Anomalous Origin Of Pulmonary Artery Research Articles

Autologous Reconstruction of Anomalous Origin of a Right Pulmonary Artery From the Aorta.

Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages.

EP24.17: Prenatal diagnosis and postnatal follow‐up of anomalous origin of pulmonary artery from ascending aorta by echocardiography

EP24.17: Prenatal diagnosis and postnatal follow‐up of anomalous origin of pulmonary artery from ascending aorta by echocardiography

Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot.

The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1-3% of the patients with tetralogy of Fallot will have unilateral absence of pulmonary artery (UAPA). However, coexistence of APV with tetralogy of Fallot (TOF) and UAPA is extremely rare. This rare subset can present in two forms. In one group, the main pulmonary artery continues as either left or right pulmonary artery (UAPA) and there is true absence of contralateral pulmonary artery. The second group is termed as unilateral anomalous origin of pulmonary artery (UAOPA), in which the contralateral lung is supplied either by patent ductus arteriosus or a collateral from the aorta. There are a limited number of these cases in the literature. We present a rare case of TOF with APV and UAPA managed using a different surgical technique. Also, we have done contemporary literature review.

Aortic Arch Anomalies and Pulmonary Artery Anomalies : Echocardiographic Evaluation

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and review other malformations of the aortic arch, including interrupted aortic arch (IAA), hypoplastic aortic arch, and coarctation of aorta. I have also described few anomalies of pulmonary artery (PA) origin and bifurcation like PA sling which has clinical presentation similar to vascular ring and anomalous origin of PA from aorta which present as large aorto-pulmonary communication with congestive heart failure. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression.

The surgical treatment of anomalous origin of one pulmonary artery from the ascending aorta

ObjectiveThis study sought to summarize the clinical experience of surgical treatment of anomalous origin of pulmonary arteries from the ascending aorta (AOPA) in Fuwai hospital.MethodsFifty-two patients (28 males, 17.2 ± 27.2 months old and 8.7 ± 10.2 Kg weight) who have AOPA undertook surgical treatment between 1998 and 2017 were reviewed in this study, 47 out of 52 are anomalous origin of the right pulmonary artery (AORPA), among the rest of the patients are anomalous origin of left pulmonary artery (AOLPA). 27 out of 52 associate with simple cardiac abnormalities, 20 out of 52 associate with complex cardiac malformations, the remaining 5 patients without cardiac abnormalities. Among all patients who underwent surgical treatment, the direct end-to-side anastomosis strategy was applied in 26 patients, autologous pericardial-homograft patch and aortic flap were employed in 20 patients, and synthetic graft was used in 6 patients.ResultsNo patient died during the perioperative period. 50 out of 52 patients were followed-up for 100.1 ± 70.9 months. The rate of pulmonary arterial free restenosis for 2 years, 5 years, and 10 years is 98.0, 96.0 and 92.0%, respectively.ConclusionsThe correct diagnosis and appropriate surgical treatment for AOPA could obtain excellent early and medium-term result.

The prenatal diagnosis, classification and outcome of anomalous origin of pulmonary artery from ascending aorta

Objective To investigate the clinical value of fetal echocardiography in prenatal diagnosis, classification and outcome of abnormal origin of pulmonary artery from ascending aorta (AOPA). Methods From January 2014 to June 2018, 18 cases of AOPA diagnosed by echocardiography in 43 966 fetuses from Shaanxi Fetal Congenital Heart Disease Diagnostic Center were retrospectively analyzed. The echocardiographic features, pathological and anatomical classification, genetic characteristics and outcome of postnatal echocardiography were summarized. Results Abnormal origin of pulmonary artery branches could be demonstrated by color Doppler imaging system in 18 cases. Ten cases (55.6%) of right pulmonary artery abnormalities originated from ascending aorta (AORPA), in which 6 cases (60%) were distal pulmonary artery abnormalities, 4 cases (40%) were proximal pulmonary artery abnormalities. Eight cases (44.4%) of left pulmonary artery abnormalities originated from ascending aorta (AOLPA), including 7 cases (87.5%) of distal pulmonary artery abnormalities and 1 case (12.5%) of proximal pulmonary artery abnormalities. Twelve AOPAs were associated with other intracardiac malformations with 6 right ventricular double outlet (DORV) accompanying with pulmonary stenosis, 3 tetralogy of fallots(TOF), 2 atrioventricular septal defects(AVSD), 1 single ventricle with single atrium, and 1 Berry syndrome, and no casese were associated with extracardiac malformations.Amniocentesis karyotype analysis and gene chip detection in 5 cases showed normal results. Four of 18 cases were born, in which 3 cases died and 1 case was progressively suffered with right pulmonary artery atresia, 10 cases were inducted of labor (4 autopsy) and following-up was lost in 4 cases. Conclusions Incidence of AOLPA and distal type in fetal AOPA is higher than that in child and adult from references, DORV is abnormal type mostly associated with intracardiac malformation. AOPA has no obvious genetic result for less cases. The main risk after AOPA birth is pulmonary infection. Echocardiography is of great value for AOPA in prenatal diagnosis, outcome observation and surgical planning after birth. Key words: Ultrasonography, prenatal; Fetus; Abnormal origin of pulmonary artery from ascending aorta

A Rare Combination of Anomalous Origin of Right Pulmonary Artery from the Ascending Aorta with Congenital Tricuspid Valve Anomaly and Severe Tricuspid Regurgitation

Anomalous origin of a pulmonary artery (PA) from the aorta is a rare presentation in adulthood. As these patients invariably develop severe pulmonary hypertension in infancy itself, prognosis is poor. Here, we present a patient with anomalous origin of right PA from the aorta and congenital tricuspid valve anomaly with severe tricuspid regurgitation, a very rare combination surviving to adult life.

Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.

The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic imaging technology have led to better delineation of the vasculature anatomy and the identification of previously unrecognized and unclassified anomalies. In this manuscript, we review the existing literature and propose a series of modifications to the original Stewart classification of congenital aortic arch abnormalities to incorporate this new knowledge. In brief, we propose the following modifications: (1) In Group I, we further divide subgroup B into left arch atretic and right arch atretic; (2) In Group II, we add three more subgroups, including aberrant right innominate artery, "isolated" right innominate artery (RIA), "isolated" right carotid artery with aberrant right subclavian artery; (3) In Groups I, II, and III, we add a subgroup of absence of both ductus arteriosus; and (4) In Group IV, we add three subgroups, including circumflex retro-esophageal aorta arch, persistent V aortic arch, and anomalous origin of pulmonary artery from ascending aorta.

Prenatal diagnosis of anomalous origin of pulmonary artery.

Anomalous origin of the pulmonary arteries is a rare congenital pulmonary vascular malformation that includes unilateral absence of the pulmonary artery (UAPA), anomalous origin of unilateral pulmonary artery (AOPA), and left pulmonary artery sling (LPAS). We analyze 15 cases of fetal pulmonary artery abnormalities from 2011 to 2017, detected via prenatal ultrasound at our center. The 15 cases include UAPA (5), AOPA (6), and LPAS (4). Of the UAPA cases, 2 had pulmonary atresia and 3 had pulmonary artery stenosis. In 5 cases, the descending aorta issued collateral vessels to the left lung. Of the AOPA cases, 2 had left pulmonary artery abnormalities originating in the ascending aorta and 4 showed right pulmonary artery abnormalities originating in the ascending aorta. Of the LPAS cases, 2 were type IA, 1 was type IIA, and 1 was type IIB. Two of the LPAS were associated with right lung dysplasia. Abnormal origin of the pulmonary artery has a characteristic ultrasonographic appearance. The branch of the pulmonary artery cross section can provide important clues to the diagnosis of abnormal pulmonary artery origin. Pulmonary artery abnormalities are often associated with pulmonary atresia or stenosis.

Double ductus arteriosus and anomalous origin of the right pulmonary artery from the right-sided duct

Double ductus arteriosus with anomalous origin of the right pulmonary artery is a rare congenital heart disease. Echocardiography is the first-choice method to diagnose pulmonary arteries and presence of the arterial ductus. Computed tomography (CT) angiography confirms the final diagnosis of the anomalous origin of the pulmonary artery. We report a case diagnosed using 40-slice CT angiography where this case demonstrated presence of both ducts and an anomalous origin of right pulmonary artery from the right-sided ductus arteriosus.<Learning objective: Anomalous origin of the right pulmonary artery usually presents in early infancy with heart failure and rapid development of pulmonary hypertension. An association of two patent arterial ducts and an anomalous origin of the right pulmonary artery from the right-patent arterial duct is a rare finding. Computed tomography angiography is the best diagnostic modality to establish the correct diagnosis, which is crucial for further decision-making and optimal patient management.>

Anomalous Origin of the Right Pulmonary Artery from the Abdominal Aorta With Aberrant Right Subclavian Artery and Left Patent Ductus Arteriosus

Anomalous origin of the pulmonary artery (AOPA) from the aorta is a rare congenital heart malformation. This report describes a case of AOPA from the abdominal aorta in association with an aberrant right subclavian artery and a patent ductus arteriosus, which never has been reported previously in the literature.

Repair of Anomalous Origin of Right Pulmonary Artery from Ascending Aorta without Cardiopulmonary Bypass

Anomalous origin of a pulmonary artery from the ascending aorta (AORPA) is a rare congenital cardiac malformation that needs prompt surgical repair; otherwise it is associated with poor prognosis. We describe 3 cases of AORPA that successfully underwent complete surgical correction without extracorporeal circulation.

Anomalous origin of the pulmonary artery from the aorta: early diagnosis and repair leading to immediate physiological correction

Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction. Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Children's Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed. Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operative mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised. Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.

Anomalous origin of the pulmonary artery from the ascending aorta in a neonate, assessed by two-dimensional echocardiography

MOTS CLES Shunt ; Cardiopathie congenitale ; Nouveau-ne ; Echocardiographie 2D A full-term baby presented at 2 weeks of life with severe congestive heart failure. On clinical examination a 2/6 holosystolic murmur was found, bounding femoral pulses and hepatomegaly. Echocardiography found massive dilatation of both ventricles with severe atrioventricular regurgitation and pulmonary hypertension (Fig. 1). No intracardiac shunt was found but an abnormal right pulmonary artery arising from the ascending aorta was diagnosed (Figs. 2 et 3). On Doppler investigation of the aortic arch, diastolic reflux from the aorta to the right pulmonary artery was found (Fig. 4). The patient underwent immediate and successful complete surgical repair, consisting of detachment of the anomalous pulmonary artery from the ascending aorta with direct anastomosis to the main pulmonary artery. Anomalous origin of the right pulmonary arteries arising from the ascending aorta, also called hemitruncus, is a very rare anomaly. This lesion with two normal semilunar valves should be distinguished from truncus arteriosus communis, in which there is only one semilunar valve. Associated defects such as tetralogy of Fallot or aortopulmonary window are present occasionally. The right lung receives blood directly from the aorta, with resulting massive volume and/or pressure overload of the left pulmonary branch. The right lung receives the entire right ventricular output, resulting in volume overload. Biventricular cardiomyopathy and pulmonary hypertension occur rapidly in neonates, leading to respiratory distress and congestive heart failure. Diagnosis is by two-dimensional echocardiography and surgery should be performed as soon as possible. Treatment consists of surgical division of the anomalously connected pulmonary artery branch and reanastomosis

Long-Term Outcome of Surgically Repaired Unilateral Anomalous Pulmonary Artery Origin

The long-term outcome after surgical reimplantation of a unilateral congenitally anomalous pulmonary artery (UCAPA) origin is inadequately described. We performed a single-center retrospective review of patients with pulmonary artery (PA) sling or anomalous origin of 1 PA from the ascending aorta who underwent surgical repair from 1980 to 2006. Baseline data were available for 20 patients with PA sling and 29 patients with anomalous origin of 1 PA from the ascending aorta. Follow-up data for 38 patients, representing 190 cumulative patient-years, were available. Of 28 (74%) patients who developed pulmonary artery stenosis (PAS), 17 (45%) patients underwent a first intervention for PAS, and 9 (24%) patients underwent a second intervention. Median time from surgical repair to diagnosis of PAS was 0.4 years (95% confidence interval [CI] 0, 0.9). Median time from diagnosis of PAS to first intervention was 2.7 years (95% CI 0.07, 5.4). In the 29 patients initially repaired at our center, no first intervention occurred later than 2.8 postoperative years. Median time from first to second intervention was 2 years (95% CI 0, 4.8). Percutaneous intervention for postoperative PAS resulted in acute hemodynamic and anatomic improvement with modest risk profile. On multivariate analysis, recent surgical repair (after 1993) was a risk factor for PAS (p = 0.03, hazard ratio [HR] 1.9), and operative first intervention (vs. percutaneous) was a risk factor for second intervention (p = 0.05, HR 11.2). After reimplantation of a UCAPA origin, PAS is frequent and occurs early postoperatively. Close follow-up after repair is necessary.

Anomalous origin of the pulmonary artery from the right coronary artery

A 5-year-old boy who had an anomalous origin of a stenotic pulmonary artery from the right coronary artery with ventricular septal defect and pulmonary atresia is reported. The diagnosis was made at operation. Successful repair was achieved for the complex anomaly by using a pulmonary artery homograft as a conduit and closing the ventricular septal defect.

Use of autogenous aortic and main pulmonary artery flaps for repair of anomalous origin of the right pulmonary artery from the ascending aorta

in patients with origin of the right (RPA) or left pulmonary artery from the ascending aorta, the RPA or, much less commonly, the left pulmonary artery arises from the ascending aorta in the presence of separate aortic and pulmonary valves and without the interposition Of ductal tissue. 1" 2 The RPA usually originates from the right posterior aspect of the ascending aorta. 3 Physiologically, the alteration creates a large left-to-right shunt. The contralateral lung is therefore subjected to the entire right ventricular output in addition to flow contributed by associated anomalies, such as patent ductus arteriosus, aortopulmonary septal defect, atrial septal defect, or ventricular septal defect, which have been present in greater than 60% of previous reports. 1 4 Without early operation the natural history of this condition is dismal, with a mortality rate of approximately 70% at 6 months of age and 80% at I year of age. 5 Armer and associates 6 reported the first successful anatomic repair of anomalous origin of the RPA from the ascending aorta with interposition of a polyester fiber graft between the RPA and the main pulmonary artery (MPA). The first successful primary repair was reported by Kirkpatrick, Girod, and King. 7 Traditionally, primary repair is generally reserved for those patients in whom the RPA originates from the posterior aspect of the aorta in close proximity to the MPA. s When the RPA arises from the right lateral aspect of the aorta, the right hilum is often mobilized medially to allow apposition of the RPA and MPA without tension. Alternatively, an interposition synthetic graft is placed either anterior or posterior to the aorta 5, 9 We propose a modified native tissue repair that reduces tension on the RPA-MPA anastomosis. The ascending aorta, proximal aortic arch vessels, the MPA, and both branch pulmonary arteries are widely mobilized. As soon as cardiopulmonary bypass has been established, the RPA is temporarily clamped or snared. The patent ductus arteriosus, if present, is doubly ligated and divided. After crossclamping of the aorta, the anterior aortic circumference is incised transversely at the level of the RPA origin (Fig. 1). Under direct vision, the remaining aortic circumference is transected, with a generous cuff of posterior aortic wall left around the RPA origin (Fig. 2). Alternatively, the posteromedial aspect of the aortic wall can be left intact. Utmost care should be taken to avoid compromise of the left

Anomalous origin of one pulmonary artery from the ascending aorta: a review of echocardiographic, catheter, and morphological features.

Six patients with anomalous origin of one pulmonary artery from the ascending aorta were reviewed. Four had anomalous origin of the right pulmonary artery and two had anomalous origin of the left pulmonary artery from the ascending aorta. Two of these six patients had tetralogy of Fallot. Two patients died in the first month of life. No changes in the pulmonary vasculature were seen at necropsy. Corrective surgery was attempted in two patients with associated tetralogy of Fallot when they were two years old but both died. At necropsy there was severe pulmonary vascular disease in the lung supplied by the anomalous pulmonary artery but no pulmonary vascular hypertensive changes in the lung supplied by the pulmonary artery from the right ventricle. Two recent patients underwent successful anastomosis of the anomalous pulmonary artery to the main pulmonary artery at three months and one month and three weeks of age respectively. Intraoperative lung biopsy in the latter patient showed early changes in both lungs. Both echocardiography and cardiac catheterisation were used in the diagnoses. Systemic or suprasystemic pressures were found in the pulmonary artery arising from the right ventricle as well as the anomalous pulmonary artery in the three patients without tetralogy of Fallot. Anomalous origin of a pulmonary artery from the ascending aorta is a distinct entity and differs from other aorto-pulmonary arterial connections. Early surgical intervention is recommended in all patients (including those patients with associated tetralogy of Fallot) because of the risk of rapid development of irreversible pulmonary vascular disease.

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

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.

Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

Absent pulmonary valve leaflets and anomalous origin of a pulmonary artery from the ascending aorta have been reported as separate lesions associated with tetralogy of Fallot but not in combination. The clinical, radiologic and pathologic findings are presented in three patients with this previously undescribed combination. A search of the literature for similar cases revealed 233 patients with absent pulmonary valve leaflets and 56 patients with anomalous origin of a pulmonary artery from the ascending aorta; these cases are reviewed. Although 17 patients with absent pulmonary valve leaflets and tetralogy of Fallot also had absence of the proximal portion of the left pulmonary artery, none had an anomalously arising pulmonary artery from the ascending aorta. In the reviewed cases, anomalous origin of the right pulmonary artery from the ascending aorta was more frequent than that of the left pulmonary artery (44 compared with 12 cases, respectively), but the latter was more often associated with tetralogy of Fallot.