Rubin S. Cooper

Atherosclerosis in Survivors of Kawasaki Disease

OBJECTIVES To test the hypothesis that long-term survivors of low-risk Kawasaki disease (KD) have ongoing vascular inflammation and dysfunction and a higher risk of accelerated atherosclerosis than healthy control subjects. STUDY DESIGN Twenty-eight patients with KD (7-20 years after acute illness) and 27 age-matched healthy control subjects were examined for medical and dietary history, serum markers of atherosclerotic risk and inflammation, carotid intimal-medial thickness (CIMT) with vascular ultrasound scanning and arterial stiffness with applanation tonometry. RESULTS Patients and control subjects were similar in age, sex, body mass index, waist-to-hip ratio, blood pressure, cigarette smoking, family history, diet, high-density lipoprotein cholesterol level, lipoprotein (a) level, homocysteine level, glucose level, insulin level, CIMT, arterial stiffness, C-reactive protein level, and inflammatory cytokine level. Levels of total cholesterol and apolipoprotein B were significantly higher in patients with KD than in control subjects. CONCLUSIONS There was no evidence of increased atherosclerosis. Small but significant differences in cholesterol and apolipoprotein B levels could suggest increased future risk for atherosclerosis and warrant further study.

Psychiatric disorders in youth with medically unexplained chest pain versus innocent heart murmur.

OBJECTIVE To examine the prevalence of Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition psychiatric disorders in youth with chest pain compared with a control sample with innocent heart murmur. STUDY DESIGN We assessed youth ages 8 to 17 years who were examined in cardiology settings for medically unexplained chest pain (n=100) or innocent heart murmur (n=80). We conducted semi-structured interviews and assessed medical history, quality of life, and disability. RESULTS Youth with chest pain had a higher prevalence of psychiatric disorders compared with youth with murmur (74% versus 47%, χ(2)=13.3; P<.001). Anxiety disorders predominated, although major depression was also more common in the chest pain group (9% versus 0%; Fisher exact tests; P<.01). Onset of psychiatric disorders generally preceded chest pain. Patterns were similar for boys and girls and for children and adolescents. Chest pain was associated with poorer quality of life and with pain-related disability for youth with co-morbid psychiatric disorder. CONCLUSIONS In childhood and adolescence, medically unexplained chest pain is associated with a high prevalence of psychiatric disorders. Systematic mental health screening may improve detection and enhance treatment of these patients.

Cardiac MR imaging in Pompe disease.

The magnetic resonance (MR) imaging findings in a 5-month-old infant with glycogen storage disease of the heart revealed hypertrophy of the right and left ventricles and the interventricular septum with an irregular inhomogeneous appearance of the myocardium. The descriptive features of the MR study are correlated with cardiac angiography and echocardiography in Pompe disease.

Atrioventricular canal ventricular septal defect with cleft mitral valve. Angiographic and echocardiographic features.

SummaryA rare form of endocardial cushion defect is an atrioventricular (AV) canal-type ventricular septal defect with cleft mitral valve in the absence of a primum atrial septal defect. In two infants with Downs syndrome, preoperative delineation of this entity was accomplished. M-mode echocardiographic features included presence of two distinct AV valves, attachment of the anterior portion of the auterier leaflet of the mitral valve to the interventricular septum, and normal septal motion. Two-dimensional echocardiography demonstrated the intact atrial septum, ventricular septal defect, presence of two distinct AV valves, and the cleft mitral valve. Angled (axial) cineangiography was employed to delineate the AV valve morphological characteristics, position of ventricular septal defect, and absence of a primum atrial septal defect. Since the surgical approach to this lesion is different from other forms of endocardial cushion defects and specific problems related to AV valve morphology may be encountered, the preoperative diagnosis of this entity is important.

The Malignant Course of Acute Rheumatic Fever in the Modern Era: Implications for Early Surgical Intervention in Cases of Bivalvular Insufficiency With Impaired Ventricular Function

In recent years, the incidence of acute rheumatic fever (ARF) has drastically decreased in the United States while remaining common in developing nations. However, immigration to the United States from developing nations has resulted in the continued prevalence of ARF. Patients with ARF face the potential development of significant valvular insufficiency and rheumatic carditis, which are associated with significant morbidity and mortality. Potentially adverse outcomes may be avoided with advanced interventions such as aortic and/or mitral valve replacement or repair, the use of inotropic agents, and ventricular assist devices for mechanical circulatory support. This report describes a series of three ARF carditis cases. This series serves not only to emphasize proper ARF prevention, but also to demonstrate the need for timely surgical intervention once medical therapy has failed.

Endothelial function evaluation in patients with anorexia nervosa

Introduction: This study evaluated endothelial function in patients with anorexia nervosa (AN) using Endothelial Pulse Amplitude Testing (Endo-PAT) and correlated findings with the patients’ history and biochemical data. Method: Twenty-one patients age 13-21 years diagnosed with AN by the Division of Adolescent Medicine at Cohen Children’s Medical Center of New York between 6/1/2012 and 5/31/2013 were studied along with 19 healthy controls similar in age and gender distribution. Digital pulse amplitude was examined using Endo-PAT. Raw data were automatically transferred into a reactive hyperemia index (RHI) and the natural log transformation of RHI (LnRHI). Subjects’ and controls’ electrocardiograms and biochemical markers were obtained. Results: AN and controls had similar RHI (P=0.7542) and LnRHI (P=0.9497). AN had lower mean weight (P<0.0001), height (P=0.0207), BMI (P<0.0001), resting HR (P<0.0001), systolic (P<0.0001) and diastolic BP (P=0.0141). AN also had lower mean HR during EndoPAT testing (P<0.0001), triiodothyronine (T3) (P<0.0001), luteinizing hormone (LH) (P=0.0055) and estradiol (E2) (P=0.0052). Total cholesterol (Chol) (P=0.0004) was higher in AN subjects. No correlation was observed between RHI and other parameters. Conclusion: No significant differences in RHI or LnRHI were found between the two groups. There were significantly higher Chol and lower HR, T3, LH and E2 levels in the AN group compared to controls. There were no correlations of these parameters to RHI. Abbreviations: AN: anorexia nervosa; BMI: body mass index; Chol: total cholesterol; DBP: diastolic blood pressure; E2: estradiol; ECG: electrocardiogram; Endo-PAT: Endothelial Pulse Amplitude Testing; FSH: follicle-stimulating hormone; Hcy: homocysteine; HDL: high-density lipoprotein; LDL: low-density lipoprotein; LH: luteinizing hormone; LnRHI: natural logarithm transformation of Reactive Hyperemia Index; ln(Lp(a)): natural logarithm transformation of lipoprotein A; Lp(a): lipoprotein A; PAT: Peripheral Arterial Tone; PRL: prolactin; RHI: Reactive Hyperemia Index; SBP: systemic blood pressure; T3: triiodothyronine; TG: triglyceride; TSH: thyroid stimulating hormone

Balloon Dilatation Angioplasty for Discrete Coarctation of the Aorta

The introduction and availability of balloon dilatation catheters has led to the use of percutaneous transluminal angioplasty in infants and children with congenital heart disease. The conditions that have been treated with this technique include: pulmonary valvular stenosis [1], aortic valvular stenosis, peripheral pulmonary artery and pulmonary vein stenosis, superior and inferior vena cava obstruction, coarctation of the aorta [2–4], and restenosis of coarctation [5]. This report describes the results of balloon dilatation angioplasty in seven patients with discrete coarctation of the aorta. Pulsed Doppler echocardiography was used to assess this condition before and after balloon dilatation Magnetic resonance imaging (MRI) was used in the postangioplasty assessment of all patients and in the preangioplasty assessment of two patients.

COST EFFECTIVENESS IN PEDIATRIC CARDIOLOGY

The usefulness and cost-effectiveness of non-invasive procedures performed on 77 infants and children at their initial cardiac evaluation were determined. Over a 6 month period, patients (pts) seen for the first time in the pediatric cardiology clinics at Downstate Medical Center and Brookdale Hospital Medical Center, were examined separately by 3 board certified pediatric cardiologists. In addition, a chest X-ray,electrocardiogram (ECG), and echocardiogram were obtained on each patient and the results were compared with published standard norms for age. Dr. A performed a history (H) and physical (P) only. Dr. B performed an H and P and reviewed the ECG and chest X-ray only. Dr. C performed an H and P and reviewed the echocardiogram only. Each doctor then independently classified a patient as having (I) definite heart disease, (II) possible heart disease, or (III) no heart disease. The 3 doctors agreed in 68 pts (88%). In 9 pts there was an interobserver disagreement. 2 pts were classified as I and II and 7 pts were classified as II and III. No pts were classified as I and III or I, II, and III. We conclude that routine non-invasive studies are not necessary at initial cardiac evaluation in order to differentiate heart disease from no heart disease. Therefore, selective use of these studies should result in significant reduction of health care costs.(Supported by HHS Grant #1RO1 HSO4935-01).