Origin Of Pulmonary Artery Research Articles

Prenatal sonographic appearance of truncus arteriosus on wide‐band Doppler imaging

Persistent truncus arteriosus is an uncommon congenital cardiac anomaly. In most patients, this condition is not diagnosed prior to birth. We report a case in which this uncommon cardiac anomaly was diagnosed prenatally using wide-band Doppler imaging. When diagnosing fetal truncus arteriosus, sonologists should carefully search for the origin of the main pulmonary artery and for its 2 branches. Our experience suggests that wide-band Doppler imaging facilitates the prenatal diagnosis of truncus arteriosus.

Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta: 64-Slice MDCT Findings

Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta: 64-Slice MDCT Findings

Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta: A Surgical Case Study in an Adult Patient with “Irreversible” Pulmonary Vascular Disease

An adult patient presented with an anomalous right pulmonary artery arising from the ascending aorta with severe unilateral pulmonary vascular disease of the left lung due to a large left-sided patent ductus arteriosus. A stenosis in the right pulmonary artery protected the right pulmonary vasculature. Right ventricular pressure was suprasystemic. After ductal ligation and surgical repair, pulmonary artery pressures fell dramatically. An immediate postoperative angiography confirmed extremely poor perfusion of the left lung. At four-and-a-half years of follow-up, the patient's quality of life had improved dramatically, main pulmonary artery pressure was one-sixth of systemic pressures, and there was vastly improved perfusion of the left lung at this lower perfusion pressure. The calculated pulmonary vascular resistance of the left lung was within normal limits. These findings suggested a significant degree of reversal of pulmonary hypertensive disease in the left lung sustained to 54 months postoperatively.

Multislice Spiral Computed Tomography in an Infant with Pulmonary Arterial Sling Compressing the Trachea

A 4-month-old male infant was referred to Kyushu Koseinenkin Hospital for respiratory insufficiency. He was born at term after an uncomplicated pregnancy, weighing 2.7 kg. He was noted to have generalized pallor and decreased breath sound. Arterial blood gas analysis revealed hypercapnea (Pco2 84 mm Hg) and acidosis (pH 7.11). He underwent assisted ventilation with high peak-inspiratory and end-expiratory pressures. Chest radiography raised the suspicion of distal tracheal stenosis. Echocardiography demonstrated aberrant origin of the left pulmonary artery with a small ventricular septal defect.

Transpulmonary Stenting of Both Pulmonary Arteries with a Surgical Access through Redo Sternotomy

We report the case of a four-year-old boy with pulmonary atresia (PA) and ventricular septal defect (VSD) associated with MAPCAs who had a complete repair two times. He developed 21 months later stenosis at the origin of both pulmonary arteries. The conventional interventional catheterization approaches were not available due to femoral vein thrombosis and severe transient complete atrioventricular block due to the manipulations to catheterize the left pulmonary artery. The stenoses were treated by implant of stents using a surgical access to the proximal portion of a Contegra valved conduit (Medtronic, Inc., Minneapolis, MN, USA) by sternotomy.

Isolated origin of the left internal carotid artery from the pulmonary artery

The authors describe what is, to their knowledge, the first reported case of the anomalous origin of an internal carotid artery from the pulmonary artery. An otherwise asymptomatic 6-year-old girl, who presented with headaches and hypertension, underwent a comprehensive workup that revealed extensive meningeal and cerebral artery anastomoses to the left internal carotid artery--itself arising from the origin of the left pulmonary artery. This unique anatomical anomaly, caused by a disturbed pattern of aortic arch regression, resulted in a right-to-left vascular shunt into the pulmonary artery and a disturbance of intracranial artery flow patterns, complicating the management options.

Pseudopulmonary Artery Sling in a 22q11 Deletion Newborn with Tetralogy of Fallot and Isolated Left Subclavian Artery

We report an interest and mystifying cardiovascular imaging in a 22q11 deletion neonate with rare congenital heart defects, including anomalous origin of the left pulmonary artery arising from the right pulmonary artery and in front of the trachea, called pseudo-pulmonary artery sling, combined with isolated left subclavian artery confirmed by multidetector row computed tomography.

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. Fetal four-chamber view showing hypoplastic right ventricle. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. Fetal echocardiogram showing the anomalous origin of the right pulmonary artery from the ascending aorta and the normal left pulmonary branch from the main pulmonary artery. Ao, aorta; Lpa, left pulmonary artery; Pa, pulmonary artery; Rpa, right pulmonary artery. 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 cm3, left ventricular volume was 8.7 cm3 and the pulmonary valve was thick and stenotic with a gradient of 85 mmHg. The right pulmonary artery diameter was 6.7 mm and the left pulmonary artery diameter was 5.0 mm. Anomalous origin of a branch of the pulmonary artery from the ascending aorta is an extremely rare conotruncal malformation which accounts for only 0.12% of all congenital heart defects1. In most cases the anomalous branch originates on the posterolateral wall of the ascending aorta near the aortic valve. In 15% of cases the origin is distal near the base of the innominate artery. Anomalous origin of the right pulmonary branch is 5–6 times more frequent than that of the left1, 2. This anomaly can present alone or in association with other congenital heart defects, the most common being patent ductus arteriosus, tetralogy of Fallot, aorticopulmonary window and ventricular septal defect1-5. A search of the literature found only a few cases in which this anomaly was associated with right ventricular outflow obstruction (i.e. pulmonary stenosis and atresia)3-5. No cases were found in which this anomaly was associated with inflow and outflow obstruction and hypoplastic right ventricle. We could find only one published report on the prenatal diagnosis of this cardiac lesion6 and no other in which there was also hypoplastic right ventricle and inflow and outflow obstruction. Because surgical intervention is recommended as soon as possible, the prenatal diagnosis of this anomaly is important. Unfortunately, in our case, the family declined surgery and the baby was discharged from hospital. F. Öztunç*, A. Güzeltaş*, * Nural Köşkü Konutları B/31 Tepegöz Sok. Çiftehavuzlar—Istanbul-Turkey

Anomalous Origin of Left Pulmonary Artery from Aorta with Atrial Septal Defect

Anomalous origin of left pulmonary artery (ALPA) from aorta with atrial septal defect (ASD) is a rare congenital cardiac malformation. We report a case of ALPA from the aorta with ASD in a 1.8-kg baby. Repair was needed at an early age due to progressive congestive heart failure. Surgical correction was performed with cardiopulmonary bypass for closure of ASD and direct anastomosis of the ALPA onto the main pulmonary artery.

Double Outlet Right Ventricle with Anomalous Left Pulmonary Artery

We describe a rare case of anomalous origin of the left pulmonary artery from the ascending aorta with concomitant double-outlet right ventricle in a 2-year-old boy. He underwent successful 2-stage surgical treatment with transluminal balloon pulmonary valvuloplasty, followed by complete repair. A follow-up examination at 4 years after the operation showed good results.

Surgical Results of Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta Including Reoperation for Infrequent Complications

We evaluated the results of surgery for an anomalous origin of the right pulmonary artery from the ascending aorta. From August 1986 to December 2005, 8 children (6 neonates) aged 7 to 180 days (mean, 35 +/- 59 days) with anomalous origin of the right pulmonary artery from the ascending aorta underwent surgical repair at our institute. All except one child, who had the distal form, had the proximal form. Cardiac catheterization showed that the left pulmonary artery to systemic pressure ratio was 1.0 or more. Surgery was performed by direct anastomosis in 7 patients and by graft interposition in 1. There were no operative or late deaths. All patients postoperatively underwent cardiac catheterization that showed decreased left pulmonary artery to systemic pressure ratio ranging from 0.2 to 0.6. Follow-up periods ranged from 2 months to 13 years. We undertook reoperations for two infrequent postoperative causes. One patient exhibited significant supravalvar aortic stenosis and required patch enlargement of the ascending aorta 3 years after operation. The other patient (with the distal form) needed a reoperation after 1 month because of progressive stenosis at the anatomic site. Graft interposition was performed, and histopathologic examination showed that the tissue from the stenotic region looked like that of a ductus. We undertook surgical repair for anomalous origin of the right pulmonary artery from the ascending aorta. Pulmonary hypertension was improved in all patients. Careful follow-up was necessary to detect supravalvar aortic and anastomotic stenosis early and late after operation.

Isolated Atrioventricular Discordance: Surgical Experience

Isolated ventricular inversion (atrioventricular discordance with ventriculoarterial concordance) is an extremely rare presentation of cyanotic congenital heart disease. The mode of presentation is akin to D-transposition of great arteries as systemic venous drainage and systemic arterial output connect to the same side of the cardiac septae, and pulmonary venous drainage and pulmonary arterial outflow to the opposite. Systemic oxygenation relies on intracardiac or extracardiac modes of mixing, as does survival, similar to transposition of the great arteries. Published literature is scant, mainly because of the rarity of this cardiac anomaly. We review our surgical experience with this lesion. Five children with isolated ventricular inversion presented to us between the ages of 6 days and 22 months (mean, 12 months). Preoperative echocardiogram diagnosed large interventricular communication in 4, a patent ductus arteriosus in 4, and total anomalous pulmonary venous drainage with supracardiac connection in 1. One had associated narrowing of the left pulmonary artery origin. Four patients had atrial situs solitus, whereas 1 had right atrial isomerism. Three hearts had normally related great arteries whereas in 2, the aorta was to the right and anterior to the main pulmonary artery and arising in parallel fashion from the cardiac mass. Four children underwent trans-right atrial patch closure of the interventricular communications, with ligation of the patent ductus arteriosus. All 4 underwent a concomitant modified Senning's repair. The fifth patient underwent repair of total anomalous pulmonary venous drainage with a Mustard-type repair. One needed concomitant repair of the mitral valve for injury to a free edge chorda sustained during closure of the ventricular defect. There was 1 early death. Complete heart block developed in 2 children, of which 1 needed permanent pacemaker insertion, whereas the other converted to sinus rhythm with intermittent atrial tachycardia. All survivors are doing well on follow-up, at a follow-up duration ranging from 6 to 48 months (mean, 18). Repair of isolated atrioventricular discordance can be successfully achieved in the majority of patients presenting with this complex anomaly.

Unusual case of pulmonary valve atresia

No abstract available.

Crossed Pulmonary Arteries: Report of Two Cases with Emphasis on Three-dimensional Helical Computed Tomographic Imaging

Crossed pulmonary arteries are due to an anomalous origin of both pulmonary arteries from the main pulmonary trunk. This anatomy is often associated with other congenital cardiac and extracardiac diseases. We report two neonates with complex congenital heart disease who had this disorder, which was detected during cardiac computed tomography (CT) with three-dimensional reconstruction but not during echocardiography or angiography. The first patient was a 3-day-old male neonate who had tachypnea and feeding problems since birth. Cardiac CT showed crossed pulmonary arteries, type B interruption of the aortic arch, a ventricular septal defect, and a large patent ductus arteriosus. He received an emergency T-colostomy at 3 days of age because of severe bowel distention. Low-type imperforated anus was diagnosed. His postoperative course was complicated with fluctuated saturation, seizure, hypocalcemia, hyperphosphatemia, and sepsis. Also found were cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia, and a variable deletion on chromosome 22q11 (CATCH 22 disorder). Because of his poor prognosis, the patient was transferred to another hospital on day 16 for further care, at the family's request. The other patient was a 5-day-old female neonate who had a heart murmur since birth. Cardiac CT showed crossed pulmonary arteries, truncus arteriosus, type A interruption of the aortic arch, a ventricular septal defect, an atrial septal defect, and a large patent ductus arteriosus. She received complete surgical correction, including division of the patent ductus arteriosus and repair of the other defects. Intermittent respiratory distress and decreased blood pressure complicated her postoperative course, and she died on the eighth day after surgery. Crossed pulmonary arteries complicated accurate interpretation of two-dimensional echocardiographs of the great vessels, as well as the course and location of catheters during cardiac catheterization. Three-dimensional CT provided a noninvasive approach to clearly recognize these malformations and the related anatomic structures. This information is important in planning and performing surgery in neonates with crossed pulmonary arteries.

Multidetector Computed Tomography for Assessing a Pulmonary Artery Sling in a Pediatric Patient

A 4-month-old girl was referred to our department for evaluation of a heart murmur. There was no history of cardiac failure or cyanosis, but there was respiratory difficulty. Examination revealed a grade 3/6 continuous murmur at the right upper sternal border. At echocardiography, an aberrant origin of the left pulmonary artery was demonstrated (Fig. 1). The barium-enhanced esophagogram showed an anterior indentation at the level of the tracheal bifurcation. To assess the pulmonary vascular anatomy, we performed a multidetector computed tomography angiography (MDCTA). In this case we used a 64-detector CT scanner (VCT Lightspeed; GE Healthcare), with 0.625-mm slice thickness. The time required for the study was\10 s. CT at the level of the main pulmonary artery (MPA) showed the anomalous origin of the left pulmonary artery (LPA) from the posterior aspect of the proximal right pulmonary artery (RPA) as it coursed posteriorly between the trachea and the oesophagus to reach the left hilum (Fig. 2). CT described the compressed airway and stenosis of the right branch of the trachea (Fig. 3). The diagnosis of an abnormal origin of the LPA was confirmed at CTA. Surgery with total cardiopulmonary bypass was performed, and the left pulmonary artery was attached to the pulmonary trunk ventrally to the trachea. In patients with a suspected pulmonary artery sling, conventional angiography is the current diagnostic standard. CTA is a novel imaging tool that provides good anatomic detail information, with the advantage of being noninvasive, and is therefore very useful for the diagnosis of pulmonary vascular anomalies in pediatric patients [2]. The literature demonstrated the powerful ability of CTA to delineate the exact location and morphology of the pulmonary artery and trachea [1, 3].

Anomalous Origin of the Right Pulmonary Artery from the Aorta in a Cattle

Anomalous Origin of the Right Pulmonary Artery from the Aorta in a Cattle

Berry Syndrome Two Cases of Successful Surgical Repair

Berry syndrome is a very rare congenital cardiovascular anomaly that consists of a distal aortopulmonary window, aortic origin of the right pulmonary artery, an intact ventricular septum, a patent ductus arteriosus, and an interrupted aortic arch. Two cases of Berry syndrome are presented. A one-stage surgical correction for this complex anomaly was successfully performed, and a 1-year follow-up demonstrated favorable outcomes.

Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy

The most commonly reported coronary artery malformation leading to sudden death in children and young athletes is an anomalous coronary origin from the wrong aortic sinus, with or without a proximal course of the coronary artery between the aorta and the pulmonary artery. Although the incidence is difficult to assess, there is a 0.17% incidence in autopsy series (all variants included) and a 0.1 to 0.3% in catheter-echo diagnostic series. There may be a regional variation. In a prospective study of 1950 consecutive patients undergoing coronary angiography, anomalous origin of the right coronary artery from the left aortic sinus was found in 0.92% of cases, whereas the incidence of left coronary artery arising from the right aortic sinus was 0.15%. 1 In a study of 2388 patients who had transthoracic echocardiographic examination of their proximal coronary anatomy as part of innocent murmur investigation and evaluation of performance status, four patients (0.2%) were found to have an anomalous origin of the coronary artery from the wrong sinus. 2 Whatever the true prevalence, this lesion predisposes to fatal episodes of myocardial ischemia that may occur during or shortly after strenuous exercise. 3-5 Timely diagnosis requires a high index of suspicion but is frequently impossible, as the majority of patients with the anomaly are asymptomatic and thus do not seek medical attention. Therefore, sudden death is frequently the first manifestation, especially in young athletes, with only 30% of patients reporting prodromal symptoms. 5,6 Screening young adults before participation in competitive sports has been unsuccessful. Stress testing is unreliable because of high false-positive and falsenegative results; echocardiogram, echocardiography, and stress testing all lack predictive value. 4,5 Finally, although the major association of sudden death is with young athletes, cases have also been reported in children under 1 year of age.

Severe Hemoptysis of Pulmonary Arterial Origin: Signs and Role of Multidetector Row CT Angiography

Hemoptysis of pulmonary arterial origin is a diagnostic challenge in patients admitted to a respiratory ICU (RICU) for treatment of hemoptysis. Its early accurate recognition and treatment reduce morbidity and prevent mortality. Multidetector row CT angiography (MDCTA) is an accurate method for imaging the systemic vascular network. Our aim was to assess the MDCTA signs and role in managing hemoptysis of pulmonary arterial origin.We performed a retrospective clinical and radiologic analysis of all consecutive patients who were referred for severe hemoptysis to our RICU and were treated by endovascular means between January 2004 and December 2006. We reviewed all of those cases with hemoptysis of pulmonary arterial origin.Of 272 patients who were referred for severe hemoptysis to the RICU, 189 patients were treated by endovascular means. Thirteen patients (nine men, four women; mean age, 45 years) had hemoptysis of pulmonary arterial origin. Signs of pulmonary arterial hemoptysis seen on MDCTA were of the following three types: pseudoaneurysm (n = 5); aneurysm of the pulmonary artery (n = 3); or the presence of a pulmonary artery in the inner wall of a cavity (n = 5). Hypertrophy of the bronchial arteries seen on MDCTA associated with any of these signs predicted the necessity to treat both the bronchial and pulmonary arteries. Pulmonary artery vasoocclusion was performed as a first treatment in eight patients with such an association (n = 1) or without such an association (n = 7) along with bronchial artery embolization. The remaining five patients were treated with systemic artery embolization, followed by surgery (n = 1), pulmonary artery vasoocclusion (n = 3), and death from massive hemoptysis (n = 1).MDCTA performed prior to endovascular treatment allows the correct identification and early appropriate management of severe hemoptysis of pulmonary arterial origin.

A Simple and Feasible Technique for Repair of Anomalous Origin of the Left Pulmonary Artery from the Ascending Aorta

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare congenital cardiac malformation and the direct reimplantation technique has been the strategy of choice, although postoperative restenosis across the anastomosis site is frequently observed. In the described case, a simple and feasible technique was used with satisfactory results at 20-month follow-up.