Insuficiencia cardíaca secundaria a distrofia de Steinert / Congestive heart failure due to Steinert's muscular dystrophy: case report

Se presenta el caso de un hombre de 51 años, portador de Distrofia de Steinert, que desarrolla insuficiencia cardíaca congestiva e insuficiencia respiratoria. Se describe el tipo de compromiso cardíaco y la evolución del paciente y se discute la prevalencia de insuficiencia cardíaca en estos pacientes.

A 51 year old male with Steinert’s muscular dystrophy is admitted for congestive heart failure, along with his chronic respiratory insufficiency. Presenting findings, treatment and clinical course are described. The prevalence of heart failure in Steinert’s dystrophy is discussed.

[Treatment of advanced heart failure by heart transplantation]. / Tratamiento de la insuficiencia cardíaca avanzada mediante transplante de corazón.

BACKGROUND: Heart transplantation currently provides the most effective treatment for advanced heart failure. However, medical therapy for this condition has also improved, heart donors are scarce and the cost of the procedure is high. Therefore the indications and management of these patients need reevaluation. AIM: To analyze the results of 24 patients submitted to heart transplantation for end-stage heart failure needing repeated hospitalizations and i.v. inotropes for compensation. PATIENTS AND METHODS: The group was comprised by 21 men and 3 women with a mean age of 36.8 years, mean left ventricular ejection fraction 19 +/- 4.5%, mean systolic pulmonary artery pressure 48 +/- 13 mmHg (24-70) and mean pulmonary vascular resistance 2.6 Wood Units (1-5). Fourteen patients (58%) had a previous median sternotomy. Immunosuppression did not include induction therapy and steroids were discontinued early. RESULTS: Operative mortality was 4% at 30 days. Actuarial survival at one year was 90% and at 5 years 72%. Freedom from rejection at one year was 76% and at 5 years 50%. Freedom from infection was 70% at one year and 56.5% at five years. All patients with more than 3 months of follow-up were in functional class I. CONCLUSIONS: These results justify the proposed modifications for transplantation protocols.

[The collagen of the restenosis post angioplasty with stent: Is its origin in intima or adventitia?]. / El colágeno de la reestenosis post angioplastia con stent: se origina en la íntima o en la adventicia?

BACKGROUND: Restenosis post stenting is due to the deposit of extracellular matrix, mainly collagen in the neointima. Controversy exists regarding if collagen is generated locally or by immigration from the adventitia. AIM: To study the fibrocellular response after stent implantation in rabbit iliac arteries. To observe, by immunohistochemistry and in situ hybridization, if collagen type I mRNA is expressed in the neointima, in the media or in the adventitia. MATERIAL AND METHODS: Thirty eight white rabbits (New Zealand) of 4 kg received an hypercholesterolemic diet during 1 month. After this period, in all but 6 of them, an angioplasty with stent implantation was performed via right carotid artery in both iliac arteries, using a 1:1.3 relationship regarding the reference vessel. Angiograms were performed at day 0, 4, 21, and 40, followed by paraffin fixation of the injured segments, immunohistochemistry for alpha-actin and in situ hybridization to detect procollagen type I (alpha 1R1) mRNA. RESULTS: No hybridization was observed in non injured arteries or at day 0 (n = 6). Expression of alpha 1R1 mRNA was observed in the neointima starting at day 4 after stenting (n = 8). At day 21 (n = 8) hybridization of procollagen type I was not only observed in the neointima, but also in the media, which became equally intense in both areas. At day 40 (n = 6) hybridization was observed similarly in the media and adventitia. CONCLUSIONS: In this model, hybridization of procollagen type I started in the neointima, then involved the media and finally the adventitia. This finding might be useful for designing therapies to be delivered locally at the end of an angioplasty to prevent collagen deposition in the neointima.

[Reversible myocardial ischemia in hypothyroidism: Case report]. / Isquemia miocárdica reversible en el hipotiroidismo: comunicación de un caso.

A silent, reversible myocardial ischemia with normal coronary angiography and reversible with thyroid hormone substitution, has been recently described in hypothyroid patients. We report a 49 years old male with an abnormal exercise electrocardiogram detected in a preventive medical examination. He had laboratory evidence of hypothyroidism and a history of two years of asthenia and progressive coarsening of the voice. The Thallium myocardial perfusion study, showed an alteration of coronary flow during exercise in the septum and lower wall of the left ventricle. Thyroid hormone substitution was started and three months later, a coronary angiography was normal. After six months a repeated Thallium perfusion study and exercise electrocardiogram were informed as normal.

[Refractory arterial hypertension and the use of anticonvulsant drugs. Case report]. / Hipertensión arterial refractaria y uso de drogas anticonvulsivantes. Caso clínico.

Resistant arterial hypertension is uncommon when there is good compliance to antihypertensive therapy and secondary causes have been ruled out. We report a 41 years old male that suffered hypertensive encephalopathy and received prophylactic anticonvulsant therapy showing progressive raise of arterial pressure levels. Renovascular hypertension, aldosteronism and pheochromocytoma were discarded and, in spite of combined use of antihypertensive drugs, he did not achieve normal blood pressure. When phenytoin was discontinued, blood pressure temporarily normalized. Carbamazepine was started and blood pressure raised again. It fell when this medication was discontinued. Antiepileptic agents could induce drug metabolizing system and thus reduce the effects of antihypertensive medications.

[Use of dual-chamber pacemaker. Short and long-term results]. / Marcapasos bicamerales. Resultados inmediatos y alejados.

BACKGROUND: Continuous improvement of dual chamber DDD pacemakers, electrode stability and programmed sequential stimulation changed the prognosis of patients implanted with these devices. AIM: To report our experience with the use of dual chamber pacemakers. MATERIAL AND METHODS: One hundred seventy six patients (116 male), aged 13 to 91 years old, who received a dual chamber pacemaker implant, are reported. Patients were followed for a mean of 2.6 years. RESULTS: Indications for DDD pacemaker were complete atrioventricular block in 43%, sick sinus syndrome in 32%, paroxysmal A-V block in 24%. All pacemakers were Siemens-Pacesetter and were provided with an automatic sensing and threshold device. J shaped atrium electrodes were used in 78% of patients and screw-in electrodes in 22%. Post operative complications were displacement of atrial electrode in 8 patients, of ventricular electrode in 6 patients, infection in two patients and a hematoma in one. Chronic parameters, measured after six months, were within expected ranges and allowed a good reprogramming of pacemakers. Long term programming aimed to reduce battery depletion, enhance device performance and improve hemodynamic conditions. Normal sequential stimulation was achieved in 154 patients (87%), 14 (85) patients died of cardiovascular disease not related to pacemaker function. Eight patients were in atrial fibrillation and were reprogrammed to VVI and DDI modes. CONCLUSIONS: DDD pacemakers are reliable and afford symptomatic relief in a broad spectrum of patients.

[D/D genotype of the gene for angiotensin converting enzyme as a risk factor for post-stent coronary restenosis]. / El genotipo D/D del gen de la enzima conversiva de la angiotensina como factor de riesgo de reestenosis post-stent coronario.

INTRODUCTION: Although intracoronary stenting has decreased restenosis rate compared to percutaneous balloon angioplasty, still a high number of patients develop in-stent restenosis, which is an entity primarily due to tissue proliferation. Experimental studies have indicated that the renin-angiotensin system is involved in neointimal hyperplasia. Plasma and cellular levels of ACE are associated with an I/D polymorphism in the ACE gene. Indeed, DD subjects have the higher ACE levels. The purpose of this study was to explore the possibility that the I/D polymorphism might be related with in-stent restenosis. METHODS: We studied the ACE polymorphism in 48 consecutive patients who underwent successful implantation of an elective coronary stent in native coronary vessels and had a 6 month angiographic follow up. Restenosis (50% of the reference vessel) was observed in 23/48 patients. Patients with or without restenosis did not differ in demographic or clinical variables like diabetes, plasma cholesterol levels or in quantitative angiographic parameters such as vessel reference size or minimal lumen diameter after stent implantation. RESULTS: I/D polymorphism was distributed as follows: 22.9% of the patients were D/D; 14.5% were I/I and 62.5% of the patients were heterozygous I/D. The presence of restenosis was strongly related with the I/D polymorphism: 81.8% of the patients with D/D genotype had restenosis, compared with 40.0% of I/D patients and only 14.2% of the I/I patients (chi 2 p < 0.01). CONCLUSIONS: In this limited cohort, homocygous D/D of the ACE gene was significantly associated with in-stent restenosis, whereas restenosis was infrequent in patients with the I/I genotype.

[Chronic atrial flutter: an infrequent manifestation of sick sinus syndrome. Clinical case]. / Flutter auricular crónico: una manifestación infrecuente de enfermedad del nódulo sinusal. Caso clínico.

We report a 41 years old female, previously operated of an atrial septal defect, presenting with a persisting atrial flutter. Sinus node dysfunction became evident during an electrophysiological study at the moment of interrupting the flutter with electrical stimulation. The patient was treated with his bundle ablation and implantation of a definitive pacemaker. After one year of follow up, she is devoid of symptoms.

[Unstable angina: immediate and one-year results of transluminal coronary angioplasty treatment]. / Angina inestable: resultados inmediatos y a un año del tratamiento con angioplastía coronaria transluminal percutánea.

BACKGROUND: Unstable angina is characterized by angina at rest, angina of recent onset or accelerating angina. It is caused by a fissure or ulceration of an atheromatous plaque leading to thrombi formation and coronary spasm. AIM: To report the immediate and late results of coronary angioplasty in patients with unstable angina. PATIENTS AND METHODS: Eight hundred twenty eight patients were subjected to coronary arteriography between January 1994 and June 1996. Of these, 242 were subjected to a transluminal coronary angioplasty, 245 patients were subjected to surgical revascularization and 341 patients were treated without revascularization. RESULTS: A total of 323 stenotic lesions (1.3 lesions per patient) were subjected to angioplasty. Angiographic success was obtained in 93% of patients. Angiographic success and lack of major complications such as death, infarction of the need for surgery, was obtained in 90% of patients. Five patients (2.1%) had a non fatal infarction and five required emergency surgery. Hospital mortality was 1.2%. During the year of follow up, 15% required a new revascularization, 3.3% had a non fatal infarction and 3.3% died. CONCLUSIONS: Coronary angioplasty had a 90% immediate success and 78% of patients were free of ischemic events after one year of follow up.

[Cardiovascular effects of AV interval shortening with electrical stimulation in dilated cardiomyopathy]. / Efectos cardiovasculares del acortamiento del intervalo AV mediante estimulación eléctrica del corazón en miocardiopatía dilatada.

BACKGROUND: A temporal alteration between atrial and ventricular contraction, in which the last one would be abnormally retarded, could exist in patients with dilated cardiomyopathy. This alteration could have adverse hemodynamic effects. AIM: To study the hemodynamic modifications caused by an artificial shortening of AV interval in patients with dilated cardiomyopathy. PATIENTS AND METHODS: Nine patients with dilated cardiomyopathy were studied. Hemodynamic and tissular perfusion values, echocardiographic and radioisotopic ventricular function parameters were measured before and after six hours of AV interval shortening with electrical stimulation of the heart. RESULTS: After electrical stimulation, cardiac output increased from 3.38 +/- 0.8 to 3.87 +/- 0.79 l/min (p< 0.05). Pulmonary capillary pressure decreased from 23.8 +/- 8.9 to 19.8 +/- 9.2 mm Hg (p = NS). There were no significant changes in ventricular function parameters or in systemic and pulmonary pressures. CONCLUSIONS: Electrical shortening of AV interval in patients with dilated cardiomyopathy increases cardiac output but does not change ventricular function parameters.

[Rotational atherectomy (Rotablator) for percutaneous transluminal coronary angioplasty]. / Aterectomía rotacional (Rotablator) en la angioplastía coronaria percutánea transluminal.

BACKGROUND: The use of Rotablator in percutaneous transluminal coronary angioplasty attempts to reduce the atheromatous plaque abrading it and fragmenting the parietal calcium of the artery. AIM: To report our experience with the use of Rotablator. PATIENTS AND METHODS: Rotational atherectomy was performed in 189 patients aged 60.8 +/- 11 years (154 men). The clinical indication for the procedure was chronic angina in 22%, unstable angina in 44%, myocardial infarction in 21%, silent angina in 7% and re-stenosis in 6%. One hundred seventy seven patients were followed for a mean of 15.9 +/- 6.3 months. RESULTS: Two hundred thirty six stenoses in 215 coronary arteries were treated with a 98.7% angiographic success rate. One patient had a Q infarction and no patient died or required emergency surgery. Fourteen patients had rises in CK MB enzymes (non Q infarction). Three patients had a pseudoaneurism and three had bleedings that required transfusion. Of the followed patients, 33 had a clinically suspected re-stenosis, that was angiographically confirmed in 23. Cardiac mortality was 2.3%. Seventy nine percent of patients had an evolution without angina or coronary events. CONCLUSIONS: Percutaneous transluminal coronary angioplasty with the use of Rotablator had a high immediate success rate and a low incidence of complications. The clinical evolution of patients has been favorable with a low incidence of mortality and ischemic events.

[Radiofrequency catheter ablation in patients with common atrial flutter]. / Fulguración con radiofrecuencia en pacientes con flutter auricular típico.

BACKGROUND: Type 1 atrial flutter is produced by a reentry circuit located in the right atrium that can be interrupted applying radiofrequency in the inferior cava-tricuspid valve isthmus. AIM: To report our experience in the treatment of atrial flutter with radiofrequency ablation. PATIENTS AND METHODS: Nine patients (eight male) whose ages ranged from 6 to 72 years old were studied. Two patients had an operated congenital cardiopathy, two had high blood pressure, one was subjected previously to radiofrequency ablation due to a left paraspecific pathway, one developed a cardiac failure secondary to tachycardia and three did not have evidences of cardiopathy. RESULTS: In two patients, atrial flutter was not interrupted. In the other seven patients, radiofrequency ablation was successful. There were three relapses in the first month after the procedure, of these, two patients were successfully treated again. After a mean follow up of 4.5 months, these patients are asymptomatic and without antiarrhythmic drugs. Analysis of obtained signals, showed that radiofrequency that interrupted atrial flutter always occurred in zones of double potentials. CONCLUSIONS: Radiofrequency ablation is an effective treatment for atrial flutter and the zone of successful ablation is associated to the presence of double atrial potentials.

[Radiofrequency fulguration for supraventricular tachycardia in pediatrics]. / Fulguración por radiofrecuencia de las taquicardias supraventriculares en pediatría.

Supraventricular tachycardias (SVT) are the most frequent cause of tachycardia in children. Its pharmacological treatment has adverse effects, is not curative, and is not always effective. During the last few years radiofrequency ablation (RF-A) has changed the treatment. The purpose of this study is to evaluate our experience in RF-A in children with SVT. Between 1990 and 1995, 92 patients (1 month to 17 years old) underwent electrophysiological study after the diagnosis of SVT. RF-A was attempted in 55 patients with accessory pathways (AP), slow-pathway of the atrioventricular node, or ectopic focus. The site of ablation was decided according to the electrical signals and the catheter position. The success of the RF-A was confirmed by the interruption of the tachycardia, the change in the sequence of activation of the intracardiac signals, the regression of the preexcitation, and the inability to reinduce tachycardia. RF-A was successful in 81% of the patients; 88% in those with a left AP, 56% in those with a right AP, and 100% in those with nodal reentry. Complications were seen in 7% of the patients: 3 with arterial obstruction, one with a minimal pneumothorax, and one with cardiac tamponade. During a follow up of 16.6 months there was no relapse nor late complications. We conclude that RF-A is a safe and effective procedure in pediatric patients with SVT.

[Noncardiogenic pulmonary edema associated with hydrochlorothiazide use]. / Edema pulmonar no-cardiogénico relacionado con la hidroclorotiazida.

A 54 years old female patient with a mitral valve prolapse and a rheumatoid arthritis treated with steroids was admitted with dyspnea and hypotension, that started 30 min after taking a pill containing enapril and hydrochlorothiazide. Hemodynamic monitoring with a Swan-Ganz catheter showed a pulmonary capillary pressure of 5 mm Hg, a systemic vascular resistance of 887 (dyn sec)/cm5 and a cardiac output of 10 l/min. The patient had a history of adverse reactions to thiazides and responded to volume replacement, dopamine and steroids.

[Fulguration with radiofrequency of the slow nodal pathway: experience in 30 consecutive cases]. / Fulguración con radiofrecuencia de la vía nodal lenta: experiencia en 30 casos consecutivos.

Atrioventricular nodal reentry tachycardia (AVNRT) is one of the most frequent mechanisms of paroxysmal supraventricular tachycardia. In these patients tachycardia is maintained due to anterograde conduction through a slow pathway and retrograde conduction to the atrium via a fast pathway. We present herein our experience in ablation of the slow pathway. Since January 1993, 30 consecutive patients with AVNRT underwent attempted catheter ablation of the slow pathway. Mean age was 35 +/- 3.7 years. All patients had symptomatic tachycardia and six had history of syncope. Electrophysiologic studies revealed AVNRT in all patients, in addition, two patients had a left accessory pathway. Slow pathway ablation was performed with a Mansfield 7 F catheter, guided by both fluoroscopic positioning and endocardial signals. A mean of 13 bursts were applied. In the 30 patients conduction through the slow pathway was interrupted, and thus tachycardia was no longer inducible. Retrograde conduction post ablation was evaluated in 17 of the 30 patients, significant changes were observed in three of them. One patient developed second degree AV block and a permanent pacemaker was implanted. Another patient had recurrence of tachycardia three months post ablation. After a second attempt she is arrhythmia free. Patients have been followed for a mean of 15.7 +/- 2.5 months and are asymptomatic in the absence of antiarrhythmic therapy.

[Junctional reciprocating tachycardia: clinical and electrophysiological characteristics, and the role of fulguration in 5 cases]. / Taquicardia incesante de la unión: características clínicas, electrofisiológicas y rol de la fulguración en 5 casos.

The permanent form is a variety of junctional reciprocating tachycardia that is refractory to medical treatment. The anterograde arm of the circuit is formed by the His Purkinje bundle and the retrograde conduction is through a slow conduction Accessory Atrioventricular Pathway. We report five patients with this type of arrhythmia, subjected to electrophysiological assessment. Their mean age was 37 years, all suffered from palpitations and several medical treatments had failed. During tachycardia, electrocardiogram had a negative P wave in inferior leads and RP interval was bigger than PR interval. Accessory Pathway were located in the right postero-septal region in three patients, in the left postero-septal region in one and in the left lateral in one. Specific bundle fulguration was successfully attempted in four patients, in whom arrhythmias did not recur without medical treatment.

[Arrhythmogenic right ventricular dysplasia. Options of treatment during a 12-year follow-up of a patient]. / Displasia arritmogénica de ventrículo derecho. Opciones de tratamiento durante 12 años de seguimiento en un caso.

The replacement of muscle by fibrous and adipose tissue leads to arrhythmogenic right ventricular dysplasia. We report the clinical features and therapeutic options of a 50 years old male with the disease followed during 12 years. The latter included pharmacological therapy, surgical pseudoaneurysmal resection and radiofrequency fulguration of a second arrhythmogenic focus that appeared 10 after the surgical procedure. The patient remained asymptomatic after each therapy, until the disease progressed again. This follow up is one of the longest reported and documents the disease's clinical presentation, evolution and treatment.

[Hypertrophic cardiomyopathy with two episodes of recuperated sudden death. Report of the first implantable defibrillator cardioverter inserted in Chile]. / Miocardiopatia hipertrófica y doble episodio de muerte subita recuperada. Primer cardiovertor desfibrilador automático implantable colocado en Chile.

We report a 29 years old male with a non obstructive hypertrophic cardiomyopathy that survived two episodes of cardiac arrest and with a familiar history of the disease and sudden death. He had an implant of an automatic implantable cardioverter defibrillator by a left anterior thoracotomy with intraoperative electrophysiology. The postoperative outcome was uneventful. After one year of follow up, the patient is in good functional capacity and the implanted device has not performed defibrillations.