Pediatr Cardiol l 1:69-71, 1990
Pediatric Cardiology 9 Springer-VerlagNew York Inc. 1990
Abnormal Development of Fourth Aortic Arch Derivatives in the P a t h o g e n e s i s o f T e t r a l o g y o f F a l i o t Yuji Nakajima, Makoto Nishibatake, Kazuo Ikeda, Kazuo Momma, Atsuyoshi Takao, and Masaru Terai Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
SUMMARY. Aortic arch (AoA) anomalies were studied in 233 patients with tetralogy of Fallot (TOF) of whom some had coexisting pulmonary atresia (PA). There was a 23% incidence of a right AoA in patients without PA, 21% in those with both PA and persistent ductus arteriosus (PDA), and 50% in those with PA and major aortopulmonary collateral arteries (MAPCAs). There was a 5% incidence of an aberrant subclavian artery in patients without PA and a 16% incidence in those with PA and MAPCAs. In this cohort an elongated ascending aorta was observed both with and without high aortic arch. These aortic arch anomalies were frequently associated with PA and MAPCAs. KEY WORDS: Tetralogy of Fallot - - Pulmonary atresia - - Aortic arch anomalies
Among a wide variety of associated anomalies in tetralogy of Fallot (TOF), aortic arch (AoA) anomalies are important in considering its morphogenesis. The incidence of a right AoA and an aberrant subclavian artery in TOF patients with pulmonary atresia (PA) has not previously been thoroughly investigated. In this study, we also observed an elongated ascending aorta and a high aortic arch in many patients with TOF. The purpose of this study was to determine the incidence of AoA anomalies in TOF patients with or without PA.
Patients and Methods Two hundred thirty-three (l 31 male and 102 female) patients with TOF were the subjects of this study. Their mean age was 5 years (range 2 months to 12 years). One hundred fifty-six patients had TOF without PA, 39 had both PA and persistent ductus arteriosus (PDA), and 39 with PA and major aortopulmonary collateral arteries (MAPCAs). The cardiovascular anomalies were diagnosed by cardiac catheterization, angiocardiography, operation, and/or autopsy. The term "elongated ascending aorta" is used when the first branch (innominate or common carotid artery) originates from the proximal half of the the ascending aorta. Address offprint requests to: Dr. Yuji Nakajima, Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Kawada-cho 8-1, Shinjuku-ku, Tokyo, 162 Japan.
In addition to this term, we also used "high aortic arch" when the apex of the aortic arch extended to the clavicular line on the anteroposterior view of the cineangiography (Fig. 1). Statistical analysis was performed with the x-square test. A p value of ~
Pediatric Cardiology 9 Springer-VerlagNew York Inc. 1990
Abnormal Development of Fourth Aortic Arch Derivatives in the P a t h o g e n e s i s o f T e t r a l o g y o f F a l i o t Yuji Nakajima, Makoto Nishibatake, Kazuo Ikeda, Kazuo Momma, Atsuyoshi Takao, and Masaru Terai Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
SUMMARY. Aortic arch (AoA) anomalies were studied in 233 patients with tetralogy of Fallot (TOF) of whom some had coexisting pulmonary atresia (PA). There was a 23% incidence of a right AoA in patients without PA, 21% in those with both PA and persistent ductus arteriosus (PDA), and 50% in those with PA and major aortopulmonary collateral arteries (MAPCAs). There was a 5% incidence of an aberrant subclavian artery in patients without PA and a 16% incidence in those with PA and MAPCAs. In this cohort an elongated ascending aorta was observed both with and without high aortic arch. These aortic arch anomalies were frequently associated with PA and MAPCAs. KEY WORDS: Tetralogy of Fallot - - Pulmonary atresia - - Aortic arch anomalies
Among a wide variety of associated anomalies in tetralogy of Fallot (TOF), aortic arch (AoA) anomalies are important in considering its morphogenesis. The incidence of a right AoA and an aberrant subclavian artery in TOF patients with pulmonary atresia (PA) has not previously been thoroughly investigated. In this study, we also observed an elongated ascending aorta and a high aortic arch in many patients with TOF. The purpose of this study was to determine the incidence of AoA anomalies in TOF patients with or without PA.
Patients and Methods Two hundred thirty-three (l 31 male and 102 female) patients with TOF were the subjects of this study. Their mean age was 5 years (range 2 months to 12 years). One hundred fifty-six patients had TOF without PA, 39 had both PA and persistent ductus arteriosus (PDA), and 39 with PA and major aortopulmonary collateral arteries (MAPCAs). The cardiovascular anomalies were diagnosed by cardiac catheterization, angiocardiography, operation, and/or autopsy. The term "elongated ascending aorta" is used when the first branch (innominate or common carotid artery) originates from the proximal half of the the ascending aorta. Address offprint requests to: Dr. Yuji Nakajima, Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Kawada-cho 8-1, Shinjuku-ku, Tokyo, 162 Japan.
In addition to this term, we also used "high aortic arch" when the apex of the aortic arch extended to the clavicular line on the anteroposterior view of the cineangiography (Fig. 1). Statistical analysis was performed with the x-square test. A p value of ~
