Manojkumar Rohit

Kawasaki disease in infants below 6 months: a clinical conundrum?

Kawasaki disease (KD) is a medium vessel vasculitis of childhood. In infancy KD is often characterized by incomplete and atypical forms. There is paucity of literature on KD in children below 6 months and there are no data from any developing country. This study defines the profile of children with KD below 6 months at our centre.

A fast region-based active contour model for boundary detection of echocardiographic images.

This paper presents the boundary detection of atrium and ventricle in echocardiographic images. In case of mitral regurgitation, atrium and ventricle may get dilated. To examine this, doctors draw the boundary manually. Here the aim of this paper is to evolve the automatic boundary detection for carrying out segmentation of echocardiography images. Active contour method is selected for this purpose. There is an enhancement of Chan–Vese paper on active contours without edges. Our algorithm is based on Chan–Vese paper active contours without edges, but it is much faster than Chan–Vese model. Here we have developed a method by which it is possible to detect much faster the echocardiographic boundaries. The method is based on the region information of an image. The region-based force provides a global segmentation with variational flow robust to noise. Implementation is based on level set theory so it easy to deal with topological changes. In this paper, Newton–Raphson method is used which makes possible the fast boundary detection.

Sudden Death of a Young Child Due to Cardiac Rhabdomyoma

This report describes a 1½-year-old boy who succumbed to acute obstruction of the left ventricular outflow tract by a cardiac rhabdomyoma. He was admitted to have a transient loss of consciousness episode evaluated. A mobile intracardiac mass obstructing the left ventricular outflow tract and protruding into the aortic root during systole was detected by transthoracic echocardiography. At autopsy, it was confirmed to be a rhabdomyoma.

An Adolescent with Kawasaki Disease.

Kawasaki disease is an acute vasculitis of unknown etiology that predominantly affects children <5 years of age. The incidence and the severity of myocarditis in this disease is variable and depends upon the stage of the disease, acute or chronic. Acute-stage Kawasaki disease shows relatively high incidence of myocarditis, but almost all cases are clinically mild. We describe teenage boy presenting with atypical/incomplete manifestations of Kawasaki disease and developing fulminant myocarditis within a week of illness resulting in death. The case underscores the importance of suspecting Kawasaki disease in a young child presenting with features of myocardial ischemia.

Stroke in a case of neonatal lupus: an uncommon complication.

Stroke is an extremely rare complication of congenital heart block in children. We report a 2-year-old girl with congenital complete heart block who presented with acute-onset right middle cerebral artery territory stroke. The congenital heart block was secondary to maternal lupus.

Giant Coronary Aneurysms in Kawasaki Disease

Tothe Editor: Coronary sequelaeof Kawasaki Disease (KD)occur due to focal panarteritis during acute phase of diseaseand presents as coronary aneurysms [1]. Though use ofintravenous immunoglobulin (IVIg) has brought downthe risk of coronary aneurysms from 25 % to 4 −5%,giantaneurysms (GA) which are described as aneurysms of≥8mmsize, do continue to occur in 0.3−0.4 % patients [2]. Wedescribe our experience of managing two children with GAsecondary to KD.A three-year-old girl presented with high grade fever,generalized maculopapular rash and redness of eyes, lipsand tongue of 15 d duration. With a clinical diagnosis ofKD, she was given IVIg at 2 g/kg along with high doseaspirin. She showed defervescence only to mount feveragain within 48 h of IVIg infusion. She was given Infliximabonday22,followingwhichshebecameafebrilewithreturnofinflammatory parameters to normal. Echocardiogram(ECHO) showed GA involving mid-left anterior descending(LAD) artery. She was started on low dose aspirin and warfa-rin. Two years post diagnosis; she continues to beasymptom-atic with echocardiographic evidence of GA (8×9 mm)involving mid-LAD artery (Fig. 1).A two-year-old boy presented with high grade fever of9 d duration with nonpurulent conjunctivitis, erythematousoral mucosa and discrete nontender left cervical adenopathy.With a clinical diagnosis of KD, he was given IVIg at 2 g/kgalong with high dose aspirin. He showed defervescence, how-ever became febrile within 36 h. He was given a repeat dose ofIVIgandinfliximabonday17andday23ofillness,respec-tively. ECHO showed diffuse rig ht coronary artery dilatationwithGAofLADartery.Heisonlowdoseaspirinandwarfarin.Four years post diagnosis; he continues to be asymptomaticwith a 4 mm aneurysm in mid-LAD artery.Thrombosis is one of the main complications of GA;hence prevention of thrombosis is considered a mainstay[3]. Combination therapy of low dose aspirin and warfarinhas been shown to reduce risk of myocardial infarction com-paredtoaspirinalone[4].Besidesthrombosis,GAalsocarryahighriskofdevelopingprogressivestenosisoverseveralyearsand may need interventions [1]. Out of 76 patients with GAover a median follow-up period of 19 y, 59 % patientsunderwent coronary interventions, with a median of 1

Late Symptomatic Myocarditis in Kawasaki Disease: An Unusual Manifestation

To the Editor:Myocarditis is known to occur in 50 % patients in the acute phase of Kawasaki disease (KD) [1]. However, symptomatic myocarditis in the subacute/convalescent phase has not been reported. We report a boy with KDwho presented with congestive heart failure in the fourth week of illness. A 5-y-old boy presented with fever, swelling of extremities, an erythematous rash on the face, crusted lips, red tongue, bilateral submandibular lymphadenopathy and perianal desquamation. Echocardiogram was normal with an ejection fraction (EF) of 60 %. A clinical diagnosis of KD was made and 2 g/kg intravenous immunoglobulin (IVIg) was given on day 10 of illness. He became asymptomatic and was discharged on aspirin. In the fourth week of illness, he presented with fever, pedal edema and peripheral desquamation. Chest X-ray showed cardiomegaly (cardiothoracic ratio: 56.5 %). Electrocardiogram revealed sinus tachycardia at 110/min. Creatine-phosphokinase MB was 33.5 U/L. Echocardiogram showed dilated left ventricle with EF 30–35%with mild mitral regurgitation. Erythrocyte sedimentation rate was 48mm in the first hour (Westergren). Intravenous methylprednisolone was given for 5 d. EF was 60 % on day 10 of the second admission. He was discharged on prednisolone which was tapered and stopped after 4 mo. After 6 mo, he was asymptomatic with an EF of 61 % with normal coronary arteries. Evidence of myocarditis has been seen in more than 50 % patients in the acute phase of KD on myocardial biopsy or nuclear scans [1]. This involvement can present as heart failure and dysrhythmia. Majority would show a brisk response to IVIg therapy [2]. Though long-term abnormalities in cardiac contractility are less frequently described, histopathological evidence (cellular infiltration, fibrosis and disarray of muscle fibers) has been seen even after years of initial diagnosis [3]. This child is unique as he had symptomatic myocarditis 4 wk after the initial illness, despite a timely diagnosis, appropriate therapy and a normal initial echocardiogram. Coronary artery abnormalities were absent, though their association with myocarditis is controversial [4]. In the absence of myocardial infarction and precipitating factors like anemia, the cause for late myocarditis is unclear. Brisk response to corticosteroids emphasizes their role in KD and symptomatic myocarditis. Clinicians managing children with KD should watch for myocarditis and its complications even during the convalescent phase of KD.

Heart failure in children in tropical regions.

With the ever increasing younger population in tropical countries, the number of children with heart failure is increasing. However, the etiology of heart failure in this region varies considerably from that in the temperate region, with infectious causes leading the list. In this review, we have summarized the important causes of heart failure seen in the pediatric population in tropical regions.

Acyanotic and Cyanotic Congenital Heart Diseases

There has been tremendous progress in treatment of heart disease in children. Device therapy is increasingly being used in acyanotic congenital heart disease, while surgical results have improved significantly to give smile to many cyanotic heart disease children and their parents. This article makes an attempt to increase awareness of general pediatricians about common congenital heart diseases.

Loeffler’s Syndrome

A 9-year-old boy presented to the emergency room in shock with a 3-month history of low-grade fever and vomiting. He had no history of heart disease or contact with animals. The boy had tachycardia, tachypnea, a systolic blood pressure of 70 mmHg, pedal edema, hepatomegaly, and prominent neck veins. Precordial examination showed cardiomegaly, a S4 gallop rhythm, and a pansystolic murmur at the left lower sternal border. Radiography showed an enlarged heart. Echocardiography showed a dilated and noncollapsing inferior vena cava and a dilated right atrium. There was a gross encroachment into the right ventricular cavity by an echo-dense mass throughout the cardiac cycle (Fig. 1) and further, although less extensive, involvement of the left ventricle (Fig. 2). There was lowpressure severe tricuspid regurgitation with a normally positioned tricuspid valve and mild mitral regurgitation. The use of M-mode showed a fractional shortening of 20% and an ejection fraction of 50%. There was mild pericardial effusion but no pericardial thickening (see supplementary material 1, 2, 3, and 4). The echo findings were interpreted as endomyocardial fibroelastosis. Later, a complete review of the patient’s admission blood count showed leukocytosis (56,000/cubic millimeter or 56 9 10/litre) with an abnormally elevated level of peripheral eosinophils, an absolute count of 4,480/ cubic millimeter, and an erythrocyte sedimentation rate of 68 millimeter/hour. In view of the elevated absolute eosinophil count, Loeffler’s endocarditis was diagnosed, and the patient was managed accordingly. The patient was started on immunosuppressive therapy with prednisolone. The anticipated appropriate decline in the eosinophilic count was noted to be within the normal range. The boy unfortunately discontinued prednisone due to weight gain and was lost to follow-up evaluation. Loeffler’s endocarditis is a condition in which mature eosinophils infiltrate and damage the endocardium and myocardium [3]. This syndrome shows persistent apical obliteration, valve involvement, and progressive features of a restrictive cardiomyopathy apart from hematologic abnormalities [2]. With the use of echocardiography, 40% to 50% of patients with Loeffler’s syndrome are shown to have some form of cardiac involvement [1]. The reported patient demonstrated an interesting case of Loeffler’s syndrome with cardiac involvement well delineated by echocardiographic evaluation. Electronic supplementary material The online version of this article (doi:10.1007/s00246-010-9732-7) contains supplementary material, which is available to authorized users.