Heart transplantation in children: risk factors of early and late mortality

In order to identify predictive risk factors of poor outcome following heart transplantation in children, we performed a retrospective analysis of our pediatric recipient population: 31 children, aged 15 days to 15 years (mean = 5.2 +/- 4.9 years). The preoperative diagnosis was cardiomyopathy in 17 (55%), congenital heart disease in 13 (42%) and end-stage valvular disease in 1 (3%). There were 5 operative deaths: hyperacute rejection (2), low cardiac output syndrome (3); 4 in-hospital deaths: infection (2), multiorgan failure (2) and 4 late deaths: acute rejection (1), chronic rejection (1), lymphoma (1), unknown (1). The actuarial probability of survival (+/- SE) was 62% +/- 10% at 1 year and 53% +/- 12% at 2 years. Univariate analysis was used to evaluate the following risk factors: age, diagnosis, hemodynamic decompensation, previous cardiac surgery, ischemic time of the graft, technique of graft preservation, preoperative pulmonary artery pressure, occurrence of postoperative low cardiac output syndrome (LCOS) with pulmonary hypertension (PHT). The occurrence of early LCOS with PHT significantly increased both early and late mortality (78% early mortality, 100% overall mortality). This syndrome occurred in 9 patients (29%) and was attributed to primary graft failure in 2, increased pulmonary vascular resistances in 6 and multiple factors in 1. Although not significant, two factors may increase early survival: young age (less than or equal to 1 year) at operation and improved technique of graft preservation.     

Outcome of previous tricuspid valve operation and arrhythmias in adult patients with congenital heart disease

Background. Tricuspid valve operation or replacement has been associated with high perioperative mortality and poor long-term results. The prevalence of atrial arrhythmias before and after operation in these patients is undefined.

Methods. We retrospectively examined the outcome and frequency of atrial arrhythmias in 85 adult patients (46% men) with congenital heart defects who underwent tricuspid valve operation between 1961 and 1995.

Results. The majority had either Ebstein’s anomaly (22%), congenitally corrected transposition (19%), tetralogy of Fallot (15%), atrial (13%), or ventricular (11%) septal defects. Forty-two (49%) patients had sustained arrhythmias within 1 year before operation. After tricuspid valve operation, 21 patients (50%) had recurrence of atrial arrhythmias, and 7 in preoperative sinus rhythm developed late rhythm disturbances. Multivariate analysis identified age at operation and preoperative arrhythmias as independent predictors of late arrhythmias. Perioperative mortality was 5%, and there were seven late deaths. Survival was 91% at 5 years, and 83% at 10 years.

Conclusions. Surgical intervention does not prevent recurrence of atrial arrhythmias. Tricuspid valve operation in patients with congenital heart disease can be performed with a low risk of perioperative mortality and good long-term outcome.     

Early and mid-term outcomes of cardiac and thoracic aortic surgery in over-75-year-olds with postoperative quality of life assessment

The early and mid-term outcomes of cardiac and thoracic aortic surgery were reviewed in seventy-two consecutive patients aged 75 years and older, together with assessment of postoperative quality of life. Twenty-six patients had ischemic heart disease, twenty had valvular heart disease, one had congenital heart disease, and twenty-five had thoracic aortic aneurysm. Twenty-five (34.7%) required an emergency operation. There were 6 early deaths (8.3%) and 11 late deaths (17.2%), of which the emergency cases had higher mortality of 5 early deaths (20.0%) and 3 late deaths (15.0%). In particular, most cases with a ruptured thoracic aortic aneurysm died eventually from various complications including neurological dysfunction. The others with a non-ruptured aneurysm also had atherosclerotic aortic or arterial lesions which caused a lethal cerebrovascular accident or ischemic heart disease. The quality of life of 51 of 53 survivors was assessed using the Rosser and Watts index being based on disability and distress scores. The response was satisfactory — the disability score was 2.6 ± 1.9 and the distress score was 1.4 ± 0.4. The patients with a thoracic aortic aneurysm had worse quality of life scores than those of the ischemic heart disease or valvular heart disease patient-groups because of various perioperative complications. Our experiences demonstrate that the results including the postoperative quality of life following cardiac and aortic surgery in the elderly is satisfactory except for emergency cases. The results would prompt us to operate, if possible, electively in their stable conditions, even on elderly over-75-year-olds.     

Early and mid-term outcomes of cardiac and thoracic aortic surgery in over-75-year-olds with postoperative quality of life assessment.

The early and mid-term outcomes of cardiac and thoracic aortic surgery were reviewed in seventy-two consecutive patients aged 75 years and older, together with assessment of postoperative quality of life. Twenty-six patients had ischemic heart disease, twenty had valvular heart disease, one had congenital heart disease, and twenty-five had thoracic aortic aneurysm. Twenty-five (34.7%) required an emergency operation. There were 6 early deaths (8.3%) and 11 late deaths (17.2%), of which the emergency cases had higher mortality of 5 early deaths (20.0%) and 3 late deaths (15.0%). In particular, most cases with a ruptured thoracic aortic aneurysm died eventually from various complications including neurological dysfunction. The others with a non-ruptured aneurysm also had atherosclerotic aortic or arterial lesions which caused a lethal cerebrovascular accident or ischemic heart disease. The quality of life of 51 of 53 survivors was assessed using the Rosser and Watts index being based on disability and distress scores. The response was satisfactory--the disability score was 2.6 +/- 1.9 and the distress score was 1.4 +/- 0.4. The patients with a thoracic aortic aneurysm had worse quality of life scores than those of the ischemic heart disease or valvular heart disease patient-groups because of various perioperative complications. Our experiences demonstrate that the results including the postoperative quality of life following cardiac and aortic surgery in the elderly is satisfactory except for emergency cases. The results would prompt us to operate, if possible, electively in their stable conditions, even on elderly over-75-year-olds.     

Amrinone versus conventional therapy in pulmonary hypertensive patients awaiting cardiac transplantation

Pulmonary hypertension is associated with an increased perioperative mortality for orthotopic heart transplantation. A transpulmonary gradient greater than 15 mm Hg or a pulmonary vascular resistance greater than 5 Woods units increases mortality secondary to right heart failure. This study compares amrinone with conventional therapy in 38 transplant candidates with pulmonary hypertension. All patients had elevated transpulmonary gradient, pulmonary vascular resistance, or both. Group 1 (n = 21) received prolonged continuous intravenous amrinone therapy, whereas group 2 (n = 16) received high-dose oral diuretics, digitalis, and captopril. Both groups 1 and 2 had decreased pulmonary hypertension, transpulmonary gradient, and pulmonary vascular resistance. However, amrinone was more effective, with a 86% response rate versus 63% response for conventional therapy. Survival awaiting transplantation was significantly higher in group 1 (20 of 22, 91%) than in group 2 (10 of 16, 63%). Although both groups 1 and 2 had significantly decreased pulmonary vascular resistance, only group 2 had significantly decreased systemic vascular resistance. Comparison of pulmonary vascular resistance after therapy showed that the response in group 1 (amrinone) was significantly lower than the response in group 2 (conventional therapy), suggesting that amrinone may function as a direct vasodilator of the pulmonary vasculature. There were no operative deaths or episodes of perioperative right heart failure in either group. Amrinone appears to be more effective and safe than conventional therapy in the treatment of prospective heart transplant candidates with pulmonary hypertension.     

Mechanical Aortic Valve Replacement in Children and Adolescents After Previous Repair of Congenital Heart Disease

Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown‐ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart defects. Between July 2002 and November 2008, 15 (10 male and 5 female) consecutive patients (mean age 14.5 ± 10.5 years) underwent mechanical AVR. Hemodynamic indications for AVR were aortic stenosis in four (27%), aortic insufficiency in eight (53%), and mixed disease in three (20%) after previous repair of congenital heart defects. All patients had undergone one or more previous cardiovascular operations due to any congenital heart disease. Concomitant cardiac procedures were performed in all of them. In addition to AVR, in two patients, a mitral valve exchange was performed. One patient received a right ventricle‐pulmonary artery conduit replacement as concomitant procedure. The mean size of implanted valves was 23 mm (range 17–29 mm). There were neither early deaths nor late mortality until December 2008. Reoperations were necessary in five (33%) and included implantation of a permanent pacemaker due to complete atrioventricular block in two (15%), mitral valve replacement with a mechanical prosthesis due to moderate to severe mitral regurgitation in one (7%), aortocoronary bypass grafting due to stenosis of a coronary artery in one (7%), and in one (7%), a redo subaortic stenosis resection was performed because of a secondary subaortic stenosis. At the latest clinical evaluation, all patients were in good clinical condition without a pathological increased gradient across the aortic valve prosthesis or paravalvular leakage in echocardiography. Mechanical AVR has excellent results in patients after previous repair of congenital heart defects in childhood, even in combination with complex concomitant procedures. Previous operations do not significantly affect postoperative outcome.     

动脉转位术的临床应用

目的 总结动脉转位术(arterial switch operation，ASO)治疗完全型大动脉转位(transposition of the great arteries，TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(VSD)的临床经验。方法 采用ASO治疗小儿先天性心脏病32例，其中TGA22例，伴室间隔完整型(intact ventricular septum，IVS)9例，伴VSDl3例；右心室双出口伴肺动脉瓣下VSD(Taussig—Bing)10例。结果TGA／IVS9例中死亡1例，TGA／VSD13例中死亡4例，Taussig-Bing10例死亡3例，总手术死亡率25％(8／32)。术后随访3个月～2年，所有患者紫绀消失，活动能力明显增强。1例Taussig—Bing术前二尖瓣轻-中度反流，术后仍为中度反流；2例TGA主动脉和肺动脉瓣上狭窄，压差40mmHg(1kPa=7．5mmHg)，1例肺动脉瓣下狭窄和残余VSD，3个月后再次手术治愈。结论 ASO已广泛应用于TGA的纠治，手术效果满意；应用于右心室双出口肺动脉瓣下VSD的早期纠治，不但可防止发生肺血管阻塞性病变，而且避免了心内修补左心室流出道梗阻的远期并发症。     

Pediatric heart transplantation after operations involving the pulmonary arteries

A prohibitive perioperative mortality has been previously ascribed to pediatric heart transplantation after palliative operations for congenital heart disease involving the pulmonary arteries. Of 46 children who have undergone heart transplantation at our institution between June 1984 and February 1990, 7 (15%; mean age 8 +/- 3 years; range 1 to 18 years) have previously undergone such operations: right ventricle to pulmonary artery conduit/homograft for levo-transposition of the great arteries (2), Waterston shunt for tricuspid and pulmonary atresia (1), pulmonary artery banding for single ventricle (1), Fontan procedure for single ventricle (1), first-stage Norwood procedure for hypoplastic left heart syndrome (1), and classic right Blalock-Taussig shunt for atrioventricular canal with pulmonic stenosis (1). Three categories of pulmonary artery anatomy that require different approaches to reconstruction at the time of transplantation are recognized: abnormalities of position, pulmonary outflow obstruction, and previous systemic- or atrial-pulmonary connections. At operation, individualized pulmonary arterial reconstruction was employed, including use of previously created right ventricular-pulmonary artery conduits/homografts and angioplasty (with and without pericardial patches). Transplantation was successful in all patients. Posttransplant right ventricular-pulmonary artery pressure gradients and pulmonary vascular resistance indices were acceptable, with a tendency to decrease with time. Two patients had critical right ventricular failure postoperatively; one of them required support with extracorporeal membrane oxygenation. There was no perioperative mortality, with three deaths occurring from 5 to 39 months after transplantation. All surviving patients are in New York Heart Association functional class I. Techniques borrowed from the repair of congenital cardiac lesions can be applied to subgroups of children undergoing heart transplantation. Additional length of donor aorta and pulmonary artery should be harvested for possible use in designing pulmonary artery connections. Previous palliative operations involving the pulmonary arteries with associated complex pulmonary artery anatomy are not of themselves an insurmountable obstacle to successful heart transplantation.     

2.3～4.5 kg婴儿先天性心脏病的外科治疗

目的：总结2．3～4．5kg婴儿先天性心脏病(先心病)外科治疗结果，探讨低体重要儿围手术期的处理。方法：1998年1月至2001年12月的4年中，共收治体重2．3～4．5k的先心病婴儿115例，其中男76例，女39例；早产儿5例、双胞胎病儿1例。年龄2～364d，其中新生儿34例，1～6月龄68例，6月龄以上13例；体重2．3～2．9kg者16例，3．0～3．5kg者2l例，3．6～4．0kg者4l例，4．1～4．5kg者37例。术前诊断包括简单先心病中动脉导管未闭3例、房间隔缺损5例、室间隔缺损34例、肺动脉瓣狭窄1例，复杂先心病中完全性大血管错位38例、主动脉缩窄合并心内畸形12例、室间隔完整型肺动脉闭锁6例、完全性肺静脉异位引流6例、右室双出口3例以及其它复杂先心病7例。结果：术前合并症发生率高(4l％)。全组死亡19例，死亡率为16．5％。术中死亡者中大动脉换位手术6例，Senning手术l例，IAA 1例及CoA合并CAVC 1例，术毕均因低心输出量综合征(低心排)不能脱离心肺机死亡；术后早期死于低心排1例、多脏器功能衰竭5例，呼吸衰竭、反复气管插管2例，误吸l例以及BTS闭塞1例。生存的96例中残余分流2例、残余梗阻2例、低心排心肺复苏1例、心包积液2例、多次气管插管5例、多脏器损害2例、延迟关胸13例、肺不张31例、肺部感染16例、纵隔感染1例、败血症2例、切口感染2例。随访6～48个月，无中期死亡，再手术2例。结论：低体重儿早期手术可以获得良好结果；一期根治手术、减轻体外循环损伤、综合措施可降低低体重要儿先心病围手术期并发症的发生率和病死率。     

Classic Blalock-Taussig shunt in neonates

BACKGROUND: The choice of palliative procedure for neonates with congenital cyanotic heart defects remains controversial. Several reports have questioned whether the classic Blalock-Taussig shunt is adequate in neonates, because it shows a high rate of mortality and early shunt failure. The purpose of this study is to evaluate the early and late results of classic Blalock-Taussig shunt in neonates. METHODS: From December 1981 to December 1996, 31 neonates underwent a classic Blalock-Taussig shunt. Mean age at operation was 15.9 days (range, 2 to 28 days), and mean body weight was 3214 g (range, 2229 to 4468 g). The major diagnoses were pulmonary atresia with intact ventricular septum (6 patients) and with ventricular septal defect (4), univentricular heart (6), tricuspid atresia (5), tetralogy of Fallot (4), transposition of the great arteries (3), and double outlet right ventricle (3). RESULTS: There were no early deaths. There was one early shunt failure. The patient underwent a replacement of Blalock-Taussig shunt by vascular graft on the 1st postoperative day. Two patients with non-confluent central pulmonary artery underwent a contralateral modified Blalock-Taussig shunt on the 22nd and 42nd postoperative day, respectively. There were two late deaths before the definitive repair was performed. Twelve patients required an additional shunt operation. The mean interval between the initial and the second shunt procedure was 27.7 months (range, 6 to 67 months). Ten patients underwent definitive operation and the mean interval to that procedure was 55.5 months (range, 14 to 121 months). Shunt patency was 94.7+/-5.1% at 3 years. The freedom from cardiac event (cardiac deaths or repeat shunting procedure) was 80.5+/-7.1% at 1 year and 54.9+/-9.4% at 3 years. CONCLUSIONS: The classic Blalock-Taussig shunt has a low operative mortality and has provided excellent long-term palliation in the neonate. We conclude that a classic Blalock-Taussig shunt should be considered a reasonable choice in neonates requiring a palliative procedure for congenital cyanotic heart defects.     

Ascending aorta-to-pulmonary artery anastomosis for cyanotic congenital heart disease

A total of 101 infants and children with cyanotic congenital heart disease and inadequate pulmonary blood flow underwent an ascending aorta-to-pulmonary artery anastomosis at the University of Florida from January, 1964, to December, 1972. The operative mortality was 37%. The 64 surviving patients have experienced improved oxygenation of the blood and disappearance of hypoxic episodes for up to 8 years postoperatively. Two of 4 late deaths were related to inadequate pulmonary blood flow. Cardiac catheterization was performed in 16 patients 1 week to 6 years after the operation. Eight patients later underwent complete cardiac repair and discontinuance of the shunt.     

Extracardiac valved conduits in the pulmonary circuit

Extracardiac valved conduits represent one of the weakest facets of reconstructive surgery for congenital heart disease in that they invariably need to be replaced because of growth of the patient or because of valve or conduit failure. Between 1979 and 1989, 141 patients had 169 valved conduits placed between the heart and the pulmonary artery circuit. There were 81 male and 60 female patients, aged 2 days to 35 years (mean age, 5.9 years), with 46 patients less than 1 year of age. We performed primary repair in 117 patients; in this group, there have been 28 conduit replacements in 27 patients. In 17 patients initial repair with a conduit was performed elsewhere and we replaced these conduits in 15 and removed them in 2. A further group of 9 patients were seen after repair of tetralogy of Fallot or double-outlet right ventricle, with severe pulmonary incompetence or right ventricular outflow tract aneurysm. All had valved conduits inserted as secondary procedures. The types of valved conduits used were xenograft (n = 126) and homograft (n = 43). There were six hospital deaths (3.6%; 70% confidence limits [CL], 2% to 6%) and seven late deaths (4.1%; CL, 2.5% to 6.5%) in a total of 169 conduit insertions. Forty-five conduits have been removed and 43 reinserted without early or late mortality (0%; CL, 0% to 4%). Actuarial survival after conduit insertion was 87% at 5 years (CL, 80% to 92%), including operative mortality. Actuarial freedom from conduit replacement was 37% at 5 years (CL, 20% to 56%). Conduit insertion in infants and small children ensures subsequent replacement, but this can be done at low risk.(ABSTRACT TRUNCATED AT 250 WORDS)     

Late results after resection of fixed subaortic stenosis

Resection of fixed subaortic stenosis was performed on 44 patients with median age 14 (range 2-61) years. Concomitant aortic valve pathology was present in 14 (32%) cases (congenital stenosis in 2, thick fibrotic cusps in 8 and incompetent cusps in 4) and other congenital cardiovascular malformations in eight (18%). There was no perioperative mortality. Of the six late deaths, three were due to non-cardiac causes. During follow-up (median 6, range 2-21 years), six reoperations were performed for residual or recurrent obstruction and/or aortic incompetence. Aortic valve replacement was required at two primary and four second operations. Actuarial 5-year and 10-year survival rates were 89% and 76%, respectively, and rates with freedom from cardiac death endocarditis and reoperation 83% and 64%. At follow-up evaluation two patients had significant aortic regurgitation and all survivors had a systolic ejection murmur. At Doppler echocardiography in 29 patients without reoperation, the median pressure difference in the left ventricular outflow tract was 10 (range 0-55) mmHg--in three cases greater than or equal to 30 mmHg. Careful follow-up is advisable after resection of fixed subaortic stenosis, because of the risk of residual or recurrent obstruction and of significant aortic valve incompetence.     

Outcome of cardiac surgery in low birth weight and premature infants.

OBJECTIVE: Low birth weight or premature infants may require early surgical treatment of congenital cardiac lesions because of their poor clinical status. Even thought early repair or palliation is carried out with incremental risk factor for morbidity and mortality, it has been demonstrated to be preferable to medical management and delayed surgery. This retrospective study was undertaken to evaluate early and mid-term results in infants, weighing less than 2500 g, who underwent surgery other than patent ductus arteriosus closure. METHODS: Since January 1993 to August 2002, 60 consecutive patients underwent early surgical treatment of congenital heart malformations at our institution. 27 patients were premature (born before 37 weeks of gestation). Ninety percent were severely symptomatic. Mean age at operation was 15.5 days (range 4-68 days). Mean weight was 2120 g (range 900-2500 g). Indications for surgery were: coarctation complex 11, transposition of great arteries 9, interrupted or severely hypoplastic aortic arch 9, hypoplastic left heart syndrome 7, truncus arteriosus 5, other 19. Thirty-five patients were operated on CPB, Deep Hypothermia with Circulatory Arrest was used in 9. Complete repair was achieved in 32 patients. Aortic arch reconstruction was required in 32 cases. RESULTS: There were nine early deaths (15%): heart failure (5), multiorgan failure (3), sepsis (1). Age, weight, prematurity, type of surgery and use of cardio pulmonary by-pass did not influence early mortality. Mean intensive care unit stay and duration of mechanical ventilation were 5.8 days and 75.5 h, respectively. Postoperative neurological complications did not occur in any patient. At follow-up (mean 48 months) there were nine late deaths. Kaplan-Meier survival at 60 months was 70%. CONCLUSIONS: Surgery for congenital heart disease can be performed in low weight critically ill infants with reduced, but still acceptable early and mid-term survival.     

前列腺素E1不同给药时机治疗围术期先天性心脏病肺动脉高压的观察

目的　观察先天性心脏病合并重度肺动脉高压（ＰＨ）患者围术期血液动力学的变化。方法　２０ 例先天性心脏病合并重度肺动脉高压患者以前列腺素Ｅ１ 应用不同时机分对照组和试验组,每组１０例。试验组在体外循环开始后从中心静脉持续泵注前列腺素Ｅ１２０ｎｇ·ｋｇ－ １ ·ｍ ｉｎ－ １;对照组在体外循环中,开放升主动脉后开始用前列腺素Ｅ１ 。观察围术期平均动脉压（ＭＡＰ）、平均肺动脉压（ＰＡＰ）、动脉压与肺动脉压之比（ＰＰ／ＰＳ）、心脏指数（ＣＩ）、肺阻力指数（ＰＶＲＩ）、体循环阻力指数（ＳＶＲＩ）的动态变化。结果　体外循环后各时点的肺动脉压力与主动脉压力之比较术前显著降低,（Ｐ＜ ０．０１）。肺阻力指数和体循环阻力指数在体外循环后逐渐升高,试验组肺阻力指数在升主动脉开放后６ 小时显著低于对照组,（Ｐ＜ ０．０１）。试验组心指数在开放循环后２～４ 小时高于对照组,（Ｐ＜０．０５）。结论　重度肺动脉高压心内畸形矫正手术中,体外循环开始即应用前列泉素Ｅ１ 的效果优于传统的开放升主动脉后给药的效果。     

Results of bioprosthetic versus mechanical aortic valve replacement performed with concomitant coronary artery bypass grafting

BACKGROUND: Concomitant coronary artery disease with aortic valve disease is an established risk factor for diminished late survival. This study evaluated the results of bioprosthetic (BAVR) or mechanical aortic valve replacement (MAVR) performed with coronary artery bypass grafting (CABG). METHODS: From January 1984 through July 1997, combined AVR + CABG was performed in 750 consecutive patients; 469 received BAVR and 281 received MAVR. BAVR recipients were significantly older (mean age, 75 vs 65 years), and had more nonelective operations, congestive heart failure, peripheral vascular disease, preoperative intraaortic balloons, lower cardiac indices, more severe aortic stenosis, less aortic regurgitation, and more extensive coronary artery disease. RESULTS: Early complications included operative mortality, 32 patients (4.3% total: 3.8% BAVR and 5.0% MAVR); perioperative infarction, 10 (1.3%); and perioperative stroke, 22 (2.9%). Significant multivariable predictors of early mortality were age, perioperative infarction or stroke, nonelective operation, operative year, ventricular hypertrophy, and need for intraaortic balloon. Ten-year actuarial survival was 41.7% for all patients. Predicted survival for age- and gender-matched cohorts from the general population versus observed survival were BAVR, 45% versus 36%; MAVR, 71% versus 48% (survival differences BAVR 9% vs MAVR 23%, p < 0.007). Significant multivariable predictors of late mortality included age, congestive failure, perioperative stroke, extent of coronary disease, peripheral vascular disease, and diabetes. Valve type was not significant. Ten-year actuarial freedom from valve-related complications were (BAVR vs MAVR) structural deterioration, 95% versus 100%, p = NS; thromboembolism, 86% versus 84%, p = NS; anticoagulant bleeding, 93% versus 88%, p < 0.005; reoperation, 98% versus 98%, p = NS. CONCLUSIONS: AVR + CABG has diminished late survival despite the type of prosthesis inserted. Although valve type did not predict late mortality, mechanical AVR was associated with worse survival compared with predicted and more valve-related complications due to anticoagulation requirements.     

Repair of double-outlet right ventricle. An analysis of 62 cases.

Sixty-two patients with double-outlet right ventricle (DORV) underwent complete intracardiac repair between 1967 and July, 1978. Five patients (three deaths) with DORV and complete atrioventricular (AV) canal are the subject of a separate report and are not discussed further here. Twenty-eight patients had relatively uncomplicated DORV (subaortic ventricular septal defect [VSD], doubly committed VSD, or 1-malposition of the aorta with subaortic VSD) with or without pulmonary stenosis. Two (14%) of 14 died after a completely intraventricular repair, and one (12%) of eight after repair including use of a valved external conduit. Use of a transannular patch in this group was an incremental risk factor, five (83%) dying among six treated in this way. Anterior enlargement of a restrictive or unfavorably located VSD did not increase risk. A noncommitted VSD was an incremental risk factor, two (50%) of four dying after repair. No instances of complete heart block occurred in these groups. Two late deaths occurred in the uncomplicated group, from persisting servere pulmonary hypertension. The late results were good in the remainder and in the two surviving patients with noncommitted VSD. After repair of the Taussig-Bing type of DORV, eight deaths (32%) occurred among 25 patients. Complete heart block developed in two patients. In the Taussig-Bing type of DORV, the 6 year actuarial survival rate was only 38%. Most late deaths were related to improtant pulmonary vascular disease. The surgical technique that has evolved for the basic tunnel repair in the various types of DORV is described.     

Perioperative and late outcome in patients with left ventricular ejection fraction of 35% or less who require major vascular surgery

Survival in patients with diminished left ventricular ejection fraction (EF) is reduced after major vascular surgery. The objective of this study was to determine perioperative (30-day) and subsequent outcome after major vascular surgery in those with severe cardiac dysfunction, defined by EF being 35% or less (normal EF greater than 50%). From Aug. 1, 1984 to Jan. 1, 1988, 35 patients with EF equal to 27.7% +/- 6.1% (mean +/- 1 standard deviation) have required 47 major vascular procedures: 53% (n = 25) were limb revascularizations; 21% (n = 10) were direct aortoiliac aneurysm repairs: 23% (n = 11) were carotid endarterectomies: one patient had transaortic renal endarterectomy. Two deaths occurred within the first 30 days, yielding a 4.3% perioperative mortality rate (2 of 47 procedures). The cumulative mortality rate for the entire group during follow-up of 410 +/- 390 days was 40% (14 of 35 patients). Most late deaths (71%) occurred within the first 6 months after surgery and each was due to cardiovascular complications. Survival for those with an EF of 29% or less was significantly worse than for those with an EF greater than 29%, determined by life-table analysis (p less than 0.012, Mantel-Cox). The cumulative mortality rate was 59% with an EF of 29% or less and 18% in those with an EF greater than 29% (p less than 0.029, two-tailed Fisher exact test). The perioperative mortality rate for those with an EF of 35% or less who require major vascular surgery is acceptable, but overall survival during follow-up is diminished.(ABSTRACT TRUNCATED AT 250 WORDS)     

Our experience with the Carpentier-Edwards bioprosthesis.

Sixty Carpentier-Edwards porcine valve bioprostheses stabilized with glutaraldehyde were implanted in 55 patients with acquired and congenital heart disease. The follow-up period ranged between 1 and 12 months. There were 3 hospital deaths (5%) and 2 late deaths (4%) in 24 mitral, 24 aortic, 5 mitral-aortic, 1 tricuspid and 1 pulmonary valve replacements. All patients were anticoagulated from the second postoperative day onwards for a period of 3 months after which those with sinus rhythm had their anticoagulants withdrawn. Paravalvular leakage led to re-operation in 3 cases (4%). No valve failure due to cusp rupture was encountered and no thromboembolic complications have occurred. Thirty-three patients were studied postoperatively by non-invasive methods and the results are presented.     

Improved results after heart-lung transplantation: a 17-year experience

OBJECTIVE: In selected patients with severe end-stage combined cardiopulmonary diseases, heart-lung transplantation (HLTx) remains the only therapeutical option for improving survival and quality of life. PATIENTS AND METHODS: Since 1983, 51 HLTx were done at our institution. Mean patient age was 27+/-12 years with a mean donor age of 25+/-11 years. Indications for HLTx were primary pulmonary hypertension (PPH) in 49% of patients, congenital heart disease in 39%, cystic fibrosis in 6%, and repeat-HLTx in 6%. Eleven patients were younger than 14 years. Among these pediatric patients, the indications were PPH in 55% of patients, pulmonary atresia with severe pulmonary artery hypoplasia in 27%, and cystic fibrosis and cardiomyopathy with fixed pulmonary hypertension in 9% of patients each. Two patients had additional liver transplantation because of chronic aggressive virus hepatitis. For organ preservation, Euro-Collins solution (lung perfusion) and cardioplegic solution according to Bretschneider (heart perfusion) were used until 1994. The University of Wisconsin solution replaced Bretschneider's solution in 1994. Since 1996, Perfadex, a low-potassium dextran-based preservation solution, replaced Euro-Collins. All transplantations were done through a median sternotomy until 1994. Thereafter, a transverse thoracotomy was used in patients with suspected adhesions. Until 1995, cyclosporine A, azathioprine, and prednisolone were used for immunosuppression. Since then, tacrolimus replaced cyclosporine A. RESULTS: From 1983 until 1993, perioperative mortality was 35% (6/19). From 1994 on perioperative mortality decreased to 12.5% (4/32). Early mortality was caused by graft failure (n=5), severe bleeding (n=2), multi-organ failure (n=2), and acute rejection (n=1). Cumulative survival rates were 81% after 30 days, 63% after 1 year, and 54% after 5 years, respectively. Since 1994, cumulative survival rates were markedly improved to 87% after 30 days, 81% after 6 months, and 78% after 1 year. There was no death during the first postoperative year among the 11 pediatric patients. Late death was mainly caused by obliterative bronchiolitis (OB; 76%); two patients died because of multi-organ failure or septic complications, respectively, and one patient died within the first postoperative year because of aspergillosis. CONCLUSION: Changes in organ preservation management, surgical techniques, and immunosuppressive therapy significantly improved the short- and mid-term results after HLTx. Long-term results can only be improved in cases of successful prevention and treatment of OB.