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3. Edoxaban for Thromboembolism Prevention in Pediatric Patients With Cardiac Disease

Author

Brothers, Matthew, Marino, Bradley, Canter, Charles, Law, Mark, Nguyen, Nguyenvu, Sang, Charlie, Shimano, Kristin, Gupta, Dipankar, Portman, Michael, Williams, Derek, Glass, Lauren, Sperrazza, Charles, Herold, Steven, Garg, Ruchira, Vranicar, Mark, Awad, Sawsan, Asante-Korang, Alfred, Druzgal, Colleen, Ozment, Caroline, Del Toro, Kamill, Roses, Ferran, Jux, Christian, Gravenhorst, Verena, Schweigmann, Ulrich, Bhatt, Mihir, Sabapathy, Christine, Dahdah, Nagib, Bartonicek, Dototea, Tulzer, Gerald, Basargina, Elena, Zvereva, Tatiana, Pertels, Tatiana, Plotnikova, Irina, Pierre-Emmanuel, S.E.G.U.E.L.A., Amedro, Pascal, Yves, Dulac, BONNET, Damien, Saraco, Paola, Rimini, Alessandro, Digtiar, Valerii, Gonchar, Margaryta, Kryuchko, Tetyana, Yablon, Olga, Bedi, Varinder Singh, Patel, Jashvant, Mitra, Monjori, Kusa, Jacek, Domagala, Kowalczyk, KÖRNYEI, László, BERECZKI, Csaba, ABLONCZY, László, Levitas, Vivianne Aviva, Mishali, David, Revel-Vilk, Shoshana, Harlev, Dan, Sasmaz, Hatice Ilgen, Ozbek, Namik Yasar, Unal, Sule, Patıroglu, Türkan, Malbora, Baris, Agin, Hasan, Karakas, Zeynep, Kavakli, Ramazan Kaan, Chalmers, Elizabeth, Bu'Lock, Frances, Daubeney, Piers, Hamza, Hala, Badr, Mohamed, Elalfy, Mohsen, Mansour, Ahmed, Hassab, Hoda, Sabry, Ayman, Daou, Linda, Bitar, Fadi, Portman, Michael A., Jacobs, Jeffrey P., Newburger, Jane W., Berger, Felix, Grosso, Michael A., Duggal, Anil, Tao, Ben, and Goldenberg, Neil A.

Published
2022
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4. Histopathology of congestive nephropathy: a case description and literature review.

Author

Husain‐Syed, Faeq, Rangaswami, Janani, Núñez, Julio, Skrzypek, Susanne, Jux, Christian, Gröne, Hermann‐Josef, and Birk, Horst‐Walter

Subjects
CARDIO-renal syndrome, ACUTE kidney failure, LITERATURE reviews, HEART failure, CELL differentiation, KIDNEY diseases, INTRA-abdominal hypertension
Abstract

Congestive nephropathy is an underappreciated manifestation of cardiorenal syndrome and is characterized by a potentially reversible kidney dysfunction caused by a reduced renal venous outflow secondary to right‐sided heart failure or intra‐abdominal hypertension. To date, the histological diagnostic criteria for congestive nephropathy have not been defined. We herein report a case of acute renal dysfunction following cardiac allograft failure and present a review of the relevant literature to elucidate the current understanding of the disease. Our case demonstrated that congestion‐driven nephropathy may be histopathologically characterized by markedly dilated veins and peritubular capillaries, focally accentuated low‐grade acute tubular damage, small areas of interstitial fibrosis, and tubular atrophy on a background of normal glomeruli and predominantly normal tubular cell differentiation. [ABSTRACT FROM AUTHOR]

Published
2024
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8. Left Ventricular Physiology and Ventricular‐Vascular Interactions in Young Patients After Heart Transplantation

Author

Latus, Heiner, primary, Raap, Ramona, additional, Klingel, Karin, additional, Happel, Christoph, additional, Moysich, Axel, additional, Khalil, Markus, additional, Kerst, Gunter, additional, Milla, Jakob, additional, Skrzypek, Susanne, additional, Thul, Josef, additional, Jux, Christian, additional, Schranz, Dietmar, additional, and Apitz, Christian, additional

Published
2023
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10. Edoxaban for Thromboembolism Prevention in Pediatric Patients With Cardiac Disease

Author

Portman, Michael A., primary, Jacobs, Jeffrey P., additional, Newburger, Jane W., additional, Berger, Felix, additional, Grosso, Michael A., additional, Duggal, Anil, additional, Tao, Ben, additional, Goldenberg, Neil A., additional, Brothers, Matthew, additional, Marino, Bradley, additional, Canter, Charles, additional, Law, Mark, additional, Nguyen, Nguyenvu, additional, Sang, Charlie, additional, Shimano, Kristin, additional, Gupta, Dipankar, additional, Portman, Michael, additional, Williams, Derek, additional, Glass, Lauren, additional, Sperrazza, Charles, additional, Herold, Steven, additional, Garg, Ruchira, additional, Vranicar, Mark, additional, Awad, Sawsan, additional, Asante-Korang, Alfred, additional, Druzgal, Colleen, additional, Ozment, Caroline, additional, Del Toro, Kamill, additional, Roses, Ferran, additional, Jux, Christian, additional, Gravenhorst, Verena, additional, Schweigmann, Ulrich, additional, Bhatt, Mihir, additional, Sabapathy, Christine, additional, Dahdah, Nagib, additional, Bartonicek, Dototea, additional, Tulzer, Gerald, additional, Basargina, Elena, additional, Zvereva, Tatiana, additional, Pertels, Tatiana, additional, Plotnikova, Irina, additional, Pierre-Emmanuel, S.E.G.U.E.L.A., additional, Amedro, Pascal, additional, Yves, Dulac, additional, BONNET, Damien, additional, Saraco, Paola, additional, Rimini, Alessandro, additional, Digtiar, Valerii, additional, Gonchar, Margaryta, additional, Kryuchko, Tetyana, additional, Yablon, Olga, additional, Bedi, Varinder Singh, additional, Patel, Jashvant, additional, Mitra, Monjori, additional, Kusa, Jacek, additional, Domagala, Kowalczyk, additional, KÖRNYEI, László, additional, BERECZKI, Csaba, additional, ABLONCZY, László, additional, Levitas, Vivianne Aviva, additional, Mishali, David, additional, Revel-Vilk, Shoshana, additional, Harlev, Dan, additional, Sasmaz, Hatice Ilgen, additional, Ozbek, Namik Yasar, additional, Unal, Sule, additional, Patıroglu, Türkan, additional, Malbora, Baris, additional, Agin, Hasan, additional, Karakas, Zeynep, additional, Kavakli, Ramazan Kaan, additional, Chalmers, Elizabeth, additional, Bu'Lock, Frances, additional, Daubeney, Piers, additional, Hamza, Hala, additional, Badr, Mohamed, additional, Elalfy, Mohsen, additional, Mansour, Ahmed, additional, Hassab, Hoda, additional, Sabry, Ayman, additional, Daou, Linda, additional, and Bitar, Fadi, additional

Published
2022
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11. Inhibition of mitochondrial respiration has fundamentally different effects on proliferation, cell survival and stress response in immature versus differentiated cardiomyocyte cell lines

Author

Grün, Bent, Tirre, Michaela, Pyschny, Simon, Singh, Vijay, Kehl, Hans-Gerd, Jux, Christian, and Drenckhahn, Jörg-Detlef

Subjects
Cell Biology, Developmental Biology
Abstract

Myocardial tissue homeostasis is critically important for heart development, growth and function throughout the life course. The loss of cardiomyocytes under pathological conditions ultimately leads to cardiovascular disease due to the limited regenerative capacity of the postnatal mammalian heart. Inhibition of electron transport along the mitochondrial respiratory chain causes cellular stress characterized by ATP depletion as well as excessive generation of reactive oxygen species. Adult cardiomyocytes are highly susceptible to mitochondrial dysfunction whereas embryonic cardiomyocytes in the mouse heart have been shown to be resistant towards mitochondrial complex III inhibition. To functionally characterize the molecular mechanisms mediating this stress tolerance, we used H9c2 cells as an in vitro model for immature cardiomyoblasts and treated them with various inhibitors of mitochondrial respiration. The complex I inhibitor rotenone rapidly induced cell cycle arrest and apoptosis whereas the complex III inhibitor antimycin A (AMA) had no effect on proliferation and only mildly increased cell death. HL-1 cells, a differentiated and contractile cardiomyocyte cell line from mouse atrium, were highly susceptible to AMA treatment evident by cell cycle arrest and death. AMA induced various stress response mechanisms in H9c2 cells, such as the mitochondrial unfolded protein response (UPRmt), integrated stress response (ISR), heat shock response (HSR) and antioxidative defense. Inhibition of the UPR, ISR and HSR by siRNA mediated knock down of key components does not impair growth of H9c2 cells upon AMA treatment. In contrast, knock down of NRF2, an important transcriptional regulator of genes involved in detoxification of reactive oxygen species, reduces growth of H9c2 cells upon AMA treatment. Various approaches to activate cell protective mechanisms and alleviate oxidative stress in HL-1 cells failed to rescue them from AMA induced growth arrest and death. In summary, these data show that the site of electron transport interruption along the mitochondrial respiratory chain determines cell fate in immature cardiomyoblasts. The study furthermore points to fundamental differences in stress tolerance and cell survival between immature and differentiated cardiomyocytes which may underlie the growth plasticity of embryonic cardiomyocytes during heart development but also highlight the obstacles of cardioprotective therapies in the adult heart.

Published
2022

12. Impact of Right Ventricular Pressure Load After Repair of Tetralogy of Fallot

Author

Latus, Heiner, primary, Stammermann, Jana, additional, Voges, Inga, additional, Waschulzik, Birgit, additional, Gutberlet, Matthias, additional, Diller, Gerhard‐Paul, additional, Schranz, Dietmar, additional, Ewert, Peter, additional, Beerbaum, Philipp, additional, Kühne, Titus, additional, Sarikouch, Samir, additional, Kerst, Gunter, additional, Kanaan, Majed, additional, Lebherz, Corinna, additional, Rüffer, André, additional, Gkalpakiotis, Dimitrios, additional, Schedifka, Andrea, additional, Buheitel, Gernot, additional, Streble, Joachim, additional, Willing, Rainer, additional, Schubert, Stephan, additional, Laser, Kai Thorsten, additional, Sandica, Eugen, additional, Trusen, Burkhard, additional, Berger, Felix, additional, Miera, Oliver, additional, Ovroutski, Stanislav, additional, Schmitt, Katharina, additional, Photiadis, Joachim, additional, Opgen‐Rhein, Bernd, additional, Weiss, Katja, additional, Klaassen, Sabine, additional, Berns, Christoph, additional, Boeckel, Thomas, additional, Haverkämper, Guido, additional, Kästner, Andreas, additional, Koch, Heike, additional, Peters, Björn, additional, Schmidt, Florian, additional, Timme, Jens, additional, Engel, Konstanze, additional, Franzbach, Birgit, additional, Senft, Gabriela, additional, Beyer, Frank, additional, Winter, Klaus, additional, Breuer, Johannes, additional, Schneider, Martin, additional, Bahlmann, Jens, additional, Griese, Eberhard, additional, Lê, Trong Phi, additional, Hebe, Joachim, additional, Nürnberg, Jan‐Hendrik, additional, Magsaam, Annette, additional, Müller, Ronald, additional, Potthoff, Ludger, additional, Voigt, Renate, additional, Krüger, Tim, additional, Gerleve, Hubert, additional, Kleideiter, Ulrich, additional, Schneider‐Kulla, Dirk, additional, Krülls‐Münch, Jürgen, additional, Menke, Thomas, additional, Lehn, Martin, additional, Heilmann, Antje, additional, Tomczak, Helge, additional, Tarusinov, Gleb, additional, Scheid, Michael, additional, Mayatepek, Ertan, additional, Pillekamp, Frank, additional, Lichtenberg, Artur, additional, Terpeluk, Christiane, additional, Kolterer, Bruno, additional, Dittrich, Sven, additional, Gundlach, Ulrike, additional, Cesnjevar, Robert, additional, Müntjes, Carsten, additional, Morf, Geert, additional, Esmaeili, Anoosh, additional, Backhoff, Stephan, additional, Stiller, Brigitte, additional, Beyersdorf, Friedhelm, additional, Kroll, Johannes, additional, Häffner, Nicole, additional, Siaplaouras, Jannos, additional, Masri‐Zada, Antje, additional, Jux, Christian, additional, Böning, Andreas, additional, Akintürk, Hakan, additional, Paul, Thomas, additional, Sigler, Matthias, additional, Tirilomis, Theodor, additional, Schürer, Gabriele, additional, Hartmann, Johannes, additional, Grabitz, Ralph, additional, Liebaug, Uta, additional, Rotzsch, Claudius, additional, Kozlik‐Feldmann, Rainer, additional, Rickers, Carsten, additional, Mir, Thomas, additional, Hübler, Michael, additional, Sachweh, Jörg, additional, Renz, Stefan, additional, Schemm, Andreas, additional, Friedrich, Bernd, additional, Schlobohm, Otmar, additional, Böthig, Dietmar, additional, Wermter, Burkhard, additional, Kelter‐Klöpping, Andrea, additional, Horke, Alexander, additional, Bauersachs, Johann, additional, Westhoff‐Bleck, Mechthild, additional, Gorenflo, Matthias, additional, Karck, Matthias, additional, Loukanov, Tsvetomir, additional, Schrüfer, Hermann, additional, Wilken, Martin, additional, Abdul‐Khaliq, Hashim, additional, Rädle‐Hurst, Tanja, additional, Rentzsch, Axel, additional, Schäfers, Hans‐Joachim, additional, Reichert, Hagen, additional, Vilser, Daniel, additional, Kriebel, Thomas, additional, Boysen, Arnulf, additional, Uebing, Anselm, additional, Attmann, Tim, additional, Cremer, Joachim Thomas, additional, Scheewe, Jens, additional, Buchholz‐Berdau, Regina, additional, Möller, Peter, additional, Horter, Thorsten, additional, Brockmeier, Konrad, additional, Bennink, Gerardus B. W. E, additional, Baldus, Stephan, additional, Gillor, Alex, additional, Niehues, Tim, additional, Lawrenz, Wolfgang, additional, Leidig, Steffen, additional, Dähnert, Ingo, additional, Riede, Frank‐Thomas, additional, Kostelka, Martin, additional, Kändler, Liane, additional, Bethge, Martin, additional, Köster, Stefan, additional, Schröder, Christoph, additional, Karstedt, Jens, additional, Seitz, Uwe, additional, Kampmann, Christoph, additional, Dohle, Daniel‐Sebastian, additional, Stahl, Frank, additional, Abedini, Mojtaba, additional, Müller‐Scholden, Joachim, additional, Hager, Alfred, additional, Huntgeburth, Michael, additional, Kaemmerer, Harald, additional, Nagdyman, Nicole, additional, Schoetzau, Jörg, additional, Tutarel, Oktay, additional, Lange, Rüdiger, additional, Hörer, Jürgen, additional, Haas, Nikolaus A, additional, Hauser, Michael, additional, Roithmaier, Alexander, additional, Kehl, Hans‐Gerd, additional, Lammers, Astrid, additional, Malec, Edward, additional, Baumgartner, Helmut, additional, Diller, Gerhard, additional, Bahle, Roswitha, additional, Hofner, Gerald, additional, Zink, Stefan, additional, Reif, Roland, additional, Singer, Helmut, additional, Parlasca, Christoph, additional, Freund, Matthias W, additional, Schumacher, Michael, additional, Dewald, Oliver, additional, Darrelmann, Christine, additional, Willmann, Olaf, additional, Schmiedl, Norbert, additional, Quick, Peter, additional, Hillebrand, Dirk, additional, Michele Eiselt, Stephan, additional, Nekarda, Torsten, additional, Eberhard, Michael, additional, Baier, Georg, additional, Uhlemann, Frank, additional, Tzanavaros, Ioannis, additional, Beyer, Alexander, additional, Binz, Gudrun, additional, Hess, Steffen, additional, Teufel, Thomas, additional, Handke, Ronald‐Peter, additional, Hofbeck, Michael, additional, Kaulitz, Renate, additional, Sieverding, Ludger, additional, Schlensak, Christian, additional, Apitz, Christian, additional, Kaestner, Michael, additional, Holtvogt, Jürgen, additional, Wippermann, Carl‐Friedrich, additional, Hinz, Sönke, additional, Heusch, Andreas, additional, Wirbelauer, Johannes, additional, and Brosi, Wolfgang, additional

Published
2022
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13. Exercise-dependent changes in ventricular–arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study.

Author

Latus, Heiner, Hofmann, Lucas, Gummel, Kerstin, Khalil, Markus, Yerebakan, Can, Waschulzik, Birgit, Schranz, Dietmar, Voges, Inga, Jux, Christian, and Reich, Bettina

Subjects
PULMONARY circulation, VENA cava superior, COLLATERAL circulation, MAGNETIC resonance imaging, EXERCISE, RESEARCH funding, EMPLOYEES' workload, AORTA, CARDIOPULMONARY bypass, ERGOMETRY, VENA cava inferior, HEMODYNAMICS, HEART failure
Abstract

Aims Inefficient ventricular–arterial (V–A) coupling has been described in Fontan patients and may result in adverse haemodynamics. A varying amount of aortopulmonary collateral (APC) flow is also frequently present that increases volume load of the single ventricle. The aim of the study was to assess changes in V–A coupling and APC flow during exercise CMR. Methods and results Eighteen Fontan patients (age 24 ± 3 years) and 14 controls (age 23 ± 4 years) underwent exercise CMR using a cycle ergometer. Ventricular volumetry and flow measurements in the ascending aorta (AAO), inferior (IVC), and superior (SVC) vena cava were assessed using real-time sequences during stepwise increases in work load. Measures of systemic arterial elastance Ea, ventricular elastance Ees, and V–A coupling (Ea/Ees) were assessed. APC flow was quantified as AAO − (SVC + IVC). Ea remained unchanged during all levels of exercise in both groups (P = 0.39 and P = 0.11). Ees increased in both groups (P = 0.001 and P < 0.001) with exercise but was lower in the Fontan group (P = 0.04). V–A coupling was impaired in Fontan patients at baseline (P = 0.04). Despite improvement during exercise (P = 0.002) V–A coupling remained impaired compared with controls (P = 0.001). Absolute APC flow in Fontan patients did not change during exercise even at maximum work load (P = 0.98). Conclusions Inefficient V–A coupling was already present at rest in Fontan patients and aggravated during exercise due to a limited increase in ventricular contractility which demonstrates the importance of a limited functional reserve of the single ventricle. APC flow remained unchanged suggesting no further increase in volume load during exercise. [ABSTRACT FROM AUTHOR]

Published
2023
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17. Cardiomyocyte Hyperplasia and Immaturity but not Hypertrophy are Characteristic Features of Patients with Rasopathies

Author

Drenckhahn, Jörg-Detlef, primary, Nicin, Luka, additional, Akhouaji, Sara, additional, Krück, Svenja, additional, Blank, Anna Eva, additional, Schänzer, Anne, additional, Yörüker, Uygar, additional, Jux, Christian, additional, Abplanalp, Wesley, additional, John, David, additional, Zeiher, Andreas, additional, Dimmeler, Stefanie, additional, and Rupp, Stefan, additional

Published
2022
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18. Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry

Author

Kaemmerer, Ann-Sophie, primary, Gorenflo, Matthias, additional, Huscher, Dörte, additional, Pittrow, David, additional, Ewert, Peter, additional, Pausch, Christine, additional, Delcroix, Marion, additional, Ghofrani, Hossein A., additional, Hoeper, Marius M., additional, Kozlik-Feldmann, Rainer, additional, Skride, Andris, additional, Stähler, Gerd, additional, Vizza, Carmine Dario, additional, Jureviciene, Elena, additional, Jancauskaite, Dovile, additional, Gumbiene, Lina, additional, Ewert, Ralf, additional, Dähnert, Ingo, additional, Held, Matthias, additional, Halank, Michael, additional, Skowasch, Dirk, additional, Klose, Hans, additional, Wilkens, Heinrike, additional, Milger, Katrin, additional, Jux, Christian, additional, Koestenberger, Martin, additional, Scelsi, Laura, additional, Brunnemer, Eva, additional, Hofbeck, Michael, additional, Ulrich, Silvia, additional, Noordegraaf, Anton Vonk, additional, Lange, Tobias J., additional, Bruch, Leonhard, additional, Konstantinides, Stavros, additional, Claussen, Martin, additional, Löffler-Ragg, Judith, additional, Wirtz, Hubert, additional, Apitz, Christian, additional, Neidenbach, Rhoia, additional, Freilinger, Sebastian, additional, Nemes, Attila, additional, Opitz, Christian, additional, Grünig, Ekkehard, additional, and Rosenkranz, Stephan, additional

Published
2021
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21. Fetal Echocardiography in Predicting Postnatal Outcome in Borderline Left Ventricle.

Author

Vorisek CN, Bischofsberger L, Kurkevych A, Yürökür U, Wolter A, Gembruch U, Berg C, Hudel H, Thul J, Jux C, Akintürk H, Schranz D, and Axt-Fliedner R

Subjects
Pregnancy, Female, Humans, Retrospective Studies, Prospective Studies, ROC Curve, Ultrasonography, Prenatal methods, Heart Ventricles, Echocardiography methods
Abstract

Objectives:  Prenatal prediction of postnatal univentricular versus biventricular circulation in patients with borderline left ventricle (bLV) remains challenging. This study investigated prenatal fetal echocardiographic parameters and postnatal outcome of patients with a prenatally diagnosed bLV., Methods:  We report a retrospective study of bLV patients at four prenatal centers with a follow-up of one year. BLV was defined as z-scores of the left ventricle (LV) between -2 and -4. Single-ventricle palliation (SVP), biventricular repair (BVR), and no surgical or catheter-based intervention served as the dependent outcome. Prenatal ultrasound parameters were used as independent variables. Cut-off values from receiver operating characteristic curves (ROC) were determined for significant discrimination between outcomes., Results:  A total of 54 patients were diagnosed with bLV from 2010 to 2018. All were live births. Out of the entire cohort, 8 (15 %) received SVP, 34 (63 %) BVR, and 12 (22 %) no intervention. There was no significant difference with regard to genetic or extracardiac anomalies. There were significantly more patients with endocardial fibroelastosis (EFE) in the SVP group compared to the BVR group (80 % vs. 10 %), (p < 0.001). Apex-forming LV (100 % vs. 70 %) and lack of retrograde arch flow (20 % vs. 80 %) were associated with no intervention (p < 0.001). With respect to BVR vs. SVP, the LV sphericity index provided the highest specificity (91.7 %) using a cutoff value of ≤ 0.5., Conclusion:  The majority of bLV patients maintained biventricular circulation. EFE, retrograde arch flow, and LV sphericity can be helpful parameters for counseling parents and further prospective studies can be developed., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Published
2023
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22. Exercise-dependent changes in ventricular-arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study.

Author

Latus H, Hofmann L, Gummel K, Khalil M, Yerebakan C, Waschulzik B, Schranz D, Voges I, Jux C, and Reich B

Subjects
Humans, Young Adult, Adult, Arteries, Heart Ventricles, Hemodynamics, Aorta, Fontan Procedure
Abstract

Aims: Inefficient ventricular-arterial (V-A) coupling has been described in Fontan patients and may result in adverse haemodynamics. A varying amount of aortopulmonary collateral (APC) flow is also frequently present that increases volume load of the single ventricle. The aim of the study was to assess changes in V-A coupling and APC flow during exercise CMR., Methods and Results: Eighteen Fontan patients (age 24 ± 3 years) and 14 controls (age 23 ± 4 years) underwent exercise CMR using a cycle ergometer. Ventricular volumetry and flow measurements in the ascending aorta (AAO), inferior (IVC), and superior (SVC) vena cava were assessed using real-time sequences during stepwise increases in work load. Measures of systemic arterial elastance Ea, ventricular elastance Ees, and V-A coupling (Ea/Ees) were assessed. APC flow was quantified as AAO - (SVC + IVC). Ea remained unchanged during all levels of exercise in both groups (P = 0.39 and P = 0.11). Ees increased in both groups (P = 0.001 and P < 0.001) with exercise but was lower in the Fontan group (P = 0.04). V-A coupling was impaired in Fontan patients at baseline (P = 0.04). Despite improvement during exercise (P = 0.002) V-A coupling remained impaired compared with controls (P = 0.001). Absolute APC flow in Fontan patients did not change during exercise even at maximum work load (P = 0.98)., Conclusions: Inefficient V-A coupling was already present at rest in Fontan patients and aggravated during exercise due to a limited increase in ventricular contractility which demonstrates the importance of a limited functional reserve of the single ventricle. APC flow remained unchanged suggesting no further increase in volume load during exercise., Competing Interests: Conflict of interest: none declared., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Published
2022
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