小儿原发性心脏肿瘤27例分析

目的 探讨原发性心脏肿瘤的治疗经验.方法 回顾性分析1999年5月至2009年5月27例经心脏超声检查确诊为心脏肿瘤患者的临床资料.男性20例,女性7例;年龄24 d～12.6岁,＜1岁者16例,占59.2%.22例为单发心脏肿瘤,5例为多发.其中22例因心律失常、心包积液、呼吸困难、晕厥等症状进行手术切除,5例未施行手术.根据肿瘤的具体位置选择不同的切口径路,14例完整切除肿瘤,8例部分切除;5例行二尖瓣整形,2例行三尖瓣整形.结果 术后肿瘤组织学结果:纤维瘤5例,横纹肌瘤8例,黏液瘤4例,毛细血管瘤3例,纤维肉瘤和卵黄囊瘤各1例.16例术后循环稳定;2例出现低心排血量,经治疗后心功能恢复;4例因严重低心排血量并发多器官功能衰竭死亡.18例术后随访1～10年,2例横纹肌瘤残余肿块缩小,1例消失;1例纤维肉瘤、1例血管瘤残余肿块未增大;黏液瘤术后无复发或远处种植转移.5例未手术者随访1～3年,其中2例无明显症状;1例左心室游离壁肿瘤因心律失常死亡,另1例仍存活;1例多发性心脏肿瘤伴低心排血量患者因心力衰竭死亡.结论 小儿原发性心脏肿瘤应采用个体化治疗原则,对有明显症状者应行手术治疗,对无明显症状者要密切随访.手术的目的 不是完整切除肿瘤,而是要恢复正常的血流动力学状态.

Abstract：

Objective To analyze the experience of treatment strategies for pediatric patients with primary cardiac tumors. Methods The clinical data of 27 patients with primary cardiac tumors which detected by echocardiography from May 1999 to May 2009 was analyzed retrospectively. There were 20 male and 7 female patients, aged from 24 d to 12. 6 years. There were 59. 2% less than 1 year old at the time of diagnosis. A single tumor were present in 22 cases and multiple in 5 cases. Surgery was performed for 22 patients due to the varied significant symptoms such as arrhythmia, pericardial effusion, swoon and congestive heart failure with dyspnoea. Five patients were discharged hospital without surgical treatment. The surgical approachs were adopted according to tumor location. Complete surgical resection was performed in 14 patients and partial resection in 8 patients. Seven patients were underwent valve reconstruction,5 involving the mitral valve and 2 involving the tricuspid valve. Results Histologic examination of the surgically resected tumors showed rhabdomyomas in 8 cases, fibromas in 5 cases, hemangiomas 3 cases,myxomas in 4 cases, fibrosarcoma in 1 case and yolk sac sarcoma in 1 case. Sixteen cases revealed stable haemodynamic status postoperative. Two cases occurred apparent symptoms of low cardiac output and significant arrhythmias, finally recovery after comprehensive treatment of restoration the heart function. There was a total of 4 patients in-hospital death following surgery due to multiorgan system failure. Of the 18 patients who survived after the surgery were followed up from 1 to 10 years, echocardiography showed the residual mass of the tumor with partial resection, rhabdomyoma diminishing in 2 patients and almost vanishing in 1 patient. The residual mass of one fibrosarcoma patient and one hemangioma patient were not increased. Patients with myxomas had no recur or systemic embolisation after the initial surgery. Five nonsurgical patients were followed up from 1 to 3 years, 2 patients without haemodynamic alterations,1 patients with giant tumor of left ventricular free wall was died of arrhythmia, the other one was alive; the patient of multiple cardiac tumor with low cardiac output was died of heart failure. Conclusions Despite the benign histology of most paediatric primary cardiac tumours, there may be significant associated with morbidity and occasional mortality. Therapy strategies should be individualised: surgery is indicated in cases with significant clinical symptoms and close follow-up is necessary for asymptomatic patients. Total resection is not the only therapeutic aim. Most important is the restoration of the normal haemodynamic heart function.     

Right ventricular hydatid cyst causing recurrent pulmonary emboli.

A hydatid cyst of the heart is rare. Surgical treatment is the preferable method in the treatment of cardiac echinococcosis. A 27-year-old patient with right ventricular hydatid cyst causing recurrent pulmonary emboli and diagnosed by 2-dimensional echocardiography and treated surgically is presented.     

Combined heart and lung transplantation for unresectable primary cardiac sarcoma

OBJECTIVE: The prognosis for patients with primary cardiac sarcoma is poor. Median survival is less than 10 months, especially when complete surgical excision is not feasible. Removal of all cardiopulmonary structures involved by tumor followed by orthotopic allotransplantation has been proposed to improve long-term survival. METHODS: From 1996 through 1999, we performed combined heart and lung resection followed by en bloc heart and bilateral lung transplantation in 4 patients (2 men and 2 women): 2 with inoperable pulmonary arterial sarcoma and 2 with left atrial sarcoma extending into the pulmonary vein. RESULTS: Median age at diagnosis was 39 years (range 37-45 years). All 4 patients were given chemotherapy before transplantation: doxorubicin and ifosfamide in 2 cases, and doxorubicin, ifosfamide, mesna, and dacarbazine in 2 cases. There were no operative deaths. Median survival after transplantation was 31 months (range 5-49 months). All patients had tumor recurrence: local recurrence in the chest (n = 1) and distant metastases in the brain (n = 2) and abdomen (n = 1). One patient remains alive 49 months after disease progression with cerebral metastasis as the only site of recurrence treated with whole-brain irradiation, resection, and stereotactic radiosurgery. CONCLUSIONS: Combined heart and lung transplantation is a technically feasible treatment for highly selected patients with localized advanced primary cardiac sarcomas. The high incidence of metastatic disease, however, limits its utility.     

Cardiomyoplasty: ventricular reconstruction after tumor resection

OBJECTIVE: Although cardiac transplantation has been performed for complete removal of ventricular tumors, complete surgical resection with ventricular reconstruction is desirable. Thus patients with benign tumors would probably be cured, and those with malignant tumors would have a better prognosis. In this study extensive and complete surgical resection of ventricular tumors is followed by anatomic and functional ventricular reconstruction with a dynamic cardiomyoplasty procedure. METHODS: Seven patients (mean age, 32.7 years) underwent complete resection of ventricular tumors. Histologic types were distributed as follows: fibroma in 2 patients and sarcoma, lymphosarcoma, hemangioma, lipoma, and metastatic angiosarcoma, respectively, in the remaining 5 patients. Six of the patients were considered candidates for heart transplantation because of the extent of tumor invasion. Surgery consisted of 4 steps: (1) tumor resection; (2) coronary artery resection (when invaded by the tumor) and coronary artery bypass grafting; (3) valvular reconstruction (when possible) or replacement; and (4) ventricular wall reconstruction with a pericardial patch for closure of the ventricular defect (neoendocardium) covered by the electrostimulated latissimus dorsi muscle flap (neomyocardium). RESULTS: All patients survived surgical intervention, but 2 late postoperative deaths are reported. Among the surviving patients, early complications played a major role in their postoperative course and consisted of arrhythmias, atrioventricular block necessitating a dual-chamber pulse generator, respiratory insufficiency, and heart failure. Two patients were assisted postoperatively with an intra-aortic balloon pump. On postoperative follow-up (mean, 72.4 +/- 8.5 months), an improvement in the patients' functional status was observed. Patients moved from a mean New York Heart Association functional class of 2.8 to a mean functional class of 1.2. CONCLUSIONS: The excellent long-term evolution without recurrence, ventricular dysfunction, and/or thromboembolic complications implies that cardiomyoplasty could be recommended as an alternative to heart transplantation for the therapy of large ventricular tumors.     

Outcome of patients surviving to heart transplantation after being mechanically bridged for more than 100 days.

OBJECTIVE: The effect of long-term mechanical support on subsequent heart transplantation is still debated. METHODS: We report the outcome of 41 patients (42 +/- 12 years) bridged with left ventricular assist devices (VAD; 28 Novacor, 9 HeartMate, 2 Thoratec, and 2 DeBakey) for >100 days (218 +/- 76 days) between April 1994 and March 2000). We compared follow-up with 146 patients (55 +/- 13 years) who underwent heart transplantation during the same time without prior long-term mechanical support. RESULTS: Thirty-two of the 41 patients (78%) underwent heart transplantation, 9 patients (22%) died of multi-organ (n = 5), cardiac (n = 2), or cerebral failure (n = 2). Thirty-day post-transplant mortality includes 5 cases (3 graft failures). Within the following 2 years, another 5 patients expired, 2 of cardiac failure/sudden death. Currently, 21 of 41 patients (51%) are still alive 10 to 77 months (41 +/- 22 months) after heart transplantation (1 patient was lost for follow-up). One-year and 5-year survival rates were compared with the control group (VAD vs control, 1-year survival was 75% vs 74% and 5-year survival was 60% vs 66%). Fifteen patients are doing well in New York Heart Association Class I), and 6 are NYHA Class II despite normal left ventricular ejection fraction. Episodes of moderate acute rejection (International Society for Heart and Lung Transplantation Grade 3) occurred in 10 patients (1.3 episodes per patient), not significantly different from that of the control group (1.2 episodes per patient). Scintigraphy showed regional myocardial ischemia/transplant vasculopathy in 4 patients, and coronary angiography detected the same in 2. One patient has undergone successful retransplantation. Two patients had increased right ventricular pressure. Six patients had impaired kidney function, and 3 had impaired liver function. Seven patients experienced cytomegalovirus infection. CONCLUSIONS: Our data indicate that patients who underwent heart transplantation after long-term mechanical support have a similar survival rate and comparable cardiac morbidity associated with acute rejection episodes.     

原发性左心室肿瘤的病理特点分析

目的 总结原发性左心室肿瘤病理特点及其对外科治疗的影响.方法 回顾性分析中国医学科学院阜外医院2008年1月至2019年3月32例原发性左心室肿瘤患者的临床资料,其中男17例、女15例,平均年龄(33.88±17.89)岁.分析不同的左心室肿瘤病理类型对手术结果的影响.结果 32例左心室原发性肿瘤患者接受外科手术,术后...     

Primary cardiac tumors: early and late results of surgical treatment in 91 patients.

BACKGROUND: Between March 1980 and September 1997, 91 patients underwent evaluation and treatment for primary cardiac neoplasms. METHODS: Tumors were grouped into three categories: atrial myxomas, benign nonmyxomas, and malignant tumors. Survivors were contacted; no one was lost to follow-up. The mean follow-up for this series is 7 +/- 5 years. RESULTS: Eighty-three patients were diagnosed with atrial myxomas (Male/Female: 29/54), average age 55 +/- 13 years. The hospital mortality was 3.6% (3/83), the late mortality was 6.5% (5/80). No recurrent myxomas have been identified clinically or by echocardiography in any patient. Three patients were diagnosed with benign nonmyxoma tumors. (Male/Female: 2/1), average age 64 +/- 8 years. There were no perioperative deaths and 1 patient died 4 years postoperatively from fibroma, with no linked causes. No recurrent tumors have been identified. Five patients were diagnosed with malignant tumors. (M/F: 1/4), average age 53 +/- 16 years. The hospital mortality was 20% (1/5); in 3 patients a redo-operation was necessary after 8, 11, and 12 months because of tumor recurrence. All patients died within 3 years of the first operation (mean 13 +/- 14 months). CONCLUSIONS: Surgical resection, when possible, is the treatment of choice for all primary cardiac tumors. Patients with benign tumors are probably cured by resection and in our experience there was no known tumor recurrence. Effective palliation is possible with resection of malignant tumors, but more effective adjuvant therapy will be necessary to improve long-term prognosis.     

Dobutamine stress echocardiography for the preoperative evaluation of patients undergoing lung volume reduction surgery.

BACKGROUND: Lung volume reduction surgery has been proposed as a bridge to lung transplantation and as definitive therapy for advanced chronic obstructive lung disease. However, patient selection criteria and optimal preoperative assessment have not been clearly defined. OBJECTIVE: We investigated the feasibility, safety, and value of dobutamine stress echocardiography as a predictor of major early cardiac events in patients who underwent lung volume reduction surgery. METHODS: The study population consisted of 46 patients (21 men and 25 women, mean age 59 +/- 9 years) who underwent dobutamine stress echocardiography (maximum dose 40 microg. kg(-1). min(-1) plus atropine if needed) 180 days or less before lung volume reduction surgery. Adverse cardiac events were prospectively defined and tabulated during hospitalization after the operation and at subsequent outpatient visits. RESULTS: Dobutamine stress echocardiography was interpretable in 45 of 46 (98%) patients. There were no adverse events during testing. The studies revealed normal left ventricular systolic function at rest in all patients and normal right ventricular function in all patients but one. Thirteen patients had right ventricular enlargement. Estimated right ventricular systolic pressure was mildly elevated (>40 mm Hg) in 5 patients. Four patients (9%) had stress tests positive for ischemia. There were no perioperative deaths. Follow-up was available for 44 of 45 patients at a duration of 20.0 +/- 7.0 months. Two major adverse cardiac events occurred in the same patient in whom the results of dobutamine stress echocardiography were positive for ischemia (positive predictive value 25%, 95% confidence interval 0% to 83%; negative predictive value 100%, 95% confidence interval 90 to 100%). CONCLUSION: Despite end-stage chronic obstructive lung disease and poor ultrasound windows, dobutamine stress echocardiography is feasible and safe in patients undergoing evaluation for lung volume reduction surgery. It yields important information on right and left ventricular function and has an excellent negative predictive value for early and late adverse cardiac events.     

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.     

Tumors of the heart: surgical considerations

A series of 23 cardiac tumors is reported. Six were diagnosed at autopsy; 17 tumors were surgically explored. Eleven of the 17 were myxomas within the left atrium. Eight of the 17 patients presented with congestive heart failure; peripheral and cerebral emboli were also common. The diagnosis was made preoperatively in 10 patients. Two-dimensional echocardiography was the most reliable diagnostic tool. Follow-up averages 55 months; there have been no late deaths or recurrences. The other six surgically treated tumors were: a left ventricular rhabdomyoma, a septal lipoma, a right atrial calcified endocardial mass, a right ventricular fibrosarcoma, a rhabdomyosarcoma, and a sarcoma metastatic to the pericardium and right atrium. From this series and a review of the literature, we concluded that: benign cardiac tumors can usually be excised with a low morbidity and excellent long-term results; malignant cardiac tumors have a dismal prognosis, and operation is primarily diagnostic; tumors metastatic to the heart should be operated upon only if successful palliation seems possible.     

Primary cardiac valve tumors: early and late results of surgical treatment in 10 patients

BACKGROUND: Our goal was to study the clinical characteristics of primary valve tumors and the late surgical results of their resection. METHODS: We reviewed our clinical experience with the surgical treatment of ten primary valve tumors at Fu Wai Hospital over the past 19 years. During that time, cardiac valve tumors accounted for 2.65% of all primary cardiac tumors at our hospital, and the incidence of primary valve tumors was roughly one in 4,000 cardiac operations. There were 5 male and 5 female patients aged 2 to 66 years (mean age, 30.1 years). The clinical presentation included exertional dyspnea in 7 patients, neurological symptoms in 2, and cyanosis at rest in 1 patient. The diagnosis was established by preoperative echocardiography in 8 patients, and in the other 2, it was confirmed by the findings at operation. All of the tumors were resected. Eight of the ten tumors were benign, and two were malignant. RESULTS: All patients survived the operation and recovered uneventfully. Late outcomes were known for all patients. There were three late deaths. One patient with a benign tricuspid valve tumor died 2 months postoperatively of an electrolyte disorder. The other 2 patients with a malignant mitral valve tumor died within 1 year postoperatively. The 7 survivors, all with a benign valve tumor, were followed for an average of 5.7 years (range, 8 months to 19 years), and all were in functional class I. Exercise tolerance improved to normal levels. The latest follow-up echocardiograms showed no evidence of local recurrence in any patient. CONCLUSIONS: Excellent early and late surgical results can be obtained in patients with benign valve tumors. The prognosis for patients with a malignant valve tumor is poor.     

Mid-term follow up results of Japanese heart transplant patients operated in UCLA Medical Center.

Japanese candidates have been accepted for heart transplantation by the UCLA Medical Center in the US since 1993 due to the lack of donors available from brain-dead patients. OBJECTIVES AND METHODS: We monitored to patients who underwent such heart transplantation and have been seen at the out-patient clinic at Tokyo Women's Medical University following transplantation. Pre-operative diagnosis was dilated cardiomyopathy in all patients. One patient underwent Novacor implantation as a bridge to heart transplant. All patients underwent cardiac echocardiography and cardiac catheterization including intraluminal echography. RESULTS: All patients survived with an actuarial survival curve of 100% at 1 year, 100% at 3 years and 87% at 5 years in 4.15 years of average follow-up. Two patients died due to liver dysfunction and cerebral emboli. The postoperative functional status of patients was New York Heart Association classification I in 8 (100%). Immunosuppressive therapies included triple drug therapy using either cyclosporin or tacrolimus. The incidence of acute rejection (/pt) exceeding grade 3 was 4% within three months, 3.5% in 3-6 months, and no significant rejection episode more than 6 months after transplantation. Posttransplantation coronary artery disease was seen in 2 patients, but no progression was seen after diltiazem therapy. CONCLUSION: Our postoperative follow-up after cardiac transplantation appears to be satisfactory.     

Long-term follow-up of Thoratec ventricular assist device bridge-to-recovery patients successfully removed from support after recovery of ventricular function.

BACKGROUND: In certain forms of severe heart failure there is sufficient improvement in cardiac function during ventricular assist device (VAD) support to allow removal of the device. However, it is critical to know whether there is sustained recovery of the heart and long-term patient survival if VAD bridging to recovery is to be considered over the option of transplantation. METHODS: To determine long-term outcome of survivors of VAD bridge-to-recovery procedures, we retrospectively evaluated 22 patients with non-ischemic heart failure successfully weaned from the Thoratec left ventricular assist device (LVAD) or biventricular assist device (BVAD) after recovery of ventricular function at 14 medical centers. All patients were in imminent risk of dying and were selected for VAD support using standard bridge-to-transplant requirements. There were 12 females and 10 males with an average age of 32 (range, 12-49). The etiologies were 12 with myocarditis, 7 with cardiomyopathies (4 post-partum [PPCM], 1 viral [VCM], and 2 idiopathic [IDCM]), and 3 with a combination of myocarditis and cardiomyopathy. BVADs were used in 13 patients and isolated LVADs in 9 patients, for an average duration of 57 days (range, 11-190 days), before return of ventricular function and successful weaning from the device. Post-VAD survival was compared with 43 VAD bridge-to-transplant patients with the same etiologies who underwent cardiac transplantation instead of device weaning. RESULTS: Nineteen of the 22 patients are currently alive. Three patients required heart transplantation, 1 within 1 day, 2 at 12 and 13 months post-weaning, and 2 died at 2.5 and 6 months. The remaining 17 patients are alive with their native hearts after an average of 3.2 years (range, 1.2-10 years). The actuarial survival of native hearts (transplant-free survival) post-VAD support is 86% at 1 year and 77% at 5 years, which was not significantly different (p = 0.94) from that of post-VAD transplanted patients, also at 86% and 77%, respectively. CONCLUSIONS: Long-term survival for bridge-to-recovery with VADs for acute cardiomyopathies and myocarditis is equivalent to that for cardiac transplantation. Recovery of the native heart, which can take weeks to months of VAD support, is the most desirable clinical outcome and should be actively sought, with transplantation used only after recovery of ventricular function has been ruled out.     

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.     

Outcomes of intraoperative device closure of muscular ventricular septal defects

BACKGROUND: The surgical management of muscular ventricular septal defects (mVSD) in the small infant is a challenge particularly when multiple and associated with complex cardiac lesions. Devices for percutaneous implantation have the advantage of ease of placement and for the double umbrella designs a wide area of coverage. We reviewed our experience and clinical outcomes of intraoperative mVSD device closure for such defects in small infants. METHODS: Since October 1989, intraoperative VSD device closure was a component of the surgical strategy in 14 consecutive patient implants (median age, 5.5 months; range, 3 to 11 kg), whose defects were thought difficult to approach using conventional techniques. Nine patients had associated complex cardiac lesions, 10 multiple mVSDs, and 4 patients had a previous pulmonary artery banding. RESULTS: There were 2 early deaths, 1 in a severely ill child who preoperatively had pulmonary hypertension and left ventricular failure and another in a patient with a hypoplastic left heart. Mean pulmonary to systemic flow ratio before device insertion was 3.5:1. Complete closure was achieved in 5 patients and clinically insignificant residual shunts persisted in 7. In 2 infants with significant residual lesions concomitant pulmonary artery banding was required. Postoperative mean pulmonary to systemic flow ratio was 1.7:1. In follow-up of the 12 surviving infants (mean, 41 months), 8 had complete closure and 3 persistent residual shunts. One patient with no residual shunting required heart transplantation for progressive ventricular failure 9 years after operation. All devices were well positioned on postoperative echocardiograms. There was 1 late death due to aspiration in a patient with a tiny residual shunt. CONCLUSIONS: Infants requiring operative intervention with mVSDs are difficult to manage and have an increased mortality and morbidity. Intraoperative VSD device placement for closure of mVSDs is feasible, can avoid ventriculotomy, division of intracardiac muscle bands, and is ideally suited for the neonate or infant.     

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.     

Progression of cardiomyopathy after liver transplantation in patients with familial amyloidotic polyneuropathy, Portuguese type

BACKGROUND: Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). For several mutations progression of the cardiomyopathy has been observed after OLT. The aim of this study was to assess the course of cardiac involvement in Swedish familial amyloidotic polyneuropathy (FAP), Portuguese type, after OLT. By comparison of the echocardiographic findings before OLT with those obtained after, the course of the heart involvement was followed. METHODS: Twenty-three patients, who had undergone OLT and were examined with echocardiography 1-12 months before OLT, were available for the study. Twenty-one patients were examined 12-27 months after OLT, and 12 were re-examined 52-71 months after OLT. Two-dimensional and M-mode echocardiography were performed in accordance with the standards of the American Society of Echocardiography. RESULTS: A significantly increased septal and left ventricular posterior wall thickness and a significantly increased left atrial dimension was observed at the post-OLT examinations, indicating a progression of the amyloid heart disease. This increase of the cardiac involvement was neither correlated to waiting time for OLT or to pre-operative signs of cardiomyopathy. CONCLUSIONS: Even though the production of the amyloidogenic-mutated transthyretin is stopped by OLT, the cardiomyopathy may progress after the operation even for the Portuguese type of FAP. The increase of the septal and left ventricular posterior wall thickness after OLT is not restricted to patients with signs of left ventricular hypertrophy before the transplantation. The findings have important implications for the follow-up of FAP patients after OLT.     

Volume Reduction Surgery for End-Stage Heart Failure: Experience in Korea

Partial left ventriculectomy (PLV) is regarded as one of the alternatives to heart transplantation for idiopathic dilated cardiomyopathy (d-CMP). Between June 1996 and March 2000, 20 patients underwent left ventricular volume reduction surgery at five major cardiac centers in Korea. PLV was performed in 16 patients with d-CMP and in 1 patient with ischemic CMP. The modified Dor procedure was performed in three patients; two patients with d-CMP and one patient with ischemic CMP. Median age was 35 years (range 3-64 years). There were 13 male and 7 female patients; there were 4 patients in Class III and 16 patients in Class IV. Among the 16 patients in Class IV, 5 patients were inotropic dependent, 2 patients were resuscitated from cardiac arrest or shock in hospital, and 1 patient was treated with intra-aortic balloon pumping. Operative technique for PLV was the same as described by Batista and colleagues. For the modified Dor procedures, the apical left ventricle was opened and a circumferential pursestring suture was placed at the base of both papillary muscles to reduce the diameter of the left ventricle concomitant with mitral annuloplasty. Mitral valve repair was performed in 15 patients and mitral valve replacement was performed in 1 patient. Moderate-to-severe tricuspid regurgitation was noted in 12 patients (with tricuspid annuloplasty in 11 of these patients and replacement in 1 patient). Postoperatively, there were seven operative deaths after PLV and one death after the modified Dor procedure. Cause of death after PLV was right heart failure in four of the seven cases, sepsis in one case, and ventricular tachyarrhythmia in the remaining two cases. After the modified Dor procedure, there was one operative death with left ventricular failure. Postoperatively, mean ventricular end-diastolic dimension markedly decreased from 75.3 mm to 50.9 mm. However, this dimension had increased slightly to 58.2 mm, an average observed 22 months later. Mean left ventricular ejection fraction (LVEF) improved significantly from 20.6% to 33.5% (p < 0.0001), but decreased to 28.5% on average 22 months later (p = 0.058). Eleven patients were discharged from the hospital and followed-up for a mean of 20.2 months (range 1-41 months). During the early postoperative period, most were in good condition. However, heart failure progressed with mitral regurgitation in four patients, two of whom underwent heart transplantation. In conclusion, PLV for d-CMP seems to be an effective alternative surgical procedure to heart transplantation in Korea. The modified Dor procedure may be another alternative to transplantation for left ventricular volume reduction. However, in patients showing progression of heart failure, early intervention with ventricular assist or heart transplantation will be necessary. Also, further studies will be necessary for selection criteria and for prevention of ventricular tachyarrhythmia.     

Aneurysmal aorto-right ventricular tunnel

A successful closure of an aneurysmal aorto-right ventricular tunnel (ARVT) in a 16-year-old male patient is reported here. An attempt at device closure had failed in this patient. Diagnosis was confirmed by Doppler echocardiography, 3-dimensional computed tomography, and cardiac catheterization. Surgical closure with a Dacron patch (W.L. Gore & Associates, Flagstaff, AZ) at the aortic end and direct closure at the ventricular end was done successfully with the patient under mild hypothermia. The postoperative echocardiogram showed a competent aortic valve with a closed ARVT.     

Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience

OBJECTIVE: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. METHODS: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. RESULTS: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. CONCLUSION: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.