Growth hormone deficiency and other endocrinopathies after childhood brain tumors: results from a close follow-up in a cohort of 242 patients.

PURPOSE: Brain tumors are the most common solid tumor in children. The prevalence of survivors from these cancers has been increasing, presenting endocrine sequelae in more than 40% of the cases. Our aim was to characterize the endocrinopathies diagnosed in this population, exploring the outcomes of growth hormone treatment. METHODS: We have performed a retrospective analysis of the survivors that were followed-up through a close protocol at our endocrine late-effects clinic. RESULTS: 242 survivors, followed during 6.4 (0-23.4) years, were considered. The median age at tumor diagnosis was 6.7 (0-18) years and pilocytic astrocytoma was the most frequent neoplasm (33.5%). The prevalence of endocrinopathies was of 71.5%, with growth hormone deficiency being the most frequent (52.9%). An indirect correlation between the age at the beginning of somatropin and growth velocity in the first year of treatment was observed. Those treated with craniospinal radiotherapy presented a smaller final upper/lower segments ratio comparing with those that only received cranial radiotherapy. However, their final height was not compromised when compared to their family height target. We found pubertal delay in 12%; accelerated/precocious puberty in 13.2%; central and primary hypogonadism in 21.9% and 3.3%, respectively; primary and central hypothyroidism in 23.6% and 14.5%, respectively; thyroid nodules in 7.4%; ACTH deficiency in 10.3% and diabetes insipidus in 12%. CONCLUSION: This study reveals a higher prevalence of endocrinopathies in brain tumors survivors and explores the influence of craniospinal irradiation in the adult body proportions. It reinforces the importance of routine follow-up among survivors.

Ablation of pulmonary vein foci for the treatment of atrial fibrillation; percutaneous electroanatomical guided approach.

AIMS: To evaluate the usefulness of three-dimensional (3D) electroanatomical mapping of the pulmonary veins (PV) for guiding radiofrequency (RF) ablation of focal atrial fibrillation (AF) in a single session and to correlate the electrophysiological results with the six month clinical outcome. METHODS AND RESULTS: Sixteen consecutive patients with idiopathic paroxysmal AF (more than 1 episode/month) were studied. A non-fluoroscopic mapping system was used to generate 3D electroanatomic maps of the left atrium and deliver RF energy. In patients with frequent ectopies, mapping was performed using the 'hot-cold' approach (looking for the earliest electrogram in the 3D reconstruction). In patients with infrequent/no ectopies, double/ multiple potentials recorded at the PV were tagged. Pacing at these sites to test for inducibility of ectopy or atrial fibrillation was used to define PV foci. The therapeutic endpoint was defined as suppression of premature beats, dissociation of PV potentials and inability to induce AF. Twenty-five foci were identified (multiple foci in 38%). In the 4 pts with frequent ectopies, Group A, these were suppressed by 4 +/- 4.7 applications. In the 12 pts with infrequent/no ectopies, Group B, an average 4.7 +/- 1.8 applications were delivered per focus; the endpoint was achieved in eight of the patients (13 of 21 foci). By 180 days follow-up, 11 patients were free of symptoms and in sinus rhythm, two had paroxysmal AF episodes and 3 have symptomatic ectopies and are receiving antiarrhythmic drugs. The overall success rate at six months was thus 69%, 100% for group A and 58% for group B. CONCLUSION: Electroanatomic guided RF ablation of paroxysmal AF was highly successful in patients with frequent ectopies. The use of electroanatomical mapping for precise anatomical localization of multiple potentials and for guiding the PV ostia isolation allowed successful RF ablation in 50% of pts with infrequent/no ectopies.

Implantable cardioverter-defibrillator: beyond efficacy.

OBJECTIVES: The introduction of the implantable cardioverter-defibrillator was a very important advance in the treatment of malignant ventricular arrhythmias. However, its use is associated with some possible adverse events, which should be taken into consideration when a patient is proposed for implantation. These complications may occur early after implantation and be associated with the procedure itself, or they may be late complications, usually associated with the device or the arrhythmia. It was our objective to assess the incidence of these complications in our population of patients. POPULATION AND METHODS: We describe the complications found in 98 patients (55.9 +/- 13.9 years, 89% male) with an implantable cardioverter-defibrillator and compare our results with the incidence of complications described by other authors. RESULTS: We found complications associated with the presence of the implantable cardioverter-defibrillator in 32% of patients. The most frequent complication was inappropriate shocks in 13%. The presence of infection was detected in 4%, lead insulation faults in 5%, need for lead extraction in 2%, repositioning in 1% and re-establishment of the connection with the generator in 2%. In 5% of patients, there was inefficacy of the device, 3% due to non-detected ventricular tachycardias (slow tachycardias) and 2% due to electrical storms. There was syncope in 2% of patients. The total mortality in a 2.9 +/- 1.9 year follow-up was 13% (sudden death in 3%). CONCLUSIONS: Major complications associated with implantable cardioverter-defibrillators were in our experience relatively rare, our results agreeing with those of other centers.

[Constrictive pericarditis: an early diagnosis?]. / Pericardite constritiva: um diagnóstico precoce?

The authors report a case study of a 73-year-old male, with signs of right-sided heart failure with 6 months of evolution. It was constrictive pericarditis, without radiologic, echocardiographic and magnetic resonance imaging manifestations, diagnosed on the basis of the clinical situation and hemodynamic evaluation during cardiac catheterization. The authors suggest that the lack of imagiologic manifestations might have been the result of an early diagnosis, before the development of the classic picture. Pericardiectomy, performed with success, was followed by resolution of the heart failure. It was not possible, histologically, to draw conclusions about the etiology. The development of mitral regurgitation after pericardiectomy has enriched this case with an unusual, but previously described, complication of this type of surgery.

Use of stents for small coronary arteries. Results of the Multi-Link 2.5 Portuguese Registry.

INTRODUCTION: Stents are being used with increasing frequency in percutaneous transluminal coronary angioplasty (PTCA) but their use in small vessels is still controversial, due to the possibility of excessively high rates of adverse events and restenosis. OBJECTIVE: To assess the safety and clinical efficacy of ACS RX Multi-Link (ML) 2.5 mm stents, in "de novo" coronary stenosis. DESIGN: Prospective Registry, with 6 months clinical follow-up, involving all Portuguese centers of Interventional Cardiology. POPULATION: Between April 7 and November 20 1998, 102 patients were enrolled, 82 male and with ages ranging from 30 to 86 years (average 58 +/- 11). Clinical presentation for PTCA was stable angina in 53%, unstable angina in 36% and silent ischemia in 11%. There was a history of previous myocardial infarction in 29% of patients. The main risk factors were hypertension (58%), hyperlipidemia (57%), smoking (25%) and diabetes (20%). Multivessel coronary artery disease was present in 46% of patients and left ventricular function was normal in 89%. Of the 217 existing lesions, 188 (87%) were treated: 35 with balloon angioplasty and 153 with stent implantation, 114 of which were ML 2.5 mm: 79 of 15 mm in length and 35 of 25 mm. METHODS: Angiographic success with ML stent implantation and major adverse cardiac events (MACE)--myocardial infarction (MI), coronary artery bypass graft (CABG), new target vessel revascularizations and death--were evaluated during hospital stay, and at 1 and 6 months clinical follow-up. RESULTS: Angiographic success was 97.4%. In one patient it was not possible to cross the lesion, in another there was stent migration and in a third distal coronary flow after stenting was TIMI grade 1. Clinical success was 96.1% and there were no cases of death, Q-wave MI or urgent CABG. Two patients had non-Q wave MI and two required urgent repeat angioplasty. Subacute stent thrombosis occurred in 1 patient. There were no additional MACE at 1 month follow-up. At 6-month follow-up (in 97% of patients) MACE had occurred in 14.1%: 2 deaths (one non-cardiac), 3 MI (one non-Q) and 14 new PTCA (one in a non-ML stent). There was no need for CABG in any patient. Six-month survival rate was 97.9%, 94.9% were free of infarction and 84.8% were free of infarction and new revascularization. CONCLUSIONS: Multi-Link 2.5 mm stent implantation appears to be safe and efficient with a low incidence of immediate and 6-month adverse events in the range of centers and operators of the Registry.

Results of the quantitative coronary angiographic analysis in the angiographic subgroup of the Multi-Link 2.5 Portuguese Registry.

INTRODUCTION: The restenosis rate of stents implanted into small coronary arteries is considered higher than that of stents in arteries > or = 3 mm, but could be influenced by clinical parameters and by the type and length of the stents. OBJECTIVE: To assess the incidence of angiographic restenosis at 6 months after implantation of 2.5 mm ACS RX Multi-Link (ML) stents, 15 and 25 mm length, in de novo coronary stenosis. DESIGN: Angiographic substudy of the Portuguese Multi-Link 2.5 Registry, which included Interventional Cardiology centers with facilities for coronary angiographic recordings allowing quantitative analysis. POPULATION: Between April 7 and November 20 1998 and in the 5 hospitals that agreed to participate, 61 patients were enrolled and 40 selected as having procedural and 6-month angiographies of sufficient quality for quantitative analysis. The only significant differences in the demographic, clinical and angiographic characteristics of the patients relative to those of the Registry were the lower prevalence of unstable angina and smoking in the angiographic substudy. There were 43 lesions and 46 ML stents were implanted. In 37% the lesions were located in the LAD, in 37% in the LCx and 26% in the RCA. 29 stents of 15 mm length and 17 stents of 25 mm were used. METHODS: The morphologic characteristics of the lesions were analyzed subjectively using the AHA/ACC classification. Quantitative coronary analysis (QCA) was performed, in an independent laboratory using the CAAS 2 system, for reference vessel diameter, lesion length, percentage of stenosis, minimum lumen diameter (MLD) and related parameters. Restenosis was defined as > or = 50% lumen obstruction at 6-month angiography. RESULTS: In the subjective analysis, 2 lesions were type B1, 27 type B2 and 14 type C. The overall restenosis rate was 32.6%. Restenosis was 27.7% for 15 mm stents and 36% for 25 mm stents (p = 0.4). For the QCA parameters analyzed, only MLD at the end of stent implantation was a predictor of 6-month restenosis (2.19 +/- 0.30 without vs. 2.03 +/- 0.18 with restenosis, p = 0.048). CONCLUSIONS: The restenosis rate of the Multi-Link 2.5 mm stents, of 15 and 25 mm length, was similar to that described with other types of stents in small coronary arteries. Minimum lumen diameter after stenting was found to be the best predictor of 6-month restenosis.

[The angioplasty of chronic coronary occlusions with the excimer laser for debulking followed by stent implantation]. / Angioplastia de oclusões coronárias crónicas com laser excimer para debulking seguido de implantação de stent.

UNLABELLED: Coronary angioplasty (PCI) of chronic total occlusions (CTO), even when successful, are associated to less favourable long term results. The recent use of coronary stents has improved the long term outcome of those interventions. PURPOSE: To evaluate the short term results and long term occurrence of major adverse coronary events (MACE): death, MI, urgent revascularization and the need for a new target vessel revascularization (TVR) in patients with CTO who had previously been submitted to a PCI with excimer laser for plaque debulking followed by a provisional stent. POPULATION: From our database, we selected 19 patients with a mean age of 51 +/- 13 years (18 male) submitted to PCI between 1994 and 1998. Of those patients, 10 had had of a previous MI. Hypertension, smoking habits and hypercholesterolemia were present in 9 patients (42%). Two patients had diabetes. The main reason for PCI was stable angina in 16 patients (84%) and unstable in 3 patients (16%). Single-vessel disease was present in 18 patients (94%) and multiple-vessel in 1 patient (6%); left ventricular ejection fraction was preserved in 18 patients (94%). Single vessel PCI was performed in 16 patients (84%) and double vessel in 3 patients. Plaque debulking with excimer laser was performed in all patients, followed by 23 stents (Multilink--8; Gianturco Roubin--5; Palmaz Schatz--4; others--6). The mean clinical follow-up was 19 months. RESULTS: There were no major short-term clinical events (death, MI or urgent revascularization). During follow-up, TVR was only required in 5 patients (26%), all of them in the first 7 months after PCI. CONCLUSIONS: In the highly selected population, PCI for chronic total occlusion, with excimer laser plaque debulking followed by provisional stents, was a safe procedure with a very acceptable rate of new target vessel revascularization in the follow-up period.

[The Brugada syndrome--a clinical case]. / Síndrome de Brugada--caso clínico.

A case report of a patient with syncope and family history of sudden death is presented. The precordial recordings in the standard 12-lead ECG showed a right bundle-branch block pattern with persistent ST elevation in V1 and V2-V3. After a thorough evaluation, we found no underlying organic cardiomyopathy. The diagnosis of symptomatic Brugada syndrome was made. A cardioverter-defibrillator was implanted.