Surgical treatment of cardiac pheochromocytomas

The development at our institution of the radiopharmaceutical 131-I-metaiodobenzylguanidine has permitted for the first time scintigraphic localization of pheochromocytomas. By the use of this scan in combination with contrast-enhanced computed tomography, intrapericardial pheochromocytomas have been demonstrated in eight patients at our hospital during the past 2 years. Four of these patients have been operated upon by us, and each was found to have a pheochromocytoma arising from the heart (left atrium in three and interventricular groove at the aortic root in one). While in one patient it was possible to "shell" the tumor away from the left atrial wall without cardiopulmonary bypass, in the remaining patients, bypass and cardioplegia were required to resect the pheochromocytomas without inducing life-threatening intraoperative hypertension and cardiac arrhythmias. One patient required coronary artery reconstruction and two, excision of the posterior left atrial wall with pericardial replacement. One of these latter two patients died intraoperatively of uncontrollable hemorrhage. The three remaining patients are well and normotensive after more than 1 year of follow-up. Cardiac pheochromocytomas should not be approached as typical posterior mediastinal tumors, or as they are in the abdomen, with the expectation that they will "shell away" from contiguous structures. Cardiopulmonary bypass should be available, and resection of involved myocardium may be necessary for complete removal.     

Surgical Experience of Cardiac Tumors: Early and Late Results

Purpose Cardiac tumors include benign and malignant neoplasms that arise within the cardiac chambers or myocardium. This study summarizes our surgical experiences with cardiac tumors. Methods Between 1975 and 2003, 51 patients with cardiac tumors were surgically treated. Results Myxomas. Forty-seven cardiac myxomas were excised in 46 patients with an average age of 51.7 ± 18 years. The preoperative symptoms included congestive heart failure (37%) and embolism (30%). The incidence of preoperative embolization was significantly higher in the gelatinous and lobated myxomas than in the solid and smooth form (P = 0.017). The early mortality rate was 2.2%. Although the late mortality rate was 9.7%, no patients died from cardiological causes (mean follow-up, 11.2 years). Only 1 patient required surgery for recurrence. Benign nonmyxomatous tumors. Three patients with a mean age of 26.3 ± 19.0 years showed benign nonmyxomatous tumors. There were no perioperative or late deaths. Malignant tumors. Two patients were diagnosed to have malignant tumors and although there was no perioperative death, both died postoperatively within 6 months. Conclusions Cardiac myxomas and nonmyxomatous benign cardiac tumors show excellent results after a surgical excision, with a low morbidity and mortality. A surgical resection should thus be considered as a treatment option for patients with malignant tumors.     

Surgical resection of ventricular cardiac fibromas: early and late results

BACKGROUND: Cardiac fibromas are rare tumors. Indications for and preferred type of operation are controversial, and little is known about early and late results of operation. METHODS: We retrospectively reviewed records of 18 patients who underwent resection of ventricular cardiac fibromas from 1964 to October 2002. Follow-up was obtained from current medical records and recent telephone and written correspondence. RESULTS: Seventeen patients had complete resection and 1 had subtotal resection of one or more fibromas. Tumors were located in the left ventricle (n = 12), septum (n = 4), or right ventricle (n = 2). A 2-month-old infant died intraoperatively. None of the surviving 17 patients had complete heart block. Follow-up lasted up to 33.7 years for a total of 172.1 patient-years. There were no late deaths. Fourteen patients were asymptomatic, 1 was in New York Heart Association functional class II, and 2 were in class III. There was no recurrence of tumor after complete resection and no change in size of residual tumor in the 1 patient who underwent subtotal resection. CONCLUSIONS: Although cardiac transplantation has been suggested by some as a preferable operation, most ventricular fibromas, even though extensive, can be completely resected with excellent early and late results. For patients with tumors extending into critical locations, subtotal excision can also give excellent late survival.     

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.     

Survival benefits of heart and lung transplantation.

OBJECTIVE: Heart and lung transplantation has gained acceptance as therapy for end-stage cardiac and pulmonary failure. The early and intermediate survival benefits of one center's 10-year experience with 177 patients undergoing thoracic transplantation were examined. SUMMARY BACKGROUND DATA: As experience in cardiac and pulmonary transplantation has increased, improvements in patient selection, organ preservation, preoperative support, and perioperative care have significantly reduced the early threats to patient survival. Graft dysfunction due to chronic rejection appears to be the main risk for longer-term survival, and data compiled by the United Network for Organ Sharing (UNOS) indicate a 70% 5-year survival for heart transplants and a 50% 5-year survival for lung transplant recipients. METHODS: The medical records of 120 heart recipients, 52 lung transplant recipients, and 5 heart-lung recipients were reviewed. Cumulative survival estimates were made using Kaplan-Meier analysis. The etiologies of operative and long-term mortality in each transplant population were identified. A comparison of long-term survival after heart transplantation versus coronary revascularization in a group of patients with ischemic cardiomyopathy was performed. RESULTS: Operative mortality in both the cardiac and pulmonary transplant recipients was 8%. From 1990 to 1995, 70 consecutive adult cardiac transplant procedures were performed without an operative mortality. Three of five patients survived heart-lung transplantation. The extended actuarial survival rate at 5 years was 80% for the cardiac transplant recipients. The 2-year actuarial survival rate for the lung transplant recipients was 88%. Graft dysfunction was the most common cause of operative mortality in the heart transplant group whereas infection was responsible for most of the operative mortality after lung transplantation. CONCLUSIONS: Cardiac and pulmonary transplantation can be applied to morbidly ill patients with excellent operative and intermediate-term survival.     

Clinical experience with the Novacor ventricular assist system. Bridge to transplantation and the transition to permanent application

At Stanford University, a Novacor left ventricular assist system (Baxter Healthcare Corporation, Novacor Division, Oakland, Calif.) was placed as a bridge to heart transplantation in 13 patients. During the hospitalization preceding device implantation, all patients were receiving inotropic support for biventricular failure, 11 had pulmonary edema, 6 had life-threatening ventricular arrhythmias, 5 had liver dysfunction with coagulopathy, and 2 had renal failure necessitating artificial support. The mean cardiac index before implantation of the Novacor system was 1.5. All survivors with the Novacor device had a dramatic increase in cardiac output (mean cardiac index = 3.1). One patient with cardiac allograft rejection died during implantation of the left ventricular assist system. Two patients died of pulmonary sepsis and multiorgan failure after the device was implanted. All patients who had the Novacor device implanted for more than 7 days were able to walk and ride stationary bicycles while awaiting transplantation. Ten patients (77%) underwent successful heart transplantation after a mean of 18 days' support with the Novacor device. One patient died of presumed sepsis 2 days after transplantation. Nine patients (90%) are alive 4 months to 6 years after transplantation. In the overall United States experience, 68 patients (as of May 1990) have had a Novacor left ventricular assist device implanted. Five were still being supported, 39 had received a transplant (62%), and 35 patients (90%) survived the transplant hospitalization (1 died later). No instances of device failure have occurred. Overall, the Novacor assist system provided effective bridging to transplantation, with posttransplant survival similar to results after routine transplantation. Modifications and improvements based on this clinical experience have been made in the areas of patient selection, techniques of operative placement, postoperative management, and design of the assist system. Isolated left heart support with a fully implantable left ventricular assist system will be offered as an alternative to heart transplantation for selected patients by 1992.     

Permanent ventricular assist device support versus cardiac transplantation.

BACKGROUND: Although cardiac transplantation provides excellent therapy for some patients with terminal heart failure, the results are limited by the scarcity of donor organs, reduced long-term survival, and comorbid conditions. Current experience with temporary left ventricular assist devices suggest that a permanent, totally, or near totally implantable device may be a viable alternative. METHODS: We analyzed data from the 1997 International Society for Heart and Lung Transplantation (ISHLT) Registry and other literature on heart transplantation and compared survival and complication rates with our experience and that of others with temporary ventricular assist devices. From these data, we attempted to identify those patients who would benefit most from permanent left ventricular assist systems (LVASs). RESULTS: Among heart transplant candidates, United Network for Organ Sharing (UNOS) status II, O blood type, weight >180 lb, older age, and preformed antibodies are negative factors for receipt of donor hearts. Of patients transplanted, women and nonwhites have poorer outcomes. Success with wearable LVAS's suggest some of these patients might be better served with an LVAS than with cardiac transplantation. CONCLUSIONS: Because LVAS's could be made readily available without the need for a waiting list, they might compete well with the strategy of cardiac transplantation.     

Thoracic and cardiovascular interventions after orthotopic heart transplantation.

BACKGROUND: The long-term outcome of orthotopic heart transplantation is limited by the development of cardiac allograft vasculopathy, rejection, infection, and malignancy. METHODS: After heart transplantation, we treated patients with thoracic and cardiovascular diseases: preexisting coronary artery sclerosis in 2 patients, cardiac allograft vasculopathy in 19, valvular disease in 3, mycotic ascending aortic aneurysm in 2, superior vena cava stenosis in 2, and lung neoplasm in 10 patients. RESULTS: We successfully performed coronary artery bypass grafting for preexisting coronary artery sclerosis, valve replacement for valvular disease, and patch enlargement for superior vena cava stenosis. Percutaneous transluminal coronary angioplasty for cardiac allograft vasculopathy achieved excellent initial results, but the incidence of restenosis was high (67%). One patient who underwent coronary artery bypass grafting for cardiac allograft vasculopathy died immediately after operation. Graft replacement was performed for mycotic aortic aneurysm, but 1 patient required reoperation because of recurrent aneurysm. The long-term survival rate in patients undergoing surgical resection for lung neoplasm was poor (50%). CONCLUSIONS: The need for thoracic and cardiovascular interventions in patients after heart transplantation was low (4.7%). Use of the appropriate procedures can improve the long-term survival after heart transplantation.     

The first clinical experience with heart transplantation

The article analyses the first clinical experience in orthotopic allotransplantation of the heart at the Scientific Research Institute of Transplantology and Artificial Organs, USSR Ministry of Health, in the period from October, 1986 to July, 1989 (26 transplantations of the heart and one two-stage transplantation of the heart with the use of "Poisk-10M" artificial heart for 3.5 days in the first stage). Eleven patients are alive. Follow-up periods: maximum over 2.5 years, minimum 3 months. Sixteen patients died in different periods (up to 12 months). Three-component therapy (cyclosporine A, methylprednisolone, azathioprine) was applied for immunosuppression. The authors analyse the problems of the selection of potential recipients (indications and contraindications) and donor, the optimum surgical techniques, complications of immunosuppressive therapy, and infectious complications. The first clinical experience in two-stage transplantation of the heart is analysed. The first experience in transplantation of the heart in patients allows the conclusion that the use of this method for the management of the terminal stage of congestive cardiac insufficiency is a reality.     

Primary cardiac tumors.

Cardiac tumors are a rare, but potentially curably form of heart disease. A high index of clinical suspicion is necessary for diagnosis as these tumors have protean manifestations that mimic a variety of other cardiac and noncardiac diseases. Presently, M-mode and two-dimensional echocardiography are utilized as safe, reliable, and noninvasive imaging modalities. Seventy-five per cent of these tumors are benign, with myxoma accounting for 50% and rhabodomyoma comprising 20% of lesions. Various histologic types of sarcoma are the predominant malignant cardiac neoplasms. With strict attention to avoiding perioperative tumor embolization, surgical resection of these lesions can be accomplished with minimal morbidity and mortality. Sixteen consecutive primary tumors of the heart have been surgically treated at Duke University Medical Center since 1966 with no perioperative deaths and no late recurrences.     

Pheochromocytoma. Lateral versus anterior operative approach.

The possibility of bilateral, extra-adrenal, and malignant tumors has dictated a thorough abdominal exploration through an anterior incision in the management of patients with pheochromocytomas. Careful visualization or palpation of the sites known to harbor secondary tumors is still recommended by many surgeons. The present study contrasts the results and morbidity of the retroperitoneal approach with that of the intraperitoneal operative approach for resection of pheochromocytoma. In the last 14 years, 37 patients had successful total resections of their pheochromocytomas, excluding one patient with metastasis to the liver at the time of surgery who died 10 years after operation. After preoperative localization of their tumors, 17 patients were explored anteriorly and 20 underwent resection using a lateral approach. Thirty-one patients have been followed from 2 to 141 (average 56) months. All patients have either returned to a normotensive state on no medication (27 patients) or, while requiring medication (9 patients), have had normal urinary metanephrine/catecholamine levels, except for the one patient with metatastic disease. There were substantial differences in morbidity rates between the two groups, however. Four patients (20%) had minor postoperative complications, following retroperitoneal resection that included pleural effusion, urinary retention, pulmonary congestion, and fever. Nine patients (53%) had complications when the anterior approach was used, including splenectomy in two, pneumonia, and postoperative fever. Postoperative hospital stay averaged 9.8 days (range, 4 to 21 days) for the anterior group and 6.1 days (range, 4 to 12 days) when a lateral approach was used (p = 0.002). Our data suggest that, with accurate unilateral localization, the flank, retroperitoneal approach for resection of pheochromocytoma can be used successfully with less morbidity.     

Cardiac endothelin-like immunoreactivity and preproendothelin-1 mRNA expression in human heart failure

Endothelin (ET) induces hypertrophy of cardiomyocytes and increases synthesis of collagen in vitro. Interestingly, these features are hallmarks of the cardiac remodeling taking place in heart failure. The aim of the present study was to examine cardiac ET peptide and preproET-1 mRNA synthesis in human heart failure. Cardiac tissue was obtained from 11 patients with end-stage heart failure undergoing orthothopic heart transplantation (NYHA III-IV). Cardiac tissue from nine organ donors served as controls. The specimens were examined by immunohistochemistry and mRNA slot blot analyses. Significantly stronger ET-1-like immunoreactivity (ET-1-ir) was seen in the left atrial myocardium of failing hearts compared to the left atrial myocardium of donor hearts. Within each heart, the epicardium showed the strongest ET-1-ir. Left ventricular preproET-1 mRNA expression in the entire group of patients did not differ significantly from that of donor hearts. However, hypertrophic obstructive cardiomyopathy may be associated with a twofold increase in left ventricular preproET-1 mRNA. We report an increase in cardiac ET peptide in human heart failure.     

Paraganglioma of the bladder: controversy regarding treatment

Paragangliomas or extra-adrenal pheochromocytomas account for approximately 15% of all pheochromocytomas. They are usually found in an intradiaphragmatic location. Paragangliomas of the urinary bladder are infrequent and represent 6% of the paragangliomas and 0.06% of all bladder tumors. We describe 2 cases of urinary bladder paragangliomas attended to our center and review the literature with special attention to treatment, since there is some controversy around the management of these tumors. Surgical options include partial or total cystectomy and transurethral resection, but there is no consensus regarding these treatment modalities. The 2 patients presented underwent transurethral resection with a good outcome and no recurrence or residual disease.     

Cardiac Endothelin-like Immunoreactivity and Preproendothelin-1 mRNA Expression in Human Heart Failure

Endothelin (ET) induces hypertrophy of cardiomyocytes and increases synthesis of collagen in vitro. Interestingly, these features are hallmarks of the cardiac remodeling taking place in heart failure. The aim of the present study was to examine cardiac ET peptide and preproET-1 mRNA synthesis in human heart failure. Cardiac tissue was obtained from 11 patients with end-stage heart failure undergoing orthothopic heart transplantation (NYHA III-IV). Cardiac tissue from nine organ donors served as controls. The specimens were examined by immunohistochemistry and mRNA slot blot analyses. Significantly stronger ET-1-like immunoreactivity (ET-1-ir) was seen in the left atrial myocardium of failing hearts compared to the left atrial myocardium of donor hearts. Within each heart, the epicardium showed the strongest ET-1-ir. Left ventricular preproET-1 mRNA expression in the entire group of patients did not differ significantly from that of donor hearts. However, hypertrophic obstructive cardiomyopathy may be associated with a twofold increase in left ventricular preproET-1 mRNA. We report an increase in cardiac ET peptide in human heart failure.     

Cardiac pheochromocytomas.

Cardiac pheochromocytomas are rare. Thirty cases have been reported in the literature. We report the cases of 2 more patients in whom the diagnosis was established using coronary angiography and who underwent surgical resection using cardiopulmonary bypass. We also review the literature on the subject.     

Heart transplantation in patients with amyloidosis: single-center experience

Introduction

Amyloidosis is a systemic disease. Heart transplantation in this subset of patients is contraindicated by the majority of authors. In our center, patients with heart failure due to amyloidosis have been evaluated for cardiac transplantation since 1991. The aim of this study was to analyze the outcome of these patients waiting for transplant and the effectiveness of this therapy.

Materials and methods

Since 1991, eight patients affected by amyloidosis have been evaluated and enrolled on the waiting list for transplant: five affected by AL lambda type; two by APO A1; and one by TTR. Four were transplanted, three died waiting for a donor (two from cardiac failure, one from sudden death), and one has been recently transplanted after 17 months on waiting list.

Results

Since 1985, 713 patients underwent heart transplantation in our center, five of whom were affected by amyloidosis (0.7%). Two are still alive (60 and 41 months) without evidence of cardiac amyloidotic infiltration. One patient recently underwent a combined heart-liver transplantation. Two patients died after the intervention: one sudden death after 23 months with amyloidotic infiltration of transplanted heart, and one multiple organ failure (MOF) due to progression of the systemic disease.

Conclusions

Despite the small size of the group preventing us from drawing definitive conclusion, heart transplantation may prevent therapy to arrest organ damage in patients with isolated cardiac involvement. Cardiac events are the main cause of death. Patients must be followed-up for evolution of systemic disease. The midterm survival is encouraging.     

Cardiac transplantation for hypertrophic cardiomyopathy: a valid therapeutic option.

BACKGROUND: Hypertrophic cardiomyopathy is a rare indication for cardiac transplantation, with only anecdotal reports in the literature. Transplantation has been proposed to patients with hypertrophic cardiomyopathy who remained symptomatic despite optimal medical treatment or who progressed to congestive heart failure, and when conventional surgical correction was not or was no longer possible. In this report, we analyze the clinical results of cardiac transplantation in these patients. METHODS: We retrospectively reviewed complete clinical data and prospectively gathered complete follow-up data for 14 patients who underwent orthotopic cardiac transplantation for hypertrophic cardiomyopathy at the Montreal Heart Institute and Ste-Justine Hospital between 1984 and 2001. RESULTS: Ten male (71.4%, 5 adults and 5 children) and 4 female (28.6%, 2 adults and 2 children) patients underwent heart transplantation for hypertrophic cardiomyopathy. The median age of the recipients in the pediatric group was 13 years (range, 6-16) and was 40 years (range, 22-46) in the adult group. Median duration of follow-up was 9.5 years (mean, 8.8 +/- 4.8 years; range, 1-18) and was 100% complete. We found no operative mortality and found 2 late deaths related to coronary graft atherosclerosis. Long-term survival at 5, 10, and 15 years was 100%, 85%, and 64%, respectively. Freedom from acute rejection at 1, 5, and 10 years was 73%, 32%, and 9%, respectively. The remaining 11 survivors exhibit few symptoms and are currently in New York Heart Association Class I or II. CONCLUSIONS: Cardiac transplantation is a valid therapeutic option for patients with symptomatic hypertrophic cardiomyopathy who do not respond to optimal medical management and who are not candidates for conventional surgical treatment. The long-term outcome is excellent in these patients.     

Malignant pheochromocytoma

Background Malignant pheochromocytomas are rare tumors comprising about 15% of all pheochromocytomas. A specific diagnosis of malignancy requires evidence of local invasion or metastases. The purpose of this paper is to review current management of pheochromocytomas proven to be malignant. Methods Both a review of the literature and case studies of more than 100 patients with proven malignant pheochromocytoma seen at the University of Michigan Hospitals from 1980 to 1992 are the basis of management recommendations. Representative individual cases are used to illustrate treatment principles. Results Malignancy as evidence by local invasion, particularly peritumor veins, does not preclude complete resection. Bone is the most common site of distant metastases. The¹³¹I-MIBG scintiscan is the most useful means of identifying occult metastases. Conclusions Aggressive surgical resection of local recurrences and soft tissue metastases can result in excellent palliation. Adrenergic blockade is a cornerstone of treatment, regardless whether surgical resection, triple drug therapy,¹³¹I-MIBG or external radiation therapy is ultimately utilized.      

Dobutamine stress echocardiography predicts cardiac events or death in asymptomatic patients long-term after heart transplantation: 4-year prospective evaluation.

BACKGROUND: Cardiac allograft vasculopathy (CAV) remains the major cause of death after cardiac transplantation during long-term follow-up. Nevertheless, annual angiographic evaluation is difficult to perform routinely. We evaluated the value of clinical risk factors and non-invasive testing for cardiac allograft vasculopathy in predicting cardiac events or death in asymptomatic patients with normal ventricular function during long-term follow-up after heart transplantation. METHODS: We studied 39 patients, mean aged 48 +/- 13 years, at 86 +/- 31 months after heart transplantation. Patients underwent thallium scintigraphy, treadmill stress testing, dobutamine stress echocardiography, and angiography to detect CAV. We prospectively observed all patients an additional 4 years for acute myocardial infarction, congestive heart failure, or death. RESULTS: Angiography detected CAV in 15 patients (38%). Three patients had acute myocardial infarction and another 7 had congestive heart failure, representing 25% of cardiac events during the study period. Nine deaths (23%) occurred during the same observation time. Univariate analysis showed that increased body mass index, positive dobutamine stress echocardiography results, and positive angiography results were associated significantly with cardiac events or death during follow-up. In the absence of coronary angiography, stepwise logistic regression identified positive dobutamine echocardiography results as the unique independent predictor of cardiac events (p = 0.001) or death (p = 0.002). CONCLUSION: Cardiac events and death after heart transplantation increased during long-term follow-up of this population. However, dobutamine stress echocardiography is well tolerated and, in the absence of routine angiographic evaluation, may be a strong predictor of these events.     

Pancreas transplantation: problems and prospects in intensive care units

AIM: The aim of this study is to describe personal experience in the intensive management of patients with severe diabetes undergoing pancreas transplantation. METHODS: Clinical records of subjects consecutively undergoing an isolated or combined pancreas transplant have been examinated. RESULTS: During the considered period, 10 patients received an isolated pancreas transplant and 43 a simultaneous kidney-pancreas transplantation (SPKT), including 6 using a kidney from a living donor. The mean stay in the Intensive Care Unit (ICU) was 4.7 days: 52 patients (98.2%) were transferred to the Surgical Department, whereas one (1.8%) belonging to the SPKT group died with a non-functioning graft. Ten patients (18.6%) were re-admitted because of the onset of late complications, including one SPKT who died of sudden cardiac death with functioning grafts. Arterial hypertension appeared in 51% of the recipients, and 5.6% experienced at least one hypotensive episode. Cardiac rhythm alterations were diagnosed in 5 subjects (9.4%), and myocardial ischemia in 9 (17%). CONCLUSIONS: Pancreas transplantation is a therapeutic option that can improve patients' quality of life by also slowing down the evolution of diabetes; however, it is important to bear in mind the associated risks. The best results are obtained in patients in whom the disease has not already seriously impaired the function of the various target organs.