Safety and Clinical Outcomes of Endovascular Treatment of Adult-onset Pulmonary Artery Stenosis

Mujeeb A. Sheikh, MD; Mohammed A. Chowdhury, MD, MRCP (UK); George V. Moukarbel, MD

J Invasive Cardiol. 2016;28(5):202-208.

Abstract and Introduction

Abstract

Background. Pulmonary artery stenosis (PAS) in adults is a rare condition. The role of endovascular stent implantation as a therapeutic option has not been clearly defined.

Methods and Results. We performed a retrospective review of all cases of adult-onset PAS published in Pubmed/Medline from 1990 to 2013. A total of 126 cases of adult-onset PAS were identified, out of which 46 cases (37%) were treated with an endovascular intervention. Symptomatic improvement was reported in 98% of cases. Average translesional gradient at baseline was 47 ± 20 mm Hg, which reduced to 8 ± 11 mm Hg after stenting (P<.001). Mean preprocedural pulmonary systolic artery pressures were 79 ± 26 mm Hg, which reduced post procedure to 50 ± 20 mm Hg (P=.02). There were no immediate adverse events reported related to procedure. In-stent restenosis was reported in 7 cases on follow-up.

Conclusion. Endovascular treatment appears safe and effective for symptom relief in adult-onset PAS and should be considered as an alternative treatment option in patients with prohibitive surgical risk.

Introduction

Pulmonary artery stenosis (PAS), particularly branch stenosis, is one of the most common forms of vascular stenosis associated with congenital heart disease.^[1,2] There has been extensive experience with pulmonary artery stenting in children, demonstrating significant improvement in symptoms and improved outcomes.^[3–5] In contrast to pediatric PAS, adult-onset PAS is rare and has been associated with a wide variety of clinical conditions including fibrosing mediastinitis, lung parenchymal tumors, primary vascular tumors, and lung transplantation. PAS in these cases results from extrinsic compression or direct vessel involvement from inflammatory or neoplastic process in the adjacent structures. Reported clinical manifestations of PAS are variable and range from dyspnea due to inadequate pulmonary perfusion to right heart failure from chronic pressure overload. Multiple published anecdotal cases and case series have documented efficacy of endovascular stent implantation in the treatment of symptomatic adult-onset PAS. We therefore reviewed the published literature on adult-onset PAS treated with endovascular approach and provide a comprehensive report on the etiology, clinical presentation, diagnostic modalities, procedural details, and outcome of these patients.

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Abstract and Introduction
Methods
Results
Discussion
Conclusion
References

Table 1. Characteristics and outcomes of cases of PAS treated with stenting, grouped according to etiology.
Case Number/Study Author	Etiology of PAS	Findings	Stent Size (mm) and Type	Outcome	Complication
Fibrosing mediastinitis: Infectious causes
(1) Gustafson et al¹⁰	Histoplasma capsulatum	B/L branch PA stenosis	23: Palmaz Genesis (Cordis)	Symptoms improved	Complete occlusion of left PA stent after 6 months
(2) Smith et al¹¹	Histoplasma capsulatum	Severe right PA stenosis	N/A	Symptoms & gradient improved	None
(3) Satpathy et al¹²	Histoplasma capsulatum	Rt PA compression	N/A	Symptoms & gradient improved	None
(4) Thiessen et al¹³	Histoplasma capsulatum	Rt PA narrowing	30 x 12: Palmaz-Schatz stent (Cordis)	Symptoms & gradient improved	65% intrastent stenosis at 6 months
(5) Kandzari et al¹⁴	Histoplasma capsulatum	Severe right PA stenosis	12 x 40: 308 Palmaz stent (JJIS)	V/Q: right lower lobe perfusion, gradient improved	None
(6) Ferguson et al¹⁵	Histoplasma capsulatum	Complete occlusion of SVC, LUPV, severe stenosis of RUPV, RPA	29: Genesis stent (Cordis) followed by a Palmaz 308 stent (Cordis)	Symptom improved	Rt upper PA occlusion due to jailing by the distal stent
Fibrosing mediastinitis: Non-infectious causes
(7) Fontaine et al¹⁶	Idiopathic	Rt PA in-stent restenosis	10 x 40: 308 Palmaz stent (JJIS)	Symptoms & gradient improved	None
(8) Fontaine et al¹⁶	Idiopathic	Rt PA occlusion	10 x 40: Palmaz 424 long medium stent (JJIS)	Symptoms & gradient improved	None
(9) Fontaine et al¹⁶	Idiopathic	Severe B/L stenosis of proximal PA	12 x 40: 308 Palmaz stent (JJIS)	Symptoms & gradient improved	None
(10) Guerrero et al¹⁷	Idiopathic	95% stenosis of Rt PA	Palmaz 424 medium catheter (Cordis/Johnson and Johnson)	Symptoms improved	None
(11) Murphy et al¹⁸	Idiopathic	Severe B/L proximal PA stenosis	45: 8 Zig CP (NuMED)	Symptoms & gradient improved	Lung reperfusion injury
(12) Doyle et al¹⁹	Idiopathic	Complete occlusion of Rt PA	P308 Palmaz stents (Johnson and Johnson)	Symptoms improved	None
(13) Doyle et al¹⁹	Idiopathic	Occlusion of Rt PA, stenosis of Rt lower pulmonary vein	P308 Palmaz stents (Johnson and Johnson); 2 in Rt PA and 1 in Rt PV	Symptoms improved	Complete occlusion of Rt PA despite anticoagulation in 1 month
(14) Ferguson et al¹⁵	Sarcoidosis	Rt PA stenosis	10: Genesis stent (Cordis)	Symptoms & gradient improved	PVR increased to 11 in 6 weeks; started on sildenafil
(15) Ferguson et al¹⁵	Idiopathic	RPA stenosis, thrombus at SVC-Rt atrial junction	29: Intrastent double-strut LD (ev3)	Hemodynamically improved	Progressive RPA stenosis proximal to stent, all stents had in-stent stenosis in 1 yr
(16) Hamilton-Craig et al²⁰	Sarcoidosis	Severe narrowing of left main PA and origin of right PA due to extrinsic compression	8x17: Express stent in Rt middle lobe artery	Symptoms & gradient improved discharged with oral anticoagulation	None
(17) Hamilton-Craig et al²⁰	Sarcoidosis	Mediastinal artrerial compression	N/A	Symptoms improved	In-stent restenosis at 1 year
Anastomotic stenosis post lung transplantations
(18) Banerjee et al²¹	Interstital pnemonitis	Severe stricturing of pulmonary artery at anastomatic site; left main PA	8 x 20: Cheatham platinum stent	Symptoms improved	None
(19) Ferretti et al²²	Idiopathic pulmonary fibrosis	Anastomotic stenosis associated with kinking of donor PA	17 x 40: Strecker balloon-expandable stent (Boston Scientific)	Weaned from ventilator Symptoms & gradient improved	None
(20) Waurick et al²³	Primary pulmonary hypertension	Stenosis in the region of vessel anastomosis	20 x 40: Johnson & Johnson stent	Symptoms & gradient improved	None
(21) Gaubert et al²⁴	Cystic fibrosis	Subtotal stenosis at anastomotic site of left pulmonary artery	35 x 14: Self-expanding Wallstent (Schneider)	Symptoms improved Pt died 15 months after stent due to cerebellar hematoma	None
(22) Chen et al²⁵	Idiopathic pulmonary fibrosis	Severe stenosis distal to the left PA anastomosis	6 x 18: Palmaz Genesis (Johnson & Johnson	Weaned off ECMO	None
(23) Lumsden et al²⁶	End-stage lung disease	Stenosis of Lt PA stent	16: Self-expanding Wallstent (Schneider)	Symptoms improved	None
(24) Grubstein et al²⁷	Pulmonary fibrosis	Stenosis of Rt PA	CP stent (NuMED)	Hypoxia and graft perfusion improved	Postprocedure hemothorax requiring drainage
(25) Grubstein et al²⁷	Emphysema	Stenosis of Lt PA	CP stent (NuMED)	hypoxia and graft perfusion improved; died from chronic graft rejection 2 years post procedure without any graft restenosis	None
(26) Grubstein et al²⁷	Emphysema	Stenosis of Lt PA	CP stent (NuMED)	Hypoxia and graft perfusion improved	None
(27) Grubstein et al²⁷	Sarcoidosis	Stenosis of Rt PA	CP stent (NuMED)	Died from graft rejection 2 months post angioplasty + mycobacterium abscesses
(28) Grubstein et al²⁷	Pulmonary fibrosis	Stenosis of Rt PA	Jomed (Jomed International AB)	Lung function improved	None
(29) Najafizadeh et al²⁸	Pulmonary fibrosis	Stenosis of Rt PA	N/A	Symptoms & gradient improved	None
Extrinsic malignancy/Tumors
(30) Fierro Renoy et al²⁹	Lymphadenopathy	Rt PA 73% stenosis; Lt PA 80% stenosis	8 x 29: Corinthian stent (Cordis)	Symptoms & gradient improved	None
(31) Desai et al³⁰	Thymoma	90% stenosis of Rt PA	10 x 40: Wallstent (Boston Scientific) Smart stent (Cordis)	Symptoms improved; pt died 2 years after stent due to cancer	None
(32) Asato et al³¹	Lung cancer	Mass in mediastinum, occlusion of left innominate vein, Rt PA severe stenosis	16 x 45: Easy Wallstent (Boston Scientific) self-expandable endoprosthesis	Symptoms & gradient improved	None
(33) Balkin et al³²	Sarcoma	Tumor extending into proximal Rt PA and into the origins of Lt PA	16 x 60: Wallstent (Schneider)	Symptoms & gradient improved Pt died 8 weeks after stent due to cancer	None
(34) Misselt et al³³	Small cell	Lt PA: 90% stenosis Rt PA: 70% stenosis	10 x 57: Express Biliary LD (Boston Scientific)	Symptoms improved	None
(35) Hirota et al³⁴	Tumor	Rt & Lt PA trunk stenosis, Rt PA 4.5 cm stenosis	5 x 10: Wallstent (Boston Scientific)	Symptoms & gradient improved Pt died 1 month after stent due to cancer	None
(36) Gutzeit et al³⁵	Non-small cell	Lt PA stenosis (6 cm)	10 x 68: SelfX (Abbott)	Symptoms improved	None
(37) Bydawell et al³⁶	Lymphadenopathy	Rt main artery occlusion	E-Luminexx (C.R. Bard)	Symptoms improved	None
(38) Bydawell et al³⁶	Lymphadenopathy	Rt middle lobe compression	8 x 30: E-Luminexx (C.R. Bard)	Symptoms improved	None
(39) Chan et al³⁷	Tumor	Distal pulmonary trunk, Rt PA	12 x 80: Wallstent (Schneider)	Symptoms & gradient improved	None
(40) Vawdrey et al³⁸	Bronchial carcinoid	Rt PA stenosis	18 x 60 mm: Sinus XL stent (Optimed)	Symptoms & gradient improved	None
Intrinsic malignancy/Tumors
(41) Smith CA et al³⁹	Bronchial adenoid cystic CA	Rt PA stenosis	39: Palmaz Genesis stent (Cordis)	Symptomatic improvement; died several months later due to metastases within 9 months	Stent dislodgment
(42) Meckel et al⁴⁰	Leiomyosarcoma	Mass growing onto vessel wall from bifurcation of pulmonary trunk into both main PAs	14 x 60: Self-expanding nitinol stent (Symphony; Boston Scientific/Meditech)	Postoperative deep vein thrombosis Symptoms & gradient improved
(43) Mendiz et al⁴¹	Sarcoma	Critical stenosis of pulmonary trunk by mass involving ostium of both PAs	24 x 60: Sinus XL self-expandable nitinol stent (Sinus XL; OptiMed)	Symptoms & gradient improved	None
(44) Totaro et al⁴²	Angiosarcoma	Main PA severe narrowing	25x30: Palmaz stent (Cordis)	Symptoms & gradient improved	None
(45) Heid et al⁴³	Sarcoma	Rt main PA		Stenosis improved	None
(46) Bruhl et al⁴⁴	Radiation-small cell	Rt main PA 90% stenosis with post-stenotic dilation	17: Palmaz XL stent (Cordis)	Symptoms & gradient improved	None

PA = pulmonary artery. Lt = left; Rt = right.

Table 2. Summary of results.
Adult-onset pulmonary artery stenosis (n = 126 cases)	46 cases underwent endovascular intervention
Adult-onset pulmonary artery stenosis (n = 126 cases)	80 cases were treated with standard modalities of treatment
Endovascular intervention	Balloon angioplasty + stent used in 39% of cases
Endovascular intervention	Direct stenting without prior angioplasty used in 61% of cases
Endovascular stents	Balloon-expandable stents used in 74% of cases
Endovascular stents	Self-expanding stents used in 26% of cases
Intervention outcomes
Preprocedure	Average translesional gradient: 47 ± 20 mm Hg Mean pulmonary systolic artery pressure: 79 ± 26 mm Hg
Postprocedure	Average translesional gradient: 8 ± 11 mm Hg (P<.001) Average reduction: 82 ± 22% Mean pulmonary systolic artery pressure: 50 ± 20 mm Hg (P=.02) Average reduction: 36 ± 15% Symptomatic improvement in 98% of cases
Complications	Immediate procedural complications: 3 cases (7%) Long-term complications/in-stent restenosis: 7 cases (24%)