Pulmonary Artery Agenesis Research Articles

Isolated right pulmonary artery agenesis

Isolated right pulmonary artery agenesis

Unilateral Pulmonary Artery Agenesis with Right Sided Aortic Arch

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly due to a malformation of the sixth aortic arch during embryogenesis. It can occur as isolated or may be associated with other congenital cardiovascular malformations. Right-sided aortic arch (RAA) is infrequently seen entity that can be related to various forms of congenital heart disease. Among these, with mirror-image branching of the major arteries is the most encountered subtype. We have presented here a rare case of UPAA with RAA and Kommerell’s diverticulum.

Catheter ablation for atrial fibrillation in a patient with unilateral left pulmonary artery agenesis: an enlarged right pulmonary vein caused arrhythmogenicity of atrial fibrillation

A 55-year-old male with persistent atrial fibrillation (AF) was admitted to our hospital for catheter ablation (CA). Enhanced computerized tomography before CA demonstrated a left shrunken lung and absence of the left main pulmonary artery ( Panel A ). Collateral flow via the coronary and bronchial …

A Case of Unilateral Pulmonary Artery Agenesis

A Case of Unilateral Pulmonary Artery Agenesis

Konjenital mitral kapak prolapsusuna eşlik eden nadir sağ taraflı pulmoner arter agenezisi olgusu

Konjenital mitral kapak prolapsusuna eşlik eden nadir sağ taraflı pulmoner arter agenezisi olgusu

Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

Abstract Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH), that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner's syndrome.

We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome).

Chronic Recurrent Unilateral Pulmonary Infection: Result of Congenital Unilateral Agenesis of Pulmonary Artery

Unilateral agenesis of the pulmonary artery (UAPA) is a rare congenital anomaly. This report describes a 52-year-old female who gave a long history of chronic, recurrent, left-sided pulmonary infections related to UAPA. For many years, she was managed medically but the infection continued to recur. She eventually underwent left pneumonectomy and made a good recovery.

Left pulmonary artery agenesis

Unilateral pulmonary artery agenesis is a rare malformation accounting for 1% of congenital heart defects. It can lead to several complications. We report the case of a 16-year-old female patient with no particular past medical history, presenting with recurrent low abundance hemoptysis within a context of preservation of patient’s general condition. Clinical examination showed no abnormalities and frontal (A) and lateral (B) chest x-ray revealed small left lung-field associated with decreased vascular pattern and mediastinal shift to the left. Given this radio-clinical presentation, diagnosis of suspected left pulmonary hypoplasia, sequelae of a previous pulmonary infection and endobronchial foreign body were made. Thoracic CT scan objectifies the absence of left pulmonary artery (C) and multiple systemic collateral circulation Left pulmonary parenchyma was hypoplastic, well ventilated and without bronchial abnormality (D). The descending aorta was located on the right. The electrocardiogram showed right bundle-branch block and transthoracic ultrasound did not reveal cardiac abnormality including sign of pulmonary arterial hypertension. Spirometry showed normal respiratory function as well as laboratory tests. Hemoptysis treatment was symptomatic and the patient was kept under medical monitoring.

Reparación completa quirúrgica en adultos con situación Fallot no operada o solamente paliada: ¿ficción o realidad?

Introduction-objectivesFallot patients, unoperated or just palliated, exceptionally survive into adulthood. Complete surgical repair in these patients is possible if they have adequate size of their pulmonary arteries. We present our experience. MethodsWe studied retrospectively 27 Fallot patients operated (complete surgical repair) from 1991 to 2014 in our adult congenital heart unit. ResultsMean age: 35,6±10,6 years, 59% male, 18,5% prior palliation. Principal diagnosis: double chamber right ventricle+ventricular septal defect (48%), tetralogy of fallot (30%). nyha functional class: i-26%, ii-30%, iii-44%. arrhythmia: 22%. Mean Hematocrit: 49±11%. Associated left pulmonary artery agenesis: 11%, severe aortic regurgitation: 11%. Additional tests showed high gradients between the right ventricle and pulmonary artery, and good biventricular function. We operated by median sternotomy with cardiopulmonary bypass, moderate hypothermia and aortic clamping. Ventricular septal defect was closed from right atrium: 63%. Reconstruction of the right outflow tract was done by preserving the pulmonary valve (78%), interposing bioprosthesis (15%), and using transannular patch (7%). Associated surgery in 5 patients (18.5%): tricuspid (1), aortic (4). Nobody died during inmediate postoperative period. Mean follow-up: 8.4±6.3 years (maximum-19.75). Late mortality: 1-patient. Reoperation in 3 patients (11%) because of residual lesions. Currently functional class i-65%, ii-31%, 4%-iii; sinus rhythm in 92%. ConclusionsComplete surgical repair in adulthood of Fallot patients achieves clinical improvement by eliminating the cyanosis, closing intracardiac shunts, and decreases right ventricular pressure overload. Our results are excellent and allow higher rates of pulmonary annulus preservation regarding children's series.

Unilatereal Pulmonary Artery Agenesis: A Rare Case as an Incidental Finding in an Elderly Male

Unilatereal Pulmonary Artery Agenesis: A Rare Case as an Incidental Finding in an Elderly Male

Pulmonary Artery Agenesis: A Case Series

Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis. The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.

Rare case of truncus arteriosus with anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) and unilateral left pulmonary artery agenesis.

The incidence of congenital heart disease (CHD) is 2.4-3.8/1000 live births. Up to 70.7 % of all cases of CHD are reported to be benign; complex heart anomalies are extremely rare. Our case is extremely rare, as we report three very rare findings-truncus arteriosus, anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), and unilateral left pulmonary artery agenesis-in a single patient. Congenital complex cardiac abnormalities are very rare, and two-dimensional echocardiography screening should be supported by cardiac computed tomography (CT). We report a case of truncus arteriosus associated with ARCAPA and left pulmonary artery agenesis diagnosed by cardiac computed tomography; we believe that such an unusual case with all three of these entities has never been reported before.

Isolated right pulmonary artery agenesis with aplasia of right upper lobe and with anomalous arterial supply from celiac axis, anomalous venous drainage

Pulmonary artery agenesis is the complete absence of the right or left pulmonary artery. This lesion is commonly associated with other cardiac anomalies and is usually diagnosed incidentally. Unilateral absence of pulmonary artery with lung hypoplasia is very rare. Ipsilateral hypoplastic or absent central pulmonary artery in these patients will have a systemic arterial supply to peripheral pulmonary arteries of the affected lung from the descending thoracic or upper abdominal aorta. These findings are usually characterized as pulmonary veno lobar syndrome or scimitar syndrome when associated with ipsilateral total or partial anomalous pulmonary venous return from the lower lobe which drains into the inferior vena cava or less commonly to the hepatic, azygous, or portal vein, or into the right atrium and lung agenesis/hypoplasia.

Looking beyond the thrombus: essentials of pulmonary artery imaging on CT.

BackgroundPulmonary arteries are not just affected by thrombus. Congenital and acquired conditions can also involve the pulmonary arteries. An awareness of these conditions is important for the radiologist interpreting chest computed tomography (CT).MethodsThe anatomy of the pulmonary arteries was reviewed. CT and magnetic resonance (MR) acquisition protocols for imaging the pulmonary arteries were discussed. The imaging appearances of congenital and acquired anomalies involving the pulmonary arteries, using CT and other modalities, were presented.ResultsImaging features of congenital anomalies presented include pulmonary agenesis, partial pulmonary artery agenesis, patent ductus arteriosus, pulmonary artery sling, congenital pulmonary artery stenosis and coronary to pulmonary artery fistula. Acquired pulmonary artery anomalies discussed include arteritis, infected aneurysm and sarcoma. Pulmonary artery filling defects besides thromboembolism are also discussed, including foreign body emboli. Imaging features of bronchogenic carcinoma and mediastinal fibrosis demonstrating compression of the pulmonary arteries are presented, followed by a brief discussion of post repair appearance of the pulmonary arteries for congenital heart disease.ConclusionsCongenital and acquired pulmonary artery anomalies have a characteristic appearance on a variety of imaging modalities. An acquaintance with the imaging features of these anomalies is needed to avoid misinterpretation and reach the correct diagnosis. Teaching Points • Discuss a variety of congenital and acquired anomalies of the pulmonary arteries.• Discuss the imaging appearance of the presented congenital or acquired pulmonary artery anomalies.• Describe CT and MR acquisition protocols for imaging the pulmonary arteries.• Review the anatomy of the pulmonary arteries.

Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: A rare case diagnosed during the postpartum period

Unilateral absence of a pulmonary artery a very rare congenital disorder. We here present a case of a 22-year-old female patient with agenesis of the right pulmonary artery accompanying patent ductus arteriosus and Eisenmenger syndrome, diagnosed by chest X-ray and multidetector computed tomography 5 days after giving birth.

Repair of congenital heart defects associated with single pulmonary artery.

Experience with complete repair of congenital heart defects associated with unilateral absence of a pulmonary artery is limited. The aim of this retrospective study was to present our surgical experience of this complex category of patients, to analyze immediate results of surgical interventions, and to suggest a rational surgical strategy. Of 37 patients with a single pulmonary artery who underwent complete repair of associated heart defects, the left or right pulmonary artery was absent in 32 and 5, respectively. The most frequent heart defects were tetralogy of Fallot (n = 25) and ventricular septal defect (n = 8). The median age of these patients was 7.1 years. Preoperative examinations included echocardiography, cardiac catheterization and angiocardiography, with quantitative assessment of the single pulmonary artery. In-hospital parameters of surgical outcome were analyzed. Recorded hospital mortality was 2.7% (1/37). The single death was in a patient with tetralogy of Fallot, agenesis of the left pulmonary artery, and a small diameter of the contralateral pulmonary artery (Nakata index 174 mm(2)·m(-2)). The right-to-left ventricular systolic pressure ratio after complete tetralogy of Fallot repair in patients who survived the operation was 0.58 ± 0.11. Complete repair of congenital heart defects in patients with unilateral absence of a pulmonary artery is associated with a relatively low risk. If the hilar artery is of adequate size, surgical intervention should attempt restoration of the communication between the disconnected hilar artery and the pulmonary trunk, in addition to repairing the heart defects.

Pulmonary Artery Agenesis with Bronchial Asthma

Unilateral Pulmonary Artery Agenesis (UPAA) is a rare congenital anomaly during the 4 th week of gestational age. It is defined as an absence of pulmonary parenchyma and its supporting artery. A 9-year-old girl was admitted to our hospital because of chronic cough. Chest examination showed a decrement in lung sound of right hemi-thorax with expiratory wheeze. Chest radiography (CXR) revealed a semi-opaque right hemi-thorax. Chest CT with intra-venous contrast demonstrated absence of the right pulmonary artery and lung parenchyma with hyper-inflated left lung and dextro-position of mediastinum. This case emphasizes that in patients with respiratory compliant and chronic cough CXR must be done to rule out similar diagnosis other than asthma.

Added value of cardiac magnetic resonance in a case of multiple congenital abnormalities

Sub-infundibular stenosis is a rare form of congenital heart disease, difficult to precisely diagnose, especially in adult patients. We report a case of an adult patient, referred to our hospital with long-standing dyspnea on exertion. She was finally diagnosed with sub-infundibular stenosis. During cardiovascular magnetic resonance imaging, right-sided aortic arch and left pulmonary artery agenesis were also identified.<Learning objective: The case described illustrates that cardiovascular magnetic resonance study is a powerful imaging technique in the congenital heart disease population, especially in the setting of multiple congenital abnormalities.>

Left pulmonary artery agenesis with pulmonary hypoplasia in an elderly patient: a rare case report

Proximal interruption of the unilateral pulmonary artery is a rare congenital anomaly, which is often associated with other cardiovascular abnormalities. It is usually diagnosed in children but rarely discovered in adulthood as an isolated phenomenon, occurring more frequently on the right side and is often associated with a contralateral aortic arch. We are presenting a rare case of a sixty year old male who was diagnosed with left lung hypoplasia due to proximal interruption of left pulmonary artery with left sided aortic arch without any associated cardiovascular anomalies. DOI: http://dx.doi.org/10.3329/bjms.v12i4.13689 Bangladesh Journal of Medical Science Vol. 12 No. 04 October ’13 Page 462-466