Non-transplant cardiac surgery for end-stage dilated cardiomyopathy in small children.

Between October 1997 and December 2000, a total of 7 pediatric patients with end-stage dilated cardiomyopathy (DCM) were referred to our service for heart transplantation; non-transplant cardiac surgery was offered as a biologic bridge. Two patients died before surgery and the in-hospital surgical mortality rate was 50%: 75% in 4 patients who received emergency surgery and 0% in 2 patients who received urgent surgery. Non-transplant cardiac surgery improved clinical outcome and acted as a biologic bridge, instead of a mechanical bridge, to heart transplantation in small children with DCM and severe heart failure.     

Partial left ventriculectomy in pediatric end-stage dilated cardiomyopathy

OBJECTIVE: Although a recent survey on pediatric cardiomyopathy in Japan showed that 48% of patients died despite the medical treatment, pediatric cardiac transplantation is not legal in Japan. We determined the feasibility of partial left ventriculectomy as an alternative to end-stage dilated cardiomyopathy. METHOD: We retrospective analyzed partial left ventriculectomy in 4 pediatric patients with end-stage dilated cardiomyopathy. RESULTS: In case 1, an 8-month-old girl underwent semiemergency partial left ventriculectomy. Her ejection fraction increased from 10% to 25%, and her condition improved initially, but she developed heart failure and underwent cardiac transplantation 6 months later in the US. In case 2, a 3-year-old boy developed severe heart failure 2 months after ventricular septal defect repair. Intensive medical therapy failed, so partial left ventriculectomy was done, which increased his ejection fraction from 15% to 35%. His condition is stable 35 months after surgery. In case 3, a 2-year-old girl with a chromosomal anomaly undergoing ventricular septal defect repair developed progressive heart failure 1 year later. Despite emergency partial left ventriculectomy, she died of hemoptysis 2 weeks postoperatively. In case 4, a 2-year-old girl developing progressive heart failure unresponsive to medical therapy after 10 months underwent elective partial left ventriculectomy and remains in stable condition 18 months postoperatively. CONCLUSION: Partial left ventriculectomy is appropriate for selected patients with end-stage dilated cardiomyopathy if medical therapy is not effective and heart transplantation is not possible.     

Improved early outcome for end-stage dilated cardiomyopathy in children

OBJECTIVE: To review the impact of management changes on the early outcomes of end-stage dilated cardiomyopathy in children. METHODS: We conducted a retrospective study of all consecutive children with end-stage dilated cardiomyopathy who received hospital treatment since 1992. Over the past 3 years the following management changes were made: (1) more aggressive use of mechanical cardiac assistance; (2) high priority listing for transplantation; and (3) ABO incompatible transplants for infants. Outcomes for 46 patients admitted between 1992 and 1999 (group I) were compared with 53 patients between 2000 and March 2003 (group II). RESULTS: In group I, 12 (26%) patients received mechanical support with recovery in 3 and transplantation in 5 (1 died). In group II, 19 (36%) patients received extracorporeal membrane oxygenation, with recovery in 5 and transplantation in 12 (all survived). The use of mechanical assistance was associated with high morbidity related to bleeding, end-organ failure, and long-term mechanical ventilation. Five patients in group II received ABO incompatible transplants and all survived. There have been no episodes of rejection or need for increased immunosuppressive therapy. Hospital mortality has been significantly reduced (group I, 37% vs group II, 11%; P <.05). CONCLUSIONS: Recent refinements in the management of end-stage dilated cardiomyopathy in children have significantly reduced early mortality. Identification of markers of early myocardial recovery and development of mechanical devices for longer term and more physiologic support are essential to achieve further improvements in outcome.     

Nontransplant cardiac surgery for end-stage cardiomyopathy

OBJECTIVE: To treat end-stage cardiomyopathy, we evaluated endoventricular circular patch plasty, partial left ventriculectomy, and valvular reconstruction alone in our 2-year experience. METHODS: Among 86 patients with heart failure evaluated between December 1996 and February 1999, 33 patients with ischemic cardiomyopathy (25 men and 8 women; mean age 61 +/- 7.8 years; New York Heart Association class 3.5 +/- 0.5) were treated with endoventricular circular patch plasty combined with coronary bypass grafting (84%) and mitral reconstruction (36%). The other 53 patients with nonischemic cardiomyopathy (45 men and 8 women; mean age 48 +/- 14.3 years, New York Heart Association class 3.7 +/- 0.5), were treated by left ventricular reduction by partial left ventriculectomy (n = 37) or patch plasty (n = 3) and valve reconstruction alone (n = 13). The first 24 patients (group I) underwent ventriculectomy with or without valve reconstruction; the more recent 29 patients (group II) underwent left ventricular reduction (n = 16) or valve reconstruction alone (n = 13) on the basis of the intraoperative echocardiographic evaluation to observe changes of wall motion and thickness during cardiopulmonary bypass. RESULTS: Ischemic Group: Hospital mortality in elective (n = 26) and emergency (n = 7) operations was 4% and 43%, and 3 patients died in the late postoperative period. Mean New York Heart Association class and ejection fraction improved from 3.5 +/- 0.5 to 1.5 +/- 0.7 and from 23% +/- 7.7% to 36% +/- 8.6%, respectively. Left ventricular end-diastolic and end-systolic volume indexes decreased from 162 +/- 46 mL/m(2) to 110 +/- 39 mL/m(2) and from 130 +/- 47 mL/m(2) to 70 +/- 32 mL/m(2), respectively. Nonischemic Group: In 40 patients with left ventricular reduction, hospital mortality in elective (n = 33) and emergency (n = 7) operations was 6% and 86%, and 5 patients died in the late postoperative period. Mean New York Heart Association class and ejection fraction improved from 3.7 +/- 0.5 to 1.7 +/- 0.6 and from 18% +/- 6.4% to 31% +/- 5.9%. Left ventricular end-diastolic and end-systolic volume indexes decreased from 203 +/- 45 mL/m(2) to 110 +/- 37 mL/m(2) and from 164 +/- 40 mL/m(2) to 79 +/- 33 mL/m(2), respectively. In 13 patients undergoing valve reconstruction alone (12 mitral with or without tricuspid and 1 tricuspid plus left ventricular assist device), hospital mortality in elective (n = 9) and emergency (n = 4) operations was 0% and 50% with no late deaths. Mean New York Heart Association class and ejection fraction improved from 3.6 +/- 0.5 to 2.0 +/- 0.5 and from 22% +/- 6.0% to 30% +/- 14.5%, respectively. Mean left ventricular end-diastolic and end-systolic volume indexes decreased from 170 +/- 34 mL/m(2) to 150 +/- 50 mL/m(2) and from 140 +/- 38 mL/m(2) to 104 +/- 40 mL/m(2), respectively. Overall mortality decreased from 50% in group I to 10% in group II. The survival estimates at 2 years were 77% (confidence limits 57%-88%) in the ischemic group and 63% (confidence limits 47%-75%) in the nonischemic group (no significant difference). The analysis of our data showed that the factors influencing the surgical results for dilated cardiomyopathy were presence of severe mitral regurgitation, preoperative New York Heart Association functional class IV with emergency operation, and operative procedures with randomly performed partial left ventriculectomy without an intraoperative echo test. CONCLUSION: Endoventricular circular patch plasty, partial left ventriculectomy, and solo valve reconstruction can be performed with an acceptably low risk as elective operations. The selection of operative procedures in idiopathic dilated cardiomyopathy and avoidance of emergency surgery improved operative mortality and morbidity. Among patients who survived at least 1 year, there were no late deaths up to 30 months' follow-up.     

Early and mid-term results of left ventricular volume reduction surgery for dilated cardiomyopathy

OBJECTIVE: To evaluate structure-oriented left ventricular volume reduction surgery (LVVRS). The purpose of this study was to report the early and mid-term results of left volume reduction surgery for dilated cardiomyopathy (DCM). METHODS: We performed LVVRS on 29 patients with DCM. The age of the patient ranged from 8 to 73 years (mean 58 +/- 18 years). There were 19 male patients (63%). Twenty-three patients were ischemic, 5 were non-ischemic, and 1 had salcoidosis. Twenty-three patients were in New York Heart Association class III or IV. Fourteen patients underwent the Dor operation, 11 underwent a septal anterior ventricular exclusion operation, and 6 underwent a modified Batista operation. Fifteen patients underwent mitral annuloplasty and 2 patients had mitral valve replacement. All patients were divided into two groups, a Dor group (n = 14) and non-Dor group (n = 15). Postoperative early results and mid-term survival rate were compared between the two groups. RESULTS: Hospital mortality was 13.8% (4/29). The causes of death were low-output syndrome (n = 3) and septic shock (n = 1). Survival rate was 80% at 1 year and 72% at 3 years. Two-year survival rate of Dor and non-Dor groups were 69.8% and 93.8%, respectively (p = 0.099). CONCLUSIONS: Early and mid-term results of LVVRS were satisfied, and the non-Dor operation tended to be superior in mid-term survival to the Dor operation. Long-term follow-up is warranted.     

Histopathologic findings in explanted heart tissue from patients with end-stage idiopathic dilated cardiomyopathy

Explanted hearts were examined to determine whether specific histopathologic features are present in the myocardium of patients with end-stage idiopathic dilated cardiomyopathy (IDC). Extensive histopathologic examination by light microscopy, electron microscopy and immunohistochemistry revealed marked fibrosis in the hearts of 21 of 37 IDC patients and in 26 of 35 patients with heart diseases of known causes. Reactive (interstitial and perivascular) fibrosis predominated in the IDC hearts, whereas both reparative (replacement) fibrosis and reactive fibrosis were found in the comparison group. Endocardial fibroelastosis was found in nine patients with IDC and in 14 patients from the comparison group. Distinct patterns of fibrosis were the sole significant histopathologic difference between myocardial samples from patients with IDC and from those with heart diseases of known causes. The diffuse presence of reactive fibrosis in IDC patients suggests a more generalised dysfunction that affects the composition of the myocardial extracellular matrix. Received: 8 May 2000 Revised: 1 February 2001 Accepted: 8 June 2001     

Novel adjunct to surgery for end-stage cardiomyopathy receiving hemodialysis

Surgical management of heart failure patients receiving hemodialysis (HD) is a challenge to surgeons and reports are limited. Five patients receiving HD underwent a mitral annuloplasty with or without restoration of the left ventricle because of class III or IV heart failure due to mitral regurgitation and poor ventricular functions. Of those, three fully recovered to NYHA class I after the cardiac procedure, however, two patients remained symptomatic and required an adjunctive procedure. For that, we converted the arteriovenous dialysis shunt to an inter-arterial bypass by dividing the venous side of the shunt and anastomosing it to the proximal radial artery (RA), followed by ligation of the RA between the two anastomoses so that the RA was bypassed by the cephalic vein. Following this procedure, left ventricular end-diastolic pressure and volume were reduced, and heart failure symptoms diminished. This simple procedure was able to reduce the cardiac overload, while keeping the vascular access intact and may be a relevant adjunct to surgical reverse remodeling in end-stage heart failure patients receiving HD.     

Dynamic cardiomyoplasty: long-term clinical results in patients with dilated cardiomyopathy

BACKGROUND: Dynamic cardiomyoplasty has been considered to be an effective method of surgical treatment of patients with end-stage heart failure, and is an alternative to heart transplantation. METHODS: We critically evaluated the long-term course of 52 patients with dilated cardiomyopathy who underwent dynamic cardiomyoplasty and were followed-up for up to 110 months. RESULTS: Dilated cardiomyopathy was due to undetermined cause in 42 patients (80.8%), Chagas disease in 8 (15.4%), viral infection in 1 (1.9%), and peripartum cardiomyopathy in 1 (1.9%). In the nonchagasic group the survival rates were 79.5% +/- 6.1%, 67.8% +/- 7.1%, 53.7% +/- 8.3%, 49.9% +/- 8.3%, 14.9% +/- 12.2%, and 14.9% +/- 12.2%, respectively, at 12, 24, 48, 60, 80 and 110 months of follow-up. In the chagasic patients the survival rates were 37.5% +/- 17.1%, 12.5% +/- 11.7%, 12.5% +/- 11.7% and 0%, respectively, at 12, 24, 48, and 60 months of follow-up, making chagasic cardiomyopathy a possible contraindication for dynamic cardiomyoplasty. CONCLUSIONS: There was no correlation between the clinical improvement and hemodynamic data. Ventricular fibrillation was a frequent cause of immediate and late death, suggesting the need for prophylactic use of antiarrhythmic drugs or implantable cardioverter/ defibrillators.     

Acute clinical deterioration in a patient with end-stage dilated cardiomyopathy and left ventricular thrombus formation

The case of a 17-year-old male patient with severe end-stage dilated cardiomyopathy and a large thrombus formation within the cavum of the left ventricle is reported. After an acute thrombectomia combined with a partial left ventriculectomy (Batista procedure), the patient was successfully treated with an appropriate left ventricular assist device (LVAD) system using a centrifugal nonocclusive pump (Biomedicus, Medtronic, Anaheim, CA, U.S.A.). Mechanical support was removed on Day 9, and the patient was discharged from the hospital on Day 19. The effectiveness of emergency mechanical support in patients with very unfavorable prognoses is discussed.     

Anesthetic management of patients with dilated cardiomyopathy undergoing non-cardiac surgery

BACKGROUND: A considerable amount of data are available regarding cardiac risk in patients with coronary artery disease, but not with patients with cardiomyopathy, undergoing non-cardiac surgery. METHODS: Reports of the anesthetic management of patients with dilated cardiomyopathy (DCM) undergoing non-cardiac surgery were identified using Medline and the Igaku-chuou-zassi (Japana Centra Revuo Medicina) database (1981-2001). The data were analyzed in terms of patient characteristics, methods of intraoperative care, and clinical outcome. RESULTS: Seventy-three patients were included. The mean value of the preoperative left ventricular ejection fraction (EF) was 31%. About 70% of patients revealed poor ventricular function (EF < 35%). EF did not correlate with the severity of congestive heart failure (CHF). Major complications occurred in 6 cases and minor ones in 23 cases. A history of CHF, advanced NYHA classification and lack of preoperative diagnosis of DCM were suggested as perioperative risk factors. CONCLUSIONS: Careful planning is inevitable in anesthesia for patients with DCM, although the rate of major perioperative complications is relatively low. Evaluation of cardiac reserve is more important than the resting value of ejection fraction. In order to clearly elucidate risk factors for adverse perioperative outcomes, further analysis will be necessary as more cases are documented.     

Systematic reductive annuloplasty of the mitral and tricuspid valves in patients with end-stage ischemic dilated cardiomyopathy

OBJECTIVE: Patients with ischemic dilated cardiomyopathy exhibit extensive remodeling of the left ventricle, annular dilation, and significant mitral and tricuspid regurgitation. These changes increase per operative morbidity and mortality, and emphasize patient candidacy for heart transplantation. The aim of this study is to show immediate and long-term results after reductive annuloplasty of double (mitral and tricuspid) orifices, performed at the time of coronary artery bypass grafting, as an alternative to heart transplantation. METHODS: There were 226 consecutive patients (205 males, 21 females) with ischemic dilated cardiomyopathy, mean ejection fraction below 30% [(26.6 +/- 3.1)%], and mean left ventricle end-diastolic internal diameter greater than 7.0 cm (7.3 +/- 0.3 cm). In addition to myocardial revascularization, Carpentier's mitral annuloplasty and posterior semicircular reductive annuloplasty were performed in 37 and 189 patients, respectively. In all 226 patients, a modified De Vega's tricuspid annuloplasty was performed. RESULTS: Postoperative 30-day mortality was 7.5% (17 patients). Survival rates after 5 and 10 years were (61.5 +/- 4.0)% and (38.05 +/- 8.0)%, respectively. CONCLUSION: Reductive annuloplasty of mitral and tricuspid orifices performed at the time of myocardial revascularization could be beneficial in selective patients with ischemic dilated cardiomyopathy. Results indicate that this method should not be recognized as a valve repair, but ventricular repair procedure also.     

Immediate results of reconstructive operations in arteriosclerosis obliterans of the iliac and femoral arteries]

The work generalizes the immediate results of restoration of the main and collateral blood flow in 240 patients with arteriosclerosis obliterans of the iliac and femoral arteries. The outcomes of surgical treatment were judged according to the clinical and ultrasonic evaluation of regional hemodynamics. The results were found to be good in 84 (35.0%), satisfactory in 104 (47.5%), and poor in 42 (17.5%) patients. The results of operations on the iliac and femoral arteries are shown separately, as well as the results of surgery in their combined lesions. Thromboses of the reconstructed arteries and prostheses were encountered in 10 (4.17%), hemorrhages in 8 (3.33%), wound infection in 16 (6.67%), and venous thromboses in 4 (2.5%) patients. For the prevention of thromboses of the deep veins the author claims dosed physical exercises on a constructed training apparatus to be advisable. Amputation of the extremity was carried out in 10 (4.17%) patients because surgical correction of the blood flow proved ineffective and specific complications occurred.     

Long-term results of reconstructive surgery for aorto-iliac arterial occlusive lesions

We report a review of 215 reconstructions for aorto-iliac arterial occlusive lesions, involving 156 patients. At 13 years, the cumulative patency rate of 158 aorto-femoral or aorto-iliac dacron synthetic bypass prostheses was 71.6 per cent as compared to 52.7 per cent at 5 years for 22 blunt endarterectomies and 47.4 per cent at 6 years for 35 axillo-femoral bypass prostheses. Of the 158 synthetic bypass prostheses, the cumulative patency rate at 13 years was 85.1 per cent in cases with no occlusive involvement of the peripheral arteries, significantly higher (p<0.03) than the 39.3 per cent patency rate at 7 years in cases in which associated femoro-politeal occlusive lesions were not treated. At 12 years, the patency rate was 53.2 per cent in cases in which associated femoro-popliteal occlusive lesions were treated during the same reconstructive intervention, i.e. markedly higher (p<0.08) than in patients in whom the degree of outflow in the distal vessels was poor. Our findings indicated that, in order to achieve a satisfactory long-term patency rate, it is important to treat associated occulusive lesions in the femoro-popliteal arteries.