Causes of Exercise Intolerance in Pectus Excavatum

Background: Despite several studies have reported lower exercise capacity in patients with pectus excavatum, none of them could demonstrate a clear pathophysiology. Objective: The aim of this study is to evaluate cardiac hemodynamics and systolic and diastolic function at rest and during exercise in patients with pectus excavatum and compare it with healthy controls. Methods: Stress echocardiography was performed in 111 subjects with pectus excavatum and 20 healthy controls. Results: Patients with pectus excavatum had lower right ventricular inflow minimum diameter: 1.29±0.26 cm/m2 versus 1.89±0.25 cm/m2 (p <0.01). Peak exercise capacity was lower in patients with pectus excavatum: 8.3±1.4 METs versus 15±4.5 METs (p <0.0001). Left ventricular diastolic dysfunction was observed in 34.6% of the patients with pectus excavatum and in 5% of the healthy controls (p=0.007), while 40% of the subjects with pectus excavatum and 15% of the healthy controls presented right ventricular diastolic dysfunction (p=0.04). Medium tricuspid pressure gradient during exercise was higher in patients with pectus excavatum: 6.21±2.29 mm Hg versus 4.8±1.17 mm Hg in healthy controls (p=0.01). The tricuspid valve area remained fixed during exercise in patients with pectus excavatum: 1.48±0.57 cm2/m2 versus 2.11±0.88 cm2/m2 in healthy controls (p=0.0001). Conclusions: Patients with pectus excavatum present functional abnormalities, probably due to external compression of the heart, which are evident by a small tricuspid annulus, a higher tricuspid pressure gradient during exercise, a tricuspid areaIntroducción: En múltiples estudios se ha determinado menor capacidad de esfuerzo en pacientes con pectus excavatum, pese a lo cual no se ha logrado demostrar claramente un mecanismo fisiopatológico que la explique. Objetivo: Evaluar la hemodinamia cardíaca y la función sistodiastólica en reposo y en esfuerzo en pacientes con pectus excavatum, comparándolos con controles sanos. Material y métodos: Se estudiaron con eco estrés 111 sujetos portadores de pectus excavatum y 20 controles sanos. Resultados: El diámetro mínimo a nivel del tracto de entrada del ventrículo derecho fue menor en los pacientes con pectus excavatum: 1,29 ± 0,26 cm/m2 versus 1,89 ± 0,25 cm/m2 (p < 0,01). La capacidad de esfuerzo máxima fue menor en los pacientes con pectus excavatum: 8,3 ± 1,4 MET versus 15 ± 4,5 MET (p < 0,0001). Se observaron signos de disfunción diastólica del ventrículo izquierdo en el 34,6% de los pacientes con pectus excavatum y en el 5% de los controles sanos (p = 0,007), y de disfunción diastólica del ventrículo derecho en el 40% de los portadores de pectus excavatum y el 15% de los controles sanos (p = 0,04). El gradiente tricuspídeo medio en el esfuerzo fue mayor en los pacientes con pectus excavatum: 6,21 ± 2,29 mm Hg versus 4,8 ± 1,17 mm Hg en los controles sanos (p = 0,01). El área tricuspídea en el esfuerzo se mantuvo fija en los portadores de pectus excavatum: 1,48 ± 0,57 cm2/m2 versus 2,11 ± 0,88 cm2/m2 en los controles sanos (p = 0,0001). Conclusiones: Los pacientes con pectus excavatum presentan alteraciones funcionales, probablemente producto de la compresión cardíaca externa, que se evidencian por un diámetro del anillo tricuspídeo menor, un gradiente diastólico tricuspídeo mayor en el esfuerzo, un área tricuspídea fija en reposo y en esfuerzo, y signos que sugieren disfunción diastólica del ventrículo izquierdo y el ventrículo derecho. Dichas alteraciones contribuyen a explicar la menor performance en el esfue

Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT)

Objective Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. Methods Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered. Results Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). Conclusion The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments