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According to the description made by Holt and Oram in 1960, a wide group of associated skeletal and cardiac malformations was named as syndrome of Holt-Oram. The morpho-anatomic and functional expression of the syndrome are so great in extension, that it is possible to think of several clinical entities with common manifestations; or perhaps, in a common entity with different clinical and functional expressions. Undoubtedly, the interest in the knowledge and diagnostic of the disease is due mainly to the hereditary condition, as demonstrated in a great number of cases, though they had been observed some generations without antecedents, with the possible explanation of genetic mutation. In this paper, are demonstrated the descriptive findings in a patient, male, 32 years old, with the diagnostic of Holt-Oram syndrome, and also the results of the study of the near members of his family. This study allowed to find other affected persons with manifestations that probably can be the result of new mutations. The paper is completed with a wide review of the pertinent bibliography.

The increasing incidence of abdominal aortic aneurysm (AAA), mainly due to the aging population, and its mortality of 85-90% in the event of rupture justify opting for early diagnosis and elective treatment to repair it. The main aim of this paper is to analyze the utility of transthoracic echocardiography (TTE) in the study of infrarenal aorta and AAA screening.The study included 512 patients (309 men and 203 women) consecutively assessed by TTE and, where possible, abdominal ultrasound for any reason in a cardiology department.An AAA was detected in 25 patients (5.1%), the minimum age at diagnosis was 55 years, the ratio of men to women was 7.3:1 and the mean diameter of the aneurysms was 39.5 ± 12.2mm. Risk factors associated with AAA were to current and former smoking, age, and presence of femoral murmur. The TTE results were equivalent to those of abdominal ultrasound. All patients with AAA studied by coronary angiography showed significant coronary lesions.It is feasible and useful to complement conventional TTE with the study of the infrarenal aorta for AAA screening in patients visited at the department of cardiology. This study should be performed mainly in patients ≥55 years old or with risk factors to develop an AAA.

This paper describes the general situation regarding heart transplantation in Spain and the results obtained using the technique, incorporating data for the year 2004. In 2004, 294 heart transplants were carried out, which brings the total number of procedures performed since 1984 to 4680. Clinically, the typical heart transplant recipient in Spain is male, aged around 50 years, has blood group A, has non-revascularizable coronary artery disease, and is in NYHA functional class IV/IV. The percentage of emergency heart transplantations was 35%, which is higher than in the previous year (29%), and higher than the mean for the preceding 5 years (22%). The early mortality rate was 10%, which is lower that the mean for the preceding 5 years (13%). After combining the results for 2004 with those of previous years, the probability of survival at 1, 5 and 10 years was 80%, 70% and 60%, respectively. When the survival rates for different time periods were analyzed, a significant improvement could be seen in the last 5 years, with recent survival rates being 85% and 72% at 1 and 5 years, respectively. The most frequent cause of death in the first month was acute graft failure; in the first year, infection and rejection; and, over the long term, tumors and a combination of graft vasculopathy and sudden death. A comparative analysis of survival rates showed that long-term results in Spain are slightly better than those published in the world literature. Moreover, survival has tended to improve gradually in recent years.

Mitral valve disease often is accompanied by chronic atrial fibrillation, especially when the left atrium is enlarged. Mitral valve surgery alone cannot resolve the arrhythmia in most cases. Several surgical techniques have been proposed. We have used surgical left atrial reduction and pulmonary vein isolation to eliminate chronic atrial fibrillation associated with mitral valve disease. The aim of this paper is to report our experience with this new surgical concept.Twenty-three patients (18 women and 5 men, mean age 44.1 14.7 years) with mitral valve disease and chronic atrial fibrillation for more than 3 months underwent surgery. Mitral valvuloplasty was performed in 11 cases, and mitral valve replacement in 12. Left atrial reduction was used to eliminate chronic atrial fibrillation in all cases.There was no operative mortality. Three patients (13%) had recurrent atrial fibrillation between postoperative days 6 and 8. No atrioventricular conduction disturbances were observed. An important reduction in left atrial size was evident on echocardiography (4.8 0.77 vs. 8.1 1.47 cm, p0.01). In 3 to 36 months of follow-up (13.9 11 months), all patients preserved sinus rhythm.Left atrial reduction seems to be an effective and easily applied alternative method for treating mitral valve disease with chronic atrial fibrillation.

Multiple clinical and echocardiographic parameters have been shown to have prognostic value in cases of left ventricular dysfunction. The purpose of this paper was to evaluate the relative predictive power of such parameters.Ninety-one patients with systolic dysfunction were prospectively studied. Functional status was evaluated using the New York Heart Association classification and the 6-minute walking test. Other clinical and biochemical parameters were assessed, and an anatomic and functional echocardiographic study was performed.Mean follow-up was 16.5 months (SD: 6.95). Eighteen patients died and two underwent heart transplantation (cardiac death 22%). Multiple regression analysis showed that the only independent predictor of death was functional status. Functional classes I and II showed a 16-month mortality rate of 10%, class III 40% and class IV 83%. The mortality rate was 67% for patients who walked300 meters and 0% for those who reached500 meters. When echocardiographic results were analyzed separately, the only independent predictors of outcome were left atrial diameter and the E wave deceleration time. Deceleration times100 ms or atrial diameters5 cm were associated with a mortality rate of 46%. The correlation between E wave deceleration time and the walking test was r = 0.55, p0.0001.Functional status is the main predictor of outcome in patients with systolic dysfunction, whether assessed subjectively or estimated objectively by a walking test. Among echo-Doppler parameters, the deceleration time of the E wave and left atrial diameter gave similar prognostic information, although with less statistical significance. They can confirm or substitute the prognosis obtained by the functional classification.

The large amount of information on rates of acute coronary syndrome accumulated in Spain over the last two decades is summarized in this paper, which also estimates the number of cases expected in 2002 and the trend for 1997 to 2005.Published information on the situation in the 90's was reviewed and summarized. We present the incidence of acute myocardial infarction (AMI), and an estimate of the absolute number of patients expected for various acute coronary syndromes in each autonomous community in Spain in 2002, along with the trend for 1997 to 2005.Approximately 68,500 patients will suffer AMI in 2002 and 40,989 of them will be hospitalized, while the rest will die before admission. A further 24.9% of admitted patients will not survive 28 days. Slightly less than half will be younger than 75 years old, an age with a better prognosis (28-day mortality 38.8%). Approximately 33,500 patients with unstable angina will be admitted, and 4.5% of them will die within 3 months of admission. Assuming the incidence of AMI remains stable, the absolute number of cases will increase by 2.28% yearly (9,847 cases in total) and hospitalizations for acute coronary syndromes will increase by 1.41% (8,817 cases in total) between 1997 and 2005.Ischaemic heart disease generates increasing demand for health care in Spain. Case fatality is high among the approximately 68,500 AMI patients, given that scarcely two thirds will have been hospitalized in 2002.

Recurrent symptomatic pericardial effusion can complicate different cardiac and extracardiac diseases. When recurrent pericardial effusion after drainage with conventional catheter techniques occurred the creation of a pericardial window by open surgery used to be the unique treatment available until the recent development of percutaneous balloon pericardiotomy.The aim of this paper is to review our initial experience with percutaneous balloon pericardiotomy for the treatment of patients with recurrent pericardial effusion.Five patients with recurrent pericardial effusion have been treated with percutaneous pericardiotomy until now. Four patients had malignant pericardial effusion secondary to metastasis of extracardiac tumors, in one patient recurrent pericardial effusion was idiopathic. In all patients percutaneous balloon pericardiotomy was performed with a pediatric valvuloplasty balloon catheter, through a subxiphoid approach.Successful drainage and balloon pericardiotomy was achieved in all patients without severe complications. In all cases only one pericardial site was dilated. Minor complications were registered, which included mainly mild pleural effusion occurring in all patients with spontaneous resolution. During a mean follow-up period of 8.6 6.5 months (range 2 to 18 months) there were no recurrences of effusion or tamponade. Two patients died, 1 month and 9 months after the procedure, due to their malignant condition.Percutaneous balloon pericardiotomy is an easy and useful technique to manage patients with large recurrent pericardial effusion with a low r

This paper aimed to study the usefulness of the Statistical Bulletin of Deaths (SBD) for identifying extrahospital deaths due to acute coronary ischaemia, and to determine the most efficient strategy in the selection of death causes that appear in the SBD, which are the most efficient for the identification.Those extrahospital deaths, which, among the causes of death, recorded a diagnostic code indicating that they might have been caused by coronary ischaemia, were included. To study the usefulness of the death certificate we calculated the sensitivity and the positive predictive value of cardiac ischaemia. To determine the most efficient strategy for selecting causes of death we compared two selection strategies: the first, using only the basic cause of death; and the second using all the causes appearing in the SBD.Of the 395 SBD selected, 161 were classed as acute heart attacks. In those SBD in which cardiac ischaemic disease was given as the basic cause of death, we obtained a sensitivity of 82.6% (CI 95%: 75.9-88.1) and a positive predictive value of 72.7% (CI 95%: 65.6-79). The most efficient strategy in SBD selection proved to be the investigation of death certificates in which cardiac ischaemia appeared as one of all the causes of death, and death certificates in which the basic cause of death was coded as diabetes mellitus, essential arterial hypertension, hypertensive heart disease, cardiac dysrhythmia, and cardiac insufficiency.The information provided by death certificates for extrahospital deaths due to coronary ischaemia is reliable. A sensitive and efficient SBD selection strategy is proposed for the detection of cases.

Renewed interest for aortic valve disease has evolved in recent years. Aortic valve replacement has become the second most frequent cause of cardiac surgery, following coronary bypass surgery. In addition, the etiologic and physiopathologic knowledge of this disorder has improved. In the present paper we analyze three aspects of the disease which are, at present, the subject of study and controversy: first, we discuss the possible relationship between degenerative aortic stenosis and atherosclerosis; second, the involvement of the aortic root in cases of bicuspid aortic valve; and third, the surgical indications in asymptomatic patients with either aortic stenosis or regurgitation.

Most exercise testing is performed in adults with known or suspected ischemic heart disease. In the last few years cardiac imaging techniques have been applied in this field, improving the information obtained with the procedure. However, the exceptions to this rule are emerging rapidly not only in healthy people (asymptomatic individuals, athletes, handicapped people) but also in cardiac patients (advanced congestive heart failure, hypertension, rhythm disorders, congenital heart disease, etc.). All the-se issues justify the need for a multidisciplinary consensus document in Spain. This paper reviews and updates the methodological aspects of the stress test, including those related to oxygen consumption measurements. The main aim of this review was to determine the role of exercise testing in the evaluation of ischemic heart disease as well as the applications of imaging stress testing. The usefulness of this test in other non-ischemic cardiac disorders and in selected subsets of healthy people is also reviewed.

The information concerning stroke mortality is limited in Spain, and the information on morbidity is even scarcer similarly to other countries. This is true also for the decrease of frequency observed in the last decades. The objective of this paper is to provide data in the incidence, mortality and cardiovascular risk factors associated to stroke in our surrounding through by the prolonged observation of a working population.In the Manresa Study, which began in 1968, a cohort of 1,059 men, from 30 to 59 years old, was followed for 28 years. We recorded new cases of fatal and nonfatal stroke and the relationship between stroke incidence and risk factors of cardiovascular disease found in the initial examination.Incidence rate for stroke was 183 x 100,000 per year, 64% of the cases were registered after they turned 60 years of age. Mortality rate due to stroke was 88 x 100,000 per year, 91.6% of fatal cases were over 60 years old. Factors associated to the stroke morbimortality incidence were age, high blood pressure and overweight. In a bivariate regression model, stroke mortality was found significantly associated to the presence of atrial fibrillation, diabetes, hypercholesterolemia and tobacco smoking.Stroke frequency rates in the Manresa cohort are ranged at a medium level compared to data from other general population studies. The role of atrial fibrillation in the stroke morbimortality has been confirmed. The associated factors, age, high blood pressure and overweight, are similar role to that which was found in other research studies. The priorities in the cerebrovascular disease prevention in our surroundings are discussed.

Coronary by-pass grafting is a well established procedure for ameliorating ischemic coronary disease. From time to time it is necessary to re-operate these patients. The objective of our paper is to present our experience in this field. Retrospective analysis of 128 patients operated on between February 1978 and November 1996, has been analyzed. The mean age was 57.4 +/- 0.7 years. 77.2 +/- 5 months elapsed between operations. Stable angina (20.4%) or unstable angina (76.3%), myocardial infarction (48%) and congestive heart failure (17%) were the predominant clinical manifestations.Hospital mortality was 10.9% (14 patients) and in the follow-up there were 16 deaths (14%). Perioperative myocardial infarction was the main cause of in-hospital mortality. In the follow-up there were 4 deaths due to myocardial infarction and another 4 patients died from neoplasms. Perioperative myocardial infarction was present in 9.3% (12 patients)a) Re-do coronary by-pass grafting is still a good procedure for solving myocardial ischemia in spite of a higher mortality and morbidity than in the original operation. b) There is no progression in the number of patients according to our experience, probably due to better techniques and the frequent actions by an intervention cardiologist. c) The long-term results are good enough, but with a higher mortality.

In Spain ischemic heart disease mortality was increasing prior to 1975 and has decreased since 1975. This trend is common to both genders. The goal of this paper is to separate the genetic, environmental and competitive risk factors influencing this evolution.The Gompertz function was adjusted cross-sectionally to age-specific mortality due to ischemic heart disease for each year from 1951 to 1992. The Gompertzian longitudinal analysis was applied to the coefficients obtained to estimate the effect due to environmental and competitive factors.Ischemic heart disease in Spain is a Gompertzian disease with an intersection point at 67 years for men and 40 years for women. Environmental factors were increasing before 1975 and have decreased since then. However, the competitive factors decreased in men since 1980 and in women since 1951 on.The evolution of risk factors (smoking, hypercholesterolemia and hypertension) is responsible for the major proportion of ischemic disease mortality changes. Treatment of instaured ischemic disease has a low influence.

The superiority of surgical treatment over other procedures in the left main coronary artery stenosis is well known, being today the therapy of choice. The purpose of this work is to analyze the clinical characteristics and the immediate results of surgery in our patients.In this paper we under-took a retrospective study of 147 consecutive patients, 129 men and 18 women with a 50% or more left main coronary artery stenosis without associated valvular disease, operated on at our institution during a period of 3.5 years, between January 1992 and May 1995. Thirty-one variables were analyzed under Chi-square, comparison of proportions and Student's t-tests. Then, it has been developed into a multivariant logistic regression of significant variables (p less than 0.05) of factors influencing mortality and rhythm disturbances which have been the most frequent postoperative complication.The mean age was 65 years. Sixty-two per cent had unstable angina and 51.7% had previous myocardial infarction. An average of 3.1 grafts were performed. Total mortality was 6.8%. The complications were 17% arrhythmias, 8% low cardiac output and 6% perioperative myocardial infarction. In the multivariate analysis, mortality has been strongly related to the presence of perioperative myocardial infarction and also with moderate to severe cardiomegaly and a high left ventricular end-diastolic pressure. Arrhythmias were related to an advanced age.1) In hospital mortality remains within acceptable limits and is influenced by the presence of perioperative myocardial infarction, cardiomegaly and a high left ventricular end-diastolic pressure, and 2) elderly patients have more damaged vessels, more diseased coronary segments, and more complications, especially rhythm disturbances.

This paper describes our preliminary experience with left main coronary angioplasty in 8 patients (9 procedures). In 6 patients the left main coronary artery was "protected" either by previous by-pass surgery (4 patients) or by collateral vessels from the right coronary artery (2 patients). Three patients had a total occlusion of the left main coronary artery and 2 of them had a recent or acute myocardial infarction and the coronary angiogram suggested a thrombotic occlusion of the infarct-related artery. Three patients were not considered surgical candidates and an additional patient, who was in cardiogenic shock, required an emergency coronary angioplasty as "rescue" procedure. A successful dilatation was achieved in 6 patients (including a patient with successful deployment of a Palmaz-Schatz stent) but, unfortunately, one them eventually died 7 days later from a femoral sepsis related to the procedure. However in the 2 remaining patients--with a total occlusion of the left main coronary artery in relation with a myocardial infarction--the dilatation procedures were unsuccessful. One patient underwent a successful repeat coronary angioplasty for restenosis of left main coronary artery. Our preliminary experience confirms previous reports suggesting the value of coronary angioplasty in patients with left main coronary artery disease providing a careful selection of possible candidates is performed prior to the procedure.

Double-orifice mitral valve is an uncommon congenital heart defect. The isolated occurrence of this anomaly is exceptional and, more often, is encountered in association with other congenital cardiac abnormalities. Principal among these are the partial and complete forms of the atrioventricular canal. In this paper, we present two cases of double-orifice mitral valve. Our first case is associated with subaortic stenosis and coarctation of aorta. The second case is an isolated one resembling a severe mitral stenosis. As we know, the diagnosis of this rare anomaly by color-Doppler technique has not been previously reported. We believe this technique provides definite anatomical and functional information about double-orifice mitral valve.

In this paper, correlations between ischaemic heart disease and several cancers associated with cigarette smoking are examined. Also, tobacco consumption data are correlated against ischaemic heart disease mortality incidence rates. Furthermore, correlations between ischaemic heart disease incidences and per capita consumption of fattening foods (meat, milk and eggs) are analyzed. In this work, results referring to Spain, and at an ecologic level, confirm conclusions of observational and experimental studies carried out in other countries, which points out a strong association between ischaemic heart disease, cigarette consumption and fattening diet.