- Email: [email protected]
Effectiveness of cardiac surgery in trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium)
quantification of the left ventricle by two-dimensional echocardiography. J Am Soc Echocardiogr 1989;2:358 –367. 10. Vogel M, Staller W, Buhlmeyer K. Left ventricular myocardial mass determined by cross-sectional echocardiography in normal newborns, infants, and children. Pediatr Cardiol 1991;12:143–149. 11. Du Bois D, Du Bois EF. A formula to estimate the approximate surface area if height and weight be known. Arch Intern Med 1916;17:863–871. 12. Fulton RM, Hutchinson EC, Jones AM. Ventricular weight in cardiac hypertrophy. Br Heart J 1952;14:413–420. 13. Fast J, Jacobs S. Limits of reproducibility of cross-sectional echocardiographic measurements of left ventricular muscle mass. Int J Cardiol 1991;31: 213–216. 14. Youn H-J, Rokosh G, Lester SJ, Simpson P, Schiller NB, Foster E. Twodimensional echocardiography with a 15-MHz transducer is a promising alternative for in vivo measurement of left ventricular mass in mice. J Am Soc Echocardiogr 1999;12:70 –75. 15. Collins KA, Korcarz CE, Shroff SG, Bednarez JE, Fentzke RC, Lin H, Leiden
JM, Lang RM. Accuracy of echocardiographic estimates of left ventricular mass in mice. Am J Physiol Heart Circ Physiol 2001;280:H1954 –H1962. 16. Kiatchoosakun S, Restivo J, Kirkpatrick D, Hoit BD. Assessment of left ventricular mass in mice: comparison between two-dimensional and M-mode echocardiography. Echocardiography 2002;19:199 –205. 17. Mercier JC, DiSessa TG, Jarmakani JM, Nakanishi T, Hiraishi S, Isabel-Jones J, Friedman WF. Two-dimensional echocardiographic assessment of left ventricular volumes and ejection fraction in children. Circulation 1982;65:962–969. 18. Sandstede J, Lipke C, Beer M, Hofman S, Pabst T, Kenn W, Neubauer S, Hahn D. Age- and gender-specific differences in left and right ventricular cardiac function and mass determined by cine magnetic resonance imaging. Eur Radiol 2000;10:438 –442. 19. Byrd BF, Wahr D, Wang YS, Bouchard A, Schiller NB. Left ventricular mass and volume/mass ratio determined by two-dimensional echocardiography in normal adults. J Am Coll Cardiol 1985;6:1021–1025. 20. Nagasawa H, Arakaki Y, Yamada O, Nakajima T, Kamiya T. Longitudinal observations of left ventricular end-diastolic dimension in children using echocardiography. Pediatr Cardiol 1996;17:169 –174.
Effectiveness of Cardiac Surgery in Trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium) Eric M. Graham, MD, Scott M. Bradley, MD, Girish S. Shirali, Christine B. Hills, BA, and Andrew M. Atz, MD Because of severely reduced lifespan in children with trisomies 13 and 18, surgical repair of congenital heart lesions has rarely been offered. With data from a multicenter registry, we report 35 cases of cardiac surgery in infants and children with trisomy 13 or 18 with a 91% hospital survival rate. Those patients without an extended preoperative ventilatory requirement did not require prolonged mechanical ventilation after surgery. 䊚2004 by Excerpta Medica, Inc. (Am J Cardiol 2004;93:801– 803)
P
atients with trisomies 13 and 18 frequently have multiple congenital anomalies and a markedly reduced lifespan. Congenital heart disease occurs in 60% to 90% of these patients.1–3 The most common defects are ventricular septal defect (VSD), atrial septal defect, and patent ductus arteriosus (PDA),4 – 6 although a variety of other lesions, including complicated single-ventricle defects, have been reported.2 Population-based studies have shown that median survival after birth ranges from 3 to 14 days,2,3,7,8 with 5% to 10% surviving ⬎1 year.8 However, observational studies have reported an average survival rate of 12.8 and 6.6 months for trisomies 13 and 18, respectively, with almost 40% alive at 12 months and ⬎10% alive at 5 years.1 Historically, congenital heart disease From the Divisions of Pediatric Cardiology and Cardiothoracic Surgery, Medical University of South Carolina, Charleston, South Carolina; and the Department of Pediatrics and Pediatric Cardiac Care Consortium, University of Minnesota School of Medicine, Minneapolis, Minnesota. Dr. Atz’s address is: Pediatric Cardiac Intensive Care, Children’s Heart Program of South Carolina, Medical University of South Carolina, 165 Ashley Avenue, PO Box 250915, Charleston, South Carolina 29425. E-mail: [email protected]. Manuscript received August 26, 2003; revised manuscript received and accepted December 2, 2003. ©2004 by Excerpta Medica, Inc. All rights reserved. The American Journal of Cardiology Vol. 93 March 15, 2004
MBBS,
has been considered a major cause of early death in these infants, and the overall prognosis so poor that cardiac surgery has been offered infrequently. More recent studies have determined central apnea to be the primary cause of death.2,3 It is currently less clear as to whether the presence of a heart defect affects survival.8 Outcome data after cardiac surgery in this population have been limited to single-center case reports.1,9 –11 This report reviews the results of cardiac surgery for infants and children with trisomies 13 and 18 by using the multicenter Pediatric Cardiac Care Consortium cardiac registry. •••
The Pediatric Cardiac Care Consortium has collected data on infants and children with cardiac anomalies since 1982. The registry currently includes data on ⬎83,000 patients from 48 centers in the United States, Canada, and Europe. Participating centers submit a data form and brief patient history for each child undergoing a cardiac operation or catheterization. For this study, the Pediatric Cardiac Care Consortium database was searched for all children (⬍18 years) with diagnostic codes trisomy 13 and trisomy 18. Cytogenetic confirmation was verified in 70 cases. Further analysis was limited to the 35 patients who underwent cardiac surgery (trisomy 13, n ⫽ 11; trisomy 18, n ⫽ 24). Most patients had multiple heart lesions. Patients were stratified into 5 primary diagnostic categories based on the most hemodynamically significant component: VSD (n ⫽ 20), tetralogy of Fallot (n ⫽ 6), coarctation with or without VSD (n ⫽ 4), isolated PDA (n ⫽ 3), and atrioventricular septal defect (AVSD; n ⫽ 2). Distributions of diagnosis, weight at surgery, and length of stay were no different between those with trisomy 13 and those with trisomy 18 (Table 1). Complete hemodynamic repair was performed in 21 patients (12 with VSD, 3 with coarcta0002-9149/04/$–see front matter doi:10.1016/j.amjcard.2003.12.012
801
(91%) were discharged alive, 28 to home and 4 to referring institutions. Three patients died, all of whom had Total trisomy 18: an 11-day-old infant on (n ⫽ 35) the evening after VSD repair, a 3.7 (2.1–16.0) 5-year-old child 46 days after com14/21 plete repair of tetralogy of Fallot, and 20 a 6-year-old child 9 days after shunt 6 placement for tetralogy of Fallot. 4 3 Given the potential effect of cen2 tral apnea on mortality in these pa128 (4–2,479) tients, we also evaluated outcome 10 (3–48) based on preoperation ventilatory re32 (91%) quirements. Patients were divided into 2 groups: those requiring mechanical ventilation for ⬎2 days (Figure 1) and those requiring mechanical ventilation for ⬍2 days before surgery (Figure 2). Nine patients were ventilated mechanically for ⬎2 days before surgery. Of these, 1 patient died, 3 were extubated before discharge, and 5 remained mechanically ventilated at the time of discharge. In stark contrast, of the 26 patients who were not mechanically ventilated or ventilated ⱕ2 days before surgery, all 24 survivors were extubated before discharge.
TABLE 1 Characteristics at the Time of Initial Surgery in 35 Patients With Trisomies 13 and 18*
Weight (kg) Male/female Ventricular septal defect Tetralogy of Fallot Coarctation of aorta Isolated patent ductus arteriosus Atrioventricular septal defect Age at surgery (d) Hospital stay (d) Hospital survival
Trisomy 13 (n ⫽ 11)
Trisomy 18 (n ⫽ 24)
3.8 (2.8–11.3) 6/5 6 1 2 1 1 77 (4–2,375) 11 (3–48) 11 (100%)
3.6 (2.1–16.0) 8/16 14 5 2 2 1 145 (6–2,479) 9 (4–27) 21 (86%)
*Continuous variables are expressed as median (range).
•••
FIGURE 1. In patients mechanically ventilated before surgery for >2 days, only 3 of 8 survivors were extubated at the time of hospital discharge.
FIGURE 2. Of those patients mechanically ventilated for <2 days before cardiac surgery, all survivors were discharged home without mechanical ventilatory assistance.
tion, 3 with PDA, 2 with tetralogy of Fallot, and 1 with AVSD). Four patients underwent palliative surgery with subsequent complete repair, and all had pulmonary artery band placement for VSD. Ten patients underwent palliative surgery only, including 5 pulmonary artery bands (3 with VSD, 1 with coarctation and VSD, and 1 with AVSD), 4 systemic-topulmonary artery shunts (4 with tetralogy of Fallot), and 1 PDA ligation in 1 patient with VSD and PDA. Of the 35 patients who underwent cardiac surgery, 32 802 THE AMERICAN JOURNAL OF CARDIOLOGY姞
VOL. 93
This retrospective analysis describes the largest series to date of patients with trisomy 13 or 18 who have undergone cardiac surgery. Thirty-five infants and children underwent cardiac surgery, with a 91% hospital survival rate. Although longevity in patients with trisomy 13 or 18 remains limited, families, health care providers, and support groups are increasingly interested in maximizing the quality of life for infants and children with these disorders. Increasing numbers of affected children are receiving noncardiac surgical intervention, with an average of 2 surgeries per year for each patient surviving to age 1 year, including complicated abdominal and neurologic procedures.1 Our findings demonstrate that most patients with trisomy 13 or 18 can survive palliative and corrective heart surgeries. Those without a prolonged preoperative ventilatory requirement are unlikely to require continued mechanical ventilation after surgery. The present study was not designed to determine whether (or how much) lifespan is increased by performing cardiac surgery on these patients. Significant changes in attitudes, diagnosis, and health care over the 18 years required to accumulate this series confound the ability to compare this group with those who did not undergo cardiac surgery. The present study is not unique in this limitation. Recently published analyses of mortality in cases of trisomy 13 or 18 involving thousands of patients could not determine whether prolonged survival was related to more aggressive care.8 We did not address the issue of improved quality of life after surgery, although some improvements in this area might be expected. Information is provided to the Pediatric Cardiac Care Consortium registry on a voluntary basis and does not include follow-up information. Therefore, some cases may not be represented. There is inherent ascertainment bias toward patients who MARCH 15, 2004
survived past the neonatal period, so the present results cannot necessarily be extended to the population at large (e.g., a fetus diagnosed prenatally). 1. Baty BJ, Blackburn BL, Carey JC. Natural history of trisomy 18 and trisomy 13: I. Growth, physical assessment, medical histories, survival, and recurrence risk. Am J Med Genet 1994;49:175–188. 2. Embleton ND, Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 18. Arch Dis Child 1996;75:F38 –F41. 3. Martlew RA, Sharples A. Anaesthesia in a child with Patau’s syndrome. Anaesthesia 1995;50:980 –982. 4. Matsuoka R, Misugi K, Goto A, Gilbert EF, Ando M. Congenital heart anomalies in the trisomy 18 syndrome, with reference to congenital polyvalvular disease. Am J Med Genet 1983;14:657–668.
5. Musewe NN, Alexander DJ, Teshima I, Smallhorn JF, Freedom RM. Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18. J Am Coll Cardiol 1990;15:673–677. 6. Root S, Carey JC. Survival in trisomy 18. Am J Med Genet 1994;49:170 –174. 7. Stromme P, Thaulow E, Geiran O. Cardiac surgery in a girl with trisomy 13. Cardiol Young 2000;10:638 –640. 8. Van Dyke DC, Allen M. Clinical management considerations in long-term survivors with trisomy 18. Pediatrics 1990;85:753–759. 9. Van Praagh S, Truman T, Firpo A, Bano-Rodrigo A, Fried R, McManus B, Engle MA, Van Praagh R. Cardiac malformations in trisomy-18: a study of 41 postmortem cases. J Am Coll Cardiol 1989;13:1586 –1597. 10. Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 13. Arch Dis Child 1994;71:343–345. 11. Rasmussen SA, Wong LC, Yang Q, May KM, Friedman JM. Populationbased analyses of mortality in trisomy 13 and trisomy 18. Pediatrics 2003;111: 777–784.
Risk of Recoarctation Should Not Be a Deciding Factor in the Timing of Coarctation Repair Jeffrey M. Pearl, MD, Peter B. Manning, MD, Cheri Franklin, Robert Beekman, MD, and Linda Cripe, MD To determine whether early coarctation repair is a significant risk for recoarctation in the modern era, 120 patients, including 87 infants, who underwent isolated coarctation repair at a single institution, were reviewed. At a mean follow-up of 44.4 months, there have were no late reoperations, and 2 patients required balloon aortoplasty. The overall incidence of late reintervention was 1.7%, with only 2.4% (2 of 83) in those <1 year old. 䊚2004 by Excerpta Medica, Inc. (Am J Cardiol 2004;93:803– 805)
epair of native coarctation of the aorta has become a routine, low-risk operation with predictR able outcomes. Diagnosis is made currently and 1,2
frequently during early infancy secondary to improved diagnostic modalities. Traditional thought has been to delay elective repair due to reported experience demonstrating a higher incidence of recoarctation associated with early repair.3–5 However, delay in repair is associated with an increased risk of long-term hypertension,6 – 8 heightened parental anxiety, and additional medical costs associated with medical surveillance before repair. Influenced by low rates of recurrence, there has been a recent trend toward early elective repair in some centers. To guide our own practice and to determine whether early elective repair at the time of diagnosis is a reasonable approach, we reviewed our institutional results. ••• From the Division of Pediatric Cardiothoracic Surgery, Children’s Hospital Medical Center, and the Department of Surgery, University of Cincinnati; and the Division of Pediatric Cardiology, Children’s Hospital Medical Center, Cincinnati, Ohio. Dr. Pearl’s address is: Division of Pediatric Cardiothoracic Surgery, Children’s Hospital Medical Center, 3333 Burnet Avenue, OSB-3, Cincinnati, OH 45229. E-mail: [email protected]. Manuscript received September 26, 2003; revised manuscript received and accepted November 26, 2003. ©2004 by Excerpta Medica, Inc. All rights reserved. The American Journal of Cardiology Vol. 93 March 15, 2004
RN, MSN, PNP,
All isolated coarctation repairs during the 6-year period from January 1997 to January 2003 were included. One-hundred twenty patients met inclusion criteria; 87 were ⬍1 year of age, with 64 of these being neonates (⬍30 days). Additional cardiac diagnoses were present in 50%, with bicuspid aortic valve (n ⫽ 66) and ventricular septal defect (n ⫽ 29) being most common. An extended end-to-end anastomosis was used in nearly all patients (n ⫽ 117). A subclavian flap aortoplasty was used in 3 patients secondary to unfavorable arch anatomy for clamping or a distal coarctation. All obvious ductal tissue was removed, and a continuous running anastomosis was performed with 7.0 or 6.0 absorbable suture (PDS or Maxon; 70%) or 6.0 polypropylene suture (30%), depending on the patient’s size and the surgeon’s preference. All patients had 4 extremity blood pressure measurements after repair and 75% had echocardiography before discharge. A mean systolic pressure gradient ⬎10 mm Hg by blood pressure cuff or a peak systolic echo gradient ⬎15 mm Hg was considered residual or recurrent coarctation. Most recent upper and lower limb cuff blood pressures and echocardiographic evaluation were reviewed. Late hypertension was defined as the need for chronic antihypertensive medication. Late reintervention was considered in patients with a cuff blood pressure gradient of ⬎30 to 40 mm Hg with a corresponding echocardiographic gradient. Recurrence and degree of obstruction were confirmed by catheterization and angiography. There were 2 early deaths and 1 late death unrelated to the coarctation repair in infants with additional cardiac and noncardiac anomalies. The 2 early deaths included 1 neonate with collodion syndrome and 1 neonate with a chromosomal anomaly and multiple congenital defects. The late death was secondary to pulmonary hypertension. Two neonates required very early reoperation for significant arch hypoplasia, 0002-9149/04/$–see front matter doi:10.1016/j.amjcard.2003.11.064
803
JM, Lang RM. Accuracy of echocardiographic estimates of left ventricular mass in mice. Am J Physiol Heart Circ Physiol 2001;280:H1954 –H1962. 16. Kiatchoosakun S, Restivo J, Kirkpatrick D, Hoit BD. Assessment of left ventricular mass in mice: comparison between two-dimensional and M-mode echocardiography. Echocardiography 2002;19:199 –205. 17. Mercier JC, DiSessa TG, Jarmakani JM, Nakanishi T, Hiraishi S, Isabel-Jones J, Friedman WF. Two-dimensional echocardiographic assessment of left ventricular volumes and ejection fraction in children. Circulation 1982;65:962–969. 18. Sandstede J, Lipke C, Beer M, Hofman S, Pabst T, Kenn W, Neubauer S, Hahn D. Age- and gender-specific differences in left and right ventricular cardiac function and mass determined by cine magnetic resonance imaging. Eur Radiol 2000;10:438 –442. 19. Byrd BF, Wahr D, Wang YS, Bouchard A, Schiller NB. Left ventricular mass and volume/mass ratio determined by two-dimensional echocardiography in normal adults. J Am Coll Cardiol 1985;6:1021–1025. 20. Nagasawa H, Arakaki Y, Yamada O, Nakajima T, Kamiya T. Longitudinal observations of left ventricular end-diastolic dimension in children using echocardiography. Pediatr Cardiol 1996;17:169 –174.
Effectiveness of Cardiac Surgery in Trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium) Eric M. Graham, MD, Scott M. Bradley, MD, Girish S. Shirali, Christine B. Hills, BA, and Andrew M. Atz, MD Because of severely reduced lifespan in children with trisomies 13 and 18, surgical repair of congenital heart lesions has rarely been offered. With data from a multicenter registry, we report 35 cases of cardiac surgery in infants and children with trisomy 13 or 18 with a 91% hospital survival rate. Those patients without an extended preoperative ventilatory requirement did not require prolonged mechanical ventilation after surgery. 䊚2004 by Excerpta Medica, Inc. (Am J Cardiol 2004;93:801– 803)
P
atients with trisomies 13 and 18 frequently have multiple congenital anomalies and a markedly reduced lifespan. Congenital heart disease occurs in 60% to 90% of these patients.1–3 The most common defects are ventricular septal defect (VSD), atrial septal defect, and patent ductus arteriosus (PDA),4 – 6 although a variety of other lesions, including complicated single-ventricle defects, have been reported.2 Population-based studies have shown that median survival after birth ranges from 3 to 14 days,2,3,7,8 with 5% to 10% surviving ⬎1 year.8 However, observational studies have reported an average survival rate of 12.8 and 6.6 months for trisomies 13 and 18, respectively, with almost 40% alive at 12 months and ⬎10% alive at 5 years.1 Historically, congenital heart disease From the Divisions of Pediatric Cardiology and Cardiothoracic Surgery, Medical University of South Carolina, Charleston, South Carolina; and the Department of Pediatrics and Pediatric Cardiac Care Consortium, University of Minnesota School of Medicine, Minneapolis, Minnesota. Dr. Atz’s address is: Pediatric Cardiac Intensive Care, Children’s Heart Program of South Carolina, Medical University of South Carolina, 165 Ashley Avenue, PO Box 250915, Charleston, South Carolina 29425. E-mail: [email protected]. Manuscript received August 26, 2003; revised manuscript received and accepted December 2, 2003. ©2004 by Excerpta Medica, Inc. All rights reserved. The American Journal of Cardiology Vol. 93 March 15, 2004
MBBS,
has been considered a major cause of early death in these infants, and the overall prognosis so poor that cardiac surgery has been offered infrequently. More recent studies have determined central apnea to be the primary cause of death.2,3 It is currently less clear as to whether the presence of a heart defect affects survival.8 Outcome data after cardiac surgery in this population have been limited to single-center case reports.1,9 –11 This report reviews the results of cardiac surgery for infants and children with trisomies 13 and 18 by using the multicenter Pediatric Cardiac Care Consortium cardiac registry. •••
The Pediatric Cardiac Care Consortium has collected data on infants and children with cardiac anomalies since 1982. The registry currently includes data on ⬎83,000 patients from 48 centers in the United States, Canada, and Europe. Participating centers submit a data form and brief patient history for each child undergoing a cardiac operation or catheterization. For this study, the Pediatric Cardiac Care Consortium database was searched for all children (⬍18 years) with diagnostic codes trisomy 13 and trisomy 18. Cytogenetic confirmation was verified in 70 cases. Further analysis was limited to the 35 patients who underwent cardiac surgery (trisomy 13, n ⫽ 11; trisomy 18, n ⫽ 24). Most patients had multiple heart lesions. Patients were stratified into 5 primary diagnostic categories based on the most hemodynamically significant component: VSD (n ⫽ 20), tetralogy of Fallot (n ⫽ 6), coarctation with or without VSD (n ⫽ 4), isolated PDA (n ⫽ 3), and atrioventricular septal defect (AVSD; n ⫽ 2). Distributions of diagnosis, weight at surgery, and length of stay were no different between those with trisomy 13 and those with trisomy 18 (Table 1). Complete hemodynamic repair was performed in 21 patients (12 with VSD, 3 with coarcta0002-9149/04/$–see front matter doi:10.1016/j.amjcard.2003.12.012
801
(91%) were discharged alive, 28 to home and 4 to referring institutions. Three patients died, all of whom had Total trisomy 18: an 11-day-old infant on (n ⫽ 35) the evening after VSD repair, a 3.7 (2.1–16.0) 5-year-old child 46 days after com14/21 plete repair of tetralogy of Fallot, and 20 a 6-year-old child 9 days after shunt 6 placement for tetralogy of Fallot. 4 3 Given the potential effect of cen2 tral apnea on mortality in these pa128 (4–2,479) tients, we also evaluated outcome 10 (3–48) based on preoperation ventilatory re32 (91%) quirements. Patients were divided into 2 groups: those requiring mechanical ventilation for ⬎2 days (Figure 1) and those requiring mechanical ventilation for ⬍2 days before surgery (Figure 2). Nine patients were ventilated mechanically for ⬎2 days before surgery. Of these, 1 patient died, 3 were extubated before discharge, and 5 remained mechanically ventilated at the time of discharge. In stark contrast, of the 26 patients who were not mechanically ventilated or ventilated ⱕ2 days before surgery, all 24 survivors were extubated before discharge.
TABLE 1 Characteristics at the Time of Initial Surgery in 35 Patients With Trisomies 13 and 18*
Weight (kg) Male/female Ventricular septal defect Tetralogy of Fallot Coarctation of aorta Isolated patent ductus arteriosus Atrioventricular septal defect Age at surgery (d) Hospital stay (d) Hospital survival
Trisomy 13 (n ⫽ 11)
Trisomy 18 (n ⫽ 24)
3.8 (2.8–11.3) 6/5 6 1 2 1 1 77 (4–2,375) 11 (3–48) 11 (100%)
3.6 (2.1–16.0) 8/16 14 5 2 2 1 145 (6–2,479) 9 (4–27) 21 (86%)
*Continuous variables are expressed as median (range).
•••
FIGURE 1. In patients mechanically ventilated before surgery for >2 days, only 3 of 8 survivors were extubated at the time of hospital discharge.
FIGURE 2. Of those patients mechanically ventilated for <2 days before cardiac surgery, all survivors were discharged home without mechanical ventilatory assistance.
tion, 3 with PDA, 2 with tetralogy of Fallot, and 1 with AVSD). Four patients underwent palliative surgery with subsequent complete repair, and all had pulmonary artery band placement for VSD. Ten patients underwent palliative surgery only, including 5 pulmonary artery bands (3 with VSD, 1 with coarctation and VSD, and 1 with AVSD), 4 systemic-topulmonary artery shunts (4 with tetralogy of Fallot), and 1 PDA ligation in 1 patient with VSD and PDA. Of the 35 patients who underwent cardiac surgery, 32 802 THE AMERICAN JOURNAL OF CARDIOLOGY姞
VOL. 93
This retrospective analysis describes the largest series to date of patients with trisomy 13 or 18 who have undergone cardiac surgery. Thirty-five infants and children underwent cardiac surgery, with a 91% hospital survival rate. Although longevity in patients with trisomy 13 or 18 remains limited, families, health care providers, and support groups are increasingly interested in maximizing the quality of life for infants and children with these disorders. Increasing numbers of affected children are receiving noncardiac surgical intervention, with an average of 2 surgeries per year for each patient surviving to age 1 year, including complicated abdominal and neurologic procedures.1 Our findings demonstrate that most patients with trisomy 13 or 18 can survive palliative and corrective heart surgeries. Those without a prolonged preoperative ventilatory requirement are unlikely to require continued mechanical ventilation after surgery. The present study was not designed to determine whether (or how much) lifespan is increased by performing cardiac surgery on these patients. Significant changes in attitudes, diagnosis, and health care over the 18 years required to accumulate this series confound the ability to compare this group with those who did not undergo cardiac surgery. The present study is not unique in this limitation. Recently published analyses of mortality in cases of trisomy 13 or 18 involving thousands of patients could not determine whether prolonged survival was related to more aggressive care.8 We did not address the issue of improved quality of life after surgery, although some improvements in this area might be expected. Information is provided to the Pediatric Cardiac Care Consortium registry on a voluntary basis and does not include follow-up information. Therefore, some cases may not be represented. There is inherent ascertainment bias toward patients who MARCH 15, 2004
survived past the neonatal period, so the present results cannot necessarily be extended to the population at large (e.g., a fetus diagnosed prenatally). 1. Baty BJ, Blackburn BL, Carey JC. Natural history of trisomy 18 and trisomy 13: I. Growth, physical assessment, medical histories, survival, and recurrence risk. Am J Med Genet 1994;49:175–188. 2. Embleton ND, Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 18. Arch Dis Child 1996;75:F38 –F41. 3. Martlew RA, Sharples A. Anaesthesia in a child with Patau’s syndrome. Anaesthesia 1995;50:980 –982. 4. Matsuoka R, Misugi K, Goto A, Gilbert EF, Ando M. Congenital heart anomalies in the trisomy 18 syndrome, with reference to congenital polyvalvular disease. Am J Med Genet 1983;14:657–668.
5. Musewe NN, Alexander DJ, Teshima I, Smallhorn JF, Freedom RM. Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18. J Am Coll Cardiol 1990;15:673–677. 6. Root S, Carey JC. Survival in trisomy 18. Am J Med Genet 1994;49:170 –174. 7. Stromme P, Thaulow E, Geiran O. Cardiac surgery in a girl with trisomy 13. Cardiol Young 2000;10:638 –640. 8. Van Dyke DC, Allen M. Clinical management considerations in long-term survivors with trisomy 18. Pediatrics 1990;85:753–759. 9. Van Praagh S, Truman T, Firpo A, Bano-Rodrigo A, Fried R, McManus B, Engle MA, Van Praagh R. Cardiac malformations in trisomy-18: a study of 41 postmortem cases. J Am Coll Cardiol 1989;13:1586 –1597. 10. Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 13. Arch Dis Child 1994;71:343–345. 11. Rasmussen SA, Wong LC, Yang Q, May KM, Friedman JM. Populationbased analyses of mortality in trisomy 13 and trisomy 18. Pediatrics 2003;111: 777–784.
Risk of Recoarctation Should Not Be a Deciding Factor in the Timing of Coarctation Repair Jeffrey M. Pearl, MD, Peter B. Manning, MD, Cheri Franklin, Robert Beekman, MD, and Linda Cripe, MD To determine whether early coarctation repair is a significant risk for recoarctation in the modern era, 120 patients, including 87 infants, who underwent isolated coarctation repair at a single institution, were reviewed. At a mean follow-up of 44.4 months, there have were no late reoperations, and 2 patients required balloon aortoplasty. The overall incidence of late reintervention was 1.7%, with only 2.4% (2 of 83) in those <1 year old. 䊚2004 by Excerpta Medica, Inc. (Am J Cardiol 2004;93:803– 805)
epair of native coarctation of the aorta has become a routine, low-risk operation with predictR able outcomes. Diagnosis is made currently and 1,2
frequently during early infancy secondary to improved diagnostic modalities. Traditional thought has been to delay elective repair due to reported experience demonstrating a higher incidence of recoarctation associated with early repair.3–5 However, delay in repair is associated with an increased risk of long-term hypertension,6 – 8 heightened parental anxiety, and additional medical costs associated with medical surveillance before repair. Influenced by low rates of recurrence, there has been a recent trend toward early elective repair in some centers. To guide our own practice and to determine whether early elective repair at the time of diagnosis is a reasonable approach, we reviewed our institutional results. ••• From the Division of Pediatric Cardiothoracic Surgery, Children’s Hospital Medical Center, and the Department of Surgery, University of Cincinnati; and the Division of Pediatric Cardiology, Children’s Hospital Medical Center, Cincinnati, Ohio. Dr. Pearl’s address is: Division of Pediatric Cardiothoracic Surgery, Children’s Hospital Medical Center, 3333 Burnet Avenue, OSB-3, Cincinnati, OH 45229. E-mail: [email protected]. Manuscript received September 26, 2003; revised manuscript received and accepted November 26, 2003. ©2004 by Excerpta Medica, Inc. All rights reserved. The American Journal of Cardiology Vol. 93 March 15, 2004
RN, MSN, PNP,
All isolated coarctation repairs during the 6-year period from January 1997 to January 2003 were included. One-hundred twenty patients met inclusion criteria; 87 were ⬍1 year of age, with 64 of these being neonates (⬍30 days). Additional cardiac diagnoses were present in 50%, with bicuspid aortic valve (n ⫽ 66) and ventricular septal defect (n ⫽ 29) being most common. An extended end-to-end anastomosis was used in nearly all patients (n ⫽ 117). A subclavian flap aortoplasty was used in 3 patients secondary to unfavorable arch anatomy for clamping or a distal coarctation. All obvious ductal tissue was removed, and a continuous running anastomosis was performed with 7.0 or 6.0 absorbable suture (PDS or Maxon; 70%) or 6.0 polypropylene suture (30%), depending on the patient’s size and the surgeon’s preference. All patients had 4 extremity blood pressure measurements after repair and 75% had echocardiography before discharge. A mean systolic pressure gradient ⬎10 mm Hg by blood pressure cuff or a peak systolic echo gradient ⬎15 mm Hg was considered residual or recurrent coarctation. Most recent upper and lower limb cuff blood pressures and echocardiographic evaluation were reviewed. Late hypertension was defined as the need for chronic antihypertensive medication. Late reintervention was considered in patients with a cuff blood pressure gradient of ⬎30 to 40 mm Hg with a corresponding echocardiographic gradient. Recurrence and degree of obstruction were confirmed by catheterization and angiography. There were 2 early deaths and 1 late death unrelated to the coarctation repair in infants with additional cardiac and noncardiac anomalies. The 2 early deaths included 1 neonate with collodion syndrome and 1 neonate with a chromosomal anomaly and multiple congenital defects. The late death was secondary to pulmonary hypertension. Two neonates required very early reoperation for significant arch hypoplasia, 0002-9149/04/$–see front matter doi:10.1016/j.amjcard.2003.11.064
803