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VOLUME 55 , ISSUE 2 ( April-June, 2013 ) > List of Articles

REVIEW ARTICLE

Manifestations of Pulmonary Disease in Adults with Congenital Heart Disease

Anup Kumar Singh, Nick Patel, Kevin Leung, Arunabh Talwar

Keywords : Congenital heart disease, Adults, Pulmonary manifestations

Citation Information : Singh AK, Patel N, Leung K, Talwar A. Manifestations of Pulmonary Disease in Adults with Congenital Heart Disease. Indian J Chest Dis Allied Sci 2013; 55 (2):85-95.

DOI: 10.5005/ijcdas-55-2-85

License: CC BY-NC 4.0

Published Online: 16-06-2022

Copyright Statement:  Copyright © 2013; The Author(s).


Abstract

Children with congenital heart disease (CHD) are more frequently living into adulthood as their survival has improved due to availability of better medical and surgical management in recent times. Management of adults with CHD is emerging as new challenge in the field of medical science. Adults surviving with CHD for longer duration have been observed to develop more complications as compared to children. It is important to recognise and treat these complications early to reduce the morbidity. Pulmonary diseases are the most common systemic complications associated with adults having CHD. These individuals are presenting to clinics or emergency for pulmonary complaints, hence, pulmonologist must be aware about the pulmonary manifestations of CHD and their management.

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  1. Ferencz C, Rubin JD, McCarter RJ, Brenner JI, Neill CA, Perry LW, et al. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol 1985;121:31-6.
  2. Tanner K, Sabrine N, Wren C. Cardiovascular malformations among preterm infants. Pediatrics 2005;116: e833-8.
  3. Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008;153:807-13.
  4. Khalil A, Aggarwal R, Thirupuram S, Arora R. Incidence of congenital heart disease among hospital live births in India. Indian Pediatr 1994;31:519-27.
  5. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: first of two parts. N Engl J Med 2000;342:256-63.
  6. Srinathan SK, Bonser RS, Sethia B, Thorne SA, Brawn WJ, Barron DJ. Changing practice of cardiac surgery in adult patients with congenital heart disease. Heart 2005;91:207-12.
  7. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007;115:163-72.
  8. Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, Vliegen HW, et al. Gender and outcome in adult congenital heart disease. Circulation 2008;118:26-32.
  9. Shah P. eComment: cardiac surgery in grown-up congenital heart patients: will the surgical workload increase? Interact Cardiovasc Thorac Surg 2008;7:89.
  10. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008;52:e1-121.
  11. Williams AM, Crabbe DC. Pectus deformities of the anterior chest wall. Paediatr Respir Rev 2003;4:237-42.
  12. Westphal FL, Lima LC, Lima Neto JC, Chaves AR, Santos Júnior VL, Ferreira BL. Prevalence of pectus carinatum and pectus excavatum in students in the city of Manaus, Brazil. J Bras Pneumol 2009;35:221-6.
  13. Shamberger RC, Welch KJ, Castaneda AR, Keane JF, Fyler DC. Anterior chest wall deformities and congenital heart disease. J Thorac Cardiovasc Surg 1988;96:427-32.
  14. Davis WC, Berley FV. Pectus excavatum and pectus carinatum: report on the surgical treatment of eleven patients. Am J Surg 1956;91:770-6.
  15. Ravitch MM. Operative treatment of congenital deformities of the chest. Am J Surg 1961;10:588-97.
  16. Molik KA, Engum SA, Rescorla FJ, West KW, Scherer LR, Grosfeld JL. Pectus excavatum repair: experience with standard and minimal invasive techniques. J Pediatr Surg 2001;36:324-8.
  17. Fonkalsrud EW. Current management of pectus excavatum. World J Surg 2003;27:502-8.
  18. Goretsky MJ, Kelly RE Jr, Croitoru D, Nuss D. Chest wall anomalies: pectus excavatum and pectus carinatum. Adolesc Med Clin 2004;15:455-71.
  19. Saxena AK. Pectus excavatum, pectus carinatum and other forms of thoracic deformities. J Indian Assoc Pediatr Surg 2005;10:147-57.
  20. Maeda T, Suzuki Y, Haeno S, Asada M, Hiramatsu R, Tanaka F, et al. Ehlers-Danlos syndrome and congenital heart anomalies. Intern Med 1996;35:200-02.
  21. Castile RG, Staats BA, Westbrook PR. Symptomatic pectus deformities of the chest. Am Rev Respir Dis 1982;126:564-8.
  22. Quigley PM, Haller JA Jr, Jelus KL, Loughlin GM, Marcus CL. Cardiorespiratory function before and after corrective surgery in pectus excavatum. J Pediatr 1996;128:638-43.
  23. Kaguraoka H, Ohnuki T, Itaoka T, Kei J, Yokoyama M, Nitta S. Degree of severity of pectus excavatum and pulmonary function in preoperative and postoperative periods. J Thorac Cardiovasc Surg 1992;104:1483-8.
  24. Haller JA Jr, Kramer SS, Lietman SA. Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report. J Pediatr Surg 1987;22:904-6.
  25. Derveaux L, Clarysse I, Ivanoff I, Demedts M. Preoperative and postoperative abnormalities in chest xray indices and in lung function in pectus deformities. Chest 1989;95:850-6.
  26. Morshuis WJ, Folgering HT, Barentsz JO, Cox AL, van Lier HJ, Lacquet LK. Exercise cardiorespiratory function before and one year after operation for pectus excavatum. J Thorac Cardiovasc Surg 1994;107:1403-9.
  27. Goretsky MJ, Kelly RE Jr, Croitoru D, Nuss D. Chest wall anomalies: pectus excavatum and pectus carinatum. Adolesc Med Clin 2004;15:455-71.
  28. Shamberger RC, Welch KJ. Surgical correction of pectus carinatum. J Pediatr Surg 1987;22:48-53.
  29. Fonkalsrud EW, Anselmo DM. Less extensive techniques for repair of pectus carinatum: the undertreated chest deformity. J Am Coll Surg 2004;198:898-905.
  30. Shamberger RC, Welch KJ. Surgical correction of chondromanubrial deformity (Currarino Silverman syndrome). J Pediatr Surg 1988;23:319-22.
  31. Chidambaram B, Mehta AV. Currarino-Silverman syndrome (pectus carinatum type 2 deformity) and mitral valve disease. Chest 1992;102:780-2.
  32. Robicsek F. Surgical treatment of pectus carinatum. Chest Surg Clin N Am 2000;10:357-76.
  33. Motro M, Fisman EZ, Tenenbaum A. Cardiovascular management of Marfan syndrome. Isr Med Assoc J 2008;10:182-5.
  34. Bruno E, Rossi N, Thuer O, Córdoba R, Alday LE. Cardiovascular findings, and clinical course, in patients with Williams syndrome. Cardiol Young 2003;13:532-6.
  35. Albano LM, Nishioka SA, Moysés RL, Wagenführ J, Bertola D, Sugayama SM, et al. Friedreich's ataxia: cardiac evaluation of 25 patients with clinical diagnosis and literature review. Arq Bras Cardiol 2002;78:444-51.
  36. Rinsky LA. Quantifying scoliosis: we are still not there. Spine J 2010;10:813-4.
  37. Bergofsky EH. Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis 1979;119:643-69.
  38. Kaplan J. Sleep and breathing in restrictive thoracic cage and lung disease. Sleep Med Clin 2008:3:517-24.
  39. Lisboa C, Moreno R, Fava M, Ferretti R, Cruz E. Inspiratory muscle function in patients with severe kyphoscoliosis. Am Rev Respir Dis 1985;132:48-52.
  40. Leger P, Bedicam JM, Cornette A, Reybet-Degat O, Langevin B, Polu JM, et al. Nasal intermittent positive pressure ventilation: long-term follow-up in patients with severe chronic respratory insufficiency. Chest 1994;105: 100-5.
  41. Rawlings MS. Straight back syndrome: a new heart disease. Dis Chest 1961;39:435-43.
  42. Saenger P. Turner's syndrome. N Engl J Med 1996;335: 1749-54.
  43. Sarkozy A, Digilio MC, Dallapiccola B. Leopard syndrome. Orphanet J Rare Dis 2008;3:13.
  44. Thakral CL, Maji DC, Sajwani MJ. Congenital lobar emphysema: experience with 21 cases. Pediatr Surg Int 2001;17:88-91.
  45. Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am 1994;41:453-72.
  46. Cunha Fatureto M, Pinheiro Ferreira D, Amaro Ferraz D, Vieira Dos Santos JP, Andrade Maia S. Congenital lobar emphysema: study of a case. Rev Port Pneumol 2008;14:893-6.
  47. Stanger P, Lucas RV, Edwards JE. Anatomic factors causing respiratory distress in acyanotic congenital cardiac disease. Pediatrics 1969;43:760-9.
  48. Pierce WS, DeParedes CG, Friedman S, Waldhausen JA. Concomitant congenital heart disease and lobar emphysema in infants: incidence, diagnosis and operative management. Ann Surg 1970;172:951-6.
  49. Khemiri M, Ouederni M, Ben Mansour F, Barasaoui S. Bronchogenic cyst: an uncommon cause of congenital lobar emphysema. Respir Med 2008;102:1663-6.
  50. Mei-Zahav M, Konen O, Manson D, Langer JC. Is congenital lobar emphysema a surgical disease? J Pediatr Surg 2006;41:1058-61.
  51. Logan JW, Rice HE, Goldberg RN, Cotten CM. Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol 2007;27: 535-49.
  52. De Troyer A, Yernault JC, Englert M. Lung hypoplasia in congenital pulmonary valve stenosis. Circulation 1977;56:647-51.
  53. Dupuis C, Charaf LA, Breviere GM, Abou P, Rémy- Jardin M, Helmius G. The “adult” form of the scimitar syndrome. Am J Cardiol 1992;70:502-7.
  54. Johnson RJ, Haworth SG. Pulmonary vascular and alveolar development in tetralogy of Fallot: a recommendation for early correction. Thorax 1982;37:893-901.
  55. Noone PG, Leigh MW, Sannuti A, Minnix SL, Carson JL, Hazucha M, et al. Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med 2004;169:459-67.
  56. Kennedy MP, Omran H, Leigh MW, Dell S, Morgan L, Molina PL, et al. Congenital heart disease and other heterotoxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation 2007;115:2814-21.
  57. Counil F, Ichay L, Guillaumont S, Dumas R, Voisin M. Association of severe bronchial disease (bronchial casts, bronchiectasis) and partial abnormal pulmonary venous drainage in 2 children with Turner's syndrome. Arch Pediatr 1999;6:1070-4.
  58. Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010;137(6 Suppl.):52S-61S.
  59. Rubin LJ, Badesch DB. Evaluation and management of the patient with pulmonary arterial hypertension. Ann Intern Med 2005;143:282-92.
  60. Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007;120:198-204.
  61. Engelfriet PM, Duffels MG, Möller T, Boersma E, Tijssen JG, Thaulow E, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart 2007;93:682-7.
  62. Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010;137:376-87.
  63. Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004;25:2243-78.
  64. Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43(12 Suppl. S):5S-12S.
  65. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54(1 Suppl.):S43-54.
  66. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958;2: 701-9.
  67. Cantor WJ, Harrison DA, Moussadji JS, Connelly MS, Webb GD, Liu P, et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol 1999;84:677-81.
  68. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Artery Dis 2005;16:13-8.
  69. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-65.
  70. Schulze-Neick I, Gilbert N, Ewert R, Witt C, Gruenig E, Enke B, et al. Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy. Am Heart J 2005;150:716.
  71. Galie N, Hoeper MM, Humbert M, Vachiery JL, Barbera JA, Beghetti M, et al. 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). Euro Heart J 2009;30:2493-537.
  72. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007;115: 1039-50.
  73. Dimopoulos K, Giannakoulas G, Wort SJ, Gatzoulis MA. Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications. Curr Opin Cardiol 2008;23:545-54.
  74. Silversides CK, Granton JT, Konen E, Hart MA, Webb GD, Therrien J. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003;42:1982-7.
  75. Broberg C, Ujita M, Babu-Narayan S, Rubens M, Prasad SK, Gibbs JS, et al. Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger's syndrome: a clinical dilemma. Heart 2004;90:e63.
  76. Broberg CS, Ujita M, Prasad S, Li W, Rubens M, Bax BE, et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol 2007;50:634-42.
  77. Noonan JA. Noonan syndrome revisited. J Pediatr 1999;135:667-8.
  78. Morice A, Ulloa-Gutierrez R, Avila-Agüero ML. Congenital rubella syndrome: progress and future challenges. Expert Rev Vaccines 2009;8:323-31.
  79. Hall JR, Pyeritz RE, Dudgeon DL, Haller JA Jr. Pneumothorax in the Marfan syndrome: prevalence and therapy. Ann Thorac Surg 1984;37:500-4.
  80. O'Neill S, Sweeney J, Walker F, O'Dwyer WF. Pneumothorax in the Ehlers-Danlos syndrome. Ir J Med Sci 1981;150:43-4.
  81. Chatzis AC, Giannopoulos NM, Tsoutsinos AI, Chryssostomidis G, Panagiotou C, Zavaropoulos P, et al. Successful surgical correction of congenital heart disease in adults: seven years’ experience. Hellenic J Cardiol 2005;46:128-34.
  82. Yap SC, Roos-Hesselink JW, Hoendermis ES, Budts W, Vliegen HW, Mulder BJ, et al. Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study. Eur Heart J 2007;28:1854-61.
  83. Book WM. Heart failure in the adult patient with congenital heart disease. J Card Fail 2005;11:306-12.
  84. Perens GS, Shannon KM, Levi DS, Drant S. Successful treatment of eosinophilic pleural effusions following congenital heart surgery. Pediatr Cardiol 2006;27:168-9.
  85. Doerr CH, Allen MS, Nichols FC, Ryu JH. Etiology of chylothorax in 203 patients. Mayo Clin Proc 2005;80:867-70.
  86. Milonakis M, Chatzis AC, Giannopoulos NM, Contrafouris C, Bobos D, Kirvassilis GV, et al. Etiology and management of chylothorax following pediatric heart surgery. J Card Surg 2009;24:369-73.
  87. Marino BS, Pudusseri A, Wernovsky G, Paridon SM, Walker SA, Cohen MS. Risk factors and comorbidities associated with obesity in children and adolescents after the arterial switch operation and Ross procedure. Am Heart J 2009;158:473-9.
  88. Dua JS, Cooper AR, Fox KR, Graham Stuart A. Physical activity levels in adults with congenital heart disease. Eur J Cardiovasc Prev Rehabil 2007;14:287-93.
  89. Fernhall B, Otterstetter M. Attenuated responses to sympathoexcitation in individuals with Down syndrome. J Appl Physiol 2003;94:2158-65.
  90. Morgan T. Turner syndrome: diagnosis and management. Am Fam Physician 2007;76:405-10.
  91. Hidestrand P, Vasconez H, Cottrill C. Carpenter syndrome. J Craniofac Surg 2009;20:254-6.
  92. Karska-Basta I, Kubicka-Trzaska A, Filemonowicz- Skoczek A, Romanowska-Dixon B, Kobylarz J. Alström syndrome: a case report and literature review. Klin Oczna 2008;110:188-92.
  93. Mykytyn K, Nishimura DY, Searby CC, Beck G, Bugge K, Haines HL, et al. Evaluation of complex inheritance involving the most common Bardet-Biedl syndrome locus (BBS1). Am J Hum Genet 2003;72:429-37.
  94. Morgan PJ. Peripartum management of a patient with Isaacs’ syndrome. Can J Anaesth 1997;44:1174-7.
  95. Nystad W, Meyer HE, Nafstad P, Tverdal A, Engeland A. Body mass index in relation to adult asthma among 135,000 Norwegian men and women. Am J Epidemiol 2004;160:969-76.
  96. Dixon AE, Shade DM, Cohen RI, Skloot GS, Holbrook JT, Smith LJ, et al. Effect of obesity and clinical presentation and response to treatment in asthma. J Asthma 2006;43:553-8.
  97. Saint-Pierre P, Bourdin A, Chanez P, Daures JP, Godard P. Are overweight asthmatics more difficult to control? Allergy 2006;61:79-84.
  98. Vestbo J, Prescott E, Almdal T, Dahl M, Nordestgaard BG, Andersen T, et al. Body mass, fat-free body mass, and prognosis in patients with chronic obstructive pulmonary disease from a random population sample: findings from the Copenhagen city heart study. Am J Respir Crit Care Med 2006;173:79-83.
  99. Nishimura Y, Tsutsumi M, Nakata H, Tsunenari T, Maeda H, Yokoyama M. Relationship between respiratory muscle strength and lean body mass in men with COPD. Chest 1995;107:1232-6.
  100. Koenig SM. Pulmonary complications of obesity. Am J Med Sci 2001;321:249-79.
  101. Karamanolis G, Sifrim D. Developments in pathogenesis and diagnosis of gastroesophageal reflux disease. Curr Opin Gastroenterol 2007;23:428-33.
  102. Brodsky JB, Lemmens HJ, Brock-Utne JG, Vierra M, Saidman LJ. Morbid obesity and tracheal intubation. Anesth Analg 2002;94:732-6.
  103. Söderberg M, Thomson D, White T. Respiration, circulation and anaesthetic management in obesity: investigation before and after jejunoileal bypass. Acta Anaesthesiol Scand 1977;21:55-61.
  104. Mickelson SA. Preoperative and postoperative management of obstructive sleep apnea patients. Otolaryngol Clin North Am 2007;40:877-89.
  105. Pianosi PT, Johnson JN, Turchetta A, Johnson BD. Pulmonary function and ventilatory limitation to exercise in congenital heart disease. Congenit Heart Dis 2009;4: 2-11.
  106. Joho-Arreola AL, Bauersfeld U, Stauffer UG, Baenziger O, Bernet V. Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children. Eur J Cardiothorac Surg 2005;27:53-7.
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