 12 patients (mean age 28 +/- 5 years) referred for percutaneous pulmonary valve implantation to treat right ventricle-pulmonary artery conduit failure 11.1 +/- 3.3 years after Ross procedure. RESULTS: Percutaneous pulmonary valve implantation was feasible in all 12 patients, with no procedural complications (procedure time 99 +/- 16 minutes, fluoroscopy time 21 +/- 6 minutes). Right ventricular outflow tract gradient during catheterization and pulmonary regurgitant fraction on magnetic resonance imaging fell after valve implantation (gradient 34 +/- 6 to 14 +/- 3 mm Hg, P < .01, regurgitant fraction 20% +/- 6% to 2% +/- 1%, P < .05). After restoration of right ventricular outflow tract function, indexed right ventricular end-diastolic volume decreased (91 +/- 13 to 78 +/- 12 mL x beat(-1) x m(-2), P < .01) and maximal cardiopulmonary exercise performance improved (peak oxygen consumption 25.4 +/- 2.3 to 30.8 +/- 3.0 mL x kg(-1) x min(-1), P < .01). During follow-up (18.8 +/- 4.6 months), there was 1 device explantation (restenosis). The probabilities of freedom from right ventricular outflow tract reoperation were 100% at 1 year and 90% at 3 years. CONCLUSIONS: Percutaneous pulmonary valve implantation provides an effective transcatheter treatment strategy to prolong the lifespan of right ventricle-pulmonary artery conduits after the Ross procedure, reducing the reoperation burden on patients with aortic valve disease.

Quantitative assessment of homograft function 1 year after insertion into the pulmonary position: impact of in situ homograft geometry on valve competence.

AIMS: To prospectively evaluate homograft function with cardiac magnetic resonance (CMR) imaging 1 year after insertion into the pulmonary position, and to assess the impact of in situ homograft geometry, surgical factors, and 'intrinsic' homograft properties on early valve incompetence. METHODS AND RESULTS: A total of 60 patients (mean age 21 +/- 10 years; 35 females) with congenital heart disease underwent pulmonary valve replacement with homograft insertion and were prospectively enrolled into a study protocol that included serial echocardiography and CMR 1 year after surgery. None of the patients had homograft stenosis but 10 (17%) had significant homograft incompetence (i.e. pulmonary regurgitation fraction >20% on CMR). A higher incidence of 'eccentric' pulmonary forward flow pattern (P < 0.001, Fisher's exact test), more acute 'homograft distortion angle' (P < 0.001), larger relative 'annular' size (P < 0.01), and greater pre-homograft right ventricular outflow tract (RVOT) diameters (P = 0.01) at CMR was seen in those with worse homograft function. In a backward multivariate linear regression model, 'eccentric' pulmonary forward flow pattern (r(part) = 0.36, P < 0.001), 'homograft distortion angle' (r(part) = 0.31, P = 0.001), and pre-homograft RVOT diameter (r(part) = 0.19, P = 0.03) were independently associated with the degree of pulmonary regurgitation (in %) at 1 year. CONCLUSION: Using CMR, in this prospective cohort study, we have shown that significant valve incompetence is present in one-sixth of patients after homograft insertion into the pulmonary position, and that alterations in the in situ homograft geometry were associated with the likelihood of developing valve incompetence. These findings imply that mechanical factors may have an important impact on homograft performance.

Percutaneous pulmonary valve replacement.

Percutaneous pulmonary valve implantation helps in prolonging the lifespan of surgically placed right ventricle-to-pulmonary artery (RV-PA) conduits, and represents a less invasive alternative to repeat open-heart surgery. The clinical indications for treatment match those of surgery. As far as the suitability is concerned, the current ideal substrate is a degenerated RV-PA conduit, because of the presence of a certain degree of calcification that offers a safe anchoring point. So far, patients have been treated with low morbidity and mortality that were further improved with increasing experience. After percutaneous pulmonary valve implantation, patients experience a subjective improvement, mirrored by an objective increase of measured exercise capacity parameters and by reduced RV-PA gradient, regurgitant fraction, and RV volumes. New developments in the experimental field should result in an extension of indications in the future.

Current approaches to pulmonary regurgitation.

OBJECTIVE: To evaluate the effects on ventricular function and volumes following right ventricular outflow tract reconstruction (RVOTR) with pulmonary homograft replacement (PVR) and percutaneous pulmonary valve implantation (PPVI) for predominant pulmonary regurgitation. This study was not intended to compare the two approaches. METHODS: We prospectively examined 25 patients (mean age 21+/-13 years, 96% tetralogy of Fallot, 1/25 with conduit dysfunction) who had PVR with RVOTR for severe pulmonary regurgitation (PR), and 11 patients (mean age 20+/-9 years, 64% tetralogy of Fallot, 9/11 with conduit dysfunction) who underwent PPVI for predominant PR. Mean age at primary repair in both groups was 4.3+/-6.6 years. Magnetic resonance imaging was performed prior to, and 1 year following, interventions. RESULTS: Before procedure, NYHA classification was similar in both groups 2.1+/-0.5. Following interventions, there was a significant reduction in RV volumes in both groups. In the surgical (PVR) group, RV end-diastolic volume (EDV) decreased from 151+/-49 to 97+/-32 ml/m(2) (p<0.0001) whereas end-systolic volume (ESV) decreased from 80+/-43 to 46+/-23 ml/m(2) (p<0.0001). In the PPVI group, RV EDV decreased from 106+/-27 to 89+/-25 ml/m(2) (p=0.002) and RV ESV from 49+/-20 to 40+/-16 ml/m(2) (p=0.034). Both groups had a significant improvement in RV (63+/-20 to 72+/-16 ml/beat, p=0.003 (PVR group), 53+/-14 to 67+/-16 ml/beat, p=0.030 (PPVI group)) and LV effective stroke volume (61+/-18 to 73+/-16 ml/beat, p=0.001 (PVR group); 59+/-24 to 75+/-16 ml/beat, p=0.009 (PPVI group)). CONCLUSIONS: Following either PVR with RVOTR or PPVI, there was a significant reduction in RV volumes and an improvement in RV function. Importantly, in both groups, LV effective SV increased, and this may be the parameter to judge the benefit of the procedure. These results also support PPVI as an extra dimension in complex RVOT management.

Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome.

BACKGROUND: Percutaneous pulmonary valve implantation was introduced in the year 2000 as a nonsurgical treatment for patients with right ventricular outflow tract dysfunction. METHODS AND RESULTS: Between September 2000 and February 2007, 155 patients with stenosis and/or regurgitation underwent percutaneous pulmonary valve implantation. This led to significant reduction in right ventricular systolic pressure (from 63+/-18 to 45+/-13 mm Hg, P<0.001) and right ventricular outflow tract gradient (from 37+/-20 to 17+/-10 mm Hg, P<0.001). Follow-up ranged from 0 to 83.7 months (median 28.4 months). Freedom from reoperation was 93% (+/-2%), 86% (+/-3%), 84% (+/-4%), and 70% (+/-13%) at 10, 30, 50, and 70 months, respectively. Freedom from transcatheter reintervention was 95% (+/-2%), 87% (+/-3%), 73% (+/-6%), and 73% (+/-6%) at 10, 30, 50, and 70 months, respectively. Survival at 83 months was 96.9%. On time-dependent analysis, the first series of 50 patients (log-rank test P<0.001) and patients with a residual gradient >25 mm Hg (log-rank test P=0.01) were associated with a higher risk of reoperations. CONCLUSIONS: Percutaneous pulmonary valve implantation resulted in the ability to avoid surgical right ventricular outflow tract revision in the majority of cases. This procedure might reduce the number of operations needed over the total lifetime of patients with right ventricle-to-pulmonary artery conduits.

Rescue surgery following percutaneous pulmonary valve implantation.

OBJECTIVE: Percutaneous pulmonary valve insertion (PPVI) is an evolving alternative to surgical pulmonary valve insertion. The aim of this study is to review the acute complications of PPVI requiring emergency rescue surgery. PATIENTS AND METHODS: Between 09/2000 and 01/2007, 152 patients (pts), received a PPVI. Patient's charts were reviewed in retrospect. RESULTS: Emergency rescue surgery (ERS) took place in 6 pts (3.9%). Indications for ERS were: homograft rupture two pts, dislodgment of the stented valve in a dilated right ventricular outflow tract two pts, occlusion of the right pulmonary artery one pt and compression of the left main coronary artery one pt. Cardiopulmonary bypass was established through repeat sternotomy incision with femorofemoral cannulation in 2/6 pts. The stented valve was removed in five and replaced with a homograft in three and a valved conduit in two pts. One ruptured homograft was repaired leaving the stented valve in situ. All patients survived, one sustained mild neurological impairment. CONCLUSION: Although some of the acute complications of PPVI were probably related to a learning curve (4 among the first 50 pts and 2 among the last 102 patients) the need for ERS is unlikely to be completely abolished. This experience highlights the importance of close collaboration between cardiologists and surgeons in these evolving technologies. Highly skilled and responsive surgical back up is necessary to support the introduction and to sustain institutional programmes such as PPVI.

Percutaneous implantation of the pulmonary and aortic valves: indications and limitations.

Percutaneous transcatheter intervention for valvular heart disease is the new horizon in transcatheter therapeutics. Balloon dilatation has been used successfully for treatment of congenital and acquired stenotic lesions of semilunar and atrio-ventricular valves. Although attempts have been made to repair and replace cardiac valves without cardiopulmonary bypass and through percutaneous techniques, this has only recently become a reality. The semilunar valves have preceded atrioventricular valves in successful application in animals and humans. Morphological features play an important role in determining the design of the valve and technique and site of implantation. The major deviations in research and development in artificial or tissue valves have included attempts at delivery of these valves to the site of implantation without open heart surgery. Successful implantation needs long-term follow-up for the durability of the valve and freedom from re-intervention.

Current experience with percutaneous pulmonary valve implantation.

Transcatheter valve replacement has recently been introduced into clinical practice and has the potential to transform the management of valvular heart disease. To date, the largest human experience exists with percutaneous pulmonary valve implantation in patients with repaired congenital heart disease who require re-intervention to the right ventricular outflow tract. The application of this approach, however, is presently restricted to certain right ventricular outflow tract morphologies, because the device needs to be anchored safely to prevent device dislodgement. Early results of percutaneous pulmonary valve implantation show lower morbidity than surgery and significant early symptomatic improvement. In the future, the challenge will be to extend percutaneous pulmonary valve implantation to all patients with a clinical indication to delay or avoid repeat open-heart surgery.