Diagnosis Of Dilated Cardiomyopathy Research Articles

Novel left ventriculoplasty for nonischemic dilated cardiomyopathy with functional mitral regurgitation

Novel left ventriculoplasty for nonischemic dilated cardiomyopathy with functional mitral regurgitation

Genetic Predictors and Remodeling of Dilated Cardiomyopathy in Muscular Dystrophy

Dystrophin gene mutations cause 2 common muscular dystrophies, Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). Both are frequently associated with dilated cardiomyopathy (DCM) and premature death. We hypothesized that early diagnosis and treatment of DCM in DMD/BMD patients would lead to ventricular remodeling and that specific dystrophin gene mutations would predict cardiac involvement. Sixty-nine boys with DMD (n=62) and BMD (n=7) (mean age, 12.9 and 13.7 years, respectively) were referred to our Cardiovascular Genetics Clinic for evaluation, including echocardiography and DNA analysis. Follow-up evaluations were scheduled yearly until the first abnormal echocardiogram indicative of DCM and quarterly thereafter. After the first abnormal echocardiogram, angiotensin-converting enzyme inhibitor or beta-blocker therapy was started. beta-Blockers were added if echocardiography showed no ventricular remodeling in angiotensin-converting enzyme inhibitor-treated patients after 3 months. DCM was diagnosed in 31 subjects (DMD, 27/62, 44%; BMD, 4/7, 57%) (mean age at onset, 15.4+/-2.8 years; range, 10.4 to 21.2 years). All 31 subjects were begun on pharmacological therapy after diagnosis. On follow-up (n=29), 2 subjects (both DMD) showed stable DCM, 8 subjects (all DMD) showed improvement, and 19 subjects (16 DMD; 3 BMD) showed normalization of left ventricular size and function (total improvement, 27/29 [93%]). DNA analysis in 47 cases (68%) revealed a significant association between DCM and exon 12 and 14 to 17 mutations, possible protection against DCM by exon 51 to 52 mutations, and a trend toward significant association between onset of DCM and exon 31 to 42 mutations. Statistical significance was based on nominal probability values. Early diagnosis and treatment of DCM may lead to ventricular remodeling in DMD/BMD patients. Specific dystrophin gene mutations appear to be predictive of cardiac involvement, while other mutations may protect against or inhibit development of DCM. Further studies evaluating the impact of early intervention strategies on left ventricular geometry and function in muscular dystrophy patients seem warranted.

Left main coronary artery atresia masquerading as dilated cardiomyopathy treated with aortic reimplantation

Left main coronary artery atresia masquerading as dilated cardiomyopathy treated with aortic reimplantation

Idiopathic Dilated Cardiomyopathy in Children: A Single Medical Center's Experience

The prognosis of patients with idiopathic dilated cardiomyopathy (DCM) is poor. Most patients die while waiting for cardiac transplantation because of the small number of donors in Taiwan. The purpose of this study was to review our experience with pediatric patients diagnosed with idiopathic DCM and attempt to discover prognostic factors. Eighteen patients with idiopathic DCM presenting between 1990 and 2004 were identified. They were classified into 2 groups according to outcome: group 1 comprised 13 patients who died; group 2 comprised 5 who survived. Clinical findings and laboratory investigations were compared between the 2 groups. The age at initial diagnosis for the 18 patients (11 males, 7 females) ranged from fetus to 13 years (median, 3 months). The follow-up period ranged from 12 days to 44 months (median, 7 months) in group 1, and from 1 to 48 months (median, 39 months) in group 2. Of the 18 patients, 13 (72%) died: 11 died from severe heart failure while waiting for cardiac transplantation. The cumulative survival rate was 50% at 1 year and 28% at 4 years. The presence of arrhythmia and low left ventricular ejection fraction were predictive of a poor outcome. The diagnosis of idiopathic DCM in children is associated with a generally poor prognosis. The lack of available donors results in significant mortality for pediatric patients awaiting transplantation. Advocating organ donation to increase the size of the organ donor pool is needed to significantly reduce the mortality rate in such patients.

Evaluation of the phospholamban gene in purebred large-breed dogs with dilated cardiomyopathy

To evaluate the role of the phospholamban gene in purebred large-breed dogs with dilated cardiomyopathy (DCM). 6 dogs with DCM, including 2 Doberman Pinschers, 2 Newfoundlands, and 2 Great Danes. All dogs had clinical signs of congestive heart failure, and a diagnosis of DCM was made on the basis of echocardiographic findings. Blood samples were collected from each dog, and genomic DNA was isolated by a salt extraction method. Specific oligonucleotides were designed to amplify the promoter, exon 1, the 5'-part of exon 2 including the complete coding region, and part of intron 1 of the canine phospholamban gene via polymerase chain reaction procedures. These regions were screened for mutations in DNA obtained from the 6 dogs with DCM. No mutations were identified in the promoter, 5' untranslated region, part of intron 1, part of the 3' untranslated region, and the complete coding region of the phospholamban gene in dogs with DCM. Results indicate that mutations in the phospholamban gene are not a frequent cause of DCM in Doberman Pinschers, Newfoundlands, and Great Danes.

Dilatative Kardiomyopathie bei tachykardem Vorhofflattern

We present a 38-year-old male patient with progressive exertional dyspnoea and intermittent palpitations. The initial examination detected a regular tachycardia with a heart rate of 160 bpm. EXAMINATIONS, DIAGNOSIS: In association with intermittent palpitations the 12-lead ECG detected a typical type of atrial flutter. A transthoracic echocardiography and cardiac catheterization revealed a left ventricular dilation and marked systolic dysfunction (EF 39 %) with normal coronaries. The diagnosis of dilated cardiomyopathy was made on these findings. On the basis of this diagnosis, we treated our patient by radiofrequency catheterablation, which successfully terminated atrial flutter. Three month after ablation the echocardiography was normal with an increase of the shortening fraction from 18 to 37 % and the patient was asymptomatic. Retrospectively the diagnosis of tachycardia-induced cardiomyopathy was made. Tachycardia-induced cardiomyopathy is a rare, but reversible form of dilated cardiomyopathy. The cornerstone in the management of these patients is to achieve a normal ventricular beating rate. Radiofrequency catheterablation is a curative therapeutic option that can terminate tachyarrhythmias and lead to significant improvement of left ventricular diameters, systolic function and symptoms in patients with tachycardia-induced cardiomyopathy.

Histologic Characterization of Canine Dilated Cardiomyopathy

Dilated cardiomyopathy (DCM), characterized by chamber dilatation and myocardial systolic and diastolic dysfunction, is one of the most common heart diseases in dogs. The clinical diagnosis is based on findings on echocardiographic and Doppler examinations, with the active exclusion of other acquired or congenital heart diseases. However, the echocardiographic criteria for the diagnosis of DCM are not wholly specific for the disease, and histologic examination may be necessary for final diagnosis. Review of reports on histologic findings in dogs with clinically diagnosed DCM reveals two histologically distinct forms of DCM: 1) cardiomyopathy of Boxers and Doberman Pinschers, corresponding to the "fatty infiltration-degenerative" type and 2) the form seen in many giant, large-, and medium-sized breeds, including some Boxers and Doberman Pinschers, classified as the "attenuated wavy fiber" type of DCM. The histologic changes of the attenuated wavy fiber type of DCM may precede clinical and echocardiographic signs of heart disease, thus indicating an early stage of DCM.

Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation

ObjectivesWe studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation. BackgroundThe outcome of pediatric DCM patients ranges from recovery to a 50% to 60% chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain. MethodsWe performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation. ResultsAt the time of last follow-up, 11 patients (12%) had died without transplantation; 20 (22%) underwent transplantation; 27 (30%) had persistent cardiomyopathy; and 33 (36%) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90%, and five-year survival was 83%. However, actuarial freedom from “heart death” (death or transplantation) was only 70% at one year and 58% at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation. ConclusionsPediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.

Proposed Guidelines for the Diagnosis of Canine Idiopathic Dilated Cardiomyopathy

Dilated cardiomyopathy (DCM) is a major cause of morbidity and mortality in various dog breeds. The diagnosis of overt DCM is not normally problematic, although the importance of active exclusion of other causes of the dilated, hypokinetic heart is emphasised. Recent interest in human familial DCM has prompted a number of researchers to investigate the genetic basis of canine DCM. Prospective screening of dogs from lines with familial prevalence of DCM may identify dogs with pre-clinical (“occult”) DCM. Dogs with other echocardiographic abnormalities or arrhythmias may also be identified. It is clear that dogs, like humans, have a prolonged pre-symptomatic phase of the disease extending over years. The ESVC DCM taskforce was established to provide the veterinary cardiology community with guidelines for the diagnosis of DCM, predominantly based on 2D and M-mode echocardiography. Diagnosis of DCM requires all of the following: (i) Left ventricular dilatation (ii) Reduced systolic function (iii) Increased sphericity of the left ventricle. We propose a scoring system for the identification of dogs in the pre-clinical stages. These include a number of major criteria and minor criteria. Future prospective longitudinal studies are required to test these in different breed populations to assess their predictive power and further refinements may be required. The importance of post mortem confirmation of disease is emphasised, and the two major histopathological features associated with DCM, the attenuated wavy fibre and the fibro-fatty infiltration-degenerative forms, require further investigation to identify the different aetiopathogenetic factors which may be involved.

Heart transplantation in chemotherapeutic dilated cardiomyopathy

Anthracycline cardiotoxicity can induce dilated cardiomyopathy (DCM). Nine patients (four men) experienced postchemotherapy DCM: age at time of tumour diagnosis ranged from 1–45 years (mean 13.5 ± 19 years); interval time between tumour and HT was 3–23 years (mean 10.8 ± 6.6) and age at HT ranged from 10–65 years (30.8 ± 20.1). Interval between end of chemotherapy and beginning of cardiac symptoms was 5.71 ± 4.6 years. Mean age at DCM diagnosis was 19.2 ± 19.7 (range 1–50 years). Interval between start of chemotherapy and DCM ranged from 1 month to 10 years (mean 3.15 ± 3.6 years). Tumours were Ewing sarcoma (7-year-old boy), paratesticular rabdomyosarcoma (1-year-old boy), Wilms tumor with pulmonary metastasis (3-year-old girl), bilateral breast carcinoma (45-year-old woman), uterine leiomyosarcoma (44-year-old woman), acute myelocytic leukemia (1.5-year-old boy and 17-year-old girl), and chronic myelocytic leukemia (5-year-old boy). All patients had high pulmonary resistance values. One patient with chronic myelocytic leukemia (14 year-old at HT) died due to graft failure on the first postoperative day. At follow-up (mean, 80.4 ± 69.3 months) two patients died: a 32-year-old woman (acute myelocytic leukemia) 1 year after HT for sepsis and a 68-year-old woman who had breast adenocarcinoma recurrence 81 months after HT. The remaining patients are alive, in good condition with no difference in survival from other transplanted patients (P = .757). Patients with end-stage postchemotherapy DCM without evidence of tumour recurrence can safely undergo HT.

Aortic distensibility independently affects exercise tolerance in patients with dilated cardiomyopathy.

Peak exercise oxygen consumption (VO2) is crucial for the prognostic stratification of patients with congestive heart failure, but its hemodynamic determinants are still not completely understood. Aortic wall elasticity modulates left ventricular function and coronary blood flow. Whether an increased aortic pulse-wave velocity (PWV), a known marker of arterial stiffness, may predict peak VO2 in patients with dilated cardiomyopathy (DCM) has to be clarified. A total of 78 patients with clinical diagnosis of DCM (aged 62+/-11 years; female 29%; mean ejection fraction 34+/-9%) were selected. All patients underwent a complete echocardiographic-Doppler evaluation. Aortic PWV was measured by Doppler ultrasonography immediately before the exercise. A bicycle exercise test with expiratory gas exchange monitoring was performed to determine VO2 . Plasma concentration of the amino-terminal propeptide of type III procollagen (PIIINP), a marker of extracellular matrix turnover, was determined. Mean PWV was 5.7+/-2.2 m/s, and VO2 was 16.5+/-4.5 mL x kg(-1) x min(-1). The hemodynamic variables correlated with VO2 were PWV (r=-0.39, P=0.0007) and stroke volume (r=0.38, P=0.002). In a multivariate analysis, PWV (P=0.04) and stroke volume (P=0.05) were independently correlated with VO2 , accounting for 34% of its variance. PIIINP levels correlated with PWV (r=0.35, P=0.002) and a more restrictive diastolic filling pattern (r=0.40, P=0.02). Increased aortic stiffness measured by PWV is an independent predictor of peak VO2 and could partially explain exercise intolerance in patients with DCM.

Determinants and prognostic value of left atrial volume in patients with dilated cardiomyopathy

ObjectivesWe aimed to investigate the determinants of left atrial (LA) volume and its prognostic value in patients with dilated cardiomyopathy (DCM). BackgroundEnlargement of the LA is a marker of mortality in the general population. Patients with DCM are characterized by a wide range of LA sizes, but the clinical role of this observation has been played down. MethodsA complete echocardiographic Doppler examination was performed in 337 patients (age 60 ± 13 years; 84% male) with the diagnosis of DCM. Left atrial maximal volume (LAmax) was measured at left ventricular (LV) end systole (four-chamber view; area–length method). Left ventricular end-diastolic and end-systolic volumes (LVEDV and LVESV) and ejection fraction (EF) were also measured. Mitral regurgitation (MR) was graded using a 5-point scale. Mitral E-wave (E) and A-wave (A) velocities, as well as their ratio (E/A), were measured off-line. ResultsDeterminants of LAmax were: atrial fibrillation (r = 0.34, p < 0.0001), LVEDV (r = 0.46, p < 0.0001), EF (r = 0.40, p < 0.0001), MR (r = 0.39, p < 0.0001), and E/A ratio (r = 0.36, p < 0.0001). During follow-up (41 ± 29 months), 77 patients died and 12 underwent heart transplantation. Univariate Cox analysis showed that LAmax (hazard ratio [HR] 1.01, 95% confidence interval [CI] 1.007–1.013, p < 0.0001), LVESV (HR 1.003, CI 1.001–1.005, p = 0.0003), E/A ratio (HR 1.6, CI 1.3–2.005, p < 0.0001), and MR (HR 1.21, CI 1.03–1.44, p = 0.02) were related to the outcome. On bivariate Cox analysis, LAmax predicted the prognosis independently of each determinant. Patients with a larger LA volume (LAmax/m2 >68.5 ml/m2) had a risk ratio of 3.8 compared with those with a smaller LA volume. ConclusionsIn patients with DCM, LA volume is associated with LV remodeling, diastolic dysfunction, and the degree of MR. The maximal volume of the LA has an independent and incremental prognostic value, compared with all its determinants.

Infantile Dilated X-Linked Cardiomyopathy, G4.5 Mutations, Altered Lipids, and Ultrastructural Malformations of Mitochondria in Heart, Liver, and Skeletal Muscle

Mutations in the Xq28 gene G4.5 lead to dilated cardiomyopathy (DCM). Differential splicing of G4.5 results in a family of proteins called “tafazzins” with homology to acyltransferases. These enzymes assemble fatty acids into membrane lipids. We sequenced G4.5 in two kindreds with X-linked DCM and in two unrelated men, one with idiopathic DCM and the other with DCM of arrhythmogenic right ventricular dysplasia. We examined the ultrastructure of heart, liver, and muscle biopsy specimens in these three DCM types; we used gas chromatography to compare fatty acid composition in heart, liver, and muscle autopsy specimens of two patients of kindred 1 with that of controls. In X-linked DCM, G4.5 had a stop codon (E188X), a nonsense mutation, in kindred 1 and an amino acid substitution (G240R), a missense mutation, in kindred 2. In the two men with isolated DCM, G4.5 was not mutated. Ultrastructural mitochondrial malformations were present in the biopsy tissues of the patients with DCM. Cardiac biopsy specimens of both kindreds with X-linked DCM exhibited greatly enlarged mitochondria with large bundles of stacked, compacted, disarrayed cristae that differed from those of the two types of isolated DCM. Autopsy tissue of patients with X-linked DCM had decreased unsaturated and increased saturated fatty acid concentrations. Seven of 13 published G4.5 missense mutations, including the one presented here, occur in acyltransferase motifs. Impaired acyltransferase function could result in increased fatty acid saturation that would decrease membrane fluidity. Mitochondrial membrane proliferation may be an attempt to compensate for impaired function of acyltransferase. Cardiac ultrastructure separates X-linked DCM with G4.5 mutations from the two types of isolated DCM without G4.5 mutations. Electron microscopy of promptly fixed myocardial biopsy specimens has a role in defining the differential diagnosis of DCM. Mutational analysis of the G4.5 gene also serves this purpose.

Miocardiopatía dilatada familiar en pacientes trasplantados por miocardiopatía dilatada idiopática

To evaluate the prevalence, clinical features, and pattern of inheritance of familial dilated cardiomyopathy (DCM) in heart transplant patients. Patients with idiopathic DCM who had undergone heart transplantation were invited to participate. Patients with alcohol abuse were excluded. A clinical evaluation, 12-lead ECG, echocardiogram, blood tests, and DNA extraction were performed in patients and relatives. Familial DCM was defined as the presence of at least one relative with idiopathic DCM. Possible familial DCM was considered when at least one relative had left ventricular enlargement (LVE) (> 112% predicted LVEDD). One hundred and ninety-nine relatives of 43 families were studied. DCM was familial in 11 probands (25.6%) and possibly familial in 11 (25.6%). Fifteen relatives had DCM (7.5%), 26 (13.1%) LVE, and 5 (2.5%) hypertrophic cardiomyopathy. The pattern of inheritance was autosomal dominant in most families. Five probands (3 with familial DCM) had antecedents of consanguinity and possible recessive inheritance. Six probands (14%, 1 with familial DCM) had relatives with conduction system defects. Creatine kinase was moderately increased in 9 relatives (4.5%), 3 of them with LVE. Fifteen patients had at least moderate alcohol intake. Three of them had familial DCM (relatives without alcohol abuse) and 6 had possible familial DCM. The prevalence of familial DCM is high in patients who undergo heart transplant. Left ventricular enlargement, conduction system abnormalities, and elevated creatine kinase may be early markers of familial disease. Hypertrophic cardiomyopathy is present in some relatives of patients with idiopathic DCM. Familial DCM is present in patients with a previous diagnosis of alcoholic DCM.

Polymerase chain reaction analysis for viruses in paraffin-embedded myocardium from dogs with dilated cardiomyopathy or myocarditis.

To perform polymerase chain reaction (PCR) analysis on paraffin-embedded myocardium from dogs with dilated cardiomyopathy (DCM) and dogs with myocarditis to screen for canine parvovirus, adenovirus types 1 and 2, and herpesvirus. Myocardial specimens from 18 dogs with an antemortem diagnosis of DCM and 9 dogs with a histopathologic diagnosis of myocarditis were evaluated. Paraffin-embedded myocardial specimens were screened for viral genome by PCR analysis. Positive-control specimens were developed from cell cultures as well as paraffin-embedded tissue specimens from dogs with clinical and histopathologic diagnoses of viral infection with canine parvovirus, adenovirus types 1 and 2, and herpesvirus. The histologic characteristics of all myocardial specimens were classified regarding extent, location, and type of inflammation and fibrosis. Canine adenovirus type 1 was amplified from 1 specimen from a dog with DCM. Canine parvovirus, adenovirus type 2, and herpesvirus were not amplified from any myocardial specimens. Histologic analysis of specimens from dogs with DCM revealed variable amounts of fibrosis; myocardial inflammation was observed in 1 affected dog. Histopathologic analysis of specimens from dogs with myocarditis disclosed variable degrees of inflammation and fibrosis. Viral agents canine parvovirus, adenovirus types 1 and 2, and herpesvirus are not commonly associated with DCM or active myocarditis in dogs. Additional studies evaluating for nucleic acid from viruses that less commonly affect dogs or different types of infectious agents may be warranted to gain insight into the cause of DCM and myocarditis in dogs.

Norwood/Batista operation for a newborn with dilated myopathy of the left ventricle

Partial left ventriculectomy for dilated cardiomyopathy was first reported by Batista and associates1 in 1996. The rationale for this procedure is the increase in left ventricular cavity size in the absence of compensatory left ventricular wall thickness that is observed in dilated cardiomyopathy. This combination of factors results in an increase in wall stress per unit muscle mass, as predicted by the LaPlace equation. As wall stress increases, mechanical load eventually becomes nonsustainable and contributes to further dilation of the ventricle. Partial left ventriculectomy has been advocated as a method of restoring the balance between cavity size and wall thickness. Fundamental to this concept is the assumption that the left ventricular muscle is intrinsically normal or has recoverable contractile function. The variable results that have been reported in the adult literature may reflect patient selection according to the reversibility or recoverability of the underlying disease process. Despite the substantial worldwide experience with the Batista procedure in adult patients, there is limited experience with this procedure in children. The current case report describes the treatment of a patient in whom the diagnosis of dilated cardiomyopathy was made in utero. Clinical summary. A female infant was recognized in utero as having a dilated, poorly functioning left ventricle. Labor was induced at 36 weeks’ gestation, and she was delivered by normal, spontaneous, vaginal delivery. She underwent tracheal intubation in the delivery room because of poor tone and cyanosis. After her condition was stabilized, she was transferred to this institution. A postnatal echocardiogram again revealed massive dilation of the left ventricle with severely depressed systolic function. There was mild mitral valve hypoplasia with severe insufficiency. The aortic valve anulus measured 7 mm in diameter, and the valve appeared normal. The aortic arch was unobstructed, but there was retrograde flow via the ductus into the transverse aortic arch. The right ventricular systolic function appeared to be normal. A chest film revealed marked cardiac enlargement with compression of the left lung. From Nemours Cardiac Center, Alfred I. duPont Hospital for Children, Wilmington, Del. Received for publication Jan 18, 2000; accepted for publication Feb 18, 2000. Address for reprints: Richard D. Mainwaring, MD, Nemours Cardiac Center, Alfred I. duPont Hospital for Children, PO Box 269, Wilmington, DE 19899 (E-mail: rmainwar@nemours.org). J Thorac Cardiovasc Surg 2000;120:612-5 Copyright © 2000 by The American Association for Thoracic Surgery 0022-5223/2000 $12.00 + 0 12/54/106657 doi:10.1067/mtc.2000.106657 NORWOOD/BATISTA OPERATION FOR A NEWBORN WITH DILATED MYOPATHY OF THE LEFT VENTRICLE

Comparison of Coronary Flow Reserve According to The Degree of Hypokinesia in Dilated Cardiomyopathy With Regional Asynergy

Background and Objectives:Dilated cardiomyopathy (DCMP) is a primary myocardial disease of unknown cause characterized by left ventricular or biventricular dilatation and impaired myocardial been reported in DCMP with generalized hypok contractility. In 1973, Kreulen et al. classified DCMP into two groups-one with generalized hypokinesia and the other with regional asynergy in addition to generalized hypokinesia. Diminished coronary flow reserve has inesia but its mechanism remains obscure. The aim of this study was to investigate the relationship between the degree of microvascular dysfunction and the difference of regional wall motion abnormality in DCMP with regional asynergy. Methods:The subjects of this study were 11 patients (M:F=6:5, mean age:60±15 yrs) a diagnosis of DCMP with regional asynergy, normal sinus rhythm without left bundle branch block and normal coronary angiogram who underwent Doppler wire from September 1997 to December 1999. Left ventricle was divided into three territories according to the coronary arterial distribution by echocardiography (A:coronary artery territory showing regional asynergy, I:coronary artery territory showing intermediate wall motion, P:coronary artery territory showing relatively preserved wall motion). Coronary flow reserve (CFR) was measured at the mid portion of left anterior descending artery (LAD), left circumflex artery (LCX) and right coronary artery (RCA) with 0.014 inch Doppler guide wire before and during intracoronary injection of 12-18 μg of adenosine. Relative coronary flow reserve (rCFR) was obtained by the ratio of the CFR in coronary artery of the territory showing regional asynergy and relatively preserved wall motion to the CFR in coronary artery of the territory showing intermediate wall motion (CFRA/CFRI, CFRP/CFRI). Results:Regional asynergy was observed in LAD The mean CFR was 2.5±0.6 in LAD, territory in 4 (36%) patients, LCX territory in 4 (36%) patients, RCA territory in 3 (28%) patients (p=ns).2.4±0.5 in LCX, 2.4±0.6 in RCA (p=ns). The mean CFR and rCFR in coronary arteries showing regional asynergy were significantly lower than those in coronary arteries showing relatively preserved wall motion (2.1±0.5 vs 2.7±0.6, p<0.05, 0.84±0.12 vs 1.11±0.11, p<0.001). 논문접수일:1999년 5월 29일 심사완료일:1999년 9월 25일 교신저자:서정기, 400-103 인천광역시 중구 신흥동 3가 7-206 인하대학교 의과대학 순환기내과학교실 전화:(032) 890-2445·전송:(032) 890-2447 E-mail:jksmd@inha.com

Use of echocardiography in the diagnosis of dilated cardiomyopathy in Irish wolfhounds.

The purpose of this study was to compare the echocardiographic features of Irish wolfhounds with clinically inapparent dilated cardiomyopathy (DCM) (n = 33) to dogs with advanced DCM (n = 33) and to normal dogs (n = 262). Significant differences were detected between the three groups. In dogs with DCM, the most sensitive diagnostic measurements were: end-systolic volume index (ESVI), E-point to septal separation (EPSS), fractional shortening (FS), and left ventricular internal dimensions (LVIDd and LVIDs). Left atrial diameter was increased markedly in dogs with DCM and 83.3% of affected Irish wolfhounds had concurrent atrial fibrillation. Compared with early DCM, in advanced DCM there was a significant increase in end-diastolic right ventricular diameter, often combined with extensive pleural effusion, the leading sign of congestive heart failure in Irish wolfhounds.

Cardioprotection for Duchenne’s muscular dystrophy

Purpose To explore the use of neuroendocrine monitoring for more timely diagnosis of dilated cardiomyopathy (DCM) in Duchenne’s muscular dystrophy (DMD) and to determine the effects of angiotensin-converting enzyme inhibitors (ACEI) and β-blockers on neuroendocrine levels, left ventricular diastolic diameter (LVDd), ejection fraction, and mortality rate on DMD. Methods Eighty-five patients with DMD underwent yearly cardiac monitoring including neuroendocrine screening. Eleven patients had symptoms of DCM develop once plasma neuroendocrine levels increased. At this point the patients received ACEI for 9 to 62 months (35.8 ± 18.4 months) and β-blockers for 7 to 60 months (31.6 ± 20.1 months). Results The combination of ACEI and β-blockers relieved symptoms and signs of heart failure in all 11 patients and significantly reduced atrial natriuretic protein (ANP) levels from 197.5 ± 152.1 pg/mL to 25.5 ± 16.2 pg/mL ( P < .002) at 15.5 ± 8.2 months, brain natriuretic protein from 523.8 ± 434.8 pg/mL to 59.3 ± 24.2 pg/mL ( P < .05) at 12.2 ± 3.1 months (data complete for 5 patients), norepinephrine levels from 1114 ± 689 pg/mL to 360 ± 257 pg/mL at 20.5 ± 9.6 months for 11 patients (P = .001), and LVDd from 65.9 ± 9.2 mm to 63.3 ± 6.3 mm (P = .15) at 15.0 ± 7.4 months for 10 patients, including 3 for whom the LVDd increased by 2 to 6 mm. The combination increased left ventricular ejection fraction (LVEF) from 25.1% ± 9.2% to 36.5% ± 5.8% (P < .001) at 17.1 ± 11.0 months for 10 patients. For 9 of the patients ANP levels remained lower throughout the 36.8 ± 20.1 month course of the follow-up. Two patients had sudden severe re-elevations of ANP levels just before death from congestive heart failure after 44 and 23 months of therapy, respectively. Conclusion Neuroendocrine level monitoring can assist in the diagnosis of DCM in patients with DMD. Combination therapy with ACEI and β-blockers can significantly decrease neuroendocrine activation and LVDd and reverse symptoms and signs of congestive heart failure for patients with DMD. (Am Heart J 1999;137:895-902.)

Três anos de ventriculectomia parcial esquerda: resultados globais e tardios em 41 pacientes

Objetivos: Avaliar a indicação, sobrevida, qualidade de vida e arritmias após Ventriculectomia Parcial Esquerda (VPE) e relatar seu uso como ponte para transplante. Casuística e Métodos: Quarenta e um pacientes (pt) operados no período de dezembro de 1994 a dezembro de 1997,com idades de 32 a 70 anos, com miocardiopatia dilatada, classe funcional IV-92,6% e III-7,3% com contra-indicação para transplante e má qualidade de vida. Ecocardiogramas e estudos eletrofisiológicos (EEF) pré e pós-operatórios foram realizados. Clinicamente, agrupados pela NYHA e pelo Protocolo de Qualidade de Vida (QV). Resultados: Em 15 pacientes examinados aos 30 e 90 dias de pós-operatório por ecocardiograma obtivemos os dados da tabela abaixo: Sobrevida de 66%, 53%, 40% e 36,6% aos 3, 6, 12 e 24 meses, respectivamente. Aos 3 anos a mortalidade imediata e tardia é de 21,5 % e 42,1%,com total de 63,6 %. Menor mortalidade relacionada a arritmia quando não se conseguiu induzí-la no pós-operatório. A qualidade de vida melhorou (72,7 % em NYHA I e II e 81,8% em QV I e II) e dois foram transplantados posteriormente. Conclusões: Melhoraram a dinâmica cardíaca, a QV e a classe funcional (NYHA).O EEF identificou pacientes com maior risco de arritmias pós-operatórias. A sobrevida de 36% em 3 anos é encorajadora, levando-se em conta a gravidade dos casos. A seleção de pacientes não tem ainda critérios inequívocos.