Isolated Unilateral Absence of Right Proximal Pulmonary Artery: Surgical Repair and Follow-Up

Welch, Kristin; Hanley, Frank L.; Johnston, Troy A.; Cailes, Colleen; Shah, Maully J.

doi:10.1016/j.athoracsur.2003.10.037

Cited by 67 publications

(69 citation statements)

References 8 publications

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“…Its prevalence as an isolated lesion is estimated at 1 in 200 000‐to‐300 000 adults,10, 18, 19, 20 and 80% of reported cases involving the left PA have been associated with coexisting CHD, such as TOF or truncus arteriosus 10, 19. In 2011, a review of the literature reported 352 cases of unilateral “absence” of pulmonary artery; two thirds (n=237) were associated with other CHD 21.…”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

“…In 2011, a review of the literature reported 352 cases of unilateral “absence” of pulmonary artery; two thirds (n=237) were associated with other CHD 21. PH is present in 44% of cases and, in conjunction with the underlying CHD, affects appropriate management and outcomes for these patients 18, 19, 22. PH may occur as a result of increased flow to the “healthy” lung, or in the “disconnected” lung supplied by large collaterals or a large PDA (Figure 4).…”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

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Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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“…In asymptomatic patients, some clinicians adopt conservative management with close follow-up. Others opt for early revascularisation of the interrupted pulmonary artery to restore a physiological pulmonary circulation, which may lead to regression of pulmonary hypertension 8,9 . If massive haemoptysis ensues, selective embolization of systemic collaterals may be an option 10 .…”

Section: Njr I Vol 1 I No 1 I Issue 1 I July-dec 2011mentioning

confidence: 99%

Interruption of Left Pulmonary Artery with Right Sided Aortic Arch with TOF: A Complete Anomaly Combination

Gangadhar

2012

Nep J Radiology

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Interruption of the pulmonary artery is a rare congenital disorder. The clinical symptoms are non-specific and radiological investigations are the key to diagnosis. We present a case of interruption of the left pulmonary artery associated with Tetralogy of Fallot in a young male. The radiological and clinical features of this condition are also reviewed.Key words: Right sided aortic arch: Interruption of the pulmonary artery: Ventricular Septal Defect (VSD) Case HistoryAn 18-year-old male presented with an episode of hemoptysis and cough. He had a significant past history of recurrent chest infections and few episodes of hemoptysis. There was no history of tuberculosis in the past. On examination patient was mildly tachypneic (20 breaths per minute) with normal pulse rate and blood pressure. Chest examination revealed reduced breath sounds in left side with more laterally shifted apical pulse. Right side of chest revealed mild hyper resonant percussive noted with retrosternal mild hyper resonance.Review of his chest radiographs ( Figure A) showed a grossly enlarged heart (probably related to Tetralogy of Fallot), a right-sided ____________________________________ Correspondence to: Dr. Kiran Gangadhar Department of Radiodiagnosis and Imaging, Banaras Hindu University, Varanasi, India Email: kirang.585@googlemail.com aortic arch, and an oligaemic hypoplastic left lung. The right pulmonary artery and pulmonary trunk were enlarged, but the left hilum was indistinct. Rib notchings were seen at the left 4th to 6th ribs.Computed tomographic (CT) pulmonary angiography was obtained during the patient's stay in the hospital revealed dilated right main pulmonary artery and its divisions ( Figure B). There was, however, complete absence of the mediastinal portion of the left main pulmonary artery. Right sided aortic arch seen with mirror image branching with a large perimembranous VSD with dilated right ventricle suggestive of probable TOF. Prominent intercostals arteries and bronchial arteries were coursing within areas of extra pleural fat and mediastinum at several levels, supplying collateral circulation to the smaller left lung. Although the left lung was small, the bronchial branching pattern was normal with few patchy foci of pleural thickening. There was no evidence of bronchiectasis. Mild hyperinflation of the right lung was

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“…However, cardiac malformation is more common in the left sided agenesis (5). When cardiac malformation coexists with UPAA, the patients are more symptomatic and diagnosed during childhood (7). The most common symptoms in UPAA are recurrent pulmonary infections, hemoptysis, and dyspnea (6).…”

mentioning

confidence: 99%

A rare case of right sided pulmonary artery agenesis associated with congenital mitral valve prolapse

Iliaz¹,

Aslan²,

Ozturk

et al. 2016

Tuberk Toraks

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Unilateral pulmonary artery agenesis (UPAA) is a rare condition. It is caused by an embryogenic malformation of the 6 th aortic arch of the affected side (1,2). The first case was reported in 1868 and the estimated prevalence is 1/200.000 people (3-5). Right-sided UPAA is twice as common as the left-sided (6). However, cardiac malformation is more common in the left sided agenesis (5). When cardiac malformation coexists with UPAA, the patients are more symptomatic and diagnosed during childhood (7). The most common symptoms in UPAA are recurrent pulmonary infections, hemoptysis, and dyspnea (6). If the agenesis is isolated, the symptoms may be minor or absent, the diagnosis can be made in the adulthood (5). A study by Bouros D. et al reported that 30% of the adult patients with UPAA haven't any symptoms (5). In this study, 75% of the left-sided UPAA was associated with congenital cardiac anomalies, whereas right-sided agenesis was not associated with any cardiac malformation (5). The most common associated cardiovascular abnormalities are tetralogy of Fallot, septal defects, patent ductus arteriosus, coarctation of the aorta and transposition of great vessels (4). Mitral valve prolapse (MVP) association with UPAA is very rare. In the literature, there are only two cases (5,8). To the best of our knowledge, it is the third reported right-sided UPAA case with coexisting MVP. Here, we present a rare case of right-sided UPAA and severe MVP diagnosed at adult age.A 43-year-old man was referred to pulmonary medicine outpatient clinic for investigation of dyspnea after the prosthetic ring valvuloplasti. He was on anti-hypertensive treatment for a year. One month prior to his routine evaluation for cardiology, he reported exertional dyspnea and orthopnea. He was non-smoker. Had no other systemic symptoms. On the physical examination he had 4/6 systolic murmur at mitral area. In the echocardiographic evaluation, there was grade 3-4 mitral regurgitation, mitral valve prolapse, and chordal rupture. Systolic pulmonary arterial pressure was 50 mmHg, the left ventricular ejection fraction was 65%. The coronary angiography was normal.A rare case of right sided pulmonary artery agenesis associated with congenital mitral valve prolapse

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Isolated Unilateral Absence of Right Proximal Pulmonary Artery: Surgical Repair and Follow-Up

Cited by 67 publications

References 8 publications

Definition and Management of Segmental Pulmonary Hypertension

Definition and Management of Segmental Pulmonary Hypertension

Interruption of Left Pulmonary Artery with Right Sided Aortic Arch with TOF: A Complete Anomaly Combination

A rare case of right sided pulmonary artery agenesis associated with congenital mitral valve prolapse

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