Unilateral Absence Of Pulmonary Artery Research Articles

A silent anomaly: congenital unilateral absence of pulmonary artery incidentally discovered in adulthood, presenting as pulmonary hypertension and chronic respiratory failure—case report

A silent anomaly: congenital unilateral absence of pulmonary artery incidentally discovered in adulthood, presenting as pulmonary hypertension and chronic respiratory failure—case report

Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review.

Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation associated with hemoptysis, pulmonary hypertension, and infection. Little is known about the impact on pregnancy outcomes. We sought to synthesize the existing literature on pregnancy outcomes in patients with maternal UAPA. We report a case of maternal UAPA and performed a systematic review of the existing literature. Articles in English reporting pregnancy outcomes among women with unilateral absence or hypoplasia of the pulmonary artery were included. Articles were reviewed at the abstract level and, if eligible, at the full-text level by two independent reviewers with disagreements adjudicated by a third reviewer. Data were abstracted by two independent reviewers. Outcomes of interest were mode of delivery, gestational age at delivery, intensive care admission, maternal death, and length of stay. Summary statistics for each outcome are presented. We identified 14 studies, including the presented case, reporting outcomes in 22 pregnancies impacted by maternal UAPA. Median age at diagnosis was 21 years. Concurrent cardiac comorbidities were reported in 6/13 (46.2%) with pulmonary hypertension in 5/20 (25%) of cases where this information was reported. We observed high rates of preterm birth (4/12, 33.3%), cesarean delivery (10/15, 66.7%), and operative vaginal delivery (2/5, 40.0%). There was one maternal death occurring in the immediate postpartum period for a mortality rate of 4.5%. Our study provides a comprehensive review of existing literature on maternal UAPA. Our findings suggest increased rates of adverse outcomes and underscore the importance of early diagnosis, identification of pulmonary hypertension, and multidisciplinary care. · There may be increased adverse outcomes in maternal UAPA.. · Concurrent cardiac abnormalities are common in maternal UAPA.. · Early diagnosis, identification of pulmonary hypertension, and multidisciplinary care are important..

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.

Isolated unilateral absence of pulmonary artery- an unusual cause of hemoptysis: a case report

BackgroundIsolated unilateral absence of one pulmonary artery (IUAPA) is a rare malformation and a rare cause of hemoptysis. It occurs due to malformation of the sixth aortic arch during embryogenesis. This condition has variable presentations like dyspnoea, reduced exercise tolerance, chest pain, pleural effusion, recurrent bronchopneumonia, pulmonary hypertension, and haemoptysis can be asymptomatic.Case PresentationWe describe a case of a 34-year-old female presenting with recent onset hemoptysis associated with mild dyspnoea and cough. Chest Radiograph revealed unilateral hyperlucent lung field which was later diagnosed as Isolated unilateral absence of pulmonary artery on high resolution computed tomography of chest with angiography.ConclusionAbsent pulmonary artery should be considered as an important differential in cases presenting with hemoptysis. This case emphasises the significance of combining computed tomography with pulmonary angiography to accurately evaluate and define congenital lung abnormalities.

Pulmonary vein wedge angiography in a child with unilateral absence of pulmonary artery.

A 3-month-old boy was admitted to Hainan Women and Children's Medical Center on November 17, 2019, because of feeding difficulty, without a history of cyanosis, anemia, edema, oliguria, or chronic cardiac diseases, as well as a family history of cardiac diseases or other disorders. Physical examination revealed a systolic murmur of grade II at the left ventricular apex. Laboratory findings showed no abnormalities in either the serum creatine kinase or the serum creatine kinase isoenzyme and anemia, with arterial oxygen partial pressure (PaO2) of 71 mmHg and arterial oxygen saturation (SaO2) of 94%.

Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: a single-center retrospective study.

The early diagnosis of unilateral absence of pulmonary artery (UAPA) in children offers an opportunity for effective intervention. Due to the lack of clinical evidence, a consensus regarding surgical treatment has yet to be reported. The aim of this study is to evaluate the effectiveness and safety of pulmonary artery (PA) reconstruction with a "two-segment" technique to repair UAPA in patients with pulmonary hypertension. Intraoperatively, the ligamentum arteriosum connecting the innominate artery and distal PA was dissected and occluded. A conduit created by fresh autologous pericardium formed the first "segment" of the neo-PA. The second "segment" was a Gore vascular graft with integrated rings anastomosed between the proximal end of the pericardial conduit and the main pulmonary artery (MPA). A total of five consecutive patients were included, and the absent PA was successfully reconstructed using the "two-segment" technique in all patients. Following revascularization, the direct measurement of the pressure in MPA during the operation showed that the average mean pulmonary artery pressure (mPAP) decreased from 31.3±16.0 to 16.8±4.2 mmHg (P=0.047). The average mPAP/radial mean arterial pressure (rMAP) ratio decreased from 0.59±0.27 preoperatively to 0.30±0.10 postoperatively (P=0.028). The mean follow-up period was 18.85±4.67 months. The median diameter of the reconstructed PA (pericardial segment) measured by transthoracic echocardiography (TTE) was 6.1 mm. One patient safely underwent a redo operation to repair relative stenosis in the neo-PA. Early PA reconstruction may effectively alleviate pulmonary hypertension in children with UAPA. The "two-segment" technique is safe and can facilitate potential redo pulmonary arterioplasty. Anticoagulation and antiplatelet therapy, as well as frequent follow-up, is required after the operation.

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

An isolated congenital absence of right pulmonary artery associated with pulmonary hypertension with coronary collaterals: A case report

Unilateral absence of pulmonary artery (UAPA) resulting from a defect in the aortic arch's embryogenesis. Patients who survive into adulthood is rare. Herein, we presented a case of UAPA with pulmonary hypertension in a 49-year old female.

Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot.

The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1-3% of the patients with tetralogy of Fallot will have unilateral absence of pulmonary artery (UAPA). However, coexistence of APV with tetralogy of Fallot (TOF) and UAPA is extremely rare. This rare subset can present in two forms. In one group, the main pulmonary artery continues as either left or right pulmonary artery (UAPA) and there is true absence of contralateral pulmonary artery. The second group is termed as unilateral anomalous origin of pulmonary artery (UAOPA), in which the contralateral lung is supplied either by patent ductus arteriosus or a collateral from the aorta. There are a limited number of these cases in the literature. We present a rare case of TOF with APV and UAPA managed using a different surgical technique. Also, we have done contemporary literature review.

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.

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.

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.

UNILATERAL ABSENCE OF PULMONARY ARTERY COMPLICATED BY SEVERE PULMONARY HYPERTENSION: FAVORABLE RESPONSE TO PARENTERAL THERAPY

TOPIC: Pulmonary Vascular Disease TYPE: Fellow Case Reports INTRODUCTION: Unilateral absence of pulmonary artery (UAPA) is a rare disorder that can present as an isolated lesion or in association with other congenital heart defects. An important complication of UAPA is the development of pulmonary hypertension (PH). Herein, we discuss a patient with severe PH in the setting of congenital UAPA and highlight the hemodynamic response to combination vasodilator therapy that included parenteral treprostinil. CASE PRESENTATION: A 58-year-old female with a history of congenital left UAPA, left lung hypoplasia and severe PH presented for evaluation of repeated syncopal episodes on minimal exertion. Two years prior to presentation, RHC showed mPAP 56 mmHg, PAWP 11 mmHg, CO 3.63 L/min, CI 1.9 L/min/m2, and PVR 12.4 WU. The patient was receiving riociguat 2.5 mg po TID, ambrisentan 10 mg po daily, and selexipag 1600 mcg po BID. She was NYHA FC IV and required oxygen at 6 L/min via oxymizer. Upon presentation, she was severely hypoxic requiring HFNC at 50 L/min and fraction of inspired oxygen of 0.9. Evaluation revealed Pro-BNP of 6170 pg/ml and cardiac echo showed an EF of 45%, severe RV dilation with severe hypokinesis and RVSP of 100 mmHg. Chest CT with contrast showed absence of the left pulmonary artery, enlargement of the main and right pulmonary arteries without pulmonary embolism, hyperinflation of right lung, and hypoplasia of the left lung. PFTs showed TLC 64%, FEV1/FVC 86, FEV1 43%, FVC 39%, and DLco 23% of predicted. VQ scan showed marked decreased perfusion of the left lung, decreased ventilation of the anterior left lung with some patchy ventilation of the posterior aspect of the left lung, and normal right lung perfusion. Repeat RHC showed significant worsening of hemodynamics with mPAP of 76 mmHg and PVR of 28.6 WU. The patient was transitioned from selexipag to IV treprostinil with rapid titration to 40 ng/kg/min over a few days. There was improvement in her dyspnea and oxygenation with weaning of supplemental oxygen from HFNC to 6 L/min oxymizer. Pro-BNP trended down to 332 pg/ml and she had no recurrence of syncopal episodes. The patient was transitioned from IV to SQ treprostinil and further titration of SQ treprostinil was continued at home to 64 ng/kg/min. After 2 months, repeat echo showed improvement with an EF of 67%, moderate RV dilation with mild hypokinesis and RVSP 60 mmHg. Repeat RHC showed hemodynamic improvement with mPAP of 51 mmHg and PVR of 10.5 WU. DISCUSSION: Patients with UAPA and lung hypoplasia who develop PH pose a management dilemma as no treatment consensus exists. Therapeutic approaches should be based on severity of symptoms, pulmonary artery anatomy, associated cardiovascular anomalies, collateral circulation and hemodynamic parameters. CONCLUSIONS: Select patients with severe PH may benefit from a cautious trial of combination vasodilator therapy under close observation. REFERENCE #1: 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(8-9):448-454. doi:10.1016/j.acvd.2013.05.004 DISCLOSURES: Speaker/Speaker's Bureau relationship with United therapeutics Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Honoraria Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with United therapeutics Please note: $1001 - $5000 by Karim El-Kersh, source=Web Response, value=Consulting fee Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with Actelion Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Consulting fee Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with United Therapeutics Please note: Current Added 04/23/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Actelion Please note: 2019 and 2020 Added 04/23/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with United Therapeutics Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Advisory Committee Member relationship with United Therapeutics Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Advisory Committee Member relationship with Actelion Please note: 2019 and 2020 Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Consultant relationship with Acceleron Pharma Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee No relevant relationships by Meliha Hrustanovic-Kadic, source=Web Response

A CASE OF RECURRENT PNEUMOTHORACES IN A NEWBORN

TOPIC: Pediatrics TYPE: Fellow Case Reports INTRODUCTION: Recurrent pneumothroaces (PTX) is an uncommon presentation in the newborn period, often associated with prematurity or ventilator use. This is a case of rapidly progressive cystic disease in the setting of an absent right pulmonary artery. CASE PRESENTATION: Patient is a 18do female, ex-37 weeks gestation, with negative prenatal history. Noted at birth to have meconium aspiration at birth, cyanosis and non-invasive positive pressure ventilation (PPV) at 9 hours of life. CXR showed R PTX, that was resolved with chest tubes and discharged on day of life (DOL) 11. Presented on DOL) 17 with respiratory distress and was found to have a PTX. Chest CT showed R PTX with cystic foci in all three lobes thought to be related to pulmonary interstitial emphysema (PIE). Cardiothoracic surgery felt patient was too young for surgery and recommended more conservative management. Presented on DOL 27 with recurrrent PTX and then again at 3 months of life. Chest CT at this time showed a absent R pulmonary artery not previously recognized. ECHO confirmed absence of R PA. Patient underwent R pneumonectomy with mediastinal tent placement. Pathology showed pneumatoceles in lower lobes, focal areas of dysplastic lung development, absent large hilar pulmonary arteries, lymphangiectasis and pleural fibrosis. Cytology showed few multinucleated giant cells and fibroblastic proliferation along pneumatocele wall. DISCUSSION: Air leak syndromes such as PTX and PIE are more common in neonates with prematurity, need for PPV, meconium aspiration, and pulmonary hypoplasia. The presence of meconium aspiration and need for PPV likely contributed to the initial pulmonary insult that led to her clinical picture. Persistent PIE can associated with multiple large pseudocysts. Presence of multinucleated giant cells with fibroblast proliferation in cytology further supports presence of persistent PIE. In addition the unilateral absence of the R pulmonary artery (UAPA) can contribute to hypoperfusion and hypoplasia. This can subsequently lead to the cystic development especially when there are multiple other insults to the lung as seen in this patient. Post-pneumonectomy complications include concern for development of pulmonary hypertension as well as complication related to the post-pneumonectomy space, such as mediastinal shift causing compression of the airways. CONCLUSIONS: Recurrent PTX can be due to a wide variety of etiologies based on patient's age and presentation. This case highlights recurrent PTX in a neonate in the setting of cystic lung disease that is likely from persistent PIE with further contribution from a unilaterally absent pulmonary artery. REFERENCE #1: Jeng, M.J., Lee, Y.S., Tsao, P.C. & Soong, W.J. Neonatal air leak syndrome and the role of high-frequency ventilation in its prevention. J Chin Med Assoc 75, 551-559 (2012). REFERENCE #2: Agrons, G.A., Courtney, S.E., Stocker, J.T. & Markowitz, R.I. From the archives of the AFIP: Lung disease in premature neonates: radiologic-pathologic correlation. Radiographics 25, 1047-1073 (2005). REFERENCE #3: Kruzliak, P., Syamasundar, R.P., Novak, M., Pechanova, O. & Kovacova, G. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis 106, 448-454 (2013). DISCLOSURES: No relevant relationships by Ashish George, source=Web Response no disclosure on file for Robin Ortenberg; No relevant relationships by Swaroop Pinto, source=Web Response

Surgical management in tetralogy of Fallot with rare unilateral pulmonary anomalies: A literature review.

Tetralogy of Fallot with unilateral pulmonary anomalies such as the unilateral absence of pulmonary artery or unilateral pulmonary agenesis is an extremely rare complex congenital heart anomaly. There is no established surgical algorithm for tetralogy of Fallot with concomitant unilateral pulmonary anomalies. This condition is still challenging, especially in the surgical field. In this review we also present our experiences in our center, Dr Cipto Mangunkusumo General Hospital, Jakarta, Indonesia. This literature review aimed to discuss systematic treatment options and hoped to help the decision-making process when surgeons face these rare anomalies.

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.

A comprehensive study of congenital unilateral absence of branch pulmonary artery associated with other congenital heart defects and ipsilateral non-unifocalizable major aorto-pulmonary collateral arteries: A single-center retrospective study.

Introduction:Congenital unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with the complete absence of intrapericardial segment of one of the branch pulmonary arteries. Sixty percent are associated with other congenital heart defects (CHD) that often need correction.Aim:To analyze the data of patients with UAPA and ipsilateral non-unifocalizable major aortopulmonary collateral arteries (MAPCAs) associated with other CHD to identify the commonly associated CHD, their management strategies and outcomes.Materials and Methods:Retrospective data of patients admitted for congenital UAPA with other CHD was compiled from hospital records from 2002 to 2015. The associated CHD were categorized as group I with the decreased pulmonary flow and group II with increased pulmonary flow to the unaffected contralateral pulmonary artery. The determinants of their management were analyzed.Results:Sixty-five patients of UAPA and ipsilateral non-unifocalizable MAPCAs associated with other CHD were identified. Group I had 41 patients and Group II had 24. The most common CHD associated with UAPA was tetralogy of Fallot (TOF) in 31 patients (47.7%). Fifty-three patients underwent surgery, 48 (73.8%) underwent single lung corrective surgery, 5 (7.6%) palliative surgery and 12 (18.4) received no surgery. Four operated patients died in the immediate postoperative period. The lowest Mc Goon ratio and Nakata index to undergo corrective surgery were 1.0 and 87.4 mm2/m2. A follow-up of 21 patients was done, among which 11 patients who underwent single-stage corrective surgery, all are in NYHA class II and saturating above 95%.Conclusions:Congenital UAPA is a rare anomaly and associated with a variety of CHDs, TOF being the most common. Single lung corrective surgery in patients with ipsilateral non-unifocalizable MAPCAs has good immediate and long term survival.

Surgical treatment of isolated unilateral absence of pulmonary artery with pulmonary hypertension.

A one-and-a-half-month-old patient with isolated unilateral absence of the right proximal pulmonary artery with prominent left pulmonary hypertension was diagnosed using CT. Medication therapy was initiated first. Left pulmonary artery pressure decreased after the initiation of medication therapy, and single-stage reconstruction of the right pulmonary artery was performed. The patient is asymptomatic 14 months postoperatively.

Unilateral absence of pulmonary artery analysis based on echocardiographic feature.

The unilateral absence of pulmonary artery (UAPA) is a rare congenital cardiovascular malformation, which is asymptomatic and easy to be ignored in early stage. A large number of complications may occur in the later stage. Therefore, early diagnosis and treatment is of great significance. The imaging data of 49 patients with UAPA discovered and confirmed clinically by the echocardiography in our hospital are analyzed. The results show that left pulmonary artery absence is more common (55%) and most of them are associated with other cardiovascular malformations (92%). Atrial septal defect and patent foramen ovale were most common in 56%. In which the absence of isolated pulmonary artery was 8% (4/49), and the absence of right pulmonary artery was 75% (3/4). Especially in the patients with tetralogy of Fallot, 77% (5/9) of them miss the diagnosis of UAPA. This suggests that doctors and sonographers should pay attention to the development of pulmonary artery bifurcation and left and right branches in multi-section, and strengthen the scanning of short axis section of high large artery.

Segmental Pulmonary Hypertension in Children with Congenital Heart Disease.

Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies.