Lung Function after the Minimal Invasive Pectus Excavatum Repair (Nuss Procedure)

Background The Nuss procedure was introduced at our center in 1999. The operation was mainly performed for cosmesis. Little information is available regarding the influence of this operation on lung function. Methods The aim of this study, a prospective analysis, was to analyze the effect of the Nuss procedure on lung function variables. Between 1999 and 2007 a total of 203 patients with pectus excavatum were treated with the Nuss procedure, of whom 145 (104 male, 41 female) were located at Emma Children’s Hospital. In the latter subset of consecutive patients, static lung function variables [total lung capacity (TLC), functional residual capacity (FRC), vital capacity (VC)] and dynamic lung function variables [forced expired volume in 1 s (FEV1), maximum expiratory flow (MEF50)] were performed using spirometry and body box measurements at four time points: prior to operation Some of these data were presented at the International Surgical Week

Atrial septal defect in adults is associated with airway hyperresponsiveness

Objective: The association between secundum atrial septal defects (ASD) and asthma-like dyspnea with consequent long-term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short-and long-term postprocedural follow-up. Methods: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and bronchoprovocation testing pre-and six-month postprocedurally, with additional bronchoprovocation at 2-year follow-up. Airway hyperresponsiveness was defined as ≥20% fall of forced expiratory volume in 1-second (FEV1) following <8.0 mg/mL of inhaled methacholine. Results: Airway hyperresponsiveness was found in 19/30 patients (63%[95%CI 45%-81%]; post hoc statistical power = 89%). Asthma-like symptoms wheezing, chest tightness, and cough were more frequently reported in airway hyperresponsive patients. Airway responsiveness was not influenced by successful percutaneous ASD closure, corresponding to persistence of asthma-like symptoms postclosure. Regardless of airway responsiveness, postprocedural right-sided reverse remodeling significantly improved dyspnea and pulmonary function. Conclusions: This study is the first to report a high prevalence of airway hyperresponsiveness in a cohort of unrepaired adult ASD patients, and confirms the association between asthma-like symptoms and ASD in adults. Attention to symptoms and pulmonary function should be given during clinical follow-up of adult ASD patients, both before and long after repair

Pulmonary Endarterectomy Improves Dyspnea by the Relief of Dead Space Ventilation

Background. In chronic thromboembolic pulmonary hypertension (CTEPH), dyspnea is considered to be related to increased dead space ventilation caused by vascular obstruction. Pulmonary endarterectomy releases the thromboembolic obstruction, thereby improving regional pulmonary blood flow. We hypothesized that pulmonary endarterectomy reduces dead space ventilation and that this reduction contributes to attenuation of dyspnea symptoms. Methods. In this follow-up study we assessed dead space ventilation, hemodynamic severity of disease, and symptomatic dyspnea in 54 consecutive CTEPH patients, before and 1 year after pulmonary endarterectomy. Dead space ventilation was calculated using the Bohr-Enghoff equation. Dyspnea was assessed by Borg scores and the New York Heart Association functional classification. Results. Preoperatively, dead space ventilation was increased (0.40 +/- 0.07) and correlated with severity of disease (mean pulmonary artery pressure: r = 0.49, p <0.001; total pulmonary resistance: r = 0.53, p <0.001), and resting (r = 0.35, p <0.05) and post-exercise Borg dyspnea scores (r = 0.44, p <0.01). Postoperatively, dead space ventilation (0.33 +/- 0.08, p <0.001) and dyspnea symptoms decreased significantly. Changes in symptomatic dyspnea were independently associated with changes in pulmonary hemodynamics and absolute dead space. Conclusions. Dead space ventilation in CTEPH is increased and correlates significantly with hemodynamic severity of disease and dyspnea symptoms. Pulmonary endarterectomy decreases dead space ventilation. The induced change in dead space upon surgical removal of chronic thromboembolism contributes to the postoperative recovery of symptomatic dyspnea. (Ann Thorac Surg 2010;89:347-52) (C) 2010 by The Society of Thoracic Surgeon

Six-minute walk distance as parameter of functional outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension

OBJECTIVES: In chronic thromboembolic pulmonary hypertension, objective data to assess the functional outcome after pulmonary endarterectomy are lacking. We studied the 6-minute walk distance in relation to the clinical and hemodynamic severity of disease, and assessed the effect of pulmonary endarterectomy on the 6-minute walk distance. METHODS: A total of 50 consecutive patients with chronic thromboembolic pulmonary hypertension were studied. Subsequently, pulmonary endarterectomy was performed in 42 patients, 35 of whom underwent a 6-minute walk distance 1 year after surgery. RESULTS: The mean +/- standard error of the mean 6-minute walk distance was 391 +/- 19 m. The 6-minute walk distance decreased in proportion to New York Heart Association functional class and correlated (all P < .0001) with mean pulmonary artery pressure (r = -0.62), cardiac output (r = 0.76), total pulmonary resistance (r = -0.75), mixed venous oxygen saturation (r = 0.77), and brain natriuretic peptide (r = -0.65). One year after pulmonary endarterectomy, the 6-minute walk distance increased from 417 +/- 19 m to 517 +/- 16 m (P < .0001). The change from baseline in 6-minute walk distance correlated with the changes after 1 year in New York Heart Association functional class (P < .01) and brain natriuretic peptide (r = 0.57, P < .002), and with the observed hemodynamic changes directly after pulmonary endarterectomy (change in mean pulmonary artery pressure: r = 0.52; change in cardiac output: r = 0.70; change in total pulmonary resistance r = 0.70; all P < .001). In patients with residual pulmonary hypertension after pulmonary endarterectomy, the 6-minute walk distance was significantly lower than in hemodynamically normalized patients. However, the absolute increase in the 6-minute walk distance was higher in patients with residual pulmonary hypertension (137 +/- 26 m and 82 +/- 20 m, respectively; P = .03). CONCLUSIONS: The 6-minute walk distance was demonstrated to reflect the clinical and hemodynamic severity of disease in patients with chronic thromboembolic pulmonary hypertension. One year after pulmonary endarterectomy, the 6-minute walk distance had increased significantly, and the change in the 6-minute walk distance correlated with the observed clinical and hemodynamic improvemen

Exercise tolerance, lung function abnormalities, anemia, and cardiothoracic ratio in sickle cell patients

Many patients with sickle cell disease (SCD) have a reduced exercise capacity and abnormal lung function. Cardiopulmonary exercise testing (CPET) can identify causes of exercise limitation. Forty-four consecutive SCD patients (27 HbSS, 11 HbSC, and 6 HbS-beta thalassemia) with a median age (interquartile range) of 26 (21-41) years underwent pulmonary function tests, CPET, chest x-ray, and echocardiography to further characterize exercise limitation in SCD. Peak oxygen uptake (V'O-2-peak), expressing maximum exercise capacity, was decreased in 83% of the studied patients. V'O-2-peak correlated with hemoglobin levels (R = 0.440, P = 0.005), forced vital capacity (FVC) (R = 0.717, P <0.0001). Cardiothoracic ratio on chest x-ray inversely correlated with FVC (R = -0.637, P <0.001). According to criteria for exercise limitation, the patients were limited in exercise capacity due to anemia (n = 17), cardiovascular dysfunction (n = 2), musculoskeletal function (n = 10), pulmonary ventilatory abnormalities (n = 1), pulmonary vascular exercise limitation (n = 1), and poor effort (n = 3). In the present study we demonstrate that anemia is the most important determinant of reduced exercise tolerance observed in SCD patients without signs of pulmonary hypertension. We found a strong correlation between various parameters of lung volume and cardiothoracic ratio and we hypothesize that cardiomegaly and relative small chest size may be important causes of the impairment in pulmonary function, that is, reduced long volumes and diffusion capacity, in SCD. Taking into account anthropomorphic differences between SCD patients and controls could help to interpret lung function studies in SCD better. (C) 2014 Wiley Periodicals, In

Hemodynamic and ventilatory responses during exercise in chronic thromboembolic disease

Background: Patients with chronic thromboembolic disease (CTED) may suffer from exercise intolerance without pulmonary hypertension at rest. Pulmonary endarterectomy (PEA) for symptomatic CTED results in improvement of symptoms and quality of life. Neither the pathophysiology of the exercise limitation nor the underlying mechanisms of the PEA-induced improvement have been studied previously. Objectives: We studied hemodynamic and ventilatory responses upon exercise in 14 patients with CTED. After 1 year, we studied the underlying physiologic mechanisms of the PEA-induced symptomatic improvement. Methods: Cardiopulmonary exercise testing (CPET) was performed during right heart catheterization, and noninvasive CPET was performed 1 year postoperatively. Results: During exercise, we observed abnormal pulmonary vascular responses, that is, a steep mean pulmonary artery pressure/cardiac output (2.7 +/- 1.2 mm Hg.min.L-1), and low pulmonary vascular compliance (2.8 +/- 1.1 mL.mm Hg-1); mean pulmonary artery pressure/cardiac output slope correlated with dead-space ventilation (r = 0.586; P =.028) and ventilatory equivalents for carbon dioxide slope (r =.580; P =.030). Postoperatively, the improvement observed in exercise capacity was related to improvements in CPET-derived parameters pointing to restoration of right ventricle stroke volume response (oxygen pulse: 11.7 +/- 3.1 to 13.3 +/- 3.3; P = .027; heart rate response: 80.9 +/- 12.4 to 72.0 +/- 5.7; P = .003); and, indicating improved ventilatory efficiency, the ventilatory equivalents for carbon dioxide slope decreased from 38.2 +/- 3.6 to 32.8 +/- 7.0 (P = .014). Conclusions: Patients with CTED showed an abnormal pulmonary vascular response to exercise and a decreased ventilatory efficiency. Responses after PEA point to restoration of right ventricle stroke volume response and ventilatory efficienc

Airway inflammation and mannitol challenge test in COPD

Abstract Background Eosinophilic airway inflammation has successfully been used to tailor anti-inflammatory therapy in chronic obstructive pulmonary disease (COPD). Airway hyperresponsiveness (AHR) by indirect challenges is associated with airway inflammation. We hypothesized that AHR to inhaled mannitol captures eosinophilia in induced sputum in COPD. Methods Twenty-eight patients (age 58 ± 7.8 yr, packyears 40 ± 15.5, post-bronchodilator FEV1 77 ± 14.0%predicted, no inhaled steroids ≥4 wks) with mild-moderate COPD (GOLD I-II) completed two randomized visits with hypertonic saline-induced sputum and mannitol challenge (including sputum collection). AHR to mannitol was expressed as response-dose-ratio (RDR) and related to cell counts, ECP, MPO and IL-8 levels in sputum. Results There was a positive correlation between RDR to mannitol and eosinophil numbers (r = 0.47, p = 0.03) and level of IL-8 (r = 0.46, p = 0.04) in hypertonic saline-induced sputum. Furthermore, significant correlations were found between RDR and eosinophil numbers (r = 0.71, p = 0.001), level of ECP (r = 0.72, p = 0.001), IL-8 (r = 0.57, p = 0.015) and MPO (r = 0.64, p = 0.007) in sputum collected after mannitol challenge. ROC-curves showed 60% sensitivity and 100% specificity of RDR for >2.5% eosinophils in mannitol-induced sputum. Conclusions In mild-moderate COPD mannitol hyperresponsiveness is associated with biomarkers of airway inflammation. The high specificity of mannitol challenge suggests that the test is particularly suitable to exclude eosinophilic airways inflammation, which may facilitate individualized treatment in COPD. Trial registration Netherlands Trial Register (NTR): NTR1283</p

Use of pulmonary inhalants remains remarkably high after atrial septal defect closure

Background: Post-repair atrial septal defects (ASD) patients are frequently discharged from follow-up, but the extent of pulmonary symptoms long-term post-repair is unknown. Methods and Results: The national CONgenital CORvitia registry was linked to the national Drug Registry to investigate all ambulatory-dispensed pulmonary inhalants for 2006–2014. ASD patients were compared with age-and sex-matched referents from the general population. A total of 1,959 adult patients (age 42±17 years; 66% female; 1,223 [62%] repaired) were included. Compared with the referents, ASD patients had more inhalant use, even at long-term post-repair follow-up (OR=1.81 [95% CI 1.62–2.03]; P<0.001). Conclusions: ASD patients had 2-fold higher inhalant use compared with referents even at long-term post-repair follow-up, suggesting persistent pulmonary functional impairment