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.

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.

Repair of posterior left ventricular aneurysm in a six-year-old boy.

Left ventricular aneurysms and diverticula are rarely encountered in the pediatric age group. This paper reports a case of congestive heart failure and mitral regurgitation in a 6-year-old boy with a large posterolateral left ventricular aneurysm. Complete repair was successfully performed by excision of the aneurysm and Dacron patch reconstruction of the left ventricular free wall. The patch extended onto the posterior annulus of the mitral valve, thus restoring the mitral valve to normal geometry and correcting the mitral insufficiency. The surgical literature on congenital cardiac diverticula and acquired aneurysms in children is reviewed and summarized.

Echocardiographic patterns after mitral annuloplasty.

18 adolescents and children who underwent mitral annuloplasty for severe mitral regurgitation were evaluated by echocardiography. In 7 patients studied preoperatively, mitral valve excursion ranged from 25 to 56 mm with E-F slopes from 92 to 160 mm/sec. 5 of the 7 patients showed mitral valve prolapse. The left ventricle was dilated in 6 and the left atrium was dilated in 4 patients. Postoperatively, the mitral valve excursion decreased ( range 15-26 mm; p less than 0.01) as did the E-F slope (range 44-100 mm/sec; 0.10 greater than p greater than 0.05). The prolapse pattern disappeared and the left heart chambers decreased in size. In these 7 patients and in 11 others studied postoperatively only, a common echocardiographic pattern was that of early anterior motion of the posterior mitral valve leaflet, which was not present preoperatively and was, therefore, tentatively ascribed to the technique of annuloplasty repair. No calciferation and no definite mitral steosis has occurred in the follow-up of these patients to date. Persistent left atrial/left ventricular dilatation appeared related to residual mitral regurgitation with or without atrial fibrillation.

Successful excision of an atrial fibroma in a four-month-old male infant.

This report presents an infant who was initially admitted with a pericardial effusion. Subsequent evaluation led to the diagnosis of a primary cardiac tumor. Excision of the tumor which arose from the atrial septum was possible only by removing most of the free right atrial wall and a portion of the septal leaflet of the tricuspid valve. Reconstruction of the tricuspid valve leaflet by reattachment of chordae and a partial annuloplasty was done with the right atrial wall being replaced using pericardial tissue. The histology of the tumor was compatible with a fibroma, a type not previously reported in this location in the pediatric age group. Twenty-one months following operation, the child was restudied and the catheterization revealed mild to moderate tricuspid regurgitation and no recurrence of the tumor. The child is now 4 years of age and remains asymptomatic with normal growth and development and no evidence of fibromatosis.

Digoxin toxicity in a premature infant: treatment with Fab fragments of digoxin-specific antibodies.

The first use of Fab fragments to treat digoxin toxicity in a premature infant with renal failure, 18 h after the onset of severe arrhythmias, is reported with dramatic results. The development of digoxin toxicity in the context of accepted therapeutic dosing to treat heart failure due to a cerebral arteriovenous malformation is discussed.

Annuloplasty in children and young adolescents with severe rheumatic mitral insufficiency.

Eleven patients aged 8 to 15 years underwent measured asymmetrical annuloplasty for severe mitral regurgitation in the years 1961 through 1966. They had had a total of 20 attacks of acute rheumatic fever. The intervals between the last attack of acute rheumatic fever and operation ranged from 2 to 8 years. The criteria for surgery were congestive failure and progressive cardiac enlargement. Using the hydraulic formula of Gorlin, a mitral annuloplasty was tailored to the size of each patient so that insufficiency was eliminated without producing hemodynamically significant stenosis. In this group of 11 children there has been one death. The majority of our 11 patients reacquired murmurs of mitral regurgitation. Satisfactory results, however, are not dependent on complete hemodynamic correction. All patients have improved remarkably and have sustained this improvement up to 7 years. These results suggest that mitral annuloplasty should be the operation of choice in children with severe mitral regurgitation.

Churg-Strauss syndrome. Development of cardiomyopathy during corticosteroid treatment.

A patient with Churg-Strauss syndrome had pulmonary lesions, vasculitis, gastrointestinal tract involvement, and leukocytosis with hypereosinophilia. A wedge section from the lung showed necrotizing granulomata filled with necrotic eosinophils and fibrinoid, and rimmed by giant cells and eosinophils. Arteries and veins of all sizes were affected with perivascular eosinophilic-rich, palisaded, giant cell granulomata with evidence of partial or complete occlusion. The patient was treated with prednisone, with excellent resolution of his symptoms. Recurrent episodes of pneumonia and wheezing responded to increased levels of prednisone plus bronchodilators and antibiotics. Cardiomyopathy developed after two years while taking prednisone. His condition has been managed with digoxin, diuretics, and increased amount of prednisone. Although our patient has shown clinical improvement in his cardiac status, objective studies show minimal resolution.

Use of saphenous vein allografts for aortopulmonary artery anastomoses in neonates with complex cyanotic congenital heart disease.

A saphenous vein allograft was used to create an aortopulmonary communication in 16 infants with cyanotic congenital heart disease and ductus-dependent pulmonary blood flow. These grafts measured from 3 to 8 mm in diameter and were placed between the aorta and main pulmonary artery in eight patients, between aorta and right pulmonary artery in eight, and between aorta and left pulmonary artery in one (one child had two grafts). Before heparin was used, early in the series, four of these grafts occluded and three of the four infants died during attempted revision. Another infant died early from renal failure. Late mortality has claimed four: one from cerebral hemorrhage, two from hypoxia, and one at open-heart surgery for repair. There are eight late survivors (50%). Most of the allografts were used before small diameter Gore-Tex was available; in more recent patients, 4- to 6-mm Gore-Tex grafts have been used. In our most recent patient, however, the attempt to place a Gore-Tex graft was unsuccessful, but the more pliable saphenous vein graft was readily placed and an adequate shunt obtained. Both the saphenous vein graft and the Gore-Tex have the advantage of providing pulmonary flow without the higher risk of congestive failure or pulmonary hypertension seen in patients with a Waterston or Potts anastomosis. They are easier to perform, require less anesthesia time than the Blalock-Taussig shunt, last as long as the Blalock-Taussig when done under similar conditions, and are easy to take down at the time of total repair.