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Introduction and Objectives: Risk stratification of ventricular arrhythmias in patients with repaired tetralogy of Fallot (rTOF) remains unresolved. We aimed to identify right ventricular (RV) electrophysiological parameters potentially associated with a higher risk of ventricular arrhythmias in patients with rTOF., Methods: We included all consecutive patients with rTOF who underwent RV electroanatomical mapping at a single tertiary center. We used logistic regression modeling to identify those variables associated with an increased risk of clinical or induced ventricular tachycardia (VT), or clinical VT exclusively., Results: Twenty-one of the 56 patients included had clinical or induced VT. A high-frequency of premature ventricular contractions/nonsustained VT (OR, 11.34; 95%CI, 1.50-85.97; P=.019), an HV interval > 55 ms (OR, 21.20; 95%CI, 3.12-144.14; P=.002), and RV activation time (ms) (OR [per 10ms intervals], 1.34; 95%CI, 1.02-1.75; P=.035) proved to be associated with clinical or induced VT. The model including this information had good discrimination ability, with an area under the curve of 0.884 (95%CI, 0.79-0.97; P <.001). When considering only clinical VT as the outcome of interest, only an HV interval > 55ms (OR, 9.65; 95%CI, 1.41-66.14; P=.021) and high-frequency of premature ventricular contractions/nonsustained VT (OR, 13.14; 95%CI, 1.95-88.54; P=.008) were independently associated (area under the curve of 0.836 [95%CI, 0.663-1.000; P=.002])., Conclusions: High-frequency of premature ventricular contractions/nonsustained VT, an HV interval> 55ms and RV activation time are factors associated with an increased risk of ventricular arrhythmias in patients with rTOF., (Copyright © 2020 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

Aim: Intra-atrial re-entrant tachycardia (IART) is a common complication in patients with congenital heart disease (CHD) and is related to increased morbidity and mortality. Few reports have been published about factors associated to IART severity. The aim of this study is to analyse factors associated to severe clinical presentation of IART., Methods and Results: Observational study of all consecutive CHD patients who underwent a first IART ablation from January 2009 to December 2015 (94 patients, 39.4% female, and age: 36.55 ± 14.9 years). Severe clinical presentation was defined as heart failure, syncope, shock, electromechanical dissociation (EMD), or aborted sudden death. The majority of patients had moderately or highly complex cardiac defect (90.4%). Types of IART included cavotricuspid isthmus(CTI) dependent in 51% (48), non-CTI-related in 22.3% (20), and both types in 27.7% (26). In 38 patients (40.4%), a severe event occurred and in 16 (17%), the symptoms included shock, syncope, sudden death, or EMD. In 21 (22.3%), severe symptoms were the first manifestation of IART. In multivariate analysis, transposition of the great arteries (TGA) with right systemic ventricle (OR 5.32, 95% C.I. 1.6-7.02, P = 0.0005) and severe dilation of the venous atrium (VsA) (OR 4.17; 95% CI 1.4-8.12, P = 0.0009) were factors independently associated with severity., Conclusion: In our series of 94 CHD patients with a high proportion of moderately to highly complex cardiac defects, severe consequences of IART were frequent. Transposition of the great arteries with systemic right ventricle and severe dilation of VsA were independently associated to severity. Early invasive procedures should be considered for these high-risk patients.

Background: Intra-atrial re-entrant tachycardia (IART) is a frequent and severe complication in patients with congenital heart disease (CHD). Cavotricuspid isthmus (CTI)-related IART is the most frequent mechanism. However, due to fibrosis and surgical scars, non-CTI-related IART is also frequent., Objective: The main objective of this study was to describe the types of IART and circuit locations and to define a cut-off value for unhealthy tissue in the atria., Methods and Results: This observational study included all consecutive patients with CHD who underwent a first ablation procedure for IART from January 2009 to December 2015 (94 patients, 39.4% female, age: 36.55 ± 14.9 years, 40.4% with highly complex cardiac disease). During the study, 114 IARTs were ablated (1.21 ± 0.41 IARTs per patient). Cavotricuspid isthmus-related IART was the only arrhythmia in 51% (n = 48) of patients, non-CTI-related IART was the only mechanism in 27.7% (n = 26), and 21.3% of patients (n = 20) presented both types of IART. In cases of non-CTI-related IART, the most frequent location of IART isthmus was the lateral or posterolateral wall of the venous atria, and a voltage cut-off value for unhealthy tissue in the atria of 0.5 mV identified 95.4% of IART isthmus locations., Conclusion: In our population with a high proportion of complex CHD, CTI-related IART was the most frequent mechanism, although non-CTI-related IART was present in 49% of patients (alone or with concomitant CTI-related IART). A cut-off voltage of 0.5 mV could identify 95.4% of the substrates in non-CTI-related IART., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices., (Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

In the year 2012, 3 scientific sections-heart failure and transplant, congenital heart disease, and clinical cardiology-are presented together in the same article. The most relevant development in the area of heart failure and transplantation is the 2012 publication of the European guidelines for heart failure. These describe new possibilities for some drugs (eplerenone and ivabradine); expand the criteria for resynchronization, ventricular assist, and peritoneal dialysis; and cover possibilities of percutaneous repair of the mitral valve (MitraClip(®)). The survival of children with hypoplastic left heart syndrome in congenital heart diseases has improved significantly. Instructions for percutaneous techniques and devices have been revised and modified for the treatment of atrial septal defects, ostium secundum, and ventricular septal defects. Hybrid procedures for addressing structural congenital heart defects have become more widespread. In the area of clinical cardiology studies have demonstrated that percutaneous prosthesis implantation has lower mortality than surgical implantation. Use of the CHA2DS2-VASc criteria and of new anticoagulants (dabigatran, rivaroxaban and apixaban) is also recommended. In addition, the development of new sequencing techniques has enabled the analysis of multiple genes., (Copyright © 2012 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

This article contains a review of some of the most important publications on congenital heart disease and pediatric cardiology that appeared in 2010 and up until September 2011. Of particular interest were studies on demographic changes reported in this patient population and on the need to manage the patients' transition from the pediatric to the adult cardiology department. This transition has given rise to the appearance of new areas of interest: for example, pregnancy in women with congenital heart disease, and the effect of genetic factors on the etiology and transmission of particular anomalies. In addition, this review considers some publications on fetal cardiology from the perspective of early diagnosis and, if possible, treatment. There follows a discussion on new contributions to Eisenmenger's syndrome and arrhythmias, as well as on imaging techniques, interventional catheterization and heart transplantation. Finally, there is an overview of the new version of clinical practice guidelines on the management of adult patients with congenital heart disease and of recently published guidelines on pregnancy in women with heart disease, both produced by the European Society of Cardiology., (Copyright © 2012 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

Objectives: Assessment of the long term outcome of mechanical valve prosthesis at pulmonary position in a population of grown-up congenital heart disease patients from a tertiary referral center., Methods: From 1977 to 2007, 22 consecutive patients underwent a total of 25 pulmonary valve replacements with mechanical prosthesis. The most frequent underlying cardiac condition was tetralogy of Fallot (n=16, 64%) and the mean age at the time of pulmonary valve replacement was 32 ± 11 years (range 14-50 years)., Results: The postoperative mortality rate was 4% (n=1) with no late deaths documented after a mean follow-up of 7.6 ± 7.6 years (range 0.29-24 years). No major bleeding episodes occurred. Three patients presented with valve thrombosis in the setting of long term anticoagulation withdrawal and required valve re-replacement. Two of these patients, both with poor right ventricular function and overt clinical signs of right heart failure at the time of valve re-replacement, experienced further episodes of thrombosis despite correct anticoagulation. All episodes resolved with thrombolysis. After addition of antiplatelet treatment in one case and anticoagulation self-control, in the other, no further thrombosis has been documented., Conclusions: Mechanical valve prosthesis may be an alternative to tissue valve prosthesis in patients with congenital heart disease requiring pulmonary valve replacement. Optimal anticoagulation is crucial and additional antiplatelet treatment should be considered. Our data also suggest that patients with severe right ventricular dysfunction and congestive heart failure might be at particular risk for valve thrombosis., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Most of the data reported on sudden cardiac death has been from studies of Anglo-Saxon patients. We conducted a study to ascertain the relation between sudden death (SD) and some epidemiologic, clinical, and biochemical parameters and to assess the coronary histopathologic aspects of subjects in a Spanish population who had died suddenly. A total of 204 subjects (86% men), aged 12 to 80 years (mean 54 ± 15), who had died from out-of-hospital natural SD were evaluated. Only 15% of subjects had been previously diagnosed with heart disease. Pathologic evidence of underlying cardiovascular disease was found in 90% of cases, with coronary heart disease (CHD) the most frequent (58%). The CHD was acute coronary thrombosis in 41% and a stable plaque with luminal narrowing of ≥75% in 59%. An old myocardial infarction was found in 31% of the SD victims. Cardiac hypertrophy was found in 48%, with no relation between the presence of cardiac hypertrophy and CHD. Patients with stable plaques had a greater heart weight than did those with acute coronary thrombosis (p = 0.02). Male gender, older age, smoking, and low-density lipoprotein cholesterol/high-density lipoprotein cholesterol ratio of ≥3 were associated with CHD. A greater percentage of patients with an eroded and/or ruptured plaque than patients with a stable plaque were smokers. Only smoking and a low-density lipoprotein/high-density lipoprotein cholesterol ratio of ≥3 were associated with an eroded and/or ruptured plaque. In conclusion, compared with the findings from studies of Anglo-Saxon patients, a lower incidence of CHD and acute coronary thrombosis and a greater incidence of cardiac hypertrophy were found in SD victims of a Mediterranean Spanish population., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Cardiac magnetic resonance imaging (CMR) in hypertrophic cardiomyopathy (HCM) often shows delayed contrast enhancement (DE) representing regions of focal myocardial fibrosis. Atrial fibrillation (AF) is a commonly reported complication of HCM. We determined the relationship between the presence of left ventricular myocardial fibrosis (LVMF) detected by DE-CMR and the occurrence AF in a series of patients with HCM. 67 patients with HCM (47 males; mean age 50.1+/-18.5 years) were studied by CMR measuring mass of LVMF, left ventricular mass, volume and function, and left atrial (LA) area. AF was present in 17 (25%) patients. LVMF was observed in 57% of patients. AF was significantly more frequent in patients who also showed LVMF, compared with the group without LVMF (42.1% vs. 3.4%, respectively; p<0.0001). LA size was larger in patients showing DE (LA area: 37.4+/-11.1 vs. 25.9+/-6.8 cm(2); respectively, p=0.0001). AF in HCM is related with myocardial fibrosis detected by DE-CMR and dilatation of the LA. This fact adds to the proven adverse prognostic value of myocardial fibrosis in HCM, thus, reinforcing the usefulness of this technique in the assessment of these patients., (Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.)

Substantial progress in the diagnosis and treatment of patients with pulmonary hypertension in recent years has led to significant improvement in survival. Evidence-based clinical practice guidelines issued by scientific societies reflect these new developments. However, certain clinically relevant issues have not been covered in consensus guidelines because of the lack of conclusive scientific evidence. Therefore, the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC) have promoted the present consensus statement in order to define national standards of care in the evaluation and management of pulmonary hypertension in its various forms, as well as to outline a clinical pathway and the basic principles for organizing health care in this clinical setting, with special emphasis on the requirements for and functions of specialized referral units. To prepare the statement, SEPAR and SEC formed a task force composed of national experts in various aspects of pulmonary hypertension. The resulting consensus is based on international clinical guidelines, a review of available scientific evidence, and panel discussion among the task force members. The final statement, approved by all participants, underwent external review.

Consensus Statement of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC). Substantial progress in the diagnosis and treatment of patients with pulmonary hypertension in recent years has led to significant improvement in survival. Evidence-based clinical practice guidelines issued by scientific societies reflect these new developments. However, certain clinically relevant issues have not been covered in consensus guidelines because of the lack of conclusive scientific evidence. Therefore, the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC) have promoted the present consensus statement in order to define national standards of care in the evaluation and management of pulmonary hypertension in its various forms, as well as to outline a clinical pathway and the basic principles for organizing health care in this clinical setting, with special emphasis on the requirements for and functions of specialized referral units. To prepare the statement, SEPAR and SEC formed a task force composed of national experts in various aspects of pulmonary hypertension. The resulting consensus is based on international clinical guidelines, a review of available scientific evidence, and panel discussion among the task force members. The final statement, approved by all participants, underwent external review., (Copyright © 2008 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Baló's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures.

Introduction and Objectives: Severity of hypertrophic cardiomyopathy has been associated with the amount of myocardial fibrosis in autopsy studies. Cardio-vascular magnetic resonance allows, by means of the delayed contrast-enhancement technique, an in vivo detection of focal myocardial fibrosis. Our aim was to study myocardial fibrosis in patients with hypertrophic cardiomyopathy by means of contrast-enhance cardio-vascular magnetic resonance., Methods: 43 patients (30 males; mean age 47 [18] years) were studied by cardio-vascular magnetic resonance. In all patients left ventricular function and mass was analyzed. Total mass of myocardial fibrosis, as identified by delayed contrast-enhancement, was also calculated., Results: In 63% of patients some degree of myocardial delayed contrast-enhancement was observed, total mass of myocardial fibrosis ranging between 1 and 59 g (mean: 17 g). There was a positive correlation between the amount of myocardial fibrosis and the degree of hypertrophy. Maximal wall thickness was higher in patients with myocardial fibrosis (23 [7] vs 18 [4] mm, respectively, P=.04). Familial cases were also more prevalent among this group (48% vs 13%, respectively), as well as conventional clinical risk factors., Conclusions: Myocardial fibrosis as detected by contrast-enhanced cardio-vascular magnetic resonance is highly prevalent in hypertrophic cardiomyopathy patients, particularly in familial cases with severe hypertrophy and associated risk factors.

Closed chest trauma can cause rupture of intracardiac structures. We report the case of a 17-year-old boy whose chest was trodden by a horse. He sustained rupture of tricuspid and aortic valve leaflets and rupture of the interventricular septum. He underwent surgical repair of these lesions, but aortic insufficiency developed 2 years later and the aortic valve was replaced with a mechanical prosthesis.

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.

Background: Most patients with uncorrected coarctation of the aorta die before reaching age 50 years. In those who survive, the beneficial effect of surgical repair on systolic hypertension has been questioned., Methods: Surgical repair of aortic coarctation was performed in 8 patients aged 51 to 73 years (mean, 58+/-9 years). Preoperative mean systolic pressure was 185+/-34 mm Hg and systolic gradient, 70+/-11 mm Hg. In addition, 3 patients had significant coronary artery disease. Severe calcification of the aortic arch and left subclavian artery was found in 3 patients. The surgical technique involved bypass of the coarctation with a Dacron tube graft (16 or 18 mm) in all patients. One patient underwent concomitant coronary artery bypass grafting., Results: There were no operative or late deaths during a mean follow-up of 4.3 years. Mean systolic blood pressure decreased significantly in the postoperative period to 128+/-16 mm Hg (p < 0.001). At the last visit, systolic blood pressure was a mean of 127+/-9 mm Hg. Five patients were not taking antihypertensive medication., Conclusions: Surgical repair of aortic coarctation in patients more than 50 years of age with a Dacron tube bypass graft reduces systolic hypertension and the need of antihypertensive medication.

A 2-year-old patient with severe mitral regurgitation associated with a mass originating from the anterior leaflet of the mitral valve is reported. Excision of the tumor and mitral valve repair was performed. Four years later the child remains asymptomatic with no recurrence of the tumor or regurgitation.

The presence of liver methastasis and serotonin in plasma in the carcinoid tumor are responsible for the carcinoid syndrome. We present a case of tricuspid and pulmonary valvular disease secondary to this syndrome. The finding of liver hyperechogenic nodules added to the described valvular disease by subcostal echocardiogram oriented the diagnosis.

A child with a membranous obstruction of the inferior vena cava (MOIVC) is presented. The diagnosis was confirmed by angiography. The clinic significance, the evolution, the surgical treatment and the possible complications are described.

A 7 months-old child affected of West syndrome presented a cardiac hypertrophy with subaortic obstruction induced by adrenocorticotropic hormone treatment. The administration of this hormone may induce a Cushing syndrome; at high doses has chronotropic and inotropic positive effects and may produce arterial hypertension and cardiac hypertrophy. We pointed out the importance of the echocardiography-Doppler in the diagnosis and follow-up of the cardiac hypertrophy with subaortic obstruction as well as the reversibility of the cardiac involvement when the drug is suppressed.

A case of anomalous origin of the left coronary artery from the pulmonary artery (ALCA) is presented. The noninvasive diagnosis was made by 2D-color flow Doppler. Application of this technique may alleviate the necessity for angiography in patients with ALCA.

Recurrence is one of the major complications of pericarditis. Treatment of recurrence is often difficult, and immunosuppressive drugs or surgery may be necessary. We conducted an open-label prospective study of nine patients (seven men and two women; age, 18-64 years; mean age, 41.7 +/- 13.7 years). Patients were treated with colchicine (1 mg/day) to prevent recurrences. All patients had suffered at least three relapses despite treatment with acetylsalicylic acid, indomethacin, prednisone, or a combination. Pericarditis was classified as idiopathic in five patients, postpericardiotomy in two, post-myocardial infarction in one, and associated with disseminated lupus erythematosus in one. For statistical analysis, we conducted a paired comparison design (Student's t test). All patients treated with colchicine responded favorably to therapy. Prednisone was discontinued in all patients after 2-6 weeks (mean, 26.33 +/- 10.9 days), and colchicine alone was continued. After a mean follow-up of 24.3 months (minimum, 10 months; maximum, 54 months), no recurrences were observed in any patient; there was a significant difference between the symptom-free periods before and after treatment with colchicine (p less than 0.002). Our study suggests that colchicine may be useful in avoiding recurrence of pericarditis, although these results need to be confirmed in a larger, double-blind study.

A case of a 29 year old man with congenital aortic stenosis who developed a subvalvular annular aneurysm of the left ventricle after being run by a car is presented. At surgery his aortic valve was replaced and the opening of the aneurysm was closed with a patch of Dacron. Due to the dense adhesions to the aorta and left atrium no attempts were made to excise the sac, that was not compromising the dynamics of any one of these structures.

A patient is described with ventricular septal defects and two innominate veins which gave rise to an unusual cross-sectional echocardiographic finding thought to be due to a left superior caval vein without connexion to the coronary sinus.

We studied three groups of individuals by means of spatial-velocity electrocardiograms and thallium-201 myocardial imaging to figure out the electrophysiological explanation of the SI SII SIII electrocardiographic morphology. We studied twelve healthy individuals without SI SII SIII, seven healthy individuals with SI SII SIII and fifteen patients with chronic obstructive pulmonary disease with SI SII SIII. The average values of the QRS-E and QRS-F intervals were higher in the second (P less than 0.05 and P less than 0.005) and third groups (P less than 0.01 and P less than 0.001) than in the first. One patient of the second group and thirteen of the third showed right ventricular enlargement. The slowing down of the right ventricular conduction explained the SI SII SIII morphology in normal individuals in more than half the cases. In patients with chronic obstructive pulmonary disease with SI SII SIII the conduction delay plays an important part in the electrogenesis of the right ventricular enlargement electrocardiographic morphology. We think that these observations can give further data about the electrophysiologic mechanism of the SI SII SIII morphology.