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.
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.
[FIGURE 1 OMITTED]
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).
[FIGURE 2 OMITTED]
[FIGURE 3 OMITTED]
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.
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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- old 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 graphy Absence of Right Right Left Left pulmonary artery 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 + - + + arch Additional CoA, PDA, PAPVR ASD MVP MR echocardio- VSD, ASD graphic findings 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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|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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