Stochastic analysis of heart rate variability and its relation to echocardiography parameters in hypertrophic cardiomyopathy patients.

The heart rate variability of 10 healthy males (age 26 - 4/+ 3 y) and 49 patients with hypertrophic cardiomyopathy (HCM) (25 males, 24 females, age 29.5 - 11.5/+ 10.5 y) was studied. We applied Kramers-Moyal expansion to extract the drift and diffusion terms of the Langevin equation for the RR interval time series. These terms may be used for a stochastic reconstruction of the time series and for description of the properties of heart rate variability. New parameters characterizing the diffusion term are proposed: the coefficients of the linear fit to the left (LCF) and right (RCF) branch of the dependence of the diffusion term on the rescaled heart rate. Relations of the new parameters to classical echocardiography parameters were studied. Using the relation between the difference LCF-RCF and the left ventricular systolic diameter, the HCM patients studied were divided into three groups. In addition, comparison of the properties of the heart rate variability in the HCM group with that obtained for the healthy young men showed that the parameter LCF-RCF may be treated as a measure of the effect of HCM on heart rate variability and may have diagnostic value.

Heart rate variability analysis based on time-frequency representation and entropies in hypertrophic cardiomyopathy patients.

In hypertrophic cardiomyopathy (HCM) patients there is an increased risk of premature death, which can occur with little or no warning. Furthermore, classification for sudden cardiac death on patients with HCM is very difficult. The aim of our study was to improve the prognostic value of heart rate variability (HRV) in HCM patients, giving insight into changes of the autonomic nervous system. In this way, the suitability of linear and nonlinear measures was studied to assess the HRV. These measures were based on time-frequency representation (TFR) and on Shannon and Rényi entropies, and compared with traditional HRV measures. Holter recordings of 64 patients with HCM and 55 healthy subjects were analyzed. The HCM patients consisted of two groups: 13 high risk patients, after aborted sudden cardiac death (SCD); 51 low risk patients, without SCD. Five-hour RR signals, corresponding to the sleep period of the subjects, were considered for the analysis as a comparable standard situation. These RR signals were filtered in the three frequency bands: very low frequency band (VLF, 0-0.04 Hz), low frequency band (LF, 0.04-0.15 Hz) and high frequency band (HF, 0.15-0.45 Hz). TFR variables based on instantaneous frequency and energy functions were able to classify HCM patients and healthy subjects (control group). Results revealed that measures obtained from TFR analysis of the HRV better classified the groups of subjects than traditional HRV parameters. However, results showed that nonlinear measures improved group classification. It was observed that entropies calculated in the HF band showed the highest statistically significant levels comparing the HCM group and the control group, p-value < 0.0005. The values of entropy measures calculated in the HCM group presented lower values, indicating a decreasing of complexity, than those calculated from the control group. Moreover, similar behavior was observed comparing high and low risk of premature death, the values of the entropy being lower in high risk patients, p-value < 0.05, indicating an increase of predictability. Furthermore, measures from information entropy, but not from TFR, seem to be useful for enhanced risk stratification in HCM patients with an increased risk of sudden cardiac death.

Left ventricular outflow tract gradient decrease with non-surgical myocardial reduction improves exercise capacity in patients with hypertrophic obstructive cardiomyopathy.

OBJECTIVES: This study was undertaken to evaluate mid-term clinical results of non-surgical myocardial reduction in patients with hypertrophic obstructive cardiomyopathy. METHODS: Twenty-five patients with left ventricular outflow tract obstruction (mean gradient of 84. 54+/-31.38 mmHg) and symptoms of dyspnoea, angina and/or syncope were treated with non-surgical myocardial reduction. The patients were followed-up for a mean period of 10.44+/-1.8 months. In all patients clinical examination with echocardiography was repeated after every 3 months of follow-up, and a symptom-limited treadmill test was repeated at the 6 month follow-up. Eighteen patients underwent simultaneous respiratory gas analysis. RESULTS: Clinical follow-up examinations were achieved in all 25 patients. Persistent left ventricular outflow tract gradient reduction was seen in 23 patients. Seventeen patients had a reduction of left ventricular outflow tract gradient >50% of baseline value. Twenty patients showed a clinical improvement from 2.8+/-0.5 up to 1.2+/-0.5 NYHA class (P<0.001). The clinical improvement was matched by an improvement in objective measures of exercise capacity in patients with significant left ventricular outflow tract gradient reduction. Exercise time increased from 571.9+/-192.2 to 703.5+/-175.4 s, P<0. 001, and peak VO(2)increased from 14.6+/-5.2 to 20.5+/-8.6 ml. kg(-1)min(-1), P<0.05. CONCLUSION: Significant left ventricular outflow tract gradient reduction with exercise capacity improvement was achieved in the majority of patients treated with non-surgical myocardial reduction. We recommend this method as an alternative to surgery for symptomatic patients with hypertrophic obstructive cardiomyopathy.

Time-frequency analysis of the RT and RR variability to stratify hypertrophic cardiomyopathy patients.

The RT interval is a measure of the ventricular repolarization and is partially influenced by the sympathovagal balance. The analysis of the variation of the duration of the RT and RR intervals might bring new information about the arrhythmogenic vulnerability and autonomic imbalance. The RR signal and its spectral density (SD) are characterized by two different patterns during the sleep period. On the basis of this information, RT and RR sequences have been automatically classified into two patterns, R and N. In this work, we propose a methodology to define new variables that are able to distinguish patients with hypertrophic cardiomyopathy (HCM) who later developed sudden cardiac death (SCD) from HCM patients without such episode during the follow-up. These variables are based on the instantaneous frequency calculation using time-frequency representation of the RT and RR signals previously classified into R and N patterns. In this study, three spectral bands have been considered: low-frequency band (LF, 0-0.07 Hz), mid-frequency band (MF, 0.07-0.15 Hz), and high-frequency band (HF, 0.15-0.45 Hz). Then a suitable combination of mean energy and mean frequency of the RT and RR signals in the MF and HF bands has allowed HCM patients with SCD to be discriminated from HCM patients without SCD (P < 0.001).

Evaluation of the impact of transmyocardial laser (CO2) revascularisation on myocardial perfusion--6-months observations.

BACKGROUND: Transmyocardial laser revascularisation (TMLR) is a relatively new surgical approach to symptomatic coronary artery disease patients otherwise inoperable by classical revascularisation methods. Perfusion improvement after TMLR is considered as one possible mechanism causing alleviation of symptoms in a significant percentage of operated patiens. The goal of this work was to assess the history f myocardial perfusion during the first 6 month after sole TMLR operation. METHODS: TMLR was performed by using high-power (800 W) CO(2) laser. Tc-99m-Sestamibi single photon emission computed tomography (SPECT), both in rest and stress, was performed 4 times: before TMLR [SPECT-0], very early (mean: 3 weeks) after TMLR [SPECT-I], 3 months after TMLR [SPECT-II] and 6 months after TMLR [SPECT-III] in every patient. The group consisted of 25 patients, including 21 patients with previous myocardial infarctions. The patients subjected to the operation were those suffering from angina in spite of pharmacological therapy, with diffuse changes in the peripheral parts of coronary arteries, with left ventricle (LV) ejection fraction not lower than 0.30 and with at least one transient or small persistent defect in preoperative SPECT. Perfusion was assessed in 13 of 17 segments of the LV (after exclusion of 4 septal segments). Only a history of transient or small persistent perfusion defects ('viable' segments) detected in SPECT-0 is discussed. RESULTS: In comparison to SPECT-0: in SPECT-I perfusion did not change in 52% of segments, improved--in 31%, and deteriorated--in 17%: in SPECT-II perfusion did not change in 48% of segments, improved--in 34%, and deteriorated--in 18%; in SPECT-III perfusion did not change in 52%, improved--in 25%, and deteriorated--in 22% of segments. No significant difference in the number of segments with perfusion preservation, improvement or deterioration in comparison to SPECT-0 was found in SPECT-I,-II or III. In SPECT-II in comparison to SPECT-I, no changes in perfusion were found in 66% of segments, perfusion improved in 20% and deteriorated in 14%. In SPECT-III in comparison to SPECT-II, no changes in perfusion were found in 79% of segments, perfusion improved in 5% and deteriorated in 15%. CONCLUSIONS: Our evaluation of the history of segments with preoperatively transient or small persistent ('viable') defects indicates that during the first 6 months after TMLR: 1) perfusion is better than before the operation in about one third of the segments, and 20 in some of these segments there are dynamic perfusion changes (improvement or deterioration) from one to the next postoperative moment of observation.

Significant improvement of quality of life following atrioventricular synchronous pacing in patients with hypertrophic obstructive cardiomyopathy. Data from 1 year of follow-up. PIC study group. Pacing In Cardiomyopathy.

AIMS: Atrioventricular synchronous pacing exerts beneficial effects, including reduction of left ventricular outflow tract gradients, in patients with hypertrophic obstructive cardiomyopathy. The Pacing in Cardiomyopathy study was initiated to explore the effects of pacing in a double-blind randomized crossover fashion. The aims were to ascertain the beneficial effects of pacing in a controlled study and to rule out a placebo effect by pacing. This paper deals with the outcome of pacing on quality of life during 1 year of follow-up. METHODS: Quality of life was evaluated with the Karolinska questionnaire, validated for patients paced for bradyarrhythmias and ischaemic heart disease. Drug-refractory patients with hypertrophic obstructive cardiomyopathy were recruited for the study and after a temporary pacing procedure implanted with permanent pacemakers. Patients were randomized to two study arms defining the sequence of pacemaker programming. In one arm the pacemaker was inactive, in the other active. After 3 months the pacemaker was reprogrammed to the alternate mode and a further 3 months followed. After this period subsequent pacemaker programming corresponded to the mode preferred by the patient. A last assessment was made 1 year after baseline examinations. RESULTS: Eighty patients completed the first crossover period and 75 completed the full 1 year of follow-up. Active pacing induced significant quality of life improvements, in the order of 9-44%, regardless of programming sequence. Discontinuation of pacing after a first active period resulted in the return of symptoms. Fourteen patients requested early reprogramming after having been programmed to inactive pacing after a first period of active pacing. Seventy-six patients preferred active pacing after the crossover period. A further 6 months of pacing induced progressive improvement in symptoms already favourably influenced. CONCLUSION: Atrioventricular synchronous pacing has a profound beneficial effect on most domains of quality of life in patients with hypertrophic obstructive cardiomyopathy refractory to drug treatment.

Clinical progress after randomized on/off pacemaker treatment for hypertrophic obstructive cardiomyopathy. Pacing in Cardiomyopathy (PIC) Study Group.

BACKGROUND: The therapeutic options for hypertrophic obstructive cardiomyopathy (HOCM) classically include medical treatment with beta-blockers and calcium antagonists or myectomy-myotomy as a surgical possibility for refractory cases. The observation that pacemaker activation of the heart in HOCM reduces the subaortic gradient is well known but less well investigated. METHODS: Eighty-three patients (33 female and 50 male) mean age 53 (18-82) years, with symptoms refractory to drug treatment and a resting gradient above 30 mmHg, who responded favourably to temporary pacing, were included in this prospective study and had a pacemaker (DDD) implanted. After an initial double-blind crossover phase of 6 months, patients were reinvestigated at 12 months and followed for a mean of 36 months. RESULTS: As observed during a screening investigation, the obstruction was significantly reduced from 72 +/- 35 mmHg to 29 +/- 24 mmHg (P < 0.01) when the pacemaker was on, while no major effect was seen during the sham phase. The effect was persistent at 1 year with a remaining resting gradient of 28 +/- 24 mmHg. In parallel, we documented an improvement in functional capacity, according to the NYHA classification and by quality of life analysis, and a significant improvement in dyspnoea and angina. Exercise on treadmill improved only in patients with reduced initial tolerance (< 8 min). During the mean follow-up of 36 months, 65 patients remained on pacing alone, with eight patients having additional AV-node ablation and five patients finally having surgery. CONCLUSION: This controlled multicentre study shows that pacemaker treatment is an option for HOCM patients; it is inoffensive and does not exclude alternative methods, but satisfies 79% of patients beyond 3 years.

Pacing in hypertrophic obstructive cardiomyopathy. A randomized crossover study. PIC Study Group.

BACKGROUND: Uncontrolled studies have shown that short atrioventricular delay dual chamber pacing reduces outflow tract obstruction in hypertrophic obstructive cardiomyopathy. Although the exact mechanism of this beneficial effect is unclear, this seems a promising potential new treatment for hypertrophic obstructive cardiomyopathy. METHOD: In order to evaluate the impact of pacing therapy, were performed a randomized multicentre double-blind cross-over (pacemaker activated vs non activated) study to investigate modification of echocardiography, exercise tolerance, angina, dyspnoea and quality of life in 83 patients with a mean age of 53 (range 22-87) years with symptoms refractory or intolerant to classical drug treatment. RESULTS: After 12 weeks of activated or inactivated pacing, independent of which phase was first, the pressure gradient fell from 59 +/- 36 mmHg to 30 +/- 25 mmHg (P < 0.001) with active pacing. Exercise tolerance improved by 21% in those patients who at baseline tolerated less than 10 min of Bruce protocol; symptoms of dyspnoea and angina also improved significantly from NYHA class 2.4 to 1.4 and 1.0 to 0.4, respectively (P < 0.007). Quality of life assessment with a validated questionnaire objectivated the subjective improvement. CONCLUSION: Pacemaker therapy is of clinical and haemodynamic benefit for patients with hypertrophic obstructive cardiomyopathy, left ventricular outflow gradient at rest over 30 mmHg who are symptomatic despite drug treatment.

The influence of the angiotensin I converting enzyme genotype in familial hypertrophic cardiomyopathy varies with the disease gene mutation.

Familial hypertrophic cardiomyopathy is an autosomal dominant genetically heterogeneous disease characterized by a partial penetrance and variable expressivity. Previous studies showed that the extent of hypertrophy is influenced by the angiotensin I converting enzyme insertion/deletion (I/D) polymorphism. Recently, molecular genetic analysis revealed the existence of healthy carriers and that as many as a quarter of genetically affected individuals do not express the disease. This data prompted us to re-investigate the role of the angiotensin I converting enzyme polymorphism on hypertrophy by assessing both clinically affected individuals and healthy carriers. For this, several families with mutations in the cardiac myosin binding protein C or the beta-myosin heavy chain genes were analysed. The mean maximal intraventricular septum thickness was compared as a function of angiotensin I converting enzyme genotypes in all genetically affected individuals (n = 114), and in subsets of subjects carrying either a splice acceptor site mutation in the cardiac myosin binding protein C gene (n = 33), or various missense mutations in the cardiac beta-myosin heavy chain gene (n = 81) or finally, mutation in the Arg403 codon of the beta-myosin heavy chain gene (n = 54). Significant association between the D allele and hypertrophy was observed only in the case of Arg403 codon mutations (mean septum thickness for subjects with the DD genotype: 19.3 +/- 2.7 mm: with the ID genotype: 13.4 +/- 1.3 mm and with the II genotype: 11.0 +/- 0.9 mm; P < 0.02). These results were confirmed by the chi 2 test showing an over-representation of DD genotype in patients carrying an Arg403 codon mutation associated with septal hypertrophy (P < 0.05). Our data confirms that the angiotensin I converting enzyme genotypes can influence the phenotypic expression of hypertrophy and shows that this influence depends on the mutation, raising the concept of multiple genetic modifiers in familial hypertrophic cardiomyopathy.

DDD pacing in hypertrophic cardiomyopathy: a multicentre clinical experience.

BACKGROUND: DDD pacing has been advocated as an effective treatment for drug refractory obstructive hypertrophic cardiomyopathy. This study reports the outcome of pacing in 56 patients with refractory symptoms referred to four tertiary centres. METHODS: Core data on symptoms, drug burden, and left ventricular outflow tract gradient were recorded. Patients underwent a temporary pacing study with optimisation of the atrioventricular (AV) delay for greatest gradient reduction without haemodynamic compromise. Patients were assessed after implantation in terms of changes in symptoms, drug load, and outflow tract gradient. RESULTS: 56 patients underwent pacing assessment. The mean (SD) left ventricular outflow tract gradient before pacing was 78 (31) mm Hg. At temporary study the mean (SD) left ventricular outflow tract gradient was 38 (24) mm Hg with a median (range) optimised sensed AV delay of 65 (25-125) ms. Fifty three patients were implanted and followed up for a mean (SD) of 11 (11) months. The median (range) programmed sensed AV delay was 60 (31-200) ms. Left ventricular outflow tract gradient at follow up was 36 (25) mm Hg. Forty four patients had improved functional class. Although a correlation (r = 0.69) was shown between acute and chronic left ventricular outflow tract gradient reduction, there was no correlation between magnitude of gradient reduction and functional improvement, and no appreciable change in pharmacological burden. CONCLUSION: This series confirms symptomatic improvement after DDD pacing in hypertrophic cardiomyopathy. There remains, however, a discrepancy between perceived symptomatic benefit and modest objective improvement. Furthermore, the optimal outcome has been achieved only with continued pharmacological treatment. Current methods of temporary evaluation do not predict functional outcome which seems to be independent of the magnitude of gradient reduction.

The standard electrocardiogram as a screening test for hypertrophic cardiomyopathy.

Phenotypic heterogeneity in hypertrophic cardiomyopathy (HC) makes definitive diagnosis difficult, particularly during family screening. We studied the electrocardiogram (ECG) as a potential initial screening test in patients with HC. Using accepted diagnostic criteria, we examined the ECGs and echocardiograms of 159 patients with a confirmed clinical or genetic diagnosis of HC. An abnormal ECG was found in 154 patients (97%) while only 146 (92%) showed an abnormal echocardiogram. Of the former, 9 patients (6%) had normal echocardiograms and had been diagnosed on the basis of identification of a mutation in the beta myosin heavy chain gene (n = 8) or obligate carrier status (n = 1). Only 1 of these 9 patients was under age 20, the time at which hypertrophy is normally expressed on the echocardiogram. The remaining 5 patients (3%) without ECG abnormality consisted of 1 patient with an echocardiogram clearly diagnostic of HC and 4 clinically normal patients (aged 13, 24, 29, and 33 years) with normal echocardiograms who had been diagnosed by mutation identification (n = 3) or obligate carrier status (n = 1). Thus only these latter 4 patients (3%) would not have been diagnosed as having HC based on an abnormal ECG and/or abnormal echocardiogram. Screening relatives for HC by ECG criteria alone detects all those whom an echocardiogram will diagnose. While echocardiography aids in the specificity of HC diagnosis, the ECG, within the context of a family with a proven case of HC, is a more sensitive marker of the disease. It is therefore both a cost-effective and useful tool for screening those to proceed to echocardiography.

The electrocardiogram is a more sensitive indicator than echocardiography of hypertrophic cardiomyopathy in families with a mutation in the MYH7 gene.

BACKGROUND: Mutations in the cardiac beta myosin heavy chain gene causing hypertrophic cardiomyopathy have been identified, and to assist both diagnosis and prediction of outcome attempts have been made to correlate phenotype and genotype. Two new mutations in codon 403 of the gene in three unrelated families are described and attention drawn to variable or even absent phenotypic expression in different family members. METHODS AND RESULTS: The polymerase chain reaction and heteroduplex analysis on Mutation Detection Enhancement gels were used to search for mutations in the globular head of the beta myosin heavy chain gene in families with hypertrophic cardiomyopathy. Two mutations were found in exon 13 (codon 403) of the gene. In two unrelated Polish families the mutation resulted in the conversion of arginine to tryptophan (CGG: >TGG). A second mutation, found in a British family, converted the same arginine to leucine (CGG: >CTG). These mutations were detected in family members who had electrocardiographic and echocardiographic features typical of hypertrophic cardiomyopathy; however, they were also detected in 7 other adult relatives with an abnormal electrocardiogram but a normal echocardiogram. Two unrelated adult relatives had completely normal clinical findings but carried the gene mutation. CONCLUSIONS: Identification of a specific mutation gives no guide to the clinical phenotype. There is considerable variability in the phenotypic expression of hypertrophic cardiomyopathy. Mutations were detected in adults previously regarded as normal or in whom the diagnosis was questionable. The fact that the clinical significance of the mutation in these people is still unknown emphasises the dilemma facing screening programmes. Isolated, unexplained electrocardiographic abnormalities in first degree relatives in a family with a definitive diagnosis of hypertrophic cardiomyopathy should be regarded as evidence of a carrier state.

[Familial dilated cardiomyopathy with autosomal mode of inheritance. Case report]. / Kardiomiopatia rozstrzeniowa z dominujacym autosomalnym sposobem dziedziczenia. Opis rodziny.

Familial occurrence of dilated cardiomyopathy is estimated by 2-20%. We present a family with dilated cardiomyopathy inherited in an autosomal dominant way. We examined 9 members of the family, most of them are asymptomatic.

[Constriction of the left main coronary artery. Early and long term treatment outcome]. / Zwezenie pnia lewej tetnicy wiencowej. Wczesne i odlegle wyniki leczenia.

Clinical presentation and course were studies in 45 consecutive patients (p)--39 males, 6 females with angiographically proven left main coronary artery stenosis (LMCA) > 50%. Mean age was 54.7 years. Three (6%) had no history of chest pain, 2 p (4%) had atypical chest pain, and the remaining (90%) had typical angina pectoris. 19 p (42%) had unstable angina, 20 p (44%) had suffered a myocardial infarction in the past. Outside an episode of chest pain most of the patients had an abnormal ECG with ST-T segment depression 2 mm or more in leads V3-6 and ST-T elevation in leads V1 and aVR. No significant differences were found when the abnormalities of the ST-T segment were compared to severity of LMCA obstruction. A symptom limited exercise test was performed in 17 (37%) p. It was abnormal in 13 p (29%). Thirty eight patients (85%) underwent bypass surgery and the mean number of bypass graft was 3.3. Seven patients were treated medically. In the surgical group four p (10.5%) died perioperatively. All of them had subtotal occlusion of LMCA, without significant lesions in the remaining coronary arteries, the ejection fraction (EF) was above 66%. Among thirty four living patients thirty have been asymptomatic. In the medically treated group 3 p (42%) died and only two of four survivors were asymptomatic at a mean follow-up 35.7 months. Left ventricle of all died patients were severely damaged (EF mean 28%), right coronary artery (RCA) was totally occluded and all had rythm disturbances. We conclude, that patients with significant LMCA stenosis had a good prognosis when treated surgically.(ABSTRACT TRUNCATED AT 250 WORDS)

[Increased tension on the sympathetic nervous system in arrhythmogenic right ventricular dysplasia. Participation in etiopathogenesis?]. / Wzmozone napiecie ukladu sympatycznego w arytmogennej dysplazji prawej komory. Udzial w etiopatogenezie?

UNLABELLED: To evaluate the suspected role of sympathetic nerve tone in pathogenesis of arrhythmogenic right ventricle dysplasia (ARVD), noradrenaline (NOR) and adrenaline (AD) plasma levels were determined by radioenzymatic method in 10 pts with ARVD (mean age 36 y, VT-8 pts, syncope-5) at rest and during peak exercise. 9 healthy men (mean age 28 y) served as control. RESULTS: [table: see text] CONCLUSION: Significant increase of norepinephrine serum level during peak exercise suggests essential role of sympathetic nerve tone in pathogenesis of ARVD.

[Surgical treatment of ventricular tachycardia in patients with post-infarction aneurysms]. / Leczenie operacyjne czestoskurczów komorowych u chorych z tetniakami pozawalowymi serca.

4 patients (P) with recurrent, sustained ventricular tachycardia (VT) resistant to medical treatment, underwent surgery for cure of this arrhythmia. Each P had episodes of VT lasting 30 or more seconds, 3 of them had episodes of ventricular fibrillation. In all cases rhythm disturbances were secondary to post myocardial infarction aneurysm. Coronary angiography showed in all P total occlusion of LAD, in 2 cases significant lesion in RCA were found. 1 P had lung cancer. All P underwent aneurysmectomy and an excision of the altered endocardium by Harken's method. The endocardial excision was performed without endocardial mapping. 2 P had concomitant CABG to RCA. In the P with lung cancer lobectomy was performed. There were 2 ++non-arrhythmic death. The P with lung cancer died because of sepsis due to lung abscess. One P died because of heart failure (preoperative EF 10%), 6 months after the surgery. The 2 survivors remained free of VT during a follow-up period 8 months. In conclusion, endocardial excision by Harken's method is efficient in treating recurrent sustained VT, resistant to medical treatment, in patients with post myocardial infarction aneurysm. The surgical procedure can be performed without intraoperative endocardial mapping.

[Disturbances of myocardial perfusion by exertion scintigraphy in patients with hypertrophic cardiomyopathy and their relationship with sudden death risk factors]. / Zaburzeniom perfuzji miesnia sercowego w kardiomiopatii przerostowej towarzysza czynniki ryzyka naglej smierci.

Heart muscle perfusion was studied by exertion scintigraphy Tal-201 in 24 patients, 16M and 8F, aged 16-45 years, means--28 +/- 7.4 years with hypertrophic cardiomyopathy. The relationship between perfusion disturbances and sudden death risk factors occurring in this group of patients was evaluated. Disturbances of heart muscle perfusion were found in 20 pts (83%); 2 pts had permanent perfusion defects, in 18 pts these defects were completely or partially reversible at rest. Only 4 pts (17%) had normal heart muscle perfusion. In patients with perfusion disturbances there was found a significantly more frequent occurrence of the following sudden death risk factors: 1. syncope (p less than .01) 2. ventricular arrhythmia of IV b class according to Lown (p less than .01) 3. advanced hypertrophy of intraventricular septum (p less than .01) 4. sudden death in patients families (p less than .05) The evaluation of the heart muscle perfusion confirmed the occurrence of myocardial ischemia in most of the examined patients. Normal coronaro-angiography in all the patients over 35 years as well as the young age of the other patients exclude atherosclerosis as the cause of myocardiac ischemia in the group under study. This is a confirmation of nonatherosclerotic etiology of myocardiac ischemia in hypertrophic cardiomyopathy patients. The correlation between perfusion disturbances and sudden death risk factors points to the role of ischemia in the natural course of disease and the value of exertion scintigraphy TI-201 in prognosing patients with hypertrophic cardiomyopathy.

[Indications for emergency cardiovascular surgery at the Institute of Cardiology]. / Operacje kardiochirurgiczne ze wskazan zyciowych w materiale Instytutu Kardiologii.

Between 1981 and 1989 73 patients (46 males, 27 females aged 15-69; mean 42) underwent emergency cardiac operation in the I Clinic of Cardiosurgery. There were 39 (53%) patients with valvular disease, 18 (25%) with prosthetic valve dysfunction and endocarditis, 9 (12%) with acute aortic dissection (2 of them had Marfan's syndrome), 5 (7%) with atrial myxoma and 2 (3%) with malignant pericardial mesothelioma. All of them were treated in C.C.U. before surgery. 27 urgent operations were performed within the first 24 hours after the admission to C.C.U. 30 of 73 patients died (41%). The IV NYHA functional class was stated as the most significant predictive factor for in-hospital mortality. The greatest mortality was observed in the group of patients with valvular disease (53%). In hospital survival rate reached 50% after reoperations as a procedure of choice in prosthetic valve dysfunction. Good early results were observed in patients with aortic dissection (survival rate--66%). The presence of Marfan's syndrome was associated with a poor surgical prognosis. There were no deaths after atrial myxoma removal, but all patients with malignant mesothelioma died. The most common cause of all deaths was congestive heart failure.