Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta

Prifti, Edvin; Bonacchi, Massimo; Murzi, Bruno; Crucean, Adrian; Leacche, Marzia; Bernabei, Massimo; Bartolozzi, Fabio; Nathan, Neera; Vanini, Vittorio

doi:10.1111/j.0886-0440.2004.04023.x

Cited by 48 publications

(58 citation statements)

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“…In the oblique parasagittal plane this gave the appearance of a 'tobacco pipe', the bowl of the pipe represented by the coronary sinus and the stem by the LSVC and left internal jugular vein (Figures 1 and 2). This appearance has been described in pediatric cardiology 5 , but not to our knowledge in fetal studies. The fetus we describe had no additional anomalies and the neonatal course was uneventful, similar to that of other reported cases 3,5 .…”

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confidence: 62%

“…This appearance has been described in pediatric cardiology 5 , but not to our knowledge in fetal studies. The fetus we describe had no additional anomalies and the neonatal course was uneventful, similar to that of other reported cases 3,5 .…”

mentioning

confidence: 62%

“…Anomalies of the superior vena cava (SVC) include the presence of two veins as the right SVC (RSVC) and persistent left SVC (LSVC) 1 , the presence of an LSVC with an absent RSVC 2,3 and the very rare condition of the absence of both the right and left SVC 4 . In most cases with two SVCs the innominate vein connecting the jugular vein with the opposite SVC is absent 1,5 . Absent RSVC with LSVC is often an isolated anomaly 3,6 .…”

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confidence: 99%

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Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta with hypoplastic right ventricle and pulmonary stenosis

Öztunç¹,

Güzeltaş²

2008

Ultrasound in Obstet & Gyne

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Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta with hypoplastic right ventricle and pulmonary stenosis A 24-year-old woman was referred at 35 weeks' gestation for prenatal echocardiography due to a suspected cardiac anomaly on routine ultrasound examination. There was no history of medication use during pregnancy. Ultrasound examination showed normal fetal growth and no other organ anomalies or hydrops fetalis. Fetal echocardiography detected a hypoplastic right ventricle (diameter, 10 mm, vs. left ventricle, 25 mm) and tricuspid hypoplasia (annulus diameter, 5 mm) with valvar pulmonary stenosis (velocitiy, 2.5 m/s) (Figure 1). The main pulmonary artery was continuous with the left pulmonary artery and there was no confluence with the right pulmonary artery. The ventricular septum was intact. On detailed examination it was noted that the right pulmonary artery arose from the ascending aorta with a normal left pulmonary branch from the main pulmonary artery (Figure 2). The pregnancy was uneventful, and spontaneous vaginal delivery occurred at 38 weeks' gestation.Echocardiographic examination on the first postnatal day confirmed the above findings. Additionally, a small secundum atrial septal defect was detected. The tricuspid annulus Z-value score was −4, right ventricular volume was 1.5 cm 3 , left ventricular volume was 8.7 cm 3 and the pulmonary valve was thick and stenotic with a gradient of 85 mmHg. The right pulmonary artery diameter was

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confidence: 62%

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confidence: 99%

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Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta with hypoplastic right ventricle and pulmonary stenosis

Öztunç¹,

Güzeltaş²

2008

Ultrasound in Obstet & Gyne

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“…Right pulmonary artery originating from ascending aorta is commonly associated with atrial septal defect (ASD) (8,9). Also, left pulmonary artery originating from aorta associated with ASD was reported in the literature (8).…”

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confidence: 99%

Unilateral pulmonary artery agenesis: Clinical and laboratory findings of four cases and diagnostic clues for pediatricians

Karataş¹,

Alp²,

Altın³

et al. 2012

Anadolu Kardiyol Derg

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“…In aortic origin of a branch PA the PAs are discontinuous and the normal bifurcation of the MPA is not seen from any angle. The arch sidedness and the PDA are usually contralateral to the anomalous PA [64]. The echocardiogram typically exhibits evidence of pulmonary hypertension.…”

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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Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta

Cited by 48 publications

References 49 publications

Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta with hypoplastic right ventricle and pulmonary stenosis

Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta with hypoplastic right ventricle and pulmonary stenosis

Unilateral pulmonary artery agenesis: Clinical and laboratory findings of four cases and diagnostic clues for pediatricians

Abnormalities of the Ductus Arteriosus and Pulmonary Arteries

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