Cor triatriatum: operative results in 25 patients.

Twenty-five patients with cor triatriatum underwent surgical correction at the Texas Heart Institute during a 21-year period from 1959 to 1980. Patients ranged in age from 4 months to 38 years. Diagnosis was established preoperatively in 14 patients (56%), at the time of operation for correction of associated lesions in 10 patients (40%), and during reoperation in 1 (4%). In the earlier part of this series, diagnosis was more common at the time of operation. Associated cardiovascular anomalies were present in 20 patients (80%), major anomalies in 14 others (56%). Simultaneous correction or palliation of associated lesions and total excision of the anomalous membrane were done in 18 patients utilizing temporary cardiopulmonary bypass. Cor triatriatum alone was repaired in 5 patients (20%) with the aid of extracorporeal circulation. Two patients underwent reoperation: 1 because of incomplete excision of the septum and the other because the condition had not been diagnosed during a first operation for correction of total anomalous pulmonary venous return. Excision of the membrane was accomplished utilizing the left atrium in 10 patients (40%), the right atrium in 12 (48%), or both in 3 (12%). Four patients (16%) died early after operation; all were infants who had severe associated cardiac anomalies. Among the 21 surviving patients, results were excellent in 20.

Effect of sodium bicarbonate on canine left ventricular function.

Left ventricular performance was evaluated in seven canine right heart bypass preparations during and after the intraauricular administration of sodium bicarbonate (100 ml, 1100 Osm, 60 mEq). Under conditions of constant cardiac input and left ventricular volume, sodium bicarbonate produced mild and brief left ventricular depression, as manifested by a decline of left ventricular dp/dt with simultaneous rise of left ventricular end-diastrolic and left atrial pressures. These findings occurred as aortic impedance fell. Identical results accrued in paced and unpaced preparations. The data lends credence to the proposition that a sudden rise in blood pCO2 produces increased transport of CO2 across the cardiac membranes with intracellular acidosis. However, the data is also compatible with hyperosmolality induced contractile depression. Possible clinical implications are discussed.

Growth hormone treatment for sustained pain reduction and improvement in quality of life in severe fibromyalgia.

Functional defects in growth hormone (GH) secretion and its efficacy as a complementary treatment have been suggested for fibromyalgia. This study investigated the efficacy and safety of low-dose GH as an add-on therapy in patients with both severe FM and low insulin-like growth factor 1 levels. A total of 120 patients were enrolled in a multicenter, placebo-controlled study for 18 months. They were randomly assigned to receive either 0.006 mg/kg/day of GH subcutaneously (group A, n=60) or placebo (group B, n=60) for 6 months (blind phase). The placebo arm was switched to GH treatment from month 6 to month 12 (open phase), and a follow-up period after GH discontinuation was performed until month 18. Standard treatment for fibromyalgia (selective serotonin re-uptake inhibitors, opioids, and amitriptyline) was maintained throughout the study. Number and intensity of tender points, Fibromyalgia Impact Questionnaire (FIQ) with its subscales, and EuroQol 5 dimensions test (EQ5D) with visual analogue scale (VAS) were assessed at different time points. At the end of the study, 53% of group A patients obtained fewer than 11 positive tender points, vs 33% of group B patients (P<.05). 39.1% vs 22.4% reached more than 50% improvement in VAS (P<.05). Group A patients showed significantly improved FIQ scores (P=.01) compared with group B. Although GH discontinuation worsened all scores in both groups during follow-up, impairment in pain perception was less pronounced in the GH-treated group (P=.05). In this largest and longest placebo-controlled trial performed in FM (NCT00933686), addition of GH to the standard treatment is effective in reducing pain, showing sustained action over time.

High prevalence of growth hormone deficiency in severe fibromyalgia syndromes.

CONTEXT: Fibromyalgia (FM) is characterized by widespread pain and fatigue and is considered a syndrome with different pathogenic mechanisms. Controversial data on GH axis disturbances have been published. Some preliminary trials have shown promising effects of GH therapy on tender points and quality of life in FM. AIM: The aim was to study the patterns of GH secretion/sensitivity in a cohort of severe FM patients. SETTING: The study was conducted in five tertiary hospitals. METHODS: A total of 493 FM women (1990 American College of Rheumatology criteria) recruited from five centers, having more than 16 tender points, Fibromyalgia Impact Questionnaire scores above 75, more than 1 yr of stable medication (serotonin reuptake inhibitors, amitriptyline, and opioids), and body mass index below 35 kg/m(2) underwent baseline IGF-I/GH determinations; an insulin tolerance test (ITT) and a modified IGF-I generation test were performed in those cases showing IGF-I of 150 microg/liter or less. RESULTS: A total of 169 of the 493 patients (34.2%) showed IGF-I of 150 microg/liter or less. Mean peak GH during ITT was 13.3 +/- 9.9 ng/ml in 127 patients in which the test was performed. In 22 of 127 (17.3%), ITT peak GH was 5 microg/ml or less, and in eight of them (6.3%), the peak GH was 3 ng/ml or less. Mean baseline GH (n = 127) was 1.47 +/- 2.58 ng/dl, and eight of 120 (6.8%) showed an insufficient IGF-I response (<50% over baseline) to the IGF-I generation test. CONCLUSION: FM patients show a high prevalence of GH axis dysfunction. A significant number of patients show biochemical patterns of GH deficiency as well as some degree of GH resistance and might be potential candidates for substitution treatment.

Effects of ketamine on canine cardiovascular function.

Left ventricular contractility was assessed before and during the peak effect of ketamine in eight canine right heart bypass preparations. Myocardial contractility was defined in terms of maximum left ventricular dp/dt, the ejection fraction, and left ventricular end-diastolic and left atrial pressures at constant heart rate and cardiac inflow. Ketamine produced significant decreases in contractility and there were some indications of a dose-response pattern. The action of ketamine was dependent partly on changes in arterial pressure, as the drug caused a mild but sustained vasodilatation which was not dose-dependent. These data offer further evidence of a small and transient negative inotropic effect of ketamine.