Lung Hypoplasia Research Articles

A rare case of Sprengel deformity associated with spondylocostal dysostosis in a four-year child

Sprengel deformity is a complex congenital anomaly affecting one or both scapulae with mispositioning and dysplasia of scapula. It occurs due to failure of descent of scapula during intrauterine development and is the commonest congenital skeletal deformity of the scapula, often associated with other skeletal deformities. spondylocostal dysostosis is a rare genetic disorder which is characterized by malformation of the bones of the spine and ribs. It occurs in approximately 1 in 2,00,000 people worldwide. In spondylocostal dysostosis, affected children have wedge shaped vertebrae along with ribs that are fused, forked or sometimes missing. Based on the severity of the defect’s children may present with short stature, scoliosis or hypoplasia of lungs causing difficulty in breathing and recurrent respiratory tract infections. Management of both these skeletal deformities depends on their severity and the presence of associated deformities. Surgical intervention might be required for cosmetic and functional recovery of the shoulder. Authors had a rare and interesting case of a 4-year-old boy who had features of Sprengel deformity affecting left scapula which was associated with features of spondylocostal dysostosis.

MIF inhibition enhances pulmonary angiogenesis and lung development in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a complex birth anomaly with significant mortality and morbidity. Lung hypoplasia and persistent pulmonary hypertension (PPHN) limit survival in CDH. Macrophage migration inhibitory factor (MIF), a key regulator of innate immunity, is involved in hypoxia-induced vascular remodeling and PPHN. We hypothesized that antenatal inhibition of MIF in CDH fetuses, would reduce vascular remodeling, and improve angiogenesis and lung development. Pregnant rats were randomized into three groups: Control, nitrofen, and nitrofen + ISO-92. Lung volumes of pups were measured by CT scanning. Right ventricular systolic pressure (RVSP) and vascular wall thickness (VWT) were measured together with MIF concentration, angiogenesis markers, lung morphometry, and histology. Prenatal treatment with ISO-92, an MIF inhibitor, improved normalization of static lung volume, lung volume-to-body weight ratio, decreased alveolar septal thickness, RVSP and VWT and improved radial alveolar count as compared to the non-treated group. Expression of MIF was unaffected by ISO-92; however, ISO-92 increased p-eNOS and VEGF activities and reduced arginase 1, 2 and Sflt-1. Prenatal inhibition of MIF activity in CDH rat model improves angiogenesis and lung development. This selective intervention may be a future therapeutic strategy to reduce the morbidity and mortality of this devastating condition.

Neonatal cardiopulmonary transition in an ovine model of congenital diaphragmatic hernia

ObjectiveInfants with a congenital diaphragmatic hernia (CDH) are at high risk of developing pulmonary hypertension after birth, but little is known of their physiological transition at birth. We aimed to...

Evaluation and Monitoring of Pulmonary Hypertension in Neonates With Congenital Diaphragmatic Hernia.

This review aims to describe the assessment of pulmonary hypertension and ventricular function in neonates with congenital diaphragmatic hernia and the long-term follow-up of their pulmonary vascular disease. In 2015, the pediatric pulmonary hypertension guidelines from the American Heart Association and American Thoracic Society suggested class I level of evidence B guidelines for routine evaluation of patients with congenital diaphragmatic hernia, including longitudinal care in an interdisciplinary pulmonary hypertension program and following the recommendations offered for all children with pulmonary hypertension. Congenital diaphragmatic hernia causes compression of the lungs during critical stages of fetal development and results in lung hypoplasia. As a result, there is abnormal development of pulmonary vasculature that leads to post-natal pulmonary hypertension and increased afterload to the right ventricle. Left ventricular filling is affected by decreased pre-load and mechanical compression by abdominal content leading to decreased systemic perfusion. Persistent pulmonary hypertension after surgical repair of congenital diaphragmatic hernia is associated with increased mortality. Assessment and monitoring of pulmonary hypertension and ventricular function in this population of neonates is crucial to determine response to medical treatment, the need for extracorporeal membrane oxygenation, and the timing of surgical repair.

Complex Compound Inheritance of Lethal Lung Developmental Disorders Due to Disruption of the TBX-FGF Pathway

Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving TBX4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to TBX4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving TBX4 or FGF10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung.

Application of a postnatal prediction model of survival in CDH in the era of fetal therapy

Background: The disease severity in patients with a congenital diaphragmatic hernia (CDH) is highly variable. To compare patient outcomes, set up clinical trials and come to severity-based treatment guidelines, a performant prediction tool early in neonatal life is needed.Objective: The primary purpose of this study was to validate the CDH study group (SG) prediction model for survival in neonates with CDH, including patients who had fetal therapy. Secondary, we aimed to assess its predictive value for early morbidity.Methods: This is a retrospective single-center study at the University Hospitals Leuven on all infants with a diagnosis of CDH live-born between April 2002 and December 2016. The prediction model of the CDHSG was applied to evaluate its performance in determining mortality risk. Besides, we examined its predictive value for early morbidity parameters, including duration of ventilation, respiratory support on day 30, time to full enteral feeding and length of hospital stay.Results: The CDHSG prediction model predicted survival well, with an area under the curve of 0.796 (CI: 0.720–0.871). It had poor value in predicting infants who needed respiratory support on day 30 (area under the curve (AUC) 0.606; CI: 0.493–0.719), and correlated poorly with duration of ventilation, time to full enteral feeding and length of hospital stay.Conclusion: The CDHSG prediction model was in our hands also a useful tool in predicting mortality in neonates with CDH in the fetal treatment era. Correlation with early morbidity was poor.RationaleObjectives: (1) Validation of the CDHSG prediction model for survival in a cohort of neonates with CDH, in whom fetal endoscopic tracheal occlusion was applied according to the severity of lung hypoplasia. (2) Evaluation of performance of the model in the prediction of early morbidity.Main results: (1) Confirmation of the predictive value of the model for survival in neonates with CDH in the era of fetal therapy. (2) No correlation of the model with early morbidity parameters.

Scimitar Syndrome and Coronary Artery Disease–an Unusual Blend of Congenital and Acquired Cardiac Disease: A Case Report

Introduction: Scimitar syndrome and co-existent coronary artery disease is an unusual blend of congenital and acquired cardiac disease. Scimitar syndrome is characterised by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. Co-existing abnormalities include atrial septal defects and lung hypoplasia. Due to the rarity of Scimitar syndrome, much less the combination of both pathologies, there are no established guidelines. This study reported a case of a 71-year-old man who underwent coronary revascularisation and conservative treatment of Scimitar syndrome.

Congenital Pulmonary Adenomatoid Malformation: Unusual Revelation

Congenital pulmonary adenomatoid malformation (CPAM) is a congenital lung malformation that is distinguished by abnormal airway patterning during branching morphogenesis. It may lead to significant morbidity and mortality in infants due to complications such as pulmonary infections, lung hypoplasia, respiratory distress, and fetal hydrops. The diagnosis is usually prenatal thanks to morphological ultrasound; in postnatal, the anomaly may remain asymptomatic or show respiratory signs or complications. The postnatal diagnosis is based on computed tomography. In this article, we report a case of congenital pulmonary adenomatoid malformation in a 7-month-old patient who presented respiratory distress that is due to voluminous infected pleural effusion. The diagnosis of CPAM was suspected at the initial CT and confirmed at the control CT that was realized after treatment of the infection. The therapeutic management of CPAM is depending on the severity of complications and its evolution.

Surgical technique for developing a rabbit model of congenital diaphragmatic hernia and tracheal occlusion

The surgical model of congenital diaphragmatic hernia (CDH) has been utilized in exploring treatments and innovative therapies, such as tracheal occlusion (TO). The rabbit is an excellent surgical model compared to others due to lower cost, ease of care, short gestational period, and large litter size. This model is also ideal in studying lung hypoplasia of CDH because rabbit lung development is most similar to humans as alveolarization begins prior to birth and continues post-natally. However, the surgical technique in creating a rabbit model of CDH is quite difficult and information is lacking on how to establish this model. Therefore, the aim of this paper is to describe:•Surgical technique in establishing a rabbit model of CDH and TO•Perioperative care for pregnant rabbit does

Ultrasound Guided Supraclavicular Block in Poland Syndrome : A Safe Alternative to General Anaesthesia

Poland syndrome is an uncommon, congenital anomaly characterized by multiple systems and organs involvement like musculoskeletal, lungs, heart, kidneys and haemopoetic system with absence of functional disability. Right sided Thorax deformity is the most common feature of this syndrome with the absence of underlying bones and muscles making this condition similar to open chest along with ipsilateral hand anomalies such as brachydactyly, syndactyly or ectodactyly [1]. The incidence of this syndrome is 1 per 30,000-50,000 live births [2]. Patients with Poland syndrome require anesthesia during surgical procedures to correct the associated deformities or the organ systems associated with the disease process. Literature shows that these patients may be more susceptible for malignant hyperthermia due to associated musculoskeletal abnormalities under general anesthesia [3]. Moreover respiratory problems and hypoxia in the perioperative period may be related to associated paradoxical breathing, lung hypoplasia and chest wall abnormalities in otherwise asymptomatic patient at rest. We report a diagnosed case of Poland syndrome presented for syndactly release Keywords: USG blocks, Supraclavicular approach.

An Asian multicenter retrospective study on persistent pulmonary hypertension of the newborn: incidence, etiology, diagnosis, treatment and outcome

Objectives: To explore the incidence, etiologies, diagnostic methods, treatment options and outcomes in neonates with persistent pulmonary hypertension of the newborn (PPHN) and to identify mortality risk factors in a study from six Asian countries.Methods: A retrospective chart review of patients with documented PPHN from seven centers in six Asian countries (Japan, Kuwait, India, Pakistan, Singapore, and Thailand) between 1 January, 2014 and 31 December, 2016, was performed.Results: A total of 369 PPHN infants were identified. The incidence of PPHN ranged from 1.2 to 4.6 per 1000 live births. The all-cause mortality rate was 20.6% (76 of 369). Meconium aspiration syndrome was the primary cause of PPHN (24.1%). In most cases (84.8%) echocardiography was used to establish the diagnosis of PPHN. Sildenafil was the most commonly used pulmonary vasodilator (51.2%). Multivariate multiple regression analysis indicated gestational age <34 weeks (adjusted odds ratio (OR) = 3.27; 95% CI 1.56–6.74), congenital diaphragmatic hernia (CDH)/lung hypoplasia (LH) (adjusted OR = 6.13 (95% CI 2.28–16.42)), treatment with high frequency oscillation ventilation (HFOV) with or without inhaled nitric oxide (iNO) (adjusted OR = 3.11 (95% CI 1.52–6.34)), and inotropic agents (adjusted OR = 9.43 (95% CI 2.71–32.83)) were independently associated with increased risk of death.Conclusions: The incidence of PPHN in the current study was higher than in western settings. Birth weight, gestational age, CDH/LH, HFOV/iNO, and inotropic agents were significant mortality risk factors.

A new ovine model of spine and chest wall deformity at birth with alteration of respiratory system mechanics and lung development: a feasibility study.

To develop an animal model of spine and chest wall deformity (CWD) at birth and to evaluate its effects on respiratory system mechanics and lung development. A spine and CWD was created in utero between 70 and 75days of gestation in six ovine fetuses by resection of the seventh and eighth left ribs. Two days after birth, respiratory system mechanics was assessed in anesthetized lambs using the flexiVent apparatus, followed by postmortem measurement of lung mechanics as well as histological lung analysis. A range of severity of CWD was found (Cobb angle from 0° to 48°) with a mean decrease in compliance of 47% and in inspiratory capacity of 39% compared to control lambs. Proof-of-concept histological analysis in one lamb showed marked lung hypoplasia. Our ovine model represents a pilot proof-of-concept study evaluating the impact of a spine and CWD present at birth on lung respiratory mechanics and development. This study lays down the groundwork for future studies evaluating the impact of these deformities on lung development and potential treatments. These slides can be retrieved under Electronic Supplementary Material.

Fetal pulmonary artery acceleration/ejection time prognostic accuracy for respiratory complications in preterm prelabor rupture of membranes

Objective: To determine the prognostic accuracy of the fetal pulmonary artery acceleration time/ejection time (PATET) for the prediction of neonatal respiratory complications (NRCs) in pregnancies with preterm premature rupture of membranes (PPROM).Methods: This is a prospective cohort of singleton pregnancies complicated by PPROM managed in our institution from October 2015 to April 2018. Inclusion criteria included mothers from 13 to 46 years of age and singleton pregnancies with PATET measurements <7 days prior to delivery. PATET was obtained by placing the Doppler caliper in the main pulmonary artery proximal to the bifurcation of this vessel. NRC was defined as: need for ventilatory support, respiratory distress syndrome (RDS), or lung hypoplasia. Logistic regression models and area under the receiver operating characteristic curves (ROC) were utilized to determine the prognostic accuracy of PATET and gestational age for NRC and RDS.Results: Of 95 patients included, 46 had NRC (RDS = 33). PATET was a significant predictor of NRC (AUC 0.74; 95%CI: 0.61–0.83; p < .001) and RDS (AUC 0.69; 95%CI: 0.57–0.80; p = .021) in PPROM. Gestational age at delivery and gestational age at PPROM were also significantly associated with NRC and RDS. Their predictive accuracy for NRC was 0.87 and 0.84, and for RDS 0.85 and 0.86, respectively.Conclusions: PATET is a statistically significant predictor for NRC in pregnancies with PPROM; however, its clinical use may be limited as gestational age is a better predictor of these outcomes.Rationale: NRCs are common in pregnancies complicated by preterm prelabor rupture of membranes (PPROM). We aim to determine the prognostic accuracy of the fetal PATET for the prediction of neonatal NRC in these pregnancies. Our results indicate that PATET is a statistically significant predictor for NRC in pregnancies with PPROM; however, its clinical use may be limited, as gestational age is a better predictor of these outcomes.

Unilateral lung hypoplasia presenting in seventh decade of life: a rare case report

Pulmonary hypoplasia is a bronchopulmonary foregut anomaly in which gross morphology of the lung is preserved but there is decrease in the number or size of airways, vessels, and alveoli. Unilateral pulmonary hypoplasia is a rare clinical condition and most patients reported in the literature are newborns and infants, but patients may remain asymptomatic until late adolescent or till adulthood 65 years old male with parkinsonism was referred to our department with complaints of recent chest symptoms. Clinical examination revealed loss of lung volume in left hemithorax. CT scan thorax and bronchoscopy confirmed the diagnosis of left sided pulmonary hypoplasia. This case report highlights the possibility of pulmonary hypoplasia as one of the differential diagnoses in adult patients presenting with loss of lung volume and to the best of our knowledge he is the eldest patient so far reported.

Tailored Management of Airway Abnormalities in Children with Congenital Single Functional Lung

ObjectivesPediatric patients born with single functional lung can be associated with symptomatic airway abnormalities. Management of such patients is not only technically demanding but is also ethically challenging. This study reports our experience of managing symptomatic airway abnormalities in pediatric patients with congenital single functional lung. MethodsObservational retrospective cohort study performed at a tertiary children’s hospital from January 2001 to September 2017. All children (0 to 18 years old) with congenital single functional lung (agenesis and hypoplasia) presenting with symptomatic airway abnormalities (long segment congenital tracheal stenosis and tracheomalacia) and requiring surgical interventions were included in the study. Children with single functional lung secondary to non-congenital causes were excluded. ResultsA total of 16 patients with single functional lung (agenesis=8 and hypoplasia=8) and airway abnormalities (long segment congenital tracheal stenosis=12 and tracheomalacia =4) were eligible for the study. Lung abnormalities were common on the right side (n = 10, 62.5%). Associated abnormalities (cardiac and non-cardiac) were seen in 11 patients (68.8%). Surgical interventions for airway abnormalities, alone or in combination, included slide tracheoplasty (n=12), aortopexy with or without pericardiopexy (n=7), excision of rudimentary lung (n=4) and placement of intrathoracic tissue expanders to reposition the mediastinum (n=3). Nine patients (56.3%) underwent a one-stage repair while staged repairs (airway and cardiac) were performed in 7 (43.7%). Fourteen patients (87.5%) survived to hospital discharge. Of the survivors, 9 (64.2%) had stable airways not requiring respiratory support at home. ConclusionManagement of pediatric patients with airway abnormalities in the setting of congenital single functional lung is feasible with acceptable surgical outcomes. This is facilitated by staged repairs and repositioning of mediastinum before a definitive airway repair in patients with significant comorbidities. Treatment should not be deferred to these patients if there are no obvious contraindications. Type of StudyRetrospective Case Control Study Level of EvidenceLevel III.

LETHAL CASE OF PARROT ACHONDROPLASIA IN A NEWBORN BOY

Achondroplasia is an inherited monogenic disease from the group of osteochondroplasia with defects of tubular bones and axial skeleton characterized by autosomal-dominant type of heredity with complete penetrance. Occurrence of the disease in the world is 0,4:10000, and in European population – 2:10000 of neonates. In 99% cases achondroplasia is caused by mutations of G380R gene FGFR3, located on a short arm of the fourth chromosome (locus 4p16.3). Missense mutations in FGFR3 gene are the most frequent associated with changes of glycine into arginine in the protein coding the receptor of fibroblast growth factor responsible for proliferation of cartilages in joints of the long bones. The article presents the original case of observation of a child with achondroplasia with lethal outcome. The boy was born preterm on the 35th week of pregnancy with 2460 g of body weight, and body length was 36 cm. Physical examination determined the phenotypic signs of Parrot achondroplasia. The boy’s condition at birth and during his life was assessed as severe due to manifestation of multiple organ failure syndrome against the ground of lung hypoplasia and hydrocephaly with gradual decompensation of vital functions resulting in lethal outcome at the age of 1 month and 24 days. Prenatal screening determined maternal decreased levels of β-chorial human gonadotropin on the 10th week of gestation and α-fetoprotein – on the 17th week of gestation; ultrasound examination diagnosed multiple developmental defects of the muscular-skeletal system. The woman refused from invasive examination and interruption of pregnancy at all the stages of examination and consulting. Medical-genetic examination of the child was not performed due to considerable severity of disorders of his general condition. The necessity to give neonatal palliative care to neonates with critical congenital developmental defects has been discussed.

General Anesthetic Management of a Child With Horseshoe Lung and Left Lung Hypoplasia for Cheiloplasty: A Case Report

Horseshoe lung is an extremely rare congenital malformation in which the right and left lungs are fused due to stenosis of the lung parenchyma. In anesthetic management, it is important to avoid hypoxemia and hypercapnia caused by a decline in lung capacity and functional residual capacity. A 3-year-old boy with horseshoe lung and left lung hypoplasia was scheduled to undergo cheiloplasty. Regarding respiratory management, to prevent hypoxemia and hypercapnia, we avoided intraoperative peripheral airway obstruction with positive end-expiratory pressure, set a long inspiratory phase time for sufficient alveolar expansion, and maintained sufficient gas exchange in lungs with low reserve capacity.

The receptor for hyaluronan-mediated motility (RHAMM) expression in neonatal bronchiolar epithelium correlates negatively with lung air content

IntroductionHyaluronan (HA) and the receptor for hyaluronan-mediated motility (RHAMM) may play an important role in lung development. We examined the expression of HA content and RHAMM during postnatal lung development by analyzing human lung specimens from newborn infants with a variety of lung diseases at different gestational (GA) and postnatal (PNA) ages. Materials and methodsNinety-four patients were evaluated. Immunohistochemical RHAMM expression was studied with digital image analysis, followed by hierarchical cluster analysis of both these data and clinical data to define subgroups. The air content of the lung was determined by computerized analysis. HA content was estimated by radiometric assay. ResultsCluster analysis defined six distinct patient groups (Group 1-2: 34-41 weeks GA; Group 3-5: 23-27 weeks GA; Group 6: mixed population). Group 1-5 showed individual patterns in RHAMM expression and HA content (Group 1: high RHAMM/low HA; Group 2: low RHAMM/low HA; Group 3: low RHAMM/low HA; Group 4: low RHAMM/high HA; Group 5: high RHAMM/high HA). HA content decreased with increasing PNA independently of GA. Negative correlation was observed between air content and RHAMM expression in the bronchiolar epithelium irrespective of clustered groups. Lung hypoplasia appeared in two distinctive groups, with significant differences in lung development and RHAMM expression. ConclusionsRHAMM expression may show dynamic changes during pathological processes in the neonatal lung. The distribution of RHAMM in the lung tissue is heterogeneous with a predominance to the bronchiolar epithelium. We found a negative correlation between lung air content and RHAMM expression in bronchiolar epithelium.

Fetal brain morphometry on prenatal magnetic resonance imaging in congenital diaphragmatic hernia

Many infants with congenital diaphragmatic hernia (CDH) show brain abnormality on postnatal brain MRI related to severity of CDH, degree of lung hypoplasia, intrathoracic liver, right diaphragmatic hernia and large diaphragmatic defect. It is not known whether these factors affect brain growth in utero in CDH. To assess prenatal brain morphometry and abnormalities on fetal MR in congenital diaphragmatic hernia. We retrospectively reviewed 109 fetal MRIs in 63 fetuses with CDH from 2009 to 2014 (27 died before discharge, 36 survived to discharge). We compared brain injury and gestational-age-corrected z-scores of brain measurements between survivors and non-survivors. We assessed correlations between brain abnormalities and CDH severity. Enlarged extraaxial space was the most common abnormality, frequently seen on fetal MRI at >28weeks of gestation, similar in survivors versus non-survivors. Anteroposterior cerebellar vermis dimension at >28weeks of gestation was smaller in non-survivors compared to survivors (P=.02) and positively correlated with observed/expected total fetal lung volume (P=.01). Transverse cerebellar diameter at >28weeks of gestation was also positively correlated with observed/expected total fetal lung volume (P=.04). We did not identify maturational delay, abnormal parenchymal signal or intracranial hemorrhage on fetal MRI. Enlarged extraaxial spaces in the third trimester was the most common abnormality on fetal MRI in congenital diaphragmatic hernia. Cerebellar dimensions on fetal MRI are associated with CDH severity. There was no major brain parenchymal injury on fetal MRI, even in the third trimester, in CDH survivors and non-survivors.

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