Right and left ventricular mass development in early infancy: Correlation of electrocardiographic changes with echocardiographic measurements.

Background: Right ventricular mass indexed to body surface area (RVMI) decreases and left ventricular mass index (LVMI) increases rapidly and substantially during early infancy. The relationship between these sizeable mass transformations and simultaneous electrocardiographic changes have not been previously delineated.

Methods: Normal term infants (#45 initially enrolled) were prospectively evaluated at 2 days and at 2-week, 2-month, and 4-month clinic visits.

Plasma biomarkers and echocardiographic indices of left ventricular function in very low birth weight infants.

Objective: Evaluate plasma markers of myocardial function and ischemia [B-type natriuretic peptide] (BNP) and cardiac troponin T (cTnT)] in relationship to echocardiographic indices of left ventricular (LV) function and severity of illness score (SNAPPE-II) in Very-Low-Birth-Weight-Infants (VLBWIs) prospectively.

Study Design: Serial echocardiography studies, clinical data, BNP and cTnT were obtained in thirty VLBWIs on postnatal days 1, 2, 3 and 7.

Results: BNP increased and cTnT decreased significantly day 1 through 3.

Death from cardiac failure in a child with ruptured cerebral arteriovenous malformation.

We present the case of a 13-year-old boy with a ruptured cerebral arteriovenous malformation who had rapidly progressive cardiac failure leading to death. Serial electrocardiograms, cardiac enzymes, echocardiograms, and pulmonary artery catheter data confirmed severe ventricular dysfunction related to myocardial ischemia and infarction. Cardiac dysfunction after cerebral insult is commonly described in adults with aneurysmal subarachnoid hemorrhage and has been termed "neurogenic stunned myocardium" because of its transient nature in most of patients.

Costello syndrome with pancreatic islet cell hyperplasia.

A 3-month-old boy with Costello syndrome (CS) developed respiratory distress and fatal arrhythmias. An autopsy showed cardiac hypertrophy, mild coarctation of the aorta, and pancreatic islet cell hyperplasia. To our knowledge, this is the first report of a pancreatic abnormality in CS.

Normal right and left ventricular mass development during early infancy.

The average newborn's right ventricular mass, indexed to body surface area, is approximately 20% above the adult level, consistent with in utero physiologic hypertrophy. In contrast, the left ventricular mass index is underdeveloped by about 30% at birth, resulting in a ratio of right to left ventricular mass that is nearly 75% higher than at maturity. Left ventricular mass index increases rapidly during the first 2 weeks of life and then more slowly thereafter, whereas the right ventricular mass index decreases steadily during the first 4 months of life.

Echocardiographic assessment of the right ventricular stress-velocity relationship under normal and chronic overload conditions.

Unlabelled: The effects of chronic volume or pressure overload on the velocity of right ventricular ejection have not been previously well defined. We hypothesized that, as formerly shown for the left ventricle, there would be a direct relationship between the velocity of ejection and an estimate of systolic wall stress.

Methods: Echocardiograms of asymptomatic patients, not on cardiac medications, with either an isolated secundum atrial septal defect > or = 5 mm in diameter or isolated pulmonic stenosis with a peak instantaneous pressure gradient > or = 20 mmHg, were reviewed.

Estimation of right ventricular free-wall mass using two-dimensional echocardiography.

Echocardiographic methods based on geometric models have long been in use for estimating left ventricular mass, but there is currently no similar method for estimating right ventricular (RV) free-wall mass. We hypothesized that a one-quarter prolate ellipsoid model could be used with two-dimensional echocardiography to approximate RV mass. Over a 2-year period 39 patients who had both a complete cardiac magnetic resonance imaging (MRI) scan and an echocardiogram within 2 weeks of each other were retrospectively analyzed.

Transvascular catheter infiltration of the ductus arteriosus with formalin in the newborn lamb: a novel method of establishing and maintaining ductal patency.

Objectives: This study sought to establish and maintain patency of the ductus arteriosus by a new method of transvascular formalin infiltration of the ductus arteriosus wall.

Background: Maintaining patency of the ductus arteriosus postnatally is necessary with many forms of congenital heart disease to ensure survival until definitive surgical repair. A variety of approaches have to date met with variable success.

Magnetic resonance imaging demonstration of "remodeling" of the aorta following balloon angioplasty of discrete native coarctation.

Assessment of the hemodynamic and anatomic results following balloon angioplasty of discrete native coarctation of the aorta, with particular attention to "remodeling," has required repeat cardiac catheterization and angiography, which is invasive and has limited resolution. Eight patients with hypertension and discrete native coarctation with an otherwise normally developed aortic arch underwent angioplasty at 5.0 +/- 6.

Transcatheter vascular occlusion of the small patent ductus arteriosus: an alternative method.

The current strategies concerning a small ( View Article and Find Full Text PDF

Association of a mosaic chromosomal 22q11 deletion with hypoplastic left heart syndrome.

The atypical presentation of CATCH 22 raises several important concerns. First, in this patient, as in others, the heart defects were found in association with subtle facial abnormalities but with few of the other criteria normally seen in CATCH 22. This association alone may be sufficient to raise suspicion that an interstitial 22q11 deletion may be present.

Outcome after open-heart surgery in infants and children.

We have studied the neurodevelopmental outcome of 104 consecutive unselected children who underwent open-heart surgery from 1987 through 1989. Survivors had formal neurologic and psychometric examinations after 2 years of age. Mean IQ was 90, and 78% had scores above 70.

Postoperative neurologic complications after open heart surgery on young infants.

Objective: To ascertain the relation between postoperative neurologic complications and variables occurring before, during, and after hypothermic cardiopulmonary bypass surgery to correct congenital heart disease in young infants.

Design: Prospective analysis of mortality and neurologic morbidity before hospital discharge; systematic comparison with patient characteristics, metabolic status, surgery variables; and preoperative neurologic findings of the patients.

Setting: Intensive care unit in tertiary care center.

The Norwood operation and subsequent Fontan operation in infants with complex congenital heart disease.

From April 1987 to September 1993, 60 infants underwent a Norwood operation for complex congenital heart disease including hypoplastic left heart syndrome (n = 41), ventricular septal defect and subaortic stenosis with aortic arch interruption/severe coarctation (n = 7), complex single right ventricle with subaortic stenosis (n = 8), critical aortic stenosis with endocardial fibroelastosis (n = 2), and malaligned primum atrial septal defect with coarctation (n = 2). Age at operation ranged from 1 day to 3.9 months (mean 9 days, median 3.

Long-term MRI changes in brain after pediatric open heart surgery.

We performed magnetic resonance imaging (MRI) on the brain and neurologic examinations on 23 children after open heart surgery for congenital heart disease. Twenty children also had psychometric assessments. Examinations were performed at a mean age of 66 months (range, 26 to 180 months).

Sequential longitudinal evaluation of cardiac growth and ventricular diastolic filling in fetuses of well controlled diabetic mothers.

Hypertrophic cardiomyopathy and abnormal ventricular diastolic filling in the infant of the diabetic mother is related to poor maternal glycemic control. Evaluation of fetuses of well controlled diabetic mothers has not been examined. Eleven fetuses of nondiabetic mothers (normals) and 9 fetuses of well controlled insulin-dependent diabetic mothers (FODMs) underwent serial evaluation of cardiac growth and ventricular diastolic filling using M-mode and Doppler echocardiography at 20-26 weeks' (period 1), 27-33 weeks' (period 2), 34-40 weeks' (period 3),m and 48-72 hours after birth (period 4).

EEG changes during open heart surgery on infants aged 6 months or less: relationship to early neurologic morbidity.

In a prospective study, we analyzed the intraoperative electroencephalographic (EEG) changes during open heart surgery with deep hypothermia in 66 infants aged 6 months or younger, 70% of whom were neonates. Suppression of amplitude and continuity at the nadir of temperature reduction and following rewarming, and the appearance of periodic paroxysmal activity, was compared with neurologic abnormalities before and following operation, patient characteristics, and operation variables. EEG changes disclosed no relationship to abnormal neurologic findings, age at operation, type of anesthetic, duration of cardiopulmonary bypass (CPB), duration of low-flow CPB or cooling, temperature at circulatory arrest (HCA) or low flow, or nasopharyngeal-venous return temperature differences.

Captopril-induced bone marrow suppression in two cardiac patients with trisomy 21.

Neutropenia is an infrequent complication following administration of the angiotensin-converting enzyme (ACE) inhibitor, captopril. Most reports have been in adult patients, with rare reports in the pediatric population. We report two cases of neutropenia following captopril use in cardiac patients with trisomy 21.

Developmental differences in cardiac myocyte calcium homeostasis after steady-state potassium depolarization: mechanisms and implications for cardioplegia.

We previously reported developmental differences in the response of cytosolic free calcium ([Cai]) to extracellular potassium ([Kex]) depolarization (cardioplegia) in juvenile (4 weeks post partum) and mature (12 to 18 months post partum) cardiac myocytes (rabbit). Our present study explored the physiologic basis for these observations. Single calcium-tolerant cardiac myocytes were isolated by sequential exposure to proteolytic agents, loaded with a fluorescent probe for calcium (fura-2) and [Cai] measured by standard fluorescence techniques.

Aortic "recoarctation" at rest versus at exercise in children as evaluated by stress Doppler echocardiography after a "good" operative result.

The mechanism for exercise systolic hypertension after a "good" operative repair of coarctation of the aorta remains speculative. Twenty-four children (mean age +/- SD 10.3 +/- 3.

Discrepancies in aortic growth explain aortic arch gradients during exercise.

Objectives: This study was conducted to evaluate the incidence and etiology of hypertension and aortic arch gradients during exercise in patients who have apparent good coarctation repair assessed at rest.

Background: The reported incidence of recurrent aortic arch obstruction (rest gradient > 20 mm Hg) after previous successful surgical repair varies from 0% to 60% and usually is associated with recurrent stenosis at the site of surgical repair.

Methods: Maximal treadmill exercise with Doppler echocardiographic gradient estimation was performed in 28 patients with a good coarctation repair at rest (normal blood pressure and arch gradient < 20 mm Hg) who had isolated coarctation repair a mean of 7.