Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy

Surgical mortality is high in children with visceral heterotaxy (VH), particularly if atrioventricular valve insufficiency, ventricular dysfunction, or aortic atresia is present. This study reviews the outcome of cardiac transplantation (CT) in infants and children with VH and congenital heart disease who are at high risk for standard palliative or corrective surgery. We reviewed CT outcomes in 29 children with VH, congenital heart disease, atrioventricular valve insufficiency, ventricular dysfunction, and/or aortic atresia. Median age at CT was 3.1 years. Cardiac surgery had been performed in 20 patients (69%) before CT. Follow-up since CT has been 8.5 +/- 2.2 years. Outcomes were compared with 45 children who underwent transplantation for dilated cardiomyopathy. Actuarial graft survival in the VH group at 30 days and 1, 5, and 10 years was 100%, 86%, 68%, and 50%, respectively, compared with 100%, 96%, 83%, and 68% in children who underwent transplantation for dilated cardiomyopathy (p = 0.12). Splenic status, cardiac position, age at CT, number of prior cardiac surgeries, or systemic venous anomalies were not predictors of mortality after CT. Cardiopulmonary bypass and graft ischemic times were longer in the VH group; time on the ventilator after CT, length of hospitalization, and rejection, infection, post-transplant lymphoproliferative disease, and transplant coronary artery disease rates were equal. Thus, CT is a viable alternative therapy for high-risk patients with VH, possibly offering improved survival over standard surgical management.     

Assisted circulation by external heterotopic prosthesis as a bridge to heart transplantation

Eleven patients aged 7 to 58 years were placed on assisted circulation with Pierce (2 cases) or Abiomed (9 cases) external prosthetic ventricles as a bridge to cardiac transplantation. The indications were terminal cardiac failure following cardiomyopathy (7 cases), decompensated ischemic heart disease (3 cases) and subacute post-transplantation rejection (1 case). The duration of the assisted circulation ranged from 24 hours to 11 days. All patients were transplanted but 3 died after transplantation (27%). The circulatory assistance was satisfactory in all patients as shown by the regression of clinical signs of low cardiac output and the normalisation of diuresis. The complications observed during assisted circulation and after cardiac transplantation were: haemorrhage (36%), infection (27%) and thromboembolism (9%). These preliminary results with a 72% post-transplantation survival rate, show that both systems are effective "bridges to cardiac transplantation". The Abiomen device is excellent value for money and relatively simple to install and represents a good compromise between the sophisticated techniques of circulatory assistance and the problems of the cost of health care.     

The contribution of pathology sections to the Italian Heart Transplant Project in the first 5 years of its activities (1985-1990)]

All the transplantation units within the Italian Heart Transplantation Project are supported by a section of pathology, devoted to the study of the recipient's heart, to patient monitoring by means of a schedule of endomyocardial biopsies, and, if that was the case, to examine the donor's heart and to analyse the causes of death. When successes and failures of the first five years of the Project's activity are weighed up, good results are observed: of the 847 operations performed (orthotopic, heterotopic and heart-lung transplants, and re-transplants) an actuarial survival rate of 77% at 5 years has been achieved. The sections of pathology believe to have contributed significantly to these results, examining as many as 10,446 endomyocardial biopsies. The indications for transplantation were: dilated cardiomyopathy (48.5%); ischemic (35.3%); valvular (5.9%) and congenital (2.4%) heart disease; hypertrophic cardiomyopathy (2.2%); endocardial fibroelastosis (1.7%); restrictive cardiomyopathy (1.4%); anthracycline cardiotoxicity (0.8%); myocarditis (0.8%); cardiac tumours (0.5%) and arrhythmogenic cardiomyopathy (0.2%). Distribution of recipients by sex and age varied according to the indications for transplantation: males were more common among the patients transplanted for ischemic (97%) and valvular (84%) heart disease, as well as for dilated (82%) and hypertrophic (78%) cardiomyopathy, whereas the opposite was true for endocardial fibroelastosis (males constituting 21%) and cardiac tumours (25%). Mean age at transplantation ranged from 49 years (ischemic heart disease) to 6 years (endocardial fibroelastosis). In the follow-up period, a 17.5% death rate was recorded; the main causes of death were the early failure of the transplanted heart (27 pts), postoperative complications (16), hyperacute rejection (4), acute rejection (18), infections (the singular most frequent cause of death, 35 pts), the proliferative endoarteritis of coronary branches (the so-called chronic rejection, that caused 21 deaths and required 14 re-transplants) and the development of neoplasms (11). The actuarial survival curve drops to 89% after the first postoperative month, abates to 82% at the end of the first year, and progressively decreases to 77% at the end of the fifth follow-up year. Rejection monitoring required an average number of 12.5 endomyocardial biopsies per recipient, and allowed 1.7 rejection episodes per patient to be diagnosed. The fewer were the rejection episodes occurring in a unit, the higher was the percentage of deaths due to infections.(ABSTRACT TRUNCATED AT 400 WORDS)     

Influence of race in heart failure and cardiac transplantation: mortality differences are eliminated by specialized,comprehensive care

BACKGROUND: Differences in mortality are thought to exist between African Americans and Caucasians with heart failure. These differences may be due to a variety of factors, including differences in disease process, socioeconomic status, and access to health care. Additionally, little data exist on racial differences between these two groups after cardiac transplantation. This study examines a single center, urban experience in treating African Americans and Caucasians with heart failure and after cardiac transplantation. We hypothesize that treatment in a specialized, comprehensive heart failure/cardiac transplantation program results in similar survival between African Americans and Caucasians. METHODS: We retrospectively reviewed the Rush Heart Failure and Cardiac Transplant Database from July 1994 to August 2000. Variables analyzed in the cardiomyopathy patients included survival (until death, placement of left ventricular assist device or cardiac transplantation), number of hospitalizations per year, length of stay per year, and utilization of outpatient resources. Follow-up period was from initial visit to death, transplantation, or implantation of left ventricular assist device. In those who underwent cardiac transplantation, we examined rejection rates (cellular and humoral), rejection burden, hospitalization data, and 5-year survival. A subgroup bridged to cardiac transplantation with a left ventricular device was also analyzed. RESULTS: Seven hundred thirty-four cardiomyopathy patients were identified: 203 were African Americans and 531 were Caucasians. The etiology of cardiomyopathy was more commonly ischemic in Caucasians as compared to non-ischemic in African Americans (P <.01). African Americans had more admissions to the hospital per year compared with Caucasians, 1.2 +/- 2.1 versus.5 +/- 1.1 (P <.01) with longer length of stay per year, 1.4 +/- 25.2 days versus 4.4 +/- 14.3 days (P <.01). Utilization of outpatient resources was significantly higher in African Americans compared with Caucasians with more use of continuous inotropes (13% versus 6%, P <.01), intermittent inotropes (11% versus 5%, P <.01), and home nursing after hospital discharge (52% versus 32% of hospital discharges, P <.01). Survival by Kaplan-Meier analysis was comparable between the two groups (mean survival 1,470 +/- 72 days in African Americans versus 1521 +/- 46 days in Caucasians, log rank test [P =.6]). During this time, 30 African Americans and 73 Caucasians underwent cardiac transplantation. Fifty-three were bridged to transplantation with a left ventricular assist device (20 African Americans, 33 Caucasians). There were no differences in 5-year survival by Kaplan-Meier analysis despite higher peak preoperative panel reactive antibody levels in African Americans versus Caucasians (12% +/- 30% compared with 5% +/- 15%, P =.04), more overall treated rejection episodes per year in the African Americans (P <.01), as well as more posttransplant hospitalizations (2.2 +/- 1.2 times per year as compared with 1.7 +/- 2.1 times per year, P =.04). CONCLUSION: Delivery of care to heart failure patients in a comprehensive, specialized program results in similar survival regardless of race despite higher utilization of inpatient and outpatient resources. The finding that, after cardiac transplantation, African Americans do not have higher mortality rates, despite having higher rates of rejection overall and more hospitalizations, further supports the hypothesis that optimal care can improve outcomes despite unfavorable baseline clinical characteristics.     

11例原位心脏移植成功的初步经验

目的 报告11例原位心脏移植成功的初步经验。方法 2000年5月－2001年5月连续为11例患者行原位心脏移植术，其中扩张型心肌病10例，复杂性先天性心脏病1例。标准原位心脏移植手术10例，双腔静脉吻合法1例。结果 所有病例均存活，心功能恢复至I－Ⅱ级（NYHA），围术期无感或严重排异反应发生。随访期间有1例巨细胞病毒感染，1例排反应发生。结论 良好的心肌保护、术后合理的监测与抗排异治疗是心脏移植成功的关键。     

肥厚型心肌病原位心脏移植后心肌再肥厚初探

目的 探讨原位心脏移植治疗肥厚型心肌病(hypertrophic cardiomyopathy,HCM)的经验和疗效以及移植后心肌再肥厚分析.方法 9例晚期HCM患者接受同种异体原位心脏移植,男7例,女2例,采用免疫诱导方案,维持治疗采用环孢素A+霉酚酸酯+泼尼松三联方案.结果 1例移植后因肾动脉栓塞发生急性肾功能衰竭、急性排斥反应,行持续肾脏替代治疗及冲击治疗后好转,移植后32个月心跳骤停死亡.1例并发额顶叶脱髓鞘病变经内科治疗后好转.8例长期存活,3例移植后半年发现心肌再肥厚,未发现移植物血管病.结论 心脏移植是治疗晚期HCM的最佳方法之一,临床疗效良好,移植后心肌再肥厚可能与心肌微环境等有关.

Abstract：

Objective To investigate the outcome of orthotopic heart transplantation for patient with end-stage hypertrophic cardiomyopathy. Methods This retrospective review analyzed the clinical data of nine patients (7 males ) undergoing orthotopic heart transplantation for end-stage hypertrophic cardiomyopathy in our center. All patients received induced therapy protocols peri-operative and standard triple maintenance immunosuppressive therapy postoperative. Results One recipients developed acute renal failure due to renal artery embolism and allograft rejection in the early posttransplantive course, symptoms and signs were improved under continuous renal replacement therapy and steroid-pulse therapy, this patient died of sudden cardiac arrest at 32 months post transplantation. Another recipient developed demyelinating disease in frontal and parietal lobe and finally recovered with medical therapy. Eight patients survived the operation with good quality of life and there was no episode of rejection or infection or chronic graft arteriosclerosis during follow-up time. Three recipients developed left ventricular hypertrophy and there were no signs of grapg-vessle diseases in the survivals. Conclusion Heart transplantation is the best therapeutic option for selected patients with end-stage hypertrophic cardiomyopathy.     

Heart transplantation and the Batista operation for children with refractory heart failure

Medically refractory heart failure may be present in children with cardiomyopathy (CMP) or complex congenital heart disease (CHD). In adults, the surgical management of this condition is either heart transplantation or the Batista operation. From March 1995 to January 2000, a total of 6 children, aged from 1 to 16 years, with medically refractory heart failure associated with CMP or complex CHD underwent cardiac transplantation and one of them also had the Batista operation as a bridge to transplantation. One of the 6 patients died of intractable sepsis 17 days after the operation, but the other 5 were discharged with satisfactory hemodynamics. Immunosuppressive agents, including azathioprine, cyclosporin or FK-506, were given. One patient experienced moderate acute rejection, but it was controlled by FK-506, OKT-3 and solumedrol. However, another suffered from lymphoproliferative disease 8 months after transplant, but it was controlled by intravenous immunoglubulin, alpha-interferon and acyclovir. Cardiac function during serial follow-up (range, 1 month to 5 years) revealed normal systolic and diastolic function and none received any anticongestive medications. Almost all patients received an oversized donor heart. The left ventricle (LV) mass was remodeled, initially as an decrease and later as an increase. The patient who underwent the Batista operation was discharged 1 month after the operation with an increased LV ejection fraction (from 10% to 22%). She was successfully bridged to heart transplantation 7 months after the Batista operation. The results of cardiac transplantation in growing children are satisfactory and remain the mainstay of surgical treatment for medically refractory heart failure in these patients. However, with a shortage of donor hearts, the Batista operation may be adopted as a bridge to heart transplant with a fair response.     

Ten year survival after heart transplantation: palliative procedure or successful long term treatment?

OBJECTIVE: To investigate the long term outcome and prognostic factors after heart transplantation. SETTING: University hospital. SUBJECTS: 120 heart transplant patients (98 male, 22 female; underlying disease: dilated cardiomyopathy in 69, coronary artery disease in 42, miscellaneous in nine) who had undergone heart transplantation between October 1984 and October 1987. Immunosuppressive treatment was comparable in all patients and rejection episodes were treated in a uniform manner. METHODS: Functional status, quality of life, and potential predictors for long term survival were investigated. RESULTS: Actuarial survival rates were 65% at five years and 48% at 10 years; 58 patients survived > 10 years. The major causes of death were cardiac allograft vasculopathy (39%), acute rejection (18%), infection (11%), and malignancy (11%). Long term survivors had good exercise tolerance assessed by the New York Heart Association classification: 47 (81%) in grade I/II; 11 (19%) in grade III/IV. Echocardiography showed good left ventricular function in 48 patients. On angiography, severe allograft vasculopathy was present in only 16 patients (28%). Renal function was only slightly impaired, with mean (SD) serum creatinine of 148.5 (84.9) micromol/l. Multiple potential predictors of long term survival were analysed but none was found useful. CONCLUSIONS: Heart transplantation represents a valuable form of treatment. Survival for more than 10 years with a good exercise tolerance and acceptable side effects from immunosuppression can be achieved in about 50% of patients.     

巴利昔单抗作为免疫诱导剂预防国人心脏移植术后早期急性排斥反应的疗效和安全性的观察

目的观察国人心脏移植用巴利昔单抗作为免疫诱导剂与传统的三联免疫抑制剂合用的耐受性和预防术后早期急性排斥反应的效果。方法心脏移植患者47例,男38例,女9例,平均年龄(44.9±13.4)岁,包括扩张型心肌病20例(42.5%),缺血性心肌病12例(25.5%),致右室心律失常性心肌病8例(17.0%),肥厚型心肌病2例(4.2%),心脏肿瘤2例(4.2%),瓣膜性心肌病1例(2.1%),高血压心脏病1例(2.1%),巨细胞性心肌炎1例(2.1%)。术前淋巴细胞群体反应抗体(PRA)>10%者4例,交叉配型均<5%。用巴利昔单抗诱导治疗20mg×2次。三联免疫抑制剂用法:术中给予甲基强的松龙500mg×2次,术后125mg每8h1次;拔除气管插管后给予强的松1mg.kg-1.d-1,以后每3天减量10mg,至总量10mg/d维持。环孢素A(CsA)于术后血肌酐<150μmol/L开始服用,剂量3～6mg.kg-1.d-1,分2次服用,血药谷值浓度维持在180～300ng/ml。术后3周作1次心内膜活检。霉酚酸酯(MMF)1.0～2.0mg/d,分2次服用。急性排斥反应分级按照国际心肺移植协会(ISHLT)的标准。巨细胞病毒感染的监测用PP65抗原血症试验,EB病毒感染用ELISA方法查抗体。结果47例患者全部存活。急性排斥反应分级结果:0级30(63.8%)人,ⅠA级11(23.4%)人,ⅠB级3(6.3%)人,Ⅱ级3(6.3%)人。MMF平均剂量(1.2±0.3)g/d。CsA于术后平均(3.4±2.1)d开始服用,平均累积剂量(4.1±1.2)mg.kg-1.d-1,平均谷值浓度(237.0±76.2)ng/ml。术后1个月内感染5人,但无巨细胞病毒和EB病毒感染。结论国人心脏移植用巴利昔单抗作为免疫诱导剂与传统的三联免疫抑制剂合用的耐受性良好,预防术后早期急性排斥反应有效。     

Heart transplantation in patients with end-stage heart failure and cardiac ascites.

BACKGROUND: Clinical outcome of heart transplantation in patients with heart failure and ascites has not been reported. Here, the clinical outcome of heart transplantation in patients with heart failure and ascites is evaluated. METHODS AND RESULTS: Between 1989 and 2005, 45 patients with heart failure and ascites underwent an orthotopic heart transplantation. Of the 45 patients, 33 were men (median age 44 years, range 10-63 years). Causes of heart failure included congenital heart disease in 4 patients (9%), dilated cardiomyopathy in 21 patients (47%), rheumatic heart disease in 7 patients (16%), coronary artery disease in 10 patients (22%), and others. Twenty of the 45 patients (44%) had undergone a previous cardiac operation. Hospital mortality occurred in 10 patients (22%) because of bleeding in 4, sepsis with multiple organ failure in 5 and non-diagnostic graft failure in 1 patient. Re-operation for postoperative bleeding occurred in 14 patients (31%). Independent risk factors for hospital death were low serum albumin (odds ratio 0.05; 95% confidence interval 0.003-0.591; p=0.018) and re-operation for bleeding (odds ratio 30.11; 95% confidence interval 2.38-380.26; p=0.009). CONCLUSIONS: Heart transplantation in patients with heart failure and ascites was associated with high hospital mortality and morbidity. The co-existence of ascites and hypoalbuminemia implied poor prognosis.     

Frequency of familial nature of dilated cardiomyopathy and usefulness of cardiac transplantation in this subset

A familial etiology was identified on the basis of family history in 16 (8.75%) of 184 patients undergoing cardiac transplantation at Stanford for endstage dilated cardiomyopathy (DC). These 16 patients, from 11 families, included 5 sibling pairs. To help determine optimal management of such patients, their case histories and posttransplant courses were reviewed. Mean age of patients at presentation was 23 +/- 15 years. In sibling pairs, duration of symptoms from onset to diagnosis was 14 +/- 5 weeks for the first sibling, but only 4 +/- 2 weeks for the second. Progressive cardiac enlargement was documented radiographically in siblings of transplant recipients in 2 families before the onset of symptoms. The posttransplant course with regard to rejection incidence, infectious complications, coronary artery disease and malignancy was similar to that of the 168 patients with nonfamilial DC. Actuarial survival at 5 years after transplantation was 80%. Thirteen patients (including all sibling pairs) are alive 1 to 11 years after transplantation. Sepsis was the cause of death in 3 patients, occurring during the early postoperative period in 2 and following retransplantation for graft atherosclerosis 7 years after the initial transplant in the third patient. Thus, diagnosis of DC in childhood or adolescence mandates evaluation and surveillance of family members, because this disease can progress rapidly. The favorable results of cardiac transplantation for familial DC suggest that it should be promptly considered for such patients with end-stage disease.     

Heart failure etiology affects peripheral vascular endothelial function after cardiac transplantation

OBJECTIVES: The goal of this study was to examine the effect of heart failure etiology on peripheral vascular endothelial function in cardiac transplant recipients. BACKGROUND: Peripheral vascular endothelial dysfunction occurs in patients with heart failure of either ischemic or nonischemic etiology. The effect of heart failure etiology on peripheral endothelial function after cardiac transplantation is unknown. METHODS: Using brachial artery ultrasound, endothelium-dependent, flow-mediated dilation (FMD) was assessed in patients with heart failure with either nonischemic cardiomyopathy (n = 10) or ischemic cardiomyopathy (n = 7), cardiac transplant recipients with prior nonischemic cardiomyopathy (n = 10) or prior ischemic cardiomyopathy (n = 10) and normal controls (n = 10). RESULTS: Patients with heart failure with either ischemic cardiomyopathy or nonischemic cardiomyopathy had impaired FMD (3.6 +/- 1.0% and 5.1 +/- 1.2%, respectively, p = NS) compared with normal subjects (13.9 +/- 1.3%, p < 0.01 compared with either heart failure group). In transplant recipients with antecedent nonischemic cardiomyopathy, FMD was markedly higher than that of heart failure patients with nonischemic cardiomyopathy (13.0 +/- 2.4%, p < 0.001) and similar to that of normal subjects (p = NS). However, FMD remained impaired in transplant recipients with prior ischemic cardiomyopathy (5.5 +/- 1.5%, p = 0.001 compared with normal, p = 0.002 vs. transplant recipients with previous nonischemic cardiomyopathy). CONCLUSIONS: Peripheral vascular endothelial function is normal in cardiac transplant recipients with antecedent nonischemic cardiomyopathy, but remains impaired in those with prior ischemic cardiomyopathy. In contrast, endothelial function is uniformly abnormal for patients with heart failure, regardless of etiology. These findings indicate that cardiac transplantation corrects peripheral endothelial function for patients without ischemic heart disease, but not in those with prior atherosclerotic coronary disease.     

Progressive Left Ventricular Hypertrophy after Heart Transplantation: Insights and Mechanisms Suggested by Multimodal Images

Immunosuppression is the typical measure to prevent rejection after heart transplantation. Although rejection is the usual cause of cardiac hypertrophy, numerous other factors warrant consideration. Calcineurin inhibitors rarely cause hypertrophic cardiomyopathy; the few relevant reports have described children after orthotopic kidney or liver transplantation. We present the case of a 73-year-old woman, an asymptomatic orthotopic heart transplantation patient, in whom chronic immunosuppression with prednisone and cyclosporine apparently caused a phenotype of hypertrophic cardiomyopathy. The natural course of her midapical hypertrophy was revealed by single-photon-emission computed tomography, positron-emission tomography, and 2-dimensional echocardiography.Clinicians and radiographers should be alert to progressive left ventricular hypertrophy and various perfusion patterns in heart transplantation patients even in the absence of underlying coronary artery disease. Toward this end, we recommend that advanced imaging methods be used to their fullest extent.     

Analysis of morbid events and risk factors for death after cardiac transplantation

Risk factors for death after cardiac transplantation performed at the University of Alabama at Birmingham from January 1981 to July 1985 included (by multivariate analysis) higher calculated preoperative pulmonary vascular resistance (early and constant phases), morphology of cardiomyopathy (versus ischemic heart disease) (constant phase only) and black race (constant phase). Overall actuarial survival was 71% at 1 year and 48% at 3 years (including azathioprine and cyclosporine eras). The hazard function for death was highest immediately after operation and declined rapidly thereafter, merging with a constant phase of risk at about 3 months. The most favorable group for long-term survival was the group of white patients with ischemic heart disease and low pulmonary vascular resistance. When such patients had a pulmonary vascular resistance less than 3 units.m2, the 3 year survival rate exceeded 85%. The most common causes of death were acute rejection (24%) and infection (17%). The risk of infection remained highest during the first several months after any period of augmented immunosuppression.     

Risk stratification in a Brazilian hospital-based cohort of 1220 outpatients with heart failure: role of Chagas' heart disease

BACKGROUND: Few studies evaluated prognostic factors of outpatients with heart failure of different etiologies including Chagas' heart disease. METHODS: We studied 1220 outpatients with heart failure in functional classes III and IV (NYHA) to evaluate prognostic factors. Patients aged 13-72 years (mean 45.5, standard deviation 11); 952 men (78%) and 268 women (22%) were followed up for 25.6+/-26 months from 1991 to 2000. Heart failure was attributed to idiopathic dilated cardiomyopathy in 454 (37%) patients. Etiologies were Chagas' heart disease in 242 (20%) patients, ischemic cardiomyopathy in 212 (17%), hypertensive cardiomyopathy in 170 (14%) and others in 142 (12%). Statistical analyses were performed with Kaplan-Meier and Cox proportional hazards methods, following a strategy of noninvasive model as well as in an invasive model to identify the risk of death. RESULTS: Four hundred fifteen (34%) patients died in the follow-up period, 71 (6%) patients underwent heart transplantation and 28 (2%) underwent other surgical interventions. In the noninvasive model, Chagas' heart disease (relative risk compared with other etiologies 2.26 to 2.97), left ventricular end diastolic diameter on echocardiography (relative risk 1.13) and left ventricular ejection fraction on radionuclide angiography (relative risk 0.96) were associated with higher mortality. In the invasive model, Chagas' heart disease (relative risk compared with other etiologies 2.66 to 9.13) was the most important determinant of mortality in association with the cardiac index (relative risk 0.40). CONCLUSIONS: In this cohort of patients with heart failure of different etiologies, Chagas' heart disease was the main prognostic factor for mortality.     

A survey of nine years heart transplantation at Erasme Hospital, University of Brussels.

Between March 1982 and March 1991, 225 heart transplantations (HTx) have been performed in 220 patients suffering end stage cardiac disease. Thirteen percent were females and 87% were males. Age range was from 5 to 68 years. The underlying cardiac disease was ischemic cardiopathy in 51.5%, congestive dilated cardiomyopathy in 42%, valvular cardiomyopathy in 3.5%, toxic myocarditis (post-adriamycin) in 1.5% and chronic rejection in 2.5% (retransplantation). Selection of the recipients was done following the currently well established criteria also taking into account the absolute major contraindications for HTx. Due to the still increasing demand of donor organs, currently donor age has been extended up to 50 years for male and 55 years for female donors. One quarter of the grafts were harvested on site in our institution, two other quarters were harvested somewhere else in Belgium and the last quarter provided by other countries cooperating with Eurotransplant. All patients have undergone orthotopic cardiac transplantation using the standard Lower and Shumway technique. Immunosuppression protocols have changed four times throughout the years. Nevertheless all were based on the use of Ciclosporine variously combined with other current immunosuppressive drugs. Rejection monitoring relied on routine endocardiac biopsy and was diagnosed according to the Billingham criteria. The in-hospital mortality is currently 11%. Infection, early right heart graft failure and acute rejection were the leading causes of death. The major causes of early morbidity were several curable infections, reversible rejection episodes, transient acute renal failure and controllable arterial hypertension. Among the survivors followed for at least one month up to nine years, half of late mortality was caused by chronic rejection followed by infection, sudden death, metabolic disorders, stroke and malignancy. Late morbidity involves cases of mild coronary graft diseases, biological renal insufficiency, some degree of arterial hypertension, dislipidemia. Current actuarial survival rate is 87% at one year, 76% at 5 years up to 9 years. Our experience confirms that HTx represents today and effective therapy for selected patients suffering end stage cardiac disease.     

Clinical features of hypertrophic cardiomyopathy in the young

BACKGROUND: We analysed the experience with hypertrophic cardiomyopathy in two paediatric centres to establish the differences from older patients. METHODS: Out of 45 young patients seen from 1974 to 1999, we included 38. Criterions for exclusion were secondary forms, or association with severe congenital cardiac disease which could alter the outcome. RESULTS: The patients presented at the age of 5.7 years, and were followed for 7.0 years. The 34 patients referred because of a murmur or cardiomegaly were older than the four with heart failure, presenting at 6.2 as opposed to 2.1 years of age, p = 0.08. Of the patients, 29 (76%) had primary cardiomyopathy, while 9 (24%) had secondary forms associated with Noonan's and LEOPARD syndromes. Familial tendency was ascertained in 7 patients (18%). The septal thickness in mm/m2 at presentation was greater in patients under 2 years than in older children (29 vs 18, p = 0.02). Obstructive hypertrophic cardiomyopathy was found in 17 patients (45%), with six of these having mild associated congenital cardiac defects. Nine had symptomatic arrhythmias. Overall, treatment was medical in 31, with DDD pacing used in 5, and surgery, radiofrequency ablation, and transplantation in one patient each. Total mortality was 24%, at a rate of 4.3% per year. Four patients died in heart failure and 5 had sudden death. Those in failure were significantly younger (p = 0.01). CONCLUSIONS: Hypertrophic cardiomyopathy in the young is characterized by referral for murmur or heart failure; frequent secondary forms; the obstructive variant being as common as the non-obstructive form; a mortality rate similar to that for adults attending tertiary centres; and less frequent familial forms than in older populations.     

Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela

OBJECTIVE: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND: Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS: A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS: Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 +/- 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% +/- 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 +/- 2%). CONCLUSIONS: Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.     

Pharmaceutical management of decompensated heart failure syndrome in children: Current state of the art and a new approach

Prompt initiation of appropriate and intensive treatment in children with decompensated heart failure is crucial to avoid irreversible end-organ dysfunction. Initial management of these children includes transfer to the pediatric cardiac intensive care unit, basic hemodynamic monitoring, and establishment of intravenous access. Inotropic support should be instituted peripherally before obtaining central venous and arterial access. The team should be prepared for emergent intubation and initiation of mechanical circulatory support. Two experienced physicians should work together to obtain vascular access and manage sedation, airway control, and cardiovascular support. Acute heart failure syndrome in children may be related to cardiomyopathy, myocarditis, congenital heart disease, and acute rejection post heart transplantation. Each of these causes requires a different approach. Fulminant myocarditis may lead to severe morbidity and requires intensive support, although its outcome is considered to be good. Acute heart failure related to newly diagnosed dilated cardiomyopathy may represent end-stage heart failure; therefore, long-term mechanical circulatory support and heart transplantation may be considered to avoid other end-organ dysfunction. Hypertrophic cardiomyopathy may lead to acute decompensation due to 1) left ventricular outflow obstruction, 2) restrictive physiology leading to pulmonary hypertension, or 3) myocardial is chemia associated with coronary artery bridging. Decompensated heart failure associated with congenital heart disease usually represents end-stage heart failure and requires thorough evaluation for heart transplantation. Children with single-ventricle physiology who develop decompensated heart failure after a Fontan procedure are not candidates for mechanical circulatory support and therefore may not survive to heart transplantation. Acute heart failure due to posttransplantation acute rejection requires aggressive antirejection treatment, which places these patients at significant risk for overwhelming opportunistic infections. In our opinion, mechanical circulatory support should be initiated early in children who present with end-stage heart failure associated with hemodynamic instability to avoid end-organ damage.     

Cardiac transplantation in the infant and young child. Preliminary results

Between January and December, 1987, a programme of heart transplantation in paediatrics was designed and carried out in 9 children by the medical and surgical teams of the Necker/Enfants Malades-La?nnec hospitals group, Paris. Six of the patients were infants of less than 2 years (4 were under one year), and the oldest child was 10 years old. All patients seemed to be condemned to an early death either because their congenital heart disease was beyond the resources of conventional surgery (6 cases) or because their dilated cardiomyopathy was refractory to all medical treatments. Three children died at the end of the operation or a few days afterwards, due to poor quality graft (1 case), fulminating bacterial superinfection (1 case) or intractable pulmonary hypertension (1 case). The remaining 6 children are now living as normally as possible in their respective families. The long-term immunosuppressive treatment consists of cyclosporine and azathrioprine; corticosteroids are only used at the very beginning of treatment or in case of graft rejection. Only two episodes of rejection, confirmed by endomyocardial biopsy, were observed in the same patient during the first postoperative month. Biopsy was never performed systematically in order to spare the patient's vein, and the diagnosis of rejection was suspected on clinical grounds.(ABSTRACT TRUNCATED AT 250 WORDS)