Risk stratification of ventricular arrhythmias in repaired tetralogy of Fallot.

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.

Unusual case of new-onset heart failure due to cor triatriatum sinister.

We report the case of a 30-year old man who came to the emergency department of our hospital with acute left heart failure, and was diagnosed with a rare congenital anomaly (cor triatriatrum sinister), which can mimic a severe mitral stenosis. Cor triatriatum sinister is a rare anomaly (0.1% of all cases of congenital heart disease) that is seldom diagnosed in adult patients. The hallmark of this congenital defect is the presence of a fibromuscular membrane that divides the left atrium (LA) into two chambers: a postero-superior chamber into which the pulmonary veins drain and an infero-anterior chamber (true LA) containing the mitral valve and atrial appendage. Both chambers communicate through a membrane in which one or more drain holes can be found. When the hole is significantly obstructive, it results in increased venous and arterial pressures. Even though the definitive treatment of cor triatriatum is the surgical excision of the membrane, we present a balloon dilatation case with a good response to percutaneous therapy, both initially and in the ensuing months.

Gestación y anticoncepción / Pregnancy and Contraception

En la actualidad, un gran número de pacientes con cardiopatía congénita (CC) ha alcanzado la edad adulta, por lo que la problemática de la anticoncepción y los riesgos en caso de embarazo deberían abordarse en todas las jóvenes con estas enfermedades y ofrecerles, en el momento adecuado (posiblemente al inicio de la adolescencia), consejo preconcepcional con el objetivo de prevenir riesgos evitables y ayudarlas en la planificación de su futuro. Para ello, a la vez que para lograr un cuidado adecuado del embarazo, el feto, el parto y el posparto, es importante que un equipo multidisciplinario, constituido por cardiólogos, obstetras, ginecólogos, anestesiólogos, neonatólogos y expertos en medicina fetal, elabore y ponga en práctica unos protocolos de actuación consensuados (AU)

Since today a substantial number of patients with congenital heart disease (CHD) will have reached adulthood, it is important that the question of contraception and the risks associated with pregnancy are discussed with young women with the disease. At the appropriate time (possibly at the start of adolescence), these young women should be offered preconception counseling to help them avoid preventable risks and plan their future. With this aim in mind and to ensure that the appropriate level of care is provided for mothers during pregnancy, delivery and the puerperium and for the fetus and newborn child, it is essential that a multidisciplinary team of cardiologists, obstetricians, gynecologists, anesthesiologists, neonatologists and experts in fetal medicine develop and implement consensual clinical practice guidelines (AU)

[Standards of care in pulmonary hypertension. Consensus Statement of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC)]. / Estándares asistenciales en hipertensión pulmonar. Documento de consenso elaborado por la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) y la Sociedad Española de Cardiología (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.

Estándares asistenciales en hipertensión pulmonar*. Documento de consenso elaborado por la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) y la Sociedad Española de Cardiología (SEC) / Standards of care in pulmonary hypertension

En los últimos años se han producido importantes avances en el diagnóstico y tratamiento de la hipertensión pulmonar que han logrado una mejoría significativa en la supervivencia de esta enfermedad. Estas innovaciones se han recogido en guías de práctica clínica basadas en la evidencia elaboradas por las sociedades científicas. Sin embargo, no se incluyen en ellas, por falta de evidencia científica concluyente, algunos aspectos que inciden en la práctica asistencial. Conscientes de ello, la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) y la Sociedad Española de Cardiología (SEC) han promovido la elaboración de un documento de consenso para definir en nuestro medio los estándares de calidad adecuados para el diagnóstico y tratamiento de la hipertensión pulmonar en sus diversas formas de presentación, así como la vía clínica y las directrices básicas de la organización asistencial del cuidado de estos pacientes, haciendo especial hincapié en los requisitos y funciones de las unidades de referencia. Para su redacción la SEPAR y la SEC designaron a un grupo de trabajo formado por expertos en los distintos aspectos de la enfermedad. Para la elaboración del documento se han utilizado las guías clínicas internacionales existentes, la revisión de la evidencia científica disponible y el debate en panel entre los expertos. El documento final, aprobado por todos los participantes, ha sido evaluado por revisores externos

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 (AU)

[Detection and quantification of myocardial fibrosis in hypertrophic cardiomyopathy by contrast-enhanced cardiovascular magnetic resonance]. / Detección y cuantificación de la fibrosis miocárdica en la miocardiopatía hipertrófica mediante cardiorresonancia magnética con contraste.

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.

Detección y cuantificación de la fibrosis miocárdica en la miocardiopatía hipertrófica mediante cardiorresonancia magnética con contraste / Detection and quantification of myocardial fibrosis in hypertrophic cardiomyopathy by contrast-enhanced cardiovascular magnetic resonance

Introducción y objetivos. Estudios necrópsicos han asociado la severidad de la miocardiopatía hipertrófica con la cantidad de fibrosis miocárdica. La cardiorresonancia magnética con contraste permite, mediante la secuencia de realce tardío, la detección in vivo de la fibrosis miocárdica focal. El objetivo del presente estudio fue cuantificar la fibrosis miocárdica mediante el realce tardío en pacientes con miocardiopatía hipertrófica. Métodos. Se practicó un estudio de cardiorresonancia magnética con contraste en 43 pacientes (30 varones; edad media 47 ± 18 años) con miocardiopatía hipertrófica. En todos ellos se realizaron secuencias de cine-resonancia para el estudio de la función ventricular izquierda y la masa ventricular izquierda. Además, se calculó la masa total de fibrosis miocárdica mediante la identificación del realce tardío a los 10 min de la administración de contraste paramagnético. Resultados. En el 63% de los pacientes se observó realce tardío con una masa total de fibrosis miocárdica media de 17 g (intervalo, 1-59 g). Se evidenció una correlación positiva entre la cantidad de fibrosis miocárdica y el grado de hipertrofia. El grosor parietal máximo era superior en pacientes con fibrosis miocárdica (23 ± 7 frente a 18 ± 4 mm, respectivamente; p = 0,04). Los casos familares también eran más prevalentes en este grupo (el 48 frente al 13%, respectivamente), así como la presencia de factores de riesgo convencionales. Conclusiones. La fibrosis miocárdica detectada por cardiorresonancia magnética es altamente prevalente en pacientes con miocardiopatía hipertrófica, en particular en casos familiares con severa hipertrofia y factores de riesgo asociados

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

Guías de práctica clínica de la Sociedad Española de Cardiología en pruebas de esfuerzo / Guidelines of the Spanish Society of Cardiology for clinical practice in exercise testing

La mayor parte de las pruebas de esfuerzo se realizan a pacientes adultos con cardiopatía isquémica en estudio o ya conocida. En los últimos años se ha producido la incorporación de las técnicas de imagen en este campo, mejorando así la información aportada por la prueba de esfuerzo convencional. Pero cada vez existen más situaciones que escapan a esta norma general, tanto en sujetos sanos (asintomáticos, atletas, discapacitados, etc.) como en pacientes con cardiopatías diferentes de la isquémica (insuficiencia cardíaca congestiva avanzada, hipertensión, trastornos del ritmo, cardiopatías congénitas etc.). Todos estos aspectos justifican un documento de consenso en España, necesariamente multidisciplinario. Este documento revisa en profundidad la metodología de las pruebas de esfuerzo convencionales, sin olvidar las realizadas con determinación de consumo de oxígeno. El papel de esta exploración en el manejo de la cardiopatía isquémica, así como las aplicaciones de las técnicas de imagen al campo de estrés, ocupan un lugar fundamental en esta revisión. Por último, se analiza la utilidad de las pruebas de esfuerzo en diversas cardiopatías no isquémicas y en diferentes poblaciones de sujetos sanos (AU)