Echocardiography in cystic fibrosis: A proposed scoring system. 1980

L A Lester, and A C Egge, and V S Hubbard, and C S Camerini-Otero, and R J Fink

An echocardiographic scoring system was developed on the basis of an observed sequence of echo abnormalities, beginning with RV hypertrophy, through RV dilation, to abnormal RV systolic time intervals, noted to be associated with the progressive pulmonary disease of cystic fibrosis. This score correlated significantly with both the Shwachman-Kulczycki (r = 0.87, P < 0.001) and Taussig-NIH (r = 0.86, P < 0.001) clinical scoring systems, the Brasfield chest roentgenogram score (r = 0.86, P < 0.001), and pulmonary function test results. The scoring of echos appears to be useful for the early detection and systematic quantitation of the cardiac effects of the progressive pulmonary disease. Preliminary sequential echo studies suggest that this system provides a method for assessing the progression of cardiac disease and evaluating prognosis in individual patients, and may prove to be useful in monitoring therapeutic interventions for cor pulmonale.

UI MeSH Term Description Entries
D008297 Male Males
D008450 Maximal Midexpiratory Flow Rate Measurement of rate of airflow over the middle half of a FORCED VITAL CAPACITY determination (from the 25 percent level to the 75 percent level). Common abbreviations are MMFR and FEF 25%-75%. Forced Expiratory Flow 025-075 Percent,FEF 25-75 Percent,Flow Rate, Maximal Midexpiratory,MMFR,25-75 Percent, FEF,25-75 Percents, FEF,FEF 25 75 Percent,FEF 25-75 Percents,Forced Expiratory Flow 025 075 Percent,Percent, FEF 25-75,Percents, FEF 25-75
D011660 Pulmonary Heart Disease Hypertrophy and dilation of the RIGHT VENTRICLE of the heart that is caused by PULMONARY HYPERTENSION. This condition is often associated with pulmonary parenchymal or vascular diseases, such as CHRONIC OBSTRUCTIVE PULMONARY DISEASE and PULMONARY EMBOLISM. Cor Pulmonale,Disease, Pulmonary Heart,Diseases, Pulmonary Heart,Heart Disease, Pulmonary,Heart Diseases, Pulmonary,Pulmonary Heart Diseases
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003550 Cystic Fibrosis An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D004452 Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Echocardiography, Contrast,Echocardiography, Cross-Sectional,Echocardiography, M-Mode,Echocardiography, Transthoracic,Echocardiography, Two-Dimensional,Transthoracic Echocardiography,2-D Echocardiography,2D Echocardiography,Contrast Echocardiography,Cross-Sectional Echocardiography,Echocardiography, 2-D,Echocardiography, 2D,M-Mode Echocardiography,Two-Dimensional Echocardiography,2 D Echocardiography,Cross Sectional Echocardiography,Echocardiography, 2 D,Echocardiography, Cross Sectional,Echocardiography, M Mode,Echocardiography, Two Dimensional,M Mode Echocardiography,Two Dimensional Echocardiography
D005260 Female Females
D005541 Forced Expiratory Volume Measure of the maximum amount of air that can be expelled in a given number of seconds during a FORCED VITAL CAPACITY determination . It is usually given as FEV followed by a subscript indicating the number of seconds over which the measurement is made, although it is sometimes given as a percentage of forced vital capacity. Forced Vital Capacity, Timed,Timed Vital Capacity,Vital Capacity, Timed,FEVt,Capacities, Timed Vital,Capacity, Timed Vital,Expiratory Volume, Forced,Expiratory Volumes, Forced,Forced Expiratory Volumes,Timed Vital Capacities,Vital Capacities, Timed,Volume, Forced Expiratory,Volumes, Forced Expiratory
D006332 Cardiomegaly Enlargement of the HEART, usually indicated by a cardiothoracic ratio above 0.50. Heart enlargement may involve the right, the left, or both HEART VENTRICLES or HEART ATRIA. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (HEART FAILURE) or several forms of CARDIOMYOPATHIES. Cardiac Hypertrophy,Enlarged Heart,Heart Hypertrophy,Heart Enlargement,Cardiac Hypertrophies,Enlargement, Heart,Heart Hypertrophies,Heart, Enlarged,Hypertrophies, Cardiac,Hypertrophies, Heart,Hypertrophy, Cardiac,Hypertrophy, Heart

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