Quadricuspid Aortic Valve

Characteristics, Associated Structural Cardiovascular Abnormalities, and Clinical Outcomes

Michael Y C Tsang, Muaz M. Abudiab, Naser M. Ammash, Tasneem Zehra Naqvi, William D. Edwards, Vuyisile T Nkomo, Patricia Pellikka

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes. Methods and Results-Fifty patients (mean±SD age, 43.5±21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean±SD follow-up of 4.8±5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years. Conclusions-Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.

Original languageEnglish (US)
Pages (from-to)312-319
Number of pages8
JournalCirculation
Volume133
Issue number3
DOIs
StatePublished - Jan 19 2016

Fingerprint

Cardiovascular Abnormalities
Aortic Valve
Aortic Valve Insufficiency
Dilatation
Heart Septal Defects
Superior Vena Cava
Patent Ductus Arteriosus
Survival
Heart Valves
Endocarditis
Echocardiography
Dissection
Pathologic Constriction
Hemodynamics
Databases

Keywords

  • Aortic valve
  • aortic valve insufficiency
  • echocardiography
  • survival

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Quadricuspid Aortic Valve : Characteristics, Associated Structural Cardiovascular Abnormalities, and Clinical Outcomes. / Tsang, Michael Y C; Abudiab, Muaz M.; Ammash, Naser M.; Naqvi, Tasneem Zehra; Edwards, William D.; Nkomo, Vuyisile T; Pellikka, Patricia.

In: Circulation, Vol. 133, No. 3, 19.01.2016, p. 312-319.

Research output: Contribution to journalArticle

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abstract = "Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes. Methods and Results-Fifty patients (mean±SD age, 43.5±21.8 years at the time of the index diagnosis; female sex, 52{\%}) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006{\%}). The QAV was type A in 32{\%} and type B in 32{\%} (Hurwitz and Roberts classification). Aortic dilatation was present in 29{\%} of the patients, and 26{\%} had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8{\%} of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32{\%} of patients. During a mean±SD follow-up of 4.8±5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5{\%} and 87.7{\%} at 5 and 10 years. Conclusions-Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.",
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AU - Naqvi, Tasneem Zehra

AU - Edwards, William D.

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N2 - Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes. Methods and Results-Fifty patients (mean±SD age, 43.5±21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean±SD follow-up of 4.8±5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years. Conclusions-Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.

AB - Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes. Methods and Results-Fifty patients (mean±SD age, 43.5±21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean±SD follow-up of 4.8±5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years. Conclusions-Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.

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KW - echocardiography

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