Arrhythmias in the athlete with congenital heart disease: guidelines for participation.

Advances in the diagnosis and early management of congenital heart disease in recent decades have led to increasing numbers of individuals being sufficiently well to participate in social as well as truly competitive sports. Physicians are increasingly asked whether such participation is safe, advisable, and efficacious, yet few guidelines exist to help them make these decisions. There are three apparent subgroups of patients: (a) those with mild or repaired problems, who function normally or nearly so and may fully participate; (b) those with severe functional deficit or known high risk, for whom strenuous exertion must be strictly proscribed; and (c) those who fall in between, with some limitations and some risk--these patients present a great challenge to the wisdom and clinical skill of the physician.

Anesthetic management and outcome following noncardiac surgery in nonparturients with Eisenmenger's physiology.

STUDY OBJECTIVE: To evaluate the perioperative risk to nonparturients with Eisenmenger's physiology for noncardiac surgical procedures. DESIGN: Retrospective chart review. SETTING: University-affiliated hospital. PATIENTS: 12 nonparturients with Eisenmenger's physiology who underwent 25 noncardiac surgical procedures requiring care by an anesthesiologist. MEASUREMENTS AND MAIN RESULTS: Preoperative, intraoperative, and postoperative records were retrospectively analyzed. Data examined included patient age, gender, symptoms, laboratory values, monitors used, surgical procedure, and outcome. Twenty-five procedures were performed on 12 patients; 13 procedures were performed with general anesthesia, 6 with peripheral nerve blocks, 5 with sedation by an anesthesiologist with or without local anesthetic infiltration, and one with epidural anesthesia. One patient died perioperatively. Review of the literature revealed two deaths in 32 procedures for nonparturients with Eisenmenger's physiology undergoing noncardiac surgery. CONCLUSIONS: A variety of anesthetic techniques and drugs may be used successfully in nonparturients with Eisenmenger's physiology undergoing noncardiac surgery. Although the study group is small, the perioperative mortality risk is lower than that for parturients undergoing either labor and delivery or cesarean section and is probably in the range of approximately 10%.

Imaging and sizing of atrial septal defects by magnetic resonance.

BACKGROUND: Development of techniques for percutaneous closure of atrial septal defects (ASDs) makes accurate noninvasive sizing of ASDs important for appropriate patient selection. METHODS AND RESULTS: Magnetic resonance (MR) images of ASDs were obtained in 30 patients (mean age, 41 +/- 16 years) by both spin-echo and phase-contrast cine MR imaging. Spin-echo images were obtained in two orthogonal views (short-axis and four-chamber) perpendicular to the plane of the ASD. Spin-echo major and minor diameters were measured, and spin-echo defect area was calculated. Phase-contrast cine MR images were obtained in the plane of the ASD, and cine major diameter and defect area were measured from the region of signal enhancement or phase change due to shunt flow across the defect. MR measurements were compared with templates cut during surgery to match the defect or with ASD diameter determined by balloon sizing at catheterization. ASD size measured from cine MR images (y) agreed closely with catheterization and template standards (x). For major diameter, y = 0.78x + 5.7, r = .93, and SEE = 3.4 mm. On average, spin-echo measurements overestimated major diameter and area of secundum ASDs by 48% and 125%, respectively. CONCLUSIONS: Phase-contrast cine MR images acquired in the plane of an ASD define the defect shape by the cross section of the shunt flow stream and allow noninvasive determination of defect size with sufficient accuracy to permit stratification of patients to closure of the defect by catheter-based techniques versus surgery. Spin-echo images, on the other hand, are not adequate for defining ASD size, because septal thinning adjacent to a secundum ASD may appear to be part of the defect.

Magnetic resonance imaging of thoracic aortic aneurysms: comparison with other imaging methods.

Fifteen patients with thoracic aortic aneurysms had magnetic resonance images (MRI) and at least one additional diagnostic image study: thoracic aortography, computed tomography (CT), or two-dimensional echocardiography. Twenty aneurysms were demonstrated by MRI, 19 by the other studies. One small saccular aneurysm was missed by CT. There was complete agreement between MRI and other studies regarding aneurysm morphology, and good correlation in diameter measurements of the aneurysms and at multiple additional aortic levels.

Perioperative anesthetic risk of noncardiac surgery in hypertrophic obstructive cardiomyopathy.

To determine their perioperative risk, we reviewed the records of 35 patients with hypertrophic cardiomyopathy diagnosed by cardiac ultrasound and/or catheterization who underwent general (52) or spinal (four) anesthesia--a total of 56 major surgical procedures. There were no operative or related perioperative deaths and no significant ventricular tachyarrhythmias. Intraoperative or postoperative complications included: myocardial infarction with heart failure in one patient who also had coronary artery disease and was one of three patients who had spinal anesthesia, arrhythmia requiring therapy in eight, and angina during supraventricular tachycardia in one. We conclude that the risk of general anesthesia and major noncardiac surgery is low in patients with hypertrophic obstructive cardiomyopathy. Spinal anesthesia, which decreases systemic vascular resistance and increases capacitance, may be relatively contraindicated. Concomitant coronary artery disease may increase the risk.

Incidence and significance of primary abnormalities of cardiac rhythm in infants at high risk for sudden infant death syndrome.

The exact relationship between cardiac arrhythmias and sudden infant death syndrome (SIDS) is uncertain. Several reports have implicated both ventricular and supraventricular arrhythmias in isolated cases, but there have been no studies of the incidence or type of arrhythmias that occur in populations at risk for SIDS. Of 1699 infants at high risk for SIDS, 60 (4%) were found to have a primary cardiac arrhythmia (i.e., not associated with disordered respiration or apnea). The incidence of atrial and ventricular premature beats, supraventricular tachycardia, and Wolff-Parkinson-White syndrome was similar to the incidence found in normal infants. Primary bradycardia (defined as a heart rate less than 60 for greater than 10 s not associated with abnormal respiration) was the most common arrhythmia, occurring with a frequency and severity not seen in normal infants. Thirty-two infants experienced periodic bradycardia. In 19 of these latter infants, there were symptoms associated with these bradyarrhythmias that necessitated treatment. Heart rates as low as 20 beats/min were recorded. One infant presented with an episode of ventricular fibrillation and on further evaluation was noted to have recurrent bradyarrhythmias. In no infant was there abnormal prolongation of the QT interval. Primary bradyarrhythmias are seen at an increased incidence in infants at high risk for SIDS and may play a causal role in this syndrome. Most symptomatic infants can be adequately controlled with sympathomimetic or parasympatholytic therapy. Other cardiac arrhythmias occur at a rate similar to that in normal infants and are therefore unlikely to play a major role in SIDS.

Exercise testing in asymptomatic or minimally symptomatic aortic regurgitation: relationship of left ventricular ejection fraction to left ventricular filling pressure during exercise.

Exercise radionuclide angiography is being used to evaluate left ventricular function in patients with aortic regurgitation. Ejection fraction is the most common variable analyzed. To better understand the rest and exercise ejection fraction in this setting, 20 patients with asymptomatic or minimally symptomatic severe aortic regurgitation were studied. All underwent simultaneous supine exercise radionuclide angiography and pulmonary gas exchange measurement and underwent rest and exercise measurement of pulmonary artery wedge pressure (PAWP) during cardiac catheterization. Eight patients had a peak exercise PAWP less than 15 mm Hg (group 1) and 12 had a peak exercise PAWP greater than or equal to 15 mm Hg (group 2). Group 1 patients were younger and more were in New York Heart Association class I. Group 1 patients also had a higher mean rest ejection fraction (0.64 +/- 0.08 vs 0.49 +/- 0.13, p less than 0.01, higher exercise ejection fraction (0.63 +/- 0.10 vs 0.40 +/- 0.18, p less than 0.01), lower end-systolic volume (38 +/- 13 vs 79 +/- 36 ml/m2, p less than 0.01) and higher peak oxygen uptake (24.9 +/- 5.1 vs 16.6 +/- 4.9 ml/kg/min, p less than 0.01) than group 2 patients. However, the two groups had similar cardiothoracic ratios, changes in ejection fractions with exercise, and rest and exercise regurgitant indexes. Using multiple regression analysis, the best correlate of the exercise PAWP was peak oxygen uptake (r = -0.78, p less than 0.01). No other measurement added significantly to the regression. When peak oxygen uptake was excluded, rest and exercise ejection fraction also correlated significantly (r = -0.62 and r = -0.60, respectively, p less than 0.01). Patients with asymptomatic or minimally symptomatic severe aortic regurgitation have a wide spectrum of cardiac performance in terms of the PAWP during exercise. The absolute rest and exercise ejection fraction and the level of exercise achieved are noninvasive variables that correlate with exercise PAWP in aortic regurgitation, but the change in ejection fraction with exercise by itself is not.

Cerebral hemorrhage in neonates with coarctation of the aorta.

Coarctation of the aorta is an uncommon cause of cerebral hemorrhage in the full- or near-term infant. The clinical, radiologic, and neurologic findings of four infants with aortic coarctation and cerebral hemorrhage are presented. In all four infants, cerebral hemorrhage was associated with only moderate elevation of systolic blood pressure (90-110 mmHg).

Aberrant origin of the left coronary artery from the proximal right coronary artery: diagnostic features and pre- and postoperative course.

In this report, we present the pre and late postoperative course of a patient with severe angina secondary to aberrant origin or the left coronary artery from the proximal righ coronary artery (Fig. 1). We illustrate the noninvasive diagnosis and evaluation of this patient by two-dimensional ultrasound and stress thallium imaging, and the pre and late postoperative angiographic and thallium perfusion findings.

Ischemic heart disease in systemic lupus erythematosus in the young patient: report of six cases.

To clarify the clinical spectrum of coronary arterial abnormalities in systemic lupus erythematosus, the data were reviewed on six patients who had a diagnosis of lupus at ages 15 to 29 years and who had ischemic heart disease before age 35. Two patients had coronary arteritis diagnosed on postmortem examination. In a third patient alterations in coronary arterial anatomy occurred with angiographic improvement temporally related to the initiation of steroid therapy. The other three patients had severe diffuse atherosclerotic coronary disease that was identified in two at postmortem examination. In the third patient the course of the disease strongly suggested coronary atherosclerosis, and eventually coronary bypass grafting was performed for relief of angina. In summary, clinically important extramural coronary arteritis and atherosclerosis both occur, although rarely, in young patients with lupus. Coronary artery disease may occur with or without coexisting active extracardiac lupus manifestations. Short-term steroid therapy and follow-up angiography for those with angina and in whom coronary arteritis is suspected warrant consideration. When stable coronary arterial anatomy is demonstrated on follow-up angiography, management is determined by the patient's symptoms irrespective of the prior history of lupus and, if indicated, cardiac surgery for symptomatic relief can be safely performed.

Multifocal or chaotic atrial rhythm: report of nine infants, delineation of clinical course and management, and review of the literature.

Nine infants with episodic or continuous chaotic atrial rhythm (CAR) are presented. In addition to 3 or more different P-wave contours, atrial rates greater than 100 per minute, variable PP, RR, and PR intervals, and a discrete isoelectric baseline, findings included atrial rates that varied from a low of 50 to 120 to a high of 140 to 270 per minute, ventricular rates that varied from a low of 40 to 50 to a high of 180 to 270 per minute, and periodic sinus arrest with junctional escape rhythm. Except for the arrhythmia, all had a normal cardiac examination, ECG, chest x-ray film, and echocardiogram. Six infants were otherwise normal; one had an orbital rhabdomyosarcoma; one had neonatal asphyxia; and one had respiratory distress, bronchopulmonary dysplasia, and an intraventricular cerebral hemorrhage. The CAR persisted from 3 days to 20 months; it spontaneously reverted to normal sinus rhythm in 8 infants and persists in 1 infant at age 7 months. Digoxin (4 patients), propranolol hydrochloride (3 patients), quinidine sulfate (2 patients), and lidocaine (1 patient) did not alter the CAR. No patient had heart failure secondary to the CAR, although three also had episodes of sustained atrial tachycardia, which while present caused heart failure. All patients are functioning normally at home and have normal findings on cardiac examination and have normal ECGs at ages 3 to 38 months. Seven are in normal sinus rhythm, one has rare atrial premature contractions, and one has persistent CAR. We conclude that specific treatment was not necessary in these infants with CAR, except in those with associated sustained atrial tachycardia, which itself may cause heart failure.

Severe mitral regurgitation: a common occurrence in the aging patient with secundum atrial septal defect.

Severe mitral valve regurgitation (MR) which necessitated mitral valve replacement was identified in 19 (3.9%) of 498 consecutive patients (age range 1-83 years) with secundum atrial septal defects (ASD). The incidence of severe MR was significantly higher in patients older than age 50 years, 15 of 98 (15%), than in patients either below 21 years, 1 of 213 (0.4%), or between ages 21 to 49 years, 3 of 187 (2%). The higher frequency and severity of MR in the older ASD patient has not previously been appreciated. The morphology of severe MR in the older ASD patient consists of fibrous thickening and deformity of the mitral leaflets with shortening and thickening of the chordae tendineae. Because of the rarity of severe MR in the young patient with ASD, the mitral valve pathology is still poorly defined.