Fungal Endocarditis, 1995–2000

Recurrent inflammatory pseudotumors in children.

https://www.jtcvs.org/article/S0022-5223(99)70286-2/pdf

Surgical treatment of subaortic stenosis: a seventeen-year experience.

Bach JR: Medical considerations of long-term survival of Werdnig–Hoffmann disease. Am J Phys Med Rehabil 2007;86:349–355.Objectives:To report intercurrent nonrespiratory complications of unprecedented survival for Werdnig–Hoffman disease (spinal muscular atrophy type 1 [SMA 1]).Design:A retr

Medical considerations of long-term survival of Werdnig-Hoffmann disease.

Cardiac tumors other than myxomas are rare. We report a series of 10 intracavitary polypoid myofibroblastic proliferations in children and young adults emphasizing gross, histologic, and clinical features. There were 6 females and 4 males, with a mean age of 10 years (range 5 wk to 21 y). All lesions were endocardial-based, located in the right atrium (1), right ventricular inflow/tricuspid valve (1), right ventricular outflow (3), mitral valve (3), aortic valve/left coronary sinus (1), and left ventricular free wall (1). Symptoms included shortness of breath or dyspnea (3), syncope (2), chest pain (1), transient ischemic attacks (1), and fever with myalgias (1). All tumors were surgical resections, except 1 tumor that resulted in sudden coronary death and that was diagnosed at autopsy, and 1 tumor that embolized into the coronary artery and was treated by cardiac transplant. Two tumors, present in the aortic and mitral valves, respectively, caused cardiac ischemia. The tumors were polypoid or filiform and histologically resembled inflammatory myofibroblastic tumors of extracardiac sites, with loose spindle cell growth with sparse inflammation. Although there were frequent collagen bundles interspersed among the tumor cells, there were no large areas of dense fibrosis. Surface fibrin was present on the polypoid projections in 7 cases. Symptoms resulted from prolapse into coronary ostia or embolization, but no patient developed metastasis. Long-term follow-up in 2 patients demonstrated no evidence of disease or recurrence. Although metastatic potential was not identified, these tumors may result in serious symptoms, including myocardial infarct, syncope, and sudden death. These cardiac myofibroblastic tumors are readily distinguished from other endocardial-based cardiac tumors, including papillary fibroelastoma and myxoma, which may present clinically in the same manner.

Cardiac inflammatory myofibroblastic tumor: a "benign" neoplasm that may result in syncope, myocardial infarction, and sudden death.

We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant The infant is asymptomatic 18 months after the second surgery The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combinationThe inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes To our knowledge only 9 cases have been published of such tumor arising within the heart1–6

Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract.

Transposition of great arteries is the consequence of abnormal aorticopulmonary septation. Animal embryonic data indicate that septation and elastogenesis are related events, but human and clinical data are not available. We tested the hypothesis that large artery elastic function was impaired in patients with transposition of great arteries. We studied 34 patients aged 9 to 19 years, 12±3 years after atrial switch operation; 14 patients aged 7 to 9 years, 8±1 years after arterial switch operation; and 108 healthy control subjects matched for age. Carotid artery diastolic diameter and pulsatile distension were determined by echo wall-tracking; carotid blood pressure was measured by tonometry. Systolic pressure was higher and diastolic pressure was lower in patients than in controls. Patients with atrial and arterial switch repair were compared with their respective controls by 2-factor ANOVA. For patients with atrial switch repair versus control, stiffness index β was 4.9±1.5 versus 3.1±1.0 ( P P

Transposition of great arteries is associated with increased carotid artery stiffness.

The echocardiographic features and management of 10 children with right heart thrombus (RHT) are presented. The patients' ages ranged from 2 weeks to 12 years. Three patients were less than 3 weeks old. The echocardiographic diagnosis was confirmed in five patients by surgery or autopsy. The thrombus was detected in the right atrium (five patients), in the right ventricle (three), on the tricuspid valve or within the inferior vena cava, the superior vena cava, or its extension following Senning's operation for transposition of great arteries (TGA). Seven patients had structural heart disease: TGA in four, tricuspid atresia in three. In two of three patients with anatomically normal heart, the thrombus occurred as a complication of a ventriculoatrial and a lymphovenous shunt system. The third patient had no predisposing factor. Five patients were treated with anticoagulants or platelet anti-aggregating agents and four by surgery. Seven patients survived and were followed by echocardiography.

Right heart thrombus in infants and children

Complex congenital heart diseases with abnormal formation of the aorticopulmonary septum are also associated with defective large artery elastogenesis. In the current study, we tested the hypothesis that carotid artery elastic function was impaired in patients with tetralogy of Fallot (ToF). The study included 45 Fallot-patients (male:female 27:18; age 21.0 ± 11.8 years) and 45 age- and gender-matched healthy control individuals. Carotid artery diameter, pulsatile distension, and intima-media thickness (IMT) were measured by echotracking device, and carotid blood pressure was determined using applanation tonometry. Carotid artery elasticity was characterized by compliance and distensibility coefficients, stiffness index β, and incremental elastic modulus. All carotid artery elastic parameters showed significant differences between groups. The compliance coefficient was 36%, and the distensibility coefficient was 33% smaller, whereas stiffness index β was 46% and incremental elastic modulus was 40% larger in Fallot-patients. Fallot-patients also had larger carotid artery IMT as compared to that of healthy individuals. Carotid artery is markedly stiffer in Fallot-patients suggesting that impaired elastogenesis is a component of the congenital abnormality. Increased large artery stiffness might contribute directly and indirectly (through impairment of baroreflex function) to the higher mortality found in ToF patients.

Impaired carotid artery elastic function in patients with tetralogy of Fallot

We have shown previously that TGA (transposition of great arteries) is associated with increased carotid artery stiffness. It has been established that stiffening of the barosensory vessel wall results in reduced baroreceptor activation and impaired BRS (baroreflex sensitivity). In the present study we tested the hypothesis that the increased carotid artery stiffness in TGA patients was associated with reduced cardiovagal BRS. We studied 32 TGA patients aged 9-19 years, 12+/-3 years after surgical repair and 32 age-matched healthy control subjects. Carotid artery diastolic diameter and pulsatile distension was determined by echo wall tracking; carotid blood pressure was measured by tonometry. BRS was measured using spontaneous techniques [BRS(seq) and LF(gain) (low-frequency transfer function gain)] and by the phenylephrine method (BRS(phe)). Carotid artery distensibility was markedly reduced in patients as compared with controls (5.6+/-1.9 x 10(-3) compared with 8.7+/-2.7 x 10(-3)/mmHg P<0.05, as determined using an unpaired Student's t test), but BRS was not different in patients and controls (20.3+/-14.7 compared with 21.7+/-12.7 for BRS(seq); 13.1+/-9.2 compared with 10.6+/-4.5 for LF(gain); and 19.1+/-8.6 compared with 24.8+/-7.2 for BRS(phe) respectively). Carotid artery elastic function was markedly impaired in patients with TGA, but the increased stiffness of the barosensory vessel wall was not associated with reduced BRS. It appears that attenuation of baroreceptor stimulus due to arterial stiffening may be compensated by other, possibly neural, mechanisms when it exists as a congenital abnormality.

/pdf/adaptation-of-baroreflex-function-to-increased-carotid-3vz8evz767.pdf

Krisztina Kádár

Papers

Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract.

Transposition of great arteries is associated with increased carotid artery stiffness.

Right heart thrombus in infants and children

Impaired carotid artery elastic function in patients with tetralogy of Fallot

Adaptation of baroreflex function to increased carotid artery stiffening in patients with transposition of great arteries