Syndrome of anomalous left coronary artery
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REFERENCES 1. Fitzsimmons SC. The changing epidemiology of cystic fibrosis. J Pedi- atr 1993;122:1–9. 2. Halliburton CS, Mannino DM, Olney RS. Cystic fibrosis deaths in the United States from 1979 through 1991: an analysis using multiple-cause mor- tality data. Arch Pediatr Adolesc Med 1996;150:1181–5. 3. Elborn JS, Shale DJ, Britton JR. Cystic fibrosis: current survival and population estimates to the year 2000. Thorax 1991;46:881–5. 4. Frederiksen B, Lanng S, Koch C, Hoiby N. Improved survival in the Danish center-treated cystic fibrosis patients: results of aggressive treatment. Pediatr Pulmonol 1996;21:153–8. 5. Fogarty A, Hubbard R, Britton J. International comparison of median age at death from cystic fibrosis. Chest 2000;117:1656–60. 6. Corey M, Farewell V. Determinants of mortality from cystic fibrosis in Canada, 1970–1989. Am J Epidemiol 1996;143:1007–17. 7. Hill DJ, Martin AJ, Davidson GP, Smith GS. Survival of cystic fibrosis patients in South Australia: evidence that cystic fibrosis centre care leads to better survival. Med J Aust 1985;143:230–2. 8. Cystic Fibrosis Foundation National Patient Registry Annual Data Re- port 2000. Bethesda (MD): Cystic Fibrosis Foundation; 2001. 9. Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B. Gender gap in cystic fibrosis mortality. Am J Epidemiol 1997;145:794–803. 10. Demko CA, Byard PJ, Davis PB. Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection. J Clin Epidemiol 1995;48:1041–9. 11. Corey M. Survival estimates in cystic fibrosis: snapshots of a moving target. Pediatr Pulmonol 1996;21:149–50. 12. Wesley AW, Smith PA, Elliott RB. Experience with neonatal screening for cystic fibrosis in New Zealand using measurement of immunoreactive trypsinogen. Aust Paediatr J 1989;25:151–5. 13. Hudson I, Phelan PD. Are sex, age at diagnosis, or mode of presenta- tion prognostic factors for cystic fibrosis? Pediatr Pulmonol 1987; 3:288–97. 14. Liou TG, Adler FR, Fitzsimmons SC, Cahill BC, Hibbs JR, Marshall BC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol 2001;153:345–52. 15. Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988;41:583–91. 16. Tonelli MR, Aitken ML. New and emerging therapies for pulmonary complications of cystic fibrosis. Drugs 2001;61:1379–85. 636 Kulich et al The Journal of Pediatrics • June 2003 50 Years Ago in The Journal of Pediatrics SYNDROME OF ANOMALOUS LEFT CORONARY ARTERY Kelly VC,Wilkins WS, Scott RB. J Pediatr 1953;42:731-3 In June 1953, when this report of the death of a 3-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCA) was published, corrective surgery for congenital cardiac anomalies with cardiopulmonary by- pass had not yet been done successfully. Thus, the discussion in 1953 focused on the typical clinical, electrocardiographic, and roentgenographic features of ALCA, suggesting that the diagnosis could be made before death. A brief mention of a possible closed heart surgical approach, based on the Potts-Smith shunt procedure is made. The uniformly fatal outcome in the 40 cases reported by 1953 in symptomatic infants was emphasized. An intriguing feature of the report is left ventricular rupture and death secondary to the tamponade induced by accumula- tion of pericardial blood. This complication still occurs in adults with myocardial infarction, albeit rarely. In the intervening 50 years, a remarkable transformation in outcome for ALCA has occurred. Early treatment of ALCA by ligation of the anomalous vessel at the pulmonary arterial connection, with the goal of interrupting the flow of poorly oxy- genated blood at low pressure to the myocardium, did improve outcome, reducing mortality to ~50%. Other procedures based on coronary bypass experience to re-establish flow to the ALCA via venous or aortico-ALCA shunts had modest success be- cause of the small size of the bypass grafts or vessels. Anatomical correction of ALCA by reimplantation of the left coronary artery to the aorta has become the standard surgical approach over the last 10 to 15 years, as experience with coronary reimplantation for other congenital anomalies, such as trans- position of the great vessels, has become common and successful. The results are quite amazing, with more than 90% perioper- ative survival in many institutions and good long-term outcomes despite the severe and extensive infarction that occurs before diagnosis. It is truly remarkable that a disorder with 100% mortality 50 years ago now results in nearly 100% early survival. This incredible change in outcome parallels the progress in surgical correction of many congenital cardiac anomalies in the same time span. Arnold Strauss, MD Department of Pediatrics Vanderbilt University Medical Center Nashville, TN 37232-2574 YMPD255 10.1067/mpd.2003.255
Transcript of Syndrome of anomalous left coronary artery
REFERENCES1. Fitzsimmons SC. The changing epidemiology of cystic fibrosis. J Pedi-atr 1993;122:1–9.2. Halliburton CS, Mannino DM, Olney RS. Cystic fibrosis deaths in theUnited States from 1979 through 1991: an analysis using multiple-cause mor-tality data. Arch Pediatr Adolesc Med 1996;150:1181–5.3. Elborn JS, Shale DJ, Britton JR. Cystic fibrosis: current survival andpopulation estimates to the year 2000. Thorax 1991;46:881–5.4. Frederiksen B, Lanng S, Koch C, Hoiby N. Improved survival in theDanish center-treated cystic fibrosis patients: results of aggressive treatment.Pediatr Pulmonol 1996;21:153–8.5. Fogarty A, Hubbard R, Britton J. International comparison of medianage at death from cystic fibrosis. Chest 2000;117:1656–60.6. Corey M, Farewell V. Determinants of mortality from cystic fibrosis inCanada, 1970–1989. Am J Epidemiol 1996;143:1007–17.7. Hill DJ, Martin AJ, Davidson GP, Smith GS. Survival of cystic fibrosispatients in South Australia: evidence that cystic fibrosis centre care leads tobetter survival. Med J Aust 1985;143:230–2.8. Cystic Fibrosis Foundation National Patient Registry Annual Data Re-port 2000. Bethesda (MD): Cystic Fibrosis Foundation; 2001.
9. Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B. Gendergap in cystic fibrosis mortality. Am J Epidemiol 1997;145:794–803.10. Demko CA, Byard PJ, Davis PB. Gender differences in cystic fibrosis:Pseudomonas aeruginosa infection. J Clin Epidemiol 1995;48:1041–9.11. Corey M. Survival estimates in cystic fibrosis: snapshots of a movingtarget. Pediatr Pulmonol 1996;21:149–50.12. Wesley AW, Smith PA, Elliott RB. Experience with neonatal screeningfor cystic fibrosis in New Zealand using measurement of immunoreactivetrypsinogen. Aust Paediatr J 1989;25:151–5.13. Hudson I, Phelan PD. Are sex, age at diagnosis, or mode of presenta-tion prognostic factors for cystic fibrosis? Pediatr Pulmonol 1987;3:288–97.14. Liou TG, Adler FR, Fitzsimmons SC, Cahill BC, Hibbs JR, MarshallBC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol2001;153:345–52.15. Corey M, McLaughlin FJ, Williams M, Levison H. A comparison ofsurvival, growth, and pulmonary function in patients with cystic fibrosis inBoston and Toronto. J Clin Epidemiol 1988;41:583–91.16. Tonelli MR, Aitken ML. New and emerging therapies for pulmonarycomplications of cystic fibrosis. Drugs 2001;61:1379–85.
636 Kulich et al The Journal of Pediatrics • June 2003
50 Years Ago in The Journal of PediatricsSYNDROME OF ANOMALOUS LEFT CORONARY ARTERY
Kelly VC,Wilkins WS, Scott RB. J Pediatr 1953;42:731-3
In June 1953, when this report of the death of a 3-month-old infant with anomalous origin of the left coronary artery fromthe pulmonary artery (ALCA) was published, corrective surgery for congenital cardiac anomalies with cardiopulmonary by-pass had not yet been done successfully. Thus, the discussion in 1953 focused on the typical clinical, electrocardiographic, androentgenographic features of ALCA, suggesting that the diagnosis could be made before death. A brief mention of a possibleclosed heart surgical approach, based on the Potts-Smith shunt procedure is made. The uniformly fatal outcome in the 40cases reported by 1953 in symptomatic infants was emphasized.
An intriguing feature of the report is left ventricular rupture and death secondary to the tamponade induced by accumula-tion of pericardial blood. This complication still occurs in adults with myocardial infarction, albeit rarely.
In the intervening 50 years, a remarkable transformation in outcome for ALCA has occurred. Early treatment of ALCA byligation of the anomalous vessel at the pulmonary arterial connection, with the goal of interrupting the flow of poorly oxy-genated blood at low pressure to the myocardium, did improve outcome, reducing mortality to ~50%. Other procedures basedon coronary bypass experience to re-establish flow to the ALCA via venous or aortico-ALCA shunts had modest success be-cause of the small size of the bypass grafts or vessels.
Anatomical correction of ALCA by reimplantation of the left coronary artery to the aorta has become the standard surgicalapproach over the last 10 to 15 years, as experience with coronary reimplantation for other congenital anomalies, such as trans-position of the great vessels, has become common and successful.The results are quite amazing, with more than 90% perioper-ative survival in many institutions and good long-term outcomes despite the severe and extensive infarction that occurs beforediagnosis. It is truly remarkable that a disorder with 100% mortality 50 years ago now results in nearly 100% early survival.This incredible change in outcome parallels the progress in surgical correction of many congenital cardiac anomalies in thesame time span.
Arnold Strauss, MDDepartment of Pediatrics
Vanderbilt University Medical CenterNashville, TN 37232-2574
YMPD25510.1067/mpd.2003.255
