Familial nonobstructive cardiomyopathy with endocardial fibroelastosis beyond infancy.

A 10-year-old boy with congestive heart failure died in five months in spite of comprehensive medical treatment. Autopsy showed patchy areas of endocardial fibroelastosis of the left ventricle. The sister of this patient had followed a similar course at 13 years of age with death within six months of the onset of congestive failure. Her postmortem examination also showed endocardial fibroelastosis. The clinical presentation of familial endocardial fibroelastosis in the preteen and teenage years is a rare event. Probably the endocardial fibroelastosis was secondary to a familial nonobstructive cardiomyopathy.

Use of contrast echocardiography in the diagnosis of partial anomalous pulmonary venous connection.

Contrast echocardiography is useful in diagnosing the presence of partial anomalous pulmonary venous connection, particularly when it occurs in association with an atrial septal defect. Injections of saline solution, Cardiogreen or the patient's own blood were made in the inferior vena cava, left atrium and pulmonary veins of 27 patients. In all patients, the inferior vena caval injections showed only right heart clouding with no evidence of a right to left shunt. In 21 patients, the left atrial and pulmonary venous injections showed clouding of both the right and left heart structures, indicating a left to right atrial shunt and normal pulmonary venous connection. In six patients, the left artrial injections showed right and left heart clouding, but the right pulmonary venous injections showed only right heart structures, indicating a left to right atrial shunt with partial anomalous pulmonary venous connection. Contrast echocardiography proved to be a sensitive method of diagnosing the anomalous venous connection without the use of dye curves. The method is not useful when a right to left atrial shunt is present and may show false positive results for partial anomalous pulmonary venous connection it left atrial visualization is not adequate during injection into the pulmonary vein.

Masked abnormal drainage of the inferior vena cava into the left atrium.

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.

Cardiac tamponade secondary to chylopericardium following cardiac surgery: case report and review of the literature.

Only four instances of isolated chylopericardium following cardiac operation have been reported previously. We encountered this complication in a young patient who was operated on for infundibular pulmonary stenosis. The etiology appears to be related to a combination of intraoperative lymphatic injury and catheter-related subclavian vein thrombosis, the latter potentially elevating pressure within the thoracic duct and thereby resulting in an accumulation of chyle within the pericardial space. Therapy for this problem usually includes partial pericardiectomy with or without ligation of the thoracic duct.

Saphenous vein graft growth 13 years after coronary bypass in a child with Kawasaki disease.

The presumed limited growth potential of saphenous vein grafts has led many authorities to discourage their use in young children. We documented excellent growth and patency of a saphenous vein graft 13 years after operation in a 7-year-old child with coronary artery obstruction caused by Kawasaki disease.

Anomalous right ventricular muscle bundle: clinical pitfalls and extracardiac anomalies.

Seven patients with anomalous right ventricular muscle bundle are presented to emphasize possible pitfalls in the clinical diagnosis as based on physical examination, chest x ray, and electrocardiogram. Echocardiography, on the other hand, revealed abnormalities in all four of the patients on whom the study was done. The echocardiogram was directly responsible for a change in diagnosis and referral for a cardiac catheterization in one patient who had been followed for 19 years with the clinical diagnosis of a small ventricular septal defect. Four of the seven patients with anomalous right ventricular muscle bundle had extracardiac anomalies, one girl with an absent left thumb, and three patients, one girl and two boys, with left-sided renal anomalies. This high incidence of renal anomalies has not been previously reported in clinical or postmortem series.

Prognostic value of the electrocardiogram in endocardial fibroelastosis.

Nine children with endocardial fibroelastosis were followed from the time of admission with congestive heart failure until either death or discharge. Review of multiple clinical features showed that only the electrocardiographic pattern could be correlated with death or survival. The presence of a delayed transition zone with anterior force loss on the initial electrocardiogram ('infarct pattern') was noted in all the children who died. Progression of these changes with a pattern of anterolateral 'infarct' in two and inferior wall 'infarct' in two occurred before death. Necropsy on three of the four children confirmed the diagnosis of endocardial fibroelastosis. There was extensive fibrosis and thinning of the left ventricular myocardium as well as involvement of the mitral valve structures. Review of published cases supports the view that an 'infarct' pattern in a child with endocardial fibroelastosis is usually associated with death and that this pattern is a negative prognostic sign for survival.

Severe hemolysis following partial coil-occlusion of patent ductus arteriosus.

Severe hemolysis occurred in a one-year-old boy following partial coil occlusion of a patent ductus arteriosus with a small but high-velocity residual shunt. Hemolysis abated rapidly and completely after placement of a second coil fully occluded the ductus.

Postoperative responses in 'prepared' child after cardiac surgery.

Postoperative reactions of children and adolescents realistically prepared for cardiac surgery were analysed for acute and long-term responses. No cases of delirium or psychoses occurred in the 60 children while they were in hospital. Fifty sets of parents and children answered questionnaires 6 months to 4 years after operation (36 under 1 year, 8 from 1 to 3 years, and 6 over 3 years) and assessment of these showed only long-term reaction. Evaluation of reactions is presented by age and in detail. The children aged 2 to 7 years had the greatest number of negative reactions and the only long-term reaction also came from this group. Children 8 to 11 years of age had the least number of negative reactions and the highest number of positive gains. Adolescents, 12 to 19 years of age, were often concerned with their body image (wanted to be considered 'well', resented the scar, etc). From the standpoint of emotional stability and after effects, the 8 to 11-year-old group seemed to do best.