Marie M. Gleason

Guidelines for the Outpatient Management of Complex Congenital Heart Disease

An increasingly complex group of children is now being followed as outpatients after surgery for congenital heart disease. A variety of complications and physiologic perturbations, both expected and unexpected, may present during follow-up, and should be anticipated by the practitioner and discussed with the patient and family. The purpose of this position article is to provide a framework for outpatient follow-up of complex congenital heart disease, based on a review of current literature and the experience of the authors.

A pilot study of the feasibility of heart screening for sudden cardiac arrest in healthy children

BACKGROUND In children, sudden cardiac arrest (SCA) is associated with structural and electrical cardiac abnormalities. No studies have systematically screened healthy school children in the United States for conditions leading to SCA to identify those at risk. METHODS From June 2006 to June 2007, we screened 400 healthy 5- to 19-year-olds (11.8 ± 3.9 years) in clinical offices at The Childrens Hospital of Philadelphia using a medical and family history questionnaire, weight, height, blood pressure, heart rate, cardiac examination, electrocardiogram (ECG), and echocardiogram (ECHO). Our goals were to determine the feasibility of adding an ECG to history and physical examination and to identify a methodology to be used in a larger multicenter study. A secondary objective was to compare identification of cardiovascular abnormalities by history and physical examination, ECG, and ECHO. RESULTS Previously undiagnosed cardiac abnormalities were found in 23 subjects (5.8%); an additional 20 (5%) had hypertension. Potentially serious cardiac conditions were identified in 10 subjects (2.5%); 7 were suspected or identified by ECG and 3 more only by ECHO. Only 1 of the 10 had symptoms (previously dismissed); none had a positive family history. CONCLUSIONS It is feasible to screen for conditions associated with SCA in healthy children by adding ECG to history and physical examination. In this nongeneralizable sample, ECG identified more cases compared to history and physical examination alone, with further augmentation from ECHOs. Improvements in ECG and echocardiographic normative standards, representing age, gender, race, and ethnicity, are needed to increase the efficacy of screening in a young population.

Two-dimensional and doppler echocardiographic assessment of neonatal arterial repair for transposition of the great arteries

The arterial switch procedure has become an accepted reparative technique for transposition of the great arteries with or without ventricular septal defect. In this study the accuracy of prospective noninvasive imaging in detecting arterial tract obstruction and the prevalence and severity of arterial valvular regurgitation (as assessed by Doppler ultrasound) were evaluated in survivors of arterial repair. All 53 study patients underwent two-dimensional echocardiographic examination 2 days to 20 months (median 7 months) postoperatively; 43 patients also had pulsed and continuous wave Doppler studies. The accuracy of the noninvasive evaluation of arterial tract obstruction was determined by comparison of Doppler maximal instantaneous gradients with peak to peak gradients at nonsimultaneous catheterization in 26 patients. Twenty-one (81%) of the 26 patients underwent catheterization and successful pulsed and continuous wave Doppler examination of the right heart; 17 (81%) of these 21 had a maximal pressure gradient within 20 mm Hg of the peak to peak gradient obtained at catheterization. Echocardiographic identification of the stenotic site was correct in all eight of the patients in this group requiring reoperation. Twenty-three (88%) of the 26 patients who underwent catheterization had successful Doppler interrogation of the aortic tract; 22 (96%) of these 23 had a maximal instantaneous gradient within 20 mm Hg of the peak to peak catheterization gradient. Fourteen (32%) of 43 patients had mild or moderate pulmonary regurgitation by Doppler study. Three (7%) of the 43 had mild aortic regurgitation.

Mitral Arcade: A Rare Cause of Fatigue in an 18-Year-Old Female

A previously healthy 18-year-old female presented for evaluation of a 3-month history of fatigue. Other notable findings in the history were progressively worsening dyspnea on exertion, 3-pillow orthopnea, a newly developed cough, and a 20-lb weight loss. The physical examination demonstrated tachypnea, with a respiratory rate of 24 breaths per minute, and the lungs were clear. The heart rate was 88 bpm and regular. There was a regular rhythm with a normal first heart sound and a second heart sound that was slightly accentuated and narrowly split. There was a 2/6 systolic regurgitant murmur at the left ventricular apex. There was no diastolic murmur. There was no jugular venous distension or hepatic engorgement. A chest radiograph demonstrated fine, reticular prominence of the pulmonary interstitium of both lungs, increased pulmonary vascular markings, and mild prominence of the left atrial appendage (Figure 1). A nongated computed tomography scan of the chest demonstrated abnormal pulmonary parenchyma with diffuse ground-glass opacities (Figure 2). A 15-lead ECG was performed that demonstrated sinus rhythm, right-axis deviation, and biatrial enlargement (Figure 3). Figure 1. Chest radiograph in the posteroanterior projection demonstrates fine reticular prominence of the pulmonary interstitium of both lungs (asterisk), increased pulmonary vascular markings (arrow), and mild prominence of the left atrial appendage (LA) …

Ruptured sinus of Valsalva aneurysm in childhood.

papillary muscle, resulting in its avulsion. Although very short balloons are not recommended for pulmonary valvuloplasty, excessively long balloons can be harmful, and thus the appropriate diameter as well as length of the balloon should be individually selected for best results. Depending on the age of the patient, we recommend use of balloons that are 20 to 40 mm long for pulmonary valvuloplasty in infants and children.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa in a healthy child

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (P-MAIVF) is a condition that has been reported in adults as a sequela of infective endocarditis, aortic surgery, and trauma [1–3]. However, in pediatric patients, this condition is extremely rare. Morphologically, it is a false aneurysm at the fibrous, interannular zone, between the mitral and aortic valves. It communicates with the left ventricular outflow tract (LVOT) between the anterior leaflet of the mitral valve and the left coronary or non-coronary cusp of the aortic valve [1]. In adults, this is an acquired finding, however, here we describe a rare case of a P-MAIVF, where no etiology was found and the lesion had not produced any symptoms in a young child.

What the Pediatrician Needs to Know: Care for the Child with Hypoplastic Left Heart Syndrome

Rapid breakthroughs in research and technology are dramatically changing the practice of clinical medicine, as well as expectations for the future, as we transition from the 20t“ to the 21st centuries. In years past, a primary care provider would rarely be expected to provide ongoing care for children with complex congenital heart lesions due to poor survival rates. We are now faced with an ever-growing population of infants and children who are surviving into adolescence and young adulthood with congenital heart problems previously thought to be incompatible with life. It is because of this changing pediatric population that education of primary care providers and cooperation between the local physician and the pediatric cardiologist are required to provide the best of care to these unique children and their families.