Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood

Autor: Annie Dore, François-Pierre Mongeon, Sajid Aslam, Paul Khairy, Pablo Avila-Alonso, Reda Ibrahim, Nancy Poirier, Azadeh Shohoudi, Joaquim Miro, Lise-Andrée Mercier, Anita W. Asgar, François Marcotte
Rok vydání: 2016
Předmět:
Aortic valve
Adult
Heart Defects
Congenital
Male
medicine.medical_specialty
Time Factors
Heart disease
medicine.medical_treatment
Heart Valve Diseases
030204 cardiovascular system & hematology
Aortic Coarctation
03 medical and health sciences
0302 clinical medicine
Bicuspid aortic valve
Bicuspid Aortic Valve Disease
medicine.artery
Mitral valve
Internal medicine
medicine
Thoracic aorta
Ventricular outflow tract
Humans
Mitral Valve Stenosis
Abnormalities
Multiple
030212 general & internal medicine
Diagnostic Errors
Retrospective Studies
Heart transplantation
business.industry
Quebec
Aortic Valve Stenosis
medicine.disease
Surgery
Survival Rate
medicine.anatomical_structure
Heart failure
Aortic Valve
cardiovascular system
Cardiology
Female
Morbidity
Cardiology and Cardiovascular Medicine
business
Follow-Up Studies
Zdroj: The Canadian journal of cardiology. 33(2)
ISSN: 1916-7075
Popis: Background Shone complex consists of a constellation of left-sided, usually obstructive, cardiac lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation. Incomplete Shone complex consists of a mitral valve anomaly associated with lesions involving the subaortic region, aortic valve, or thoracic aorta. There is a paucity of data regarding long-term outcomes in adults with Shone complex. Methods We reviewed records of adults with complete or incomplete Shone complex followed at the Montreal Heart Institute between 1982 and 2014. Results Among 4189 adults with congenital heart disease, 28 (0.67%) patients (mean age, 35 ± 11 years; 50% women) had complete or incomplete Shone complex and were followed for a median of 8 years. Only 39% were previously diagnosed as having Shone complex. The most common defects were congenital mitral stenosis (93%), aortic coarctation (75%), and bicuspid aortic valve (71%). Heart transplantation was required in 2 patients (7.1%) at age 22 and 28 years, respectively. Overall, 48% had cardiovascular hospitalizations during adulthood, predominantly for arrhythmias or heart failure. Freedom from cardiovascular intervention was 55%, 18%, and 8% at 10, 20, and 30 years of age, respectively. Although aortic coarctation was the most common indication for initial intervention (61%), adult interventions occurred predominantly for aortic valve/left ventricular outflow tract (60%) and mitral valve (33%) lesions. Conclusions Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. Increased awareness of this condition and associated complications may allow for more tailored follow-up.
Databáze: OpenAIRE
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