Prema Ramaswamy

Relation of Coarctation of the Aorta to the Occurrence of Ascending Aortic Dilation in Children and Young Adults With Bicuspid Aortic Valves

Children with bicuspid aortic valve (BAV) have aortic dilation that is present and progressive from birth irrespective of the functional state of the valve. There are no published data comparing aortic dilation in children with BAV with and without aortic coarctation (CoA). The objectives of this study were to (1) compare differences in aortic dimensions and rates of dilation between children with isolated BAV with those of children with BAV and CoA and (2) identify risk factors for the development of aortic dilation. Patients with BAV with CoA (group A) and without CoA (group B) were identified from our echocardiographic database (1993 to 2006). Aortic measurements at 4 levels were obtained, and z scores were compared. Criteria for exclusion were severe aortic regurgitation/stenosis, previous aortic valvuloplasty, complex left-sided cardiac disease, ventricular septal defects, and Turner, Noonan, Williams, and Marfan syndromes. There were 600 echocardiograms in 247 patients. Group A had 192 echocardiograms in 53 patients (median age 11.3 years; range 0 to 30; median follow-up 7 years), and group B had 382 in 194 patients (median age 8.7 years; range 0 to 29; median follow-up 4 years). Group B had significantly greater ascending aorta dimensions (p<0.05) and significantly faster rates of aortic dilation (p<0.0001). The ascending aorta in patients with BAV and CoA did not dilate to the same degree as in patients with isolated BAV. In conclusion, valve morphologic characteristics and function and age at CoA repair had none to minimal impact on aortic dimensions.

Frequency of Aberrant Subclavian Artery, Arch Laterality, and Associated Intracardiac Anomalies Detected by Echocardiography

Tetralogy of Fallot is generally considered to be the most common congenital heart defect associated with an aberrant subclavian artery (ASA), but the prevalence of ASA in patients with other cardiac anomalies is not well described. The pediatric echocardiography database, with 15,871 initial echocardiograms, was searched for all patients with ASA. Arch laterality and associated intracardiac anomalies were documented for each patient. ASA was found in 226 patients, of whom 171 had a left aortic arch (LAA) and 55 had a right aortic arch (RAA). The occurrence of ASA was 1% in patients with LAA (171 of 15,650) and 25% in patients with RAA (55 of 221; p = 0.001). Intracardiac anatomy was normal in 32% of patients with ASA/LAA and 25% with ASA/RAA. Conotruncal anomalies occurred more frequently with ASA/RAA than ASA/LAA (36% vs 18%; p = 0.01). Atrioventricular canal defects accounted for 10% and left-sided cardiac obstructive lesions accounted for 11% of subjects with ASA/LAA. ASA was rarely associated with d-transposition of the great arteries (1 of 226) and double-outlet right ventricle (5 of 226). The prevalence of ASA was highest in patients with interrupted aortic arch (11 of 38; 29%). In patients with tetralogy of Fallot, the overall prevalence of ASA was 8% (34 of 447), but was higher with RAA (16 of 103; 16%). The highest prevalence of ASA occurred in the subgroup of patients with tetralogy of Fallot with pulmonary atresia and RAA (6 of 25; 24%). In conclusion, ASA was more common in patients with RAA, especially with conotruncal anomalies. In patients with LAA, hypoplastic left heart syndrome, aortic coarctation, and atrioventricular canal defects were commonly associated with ASA.

Neonatal Marfan syndrome : in utero presentation with aortic and pulmonary artery dilatation and successful repair of an acute flail mitral valve leaflet in infancy.

Neonatal Marfan syndrome (nMFS) is the rare and severe form of this connective tissue disorder with poor outcome due to progressive valvular insufficiency. We describe a case of nMFS suspected in utero, by fetal echocardiography, due to marked dilatation of both great arteries, which is the first description of fetal pulmonary artery dilatation in this disorder. The patient developed a flail mitral valve leaflet during the first year of life that was successfully repaired with an excellent medium-term result. This is the first report of this type of surgery in infancy.

Regression of left ventricular hypertrophy in children with antihypertensive therapy

Left ventricular hypertrophy (LVH) is a complication of hypertension that has received great attention in the adult population. Large-scale randomized control studies in adults have demonstrated that LVH regresses in response to pharmacologic intervention. While it is known that LVH occurs as a complication of hypertension in the pediatric population, few studies have focused on its regression with pharmacological intervention. We report on three cases of hypertension-induced LVH in the pediatric population and its regression after treatment with antihypertensive medications. This report brings to light the need for larger, prospective studies on the incidence, natural history, and treatment of LVH associated with hypertension in the pediatric population.

Acute Myopericarditis After Multiple Vaccinations in an Adolescent: Case Report and Review of the Literature

We report a case of postvaccination acute myopericarditis in an adolescent. The patient presented with acute chest pain, diffuse ST-segment elevation, and elevated cardiac enzyme levels. Cardiac MRI was consistent with acute myocarditis. He recovered within a few days with nonsteroidal antiinflammatory treatment and remains clinically stable, with improvement of MRI findings at the 10-week follow-up. Postvaccination cases of myopericarditis reported in the pediatric literature are also reviewed.

Electrocardiographic Predictors of Left Ventricular Hypertrophy in Pediatric Hypertension

OBJECTIVE To determine the efficacy of electrocardiography (ECG) in detecting left ventricular hypertrophy (LVH) in pediatric hypertension (HT). STUDY DESIGN Concomitant echocardiograms and electrocardiograms in 108 children with HT were reviewed. Left ventricular mass (LVM), assessed by echocardiography, was used as a basis for a diagnosis of LVH (echo LVH) using accepted pediatric criteria. Using Wilcoxons rank-sum test, 14 ECG variables were compared between subjects with and without echo LVH. Spearman correlations were used to examine the linear association between echo LVH and these ECG variables. The sensitivity and specificity of ECG in diagnosing LVH were computed. RESULTS Of the 108 subjects studied, 35 (32%) met the pediatric criteria for LVH; of these, 8 (7.4%) also met the adult criteria (>51 g/m(2.7)) for LVH. Mean values for only 5 ECG criteria differed significantly among the groups: RI, SaVR, RaVL, RI+SIII, and SVI+RV6 (P < .05). Significant correlations were found for several ECG criteria and at least 1 measure of LVM, but the magnitudes were modest. Standard ECG criteria predicted LVH with high specificity (>90%) but low sensitivity (<35%). RI >10 mm was identified as demonstrating a modestly improved positive likelihood ratio of approximately 3. CONCLUSIONS ECG is not an adequate predictor of LVH for clinical use in HT.

Neonatal Left Pulmonary Artery Occlusion and Postinfarction Cysts of the Left Lung: Cause and Effect?

An 11-day-old male presented with tachypnea and fever of 2 days’ duration. He was found to be desaturated (85% saturation by pulse oximetry on room air) and he had no breath sounds on the left hemithorax. Chest x-ray showed extensive hyperlucency on the left with shift of the mediastinum to the right. Follow-up computed tomography (CT) scan with contrast revealed a large, lobulated, thickwalled cavity occupying most of the left hemithorax and connecting with the bronchial tree at the level of the left lower lobe (Fig. 1). Echocardiogram performed preoperatively demonstrated a large, highly echogenic mass at the take-off of the left pulmonary artery (LPA) with almost complete absence of the left pulmonary arterial blood flow (Figs. 2 and 3). Thrombosis of the LPA as the primary cause of the pulmonary pathology was considered likely and was confirmed at operation and subsequent histopathology examination, which showed more than 90% occlusion of the LPA with organizing thrombi, muscular hypertrophy, and dystrophic calcification. Multiple organizing thromboemboli involving distal pulmonary vessels

Ascending aortic aneurysm in a fetus due to a benign nodular myofibroblastic lesion.

A fetal echocardiogram at 20 weeks of gestation revealed a large ascending aortic aneurysm in the presence of a normal aortic root and normal intracardiac anatomy. No other abnormalities were noted in the fetus. Upon termination of pregnancy, histopathological examination revealed an isolated benign nodular myofibroblastic lesion of likely hamartomatous origin, a first description of such pathology contributing to the formation of an aneurysm in the ascending aorta.

Enhanced coronary blood flow and abnormal blood flow in the aortic isthmus in severe fetal anemia.

Coronary arteries are not normally visualized by fetal echocardiograms. Reversal of flow in the transverse aortic arch is most often seen in association with severe coarctation. We describe a case of a near-term fetus whose fetal echocardiogram showed very prominent coronary arteries and severe reversal of flow in the transverse aorta suggestive of a coarctation who was postnatally confirmed to have normal intracardiac and aortic anatomy. We discuss the pitfalls in clinical diagnosis in this case to alert pediatric cardiologists of transient perturbations in physiology masquerading as heart disease.

Dysmorphic features, cognitive disability, chronic inflammation, and predisposition to vascular disease in two sisters: a new autosomal recessive disorder?

A 20-year-old woman presented with mental retardation and a history of stroke related to moyamoya disease at the age of 8 years. She had cognitive impairment which became more pronounced after the stroke. This patient’s parents were first cousins and six close family relatives had strokes in their 60s or 70s. The patient’s 16-year-old sister had learning disability, chronic muscle pain, and an ECG suggestive of previous hypoxemic heart injury. The two sisters had similar dysmorphic facial appearance including a prominent philtrum, bulbous nose, and severe acne. They both had increased subcutaneous tissue in their faces, whereas their bodies were slim. Both sisters were found to have elevated levels of rheumatoid factor, C-reactive protein, and erythrocyte sedimentation rate on repeat measurements. Partial autoimmunity screening in one of the patients was negative. Chromosome analysis and array comparative genomic hybridization analyses were also normal. Nerve conduction findings in the younger sister were consistent with distal, predominantly motor, demyelinating neuropathy localized to the lower extremities. We propose that these two sisters suffer from a new autosomal recessive syndrome. Carrier status for this condition may predispose to later onset stroke.