Real-world, long-term survival of incident patients with pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era. METHODS: Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7-5.4] years. Kaplan-Meier survival analysis was used to estimate 1-, 3-, and 5-year survival and to compare it with a historical PAH survival estimated from the NIH cohort. RESULTS: Mean age was 48±19 years with female preponderance (68%). The most common PAH subgroup was congenital heart disease (PAH-CHD) (n=31; 48%), followed by connective tissue disease (PAH-CTD) (n=16; 25%), idiopathic (IPAH) (n=8; 12%) and hereditary (HPAP) (n=1; 1.5%). BNP values (hazard ratio [HR] 2.07; 95%CI 1.34-3.22; P=0.001) and male gender [HR 4.34 (1.44-13.09); P=0.009] were predictors of death. Survival rates at 1-, 3- and 5-years were 95%, 77% and 71%. Survival was not statistically different between PAH etiologies (Log-rank P=0.7). However, PAH-CHD was associated with a decreased risk of the combined endpoint of all-cause mortality and admission for decompensated heart failure [HR 0.36 (0.15-0.85); P=0.02]. We found a non-significant numerically higher survival of incident IPAH, HPAH and DPAH patients in comparison with the historical NIH cohort. CONCLUSIONS: In this cohort of incident PAH patients, PAH-CHD patients had better overall prognosis. Higher BNP values and male gender were associated with higher mortality.

A case of cardiogenic shock with preserved ejection function / Un caso de shock cardiogénico con función de eyección conservada

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[Role of nitrates in pharmacologic modulation of reflected waves and their significance in the treatment of arterial hypertension in the elderly]. / Os nitratos na modulação farmacológica das ondas reflectidas e sua importância no tratamento da hipertensão arterial do idoso.

OBJECTIVE: To analyse the efficacy of a sustained release form of isosorbide mononitrate in the treatment of isolated systolic hypertension in the elderly. PATIENTS: 24 patients suffering from essential hypertension and with an average age of 68.5 +/- 1.1 years were studied: 20 male and four female patients, all with isolated systolic hypertension (systolic blood pressure (SBP) > 160 mmHg and diastolic blood pressure (DBP) < 90 mmHg). None of the patients had received pharmacological treatment for their hypertension. None were receiving other medication or displayed concomitant pathologies. METHODS: Assessment of all the patients was made with the measurement of their occasional blood pressure, ambulatory measurement of blood pressure and the measurement of pulse wave velocity in two arterial zones (carotid-femural) by mecanography before and after thirty days of monotherapy with a single 50 mg dose of a sustained release form of isosorbide mononitrate. Four patients were withdrawn from tests due to signs of intolerance to the drug. RESULTS: A fall in occasional blood pressure was recorded, with statistical significance in relation to SBP only: SBP-192 +/- 15.5-->164 +/- 10.2 mm Hg (p < 0.001); DBP-85 +/- 4.2-->83 +/- 5.4 mm Hg. Ambulatory blood pressure readings also showed a significant drop in average SBP readings over the 24 hours: SAP 152.6 +/- 13.6-->140.5 +/- 15.4 mm Hg (p < 0.03); DBP 77.2 +/- 8.7-->72.3 +/- 5.47 mm Hg. No significant changes in pulse wave velocity were recorded for the zones studied: carotid-femural -20.8 +/- 6.0-->21.7 +/- 5.1 m/sec; femural-foot -4.5 +/ -1.4-->4.4 +/- 2.6 m/sec; a marked alteration in the morphology of arterial pulse in the aortic zone was observed, however, with a clear levelling off and reduction of the systolic peak. CONCLUSION: Treatment with nitrates may be a new and effective alternative for the treatment of the age group in question. It acts specifically on the pathophysiological mechanisms of isolated systolic arterial hypertension in the elderly. Changes in reflected wave velocity (retrogrades) seem to cause the significant reduction in SBP, observed in this group of patients.

[24-hour arterial pressure study and diabetic neuropathy and angiopathy of the lower limbs]. / Estudo da tensão arterial nas 24 horas e neuropatia e angiopatia diabética dos membros inferiores.

AIMS: To study the circadian variation of BP in diabetics with chronic late complications (autonomic neuropathy, peripheral neuropathy and macroangiopathy of the lower limbs). PATIENTS AND METHODS: We studied 35 NIDDM patients, 22 male and 13 female, with a mean age of 62.17 +/- 8.66 years, all with hypertension (HBP) or with BP levels close to normal, many of them under anti-hypertensive therapy (mean BP in clinical measurements: 164 +/- 23.16 mmHg systolic and 92.14 +/- 11.06 mmHg diastolic). Twenty-four suffer from autonomic neuropathy, 23 from peripheral neuropathy and 11 from macroangiopathy of the lower limbs. The control group is composed of 10 patients with essential HBP without other observable pathology, with a mean age of 68.5 +/- 3.63 years and BP in clinical measurements: systolic 192 +/- 15.49 mmHg and diastolic 88 +/- 4.21 mmHg (without any previous therapy). The record of the BP circadian profile was made, with the use of a Spacelab monitor, model 90207, 5 days after anti-hypertensive therapy was suspended. RESULTS: The mean BPs in 24 hours were: control group-systolic BP 152.6 +/- 13 mmHg and diastolic 79.8 +/- 13 mmHg; diabetics-systolic BP 136.82 +/- 15 mmHg and diastolic 77.4 +/- 8 mmHg. The daily and nocturnal mean BPs were 158.5 +/- 8.5 and 146 +/- 8.4 mmHg (systolic) and 82.8 +/- 5.7 and 70.9 +/- 5.1 mmHg (diastolic) in the control group, while they were, in diabetics, 141.3 +/- 15.7 and 135.6 +/- 16.6 mmHg (systolic) and 79.6 +/- 7.1 and 72 +/- 8.2 mmHg (diastolic). In diabetic patients, considering the groups with and without autonomic neuropathy, the difference between daily and nocturnal mean BP was 1.6 vs 9.4 mmHg systolic and 4.9 vs 9.4 mmHg, diastolic. CONCLUSIONS: In both groups, the mean BP levels in 24 hours were notoriously lower than those obtained from the clinical measurements: the differences were 40 mmHg (control group) and 27.2 mmHg (diabetics) for systolic BP and 8.2 mmHg (control group) and 14.7 mmHg (diabetics) for diastolic. The BP circadian profiles show a lower variability, with absence of the nocturnal reduction, specially evident in diabetic patients with autonomic neuropathy versus other groups.

[Pulse wave velocity as initial marker of atherosclerosis]. / A velocidade da onda de pulso como marcador inicial da doença aterosclerótica.

OBJECTIVE: To evaluate the influence of hypercholesterolaemia on arterial distensibility. MATERIAL AND METHODS: 43 male New Zealand White rabbits, with similar ages and weights, were included in the present study. The animals were divided in two groups: Group A (n = 15) was fed a normal diet; Group B (n = 28) was fed normal diet plus 0.1% cholesterol. at the beginning and after 6 and 9 months, blood samples were obtained for determination of serum cholesterol (total, esterified, LDL) and Triglyceride levels. Pulse wave velocity (PWV) was also evaluated, by mecanography, after 6 and 9 months of the beginning of the experiment. After 6 months (Group A = 4 and Group B = 7) and 9 months (Group A = 6 and Group B = 7) of the experiment, some animals were killed for anatomopathological studies. RESULTS: Major differences were obtained between the two groups, specially in what concerns to LDL and cholesterol levels (p < 0.001). There was also a remarkable difference in PWV between the two groups (6.078 +/- 0.162/9.002 +/- 0.196 m/s at 6 months and 7.639 +/- 0.590/9.557 +/- 0.543 m/s at 9 months) from the rabbits fed normal or cholesterol diet, respectively. The anatomical lesions were only significant after 9 months. However there was a decrease in aorta internal diameters at thoracic and renal levels at 6 months (34% and 53%) and at 9 months (29% and 33%), without significant changes in their thickness. In the heart, the left ventricle (LV) had a significant thickness increase after 6 months (about 43%). CONCLUSIONS: These data indicate that even before anatomical lesions had occurred, important functional changes are present, in the arterial wall. Then, the evaluation of the PWV could be a promising non-invasive diagnostic method of early atherosclerosis, with obvious implications concerning its prophylaxis and therapy.

[Pulse wave velocity as expression of arterial compliance and its importance in the evaluation of arterial hypertension]. / A velocidade da onda de pulso como expressão da compliance arterial e sua importância na avaliação da hipertensão arterial.

STUDY OBJECTIVE: To determine the arterial compliance through the evaluation of pulse wave velocity. DESIGN: Open study with direct comparison of different groups within a 12 week evaluation period. PATIENTS: 69 patients, 49 with hypertension and 20 normals individuals. INTERVENTIONS: Different groups with the following treatments: Isradipine, Lisinopril, Dilevalol and no therapy. MEASUREMENTS AND MAIN RESULTS: There is marked differences in the pulse wave velocity when hypertensive patients are compared with normal individuals (p < 0.001). In a 12 week therapeutic evaluation there is an improvement in the pulse wave velocity particularly when the arterial pressure was lowered to normal values in the hypertensive patients: Lisinopril (p < 0.005), Isradipine (p < 0.005), Dilevalol (p < 0.025). CONCLUSIONS: It is very easy to evaluate the pulse wave velocity. Arterial compliance, which may be evaluated using the pulse wave velocity, is significantly reduced in hypertensive patients, compared with age-matched control subjects. The use of antihypertensive drugs is associated with changes in arterial compliance. There is a significant decrease in the pulse wave velocity after the administration of ACE inhibitors, calcium channel blockers and beta blockers for an equivalent fall in blood pressure. These observations emphasizes the potential importance of the changes in the large arteries, considered as either an associated factor or a consequence of elevated blood pressure, in the evaluation of the cardiovascular morbidity, and mortality of patients treated for hypertension.

Neopterin in tuberculous and neoplastic pleural fluids.

Neopterin is derived from guanosine-triphosphate, produced by stimulated macrophages under the influence of gamma interferon of lymphocyte origin. It has been suggested as an excellent marker for activation of the monocyte/macrophage axis in some clinical situations. We evaluated its concentration in the pleural effusions of 25 individuals (10 tuberculous and 15 neoplastic) as well as in the blood of 22 of them (8 tuberculous and 14 neoplastic), comparing these levels with those of a control group in 99 normal individuals. The concentration of neopterin was determined by radioimmunologic assay. This showed a significant increase (p less than 0.001) of neopterin levels in the tuberculous pleural fluid, compared to the neoplastic group (42 +/- 23/17 +/- 9 nmol/L). In the blood, values were nearly identical to the pleural fluid (41.3 +/- 25/15.8 +/- 6.9 nmol/L), although with significant differences between them and in relation to the control group (p less than 0.001), which had a normal serum value (5.11 +/- 1.92 nmol/L). We emphasize the influence of the neopterin levels in the pleural fluid on the diagnosis of causes of pleurisy and its importance as a marker of immunologic cellular activity.

[Non-invasive evaluation of left ventricular function (systolic time interval) in patients using double-chamber pacemakers programmed on DDD and VVI]. / Avaliação não invasiva da função ventricular esquerda (tempos sistólicos) em doentes portadores de pacemakers de dupla câmara programados em DDD e VVI.

OBJECTIVE: To evaluate changes on left ventricular function as assessed by systolic time intervals (STI), by programming "physiological" pacemakers either on VVI or DDD mode. STUDY DESIGN: Comparison of STI values--electromechanical systole (QS2); left ventricular ejection time (LVET); pre-ejection period (PEP); PEP/LVET--in patients submitted to double chamber pacing programmed in DDD or VVI mode. SETTING: Cardiac Pacing Center in a Department of Cardiology at a University Hospital. PATIENTS: We have studied 12 patients (3 men, 9 women), mean age 56.16 years (range 25-76 years) treated with double chamber pacing. INTERVENTIONS: Initial measurements of STI in DDD or one week in VVI mode, and again after one week in DDD mode. RESULTS: Significant changes were found after the first week in VVI pacing, versus baseline determination in DDD pacing, for LVET, PEP, and PEP/LVET (p less than 0.05). At the end of the second week (DDD pacing), there were also statistically significant differences for QS2 (P less than 0.05), LVET (P less than 0.01) and PEP/LVET (P less than 0.05) values when compared with the ones obtained in VVI pacing, at the end of the preceding week. No significant changes were found between the initial and the final STI determinations (both in DDD pacing). CONCLUSION: We concluded for a better performance of the left ventricle at rest when "physiological" pacing is compared with VVI pacing.

Efectos del hidroxido de sodio en la pleura de la rata. / Effects of sodium hydroxide on the pleura of rats

Fueron instiladas dentro del espacio pleural de la rata normal 2 concentraciones de HONa, para observar la respuesta producida por este nuevo agente esclerosante.Se concluye que la injuria producida en la pleura de la rata por HONa produce un exudado pleural rico en proteinas y que la concentracion usada es un factor determinante para la produccion de sinfisis pleural que es progresiva con el transcurso del tiemp

Efectos del hidroxido de sodio en la pleura de la rata. / Effects of sodium hydroxide on the pleura of rats

Fueron instiladas dentro del espacio pleural de la rata normal 2 concentraciones de HONa, para observar la respuesta producida por este nuevo agente esclerosante.Se concluye que la injuria producida en la pleura de la rata por HONa produce un exudado pleural rico en proteinas y que la concentracion usada es un factor determinante para la produccion de sinfisis pleural que es progresiva con el transcurso del tiemp