Unilateral Absence Of A Pulmonary Artery Research Articles

Chest Computed Tomography Findings in Unilateral Pulmonary Fibrosis Secondary to Chronic Hypoperfusion

Purpose: Unilateral lung fibrosis is uncommon and few cases secondary to parenchymal hypoperfusion have been reported, requiring further understanding of this entity. This study aims to report the chest computed tomography (CT) findings of patients with unilateral lung fibrosis related to parenchymal hypoperfusion observed in our institution. Patients and Methods: Patients with a chest CT between 2004 and 2022 showing a condition causing hypoperfusion of either lung and ipsilateral unilateral lung fibrosis were retrospectively identified. Clinical and scintigraphic data were collected. Pattern and distribution of fibrosis were recorded, and its progression was evaluated when follow-up was available. In adequate CTs, fibrosis was quantified using data-driven textural analysis (DTA). Affected and contralateral lungs and baseline and follow-up data were compared using the Wilcoxon signed-rank test. Results: Thirteen patients (male: 7, female: 6, median age: 61 y) were included; 5 with congenital unilateral absence of a pulmonary artery and 8 with fibrosing mediastinitis. The mean scintigraphic perfusion of affected lungs was 3.3% ± 1.1 compared with 96.7% ± 1.1 contralaterally (n = 7, P = 0.017). Fibrosis had a UIP pattern in one case, indeterminate in the others, and was most commonly diffuse craniocaudally and peripheral or central axially. DTA in 12 patients showed a mean fibrotic score of 32% ± 24.6 compared with 0.5% ± 0.4 in the contralateral lungs (P = 0.002). Median follow-up was 4.5 years (minimum to maximum: 1 to 13 y). Of 10 patients, fibrosis was progressive in 60%. DTA of 5 follow-up CTs showed increased reticulations (P = 0.043). Conclusion: In patients with lung hypoperfusion, the possible complication of lung fibrosis should be considered.

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

Early single-stage surgical revascularization of pulmonary artery in unilateral absence of a pulmonary artery

BackgroundThis research aims to summarize the findings of the early single-stage revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery.MethodsWe retrospectively analyzed the medical records of 10 patients with unilateral absent pulmonary artery, in which 7 were right and 3 were left, the median age and mean weight at surgery was 4 months and 5.6 kg, respectively. The patients received operation from January 2009 to June 2020.ResultsTen patients, 1 case associated with atrial septal defect, 2 cases with tetralogy of Fallot, and 1 case with aortopulmonary window. The mean diameter of the affected hilar pulmonary artery remnants was 3.14 ± 1.09 mm (1.6-5 mm), and the Z value was − 3.66 ± 1.86 (range, − 6.7 to − 1.75). All the patients received single-stage revascularization: tube graft interposition in 3 patients, autologous pericardial roll in 4, direct anastomosis in one, and main pulmonary artery flap angioplasty in the rest 3. No hospital deaths occurred. Mean follow-up in this cohort was 3.3 ± 1.9 years One case underwent percutaneous balloon dilatation due to new pulmonary artery stenosis. Nonetheless, the results were encouraging, symptoms have improved in all patients. The median Z value of the latest ipsilateral pulmonary artery diameter was − 1.88 (range, − 4.52 to − 1.35), a significantly improvement when compared to the preoperative value. The Z value of that in patients who using Gore-Tex tube increased relatively small.ConclusionsSingle-stage pulmonary artery revascularization is effective at restoring normal antegrade flow to the affected lung, resulting in improved diameter of the PA, regression of pulmonary hypertension, and patient’s symptoms. Revascularization by using the autologous tissue or autologous pericardium may obtain a preferred result. The new pulmonary artery stenosis certainly will need to be addressed in the long-term follow-up.

Long-term outcomes of percutaneous closure of patent ductus arteriosus associated with unilateral absence of a pulmonary artery.

This study aimed to evaluate the long-term outcomes of patients with patent ductus arteriosus (PDA) associated with unilateral absence of a pulmonary artery (UAPA). Patients diagnosed with PDA associated with UAPA between January 2005 and June 2019 were retrospectively enrolled in this study. Demographic and clinical characteristics, treatments, and follow-up information were evaluated. A total of 11 patients were diagnosed with PDA associated with UAPA. Percutaneous closure was successfully conducted in nine patients. The mean diameters of the PDA measured by aortogram and occluders were 5.3 ± 1.8 mm and 11.5 ± 3.9 mm, respectively. The median pulmonary systemic flow ratio (Qp:Qs) in five patients was 1.41, and the median total lung resistance was 12 Wood Units. The mean systolic pulmonary artery (PA) pressure was 68.3 ± 19.1 mmHg. In five patients with pre- and postprocedure catheter data, the systolic pulmonary arterial pressure decreased significantly after closure (from 77.0 ± 20.2 to 58.8 ± 17.5 mmHg; p = .024), as did the mean pulmonary arterial pressure (from 58.2 ± 14.6 to 39.0 ± 14.1 mmHg; p = .18). The PA pressure and heart size gradually decreased to normal levels in eight patients, and their quality of life was significantly improved. The ratio of lung to systemic circulation pressure was less than 0.75. In appropriate patients with PDA associated with UAPA, transcatheter closure of PDA has the potential to improve PA hypertension. A ratio of lung to systemic circulation pressure less than 0.75 may be an important reference index for predicting whether PA pressure can be reduced to a normal level after occlusion.

Autologous Tissue Technique to Repair Unilateral Isolated Absence of a Pulmonary Artery

Isolated unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly in which one branch pulmonary artery has no connection to the main pulmonary trunk (most often there is ductal origin). Without treatment, it may lead to ipsilateral pulmonary hypoplasia and contralateral pulmonary artery hypertension. To avoid these complications, early surgical repair of UAPA is necessary. Surgical strategies include direct anastomosis between the "isolated" branch pulmonary artery (PA) and the main pulmonary trunk or creation of an interposition graft using prosthetic material or flap techniques. We describe a surgical technique using a totally autologous interposition tube graft.

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

Unilateral Absence of a Pulmonary Artery

Unilateral Absence of a Pulmonary Artery

A case of bronchial artery aneurysm complicated with congenital unilateral absence of a pulmonary artery

A case of bronchial artery aneurysm complicated with congenital unilateral absence of a pulmonary artery

Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery

Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although there are several reports regarding pregnancy in patients with unilateral absence of a pulmonary artery, there are no case reports describing anesthesia for Cesarean section in a patient with unilateral absence of a pulmonary artery. We present a patient with unilateral absence of a pulmonary artery who underwent Cesarean sections twice at the ages of 24 and 26 years under spinal anesthesia for surgery and epidural analgesia for postoperative pain relief. Both times, spinal anesthesia and epidural analgesia enabled successful anesthesia management without the development of either pulmonary hypertension or right heart failure. Spinal anesthesia combined with epidural analgesia is a useful anesthetic method for a Cesarean section in patients with unilateral absence of a pulmonary artery.

Unusual presentation of rare congenital anomaly: Isolated unilateral absence of a pulmonary artery

Unilateral absence of a pulmonary artery (UAPA) is a rare defect. It may be associated with other congenital cardiovascular malformations which present at an early age. Isolated UAPA (IUAPA), i.e., without any other cardiovascular malformations, usually is asymptomatic and presents in adulthood. In infancy, IUAPA may be suspected by the presence of recurrent respiratory infections and pulmonary hypertension (PHT). Here, we present a child with IUAPA who presented with intermittent cyanosis which is unusual. Echocardiography done showed the presence of severe PHT with a patent foramen ovale (PFO) with a right to left shunt. Detailed echo revealed the absence of right pulmonary artery which was confirmed by a multislice detector computed tomography (MDCT). Infants with unexplained PHT should be screened for the possibility of IUAPA. MDCT scan and magnetic resonance imaging (MRI) can confirm the echocardiographic diagnosis. The treatment plan depends on the presence of symptoms, size of pulmonary artery, and collaterals. Early surgical or hybrid intervention may improve survival. Medical management includes treatment for congestive cardiac failure and pulmonary vasodilators for PHT. Infants with severe PHT are difficult to treat and have poor prognosis.

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

Bosentan in pulmonary hypertension secondary to unilateral absence of a pulmonary artery

Bosentan in pulmonary hypertension secondary to unilateral absence of a pulmonary artery

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.

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.

Agenesia de la arteria pulmonar derecha con hipoplasia pulmonar ipsilateral como hallazgo incidental en un paciente con asma

Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unusual finding in some of these patients. This paper reports the case of a 4-year-old male with recurrent respiratory infections and asthma symptoms, in who further studies found agenesia of right pulmonary artery with pulmonary hypoplasia of the same side.

A hidden cause of recurrent hemoptysis: unilateral absence of a pulmonary artery

A hidden cause of recurrent hemoptysis: unilateral absence of a pulmonary artery

Unilateral absence of right proximal pulmonary artery in the setting of severe pulmonary hypertension: considerations for surgical repair and perioperative management

We report a successfully treated case of unilateral absence of a pulmonary artery, associated with an atrial septal defect and chronic lung disease with severe pulmonary hypertension. Because this severe pulmonary hypertension could jeopardize postoperative hemodynamic, the ingenuity of surgical strategy was required. The atrial septal defect was left open as a safety "pop-off" valve, and prosthetic graft was chosen as a reconstructive material to avoid excessive dilatation from exposure to unpredictable postoperative pulmonary hypertension.

Unilateral Absence of a Pulmonary Artery: A Rare Disorder with Variable Presentation

Unilateral absence of a pulmonary artery (UAPA) is a rare condition with an estimated prevalence of 1 in 200,000 young adults. Most commonly, UAPA occurs in conjunction with cardiovascular abnormalities such as tetralogy of Fallot or cardiac septal defects, but it can also occur in an isolated manner. Patients with isolated UAPA can remain asymptomatic into late adulthood but usually report symptoms such as dyspnea or chest pain or suffer from hemoptysis or recurrent infections. Diagnosis can be difficult due to the rarity of the condition and its nonspecific presentation. We present a case of a 61-year-old man who presented for lung transplant evaluation and was found to have UAPA. Typical findings on chest radiograph, strategies for diagnosis, and available treatments are discussed.

Absence of the Right Pulmonary Artery Associated With a Partial Anomalous Pulmonary Venous Connection

Unilateral absence of a pulmonary artery is a rare anomaly. Patients may present with hemoptysis, recurrent respiratory infections, pulmonary hypertension, or congestive heart failure. This report describes the case of a missing right pulmonary artery associated with anomalous left pulmonary venous connection not previously described in the medical literature.

Age-Related Clinical Characteristics of Isolated Congenital Unilateral Absence of a Pulmonary Artery

Unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although UAPA has been reported previously, its age-related pathogenesis and symptoms remain unclear. This retrospective cohort study included cases of UAPA reported in Japan at medical meetings or in the literature from 1990 through 2009. Patients with other congenital cardiac defects were excluded from the study. Clinical status was assessed according to age, and the clinical course of patients with isolated UAPA was compared with that of patients who had UAPA with a patent ductus arteriosus (PDA). Of the 92 patients with UAPA identified, 78 had isolated UAPA (14 with PDA). Hemoptysis and collateral arteries were observed in 0 and 13% of patients with isolated UAPA who were younger than 1year, as compared with 24 and 50% of those 20years of age or older, respectively. Pulmonary hypertension was present in 5% of the patients aged 1 to 19years. Among patients 20years or older, however, 32% had pulmonary hypertension, and 8% died. Compared with isolated UAPA, UAPA with PDA was associated with an earlier diagnosis (median age, 20 vs. 0years; p=0.002), a higher prevalence of pulmonary hypertension (22% vs. 86%; p<0.0001), and a higher mortality rate (4% vs. 21%; p=0.046). Collateral artery formation and pulmonary hypertension progress with age in patients with UAPA. Early diagnosis and revascularization may prevent the age-related progression of UAPA.