Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: a single-center retrospective study

Yan, Xiaojun; Cen, Jianzheng; Luo, Xin-lan; Chen, Jimei; Wen, Shusheng; Wu, Jian; Zhuang, Jian

doi:10.21037/tp-22-491

Cited by 4 publications

(2 citation statements)

References 23 publications

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“…Sildenafil is recommended for the treatment of pulmonary hypertension ( 10 ). Also, patients with UPAA and pulmonary hypertension should undergo surgical reconstruction and pulmonary artery rehabilitation ( 12 ). Pulmonary angiography and embolization can be performed in patients with hemoptysis because of the chronically hyperperfused vessels on the contralateral side of the UPAA ( 1 , 4 , 13 ).…”

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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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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“…(17) An alternative is the interposition of a tube graft with autologous pericardium or prosthetic material and mobilization with anastomosis of the affected PA branch to the main PA. (17) Besides, there are other approaches such as embolization, lobectomy or pneumectomy in cases of recurrent haemoptysis or closure of selected collateral arteries. (1)(17) (22) In our centre, one patient underwent corrective surgery of a CHD and another right lobectomy. The remaining three patients that did not undergo any intervention for the UAPA had remained asymptomatic with no PHT.…”

Section: Treatmentmentioning

confidence: 99%

Congenital anomalies of the pulmonary arterial system in a paediatric cohort: a single- center 28-year experience

Brunet-Garcia,

Bellon,

Zuccarino

2023

Preprint

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Background and objective Congenital anomalies of the pulmonary arterial system are rare. They include left pulmonary artery sling (LPAS), unilateral absence of the pulmonary artery (UAPA) and anomalous origin of unilateral pulmonary artery from the ascending aorta (AOPA) or hemitruncus. Our aim was to review 21 paediatric cases and describe their presentation, , diagnosis, management and long-term follow-up. Material and methods A retrospective observational study of all paediatric patients with pulmonary arterial system abnormalities diagnosis (May 1995-March 2023) was conducted. Patients underwent systematic evaluation (medical and family history; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography). Results 21 paediatric patients with pulmonary arterial system abnormalities (LPAS n=11, UAPA n=6 and AOPA n=4) were included. The median age at diagnosis was 2.0 [0.1-12.0] months, the median follow-up time 2.4 [0.4-11.7] for all patients and 8.0 [0.7-12.5] years for survivors. 2 patients (9.5%) were prenatally diagnosed. Echocardiography diagnosed 18 patients (85.7%). The diagnosis was confirmed by MR (n=1), CT (n=17) or angiography (n=3). There were 5 deaths (23.8%) (median age 1.4 [0.6-5.9] months). Conclusion Prompt diagnosis and treatment of patients with pulmonary arterial system abnormalities is paramount to diminish post-operative residual lesions and morbidity, as demonstrated in our study. Nevertheless, further studies including larger cohorts are required not only to better define consensus regarding surgical management of congenital abnormalities of the pulmonary arterial system but also to improve their aetiology understanding and associated anomalies. These will enhance the clinical delineation of these entities leading to an increase of clinicians’ awareness.

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Unilateral pulmonary artery agenesis with unilateral interstitial lung disease in an allergic bronchopulmonary aspergillosis patient

George,

Anithakumari,

Philip

et al. 2023

PULMON

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Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: a single-center retrospective study

Cited by 4 publications

References 23 publications

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

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

Congenital anomalies of the pulmonary arterial system in a paediatric cohort: a single- center 28-year experience

Unilateral pulmonary artery agenesis with unilateral interstitial lung disease in an allergic bronchopulmonary aspergillosis patient

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