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Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs). This study aimed to investigate the clinical and hemodynamic characteristics and survival of anti-U1 RNP-positive patients with CTD-associated PAH, with a focus on systemic sclerosis (SSc)-associated PAH.We implemented a prospective database that included patients with CTD-associated PAH for whom there were clinical, autoantibody, and mortality data. We compared clinical and hemodynamic characteristics to anti-U1 RNP antibody status. We then assessed whether anti-U1 RNP antibodies could be a prognostic factor in CTD-associated PAH with a focus on SSc-associated PAH.We studied a total of 342 patients with CTD-associated PAH, of whom 36 (11%) were anti-U1 RNP antibody positive. Anti-U1 RNP-positive patients were younger and less functionally impaired than were anti-U1 RNP-negative patients in CTD- and SSc-associated PAH. Hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients. In CTD-associated PAH, anti-U1 RNP positivity was associated with decreased mortality in univariable analysis (hazard ratio 0.34 [95% confidence interval 0.18-0.65], P0.001). In multivariable analysis, anti-U1 RNP positivity was also associated with decreased mortality (hazard ratio 0.44 [95% confidence interval 0.20-0.97], P = 0.043) independently of age, sex, functional parameters, lung involvement, and hemodynamic parameters. Results were similar in SSc-associated PAH, although the association between anti-U1 RNP positivity and survival did not reach significance in univariable (hazard ratio 0.47 [95% confidence interval 0.22-1.02], P = 0.055) and multivariable (hazard ratio 0.47 [95% confidence interval 0.20-1.11], P = 0.085) analyses.Anti-U1 RNP positivity was associated with distinct clinical characteristics and survival in CTD- and SSc-associated PAH. While hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients, our results suggest that anti-U1 RNP positivity could be a factor protecting against mortality in CTD- and SSc-associated PAH.

To determine whether patients with systemic sclerosis (SSc) and borderline mean pulmonary artery pressure (PAP) at cardiac catheterization are more likely to develop pulmonary hypertension (PH) than those in whom pulmonary pressure is normal.Patients with SSc in whom PH and significant interstitial lung disease had been excluded at baseline were enrolled in our prospective cohort. Analysis of followup data identified patients who met prespecified criteria prompting repeat catheterization to reassess for possible PH. Using Kaplan-Meier, receiver operating characteristic, and Cox regression methods, we studied the development of PH and death.Of 228 patients in this study, 86 had borderline mean PAP (21-24 mm Hg) at baseline. Following prespecified criteria, 76 patients underwent repeat catheterization, and 29 of these developed PH. Two cases were related to disease of the left side of the heart. The average mean PAP increased from baseline (20.2 mm Hg) to followup (24.3 mm Hg) (P0.05 by Student's t-test). Patients with borderline mean PAP were more likely to develop PH than patients with mean PAP≤20 mm Hg (P0.001 by log rank test, hazard ratio [HR] 3.7). A transpulmonary gradient (TPG)≥11 mm Hg at baseline also predicted PH (P0.001 by log rank test, HR 7.9). Incident development of pulmonary arterial hypertension (PAH) was not benign, with a mortality of 18% within 3 years.Our findings indicate that borderline mean PAP and an elevated TPG in patients with SSc predict progression to PH. These patients should be monitored closely for the development of PH. Our findings indicate that catheterization data are useful in patients considered at risk of PAH.

A 35-year-old woman underwent reconstruction of her right pulmonary artery for treatment of acquired right pulmonary artery stenosis. The stenosis was secondary to tuberculosis causing both an extrinsic and an intrinsic obstructive component. After her reconstruction, the patient made an uneventful recovery, and perfusion to the right lung was subsequently restored.

We report the case of a 20-year-old man born with transposition of the great arteries who underwent emergency balloon septostomy and subsequently a Mustard procedure. When aged 20 years, he had several syncopal attacks due to sinoatrial disease for which he was simply and successfully paced transvenously in VVI mode.

Percutaneous isosorbide dinitrate cream and sustained-release tablets were compared in a double-blind randomised crossover trial in 28 patients with coronary artery disease and chronic stable angina pectoris. Twenty-two patients completed the trial. Both preparations significantly increased the mean exercise time to the onset of angina (P less than 0.001) and to termination of exercise (P less than 0.001) compared to the pre-treatment period. There were no significant differences between the cream and tablets with respect to frequency of anginal attacks, glyceryl trinitrate consumption, heart rate and ST segment depression at the onset of angina, ST segment depression at maximal exercise and the double product of heart rate and systolic blood pressure at maximal exercise. Equal numbers of patients expressed preference for cream and tablets. We conclude that in this group of patients isosorbide dinitrate sustained-release tablets have no clinical advantage over isosorbide dinitrate cream which, may, therefore, be of particular value for those patients with angina pectoris who dislike taking tablets or who prefer this form of nitrate preparation.

Isradipine, a new dihydropyridine calcium antagonist, was compared to nifedipine in the treatment of 11 male patients with angina and coronary artery disease in a randomised, double-blind cross-over study. Patients received 5 mg nifedipine three times a day rising to 20 mg three times a day in three dosage increments over six weeks or 2.5 mg isradipine three times a day rising to 7.5 mg three times a day in three dosage increments over six weeks, and then received the alternate preparation. There were no significant differences between the drugs in terms of the frequency and severity of angina attacks or the consumption of glyceryl trinitrate. The increases in systolic blood pressure and the double product during exercise were significantly less with isradipine than with nifedipine. There was a similar trend in heart rates. There was no difference between the treatments in respect of exercise induced ST-segment depression or diastolic blood pressure. We conclude that isradipine and nifedipine have similar anti-anginal effects and that isradipine may be a useful new anti-anginal agent.

The safety, tolerability and haemodynamic effects of oral prajmaline bitartrate were assessed in a double-blind, randomized, placebo-controlled, crossover trial in 21 patients with stable angina pectoris and coronary artery disease. No serious side-effects occurred. Prajmaline bitartrate produced no statistically significant changes in resting heart rate or systolic blood pressure or in work capacity on the treadmill, or in heart rate or systolic blood pressure at maximum exercise compared to placebo values. No new arrhythmias or conduction abnormalities were produced in any patient. We conclude that oral prajmaline bitartrate is well tolerated and can be given safely to patients with coronary artery disease without producing deleterious haemodynamic effects or changes in exercise capacity.

We report the case of a 20-year-old man born with transposition of the great vessels who underwent emergency balloon septostomy and subsequently a Mustard procedure. When aged 20 years, he had several syncopal attacks due to sinoatrial disease for which he was simply and successfully paced transvenously in VVI mode.

We report a 25-year-old man with a probable congenital diverticulum of the heart. The diverticulum was clearly shown using magnetic resonance imaging.

Summary A possible case of cimetidine induced extrapyramidal and cerebellar features is reported. Although confusion is a well recognized toxic effect of cimetidine, other neurotoxic features are less common, especially in patients without evidence of renal or hepatic disease. Cimetidine should be used with great care and possibly in a reduced dose in the elderly as neuropsychiatric side effects may occur.

Gated thallium-201 myocardial tomography incorporating perfusion profile analysis was used alone, to assess left ventricular wall perfusion and left ventricular wall movement together in 29 consecutive patients, without prior infarction, who presented with chest pain. All patients had had coronary and left ventricular angiography. The proportion of false positive perfusion defects was reduced when an analysis of corresponding wall movement was made. This combined technique resulted in an improved specificity compared with standard 201Tl myocardial tomography. This approach shows the functional effect of reversible ischaemia on regional myocardial contractility and would appear to be particularly useful in the assessment of patients who present with atypical chest pain.

Submaximal and maximal treadmill exercise tests were performed predischarge in 64 patients after acute myocardial infarction to assess the relative yield of residual ischaemic abnormalities. The reproducibility of individual abnormalities resulting from maximal stress tests performed predischarge and 6 weeks after infarction was also assessed in 55 of these patients. Compared with predischarge submaximal exercise testing, a maximal exercise test identified a significantly greater number of patients with residual myocardial ischaemia (26 vs. 15, P less than 0.05) and this was associated with a significantly longer average maximal exercise duration (P less than 0.001), and a higher rate-pressure product (P less than 0.001). Among the 55 patients who had maximal stress tests both predischarge and 6 weeks after infarction, there was a significant lack of reproducibility in the occurrence of exercise induced angina (P less than 0.01) and an abnormal blood pressure response (P less than 0.02). In contrast, exercise induced ST segment depression and elevation and ventricular arrhythmias were relatively reproducible. More patients had an ischaemic test result (ST depression or angina) at the later test compared to the predischarge test (33 vs. 25 patients) but this increase was not statistically significant. There were, however, significant increases at the later test in mean maximal exercise duration (P less than 0.001). mean maximal heart rate (P less than 0.001) and heart rate-systolic blood pressure double product (P less than 0.001). The majority of patients who had a cardiac event in the period between the two tests had a predischarge test abnormality. We conclude that a significantly greater number of patients with residual reversible myocardial ischaemia after infarction will be identified by symptom limited exercise testing compared with a submaximal predischarge test. Because ST depression and elevation appear reproducible, patients who develop these abnormalities during a predischarge test do not, for prognostic reasons, need retesting 6 weeks after infarction. Exercise induced angina pectoris and an abnormal blood pressure response, however, are highly variable and in these patients a repeat test may be useful.