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Biomedical subjects

J T Lie

Publications and source records attributed to J T Lie.

At least 19 recordsLinked to original sources

Malignant angioendotheliomatosis (intravascular lymphomatosis) clinically simulating primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (PACNS) and malignant angioendotheliomatosis (MAE) are two unrelated and lethal diseases that frequently affect the brain. Both are uncommon and both require histologic documentation of their characteristic lesions for a definitive diagnosis. Because brain biopsies are not routinely included in the diagnostic workup of non-neoplastic CNS diseases, a number of unconfirmed nonvasculitic conditions may mimic PACNS. To my knowledge, MAE simulating PACNS clinically and angiographically has not been previously reported, and 3 such cases are described herein.

Brain Diseases

Polyarteritis nodosa in childhood a clinical pathologic study.

The clinical and pathologic findings of 2 infants and 7 older children with polyarteritis nodosa who were autopsied are reported. The most frequent clinical features included prolonged high fever, skin rash, abdominal symptoms, leukocytosis, proteinuria, and signs of either cardiac or renal failure. The 2 infants died of cardiac arrest, whereas renal or neurologic involvement was the most common cause of death in the older children. A consistent finding at autopsy was arteritis of the epicardial coronary arteries.

Adolescent

Cardiac involvement in polymyositis: a clinicopathologic study of 20 autopsied patients.

Cardiac involvement in polymyositis was investigated in 20 autopsied cases. Clinically, 13 of 18 patients had abnormal electrocardiograms, and 9 of the 20 patients had previous evidence of congestive heart failure. Histologically documented myocarditis was detected in 6 patients (4 with congestive heart failure and 2 without), 4 of whom also had small vessel disease of the myocardium. Patients with polymyositis may have a cardiopathy in the absence of overt myocardial inflammatory disease.

Adolescent

Histopathological types of cardiac fibrosis in myocardial disease.

Myocardial fibrosis is a frequently observed pathologic finding. It is a common practice to differentiate macroscopic scarring and microscopic scarring, but there has been little attempt to distinguish the various types of interstitial fibrosis, and to determine their prevalence in different cardiac disorders. In this study, we have semiquantitatively assessed microscopic scarring, interfibre and perivascular fibrosis and a distinctive type of plexiform fibrosis. We examined ten hearts with congestive cardiomyopathy, ten with hypertrophic cardiomyopathy, ten with severe valvular aortic stenosis and ten normal hearts. Perivascular and interfibre fibrosis were quantitatively closely linked and most marked in the congestive cardiomyopathy and the valvular aortic stenosis groups. Plexiform fibrosis occurred maximally in the hypertrophic obstructive cardiomyopathy group, closely associated with myocardium exhibiting muscle fibre disarray. Our study demonstrates that although different types of interstitial fibrosis have particular associations with certain disease entities, the associations are not specific. The recognition of the prevalence of one or more types of interstitial fibrosis may, however, assist in the histopathologic diagnosis of certain myocardial disorders.

Adult

Anatomy of coronary arteries in univentricular hearts and its surgical implications.

The coronary arterial anatomy in 26 univentricular hearts, its relation to the morphologic characteristics of the ventricles and rudimentary chambers, and its surgical implications were analyzed. All of the hearts except two had been operated on; 18 had septation with or without an extracardiac conduit and 6 had had palliative procedures. Twenty-one univentricular hearts with a left ventricular type main chamber had an anterior outlet chamber (17 left-sided subaortic, 3 right-sided subaortic and 1 right-sided subpulmonary). Right and left delimiting arteries outlined the outlet chamber in 16 hearts (76 percent). In 20 of the 21 hearts, large delimiting parallel branches of the right coronary artery course over the anterior wall of the heart; 13 of these vessels had been injured surgically with resultant ischemic myocardial necrosis. Five univentricular hearts did not have an outlet chamber; two had a left ventricular type main chamber and three had a morphologically right ventricular main chamber. Three of the five hearts had rudimentary pouches, located anteriorly in one and posteriorly in two. The two rudimentary pouches lying posteriorly were not outlined by delimiting arteries. Two of the five univentricular hearts without an outlet chamber also had injured coronary arteries. Thus, the identification of outlet chambers and rudimentary pouches in univentricular hearts is facilitated by the determination of coronary anatomy. The presence of major delimiting parallel branches over the usually favored ventriculotomy sites renders them vulnerable to surgical injury; such mishaps occurred in 15 of the 24 hearts that had either corrective or palliative operations.

Adolescent

Reduction of intimal thickening in canine coronary bypass vein grafts with dipyridamole and aspirin.

The potential benefit of platelet inhibitor drugs on coronary arterial bypass vein grafts was assessed in dogs with magnification-corrected angiographic luminal measurements and quantitative histologic evaluation of the vein grafts. There were 11 control animals and 11 animals treated with dipyridamole, 55 mg/day, plus aspirin, 325 mg/day. Eighteen animals with patent grafts were studied when electively killed 2, 4 or 6 months after grafting. At 14 days, there was greater angiographic narrowing in the most distal 1 cm of vein grafts in control than in treated dogs (P less than 0.01). This same angiographic narrowing persisted in control dogs until they were killed (P less than 0.03). Computer-assisted measurements of the entire area of intimal thickening were done on vein graft cross sections taken 1 cm from the distal anastomosis. The circumference of the vein grafts at the intimal-media junction was measured from the same section and the potential maximal luminal area calculated. The calculated luminal narrowing due to intimal thickening was greater in control than in treated dogs (P less than 0.03). These data correlate well with the demonstrated angiographic narrowing. The findings indicate that the degree of early intimal thickening that persists 2 to 6 months postoperatively in canine coronary bypass vein grafts may be reduced by the platelet inhibitor combination of dipyridamole plus aspirin.

Animals

An unusual mediastinal lipoma simulating cardiomegaly.

A patient with vague, nonspecific symptoms was initially thought to have an enlarged cardiac silhouette on chest roentgenogram, and a primary intracardiac disorder was suspected. At surgery, a mediastinal lipoma was diagnosed and excised. The roentgenographic appearance represents an unusual presentation of a mediastinal tumor.

Adult

Takayasu's arteritis in Crohn's disease.

This report describes a patient with coexistent Crohn's colitis and Takayasu's arteritis involving the thoracic aorta, superior mesenteric artery, and left hepatic artery. Unlike most previously reported cases of this association, characteristic histopathology is demonstrated. This case is also unusual in that the Takayasu's arteritis presented as aneurysmal disease instead of occlusive disease. We believe that Takayasu's arteritis may represent another of the many extra-intestinal manifestations of inflammatory bowel disease.

Adolescent

Ceroidosis in the "brown bowel syndrome".

A middle-aged patient with "brown bowel syndrome" or gastrointestinal ceroidosis manifested as malabsorption of undetermined cause is described. Autopsy revealed involvement of the entire gastrointestinal tract and unusual cardiac findings. Microscopically, the pigment responsible for the discoloration is a lipofuchsin that is deposited in the smooth muscle cells of the gastrointestinal tract. In all reported cases, ceroidosis was associated with some abnormality of the gastrointestinal tract--that is, malabsorption, steatorrhea, or gross local disease--with the possible exception of prolonged malnutrition. This case is also unusual because of the unexplained multifocal proliferative endocarditis. Vitamin E deficiency may be the common denominator of all these various disorders.

Ceroid

Primary cardiac myxosarcoma in a child.

This is a detailed clinical and autopsy documentation of a rare entity--primary cardiac myxosarcoma in a 29-month-old girl. The patient had sudden onset of right hemiplegia and angiographic evidence of multiple occlusions of the left middle cerebral artery. Subsequent M-mode and sector echocardiography showed a mobile, pedunculated left atrial tumor, which was excised. No other tumor mass was noted at the time of surgical exploration, and postoperatively, the patient received a course of chemotherapy (vincristine, dactinomycin, and cyclophosphamide). After a temporary improvement in her condition, the patient died following several days of rapid deterioration; this was 3 months after the onset of symptoms. Autopsy showed that death was due to brainstem herniation secondary to massive infiltration of the brain by tumor, and there were also widespread systemic metastases.

Brain

Morphologic spectrum of Ebstein's anomaly of the heart: a review.

The rare and curious congenital malformation of the tricuspid valve known as Ebstein's disease or Ebstein's anomaly has been a subject of considerable interest to the cardiologist and the morphologist since its first description by Wilhelm Ebstein in 1866. With the evolution of various surgical techniques for the correction of this anomaly, a thorough understanding of the pathologic anatomy of the malformation assumes greater practical importance. It is now clear that although all hearts with Ebstein's anomaly have in common displaced basal insertions of the septal and posterior leaflets of the tricuspid valve, each case may be pathologically and hemodynamically unique. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. It may be found in a left-sided tricuspid valve with inversion of ventricles. It is important that these individual variations be recognized so that the operative approach can be tailored to the particular variant of the malformation at hand.

Coronary Vessels

The right ventricular myocardium in Ebstein's anomaly: a morphometric histopathologic study.

Ebstein's anomaly of the tricuspid valve is associated with right ventricular dilatation in approximately two-thirds of cases. Dilatation may be massive. It involves not only the right ventricular wall proximal to the tricuspid valve (atrialized ventricle) but also the right ventricle distal to the valve (funtional right ventricle), including the right ventricular infundibulum. For further definition of the pathogenesis of dilatation of the functional right ventricle (distal chamber), a morphometric histopathologic study was performed on 10 hearts with Ebstein's anomaly and 10 normal age-matched control hearts. In the group with Ebstein's anomaly, five hearts exhibited dilated ventricles and five did not. The study demonstrated that dilatation of the right ventricle in Ebstein's anomaly was associated not only with thinning of the wall but also with an absolute decrease in the number of myocardial fibers counted through the thickness of the wall from endocardium to epidcardium.

Dilatation, Pathologic

Anomalous origin of left coronary artery from pulmonary artery in association with intracardiac lesions. Report of two cases.

Two cases are presented to illustrate the diagnostic and therapeutic dilemma when an anomalous origin of the left coronary artery from the pulmonary artery occurs with a hemodynamically significant intracardiac lesion. Hemodynamic alterations resulting from the associated intracardiac lesion may obscure the clinical features of the coronary arterial abnormality, and the appearance of normal coronary arterial distribution with left ventricular or aortic root angiography may create a false impression of a normal origin of the coronary arterial supply. A high index of suspicion may be the only lead to the detection of an anomalous origin of the left coronary artery from the pulmonary artery in association with an intracardiac lesion. In addition to the intracardiac lesion known to be associated with coronary arterial abnormalities, the question of anomalous coronary supply should be raised by the finding of poor left ventricular function or mitral insufficiency after operation (unexplainable by other causes), by the development of poor myocardial function during cardiac catheterization or anesthesia, or by the significant enlargement of a coronary artery seen during left ventriculography or supravalvular aortography.

Aortography

A reappraisal of palliative surgery for total anomalous pulmonary venous connection: description of a new operative technique.

The need for effective palliation of critically ill infants born with total anomalous pulmonary venous connection is reappraised. Careful study of autopsy specimens in patients with this cardiac malformation (patient ages ranging from newborn to 5 1/2 mth) revealed 4 favorable anatomic dispositions in 16 selected hearts: (1) a horizontal vein or a lobar vein larger than 3-mm size was present in every case; (2) these veins were of adequate length for a shunt anastomosis; (3) the heart could be rotated for easy access to the left atrium; and (4) one of the two separate connecting veins in the mixed type could be utilized. Based on morphologic observations and trial procedures on the autopsy specimens, a new palliative operation is proposed which can be performed under normothermia and without cardiopulmonary bypass.

Blood Vessel Prosthesis