Orthopnea and pulmonary hypertension. Treat the underlying disease.

We report the case of a patient with longstanding dyspnea upon reclining, who presented with severe right heart failure due to chronic alveolar hypoventilation. Bilateral diaphragmatic paresis was discovered. She recovered from her right ventricular failure with non-invasive mechanical ventilatory support.

Comparison of ventilation-perfusion single-photon emission computed tomography (V/Q SPECT) versus dual-energy CT perfusion and angiography (DECT) after 6 months of pulmonary embolism (PE) treatment.

BACKGROUND: The natural evolution of treated symptomatic pulmonary embolism shows often incomplete resolution of pulmonary thrombi. The prevalence of perfusion defects depend on the image modality used. This study directly compares V/Q SPECT with DECT. METHODS: A single-center prospective observational cohort study of patients with intermediate risk PE, reassessed at the end of treatment with V/Q SPECT. Abnormal V/Q SPECT images were compared with DECT. RESULTS: We compared DECT en V/Q SPECT in 28 consecutive patients with persistent V/Q mismatch on V/Q SPECT, 13 men and 15 woman, mean age 60 (+17), range 23-82 year. One patient was excluded from the final analysis due to inferior quality DECT. In 18/27 (66.7%) the results were concordant between CTPA (persistent embolus visible), DECT (segmentary defects on iodine map) and V/Q SPECT (segmentary ventilation-perfusion mismatch). In 3/18 (11.1% of the total group) the partialy matched V/Q SPECT defect could be explained on DECT lung images by lung infarction. In 6/27 (22.1%) only hypoperfusion was seen on DECT iodine map. In 3/27 (11.1%) results were discordant between V/Q SPECT and DECT images. CONCLUSION: Six months after diagnosis of first or recurrent PE, residual pulmonary perfusion-defects encountered on V/Q-SPECT corresponds in the majority of patients with chronic thromboembolic disease seen on DECT. In 22.1% of patients V/Q SPECT mismatch only corresponds with hypoperfusion on iodine map DECT scan. Some (11.1%) of the chronic thromboembolic lesions seen on V/Q SPECT can not be explained by DECT results.

Phase II study of stereotactic body radiotherapy to primary tumor and metastatic locations in oligometastatic nonsmall-cell lung cancer patients.

BACKGROUND: Stereotactic body radiotherapy (SBRT) has emerged as a treatment modality in patients presenting with oligometastatic nonsmall-cell lung cancer (NSCLC). SBRT is used as a local consolidative treatment to metastatic disease sites. The majority of patients included in SBRT trials for oligometastatic NSCLC have controlled primary tumors and brain metastases. PATIENTS AND METHODS: Oligometastatic NSCLC patients with ≤5 metastatic lesions were included in a prospective phase II trial to evaluate efficacy and toxicity of SBRT to all disease sites, primary tumor and metastatic locations. SBRT to a dose of 50 Gy in 10 fractions was delivered. Positron emission tomography-computed tomography (PET-CT) was carried out at baseline and 3 months after SBRT to evaluate the metabolic response rate according to PET Response Criteria in Solid Tumors (PERCIST). The progression-free survival (PFS) and overall survival (OS) were calculated using Kaplan-Meier method from start of chemotherapy or radiotherapy. Side-effects were scored using the National Cancer Institute Common Terminology Criteria for Adverse Events (NCI CTCAE) version 3.0. RESULTS: Twenty-six patients received SBRT after induction chemotherapy (n = 17) or as a primary treatment (n = 9). Median follow-up was 16.4 months. Overall metabolic response rate was 60% with seven patients (30%) achieving a complete metabolic remission and 7 (30%) a partial metabolic response. Any acute grade 2 toxicity was observed in four patients (15%) and grade 3 pulmonary toxicity in two patients (8%). Median PFS and OS were 11.2 and 23 months. The 1-year PFS and 1-year OS rate were 45% and 67%, respectively. CONCLUSION: SBRT to all disease sites, primary tumor and metastatic locations, in oligometastatic NSCLC patients produced an acceptable median PFS of 11.2 months.

Severe right heart failure in a patient with chronic obstructive lung disease: a diagnostic challenge.

A 55-year-old male was admitted for evaluation of severe dyspnoea and hypoxaemia. Physical examination upon admission showed elevated jugular venous pressure and an accentuated second heart sound. Chest radiograph showed cardiomegaly with increased bibasilar markings. Arterial blood gas analysis while breathing room air showed marked hypoxaemia. High resolution computed tomography angiography of the chest showed modestly enlarged mediastinal lymph nodes with discrete diffuse ground-glass attenuation especially at the lower lung zones. Positron emission tomography using 18F labelled 2-deoxy-D-glucose (FDG) demonstrated the mediastinal lymph nodes were FDG-avid. Transthoracic echocardiography showed dilated hypokinetic right heart chambers with bulging of the interventricular septum to the left, compatible with acute cor-pulmonale. From the tricuspid regurgitation jet measurement a systolic pulmonary artery pressure (PAP) of 48 mmHg was estimated. Patent foramen ovale was suspected on bubble test. Right heart catheterisation confirmed pulmonary arterial hypertension: mPAP 47 mmHg, pulmonary artery occlusion pressure 5 mmHg, cardiac index 1.1 L/min/m2, pulmonary vascular resistance (PVR) 959 dyne.sec.cm(-5). Pulmonary function tests showed a marked diffusing capacity for carbon monoxide (DLCO) decrease of 32% predicted but no obstructive lung deficit. Before an open lung biopsy could be scheduled the patient developed acute cardiogenic shock. At autopsy pulmonary veno-occlusive disease with marked pulmonary hypertension was diagnosed.

A not so healthy muesli: a case report.

We present a patient with severe anaphylaxis, angioedema, hypotension and shock. The near fatal allergic reaction was caused by eating yogurt with muesli containing pine nuts. The patient developed an acute infero-posterolateral myocardial infarction due to systemic hypotension and shock. Food allergy to pine nut was demonstrated by dosage of specific IgE to pine nut.

Complete metabolic tumour response, assessed by 18-fluorodeoxyglucose positron emission tomography (18FDG-PET), after induction chemotherapy predicts a favourable outcome in patients with locally advanced non-small cell lung cancer (NSCLC).

BACKGROUND: 18FDG-PET and multislice computerized axial tomography (CT) scan are used for diagnosis, staging and response evaluation in NSCLC patients. The correlation between the response assessment by both imaging techniques and survival was assessed in patients with unresectable stage III NSCLC treated with induction chemotherapy followed by consolidation radiotherapy. METHODS: Thirty-one patients, enrolled in a phase II study evaluating the efficacy and toxicity of a novel triplet induction chemotherapy (paclitaxel, carboplatin and gemcitabine) (PACCAGE) before consolidation radiotherapy, were evaluated by CT and 18FDG-PET at baseline and after three cycles of chemotherapy. The correlation between CT and 18FDG-PET response and time to progression and overall survival was analyzed using the Kaplan-Meier estimates of survival and the log rank test. RESULTS: Ten patients with a complete response (CR) on 18FDG-PET had a significantly longer time to progression and overall survival than patients with a non-CR (median 19.9 months versus 9.8 months, p=0.026, and median >49 months versus 14.4 months, p=0.004, respectively). Twenty patients with a partial CT response (PR) had a significantly longer time to progression (median 15 months versus 9.4 months, p=0.001) than patients with a non-PR but the difference in overall survival only showed a trend (23.3 months versus 14.4 months, p=0.093). CONCLUSIONS: A CR on 18FDG-PET following induction chemotherapy for locally advanced, unresectable NSCLC seems to be a more powerful prognostic marker for survival compared to PR on CT.

Primary tracheobronchial amyloidosis: a series of 3 cases.

Primary tracheobronchial amyloidosis is a form of localized pulmonary amyloidosis, characterised by the deposition of AL-amyloid in trachea and bronchi. It is a rare and slowly progressive disease, usually requiring repeated endoscopic treatment. In this case series we describe symptoms, diagnostic and therapeutic procedures, radiological findings and pulmonary function testing in 3 cases of different presentation and severity. Two patients were treated by endoscopic debulking and stent placement during rigid bronchoscopy, both with excellent clinical and functional results. In one of these patients regular endoscopic and clinical control exams were performed in the 5 years following the initial treatment, showing stable disease, requiring no further therapeutic intervention until today.

A novel triplet regimen with paclitaxel, carboplatin and gemcitabine (PACCAGE) as induction chemotherapy for locally advanced unresectable non small cell lung cancer (NSCLC).

UNLABELLED: Phase II study of 3 cycles of triplet induction chemotherapy (response, toxicity) followed by radiotherapy in locally advanced non small cell lung cancer (NSCLC). BACKGROUND: Patients with locally advanced inoperable non-small cell lung cancer are currently treated with concomitant or sequential chemotherapy and radiotherapy. However, the outcome of existing treatment modalities is unsatisfactory. Development of new strategies including more efficient systemic chemotherapy is warranted. OBJECTIVE: To study the antitumour activity and toxicity of a triplet combination of paclitaxel, carboplatin and gemcitabine as induction chemotherapy before radiotherapy, in locally advanced NSCLC and to evaluate time to progression and survival. METHODS: Three cycles of paclitaxel (175 mg/m(2) by 3h infusion on day 1), carboplatin (AUC 5mg/(mlmin) by IV bolus on day 1) and gemcitabine (1000 mg/m(2) by IV bolus on day 1 and 8) were administered every 3 weeks in reasonably fit patients. Fractionated radiotherapy with curative intent was initiated 4 weeks after the last chemotherapy administration. Toxicity was assessed weekly during cycle 1 and on day 1 and 8 in cycles 2 and 3. Response evaluation was performed at the end of cycle 3. RESULTS: Forty-eight patients (20 stage IIIA and 28 stage IIIB) received a total of 134 cycles of chemotherapy. Forty-two patients received the intended 3 cycles. Thirty patients obtained an objective response (1 complete and 29 partial response) or 62.5% on the intent to treat analysis (95% confidence interval: 49-76%). None of the responders became eligible for surgery. The median time to progression and survival for all patients was 10.1 and 15.7 month, respectively. A significant difference was observed in survival parameters between stage IIIA and stage IIIB patients. Haematological toxicity grade 3/4, mainly neutropenia and thrombocytopenia, was most prominent on day 15 of the treatment cycles. Haematological support by means of recombinant erythropoietin, red blood cell or platelet transfusion, filgrastim administration or a combination was needed in 21 patients. None of the patients discontinued chemotherapy because of haematotoxicity. Grade 3/4 non-haematological toxicity leading to chemotherapy withdrawal occurred early during induction (2 and 1 in cycles 1 and 2, respectively). CONCLUSION: Three cycles of the novel triplet combination of paclitaxel, carboplatin and gemcitabine (PACCAGE) is an active and feasible induction regimen for patients with locally advanced inoperable NSCLC. Neutropenia and to a lesser extent thrombocytopenia represent the main haematological toxicity. Whether this triplet regimen can improve outcome when compared to specific cisplatin doublet regimens should be evaluated in a phase III study.

Stenting allows weaning and extubation in ventilator- or tracheostomy dependency secondary to benign airway disease.

Central airway obstruction can cause severe respiratory insufficiency leading to mechanical ventilation (MV) or artificial airway (AA) dependency. Interventional bronchoscopic procedures have been reported to be of help in weaning patients with malignant airway stenoses from mechanical ventilation, whereas their use in benign disease is only anecdotal. The objectives of this study are to evaluate early, intermediate and long-term outcome of interventional bronchoscopy and stent placement in the treatment of MV/AA dependency due to benign airway obstruction. In a retrospective cohort analysis for the period 1999-2004, we evaluated 15 consecutive ICU patients with documented benign central airway obstruction, who were referred for bronchoscopic management of their condition after multiple failed attempts at weaning from MV or decannulation of the AA. Indications for bronchoscopic treatment were surgery refusal, medical or surgical inoperability, or absence of alternative treatment options. Malacia, post-intubation stenosis and goiter were the main causes of airway obstruction and MV/AA dependency. All patients were treated by means of rigid bronchoscopy, dilatation procedures and stent insertion. All but one patient (93.3%) were successfully and permanently extubated/decanulated immediately after the bronchoscopy. Minor complications occurred in 6 patients (40%) leading to a second intervention. All complications could be managed endoscopically and long-term follow up was uneventful. Interventional bronchoscopy with stent insertion can allow successful withdrawal from MV/AA and can offer longstanding airway patency in selected ventilator- or tracheostomy-dependant individuals with benign airway stenoses,when surgery in not feasable or contra-indicated.

Music: a new cause of primary spontaneous pneumothorax.

Most cases of primary spontaneous pneumothorax are thought to be caused by air leaks at so-called "emphysema-like changes" or in areas of pleural porosity at the surface of the lung. Environmental pressure swings may cause air leaks as a result of transpulmonary pressure changes across areas of trapped gas in the distal lung. This is the first report of music as a specific form of air pressure change causing pneumothorax (five episodes in four patients). While rupture of the interface between the alveolar space and pleural cavity in these patients may be linked to the mechanical effects of acute transpulmonary pressure differences caused by exposure to sound energy in association with some form of distal air trapping, we speculate that repetitive pressure changes in the high energy-low frequency range of the sound exposures is more likely to be responsible. Exposure to loud music should be included as a precipitating factor in the history of patients with spontaneous pneumothorax.

Effects of formoterol (Oxis Turbuhaler) and ipratropium on exercise capacity in patients with COPD.

Although long-acting inhaled beta 2-agonists improve various outcome measures in COPD, no double-blind study has yet shown a significant effect of these drugs on exercise capacity. In a randomized, double-blind, placebo-controlled, crossover study, patients received formoterol (4, 5, 9, or 18 micrograms b.i.d. via Turbuhaler), ipratropium bromide (80 micrograms t.i.d. via pMDI with spacer), or placebo for 1 week. Main endpoint was time to exhaustion (TTE) in an incremental cycle ergometer test. Secondary endpoints were Borg dyspnoea score during exercise, lung function, and adverse events. Thirty-four patients with COPD were included, mean age 64.8 years, FEV1 55.6% predicted, reversibility 6.1% predicted. All doses of formoterol, and ipratropium significantly improved TTE, FEV1, FEF25-75%, FRC, IVC, RV and sGAW compared with placebo. A negative dose-response relationship was observed with formoterol. Ipratropium increased time to exhaustion more compared with formoterol, 18 micrograms, but not with formoterol, 4.5 and 9 micrograms. No changes in Borg score were found. There was no difference in the adverse event profile between treatments. In conclusion, 1 week of treatment with formoterol and ipratropium significantly improved exercise capacity and lung function compared with placebo. However, a negative dose-response relation for formoterol was unexpected and needs further investigation.

Bronchoscopic cryotherapy: preliminary experience.

Our preliminary experience with the bronchoscopic application of cryotherapy using rapid decompression of liquid nitrous oxide as cooling agent is reported. Seventeen applications through rigid bronchoscopy in twelve patients were performed. A single cryotherapy session was successful in the debulking of obstructive malignant lesions of the central airways in five patients (four non-small cell carcinoma, one renal cell cancer metastasis), and in the treatment of a capillary haemangioma (one patient). Two sessions were successful in the treatment of a metastatic melanoma (one patient) and benign granulation tissue (one patient). Cryotherapy was also successful in the treatment of early bronchial cancer (carcinoma in situ) in four patients, requiring repetitive sessions in two. There were no complications or side-effects. These preliminary findings confirm the safety and efficacy of bronchoscopic cryotherapy in a variety of airway lesions.

Real time CT-fluoroscopy: diagnostic and therapeutic applications.

The synergetic progression of CT technology and computer hardware has made ultrafast acquisition and image reconstruction possible. This has lead to the availability of CT interactive diagnosis and therapeutic procedures. Making use of our own material (337 intervention procedures during the last 17 months), we have compared our techniques and results to the recent literature data. One of the advantages of the biopsy technique is an improved sensitivity for neoplastic lesions, most certainly in cases of intrapulmonary lesions, surrounded by aerated tissue (now 94% compared to 87% in our previous study). A second advantage is the safety of the technique (only one major complication in our series). Fluid collection drainages, and more complex interventions like local injection of drugs, radio-frequency ablation, wire hook placement and ethanol injection were performed without complication. Yet another interesting feature is the shortening of the procedure time (reduced in average to an 'in-room' time of less than 30 min), which has definite economical implications. Furthermore it increases the patient's comfort and safety, and extends the scope of outpatient procedures (80% outpatient procedures in our material). On the other side the radiation exposure can be raised as an issue, especially when we consider the operator's hands. However, the described technique and the use of dedicated tools can alleviate the problem. As a conclusion, real time CT fluoroscopy has given a new input and broadens the scope of clinical indications of CT-guided diagnostic and therapeutic procedures.

Rib fractures induced by coughing: an unusual cause of acute chest pain.

We report three patients with stress fractures of the ribs induced by coughing. Standard radiographs of the chest and ribs did not reveal evidence of rib fractures in any of the patients. Bone scintigraphy, performed 1 to 2 weeks after initial onset of symptoms, showed a focal area of increased uptake along the chest wall in all cases. Thin section angulated helical CT directly visualized the subtle rib fractures. Initial diagnosis of a cough-induced fracture of the rib may be difficult because of the associated underlying disorder, and unnecessary examinations are commonly performed. Identification of a cough-induced fracture of the rib using helical CT may be clinically important to avoid unnecessary concern and additional examinations.