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ObjectiveTo investigate whether infusion of adrenomedullin, a potent vasorelaxant peptide, has beneficial haemodynamic and hormonal effects in patients with pulmonary hypertension.Patients and designThe haemodynamic and hormonal responses to intravenous infusion of adrenomedullin (0.05 μg/kg/min) or placebo were examined in 13 patients with precapillary pulmonary hypertension.ResultsInfusion of adrenomedullin produced a 44% increase in cardiac index (mean (SD) 1.8 (0.2) to 2.6 (0.3) l/min/m², p < 0.05) and a 32% decrease in pulmonary vascular resistance (19.7 (1.4) to 13.4 (1.3) units, p < 0.05), with a 4% reduction in mean pulmonary arterial pressure (62 (4) to 59 (4) mm Hg, NS). Adrenomedullin also decreased mean systemic arterial pressure (81 (3) to 72 (4) mm Hg, p < 0.05) and increased heart rate (73 (4) to 79 (4) beats/min, p < 0.05). Adrenomedullin decreased plasma aldosterone (9.8 (2.5) to 7.1 (1.5) ng/dl, p < 0.05) without significant changes in plasma renin activity. Plasma atrial and brain natriuretic peptides tended to decrease with adrenomedullin, although these changes did not reach significance. The haemodynamic and hormonal variables remained unchanged during placebo infusion.ConclusionsIntravenous adrenomedullin has beneficial haemodynamic and hormonal effects in patients with precapillary pulmonary hypertension.

ObjectiveTo assess haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome.DesignRetrospective observational study.SettingTertiary referral centre.Patients94 adult patients with Eisenmenger syndrome who were diagnosed between September 1982 and July 1998.Main outcome measuresSerum uric acid was measured in all patients, together with clinical and haemodynamic variables related to mortality.ResultsSerum uric acid was raised in patients with Eisenmenger syndrome compared with age and sex matched control subjects (7.0 v 4.7 mg/dl, p < 0.0001) and increased in proportion to the severity of New York Heart Association functional class. Serum uric acid was positively correlated with mean pulmonary arterial pressure (r = 0.30, p = 0.0052) and total pulmonary resistance index (r = 0.55, p < 0.0001), and negatively correlated with cardiac index (r = -0.50, p < 0.0001). During a mean follow up period of 97 months, 38 patients died of cardiopulmonary causes. Among various clinical, echocardiographic, and laboratory variables, serum uric acid remained predictive in multivariate analysis. Kaplan-Meier survival curves based on median serum uric acid showed that patients with high values had a significantly worse survival rate than those with low values (log-lank test: p = 0.0014 in male patients, p = 0.0034 in female patients).ConclusionsSerum uric acid increases in proportion to haemodynamic severity in adult patients with Eisenmenger syndrome and is independently associated with long term mortality.

To assess whether left ventricular myocardial metabolism is affected by the presence of right ventricular pressure overload, we performed 123I-β-methyl-iodophenyl pentadecanoic acid (123I-BMIPP) and 99Tcm-sestamibi (99Tcm-MIBI) single photon emission tomography in 24 patients with pulmonary hypertension and in 10 control subjects. The left ventricle was divided into six regions using a short-axis tomogram at the mid-ventricular level. The relative regional uptake (RRU, ) was determined for each region as the ratio of the regional uptake per pixel to the maximum uptake in the six regions. Right heart catheterization was performed to obtain mean pulmonary arterial pressure. 123I-BMIPP uptake in the septum and the adjacent myocardium of the left ventricle was lower in patients with pulmonary hypertension than in control subjects (septal RRU, 75 $PT 6 vs85 $PT 4; anteroseptal RRU, 76 $PT 6 vs86 $PT 3; posteroseptal RRU, 75 $PT 4 vs84 $PT 4; P< 0.001). The reduction in 99Tcm-MIBI uptake was confined to the septum in patients with pulmonary hypertension (79 $PT 5 vs85 $PT 5; P< 0.05). Interestingly, the septal RRU of 123I-BMIPP and the sum of the septal, anteroseptal and posteroseptal RRUs of 123I-BMIPP were negatively correlated with mean pulmonary arterial pressure (r= −0.70 and −0.55, respectively; P< 0.01). In conclusion, myocardial metabolism in the interventricular septum and the adjacent regions of the left ventricle may be impaired in proportion to the degree of pulmonary hypertension.

Chlorambucil (CHL) was used in combination with prednisolone in the treatment of nine children with frequently relapsing nephrotic syndrome. Serial electroencephalograms were obtained to evaluated CHL central nervous toxicity, before, during and after treatment with this agent. EEG abnormalities were observed in two of the nine children during chlorambucil therapy. EEG changes were diffuse spike and wave complexes and disappeared after discontinuation of therapy. There were no other neurological abnormalities and more particularly, no seizures or myocloni were observed. According to the literature, chlorambucil central nervous toxicity is found almost exclusively in childhood nephrotic syndrome. Strict neurological supervision of patients treated with chlorambucil is recomended.

Abstract.: An 11-month-old girl with congenital heart disease developed infective endocarditis. Blood cultures revealed an unusual gram-negative rod, Rahnella aquatilis. The patient was successfully treated with a combination of netilmicin and ceftazidime. This is the first case report of infective endocarditis caused by this organism. R. aquatilis should be recognized as a clinical pathogen capable of causing life-threatening infection in children and adults.