Cardiomyopathy and myocarditis in children with ventricular ectopic rhythm.

OBJECTIVE: The objective of this study was to evaluate the histologic features of the myocardium in children with abnormal ventricular ectopic rhythm but a structurally normal heart. BACKGROUND: Abnormal ventricular ectopic rhythm in children with a structurally normal heart is an uncommon but serious condition. Previous studies in adults with these findings have shown that approximately 10% have "primary electrical disease" and that 40% to 100% of these have abnormal histologic findings. METHODS: Endomyocardial biopsy samples were obtained prospectively in 33 subjects presenting with ventricular ectopic rhythm but a structurally normal heart by physical examination and noninvasive studies. Biopsy specimens were evaluated for histologic changes consistent with dilated cardiomyopathy or myocarditis and these results were compared with noninvasive and invasive clinical findings. RESULTS: Of the 33 subjects, 16 (48%) had normal myocardial histologic features (Group A), 14 (42%) had changes similar to the histologic features seen with idiopathic dilated cardiomyopathy (Group B) and 3 (9%) had lymphocytic myocarditis (Group C). Presenting clinical symptoms, surface electrocardiograms (ECGs), exercise stress testing and electrophysiologic stimulation tests failed to predict the biopsy results. Twenty-four-hour ambulatory ECGs showed a statistical difference between sustained and nonsustained ventricular tachycardia in Group A versus Group B (p less than 0.007), with Group A having more sustained ventricular tachycardia. Left ventricular function measured by fractional shortening on echocardiography did not differ between groups, but left ventricular end-diastolic dimension was greater in the subjects with abnormal histologic findings (Group B) (p less than 0.03). CONCLUSIONS: These results provide evidence that approximately 50% of children with abnormal ventricular ectopic rhythm but a structurally normal heart may have subclinical cardiomyopathy or unsuspected myocarditis.

New approaches to treatment of incessant ventricular tachycardia in the very young.

It was assumed that the availability of new antiarrhythmic drugs and new surgical techniques might allow medical or nonexcisional surgical treatment in many young children with incessant ventricular tachycardia. Fourteen infants and young children less than 5 years of age were evaluated and treated for incessant ventricular tachycardia. Medical treatment was pursued up to the use of amiodarone with a type Ib or Ic antiarrhythmic drug unless the patient became hemodynamically unstable. Patients underwent surgery when these drug regimens failed or when moderate congestive heart failure was present. Seven patients were successfully treated medically and seven underwent surgical treatment. Of those treated surgically, five had cryothermic lesions and two had excisions. Five of the surgically treated patients required temporary additional medical treatment. Follow-up ranged from 12 to 53 months (mean 28). Eleven of the 14 patients are currently not taking any antiarrhythmic medication. No patient required a pacemaker, none received anticongestive medications and none died.

Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.

OBJECTIVE: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND: Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS: A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS: Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 +/- 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% +/- 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 +/- 2%). CONCLUSIONS: Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.

An improved tungsten-178/tantalum-178 generator system for high volume clinical applications.

Clinical utilization of the multiwire gamma camera (MWGC) requires low-energy radionuclides. The short-lived (9.3 min) tantalum-178 (178Ta) is ideally suited for the MWGC and can be produced from long-lived (21.7 day) tungsten-178 (178W) by a previously reported 178W/178Ta generator. This generator, however, is limited by sharp increase in breakthrough after elution of only 30-60 column-volumes. To optimize the 178W/178Ta generator for clinical use, varying eluant acid concentrations were evaluated. A reduced (from 0.1 to 0.03N) HCI concentration in the eluant, coupled with low operating temperatures (3 to 5 degrees C) allowed high (40 to 60%) 178Ta yield. Minimal 178W breakthrough (less than .01%) resulted, even after elution of more than 200 column-volumes. Each of six tested generators provided sterile, high activity (up to 100 mCi) 178Ta elutions for more than 30 days. Radiation dosimetry was estimated utilizing both human and animal biodistribution data. The whole body (critical organ) dose in adults and neonates were 1/20 (1/21) and 1/19 (1/50) respectively relative to that of technetium-99m (99mTc) as sodium pertechnetate. The optimized 178W/178Ta generator provides a commercially practical, safe source of low-energy radioisotope for the MWGC with substantial dosimetry advantages over 99mTc.

Correlation of pediatric echocardiographic Doppler and catheter-derived valvar aortic stenosis gradients and the influence of aortic regurgitation.

This report suggests that in the absence of aortic regurgitation, Doppler peak and mean gradients are useful predictors of catheter peak-to-peak aortic stenosis gradients >50 mm Hg; however, in the presence of aortic regurgitation, the predictive value diminishes dramatically, but improves when electrographic data are incorporated. We present potentially useful equations to help predict the need for interventional catheterization for valvar aortic stenosis.

Prognostic features of children with idiopathic dilated cardiomyopathy.

The presenting features and long-term outcome of 39 children (median age 6.5 months, range 1 day to 16 years) with idiopathic dilated cardiomyopathy (IDC) were reviewed to help determine the appropriate management of these patients. Four outcome groups were identified: those who died, improved, had IDC resolved or received transplants. Presenting clinical features of age, sex, race, congestive heart failure, cardiomegaly, and degree of systolic ventricular dysfunction did not predict final outcome. Left ventricular hypertrophy on the electrocardiogram was seen significantly more often in children who improved than in those who died or in whom IDC resolved (p = 0.002). A rhythm disturbance was also seen more often in those who died than in those who survived (p = 0.025). Of 36 patients treated medically, 12 (33%) died, 15 (42%) improved and 9 (25%) resolved. Eighteen of 26 (69%) patients presenting at age less than or equal to 2 years survived, whereas 6 of 10 patients greater than 2 years survived. There were no differences based on age at presentation, in the time to death or time of follow-up. Three patients received orthotopic heart transplants, 1 of whom died from graft failure. Thus, no clinical feature including age at presentation consistently predicts ultimate outcome in children with IDC.

Pleuroperitoneal shunt for persistent pleural drainage after Fontan procedure.

Persistent effusions of chylous fluid may prolong the hospitalization period of many patients who have had a Fontan procedure. Herein we report the case histories of two patients in whom use of a pleuroperitoneal shunt to control the effusion shortened the hospital stay by several weeks.

Repeated endomyocardial biopsy without complication in an infant after heart transplantation.

Endomyocardial biopsy is necessary for accurate diagnosis of rejection after heart transplantation. This case illustrates the safe use of repeated endomyocardial biopsies in an infant after heart transplantation.

Right ventricular volume estimation using an ellipsoidal shell model and single-plane magnetic resonance imaging.

RATIONALE AND OBJECTIVES: To investigate whether accurate right ventricular volumes could be obtained using an ellipsoidal shell model with magnetic resonance (MR) image measurements from a single imaging plane. METHODS: An initial retrospective patient study included 10 patients with congenital cardiac defects who had undergone same-day or next-day radiographic contrast ventriculography. An expanded study included MR scans of a total of 29 patients with congenital cardiac defects. Magnetic resonance scans of 10 healthy volunteers were also included in part of the study. Right ventricular volumes were calculated from (1) model-based calculations using single-plane cine MR imaging, and (2) multislice calculations based on contrast angiography. RESULTS: Regression of angiography-based volumes against MR-based volumes showed high correlation (r = 0.97, see = 12.5 mL) and slope near unity. Regression of right against left stroke volumes, both calculated from MR data, showed excellent correlation (r = 0.90, see = 11.6 ml) and slope near unity. CONCLUSIONS: The ellipsoidal shell model can be used to reliably estimate right ventricular volume using single-plane MR images.

T-cell lymphoma in a child after heart transplantation.

Lymphoproliferative disease remains a small but worrisome problem after organ transplantation. This disorder is often a B-cell proliferation associated with Epstein-Barr virus. This article describes the unusual association of Epstein-Barr virus with a T-cell lymphoma in a child after heart transplantation and ultimately an atypical rejection episode with an increased number of eosinophils.

Subvalvar aortic stenosis: timing of operation.

Subvalvar aortic stenosis can be associated with progressive left ventricular outflow tract obstruction, aortic insufficiency, and infective endocarditis. We reviewed the records of 36 surgical patients who underwent 39 operations for subaortic stenosis. Seventeen patients had associated congenital cardiac anomalies. One perioperative death occurred in a patient with tetralogy of Fallot. The mean preoperative left ventricular outflow tract systolic pressure gradient was 64 +/- 5 mm Hg (+/- standard error of the mean) and decreased to 9 +/- 2 mm Hg postoperatively (p less than 0.001). Reliable preoperative and postoperative information regarding aortic valve function was available for 27 patients. Aortic insufficiency was found in 17 (63%) of those patients preoperatively. Postoperatively, insufficiency increased in 3 patients and decreased in 4; none of these changes was major. Severity of preoperative aortic insufficiency increased significantly with age (p less than 0.05), but did not correlate with left ventricular outflow tract gradient. The information from this study and previous studies suggests that resection of subaortic stenosis is safe and effective, and operation at the time of diagnosis, regardless of left ventricular outflow tract gradient or symptomatic status, is a reasonable therapeutic alternative.

Right ventricular volumes revisited: a simple model and simple formula for echocardiographic determination.

Our objective was to establish a crescentic model of the right ventricle as the basis of a reported 2/3 (Area)(Length) empirical formula for volume. This formula has been investigated by others without cognizance of its connection to a clear geometric model. The particular model, an ellipsoidal shell or difference of ellipsoids, has been investigated by several groups by using different volume formulas. Accordingly, we obtained echocardiographic images in 2 orthogonal planes from 7 patients and 4 volunteers. Specified area and length measurements from these images were used to calculate right ventricular volumes. These volumes were compared with values determined through multislice, magnetic resonance imaging with summation of lumen areas, a widely accepted standard. Obtained high correlations compared favorably with those of previous investigators who used equivalent but less well understood methods. We conclude that the ellipsoidal shell model of the right ventricle provides a simple area-length formula for the determination of lumen volume with echocardiography.

Noninvasive diagnosis of partial anomalous left pulmonary artery.

The branching patterns of the pulmonary arteries may vary, with one or more lobes of the left lung being supplied by a branch originating from the right pulmonary artery-a condition termed partial anomalous left pulmonary artery. If this anomalous branch courses anterior and inferior to the trachea, it is unlikely to result in airway obstruction. It is important that this anomaly not be confused with pulmonary artery sling, where the anomalous branch courses posterior to the trachea, encircling it almost completely. Partial anomalous left pulmonary artery has previously been diagnosed with angiography or by direct visualization during surgery. We present the first cases of noninvasive diagnosis of this anomaly with the use of echocardiography and magnetic resonance imaging. The complementary roles of these two modalities in detecting the anomaly and defining the spatial relationship of the anomalous pulmonary artery to the trachea are emphasized.

Imaging congenital heart disease.

When defined in a broad sense, imaging is the most important aspect of modern pediatric cardiovascular medicine. Definition of anatomic defects is now accurately and easily obtained with physical inspection, x-ray technology (including roentgenology, fluoroscopy, and cineangiography), and echocardiography. Echocardiography, with the addition of Doppler and color flow Doppler, is the most important development in clinical cardiac imaging in the past decade. The exciting new areas of "imaging" are in cardiac functional analysis and metabolic evaluation. Viewing the heart at the cellular or biochemical level is the challenge of the future. The new technology offered by computed tomography, positron emission tomography, and nuclear magnetic resonance imaging begins to provide the ability to image the domain of cellular and biochemical function.

Two cases of left superior vena cava draining directly to a left atrium with a normal coronary sinus.

The most common variation in the thoracic systemic venous system is a persistent left superior vena cava draining to a coronary sinus. A rare anomaly is a persistent left superior vena cava connecting directly to the left atrium. In this situation it is believed that the coronary sinus must be absent. This report describes two cases of a persistent left superior vena cava draining to a left atrium with a normal coronary sinus.

Pediatric cardiac auscultation.

Murmurs will be a frequent finding during the well child pediatric exam and perhaps more frequent during the evaluation of an acute illness, but most of these murmurs are innocent. Understanding the variety of causes of innocent murmurs should help identify them as such. However, referral to the pediatric cardiac specialist is appropriate in newborns and small infants, when the findings are not entirely consistent with an innocent murmur, or when there are other reasons to suspect heart disease.

Resolution of atrial standstill in a child with myocarditis.

Atrial standstill is a rare disorder usually seen in adults with extreme myocardial disease. Etiologies described in the literature have included muscular dystrophy, familial amyloidosis, and rarely myocarditis. These etiologies usually lead to permanent atrial standstill and require ventricular pacing. We present a case of an 11-year-old black female who developed atrial standstill secondary to biopsy proven acute necrotizing myocarditis. Absence of atrial function was confirmed by surface electrocardiogram, echocardiogram, and an invasive electrophysiology study. Atrial function returned within 3 days of initiation of methyl-prednisolone. In cases of atrial standstill due to myocarditis, a delay in the placement of a permanent pacemaker with or without a trial of methylprednisolone may prove beneficial.

The effect of indomethacin tocolysis on fetal ductus arteriosus constriction with advancing gestational age.

OBJECTIVE: Our purpose was to determine whether continuing exposure to indomethacin tocolysis is associated with an increased incidence of constriction of the human fetal ductus arteriosus with advancing gestational age. STUDY DESIGN: Fetal echocardiograms were reviewed in 61 cases in which the pregnant women were treated for preterm labor with indomethacin (25 mg orally every 6 hours). Density function analysis and regression analysis were used to assess the effect of indomethacin tocolysis on ductal constriction with advancing gestational age. RESULTS: A total of 193 fetal echocardiograms were obtained for 72 fetuses. Ductal constriction developed in 50% of the fetuses ranging from 24.7 to 35.0 weeks' gestation. Fetuses with indomethacin-induced ductal constriction demonstrated a greater increase in systolic flow velocities with advancing gestational age compared with the nonconstricted group (p < 0.05). Constriction was detected at a mean gestational age of 30.9 +/- 2.3 weeks at an average of 5.1 +/- 6.0 days after initiation of therapy. Ductal constriction occurred by 31 weeks' gestation in 70% of the affected fetuses. After discontinuation of indomethacin therapy, all follow-up echocardiograms demonstrated a return to nonconstricted ductal flow velocities. No significant adverse neonatal outcomes were attributed to indomethacin use. CONCLUSIONS: A dramatic yet reversible increase in the incidence of indomethacin-induced ductal constriction occurs at 31 weeks' gestation. However, ductal constriction can occur at any gestational age. With indomethacin tocolysis, weekly fetal echocardiography is warranted for the duration of therapy.

Immunosuppressive treatment for myocarditis and borderline myocarditis in children with ventricular ectopic rhythm.

OBJECTIVES: To ascertain the responsiveness to immunosuppressive treatment of myocarditis and borderline myocarditis in children with ventricular ectopic rhythm (that is, all ventricular arrhythmia except benign premature ventricular contractions). To determine the impact of the diagnostic information provided by an endomyocardial biopsy specimen in these patients. BACKGROUND: The therapeutic value of performing an endomyocardial biopsy in children with ventricular ectopic rhythm is not established. In turn, the treatment of myocarditis with immunosuppressive drugs is also controversial. METHODS: The case notes and endomyocardial biopsy findings of all children with ventricular ectopic rhythm and a biopsy diagnosis of myocarditis were reviewed. RESULTS: Ten (14%) of 69 patients with ventricular ectopic rhythm and an anatomically normal heart had histological evidence of myocarditis or borderline myocarditis. Eight patients received corticosteroids and efficacy was judged by regular 24 hour Holter monitoring. Total resolution of arrhythmia was seen in four, improvement in two, and no change in two patients. At follow up (8-39 months, mean 22 months), arrhythmia recurrence was seen in the two patients who showed an improvement but not resolution during treatment. Both received azathioprine with further reduction in ectopy rates. Patients who responded to treatment were symptomatic (six of six patients) at presentation compared with those who did not respond to treatment (none of two patients) who were not symptomatic. Five patients had a repeat biopsy specimen taken which confirmed histological improvement. CONCLUSIONS: Steroid treatment seems to benefit a subset of children with ventricular ectopic rhythm and a biopsy diagnosis of myocarditis or borderline myocarditis. Because it can identify a treatable cause for the ventricular arrhythmia, endomyocardial biopsy is a valuable investigation in these patients.