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Unilateral pulmonary artery agenesis: clinical and laboratory findings of four cases and diagnostic clues for pediatricians/Tek tarafli pulmoner arter yoklugu: cocuk uzmanlarina tani koymada ipuclari, dort olgunun klinik ve laboratuvar bulgulari.


Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly (1). The embryological explanation for the origin of UAPA is the involution of the proximal sixth aortic arch and persistence of the connection of the intrapulmonary-pulmonary artery to the distal sixth aortic arch. Some patients with isolated UAPA are completely asymptomatic; others may have severe pulmonary hypertension, pulmonary hemorrhage, congestive heart failure and cyanosis. Chest x-ray may show an absent hilar shadow, a shrunken affected lung, and a shift of the mediastinal structures to the affected side (2, 3). Subsequently, an echocardiography may confirm the diagnosis. Computerized tomography (CT) and magnetic resonance imaging (MRI) can show collateral arteries (1, 2, 4). Pulmonary artery pressure and collateral arteries can be revealed by cardiac catheterization (2, 3, 5, 6). In the present report, four cases with UAPA were discussed.

Case Reports

The demographic and clinical features of four cases are summarized in Table 1. Chest x-rays revealed absent hilar shadow and a shift of the mediastinal structures to the affected side (Fig. 1a). Echocardiography showed UAPA in all cases. Additional echocardiographic findings were coarctation of aorta (CoA) with patent ductus arteriosus (PDA) in case 1, abnormally partially venous return anomaly in case 2 and mitral valve prolapse (MVP) in case 4. Also, right aortic arch was showed in three of cases. Cardiac catheterization and imaging revealed UAPA (Fig. 1b) and many collateral arterial formations originating from descending aorta, common carotid artery and left subclavian artery (Fig. 2a, b, Fig. 3a). However, left lung of case 2 was not imaged, vertical vein was observed on MRI (Fig. 3b).


The exact prevalence of UAPA is unknown, but the current literature estimates 1:200.000 individuals (1). In the literature, more than 300 cases have been reported since 2010 and absence of right pulmonary artery is more often than the left one (2-7). Recurrent pulmonary infections, decreased exercise tolerance and mild dyspnea during exertion are the most common symptoms (3, 7). Also, some patients with isolated UAPA can be presented with severe pulmonary hypertension, pulmonary hemorrhage, congestive heart failure and cyanosis. Eventually, in our cases, newborns with cyanosis had a severe form of the disease. So, we have suggested that the severity of disease may be associated with young age and the symptom of cyanosis.

Congenital heart defects that have been associated with UAPA are the followings; tetralogy of Fallot, ventricular septal defect (VSD), right aortic arch, truncus arteriosus, patent ductus arteriosus (PDA), CoA, subvalvular aortic stenosis, transposition of the great arteries and scimitar syndrome (2, 3, 5). Right pulmonary artery originating from ascending aorta is commonly associated with atrial septal defect (ASD) (8, 9). Also, left pulmonary artery originating from aorta associated with ASD was reported in the literature (8). Additionally, in a recent study, it was reported that the incidence of pulmonary hypertension was 86% among the patients with UAPA plus PDA and these patients died of pulmonary hypertension at early ages (7). Associated cardiac anomalies in our two cases (case 1 and 3) with ASD were CoA, PDA, VSD and right aortic arch respectively. Also, abnormal partially venous return anomaly was presented in case 2. In the literature the first reported patient with dextrocardia, absent of right pulmonary artery and MVP was an adult (10). Similarly in our case 4, absence of left pulmonary artery with MVP and moderate mitral regurgitation was detected as a first association in childhood.


On chest X-ray, ipsilateral grossly diminished pulmonary vascular markings, hemidiaphragmatic elevation, cardiac and mediastinal displacement, a small hemithorax, and contralateral lung hyperinflation can be detected. Absence of pulmonary artery can be showed by echocardiography. Anatomic details and distal pulmonary arteries can be visualized by MRI and high resolution CT. The presence of hilar arteries can be demonstrated by cardiac catheterization and pulmonary venous wedge angiography.

The incidence of pulmonary hypertension varies between 18-44% in isolated UAPA and it is much higher among patients who had UAPA with PDA (1-3, 5, 7, 8). This is because of that, the contralateral pulmonary vascular bed has insufficient elasticity to cope with the increased blood flow (4).




Unilateral absence of a pulmonary artery should be considered in the patients manifested with cyanosis, recurrent respiratory infections or pulmonary hypertension. Early diagnosis can prevent further deterioration and associated comorbidities. Primarily, chest radiography and echocardiography are useful for the diagnosis of UAPA.


(1.) Bouros D, Pare P Panagou P Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995; 108: 670-6. [CrossRef]

(2.) Apostolopoulou SC, Kelekis NL, Brountzos EN, Rammos S, Kelekis DA. Absent pulmonary artery in one adult and five pediatric patients: imaging, embriology, and therapeutic implications. AJR Am J Roentgenol 2002; 179: 1253-60.

(3.) Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002; 22: 1471-7. [CrossRef]

(4.) Halit V, Olgunturk R, Erer D, Kula S. Tetralogy of Fallot and absence of left pulmonary artery. Thorac Cardiovasc Surg 2008; 56: 430-2. [CrossRef]

(5.) Atik E, Tanamati C, Kajita L, Barbero-Marcial M. Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery. Arq Bras Cardiol 2006; 87: 423-8. [CrossRef]

(6.) Prasad SK, Soukias N, Hornung T, Khan M, Pennell DJ, Gatzoulis MA, et al. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation 2004; 109: 207-14. [CrossRef]

(7.) Koga H, Hidaka T, Miyako K, Suga N, Takahashi N. Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. Pediatr Cardiol 2010; 31: 1186-90. [CrossRef]

(8.) Lee JH, Jung TE, Lee YH. Anomalous origin of left pulmonary artery from aorta with atrial septal defect. Pediatr Cardiol 2008; 29: 860-1. [CrossRef]

(9.) Prifti E, Bonacchi M, Murzi B, Crucean A, Leacche M, Bernabei M, et al. Anomalous origin of the right pulmonary artery from the ascending aorta. J Card Surg 2004; 19: 103-12. [CrossRef]

(10.) Yip AS, Chow WH, Lee DF Cheung KL. Mitral valve prolapse causing severe mitral regurgitation in a patient with absent right pulmonary artery. Clin Cardiol 1995; 18: 424-5. [CrossRef]

Zehra Karatas, Hayrullah Alp, Hakan Altin, Tamer Baysal

Department of Pediatric Cardiology, Meram Faculty of Medicine, Selcuk University, Konya-Turkey

Address for Correspondence/Yazisma Adresi: Dr. Zehra Karatas

Selcuk University Meram Faculty of Medicine, Department of Pediatric Cardiology, Beysehir Street, 42080 Meram, Konya-Turkey

Phone: +90 332 223 68 63 Fax: +90 332 223 6181


Available Online Date/Cevrimici Yayin Tarihi: 08.08.2012

Table 1. Demographic and clinical features of cases

 Case 1 Case 2 Case 3 Case 4

Age 18-day-old 6-day-old 18-mount-old 11-years-
Sex Female Female Male Male
Body weight 2.8 kg 2.7 kg 6.5 kg (< 3P) 38 kg
Complaints Cyanosis Cyanosis Recurrent Chest pain
 pulmonary and
 infections palpitation
Relativity of None Second First degree None
parents degree
Oxygen 65 76 92 93
saturation, %
Electrocardio- Right axis Right axis Right axis Normal axis
Absence of Right Right Left Left
Chest X-ray Decrease of the bronchovascular frames
 and deviation of trachea on the same
 hemithorax and increase of the aeration
 on the contra lateral
Collateral - DA Common LCA and DA
arteries carotid artery
 and DA
Right aortic + - + +
echocardio- VSD, ASD
Contralateral 60/10: 80/25: 50/20: 55/25:
PAP, mmHg mean 28 mean 60 mean 30 mean 35
Prognosis Exitus Being followed up in our clinic

ASD--atrial septal defect, CoA--coarctation of the aorta,
DA--descending aorta, LCA--left subclavian artery, MR--mitral
regurgitation, MVP--mitral valve prolapse, PAP--pulmonary
artery pressure, PAPVR--partial anomalous pulmonary venous return,
PDA--patent ductus arteriosus, VSD--ventricular septal defect
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Article Details
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Title Annotation:Case Reports/Olgu Sunumlari
Author:Karatas, Zehra; Alp, Hayrullah; Altin, Hakan; Baysal, Tamer
Publication:The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)
Date:Nov 1, 2012
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