Aortic atresia with hypoplasia of the left heart and aortic arch
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STRAUSS,BRADLEY , AND BROLIN THE JOURNAL OF PEDIATRICS APRIL 2001 lowing gastric bypass for the treatment of morbid obesity. Am Surg 1982; 48:363-5. 23. Richards DS, Miller DK, Goodman GN. Pregnancy after gastric bypass for morbid obesity. J Reprod Med 1987;32:172-6. 24. Gurewitsch ED, Smith-Levitin M, Mack J. Pregnancy following gastric bypass surgery for morbid obesity. Obstet Gynecol 1996;88:658-61. 25. Haddow JE, Hill LE, Kloza EM, Thanhauser D. Neural tube defects after gastric bypass [letter]. Lancet 1986;1:1330. 26. Werler MM, Louik C, Shapiro S, Mitchell AA. Prepregnant weight in relation to risk of neural tube defects. JAMA 1996;275:1089-92. 27. Knudsen LB, Kallen B. Gastric by- pass, pregnancy, and neural tube de- fects. Lancet 1986;2:227. 28. Borjeson M. Overweight children. Acta Paediatr 1962;51(suppl):1-76. 39. Barlow SE, Dietz WH. Obesity evalu- ation and treatment: expert committee recommendations. Pediatrics 1998; 102:e29. 30. Consensus Development Conference Panel. Gastrointestinal surgery for se- vere obesity: Consensus Development Conference Statement. Ann Intern Med 1991;115:956-61. 504 50 Years Ago in The Journal of Pediatrics AORTIC ATRESIA WITH HYPOPLASIA OFTHE LEFT HEART AND AORTIC ARCH Friedman S, Murphy L, Ash R. J Pediatr 1951;38:354-68 At the middle of the 20th century, only 50 years ago, surgical treatments for congenital heart diseases had just been initiated, with ligation of patent ductus arteriosus, closed pulmonary valvotomy, resection and repair of coarctation of the aorta, and palliation of tetralogy of Fallot by the Blalock-Taussig shunt procedure. The “glory days” of open heart surgery with heart-lung machines were several years in the future. Nonetheless, cardiologists at the Children’s Hospital of Philadelphia described in careful clinical and anatomic detail 4 original cases of the hypoplastic left heart syndrome (HLHS) and summarized some 34 more cases from the previous literature. Despite the lack of cardiac catheterization results, the authors cor- rectly predicted the precise flow pattern of systemic venous and pulmonary venous blood. An important and still relevant observation was that the foramen ovale could close, despite the left heart hypoplasia, causing pulmonary capillary dilatation, findings confirmed by chest roentgenogram. The authors noted the terrible natural history, with death in the first week. Their postulate was that surgical correction in young infants might prove feasible. They discussed potential causes, including fetal inflammation or infection, and men- tioned that “defective development may arise from an imperfect germ cell or perhaps may result from some disturbance occurring during the course of organogenesis.” The recent elucidation of genetic mutations caus- ing congenital heart diseases such as HLHS in some infants has clarified the discussion. About 30 years elapsed, during which repair of all other types of congenital heart disease was successfully achieved. Finally, the pioneering and persistent work of William Norwood and colleagues, first at Boston Children’s Hospital and then, ironically, in Philadelphia, to provide palliative treatment for infants with the HLHS proved successful. Despite many tribulations, especially a distressingly high mortality rate for more than 15 years, which led many to recommend non-intervention until very recently, the Norwood procedure has come of age, with recent 1-year survival reported in the range of 65% to 80%. Although survivors of the HLHS have an unknown long-term outcome and many have required or will need cardiac transplantation, today we can contradict the conclusion of 50 years ago that “there appears to be little hope for success in sur- gical intervention” and that “the distortion of the hemodynamics is so profound…that little permanent benefit can be anticipated from surgery.” It has taken a long time, but permanent benefit does occur with surgical in- tervention. Arnold W. Strauss, MD Chairman, Department of Pediatrics Vanderbilt University Medical Center Nashville, TN 37232-2574 9/37/114023 doi:10.1067/mpd.2001.114023
Transcript of Aortic atresia with hypoplasia of the left heart and aortic arch
STRAUSS, BRADLEY,AND BROLIN THE JOURNAL OF PEDIATRICS
APRIL 2001
lowing gastric bypass for the treatmentof morbid obesity. Am Surg 1982;48:363-5.
23. Richards DS, Miller DK, GoodmanGN. Pregnancy after gastric bypassfor morbid obesity. J Reprod Med1987;32:172-6.
24. Gurewitsch ED, Smith-Levitin M,Mack J. Pregnancy following gastricbypass surgery for morbid obesity.Obstet Gynecol 1996;88:658-61.
25. Haddow JE, Hill LE, Kloza EM,Thanhauser D. Neural tube defectsafter gastric bypass [letter]. Lancet1986;1:1330.
26. Werler MM, Louik C, Shapiro S,Mitchell AA. Prepregnant weight inrelation to risk of neural tube defects.JAMA 1996;275:1089-92.
27. Knudsen LB, Kallen B. Gastric by-pass, pregnancy, and neural tube de-fects. Lancet 1986;2:227.
28. Borjeson M. Overweight children.Acta Paediatr 1962;51(suppl):1-76.
39. Barlow SE, Dietz WH. Obesity evalu-ation and treatment: expert committeerecommendations. Pediatrics 1998;102:e29.
30. Consensus Development ConferencePanel. Gastrointestinal surgery for se-vere obesity: Consensus DevelopmentConference Statement. Ann InternMed 1991;115:956-61.
504
50 Years Ago in The Journal of PediatricsAORTIC ATRESIA WITH HYPOPLASIA OF THE LEFT HEART AND AORTIC ARCH
Friedman S, Murphy L, Ash R. J Pediatr 1951;38:354-68
At the middle of the 20th century, only 50 years ago, surgical treatments for congenital heart diseases hadjust been initiated, with ligation of patent ductus arteriosus, closed pulmonary valvotomy, resection and repairof coarctation of the aorta, and palliation of tetralogy of Fallot by the Blalock-Taussig shunt procedure. The“glory days” of open heart surgery with heart-lung machines were several years in the future.
Nonetheless, cardiologists at the Children’s Hospital of Philadelphia described in careful clinical andanatomic detail 4 original cases of the hypoplastic left heart syndrome (HLHS) and summarized some 34more cases from the previous literature. Despite the lack of cardiac catheterization results, the authors cor-rectly predicted the precise flow pattern of systemic venous and pulmonary venous blood. An important andstill relevant observation was that the foramen ovale could close, despite the left heart hypoplasia, causingpulmonary capillary dilatation, findings confirmed by chest roentgenogram. The authors noted the terriblenatural history, with death in the first week. Their postulate was that surgical correction in young infantsmight prove feasible. They discussed potential causes, including fetal inflammation or infection, and men-tioned that “defective development may arise from an imperfect germ cell or perhaps may result from somedisturbance occurring during the course of organogenesis.” The recent elucidation of genetic mutations caus-ing congenital heart diseases such as HLHS in some infants has clarified the discussion.
About 30 years elapsed, during which repair of all other types of congenital heart disease was successfullyachieved. Finally, the pioneering and persistent work of William Norwood and colleagues, first at BostonChildren’s Hospital and then, ironically, in Philadelphia, to provide palliative treatment for infants with theHLHS proved successful. Despite many tribulations, especially a distressingly high mortality rate for morethan 15 years, which led many to recommend non-intervention until very recently, the Norwood procedurehas come of age, with recent 1-year survival reported in the range of 65% to 80%. Although survivors of theHLHS have an unknown long-term outcome and many have required or will need cardiac transplantation,today we can contradict the conclusion of 50 years ago that “there appears to be little hope for success in sur-gical intervention” and that “the distortion of the hemodynamics is so profound…that little permanent benefitcan be anticipated from surgery.” It has taken a long time, but permanent benefit does occur with surgical in-tervention.
Arnold W. Strauss, MDChairman, Department of Pediatrics
Vanderbilt University Medical CenterNashville, TN 37232-2574
9/37/114023doi:10.1067/mpd.2001.114023
