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  • 1
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    Online Resource
    Modified approach for transcatheter correction of superior sinus venosus atrial septal defect: a case series
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 7, No. 1 ( 2022-12-27)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 7, No. 1 ( 2022-12-27)
    Abstract: Transcatheter correction of superior sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous drainage (PAPVD) of the right upper and/or right middle pulmonary vein (RUPV/RMPV) has recently been described as an alternative to surgical approach in a substantial number of patients. We describe a modified technical approach for transcatheter correction of SVASD using transoesophageal echocardiography (TOE) to confirm adequate stent landing zone and apposition and evaluate its feasibility, safety, and procedural success. Case summary From 2019 to 2021, three consecutive patients received a transcatheter correction of SVASD with PAPVD by redirecting the superior vena cava and RUPV/RMPV to the left atrium by implantation of a custom-made covered Cheatham platinum stent (10-zig, length: 60–80 mm). Prior to stent implantation, a balloon testing was performed in the anticipated landing zone using TOE to confirm complete defect closure and unobstructed pulmonary venous drainage. Stent deployment and flaring of the interior stent portion were performed with TOE guidance to confirm adequate landing zone and apposition and to avoid residual shunt or pulmonary vein obstruction. Conclusion Transcatheter correction of SVASD with PAPVD was performed without any complications. The follow-up period was 7.8, 13.6, and 29.8 months, respectively. During follow-up, no mortality, stent embolization, or obstruction of pulmonary venous drainage occurred. The TOE-guided modified transcatheter approach for correction of SVASD with PAPVD is safe and feasible with excellent post-procedural results and represents an alternative to surgical treatment in a pre-selected patient cohort.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytad030
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 2948381-5
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  • 2
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    Subcoronary Ross/Ross–Konno operation in children and young adults: initial single-centre experience
    Oxford University Press (OUP) ; 2021
    In:  European Journal of Cardio-Thoracic Surgery Vol. 59, No. 1 ( 2021-01-04), p. 226-233
    Details
    In: European Journal of Cardio-Thoracic Surgery, Oxford University Press (OUP), Vol. 59, No. 1 ( 2021-01-04), p. 226-233
    Abstract: We sought to evaluate the outcome after modified subcoronary Ross/Ross–Konno operation in children and young adults. METHODS Between January 2013 and January 2019, a total of 50 patients with median age of 6.3 years (range 0.02–36.5 years, 58% males), including 10 infants (20%), received modified subcoronary Ross/Ross–Konno operation at our institution. Survival, morbidity, reinterventions, aortic valve function and aortic root dimensions were analysed. RESULTS At a median follow-up of 31.2 months (range 14.4–51 months), there were 1 early death and 1 late death, both in the infant group. The overall survival at 5 years after the operation was 95%. Two patients needed aortic valve replacement, 11 and 15 months after their Ross operation. At 5 years, freedoms from reoperation on the autograft and on the right ventricle to pulmonary artery conduit were 94% and 97%, respectively. Freedom from aortic valve regurgitation greater than mild was 97% at 5 years. Median dimensions of the aortic root at all levels remained in normal range at last visit. Forty-four patients (95%) were in New York Heart Association class I with normal left ventricular function. CONCLUSIONS The initial experience with the subcoronary Ross/Ross–Konno operation in children and young adults showed excellent outcome. The mortality and morbidity among infants remain significant. The described technique is reproducible and might be advantageous in situations when prosthetic supporting techniques interfere with somatic growth.
    Type of Medium: Online Resource
    ISSN: 1010-7940 , 1873-734X
    URL: Article
    DOI: 10.1093/ejcts/ezaa307
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 1500330-9
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  • 3
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    Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics
    Springer Science and Business Media LLC ; 2020
    In:  Pediatric Cardiology Vol. 41, No. 4 ( 2020-04), p. 736-746
    Details
    In: Pediatric Cardiology, Springer Science and Business Media LLC, Vol. 41, No. 4 ( 2020-04), p. 736-746
    Abstract: Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest ® , n  = 101), liver ultrasound ( n  = 117) and transient elastography (FibroScan ® , n  = 61). The median patient age was 16.0 years [IQR 14.2], and the median duration of the Fontan circulation was 10.3 years [IQR 14.7] . Hemodynamic assessment was performed using echocardiography, cardiopulmonary exercise capacity testing and cardiac catheterization. Liver ultrasound revealed hepatic parenchymal changes in 83 patients (70.9%). Severe liver cirrhosis was detectable in 20 patients (17.1%). Median liver stiffness measured by FibroScan ® was 27.7 kPa [IQR 14.5], and the median Fibrotest ® score was 0.5 [IQR 0.3], corresponding to fibrosis stage ≥ 2. Liver stiffness values and Fibrotest ® scores correlated significantly with Fontan duration ( P 1  = 0.013, P 2  = 0.012). Exercise performance was significantly impaired in patients with severe liver cirrhosis ( P  = 0.003). Pulmonary artery pressure and end-diastolic pressure were highly elevated in cirrhotic patients ( P 1  = 0.008, P 2  = 0.003). Multivariable risk factor analysis revealed Fontan duration to be a major risk factor for the development of FALD ( P   〈  0.001, OR 0.77, CI 0.68–0.87). In the majority of patients, hepatic abnormalities suggestive of FALD were detectable by liver ultrasound, transient elastography and laboratory analysis. The severity of FALD correlated significantly with Fontan duration and impaired Fontan hemodynamics. A detailed hepatic assessment is indispensable for long-term surveillance of Fontan patients.
    Type of Medium: Online Resource
    ISSN: 0172-0643 , 1432-1971
    URL: Article
    DOI: 10.1007/s00246-020-02291-5
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2020
    detail.hit.zdb_id: 1463000-X
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  • 4
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    Image_3.pdf
    Frontiers Media SA ; 2022
    In:  Frontiers in Cardiovascular Medicine Vol. 9 ( 2022-3-10)
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    In: Frontiers in Cardiovascular Medicine, Frontiers Media SA, Vol. 9 ( 2022-3-10)
    Abstract: Despite the outstanding success of the Fontan operation, it is a palliative procedure and a substantial number of patients experience late failure of the Fontan circulation. Clinical presentation and hemodynamic phenotypes of Fontan failure are considerably variable. While various parameters have been identified as risk factors for late Fontan failure, a feasible score to classify Fontan failure and possibly allow timely risk stratification is lacking. Here, we explored the possibility of developing a score based on hemodynamic, clinical and laboratory parameters to classify Fontan failure and mortality. Methods We performed a retrospective study in our cohort of adult Fontan patients from two institutions [ n = 198, median follow-up after Fontan 20.3 (IQR 15.6–24.3) years], identifying those patients with clinical Fontan failure ( n = 52, 26.3%). Various hemodynamic, echocardiographic, laboratory and clinical data were recorded and differences between patients with and without Fontan failure were analyzed. We composed a Fontan Failure Score containing 15 parameters associated with Fontan failure and/or mortality and assessed its accuracy to discriminate between patients with and without late Fontan failure as well as late mortality and survival. Results Late failure occurred at a median of 18.2 (IQR 9.1–21.1) years after Fontan completion. Mortality associated with Fontan failure was substantial (25/52, 48.1%) with freedom of death/transplantation/take-down of 64% at 5 years and 36% at 10 years after onset of Fontan failure, respectively. Patients with Fontan failure had a significantly higher median Fontan Failure Score compared to non-failing Fontan patients [8 points (IQR 5–10) vs. 2 points (IQR 1-5), p & lt; 0.001]. The score accurately classifies Fontan failure as well as mortality as assessed with receiver operating characteristic analysis. Area under the curve of the Fontan Failure Score was 0.963 (95% CI 0.921; 0.985, p & lt; 0.001) to discriminate failure and 0.916 (95% CI 0.873; 0.959, p & lt; 0.001) to classify mortality. Conclusion We have developed an uncomplex yet remarkably accurate score to classify Fontan failure and late mortality in adult Fontan patients. Prospective validation and most likely refinement and calibration of the score in larger and preferably multi-institutional cohorts is required to assess its potential to predict the risk of Fontan failure and late mortality.
    Type of Medium: Online Resource
    ISSN: 2297-055X
    URL: Article
    URL: Article
    URL: Article
    URL: Article
    DOI: 10.3389/fcvm.2022.767503
    DOI: 10.3389/fcvm.2022.767503.s001
    DOI: 10.3389/fcvm.2022.767503.s002
    DOI: 10.3389/fcvm.2022.767503.s003
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2781496-8
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  • 5
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    Treatment strategies for protein-losing enteropathy in Fontan-palliated patients
    Cambridge University Press (CUP) ; 2020
    In:  Cardiology in the Young Vol. 30, No. 5 ( 2020-05), p. 698-709
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 30, No. 5 ( 2020-05), p. 698-709
    Abstract: Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality. Material and Methods: We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986–2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed. Results: Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001–0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival. Conclusions: Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951120000864
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2020
    detail.hit.zdb_id: 2060876-7
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  • 6
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    Online Resource
    Revascularization of Left Subclavian to Common Carotid Artery Prepares for Covered Stent Implantation in Patients With Complex Aortic Coarctation
    SAGE Publications ; 2022
    In:  Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery Vol. 17, No. 3 ( 2022-05), p. 237-243
    Details
    In: Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery, SAGE Publications, Vol. 17, No. 3 ( 2022-05), p. 237-243
    Abstract: Seven adult patients underwent a two-stage treatment of complex coarctation (CoA), including surgical revascularization of the left subclavian artery (LSA) to left common carotid artery (LCCA), followed by transcatheter covered stent implantation. The majority of patients (5 of 7, 71%) received 1 covered stent (covered Cheatham Platinum stent: 8 zig/45 mm [ n = 2], 10 zig/60 mm [ n = 1] , 10 zig/65 mm [ n = 1]; BeGraft: 24/48 mm [ n = 2] ). In 1 patient (14%), the implantation of 2 covered stents (BeGraft 20/48 mm) was necessary. During a median follow-up of 2.4 years (interquartile range, 0.1 to 4.9 years), complications occurred in 3 of 7 patients (43%), including an asymptomatic but severe stenosis of the LSA bypass ( n = 1), a recoarctation with a mild endoleak ( n = 1), and a severe endoleak ( n = 1). Surgical revascularization of the LSA to the LCCA can successfully prepare for covered stent implantation in complex CoA in adult patients. This two-stage approach was feasible and safe with complications occurring in 3 of 7 patients (43%). All complications were managed by catheter reintervention only.
    Type of Medium: Online Resource
    ISSN: 1556-9845 , 1559-0879
    URL: Article
    DOI: 10.1177/15569845221099298
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
    detail.hit.zdb_id: 2223439-1
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  • 7
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    Online Resource
    Can Left Atrioventricular Valve Reduction Index (LAVRI) Predict the Surgical Strategy for Repair of Atrioventricular Septal Defect?
    Springer Science and Business Media LLC ; 2021
    In:  Pediatric Cardiology Vol. 42, No. 4 ( 2021-04), p. 898-905
    Details
    In: Pediatric Cardiology, Springer Science and Business Media LLC, Vol. 42, No. 4 ( 2021-04), p. 898-905
    Abstract: Despite improved survival, surgical treatment of atrioventricular septal defect (AVSD) remains challenging. The optimal technique for primary left atrioventricular valve (LAVV) repair and prediction of suitability for biventricular approach in unbalanced AVSD are still controversial. We evaluated the ability of our recently developed echocardiographic left atrioventricular valve reduction index (LAVRI) in predicting LAVV reoperation rate and surgical strategy for unbalanced AVSD. Retrospective echocardiographic analysis was available in 352 of 790 patients with AVSD treated in our institution and included modified atrioventricular valve index (mAVVI), ventricular cavity ratio (VCR), and right ventricle/left ventricle (RV/LV) inflow angle. LAVRI estimates LAVV area after complete cleft closure and was analyzed with regard to surgical strategy in primary LAVV repair and unbalanced AVSD. Of the entire cohort, 284/352 (80.68%) patients underwent biventricular repair and 68/352 (19.31%) patients underwent univentricular palliation. LAVV reoperation was performed in 25/284 (8.80%) patients after surgical correction of AVSD. LAVRI was significantly lower in patients requiring LAVV reoperation (1.92 cm 2 /m 2 [IQR 1.31] vs. 2.89 cm 2 /m 2 [IQR 1.37], p  = 0.002) and significantly differed between patients receiving complete and no/partial cleft closure (2.89 cm 2 /m 2 [IQR 1.35] vs. 2.07 cm 2 /m 2 [IQR 1.69]; p  = 0.002). Of 82 patients diagnosed with unbalanced AVSD, 14 were suitable for biventricular repair (17.07%). mAVVI, LAVRI, VCR, and RV/LV inflow angle accurately distinguished between balanced and unbalanced AVSD and predicted surgical strategy (all p   〈  0.001). LAVRI may predict surgical strategy in primary LAVV repair, LAVV reoperation risk, and suitability for biventricular approach in unbalanced AVSD anatomy.
    Type of Medium: Online Resource
    ISSN: 0172-0643 , 1432-1971
    URL: Article
    DOI: 10.1007/s00246-021-02558-5
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2021
    detail.hit.zdb_id: 1463000-X
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  • 8
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    Coronary Interventions in Pediatric Congenital Heart Disease
    Springer Science and Business Media LLC ; 2022
    In:  Pediatric Cardiology Vol. 43, No. 4 ( 2022-04), p. 769-775
    Details
    In: Pediatric Cardiology, Springer Science and Business Media LLC, Vol. 43, No. 4 ( 2022-04), p. 769-775
    Abstract: Coronary artery lesions represent rare conditions in pediatric congenital heart disease and mainly include coronary artery stenoses (CAS) or coronary artery fistulae (CAF). Due to the small vessel size, pediatric percutaneous coronary interventions (PCI) are demanding and studies concerning long-term results are missing. In this retrospective study, we analyzed indications, procedural details, and post-procedural outcomes in pediatric patients who underwent PCI in our institution. For CAS treatment, procedural success was defined as efficient coronary revascularization with a significant improvement of coronary perfusion. CAF treatment was considered successful, when no residual shunt was detectable. From 1995 to 2020, 32 pediatric patients aged ≤ 18 years received interventional treatment for CAS ( n  = 24/32) or CAF ( n  = 8/32). Reasons for CAS were post-surgical ( n  = 15/24) or post-transplant ( n  = 9/24). Interventional treatment strategies included coronary angioplasty (20/43), stent placement (10/43), and a combination of both (13/43). In-hospital mortality occurred in 6/24 patients and late mortality in 5/24 patients leading to an overall 5-year survival of 62.5%. Early mortality mainly occurred due to post-ischemic myocardial failure. CAF occlusion was performed using coil embolization ( n  = 3), placement of vascular plugs ( n  = 3), a combination of both ( n  = 1), or a combination of coil embolization and a covered stent ( n  = 1). Treatment of coronary fistulae was successful in all patients with excellent post-procedural results and no follow-up death. PCI in pediatric patients with congenital heart disease can be performed safely and effectively. However, the overall 5-year survival probability of patients with CAS is reduced due to severe ischemic myocardial damage.
    Type of Medium: Online Resource
    ISSN: 0172-0643 , 1432-1971
    URL: Article
    DOI: 10.1007/s00246-021-02784-x
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
    detail.hit.zdb_id: 1463000-X
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  • 9
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    Online Resource
    Functional hepatic deterioration determined by 13C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults
    Frontiers Media SA ; 2022
    In:  Frontiers in Cardiovascular Medicine Vol. 9 ( 2022-9-7)
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    In: Frontiers in Cardiovascular Medicine, Frontiers Media SA, Vol. 9 ( 2022-9-7)
    Abstract: Despite improved survival a substantial number of Fontan patients eventually develop late failure. Fontan-associated liver disease (FALD) is the most frequent end-organ dysfunction. Although impaired hemodynamics and Fontan failure correlate with FALD severity, no association between hepatic functional metabolic impairment and Fontan hemodynamics has been established. Hypothesis Metabolic liver function measured by liver maximum function capacity test (LiMAx®) correlates with Fontan hemodynamics and Fontan failure. Methods From 2020 to 2022, 58 adult Fontan patients [median age: 29.3 years, IQR (12.7), median follow-up time after Fontan operation: 23.2 years, IQR (8.7)] were analyzed in a cross-sectional study. Hemodynamic assessment included echocardiography, cardiopulmonary exercise testing and invasive hemodynamic evaluation. Fontan failure was defined based on commonly applied clinical criteria and our recently composed multimodal Fontan failure score. Results LiMAx® test revealed normal maximum liver function capacity in 40 patients ( & gt;315 μg/h * kg). In 18 patients a mild to moderate impairment was detected (140–314 μg/h * kg), no patient suffered from severe hepatic deterioration (≤ 139 μg/kg * h). Fontan failure was present in 15 patients. Metabolic liver function was significantly reduced in patients with increased pulmonary artery pressure ( p = 0.041. r = −0.269) and ventricular end-diastolic pressure ( p = 0.033, r = −0.325), respectively. In addition, maximum liver function capacity was significantly impaired in patients with late Fontan failure (289.0 ± 99.6 μg/kg * h vs. 384.5 ± 128.6 μg/kg * h, p = 0.007). Conclusion Maximum liver function capacity as determined by LiMAx® was significantly reduced in patients with late Fontan failure. In addition, elevated pulmonary artery pressure and end-diastolic ventricular pressure were associated with hepatic functional metabolic impairment.
    Type of Medium: Online Resource
    ISSN: 2297-055X
    URL: Article
    DOI: 10.3389/fcvm.2022.952080
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2781496-8
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  • 10
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    Online Resource
    Long-term results after surgical repair of atrioventricular septal defect
    Oxford University Press (OUP) ; 2019
    In:  Interactive CardioVascular and Thoracic Surgery Vol. 28, No. 5 ( 2019-05-01), p. 789-796
    Details
    In: Interactive CardioVascular and Thoracic Surgery, Oxford University Press (OUP), Vol. 28, No. 5 ( 2019-05-01), p. 789-796
    Type of Medium: Online Resource
    ISSN: 1569-9285
    URL: Article
    DOI: 10.1093/icvts/ivy334
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2019
    detail.hit.zdb_id: 2096257-5
    detail.hit.zdb_id: 3167862-2
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