Isolated absence of right pulmonary artery in a 4-year old child: a case report

Weldetsadik, Abate Yeshidinber; Asfaw, Yemisrach Mekonnen; Tekleab, Atnafu Mekonnen

doi:10.2147/imcrj.s181571

Cited by 8 publications

(11 citation statements)

References 13 publications

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“…Chopra [17] analyzed a 77-year-old woman with UAPA. Some pediatric UAPA cases were analyzed and sorted out by Weldetsadik [18]. Raymond [19] analyzed neonatal UAPA cases and summarized diagnostic points and methods and mainly described the basic feature of echo findings.…”

Section: Discussionmentioning

confidence: 99%

Unilateral absence of pulmonary artery analysis based on echocardiographic feature

Tian¹,

Zheng²

2021

Reviews in Cardiovascular Medicine

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The unilateral absence of pulmonary artery (UAPA) is a rare congenital cardiovascular malformation, which is asymptomatic and easy to be ignored in early stage. A large number of complications may occur in the later stage. Therefore, early diagnosis and treatment is of great significance. The imaging data of 49 patients with UAPA discovered and confirmed clinically by the echocardiography in our hospital are analyzed. The results show that left pulmonary artery absence is more common (55%) and most of them are associated with other cardiovascular malformations (92%). Atrial septal defect and patent foramen ovale were most common in 56%. In which the absence of isolated pulmonary artery was 8% (4/49), and the absence of right pulmonary artery was 75% (3/4). Especially in the patients with tetralogy of Fallot, 77% (5/9) of them miss the diagnosis of UAPA. This suggests that doctors and sonographers should pay attention to the development of pulmonary artery bifurcation and left and right branches in multi-section, and strengthen the scanning of short axis section of high large artery.

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Section: Discussionmentioning

confidence: 99%

Unilateral absence of pulmonary artery analysis based on echocardiographic feature

Tian¹,

Zheng²

2021

Reviews in Cardiovascular Medicine

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“…Therefore, patients without other significant anomalies can survive until adulthood without symptoms, which is the reason for the late diagnosis of UPAA. Dyspnea, limited exercise tolerance, recurrent pulmonary infections, and hemoptysis are the most common symptoms observed in adult patients with UPAA ( 1 , 2 , 4 ). Pulmonary hypertension develops in 19%–44% of adult patients and reduces long-term survival ( 1 , 2 , 10 ).…”

Section: Discussionmentioning

confidence: 99%

Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl

Miseviciene,

Liakaite,

Zaveckiene

et al. 2023

Front. Pediatr.

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BackgroundUnilateral pulmonary artery agenesis (UPAA) and Kommerell's diverticulum (KD) are two rare embryologically unrelated congenital vascular malformations rarely diagnosed in children. This is the first report of our knowledge of the unique combination for a child as patients are at a high risk of pulmonary hypertension and rupture of the diverticulum. Our aim is to present the case of a pediatric patient with UPAA and KD with the short literature review and to highlight the importance of early diagnostics of rare congenital vascular malformations.Case reportA 1-year-old girl presented to the emergency department with prolonged cough and variable wheezing. A hypoplastic left lung was suspected in the radiographic image of the chest. A transthoracic echocardiogram revealed absence of the left pulmonary artery and right arch of aorta and anomaly of subclavian arteries was suspected. The diagnosis was confirmed by computed tomography scans of the chest that demonstrated elongation of the aorta and an aberrant right subclavian artery with KD, as well as absence of the left pulmonary artery. The patient is being followed up for the development of pulmonary hypertension and compression of vascular structures to the airways as well as any indications for surgical intervention because of the KD.ConclusionsUPAA and KD are two very rare congenital vascular anomalies usually diagnosed in adults. A high risk of pulmonary hypertension and rupture of diverticulum is noted for adult patients. This case provides us with an exclusive possibility to follow up a patient with an extremely rare combination of the two vascular anomalies with insufficiently known future complications and outcomes.

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“…In the patients with small intrapulmonary arteries, a modified Blalock-Taussig shunt is recommended before the anastomosis to aid with vessel growth prior to unifocalization. In asymptomatic patients who have no cardiopulmonary dysfunction, clinical follow-up may be reasonable ( 12 , 13 ). Our patient had pulmonary hypertension; therefore, PDA ligation was performed in hopes of decreasing the pulmonary artery pressure.…”

Section: Discussionmentioning

confidence: 99%

Interruption of the Right Pulmonary Artery in a Neonate

Khalilian

Langroudi

Dabbagh

et al. 2022

Case Reports in Cardiology

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Interruption of the right pulmonary artery is a very rare anomaly which can be associated with other congenital heart lesions or can occur in isolation. Clinical presentations of the unilateral interruption of a pulmonary artery are varied including pulmonary hypertension, recurrent infection, dyspnea, exercise intolerance, hemoptysis, and chest pain. Less commonly, patients may be asymptomatic. Diagnosis of this anomaly is made by echocardiography and CT angiography as well as cardiac MRI. Treatment options are medical versus surgical management and often recommended in symptomatic patients with pulmonary hypertension, recurrent infection, and hemoptysis. Herein, we describe a very rare case of right pulmonary artery originating from the right subclavian artery in a 12-day-old neonate.

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Isolated absence of right pulmonary artery in a 4-year old child: a case report

Cited by 8 publications

References 13 publications

Unilateral absence of pulmonary artery analysis based on echocardiographic feature

Unilateral absence of pulmonary artery analysis based on echocardiographic feature

Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl

Interruption of the Right Pulmonary Artery in a Neonate

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