Ricardo Ceballos

Congenital pulmonary vein stenosis

Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected. Of the 8 in whom the condition was diagnosed during life, it was suspected clinically in 6 and found unexpectedly at cardiac catheterization in 2. All underwent operation, and 5 were hospital survivors. In all survivors rapid and progressive restenosis of the pulmonary veins occurred over the next several months. Three of the 5 underwent reoperation, but progressive restenosis recurred and all eventually died of this condition. Thus, despite partial surgical relief of pulmonary vein stenosis, the lesion is apparently one of relentless progression. No surgical repair has been successful in the cure or long-term palliation of this lethal lesion.

Tetralogy of Fallot: an angiographic-pathologic correlative study.

The anatomic abnormalities observed by cineangiographic axial techniques of 12 patients with tetralogy of Fallot were correlated with anatomic details noted at necropsy. Right ventricular angiograms made in the right anterior oblique view best demonstrated the severity and type of infundibular obstruction and also permitted differentiation of the perimembranous, infundibular muscular and subarterial types of ventricular septal defects. The degree of aortic overriding was best displayed in the long-axis view. Comparison of the intracardiac anatomy of each postmortem specimen with the respective premortem cineangiogram has provided further clarification of the angiographic anatomy displayed by these axial techniques.

Angiographic anatomy of the normal heart through axial angiography.

We sectioned a series of hearts in a manner similar to that seen on angiographic axial views. A correlation with normal angiograms to identify the anatomic components of the four cardiac chambers showed that the components of the normal cardiac anatomy can he identified accurately through axial angiography in a manner not shown previously.

Results of Radionuclide Assessment of Cardiac Function Following Transplantation of the Heart

Radionuclide assessment of ejection fraction was determined early and late postoperatively following cardiac transplantation in 16 patients. In 11 patients, ejection fraction was determined within 48 hours of an endocardial biopsy. There was no relationship between the severity of histologically evident rejection and the ejection fraction (Pearson correlation coefficient [r] = -0.11; p = 0.47). In 2 patients, severe graft fibrosis developed with consequent diminution in ejection fraction. There was no relationship between severity and duration of rejection or the amount of immunosuppression required to treat acute rejection and the development of graft fibrosis. The mean resting ejection fraction in 7 patients in follow-up ranging from 6 to 21 months after transplantation was 0.59 +/- 0.06 (standard deviation), and the mean exercise ejection fraction in 6 of these patients was 0.72 +/- 0.08. Radionuclide-determined ejection fraction is not predictive of rejection early after operation. During short-term late follow-up, systolic left ventricular function at rest and exercise has been retained at normal levels.

Aortic dissection masquerading as gastrointestinal disease

Presentation of aortic dissection (AD) typically includes chest and/or abdominal pain. Gastrointestinal (GI) symptoms other than abdominal pain are uncommon. Two patients with AD are described in whom the dominant presenting symptom was GI hemorrhage. Mesenteric infarction complicated acute Type I dissection in one patient whose clinicalo manifestations simulated ulcerative colitis. In the other patient an old, small asymptomatic Tupe III AD resulted in a false aneurysm in the retroperitoneum which ruptured into the duodenum. In the latter case an antemortem diagnosis was not made as angiography was limited to the visceral arteries and the abdominal aorta without appreciation of the significance of a focal compression of the abdominal aorta. The possibility of AD thus should also, be considered in the evaluation of a patient with acute GI bleeding.

Heterograft Replacement of the Mitral Valve: Clinical, Hemodynamic, and Pathological Features

Seventeen patients had replacement of their diseased mitral valves with formalin-fixed, mounted porcine heterografts over a ten-month period with no operative or postoperative mortality. Hemodynamic studies early after operation indicate that patients with heterograft mitral valves behave no differently than patients who have had replacement of the mitral valve with ball valves. The early postoperative myocardial dysfunction present after mitral valve replacement is probably not related to the presence of a caged ball or other such device in the left ventricle. Late hemodynamic studies in two patients have shown complete competence of the valves and minimal mean diastolic and absent end-diastolic gradients at rest between the left atrium and left ventricle. Six of the valves became incompetent and were removed from 2 to 11 months after operation. Histological study of these valves suggests that the development of incompetence in five of them was related to absorption of muscle beneath the right coronary cusp with retraction of the tissues supporting this cusp and subsequent avulsion of the adjacent tissues from the supporting frame. The 11 patients whose valves remain competent are all clinically improved 6 to 15 months after operation. There have been no thromboembolic complications in any of the patients although long-term anticoagulation has not been used. The failure of the aortic valve heterografts mounted on rigid frames in this series appears related to mechanical stress resulting from imperfect coaptation of the valve cusps, either from resorption of heterograft tissue or improper mounting. Further studies are necessary to clarify these observations.

Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn

SummaryA one-day-old full-term female infant with massive “cardiomegaly” developed marked respiratory distress soon after birth. The two-dimensional (2D) echocardiogram demonstrated a 4×5-cm cystic mass adjacent to the left ventricular free wall. Computed tomography (CT) confirmed the presence and dimension of the cyst and CT number indicated the fluid within the cyst to be serous. A pericardial cyst was successfully removed at thoracotomy.Two-dimensional echocardiography and CT, both noninvasive procedures, are fully adequate tools in the diagnosis of pericardial cysts in the neonate, so that invasive investigations may be avoided.

Long-Term Anatomical Results of Intracardiac Repair of Tetralogy of Fallot

Abstract Two adult patients with tetralogy of Fallot died in accidents 18 and 28 months, respectively, after corrective operations. Anatomical and histological studies of their hearts showed the Dacron patches used for repairing the ventricular septal defect to be securely incorporated into the ventricular septum and evenly coated by connective tissue, endothelium, and even elastic fiber formation. A similar connective tissue reaction had covered the right ventricular outflow tract in the areas of infundibular resection. One of the patients, whose defect had necessitated extensive remodeling of the pulmonary outflow tract, showed aneurysmal dilatation of the proximal pericardial bolster. The aortic homograft with its retained valve was well preserved, but distortion and incompetence of one leaflet had resulted from dilatation of the adjacent pericardial patch.