Since 1974, late results of the Mustard procedure for correcting complete transposition of the great arteries have been evaluated by cardiac catheterization, electrocardiography, roentgenography, history, and physical examination of 48 Mayo Clinic patients. Of these, 15 were studied I month to 2 years postoperatively because of clinical deterioration. The other 33 had been asymptomatic but were asked to return for hemodynamic reevaluation one-half to 1I years postoperatively. Of the asymptomatic group, 19 underwent exercise electrocardiography prior to

14. De Leval MR, McGoon DC, Wallace RB, Danielson GK, Mair DD: Management of truncal valvular regurgitation. Ann Surg 180: 427, 1974 15. Mair DD, Ritter DG, Davis GD, Wallace RB, Danielson GK, McGoon DC: Selection of patients with truncus arteriosus for surgical correction: Anatomic and hemodynamic considerations. Circulation 49: 144, 1974 16. Arai T, Tsuzuki Y, Nogi M, Kurashige K, Koyanagi H, Nishida H, Ikeda Y, Ichikawa H: Experimental study on bypass between the right ventricle and pulmonary artery, left ventricle and pulmonary artery, and left ventricle and aorta by means of homograft with valve. Bull Heart Inst Japan 9: 49, 1965 17. Rastelli GC, Titus JL, McGoon DC: Homograft of ascending aorta and aortic valve as a right ventricular outflow: An experimental approach to the repair of truncus arteriosus. Arch Surg 95: 698, 1967 18. DuShane JW: Cited by DuShane JW, Kirklin JW: Late results of the repair of ventricular septal defect on pulmonary vascular disease. In Advances in Cardiovascular Surgery. Editedby JW Kirklin. New York, Grune & Stratton, 1973, pp 9-16 19. Parker RK, McGoon DC, Danielson GK, Wallace RB, and Mair DD: Repair of truncus arteriosus in patients with prior banding of the pulmonary artery. Surgery 78: 761, 1975 20. Gelband H, Van Meter S, Gersony WM: Truncal valve abnormalities in infants with persistent truncus arteriosus: A clinicopathologic study. Circulation 45: 397, 1972 21. McGoon DC, Wallace RB, Danielson GK: Homografts in reconstruction of congenital cardiac anomalies: Expanded operability in complex congenital heart disease. Mayo Clin Proc 47: 101, 1972 22. McGoon DC, Wallace RB, Danielson GK: The Rastelli operation: Its indications and results. J Thorac Cardiovasc Surg 65: 65, 1973 23. Brawley RK, Gardner TJ, Donahoo JS, Neill CA, Rowe RD, Gott VL: Late results after right ventricular outflow tract reconstruction with aortic root homografts. J Thorac Cardiovasc Surg 64: 314, 1972 24. Kaplan S, McKinivan CE, Helmsworth JA, Benzing G III, Schwartz DC, Schreiber JT: Complications following homograft replacement of the right ventricular outflow tract. Ann Thorac Surg 18: 250, 1974 25. Merin G, McGoon DC: Reoperation after insertion of aortic homograft

studies in anatomy and function. Radiology 92: 939, 1969 11. Solinger R, Elbi F, Minhas K: Deductive echocardiographic analysis in infants with congenital heart disease. Circulation SO: 1284, 1974 12. Corya BC, Feigenbaum H, Rasmussen S, Black MJ: Anterior left ventricular wall echoes in coronary artery disease. Am J Cardiol 34: 652, 1974 13. McDonald IG, Feigenbaum H, Chang S: Analysis of left ventricular wall motion by reflected ultrasound. Circulation 46: 14, 1972 14. Jacobs JJ, Feigenbaum H, Corya BC, Philipps JF: Detection of left ventricular asynergy by echocardiography. Circulation 48: 263, 1973 15. Manoli SH: An intraventricular ultrasound method for measurement of left ventricular dimensions. IEEE Trans Biomed Eng 21: 333, 1974

Between 1973 and March 1989, 176 patients with tricuspid atresia had the Fontan procedure performed at the Mayo Clinic. Age range at the time of surgery was 7 months to 42 years, with 43 patients (24%) 16 years old or older. Hospital mortality rates were 17% (nine of 54) from 1973 through 1980 and 8% (10 of 122) from 1981 through 1989. There have been 10 late cardiovascular deaths. Postoperative follow-up of 139 survivors (range, 6 months to 14 years; mean, 5.5 years) revealed 91% to be in excellent or good condition and 9% to be in fair or poor condition. Patients in fair or poor condition had poor stamina and/or fluid retention with intermittent pleural effusion, ascites, and so on. Two factors that clearly influence operative and late results are preoperative pulmonary arteriolar resistance (Rpa) and left ventricular diastolic function. A preoperative catheterization index devised by adding Rp. to left ventricular end-diastolic pressure divided by QpI plus QsI may be helpful in selecting candidates most likely to survive and benefit from the Fontan operation. In our experience, if this index is less than 4.0, then the postoperative right atrial mean pressure will be 20 mm Hg or less, a circumstance associated with 95% early and 89% overall survival rates. (Circulation 1990;82(suppl IV):V-164-V-169)

14. De Leval MR, McGoon DC, Wallace RB, Danielson GK, Mair DD: Management of truncal valvular regurgitation. Ann Surg 180: 427, 1974 15. Mair DD, Ritter DG, Davis GD, Wallace RB, Danielson GK, McGoon DC: Selection of patients with truncus arteriosus for surgical correction: Anatomic and hemodynamic considerations. Circulation 49: 144, 1974 16. Arai T, Tsuzuki Y, Nogi M, Kurashige K, Koyanagi H, Nishida H, Ikeda Y, Ichikawa H: Experimental study on bypass between the right ventricle and pulmonary artery, left ventricle and pulmonary artery, and left ventricle and aorta by means of homograft with valve. Bull Heart Inst Japan 9: 49, 1965 17. Rastelli GC, Titus JL, McGoon DC: Homograft of ascending aorta and aortic valve as a right ventricular outflow: An experimental approach to the repair of truncus arteriosus. Arch Surg 95: 698, 1967 18. DuShane JW: Cited by DuShane JW, Kirklin JW: Late results of the repair of ventricular septal defect on pulmonary vascular disease. In Advances in Cardiovascular Surgery. Editedby JW Kirklin. New York, Grune & Stratton, 1973, pp 9-16 19. Parker RK, McGoon DC, Danielson GK, Wallace RB, and Mair DD: Repair of truncus arteriosus in patients with prior banding of the pulmonary artery. Surgery 78: 761, 1975 20. Gelband H, Van Meter S, Gersony WM: Truncal valve abnormalities in infants with persistent truncus arteriosus: A clinicopathologic study. Circulation 45: 397, 1972 21. McGoon DC, Wallace RB, Danielson GK: Homografts in reconstruction of congenital cardiac anomalies: Expanded operability in complex congenital heart disease. Mayo Clin Proc 47: 101, 1972 22. McGoon DC, Wallace RB, Danielson GK: The Rastelli operation: Its indications and results. J Thorac Cardiovasc Surg 65: 65, 1973 23. Brawley RK, Gardner TJ, Donahoo JS, Neill CA, Rowe RD, Gott VL: Late results after right ventricular outflow tract reconstruction with aortic root homografts. J Thorac Cardiovasc Surg 64: 314, 1972 24. Kaplan S, McKinivan CE, Helmsworth JA, Benzing G III, Schwartz DC, Schreiber JT: Complications following homograft replacement of the right ventricular outflow tract. Ann Thorac Surg 18: 250, 1974 25. Merin G, McGoon DC: Reoperation after insertion of aortic homograft

was proposed by Carlon et al.1 in 1951. In 1954, Glenn and Patifio2 described a modification of this anastomosis by suturing the superior vena cava (SVC) directly to the right pulmonary artery (RPA). Theoretical hemodynamic advantages of this method of palliation, compared with operations anastomosing a systemic artery to pulmonary artery (Blalock-Taussig, Potts shunt), were increased pulmonary blood flow accompanied by an absolute reduction in intracardiac right-to-left shunt and avoidance of excessive pulmonary blood flow.3 However, reports soon began to

BACKGROUND:Palliative atrial switch (PAS) procedures that reroute pulmonary and systemic venous drainage and leave a ventricular septal defect (VSD) open have been used in the treatment of deeply cyanotic patients who have severe pulmonary vascular obstructive disease (PVOD). Palliative atrial switch is beneficial for patients with transposition of the great arteries or other complex lesions with VSD who show higher arterial oxygen saturation in the pulmonary artery than in the aorta (transposition hemodynamics/unfavorable streaming). We reviewed the early and late results of PAS (Mustard, n = 25; Senning, n = 3) in patients at two institutions. METHODS:Between April 1965 and March 2000, PAS was performed in 28 cyanotic patients (18 male, 10 female). Median age was 10 years (range, 1 to 27). Mean preoperative pulmonary arterial pressure was 68 mm Hg (range, 30 to 121 mm Hg). Mean systemic arterial oxygen saturation was 65% (range, 47% to 80%). The majority of patients (95%) were in New York Heart Association (NYHA) functional class III or IV preoperatively. RESULTS:Overall early mortality was 21%; for patients after 1972 (n = 23), the early mortality was 8.7%. Mean follow-up was 8.3 years (maximum 20). Mean postoperative systemic arterial oxygen saturation was increased significantly to 88% (p < 0.0001). Late survival for early survivors at 5, 10, and 15 years respectively was 84% (59%, 97%), 64% (39%, 88%), and 54% (15%, 72%). The NYHA functional class was significantly improved; 94% of late survivors (n = 17) were in functional class I or II (p = 0.002). CONCLUSIONS:The PAS operation significantly improves systemic arterial oxygen saturation and quality of life in selected patients with transposition hemodynamics, VSD, and severe PVOD.

It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patient's pericardium, and this patient continues to do well. In the subsequent four decades, great advances have been made in the diagnosis, with regard to the often very complex anatomy of pulmonary blood supply that this extremely heterogeneous group of patients manifest, and their subsequent surgical management. Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. Although the ideal extracardiac conduit has yet to be developed, necessitating periodic conduit replacement in corrected patients, the late results in this group of patients are generally excellent, with many now achieving survival well into the adulthood years with good quality of life. Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.

The first successful surgical correction of truncus arteriosus (common arterial trunk) was performed in 1967 and long-term follow-up is now available on 137 operative survivors. Actuarial survival was 46.4% at 24 years. Late survival was statistically significantly improved in patients having correction during the first 2 years of life when compared to those corrected after the age of 2. Late mortality was secondary to reoperation, most commonly for truncal valve replacement, progressive pulmonary vascular obstructive disease and right ventricular failure, or progressive left ventricular deterioration and failure. Reoperation for isolated conduit replacement was associated with low mortality (one death in 54 reoperations) and morbidity. Reoperation for truncal valve replacement was frequently necessary in patients showing significant truncal incompetence immediately after operative correction, but had not been observed in any patients showing no or minimal incompetence at the time of initial hospital discharge. Of the 86 presently surviving patients, many of whom are now adults, 78 (91%) were NYHA Class I or II, capable of full-time school or employment, at the time of most recent follow-up. Operative correction of truncus arteriosus is now possible at low mortality during the first few months of life, and this is the approach now utilized in all centers to relieve the large left ventricular volume load and its detrimental effects on left ventricular function as soon as possible, as well as to minimize the risk of irreversible pulmonary vascular obstructive disease. With this present approach, late mortality and morbidity could be expected to be significantly less in infants operated today than the late results seen in our unique group of patients corrected, for the most part, at older ages, many even as adolescents or young adults. An infant born with truncus arteriosus today has an excellent chance of survival with good quality of life well into adulthood years.

The Rastelli operation, first performed in 1968, was developed for repair of transposition of the great arteries with associated ventricular septal defect and severe pulmonary stenosis. This operation includes placement of an intracardiac baffle to direct left ventricular blood to the aorta and an extracardiac valved conduit to establish continuity between the right ventricle and the pulmonary arteries. Over the last 3 decades, the Rastelli operation has been performed with a progressive decline in early mortality, and it remains the preferred repair for transposition, ventricular septal defect, and severe fixed valvular or subvalvular pulmonary stenosis. This chapter examines the late results of our 33-year experience with the Rastelli operation and describes our operative technique.

In summary, surgical management of patients with pulmonary atresia and intact ventricular septum is dictated by the patient's potential for an eventual two-ventricle repair. This potential is related to the size of the tricuspid valve and right ventricle and, on occasion, to the presence of right ventricular to coronary artery fistulae with accompanying coronary artery obstructive lesions. Neonatal management involves establishing right ventricular to pulmonary artery continuity and carrying out a simultaneous systemic to pulmonary artery shunt in a child with good potential for a two-ventricle repair, a repair that then can often be performed in the first year or two of life. In neonates in whom the tricuspid and right ventricle do not have good growth potential, a systemic to pulmonary artery shunt is performed to provided reliable palliation with an eye toward the Fontan procedure at age 2 to 3 years. A few patients will exhibit a tricuspid valve and right ventricle adequate to handle a portion, but not all, of the returning systemic venous circulation, and in such patients the “ventricle and one-half repair,” routing inferior vena caval blood through the right ventricle while carrying out a bidirectional cavopulmonary anastomosis to convey superior vena caval blood directly to the pulmonary arteries may be feasible. Patients having a two-ventricle repair would be expected to have an excellent long-term prognosis, similar to that being seen in patients now many years postrepair of tetralogy of Fallot (10). The very long-term results from the Fontan approach are less certain, few of these patients presently being >15 years postrepair. However, the results from our group of 40 patients having a Fontan operation for this condition would indicate that these patients do have an excellent chance to survive the operative procedure and to continue to lead good quality lives into, at least, the early adulthood years.

Grading Key***** = outstanding;**** = excellent;*** = good;** = fair;* = poor Grading Key***** = outstanding;**** = excellent;*** = good;** = fair;* = poor Type of Book: A multiauthored text, written by prominent physicians and scientists in the field, devoted to the diagnosis and treatment of congenital and acquired cardiovascular disease in infants, children, and adolescents. Scope of Book: An overview is provided of the current state-of-the-art knowledge and techniques in pediatric cardiology aimed toward the primary-care physician (pediatrician or family physician) or physician in training. Contents: Approximately two-thirds of the book is devoted to congenital heart disease. This portion has one chapter each on risk factors and syndromes in addition to those focusing on the separate diagnostic entities of the clinical examination, electrocardiography, echocardiography, and cardiac catheterization including interventional therapeutic catheterization techniques. The chapter on surgical treatment of congenital cardiovascular malformations is excellent and includes some extremely well-done diagrams of the operative procedures. One lengthy chapter deals with acquired heart disease in young patients, including Kawasaki disease, bacterial endocarditis, rheumatic fever, and myocarditis. The book concludes with chapters on pediatric cardiac arrhythmias, cardiopulmonary resuscitation in infants and children, steps taken during childhood to attempt to prevent coronary artery disease in adulthood, and a short appendix that presents a schematic approach to the diagnosis of congenital heart disease. Strengths: The chapters are constructed concisely and logically and are structured toward establishing a pragmatic approach to the management of these problems initially encountered by the primary-care physician. The diagrams and illustrations are well done, are appropriate, and substantially enhance the text. Deficiencies: The bibliographies provided at the conclusion of each chapter are limited and perhaps somewhat skewed toward the author's personal experience, but this is not a serious shortcoming. Recommended Readership: This book would be excellent for primary-care physicians who are responsible for the care of infants, children, and adolescents. It also would be an excellent source of information for medical students and residents. Overall Grading:****

Early mortality (EM) after nonfenestrated Fontan operation (NF) has been reported to be higher for young pts, specifically those less than 4 yrs old. In our recent experience, overall EM has decreased markedly. In order to determine if the risk of EM after NF is still significantly increased for young pts, we reviewed all 839 pts who had NF at our institution between 1973–92. During this period, 123 (15%) pts younger than age 4 yrs had a NF. EM for this group of pts was 22/123 (18%). During the last 20 yrs, EM after NF for pts younger than 4 yrs has decreased progressively at our institution: 197–77 = 50%, 1978–82 = 32%, 1983-86 = 20%, 1987–92 = 12% (p < 0.04). In the 3 early time periods, EM was consistently greater in pts younger than 4 yrs than in older pts. However, in our recent experience (1987–92), EM after NF for pts younger than 4 yrs [8/69 (12%)] is not significantly different from EM after NF for older pts [22/270 (8%)]. Many factors have contributed to the improved early outcome in young pts. Among these are the fact that in our earlier experience (1973–86), pts younger than 4 yrs frequently had NF on a non-elective basis [20/54 137%)] due to progressive clinical deterioration. Also, in the more recent period, selection criteria for NF have become more stringent and staging operations (i.e. cavopulmonary connections) have been more widely utilized. Several institutions have advocated atrial baffle fenestration to improve survival for high-risk Fontan pts including those less than 4 yrs old. However, our results with nonfenestrated Fontan operations indicate that with careful pt selection, children younger than age 4 yrs, have an operative risk which is not only improved, but similar to that observed in older pts. Therefore, any technical modification of the Fontan procedure must be evaluated in light of these recent improved results.

The modified Fontan operation (MF) is the preferred palliative procedure for heterotaxy pts (HP) with complex cyanotic heart disease. Early mortality (EM) after MF has been reported to be greater for HP In our recent experience (1987 – 92) EM after MF has improved dramatically. In order to determine if these improved results applied to HP, we reviewed all HP who had a MF atour institution between 1973 – 92 (n=111). From 1973–92, 111/839 (13%) pts having a MF were HP 154 (6.5%1 polysplenia, 57 (6.8%) asplenia]. HP were significantly older than non-HP at the time of MF (median age=11 yrs vs 9 yrs; p&lt;0.05). We compared EM after MF for HP in two time periods: early 11973–86 In=49)] and recent [1987–92 (n=62)]. EM decreased significantly in the recent group [9/62 (14.5%)] compared to the early group 121/49 (43%); p&lt;0.001]. In our early experience, asplenia pts had increased EM compared to polysplenia pts (65% vs 23%) but, recent experience shows that EM is similar for these two subsets of HP [asplenia: 5/34 (15%) vs polysplenia: 4/28 (14%)]. Many factors, including use of an intra-atrial tunnel, have contributed to these improved results.

of The Western Thoracic Surgical Association will be held June 22-25, 1994, at the Resort at Squaw Creek in Olympic Valley, California.The scientific sessions are open to all duly qualified physicians.

BACKGROUND:Although corrective surgery for tetralogy of Fallot has been available for more than 30 years, the occurrence of late sudden death in patients in whom surgery was apparently successful remains worrisome.METHODS:We studied long-term survival among 163 patients who survived 30 days after complete repair of tetralogy of Fallot, examining follow-up hospital records and death certificates when relevant.RESULTS:The overall 32-year actuarial survival rate among all patients who survived surgery was 86 percent, as compared with an expected rate of 96 percent in a control population matched for age and sex (P < 0.01). Thirty-year actuarial survival rates were calculated for the patient subgroups. The survival rates among patients less than 5 years old, 5 to 7 years old, and 8 to 11 years old were 90, 93, and 91 percent, respectively--slightly less than the expected rates (P < 0.001, P = 0.06, and P = 0.02). Among patients 12 years old or older at the time of surgery, the survival rate was 76 percent, as compared with an expected rate of 93 percent (P < 0.001). The performance of a palliative Blalock-Taussig shunt procedure before repair, unlike the performance of a Waterston or Potts shunt procedure, was not associated with reduced long-term survival, nor was the need for a trans-annular patch at the time of surgery. Independent predictors of long-term survival were older age at operation (P = 0.02) and a higher ratio of right ventricular to left ventricular systolic pressure after surgery (P = 0.008). Late sudden death from cardiac causes occurred in 10 patients during the 32-year period.CONCLUSIONS:Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.

From May 1960 to January 1992, 13 patients with cor triatriatum underwent surgical correction at the Mayo Clinic. Their ages ranged from 7 months to 57 years. Four patients had isolated cor triatriatum; three others had an associated patent foramen ovale or atrial septal defect. Six patients had major associated cardiac anomalies. In patients who had isolated cor triatriatum or associated anomalies with high pulmonary blood flow, the mean gradient across the left atrial diaphragm was 24.4 mm Hg, in comparison with 11.6 mm Hg in patients who had cor triatriatum and a connection between the common pulmonary venous chamber and the right atrium or associated cardiac anomalies that restricted pulmonary blood flow. In all 11 patients who underwent angiography, echocardiography, or both at our institution, the correct diagnosis was established preoperatively. Currently, echocardiography is the procedure of choice for diagnosing cor triatriatum. The diaphragm was excised through a left atrial approach in seven patients and through a right atriotomy in six. All associated anomalies were corrected. One critically ill patient who underwent an emergency operation in 1963 died early postoperatively, and one patient with chromosomal abnormalities and multiple cardiac defects died 2 months after an uneventful postoperative course. At the time of follow-up of the 11 long-term survivors, 9 were in New York Heart Association functional class I, and 2 (both of whom had major associated cardiac anomalies) were in class II. Postoperative angiography or echocardiography (in nine patients) showed no residual interatrial shunt or recurrent left atrial diaphragm.