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      Fetal Isolated Anomalous Origin of Right Pulmonary Artery from Aorta

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          Abstract

          The anomalous origin of a branch pulmonary artery from the aorta (AOPA) is characterized by the anomalous origin of one of the branch pulmonary arteries (PA) from the ascending aorta and a normal origin of the other PA from main PA. AOPA is an extremely rare cardiac malformation. Few studies have reported fetal anomalous origin of PA from aorta with other malformation. We report a case of isolated distal anomalous origin of the right PA from the aorta that was diagnosed by fetal echocardiography at 25 weeks' of gestation. Tracing the course of PA branches is important to make diagnosis.

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          Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis.

          Geert van Leenders, Carla H van Gils, L Kutsche (1988)
          The pathologic anatomic features and associated cardiovascular anomalies of 9 patients with anomalous origin of 1 pulmonary artery (PA) from the ascending aorta seen at the University of Florida and 99 previously reported cases were analyzed. Anomalous origin of the right PA was much more common than anomalous origin of the left PA. The anomalous right PA usually arose from the posterior aspect of the ascending aorta close to the aortic valve. Less commonly, it originated from the lateral ascending aorta just proximal to the innominate artery. Patent ductus arteriosus and aorticopulmonary septal defect were commonly associated with anomalous origin of the right PA; other cardiovascular anomalies were rare. In contrast, tetralogy of Fallot and aortic arch anomalies, e.g., right aortic arch and anomalous origin of the subclavian artery, were common in anomalous origin of the left PA. An association with DiGeorge syndrome, frequently noted with persistent truncus arteriosus, was not seen with anomalous origin of a PA from the ascending aorta. The observations indicate that anomalous origin of the right PA and anomalous origin of the left PA are pathogenetically unrelated to each other and both are unrelated to persistent truncus arteriosus. Moreover, anomalous origin of the right PA arising close to the innominate artery is thought to be pathogenetically distinct from the type that arises close to the aortic valve. These pathogenetic relations should be considered in epidemiologic studies of congenital heart disease.
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            Origin of the right pulmonary artery from the ascending aorta.

            G Fontana, M S Spach, E L Effmann … (1987)
            Origin of the right pulmonary artery from the aorta is a congenital malformation usually associated with serious symptoms in the first year of life and characterized by a poor prognosis. Sixty-five patients with this disorder have been reviewed in the literature, and 95% presented during the first year with signs of congestive heart failure. All had cardiomegaly by radiographic and electrocardiographic examination. An accurate diagnosis was established by cineangiography, and associated cardiovascular anomalies were present in 85%. Origin of the right pulmonary artery from the ascending aorta is much more common than origin of the left pulmonary artery from this vessel (8 to 1). Twenty-three patients were managed without operation with a 30% 1-year survival rate. Among those patients managed surgically, the survival rate was 84% at 1 year. It is now clear that operation should be done as early as possible to prevent irreversible changes occurring in the pulmonary arterial vasculature since microscopic features of pulmonary hypertension have been seen during the first month of life. One patient was operated on at 5 months with correction of the deformity. The pulmonary arterial pressure decreased to normal after operation. This child is now asymptomatic and his 10-year postoperative follow-up is the longest found in the literature.
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              Early repair of hemitruncus: excellent early and late outcomes.

              G Piercey, J. Del Peso, Meena Nathan … (2007)
              Anomalous origin of 1 of the branch pulmonary arteries from the aorta with 2 normal semilunar valves (hemitruncus) is a rare entity. There have been several small case series reported. We report here our single-institution surgical experience with hemitruncus from 1982 to 2006. A retrospective case review of all cases of conotruncal anomalies at Children's Hospital Boston revealed 16 patients with hemitruncus. Ten patients had surgery in the neonatal period (<30 days), 4 at 1 to 6 months, 1 at 8 months, and 1 at 2 years. Diagnosis of hemitruncus was the indication for operation in all but 1. Fourteen of the 16 had anomalous right pulmonary artery from aorta, and 2 had left pulmonary artery from aorta. Common associated anomalies included patent foramen ovale in 14, patent ductus arteriosus in 11, and ventricular septal defect in 4. All patients had elevated right ventricular pressures with systemic pressures in 5 and suprasystemic pressures in 9. There was 1 operative death in this series in an infant who died from sepsis following ligation of a tracheoesophageal fistula. One patient required reoperation for supravalvular aortic stenosis and right pulmonary artery stenosis 1 year postoperatively. Three patients required 4 catheter-based interventions postoperatively. At 20 years, survival by Kaplan-Meier was 93%; freedom from reoperation, 93%; and freedom from catheter reintervention, 79%. Early repair of hemitruncus results in excellent hemodynamic and anatomic results. Survival is excellent, with a low incidence of reoperation or reintervention.
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                Author and article information

                Journal
                Journal ID (nlm-ta): AJP Rep
                Journal ID (iso-abbrev): AJP Rep
                Journal ID (doi): 10.1055/s-00000169
                Title: AJP Reports
                Publisher: Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                ISSN (Print): 2157-6998
                ISSN (Electronic): 2157-7005
                Publication date (Electronic): 04 March 2015
                Publication date (Print): April 2015
                Volume: 5
                Issue: 1
                Pages: e80-e81
                Affiliations
                [1 ]Department of Ultrasonography, The Second Xiangya Hospital, Central South University, Changsha, Hunan, People's Republic of China
                Author notes
                Address for correspondence Qichang Zhou, PhD Department of Ultrasound, Second Xiangya Hospital of Central South University 139 Renmin Road (M), Changsha 410011China hnzqc2013@ 123456163.com
                Article
                Publisher ID: 140083
                DOI: 10.1055/s-0035-1547331
                PMC ID: 4502634
                SO-VID: 5138dc63-9926-4e9c-a50a-a66911a54a7d
                Copyright © © Thieme Medical Publishers
                History
                Date received : 30 December 2014
                Date accepted : 23 January 2015
                Categories
                Subject: Article

                Keywords: anomalous origin of pulmonary artery from aorta,fetal echocardiography,prenatal diagnosis
                Data availability:
                Keywords: anomalous origin of pulmonary artery from aorta, fetal echocardiography, prenatal diagnosis

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