Situs inversus, subaortic and subpulmonic stenosis, ventricular septal defect, and single coronary artery. 1976

M Kleinfeld, and J J Rozanski, and J V Brumlik

The unusual occurrence of situs inversus totalis, ventricular septal defect, hypertrophic subaortic and subpulmonic stenosis, and single coronary artery in a 38-year-old man is presented. The clinical course was remarkably mild, as documented by data from 23 years of study including four cardiac catheterizations. At age 35 years, however, syncope, chest pain, and marked elevation of right ventricular pressure prompted complete surgical repair of the left and right ventricular outflow tract obstructions and closure of the septal defect. Three years after surgery the patient continues to lead an active life without symptoms. The unusually mild course can be attributed to the natural banding effects of the subpulmonic stenosis which prevented irreversible pulmonary hypertension.

UI MeSH Term Description Entries
D008297 Male Males
D011666 Pulmonary Valve Stenosis The pathologic narrowing of the orifice of the PULMONARY VALVE. This lesion restricts blood outflow from the RIGHT VENTRICLE to the PULMONARY ARTERY. When the trileaflet valve is fused into an imperforate membrane, the blockage is complete. Pulmonary Stenosis,Pulmonary Stenoses,Pulmonary Valve Stenoses,Pulmonic Stenosis,Stenoses, Pulmonary,Stenoses, Pulmonary Valve,Stenosis, Pulmonary,Stenosis, Pulmonary Valve,Valvular Pulmonic Stenosis,Pulmonary Stenose,Pulmonic Stenoses,Pulmonic Stenoses, Valvular,Pulmonic Stenosis, Valvular,Stenose, Pulmonary,Stenoses, Pulmonic,Stenosis, Pulmonic,Valvular Pulmonic Stenoses
D003330 Coronary Vessel Anomalies Malformations of CORONARY VESSELS, either arteries or veins. Included are anomalous origins of coronary arteries; ARTERIOVENOUS FISTULA; CORONARY ANEURYSM; MYOCARDIAL BRIDGING; and others. Anomaly, Coronary Vessel,Coronary Vessel Anomaly
D003914 Dextrocardia A congenital defect in which the heart is located on the right side of the THORAX instead of on the left side (levocardia, the normal position). When dextrocardia is accompanied with inverted HEART ATRIA, a right-sided STOMACH, and a left-sided LIVER, the combination is called dextrocardia with SITUS INVERSUS. Dextrocardia may adversely affect other thoracic organs. Dextrocardias
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
D006345 Heart Septal Defects, Ventricular Developmental abnormalities in any portion of the VENTRICULAR SEPTUM resulting in abnormal communications between the two lower chambers of the heart. Classification of ventricular septal defects is based on location of the communication, such as perimembranous, inlet, outlet (infundibular), central muscular, marginal muscular, or apical muscular defect. Ventricular Septal Defects,Intraventricular Septal Defects,Ventricular Septal Defect,Defect, Intraventricular Septal,Defect, Ventricular Septal,Defects, Intraventricular Septal,Intraventricular Septal Defect,Septal Defect, Intraventricular,Septal Defect, Ventricular,Septal Defects, Intraventricular,Septal Defects, Ventricular
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001024 Aortic Valve Stenosis A pathological constriction that can occur above (supravalvular stenosis), below (subvalvular stenosis), or at the AORTIC VALVE. It is characterized by restricted outflow from the LEFT VENTRICLE into the AORTA. Aortic Stenosis,Aortic Valve Stenoses,Stenoses, Aortic,Stenoses, Aortic Valve,Stenosis, Aortic,Stenosis, Aortic Valve,Valve Stenoses, Aortic,Valve Stenosis, Aortic

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