Anomalous origin of right pulmonary artery from ascending aorta with right ventricular endocardial fibroelastosis

Pahlajani, Dev; Pantvaidya, Shanti; Pandit, Shobha; Patwardhan, Anil; Chaukar, A. P.

doi:10.1007/bf02265408

Cited by 7 publications

(1 citation statement)

References 10 publications

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“…Other proposed theories include incomplete leftward migration of the RPA during its development [50] or failure of fusion of one of the sixth arches with the MPA, resulting in persistence of the aortic sac from which the sixth arches originate [51]. An association with chromosome 22q11 deletion has been suggested [52].…”

Section: Aortic Origin Of a Branch Pulmonary Arterymentioning

confidence: 99%

Abnormalities of the Ductus Arteriosus and Pulmonary Arteries

Tacy

2009

Echocardiography in Pediatric and Congenital Heart Disease

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The ductus arteriosus is a normal vascular structure in mammalian embryos that connects the main pulmonary artery (MPA) with the descending aorta or a subclavian artery. In left aortic arch the ductus arteriosus inserts into the proximal descending aorta at its junction with the aortic isthmus, distal to the origin of the left subclavian artery. In right aortic arch the ductus arteriosus can connect the right descending aorta with the MPA (right-sided ductus arteriosus), the proximal left subclavian artery with the MPA (left-sided ductus arteriosus), or both (bilateral ductus arteriosus). In the fetus, the ductus arteriosus carries blood from the right ventricle (RV) to the aorta, bypassing the high-resistance pulmonary vascular bed. Normally, the ductus arteriosus closes during the first day of life. Persistent ductal blood flow beyond the newborn period is termed patent ductus arteriosus (PDA). IncidenceThe incidence of PDA varies considerably depending on the postnatal age of subjects and their degree of prematurity. PDA is common in preterm infants and is considered a functional abnormality as opposed to a structural congenital cardiac anomaly. In term infants the normal ductus arteriosus may stay open for several days after birth. Echo-Doppler studies in healthy newborns have shown functional closure of the ductus arteriosus in almost all subjects by 4-7 days after birth [1,2]. Based on analysis of 40 published articles spanning several decades, Hoffman and Kaplan estimated the median incidence of PDA in term infants at 56.7 per 100 000 live births [3]. EtiologyThe etiology of isolated PDA in full-term infants older than 1 week is not known. Recent investigations have identified the genes affecting the development of the fourth and sixth pharyngeal arches, and noted their association with cardiovascular defects similar to those observed in DiGeorge syndrome [4]. Morphology Developmental considerationsThe normal anatomy and congenital malformations of the ductus arteriosus and branch pulmonary arteries are rooted in the development of the embryonic sixth aortic arches (see Chapter 30). The embryo has two dorsal aortas, which communicate with the ventrally located aortic sac via several pairs of branchial arch arteries. During development, there is an orderly appearance and regression of six pairs of primitive aortic arches. The fate of the fourth and sixth arches is most important to the topics covered in this chapter. The left fourth arch persists as the distal left aortic arch between the left common carotid and left subclavian arteries, and the left dorsal aorta persists to become the segment that extends between the left subclavian artery and the site of the entrance of the ductus arteriosus. The right fourth arch becomes the brachiocephalic trunk and proximal right subclavian artery, whereas the right dorsal aorta largely involutes. The proximal portions of the right and left sixth arches form the proximal right and left pulmonary arteries, respectively. The distal right sixth arch regresses, whereas the...

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Section: Aortic Origin Of a Branch Pulmonary Arterymentioning

confidence: 99%