Real-time wide-angle sector echocardiography: atrioventricular canal defects.
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Two hundred fifty-one patients with Down syndrome and congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients uncerwent cardiac surgery. Motality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.
Ten adult patients with Ebstein's anomaly had open-heart operation for interruption of a Kent bundle. The 4 patients in Group 1 had arrhythmias. One patient had a patent foramen ovale, which was closed. In 3 patients the Kent pathway was identified and interrupted, but in the other only the pathway's anterograde function was interrupted, leaving the patient subject to supraventricular tachycardia (SVT). The 3 patients in Group 2 had mild to moderate cyanosis and dyspnea during SVT as well as mild impairment during strenuous physical activity. Only interruption of their Kent bundles and closure of the patent foramen ovale were done. The 3 patients in Group 3 had dyspnea and cyanosis on exertion, and 1 had overt right heart failure. In each patient, obstruction between the atrialized right ventricle (RV) and functioning RV by the displaced tricuspid valve (TV) was relieved by valve excision and replacement. Interruption of the Kent bundle was successful in 2 of the 3 patients. Operation for Ebstein's anomaly is indicated when any of the following conditions are present: arrhythmias due to Kent bundles that are refractory to medical management; a defect in the atrial septum that must be closed because of a history of cyanosis or paradoxical emboli, or an arrhythmia that must be corrected by right atriotomy; and obstruction between the atrialized and functioning RV or a small functioning RV, both of which can be corrected by TV replacement.
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The present report is based on the study of 28 cases of pulmonary atresia with intact ventricular septum, all of them necropsically and/or angiographically verified. A microscopic study of the wall of both ventricles was performed in the 20 necropsy cases. The size of the right ventricular cavity, a fact of great surgical significance, did not correlate with the electrocardiogram and varied from diminute to very large. In two cases necropsically proved there was as associated atresia of the infundibular, in one of them existing additionally an Ebstein malformation of the tricuspid valve, which could be angiocardiographically diagnosed. The surgical mortality, in our hands, is 64.3%. All survivors have a small right ventricle.
It has been demonstrated that echocardiography is a useful and accurate noninvasive technique for the study of congenital cardiovascular diseases. This method provides significant information in that invasive cardiac catheterization and angiocardiography can be planned with greater facility. This is especially important in severely ill babies in whom invasive diagnostic methods are risky. It is particularly attractive in neonates in whom the clinical differentiation of lethal heart disease from septic shock is difficult, since in the latter situation the echographic findings are normal.
Echocardiography is an important noninvasive method for accurately diagnosing many congenital cardiac defects. This method provides significant information so that cardiac surgery, when necessary, can be planned with greater facility. The value of the sonar method rests not only on its ability to diagnose specific cardiac defects but also to exclude heart disease, especially in infants with shock or cyanosis from noncardiac causes. Development of two-dimensional real-time scanning systems should provide greater understanding of anatomic spatial relationships and ease of diagnosis.
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Wall thicknesses were measured and echo densities were evaluated from the left ventricular echograms of 182 patients. The echogram was considered to reflect scar tissue when 1) either the interventricular septum, the posterior left ventricular wall or the anterior left ventricular wall was less than 7 mm thick in mid-diastole and was more echo-producing than its opposing wall or another area of the same wall in a sector scan, or 2) an area of myocardium was 30% less thick than an adjacent area within a sector scan. Myocardial scarring was diagnosed by echocardiography in 52 of the 182 patients. The echocardiographic presence or absence of scarring was confirmed in 95% (173 of 182) of cases, 34 cases by microscopic examination and 139 by surgical appearance. This study shows that M-mode echocardiography is both a sensitive and specfic method for detecting myocardial scar tissue.
Management of symptomatic atrioventricular canal (AVC) in infancy may be difficult. Between July, 1969, and September, 1977, 31 infants with complete AVC presented in congestive heart failure (CHF) to the University of Minnesota Hospitals. Fifteen of these patients have responded to medical management and have been followed as outpatients. The other 16 patients remained in CHF. Six of them died of persistent heart failure within 4 months. The other 10 infants, aged 3 weeks to 1 year (mean 4 months), underwent pulmonary artery banding and seven survived operation. One of the survivors died with apparent pneumonia 1 month postoperatively. Each of the remaining six patients, who have been followed for 9 months to 9 years, had minimal mitral insufficiency and a large ventricular shunt. The three patients dying after banding had significant mitral insufficiency. We believe that pulmonary artery banding is an effective palliative procedure for infants with complete AVC and CHF who have large ventricular shunts and minimal mitral insufficiency.
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A surgical operation with a "curative" aim has been performed on four patients with a lone ventricle. It consisted of a IA type in 3 cases and IC type in one. There were one operative death and three long-term survivals. The operation consisted essentially in: a delicate step of partition of the lone cavity and the treatment of the often combined, and sometimes complex, heart malformation. The trauma to the conduction pathways is one of the major dangers of this type of operation and should be avoided or decreased by the detection during operation of the His bundle. Operation should be indicated in badly tolerated forms. Stenosis of the infundibulo-pulmonary pathway interferes with the development of a hypertensive pulmonary arteriolitis, seems to be a favourable element for surgical correction. In the absence of such stenosis, one should perform a banding early enough or discuss the indication for an intracardiac operation in relation with the pulmonary artery resistance.
Twenty cases of cor triatriatum are reported. The diagnosis was confirmed by necropsy in 16 cases and at the time of operation in 4. The lesion occurred as an isolated anomaly in 7 cases; in 13, other associated cardiac anomalies were present. Three anatomic types of cor triatriatum were identified in the cases studied at necropsy: diaphragmatic (10 cases), hourglass (3) and tubular (3). The diaphragmatic type was also present in all four cases in which the diagnosis was confirmed at operation. Associated anomalies were found in five cases of the diaphragmatic type and in each case of the hourglass of tubular types. In isolated cor triatriatum the clinical findings were characteristic of pulmonary venous and arterial hypertension. In two cases, one with a communication between the right atrium and the accessory left atrial chamber and one with partial anomalous pulmonary venous connection associated with cor triatriatum, the clinical findings suggested a large left to right shunt with pulmonary arterial hypertension. The clinical findings varied in the cases with associated anomalies, and it was difficult to determine the cause of disturbance of the circulation.
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Win 18,446, a bis(dichloroacetyl)diamine, is a drug that was previously shown to suppress spermatogenesis and to be an effective oral abortifacient in rats. The present study shows that the drug is capable of producing characteristic congenital malformations in high incidence, by a single treatment, and with high survival of fetuses to day 21. Gestation day 11 is the most sensitive time. The teratologies obtained after various schedules of treatment include malformations of the snout (100%), septal heart defects (100%) diaphragmatic hernias (100%), cryptorchism (100%), cervical pockets (100%) and absent or small irregular thymus (92%). Some of these data, namely of the heart, face and thymus, cluster in patterns that indicate that the action of the drug is upon a time-resistricted developmental process. These periods of sensitivity are subsiding or have ended before primordia of these structures appear, but they coincide with the proliferation and migration of those mesenchyme cells that will eventually form or contribute to the structures affected. It is postulated that the drug acts on these mesenchyme cells, or on the extracellular matrix that provides the necessary framework for their dispersal.