Employment and insurance for young adults with congenital heart disease.

OBJECTIVE: To determine the life and health insurability and employability of young adults with congenital heart disease. DESIGN: Questionnaire study. SETTING: Cardiac department of a tertiary referral hospital for children. PATIENTS: Young adults 18-30 years old with a variety of congenital heart defects, both simple and complex, including postoperative patients. MAIN OUTCOME MEASURES: Availability of insurance at normal or high rates, with or without special conditions or exclusions. Prospects for employment. RESULTS: Questionnaires were sent to eight large life insurance companies, five health insurance companies and, 15 employers, and 26 replies were received (93%). The consensus for life insurability was that young adults with mitral valve prolapse without regurgitation, postoperative ductus arteriosus, and aortic coarctation were insurable at standard rates. Those with any of the other heart defects listed were either insurable at high rates, or in the case of many lesions, not insurable at all. The consensus for health insurance was that insurance was available, but with complete exclusion of benefit for the cardiac disorder. Employment prospects were good for those with simple defects, but poorer for those with complex lesions. CONCLUSIONS: Prospects for insurance and employment for young adults with complex congenital heart lesions are poor. Inconsistencies found in insurance and job policies may be due to lack of appropriate guidelines for the outcome of young adults with corrected and uncorrected congenital heart disease.

Assessment of residual right ventricular outflow tract obstruction following surgery using the response to intravenous propranolol.

The response to intravenous administration of propranolol hydrochloride was studied in 24 children undergoing operation for isolated or complex right ventricular outflow tract obstruction. Ten had pulmonary valve stenosis, 4 had pulmonary valve stenosis plus ventricular septal defect, 8 had tetralogy of Fallot, and 2 had complex lesions. Propranolol (0.01 to 0.2 mg per kilogram of body weight) was given to patients with a residual right ventricular to left ventricular systolic pressure ratio greater than 0.75. Four children did not respond to propranolol and required further surgical intervention. Twenty patients responded, 2 of whom died. Fifteen were restudied by cardiac catheterization 3 weeks to 27 months postoperatively. The right ventricle-pulmonary artery gradient was 25 mm Hg or less in 13 patients. We conclude that a small dose of propranolol given intraoperatively can identify patients in whom a reduction in the right ventricle-pulmonary artery gradient to acceptable levels will occur in the months following operation. This may reduce the need for placement of a subannular or transannular patch in some patients.

Holter monitoring in assessment of angina pectoris.

Holter monitoring of ST-segment changes is a unique method of studying the character of transient myocardial ischemia that occurs during ordinary daily life. The electrocardiographic signal is a reliable marker of ischemia in defined populations of patients with angina and coronary disease, but should be interpreted with caution outside of these groups. Detailed studies in patients with chronic stable angina have shown that transient ischemia is frequently silent and prolonged, and may occur without evidence of physical exertion. Analysis of underlying changes in regional myocardial perfusion using rubidium-82 and positron tomography has shown that a decrease in myocardial perfusion (supply) is involved in the genesis of many episodes of ischemia during daily life. Clinical trials have shown that drugs that affect demand and supply are efficacious against both painful and painless ischemia and that combinations of agents can provide useful benefits. There is, however, marked natural variability in disease activity despite "stable" symptoms, which must be taken into account in individual patient assessment and the rational design of clinical trials. Ambulatory monitoring permits quantitation of previously unrecognized myocardial ischemia, and treatment can thus be assessed in terms of ischemic activity during everyday life rather than on data obtained during brief hospital visits. An active approach to the detection and monitoring of transient ischemia with and without pain will be necessary if prospective clinical research shows that treatment of silent myocardial ischemia can prevent myocardial damage and improve prognosis.

Different mechanisms for the relief of angina after coronary bypass surgery. Physiological versus anatomical assessment.

To determine the physiological effect of coronary artery bypass surgery and the mechanisms for pain relief, 15 patients with exertional angina were studied before and after operation. Before the operation conventional tests included exercise tests (all positive) and coronary angiography (all patients had greater than or equal to 70% stenosis of major vessels). In addition, ambulatory electrocardiographic monitoring during 48 hours detected 92 episodes (greater than or equal to 1 mm) of ST depression. Regional myocardial perfusion was assessed with positron tomography using rubidium-82 (t1/2 78 s) and this showed reversible inhomogeneity with absolute regional reduction of cation uptake after exercise in all 15 patients. After coronary surgery 10 of the 15 patients had (a) no angina, (b) patent grafts (three or more), (c) no evidence of ischaemia during ambulatory monitoring out of hospital, and (d) homogeneous perfusion with reversal of the disturbances in regional myocardial perfusion after exercise. After operation one of the 15 patients had no angina and showed silent infarction in the segment that was previously ischaemic but supplied by a patent graft. All but one of the remaining patients had no angina, patent grafts, but disturbances of regional myocardial perfusion with silent ischaemia on exercise. Two of these patients continued to have asymptomatic and ischaemic episodes of ST depression during ambulatory monitoring out of hospital. This physiological study of regional myocardial perfusion in patients in hospital and in those with ischaemia out of hospital showed that three different mechanisms may account for the relief of pain--improved perfusion, infarction, and silent ischaemia. Silent ischaemia in particular raises puzzling pathophysiological and therapeutic questions that may affect prognosis and the interpretation of clinical trials.

Assessment of right ventricular function and anatomy using peripheral vein infusion of krypton 81m.

A method for imaging the right side of the heart (atrium, ventricle and main pulmonary artery) and for assessment of RV systolic function (ejection fraction and ejection rate) is described. An ultra-short-lived isotope (81Krm) is continuously eluted in 5% dextrose and infused into an ante-cubital arm vein; standard multigated images are acquired using a gamma camera and commercially available software. Preliminary evaluation of the method in 55 subjects (20 with repaired tetralogy of Fallot, 14 with dilated cardiomyopathy and 21 normal volunteers) showed that the technical success rate was 100%; that RV boundaries free from LV overlap can be clearly visualised due to efficient exhalation of 81Krm through the lungs and that 81Krm measurements of RVEF are reproducible. The technique offers considerable potential for serial non-invasive assessment of RV function.