Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases
Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA) is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of ex...
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| Veröffentlicht in: | Journal of postgraduate medicine 2017-10, Vol.63 (4), p.262-264 |
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| creator | Govindaraj, V Joseph, J Kumar, B Nagamalli Soman, R |
| description | Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA) is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan. |
| doi_str_mv | 10.4103/jpgm.JPGM_562_16 |
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Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.</description><identifier>ISSN: 0022-3859</identifier><identifier>EISSN: 0972-2823</identifier><identifier>DOI: 10.4103/jpgm.JPGM_562_16</identifier><identifier>PMID: 28862244</identifier><language>eng</language><publisher>India: Medknow Publications and Media Pvt. 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Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.</description><subject>Abnormalities, Multiple - diagnostic imaging</subject><subject>Adult</subject><subject>Adults</subject><subject>Aged</subject><subject>Agenesis</subject><subject>Brief Report</subject><subject>Bronchiectasis - diagnostic imaging</subject><subject>Bronchiectasis - etiology</subject><subject>Bronchoscopy</subject><subject>Care and treatment</subject><subject>Case studies</subject><subject>Diagnosis</subject><subject>Dyspnea - etiology</subject><subject>Female</subject><subject>Health aspects</subject><subject>Health education</subject><subject>Humans</subject><subject>Infections</subject><subject>Lung</subject><subject>Lung - abnormalities</subject><subject>Lung - diagnostic imaging</subject><subject>Lung Diseases - diagnostic imaging</subject><subject>Magnetic Resonance Imaging</subject><subject>Medical imaging</subject><subject>Medicine</subject><subject>Patients</subject><subject>Pulmonary arteries</subject><subject>Pulmonary artery</subject><subject>Pulmonary Artery - abnormalities</subject><subject>Pulmonary Artery - diagnostic imaging</subject><subject>Pulmonary Artery - pathology</subject><subject>Pulmonary hypertension</subject><subject>Respiratory Tract Infections - etiology</subject><subject>Tomography, X-Ray Computed</subject><subject>Veins & arteries</subject><issn>0022-3859</issn><issn>0972-2823</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNptUk1r3DAQFaWhSdPeeyqCnr3Rl2Wph8IS2nyQ0B6aa4RWkr1abMuV5IT8-2rzHVh0mGH05s3M4wHwBaMFw4gebaZuWJz_OblUNScK83fgAMmGVEQQ-r7kiJCKilrug48pbRDCnDP6AewTITghjB2A67MUep2dhb1rc5W8Lek090MYdbyDOma3DZ0bXfIJ3vq8vkfCfh47uL6bwtTr5PV3uITRTSFmGFqYbwM0Orn0Cey1uk_u82M8BFe_fv49Pq0ufp-cHS8vKsMEyRXmAmFZU41QI_nKckr1qkGsJStNsdGWCaYNcxJZUopSWsZl00hLTMtE4-gh-PHAO82rwVnjxhx1r6boh3KGCtqrtz-jX6su3BTdeCEgheDbI0EM_2aXstqEOY5lZ1UWK-OlYPULqtO9U35sQyEzg09GLWuMSdGVsIKqdqC2EpbJYXStL-U3-MUOfHnWDd7sbEAPDSaGlKJrnw_FSG2NobbGUK-MUVq-vhboueHJCfQ_mDu1Aw</recordid><startdate>20171001</startdate><enddate>20171001</enddate><creator>Govindaraj, V</creator><creator>Joseph, J</creator><creator>Kumar, B Nagamalli</creator><creator>Soman, R</creator><general>Medknow Publications and Media Pvt. 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| subjects | Abnormalities, Multiple - diagnostic imaging Adult Adults Aged Agenesis Brief Report Bronchiectasis - diagnostic imaging Bronchiectasis - etiology Bronchoscopy Care and treatment Case studies Diagnosis Dyspnea - etiology Female Health aspects Health education Humans Infections Lung Lung - abnormalities Lung - diagnostic imaging Lung Diseases - diagnostic imaging Magnetic Resonance Imaging Medical imaging Medicine Patients Pulmonary arteries Pulmonary artery Pulmonary Artery - abnormalities Pulmonary Artery - diagnostic imaging Pulmonary Artery - pathology Pulmonary hypertension Respiratory Tract Infections - etiology Tomography, X-Ray Computed Veins & arteries |
| title | Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases |
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