Morphology of the native ascending aorta after the Norwood procedure for aortic atresia: impact on survival and right ventricular dysfunction.

OBJECTIVES: To evaluate the impact of variable morphology of the native ascending aorta after the Norwood I procedure in patients with hypoplastic left heart syndrome/aortic atresia on long-term survival and systemic right ventricular dysfunction. METHODS: Of 151 survivors of the Norwood procedure for hypoplastic left heart syndrome/aortic atresia at our institution between January 2001 and December 2020, we included patients with available and measurable aortography prior to stage II palliation. Diameter of the native ascending aorta, length of the native ascending aorta, and the angle between the the native ascending aorta and the proximal pulmonary artery were measured. We investigated the impact of these morphologic parameters on the mortality and the right ventricular dysfunction (defined as at least moderate). RESULTS: Angiography was available in 78 patients. Median diameter of native ascending aorta was 3.2 mm (2.6-3.7), median length of native ascending aorta was 15.4 mm (13.3-17.9), and median angle between the native ascending aorta and the proximal pulmonary artery was 44° (35° - 51°). During median follow-up of 6.5 years, eight (10%) patients died and systemic right ventricular dysfunction occurred in 19 patients (24%). No significant association between the aortic morphology and mortality could be detected. Right ventricular function was negatively affected by a larger angle between the native ascending aorta and the proximal pulmonary artery and (odds ratio 1.07 [1.01-1.14], P= 0.02). CONCLUSIONS: In survivors of the Norwood procedure for hypoplastic left heart syndrome/aortic atresia with available angiography, no significant association between the native aortic morphology and mortality could be demonstrated after stage II palliation, within the scope of this limited study. A larger anastomosis angle between the native ascending aorta and the proximal pulmonary artery emerged as a risk factor for right ventricular dysfunction.

Relationship of Aortopulmonary Collaterals and Pulmonary Artery Development During Staged Single Ventricle Reconstruction.

To evaluate the relationship of aortopulmonary collaterals and the development of central pulmonary arteries during staged palliation. A total of 287 patients, who underwent staged palliation with bidirectional cavopulmonary shunt and total cavopulmonary connection between 2008 and 2019, had available angiography. Pulmonary artery index was calculated using pulmonary angiography as described by Nakata and colleagues. Aortopulmonary collaterals were observed in 47 (16%) patients at stage II palliation, in 131 (46%) at total cavopulmonary connection, and afterwards in 49 (7%). The interventional closure of aortopulmonary collaterals was performed before stage II in 12 (4%) patients, before Fontan completion in 38 (13%), and afterwards in 39 (14%). Presence of aortopulmonary collaterals before stage II was not associated with the pulmonary artery index (129 vs. 150 mm2/m2, p = 0.176) at stage II. In contrast, aortopulmonary collaterals before the Fontan completion were associated with lower pulmonary artery index (154 vs. 172 mm2/m2, p = 0.005), and right pulmonary artery index (99 vs. 106 mm2/m2, p = 0.006). Patients who underwent interventional closure of aortopulmonary collaterals before total cavopulmonary connection had lower pulmonary artery index (141 vs. 169 mm2/m2, p < 0.001), lower right pulmonary artery index (93 vs. 106 mm2/m2, p = 0.007), and left pulmonary artery index (54 vs. 60 mm2/m2, p = 0.013) at Fontan completion. The presence of aortopulmonary collaterals did not influence pulmonary artery size by the time of stage II. However, presence of aortopulmonary collaterals was associated with under-developed pulmonary arteries at Fontan completion, especially in patients who needed interventional closure of aortopulmonary collaterals.

Mobile bedside ductus arteriosus closure in severely premature neonates using only echocardiographic guidance.

BACKGROUND: Transcatheter closure of the patent ductus arteriosus (PDA) in premature infants is currently dependent on fluoroscopic guidance and transportation to the catheterization laboratory. AIM: We describe a new echocardiographically guided technique to allow our team to move to the bedside at the neonatal intensive care unit (NICU) of the referring center for percutaneous treatment of PDA in premature infants. METHODS: This is a single-center, retrospective, primarily descriptive analysis. Clinical details about the procedure, its outcomes, and complications were collected. RESULTS: Fifty-eight neonates with a median weight of 1110 g (range 730-2800) and postnatal age of 28 days (range 9-95) underwent percutaneous PDA closure. Five of them were treated in our center with ultrasound guidance only and the other 53 in 18 different neonatology units in 12 towns. The median duration of the procedure was 40 min (range 20-195 min). There were no procedural deaths. There was one residual shunt for 3 weeks, in all other patients the duct closed completely in the first few hours after the intervention. In one patient the procedure had to be interrupted because of a pericardial effusion which had to be drained, the PDA was closed successfully interventionally 5 days later. One device-related aortic coarctation had to be stented. One embolization and one late migration occurred and required treatment. CONCLUSIONS: Echocardiographically guided transcatheter closure of the PDA in prematures was repeatedly possible and allowed that the procedure is performed at the bedside at the NICU with an acceptable rate of complications.

Comparison of ductus stent versus surgical systemic-to-pulmonary shunt as initial palliation in patients with univentricular heart.

OBJECTIVES: In this study, we aimed to compare infants with univentricular hearts who underwent an initial ductus stenting to those receiving a surgical systemic-to-pulmonary shunt (SPS). METHODS: All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting or a surgical SPS between 2009 and 2022 were reviewed. Outcomes were compared after ductus stenting or SPS including survival, probability of re-interventions and the probability to reach stage II palliations. RESULTS: A total of 130 patients were evaluated, including 49 ductus stenting and 81 SPSs. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19 patients. There was comparable hospital mortality (2.0% stent vs 3.7% surgery, P = 0.91) between the groups, but shorter intensive care unit stay (median 1 vs 7 days, P < 0.01) and shorter hospital stay (median 7 vs 17 days, P < 0.01) were observed in patients with initial ductus stenting, compared to those with SPS. However, acute procedure-related complications were more frequently observed in patients with ductus stenting, compared with those with SPS (20.4 vs 6.2%, P = 0.01), and 10 patients needed a shunt procedure after the initial ductus stent. The cumulative incidence of reaching stage II was similar between ductus stenting and SPS (88.0 vs 90.6% at 12 months, P = 0.735). Pulmonary artery (PA) index (median 194 vs 219 mm2/m2, P = 0.93) at stage II was similar between patients with ductus stenting and SPS. However, the ratio of the left to the right PA index [0.69 (0.45-0.95) vs 0.86 (0.51-0.84), P = 0.015] was higher in patients who reached stage II with surgical shunt physiology, compared with patients with ductus stent physiology. CONCLUSIONS: After initial ductus stenting in infants with univentricular heart, survival is comparable and post-procedural recovery shorter, but more acute stent dysfunctions and lower development of left PA are observed, compared to acute shunt dysfunctions. The less invasive procedure and shorter hospital stay are at the expense of more stent reinterventions.

Proof of Concept: A New Solution for Low-Profile Transcatheter Implantation of Optimus-L Stents in Small Children.

BACKGROUND: There is no stent designed or approved for use in infants. We sought to obtain in vitro and in vivo data on a new concept conceived to implant Optimus-L stents at infant vessel diameters and offer a potential long term stent solution. METHODS: Nineteen Optimus-L stents were mounted on 8 types of angioplasty balloons with diameters 6, 8, and 10 mm with the use of an injection-moulded hand crimper. We evaluated balloon-stent unit (BSU) stability before insertion and advancement through short Terumo introducers with incremental French size and possibility of side-arm contrast injections. Three types of long sheaths were tested. Stents were inflated to balloon nominal diameters and re-expanded to 18 and 23 mm. Stent recoil, foreshortening, and fracture were evaluated. In vivo implantations were performed afterward. RESULTS: In vitro: Medtronic Evercross balloons and modified Terumo Destination sheaths were the best combination: BSUs were inserted in 6 F sheaths with possible injections (for 6 and 8 mm balloons), and 7 F sheaths without injections (for the 10 mm balloon). Retrieving BSUs inside the sheath required 1 additional F-size. Boston Scientific Sterling and Balton Lovix balloons, as well as APT Braidin L guiding sheaths showed unsatisfactory performance. Dilation up to 23 mm was possible, and stent shortening was < 24% at 18 mm and < 37% at 23 mm. Recoil was limited, and no stent fractured. In vivo: Optimus-L stents were used to treat 2 infants with aortic coarctation and 2 children with pulmonary artery stenosis with the use of 8 mm balloons and low-profile access. CONCLUSIONS: Optimus-L stents can be implanted safely in small patients with a low-profile approach. These stents have the potential to achieve adult size while maintaining structural integrity.

Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

Mid-term results following pulmonary artery patch augmentation in congenital heart disease.

Background: Treatment of pulmonary artery (PA) stenosis in congenital heart disease is associated with adverse outcomes. The aim of this retrospective cohort study was to compare outcomes after surgical patch augmentation of PA stenosis in patients with biventricular congenital heart disease using different patch materials. Methods: We identified all patients from our institutional congenital heart disease database who underwent patch augmentation for PA stenosis on the main pulmonary artery (MPA) or PA branches between 2012 and 2018. Patch materials used were glutaraldehyde fixated autologous pericardium (AP), expanded polytetrafluoroethylene (ePTFE), equine pericardium (EP), and bovine pericardium (BP). The primary study endpoint was the composite of catheter-based re-intervention or re-operation to relieve recurrent stenosis at the site of prior implanted patch material. Results: A total of 156 patients (median age, 5 months, range, 0-85 months; median weight, 6.2 kg, range, 2.8-15.0 kg) underwent patch augmentation using 163 patches (ePTFE =99, 61%; EP =34, 21%; AP =25, 15%; BP =5, 3%). Overall, 131 (84%) patients underwent patch augmentation at the MPA, and 25 (16%) patients underwent patch augmentation at one or both PA branches. Over a mean follow-up period of 4±2 years, 30 patients (19%) reached the study endpoint. Freedom from primary endpoint was 92%±3% for the MPA and 25%±9% for PA branches at 5 years, respectively (P<0.001). Comparison of patch materials revealed similar re-intervention rates between ePTFE, AP, and EP. In contrast, outcomes were significantly decreased following the usage of BP when compared to other materials (ePTFE vs. BP, P=0.01; EP vs. BP, P=0.005). In the multivariable analysis, lower weight at index operation, patch augmentation of PA branches, and usage of BP were independently associated with re-intervention. Conclusions: Patch augmentation of the MPA was associated with acceptable outcomes, while patch augmentation of PA branch stenosis remained independently associated with re-intervention. None of the used patch materials demonstrated superiority; however, BP had a higher rate of re-interventions.

Catheter interventional closure of veno-venous collaterals in cyanotic patients after partial cavopulmonary shunts in pediatric patients: clinical practice review.

The development of veno-venous collaterals is an important and treatable cause of cyanosis in patients who had undergone partial cavo-pulmonary connection (PCPC) operations. Nevertheless, the literature on this complicated therapeutic option is sparse. Patients can present cyanosis either immediately after the operation (<30 days), which delays or hinders discharge from the intensive care unit or cyanosis may occur late: (>30 days and/or in another hospital admission), after the operation. Hence, transcatheter closure of veno-venous collaterals is the treatment of choice. Four patients were selected who showed cyanosis at variable durations after PCPC; the morphology of the collaterals and their hemodynamic effect was described and the strategy for closure of such abnormal vessels is suggested. Veno-venous collaterals described in our series were seen originating mainly or mostly from innominate vein angles. The drainage sites were either above the diaphragm into a cardiac structure: the coronary sinus (CS) and/or atria; or below the diaphragm into the inferior vena cava (IVC) or hepatic veins through the paravertebral venous system and/or the azygous system. It is stated in the literature that several types of devices and coils can be used to close the collaterals such as the Amplatzer vascular plugs (AVPs), Amplatzer duct occluder II (ADOII), non-detachable and detachable coils. In this clinical review, the technical details that determine device type and size are explained. The recent generations of hydrogel-coated coils were also used in this series of patients to close the difficult types of collaterals with better results. All described vessels were closed successfully, without any complications. The patients had a significant rise in their transcutaneous oxygen saturations and hence, a clear clinical benefit.

Umbilical catheter placement aided by coronary guidewires.

Catheterization of the umbilical vessels has proven to be an effective and relatively rapid method for gaining central vascular access in neonates. However, it can be technically difficult, the procedure may last 30 min or longer, and it can be associated with complications in some patients. We suggest using a coronary guidewire during catheterization of umbilical vessels to support the placement of umbilical catheters and significantly reduce a risk for complications. We tested the proposed technique in 6 successful ex vivo bench tests of catheterization of the umbilical vessels in stillborn piglets immediately after birth. We are confident that using coronary guidewire as a guiding tool during catheterization of the umbilical vessels is a rapid and safe method. We expect that it allows to obtain a vascular access with lower risk for dangerous procedural complications, which could be a lifesaving in critically ill patients. However, the approach needs to be validated in a comparative study in neonates.

Changes in pulmonary artery index and its relation to outcome after stage II palliation in patients with hypoplastic left heart syndrome.

OBJECTIVES: This study aimed to prove if pulmonary artery (PA) size influences survival and if an additional aortopulmonary shunt (APS) promotes left PA growth after bidirectional cavopulmonary shunt (BCPS) in patients with hypoplastic left heart syndrome. METHODS: The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and BCPS between 2007 and 2020 were reviewed. Right, left and total (right + left) PA indices were calculated according to Nakata and colleagues. RESULTS: A total of 158 patients were included in this study. The median age at Norwood and BCPS was 8 (7-11) days and 3.6 (3.1-4.6) months, respectively. There were 7 hospital deaths and 12 late deaths. Survival after BCPS was 90.3% at 1 year and 86.2% at 2 years. Total, right and left PA indices were 238 (195-316), 136 (101-185) and 102 (75-130) mm2/m2 at the time of BCPS, and they were 237 (198-284), 151 (123-186) and 86 (69-108) mm2/m2 at the time of Fontan. Left PA index decreased significantly between the time of BCPS and Fontan (P < 0.01). Nine patients needed partial takedown and additional APS due to failing BCPS, but the additional APS did not promote the PA growth significantly. CONCLUSIONS: Preoperative PA index did not affect the mortality after BCPS. The partial takedown and additional APS for failing BCPS were unable to improve left PA size.

Impact of Anatomical Sub-types and Shunt Types on Aortopulmonary Collaterals in Hypoplastic Left Heart Syndrome.

This study aims to clarify the relation of development of aortopulmonary collateral arteries (APCs) with anatomical sub-types and the shunt types at Norwood procedure in patients with hypoplastic left heart syndrome (HLHS). A total of 140 patients with HLHS who completed 3 staged palliation between 2003 and 2019 were included. Incidence of APCs and corresponding interventions were examined using angiogram by cardiac catheterization, with respect to the anatomical sub-types and shunt types. Totally, APCs were observed in 87 (62%) of the patients; pre-stage II in 32 (23%), pre-stage III in 64 (46%), and after stage III in 40 (29%). The incidence of APCs before stage II was significantly higher in patients with aortic atresia/mitral atresia (AA/MA) compared with other sub-types (P = 0.022). Patients with right ventricle to pulmonary artery conduit (RVPAC) had a higher incidence of APCs originating from the descending aorta, compared with those with modified Blalock-Taussig shunt (20% vs 2%, P= 0.002). Interventions for APCs were performed in 58 (41%) patients; before stage II in 10 (7%), after stage II in 7 (5%), before stage III in 22 (16%), and after stage III in 32 (23%). Patients with AA/MA had more interventions before stage II (P= 0.019), and patients with aortic stenosis/mitral stenosis (AS/MS) had a lower incidence of interventions after stage III (P= 0.047). More than half of the patients with HLHS developed APCs. Before stage II, patients with AA/MA sub-type had a higher incidence of APCs, and those with RVPAC had significantly more APCs from the descending aorta.

Aortopulmonary collaterals in single ventricle: incidence, associated factors and clinical significance.

OBJECTIVES: Clinical significance of aortopulmonary collaterals (APCs) in patients with univentricular heart remains controversial. This study aimed to evaluate the incidence and associated factors for APCs and their influence during staged palliation. METHODS: In total, 430 patients who underwent staged palliation by bidirectional Glenn shunt and total cavopulmonary connection between 2003 and 2019 were examined. APCs were determined by angiogram. Incidence and interventions for APCs were analysed. RESULTS: The most frequent diagnosis was hypoplastic left heart syndrome in 146 (34%) patients. The median age at Glenn and Fontan was 4.9 months and 2.1 years, respectively. APCs were observed in 54 (13%) patients at Glenn and in 179 (42%) at Fontan. Closure of APCs was performed before Glenn in 12 (3%) patients, at Glenn in 13 (3%), after Glenn in 8 (2%), before Fontan in 44 (10%), at Fontan in 26 (6%) and after Fontan in 52 (12%). Hypoplastic left heart syndrome (P < 0.01) was highly associated with the development of APCs before Glenn. Lower Nakata-Index and younger age at Glenn shunt were associated with the development of APCs at Fontan procedure. The presence of APCs or intervention for APCs before total cavopulmonary connection did not influence intensive care unit stay or mortality after total cavopulmonary connection. CONCLUSIONS: APCs were most frequently observed before Fontan procedure. Hypoplastic left heart syndrome was highly associated with the development of APCs before Glenn shunt. Lower Nakata-Index and younger age at Glenn shunt were associated with APCs before Fontan procedure.

Transcatheter implantation of covered stents serving as extravascular conduits-Proof of a CT-based approach in three cases.

BACKGROUND: Covered stents perform similar to surgically implanted conduits, although the stents work inside of vessels. We present a computed tomography (CT)-based workflow for the implantation of covered stents as extravascular conduits. METHODS: We selected three different use cases: 1. Connecting a left-sided partially anomalous drainage of a pulmonary vein to the left atrium. 2. Bypassing an outgrown Dacron conduit in aortic recoarctation. 3. Re-directing hepatic venous blood to the left lung in a Fontan patient with heterotaxy, connecting the innominate vein to the right pulmonary artery like a right-sided cavopulmonary connection. By postprocessing and analyzing CT scans for planning and by the use of long needles under biplane fluoroscopy for the realization of the procedure, we projected and performed the exit of a long needle out of a vessel, the re-entering of a target vessel, and the bridging of the extravascular distance by implantation of covered stents. RESULTS: In all three cases, the covered stents were placed successfully, connecting vessels of 15-50 mm distance from each other with very good hemodynamic results. In one case, two stents were placed consecutively, overlapping each other to accomplish an exact fitting at the connection sites to the native vessels.

Surgical reintervention on the neo-aorta after the Norwood operation.

OBJECTIVES: We sought to identify the prevalence of surgical reintervention on the neo-aorta after Norwood procedure and its impact on long-term outcomes. METHODS: We reviewed the medical records of all patients who underwent Norwood procedure. The impacts of surgical neoaortic reintervention on outcomes were analysed in each stage of palliation. RESULTS: A total of 335 patients were included in this study. Thirty patients underwent surgical reintervention on the neo-aorta after Norwood procedure. The timing of initial reintervention was before stage II in 13 patients, at stage II in 7, between stage II and stage III in 5, at stage III in 3 and after stage III in 2. A reintervention before stage II was significantly associated with mortality (HR 14.4, 95% confidence interval 6.00-34.6, P < 0.001). In patients who underwent stage II (n = 251), reintervention had no significant impact on mortality. In patients who underwent stage III (n = 188), the previous reintervention was significantly associated with higher mean pulmonary pressure (P = 0.05) and a higher rate of reduced ventricular function (P = 0.002). Greater than mild atrioventricular valve regurgitation was significantly associated with the development of a neoaortic arch stenosis after stage II (P = 0.03). CONCLUSIONS: Surgical reinterventions on the neo-aorta were required in each inter-stage phase. A surgical neoaortic reintervention was not related to increased mortality after stage II but significantly associated with a higher rate of reduced ventricular function and elevated mean pulmonary artery pressure.

Reintervention and Survival After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Transcatheter pulmonary valve (TPV) replacement (TPVR) has become the standard therapy for postoperative pulmonary outflow tract dysfunction in patients with a prosthetic conduit/valve, but there is limited information about risk factors for death or reintervention after this procedure. OBJECTIVES: This study sought to evaluate mid- and long-term outcomes after TPVR in a large multicenter cohort. METHODS: International registry focused on time-related outcomes after TPVR. RESULTS: Investigators submitted data for 2,476 patients who underwent TPVR and were followed up for 8,475 patient-years. A total of 95 patients died after TPVR, most commonly from heart failure (n = 24). The cumulative incidence of death was 8.9% (95% CI: 6.9%-11.5%) 8 years after TPVR. On multivariable analysis, age at TPVR (HR: 1.04 per year; 95% CI: 1.03-1.06 per year; P < 0.001), a prosthetic valve in other positions (HR: 2.1; 95% CI: 1.2-3.7; P = 0.014), and an existing transvenous pacemaker/implantable cardioverter-defibrillator (HR: 2.1; 95% CI: 1.3-3.4; P = 0.004) were associated with death. A total of 258 patients underwent TPV reintervention. At 8 years, the cumulative incidence of any TPV reintervention was 25.1% (95% CI: 21.8%-28.5%) and of surgical TPV reintervention was 14.4% (95% CI: 11.9%-17.2%). Risk factors for surgical reintervention included age (0.95 per year [95% CI: 0.93-0.97 per year]; P < 0.001), prior endocarditis (2.5 [95% CI: 1.4-4.3]; P = 0.001), TPVR into a stented bioprosthetic valve (1.7 [95% CI: 1.2-2.5]; P = 0.007), and postimplant gradient (1.4 per 10 mm Hg [95% CI: 1.2-1.7 per 10 mm Hg]: P < 0.001). CONCLUSIONS: These findings support the conclusion that survival and freedom from reintervention or surgery after TPVR are generally comparable to outcomes of surgical conduit/valve replacement across a wide age range.

Multicenter Study of Endocarditis After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Endocarditis has emerged as one of the most impactful adverse events after transcatheter pulmonary valve replacement (TPVR), but there is limited information about risk factors for and outcomes of this complication. OBJECTIVES: The purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort. METHODS: The authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes. RESULTS: Investigators submitted data for 2,476 patients who underwent TPVR between July 2005 and March 2020 and were followed for 8,475 patient-years. In total, 182 patients were diagnosed with endocarditis a median of 2.7 years after TPVR, for a cumulative incidence of 9.5% (95% CI: 7.9%-11.1%) at 5 years and 16.9% (95% CI: 14.2%-19.8%) at 8 years (accounting for competing risks: death, heart transplant, and explant) and an annualized incidence of 2.2 per 100 patient-years. Staphylococcus aureus and Viridans group Streptococcus species together accounted for 56% of cases. Multivariable analysis confirmed that younger age, a previous history of endocarditis, and a higher residual gradient were risk factors for endocarditis, but transcatheter pulmonary valve type was not. Overall, right ventricular outflow tract (RVOT) reintervention was less often to treat endocarditis than for other reasons, but valve explant was more often caused by endocarditis. Endocarditis was severe in 44% of patients, and 12 patients (6.6%) died, nearly all of whom were infected with Staphylococcus aureus. CONCLUSIONS: The incidence of endocarditis in this multicenter registry was constant over time and consistent with prior smaller studies. The findings of this study, along with ongoing efforts to understand and mitigate risk, will be critical to improve the lifetime management of patients with heart disease involving the RVOT. Although endocarditis can be a serious adverse outcome, TPVR remains an important tool in the management of RVOT dysfunction.

Successful percutaneous treatment with the Konar MF™-VSD Occluder in an infant with Abernethy syndrome-case report.

Cyanosis persisting after surgical repair of complex congenital heart disease (CHD) may be related to the underlying disease. However, extracardiac causes should be also considered. We report on a patient with heterotaxy syndrome and double outlet right ventricle, in whom postoperative cyanosis was associated with an Abernethy malformation type II causing a hepatopulmonary syndrome. Despite this complex anatomy, interventional closure of the portosystemic shunt was done with a Konar MF™-VSD Occluder. The patient recovered rapidly with relief of cyanosis within one month. This case highlights the importance of a careful diagnostic assessment in patients with complex CHD, who presents cyanoses after surgical repair. In addition, it shows the feasibility and safety of a percutaneous approach with complete closure of the vascular malformation in a patient with a complex anatomy.