Pulmonary Artery Agenesis Research Articles

CONGENITAL ABSENCE OF PULMONARY ARTERY

Isolated unilateral absence of a proximal pulmonary main artery is a rare congenital condition with a prevalence of 1 in 200,000 (1). The first case was reported in 1868, and to date, 350 cases have been reported (2). It usually occurs in conjunction with other cardiovascular anomalies such as tetralogy of Fallot, VSD, truncus arteriosus, etc. (2), and is typically diagnosed in childhood. Survival into adulthood is rare. Symptoms of this condition can include shortness of breath, chest pain, hemoptysis, reduced exercise tolerance, pleural effusion, and recurrent infections. We encountered a case of a 22-year-old female presenting with these symptoms. Initially, the symptoms suggested pulmonary embolism. However, further investigation through CTPA and Echocardiography revealed the absence of the left pulmonary artery. This case report emphasizes that patients showing signs and symptoms indicative of PAH should also be evaluated for the absence of a pulmonary artery." The pulmonary arteries originate from the sixth aortic arch during the sixteenth week of normal embryonic development. The proximal segment of the 6th aortic arch forms the initial portion of the right & left pulmonary arteries, respectively. The link on the arch's right side vanishes, whereas it establishes a connection through the ductus arteriosus on the left side. Pulmonary artery agenesis arises from the aberrant rotation of the afflicted segment's attachment to the aortic arch and dysfunctions during migration (3). Certain publications utilize the phrase “proximal blockage of the pulmonary artery” instead of “agenesis” to describe this anomaly. Although the intraparenchymal distal segment of the pulmonary artery is miniature, it is safeguarded (4). The prevalence of bronchopneumonia is ascribed to multiple causes, such as diminished lung tissue perfusion, impaired mucosal defense, and a possible unbalance of protease-antiprotease enzymes. Moreover, increased collateral blood flow may be associated with the onset of hemoptysis.

An Adult with Acute Breathlessness and Unilateral Diffuse Alveolar Opacities

Abstract Cardiogenic pulmonary edema is typically bilateral and results from the accumulation of excessive fluid in the interstitial and alveolar spaces of the lungs caused by left ventricular failure. Cardiogenic unilateral pulmonary edema (CUPE) is uncommon and most often documented in severe mitral regurgitation. CUPE in other cardiac diseases such as coronary artery disease is rare and occasionally associated with prolonged lateral decubitus position or vascular perfusion mismatch between lungs either congenital such as pulmonary artery agenesis or acquired due to preexisting lung conditions such as compensatory emphysema following lobectomy. The radiologic opacities of CUPE does not have characteristic features as described with bilateral pulmonary edema. The possibility of CUPE is often not considered initially as its clinical/radiologic features often resemble or are mistaken as pneumonia resulting in diagnostic delay and higher mortality.

Unmasking of Agenesis of Right Pulmonary Artery with Unilateral Absence of Perfusion on Lung Scintigraphy.

We present a rare finding on lung ventilation-perfusion (V/Q) scintigraphy for a woman with longstanding dyspnea. CT of the chest showed volume loss on the right side, which raised concern about possible bronchiolitis obliterans or Swyer-James-MacLeod syndrome; however, the right pulmonary artery could not be visualized. A subsequent V/Q scan showed absence of perfusion and decreased ventilation to the entire right lung, consistent with agenesis of the right pulmonary artery. The patient's clinical course and imaging features mimicked Swyer-James-MacLeod syndrome, which usually presents with a matched perfusion defect in a single lung or lobe on V/Q scanning. This case highlights the importance of a multimodality imaging approach to achieve a diagnosis.

Apport du scanner cardiaque dans la prise en charge des cardiopathies congénitales : expérience de l'institut de cardiologie d'Abidjan à propos de 27 cas

Introduction and objectivesIn contrast to developed countries, cardiac CT is not widely used in West Africa for the assessment of congenital heart disease, and has only recently been introduced in Côte d'Ivoire. The lack of data prompted this study, the aim of which was to describe our experience of the contribution of CT to the management of congenital heart disease in the Ivorian cardiology setting. Patients and methodThis was a prospective study which took place in the pediatric cardiology department over a period of 9 months (September 2022 to June 2023) which included all patients with congenital heart disease explored by echocardiography and cardiac scan. ResultsThe average age was 5.7 ± 4.7 years with extremes of 5 months and 16 years. We noted a female predominance with a sex ratio of 0.52. The main heart diseases were: tetralogy of Fallot (37.1%), followed by pulmonary atresia with open septum (18.52%). The assessment of the anatomy of the pulmonary arteries (81.48%), the search for aortopulmonary collaterals (59.63%) and finally the assessment of the anatomy of the aorta (18.52%) were the main indications. The practice of cardiac CT scanning has enabled better accuracy in the diagnosis and management of congenital heart disease in several cases: an interruption of the aortic arch was found in 1 case initially suspected of hypoplasia of the aortic arch, three cases of partial abnormal pulmonary venous return and one case of total abnormal pulmonary venous return. Also, agenesis of the left pulmonary artery with birth anomaly of the right coronary artery in the assessment of tetralogy of Fallot was found in 1 case. The CT scan made it possible to specify the exact topography of the aortopulmonary collaterals in four cases of pulmonary atresia with an open septum. ConclusionCT has enabled better visualization of the arterial and venous pulmonary tree, identification and exact localization of aorto-pulmonary collaterals, precise description of the three-dimensional anatomy of the coronary arteries, and better precision of congenital anomalies of the aorta.

Discovery of agenesis of the right pulmonary artery on pulmonary scintigraphy in a context of pulmonary hypertension

Discovery of agenesis of the right pulmonary artery on pulmonary scintigraphy in a context of pulmonary hypertension

Dual-Energy CT of Unilateral Pulmonary Artery Agenesis.

Dual-Energy CT of Unilateral Pulmonary Artery Agenesis.

Unilateral Pulmonary Artery Agenesis with Aneurysm of the Contralateral Pulmonary Artery.

Unilateral Pulmonary Artery Agenesis with Aneurysm of the Contralateral Pulmonary Artery.

Agenesis of the Left Pulmonary Artery, as Incidental Finding

Agenesis of the Left Pulmonary Artery, as Incidental Finding

Agenesis of the Right Pulmonary Artery Associated with Patent Ductus Arteriosus: A Case Report

Introduction: Unilateral pulmonary artery agenesis is a rare congenital malformation that can be the source of serious complications. Its diagnosis must be considered in the presence of recurrent respiratory infections. Adult patients are often asymptomatic, which makes the diagnosis difficult. Chest CT angiography is the reference examination. We report a case of agenesis of the right pulmonary artery associated with patent ductus arteriosus revealed at the age of 50 years on a thoracic CT angiography. Observation: A 50-year-old female patient, treated for pulmonary tuberculosis in childhood, presented with exertional dyspnea. Thoracic CT angiography showed agenesis of the right pulmonary artery with patent ductus arteriosus and right pulmonary hypoplasia. There was significant systemic vascularity with evidence of pulmonary hypertension and right heart failure. Discussion: Unilateral pulmonary artery agenesis has been rarely reported in association with a patent ductus arteriosus. Right pulmonary artery agenesis often presents as an isolated, asymptomatic finding without associated congenital heart defects. CT angiography is used to make the diagnosis. Management is often limited to surveillance. Conclusion: Isolated pulmonary artery agenesis is a rare congenital malformation but its true frequency is not well known because of the inherent difficulties in its diagnosis. The main problem remains the risk of serious complications which requires regular follow-up.

Unilateral pulmonary artery agenesis diagnosed in adulthood: a case report

Isolated pulmonary artery agenesis (UPAA) diagnosed in adulthood is a rare congenital anomaly. It occurs during embryological development where there is a fusion failure between sixth aortic arch and pulmonary truck of affected side. Here we report a case of an adult patient with isolated unilateral right pulmonary artery aplasia.

A Technique for Pulmonary Artery Reconstruction for Ductal Origin of the Right Pulmonary Artery

Ductal origin of the pulmonary artery is a rare cardiovascular anomaly; it is often mislabeled as absent pulmonary artery or pulmonary artery agenesis resulting in delayed interventions. We present a case of ductal origin of the right pulmonary artery highlighting the need for early detection and prompt intervention to reestablish perfusion to the lung. This approach allows for continued lung development and reduces the morbidity related to single lung physiology. The aim of this article is to describe a refinement in the operative technique used in the reconstruction of the ductal origin of the right pulmonary artery via creation of an interposition homograft roll for centralization. Ductal origin of the pulmonary artery is a rare cardiovascular anomaly; it is often mislabeled as absent pulmonary artery or pulmonary artery agenesis resulting in delayed interventions. We present a case of ductal origin of the right pulmonary artery highlighting the need for early detection and prompt intervention to reestablish perfusion to the lung. This approach allows for continued lung development and reduces the morbidity related to single lung physiology. The aim of this article is to describe a refinement in the operative technique used in the reconstruction of the ductal origin of the right pulmonary artery via creation of an interposition homograft roll for centralization.

Unilateral pulmonary artery agenesis with unilateral interstitial lung disease in an allergic bronchopulmonary aspergillosis patient

Unilateral pulmonary artery agenesis with unilateral interstitial lung disease in an allergic bronchopulmonary aspergillosis patient

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

Situación Fallot reparada en la edad adulta: resultados de 3 décadas de experiencia

IntroductionWe define “Fallot situation” as the existence of significant ventricular septal defect and right ventricular outflow obstruction. Survival into adulthood is extremely rare without complete surgical repair. We present our experience operating these adults. MethodsWe consider complete surgical repair in Fallot adult patients if they have pulmonary arteries with adequate size. Retrospective study: 45 Fallot patients operated from 1993 to 2022 in our adult congenital heart unit. Statistical analysis: SPSS® 20.0. ResultsMean age: 37±13 years, 53% male, 18% prior palliation. Principal diagnosis: double chamber right ventricle+ventricular septal defect (60%), tetralogy of Fallot (27%). NYHA functional class: i 31%, ii 38%, iii 31%. Arrhythmia 20%. Mean hematocrit: 48±10%. Associated left pulmonary artery agenesis 7%, severe aortic regurgitation 7%. Main surgical indication was pulmonary stenosis 98%. Additional tests showed high gradients between the right ventricle and pulmonary artery, and good biventricular function. Surgeries were performed by median sternotomy with cardiopulmonary bypass, moderate hypothermia and aortic clamp. Ventricular septal defect was closed through the right atrium (71%). Reconstruction of the right ventricle outflow tract was done by preserving the pulmonary valve (80%), interposing bioprosthesis (16%), and using transannular patch (4%). Associated surgery (18%): tricuspid (one), aortic (6), anomalous pulmonary venous drainage (one). Inhospital mortality: one patient (2,2%). Mean follow-up: 8.9±7.6 years. Late mortality: 2 patients (4.5%). Reintervention: 7 patients (15%) because of residual lesions (3 percutaneous, 5 surgical). Nowadays, functional class: i 68%, ii 28%, iii 4%; sinus rhythm 83%. ConclusionsComplete surgical repair in adulthood of Fallot patients achieves clinical improvement by eliminating the cyanosis, closing intracardiac shunts, and decreasing right ventricular pressure overload. Our results are excellent with good rates of pulmonary annulus preservation, low mortality and acceptable reintervention rate in the follow up.

Isolated Unilateral Pulmonary Artery Agenesis: Report of Two Cases

Isolated Unilateral Pulmonary Artery Agenesis: Report of Two Cases

Isolated Right Pulmonary Artery Agenesis with Agenesis of Right Upper Lobe and Bronchiectasis of Right Lower Lobe with Anomalous Arterial Supply from Celiac Axis with Normal Venous Drainage

Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly. When detected in infancy, the condition is commonly associated with cardiovascular defects which are more frequently associated with left pulmonary artery agenesis. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms and are diagnosed incidentally on the chest radiographs. We report a case of a 19-year-old female patient who presented to us with recurrent haemoptysis. She was symptomatic since the age of four years. We report the rare occurrence of UAPA on right side, agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply of right lung from coeliac axis in this patient.

Unilateral Pulmonary Fibrosis with Pulmonary Artery Agenesis (PAA): A Case Report

Unilateral pulmonary artery agenesis (AUAP) is a rare clinical condition that can be seen either in isolation or in combination with other congenital vascular disorders. When isolated, the patient may be almost asymptomatic and only become aware of this anomaly through medical examinations performed for other reasons.
 Here we discuss the case of a female patient with chest CT evidence of unilateral fibrous thickening, with pulmonary hypoexpansion; investigations revealed the coexistence of AUAP.
 This clinical association is described by very rare case-reports in the literature.

Isolated Unilateral Agenesis of Pulmonary Artery and Reappraisal of Embryological Evolution.

Isolated Unilateral Agenesis of Pulmonary Artery and Reappraisal of Embryological Evolution.

324 Unilateral pulmonary artery agenesis with controlateral embolism: a double vascular trouble

Abstract Methods and results A 60-year-old male patient underwent coronary angiography (CA) for a non-ST segment elevation myocardial infarction (NSTEMI). CA revealed significant multivessel disease. Both internal mammary arteries (AMI) were patent, with right IMA markedly larger than the left IMA. The exam revealed also an abnormal branch arising from the proximal right coronary artery extending backwards, likely to the right lung. Pre-operative chest radiograph demonstrated asymmetry of the two hemithoraces with slight elevation of the right hemidiaphragm, small ipsilateral lung, and mediastinal shift towards the right. The patient underwent urgent CABG surgery. Myocardial revascularization was successfully performed using both AMI and one saphenous vein segment. The postoperative course was complicated by respiratory failure requiring prolonged mechanical ventilation. A chest computed tomography angiography was performed, revealing complete absence of the right pulmonary artery and a left lower lobe segmental pulmonary embolism. Furthermore, blood in the hypoplastic right lung was supplied by multiple collaterals arising from RCA and right IMA. Intravenous heparin was started with clinical improvement. Two weeks later, a lung scintigraphy was performed, ruling out perfusion defects. The patient was discharged home on oral anticoagulation with warfarin. Conclusions Unilateral pulmonary artery agenesis (UPAA) is an uncommon congenital anomaly of the great vessels. Despite the absence of the pulmonary artery, blood supply of ipsilateral lung is provided by systemic collaterals originating from bronchial, intercostal, internal mammary, and sub-diaphragmatic arteries. More rarely, these collaterals may arise from the coronary arteries with different implications, ranging from asymptomatic condition to myocardial ischaemia and infarction. In our case, the condition was previously asymptomatic. The occurrence of pulmonary embolism contributed to worsen the ventilation–perfusion mismatch, explaining the respiratory failure during the postoperative period.

MASSIVE HEMOPTYSIS POST COVID-19 INFECTION IN THE SETTING OF UNILATERAL PULMONARY ARTERY AGENESIS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Unilateral pulmonary artery agenesis (UPAA) is a rare congenital condition with a prevalence of 1 in 200,000 adults3. It is often associated with other cardiovascular abnormalities, and occurs most frequently as an absence of the right branch. Hemoptysis has been reported in up to 10% of patients with UPAA1,2. Here we describe the case of a patient with UPAA and massive hemoptysis after SARS-CoV-2 infection. CASE PRESENTATION: A 27-year-old female with a past medical history significant for patent ductus arteriosus, atrial septal defect, hypoplastic right lung, agenesis of the right pulmonary artery, von Willebrand Disease, and iodinated contrast allergy who presented with complaint of persistent hemoptysis after COVID-19 infection. The patient had been diagnosed with COVID-19 two weeks prior, and was treated as an outpatient without significant symptoms. She subsequently developed acute onset of massive hemoptysis, and upon presentation was admitted to the intensive care unit and intubated for airway protection. The case was discussed with interventional radiology (IR). Given the patient's contrast allergy, bronchoscopy was pursued in an attempt to identify the location of bleeding. Flexible bronchoscopy showed extensive bilateral airway casting with clot as well as active bleeding from the right mainstem bronchus. Significant hypoxia was encountered due to the clot burden. Bronchial artery embolization (BAE) was discussed with IR, but there were concerns for lung necrosis due to embolization in the setting UPAA. Hematology evaluated the patient, and administered Factor VIII/von Willebrand Factor Complex. The patient was evaluated for ECMO in the setting of severe refractory hypoxia. Fortunately, the active bleeding resolved and a 14 cm clot was extracted using alligator forceps via a bronchoscope. The patient's severe hypoxia resolved and she was subsequently extubated. DISCUSSION: Massive hemoptysis is a life-threatening condition. Treatment is centered on identifying the location of the hemorrhage. BAE is often required if the bleeding cannot be managed endoscopically. Our patient presented a unique problem as her bleeding appeared to be coming from the right lung whose vascular supply subsisted from the bronchial artery system alone. There is a paucity of literature on this particular issue, and it is unclear if BAE increases the incidence of ischemia and tissue necrosis. Fortunately this patient avoided the need for BAE and ultimately had a favorable outcome. CONCLUSIONS: This case highlights not only massive hemoptysis in the setting of recent COVID-19 infection, but the difficulties that pulmonary artery agenesis can pose when attempting to provide treatment. REFERENCE #1: Lee EY, Boiselle PM, Cleveland RH. Multidetector CT evaluation of congenital lung anomalies. Radiology. 2008;247(3):632–48 REFERENCE #2: Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 2002;22 Spec No:S25–43 REFERENCE #3: Reading DW, Oza U. Unilateral absence of a pulmonary artery: a rare disorder with variable presentation. Proc (Bayl Univ Med Cent). 2012;25(2):115-118. DISCLOSURES: No relevant relationships by Arjan Ahluwalia, source=Web Response No relevant relationships by Henry Lam, source=Web Response No relevant relationships by Theresa Maitz, source=Web Response No relevant relationships by Brian Miller, source=Web Response No relevant relationships by Kaitlyn Musco, source=Web Response