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Unilateral absence of the pulmonary artery in a paediatric cohort

Published online by Cambridge University Press:  03 October 2025

Laia Brunet-GarciaOpen the ORCID record for Laia Brunet-Garcia[Opens in a new window],
Flavio Zuccarino and
Juan Manuel Carretero Bellon
Laia Brunet-Garcia
Affiliation:
Department of Paediatric cardiology, Consorci Sanitari del Maresme, Hospital de Mataró, Barcelona, Spain
Flavio Zuccarino
Affiliation:
Department of Radiology, Hospital Sant Joan de Déu, Barcelona, SpainDepartment of Radiology, Hospital del Mar, Barcelona, Spain
Juan Manuel Carretero Bellon*
Affiliation:
Department of Paediatric cardiology, Hospital Sant Joan de Déu, Barcelona, SpainDepartment of Paediatric cardiology, Hospital Universitari Joan XXIII, Universitat Rovira i Virgili, Tarragona, Spain
*
Corresponding author: Juan Manuel Carretero Bellon; Email: juan.carretero@sjd.es
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Abstract

We present six paediatric patients with unilateral absence of the pulmonary artery in pursuance of rising the clinician’s recognition. Unilateral absence of the pulmonary artery was misdiagnosed in one patient by echocardiography. One patient had corrective surgery of a complete atrioventricular septal defect, and another had a right lobectomy. The remaining patients did not undergo any intervention and were asymptomatic at the end of the study period. Although there is a lack of consensus regarding optimal surgical approach, we opted for a more conservative strategy with good results.

Keywords

Unilateral absence of pulmonary arterypulmonary artery abnormalitiesCHD

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 10 , October 2025, pp. 2172 - 2174
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Unilateral absence of the pulmonary artery is a rare congenital pulmonary vascular dysplasia characterised by the absence of unilateral pulmonary artery due to occlusion or absence of the intrapericardial segment of the pulmonary artery. Reference Huang, Ling and Wu1 Its incidence is approximately 1 in 200,000. Reference Yang, Zheng and Xu2,Reference Tian and Zheng3 It is often associated with other CHDs, with Tetralogy of Fallot being the most common. Reference Huang, Ling and Wu1 Due to the absence of typical presenting symptoms and the scarce clinical knowledge of this condition, it is usually misdiagnosed. Reference Yang, Zheng and Xu2 Herein, we report the presentation, diagnosis, management, and long-term follow-up of six paediatric patients with unilateral absence of the pulmonary artery in pursuance of raising the clinician’s recognition.

Case report

Patient 1

A 1-day-old male presented with heart failure. He was diagnosed with unilateral absence of the pulmonary artery, complete atrioventricular canal defect, and pulmonary sequestration of the lower lobe of the right lung with partial anomalous pulmonary venous drainage of this segment to the right atrium. His diagnosis was confirmed through angiography. Besides, he had anorectal malformations with anal imperforation and scrotal fistulae, enlarged philtrum, and prominent auricular helix. He had a normal karyotype.

He underwent a pulmonary artery banding at 1.7 months and complete atrioventricular canal defect surgical correction at 46.2 months. He is currently asymptomatic with moderate tricuspid regurgitation, mild mitral regurgitation, and mild pulmonary hypertension 16.2 years after corrective complete atrioventricular canal defect surgery.

Patient 2

She was born at 26 weeks of gestation (960g) secondary to chorioamnionitis. She presented progressive cyanosis and was diagnosed with Tetralogy of Fallot, severe pulmonary stenosis, and unilateral absence of the pulmonary artery at 5 days of age. Diagnosis was confirmed by angiography. She had a normal karyotype (additional genetic analysis was not available at the end of this study). Surgical intervention was excluded due to the severity of pulmonary stenosis, hypoplastic right pulmonary artery, unilateral absence of the pulmonary artery and low weight. She presented clinical deterioration despite optimisation of medical therapy and died at 5.6 months of age.

Patient 3

A 1-day-old female presented with respiratory distress. She was diagnosed with unilateral absence of the pulmonary artery, ostium secundum atrial septal defect, right lung hypoplasia, diaphragmatic hernia, and bronchopulmonary dysplasia secondary to prematurity (31 weeks of gestation). Her unilateral absence of the pulmonary artery diagnosis was confirmed through CT (Figure 1). She had a negative microarray. She underwent right lobectomy at 7.6 months of age. She is currently asymptomatic after 3.8 years of follow-up.

Figure 1.

CT, maximum intensity projection (MIP) reconstruction, and volume rendering (VR) images of patient 3 depicting right absence of the pulmonary artery. Axial CT (A, B) and VR images (B-F) show right lung agenesia. Left pulmonary artery (LPA) and left lung present normal morphology. There is a large right diaphragmatic hernia (RDH) (A, C) and the right main bronchus is absent (E, F).

Patient 4

He presented with respiratory distress at 1.5 years of age. The diagnosis of unilateral absence of the pulmonary artery was made by CT as it was misdiagnosed as pulmonary artery branch stenosis through echocardiography (Figure 2). He had major aortopulmonary collateral arteries, pyelic ectasia, and vertebral malformations. He is currently asymptomatic after 9.1 years of follow-up.

Figure 2.

CT, maximum intensity projection (MIP) reconstruction, and volume rendering (VR) images of patient 4 showing right absence of the pulmonary artery. Axial MIP reconstruction (A) and VR images (B-F) show a patent left pulmonary artery (LPA) with absent right pulmonary artery. Withe arrow: small systemic collateral vessels (A). Trachea and main bronchus have a normal morphology (D-E) with two pulmonary parenchyma (white asterisks). Vertebral bodies (V) present fusion and malformations (F).

Patient 5

A 2-day-old male presented with hypoxaemia which progressively normalised. He had a patent ductus arteriosus and an atrial septal defect with no other associated findings. His unilateral absence of the pulmonary artery diagnosis was suspected through echocardiography and confirmed by CT (Supplementary figure 1). He is currently asymptomatic after 4.5 years of follow-up.

Patient 6

He had a prenatal diagnosis of unilateral absence of the pulmonary artery and right lung agenesis, which was postnatally confirmed by CT. He had a patent ductus arteriosus, atrial septal defect, bicuspid aortic valve, dextrocardia, and right lung agenesis. He presented mixed hearing loss. He had a negative microarray. He is currently asymptomatic after 3.1 years of follow-up.

Discussion

Whilst unilateral absence of the pulmonary artery has been reported to be asymptomatic during early childhood, all our patients but one were symptomatic between days 1 and 5 of life. These could be related to the fact that two of them had concomitant CHDs, and three were very preterm or moderate preterm infants.

In our cohort, consistent with previous reports, angiography or CT confirmed unilateral absence of the pulmonary artery diagnosis. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4,Reference Tian and Zheng3 In our institution, angiography was our diagnostic technique 25 years ago when CT was not widely used. In addition, unilateral absence of the pulmonary artery can be misdiagnosed by echocardiography, as in one of our patients. Reference Tian and Zheng3 A plausible explanation for this could be the misdiagnosis of the patent ductus arteriosus as a pulmonary artery branch in the context of insufficient awareness of this entity. Reference Tian and Zheng3

Early diagnosis and prompt surgical approach are paramount to ensure adequate pulmonary artery growth and lung development, diminishing the risk of pulmonary hypertension. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4,Reference Murphy, Winlaw, Cooper and Nunn5 Nevertheless, there is a lack of consensus regarding optimal surgical approach. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4 Whilst Shakibi et al stated that no treatment is mandatory in patients without cardiopulmonary dysfunction other than regular follow-up, others advocate for prompt search of hidden pulmonary arteries irrespective of symptoms with subsequent staged repair. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4,Reference Shakibi, Rastan, Nazarian, Paydar, Aryanpour and Siassi6 Surgical interventions include the creation of an aortopulmonary shunt or anastomosis of the disconnected pulmonary artery branch to the main pulmonary artery, allowing sufficient antegrade flow to the absent pulmonary artery. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4 An alternative is the interposition of a tube graft with autologous pericardium or prosthetic material and mobilisation, with anastomosis of the affected pulmonary artery branch to the main pulmonary artery. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4 Besides, there are other approaches such as embolisation, lobectomy, pneumectomy, or closure of selected collateral arteries. Reference Kruzliak, Syamasundar, Novak, Pechanova and Kovacova4,Reference Yan, Cen, Luo, Chen, Wen and Wu7 In our institution, we preferred a more conservative strategy with good results. One patient had pulmonary hypertension at the end of the study period, which could be related to his lung pathology.

In conclusion, unilateral absence of the pulmonary artery is difficult to diagnose given its low prevalence and absence of typical presentation symptoms. Besides, there is a lack of consensus regarding optimal surgical approach. In our centre, we opted for a conservative strategy with good results. Nevertheless, timely diagnosis is crucial for initiation of regular and comprehensive surveillance in order to establish a stepwise treatment approach considering conservative or surgical treatment tailored to patients’ characteristics. However, future studies with larger sample size are needed to better define more accurate treatment approach.

Supplementary material

For supplementary material accompanying this paper visit https://doi.org/10.1017/S1047951125109979

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Competing interests

None.

Ethical standards

The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guides and have been approved by the institutional committee of Hospital Sant Joan de Déu.

References

Huang, Q, Ling, W, Wu, Q et al. Anomalous origin of the fetal pulmonary artery. Front Pediatr 2023; 11: 1–11.CrossRefGoogle ScholarPubMed
Yang, Y, Zheng, H, Xu, Z et al. Outcomes of percutaneous closure of patent ductus arteriosus accompanied with unilateral absence of pulmonary artery. Am J Med Sci 2017; 353: 389–393.CrossRefGoogle ScholarPubMed
Tian, M, Zheng, M. Unilateral absence of pulmonary artery analysis based on echocardiographic feature. Rev Cardiovasc Med 2021; 22: 483–488.CrossRefGoogle ScholarPubMed
Kruzliak, P, Syamasundar, RP, Novak, M, Pechanova, O, Kovacova, G. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis 2013; 106: 448–454.CrossRefGoogle ScholarPubMed
Murphy, DN, Winlaw, DS, Cooper, SG, Nunn, GR. Successful early surgical recruitment of the congenitally disconnected pulmonary artery. Ann Thorac Surg 2004; 77: 29–35.CrossRefGoogle ScholarPubMed
Shakibi, JG, Rastan, H, Nazarian, I, Paydar, M, Aryanpour, I, Siassi, B. Isolated unilateral absence of the pulmonary artery. Jpn Heart J 1978; 19: 439–451.CrossRefGoogle ScholarPubMed
Yan, X, Cen, J, Luo, X, Chen, J, Wen, S, Wu, J, et al. Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: a single-center retrospective study. Transl Pediatr 2022; 11: 1813–1822.CrossRefGoogle ScholarPubMed
Derk Jan Ten Harkel, A, Blom, NA, Ottenkamp, J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002; 122: 1471–1477.CrossRefGoogle Scholar
Figure 0

Figure 1.CT, maximum intensity projection (MIP) reconstruction, and volume rendering (VR) images of patient 3 depicting right absence of the pulmonary artery. Axial CT (A, B) and VR images (B-F) show right lung agenesia. Left pulmonary artery (LPA) and left lung present normal morphology. There is a large right diaphragmatic hernia (RDH) (A, C) and the right main bronchus is absent (E, F).

Figure 1

Figure 2.CT, maximum intensity projection (MIP) reconstruction, and volume rendering (VR) images of patient 4 showing right absence of the pulmonary artery. Axial MIP reconstruction (A) and VR images (B-F) show a patent left pulmonary artery (LPA) with absent right pulmonary artery. Withe arrow: small systemic collateral vessels (A). Trachea and main bronchus have a normal morphology (D-E) with two pulmonary parenchyma (white asterisks). Vertebral bodies (V) present fusion and malformations (F).

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