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We report a 51-year-old asymptomatic male, with type II diabetes, referred to our outpatient clinic due to ST and T alterations on the precordial leads on the electrocardiogram. The echocardiogram showed apical akinesia and left ventricular hypertrophy. There were no angiographic lesions in the coronary angiography. In the left ventriculography, a hyperdynamic left ventricle with suspected left ventricular hypertrophy and an apical aneurysm were found. The cardiac magnetic resonance confirmed those findings, without late gadolinium enhancement. According to the European Cardiology Society Risk Score, the patient had a low sudden death risk. However, this score does not consider the presence of an aneurysm as risk factor for sudden death, but it is considered in the 2017 ACC/AHA Heart Rhythm Society Guidelines, as a major risk factor. Therefore a defibrillator was implanted, and he was discharged on permanent oral anticoagulation.

Introduction and Objectives: Myocardial interstitial fibrosis, a hallmark of hypertrophic cardiomyopathy (HCM), has been proposed as an arrhythmic substrate. Fibrosis is associated with increased extracellular volume (ECV), which can be quantified by computed tomography (CT). We aimed to analyze the association between CT-determined ECV and malignant ventricular arrhythmias., Methods: A retrospective case-control observational study was conducted in HCM patients with implantable cardioverter-defibrillator, undergoing a CT-protocol with continuous iodine contrast infusion to determine equilibrium ECV. Left ventricular septal and lateral CT-determined ECV was compared between prespecified cases (malignant arrhythmia any time before CT scan) and controls (no prior malignant arrhythmias) and among ECV tertiles., Results: A total of 78 implantable cardioverter-defibrillator HCM patients were included; 24 were women, with a mean age of 52.1 ± 15.6 years. Mean ECV ± standard deviation in the septal left ventricular wall and was 29.8% ± 6.3% in cases (n = 24) vs 31.9% ± 8.5% in controls (n = 54); P = .282. Mean ECV in the lateral wall was 24.5% ± 6.8% in cases vs 28.2% ± 7.4% in controls; P = .043. On comparison of the entire population according to septal ECV tertiles, no significant differences were found in the number of patients receiving appropriate shocks. Conversely, we found a trend (P = .056) for a higher number of patients receiving appropriate shocks in the lateral ECV lowest tertile., Conclusions: Extracellular volume was not increased in implantable cardioverter-defibrillator HCM patients with malignant ventricular arrhythmias vs those without arrhythmias. Our findings do not support the use of ECV (a surrogate of diffuse fibrosis) as a predictor of arrhythmias in high-risk HCM patients., (Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction and Objectives: Nonischemic sudden cardiac death (SCD) is predominantly caused by cardiomyopathies and channelopathies. There are many diagnostic tests, including some complex techniques. Our aim was to analyze the diagnostic yield of a systematic diagnostic protocol in a specialized unit., Methods: The study included 56 families with at least 1 index case of SCD (resuscitated or not). Survivors were studied with electrocardiogram, advanced cardiac imaging, exercise testing, familial study, genetic testing and, in some cases, pharmacological testing. Families with deceased probands were studied using the postmortem findings, familial evaluation, and molecular autopsy with next-generation sequencing (NGS)., Results: A positive diagnosis was obtained in 80.4% of the cases, with no differences between survivors and nonsurvivors (P=.53). Cardiac channelopathies were more prevalent among survivors than nonsurvivors (66.6% vs 40%, P=.03). Among the 30 deceased probands, the definitive diagnosis was given by autopsy in 7. A diagnosis of cardiomyopathy tended to be associated with a higher event rate in the family. Genetic testing with NGS was performed in 42 index cases, with a positive result in 28 (66.6%), with no differences between survivors and nonsurvivors (P=.21)., Conclusions: There is a strong likelihood of reaching a diagnosis in SCD after a rigorous protocol, with a more prevalent diagnosis of channelopathy among survivors and a worse familial prognosis in cardiomyopathies. Genetic testing with NGS is useful and its value is increasing with respect to the Sanger method., (Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

Haemodynamic changes that occur during pregnancy are maximal between 28 and 34 weeks. In the pregnant woman with several associated diseases, such as hypertensive myocardiopathy and pre-gestational diabetes, these changes can lead to a difficult control of pulmonary hypertension and acute pulmonary oedema. We report the case of a pregnant woman with long term type 1 diabetes mellitus who suffered pre-eclampsia in a previous pregnancy, and since then developed hypertensive cardiomyopathy. She was admitted at 30 week gestation for metabolic and blood pressure control, and developed congestive cardiac failure after the administration of betamethasone for foetal lung maturity. A transthoracic echocardiogram showed a non-dilated hypertrophic left ventricle with good systolic function, restrictive diastolic dysfunction and moderate pulmonary arterial hypertension. When her general condition improved, we performed a caesarean section under regional anaesthesia to prevent the complications of pulmonary and systemic hypertension. We present the anaesthetic management and resolution of complications after oxytocin administration., (Copyright © 2011 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España, S.L. All rights reserved.)

Quality of life is seriously compromised in both severe aortic stenosis and in hypertrophic obstructive cardiomyopathy. At advanced stages of disease, symptoms become incapacitating. Surgical correction is extremely effective, reducing symptoms and improving functional capacity. Percutaneous surgical techniques facilitate the treatment of patients in high-risk categories for whom conventional surgery is not an option. We report the case of a woman with severe symptomatic aortic stenosis and hypertrophic obstructive cardiomyopathy with associated mitral regurgitation. An aortic valve was implanted percutaneously. Later septal ablation was also performed percutaneously. The postoperative course was slow and the hospital stay was long. Percutaneous procedures provide a useful alternative way to treat severe symptomatic aortic stenosis and hypertrophic obstructive cardiomyopathy with mitral regurgitation, particularly when patients are at high risk for conventional surgery. These procedures are not without risk, however.

The aims of this study were to determine the prevalence of severe cardiac conduction disturbances in a cohort of 451 patients with hypertrophic cardiomyopathy and to describe the characteristics of, and outcomes in, those who required a permanent pacemaker. A pacemaker was implanted in 48 patients (11%): 20 had sinus node dysfunction and 28 had an atrioventricular conduction disturbance. Primary bradyarrhythmia (which was not related to iatrogenic atrioventricular block or therapeutic ablation of the atrioventricular node) was the reason for permanent pacemaker implantation in 36 patients (8%). In 18% of cases, at least one other family member had a permanent pacemaker. In this patient series, a high prevalence of severe cardiac conduction disturbance leading to permanent pacemaker implantation was observed. Severe cardiac conduction disturbance in hypertrophic cardiomyopathy may also have a familial component.

Background: In hypertrophic cardiomyopathy (HCM), interstitial myocardial fibrosis is an important histological modification that has been associated with sudden death and evolution toward myocardial dilation., Objective: To prospectively evaluate the prognostic value of the collagen volume fraction in HCM., Methods: An endomyocardial biopsy of the right ventricle was successfully performed in 21 symptomatic patients with HCM. The myocardial collagen volume fraction (CVF) was determined by histology. The CVF was also determined in fragments of nine normal hearts from subjects deceased from non-cardiac causes. The patients were divided into above-median CVF and below-median CVF groups, and their clinical and echocardiographic characteristics and survival curves were compared., Results: Among the patients, the CVF ranged from 1.86% to 29.9%, median 6.19%; in normal hearts, from 0.13% to 1.46%, median 0.61% (p <0.0001 between HCM and control). There were no significant correlations between CVF and baseline echocardiographic measures. Patients with CVF < or =6.19% and CVF> 6.19% were compared and no baseline differences were observed. However, after an average follow-up period of 110 months, four deaths occurred (two sudden, two due to heart failure) in the group with increased CVF, whereas the patients of the group with lower CVF were all alive at the end of the period (p = 0.02)., Conclusion: For the first time, myocardial fibrosis was prospectively associated with a worse prognosis in patients with HCM. Efforts should be directed to the quantification of myocardial fibrosis in HCM, on the premise that its association with the prognosis can aid in the stratification of risk for defibrillator implantation, and in the prescription of drugs that potentially promote myocardial repair.

OHC is a disorder with a broad spectrum of morphological, functional and genetics abnormalities. The Obstruction on the Right Ventricular Outflow (OHCRV) is not expected most of the time, that's way it is not usually detected and rarely mentioned in the cardiological literature. Its clinical presentation may include basically systemic venous hypertension symptoms that come with the hypertrophic cardyomiopathy manifestations. The manifestations of an apparent Right Ventricular Hypertrophic (RVH) in the ECG are probably due to the huge septal vector that activates the septum with a major thickness. The clinical confirmation of the obstruction on the OHCRV produced by a considerable asymmetric septal hypertrophic is easily shown with bidimensional an Doppler echocardiography.

We present the case of a patient who was previously diagnosed of hypertrophic cardiomyopathy. The patient was admitted to our coronary unit due to a sustained ventricular tachycardia picture. A coronariography was performed as part of the ventricular tachycardia study protocol. It showed angiographically normal epicardic arteries. In the ventriculography, there was a pattern of dilated cardiomyopathy with prominent left ventricular trabeculation, which suggested the diagnosis of non-compacted cardiomyopathy (NCC). The findings of the transthoracic echocardiography, that showed a dilated and hypertrophic left ventricle, with very depressed systolic function, and ventricular myocardium with a thick internal non-compacted endocardium, with a meshwork of multiple trabeculations and intracardic recesses in communication with the ventricular cavity, confirmed this diagnosis. There continues to be little knowledge on NCC and thus it is probably underdiagnosed. It must be considered in the differential diagnosis of patients diagnosed of hypertrophic or dilated cardiomyopathy.

Introduction: Infections by Bartonella spp. include a wide spectrum of emerging and re-emerging infectious diseases, such as culture-negative endocarditis., Methods: Description of 3 cases of endocarditis due to Bartonella spp. and review of those previously reported in Spain., Results: Including these 3 new cases of endocarditis due to Bartonella spp., a total of 6 cases have been reported in Spain. The median age of the patients was 51.6 years and 83.3% were men. There was history of contact with cats in 66.7%, and 50% were alcoholic. Only one patient had prior valvular disease. There were no clinical manifestations typical to any of the Bartonella species. The aortic valve was the one most commonly affected. In all cases, B. henselae was the agent implicated. The diagnosis was made by serology in 5 cases (83.3%). The outcome was favorable in all patients, although 4 of them (66.7%) required valve replacement., Conclusion: Endocarditis due to Bartonella spp. is present in Spain and is likely to be underestimated. We should suspect this pathogen in patients with negative blood cultures and a history of chronic alcoholism, homeless patients, and those who have had contact with cats or who have been bitten by fleas or lice, as well as patients with endocarditis and positive serology against Chlamydia spp.

We report a case of hypothyroid cardiomyopathy manifested as reversible asymmetric septal hypertrophy in a 61-year-old white woman diagnosed 20 years previously with primary hypothyroidism.

Thanks to advances in molecular biology during the last decade, the etiology of hypertrophic cardiomyopathy has been elucidated. Although more than 150 causal mutations of 9 genes that encode contractile proteins have been identified, many of the pathogenetic mechanisms remain unclear. In this review we discuss the current state of knowledge of the functional effects of some mutations, particularly two of the most lethal beta-myosin mutations -Arg403Gln and Arg723Gly (Barcelona mutation)- and their contributions to the pathogenesis of hypertrophy, sudden death and ischemia. Their potential roles in diagnostic and therapeutic strategies are emphasized.

Hypertrophic cardiomyopathy is a relatively common genetic disorder with heterogeneity in mutations, forms of presentation, prognosis and treatment strategies. Hypertrophic cardiomyopathy is recognized as the most common cause of sudden cardiac death that occurs in young people, including athletes. The clinical diagnosis is complemented with the ecocardiographic study, in which an abnormal myocardial hypertrophy of the septum can be observed in the absence of a cardiac or systemic disease (arterial systemic hypertension, aortic stenosis). The annual sudden mortality rate is 1% and, in selected populations, it ranges between 3 and 6%. The therapeutic strategies depend on the different subsets of patients according to the morbidity and mortality, sudden cardiac death, obstructive symptoms, heart failure or atrial fibrillation and stroke. High risk patients for sudden death may effectively be treated with the automatic implantable cardioverter-defibrillator.

Objective: To evaluate the role of myocardial perfusion SPET and radionuclide ventriculography in patients with hypertrophic cardiomyopathy (HC)., Methods: Exercise myocardial perfusion SPET with 99mTc-tetrofosmin and radionuclide ventriculography were performed in a consecutive series of 101 patients (54 15 years, 50 women, 55 with dynamic obstruction) diagnosed of HC by echo. Follow-up from the diagnosis was 9,9 6,7 years (1 to 28 years)., Results: Thirty six percent of patients had perfusion defects (non reversible in 15 and reversible in 21). In non obstructive HC higher number of patients with non reversible defects (p = 0.01 was obseved and in patients with no reversible defects higher incidence of pathologic Q waves in ECG (p = 0.01), Higher ventricular volumes (p < 0.05), lower ejection fraction (p = 0,0001) and longer time to peak emptying velocity (p < 0.05). There were 4 cardiac deaths, 15 syncopes, 18 pacemakers and 6 myectomy. Ejection fraction was higher in patients with syncope (p = 0,034) and there was no isotopic variable predictive of mortality, pacemaker or myectomy., Conclusions: Neither SPET nor radionuclide ventriculography have a prognostic role in patients with HC, but patients with syncope have higher values of ejection fraction. Patients with non reversible defects have higher rate of pathologic Q waves in ECG, higher ventricular volumes and lower ejection fraction. This is indicative of evolution to dilated form of HC.

Introduction and Objectives: The aim of this study was to assess the value of Doppler-echocardiogram for the prognosis and follow up of a hospital-based series of adult patients with hypertrophic cardiomyopathy (HC)., Methods: One-hundred nineteen consecutive patients with HC (52 +/- 12 years, 60 women) were studied over a follow up period of 9.7 +/- 6.7 years. Echocardiographic evolution was analyzed in 104 patients (67 with dynamic obstruction) who had, at least, two echocardiograms performed within an interval of 3.7 +/- 3 years (1 to 7 years)., Results: Seven patients died during follow up and 31 patients developed severe complications (7 deaths, 15 syncopes, 4 class IV angina, 3 class IV dyspnea and 2 acute myocardial infarctions). The presence of mitral insufficiency (p = 0.001) and dynamic gradient > 50 mmHg (p = 0.02) were predictive of mortality and a left atrial index > 25 mm/m2 was predictive (p = 0.028) of severe complications. Fifteen percent of the patients without dynamic obstruction in the first Doppler-echo showed a gradient > 25 mmHg in the last echo. A greater number of patients with mitral insufficiency (80% vs 66%; p = 0.01) and an increase in its severity (p = 0.038) was observed during follow up., Conclusions: Mitral insufficiency, a dynamic gradient > 50 mmHg and a left atrial index > 25 mm/m2 are variables of a bad prognosis in adult patients with HC. An evolution to obstructive HC was observed in 15% of non obstructive HC, and a tendency to increased severity of mitral insufficiency was observed during follow up.

Echocardiography is routinely used for the evaluation of cardiac function. Definition of the endocardial border is essential for the assessment of global and regional left ventricular contractility. This is sometimes difficult due to an inadequate acoustic window. New echocardiographic techniques may be useful to accurate and noninvasively diagnose certain conditions which may otherwise remain undiagnosed with traditional techniques. We present a case of a patient diagnosed with segmental wall motion abnormalities (lateral and apical hypokinesis) by conventional echocardiography. The use of harmonic imaging with contrast changed the initial diagnosis and the patient was diagnosed with severe hypertrophic cardiomyopathy with midventricular obstruction, without segmental wall motion abnormalities.

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease, characterized by asymmetric hypertrophy of the left and/or right ventricle with disarray of myocardial fibers. In order to know its clinical and electrocardiographic manifestation in the pediatric age group, we made a retrospective study of 24 cases from 1986 to 1995. There were: 15 girls and 9 boys, with a mean age of 6 years (age range: 1 month to 17 years). Clinical manifestations were dyspnea (71%), syncope (42%) and palpitations (42%). Physical examination disclosed an aortic systolic murmur in all patients, a mitral regurgitation in 42% and physical signs of congestive heart failure in 54% of patients. Chest X rays showed cardiac enlargement in 71% and pulmonary capillary hypertension in 42%. The most frequent ECG abnormalities were: a prolonged time in the intrinsecoid deflection onset on leads corresponding to the affected region, more or less deep and clean Q waves on leads aVF, aVL, V5 and V6, as well as supraventricular and ventricular rhythm disturbances in 11 patients (46%) with and without congestive heart failure. Bidimensional echocardiography confirmed antero-septal hypertrophy in all patients. The mortality rate was 17%. HCM is rare disease in the pediatric age group. Mortality increases when congestive heart failure and arrhythmias are present. Treatment must be individualized in all cases.

It has been presented a case of an eleven-year-old patient admitted with a pattern of ventricular fibrillation and diagnosed as hypertrophic cardiomyopathy. Admission analysis and myocardia anatomy evolution suggested ischemic etiology. We checked the risk factors of sudden death, its relation with ischemic disease and the etiology of ischemia in the hypertrophic myocardiopathy.

During the last 20 years, the principal objective in hypertrophic cardiomyopathy research has been the refinement of algorithms for the identification and treatment of patients at risk of sudden death. Sudden death is an important problem in hypertrophic cardiomyopathy, with an incidence of 4-6% in referral populations and approximately 1% in non-referral centers and because it affects young and often asymptomatic patients. We now know that hypertrophic cardiomyopathy is not a single disease, but a group of diseases caused by mutations in genes encoding different sarcomeric proteins. The phenotypic expression depends on multiple modifying genetic and environmental factors. Even though genetic testing is not presently a practical approach in hypertrophic cardiomyopathy risk stratification, it is important to consider new genetic data in the prognostic evaluation of patients. In this paper, we review the published data on risk stratification in hypertrophic cardiomyopathy and we set forth our opinion with regard to the available therapeutic options and their indications in the prevention of sudden death.

The hypertrophic cardiomyopathy may be associated with a variable degree of left ventricular outflow tract obstruction. There are several available therapeutic strategies for treatment: pharmacological, dual-chamber pacing, surgery and induced septal infarction. This last one is a novel technique with less experience in practice. We present the clinical case of a patient which showed persistent and severe obstruction in spite of the medications and dual-chamber pacing, and who underwent this novel technique. The results were optimal but new observations arise from this particular case.

We report two cases of patients who underwent simultaneous triple transplants (liver-pancreas-kidney) using organs taken from a single donor in each case. The anesthetic technique and perioperative treatment of each patient is described. The favorable evolution in both cases seems to indicate that although this type of transplant may be more complex, it is nevertheless a good therapeutic option for patients suffering terminal liver failure, kidney failure or diabetes type I.

We report the case of a young man first seen by is in 1989 at the age of 20 years. The diagnoses of hypertrophic cardiomyopathy, Wolf-parkinson-White syndrome, congestive heart failure and pulmonary hypertension were made. One month later the patient had jaundice and hepatomegaly and a diagnosis of acute viral hepatitis A was established by laboratory findings. The ALT and AST levels were persistently elevated, seven times the normal mean, during six years. Two liver biopsies in 1991 and 1993 showed liver injury secondary to congestive heart failure (CHF) as the only abnormality. This case illustrates the importance of liver injury secondary to CHF as a cause of a marked and persistent increase of ALT and AST that resembles that of other liver diseases.

Introduction and Objectives: The lack of specificity of electrocardiographic (ECG) recording showing isoelectric T-waves often leads to diagnostic doubts. This study attempts to demonstrate that asymptomatic patients who demonstrate benign forms of hypertrophic cardiomyopathy may be identified from a typical ECG pattern showing isoelectric T-waves., Methods: In 45 out of 5,126 asymptomatic healthy aircrew members, an ECG pattern which showed isoelectric T-waves in all leads, but were normal in V2 and V3, was found. 12 of them had negative T-waves in III and aVF leads. Cardiac echo-Doppler, Bruce test and 24-hour Holter recordings were performed in 29 of the 45 cases; all were males aged 30-55 (43.1 +/- 7.1) without any associated disorder nor engaged in heavy exercise, and a control group of 15 healthy subjects. In each of the 29 cases, 2-21 ECG's (11.1 +/- 6.3) performed over a period of 2 to 20 years (13.0 +/- 5.9) were reviewed., Results: In 23 of the 29 cases (79.3%), echo-Doppler criteria of non obstructive mild hypertrophic cardiomyopathy were found. T-waves became normal during exercise testing, returning to isoelectric during recovery, in 19 of the 23 cases. In 17 of these cases, reversible T-wave changes were observed between successive ECG's; T-wave changes became clearly negative when the level of exercise was increased. Holter recordings did not show pathological findings., Conclusions: We believe that echo-Doppler studies must be performed in asymptomatic patients showing the ECG pattern described above, because they are strongly suspicious of having mild hypertrophic cardiomyopathy.

The aim of surgery in hypertrophic cardiomyopathy is to reduce the intraventricular obstacle, increase left ventricular compliance, correct mitral regurgitation and other associated lesions, such as coronary atherosclerosis disease or endocarditis. Several surgical techniques have been proposed; myotomy, myomectomy, mitral valve replacement and mitral valve plication. The last mentioned technique combined with myomectomy can be performed safely and may represent an alternative to mitral valve replacement in cases with enlarged and elongated mitral leaflets. We review the natural evolution of the disease and its treatment, emphasizing on the possible mechanism by which surgical treatment may ameliorate the disease.

Hypertrophic cardiomiopathy is a peculiar process with different anatomical and functional abnormalities which are present in different degrees in each case. Echocardiography and Doppler techniques have contributed definitively to the knowledge of this process and these procedures are choices for establishing the diagnosis of hypertrophic cardiomyopathy and to evaluate the clinical and morphological diversity of this process consisting of a spectrum of abnormalities with a variable presence in each case. Disproportionate septal hypertrophy is the most frecuent finding but the hypertrophy can involve other segments and different patterns can be present; concentric hypertrophy, apical, involving right ventricle, inverted asymetric, etc. Anterior mitral valve motion can be produced by the interrelation between anatomic factors of the valve, geometry of the outflow tract and physical forces produced by flow changes. Doppler echocardiography allows us to evaluate subaortic obstruction, to define its site, to demonstrate and measure the degree of mitral regurgitation and to carry out intraoperative studies. In hypertrophy cardiomyopathy the pattern of delayed relaxation is the most frequent but patients with severe obstruction and mitral regurgitation can pseudonormalize this pattern and even show restrictive patterns.

Hypertrophic cardiomyopathy is characterized by abnormalities of the myocardium, and the activation and conduction tissues, that may have separate manifestations, but often occur together in complex clinical pictures. The subaortic gradient, although not always present, is the most classical manifestation of the disease, with its typical dynamic behavior, changing with preload, afterload and contractility. In most cases it is due to systolic motion of the mitral valve against the septum in systole, but in a few it is caused by midventricular "constriction". Alteration of diastolic ventricular function is important, and probably the main cause of heart failure, that is usually accompanied by normal systolic function. Mitral insufficiency is common in the obstructive forms, due to the abnormal mitral valve motion, but in some cases it may be due to structural abnormalities of the valve. There may be systolic constriction, or nonatherosclerotic occlusion of the intramyocardial coronary arteries, causing myocardial infarction and ventricular aneurysms, that may lead to systolic dysfunction. The electrocardiogram is rarely normal. Hypertrophy patterns, deeply inverted T waves, deep Q waves, QRS slurring suggestive of WPW syndrome without true preexcitation are the most common manifestations. Rhythm disturbances are common and include sinus node dysfunction, superconductor atrioventricular node or heart block. Atrial fibrillation is frequent and may have catastrophic consequences, including systemic embolism. Non-sustained ventricular arrhythmias are often present, but its predictive value for sudden death is unclear. Monomorphic ventricular tachycardia is infrequent, and programmed stimulation is more likely to precipitate polymorphic ventricular tachycardia of difficult clinical interpretation. Sudden death may be due to multiple mechanisms, and it is difficult to predict and prevent.

Introduction and Objectives: We analyzed the characteristics of mitral valve apparatus by transesophageal echocardiography in a consecutive series of patients with hypertrophic cardiomyopathy., Methods: We performed a transesophageal study in 60 patients; 35 of them had intraventricular obstruction at basal state. The following measurements and observations were made in the frontal long-axis transesophageal plane: a) length of both mitral leaflets and dimensions of left ventricular outflow tract that were compared with those obtained from 25 normal subjects; b) structure involved in the systolic anterior mitral motion; c) mechanism of mitral regurgitation, and d) sequence of systolic events., Results: Compared with control subjects (anterior mitral leaflet: 2.86 +/- 0.3 cm, posterior mitral leaflet: 1.62 +/- 0.2 and 1.7 +/- 0.2 cm, respectively; the mitral leaflets were longer in patients with and without subaortic obstruction (anterior leaflet: 2.3 +/- 0.1; posterior leaflet: 1.07 both, p < 0.01). Systolic anterior motion was observed in 49 patients, with mitral leaflet-septal contact in 87% of patients with obstruction and in 11% of nonobstructive patients (p < 0.01). Structures participating in this phenomenon were: distal portion of the anterior mitral leaflet (77.5%), of both mitral leaflets (18.4%) and anomalous chordae (4.1%); in 5 patients the obstruction was located at a more distal level. Mitral regurgitation was observed in 43 patients; in 37 of them the jet was posteriorly directed in late systole. Patients with hypertrophic cardiomyopathy have longer mitral leaflets with frequent associated abnormalities suggesting that this disease is not confined to myocardium and that leaflet length is not the sole determinant of the obstruction; 2) in almost 80% of patients the systolic anterior motion was produced by the distal anterior mitral leaflet resulting in incomplete coaptation in mid-systole; 3) the sequence of systolic events was ejection/obstruction/leak.

A 45-year-old male with palpitations and a heart murmur was investigated. Echocardiography and haemodynamic study revealed the presence of a ostium primum type interatrial communication with left-right shunting and asymmetric hypertrophic heart disease. There was no subaortic obstruction, but anterior systolic movement of the mitral valve was detected that did not contact with the interventricular septum--in part due to the paradoxical motion of the latter. The possible benefit of surgery in this infrequent association is discussed, and a review is made of the literature.

The form of presentation of a new case of Melas Syndrome is described, together with a pathological and neuroimage study, including clinical development over a 3 year period. The usefulness of MR should be underlined here, given clinical doubts, and also normality in the EMG early phases of and the association with obstructive hypertrophic miocardiopathy.