José A. Sobrino

Mechanisms of Sustained Ventricular Tachycardia in Myotonic Dystrophy Implications for Catheter Ablation

BACKGROUND Ventricular arrhythmias have been documented and linked to the high incidence of sudden death seen in patients with myotonic dystrophy. However, their precise mechanism is unknown, and their definitive therapy remains to be established. METHODS AND RESULTS We studied 6 consecutive patients with myotonic dystrophy and sustained ventricular tachycardia by means of cardiac electrophysiological testing. Particular attention was paid to establish whether bundle-branch reentry was the tachycardia mechanism, and when such was the case, radiofrequency catheter ablation of either the right or left bundle branch was performed. Clinical tachycardia was inducible in all patients and had a bundle-branch reentrant mechanism. In 1 patient, 2 other morphologies of sustained tachycardia were also inducible, neither of which had ever been clinically documented, and both had a bundle-branch reentrant mechanism. Ventricular tachycardia was no longer inducible after bundle-branch ablation, except for a nonclinically documented and nonsustained ventricular tachycardia in the only patient who had apparent structural heart disease. CONCLUSIONS A high clinical suspicion of bundle-branch reentrant tachycardia is justified in patients with myotonic dystrophy who exhibit wide QRS complex tachycardia or tachycardia-related symptoms. Because catheter ablation will easily and effectively abolish bundle-branch reentrant tachycardia, myotonic dystrophy should always be considered in patients with sustained ventricular tachycardia. This is especially true if no apparent heart disease is found.

Pheochromocytoma-Related Cardiomyopathy Inverted Takotsubo Contractile Pattern

A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …

Images in cardiovascular medicine. Pheochromocytoma-related cardiomyopathy: inverted Takotsubo contractile pattern.

A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …

Bundle-Branch Reentry and the Postpacing Interval After Entrainment by Right Ventricular Apex Stimulation: A New Approach to Elucidate the Mechanism of Wide-QRS-Complex Tachycardia With Atrioventricular Dissociation

Background —Diagnosis of bundle-branch reentry ventricular tachycardia (BBR-VT) by the standard approach is challenging, and this may lead to nonrecognition of this tachycardia mechanism. Because the postpacing interval (PPI) after entrainment has been correlated with the distance from the pacing site to the reentrant circuit, BBR-VT entrainment by pacing from the right ventricular apex (RVA) should result in a PPI similar to the tachycardia cycle length (TCL). This factor may differentiate BBR-VT from other mechanisms of wide-QRS-complex tachycardia with AV dissociation, such as myocardial reentrant VT (MR-VT) or AV nodal reentrant tachycardia (AVNRT), in which the circuit is usually located away from the RVA. Methods and Results —Transient entrainment by RVA pacing was attempted in 18 consecutive BBR-VTs and finally achieved in 13. Results were compared with those found in 59 consecutive MR-VTs and 50 consecutive AVNRTs. The mean PPI−TCL difference was significantly (P <0.0001) shorter in the BBR-VT group (9±11 ms) than in the MR-VT (109±48 ms) and the AVNRT (150±29 ms) groups. No BBR-VT showed a PPI−TCL >30 ms (range −12 to 24 ms). Except for 2 MR-VTs, no MR-VT (range 21 to 211 ms) or AVNRT (range 100 to 215 ms) showed a PPI−TCL <30 ms. Conclusions —A PPI−TCL >30 ms, after entrainment by RVA stimulation, makes BBR-VT unlikely. Conversely, a PPI−TCL <30 ms is suggestive of BBR-VT but should lead to further investigation by use of conventional criteria.

Predisposing conditions for atrial fibrillation in atrial septal defect with and without operative closure

The aims of this study were to determine the prevalence and predisposing conditions for atrial fibrillation (AF) in adults with atrial septal defect (ASD) and to evaluate the influence of age at surgical repair. The study population consisted of 286 adults with ASD (mean age 39.5 +/- 19 years). All patients had >or = 1 follow-up visit and a Doppler echocardiographic study. One hundred ninety-two of the patients underwent surgical closure 1 to 34 years before the study. Analyzed variables were entered into univariate (Mann-Whitney U) and multivariate (stepwise logistic regression) models to assess independent predictors for AF. The prevalence of AF was similar in surgically treated patients (15.6%) and in the nonsurgical group (13.8%) (p = 0.69). Multivariate analysis showed that current age (RR 1.9 per each decade of age, 95% confidence interval [CI] 1.3 to 2.7, p = 0.001), mitral regurgitation (RR 3.0 per each degree of regurgitation, 95% CI 1.6 to 5.8, p = 0.001), left atrial enlargement (RR 2.8 per each 10 mm increase in size, 95% CI 1.5 to 5.2, p = 0.001), and tricuspid regurgitation (RR 1.9 per each degree of regurgitation, 95% CI 1.0 to 3.7, p = 0.04) were independent predictors of AF; however, gender, anatomic type, defect size, Qp:Qs, pulmonary artery pressure, right ventricular dimension, left ventricular shortening fraction, and prior surgical repair were not related to late AF development. In the surgical group, age >25 years at the time of surgery was the only predictor for AF independent of age at the time of the study (p = 0.02).

Aspergillus Aortitis After Cardiac Surgery

OBJECTIVES The aim of this study was to describe the clinical characteristics of Aspergillus aortitis in a small series of consecutive patients. BACKGROUND Aspergillus infection of the ascending aorta after cardiopulmonary bypass surgery has rarely been reported and has always resulted in death. METHODS Aspergillus aortitis was confirmed by pathologic and microbiologic analysis in eight men (61 +/- 8 years) of 9,375 consecutive patients who underwent cardiac surgery between 1975 and 2000. RESULTS Patients presented with Aspergillus aortitis after aortic valve replacement (n = 5), coronary revascularization (n = 2), or both (n = 1). Initial symptoms appeared between the immediate postoperative period and up to two years after surgery. All patients had prolonged fever. Ante-mortem diagnosis was established in only three patients for whom transthoracic echocardiography was suggestive of aortic pseudoaneurysm and was confirmed by thoracic computed tomography or aortography. All patients had negative peripheral blood cultures. Seven patients died at short-term follow-up, and the one surviving patient was promptly treated by surgery and antifungal drugs. Pathologic examination confirmed Aspergillus aortitis with multi-organ dissemination without heart involvement in all patients except for two, in whom aortic valve endocarditis was found. Fungal cultures confirmed the presence of Aspergillus fumigatus in all patients. CONCLUSIONS Aspergillus aortitis is typically found after aortic valve or coronary surgery. It commonly leads to lethal multi-organ dissemination without involvement of the intracardiac structure. This entity should be considered in patients with persistent fever and negative blood cultures after open-heart surgery involving significant aortic wall damage, irrespective of the postoperative period.Objectives The aim of this study was to describe the clinical characteristics of Aspergillusaortitis in a small series of consecutive patients.

Initial outcome of percutaneous balloon valvuloplasty in rheumatic tricuspid valve stenosis

Abstract Percutaneous balloon valvuloplasty (PBV) has been used as an effective treatment for some cases of valvular stenosis, mainly in the mitral, 1 pulmonary 2 and aortic 3 valves. Less experience has been achieved with this procedure for valvular tricuspid stenosis. 4–7 We present the immediate results of 5 cases of PBV in stenotic tricuspid valves.

Transient Entrainment of Bundle-Branch Reentry by Atrial and Ventricular Stimulation Elucidation of the Tachycardia Mechanism Through Analysis of the Surface ECG

BACKGROUND Different responses to entrainment have been reported in relation to the pacing site of a variety of tachycardias. However, transient entrainment of bundle-branch reentrant tachycardia (BBRT) has not been investigated systematically. METHODS AND RESULTS We attempted entrainment of 13 BBRTs in 9 patients by pacing first the right ventricle and then the right atrium. The initial pacing cycle length (CL) was 10 ms faster than the tachycardia CL. Subsequent pacing sequences were performed with 5- to 10-ms CL decrements until tachycardia termination or loss of postatropine 1:1 AV conduction. Both full ventricular-paced and AV-conducted QRS complex references were obtained during sinus rhythm pacing from the same sites and with similar CL as during entrainment. Transient entrainment was achieved by ventricular and atrial stimulation in 11 and 8 tachycardias, respectively. Constant fusion was always present during entrainment by ventricular stimulation. There was no change in the QRS complex (orthodromically concealed fusion) during entrainment by atrial stimulation in 6 of 6 tachycardias with left bundle-branch block morphology and in 1 of 2 tachycardias with right bundle-branch block morphology. CONCLUSIONS BBRT, especially if it has a left bundle-branch block morphology, can be differentiated from other wide-QRS-complex tachycardia mechanisms through analysis of the ECGs recorded during tachycardia entrainment by atrial and ventricular stimulation. This diagnostic approach may be especially useful when it is difficult to record a stable or sufficiently sized His bundle electrogram or when spontaneous changes in the ventricular CL precede similar changes in the His bundle CL.

Myocardial Involvement in Systemic Lupus Erythematosus: A Noninvasive Study of Left Ventricular Function

A relatively high incidence of heart failure is noted among patients with systemic lupus erythematosus (SLE) without clearly defined clinical causes. To evaluate left ventricular performance in patients with SLE without evidence of cardiovascular disease, noninvasive measurement of the systolic time intervals was carried out. Simultaneous recording of the electrocardiogram, phonocardiogram and carotid arterial pulsation were obtained in 25 patients with systemic lupus erythematosus and compared with 22 normal subjects. The patients with SLE had a shorter left ventricular ejection time (P less than 0.05), a longer pre-ejection period (P less than 0.02) and an increased ratio of pre-ejection period/left ventricular ejection time (P less than 0.005). These abnormalities on ventricular function were independent of age, duration of the disease, hypertension, renal involvement, anemia, immunologic activity and corticosteroid treatment. Several etiologic possibilities are discussed and the clinical usefulness of this method to detect and follow-up the cardiac dysfunction in systemic lupus erythematosus is emphasized.

Endocarditis por Coxiella burnetii: evolución a largo plazo de 20 pacientes

Introduccion y objetivos Coxiella burnetii es una causa cada vez mas frecuente de endocarditis infecciosa de evolucion subaguda asociada a una elevada morbimortalidad. Nuestro objetivo fue analizar, en una serie de 20 pacientes, la evolucion clinica, serologica y terapeutica a largo plazo. Metodos Se estudiaron retrospectivamente 20 pacientes ingresados (13 varones y 7 mujeres con una edad media de 42 ± 10 anos) entre 1982 y 1996, que cumplian criterios de Duke modificados por Raoult para endocarditis por fiebre Q. Resultados La endocarditis asento sobre protesis valvular en 14 pacientes y sobre valvula nativa en 6. Todos excepto uno recibieron tratamiento antibiotico, presentando la doxiciclina en monoterapia peor resultado que combinada con otros farmacos. Fueron sometidos a recambio valvular 15 pacientes, siendo la causa mas frecuente la disfuncion protesica. La mortalidad global fue del 40% (8 pacientes). Actualmente, todos los pacientes mantienen valores de anticuerpos antifase I elevados tras un seguimiento entre 19 y 156 meses (media de 74 ± 47 meses). En 5 pacientes se suspendio el tratamiento antibiotico, dada la negatividad microbiologica valvular, permaneciendo asintomaticos tras 15-65 meses (32 ± 30) de seguimiento. Conclusiones La endocarditis por fiebre Q se asocia a un alto indice de complicaciones severas que requieren cirugia de sustitucion valvular. Todos los pacientes mantienen titulos serologicos elevados de forma cronica, sin otros datos de infeccion activa, lo que plantea la posibilidad de retirar el tratamiento en algun enfermo con negatividad valvular microbiologica y cuestiona el valor de la persistencia de una serologia anormal como monitorizacion del tratamiento.