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Biomedical subjects

A J Rein

Publications and source records attributed to A J Rein.

At least 37 records · Page 2Linked to original sources
PubMed1993

Generalized arteriopathy in Williams syndrome: an intravascular ultrasound study.

OBJECTIVES: We used intraluminal ultrasound imaging to provide additional information about the vascular pathology in Williams syndrome. BACKGROUND: The cardiovascular pathology of Williams syndrome consists of medial hypertrophy in both systemic and pulmonary arteries, which results in lumen narrowing. METHODS: Systemic and pulmonary arteries were examined in vivo using intravascular ultrasound imaging (5F, 30-MHz catheter) in three children with Williams syndrome. RESULTS: All arteries exhibited severe wall thickening with secondary lumen narrowing. Balloon dilation of a branch pulmonary artery in two children resulted in a significant localized increase in lumen caliber associated with a tear in the vessel wall. CONCLUSIONS: Intravascular ultrasound imaging in patients with Williams syndrome may permit better understanding of the pathophysiology of the syndrome and a more rational approach to therapeutic interventions.

Abnormalities, Multiple↗
PubMed1992

Prenatal diagnosis of tetralogy of Fallot with absence of the leaflets of the pulmonic valve.

Tetralogy of Fallot with absence of the leaflets of the pulmonic valve is relatively rare. We describe the prenatal diagnosis of this malformation in two fetuses. The association between the agenesis of the arterial duct and absence of the valvar leaflets is discussed. The diagnosis of the syndrome in the presence of dilated and pulsatile pulmonic arteries is easy to make. It should allow early counselling and planning of early care after delivery.

Abnormalities, Multiple↗
PubMed1992

Recombinant interleukin-2 and interferon alpha immunotherapy following autologous bone marrow transplantation. A case report of cardiovascular toxicity with serial echocardiographic evaluation.

The most serious side effects of recombinant interleukin-2 (rIL-2) and recombinant interferon-alpha (rIFN alpha) immunotherapy are cardiovascular disturbances, including systemic hypotension, left-ventricular dysfunction and pulmonary edema. We present a 25-year-old female who developed reversible cardiogenic shock during intermediate dose rIL-2 and low dose rIFN alpha therapy. Rapid clinical improvement occurred after intravenous fluid and dopamine support. A serial echocardiographic evaluation, which has not been described previously in this setting, is reported.

Adult↗
PubMed1990

Genetics of conotruncal malformations: review of the literature and report of a consanguineous kindred with various conotruncal malformations.

Genetic predisposition in congenital heart disease is considered to be a component of multifactorial inheritance. Recently, monogenic inheritance in conotruncal malformations has been suggested. We describe a consanguineous kindred with various conotruncal malformations, the presence of which lends support to the idea that this spectrum of malformation is monogenically inherited. Theoretical background and experimental and clinical data are reviewed and discussed.

Consanguinity↗
PubMed1989

Right ventricular outflow tract obstruction due to extracardiac tumors. A report of three cases diagnosed and followed up by echocardiographic studies.

Echocardiography has become a valuable diagnostic tool in various clinical conditions. Its use for the detection of extracardiac tumors has seldom been reported. The majority of these descriptions are of single case reports. We have recently encountered three patients (two with lymphomas and one with seminoma) who presented with signs and symptoms suggestive of right ventricular outflow tract obstruction. Two-dimensional echocardiography enabled the prompt diagnosis of extracardiac tumors compressing the heart. Moreover, echocardiography proved to be an excellent noninvasive tool for assessing the success of therapy for mediastinal tumors.

Adult↗
PubMed1988

Intracardiac causes of superior vena cava obstruction.

Most causes of superior vena cava (SVC) obstruction are extracardiac. In rare instances, an intracardiac process may obstruct the venous return from the SVC. This is illustrated by a report of a patient with a congenital coronary artery fistula to the right atrium obstructing the SVC return. We propose a classification and clinical approach to the differential diagnosis of SVC obstruction.

Child, Preschool↗
PubMed1988

An unusual presentation of right coronary artery fistula.

A four year old girl with infective endocarditis had unexplained facial swelling. Cross sectional echocardiography showed that a large right coronary artery fistula to the right atrium was obstructing the distal superior vena cava. The diagnosis was confirmed by cardiac catheterisation and at operation. The child was symptom free one year after operation.

Child, Preschool↗
PubMed1987

Regional and global left ventricular function in infants with anomalous origin of the left coronary artery from the pulmonary trunk: preoperative and postoperative assessment.

Anomalous origin of the left coronary artery from the pulmonary trunk (ALCA) remains a diagnostic as well as a therapeutic problem. The purposes of this study were: (1) to analyze left ventricular mechanics, including regional wall motion, in infants with ALCA, (2) to determine if the pattern of wall motion in infants with ALCA distinguishes these patients from those with congestive cardiomyopathy of other causes, and (3) to evaluate the potential for recovery of left ventricular function after successful restoration of a dual coronary artery system. Left ventricular mechanics were studied before and serially after surgery in six infants (2 to 13 months old) with ALCA. Fifteen age-matched normal subjects and seven age-matched patients with idiopathic congestive cardiomyopathy were also studied for comparison. Preoperatively, the end-diastolic volume in infants with ALCA was about four times larger than normal and did not differ from that in infants with CM; the myocardial volume was also about three times larger than normal, similar to that in the patients with CM. The myocardial volume/end-diastolic volume ratio was extremely low in patients with ALCA and in those with CM. The infants with ALCA did not exhibit specific segmental wall motion abnormalities but rather had global hypokinesis indistinguishable from that in the patients with CM. After successful repair, end-diastolic volume index rapidly decreased, reaching near normal values by 7 to 22 months after surgery, while myocardial volume index decreased at a slower rate, leading to an early phase of "overshoot hypertrophy," but reaching normal values by 7 to 22 months after surgery.+

Cardiomyopathy, Dilated↗