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Related Research Units

Cardiac Surgery Research

Research Overview

Ingeborg Friehs, MD, studies congenital heart disease, with a focus on endocardial fibroelastosis (EFE) and hypoplastic left heart syndrome. Her research examines how abnormal blood flow and endothelial-to-mesenchymal transition contribute to fibroelastic remodeling and impaired heart function. She also investigates translational approaches to cardiac injury and disease, including mitochondrial transplantation. 

Research Background

Ingeborg Friehs, MD, is a Senior Scientist in the Department of Cardiac Surgery at Boston Children’s Hospital and an Instructor in Surgery at Harvard Medical School. A trained general and cardiovascular surgeon, her academic career spans more than two decades in Europe and the United States. Her work bridges clinical and basic research, and she has taught and mentored learners from undergraduate through postgraduate training

Publications

  1. Imatinib Mesylate and Sirolimus Inhibit Vascular Fibroproliferative Remodeling in Pulmonary Vein Stenosis. Children (Basel). 2026 Sep 21; 13(9). View Abstract
  2. Spatial and Single-Nucleus Multiomics Profiling Reveals Heterogeneous Endothelial Cell Overabundance in Endocardial Fibroelastosis. Circulation. 2026 Aug 25; 154(8):771-774. View Abstract
  3. Fibroelastic Remodelling of the Endocardium on the Right Side of the Heart: Endothelial-to-Mesenchymal Transition in Pulmonary Atresia With Intact Ventricular Septum. Eur J Cardiothorac Surg. 2026 04 01; 68(4). View Abstract
  4. Preclinical Assessment of Atorvastatin for Treatment of Endocardial Fibroelastosis. JACC Basic Transl Sci. 2025 Sep; 10(9):101282. View Abstract
  5. Eliminating left ventricular outlet stenosis lowers the risk for endocardial fibroelastosis recurrence†. Eur J Cardiothorac Surg. 2025 07 01; 67(7). View Abstract
  6. Quantifying the impact of mitral valve anatomy on clinical markers using surrogate models and sensitivity analysis. Comput Biol Med. 2025 Jun; 192(Pt B):110265. View Abstract
  7. Smooth muscle Cxcl12 contributions to vascular remodeling in flow and hypoxia-induced pulmonary hypertension. J Biol Chem. 2025 06; 301(6):110207. View Abstract
  8. Intrinsic cardiomyopathy in pediatric Marfan syndrome: predictive factors and risk assessments. Pediatr Res. 2025 Oct; 98(4):1467-1473. View Abstract
  9. Smooth muscle Cxcl12 activation is associated with vascular remodeling in flow-induced pulmonary hypertension. bioRxiv. 2024 Sep 10. View Abstract
  10. Predictive modeling of endocardial fibroelastosis recurrence in patients with congenital heart disease. J Thorac Cardiovasc Surg. 2025 02; 169(2):366-374. View Abstract
  11. Intravenous Thrombolysis Before Thrombectomy Improves Functional Outcome After Stroke Independent of Reperfusion Grade. J Am Heart Assoc. 2024 03 19; 13(6):e031854. View Abstract
  12. A Neonatal Heterotopic Rat Heart Transplantation Model for the Study of Endothelial-to-Mesenchymal Transition. J Vis Exp. 2023 07 21; (197). View Abstract
  13. Case report: Active clinical manifestation of endocardial fibroelastosis in adolescence in a patient with mitral and aortic obstruction-histologic presence of endothelial-to-mesenchymal transformation. Front Cardiovasc Med. 2022; 9:1041039. View Abstract
  14. Postnatal circulation in patients with aortic stenosis undergoing fetal aortic valvuloplasty: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 2022 05; 59(5):576-584. View Abstract
  15. Endothelial-to-Mesenchymal Transition as Underlying Mechanism for the Formation of Double-Chambered Right Ventricle. Pediatr Cardiol. 2022 Jun; 43(5):1084-1093. View Abstract
  16. Abnormal Flow Conditions Promote Endocardial Fibroelastosis Via Endothelial-to-Mesenchymal Transition, Which Is Responsive to Losartan Treatment. JACC Basic Transl Sci. 2021 Dec; 6(12):984-999. View Abstract
  17. Mechanical strain triggers endothelial-to-mesenchymal transition of the endocardium in the immature heart. Pediatr Res. 2022 09; 92(3):721-728. View Abstract
  18. Human endothelial colony-forming cells provide trophic support for pluripotent stem cell-derived cardiomyocytes via distinctively high expression of neuregulin-1. Angiogenesis. 2021 05; 24(2):327-344. View Abstract
  19. Autogenous mitochondria transplantation for treatment of right heart failure. J Thorac Cardiovasc Surg. 2021 07; 162(1):e111-e121. View Abstract
  20. Flow disturbances and the development of endocardial fibroelastosis. J Thorac Cardiovasc Surg. 2020 Feb; 159(2):637-646. View Abstract
  21. Flow disturbances and progression of endocardial fibroelastosis - a case report. Cardiovasc Pathol. 2019 Sep - Oct; 42:1-3. View Abstract
  22. Alloreactivity and allorecognition of syngeneic and allogeneic mitochondria. Mitochondrion. 2019 05; 46:103-115. View Abstract
  23. A Growth-Accommodating Implant for Paediatric Applications. Nat Biomed Eng. 2017; 1:818-825. View Abstract
  24. Treating a 20 mm Hg gradient alleviates myocardial hypertrophy in experimental aortic coarctation. J Surg Res. 2017 10; 218:194-201. View Abstract
  25. Vascular Endothelial Growth Factor Prevents Endothelial-to-Mesenchymal Transition in Hypertrophy. Ann Thorac Surg. 2017 Sep; 104(3):932-939. View Abstract
  26. Myocardial rescue with autologous mitochondrial transplantation in a porcine model of ischemia/reperfusion. J Thorac Cardiovasc Surg. 2017 04; 153(4):934-943. View Abstract
  27. Development of a Vascularized Heterotopic Neonatal Rat Heart Transplantation Model. Eur Surg Res. 2016; 57(3-4):240-251. View Abstract
  28. Pilomotor function is impaired in patients with Parkinson's disease: A study of the adrenergic axon-reflex response and autonomic functions. Parkinsonism Relat Disord. 2016 10; 31:129-134. View Abstract
  29. Intracoronary Delivery of Mitochondria to the Ischemic Heart for Cardioprotection. PLoS One. 2016; 11(8):e0160889. View Abstract
  30. A light-reflecting balloon catheter for atraumatic tissue defect repair. Sci Transl Med. 2015 Sep 23; 7(306):306ra149. View Abstract
  31. Distention of the Immature Left Ventricle Triggers Development of Endocardial Fibroelastosis: An Animal Model of Endocardial Fibroelastosis Introducing Morphopathological Features of Evolving Fetal Hypoplastic Left Heart Syndrome. Biomed Res Int. 2015; 2015:462469. View Abstract
  32. Endocardial fibroelastosis is caused by aberrant endothelial to mesenchymal transition. Circ Res. 2015 Feb 27; 116(5):857-66. View Abstract
  33. Pressure overload induces IL-18 and IL-18R expression, but markedly suppresses IL-18BP expression in a rabbit model. IL-18 potentiates TNF-a-induced cardiomyocyte death. J Mol Cell Cardiol. 2014 Oct; 75:141-51. View Abstract
  34. A blood-resistant surgical glue for minimally invasive repair of vessels and heart defects. Sci Transl Med. 2014 Jan 08; 6(218):218ra6. View Abstract
  35. Pressure-overload hypertrophy of the developing heart reveals activation of divergent gene and protein pathways in the left and right ventricular myocardium. Am J Physiol Heart Circ Physiol. 2013 Mar 01; 304(5):H697-708. View Abstract
  36. A highly tunable biocompatible and multifunctional biodegradable elastomer. Adv Mater. 2013 Feb 25; 25(8):1209-15. View Abstract
  37. An animal model of endocardial fibroelastosis. J Surg Res. 2013 Jun 01; 182(1):94-100. View Abstract
  38. Endogenous angiogenesis inhibitors prevent adaptive capillary growth in left ventricular pressure overload hypertrophy. Ann Thorac Surg. 2012 Nov; 94(5):1509-17. View Abstract
  39. No effect of short-term hypertension on bone matrix mineralization in a surgical animal model in immature rabbits. Clin Exp Hypertens. 2012; 34(2):107-12. View Abstract
  40. Up-regulation of soluble vascular endothelial growth factor receptor-1 prevents angiogenesis in hypertrophied myocardium. Cardiovasc Res. 2011 Feb 01; 89(2):410-8. View Abstract
  41. Electron transport chain dysfunction in neonatal pressure-overload hypertrophy precedes cardiomyocyte apoptosis independent of oxidative stress. J Thorac Cardiovasc Surg. 2010 Jun; 139(6):1609-17. View Abstract
  42. Development of left ventricular diastolic dysfunction with preservation of ejection fraction during progression of infant right ventricular hypertrophy. Circ Heart Fail. 2009 Nov; 2(6):599-607. View Abstract
  43. Myocyte apoptosis occurs early during the development of pressure-overload hypertrophy in infant myocardium. J Thorac Cardiovasc Surg. 2009 Jun; 137(6):1356-62, 1362.e1-3. View Abstract
  44. Proteasome inhibition in hypertrophied myocardium. Am J Physiol Heart Circ Physiol. 2008 Oct; 295(4):H1373-4. View Abstract
  45. Inhibition of glycogen synthase kinase-3beta improves tolerance to ischemia in hypertrophied hearts. Ann Thorac Surg. 2007 Jul; 84(1):126-33. View Abstract
  46. Age-dependent expression of VEGF isoforms and receptors in the rabbit anterior cruciate ligament. Biochim Biophys Acta. 2007 Jul; 1770(7):997-1002. View Abstract
  47. Dopamine induces postischemic cardiomyocyte apoptosis in vivo: an effect ameliorated by propofol. Ann Thorac Surg. 2006 Dec; 82(6):2192-9. View Abstract
  48. Vascular endothelial growth factor prevents apoptosis and preserves contractile function in hypertrophied infant heart. Circulation. 2006 Jul 04; 114(1 Suppl):I290-5. View Abstract
  49. Cardiac conduction through engineered tissue. Am J Pathol. 2006 Jul; 169(1):72-85. View Abstract
  50. Vascular endothelial growth factor delays onset of failure in pressure-overload hypertrophy through matrix metalloproteinase activation and angiogenesis. Basic Res Cardiol. 2006 May; 101(3):204-13. View Abstract
  51. Impaired insulin-signaling in hypertrophied hearts contributes to ischemic injury. Biochem Biophys Res Commun. 2005 May 27; 331(1):15-22. View Abstract
  52. Cyclosporin A but not FK-506 protects against dopamine-induced apoptosis in the stunned heart. Ann Thorac Surg. 2005 May; 79(5):1620-6. View Abstract
  53. Promoting angiogenesis protects severely hypertrophied hearts from ischemic injury. Ann Thorac Surg. 2004 Jun; 77(6):2004-10; discussion 2011. View Abstract
  54. Cytosolic calcium in the ischemic rabbit heart: assessment by pH- and temperature-adjusted rhod-2 spectrofluorometry. Cardiovasc Res. 2003 Sep 01; 59(3):695-704. View Abstract
  55. Postnatal increase in insulin-sensitive glucose transporter expression is associated with improved recovery of postischemic myocardial function. J Thorac Cardiovasc Surg. 2003 Jul; 126(1):263-71. View Abstract
  56. Increased susceptibility of hypertrophied hearts to ischemic injury. Ann Thorac Surg. 2003 Feb; 75(2):S678-84. View Abstract
  57. Noninvasive serial evaluation of myocardial mechanics in pressure overload hypertrophy of rabbit myocardium. Herz. 2003 Feb; 28(1):52-62. View Abstract
  58. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg. 2002 Dec; 74(6):1967-77; discussion 1978. View Abstract
  59. Dopamine treatment of postischemic contractile dysfunction rapidly induces calcium-dependent pro-apoptotic signaling. Circulation. 2002 Sep 24; 106(12 Suppl 1):I290-8. View Abstract
  60. Protective effects of protein kinase C during myocardial ischemia require activation of phosphatidyl-inositol specific phospholipase C. Ann Thorac Surg. 2002 Apr; 73(4):1236-45. View Abstract
  61. Outcome after reconstruction of discontinuous pulmonary arteries. J Thorac Cardiovasc Surg. 2002 Feb; 123(2):246-57. View Abstract
  62. Rapid endotoxin-induced alterations in myocardial calcium handling: obligatory role of cardiac TNF-alpha. Anesthesiology. 2001 Dec; 95(6):1396-405. View Abstract
  63. Insulin-like growth factor-1 improves postischemic recovery in hypertrophied hearts. Ann Thorac Surg. 2001 Nov; 72(5):1650-6. View Abstract
  64. Inhibition of tumor necrosis factor-alpha improves postischemic recovery of hypertrophied hearts. Circulation. 2001 Sep 18; 104(12 Suppl 1):I350-5. View Abstract
  65. Post-ischemic PKC inhibition impairs myocardial calcium handling and increases contractile protein calcium sensitivity. Cardiovasc Res. 2001 Jul; 51(1):108-21. View Abstract
  66. Congenital supravalvar aortic stenosis: a simple lesion? Eur J Cardiothorac Surg. 2001 Feb; 19(2):195-202. View Abstract
  67. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001 Jan; 121(1):28-41. View Abstract
  68. Differential role of sarcolemmal and mitochondrial K(ATP) channels in adenosine-enhanced ischemic preconditioning. Am J Physiol Heart Circ Physiol. 2000 Dec; 279(6):H2694-703. View Abstract
  69. Surgery for bilateral outflow tract obstruction in elastin arteriopathy. J Thorac Cardiovasc Surg. 2000 Oct; 120(4):755-63. View Abstract
  70. Impaired glucose transporter activity in pressure-overload hypertrophy is an early indicator of progression to failure. Circulation. 1999 Nov 09; 100(19 Suppl):II187-93. View Abstract
  71. Forty-one years of surgical experience with congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg. 1999 Nov; 118(5):874-85. View Abstract
  72. Bidirectional superior cavopulmonary anastomosis improves mechanical efficiency in dilated atriopulmonary connections. J Thorac Cardiovasc Surg. 1999 Oct; 118(4):681-91. View Abstract
  73. Fluid dynamic comparison of intra-atrial and extracardiac total cavopulmonary connections. J Thorac Cardiovasc Surg. 1999 Apr; 117(4):697-704. View Abstract
  74. Vesnarinone and amrinone reduce the systemic inflammatory response syndrome. J Thorac Cardiovasc Surg. 1999 Feb; 117(2):375-82. View Abstract
  75. Administration of fructose 1,6-diphosphate during early reperfusion significantly improves recovery of contractile function in the postischemic heart. J Thorac Cardiovasc Surg. 1998 Aug; 116(2):335-43. View Abstract
  76. Activation of the clotting system: heparin-coated versus non coated systems for extracorporeal circulation. Int J Artif Organs. 1997 Dec; 20(12):708-12. View Abstract
  77. Adenosine prevents protein kinase C activation during hypothermic ischemia. Circulation. 1997 Nov 04; 96(9 Suppl):II-221-5; discussion II-225-6. View Abstract
  78. Evaluation of a pulsatile pediatric ventricular assist device in an acute right heart failure model. Ann Thorac Surg. 1997 Nov; 64(5):1374-80. View Abstract
  79. Hemodynamic effect of progressive right atrial dilatation in atriopulmonary connections. J Thorac Cardiovasc Surg. 1997 Jul; 114(1):2-8. View Abstract
  80. A high-resolution esophageal electrocardiogram for monitoring atrial activity in the hypothermic potassium-arrested heart. Anesth Analg. 1997 Mar; 84(3):484-90. View Abstract
  81. Partial liquid ventilation combined with inhaled nitric oxide in acute respiratory failure with pulmonary hypertension in piglets. Pediatr Res. 1997 Feb; 41(2):172-7. View Abstract
  82. Inhaled nitric oxide versus inhaled prostacyclin and intravenous versus inhaled prostacyclin in acute respiratory failure with pulmonary hypertension in piglets. Pediatr Res. 1995 Aug; 38(2):198-204. View Abstract
  83. [Extracorporeal membrane oxygenation in newborn infants and children]. Wien Klin Wochenschr. 1995; 107(14):427-35. View Abstract
  84. [Total cavo-pulmonary connection as a therapeutic principle in anatomical and functional univentricular heart]. Wien Klin Wochenschr. 1995; 107(23):714-7. View Abstract
  85. Diagnosis and treatment of carotid body tumors. Thorac Cardiovasc Surg. 1993 Dec; 41(6):340-3. View Abstract
  86. Air embolism with bilateral pneumothorax after a five-meter dive. Undersea Hyperb Med. 1993 Jun; 20(2):155-7. View Abstract
  87. [Carotid glomus tumors: vascular surgery from the oncologic viewpoint]. Vasa Suppl. 1992; 37:73-4. View Abstract
  88. IgG-anticardiolipin-antibodies are markers for cerebral and peripheral artery disease. Vasa. 1992; 21(2):158-62. View Abstract
  89. Congenital giant arteriovenous malformation in a 31-year-old male. Thorac Cardiovasc Surg. 1991 Apr; 39(2):110-2. View Abstract
  90. Serum selenium levels in distinct manifestations of coronary artery disease. Acta Med Austriaca. 1990; 17(1):22-4. View Abstract

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