[Therapeutic approaches in symptomatic hypertrophic obstructive cardiomyopathy]. / Estrategias terapéuticas en la miocardiopatía hipertrófica obstructiva sintomática.

Hypertrophic cardiomyopathy is a complex and heterogeneous disease. Although most patients experience just a few symptoms, and have a good prognosis, there are others whose symptoms are severe and progressive, determined by different pathophysiological elements such as diastolic dysfunction, myocardial ischemia, arrhythmias and subaortic obstruction. Approximately 20-30% of hypertrophic cardiomyopathy patients develop an intraventricular dynamic gradient, which in some cases, is responsible for severe symptoms which are ameliorated once the obstruction is reduced. In many cases the symptoms can be controlled with medical treatment which includes betablockers, calcium-channel antagonists and dysopiramide, but some patients will still experience severe and refractory symptoms. This subgroup of patients, which represent approximately 5-10% of patients with hypertrophic cardiomyopathy, can be problematic from a management perspective. For many years, septal myectomy and/or mitral valve replacement offered the only effective alternative therapy for these patients. However, the high rates of morbidity and mortality associated with these procedures have necessitated the search for new and less invasive procedures such as ventricular pacing and percutaneous septal ablation. Although the initial results with sequential pacing were encouraging, further studies have suggested a significant placebo effect, which makes its application controversial. In the last 5 years selective embolization of the septal artery precipitating a localized myocardial infarction has been utilized to reduce the subaortic gradient. The potential indications and efficacy of these new forms of treatment, like ventricular pacing and percutaneous septal ablation, are presently under evaluation and are the main subject of this review. Medical treatment, with either beta-blockers, calcium channel antagonists or dysopiramide constitutes the first therapeutic step. Surgery, while alleviating the subaortic obstruction and reducing the intraventicular pressure and mitral insufficiency, produces important and long-lasting symptomatic and functional improvement in most of these patients, and it continues to be an important therapeutic alternative in these cases. If the first results with sequential pacemaker implants were encouraging, today it is alluded to an important placebo effect that causes its application to be controversial. In the last 5 years the path has been made in the creation of a septal infarction located through the embolization of the septal branches to reduce the gradient.

[The clinical practice guidelines of the Sociedad Española de Cardiología on cardiomyopathies and myocarditis]. / Guías de práctica clínica de la Sociedad Española de Cardiología en miocardiopatías y miocarditis.

Myocardial diseases are a extraordinarily heterogeneous group of processes that only have in common the fact that they involve heart muscle and that they cause a wide spectrum of myocardial dysfunction. The approach of the management and treatment of the cardiomyopathies is a continuous matter of discussion because the vast majority of alternatives in this field have not been based on the best scientific possible evidence and, since except for the case of heart failure associated with dilated cardiomyopathy. The majority of different options have not been studied by means of large (or even small) randomized trials. Nevertheless, this chapter has tried to provide the reader with different approaches on how to deal with important clinical problems in dilated, hypertrophic and restrictive cardiomyopathies, and in myocarditis as well. For this, we have utilized the most relevant information found coupled with our best clinical judgment, although we admit that many of the clinical recommendations can be controversial.

[Acute myocardial infarction after anaphylactic reaction to amoxicillin]. / Infarto agudo de miocardio tras reacción anafiláctica por amoxicilina.

A 62-year-old man was admitted to the hospital in a state of shock with electrocardiographic signs of inferior-wall acute myocardial infarction. He was initially diagnosed of cardiogenic shock. An urgent coronary angiography showed an irregular stenosis of 90% in the right coronary artery. Coronary angioplasty was performed, and a stent was placed in this lesion. In-depth questioning of the family revealed that the patient had taken an oral dose of amoxicillin 15 minutes before the onset of the symptoms. Further tests proved that he was allergic to amoxicillin, and was diagnosed of anaphylactic shock, complicated with acute myocardial infarction. There are several reports of myocardial infarction as a complication of anaphylactic reaction. We have found very few cases related to antibiotics, and none associated with amoxicillin. The most frequently quoted mechanism in these cases is coronary artery spasm due to the mediators of anaphylaxis. In the case reported herein, the irregular lesion suggests that disruption of an atherosclerotic plaque and platelet aggregation have occurred.

[The electrocardiogram in apical hypertrophic myocardiopathy. A case report with unique manifestations]. / El electrocardiograma en la miocardiopatía hipertrófica apical. Presentación de un caso con manifestaciones únicas.

We report the case of a 63-year-old female patient with apical hypertrophic cardiomyopathy, diagnosed by the presence of localized apical hypertrophy in the echocardiogram and a typical "spade like" left ventricular angiographic image, but with unique electrocardiographic features, characterized by chronic ST segment elevation, and T wave inversion, in the anterolateral leads. These changes were initially interpreted as a manifestation of acute ischemic heart disease. Chronic ST segment elevation has been occasionally described in patients with hypertrophic cardiomyopathy complicated with apical necrosis and aneurysm formation, but not in uncomplicated cases of apical hypertrophic cardiomyopathy. Its knowledge by the physician could allow avoidance of problems of differential diagnosis with more frequent heart diseases, especially acute atherosclerotic ischaemic heart disease.

[Pathology of the heart of noncardiac origin. VIII. Cocaine and the heart]. / Patología del corazón de origen extracardíaco (VIII). Cocaína y corazón.

The marked increase in cocaine consumption observed in recent decades, has led to the identification of previously unknown multiple medical problems. Cardiovascular complications related to cocaine abuse include myocardial ischemia and infarction, myocarditis, cardiomyopathy, rhythm disturbances and sudden death, endocarditis, pneumopericardium and left ventricular hypertrophy. Although the mechanisms involved in cocaine-related cardiac diseases are multiple, many cardiac complications in these patients are caused in part or totally by an increase in adrenergic activity due to the blockade of catecholamine reuptake induced by the drug.

[Myocardiopathies (III). The classification and morphological patterns of hypertrophic myocardiopathy]. / Miocardiopatías (III). Clasificación y patrones morfológicos de la miocardiopatía hipertrófica.

Hypertrophic cardiomyopathy is characterized by a great variety of clinical manifestations and morphologic patterns. Hemodynamic classification in obstructive and nonobstructive forms may be clinically relevant since, although many patients have disabling symptoms in the absence of a pressure gradient, in the subset of patients who do have a gradient, symptom severity seems to relate its magnitude; this fact supports the use of some therapeutic approaches directed to reduce or eliminate obstruction. Nevertheless, the presence and severity of the gradient do not correlate with prognosis. On the other hand, from a morphological point of view, any classification will be limited by the great diversity of morphologic patterns, which include almost all forms of ventricular hypertrophy one could imagine, and by the possibility of significant changes in the severity and distribution of the hypertrophy during the life of the patient. In general, the degree and distribution of ventricular hypertrophy do not correlate with symptoms or prognosis. The identification of different molecular genetic abnormalities responsible for the development of hypertrophic cardiomyopathy, is expected to provide new insights in the etiology and pathogenesis of this disease, which should be considered in future classifications.

[A paradoxical intraventricular gradient in a patient with atypical hypertrophic myocardiopathy]. / Gradiente intraventricular paradójico en un paciente con miocardiopatía hipertrófica apical.

A paradoxic diastolic intraventricular flow (directed from apex to base), caused by a pressure gradient between a small apical aneurysm and the main left ventricular cavity, connected by a narrow muscular tunnel, was detected by Doppler echocardiography in a 69 year old male with apical hypertrophic cardiomyopathy and effort angina. Coronary arteriography was normal and the clinical, electrocardiographic, echocardiographic and angiocardiographic features of the patient were otherwise typical of apical hypertrophic cardiomyopathy. Paradoxic diastolic gradients have been occasionally described in patients with hypertrophic cardiomyopathy, and indicate the existence of a sequestered apical cavity, which is frequently not detected by echocardiography. Identification of these gradients may have important clinical implications, since they have been related with a higher incidence of embolic events, severe ventricular arrhythmias and myocardial ischaemia and necrosis.

Family history as an independent risk factor for ischaemic heart disease in a low incidence area (Galicia, Spain).

It has been suggested that a family history positive for coronary heart disease (CHD) increases the risk of CHD. We studied this association to determine the degree of risk, the independence of this association and the presence of interaction of a family history of CHD with the major known risk factors in a low incidence area. One hundred and six hospital cases (85 males and 21 females) of CHD and 106 hospital controls individually matched with each case for sex, age and place of residence (rural-urban) were studied. From every participant, information was collected on their personal and family history of cardiovascular disease and risk factors; height, weight, lipid profile and blood pressure were measured, and an electrocardiogram was recorded. Conditional logistic regression was used in the analysis. The observed odds ratio of patients suffering from CHD among those with, compared to those without, a positive family history of CHD was 4.95 (95% confidence interval = 1.27-19.28) after adjusting for the major known risk factors in each individual and their families (no interaction term remained in the model). The results support the hypothesis that a family history of CHD, acting through mechanisms other than known risk factors or their familial aggregation, is an independent risk factor for CHD even in a low incidence area. No interaction effect was observed between family history and the presence of the three major risk factors of CHD. This should help to identify individuals at greater risk of CHD.

[Apical hypertrophic cardiomyopathy and coronary arteriovenous fistula]. / Miocardiopatía hipertrófica apical y fístula arteriovenosa coronaria.

We present a case of association of apical hypertrophic cardiomyopathy of the Japanese type and coronary arteriovenous fistula in a 56-year-old male who presented with anginal symptoms. Both cardiopathies can produce myocardial ischemia and angina, and their association could aggravate the ischemia. In our patient the symptoms were adequately controlled with Verapamil. The coexistence of these two rare entities in the same patient has recently been described in 2 other cases, allowing us to speculate on a possible etiological relation between the 2 abnormalities, probably both been originated in a common developmental error.

[Myocardial disease in acute idiopathic pericarditis (author's transl)]. / Afección miocárdica en la pericarditis aguda idiopática.

Acute idopathic pericarditis can be accompanied by myocarditis, and in all types of acute pericarditis there are electrocardiographic signs of myocardial lesions. In order to determine the severity of the myocardial disease in acute idiopathic pericarditis, a prospective study has been carried out in a group of 25 patients with this diagnosis. The clinical evaluation included phonomecardiographic measurements of the systolic intervals, Weissler index in 24 cases, and echocardiographic study of the left ventricle in eight cases. Besides that, the serum levels of the myocardial enzymes (GOT, GPT, CPK, and LDH and its isoenzymes) were determined in all cases. The results showed a gallop rhythm in 8 percent of the cases, supraventricular arrhythmias in 4 percent, dysfunction of the left ventricle by systolic intervals and/or echocardiography in 32 percent, and increase of the myocardial enzymes in 24 percent, which represents a global incidence of myocardial disease of 44 percent. However, only three patients presented clinical manifestations of myocardial disease, although the congestive cardiac failure was always secondary to cardiac tamponade and not to myocardial failure. The increase of myocardial enzymes can determine important problems of differential diagnosis with an acute myocardial infarction. The clinical course was favourable in all of the cases, including the ones which showed myocardial disease.

[Paradox arterial embolism: diagnosis during life]. / Embolia arterial paradójica: diagnóstico en vida.

Paradoxical systemic embolism in the absence of congenital heart disease is often considered a clinical or pathologic rarity. We have recently observed two cases of paradoxical systemic emboli, secondary to massive pulmonary embolization in the presence of a patient foramen ovale in an otherwise normal heart. One case was lethal and the diagnosis was confirmed at autopsy. The other case was diagnosed by cardiac catheterization with dye dilution curves and the patient recovered after recurrences were prevented by the placement of an inferior vena cava umbrella-filter. Paradoxical embolization should be suspected when systemic emboli occur without an apparent cause. The diagnosis can be established by cardiac catheterization, pulmonary angiography and phlebography, and recurrences can be prevented by anticoagulation and partial interruption of the inferior vena cava, when the emboli arise from its territory.