Keratoacanthoma-like nodules as first manifestation of metastatic epithelioid trophoblastic tumor.

Cutaneous metastases are rarely the initial manifestation of a previously undiagnosed malignancy and keratoacanthoma-like lesions are a notoriously unusual presentation pattern of cutaneous dissemination of a primary tumor. Herein, we report a 40-year-old woman presenting to our dermatology department with multiple keratoacanthoma-like scalp nodules. Subsequent investigation determined it to be the first manifestation of a disseminated endometrial epithelioid trophoblastic tumor, eventually causing the patient's death. Epithelioid trophoblastic tumor, a rare form of gestational trophoblastic disease, is a recently described neoplasm whose cutaneous metastasis has not been previously reported in the literature.

Biventricular pacing--early experience.

INTRODUCTION: Biventricular pacing has been studied for the treatment of chronic heart failure (CHF). This technique seems to be able to improve symptoms and exercise tolerance, in patients with advanced CHF and prolonged QRS duration. OBJECTIVE: To present our experience with biventricular pacing in the management of severe CHF. METHODS: Between June 2000 and March 2001, 8 patients with dilated cardiomyopathy and left bundle branch block (LBBB) were selected for transvenous biventricular pacing system implantation. Mean age: 54.12 +/- 16.8 years; 5 males. The etiology was: idiopathic in 6 cases; operated congenital heart disease in 1, and ischemic in the other. Despite tailored treatment of CHF (with all patients taking diuretics and angiotensin-converting enzyme inhibitors), 7 patients remained in NYHA class III and 1 in IV. The quality of life score (assessed by the "Minnesota living with heart failure questionnaire") was 62.25 +/- 11.29. Seven patients had sinus rhythm and only one chronic atrial fibrillation; mean PQ duration--220 +/- 76.37 ms; mean QRS duration--168.75 +/- 20.31 ms. RESULTS: Implant failure, due to coronary sinus dissection and to excessive fluoroscopy time, with no coronary sinus catheterization, occurred in 2 cases (success rate: 75%). Implant data: mean implant procedure duration: 122.5 +/- 47.82 min; mean fluoroscopy time: 35.66 +/- 22.06 min; QRS duration, after implant: 133.33 +/- 15.05 ms. Left ventricular lead final position: anterolateral in 2 patients and lateral in 4. Pacing thresholds: biventricular--1.36 +/- 0.6 V; right ventricle--0.28 +/- 0.04 V; right atrium--0.32 +/- 0.08 V. Pacing impedance (left ventricle): 1013.33 +/- 147.87 omega. Follow-up (1st and 3rd month): one patient died, suddenly, 15 days after the procedure. In the others, an improvement in the quality of life index and functional class was found. These results were independent of echocardiography data. There were no significant differences in the pacing threshold and impedance during the follow-up. CONCLUSIONS: Patients with advanced CHF and widened QRS benefited from biventricular stimulation, in which improvement on the clinical status was evident.

Repolarization abnormalities after catheter ablation of differently located overt accessory pathways.

PURPOSE: Electrical repolarization abnormalities are usually seen after radiofrequency catheter ablation in overt accessory atrio-ventricular pathways. These abnormalities have been recognised as "cardiac memory". The aim of this study was to assess the relationships between electrical repolarization abnormalities and the location of the accessory atrioventricular pathway in the atrio-ventricular junction. We also assessed the relationship between electrical repolarization abnormalities and the degree of ventricular preexcitation. MATERIAL AND METHODS: Our group consisted of 45 patients with overt and persistent preexcitation who were successfully submitted to radiofrequency catheter ablation. Mean age was 38.9 +/- 14.9 years (14 to 64), 28 of whom (62.2%) were male and 17 (37.8%) female. The location of accessory pathways the was left lateral in 14 patients, right posteroseptal in 14 patients, right mesoseptal in eight patients, right anteroseptal in four patients and other locations in the remaining four. Surface electrocardiograms were performed immediately following ablation, 24 hours later and three or more months after the procedure. RESULTS: T wave abnormalities (flattened, inverted or peaked) were detected on the first day in 51% of patients, being more frequent after right accessory pathway ablation (86.9% versus 13.1% in left accessory pathways). These electrocardiographic changes were particularly frequent in patients with right mesoseptal (5/8, 62.5%) and posteroseptal (13/14, 93.3%) pathways, of which 62.5% and 71.5%, respectively, showed marked preexcitation before ablation. These findings were significantly different from those observed in patients with left lateral pathways--only 35.7% showed marked preexcitation and only two of fourteen patients (14.3%) presented T wave abnormalities after ablation. After the third month, most of the patients that had undergone successful ablation presented completed electrocardiographic normalization. CONCLUSIONS: Patients undergoing radiofrequency catheter ablation for right accessory pathways show more frequently repolarization abnormalities than those submitted to ablation of the left lateral accessory pathways. The occurrence of these changes after preexcitation resolution may depend on the degree of previous preexcitation.

Permanent pacing in patients with congenital atrio-ventricular block--experience of 33 cases.

OBJECTIVES: The authors review permanent pacing in patients with congenital atrioventricular block (CAVB) and present their experience in permanent pacing in this pathology. STUDY POPULATION AND METHODS: In a population of 4,355 patients submitted to implantation of permanent pacing between January 1980 and January 1998, 33 (0.75%) had CAVB. The mean age of the patient population with CAVB was 16.7 years (aged from eleven days to 35 years); 33% were below 10 years of age; 16 patients were male. The majority of the patients had symptoms of brain hypoperfusion; two patients had concomitant malignant ventricular tachyarrythmias (one of these with Torsade de Pointes due to congenital long QT syndrome). Transvenous (endocardial) pacing was used in 32 patients (two with previous epicardial pacing and exit block) and epicardial pacing in one. The mode of stimulation used was VVI in three patients, DDD in eight patients, VVIR in 14 patients, DDDR in four patients and VDD in four. Smaller pulse generators were used in children of lower weight. In recent years single lead VDD systems have been preferred whenever technically possible. Vascular access was the left cephalic vein in 17 patients; the left subclavian vein in 14 patients and the right jugular vein in one patient. During a mean follow-up of 6.9 years, two patients with ventricular stimulation systems developed "Pacemaker Syndrome" and required a change of mode of stimulation. Lead fracture and posterior cutaneous necroses were observed in two other patients, who were accordingly submitted to surgical revision. It was deemed necessary, one year later, to increase the lead loop in a child with a permanent pacemaker implanted at eleven days of age. No other complications occurred with the other patients; replacement of the pulse generators was performed in an elective manner. CONCLUSIONS: CAVB is a rare indication for the implantation of a permanent pacemaker. In children, in the majority of cases, endocardial stimulation is possible in spite of the obvious technical difficulties due to low weight. Sequential, more physiological, stimulation systems should be preferred. However, VVIR stimulation systems of smaller dimensions can be the first choice of mode of stimulation in smaller children, mainly due to anatomical and technical limitations.

[Effect of programming different AV intervals on mitral insufficiency in patients with DDD pacemaker]. / Influência da programação de diferentes intervalos AV na insuficiência mitral em doentes com pacemaker em modo DDD.

OBJECTIVE: To study the effect of programming three different atrioventricular intervals (AVI) on mitral insufficiency (MI) in patients with permanent pacemaker due to complete atrioventricular block. METHODS: We included 9 patients (mean age 62 +/- 11 years; 6 men), with permanent DDD pacemaker, isolated MI and normal ventricular function. We evaluated MI (in a quantitative way) and cardiac output (CO) through echocardiographic examination with three different AVI (100, 150 and 200 ms). RESULTS: We observed a reduction in the severity of MI with the reduction of the AVI (best results obtained with AVI of 100 ms). We also observed an increase in the CO in 4 patients (those with MI of greater degree) with AVI of 100 ms. CONCLUSIONS: We observed a correlation of longer AVI with greater degree of MI; our results point to the possibility of increasing the CO of patients with greater degrees of MI with shortening of the AVI (probably due to a partial recovery of the regurgitant volume).

Haemodynamics during liver transplantation in familial amyloidotic polyneuropathy: study of the intraoperative cardiocirculatory data of 50 patients.

BACKGROUND: Circulatory instability frequently complicates liver transplantation for familial amyloidotic polyneuropathy (FAP) and may be a source of surgical morbidity and mortality. OBJECTIVE: To evaluate FAP intraoperative haemodynamic data and their relation to the duration of surgery, and need for anaesthetic drugs. RBC and sympathomimetic amines. SETTING: Clinical study during a four year period. PATIENTS (mean +/- SD): Group I included 50 consecutive FAP ATTR Met 30 recipients of first transplantation. Age was 35.3 +/- 7.1 years, neurological score 34.3 +/- 13 in 100 and time elapsed from first symptom 5.0 +/- 2.7 years. Group II (control), not different concerning age and sex, included 51 patients transplanted during the same period with other pathologies. METHOD: Anaesthetic protocol, monitoring and surgical techniques were similar in both groups. Data of the two groups were compared either by the Student's t-test or Fisher's exact test. RESULTS: Low values of systemic vascular resistance index were observed in both groups, with no differences between them. Systemic arterial pressures were usually lower in group I, because cardiac index and heart rate were also significantly lower, although within normal values. However, in group I, isoflurane (a vasodilator anaesthetic) was used during less time (p < 0.05) and in lower concentrations (p < 0.01) and phenylephrine was necessary in 26% of patients vs 0 patients in group II (p < 0.001). CONCLUSION: FAP patients presented a different intraoperative behaviour when compared to other patients submitted to liver transplantation. From a clinical point of view, the authors stress: 1--As a result of autonomic dysfunction, the administration of anaesthetic drugs to FAP patients always presents the risk of producing significant hypotension; even the use of ketamine does not prevent hypotension; 2--Safety is ensured by beat-to-beat surveillance of arterial pressures and the capacity to act immediately to support circulation; 3--These patients seem to be very sensitive to decreases in the pre-load; 4--Hypotension is also frequent with an adequate pre-load, usually as the result of low SVR; an infusion of a vasoconstrictor drug emerges as the most frequent treatment requested and our experience supports it as an effective one.

[The implantable cardioverter-defibrillator: the Portuguese experience]. / Cardioversor-desfibrilhador implantável: experiência portuguesa.

OBJECTIVE: The aim of this study was to review the Portuguese experience with implantable cardioverter-defibrillator therapy (ICD), in order to evaluate the increase in the number of ICD implanted, the main indications for this kind of therapy, the technical evolution of the procedure and the results of the follow-up of these patients during the last five years. PATIENT SELECTION: The study group consists of 58 patients, 53 male and 5 female, mean age 54 +/- 14 years with ICD implanted in our country since 1992. The ICDs were implanted in 4 Hospitals, namely, Santa Cruz Hospital with 36 patients, Santa Maria Hospital with 11, Santa Marta Hospital with 8 and Coimbra University Hospital with 3 patients. Twenty six patients were resuscitated from cardiac arrest and the other 32 had ventricular tachycardia (VT) not tolerated haemodynamically and refractory to therapy. The diagnosis was coronary artery disease in 31 patients, dilated cardiomyopathy in 8, valvular disease in 4, congenital cardiopathy in 3, right ventricular dysplasia in 2, congenital long QT syndrome in 1, hypertrophic cardiomyopathy in one. Seven patients had idiopathic ventricular fibrillation without structural heart disease and one patient had isolated right ventricular dilatation. METHODS: All patients underwent electrophysiological study before ICD implantation. In 2 patients epicardial leads were used and in the remaining 56 patients a transvenous approach was used. The device was implanted in an abdominal position in 36 patients and in a pectoral position in 22. Defibrillation and pacing thresholds were measured during the implantation procedure and whenever necessary. Patients were followed up on an outpatient basis with evaluation of the number of arrhythmic episodes, therapy efficacy with reprogramming of the device when required. RESULTS: The number of implantations has increased, from 4 devices implanted in 1992, to 32 in 1996. The implantation was successful and without mortality or complications in all patients. Defibrillation threshold was 16 +/- 3 J, with an electrode impedance of 48 +/- 9 Ohms. During a mean follow-up time of 18 +/- 15 months (1 to 56) 5 patients died, one of sudden death and 4 of non cardiac deaths, 15 patients were re-admitted to hospital and in 8 patients the device was replaced due to exhaustion. In this period, 37 patients (64%) had arrhythmic episodes detected by the device, 32 patients (55%) had shocks and 9 (16%) were treated with anti-tachycardia pacing. Inappropriate shocks were observed in 10 patients (17%). The ICD were reprogrammed in 11 patients. CONCLUSIONS: Portuguese experience with IC implantation is increasing and has shown to be a safe procedure with no operative mortality or morbidity. The incidence of appropriate shocks was high with a good efficacy in sudden death reduction.

[Classical pacemaker syndrome. Report of 2 cases with severe clinical manifestations]. / Síndroma do portador de pacemaker clássico. A propósito de dois casos com manifestações clínicas aparatosas.

We report two cases of classic pacemaker syndrome. Both patients developed severe clinical manifestations after implantation of a VVI pacemaker. One patient presented syncopal episodes and the other one manifest cardiac heart failure. Additionally, we review the mechanisms responsible for this clinical entity.

[Auricular fibrillation. Antiarrhythmic treatment revisited]. / Fibrilhação auricular. Tratamento antiarrítmico revisitado.

Atrial fibrillation is one of the most common symptomatic sustained arrhythmias seen in clinical practice. Long-term control of heart rate and maintenance of sinus rhythm often require chronic antiarrhythmic therapy. For patients with disabling symptoms of atrial fibrillation that cannot be controlled with pharmacologic therapy, catheter ablation techniques of the atrioventricular junction and surgical procedures aimed at maintaining sinus rhythm have now been effectively used. The efficacy, risks and limitations of pharmacologic and non-pharmacologic therapies are presented in this review article.

Diagnosis and treatment of tachycardias with a long BP interval.

The differential diagnosis of tachycardias with a long RP interval is reviewed and the methods of treatment of these arrhythmias catheter ablation are described. Electrocardiographic and electrophysiologic criteria for the correct diagnosis of atrial tachycardia, circus movement tachycardia using retrogradely an accessory pathway with decremental conduction properties and the uncommon form of atrioventricular nodal reentrant tachycardia are discussed. First results of our institution of radiofrequency catheter ablation of atrial tachycardias and circus movement tachycardias using retrogradely an accessory pathway with decremental conduction properties are presented. We concluded that both electrocardiographic and electrophysiologic criteria give a better understanding of the mechanism and arrhythmic site which are important markers for a safe and successful ablation procedure.

Radiofrequency ablation of atrioventricular node reentrant tachycardia: experience in 302 patients.

Radiofrequency catheter ablation was performed in 302 consecutive patients with drug refractory atrioventricular (AV) node reentrant tachycardia. Fast pathway ablation was attempted in 167 patients and was successful in 161 patients (96.4%). At a mean follow-up of 24 +/- 12 months, there were 21 tachycardia recurrences (12.5%). A second fast pathway ablation was attempted in 17 patients and was successful in all but 1 patient. Permanent complete AV block occurred in 12 patients (7.2%). Among the latter, late AV block was noted in 5 patients. Final success without pacemaker implantation was accomplished in 151 patients (90.4%). Slow pathway was attempted in 135 patients and was successful in 130 patients (96.3%). Three patients in whom slow pathway ablation failed underwent successful fast pathway ablation during the same session. At a mean follow-up of 14 +/- 11 months, there were 16 tachycardia recurrences (11.8%). A second slow pathway ablation was attempted in 16 patients and was successful in all but 1 patient. Permanent complete AV block occurred in 3 patients (2.2%). An additional patient developed 2 : 1 AV block during exercise, 3 months after ablation. Final success without pacemaker implantation was achieved in 129 patients (95.5%). Fast and slow pathway ablation had similar success and recurrence rates, procedure and fluoroscopy times, and number of radiofrequency pulses. However, the incidence of permanent complete AV block was higher following fast pathway ablation (p = 0.049). Although equally effective, slow pathway ablation is safer than fast pathway ablation, therefore, should be the first choice approach for treatment of AV node reentrant tachycardia.

Atrioventricular nodal reentrant tachycardia: a review.

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) is the most common form of regular narrow complex tachycardia. It is due to dual atrioventricular nodal conduction over two pathways with different electrophysiological properties. The first pathway ('fast' pathway) conducts faster but has longer refractory period than the second pathway ('slow' pathway). OBJECTIVES: To review AVNRT. Clinically, AVNRT patients usually have palpitations in their neck during attacks. On the surface electrocardiogram, the diagnosis is suggested by the absence of P waves during tachycardia or very discrete P waves immediately after the QRS or an rSr' pattern in lead VI. Electrophysiologically, it can be reproducibly initiated or terminated by cardiac pacing. The reentrant circuit is limited to the atrioventricular node and a small amount of perinodal atrial tissue. Acute termination of tachycardia can be achieved by vagal manoeuvres or drugs. Adenosine compounds are excellent drugs, as are calcium channel blockers, for acute termination of the arrhythmia. If chronic therapy is indicated, digitalis, calcium blockers and beta-blockers are effective and simple initial options. Catheter ablation, especially using radiofrequency energy, antitachycardia pacing and surgery are therapeutic alternatives for the resistant patient. CONCLUSION: Because of its high success rate and low incidence of complications, radiofrequency ablation is becoming the therapy of first choice for the treatment of AVNRT.

Radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia.

Catheter ablation using radiofrequency energy became the non-pharmacological therapy of first choice for patients with supraventricular tachycardias. Modification of the atrioventricular nodal conduction using this source of energy can be performed to treat patients with atrioventricular nodal reentrant tachycardia. In this article the authors present an updated review of radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia and report their own experience in this field.