Absence Of Left Pulmonary Artery Research Articles

An Adult with Haemoptysis: A Rare Case of Congenital Anomaly

A non-smoker adult male presented with haemoptysis of short duration. Chest radiograph (postero-anterior view) suggested an opaque left hemithorax. Further evaluation of lung lesion pointed towards a left lung hypoplasia with absent left pulmonary artery and a right-sided aortic arch (RAA). Both kidneys were enlarged with multiple cysts and thinning of parenchyma. This case describes a unique coexistence RAA and probable autosomal dominant polycystic kidney disease.

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

Congenital cystic adenomatoid malformation associated with right aortic arch with mirror image branching and absence of left pulmonary artery in an adult

BackgroundCongenital cystic adenomatoid malformation is an uncommon developmental anomaly that is diagnosed prenatally or during early childhood and is rare to present in adulthood. Type 2 congenital cystic adenomatoid malformation is often associated with other anomalies such as congenital cardiac anomalies. We present a case of type 2 congenital cystic adenomatoid malformation in an adult male associated with absence of left pulmonary artery and right aortic arch with mirror image branching. To our knowledge, this is the first case report with the combination of all three anomalies.Case presentationA 33-year-old male presented to the Department of Pulmonary Medicine with high-grade fever, chills and rigor, breathlessness, and productive cough for a duration of one week. He had multiple similar self-limiting episodes in the past which did not require hospitalization. The plain radiograph showed right aortic arch and a small left hilum. CT thorax showed multiple cystic lesions of size 0.5–2.0 cm in the apico-posterior segment of the left upper lobe; anterior, posterior, and lateral segments of the left lower lobe; and the apical segment of the right upper lobe with a maximal cyst wall thickness of 2 mm. Volume of left lung was reduced with trans-thoracic herniation of the medial segment of the right middle lobe; bronchiectatic changes were seen in the apical, anterior, and posterior segments of the right upper lobe; and fibrotic strands were seen in the apical segment of the right lower lobe and the apico-posterior segment of the right upper lobe. Mediastinal window showed right-sided aortic arch with mirror image branching. The pulmonary trunk was seen to continue as the right pulmonary artery with absent left pulmonary artery.ConclusionsThough congenital cystic adenomatoid malformation is a disease of infancy and childhood, it should also be considered in adults presenting with recurrent lower respiratory tract infections and/or pneumothorax. Radiologists must be aware of the types of congenital cystic adenomatous malformation (CCAM) and their associations, as type 2 CCAM has a high association with congenital cardiac disease. Proximal interruption of pulmonary artery should be considered if the pulmonary trunk continues as either right or left 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.

Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus

Objective The aim of this study was to describe the prenatal diagnosis of Major Aortopulmonary Collateral Arteries (MAPCAs), and to present a systematic ultrasound method for evaluating lung vascularity in fetuses with pulmonary atresia with ventricular septal defect (PAVSD) and agenesis of ductus arteriosus (DA). Method This retrospective study evaluated fetuses diagnosed with PAVSD with agenesis of DA, for the presence of the MAPCAs anomaly. Fetal pulmonary vasculature was investigated by 2D and 4D Spatio Temporal Image Correlation (STIC) technology using High Definition Color Doppler. Results Over a 10 year period, six fetuses were diagnosed with MAPCAs. Prenatal diagnosis was made between 17 w 6 d and 28 w 4 d in five fetuses, with the sixth diagnosed at 37 w 6 d. All six had PAVSD with agenesis DA, four exhibited pulmonic atresia without any arterial outflow, while two fetuses presented with absent left pulmonary artery, and a miniscule right pulmonary artery. In five cases, the parents elected to terminate the pregnancy and the last, although born alive, did not survive an attempt at restorative surgery and died at the age of 5 months. Postnatal CT angiography imaging of this case revealed the subclavian origin of the MAPCAs. Chromosomal micro array analysis of the amniotic fluid revealed that five of the six fetuses were normal and one was lost to follow up. Conclusion MAPCAs should be investigated in cases of PAVSD with agenesis DA. A meticulous ultrasound evaluation using 2D and 4D STIC can permit the prenatal diagnosis of this anomaly and provide the parents with the opportunity for prenatal consultation.

Phenotypic expansion of Zimmermann- Laband syndrome associated with cardiac and hearing loss: a case report

Background: ATP6V1B2 gene mutation is associated with Zimmermann-Laband syndrome 2 (ZLS2), which is a rare developmental disorder characterized by nail hypoplasia and hereditary deafness. Case Presentation: We report a new phenotypic mutation of ATP6V1B2 associated with ZLS 2. The patient has atresia of the left pulmonary artery (LPA) and features of hearing loss and nail hypoplasia. The other interesting part is that the child had two types of mutations inherited from father and mother. He is carrier for GJB2 mutation (inherited from father) and diseased with ATP6V1B2 mutation (inherited from mother). Conclusion: The association of ZLS features with absent LPA was not reported previously in the literature. This finding will add new information to the database of previously reported ATP6V1B2 rare mutations.

Tetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation

Absent left pulmonary artery with Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS), is a rare congenital cardiac anomaly. Here we present such a case of A 2 year 11 month old girl with cyanosis, exertional dyspnoea. Her diagnosis is confirmed by echocardiography and CT angiogram. There are very few cases have been reported till date with high postoperative mortality. Although per operative decision making was challenging regarding pulmonary valve and size of the RPA, we performed ICR with RPA reductionplasty and creation of monocuspid pulmonary valve with success. As it is a rare association and we have overcome the hindrance we came across per operatively, we are reporting this case.
 Bangladesh Heart Journal 2020; 35(1) : 74-77

Unilateral absence of pulmonary artery: A rare cause of dyspnea in a 77-year-old woman

Isolated unilateral absence of pulmonary artery is quite rare and usually associated with congenital cardiac defects. It is usually diagnosed in childhood with recurrent respiratory tract infections and signs of pulmonary hypertension. It rarely remains asymptomatic until adulthood and may present with dyspnea, hemoptysis, and recurrent respiratory infections later, while it can rarely be an incidental finding of other imaging. Surgical management in early days of life will prevent pulmonary hypertension while in adults it is aimed to treat the complications. Here, we report the case of a 77-year-old female, a mother of five children. She came with worsening of dyspnea for a week, and her computed tomography pulmonary angiography showed absence of left pulmonary artery accompanying hypoplastic lung. Ventilation perfusion imaging showed absence of perfusion of the left lung. She had severe pulmonary hypertension and was managed with long-term oxygen therapy, diuretics, and vasodilators.

Prenatal diagnosis of anomalous origin of one pulmonary artery branch from the aorta and unilateral absence of pulmonary artery by echocardiography

Objective To improve the prenatal diagnosis and differential diagnosis ability for anomalous origin of one pulmonary artery branch from the aorta (AOPA) and unilateral absence of pulmonary artery (UAPA) by comparing and analyzing different types of fetal echocardiography features. Methods Fetal echocardiographic features were analyzed retrospectively in fetus with prenatal diagnosis of UAPA or AOPA between June 2012 and December 2018, and the different types of fetal echocardiography characteristics and main points of identification were summarized. Results A total of 6 fetuses with AOPA and 6 fetuses with UAPA were diagnosed.Among the 6 fetuses with AOPA, 5 cases were anomalous origin of right pulmonary artery from the aorta (AORPA), including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome, and 1 case was anomalous origin of left pulmonary artery from the aorta (AOLPA) with an absent pulmonary valve. All 6 cases of AOPA had proximal form. Among the 6 fetuses with UAPA, 4 cases were isolated unilateral absence of right pulmonary artery, 1 case was isolated unilateral absence of left pulmonary artery and 1 case was unilateral absence of left pulmonary artery combined with tetralogy of Fallot (TOF). The main common characteristic sonographical findings included: there was no confluence at the bifurcation of the main pulmonary artery on multiple views, and the affected pulmonary branch was not attached to the main pulmonary artery. The different characteristic sonographical findings in fetus included: the affected pulmonary artery of AOPA arosed from the right posterior or left posterior portion of the ascending aorta on three vessels and trachea view (3VT) and long axial view of left ventricle, and 3VT and the coronary view of brachiocephalic trunk artery showed that the abnormal origin of pulmonary artery branch of UAPA connected with the root of brachiocephalic trunk artery by the ipsilateral vertical arterial duct (DA), and color Doppler flow imaging showed that the blood flow of the affected pulmonary artery branch was isolated in the pulmonary field. Conclusions AOPA and UAPA have many similarities in the characteristics of fetal echocardiography. 3VT and long axial view of left ventricle are the best views for displaying proximal AOPA, 3VT and the coronary views of the root of brachiocephalic trunk artery are the best views for displaying UAPA. Key words: Echocardiography; Fetus; Unilateral absence of pulmonary artery; Anomalous origin of one pulmonary artery branch from the aorta

AN UNUSUAL CAUSE OF PULMONARY HYPERTENSION

SESSION TITLE: Pulmonary SESSION TYPE: Global Case Reports PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM INTRODUCTION: Unilateral absence of left pulmonary artery (UAPA)is a rare congenital abnormality with variable presentation. Patients with isolated UAPA can remain symptomatic in to late adulthood.Unilateral absence of pulmonary artery is a rare congenital malformation that may develop in isolation or in association with congenital cardiovascular anomalies. 1,2Associated cardiovascular anomalies may include Tetralogy of Fallot (TOF), atrial septal defect, coarctation of the aorta, a right-sided aortic arch, truncus arteriosus, and pulmonary atresia .In reported cases of isolated UAPA incidence has been reported to range from 1 in 200,000 to 1 in 300,000. 3 Agenesis of the right pulmonary artery has a greater incidence than agenesis of the left .UAPA is believed to result from the involution of the proximal sixth aortic arch, thereby forgoing expected embryological fusion with the pulmonary trunk . Most common symptoms experienced include recurrent pulmonary infections, hemoptysis, chestpain and shortness of breath upon exertion .We present a case of 45 yrs old female referred for evaluation of unexplained exertion breathlessness found to have unilateral absent left pulmonary artery. CASE PRESENTATION: Middle age lady with moderate persistent asthma on optimised inhaler medications, referred for evaluation of unexplained exertion breathlessness.Patient had longstanding history of exercise intolerance relived by rest. X ray chest showed enlarged right pulmonary artery with prominent vascular markings. A Multi detector Computed Tomography (MDCT) pulmonary angiogram showed dilated main pulmonary artery with absent left pulmonary artery. Echocardiogram revealed severe Tricuspid regurgitation with severe pulmonary artery hypertension. Her pulmonary function test showed modern obstructive pattern. she was started on diuretics and vasodilators along with inhaler medications. DISCUSSION: Left-sided agenesis is a rare entity that occurs half as frequently as right-sided agenesis. Approximately 80% of left-sided agenesis is associated with a congenital cardiovascular malformation necessitating medical attention in early life .The other subset of isolated left pulmonary artery agenesis without associated cardiovascular anomaly, presenting later on in life.The treatment for UAPA is patient specific and will vary depending on age of presentation, associated cardiovascular anomalies, and symptomatology. The options in young patients include revascularization of the distal affected pulmonary artery.Lobectomy, or selective embolization of systemic arterial supply, may be considered for patients with hemoptysis . Asymptomatic adult patients with severe pulmonary hypertension may be started on vasodilators.Regular follow-up and observation of pulmonary hemodynamics is recommended CONCLUSIONS: Early diagnosis and treatment of pulmonary hypertension is required is a serious complication. Reference #1: 1. Aypak C, Yikilkan H, Uysal Z, Gorpelioglu S: Unilateral absence of the pulmonary arteryincidentally found in adulthood. Case Rep Med. 2012, 2012:1-3. Accessed: 2/1/2016:http://www.hindawi.com/journals/crim/2012/942074/. 10.1155/2012/942074. Reference #2: Ghanbari H, Feldman D, David S, Saba S: Unilateral absence of a left pulmonary artery:successful therapeutic response to a combination of bosentan and warfarin. CircaCardiovascImaging. 2009, 2:e46-e48. 10.1161/CIRCIMAGING.108.825745 Reference #3: Kruzliak P, Syamasundar RP, Novak M, Pechanova O, Kovacova G: Unilateral absence ofpulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. ArchCardiovasc Dis. 2013, 106:448-454. 10.1016/j.acvd.2013.05.004. DISCLOSURES: No relevant relationships by Nagarajan Appusamy, source=Web Response

A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right‑sided aortic arch

The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

Left pulmonary artery agenesis

Unilateral pulmonary artery agenesis is a rare malformation accounting for 1% of congenital heart defects. It can lead to several complications. We report the case of a 16-year-old female patient with no particular past medical history, presenting with recurrent low abundance hemoptysis within a context of preservation of patient’s general condition. Clinical examination showed no abnormalities and frontal (A) and lateral (B) chest x-ray revealed small left lung-field associated with decreased vascular pattern and mediastinal shift to the left. Given this radio-clinical presentation, diagnosis of suspected left pulmonary hypoplasia, sequelae of a previous pulmonary infection and endobronchial foreign body were made. Thoracic CT scan objectifies the absence of left pulmonary artery (C) and multiple systemic collateral circulation Left pulmonary parenchyma was hypoplastic, well ventilated and without bronchial abnormality (D). The descending aorta was located on the right. The electrocardiogram showed right bundle-branch block and transthoracic ultrasound did not reveal cardiac abnormality including sign of pulmonary arterial hypertension. Spirometry showed normal respiratory function as well as laboratory tests. Hemoptysis treatment was symptomatic and the patient was kept under medical monitoring.

Percutaneous Pulmonary Valve Implantation (PPVI) with Melody®: First Ever Case Report in South Asia

An eleven years old boy was diagnosed as a case of Tetralogy of Fallot (TOF) with absent left pulmonary artery (LPA) since two years of age. He had history of total corrective surgery in 2007 from India. He developed severe right pulmonary artery origin stenosis, severe tricuspid and pulmonary valve regurgitation after surgery and redo surgery was performed in 2009. During redo surgery an orthotropic conduit was placed between right ventricular (RV) out flow tract and pulmonary artery. He developed significant conduit dysfunction in the form of stenosis and regurgitation in 2011 and planned for Melody® trans-catheter pulmonary valve replacement. Finally in 25th December 2012, first ever case of pulmonary valve replacement in South Asia was performed in catheterization laboratory of CMH Dhaka with technical support from a Saudi cardiac team. Patient was discharged three days after the procedure with a fully functioning Melody® valve without any stenosis or regurgitation.J Bangladesh Coll Phys Surg 2014; 32: 102-106

Echocardiographic findings in children with Tetralogy of Fallot

Background and Aims: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in children. The aim of this study was to identify various cardiac defects associated with TOF by transthoracic echocardiography.Methods: All children presenting to Shahid Gangalal National Heart Centre, with the diagnosis of TOF from 2014 to 2015 were included in the study. Echocardiograpic reports of all eighty eight children were reviewed.Results: The mean age of presentation was 3.5 } 3.9 years. Fifty six (64%) children were male and thirty two (36%) were female. Pulmonary artery anomaly was found in nine (10.2%) children which included hypoplastic pulmonary arteries, absent left pulmonary artery, post stenotic and aneurysmal dilatation of pulmonary artery. Pulmonary valve anomalies were seen in 13(14.8%) children which include pulmonary atresia, pin-hole opening of valve and bicuspid and absent pulmonary valve. Double outlet right ventricle, patent ductus arteriosus/major aortico-pulmonary collaterals, atrial septal defect and persistent left superior venacava were present in 12 (13.6%), 21 (23.9%), 22 (22.7%) and nine (10.2%) respectively. Right sided aortic arch was seen in 20 (22.7%).Conclusion: TOF is associated with various anatomical variations which need to be identified to avoid surgical complications. These can be diagnosed by echocardiography which has greatly reduced the need for invasive cardiac catheterization for these patients.Nepalese Heart Journal 2015;12(2):65-68

Horseshoe Lung Associated With Holt-Oram Syndrome.

Horseshoe Lung Associated With Holt-Oram Syndrome.

Multidetector Computed Tomographic Angiography Imaging of Pentalogy of Cantrell

A 4-day-old female mildly cyanotic neonate presented clinically with ventral herniation of the heart to the anterior abdominal wall and umbilical defect containing bowel loop (Figure [A] and Movie I in the online-only Data Supplement), raising suspicion of pentalogy of Cantrell. Echocardiogram of the ectopic heart, although technically difficult, revealed overlap of the ascending aorta with small-caliber partially visualized main pulmonary artery arising from the ventricle (Figure [B]). Multidetector computed tomographic angiography (MDCTA) confirmed the diagnosis of pentalogy of Cantrell, which consisted of ectopia cordis with rightward apex containing ventrally herniated single ventricle with right- sided atrioventricular valve atresia protruding through the defective anterior diaphragm anterior to the liver, omphalocele, lower sternal and ventral thoracoabdominal wall defects, and multiple cardiac anomalies (Figure [C through E] and Movie II in the online-only Data Supplement). Cardiovascular anomalies included single ventricle with right-sided atrioventricular valve atresia giving rise to tetralogy of Fallot–type conotruncus with diffusely narrowed main pulmonary artery. Additional anomalies included large secundum atrial septal defect, absent left pulmonary artery originating from the undersurface of the aortic arch …

Absence of Left Pulmonary Artery after Delivery, a Case Report and Review of Literature

Her physical exam was significant for tachypnea with a respiratory rate 30 breaths/minute, pulse 120 beats per minute, blood pressure 150/90 mmHg, temperature 100.6 F. Lungs revealed bilateral bronchial breath sounds. Her cardiovascular examination was significant for tachycardia with normal s1 and s2 and no murmurs or gallops noted. Abdominal exam was benign. Her extremities showed normal pulses with no clubbing or cyanosis.

Toraks vasküler anomalileri ve Bilgisayarlı tomografi

lusoria caused by aberrant right subclavian artery, Kommerell's diverticulum, legamentum ring, right descending aorta, and absent left pulmonary artery: a report of a unique vascular congenital disease undetected until adulthood and a review of the literature. Pediatr Cardiol 2005; 26: 851-5

Double outlet right ventricle with unilateral absence of left pulmonary artery

Congenital absence of unilateral pulmonary artery, either as a primary defect or in combination with other congenital cardiovascular malformations, is very rare. Double outlet right ventricle pathology in combination with unilateral absence of pulmonary artery has only been reported once in the literature. In this report, we present our experience with double outlet right ventricle with unilateral absence of left pulmonary artery in a 3-year-old female patient who underwent a palliative procedure and was scheduled for correction.

Isolated Left Pulmonary Artery Agenesis: A Case Report

Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation-perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. Conflict of interest:None declared.