Long-term survival after pediatric cardiac transplantation and postoperative ECMO support

BACKGROUND: Graft failure after cardiac transplantation in children can be managed acutely with mechanical support, most commonly extracorporeal membrane oxygenation (ECMO). The purpose of this study was to evaluation the long-term outcome of ECMO support early and late after pediatric cardiac transplantation. METHODS: From February 1982 through October 2002, 168 patients underwent isolated cardiac transplantation. Twenty patients (11.9%) required mechanical support early or late after transplantation. Inpatient and outpatient records of these were reviewed. RESULTS: Indication for transplantation was complex congenital heart disease in 12, cardiomyopathy in 7, and graft failure (retransplant) in 1. One patient was also on ECMO preoperatively. Fifteen patients required circulatory support immediately or shortly (less than 6 weeks) after transplantation. The remaining 5 patients were placed on ECMO for ventricular dysfunction late (3 months to 7 years) after transplantation. In the perioperative ECMO group, 8 (53%) were successfully decannulated and subsequently discharged. Three of 5 (60%) patients placed on ECMO late were successfully decannulated, 1 of whom died in hospital 10 days later and 2 of whom are still alive. CONCLUSIONS: Mechanical circulatory support using ECMO can be a useful strategy is the management of graft dysfunction after pediatric cardiac transplantation both early and late postoperatively. The mortality rate is acceptable in this very high risk group of patients and long-term outcome is good.     

Does early cystectomy improve the survival of patients with high risk superficial bladder tumors?

PURPOSE: We compared survival after early versus delayed cystectomy in patients with high risk superficial bladder tumors. MATERIALS AND METHODS: Of 307 patients with high risk superficial bladder tumors who were treated initially with transurethral resection and bacillus Calmette-Guerin (BCG) therapy 90 (29%) underwent cystectomy for recurrent tumor during a followup of 15 to 20 years. Disease specific survival distribution of these 90 patients was determined relative to the indications for and time of cystectomy. RESULTS: Of the 90 patients who underwent cystectomy 44 (49%) survived a median of 96 months. Of 35 patients with recurrent superficial bladder tumors 92% and 56% survived who underwent cystectomy less than 2 years after initial BCG therapy and after 2 years of followup, respectively. Of 55 patients with recurrent muscle invasive bladder disease 41% and 18% survived when cystectomy was performed within and after 2 years, respectively. Multivariate analysis showed that survival was improved in patients who underwent earlier rather than delayed cystectomy for nonmuscle invasive tumor relapse. CONCLUSIONS: Earlier cystectomy improves the long-term survival of patients with high risk superficial bladder tumors in whom BCG therapy fails.     

Glans-preserving surgery for superficial penile cancer

In this study, we investigated the safety and feasibility of glans-preserving surgery for superficial penile squamous cell carcinoma. Young patients with penile primary tumors exhibiting favorable histologic features were best suited for organ-sparing procedures, enabling them to avoid sexual disturbances. The study included 12 patients, 38-53 years of age (median age 46 years), with superficial lesions involving the glans penis, coronary sulcus, or shaft skin. After clinical staging and grading, those patients were offered a glans-preserving procedure to preserve the normal appearance and functional integrity of the glans penis. Of the 12 patients referred, the tumors were TaG1 in 4 patients, TaG2 in 3, TisG1 in 1, TisG2 in 1, T1G1 in 2, and T1G2 in 1. All patients returned to normal sexual activity 1 month postoperatively. Sexual function and sexual satisfaction were well maintained after surgery. The cosmetic results were considered satisfying/very satisfying by 83% (10 of 12 patients). Follow-up data were available on 12 patients at a mean follow-up of 62.5 months. Only 1 patient had recurrence 6 months after surgery, which was managed by a second glans-preserving surgery without recurrence. With careful patient selection and vigilant follow-up, anatomically suitable superficial penile cancer can be offered this glans-preserving surgery, while preserving function of the penis wherever possible.     

Current approach to hypoplastic left heart syndrome. Palliation, transplantation, or both?

Over the past 3 years, 35 newborn infants have been referred for surgical management of hypoplastic left heart syndrome. Surgical palliation (first-stage Norwood) or cardiac transplantation was offered. Twenty-four families (68%) chose palliation and 11 families (32%) chose cardiac transplantation. Of the 11 infants listed for cardiac transplantation, five underwent transplantation. Because of a lack of donors after an average wait of 25 days (19 to 31), the remaining six infants underwent palliation, with no perioperative deaths. Of the 30 infants undergoing palliation, including crossovers, 20 (67%) survived the first operative stage. Among the last 19 infants undergoing palliation in 1990, the early survival was 84%. Risk factors determined for poor outcome were year of operation (p less than 0.001) and circulatory arrest time greater than 50 minutes (p less than 0.001). Among the 13 infants undergoing palliation with a circulatory arrest time of less than 50 minutes, there were 12 survivors (92%); among 12 having a circulatory arrest time of more than 50 minutes, there were four survivors (33%). At intermediate follow-up, six infants have undergone second-stage procedures (Glenn), with five survivors. There were eight late deaths, four caused by respiratory infections and four caused by cardiac problems, including a thrombosed shunt in one infant. Three of five infants are alive and doing well after cardiac transplantation. Size of aorta, tricuspid regurgitation, and ventricular wall thickness did not prove to be risk factors. Given the existing data, we believe these infants should be managed selectively on the basis of donor availability and family wishes.     

Heart transplantation in children.

Orthotopic cardiac transplantation has been performed in 15 consecutive neonates and children since 1987. Diagnoses include hypoplastic left heart syndrome (5 patients), critical aortic stenosis with small left ventricle (1 patient), complex cyanotic heart disease (6 patients), and cardiomyopathy (3 patients). Twelve patients survived operation and have been followed from 1 to 45 months. Patients less than 6 years of age are managed with cyclosporine +/- azathioprine; in older patients steroid weaning is attempted. Monitoring for rejection is performed with serial echocardiography in patients under 6 years of age; older patients undergo serial biopsies. Actuarial freedom from rejection was 26% 3 months after operation; 47% were free of infection 6 months after operation. There have been no late deaths. Actuarial survival at 3 years is 79%. Nine patients have undergone postoperative catheterization. Resting hemodynamics were normal in every patient. All long-term survivors are asymptomatic and fully active. It is concluded that cardiac transplantation in neonates and children is an effective treatment option for end-stage cardiomyopathy or otherwise incurable congenital heart disease. Long-term survivors have excellent potential for full rehabilitation.     

Bacterial endocarditis: a rare complication following orthotopic cardiac transplantation.

We describe a 46-year-old man who developed infective endocarditis, meningitis, sternal abscess, and infective cerebral emboli after cardiac transplantation. Staphylococcus aureus was the infective organism. We successfully managed the patient with flucloxacillin and fusidic acid to treat infection, and with prostacyclin for systemic embolization.     

Repeated paracentesis for treatment renal failure after heart transplantation in patient with ascites

We report a case of orthotopic heart transplantation in a 42-year-old man who had cardiomyopathy with severe biventricular heart failure, ascites, and large umbilical hernia. He successfully received an orthotopic heart transplantation. After heart transplantation, renal failure was noted. Ascites and renal failure were successfully managed with repeated paracentesis. His cardiac and abdominal symptoms subsided gradually following transplantation. His umbilical hernia was repaired 55 days after the heart transplantation because of strangulation. In this case study, we report a patient with ascites who was treated for postoperative renal failure with repeated paracentesis.     

Surgically treated primary cardiac tumors in early infancy and childhood

OBJECTIVE: Primary heart tumors in childhood are rare and mostly benign. Surgical treatment is advocated when symptoms or hemodynamic impairment is present. MATERIALS AND METHODS: Between 1986 and 2003, 8 children (3 males and 5 females, age ranging 5 days to 6.7 years, median 78 days) with a clinical diagnosis of cardiac mass were treated with surgery. Diagnosis was made by prenatal echocardiography in 3 patients and by 2-dimensional Doppler echocardiography in 5 patients. RESULTS: Complete surgical excision of the cardiac mass was feasible in all but 1 patient who underwent orthotopic heart transplantation. Surgical pathology examination revealed myxoma in 2 patients, fibroma in 2 patients, rhabdomyoma in 2 patients (multiple in 1), hamartoma in 1 patient, and teratoma in 1 patient. One patient died of cerebral malignancy 38 months after cardiac transplantation. At a mean follow-up of 69.2 months (range 3-190 months), all the remaining patients are asymptomatic, with good ventricular function on 2-dimensional echocardiography and no signs of residual or recurrent tumor. CONCLUSION: Surgical excision of obstructive cardiac tumors in childhood is safely feasible. Heart transplantation may represent the only therapeutic option when the tumor extensively invades the ventricular walls. Although 2-dimensional echocardiography remains a reliable diagnostic tool, a definite diagnosis of tumor histotype requires a thorough histopathologic characterization.     

Recurrence pattern for superficial bladder cancer

AIM: Patients with superficial bladder tumors sometimes have long recurrence-free intervals. We evaluated whether patients with long recurrence-free periods had subsequent recurrences. We also clarified how these patients should be followed. MATERIALS AND METHODS: We enrolled 244 patients with superficial bladder cancer (62 pTa and 182 pT1) treated by transurethral resection of bladder tumor (TURBT) and adjuvant chemotherapy with pirarubicin. Median follow up was 75.5 months. Patients were stratified by the length of their recurrence-free interval. RESULTS: Recurrences occurred in 124 patients (50.8%). Of 185 patients who did not have a recurrence for the first 3 years, subsequent recurrences occurred in 65 patients; in more than half the first recurrence developed after 5 years or more. Ta tumors had a low recurrence rate (14.5%) with the first recurrence often developing after a long recurrence-free period. Of 40 patients who remained recurrence-free for 3 years or more after at least one recurrence occurred, 16 patients (40%) had subsequent recurrences. Furthermore, most of these patients who remained free of recurrence for more than 5 years eventually had a recurrence. The overall progression rate was 15.6%, and this did not relate to the length of the recurrence-free interval. CONCLUSION: When patients did not have a recurrence for the first 3 years, tumors subsequently often recurred, even in pTa tumors. In patients with at least once recurrence, subsequent recurrences appear to occur irrespective of the length of the recurrence-free period. Thus, we recommend that all patients with superficial bladder tumors be followed for as long as possible.     

Impact on renal function of the use of sirolimus in cardiac transplantation

INTRODUCTION: During the last few years sirolimus has been introduced as an alternative to preserve renal function in transplant recipients receiving calcineurin inhibitors. MATERIALS AND METHODS: We reviewed our results on the use of sirolimus in cardiac transplant recipients. RESULTS: Twenty-seven patients with an average age of 63 years received sirolimus. The average time after transplantation was 73.4 +/- 58.9 months and the average follow-up was 31.7 +/- 18.01 months. Sirolimus was prescribed in 37% of cases due to chronic renal failure (CRF), 14.8% because of cardiac allograft vasculopathy (CAV), 11.1% for tumors, 22.2% de novo, 7.4% for CRF and tumor, and 7.4% for CRF and CAV. Among the patients with CRF (n = 14), there were 5 (35%) on dialysis at the moment of starting the treatment and 1 was retired from dialysis. The other 4 (28.5%) patients had to be treated with dialysis after starting the treatment. In all, 42.8% of the patients with nephropathy maintained stable renal function or improved. Among the 17 (63%) patients who did not require dialysis, there was no significant change in renal function after 6 months or 1, 2, and 3 years follow-up. CONCLUSIONS: The use of sirolimus in cardiac transplantation maintains stable renal function in the majority of patients in the medium term.     

6例心脏移植围手术期的处理

目的总结6例心脏移植围手术期处理经验。方法6例晚期心肌病患者接受同种体原位心脏移植术。围术期免疫抑制剂采用赛尼哌诱导方案。维持治疗为环孢素A＋吗替麦考酚酯（或硫唑嘌呤）＋泼尼松三联方案,术后保持低水平的中心静脉压。随访23～30个月,平均（25．6＋4．2）个月。结果6例受者均存活。1例因牙髓感染并发败血症致胸部伤口延期愈合．1例出现硬膜外血肿、脑疝形成行开颅手术。所有患者围术期及随访期间受者均无急性排斥反应、移植物功能不全、肝肾功能不全等并发症。结论围术期适当强度的免疫抑制治疗,合理应用强心利尿,密切监测血流动力学和重视受者的个体化治疗是防治心脏移植术后并发症的有效方法。     

保留阴茎头手术在浅表性阴茎癌治疗中的应用

目的:探讨保留阴茎头手术治疗浅表性阴茎癌的安全性和可行性。方法:分析我院2003年1月至2010年3月间保留阴茎头手术治疗的21例浅表性阴茎癌患者的临床资料。结果:21例患者行保留阴茎头手术,年龄36～57岁,平均年龄46岁,阴茎癌病灶范围侵犯到阴茎头、冠状沟和阴茎体的皮肤组织。经临床分期分级,21例患者中,TaG1期6例、TaG2期5例、TisG1期2例、TisG2期4例、T1G1期3例、T1G2期1例。术后1个月均开始性生活,性功能和性满意度良好。21例随访2～7年,平均随访5年,2例患者分别于术后6个月和9个月出现原位肿瘤复发,经再次保留阴茎头手术治疗后,随访5年未再复发。结论:保留阴茎头的阴茎癌手术是治疗浅表性阴茎癌的有效方法之一。对合适的患者,不仅疗效安全,同时可最大限度保留阴茎的外观并使患者获得满意的性生活。     

Regression of androgen-related hepatic tumors in patients with Fanconi's anemia following marrow transplantation

Two patients with Fanconi's anemia treated for 5 years with oxymetholone developed hepatic function abnormalities in association with hepatic tumors demonstrated by isotope liver-spleen scan or abdominal echogram. The lesions resolved over a period of 26 months after allogeneic marrow transplantation, and the patients are alive and well 3 and 4 years following transplantation. The course of these patients indicates that marrow transplantation for Fanconi's anemia allows the withdrawal of androgens and subsequent regression of androgen-related hepatic tumors in patients who might otherwise have a fatal outcome.     

Rescue cardiac transplantation for early failure of the Fontan-type circulation in children

OBJECTIVE: Early failure of the Fontan-type circulation is a potentially fatal complication. We review our experience with cardiac transplantation in children presenting with end-stage heart failure in this scenario. METHODS: We performed a retrospective review. Between 1985 and 2003, 6 children aged less than 16 years were referred for cardiac transplantation. The indication for cardiac transplantation was end-stage cardiac failure early after the completion of the Fontan-type operation. RESULTS: All 6 patients listed for transplantation underwent cardiac transplantation; the median interval to transplantation from the operation was 36 days (range, 6-180 days). Four patients had undergone the Fontan procedure, and 2 had one-and-a-half-ventricle repair. All 6 patients were ventilated and inotrope dependent, with varying degrees of multiorgan dysfunction. One patient was bridged to transplantation with extracorporeal membrane oxygenation. The median age at transplantation was 7.1 years (range, 3-12.5 years), and weight was 18.9 kg (range, 11-35 kg). One patient died on the operating table (graft failure and hemorrhage). In 5 survivors the median intensive care unit stay was 10 days (range, 8-61 days). On follow-up of 6 to 81 months, there have been no subsequent deaths, and the quality of life in survivors is good. CONCLUSION: Rescue cardiac transplantation in the setting of an early failure of the Fontan-type circulation and end-stage cardiac failure is an effective treatment option and can be performed with acceptable early mortality and encouraging short-term to medium-term results.     

Right lower lobectomy for pulmonary infarction before orthotopic heart transplantation

A 39-year-old potential heart transplant recipient had a right lower lobe infiltrate and on pulmonary angiography was found to have an embolous to the common basilar artery. This was successfully managed by a right lower lobectomy, after aggressive medical management failed. The patient was treated postoperatively with antibiotics and subsequently underwent orthotopic heart transplantation. At 1 year after transplant the patient has no evidence of cardiac or pulmonary insufficiency.     

Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study.

BACKGROUND: Infants with hypoplastic left heart syndrome (HLHS) commonly undergo cardiac transplantation as primary management. METHODS: We examined outcomes of primary transplantation for unpalliated HLHS. We analyzed data from the 20 institutions of the Pediatric Heart Transplant Study Group, from January 1, 1993, through December 31, 1998, using actuarial and parametric survival analysis and competing outcomes analysis. RESULTS: During the 6 years studied, 1,234 patients were listed for cardiac transplantation; 262 patients (21.2%) had unpalliated HLHS. The number (and percentage) of patients with HLHS decreased from 58 (27% of patients listed) in 1993 to 30 (14%) in 1998. Overall, 25% of infants with HLHS died while waiting; primary cause of death was cardiac failure (50%). Of the remaining patients awaiting transplantation, 23 (9%) underwent Norwood/Fontan-type surgeries as interim palliation: 52% died. Ultimately, 175 patients underwent cardiac transplantation (67%); 50% received organs by 2 months after listing. Post-transplant actuarial survival was 72% at 5 years, with 76% of deaths (35/46) occurring within 3 months; early mortality was caused primarily by graft failure within the first 30 days after transplantation (in 54%). Among 1-month survivors, survival at 1 and at 5 years was 92% and 85%, respectively. Of the 262 patients listed with unpalliated HLHS, overall survival, taking into account mortality after listing and after transplantation, was 68% at 3 months and 54% at 5 years. CONCLUSIONS: Cardiac transplantation offers good intermediate survival for infants with unpalliated HLHS.     

Mediastinitis after pediatric cardiac surgery: a 15-year experience at a single institution

BACKGROUND: The spectrum of sternal wound infections after cardiac surgery ranges from superficial infections to a deep sternal infection known as mediastinitis. Mediastinitis is a rare but clinically relevant source of postoperative morbidity and mortality in adult and pediatric patients after cardiac surgery. METHODS: We retrospectively identified all patients diagnosed with mediastinitis after cardiac surgery from January 1987 to December 2002 (17 patients/7,616 surgeries = 0.2%). Demographic data, cardiac diagnosis, cardiac surgery, hospital length of stay, associated medical diagnosis, and surgical treatment for mediastinitis were collected. RESULTS: Fifteen pediatric patients (age < 18 years) were diagnosed with mediastinitis (mean age at diagnosis 37.5 months, range 21 days to 17 years. The median postoperative day of diagnosis was 14 days (6 to 50 days). The most common organism was Staphylococcus species (n = 9). Six patients had an associated bacteremia. The median hospital length of stay for all patients was 42.5 days (range 16 to 163 days). The hospital mortality was 1 of 15 (6%). Each patient was treated with intravenous antibiotics; sternal debridement; and rectus abdominus flap reconstruction (n = 7), pectoralis muscle flap reconstruction (n = 3), omentum reconstruction (n = 1), or primary sternal closure (n = 4). Three patients have undergone redo-sternotomy with orthotopic heart transplantation, bidirectional cavopulmonary anastomosis, and replacement of a right ventricle to pulmonary artery homograft. CONCLUSIONS: Timely diagnosis, aggressive sternal debridement, and liberal use of rotational muscle flaps can potentially minimize the morbidity and mortality in pediatric postoperative cardiac patients. Subsequent redo-sternotomy has not been problematic.     

Transcatheter aortic valve replacement 24 years after cardiac transplantation

As patient survival after cardiac transplantation has improved over the course of the last several decades, clinicians are now faced with late complications. This includes aortic stenosis which, traditionally, has been treated with reoperative sternotomy and aortic valve replacement. Transcather aortic valve replacement (TAVR) offers a minimally invasive alternative in this high-risk population. A small but growing number of cases of TAVR after heart transplantation in high-risk patients have been reported in the last 10 years; we now present a case of aortic valve replacement via a transcatheter approach 24 years after cardiac transplantation.     

Surgical management of cardiac pheochromocytoma. Resection versus transplantation.

OBJECTIVE: The authors review their experience and that of others who have reported cases in the literature on the surgical management of cardiac pheochromocytomas. SUMMARY BACKGROUND DATA: Cardiac pheochromocytomas are rare cathecolamine-producing tumors that can be densely adherent to myocardium. Because resection can be associated with significant morbidity, we sought to determine the best mode of treatment for these difficult tumors. METHOD: The authors reviewed the experience for management of cardiac pheochromocytomas in their two institutions and those reported in the literature. Follow-up was available for 21 of 26 patients up to 9 years after resection. RESULTS: Twenty-five patients had reconstruction of the native heart; five (20%) died intraoperatively from hemorrhage, one (4%) died postoperatively from sepsis, three (12%) sustained myocardial infarction, one (4%) required a mitral valve replacement, and three (12%) had incomplete resections, two of whom subsequently developed metastatic disease and died. One patient, thought to be a high risk for resection, received an orthotopic heart transplantation. CONCLUSIONS: Surgical resection of cardiac pheochromocytomas can be performed successfully. However, resection of lesions that aggressively invade adjacent myocardium is associated with significant mortality and inadequate control of the neoplasm. Cardiac transplantation should be available as an option before embarking on resection, and it should be performed if mandated by intraoperative findings.     

Current expectations in cardiac transplantation.

Survival after cardiac transplantation has improved significantly over the past 3 1/2 years at our Center as compared to previous experience (Fig. 5). Currently, survival rates for 60 patients who have had heart transplantation since late in 1973 (program year mean survival +/- S.E.) are 66 percent (+/- 6.6 S.E.), 63 percent (+/- 7.0) and 58 percent (+/- 8.2) 1, 2, and 3 years after operation, respectively. In this report the major reasons for this successful trend have been summarized. These consist of transvenous endomyocardial biopsy for diagnosis and management of graft rejection, use of RATG, immunologic monitoring for early detection of impending rejection, and cardiac retransplantation in selected cases. The present expectations for survival and rehabilitation after heart transplantation are fully comparable to the current results of renal transplantation from unrelated donors. These considerations support the inclusion of cardiac transplantation as a realistic therapeutic alternative in the management of patients with advanced heart disease irremediable by standard forms of treatment.