Acute Vasoreactivity Testing and Outcomes in Pulmonary Arterial Hypertension: A Call for Increased Testing.

BACKGROUND: Pulmonary arterial hypertension (PAH) has a progressive, unremitting clinical course. Vasoreactivity testing (VdT) during right heart catheterisation (RHC) identifies a subgroup with excellent long-term response to calcium channel blockade (CCB). Reporting on these patients is limited. Established in 2011, the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) registry offers the opportunity to assess the frequency of VdT during RHC, treatment and follow up of PAH patients. METHODS: Registry data from 3,972 PAH patients with index RHC revealed 1,194 VdT appropriate patients. Data was analysed in three groups: 1) VdT+CCB+: VdT positive, CCB treated; 2) VdT+CCB-: VdT positive, no CCB prescribed, 3) VdT-/noVdT: VdT negative, or VdT not tested. Data was reviewed for adherence to guidelines, clinical response (World Health Organization functional class [WHO FC], 6-minute-walk-distance [6MWD], RHC), and outcomes (survival or lung transplantation). RESULTS: Patients included had idiopathic (IPAH=1,087), heritable (HPAH=67) and drug or toxin-induced PAH (DPAH=40). A VdT was performed in 22% (268/1,194), with incomplete data in 26% (70/268); 28% (55/198) were VdT+. Analysis group allocation was: VdT+CCB+ (33/55), VdT+CCB- (22/55), VdT- (143)/noVdT (996). From patients with 1-year data VdT+CCB+ and VdT-/noVdT patients improved WHO FC, 6MWD and cardiac index (CI); VdT+CCB- data remained similar. Within the VdT+CCB+ group, 30% (10/33) were long-term CCB responders with a 100% 5-year survival; non-responders had a 61% survival at 5.4 years. Long-term responders were younger at diagnosis (40 yrs vs 54 yrs). CONCLUSION: Use of VdT testing and documentation is poor in this contemporary patient cohort. Nonetheless, survival in VdT+CCB+ patients from the PHSANZ registry is excellent, supporting guidelines promoting VdT testing. Strategies to promote the use of VdT are warranted.

Smoking prevalence increases following Canterbury earthquakes.

BACKGROUND: A magnitude 7.1 earthquake hit Canterbury in September 2010. This earthquake and associated aftershocks took the lives of 185 people and drastically changed residents' living, working, and social conditions. AIM: To explore the impact of the earthquakes on smoking status and levels of tobacco consumption in the residents of Christchurch. METHODS: Semistructured interviews were carried out in two city malls and the central bus exchange 15 months after the first earthquake. A total of 1001 people were interviewed. RESULTS: In August 2010, prior to any earthquake, 409 (41%) participants had never smoked, 273 (27%) were currently smoking, and 316 (32%) were ex-smokers. Since the September 2010 earthquake, 76 (24%) of the 316 ex-smokers had smoked at least one cigarette and 29 (38.2%) had smoked more than 100 cigarettes. Of the 273 participants who were current smokers in August 2010, 93 (34.1%) had increased consumption following the earthquake, 94 (34.4%) had not changed, and 86 (31.5%) had decreased their consumption. 53 (57%) of the 93 people whose consumption increased reported that the earthquake and subsequent lifestyle changes as a reason to increase smoking. CONCLUSION: 24% of ex-smokers resumed smoking following the earthquake, resulting in increased smoking prevalence. Tobacco consumption levels increased in around one-third of current smokers.

Natural history of glucose intolerance in patients with cystic fibrosis: ten-year prospective observation program.

OBJECTIVE: To determine prospectively the long-term natural history of glucose homeostasis in adult patients with cystic fibrosis (CF). STUDY DESIGN: Between 1996 and 2005, a total of 971 modified oral glucose tolerance tests (OGTTs) were performed in 329 patients with CF without recognized CF-related diabetes (CFRD). Patients were classified as having normal glucose tolerance (NGT), impaired glucose tolerance (IGT), CFRD without fasting hyperglycemia (FH), or CFRD with FH. Data were collected at baseline from the Toronto Cystic Fibrosis database. RESULTS: On first OGTT, 63% of the 257 patients with pancreatic insufficiency (PI) had NGT, 23% had IGT, 11% had CFRD without FH, and 3% had CFRD with FH. Burkholderia cepacia complex colonization was correlated with worsening glucose tolerance category. There was a weak inverse relationship among weight, body mass index, forced expiratory volume in 1 minute, and 2-hour plasma glucose obtained during OGTT. Of the 168 PI patients who had a second OGTT, 17% improved their category of glucose tolerance, 70% remained unchanged, and 13% worsened. A similar trend was seen during the progression between any one test to a subsequent test. CONCLUSIONS: Annual screening of glucose tolerance in patients with CF reveals highly variable results over time. Fluctuating levels of insulin resistance, probably with variable degrees of ongoing inflammation, affect the results and hinder prediction of future development of CFRD. Home glucose monitoring following abnormal OGTT results was essential in establishing the diagnosis of CFRD.

Retrospective Validation of the REVEAL 2.0 Risk Score With the Australian and New Zealand Pulmonary Hypertension Registry Cohort.

BACKGROUND: Pulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies; however, the long-term outlook remains poor. Objective multiparametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score is a new model proposed for the follow-up of patients with PAH but has not been externally validated. METHODS: The REVEAL 2.0 risk score was applied to a mixed prevalent and incident cohort of patients with PAH (n = 1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier survival was estimated for each REVEAL 2.0 risk score strata and for a simplified three-category (low, intermediate, and high risk) model. Sensitivity analysis was performed on an incident-only cohort. RESULTS: The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ Registry cohort, with a C statistic of 0.74 (both for full eight-tier and three-category models). When applied to incident cases only, the C statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12- and 60-month survival estimates (all risk category comparisons P < .001). Although the full eight-tier REVEAL 2.0 model separated patients at low, intermediate, and high risk, survival estimates overlapped within some of the intermediate- and high-risk strata. CONCLUSIONS: The REVEAL 2.0 risk score was validated in a large external cohort from the PHSANZ Registry. The REVEAL 2.0 model can be applied for risk assessment of patients with PAH at follow-up. The simplified three-category model may be preferred for clinical use and for future comparison with other prognostic models.

Empyema and psoas abscess in a previously undiagnosed diabetic patient.

A 48-year-old man presented with a 2-month history of polyuria, polydypsia, chest pain, fever, cough and extreme weight loss. He was diagnosed with diabetic ketoacidosis and investigations revealed widespread infection with an empyema complicated by bronchopleural fistula, and iliopsoas, suprapubic and periarticular abscesses. Streptococcus milleri was cultured from all sites. A multidisciplinary medical and surgical approach was required for treatment. This case highlights the immunosuppression, and life-threatening complications arising from undiagnosed diabetes mellitus.

Prevalence of dyslipidemia in adults with cystic fibrosis.

BACKGROUND: A high fat calorie diet is advocated for patients with cystic fibrosis (CF) however the lipid profiles of individuals with CF, including those with CF-related diabetes (CFRD), are not well studied. METHODS: We conducted a retrospective review of adult CF patients attending St Michael's Hospital between January 2005 and December 2007. RESULTS: 334 patients (77% pancreatic insufficient (PI)) were included in the study. Mean HDL cholesterol was significantly lower in males (p<0.0001) with 44% of males having HDL cholesterol <38.7mg/dL(1mmol/L). Pancreatic sufficient patients were more likely than PI subjects to have total cholesterol >201mg/dL(5.2mmol/L) (p<0.01). 5% of subjects had triglyceride concentrations >195mg/dL(2.2mmol/L). Diabetes was diagnosed in 23% of subjects. Lipid profiles were similar between diabetics and non-diabetics. Total cholesterol and triglycerides both increased with increasing age and increasing BMI (p<0.01). CONCLUSION: Dyslipidemia occurs in CF patients however no differences in lipid profiles were seen between those with diabetes and those without. Fasting lipids should be monitored in CF patients, particularly those with PS, older age, and high BMI. As survival in CF increases, the prevalence of dyslipidemia may increase resulting in clinically important complications.

Interference of chlorofluorocarbon (CFC)-containing inhalers with measurements of volatile compounds using selected ion flow tube mass spectrometry.

Selected ion flow tube mass spectrometry (SIFT-MS) is a sensitive technique capable of measuring volatile compounds (VCs) in complex gas mixtures in real time; it is now being applied to breath analysis. We investigated the effect of inhalers containing chlorofluorocarbons (CFCs) on the detection and measurement of haloamines in human breath. SIFT-MS mass scans (MS) and selected ion monitoring (SIM) scans were performed on three healthy non-smoking volunteers before and after inhalation of the following medications: Combiventtrade mark metered-dose inhaler (MDI) (CFC-containing); Ventolintrade mark MDI (CFC-free); Atroventtrade mark MDI (CFC-free), Beclazonetrade mark MDI (CFC-containing); Duolintrade mark nebuliser. In addition, the duration of the persistence of the mass/charge ratios was measured for 20 h. Inhalers containing CFCs generated large peaks at m/z 85, 87, 101, 103 and 105 in vitro and in vivo, consistent with the predicted product ions of CFCs 12, 114 and 11. No such peaks were seen with Duolintrade mark via nebuliser, or CFC-free MDIs. We conclude that measurement of VCs, such as haloamines, with product ions of similar m/z values to the ions found for CFCs would be significantly affected by the presence of CFCs in inhalers. This issue needs to be accounted for prior to the measurement of VCs in breath in people using inhalers containing CFCs.

Heathrow to ICU direct.

We present the case of a witnessed massive pulmonary embolism (PE) resulting in pulseless electrical activity (PEA) following a long-haul flight. Our patient was successfully treated with cardiopulmonary resuscitation (CPR) and thrombolysis in the Emergency Department at Christchurch Hospital and went on to complete her honeymoon in New Zealand.