Isolated absence of right pulmonary artery
<b>Background</b> : Absence of right pulmonary artery (RPA) is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infants <b>Objective</b> : The aim of this study was to describe infantile presentation of isolated absence of RPA along...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2010-01-01
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| Series: | Annals of Pediatric Cardiology |
| Subjects: | |
| Online Access: | http://www.annalspc.com/article.asp?issn=0974-2069;year=2010;volume=3;issue=2;spage=119;epage=122;aulast=Singhi |
| _version_ | 1811279244199723008 |
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| author | Singhi Anil Francis Edwin Kumar Raman |
| author_facet | Singhi Anil Francis Edwin Kumar Raman |
| author_sort | Singhi Anil |
| collection | DOAJ |
| description | <b>Background</b> : Absence of right pulmonary artery (RPA) is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infants <b>Objective</b> : The aim of this study was to describe infantile presentation of isolated absence of RPA along with a brief review of the literature. <b>Methods</b> : The details of five patients diagnosed with isolated absence of RPA from April 2007 to October 2009 were reviewed retrospectively <b>Results</b> : Five patients were identified with this anomaly. The median age of presentation was 86 days (range, 40-120 days) and the median weight was 3.65 kg (range, 3.1-5.5 kg). All patients presented with breathing difficulty and had severe pulmonary hypertension (PHT) along with absent RPA on echocardiography. A multidetector computed tomographic scan was performed to confirm the diagnosis in four cases. Three patients had major aortopulmonary collateral and the hilar RPA was not well developed in all. A correct diagnosis was made before referral in one patient only. Differential vascularity in chest X-ray, a useful clue, was seen in four of five cases. Surgical correction was not considered in view of the small hilar pulmonary artery. The patients were all managed medically with diuretics and Sildenafil. <b>Conclusion </b>: Isolated absence of RPA is a rare congenital abnormality with varied presentation. Infantile presentation is marked with congestive cardiac failure and PHT. Specific diagnostic clue includes differential vascularity on the chest X-ray. |
| first_indexed | 2024-04-13T00:51:18Z |
| format | Article |
| id | doaj.art-952ac1edc641436987b9011156b53f2e |
| institution | Directory Open Access Journal |
| issn | 0974-2069 0974-5149 |
| language | English |
| last_indexed | 2024-04-13T00:51:18Z |
| publishDate | 2010-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Annals of Pediatric Cardiology |
| spelling | doaj.art-952ac1edc641436987b9011156b53f2e2022-12-22T03:09:52ZengWolters Kluwer Medknow PublicationsAnnals of Pediatric Cardiology0974-20690974-51492010-01-0132119122Isolated absence of right pulmonary arterySinghi AnilFrancis EdwinKumar Raman<b>Background</b> : Absence of right pulmonary artery (RPA) is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infants <b>Objective</b> : The aim of this study was to describe infantile presentation of isolated absence of RPA along with a brief review of the literature. <b>Methods</b> : The details of five patients diagnosed with isolated absence of RPA from April 2007 to October 2009 were reviewed retrospectively <b>Results</b> : Five patients were identified with this anomaly. The median age of presentation was 86 days (range, 40-120 days) and the median weight was 3.65 kg (range, 3.1-5.5 kg). All patients presented with breathing difficulty and had severe pulmonary hypertension (PHT) along with absent RPA on echocardiography. A multidetector computed tomographic scan was performed to confirm the diagnosis in four cases. Three patients had major aortopulmonary collateral and the hilar RPA was not well developed in all. A correct diagnosis was made before referral in one patient only. Differential vascularity in chest X-ray, a useful clue, was seen in four of five cases. Surgical correction was not considered in view of the small hilar pulmonary artery. The patients were all managed medically with diuretics and Sildenafil. <b>Conclusion </b>: Isolated absence of RPA is a rare congenital abnormality with varied presentation. Infantile presentation is marked with congestive cardiac failure and PHT. Specific diagnostic clue includes differential vascularity on the chest X-ray.http://www.annalspc.com/article.asp?issn=0974-2069;year=2010;volume=3;issue=2;spage=119;epage=122;aulast=SinghiAbsent right pulmonary arterychest X-raypulmonary hypertension |
| spellingShingle | Singhi Anil Francis Edwin Kumar Raman Isolated absence of right pulmonary artery Annals of Pediatric Cardiology Absent right pulmonary artery chest X-ray pulmonary hypertension |
| title | Isolated absence of right pulmonary artery |
| title_full | Isolated absence of right pulmonary artery |
| title_fullStr | Isolated absence of right pulmonary artery |
| title_full_unstemmed | Isolated absence of right pulmonary artery |
| title_short | Isolated absence of right pulmonary artery |
| title_sort | isolated absence of right pulmonary artery |
| topic | Absent right pulmonary artery chest X-ray pulmonary hypertension |
| url | http://www.annalspc.com/article.asp?issn=0974-2069;year=2010;volume=3;issue=2;spage=119;epage=122;aulast=Singhi |
| work_keys_str_mv | AT singhianil isolatedabsenceofrightpulmonaryartery AT francisedwin isolatedabsenceofrightpulmonaryartery AT kumarraman isolatedabsenceofrightpulmonaryartery |