(Circulation. 1996;93:1417-1423.)
© 1996 American Heart Association, Inc.
Articles |
Isolated Peripheral Pulmonary Artery Stenoses in the Adult
From the Boston (Mass) Adult Congenital Heart Service, Department of Cardiology, Children's Hospital and Division of Cardiology, Brigham and Women's Hospital, Departments of Cardiology (J.K., M.J.L., T.J.P., J.E.L.), Radiology (V.S.M., S.T.T.), and Pathology (L.M.R.).
Correspondence to Michael J. Landzberg, MD, Boston Adult Congenital Heart Disease Service, Department of Cardiology, Children's Hospital, 300 Longwood Ave, Boston MA 02115.
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Abstract |
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Background Isolated peripheral pulmonary artery stenosis (PPS) in the adult is rare and frequently unsuspected. We review in this article our experience with 12 adult patients with isolated PPS, half of whom had been previously diagnosed with chronic pulmonary thromboembolic disease.
Methods and Results The presentation,
evolution, and management of 12 adults with isolated PPS, 17 to 51
years of age (mean, 36.2±9.7 years), were evaluated. Presenting
symptoms were dyspnea and fatigue. Three patients had New York Heart
Association (NYHA) functional class III or greater. Lung perfusion
scans revealed multiple segmental abnormalities in flow distribution in
all patients. Oxygen desaturation at rest was present in 4
patients. At catheterization, right
ventricular (RV) pressure was suprasystemic in 2 patients,
systemic in 1, and more than half-systemic in 7. All had multiple
bilateral nonuniform stenoses in segmental and subsegmental
arteries. Balloon pulmonary angioplasty (BPA) to decrease RV
hypertension and improve pulmonary flow distribution was
performed in 11 patients. After BPA, vessel diameter increased >50%
in 10 patients, distal pulmonary artery pressure increased
30% in 6, and RV pressure decreased >30% in 5. One patient died
shortly after BPA as a result of pulmonary hemorrhage.
Immediate procedural success was achieved in 9 of 11 patients. At a
mean follow-up period of 52±32 months, 7 patients had sustained
symptomatic improvement (NYHA class I-II).
Conclusions We describe a severe syndrome of isolated PPS in the adult that mimics chronic pulmonary thromboembolic disease. Pulmonary hemodynamics and angiography are required for definitive diagnosis. BPA may offer these patients successful short-term reduction in RV hypertension and alleviation of symptomatology.
Key Words: balloon • arteries • pathology
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Introduction |
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Isolated peripheral pulmonary artery stenosis (PPS) in children has been well described.1 2 3 4 It is characterized by diffuse arterial involvement generally affecting the main and lobar arteries and occasionally produces symptoms of right ventricular (RV) failure when severe. PPS often is associated with a generalized congenital syndrome,2 3 4 5 6 7 8 9 10 11 12 13 including Williams syndrome. Balloon dilation of PPS either in isolation or with other forms of congenital heart disease is a common procedure at our institution.14 More than 900 balloon pulmonary angioplasties (BPAs) have been performed at Boston Children's Hospital since 1984.
In contrast to children with PPS, the first adult referred for balloon angioplasty in 1986 presented with dyspnea on exertion, cyanosis, and a presumptive diagnosis of thromboembolic disease. Since then, we have encountered 11 similar patients, many of whom were receiving therapy for chronic pulmonary thromboembolism without improvement. In this article, we review our experience with isolated PPS in these 12 patients, who presented over the age of 17 years, to better define the nature, presentation, and management of this underrecognized syndrome.
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Methods |
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Patient Population
All patients
17 years of age with isolated multifocal PPS
evaluated at Boston Children's Hospital as identified by review of the
Department of Cardiology patient database between July
1984 and January 1994 were considered for review. Patients with central
pulmonary artery hypoplasia or whose PPS either was associated
with other structural heart disease or was part of a systemic
congenital syndrome were excluded. Patient records—especially
medical histories, radionuclear scans, hemodynamics,
cineangiograms, and pathological specimens—were
reviewed.
Cardiac Catheterization and BPA
All patients underwent hemodynamic assessment of
the right and left sides of the heart. Special attention was devoted to
obtaining accurate systolic, diastolic, and mean
pressures in each pulmonary segment. As reported
previously,15 intravascular ultrasound was performed when
available. Selective right and left pulmonary arteriographies
were performed by standard techniques16 with a 7F Berman
angiographic catheter or a 7F pigtail with a cut end over a guiding
wire. The techniques and indications for BPA were described
earlier.14 17 18 19 20 21 BPA was considered only if the following
criteria were met: (1) RV systolic pressure >50% systemic
pressure or RV pressure <50% systemic pressure with either
accompanying RV dysfunction or dyspnea accountable by a marked
radionuclear perfusion inequality of pulmonary blood flow, (2)
low distal pulmonary artery pressures in the affected
pulmonary lobar arteries and elevated pressures in the less
affected ones, and (3) marked abnormality in the radionuclear perfusion
of affected areas. Lower lobe and more centrally located
stenoses were favored for initial dilations to minimize
potential ventilation perfusion abnormalities and reduce the risk of
postdilation high-flow pulmonary edema.22
BPA procedural success was defined as an increase in vessel diameter
50% with improvement of angiographic flow in the dilated
pulmonary artery segment (angiographic success) and/or a
decrease in RV systolic pressure by 30% in addition to
initial symptomatic improvement.
Data Collection and Follow-up
Data were collected retrospectively at Children's Hospital.
Permission for record review was obtained from the Institutional
Review Board.
Statistical Analysis
Significance of univariate analysis was
tested with Student's t test. A value of P<.05
was considered significant. Results are expressed as mean±SD.
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Results |
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Patient Characteristics
Between July 1986 and November 1993, 12 adult patients (7 women, 5 men) were evaluated for the diagnosis and treatment of isolated PPS (Table 1
). The median age at initial evaluation by a
cardiologist was 20 years (range, 3 months to 38 years); 4 patients had
asymptomatic heart murmurs. One patient underwent an
elective postoperative catheterization after a patent
ductus arteriosus ligation, which revealed PPS unrelated to the ductal
site.
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Dyspnea and fatigue on exertion were universal, with onset of symptomatology at 29.2 years of age (range, 16 to 49 years). Other symptoms included exercise-induced chest pain in 1 patient and palpitations without documented arrhythmia in another. Two patients had findings of RV failure, including hepatomegaly and peripheral edema.
Radionuclide perfusion scintigraphy
(99Tc–macroaggregated albumin)
demonstrated bilateral segmental and subsegmental defects in all
patients (Fig 1). In 6 patients, these findings, in
combination with suspected pulmonary hypertension, led to the
diagnosis of chronic pulmonary thromboembolism and institution
of anticoagulation therapy, with no improvement and progressive
clinical deterioration. All patients had undergone
diagnostic pulmonary angiography before referral to
our institution for possible BPA.
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No patient in this study had any evidence of systemic vasculitis or other organ involvement.
Cardiac Catheterization
At catheterization, RV pressure was found to be
50% systemic pressure in 11 of the 12 patients; RV pressure was 40%
systemic in 1 patient with significantly reduced lobar
pulmonary blood flow (Table 2). One patient with
RV pressure equal to 200% of the systemic level had elevated distal
pressures in all the pulmonary artery segments entered. All
other patients had low pulmonary artery pressure distal to the
stenoses in the affected segments. All patients had elevated
distal pulmonary artery pressure in less stenotic
segments. Angiography revealed bilateral, diffuse
peripheral pulmonary artery narrowings without
central pulmonary artery hypoplasia in all patients (Fig 2). Luminal tapering and obliteration of subsegmental
pulmonary arteries were seen in 7 patients. One patient with
diffuse luminal narrowings had a single filling defect in a left lower
lobe segmental pulmonary artery. No other evidence of emboli or
in situ thrombosis was present.
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Intravascular Ultrasound
Intravascular ultrasonography was performed in 2 patients (Figs 3 and 4). In both instances, nonuniform
and diffuse luminal narrowings were observed. Marked medial thickening
encroaching on the vessel lumen, with maintenance of total
vessel diameter, accounted for the obstructions. These narrowings were
interposed between pulmonary artery segments with normal
ultrasonographic characteristics. After BPA, intravascular ultrasound
demonstrated increase in vessel luminal diameter and an intimal tear
(Fig 3).
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Balloon Pulmonary Angioplasty
On one or more occasions, BPA was performed in all 11 patients who
had low distal pressures in affected arterial segments
(Table 2
). The remaining patient had severely elevated RV, proximal
pulmonary artery (200% systemic pressure), and distal
pulmonary artery pressures and subsequently was listed for
organ transplantation.
Both high- and low-pressure balloons were used to dilate multiple
pulmonary artery segments. Immediate procedural success was
achieved in 9 patients. Ten patients demonstrated angiographic success,
with a mean vessel diameter increase of >50% of predilation diameter,
changing from 2.3±1 to 5.2±2.3 mm (P<.001). Segmental
perfusion to the dilated area was improved in 6 of 8 patients who
underwent immediate postprocedural radionuclear lung scanning. The
systolic RV pressure decreased 30% in 5 patients. The mean
distal pulmonary artery pressure increased by >30% of
predilation pressure in 6 patients. Six patients had
radiographic evidence of pulmonary edema in a
dilated lung segment, as described previously.22 Two
patients had transient self-limited hemoptysis. Early in our
experience with the BPA technique, 1 patient with an initial systemic
level of RV pressure underwent multiple high-pressure balloon
dilations and developed acute pulmonary edema and
hemorrhage in the anterior segment of the right upper lobe
associated with a distal pulmonary artery perforation, which
initially seemed well controlled. However, in the evening after the
procedure, the patient expired suddenly with massive
hemorrhage. A second patient underwent multiple attempts at
high-pressure balloon dilations without elimination of the balloon
"waist," an increase in vessel diameter, or an increase in distal
flow and pressures. Having failed balloon dilation, the patient was
listed for organ transplantation.
Pathology
The postmortem examination of available affected tissues revealed
marked RV hypertrophy and chamber enlargement. The proximal
pulmonary arterial wall was diffusely thickened.
Multiple hardened stenotic areas with marked luminal narrowing
were seen in the branch pulmonary arteries in a bilateral,
diffuse, and nonuniform pattern, with poststenotic dilated
and thin-walled distal vessels. Microscopic examination of main and
proximal branch pulmonary arteries revealed typical changes of
pulmonary hypertension, as previously
described.1 23 24 Luminal encroachment by a marked
increase in smooth muscle cells and disorganized elastic fibers within
the media was noted in the affected stenotic segments (Figs 5 and 6). There was no evidence of either
pulmonary emboli or in situ thrombosis. The pathological
findings were consistent with the angiographic results
described above.
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Follow-up
Of the 11 survivors after catheterization and/or
BPA, 2 patients with recalcitrant symptoms of dyspnea with minimal
exertion and suprasystemic RV pressures were listed for organ
transplantation. One of them, who had not undergone BPA, died suddenly
18 months later.
Of the remaining 9 patients, symptomatic improvement was sustained at the most recent follow-up (mean follow-up period, 52±32 months; range, 18 to 104 months) in 7 patients: 3 are currently asymptomatic, and 4 have dyspnea with moderate exertion. Two patients with initial symptomatic improvement subsequently presented with increased symptomatology and dyspnea with mild exertion 2 and 4 years after BPA, respectively. Repeated cardiac catheterization is anticipated.
Echocardiographic estimation of RV pressure revealed
values of 
50% systemic levels in 6 of 8 patients at the latest
follow-up.
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Discussion |
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TopAbstractIntroductionMethodsResultsDiscussionReferences |
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Childhood PPS has been well described,2 3 4 16 25 occurring as (1) single or multiple stenoses confined to the trunk or main pulmonary artery branches, (2) multiple peripheral stenoses involving segmental pulmonary branches, or (3) central and peripheral stenoses. These lesions are frequently seen in association with other congenital heart defects4 26 or as part of well-characterized syndromes (Williams, Alagille's, Noonan's, congenital rubella, cutis laxa, Ehlers-Danlos, and Silver's).5 6 7 8 9 10 11 12 13 The hemodynamic effects of these narrowings may be mild. Noncardiac comorbid illness often determines the course and prognosis in many of these children. In general, patients are asymptomatic unless they have suprasystemic RV pressures or associated cardiac disease. Dyspnea on exertion is rare in children.
Our experience with PPS in the adult started in 1986, and by 1993 we had encountered 9 such patients.27 Prior reports had noted nondiffuse lesions of an unclear or acquired origin or diffuse lesions associated with central pulmonary artery hypoplasia.28 29 30 31 32 33 34 35 Patients with systemic vasculitis, such as Behçet's disease or Takayasu's arteritis, have been reported to have pulmonary arterial involvement, which can be associated with segmental radionuclear lung perfusion defects. Reports of such patients with systemic vasculitis,36 37 38 39 40 41 and recent case reports of two adult patients similar to the patients in our series with diffuse PPS without systemic disease or central PA hypoplasia42 43 highlight the need for recognition of a different syndrome of PPS presenting in adulthood.
Diagnosis and Therapy
We have described our experience with a clinically unrelenting
syndrome of isolated diffuse peripheral pulmonary
artery stenosis in 12 adult patients. This syndrome differed
from the more common pediatric presentation in two aspects:
Symptoms of progressive dyspnea and fatigue were severe and universal,
and all patients had segmental radionuclear lung perfusion defects
unmatched to ventilation abnormalities. Catheterization
revealed only moderate RV hypertension but diffuse, yet nonuniform,
peripheral pulmonary stenoses. There was
evidence of different degrees of severity, ranging from complete
obliteration of segmental vessels to mild stenosis. Therapy
with BPA was designed to normalize pulmonary flow abnormalities
(rather than reduce RV pressure) and has achieved short-term
clinical stability in 8 of 10 of these patients.
In patients with progressive dyspnea and elevation of pulmonary arterial pressure, the finding of unmatched segmental lung perfusion defects has frequently led to the diagnosis of subacute or chronic thromboembolic disease and institution of systemic anticoagulation.44 45 46 All the patients in our series met this criterion; 6 patients were incorrectly diagnosed and treated for chronic thromboembolic disease. Careful pulmonary hemodynamics and arteriography often are deferred until the onset of relentless symptomatology or when thromboembolectomy or organ transplantation is being considered. Systemic vasculitis with pulmonary arterial involvement should be ruled out in all such patients, and the potential for isolated PPS should be evaluated. The recognition of isolated PPS in the adult as an alternative syndrome, with a vastly different yet frequently successful therapy, that presents similarly and yields identical results on noninvasive perfusion scanning radically changes the diagnostic approach and should prompt earlier consideration of cardiac catheterization.
Origin and Pathology
The pulmonary arteries are derived from the embryological
bulbus cordis and truncus arteriosus (main trunk), as well as from the
ventral portions of the sixth branchial arches and the postbranchial
pulmonary vascular plexus (branch pulmonary
arteries).47 Childhood PPS has been noted in neonatal
life,3 and its coincidence with other embryological
malformations implicates a congenital origin. Because 6 of 12 adults
with isolated PPS in this series had murmurs consistent with
PPS in their childhood or adolescent years, it seems likely that some
patients have a congenital disorder characterized by slow, nonuniform
progression of areas of vascular obstruction. However, we cannot rule
out the possibility of a postnatally acquired cause of this
syndrome.
Postmortem studies of children with PPS3 48 have revealed primarily fibrous intimal proliferation with various degrees of accompanying medial hypoplasia and loss of elastic fibers in affected vascular segments. In the 1 patient in our series who came to postmortem examination, similar intimal proliferation was noted in stenosed segments, although this was accompanied by marked medial thickening with increased and disorganized elastic fibers. The pathological differences between childhood and adult forms of PPS may represent either different pathogenic mechanisms or a similar cause compounded by years of increased proximal intraluminal pressure. The histological findings in our series, in addition to the lack of microscopic evidence of vessel thromboembolism, emphasize the difference between isolated PPS in the adult and pulmonary thromboembolic disease. Postmortem studies in patients with systemic vasculitis and pulmonary involvement have demonstrated evidence of chronic inflammatory changes and various degrees of scarring, complicated at times by the presence of thrombi in the vascular wall. These findings differ from those in our study in that there were no other arterial involvement, no evidence of chronic inflammatory disease in any organ, and no inflammation at microscopy.
Summary
We have described 12 adults with an underrecognized form of
isolated PPS, most of whom have benefited from BPA.
Given the similarities in presentation between patients with isolated PPS and those with chronic thromboembolic disease44 45 46 49 and the marked differences in therapy for these disease processes, careful pulmonary hemodynamics and arteriography should be seriously considered in the evaluation of all adult patients presenting with insidious onset of dyspnea, fatigue, and diffuse segmental radionuclear lung perfusion mismatches. BPA may offer successful intermediate improvement of flow distribution, a short-term reduction in RV hypertension, and alleviation of symptomatology.
Received July 5, 1995; revision received October 5, 1995; accepted October 31, 1995.
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