Pediatric Pulmonology 43:822–823 (2008)

Case Report

An Uncommon Cause of a Vascular Mass inthe Left Lung in Neonate:

A Case Report With a Brief Review of Literature

Sreenivas Reddy* and Rohit Kumar

Summary. A 20-day-old female child presented with mild respiratory distress since first week of

life. On examination there was an ejection systolic murmur at left sternal border. Chest X-ray

revealed well-defined mass in the left lung. Echocardiogram showed features suggestive tetrology

of fallot with absent pulmonary valve with massively dilated main and left pulmonary artery. We

report a rare cause of vascular mass in a neonate due to tetrology fallot with absent pulmonary

valve. Pediatr Pulmonol. 2008; 43:822–823. � 2008 Wiley-Liss, Inc.

Key words: Tetralogy of Fallot; absent pulmonary valve.

CASE REPORT

A 20-day-old female child was brought to paediatricemergency department with complaints of mild res-piratory distress since first week of life. She was born ofan uncomplicated vaginal delivery at full term, with nohistory suggestive of birth asphyxia.

On examination, the child was noted to have mildcyanosis and a grade III/VI ejection systolic murmur atleft sternal border. Chest was clear. Rest of the physicalexamination was unremarkable. Chest X-ray AP viewshowed a well defined mass of approximately 6 cm� 5 cmwith well delineated superior, inferior, lateral margins,and the medial margin merging with the mediastinum(Fig. 1). There was hyperinflation of the lungs as evidentby the hyperlucency of upper and lower zones with widelyspaced left sided ribs. Electrocardiogram showed rightventricular hypertrophy. Echocardiogram revealed alarge ventricular septal defect with malalignment and50% aortic override. The main pulmonary artery andthe left pulmonary artery were aneurysmally dilated.Pulmonary valve was absent. There was pulmonaryannular narrowing with peak systolic gradient of 64 mmHgand moderate pulmonary regurgitation. A diagnosis ofTetralogy of Fallot with absent pulmonary valve wasmade. Respiratory distress improved with conservativemanagement over weeks. The oxygen saturation improv-ed from 89% to 92%.

DISCUSSION

Aneurysmal dilatation of pulmonary trunk is rare inneonates, but can occur in adults as an isolated condition1

or in association with Marfans syndrome, syphilis,bacterial endocarditis or following Blalock operation.Cystic parenchymal lesions like neuroenteric cyst, bron-chogenic cyst, esophageal duplication cyst, or epidermoidcyst can give a similar picture in infants. Thymic anddermoid cysts, although in anterior mediastinum, may beclosely related to the pulmonary trunk. Partial absence ofleft pericardium can simulate pulmonary trunk enlarge-ment in a chest skiagram.

Pulmonary valve is reported to be absent in 2.4–6.3% ofpatients with Tetralogy of Fallot. The systolic distensionof pulmonary artery in utero, caused by an increased rightventricular stroke volume, contributed by the regurgitant

Department of Cardiology, PGIMER, Chandigargh, India.

*Correspondence to: Dr. Sreenivas Reddy, Department of Cardiology,

PGIMER, Chandigargh 160012, India.

E-mail: reddycardio2911@yahoo.co.in

Received 18 August 2007; Revised 12 October 2007; Accepted 16 October

2007.

DOI 10.1002/ppul.20756

Published online 9 July 2008 in Wiley InterScience

(www.interscience.wiley.com).

� 2008 Wiley-Liss, Inc.

volume and absence of ductus is postulated to accountfor the aneurismal dilatation of pulmonary trunk andits branches. Poststenotic dilatation due to pulmonaryvalvular ring stenosis may also play a role in the patho-genesis of dilatation of the pulmonary trunk.2,3 Compres-sion of the trachea by the dilated central pulmonaryarteries and compresssion of the lesser bronchi by theabnormal branching pattern of intrapulomnry arteriesresult in respiratory distress that may be aggravated insupine posture. Air trapping occurs as a consequence ofthe compression of the bronchi leading to hyperinflationof the lung which may be either segmental or diffuse innature.

The operative management of Tetralogy of Fallot withabsent pulmonary valve syndrome remains controversialregarding the selection of palliative or single stagecorrection, and the need for pulmonary valve implantationand pulmonary arterioplasty. Pulmonary artery resectionand pulmonary valve implantation may improve outcomein patients with persistent airway compression.4

Valved homograft replacement of aneurysmally dilatedpulmonary arteries at initial repair has also been reportedto improve survival, especially in neonates with severerespiratory distress.5 Though many authors advocate aselective approach to pulmonary valve insertion at initialrepair, there is less controversy regarding the need of

pulmonary artery reduction arterioplasty as the primarytreatment in infants.6 Other surgical procedures triedinclude transection of dilated pulmonary artery with orwithout a concomitant Blalock Taussig shunt. Trans-location of the dilated pulmonary artery anterior to theaorta and away from the bronchial tree during primaryrepair of Tetralogy of Fallot with absent pulmonaryvalve syndrome to relieve the symptoms in newborns andinfants has been described with successful outcome.7

Stenting to the right and left main bronchi with the balloonexpandable metallic stents is another new experimentaltherapy that has been successfully used in neonateswith respiratory failure despite satisfactory intracardiacrepair.8,9

This case is being reported to highlight the occurrence ofthis relatively rare congenital anamoly, and the importanceto consider the dilated pulmonary trunk secondary to absentpulmonary valve syndrome in the differential diagnosis ofan unexplained parahilar vascular mass in a neonate.

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Fig. 1. Chest X-ray AP view showing aneurysmally dilated

pulmonary artery.

Pediatric Pulmonology

Case Report 823