Abstract. Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures. Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression from pulmonary arterial dilatation is rare in adults, and there are no reports in the literature of its occurrence in idiopathic pulmonary arterial hypertension. Compression in infants with congenital heart disease has been well described. We report 2 cases of tracheobronchial compression: first, an adult patient with idiopathic pulmonary arterial hypertension who presents with symptomatic left main bronchus compression, and second, an adult patient with Eisenmenger ventricular septal defect and right-sided aortic arch, with progressive intermedius and right middle lobe bronchi compression in association with enlarged pulmonary arteries

Ariff, B

Davies, R

Gibbs, JS

Gin-Sing, W

Howard, L

Jaijee, SK

O'Regan, DP

English

PubMed

Spiral - Imperial College Digital Repository

C A S E R E P O R TLeft main bronchus compression due to main pulmonary arterydilatation in pulmonary hypertension: two case reportsShareen K. Jaijee,1,2 Ben Ariff,3 Luke Howard,4 Declan P. O’Regan,2 Wendy Gin-Sing,4 Rachel Davies,4 J. Simon R. Gibbs41University of Sydney, Camperdown, New South Wales, Australia; 2MRC Clinical Sciences Centre, Imperial College London, Hammersmith Hospital Campus,London, United Kingdom; 3Department of Imaging, Imperial College London, Hammersmith Hospital, London, United Kingdom; 4Pulmonary HypertensionService, Imperial College London, Hammersmith Hospital, London, United KingdomAbstract: Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures.Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression frompulmonary arterial dilatation is rare in adults, and there are no reports in the literature of its occurrence in idiopathic pulmonary arterialhypertension. Compression in infants with congenital heart disease has been well described. We report 2 cases of tracheobronchial com-pression: first, an adult patient with idiopathic pulmonary arterial hypertension who presents with symptomatic left main bronchus com-pression, and second, an adult patient with Eisenmenger ventricular septal defect and right-sided aortic arch, with progressive intermediusand right middle lobe bronchi compression in association with enlarged pulmonary arteries.Keywords: bronchus compression, pulmonary arterial dilatation, complications.Pulm Circ 2015;5(4):723-725. DOI: 10.1086/683687.Pulmonary arterial dilatation in the setting of pulmonary hyper-tension is a common finding.1 Extrinsic compression of the leftmain coronary artery and the left recurrent laryngeal nerve due topulmonary arterial dilatation has been reported in the literature.2-5While uncommon, tracheobronchial compression due to pulmo-nary arterial dilatation is a well-known complication in infants withcomplex congenital heart disease and vascular rings.6 In absentpulmonary valve syndrome, significant symptoms in early infancyare commonly due to bronchial compression from dilated pulmo-nary arteries and an enlarged right atrium.7 In adults, respiratorysymptoms caused by tracheobronchial compression are most com-monly due to aortic aneurysms in the setting of atherosclerosis.8To our knowledge, there have been no reports of tracheobron-chial compression in patients with idiopathic pulmonary arterial hy-pertension and pulmonary arterial dilatation. We present the firstreport of an adult patient with severe idiopathic pulmonary arterialhypertension and pulmonary arterial dilatation associated with symp-tomatic left main bronchus compression. In addition, we present acase of adult congenital heart disease associated with pulmonaryarterial dilatation, with progressive, symptomatic bronchus inter-medius and right middle lobe bronchus compression.CASE DESCRIPTIONPatient A is a 73-year-old female who was diagnosed and com-menced on treatment for idiopathic pulmonary arterial hyperten-sion 11 years earlier. On diagnosis, she had a pulmonary arterialsystolic pressure of 98 mmHg, diastolic pressure of 40 mmHg,mean pulmonary artery pressure of 67 mmHg, pulmonary capillarywedge pressure of 8 mmHg, mean right atrial pressure of 10 mmHg,cardiac index of 1.92 L/min/m2, and pulmonary vascular resistanceof 1,181 dyn-s/cm5 on right heart catheterization. She was noted tohave enlarged pulmonary arteries, 1.5 times the size of the aortaon computed tomography (CT). Over time, she progressed from NewYork Heart Association (NYHA) class II to class IV symptoms.CT 10 years after diagnosis showed that the main pulmonaryartery abutted but did not compress the left main coronary artery.One year later, she noted a voice change and bovine cough, sug-gesting vocal cord dysfunction. She was admitted to the hospitalwith a lower respiratory tract infection and worsening shortness ofbreath, which only partially resolved with antibiotics and then re-curred. Because of progressive shortness of breath and sputum pro-duction, she had high-resolution CT that showed aneurysmal dilata-tion of the central pulmonary arteries. The right pulmonary arteryhad increased in size to 91 mm in diameter and was associated withleft main bronchus compression (Figs. 1, 2).Spirometry was not tolerated because of cough, and flow-volumeloops could not be recorded. Peak flow was severely reduced to anaverage of 183 L/min from 3 attempts. Her treatment includedbosentan and sildenafil. She was subsequently referred to specialistpalliative care for symptom control and has continued on medicaltherapy.Address correspondence to Dr. J. Simon R. Gibbs, Pulmonary Hypertension Service, Hammersmith Hospital, London, United Kingdom. E-mail: s.gibbs@imperial.ac.uk.Submitted March 7, 2014; Accepted May 13, 2015; Electronically published November 4, 2015.© 2015 by the Pulmonary Vascular Research Institute. All rights reserved. 2045-8932/2015/0504-0014. $15.00.This content downloaded from 155.198.008.192 on May 25, 2016 01:54:57 AMAll use subject to University of Chicago Press Terms and Conditions (http://www.journals.uchicago.edu/t-and-c).Patient B is a 63-year-old male with Eisenmenger’s complexassociated with right-sided aortic arch, chronic obstructive pulmo-nary disease, and atrial fibrillation who had a 10-year history of in-creasing shortness of breath. Severe pulmonary hypertension witha bidirectional shunt across the ventricular septal defect was con-firmed on echocardiography, with an estimated right ventricularsystolic pressure of 116 mmHg. Over time, he progressed to NYHAclass IV symptoms, and his current treatment includes warfarin,bosentan, and sildenafil.For 2 years, he has had an increasing frequency of lower respira-tory tract infections and has expectorated an eggcup full of gray-greensputum every day. CT 3 years ago showed bronchus intermediusand middle lobe bronchus compression with right middle lobe col-lapse. This progressed 2 years later with aneurysmal dilatation ofthe right pulmonary artery to 51 mm with intrinsic thrombus, oc-clusion of the right middle lobe bronchus with consequent collapseof the right middle lobe, and significant compression of the bron-chus intermedius (Fig. 3). He has continued on medical therapy.DISCUSSIONIn contrast to adults, the cartilaginous, muscular, and elastic sup-ports of the airway in infants are weak, making the tracheobron-chial tree vulnerable to compression. The left main bronchus islocated between the descending aorta and the pulmonary artery,making it susceptible to compression with dilation of these ves-sels.9,10 Acyanotic congenital heart disease including absent pulmo-nary valve syndrome and atrial septal defects causing pulmonaryarterial dilatation,9-13 right-sided aortic arch, double aortic arch, andabnormal ligamentous arteriosus insertion,6 associated with pul-monary arterial dilatation and tracheobronchial compression, havebeen reported.Pulmonary arterial dilatation is a common complication of se-vere pulmonary arterial hypertension and is not associated withincreased risk of death. Our review of the literature found 2 casesof tracheobronchial compression from dilated pulmonary arteriesin adults. Symptomatic tracheobronchial compression was reportedin an 18-year-old female with newly diagnosed absent pulmonaryvalve syndrome,14 and asymptomatic left bronchial compression wasreported in a 55-year-old male with chronic thromboembolic diseaseand congenital pulmonary stenosis with consequent pulmonary arte-rial dilatation.15 Left main coronary artery compression and leftrecurrent laryngeal nerve compression are otherwise well-reportedcomplications of pulmonary arterial dilatation.2-5Vascular tracheobronchial compression syndromes rarely pre-sent in adulthood. The most common acquired vascular tracheo-bronchial compression syndrome in adults is due to compressionfrom aortic aneurysms in the setting of either atherosclerosis orMarfan’s syndrome.8 In adults, CT is the investigation of choice andavoids invasive bronchoscopy. Flow-volume curves may show flat-tening of the expiratory portion of the curve, suggesting variableintrathoracic obstruction, and the chest x-ray may show a widenedupper mediastinum.Treatment of tracheobronchial compression in infants and chil-dren has been successful with surgical decompression6,13,16 or en-dobronchial stenting.9 In adults, patients are likely to be unsuitablefor surgical intervention. In the 2 cases previously reported in theliterature, one patient died soon after diagnosis,14 and the othermanaged with pulmonary vasodilators.15 Endobronchial stenting hasnot been performed.In conclusion, we present 2 patients with massive pulmonaryarterial dilatation causing tracheobronchial compression. Diagno-sis was made on CT, with the clinical presentation of recurrentlower respiratory tract infections in both patients. Large airway com-pression should be considered in patients with pulmonary arterialFigure 1. Contrast-enhanced computed tomography of the chestreconstructed in the coronal plane of patient A showing left mainbronchus compression (arrow) by an enlarged main pulmonaryartery.Figure 2. Contrast-enhanced computed tomography image of pa-tient A showing left main bronchus compression (arrow) by themain pulmonary artery.724 | Left main bronchus compression due to pulmonary artery dilatation Jaijee et al.This content downloaded from 155.198.008.192 on May 25, 2016 01:54:57 AMAll use subject to University of Chicago Press Terms and Conditions (http://www.journals.uchicago.edu/t-and-c).hypertension associated with markedly enlarged pulmonary arteriesas a potential cause of worsening breathlessness.Source of Support: Nil.Conflict of Interest: None declared.REFERENCES1. Badagliacca R, Poscia R, Pezzuto B, Papa S, Nona A, Mancone M,Mezzapesa M, et al. Pulmonary arterial dilatation in pulmonary hyper-tension: prevalence and prognostic relevance. Cardiology 2012;121(2):76–82.2. Heikkinen J, Milger K, Alejandre-Lafont E, Woitzik C, Litzlbauer D,Vogt J-F, Klußmann JP, Ghofrani A, Krombach GA, Tiede H.Cardiovocal syndrome (Ortner’s syndrome) associated with chronicthromboembolic pulmonary hypertension and giant pulmonary arteryaneurysm: case report and review of the literature. Case Rep Med 2012;2012:230736.3. Andjelkovic K, Kalimanovska-Ostric D, Djukic M, Vukcevic V,Menkovic N, Mehmedbegovic Z, Topalovic M, Tesic M. Two rare con-ditions in an Eisenmenger patient: left main coronary artery compres-sion and Ortner’s syndrome due to pulmonary artery dilatation. HeartLung 2013;42(5):382–386.4. Fujiwara K, Naito Y, Higashiue S, Takagaki Y, Goto Y, Okamoto M,Yoshida S, Sekii H, Tomobuchi Y. Left main coronary trunk compres-sion by dilated main pulmonary artery in atrial septal defect. Reportof three cases. J Thorac Cardiovasc Surg 1992;104:449–452.5. Lee J, Kwon HM, Hong BK, Kim HK, Kwon KW, Kim JY, Lee KJ,et al. Total occlusion of left main coronary artery by dilated mainpulmonary artery in a patients with severe pulmonary hypertension.Korean J Intern Med 2001;16:265–269.6. Bove T, Demanet H, Casmir G, Viart P, Goldstein J, Deuvaert F.Tracheobronchial compression of vascular origin: review of experiencein infants and children. J Cardiovasc Surg 2001;42:663–666.7. Pinsky W, Nihill M, Mullins C, Harrison G, McNamara D. The ab-sent pulmonary valve syndrome. Considerations of management. Cir-culation 1978;57:159–162.8. Kanabuchi K, Noguchi N, Kondo T. Vascular tracheobronchial compres-sion syndrome in adults: a review. Tokai J Exp Clin Med 2011;36:106–111.9. Saygili A, Aytekin C, Boyvat F, Barutcu O, Mercan S, Tokel K. Endo-bronchial stenting in a two-month-old infant with bronchial compres-sion secondary to tetralogy of Fallot and absent pulmonary valve. TurkJ Pediatr 2004;46:268–271.10. Berlinger N, Long CS, Foker J, Lucas RJ. Tracheobronchial compres-sion in acynoatic congenital heart disease. Ann Otol Rhinol Laryngol1983;92:387–390.11. Corno A, Picardo S, Ballerini L, Gugliantini P, Marcelletti C. Bronchialcompression by dilated pulmonary artery. Surgical treatment. J ThoracCardiovasc Surg 1985;90:706–710.12. Knauth AL, Marshall AC, Geva T, Jonas RA, Marx GR. Respiratorysymptoms secondary to aortopulmonary collateral vessels in tetralogy ofFallot absent pulmonary valve syndrome. Am J Cardiol 2004;93(4):503–505.13. Talwar S, Sharma P, Choudhary SK, Kothari SS, Gulati GS, Airan B.Le-Compte’s maneuver for relief of bronchial compression in atrial septaldefect. J Cardiac Surg 2011;26(1):111–113.14. Lanjewar C, Shiradkar S, Agrawal A, Mishra N, Kerkar P. Aneurysmallydilated major aorto-pulmonary collateral in tetralogy of Fallot. IndianHeart J 2012;64(2):196–197.15. Morjaria S, Grinnan D, Voelkel NF. Massive dilatation of the pulmo-nary artery in association with pulmonic stenosis and pulmonary hy-pertension. Pulm Circ 2012;2(2):256–257.16. Sebening C, Jakob H, Tochtermann U, Lange R, Vahl C, Bodegom P,Szabo G, et al. Vascular tracheobronchial compression syndromes—experience in surgical treatment and literature review. Thorac Cardio-vasc Surg 2000;48(3):164–174.Figure 3. Contrast-enhanced computed tomography reconstructedin the coronal plane of patient B showing right bronchus com-pression (arrow).Pulmonary Circulation Volume 5 Number 4 December 2015 | 725This content downloaded from 155.198.008.192 on May 25, 2016 01:54:57 AMAll use subject to University of Chicago Press Terms and Conditions (http://www.journals.uchicago.edu/t-and-c).

Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: two case reports

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Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: two case reports

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