Showing papers on "Stenosis published in 1984"

Thrombosis and Acute Coronary-Artery Lesions in Sudden Cardiac Ischemic Death

Abstract: The nature of the pathologic lesion in sudden cardiac ischemic death is in dispute. Among 100 subjects who died of ischemic heart disease in less than six hours, coronary thrombi were found in 74. There was no difference in incidence between those who died in less than 15 minutes, those who died in 15 to 60 minutes, and those who died after one hour. Among 26 cases without an intraluminal thrombus, plaque fissuring was found in 21; thus, in only 5 cases was no acute arterial lesion demonstrated. No intraluminal thrombi were found in age-matched controls. Forty-eight of the 74 thrombi were found at sites of preexisting high-grade stenosis; 14 were found at points of previous stenosis of less than 50 per cent of the diameter of the lumen. Forty-seven per cent of the thrombi were found in the right coronary artery. Only 30 per cent were found in the left anterior descending coronary artery. The pathologic process in sudden ischemic death involves a rapidly evolving coronary-artery lesion in which plaque fissuring and resultant thrombus formation are present. These findings have implications for the prevention of sudden cardiac death by antithrombotic therapy.

Does visual interpretation of the coronary arteriogram predict the physiologic importance of a coronary stenosis

Abstract: To assess visual interpretation of the coronary arteriogram as a means of predicting the physiologic effects of coronary obstructions in human beings, we compared caliper measurements of the degree of coronary stenosis with the reactive hyperemic response of coronary flow velocity studied with a Doppler technique at operation, after 20 seconds of coronary arterial occlusion. In 39 patients (44 vessels) with isolated, discrete coronary lesions varying in severity from 10 to 95 per cent stenosis, measurement of the percentage of stenosis from coronary angiograms was not significantly correlated (r = -0.25) with the reactive hyperemic response. Results were the same for obstructions in the left anterior descending, diagonal, and right coronary arteries. Underestimation of lesion severity occurred in 95 per cent of vessels with >60 per cent stenosis of the diameter by arteriography. Both overestimation and underestimation of lesions with <60 per cent stenosis were common. These results, together with...

Restenosis after percutaneous transluminal coronary angioplasty (PTCA): A report from the PTCA registry of the national heart, lung, and blood institute

Abstract: The results of follow-up angiography in patients from 27 clinical centers enrolled in the PTCA Registry were analyzed to evaluate restenosis after PTCA. Of 665 patients with successful PTCA, 557 (84%) had follow-up angiography (median follow-up 188 days). Restenosis, defined as an increase of at least 30% from the immediate post-PTCA stenosis to the follow-up stenosis or a loss of at least 50% of the gain achieved at PTCA, was seen in 187 patients (33.6%). The incidence of restenosis in patients who underwent follow-up angiography was highest within the first 5 months after PTCA. Restenosis was found in 56% of patients with definite or probable angina after PTCA and in 14% of patients without angina after PTCA. Twenty-four percent of patients with restenosis did not have either definite or probable angina. Multivariate analysis selected 4 factors associated with increased rate of restenosis: male sex, PTCA of bypass graft stenosis, severity of angina before PTCA and no history of MI before PTCA.

The value of lesion cross-sectional area determined by quantitative coronary angiography in assessing the physiologic significance of proximal left anterior descending coronary arterial stenoses.

Abstract: The results of previous work from this laboratory have shown a poor correlation between percent stenosis (determined visually with calipers) and the coronary reactive hyperemic response (an index of maximal coronary vasodilator capacity) determined during cardiac surgery. This study was performed to determine whether other parameters of lesion severity could predict the reactive hyperemic response and thus the hemodynamic significance of coronary stenoses in human beings. Twenty-three patients with lesions in the proximal left anterior descending coronary artery were studied. To account for differences in expected vessel size, patients with large diagonal branches (greater than one-half the diameter of the left anterior descending artery) arising before the lesion were excluded. Computer-assisted quantitative coronary angiography was used to measure percent diameter stenosis, percent area stenosis, and minimal stenosis cross-sectional area. With a pulsed Doppler velocity probe, reactive hyperemic responses were recorded after a 20 sec coronary occlusion of the left anterior descending artery at cardiac surgery before cardiopulmonary bypass and were quantified by the peak/resting velocity ratio (normal greater than 3.5:1). Percent area stenosis ranged from 7% to 54% for vessels with normal reactive hyperemic responses and from 27% to 94% for vessels with abnormal reactive hyperemic responses. With both percent diameter stenosis and percent area stenosis there was substantial overlap between vessels with normal and abnormal reactive hyperemic responses. In contrast, nine of nine vessels with normal reactive hyperemic responses had lesion minimal cross-sectional areas of greater than 3.5 mm2 and 13 of 14 vessels with abnormal reactive hyperemic responses had minimal cross-sectional areas of less than 3.5 mm2.(ABSTRACT TRUNCATED AT 250 WORDS)

Dynamic mechanisms in human coronary stenosis.

Abstract: At the clinical level, coronary stenoses frequently behave as though the obstruction to flow were variable and not as rigidly fixed as previously imagined. Pressure (energy) lost in flow through a stenosis is the primary determinant of its hemodynamic impact. Ischemic episodes occur when pressure distal to the stenosis falls below that needed to perfuse the subendocardium. Three important properties of the stenosis contribute to variation in its pressure loss. First, loss is proportional to the square of stenosis flow. Thus proper distribution of perfusion is doubly vulnerable to conditions such as exercise, anemia, or pharmacologic vasodilation, which ordinarily increase myocardial blood flow. Second, pressure loss is proportional to the inverse fourth power of minimum lumen diameter. As a result, seemingly small changes in diameter are amplified to large changes in stenosis resistance. Third, a compliant arc of normal arterial wall borders part of the lumen in the majority of coronary lesions. This extremely important morphologic feature of stenoses permits transient variation in stenosis lumen diameter in response to drugs or to variation in endogenous vasomotor activity or intraluminal pressure. Although our understanding is incomplete, many of the clinical features of coronary disease and its pharmacologic responses are explained in terms of these stenosis properties and their interaction.

Assessment of percutaneous transluminal coronary angioplasty by quantitative coronary angiography: diameter versus densitometric area measurements.

Abstract: Cineangiograms of 138 patients who underwent percutaneous transluminal coronary angioplasty (PTCA) were analyzed with a computer-based coronary angiography analysis system. The results before and after dilatation are presented. In a first study group (120 patients), the severity of the obstructive lesions derived from the automatically detected contours was evaluated in absolute terms and in percent-diameter reduction. In a second group of patients, 18 coronary lesions were selected for their extreme severity and symmetric aspect before angioplasty as assessed from multiple views. In the second group, the densitometric percent-area stenosis was used to assess the changes in cross-sectional area after PTCA and was compared with the circular percent-area stenosis computed from the diameter measurements. Before PTCA, a good agreement exists between the densitometric percent-area stenosis and the circular percent-area stenosis. After PTCA, important discrepancies between these 2 types of measurements are observed. It is suggested that these discrepancies in results after PTCA can be accounted for by asymmetric morphologic changes in luminal cross section, which cannot be assessed accurately from diameter measurements in a single-plane view.

Reflex constriction of significant coronary stenosis as a mechanism contributing to ischemic left ventricular dysfunction during isometric exercise.

Abstract: To study the mechanisms of myocardial ischemia during isometric exercise, handgrip was sustained, for 4.5 min at 25% of maximum by 11 patients with at least one significant coronary stenosis each, during cardiac catheterization. After recovery, the handgrip that was repeated with simultaneous infusion of nitroglycerin (50 micrograms over 4 min) directly into the diseased vessel. The cardiovascular response was assessed by hemodynamic and by computer-assisted measurements of stenosis. During the first handgrip test pulmonary capillary wedge pressure rose 56% (15 to 23 mm Hg; p less than .001), the heart rate-systolic pressure product rose 33% (p less than .01), and the diseased epicardial arteries constricted. Luminal area in the stenotic segment was reduced by 35% (p less than .01), resulting in a 243% increase in estimated stenotic flow resistance (30 to 103 mm Hg/ml/sec; p less than .001). During handgrip with intracoronary nitroglycerin, the pressure-rate product again increased 33%, but relative to resting control, capillary wedge pressure fell 4 mm Hg in association with a 32% increase in luminal area of the stenosis and a 28% reduction in flow resistance (all significantly different from the response to handgrip alone: p less than .001, .01, and .005, respectively). Thus, coronary vasoconstriction, not increased pressure-rate product, is the dominant mechanism for ischemic left ventricular dysfunction during isometric exercise in patients with significant coronary stenoses.

Spontaneous cervical cephalic arterial dissection and its residuum: angiographic spectrum.

Abstract: Cervical cephalic dissections are uncommon acute disruptions of the arterial wall occurring predominantly in middle-aged women. Clinically, most patients present with unilateral headache, oculosympathetic palsy, or ischemic neurologic symptoms. Usually, a single internal carotid artery, predominantly the right, is affected, but simultaneous multivessel dissections are evident in about one-third of patients. Angiographically, the appearance of the dissection varies, depending on its severity, extent, and the interval between onset and angiography. In the patients reported, the disruption was manifested initially by eccentric tapered stenosis in 47%, tapered stenosis and a dissecting aneurysm in 28%, occlusion in 18%, or a dissecting aneurysm alone in 7%. Subsequently, stenotic dissections resolved in 60%, improved in 20%, and progressed in 15%, while dissecting aneurysms diminished in half and resolved in one-fourth of patients. An angiographic residuum, temporally remote to its onset, was evident in 25% of dissections. Hence, carotid arterial dissections tend to resolve, sometimes progress, but seldom recur.

Cerebral hyperperfusion after carotid endarterectomy: a cause of cerebral hemorrhage.

Abstract: Correction of a very high grade carotid stenosis by endarterectomy in a normotensive man was followed by the development of severe unilateral head, eye, and face pain, seizures, and on the 6th day a fatal intracerebral hemorrhage. Autopsy revealed changes in the cerebral hemisphere ipsilateral to the endarterectomy that resembled the changes seen in malignant hypertension, whereas the opposite hemisphere was normal. These changes included hypercellularity and edema of arterial and arteriolar walls, with necrosis, extravasation of erythrocytes, and exudation of fibrin. We propose that the clinical and pathological features in this case were due to relative hyperperfusion of a cerebral hemisphere in which autoregulation had been impaired because of preoperative chronic hypoperfusion with chronic maximal dilatation of its blood vessels. This state of relative hyperperfusion is probably similar to the normal perfusion pressure breakthrough that occasionally occurs after the resection of cerebral arteriovenous malformations. It is similar to the breakthrough perfusion that occurs in severely hypertensive patients and results in hypertensive encephalopathy.

A new digital electronic caliper for measurement of coronary arterial stenosis: Comparison with visual estimates and computer-assisted measurements

Abstract: Visual analysis of the severity of coronary stenosis is limited by observer variability. However, more complex techniques of proved accuracy are tedious and costly. Therefore, a new digital electronic caliper (DEC) was evaluated as a potentially more accurate, rapid and less costly alternative for measuring stenosis severity. Stenosis minimum diameter (Dmin) and percent diameter reduction ( %S) were measured from the screen of the cine projector using a DEC. These measurements were compared with visual estimates (VIS) by 4 experienced angiographers and with measurements made by a computer-assisted method (QCA) of proved accuracy. In routine cineangiograms from 7 patients, 10 lesions were significant (>50%S) and 8 were mild ( 50%) coronary stenoses, and it may be applied with improved accuracy in the evaluation of cineangiograms.

Progression of atherosclerosis in coronary arteries and bypass grafts: ten years later.

Abstract: Progression of atherosclerosis in aortocoronary saphenous vein grafts is frequent and is the predominant cause of late graft closure after CABG. Only approximately 60% of grafts remain patent between 10 and 12 years after surgery. Of patent grafts, 45% show angiographie evidence of atherosclerosis between 10 and 12 years after surgery and 70% of the atherosclerotic lesions reduce the graft lumen diameter by 50% or more. Atherosclerosis of saphenous vein grafts does not appear to be related to age, sex or cigarette smoking, but is associated with abnormalities of cholesterol lipoprotein fractions. Progression of atherosclerosis in the native coronary arteries is also very significant after CABG. Progression of CAD between 10 and 12 years after surgery occurs in approximately 50% of nongrafted arteries. Between 10 and 12 years after surgery, the rate of progression of disease in nongrafted arteries is not different from that of grafted arteries with patent grafts; however, progression is more frequent in grafted arteries with occluded grafts. The rate of progression is not related to age, sex, risk factors or extent of disease at baseline coronary arteriography. Progression of preexisting stenoses is more frequent than appearance of new stenosis. Progression is related to the severity of the preexisting stenosis only in nongrafted arteries. Finally, progression is related to alterations of left ventricular function during follow-up. Because of these progressive late changes, CABG should probably remain limited to patients with incapacitating anginal symptoms or to those with severe lesions for whom surgery might enhance long-term survival, such as patients with severe left main CAD and 3-vessel CAD.

Quantification of relative coronary arterial stenosis by cinevideodensitometric analysis of coronary arteriograms.

Abstract: A computerized method for measuring relative coronary arterial stenosis by cinevideodensitometric analysis of 35 mm coronary arteriograms was developed and validated. Video images of projected coronary arteriographic frames were digitized into a 512 X 512 matrix (256 gray levels) by computer analysis that compared integrated contrast density measured over stenotic and normal arterial segments after background subtraction. Pixel density was 70 to 80 pixels/mm2 actual area. In phantom studies performed on plexiglass cylinders, cinevideodensitometric measurements correlated linearly with concentration of contrast medium (r = .99), with cross-sectional areas (r = .99) of contrast-filled cylinders 1 to 4 mm in diameter over a wide range of contrast concentrations (25% to 100%), and with relative stenosis of eccentric lesions in the cylinders (r = .99, SEE = 3.9%). In postmortem studies of patients who died after undergoing coronary arteriography, videodensitometric measurements of relative stenosis correlated highly (r = .97, SEE = 7.0%) with percentage stenosis based on actual area measurements obtained histologically with computer-assisted microscopic planimetry. Cinevideodensitometric analysis of coronary arteriograms was reproducible (r = .92, SEE = 7.7%), and interobserver variability was low (r = .99, SEE = 4.3%). In addition, videodensitometry provided comparable values for eccentric coronary lesions filmed in right anterior oblique and left anterior oblique projections (r = .99, SEE = 1.9%). Cinevideodensitometric analysis is an accurate, rapid method for quantifying the relative stenosis of eccentric coronary lesions without manual tracing of arterial borders.

Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery: the ring-sling complex.

Abstract: The persistent high mortality in infants with pulmonary artery "sling" (retrotracheal anomalous left pulmonary artery) is primarily due to the coexistence in such patients of long-segment tracheal stenosis due to complete cartilage rings. Five such patients are reported. Airway studies (by filtered high-kV radiography, bronchography, and/or CT) showed low carina, horizontal equal-length right and left mainstem bronchi, and long-segment tracheal stenosis. The length of the stenosis far exceeded the contact with the pulmonary sling. The suggested term "ring-sling complex" for such patients correctly places emphasis on detection of the tracheal malformation, which currently has no satisfactory surgical treatment.

Endoscopic laser repair of posterior glottic, subglottic and tracheal stenosis by division or micro-trapdoor flap.

Abstract: Laryngeal and tracheal stenosis have been refractory to a wide variety of treatments including dilation, stents, or have required major open operation, e.g., laryngofissure with and without skin or mucosal grafts and segmental resection with larynx release. Adequate airway even when achieved was frequently at the expense of voice quality and significant morbidity or mortality. A new highly successful endoscopic technique is described for the treatment of posterior glottic stenosis (apparent bilateral vocal cord paralysis), subglottic stenosis, and tracheal stenosis up to 1 cm thick. The procedure involves the endoscopic use of the CO2 laser, and a micro-trapdoor mucosal flap. Ninety percent of the patients in the group studied obtained an adequate airway with good voice quality and no tracheotomy was required in those patients not already having one. In 19 patients there was no mortality and essentially no morbidity.

Radiological follow-up of transluminally inserted vascular endoprostheses: an experimental study using expanding spirals.

Abstract: A technique for transluminal implantation of vascular endoprostheses was developed. Using a suitable instrument, 160 spiral-shaped prostheses of various forms and sizes were torsion-reduced in diameter and transluminally inserted under fluoroscopy in our study population consisting of 65 dogs and five calves. At the target, the spirals were enlarged and released from the carrier, whereupon they attached themselves to the vessel wall by elastic expansion. We implanted spirals into the vena cava or the thoracic and abdominal aorta, using the infrarenal aorta and the jugular or femoral vein for access. Angiography (the maximum follow-up was two years) demonstrated that the operation was reproducable and that it could be planned. Angiography also demonstrated that the position of the spiral prosthesis was stable and that the spiral did not lead to stenosis, thrombosis, or perforation, providing an adequate technique was used. The side branches of the main vessels remained patent, even with several spiral coils across their orifices. The method can be clinically implemented and lends itself to many applications in the vascular field.

Surgical Pathology of Pure Aortic Stenosis: A Study of 374 Cases

Abstract: The gross surgical pathologic features of the aortic valve were reviewed in 374 patients who had had clinically pure aortic stenosis and aortic valve replacement at our institution during the years 1965,1970,1975, and 1980. The most common cause of aortic stenosis, accounting for 46% of our cases, was calcification of a congenitally bicuspid valve. In the remainder, stenosis was produced by postinflammatory fibrocalcific disease (including rheumatic disease) in 35%, by degenerative calcification of an aging valve in 10%, and by calcification of a congenitally unicommissural valve in 6%. The cause of aortic stenosis was indeterminate in 4%. Valvular lesions included various degrees of dystrophic calcification, commissural fusion, and cuspid fibrosis. Calcification tended to occur more extensively and at a younger age in men than in women. Furthermore, it tended to produce stenosis and to necessitate valve replacement earliest in patients with unicommissural valves (mean age, 48 years), later in those with bicuspid or postinflammatory valves (mean age, 59 and 60 years, respectively), and latest in those with degenerative stenosis (mean age, 72 years). In our study, the relative incidence of postinflammatory aortic stenosis remained unchanged from 1965 to 1980, despite the steadily decreasing incidence of acute rheumatic fever reported in western countries. Our data suggest that (1) the incidence of chronic rheumatic heart disease has not yet begun to decrease appreciably, (2) many episodes of acute rheumatic fever may be subclinical, or (3) some forms of nonrheumatic aortic valve disease may produce gross alterations indistinguishable from those of classic chronic rheumatic valvulitis.

Salvage of an ischemic limb by laser angioplasty: Description of a New Technique

Abstract: A 62-year-old male with severe claudication and rest pain of the left leg resulting from a totally occluded superficial femoral artery and a 95 % stenosis of the deep femoral artery was treated with laser angioplasty after attempts at surgical revascularization were unsuccessful. A 200 μU silica fiber was inserted through a catheter and advanced into the lesion using 2 watts of delivered energy from an argon laser source. The fiber was then withdrawn from the lesion using 7 watts of energy to enlarge the lumen. No complications occurred, and posterior tibial blood flow was reestablished, as shown by Doppler flow measurements and resolution of clinical symptoms. We report a new technique of transcatheter fiberoptic-directed argon laser radiation (laser angioplasty) for the treatment of occlusive vascular disease.

Balloon dilatation of tracheal and bronchial stenosis.

Abstract: A 4Y2-month-old girl had acute respiratory arrest, which was caused by congenital tracheal and bronchial stenosis. Emergency resection of the stenotic segments of the trachea and right bronchus was performed. The distal four tracheal rings and proximal one right bronchial ring were excised, and an end-to-end anastomosis was performed. The surgical procedure has been described in detail [1]. Pathologic evaluation of the specimen revealed complete cartilage rings with reduction of the lumen diameter to 1 mm. The patient was followed with regular clinical evaluation and rigid bronchoscopy. She initially did well but later evaluation showed limited growth at the anastomotic site. At age 28 months, a bronchogram was obtained because of increasing respiratory difficulty. This showed a significant narrowing of the upper part of the trachea and also the origin of both right and left main-stem bronchi. Her symptoms increased over the next 3 months, and it was believed clinically that further treatment was needed for her stenosis. Surgical repair was not favored because previous operations would make this extremely difficult. Therefore, balloon dilatation of the trachea was carried out and was repeated 2 months later. When last seen 2 months after the second procedure, the patient was clinically well. Bronchography was not repeated.

The aortic valve. Colonic axis.

Abstract: In the past few years, a correlation has been recognized between calcific aortic stenosis and lower gastrointestinal bleeding in elderly patients. It has been suggested by several authors that mucosal arteriovenous malformations, usually in the right colon, are the cause of bleeding in those patients. Although attention is usually focused on doing a partial colectomy (usually right hemicolectomy) for treating colonic arteriovenous malformation bleeding, several patients with calcific aortic stenosis and gastrointestinal bleeding have been reported in whom bleeding stopped after aortic valve replacement alone. The purpose of this paper is to review the possible mechanisms of lower intestinal bleeding in patients with calcific aortic stenosis, delineate the methods of diagnosis, and finally, to outline the appropriate surgical management.

Vertebral artery stenosis: long-term follow-up.

Abstract: Ninety-six patients with greater than or equal to 50% unilateral vertebral artery (VA) stenosis were followed up for an average of 4.6 years. In 89 patients (93%) at least one VA origin was involved, while the intracranial VA was affected in 3 patients (3%). Seventy-four patients (77%) had greater than or equal to 50% stenosis of at least one internal carotid artery, of whom 52 underwent carotid endarterectomy. None of the patients had definite vertebrobasilar transient ischemic attacks (VB TIA). Nineteen patients (19.8%) experienced non-localizing symptoms possibly compatible with VB TIA, none of whom had a stroke. Twenty-three patients (24%) had strokes. The only two patients (2%) who sustained a brainstem infarction had fatal strokes and both were known to have basilar artery stenosis in addition to their VA stenosis. The observed stroke rate was 8.5 times the expected infarction rate for a normal population. Forty patients died during follow up. The observed 5-year survival rate was 60% compared to 87% in a matched normal population. Eight deaths (20% of all deaths) were caused by stroke and 21 deaths (52.5% of all deaths) were cardiac related. VA stenosis is most frequently located at the VA origin (93%), and is associated with a low incidence of brainstem infarction.

Value of lead V4R for recognition of the infarct coronary artery in acute inferior myocardial infarction

Abstract: In 84 patients with an acute inferior wall myocardial infarction (MI) admitted within 10 hours after the onset of chest pain, a right precordial lead V4R electrocardiogram was recorded in addition to the standard 12-lead electrocardiogram. The presence or absence of ST-segment elevation in lead V4R was correlated with results of coronary angiography performed 2 to 26 weeks (mean 10) after MI. Patients were classified into 3 groups: (1) those with a critical stenosis or occlusion proximal to the first right ventricular (RV) branch (27 patients); (2) those with stenosis distal to the right ventricular branch of the right coronary artery (36 patients); and (3) those with stenosis in the left circumflex coronary artery (21 patients). The presence of ST-segment elevation ≥ 1 mm in lead V4R has a sensitivity of 100% and a specificity of 87% for occlusion of the right coronary artery above the first RV branch; the predictive accuracy is 92%. Seven of 36 patients with a distal occlusion of the right coronary artery showed ST-segment elevation of 1 mm or more in lead V4R. The absence of ST-segment elevation ≥ 1 mm in lead V4R excluded proximal occlusion of the right coronary artery. ST-segment elevation in lead V4R was not seen either in 29 of 36 patients with a distal occlusion of the right coronary artery or in all patients with an occlusion of the left circumflex artery. Recording of lead V4R within 10 h after onset of acute inferior wall MI can give information rapidly about the vessel responsible for ML This could have implications when emergency procedures (streptokinase infusion, balloon dilatation and emergency surgery) are considered.

Prevalence of coronary artery disease in patients with isolated aortic valve stenosis.

Abstract: The prevalence of significant coronary artery disease (reduction in luminal diameter by more than 50%) among 88 consecutive patients with aortic stenosis requiring aortic valve replacement at Hammersmith Hospital was examined. Twenty two (34%) patients had significant coronary disease. Nineteen of 42 (45%) patients with typical angina had coronary disease; three of 20 (15%) patients with atypical chest pain had coronary disease, while none of 26 patients free of chest pain had significant coronary disease. Risk factors for coronary disease were equally distributed among patients with and without significant luminal obstruction. Because of the small, but definite, hazard of coronary arteriography and in the interest of cost containment it is suggested that patients with aortic stenosis who are free of chest pain do not require routine coronary arteriography. This applies particularly to patients requiring urgent aortic valve replacement.

Congenital pulmonary vein stenosis.

Abstract: Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected. Of the 8 in whom the condition was diagnosed during life, it was suspected clinically in 6 and found unexpectedly at cardiac catheterization in 2. All underwent operation, and 5 were hospital survivors. In all survivors rapid and progressive restenosis of the pulmonary veins occurred over the next several months. Three of the 5 underwent reoperation, but progressive restenosis recurred and all eventually died of this condition. Thus, despite partial surgical relief of pulmonary vein stenosis, the lesion is apparently one of relentless progression. No surgical repair has been successful in the cure or long-term palliation of this lethal lesion.