OBJECTIVETo investigate the histopathological features of primary restrictive cardiomyopathy (PRCM).

METHODSNine extransplanted hearts from heart transplantation recipients were examined. Gross and histopathological findings were observed, photographed and final pathological diagnosis was compared to clinical diagnosis. The myocardial ultrastructure changes were determined using transmission electron microscopy.

RESULTSThe hallmark pathologic feature of PRCM was distinguished by myocardial cell degeneration and hyperplastic collagen fibrils around the myocardial cells.Fibrosis was severer in left ventricle free wall than in ventricular septum and right ventricle. The degree of myocardial cell degeneration and poloidal disorder were severer in patients with reduced ejection fraction (EF) than in patients with preserved EF. Transmission electron microscope evidenced severe interstitial fibrosis, myofibrillar changes of sarcomere structure, abnormalities both on intercalated disc number and distribution.

CONCLUSIONSPRCM is characterized by hyperplastic collagen fibrils around the cardiomyocytes. Fibrosis is severer in left ventricle than in right ventricle. Sarcomere dysplasia is the main cause of PRCM, and ultrastructural examination is helpful for PRCM diagnosis.

Cardiomyopathy, Restrictive ; surgery ; Fibrosis ; Heart Transplantation ; Heart Ventricles ; Humans ; Myocardium ; pathology ; Myocytes, Cardiac ; Sarcomeres

According to the 2007 International Society for Heart and Lung Transplantation (ISHLT) report, a congenital diagnosis, infantile transplantation and being on extracorporeal membrane oxygenation (ECMO) at the time of transplant are risk factors for mortality for the patients who undergo a heart transplant, and a large body weight ratio also increases the risk of mortality. The patient of this case underwent a Ross operation and mitral valve repair due to left ventricle outflow track obstruction and mitral regurgitation. But the baby was treated with ECMO due to heart failure after the operation. When he was 3-months-old and had been on 30 days of ECMO, he underwent a heart transplant with a heart that had a high donor-recipient weight ratio (4.42). We present this case from a technical standpoint and we include a review of the relevant literature.
Body Weight ; Extracorporeal Membrane Oxygenation ; Heart ; Heart Failure ; Heart Transplantation ; Heart Ventricles ; Humans ; Infant ; Lung Transplantation ; Mitral Valve ; Mitral Valve Insufficiency ; Risk Factors ; Track and Field ; Transplants

We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.
Adult ; Azygos Vein ; Dextrocardia ; Drainage ; Heart Defects, Congenital ; Heart Transplantation* ; Heart Ventricles ; Heart* ; Humans ; Isomerism* ; Pulmonary Valve Stenosis ; Veins ; Vena Cava, Inferior ; Vena Cava, Superior

PURPOSE: Homograft benefits include excellent hemodynamics, resistance to infection, decreased thromboembolic events, ease of handling, and lack of need for anticoagulation. We examined the short and mid-term results of right ventricular outflow tract (RVOT) reconstruction using cryopreserved homografts. PATIENTS AND METHODS: From May 1998 to May 2005, 20 patients (male:female=10:10) underwent RVOT reconstruction using cryopreserved homografts. The median age was 23.8 years (range, 0.9 to 43.3 years) and the median body weight was 57kg (range, 7.3 to 80kg). Eighteen patients underwent re-operation after shunt or corrective operations. Homograft failure was defined as either re-operation for homograft replacement or patient death. Homograft dysfunction was defined as grade 3 or more than 3 of graft regurgitation and more than 40mmHg of transvalvular pressure gradient under echocardiographic examination. RESULTS: No operative mortality occurred and there were three major complications. Graft failure was observed in one male patient with tetralogy of Fallot. The 8-year freedom from graft failure was 87.5+/-11.7% and the 7-year freedom from graft dysfunction was 62.3+/-17.9%. Multivariable analysis revealed that the independent factor for graft dysfunction was age less than 10 years. In the analysis according to age group, the 7-year freedom from graft dysfunction in the group of patients older than 10 years was 100% and 25.0+/-21.7% in patients age 10 or younger (p= 0.03). CONCLUSION: Right ventricular outflow reconstruction using cryopreserved homografts provided excellent short and mid-term results in most patients in this study. However, in patients younger than 10 years old, homografts for RVOT reconstruction showed a high dysfunction rate at mid-term.
Adolescent ; Adult ; Child ; Child, Preschool ; Cryopreservation ; Female ; Heart Diseases/*surgery ; Heart Ventricles/*transplantation ; Humans ; Infant ; Intraoperative Complications ; Male ; Postoperative Complications ; Transplantation, Homologous/adverse effects ; Treatment Outcome

From August 1989 to January 1996, a total of 105 cases of bidirectional Glenn operations have been done as the interim stage for the patients with some risk of univentricular correction at Sejong General Hospital. From December 1992, we started the conversion to Fontan operations for them, and 42 cases underwent Fontan-stage operation till February 1996. Their diagnoses were univentricular heart in 19(right ventricular type : 14), tricuspid atresia 11, double outlet of right ventricle 9, and others in 3 cases. The median age of bidirectional Glenn-stage operation was 12.5 months(range 2 months to 8 years) and Fontan-stage operation was at 59.6 months of median age(range 1 year 5 months to 9 year 7 months). The mean waiting interval between the two operations was 33.88+/-17.85 months with a range of 10 months to 6 years 3 months. During the waiting periods, 18 patients developed significant systemic-pulmonary collaterals and/or systemic veno-veno collateral channels. There were 5 hospital deaths after operations due to low cardiac output in 4 and sepsis in one. Most of the Fontan-stage operations were done by the lateral tunneling with Gore-Tex tube graft patch and fenestrated with the size of 2.5~6 mm. All the patients were followed-up(7 months to 4 years 2 months, mean 21.97+/-10.82 months) and there were 5 late deaths(postoperatively 6 months to 2 years) due to thromboembolism in 1, after heart transplantation 1, plastic bronchitis 1, protein loosing enteropathy 1, and pneumonia in 1. Dividing the patients by the waiting interval of 2 years, the early correction to Fontan group (N=16) showed the better results(hospital mortality 1/16, late mortality 1/16, significant collateral development 2/16) compared to the other group(N=26) (4/26, 4/26, 16/26). In conclusion, after the bidirectional Glenn-stage operation successfully got rid of the previous risk factors, we recommand to do the Fontan-stage operation no later than 2 years of interval.
Anastomosis, Surgical ; Bronchitis ; Cardiac Output, Low ; Diagnosis ; Fontan Procedure* ; Heart ; Heart Transplantation ; Heart Ventricles ; Hospitals, General ; Humans ; Mortality ; Plastics ; Pneumonia ; Polytetrafluoroethylene ; Risk Factors ; Sepsis ; Thromboembolism ; Transplants ; Tricuspid Atresia

BACKGROUND: Recently, cell transplantation has been extensively investigated to improve heart function in dysfunctional heart. This study was designed to compare the effects of smooth muscle cells (SMC) and heart cells (HC) transplantation in dilated cardiomyopathic hamsters. MATERIAL AND METHOD: HC and SMC were isolated from heart and ductus deferens of BIO 53.58 hamsters, and cultured for transplantation. HC and SMC or culture medium were transplanted into the left ventricle of 17 weeks old adult hamsters in HC transplantation (HCTx), SMC transplantation (SMCTx), and control groups (Con) (N=10 each). Cyclosporine (5 mg/Kg) was administered subcutaneously for HCTx. Sham operated hamsters (N=10) underwent the surgery but did not receive an injection. At 4 weeks after transplantation, heart function was evaluated in all groups using a Langendorff perfusion apparatus. RESULT: Histology showed severe focal myocardial necrosis in all groups. HCTx and SMCTx formed huge muscle tissue in dilated myocardium. SMCTx and HCTx had better heart function than Con and sham (p < 0.01). And SMCTx had better peak systolic pressure (p < 0.05) and developed pressure (p < 0.05) than HCTx. But sham and Con did not any statistical make difference. CONCLUSION: SMCTx and HCTx formed muscle tissue and improved ventricular function in hamsters with dilated cardiomyopathy. And SMCTx showed better heart function in peak systolic pressure and developed pressure than HCTx.
Adult ; Animals ; Blood Pressure ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Cell Transplantation ; Cricetinae* ; Cyclosporine ; Heart Transplantation ; Heart Ventricles ; Heart* ; Humans ; Muscle Cells* ; Myocardium ; Myocytes, Smooth Muscle ; Necrosis ; Perfusion ; Transplantation ; Transplants ; Vas Deferens ; Ventricular Function

BACKGROUND: New 2-dimensional strain echocardiography enables quantification of right ventricular (RV) mechanics by assessing global longitudinal strain of RV (GLSRV) in patients with pulmonary arterial hypertension (PAH). However, the prognostic significance of impaired GLSRV is unclear in these patients. METHODS: Comprehensive echocardiography was performed in 51 consecutive PAH patients without atrial fibrillation (40 females, 48 +/- 14 years old) with long-term follow-up. GLSRV was measured with off-line with velocity vector imaging (VVI, Siemens Medical System, Mountain View, CA, USA). RESULTS: GLSRV showed significant correlation with RV fractional area change (r = -0.606, p < 0.001), tricuspid annular plane systolic excursion (r = -0.579, p < 0.001), and RV Tei index (r = 0.590, p < 0.001). It showed significant correlations with pulmonary vascular resistance (r = 0.469, p = 0.001) and B-natriuretic peptide concentration (r = 0.351, p = 0.012). During a clinical followup time (45 +/- 15 months), 20 patients experienced one or more adverse events (12 death, 2 lung transplantation, and 15 heart failure hospitalization). After multivariate analysis, age [hazard ratio (HR) = 2.343, p = 0.040] and GLSRV (HR = 2.122, p = 0.040) were associated with adverse clinical events. Age (HR = 3.200, p = 0.016) and GLSRV (HR = 2.090, p = 0.042) were also significant predictors of death. Impaired GLSRV (> or = -15.5%) was associated with lower event-free survival (HR = 4.906, p = 0.001) and increased mortality (HR = 8.842, p = 0.005). CONCLUSION: GLSRV by VVI showed significant correlations with conventional echocardiographic parameters indicating RV systolic function. Lower GLSRV (> or = -15.5%) was significantly associated with presence of adverse clinical events and deaths in PAH patients.
Atrial Fibrillation ; Disease-Free Survival ; Echocardiography ; Female ; Follow-Up Studies ; Heart Failure ; Heart Ventricles ; Humans ; Hypertension* ; Lung Transplantation ; Mechanics ; Mortality ; Multivariate Analysis ; Vascular Resistance

BACKGROUND: The aim of this study is to evaluate the long term results of creating various right ventricle to pulmonary artery conduits for treating complex congenital heart disease. MATERIAL AND METHOD: Between June 1986 and July 2006, we retrospectively reviewed 245 patients who underwent reconstruction of the right ventricular outflow tract with various kinds of conduits. 410 operations were done in 245 patients, the mean age at operation was 3.2+/-4.9 years (range: 7 days~45 years) and the mean body weight was 12.5+/-8.7 kg (range: 2.4~76.3 kg). RESULT: We used the following conduits: Polystan conduit, Shelhigh conduit, Carpenter-Edward conduit, Dacron graft with an artificial valve, valveless Gore Tex vascular graft, homograft and hand-made bovine or autologous pericardial conduit. The mean follow up duration was 6.3+/-5.2 years. Redo operation for RV-PA conduit dysfunction was performed in 131 patients, a second redo was done in 31 and a third redo was done in 3. The reoperation free rates were 67.3%, 48.5% and 39.4% for 5 years, 10 years and 15 years, respectively. The homograft showed the best durability, followed by the Dacron graft with artificial valve and the Carpentier-Edward conduit. The larger sized conduit showed better durability. CONCLUSION: The homograft showed lowest reoperation rate and a smaller size of conduit showed the highest reoperation rate. The reoperation rate for the RV-PA conduit was about 35% at 5 years, so it is mandatory to develop the more durable conduit for RV outflow.
Body Weight ; Follow-Up Studies ; Heart Diseases ; Heart Ventricles ; Humans ; Polyethylene Terephthalates ; Polytetrafluoroethylene ; Pulmonary Artery ; Pulmonary Atresia ; Reoperation ; Retrospective Studies ; Tetralogy of Fallot ; Transplantation, Homologous ; Transplants

The function of the transplanted heart will be affected by acute allograft rejection, chronic rejection, high blood pressure and so on, which may induce the reconstruction of the left ventricle and the increase of left ventricular mass (LVM), and eventually lead to left ventricular hypertrophy that will significantly affect the prognosis of heart transplantation (HT). The purpose of this study was to dynamically monitor the changes of left ventricular geometric patterns after HT using two-dimensional echocardiography and to understand the remodeling process and its possible influencing factors. The left ventricular internal diameter, interventricular septal wall thickness, posterior wall thickness at end diastole were measured and the relative wall thickness (RWT), left ventricular mass, left ventricular mass index were calculated respectively in 34 HT patients and 34 healthy volunteers by two-dimensional echocardiography. The type of left ventricular geometry was identified based on the echocardiographic determination of LVM index (LVMI) and RWT. The HT patients were divided into three groups according to the time length after surgery: A (3 months postoperatively), B (6 months postoperatively) and C (12 months postoperatively). We compared the parameters of left ventricle between HT group and normal control group, and explored the risk factors causing the increase of LVM. The results showed that 4 patients (16%) in group A had concentric remodeling. Nine patients (34.62%) in group B had reconstruction, including 5 cases of concentric remodeling, 2 cases of concentric hypertrophy and 2 cases of eccentric hypertrophy. The hypertrophy incidence rate was 15.4% in group B. 15 patients (62.5%) had reconstruction in group C, including 9 cases of concentric remodeling, 5 cases of concentric hypertrophy, and 1 case of eccentric hypertrophy. The prevalence of hypertrophy was 25%. Multivariate analysis showed that hypertension and acute rejection history were the risk factors that resulted in left ventricular hypertrophy. It is concluded that the left ventricular remodeling occurs following cardiac transplantation at an early stage and the incidence of left ventricular hypertrophy increases with survival time. In this study, the one-year prevalence of left ventricular hypertrophy was 25% after surgery. Hypertension and acute rejection history are risk factors that can predict the left ventricular hypertrophy.
Adult ; Cardiomegaly ; diagnostic imaging ; etiology ; Case-Control Studies ; Echocardiography ; Female ; Heart Transplantation ; adverse effects ; Heart Ventricles ; diagnostic imaging ; Humans ; Male ; Middle Aged ; Postoperative Period ; Ventricular Remodeling

OBJECTIVETo observe the changes of surface ECG and cell couplings between sinoatrial node cells and myocardial cells following transplantion of pedicled autologous sinoatrial node tissue graft into the right ventricle of a canine model of complete atrioventricular block.

METHODSTen healthy dogs were randomized into transplantation group and control group. Pedicled autologous sinoatrial node tissue grafts were transplanted into the right ventricle in the transplantation group, while the sinoatrial nodes were only excised in the control group after placement of temporary myocardial pacing wires. The changes of surface ECG were observed at 1, 2, 3 and 4 weeks postoperatively. At 4 weeks, complete atrioventricular block was induced in the dogs by radiofrequency ablation of the His bundle. The heart rate of the dogs in both groups were recorded after the injection of isoproternol (ISO) from the femoral vein, and the transplanted tissue graft was observed under optical and transmission electron microscopes.

RESULTSNo significant changes occurred in the surface ECG. All the dogs showed ECG waveforms specific of complete heart block after the ablation, and the ventricular heart rates were similar between the two groups (P>0.05). The ventricular heart rate did not undergo obvious changes after ISO injection (P>0.05). The transplanted pedicled autologous sinoatrial node survived in the dogs and the sinoatrial node cells established desmosome junctions with the myocardial cells, but the number of junctions was not sufficient to support heart pacing.

CONCLUSIONDesmosome junction can occur between ventricular myocardial cells and sinoatrial node cells at the edge of transplanted pedicled autologous sinoatrial node tissue.

Animals ; Atrioventricular Block ; physiopathology ; surgery ; Cardiotonic Agents ; pharmacology ; Dogs ; Electrocardiography ; Female ; Heart Rate ; drug effects ; Heart Ventricles ; surgery ; Intercellular Junctions ; Isoproterenol ; pharmacology ; Male ; Myocardium ; cytology ; Sinoatrial Node ; cytology ; transplantation ; Tissue Transplantation ; Transplantation, Autologous