Familial dilated cardiomyopathy: from clinical presentation to molecular genetics.
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Cardiomyopathy, Dilated↗
Biomedical subjects
A Gavazzi
Publications and source records attributed to A Gavazzi.
Epidemiology of desmin and cardiac actin gene mutations in a european population of dilated cardiomyopathy.
AIMS: Although dilated cardiomyopathy is the most frequent form of cardiomyopathy, its aetiology is still poorly understood. In about 20-30% of cases the disease is familial with a large predominance of autosomal dominant transmission. Ten different chromosomal loci have been described for autosomal dominant forms of dilated cardiomyopathy. Only two genes have been associated with pure forms (without myopathy and/or conduction disorders) of the disease, the cardiac actin and the desmin genes. Our aim was to determine the proportion of dilated cardiomyopathy affected individuals carrying a mutation in one of these two genes. METHODS AND RESULTS: We performed (1) a systematic polymerase chain reaction-SSCP-sequencing screening of the coding sequences of cardiac actin on DNA samples from 43 probands of dilated cardiomyopathy families and 43 sporadic cases; (2) a systematic polymerase chain reaction-SSCP-sequencing screening of the coding sequences of desmin combined with a search for the described missense mutation (Ile451Met) by restriction fragment length polymorphism analysis on DNA samples from 41 probands of dilated cardiomyopathy families and 22 sporadic cases. CONCLUSION: None of the patients presents a mutation in any of these two genes. Consequently, the proportion of European dilated cardiomyopathy affected individuals bearing a mutation in (1) the cardiac actin gene is less than 1.2%, (2) the desmin gene is less than 1.6%.
Actins↗
Five-minute recording of heart rate variability in severe chronic heart failure: correlates with right ventricular function and prognostic implications.
BACKGROUND: In advanced chronic heart failure (CHF), correlation between heart rate variability (HRV) and parameters of disease severity is still unclear. A reduced HRV has been related to left but not to right ventricular function parameters. Moreover, the prognostic role of spectral measures is not fully defined. We sought to assess HRV by using a short electrocardiographic recording in ambulatory patients with severe CHF to investigate the relation of HRV with clinical neurohormonal and hemodynamic parameters and to determine its predictive prognostic power. METHODS AND RESULTS: HRV was obtained from 5-minute electrocardiographic recordings in 75 ambulatory patients with CHF referred for heart transplantation screening. Standard frequency-domain parameters (total power, low-frequency power, and high-frequency power) were calculated. Prognostic value of these autonomic markers and their correlation with clinical and instrumental parameters were also assessed. A low low-frequency/high-frequency ratio was an independent predictor of cardiac events (P =.015). No correlation was found between New York Heart Association class and HRV, whereas significant correlations were identified between norepinephrine plasma levels, several hemodynamic parameters, and spectral measures (P < or =.03). A reduced HRV, particularly a low-frequency power reduction (P =.000), was highly related to indexes of right ventricular dysfunction. CONCLUSIONS: The current data indicate that spectral analysis of HRV, calculated from short electrocardiographic recordings, may represent a simple but effective means contributing to risk stratification of patients with severe CHF. Autonomic information obtained from this analysis suggests that right ventricular dysfunction may be a critical element determining autonomic imbalance in patients with severe CHF.
Biomarkers↗
Enteroviral infection causing fatal myocarditis and subclinical myopathy.
UNLABELLED: Enteroviral RNA detection in myocarditis and dilated cardiomyopathy is rare. Enteroviral particles and RNA have recently been identified in patient's skeletal muscle, suggesting that skeletal more than heart muscle hosts the virus in chronic infection. Enteroviral RNA and virus-like particles were found in the myocardium and in the skeletal muscle of two patients with fatal myocarditis: a 39 year old man who died five days after the onset of febrile flu; and a 49 year old woman, assisted for 50 days with a left ventricular assist device, who then died from cerebral haemorrhage. Automated sequencing, alignment, and sequence comparison confirmed the enteroviral origin of polymerase chain reaction products and excluded contamination. These findings agree with prior observations of enteroviral localisation in the skeletal muscle of patients with dilated cardiomyopathy, and further support the hypothesis that skeletal rather than heart muscle may host the virus and serve as a reservoir in cardiomyopathies related to chronic infection. KEYWORDS: enterovirus; myocarditis; viral particles; skeletal muscle
Adult↗
Risk factors for chronic renal dysfunction in cardiac allograft recipients.
Renal dysfunction is one of the most common and threatening complications in heart transplant recipients. Even if ciclosporin seems to play a central role in inducing renal damage, other factors may concur or predispose to renal injury. In order to identify factors responsible for renal dysfunction, we retrospectively studied a cohort of 114 cardiac transplant recipients during a follow-up period of at least 3 years. The patients had a normal renal function before and 0.5 months after heart transplantation. Doubling of baseline serum creatinine or attainment of serum creatinine steadily above 176.8 micromol/l (2.0 mg/dl) was used as criterion to define the end-point renal dysfunction. A series of clinical and laboratory variables were obtained from the patients' charts at different time intervals, and their prognostic value for the occurrence of renal dysfunction was calculated by Cox proportional hazards models. 23 out of 114 patients reached the end point after a median time period of 21 months. High serum triglyceride, alanine aminotransferase, alkaline phosphatase, ciclosporin, urea, glucose, and hemoglobin levels were shown to be associated with the development of renal dysfunction. Four variables, i.e., triglyceride, ciclosporin, urea, and alkaline phosphatase, had an independent prognostic value. Our results confirm a role for ciclosporin in inducing renal dysfunction and identify hyperlipidemia and an increased plasma urea level as risk factors for renal dysfunction in heart transplant recipients.
Adolescent↗
APOE-491 promoter polymorphism is a risk factor for late-onset Alzheimer's disease.
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Age of Onset↗
[Improvement of HF: initiative to increase awareness and improve the management of patients with heart failure in Europe].
Heart failure is a major healthcare problem, with possibly over 10 million patients in Europe suffering from the syndrome. Its prevalence is increasing partly because the proportion of elderly population is likewise increasing. The guidelines on the diagnosis and treatment of heart failure have been published by the European Society of Cardiology and represent a landmark for judging current medical practice. Evidence suggests that heart failure is frequently underdiagnosed and undertreated, and the majority of patients may not be receiving appropriate care. Both primary healthcare physicians and hospital cardiologists are responsible for proper diagnosis and appropriate treatment of patients with heart failure. The "Improvement of HF" initiative has been developed by an International Advisory Board to increase awareness and improve the management of heart failure among primary healthcare physicians in Europe. The study first investigates how primary healthcare physicians perceive heart failure should be diagnosed and treated, and examines the availability of required resources. Subsequently, primary healthcare physicians will be interviewed to assess their actual practice by reviewing relevant case notes, covering diagnosis and treatment. The program will consist of a research phase and an educational phase. For the research phase, 10 regional centers (including both urban and rural areas) in each of the 14 participating countries in Europe will be identified, with each center randomly selecting 10 primary healthcare physicians. Each physician will supply case notes of 9 of his/her patients with diagnosed heart failure or at high risk of having heart failure. The results of the survey would identify any need to change not only perceptions but clinical practice in the diagnosis and treatment of heart failure and will be used to organize an educational program. A further study is planned in order to assess the impact of the initiative.
Europe↗
Restrictive cardiomyopathy, atrioventricular block and mild to subclinical myopathy in patients with desmin-immunoreactive material deposits.
OBJECTIVES: We present clinical data and heart and skeletal muscle biopsy findings from a series of patients with ultrastructural accumulations of granulofilamentous material identified as desmin. BACKGROUND: Desmin cardiomyopathy is a poorly understood disease characterized by abnormal desmin deposits in cardiac and skeletal muscle. METHODS: Clinical evaluation, endomyocardial and skeletal muscle biopsy, light and electron microscopy and immunohistochemistry were used to establish the presence of desmin cardiomyopathy. RESULTS: Six hundred thirty-one patients with primary cardiomyopathy underwent endomyocardial biopsy (EMB). Ultrastructural accumulations of granulofilamentous material were found in 5 of 12 biopsy samples from patients with idiopathic restrictive cardiomyopathy and demonstrated specific immunoreactivity with anti-desmin antibodies by immunoelectron microscopy. Immunohistochemical findings on light microscopy were nonspecific because of a diffuse intracellular distribution of desmin. All five patients had atrioventricular (AV) block and mild or subclinical myopathy. Granulofilamentous material was present in skeletal muscle biopsy samples in all five patients, and unlike the heart biopsy samples, light microscopic immunohistochemical analysis demonstrated characteristic subsarcolemmal desmin deposits. Two patients were first-degree relatives (mother and son); another son with first-degree AV block but without myopathy or cardiomyopathy demonstrated similar light and ultrastructural findings in skeletal muscle. Electrophoretic studies demonstrated two isoforms of desmin--one of normal and another of lower molecular weight--in cardiac and skeletal muscle of the familial cases. CONCLUSIONS: Desmin cardiomyopathy must be considered in the differential diagnosis of restrictive cardiomyopathy, especially in patients with AV block and myopathy. Diagnosis depends on ultrastructural examination of EMB samples or light microscopic immunohistochemical studies of skeletal muscle biopsy samples. Familial desminopathy may manifest as subclinical disease and may be associated with abnormal isoforms of desmin.
Adolescent↗
Mitochondrial DNA mutations and mitochondrial abnormalities in dilated cardiomyopathy.
Mitochondrial (mt)DNA defects, both deletions and tRNA point mutations, have been associated with cardiomyopathies. The aim of the study was to determine the prevalence of pathological mtDNA mutations and to assess associated defects of mitochondrial enzyme activity in dilated cardiomyopathy (DCM) patients with ultrastructural abnormalities of cardiac mitochondria. In a large cohort of 601 DCM patients we performed conventional light and electron microscopy on endomyocardial biopsy samples. Cases with giant organelles, angulated, tubular, and concentric cristae, and crystalloid or osmiophilic inclusion bodies were selected for mtDNA analysis. Mutation screening techniques, automated DNA sequencing, restriction enzyme digestion, and densitometric assays were performed to identify mtDNA mutations, assess heteroplasmy, and quantify the amount of mutant in myocardial and blood DNA. Of 601 patients (16 to 63 years; mean, 43.5 +/- 12.7 years), 85 had ultrastructural evidence of giant organelles, with abnormal cristae and inclusion bodies; 19 of 85 (22.35%) had heteroplasmic mtDNA mutations (9 tRNA, 5 rRNA, and 4 missense, one in two patients) that were not found in 111 normal controls and in 32 DCM patients without the above ultrastructural mitochondrial abnormalities. In all cases, the amount of mutant was higher in heart than in blood. In hearts of patients that later underwent transplantation, cytochrome c oxidase (Cox) activity was significantly lower in cases with mutations than in those without or controls (P = 0.0008). NADH dehydrogenase activity was only slightly reduced in cases with mutations (P = 0.0388), whereas succinic dehydrogenase activity did not significantly differ between DCM patients with mtDNA mutations and those without or controls. The present study represents the first attempt to detect a morphological, easily identifiable marker to guide mtDNA mutation screening. Pathological mtDNA mutations are associated with ultrastructurally abnormal mitochondria, and reduced Cox activity in a small subgroup of non-otherwise-defined, idiopathic DCMs, in which mtDNA defects may constitute the basis for, or contribute to, the development of congestive heart failure.
Adolescent↗
Multiple coronary thrombosis and allograft vascular disease.
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Autopsy↗
Combined human cytomegalovirus and hepatitis C virus infections increase the risk of allograft vascular disease in heart transplant recipients.
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Adolescent↗
Long-term survival effect of metoprolol in dilated cardiomyopathy. The SPIC (Italian Multicentre Cardiomyopathy Study) Group.
OBJECTIVE: To evaluate the additive effect of metoprolol treatment on long-term incidence of fatal and non-fatal cardiac events in idiopathic dilated cardiomyopathy. DESIGN: 586 patients with idiopathic dilated cardiomyopathy were prospectively enrolled in a multicentre registry and followed up for a mean (SD) of 52 (32) months. Metoprolol, carefully titrated to the maximum tolerated dose, was added to conventional heart failure treatment in 175 patients. RESULTS: Survival and transplant-free survival at seven years were significantly higher in the 175 metoprolol treated patients than in the remaining 411 on standard treatment (81% v 60%, p < 0.001, and 69% v 49%, p < 0.001, respectively). By multivariate analysis, metoprolol independently predicted survival and transplant-free survival (relative risk reduction values for all cause mortality and combined mortality or transplantation 51% (95% confidence interval 21% to 69%), p = 0.002, and 34% (5% to 53%), p = 0.01, respectively). New York Heart Association class, left ventricular end diastolic diameter, and pulmonary wedge pressure were also predictive. Seven year survival (80% v 62%, p = 0.004) and transplant-free survival (68% v 51%, p = 0.005) were significantly higher in 127 metoprolol treated cases than in 127 controls selected from the entire control cohort and appropriately matched. Metoprolol was associated with a 30% reduction in all cause mortality (7% to 48%, p = 0.015) and a 26% reduction in mortality or transplantation (7% to 41%, p = 0.009). CONCLUSIONS: The addition of metoprolol to standard heart failure treatment, including angiotensin converting enzyme inhibitors, was effective in the long-term, reducing both all cause mortality and transplantation in patients with idiopathic dilated cardiomyopathy.
Adrenergic beta-Antagonists↗
Coexistence of mitochondrial DNA and beta myosin heavy chain mutations in hypertrophic cardiomyopathy with late congestive heart failure.
OBJECTIVE: To investigate the possible coexistence of mitochondrial DNA (mtDNA) mutations in patients with beta myosin heavy chain (beta MHC) linked hypertrophic cardiomyopathy (HCM) who develop congestive heart failure. DESIGN: Molecular analysis of beta MHC and mtDNA gene defects in patients with HCM. SETTING: Cardiovascular molecular diagnostic and heart transplantation reference centre in north Italy. PATIENTS: Four patients with HCM who underwent heart transplantation for end stage heart failure, and after pedigree analysis of 60 relatives, eight additional affected patients and 27 unaffected relatives. A total of 111 unrelated healthy adult volunteers served as controls. Disease controls included an additional 27 patients with HCM and 102 with dilated cardiomyopathy. INTERVENTION: Molecular analysis of DNA from myocardial and skeletal muscle tissue and from peripheral blood specimens. MAIN OUTCOME MEASURES: Screening for mutations in beta MHC (exons 3-23) and mtDNA tRNA (n = 22) genes with denaturing gradient gel electrophoresis or single strand conformational polymorphism followed by automated DNA sequencing. RESULTS: One proband (kindred A) (plus seven affected relatives) had arginine 249 glutamine (Arg249Gln) beta MHC and heteroplasmic mtDNA tRNAIle A4300G mutations. Another unrelated patient (kindred B) with sporadic HCM had identical mutations. The remaining two patients (kindred C), a mother and son, had a novel beta MHC mutation (lysine 450 glutamic acid) (Lys450Glu) and a heteroplasmic missense (T9957C, phenylalanine (Phe)-->leucine (Leu)) mtDNA mutation in subunit III of the cytochrome C oxidase gene. The amount of mutant mtDNA was higher in the myocardium than in skeletal muscle or peripheral blood and in affected patients than in asymptomatic relatives. Mutations were absent in the controls. Pathological and biochemical characteristics of patients with mutations Arg249Gln plus A4300G (kindreds A and B) were identical, but different from those of the two patients with Lys450Glu plus T9957C(Phe-->Leu) mutations (kindred C). Cytochrome C oxidase activity and histoenzymatic staining were severely decreased in the two patients in kindreds A and B, but were unaffected in the two in kindred C. CONCLUSIONS: beta MHC gene and mtDNA mutations may coexist in patients with HCM and end stage congestive heart failure. Although beta MHC gene mutations seem to be the true determinants of HCM, both mtDNA mutations in these patients have known prerequisites for pathogenicity. Coexistence of other genetic abnormalities in beta MHC linked HCM, such as mtDNA mutations, may contribute to variable phenotypic expression and explain the heterogeneous behaviour of HCM.
Adult↗
[Beta blockers in heart failure. Experiences and recommendations for clinical use. ANMCO-Area Heart Failure].
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Adrenergic beta-Antagonists↗
Enteroviral RNA and virus-like particles in the skeletal muscle of patients with idiopathic dilated cardiomyopathy.
The role of chronic viral infection in the etiopathogenesis of idiopathic dilated cardiomyopathy (IDC) has generated considerable research. Enteroviruses were the favorite candidates as etiologic agents of IDC. However, enteroviruses were rarely demonstrated in affected hearts. We investigated whether enteroviral infection persists in the heart and in extracardiac sites, particularly in skeletal muscle, in patients with IDC. Blood and myocardial and skeletal muscle samples were collected at cardiac transplantation from 31 IDC patients, 24 non-IDC heart disease patients, and 3 heart donors. Samples underwent ultrastructural studies and ribonucleic acid (RNA) extraction. RNA was reverse-transcribed, and 2 nested fragments (bps 179 and 126) were amplified in the highly conserved 5' noncoding region of enteroviral genomic RNA. Enteroviral RNA was found in the skeletal muscle of 12 cases, whereas only 4 hearts (2 of which with positive skeletal muscle) were positive. Of the 24 controls, 2 were positive (1 muscle and heart, 1 muscle only). Automated sequencing confirmed the enteroviral nature of the amplified products. Ultrastructural study showed enterovirus-like particles in 4 of the enterovirus-positive muscles, and myopathic changes in all enterovirus-positive cases. Skeletal muscle hosts chronic enteroviral infection in more than one third of patients with sporadic IDC. Two hypotheses may explain this link. Myocardial damage may derive directly from recurrent subclinical heart infections caused by enteroviruses harbored in skeletal muscle. Alternatively, enterovirus-related myopathy may trigger an autoimmune response to antigens shared by muscle and myocardium. Further studies are needed to assess the importance of these, non-mutually exclusive mechanisms in IDC pathogenesis.
Adult↗
Ten-year experience with endomyocardial biopsy in myocarditis presenting with congestive heart failure: frequency, pathologic characteristics, treatment and follow-up.
The present study summarizes our ten-year (1985-1995) experience with endomyocardial biopsy (EMB) in patients with idiopathic congestive heart failure (CHF), with specific reference to frequency of myocarditis, treatment policy, relative benefits, and follow-up. Of the 601 patients who constituted our series, 38 were clinically suspected of having myocarditis on the bases of a very recent onset of congestive heart failure and/or of arrhythmias and/or of conduction disturbances, and of a close-to-recent history of flu-like febrile illness. Corresponding EMBs showed myocarditis in 16 of the 38 cases (42.1%). A further 10 EMBs, from patients with a recent onset of congestive heart failure without prior infection episodes, showed myocarditis. Therefore, biopsy-proven myocarditis occurred in 26 of the 601 patients (4.3%). Of the 26 cases, 21 were lymphocytic, 1 was necrotizing granulomatous, 1 was eosinophilic and occurred in a patient who later developed overt zoonosis, 1 had some giant cells within endocardial inflammatory infiltrates, and 2 were borderline forms. In active myocarditis, inflammatory cells mostly constituted of T-lymphocytes (CD45RO+) with sparse macrophages (CD68+) and a few B cells (CD20+). B-lymphocytes and macrophages, along with activated T-lymphocytes, all expressed MHC class II HLA DR molecules, which were also expressed "de novo" by activated endothelial calls of capillaries and of small intramural vessels. HLA DR revealed itself as a very useful marker for the detection of activated inflammatory and endothelial cells. We also noted an increase in the number of perivascular and interstitial mast cells. Ultrastructural study was helpful for the characterization of myocyte damage and of interactions between inflammatory cells and myocytes. In 4 cases (1 of whom was later revealed as HIV positive, and subsequently died of AIDS), we found microreticulotubular structures in endothelial cells of small vessel and capillaries; in 7 cases, there were myocyte changes similar to those described in polymyositis; in 1 case, we observed subplasmalemmal buddings, but no viral particles; in 6 cases, there was extensive myocyte damage with myofibrillar lysis and focal adipous metaplasia; the remaining 6 cases showed myocyte damage of differing extent and severity; in the borderline forms, such damage coexisted with interstitial fibrosis. One of the 21 lymphocytic myocardites was not treated because during hospital screening the patient proved to be HIV positive; of the remaining 20 active myocardites, 11 were treated with a 6-month tapered steroid and azathioprine protocol (one was treated for 24 months), while 9 were not treated. The corresponding follow-up was: 6 deaths (congestive heart failure), 2 cardiac transplants and 3 survivals (1 with pace-maker) in the treated group, and 3 deaths (2 of congestive heart failure and 1 of sudden death), 1 cardiac transplant and 5 survivals (1 on the waiting list for transplantation) in the non-treated group. One of the 2 patients with borderline myocarditis died of congestive heart failure, and 1 is alive. Of the 22 patients with clinical diagnosis of myocarditis and negative biopsy, 7 died of congestive heart failure (2 on the waiting list for transplantation), 4 underwent cardiac transplantation, and 11 are alive (1 is awaiting transplantation). Of the 20 patients currently alive, 1 was originally in NYHA class III, 15 were in class II and 4 were in class I. Of the 20 overall patients who died, 12 were originally in NYHA class IV, 6 in class III, 2 in class II; of the 8 patients who underwent transplantation, 6 were originally in NYHA class IV and 2 in class III. Our overall experience shows that the frequency of myocarditis diagnosed according to Dallas criteria is high in patients with clinical diagnosis of myocarditis, while it is extremely low in dilated cardiomyopathy patients. This finding suggests that, although non-specific, recent onset of symptoms and prior febrile infe
Adolescent↗
Afterload mismatch-related problems after "domino" heart transplantation.
UNLABELLED: The heart transplants with domino technique, which uses donor hearts from heart-lung recipients, increases the pool of donors, provides the advantage of shortening the ischemic time and makes suitable hearts for patients with pulmonary hypertension. The present study aimed to characterise the pre- and post-transplant clinical and hemodynamic profiles of patients that underwent domino transplant in Pavia. METHODS: Between 1991 and 1992, 9 heart transplants were performed with the domino procedure at I.R.C.C.S. Policlinico S. Matteo of Pavia. Domino donors (6 with primary pulmonary hypertension, 2 with Eisenmenger's syndrome due to atrial septal defect, 1 with cystic fibrosis) underwent electrocardiographic, echocardiographic, chest roentgenogram studies, and right heart catheterization and coronary angiography (for donor older than 40). Domino recipients, 6 males and 3 females with a mean age of 44 years, had dilated cardiomyopathy (4 cases), coronary artery disease (4 cases) and valvular heart disease (1 case) (group 1). Seven of the 9 cases entered the study; 2 were excluded: one because had undergone heterotopic transplantation, the other had received the heart from another country and therefore the graft had suffered from a very long ischemic time. Controls group consisted of 12 patients who had consecutively undergone cardiac transplantation with non-domino donors during the same period (group 2). Immunosuppression was similar in both groups, and consisted of a combination of cyclosporin A, azathioprine and corticosteroids, plus a 7-day-course of antithymocyte globulin. Hemodynamic and echocardiographic controls were performed at 2, 3 and 4 weeks (short-term control) and at 2 and 6 months (mid-term control) after surgery. RESULTS: Domino donors (39 +/- 12.5 years) had significantly higher mean right ventricular end-diastolic diameter and lower left ventricular diameter than normal mean values. Domino recipients had significantly higher mean pulmonary arteriolar resistances than controls; mean ischemic time was also significantly lower in group 1 than in group 2. Short- and mid-term controls after surgery in group 1 showed persistently higher systemic vascular resistances and pulmonary vascular resistances and lower cardiac output than in group 1. Two patients developed an early and unexpected increase in pulmonary wedge pressure accompanied by severe paroxysmal nocturnal dyspnea and mitral regurgitation. In all cases, the left ventricles were relatively inadequate; the combination of low cardiac output and of high systemic vascular resistances favoured the occurrence of an afterload mismatch condition that was worsened by chronic hypoxia. This condition must be known and expected in these patients after transplantation in order to provide timely and effective treatment to potentially life-threatening left ventricular failure episodes. IN CONCLUSION, the subset of transplanted patients that receives domino donors may develop left-side afterload mismatch which, combined with low cardiac output, with high systemic vascular resistances and with the effects of chronic hypoxia originally suffered by the heart, may cause sudden and unexpected left-side heart failure which has to be timely recognised and managed. Although hemodynamic adaptation of this patients is highly problematic, it does not limit the value of the domino procedure.
Adult↗
[Heart transplant over 55 years].
BACKGROUND: The age of recipient has been thought for several years to be one of the most important predictors of survival after heart transplantation. Therefore patients older than age 50 years were usually excluded from heart transplantation. The marked improvement in survival after clinical introduction of cyclosporine made a critical revision of selection criteria for heart recipients. In this article we retrospectively analyze the outcome of heart transplantation dividing the patients into two groups: Group A < or = 55 years, Group B > 55 years respectively. METHODS: We analyzed 437 patients who underwent heart transplantation, immunosuppressive protocol for all the patients was cyclosporine, azathioprine and prednisone. Group A included 284 patients, Group B 153 patients. We studied demographic, clinical and haemodynamic data pre- and post-operatory in both groups. RESULTS: In Group A was more frequent dilated cardiomyopathy (p < 0.01) and the patients arrived at heart transplantation in worse haemodynamic conditions requiring more frequently inotropic infusion and/or mechanical support (intraaortic balloon pump, left ventricular assist device). Post-operatory course was more complicated in Group B patients where ischemic cardiomyopathy was more frequent (p < 0.001); intensive care stay, inotropic drugs infusion, mechanical ventilation and hospital mortality were higher in Group B, although non significantly. The donor age was significantly higher in Group B (p < 0.001), anyway the statistical analysis did not show a correlation between donor age and more complicated post-operative course. Follow-up did not show significative differences in the two groups considering haemodynamic data, metabolic disorders, graft coronary disease, infection and rejection rates. On Group B is significantly higher tumor incidence (p < 0.05) and peripheral vascular complications (p < 0.01). Actuarial survival at 5 and 7 years is respectively in Group A 80.3 and 72.9%, in Group B 75.4 and 71% (p = ns). CONCLUSIONS: Heart transplantation in patients older than 55 years with end-stage heart disease is a valid therapeutic option with excellent long-term survival; pre-operative screening must be particularly accurate and older donors should be considered.
Aged↗