Thesis etd-10092020-144318 |
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Thesis type
Tesi di specializzazione (4 anni)
Author
RIDOLFI, CECILIA
URN
etd-10092020-144318
Thesis title
The multiform spectrum of arterial duct in right aortic arch
Department
PATOLOGIA CHIRURGICA, MEDICA, MOLECOLARE E DELL'AREA CRITICA
Course of study
MALATTIE DELL'APPARATO CARDIOVASCOLARE
Supervisors
relatore Prof. Pedrinelli, Roberto
correlatore Dott. Chiappa, Enrico
correlatore Dott. Chiappa, Enrico
Keywords
- arterial duct
- fetus
- prenatal diagnosis
- right aortic arch
- vascular ring
Graduation session start date
06/11/2020
Availability
Withheld
Release date
06/11/2090
Summary
Aim: to study the different characteristics of arterial duct (AD) in a series of right aortic arch (RAA) prenatally detected.
Method: among 832 congenital heart diseases (CHD), referred to a tertiary center between 2005-2019, 98 cases of RAA were identified. Based on location, body planes alignment, and relationship with the trachea, we classified the AD in 7 types: 1) left-sided, transverse; 2) left-sided, vertical; 3) from the undersurface of AA, vertical; 4) right-sided, mirror-image “V”, transverse; 5) right-sided, “H” shaped, transverse; 6) bilateral; 7) absent or unidentifiable. Type 1 cases were further distinguished in: a) with aberrant subclavian artery (ALSA), b) mirror-image branching and c) double aortic arch (DAA).
Result: type 1 was the largest group (42/98), 4 cases had CHD, none pulmonary outflow obstruction (POO). Symptoms were present in 12/29 of cases. Proximal stenosis of ALSA was detected in 26% of type 1a cases. Prenatal misclassification between mirror-image branching and DAA with atretic segment was common. Type 2, type 3 and type 7 AD were associated with tetralogy of Fallot (TOF) or equivalents. In type 5 associated with CHD, none had POO; in isolated, 3 had asymptomatic hypoplasia of left pulmonary artery (LPA). Two cases with type 6 AD had disconnection of LPA.
Conclusion: in RAA, symptomatic vascular rings were limited to type 1 AD. POO is always present when the AD lies on a vertical plane or is absent but not when on the transverse plane. Bilateral AD is rare and entails the risk of functionary loss of the left lung after birth.
Method: among 832 congenital heart diseases (CHD), referred to a tertiary center between 2005-2019, 98 cases of RAA were identified. Based on location, body planes alignment, and relationship with the trachea, we classified the AD in 7 types: 1) left-sided, transverse; 2) left-sided, vertical; 3) from the undersurface of AA, vertical; 4) right-sided, mirror-image “V”, transverse; 5) right-sided, “H” shaped, transverse; 6) bilateral; 7) absent or unidentifiable. Type 1 cases were further distinguished in: a) with aberrant subclavian artery (ALSA), b) mirror-image branching and c) double aortic arch (DAA).
Result: type 1 was the largest group (42/98), 4 cases had CHD, none pulmonary outflow obstruction (POO). Symptoms were present in 12/29 of cases. Proximal stenosis of ALSA was detected in 26% of type 1a cases. Prenatal misclassification between mirror-image branching and DAA with atretic segment was common. Type 2, type 3 and type 7 AD were associated with tetralogy of Fallot (TOF) or equivalents. In type 5 associated with CHD, none had POO; in isolated, 3 had asymptomatic hypoplasia of left pulmonary artery (LPA). Two cases with type 6 AD had disconnection of LPA.
Conclusion: in RAA, symptomatic vascular rings were limited to type 1 AD. POO is always present when the AD lies on a vertical plane or is absent but not when on the transverse plane. Bilateral AD is rare and entails the risk of functionary loss of the left lung after birth.
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