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Thoracic Manifestation of Eisenmenger’s Syndrome in Adult Patients: A MDCT Review

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Abstract

Eisenmenger’s syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart diseases (CHD). It is caused by simple or complex CHD with a large systemic-to-pulmonary shunt. When the pulmonary pressure exceeds the systemic pressure, the shunt reverses and central cyanosis appears. ES is a progressive and fatal condition, and it is accompanied by an increased risk of a range of potentially life-threatening complications. Patients with ES are both at risk for bleeding, due to damaged capillaries and high pressure, and for in situ pulmonary thrombosis, related to hyper-viscosity and slow blood flow in dilated pulmonary arteries. Moreover, the enlarged main pulmonary arteries and cardiac chambers may determine displacement or extrinsic compression on airways, pulmonary veins, coronary arteries, and other mediastinal structures. The clinical effects may be diverse, such as respiratory difficulties, localized pulmonary edema, cardiac dysfunction, or sudden death. Multidetector computed tomography (MDCT) allows to accurately assess in a single examination, pulmonary parenchyma and vessels, coronary artery origin, and heart chambers. The aim of this review was to illustrate the thoracic MDCT angiography findings and complications of adult patients with PAH-CHD and in particular of those with ES.

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References

  1. Oliver Ruiz JM (2003) Congenital heart disease in adults: residua, sequelae, and complications of cardiac defects repaired at an early age. Rev Esp Cardiol 56:73–88

    Article  PubMed  Google Scholar 

  2. D’Alto M, Mahadevan VS (2012) Pulmonary arterial hypertension associated with congenital heart disease. Eur Respir Rev 21:328–337

    Article  PubMed  Google Scholar 

  3. D’Alto M, Diller GP (2014) Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. Heart 100:1322–1328. doi:10.1136/heartjnl-2014-305574

    Article  PubMed  Google Scholar 

  4. Gatzoulis MA, Beghetti M, Landzberg MJ et al (2014) Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions. Int J Cardiol 177:340–347. doi:10.1016/j.ijcard.2014.09.024

    Article  PubMed  Google Scholar 

  5. Beghetti M, Galiè N (2009) Eisenmenger syndrome. A clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol 53:733–740

    Article  PubMed  Google Scholar 

  6. Galiè N, Manes A, Palazzini M et al (2008) Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger’s syndrome. Drugs 68:1049–1066

    Article  PubMed  Google Scholar 

  7. Remy-Jardin M, Duhamel A, Deken V et al (2005) Systemic collateral supply in patients with chronic thromboembolic and primary pulmonary hypertension: assessment with multi-detector row helical CT angiography. Radiology 235:274–281

    Article  PubMed  Google Scholar 

  8. Nicol ED, Kafka H, Stirrup J et al (2009) A single, comprehensive non-invasive cardiovascular assessment in pulmonary arterial hypertension: combined computed tomography pulmonary and coronary angiography. Int J Cardiol 136:278–288. doi:10.1016/j.ijcard.2008.05.049

    Article  PubMed  Google Scholar 

  9. Hoey ET, Gopalan D, Agrawal SK et al (2009) Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT. Eur Radiol 19:2557–2568. doi:10.1007/s00330-009-1460-0

    Article  PubMed  Google Scholar 

  10. Perloff JK, Hart EM, Greaves SM et al (2003) Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 92:182–187

    Article  PubMed  Google Scholar 

  11. Frazier AA, Galvin JR, Franks TJ et al (2000) From the archives of the AFIP: pulmonary vasculature: hypertension and infarction. RadioGraphics 20:491–524

    Article  CAS  PubMed  Google Scholar 

  12. Martínez-Quintana E, Rodríguez-González F, Nieto-Lago V (2011) Pulmonary artery aneurysmal dilatation in adult patients with congenital heart disease. World J Pediatr Congenit Heart Surg 2:375–379

    Article  PubMed  Google Scholar 

  13. Silversides CK, Granton JT, Konen E et al (2003) Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 42:1982–1987

    Article  PubMed  Google Scholar 

  14. Hassell KL (1998) Altered hemostasis in pulmonary hypertension. Blood Coagul Fibrinolysis 9:107–117

    Article  CAS  PubMed  Google Scholar 

  15. Lopes AA, Caramurù LH, Maeda NY (2002) Endothelial dysfunction associated with chronic intravascular coagulation in secondary pulmonary hypertension. Clin Appl Thromb Hemost 8:353–358

    Article  PubMed  Google Scholar 

  16. Broberg C, Ujita M, Babu-Narayan S et al (2004) Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger’s syndrome: a clinical dilemma. Heart 90:e63

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  17. Lupinetti FM, Bolling SF, Bove EL et al (1994) Selective lung or heart-lung transplantation for pulmonary hypertension associated with congenital cardiac anomalies. Ann Thorac Surg 57:1545–1548. doi:10.3109/10408370490888442

    Article  CAS  PubMed  Google Scholar 

  18. Russo V, Zompatori M, Galiè N (2012) Extensive right pulmonary artery dissection in a young patient with chronic pulmonary hypertension. Heart 98:265–266

    Article  PubMed  Google Scholar 

  19. Tan RT, Kuzo R, Goodman LR et al (1998) Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Chest 113:1250–1256

    Article  CAS  PubMed  Google Scholar 

  20. Sheehan R, Perloff JK, Fishbein MC et al (2005) Pulmonary neovascularity: a distinctive radiographic finding in Eisenmenger syndrome. Circulation 112:2778–2785

    Article  PubMed  Google Scholar 

  21. Griffin N, Allen D, Wort J et al (2007) Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology? Clin Radiol 62:587–595

    Article  CAS  PubMed  Google Scholar 

  22. Modolon C, Attinà D, Buia F et al (2012) Lung neovascularity in pulmonary arterial hypertension associated with congenital heart defects and idiopathic pulmonary arterial hypertension: study of 198 patients. Eur Radiol 22:1059–1066

    Article  PubMed  Google Scholar 

  23. Mamas MA, Clarke B, Mahadevan VS (2008) Embolisation of systemic-to-pulmonary collaterals in patients with the Eisenmenger reaction presenting with haemoptysis. Cardiol Young 18:528–531. doi:10.1017/S1047951108002680

    Article  PubMed  Google Scholar 

  24. Peña E, Dennie C, Veinot J et al (2012) Pulmonary hypertension: how the radiologist can help. RadioGraphics 32:9–32

    Article  PubMed  Google Scholar 

  25. Bristow MR, Zisman LS, Lowes BD et al (1998) The pressure-overloaded right ventricle in pulmonary hypertension. Chest 114:101S–106S

    Article  CAS  PubMed  Google Scholar 

  26. McKie SJ, Hardwick DJ, Reid JH et al (2005) Features of cardiac disease demonstrated on CT pulmonary angiography. Clin Radiol 60:31–38

    Article  CAS  PubMed  Google Scholar 

  27. Groves AM, Win T, Charman SC et al (2004) Semi-quantitative assessment of tricuspid regurgitation on contrast-enhanced multidetector CT. Clin Radiol 59:715–719

    Article  CAS  PubMed  Google Scholar 

  28. Yeh BM, Kurzman P, Foster E et al (2004) Clinical relevance of retrograde inferior vena cava or hepatic vein opacification during contrast-enhanced CT. AJR Am J Roentgenol 183:1227–1232

    Article  PubMed  Google Scholar 

  29. Kaemmerer H, Mebus S, Schulze-Neick I et al (2010) The adult patient with Eisenmenger syndrome: a medical update after Dana point part I: epidemiology, clinical aspects and diagnostic options. Curr Cardiol Rev 6:343–355

    Article  PubMed Central  PubMed  Google Scholar 

  30. Geva T, Martins JD, Wald RM (2014) Atrial septal defects. Lancet 383(9932):1921–1932. doi:10.1016/S0140-6736(13)62145-5

    Article  PubMed  Google Scholar 

  31. Penny DJ, Vick GW 3rd (2011) Ventricular septal defect. Lancet 377(9771):1103–1112. doi:10.1016/S0140-6736(10)61339-6

    Article  PubMed  Google Scholar 

  32. Galiè N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537

    Article  PubMed  Google Scholar 

  33. Moceri P, Dimopoulos K, Liodakis E et al (2012) Echocardiographic predictors of outcome in Eisenmenger Syndrome. Circulation 126:1461–1468

    Article  PubMed  Google Scholar 

  34. Bonello B, Kilner PJ (2012) Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis 105:605–613

    Article  PubMed  Google Scholar 

  35. Méndez C, Soler R, Rodriguez E et al (2011) Magnetic resonance imaging of abnormal ventricular septal motion in heart diseases: a pictorial review. Insights Imaging 2:483–492

    Article  PubMed Central  PubMed  Google Scholar 

  36. Peacock AJ, Vonk Noordegraaf A (2013) Cardiac magnetic resonance imaging in pulmonary arterial hypertension. Eur Respir Rev 22:526–534

    Article  PubMed  Google Scholar 

  37. Devaraj A, Hansell DM (2009) Computed tomography signs of pulmonary hypertension: old and new observations. Clin Radiol 64(8):751–760

    Article  CAS  PubMed  Google Scholar 

  38. Nolan RL, Mcadams HP, Sporn TA et al (1999) Pulmonary cholesterol granulomas in patients with pulmonary artery hypertension: chest radiographic and CT findings. AJR Am J Roentgenol 172:1317–1319

    Article  CAS  PubMed  Google Scholar 

  39. Horton MR, Tuder RM (2004) Primary pulmonary arterial hypertension presenting as diffuse micronodules on CT. Crit Rev Comput Tomogr 45:335–341

    Article  CAS  PubMed  Google Scholar 

  40. Okada F, Ando Y, Yoshitake S et al (2007) Clinical/pathologic correlations in 553 patients with primary centrilobular findings on high-resolution CT scan of the thorax. Chest 132:1939–1948

    Article  PubMed  Google Scholar 

  41. Stanger R, Lucas RV, Edwards JE (1969) Anatomic factors causing respiratory distress in acyanotic congenital cardiac disease. Special reference to bronchial obstruction. Pediatrics 43:760–769

    CAS  PubMed  Google Scholar 

  42. Berlinger NT, Long C, Foker J et al (1983) Tracheobronchial compression in acyanotic congenital heart disease. Ann Otol Rhinol Laryngol 92:387–390

    Article  CAS  PubMed  Google Scholar 

  43. Healy F, Hanna BD, Zinman R (2012) Pulmonary complications of congenital heart disease. Paediatr Respir Rev 13:10–15. doi:10.1016/j.prrv.2011.01.007

    Article  CAS  PubMed  Google Scholar 

  44. Decuypere V, Delcroix M, Budts W (2004) Left main coronary artery and right pulmonary vein compression by a large pulmonary artery aneurysm. Heart 90:e21

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  45. Vaseghi M, Lee JS, Currier JW (2007) Acute myocardial infarction secondary to left main coronary artery compression by pulmonary artery aneurysm in pulmonary arterial hypertension. J Invasive Cardiol 19:E375–E377

    PubMed  Google Scholar 

  46. Dubois CL, Dymarkowski S, Van Cleemput J (2007) Compression of the left main coronary artery by the pulmonary artery in a patient with the Eisenmenger syndrome. Eur Heart J 28:1945

    Article  PubMed  Google Scholar 

  47. Mesquita SM, Castro CR, Ikari NM et al (2004) Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med 116:369–374

    Article  PubMed  Google Scholar 

  48. Kajita LJ, Martinez EE, Ambrose JA et al (2001) Extrinsic compression of the left main coronary artery by a dilated pulmonary artery: clinical, angiographic, and hemodynamic determinants. Catheter Cardiovasc Interv 52:49–54

    Article  CAS  PubMed  Google Scholar 

  49. Safi M, Eslami V, Shabestari AA et al (2009) Extrinsic compression of left main coronary artery by the pulmonary trunk secondary to pulmonary hypertension documented using 64-slice multidetector computed tomography coronary angiography. Clin Cardiol 32:426–428

    Article  PubMed  Google Scholar 

  50. Bonderman D, Fleischmann D, Prokop M et al (2002) Images in cardiovascular medicine. Left main coronary artery compression by the pulmonary trunk in pulmonary hypertension. Circulation 105:265

    Article  PubMed  Google Scholar 

  51. Lee MS, Oyama J, Bhatia R et al (2010) Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: a contemporary review and argument for percutaneous revascularization. Catheter Cardiovasc Interv 76:543–550

    Article  PubMed  Google Scholar 

  52. Cheitlin MD, de Castro CM, McAllister HA (1974) Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva. Circulation 50:780–787

    Article  CAS  PubMed  Google Scholar 

  53. Sivakumar K, Rajan M, Francis G et al (2010) Extrinsic compression of the left coronary ostium by the pulmonary trunk: management in a case of Eisenmenger syndrome. Tex Heart Inst J 37:95–98

    PubMed Central  PubMed  Google Scholar 

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The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Cardio-Thoracic Radiology Unit, Cardio-Thoracic-Vascular Department, University Hospital S. Orsola-Malpighi, via Massarenti 9, 40100, Bologna, Italy

    Domenico Attinà, Fabio Niro, Giorgio Garzillo, Cecilia Modolon, Vincenzo Russo, Luigi Lovato & Maurizio Zompatori

  2. Institute of Cardiology, University Hospital S. Orsola-Malpighi, Via Massarenti 9, 40100, Bologna, Italy

    Nazareno Galiè

Authors
  1. Domenico Attinà
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  2. Fabio Niro
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  4. Cecilia Modolon
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  5. Vincenzo Russo
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  7. Nazareno Galiè
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  8. Maurizio Zompatori
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Correspondence to Domenico Attinà.

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Attinà, D., Niro, F., Garzillo, G. et al. Thoracic Manifestation of Eisenmenger’s Syndrome in Adult Patients: A MDCT Review. Lung 193, 173–181 (2015). https://doi.org/10.1007/s00408-014-9681-7

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  • DOI: https://doi.org/10.1007/s00408-014-9681-7

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