Unilateral Absence Of The Pulmonary Artery Research Articles

Diagnostic Strategy for Suspected Unilateral Absence of the Pulmonary Artery.

To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam. All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue. The patients were initially examined clinically, followed by hematological testing, blood biochemistry testing, and chest X-ray radiology. The results suggested UAPA, so echocardiography and contrast-enhanced chest computed tomography (CT) were performed as soon as practical. The echocardiographic and CT imaging findings confirmed the suspected diagnosis of UAPA in all seven patients, which was accompanied by congenital heart disease in three patients. Three of the seven patients had mild and medium pulmonary hypertension. All seven patients were treated with drugs, which led to improvement in symptoms. Frontal chest X-ray provided the initial signs suggesting a diagnosis of UAPA. Subsequent echocardiography and contrast-enhanced chest CT were effective diagnostic tools for fast and accurate confirmation of UAPA.</p>.

Resection of infected bulla associated with isolated unilateral absence of pulmonary artery: a case report

BackgroundA unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly, which in most patients is associated with congenital cardiovascular abnormalities. Some patients with UAPAs do not have associated cardiovascular abnormalities, called isolated UAPA. Patients with isolated UAPA generally have a mild clinical course and may not be diagnosed until adulthood, but repeated complications such as infections may make surgery difficult.Case presentationA 34-year-old female patient with isolated right UAPA was referred to our hospital with a complaint of fever. Chest radiography and computed tomography showed an infectious cavity in the right upper lung. Surgery was scheduled for the patient; however, her preoperative condition needed to be optimized. She was evaluated for pulmonary hypertension and the collateral arteries were mapped out, and a waiting period after treatment of infection was set to avoid perioperative intrathoracic infections. Resection of the infected bulla was performed without any complications. No recurrence of infection was observed two years post-operatively. The residual lung expanded enough to fill the dead intrathoracic space.ConclusionsThis report suggests the importance of adequate preoperative evaluation and perioperative management for patients with isolated UAPA who require surgical resection.

Anomalous origin of the fetal pulmonary artery.

This study aims to investigate the efficacy of prenatal ultrasonography in diagnosing the anomalous origin of the fetal pulmonary artery (AOFPA). A total of 26 AOFPA cases were retrospectively analyzed from January 2014 to January 2023. The features of the AOFPA were characterized by comparing the prenatal ultrasonic data with the results of anatomical casting after pregnancy termination or postnatal imaging and surgical intervention. Missed diagnoses and misdiagnoses were expounded. Of the 26 AOFPA cases, there were 13 cases of pulmonary artery sling, 8 cases of anomalous origin of the unilateral pulmonary artery, and five cases of unilateral absence of the pulmonary artery; 17 cases received pathological anatomy and casting after pregnancy termination, and nine cases were confirmed by postnatal imaging and surgery. Nineteen cases were accurately prenatally diagnosed (19/26, 73.1%), and seven cases were missed or misdiagnosed (7/26, 26.9%). Prenatal ultrasonography has a favorable diagnostic efficacy for anomalous origin of the fetal pulmonary artery. The absence of either the left or right pulmonary artery from the image of pulmonary artery bifurcation may indicate origin abnormalities of the pulmonary artery in fetuses, which signifies the necessity to detect the abnormal origin of the pulmonary artery on the affected side and other potential intracardiac malformation complications.

Dynamic digital radiography: a novel quantitative modality to assess the pulmonary blood flow.

A 17-year-old girl with congenital unilateral absence of the pulmonary artery underwent surgical reconstruction of the right pulmonary artery (RPA) with a 6 mm polytetrafluoroethylene vascular graft 15 years ago. She had received stent implantation (S.M.A.R.T® 7.0 × 60 mm) to control the graft stenosis at 10-years-old. Nevertheless, regular hemodynamic assessment with balloon angioplasty was required because of the recurrence of graft stenosis. The current cardiac catheterization demonstrated RPA graft stenosis with a pressure gradient of 34 mmHg (Panel A). Tecnethium-99m-macroaggregated albumin lung perfusion scintigraphy showed decreased perfusion in the right lung (right/left: 20.1%/79.9%) (Panel B). A similar result was achieved in dynamic digital radiography (DDR) captured by a flat panel detector (Aero DR fine®, KONICAMINOLTA, Japan) and a conventional X-ray system (RAD speed Pro®, SHIMADZU, Japan), using a prototype workstation (KONICAMINOLTA, Japan) (right/left: 17.4%/82.6%). (Panel C, and Supplementary data online, Video) Lung perfusion scintigraphy has been a golden standard modality for the quantitative assessment of pulmonary blood flow laterality and is inevitably accompanied by radiation exposure. DDR is a new imaging to visualize small changes in pixel value representing the pulmonary circulation without contrast media.1,2 This imaging system can facilitate both qualitative and quantitative evaluations of the dynamic pulmonary circulation with a low cost, short examination time, and reduced radiation exposure. This convenient and non-invasive method will be an effective and alternative approach to assessing lung perfusion in patients with pulmonary artery stenosis.

Monosomy 18p and trisomy 17q in a boy with unilateral absence of the right pulmonary artery

Unilateral absence of the pulmonary artery (UAPA) is an infrequent finding, with an estimated prevalence of 1 in 200,000 young adults. The percentage of syndromic UAPA is not known but mostly does not have a syndromic association, only being reported with the 22q11 deletion syndrome.

Trans-arterial Embolization in a Patient with Unilateral Absence of Pulmonary Artery: Treatment Success and the Four-year Prognosis.

Unilateral absence of the pulmonary artery (UAPA) with or without other anomalies in the heart is a rare congenital malformation. A 55-year-old Filipino woman without a remarkable medical history was admitted to our hospital for hemoptysis. Contrast-enhanced chest computed tomography revealed the absence of the left pulmonary artery. Echocardiography and right heart catheterization showed no cardiac malformations or pulmonary hypertension. We diagnosed her with isolated left-sided UAPA and performed transarterial embolization of the left inferior phrenic artery. This resolved the hemoptysis, and there was no recurrence during the four-year follow-up period.

ISOLATED UNILATERAL ABSENCE OF THE PULMONARY ARTERY PRESENTING IN ADULTHOOD: A CASE REPORT

TOPIC: Lung Pathology TYPE: Fellow Case Reports INTRODUCTION: Isolated unilateral absence of the pulmonary artery (IUAPA) may rarely go undiagnosed into adulthood (1). CASE PRESENTATION: We report the case of a 44-year-old frequently homeless male with known history of chronic obstructive pulmonary disease (COPD) and pulmonary arterial hypertension (PAH), whose findings on computed tomography (CT) of the chest were consistent with IUAPA. DISCUSSION: This patient was referred to our advanced lung disease center following an episode of acute-onset hemoptysis, concurrent with long-standing history of progressive dyspnea. Infant and early childhood history was significant for pyloric stenosis, cryptorchidism, and scoliosis, but otherwise the patient reported being 'relatively healthy' through adolescence to early adulthood in exception of 5-6 hospitalizations for 'bronchitis treatments'. The patient was diagnosed with COPD in 2010 after complaints of increased dyspnea on exertion. Treatment with supplementary oxygen, tiotropium, and budesonide/formoterol was initiated with successful disease mitigation. In late 2019, the patient was hospitalized for the episode of hemoptysis, where he revealed non-adherence to oxygen and medical therapy, albeit infrequently, attributed to challenge in continued medication use. The patient was otherwise a poor historian, but reported frequent homelessness over the course of the last 9 years. At our center, CT of the chest revealed moderate cardiomegaly (Figure A, blue), bilateral subsegmental atelectasis, hypoplasia of the right lung with emphysematous changes and increased bronchial collateral circulation (Figure A-B, green), and absence of the right main pulmonary artery (Figure B, red). The patient is undergoing evaluation for lung transplantation at this time. CONCLUSIONS: This anomaly should be suspected if imaging reveals asymmetric lung fields, lung hypoplasia, or hyperinflation of the contralateral lung. Early recognition and management of IUAPA is important for preventing more serious complications such as PAH. REFERENCE #1: Jariwala P, Maturu VN, Christopher J, Jadhav KP. Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review. Indian J Thorac Cardiovasc Surg. 2021 Jan;37(Suppl 1):144-154. doi: 10.1007/s12055-020-01032-w. Epub 2020 Sep 23. PMID: 33584030; PMCID: PMC7859122. DISCLOSURES: No relevant relationships by Ashwini Arjuna, source=Web Response No relevant relationships by Michael Olson, source=Web Response No relevant relationships by Tejus Walia, source=Web Response

Isolated unilateral absence of the pulmonary artery.

Isolated unilateral absence of the pulmonary artery.

Intrapulmonary artery septation for unilateral absence of the pulmonary artery.

We describe a seven-month-old boy with tetralogy of Fallot and an absent left pulmonary artery. Due to the diminutive size of the left pulmonary artery, we performed a native tissue left pulmonary artery reconstruction and intrapulmonary artery septation procedure with a left modified Blalock-Taussig shunt. After confirming left pulmonary artery growth, the patient underwent tetralogy of Fallot repair, removal of septation patch, and division of the Blalock-Taussig shunt. Nine months post-surgery, we confirmed his balanced lung perfusion (R/L ratio 6:4). The intrapulmonary artery septation procedure would be suitable for both the resuscitation and reconstruction of the hypoplastic absent pulmonary artery.

Unilateral absence of the pulmonary artery: a one-stop-shop assessment with cardiac magnetic resonance imaging.

Unilateral absence of the pulmonary artery: a one-stop-shop assessment with cardiac magnetic resonance imaging.

Isolated unilateral absence of the pulmonary artery.

Isolated unilateral absence of the pulmonary artery (UAPA) is a rare malformation. It is associated with respiratory symptoms, such as dyspnea or hemoptysis. We suggest that surgical treatment should be positively considered in patients with UAPA who are severely symptomatic and who have no other cardiovascular or respiratory comorbidities.

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three-vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.

Developmental lung anomalies in adults: A pictorial review.

Developmental lung anomalies represent a heterogeneous group of diverse, yet related abnormalities that involve the lung parenchyma, pulmonary vasculature or a combination of both-which usually present prenatally and in early childhood. However, a substantial number of cases go unnoticed during childhood and present either incidentally or with recurrent respiratory infections progressing into adulthood. Defective development of the tracheobronchial tree and the pulmonary vasculature are proposed to cause these developmental anomalies. Encountering these lung anomalies in adults is a diagnostic challenge given their rarity and they are often mistaken as other serious pathological conditions, resulting in unnecessary diagnostic tests and procedures. The developmental lung anomalies in adults can be broadly divided into three categories: bronchopulmonary anomalies encompassing congenital bronchial atresia, bronchogenic cysts, congenital lobar emphysema and congenital pulmonary airway malformations. Vascular anomalies include pulmonary agenesis-aplasia- hypoplasia complex, unilateral absence of the pulmonary artery, pulmonary artery sling, partial anomalous pulmonary venous return, pulmonary venous varix and pulmonary arteriovenous malformations. Finally combined lung parenchymal-vascular anomalies of the lung involve hypogenetic lung (scimitar) syndrome and bronchopulmonary sequestration. This article discusses the spectrum of these developmental anomalies of the lung, their etiopathogenesis, clinical and radiographic presentations in adults and management in brief.

Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery.

Tetralogy of Fallot with unilateral absence of the pulmonary artery (UAPA) is a rare congenital heart disease. The aim of the present study was to examine the surgical strategy for Tetralogy of Fallot with UAPA by summarizing our experience of its treatment. We retrospectively evaluated 17 patients admitted to our hospital for treatment between 2006 and 2017. All patients were diagnosed with absence of the left pulmonary artery. The Nakata Index (NI), NI Z score, and McGoon ratio of the existing pulmonary artery were calculated. All patients were divided into one-stage complete repair (group A) or palliative procedure (group B) groups according to these criteria and surgical treatments. There were nine treated patients in group A, with a mean NI of 595.6 ± 690.32mm2/m2 (169.3-2433mm2/m2) and a mean NI Z score of - 1.57 ± 3.02 (- 4.60 to 5.27). There were eight treated patients in group B, with a mean NI of 107.61 ± 49.49mm2/m2 (53.15-216.39mm2/m2) and a mean NI Z score of - 6.27 ± 1.56 (- 8.22 to - 3.54). The mean follow-up time in group A was 5.58 ± 3.42years (1-11.7years), while that in group B was 5.4 ± 3.42years (0.6-12.1years). No hospital deaths occurred and the follow-up results were good in both groups. In conclusion, development of a single existing pulmonary artery can be evaluated using the NI and the NI Z score. These evaluations can be used to select complete repair or palliative procedures for patients and achieve good results.

Congenital unilateral absence of the pulmonary artery

Congenital unilateral absence of the pulmonary artery

ISOLATED CONGENITAL ABSENCE OF THE LEFT PULMONARY ARTERY WITH LUNG HYPOPLASIA

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Unilateral absence of the pulmonary artery (UAPA) is a rare congenital defect with significant physiologic sequelae. Here, we describe a patient presenting with congenital isolated left-sided UAPA. CASE PRESENTATION: A 27-year-old African male presented with six weeks of flu-like symptoms. He was born in Sierra Leone and immigrated to the United States at age 17. To evaluate his persistent cough and left side chest discomfort, a chest x-ray was obtained, showing significant opacification of the left hemithorax with left pneumothorax initially concerning for necrotizing pneumonia. He was placed on broad spectrum IV antibiotics and admitted. A follow-up computerized tomography of the chest found significant hypoplasia of the left lung with changes consistent with chronic infection, a significantly hypoplastic left pulmonary artery (PA), and hyperexpanded right lung. An echocardiogram found the right PA to be 1.8 cm in diameter compared to the left PA at 1.1 cm. His antibiotic regimen was changed to Augmentin to cover community-acquired pneumonia and he was discharged with plan to follow up with Cardiothoracic Surgery. DISCUSSION: UAPA is a rare condition that typically presents with associated congenital heart defects. Isolated presentation is even rarer, especially on the left side. During embryogenesis, the extrapulmonary PAs arise from the sixth aortic arch. Improper development, possibly due to persistence of a ductus arteriosus, leads to agenesis of the ipsilateral PA. While revascularization from collateral circulation arising from other arteries is possible, typically, inadequate blood flow to the ipsilateral lung during fetal development leads to hypoplasia of the lung.A UAPA essentially cuts pulmonary circulation in half, resulting in a diversion of cardiac output to the remaining PA. Studies in pigs have shown medial hypertrophy of the larger PAs in the unaffected lung and increased muscularity of the smaller arteries. Pulmonary hypertension developed in 44% of all patients with associated right ventricular hypertrophy. This can ultimately lead to right heart failure and eventually death.While many present in infancy, it is not uncommon for patients to remain asymptomatic until adulthood, when they may present with dyspnea on exertion, hemoptysis, or recurrent pulmonary infections. Patients often have a hypertrophied contralateral lung to compensate for the hypoplastic affected lung, leading to a mediastinal shift towards the affected lung. Despite this compensation, patients often have decreased pulmonary function. CONCLUSIONS: Isolated left UAPA results in left lung hypoplasia secondary to lack of blood flow during development. The diversion of cardiac output through the remaining PA may result in pulmonary hypertension and right-side heart failure. Pulmonary function is usually decreased despite contralateral lung compensation. Reference #1: Steiropoulos P. Unilateral pulmonary artery agenesis: a case series. Hippokratia. 2013;17(1):73–6. Reference #2: ADJ TH, NA B, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002;122(4):1471–7. Reference #3: Kruzliak P, Syamasundar RP, Novak M, Pechanova O, Kovacova G. Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis. 2013;106:448–54. DISCLOSURES: My spouse/partner as a Employee relationship with SAS Please note: >$100000 Added 02/28/2018 by Audrey Hopping, source=Web Response, value=Salary No relevant relationships by Vikas Pathak, source=Web Response No relevant relationships by Christine Zhou, source=Web Response

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.

Isolated Unilateral Absence of Pulmonary Artery: Adult Manifestations of a Congenital Disease

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Unilateral absence of pulmonary artery (UAPA) is a rare congenital disorder. In the absence of other lesions, is survivable into adulthood. Adult patients can present with chest pain (CP), dyspnea on exertion (DOE), recurrent infections, or hemoptysis. Clinical diagnosis is difficult without radiographic support. CASE PRESENTATION: 56 year-old female former smoker with moderate obstructive lung disease presented for second opinion after multiple hospital admissions for CP, DOE and recurrent bronchitis. Hyper-inflated left lung, right mediastinal shift, and absent right hilar shadow noted on chest x-ray (CXR). CT angiogram revealed hypoplastic right lung with right greater left centrilobular emphysema, right nodularity, ground glass, and abrupt cessation right pulmonary artery. Right and left heart catheterization revealed mean pulmonary pressure of 27, and large arterial fistula between the left circumflex coronary artery and several branches of the right pulmonary vasculature. CT coronaries showed proximal interruption of the right pulmonary artery with collateral vasculature recruited from SA nodal branch of the left circumflex, bronchial, internal mammary, intercostal, and phrenic arteries. DISCUSSION: Adult patients with isolated UAPA are often asymptomatic. Common presentations are pulmonary infections, CP, DOE, hemoptysis or pulmonary hypertension (PAH). CXR shows ipsilateral contracted lung with mediastinal, tracheal shift toward the affected side, absent hilar shadow, and contralateral lung hyperinflation. Diagnosis is confirmed with contrast-enhanced CT. Recurrent infections can be secondary to poor blood flow to the affected lung, reduced delivery of inflammatory cells and impaired ciliary function. Decreased blood flow can also result in alveolar hypocapnia causing secondary bronchoconstriction and mucus trapping. PAH results from increased blood flow to the contralateral pulmonary artery, increased shear endothelial stress, and release of vasoconstrictors. Our patient's chest pain was felt not due to coronary steal as cardiac stress testing was negative. There is no clear consensus on treatment for UAPA other than symptom management. CONCLUSIONS: Although isolated UAPA is a congenital abnormality, it can remain undetected until adulthood. Clinicians need to be aware of characteristic CXR findings that may suggest UAPA. . Reference #1: Harke, A et al. Isolated Unilateral Absence of a Pulmonary Artery: A Case Report and Review of the Literature.” CHEST 2002; (122.4) 1471-477. Reference #2: Yiu MWC et al. Radiological features of isolated unilateral absence of the pulmonary artery. J HK Coll Radiol. 2001;4:277-280. Reference #3: Kruzliak, P et al. Unilateral Absence of Pulmonary Artery: Pathophysiology, Symptoms, Diagnosis and Current Treatment. Archives of Cardiovascular Diseases 2013; 106.8-9: 448-54 DISCLOSURE: The following authors have nothing to disclose: Santosh Reddy, Kimberly Cao No Product/Research Disclosure Information

Repair of Unilateral Absence of Right Pulmonary Artery With Contralateral Pulmonary Artery Autograft Interposition in an Infant

Unilateral absence of the pulmonary artery (PA) is a rare congenital abnormality. Although a traditional 2-stage approach with a preceding systemic-to-PA shunt followed by a definitive repair is mandatory in patients with absent extrapericardial pulmonary arteries, more creative solutions are necessary when 1-stage repair is concerned. We present anovel technique for repairing the absent intrapericardial right pulmonary artery (RPA) with a contralateral PA autograft segment interposition. We believe that this technique using solely autologous tissue is effective in patients in whom the intrapericardial segment of the PAs is absent.

Unilateral absence of the pulmonary artery: a report of two cases that presented different clinical courses.

We recently diagnosed two cases of isolated unilateral absence of the pulmonary artery just after birth. Through the therapy, we could not prevent obstruction of the pulmonary artery and lead to complications. There have been no symptoms attributed to isolated unilateral absence of the pulmonary artery so far. We should carefully consider the strategy for therapeutic intervention for asymptomatic cases.