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.

[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.

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.

[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.