Hhex is Required at Multiple Stages of Adult Hematopoietic Stem and Progenitor Cell Differentiation.

Hhex encodes a homeodomain transcription factor that is widely expressed in hematopoietic stem and progenitor cell populations. Its enforced expression induces T-cell leukemia and we have implicated it as an important oncogene in early T-cell precursor leukemias where it is immediately downstream of an LMO2-associated protein complex. Conventional Hhex knockouts cause embryonic lethality precluding analysis of adult hematopoiesis. Thus, we induced highly efficient conditional knockout (cKO) using vav-Cre transgenic mice. Hhex cKO mice were viable and born at normal litter sizes. At steady state, we observed a defect in B-cell development that we localized to the earliest B-cell precursor, the pro-B-cell stage. Most remarkably, bone marrow transplantation using Hhex cKO donor cells revealed a more profound defect in all hematopoietic lineages. In contrast, sublethal irradiation resulted in normal myeloid cell repopulation of the bone marrow but markedly impaired repopulation of T- and B-cell compartments. We noted that Hhex cKO stem and progenitor cell populations were skewed in their distribution and showed enhanced proliferation compared to WT cells. Our results implicate Hhex in the maintenance of LT-HSCs and in lineage allocation from multipotent progenitors especially in stress hematopoiesis.

Clinical and laboratory features of neuropathies with serum IgM binding to TS-HDS.

INTRODUCTION: In this investigation we studied clinical and laboratory features of polyneuropathies in patients with serum IgM binding to the trisulfated disaccharide IdoA2S-GlcNS-6S (TS-HDS). METHODS: We retrospectively compared 58 patients with selective IgM binding to TS-HDS to 41 consecutive patients with polyneuropathies without TS-HDS binding. RESULTS: Patients with IgM vs. TS-HDS commonly had distal, sensory, axonal neuropathies. Weakness was associated with IgM M-proteins. Hand pain and serum IgM M-proteins were more common than in control neuropathy patients. TS-HDS antibody binding was often selectively κ class. Biopsies showed capillary pathology with thickened basal lamina and C5b9 complement deposition. IgM in sera with TS-HDS antibodies often bound to capillaries. CONCLUSIONS: Serum IgM binding to TS-HDS is associated with painful, sensory > motor, polyneuropathies with an increased frequency of persistent hand discomfort, serum IgM M-proteins, and capillary pathology. Serum IgM binding to TS-HDS suggests a possible immune etiology underlying some otherwise idiopathic sensory polyneuropathies.

Genomic Profiling of T-Cell Neoplasms Reveals Frequent JAK1 and JAK3 Mutations With Clonal Evasion From Targeted Therapies.

PURPOSE: The promise of precision oncology is that identification of genomic alterations will direct the rational use of molecularly targeted therapy. This approach is particularly applicable to neoplasms that are resistant to standard cytotoxic chemotherapy, like T-cell leukemias and lymphomas. In this study, we tested the feasibility of targeted next-generation sequencing in profiles of diverse T-cell neoplasms and focused on the therapeutic utility of targeting activated JAK1 and JAK3 in an index case. PATIENTS AND METHODS: Using Foundation One and Foundation One Heme assays, we performed genomic profiling on 91 consecutive T-cell neoplasms for alterations in 405 genes. The samples were sequenced to high uniform coverage with an Illumina HiSeq and averaged a coverage depth of greater than 500× for DNA and more than 8M total pairs for RNA. An index case of T-cell prolymphocytic leukemia (T-PLL), which was analyzed by targeted next-generation sequencing, is presented. T-PLL cells were analyzed by RNA-seq, in vitro drug testing, mass cytometry, and phospho-flow. RESULTS: One third of the samples had genomic aberrations in the JAK-STAT pathway, most often composed of JAK1 and JAK3 gain-of-function mutations. We present an index case of a patient with T-PLL with a clonal JAK1 V658F mutation that responded to ruxolitinib therapy. After relapse developed, an expanded clone that harbored mutant JAK3 M511I and downregulation of the phosphatase, CD45, was identified. We demonstrate that the JAK missense mutations were activating, caused pathway hyperactivation, and conferred cytokine hypersensitivity. CONCLUSION: These results underscore the utility of profiling occurrences of resistance to standard regimens and support JAK enzymes as rational therapeutic targets for T-cell leukemias and lymphomas.

Hemodynamic effects of intravenous administration of amiodarone in congestive heart failure from chronic Chagas' disease.

Fourteen patients with congestive heart failure due to chronic Chagas' disease had hemodynamic studies before and 20, 40 and 60 minutes and 24 hours after intravenous amiodarone. Amiodarone was given initially as a bolus (5 mg/kg); after 1 hour a continuous infusion was maintained for 24 hours (total dose 900 to 1,050 mg). During the first hour of observation, heart rate and cardiac index decreased and mean right atrial, left ventricular end-diastolic pressures and pulmonary and systemic vascular resistances increased. Except for heart rate and mean right atrial pressure, all hemodynamic variables returned to control values at 24 hours. Thus, myocardial depression occurred with a dose of 5 mg/kg within the first hour of intravenous administration. Amiodarone must be cautiously administered by bolus, especially in patients with cardiac failure.

A technique of anastomosis of the right internal mammary artery to the circumflex artery and its branches.

A new technique for direct revascularization of the left circumflex artery and its ventricular branches through anastomosis with the right internal mammary artery (RIMA) was applied in 56 patients. Postoperative angiographic studies in 17 patients showed that positioned behind the aorta, the RIMA can reach the circumflex artery with no tension, allowing adequate distal filling.

Single coronary arteries: two cases with distinct and previously undescribed angiographic patterns.

Single coronary artery is a rare congenital anomaly, sometimes associated with myocardial ischemia. We present the clinical and angiographic features of two symptomatic patients with documented myocardial ischemia and with distinct and previously undescribed patterns of single right coronary arteries. These cases are new variants of the types R-I and R-II-A, in which the most probable mechanisms of ischemia are the insufficient blood supply, due to the long trajectories of the single arteries and the presence of underdeveloped vessels. Also, our second case presented with a fistulae from the LCX to the left ventricle, which is another determinant of myocardial ischemia.

Prognostic significance of right ventricular dysfunction in patients with acute inferior myocardial infarction and right ventricular involvement.

Little is known about the influence of right ventricular (RV) dysfunction on prognosis of patients with acute inferior myocardial infarction (IMI) and RV involvement. Therefore, 99 consecutive patients (mean age 56.6 +/- 3.4 years) with RV involvement during acute IMI were followed for a 12-month period to clarify the influence of acute RV dysfunction on short- and long-term survivals. Forty-one patients with IMI evolved with severe arterial hypotension due to RV dysfunction, while 58 patients had no hemodynamic impairment due to RV involvement. Basal hemodynamic data (mean +/- SD) for patients with RV dysfunction were blood pressure (BP) 92/59 +/- 22/20 mmHg, systemic vascular resistance (SVR) 2314 +/- 252 dynes.s.cm-5, and cardiac index (CI) 1.3 +/- 0.3 l/min/m2. Patients without RV dysfunction demonstrated BP 113/74 +/- 20/16 mmHg (p < or = 0.05), SVR 1324 +/- 354 dynes.s.cm-5 (p < or = 0.01), and CI 2.6 +/- 0.5 l/min/m2 (p < or = 0.05). Angiographic differences noted were that hemodynamically compromised patients showed lower RV ejection fractions (0.27 +/- 0.08) than patients without hemodynamic disturbance [0.41 +/- 0.11 (p < or = 0.05)]; however, left ventricular ejection fractions were 0.48 +/- 0.10 and 0.52 +/- 0.12, respectively. Short- and long-term mortality rates were assessed during the follow-up period. Patients with hemodynamic impairment due to RV infarction had a higher mortality rate for the first month and for 11 subsequent months post MI than patients without hemodynamic impairment, that is 24.4 vs. 6.9 and 14.6 (p

Difference between the response of the pulmonary and systemic circulation during exercise in congestive heart failure.

Thirty nine patients with cardiomyopathy were evaluated by a hemodynamic study at rest and during isotonic exercise. These patients were divided into two groups on the basis of their left ventricular ejection fraction (LVEF): A, control group (LVEF greater than 40%, N = 19); B, severe congestive heart failure group (LVEF less than or equal to 40%, N = 20). Patients in group A showed adequate cardiovascular responses to exercise, i.e. increased cardiac index (80%) and stroke volume index (17%), decreased systemic vascular resistance (36.8%) but no significant change in pulmonary arteriolar resistance values. In contrast, patients in group B showed a slight and heart rate-dependent increase in cardiac index (38.5%), no improvement of stroke volume index (-3.4%), decreased systemic vascular resistance (21.6%) and increased pulmonary arteriolar resistance (41.2%) with exercise. This group also showed a significant increase in right atrial pressure that might reflect a decrease in right ventricular performance secondary to an abnormal pulmonary vascular response during exercise.

Anomalous origin of the right coronary artery from the pulmonary artery in association with a ventricular septal defect.

Origin of the right coronary artery from the pulmonary artery is a rare lesion occasionally found at angiography or autopsy. We report the rare preoperative diagnosis, in a child, of anomalous origin of the right coronary artery from the pulmonary artery, in association with a ventricular septal defect. The chest radiograph was normal, but auscultation revealed a continuous murmur at the left sternal border and electrocardiography showed right and left ventricular hypertrophy. A transthoracic echocardiogram depicted anomalous origin of the right coronary artery from the pulmonary artery. Color-flow Doppler echocardiography indicated possible right-coronary-artery-to-right-ventricle fistulae. Diagnosis was made by selective left coronary arteriography, which showed retrograde filling of the right coronary artery from collateral vessels. Selective left coronary arteriography depicted intercoronary flow, with no fistulae. Operative repair consisted of moving the proximal right coronary artery from its origin at the pulmonary trunk to the aorta. An associated procedure for correction of the ventricular septal defect was performed. The postoperative cardiac angiogram showed that the ventricular septal defect was closed and that flow through the right coronary artery was normal. Preoperative diagnosis of anomalous origin of the right coronary artery from the pulmonary artery is important, because this condition is surgically correctable.

[Multiple myocardial coronary bridges]. / Pontes miocárdicas múltiplas.

White woman, 31 years old, complaint pain in the left arm, at rest, and few months later she had pain in the precordial region with dyspnea, diaphoresis and dizziness without any inducing factor. One hour later, symptoms ceased spontaneously, remaining daily precordial pain. Subsidiary examinations as cineangiocoronariography revealed 100% of arterial lumen constriction in the middle portion of the descending coronary artery, during systole, as well as in the diagnoalis and marginal branches. Multiple myocardial coronary bridges has been described in necropsy, but during cinecoronarioangiography, this case appeared to be the first one.

[Hemodynamic effects of low doses of chlorthalidone in patients with mild systemic arterial hypertension]. / Efeitos hemodinâmicos de baixas doses de clortalidona em pacientes com hipertensão arterial sistêmica leve.

PURPOSE: To evaluate the hemodynamic effects of low doses of chlorthalidone (CHT) in patients with systemic arterial hypertension (SAH). METHODS: Eight patients with mild SAH, mean age of 52 +/- 8.9 years, 7 men, were studied. Clinical evaluation, systolic (SBP) and diastolic (DBP) blood pressure and heart rate (HR), in supine and standing positions, were obtained before and every two weeks, first two in placebo, during 12 weeks. Laboratory data, hemogram, sodium, potassium, urea, creatinine, glucose, hepatic aminotransferases and urinalysis, were done and at end of study. Hemodynamic monitorization was performed by Swan-Ganz catheter in pulmonary artery to obtain RAP and PAWP, in mmHg. Cardiac output (CO) was obtained by thermodilution method. Systemic vascular resistance (SVR) arose from variables above. Hemodynamic variables were measured at 2nd and 12th weeks during treatment with 50mg of chlorthalidone each 48 h. RESULTS: A significant reduction of SBP (p = 0.005 and p = 0.003), DBP (p < 0.0001 and p < 0.0001), respectively in supine and standing positions. HR did not show statistical difference. At hemodynamic monitoring was observed a significative reduction of SVR (p < 0.02), but not with CO. CONCLUSION: Chlorthalidone in low dosis was effective to treat mild SAH, basically by lowering SVR.

[Mitral catheter-balloon valvuloplasty in surgical high risk patients]. / Valvoplastia mitral por cateter-balão em pacientes de alto risco operatório.

PURPOSE: To evaluate percutaneous mitral balloon valvuloplasty (PMBV) in surgical high risk patients. METHODS: Twenty (12%) patients out of 172 submitted to a PMBV were considered high surgical risk cases; 17 (85%) were women and mean age was 43 (18-69). Thirteen (65%) were in acute pulmonary edema (3 were pregnant, 2 had previous cerebrovascular event, 1 had pulmonary thromboembolism and other had chronic renal failure), and 7 (35%) were in functional class (CF) III (2 had coronary artery disease, 2 severe obesity, 1 pulmonary neoplasia, 1 cardiac cachexia and one with previous cerebrovascular event). All patients underwent PMBV through transseptal technique. Double balloon was used in 10 (50%) patients; Inoue balloon in 5 (25%), mono-foil in 3 (15%) and bi-foil in 2 (10%). RESULTS: Seventeen (85%) obtained success and 2 (10%) had clinical improvement. The hemodynamic results pre versus post-PMBV showed: left atrium pressure (LAP) mmHg 28.2 +/- 10.0 x 15.2 +/- 9.2 (n = 20) (p < 0.001), mitral medium gradient (G) mmHg 21.2 +/- 10.7 x 10.7 +/- 6.7 (n = 18) (p < 0.001), mitral valve area (MVA) cm2 0.73 +/- 0.3 x 1.73 +/- 0.6 (n = 11) (p < 0.001), pulmonary artery pressure mmHg 52.0 +/- 18.2 x 40.1 +/- 14.7 (n = 18) (p < 0.001) and cardiac index L/min/m2 2.1 +/- 0.4 x 2.5 +/- 0.6 (n = 14) (p < 0.001). Comparative echocardiography results pre, post and 6 months after PMBV showed: MVA 1.06 +/- 0.39 x 1.92 +/- 0.51 x 1.65 +/- 0.5 and G 13.8 +/- 4.7 x 7.3 +/- 3.6 x 7.3 +/- 4.4. There were two immediate deaths (pulmonary thromboembolism and multiple organs failure) and other after hospital discharge. The complications were: 2 (10%) patients with neurological complications, 1 (5%) atrial septal defect and other right atrium perforation (5%). CONCLUSION: PMBV offers an alternative to surgical treatment in high risk surgical patients and the results are maintained in the 6 month follow-up.

[Mitral stenosis correction by double catheter-balloon valvuloplasty technique]. / Correção de estenose mitral através de valvoplastia por técnica de duplo cateter-balão.

PURPOSE: To evaluate long-term efficacy of double balloon percutaneous mitral valvuloplasty technique (PMV2B). METHODS: Sixty-eight patients (76% female), mean-age 32 (15-69) years who had been submitted to PMV2B, that completed clinical and echodopplercardiographic one year follow-up (PO12M). Admission criteria were: exertional dyspnoea, no thromboembolism antecedent up to three months before the procedure, absence of other cardiac disease requiring correction, an admissible echodopplercardiographic score, absence of intracavitary thrombus and mitral regurgitation absent or minor. RESULTS: The patients were divided in two groups: group A of 7 (11%) patients that have a cardiac event in this period, and group B of 61 patients that completed the follow-up without a cardiac event. In group A three patients have had a severe mitral regurgitation, one case was unsuccessful and other one had a re-stenosis. There were two deaths, not related to the intervention. In group B, haemodynamic results before and immediately after PMV2B (POI) showed a significant improvement, except in relation to cardiac index. There was an increase in the grade of mitral regurgitation in 17 (28%) patients and in two cases this regurgitation became moderate. The mitral valvar area (MVA) variation between PRE x POI x PO12M presented, comparing MVA between POI and PO12M, a significant reduction. Two (3%) patients with a reduction greater than 50% of the initial increase, 33 (54%) between 10 and 50% and 26 (42%) less that 10% remained in functional class I/II. CONCLUSION: PMV2B is an attractive treatment to select symptomatic mitral stenosis patients, with a low incidence of complications, symptomatic effective improvement that was maintained in one year follow-up, although there was a reduction in MVA.

[Analysis of the mechanical properties of the left ventricle in patients with aortic valve disease]. / Análise das propriedades mecânicas do ventrículo esquerdo em portadores de valvopatia aórtica.

The morphological and functional aspects of the left ventricle (LV) were assessed by echocardiography and cardiac catheterization performed simultaneously in 41 patients. Eleven were normal (N), 14 had aortic stenosis (AS) and 16 had aortic regurgitation (AR). Of the 14 patients with AS, eight were in New York Heart Association (NYHA) functional class I and II (ASA group) and six were in NYHA functional class III and IV (ASB group). Of the 16 patients with AR, seven were in NYHA functional class I and II (ARA group) and nine in functional class III and IV (ARB group). In the ASA group normal values of the LV function were obtained because of the development of an adequate hypertrophy that in normalizing the systolic stroke was able to keep a suitable function. In the ASB group there was a reduction of the LV function due to an increase of the systolic stroke and to the reduction of the contractile muscle state. Thus, in the whole AS group we found an inversed relation between the ejection fraction and the systolic stroke. In the ARA group we found a normal cardiac function as consequence of an adequate development of the LV dilation and hypertrophy. Despite the find of reduction of the contractile state, the systolic stroke normalizing was capable to keep the cardiac function at normal values. The ARB group presented an important depression of the cardiac function due the increase of the systolic stroke and to the decrease of the contractile state of the cardiac muscle.

[Treatment using balloon catheterization of pulmonary valve stenosis]. / Tratamento por cateter-balão da estenose pulmonar valvar.

From October 84 to September 88 we performed pulmonary valvuloplasty with balloon catheter (PVBC) in 90 patients (pt) with pulmonary valve stenosis (PVS). The patients were divided in 3 groups (Gr) by their ages: group I (Gr. I) 7 pt (8%) aged 4 days to 11 months, Gr. II 66 pt (73%) aged 1 year to 12 years and Gr. III 17 pt aged 13 to 34 years. This study consists of clinical and echocardiographic evaluation adopting the following parameters of indication: a) transvalvar pulmonary gradient (G); b) absence of dysplasia or hypoplasia of the pulmonary annulus. There were associated lesions which were found in each group: Gr. I atrial septal defect (ASD) in 2 pt and tricuspid insufficiency (TI) in 1 pt, in Gr. II supra valvar stenosis (SVS) in 1 pt and patent arterial ductus (PAD) in 1 pt and in the Gr. III (ASD) in 1 pt. The results after pulmonary valvuloplasty with balloon catheter (PVBC) were: (Gr.I) in 5 pt the G were reduced below 50 mmHg and the average of right ventricular systolic pressure (RVSP) changed from 92 to 62 mmHg and the average of G from 76 to 44 mmHg. One of these pt was submitted to surgery 1 month after the procedure and 2 developed restenosis in 2 and 3 years respectively, being submitted to another dilatation with good results. The 2 pt in which the procedure was not effective were submitted to surgery. In the second group the PVBC was effective in 61 pt (92%) showing the RVSP changed from 118 to 64 mmHg and G changed from 86 to 23 mmHg.(ABSTRACT TRUNCATED AT 250 WORDS)

[Comparative study between inoue single balloon and double balloon in percutaneous mitral valvuloplasty. Immediate results and after 1 year follow-up]. / Estudo comparativo entre balão único de inoue e duplo balão na valvoplastia mitral percutânea. Resultados imediatos e após seguimento de um ano.

PURPOSE: To compare early and 12 months results of mitral stenosis (MS) correction by percutaneous balloon valvuloplasty (PMBV) using Inoue's single-balloon or double balloon techniques. METHODS: We submitted 139 consecutive patients to PMBV using Inoue single-balloon (n = 56, GI) or the double balloon technique (n = 83, GII). The two groups were similar, in: age, sex, functional class (FC), echocardiographic (ECHO) score, mitral valve area (MVA), and gradient (G) or presence of regurgitation. Clinical and ECHO data were compared before (PRE), immediately after (POI) and one year following the procedure (PO12M). RESULTS: PMBV was successfully performed in 53 (95%) patients of GI and in 79 (96%) of GII. Statistical analyses showed that the groups were similar at POI but different at PO12M (p < 0.002). ECHO immediately after PMBV showed that: MVA increased from 0.99 +/- 0.23 to 2.01 +/- 0.44cm2 (p < 0.001) in GI and from 0.94 +/- 0.23 to 2.09 +/- 0.35cm2 (p < 0.001) in GII and G decreased from 11.58 +/- 5.02 to 5.16 +/- 2.23mmHg (p < 0.001) in GI and from 12.48 +/- 4.89 to 5.96 +/- 3.21mmHg (p < 0.001) in GII. After one year 36 (64%) patients in GI and 62 (74%) in GII underwent an ECHO study. A comparison between immediate and one year follow-up results showed that MVA decreased from 2.01 +/- 0.4 to 2.00 +/- 0.3cm2 (NS) in GI and from 2.09 +/- 0.3 to 1.74 +/- 0.4cm2 (p < 0.001) in GII and G decreased from 5.16 +/- 2.2 to 5.50 +/- 2.9mmHg (NS) in GI and from 5.96 +/- 3.2 to 8.61 +/- 4.8mmHg (p < 0.001) in GII. There was therefore a sustained improvement of MVA and G after one year in GI and a significant decrease in MVA and G in GII. The FC after one year was similar and satisfactory in both groups. CONCLUSION: Both techniques are equally effective in relieving MS immediately after PMBV, but after one year, despite similar FC, Inoue-balloon technique seems to be superior to maintain MVA and G.

[Early luminal diameter reduction after coronary angioplasty and its relation to the restenosis phenomenon]. / A perda precoce do diâmetro luminal após angioplastia coronária e sua influência no fenômeno da reestenose.

PURPOSE: To evaluate the early luminal diameter loss in the first 15 min after percutaneous transluminal coronary angioplasty (PTCA) and its influence on coronary restenosis. METHODS: In a prospective study, we evaluated 86 patients. The patients were divided in two groups based on the presence or absence of coronary restenosis. Thirty one lesions developed restenosis and 55 lesions did not. RESULTS: Univariate analysis showed that balloon/artery ratio was lower in the group of restenosis (0.92 +/- 0.01 vs 1.00 +/- 0.11, P = .003). Absolute and relative elastic recoil at 1 min was greater in the group that developed restenosis (0.79 +/- 0.54 vs 0.68 +/- 0.59 mm; P = .007 and 32.04 +/- 14.27 vs 22.15 +/- 16.65%; P = .006). Similarly, absolute and relative elastic recoil at 15 min were greater in the group with restenosis (1.25 +/- 0.59 vs 0.90 +/- 0.65 mm, P = .017 e 46.75 +/- 15.69 vs 29.18 +/- 17.84%, P < .00001). Minimal luminal diameter (MLD) at 1 min was lower in the group with restenosis (2.15 +/- 0.42 vs 2.43 +/- 0.58 mm; P = .022). The very early loss was greater in the group with restenosis (0.46 +/- 0.34 vs 0.22 +/- 0.35 mm, P = .004). MLD at 15 min was lower in the group of restenosis than in the group without restenosis (1.69 +/- 0.48 vs 2.20 +/- 0.61; P = .0001). Multivariate analysis revealed balloon/artery ratio and MLD at 15 min as independent correlates of the late outcome. CONCLUSION: The late outcome of PTCA is influenced by elastic recoil and the early MLD loss after PTCA. However, the strongest and most important predictors of late outcome by multivariate analysis were balloon/artery ratio and MLD at 15 min.

[Comparison between percutaneous balloon valvuloplasty and open commissurotomy for mitral stenosis]. / Avaliação comparative entre valvoplastia percutânea e comissurotomia a céu aberto na estenose mitral.

PURPOSE: To compare immediate and late (12 months) follow-up of clinical and Doppler echocardiographic results between percutaneous mitral balloon valvuloplasty and open commissurotomy in a prospective and randomized trial. METHODS: Eighty eight symptomatic patients with severe mitral stenosis and favorable anatomy were randomized in a prospective trial comparing the two procedures. All patients were submitted to clinical and Doppler echocardiographic evaluation before the procedures and immediate and twelve months thereafter. RESULTS: Mean mitral gradient (mmHg) decreased from 12.2 +/- 5.8 to 5.80 +/- 2.7 (p < 0.001) in commissurotomy group (CG) and from 11.7 +/- 6.1 to 5.0 +/- 2.4 (p < 0.001) in the balloon valvuloplasty group (VG). Mitral valve are (cm2) increased from 0.98 +/- 0.21 to 2.52 +/- 0.46 in CG and from 1.05 +/- 0.25 to 2.18 +/- 0.40 in VG (p < 0.001). In both groups there was a slight decrease in mitral valve area at 12 month follow-up. There was no death in either group. One patient in the VG had moderate mitral regurgitation and underwent surgery. At the 12 month follow-up, all patients in CG and 97.7% of patients in VG were in New York Heart Association functional class I or II. CONCLUSION: Both procedures were safe and showed similar immediate improvement in mitral gradient and functional class. Mitral valve area had a greater increase immediately after commissurotomy, however, there was a significantly greater reduction in the CG after 12 months of follow-up, when compared to balloon valvuloplasty. In both groups, mitral gradient remained reduced and most patients did not change functional class during the follow-up.

[Transluminal angioplasty of aortopulmonary anastomosis by the Jatene surgical technique. Report of 2 cases]. / Angioplastia transluminal da anastomose aorto-pulmonar da cirurgia de Jatene. Aplicação em dois casos.

Two patients with transposition of the great arteries submitted to Jatene surgery at ages of 6 days and 6 months respectively, presented in the postoperative period stenosis of the aorto-pulmonary anastomosis. The diagnosis was made three and six years after the surgery. In both patients successful percutaneous transluminal angioplasty (PTA) was performed, respectively six months and immediately after the diagnosis. Pressure gradients decreased from 83 mmHg to 24 mmHg in the first case, and from 76 mmHg to 13 mmHg in the other case, with clear improvement of the angiographic image. Complications of the procedure did not occur in any case. This application for PTA was not previously reported and should be considered as an alternative to the surgical correction of the "new" pulmonary trunk stenosis in the Jatene surgery.

[Clinical and Doppler echocardiographic follow-up of elderly patients subjected to aortic balloon valvuloplasty]. / Seguimento clínco e ecodopplercardiográfico de idosos submetidos a valvuloplastia aórtica por cateter-balão.

PURPOSE: To evaluate the evolution of patients with critical aortic stenosis (AS) submitted to balloon aortic valvuloplasty (BAV). PATIENTS AND METHODS: sixteen patients (mean age 74.0 +/- 5.1 years), 11 (68.7%) women. The study periods consisted during the 3 rd (post-1) and 15th month (post-2) after dilatation. RESULTS: The functional class of angina and dyspnea persisted in all patients during period post-1, in 75% of the patients, during period post-2 in relation to intra-hospital evaluation. However, in period post-1, 53% of the patients had significant reduction of aortic valve area, trend which remained in period post-2. Simultaneously, progressive (insignificant) elevation of the left ventricular aortic peak to peak gradient was noticed in the periods post-1 and post-2. Two deaths and two aortic valve replacements occurred during the study. CONCLUSION: BAV is associated to maintenance of functional class improvement obtained immediately after dilatation despite the aortic valve restenosis registered by echodopplercardiography.