Thoracoatrial discordance in a case of twisted atrioventricular connections: A unique configuration of visceroatrial arrangement.

We present a case of a 12-year-old boy with discordance between the atrial arrangement and the thoracoabdominal arrangement in the setting of twisted atrioventricular connections. This case highlights the importance of a separate description of all visceral organs and venous drainage for an accurate description of visceroatrial arrangement in cases of congenital heart defects.

Ruptured Sinus of Valsalva Aneurysm Associated with Situs Ambiguus, Isolated Levocardia, and Polysplenia.

Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.

Prolonged survival with isolated levocardia and situs inversus.

Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients with congenital heart diseases. It is commonly associated with both congenital heart disease and splenic abnormalities, either asplenia or polysplenia. The prognosis is poor, and only about 5% to 13% of patients survive more than 5 years. The case described here, which had computed tomographic findings, is the first case reported with multiple malignancies associated with ILSI. This patient, at 73 years of age, is the second longest survivor reported in the literature.

Ventricular septal defect and subaortic stenosis: an analysis of 41 patients.

Forty-one patients with subaortic stenosis (SAS) and ventricular septal defect (VSD) were identified from the cardiac records of the Hospital for Sick Children, Toronto, Ontario. The diagnosis of an associated SAS was made clinically in only 1 patient, who had findings of left ventricular (LV) hypertrophy with strain on the electrocardiogram. There was a delay of 3.1 years between initial presentation and detection of SAS. The SAS was not diagnosed at initial catheterization in 17 patients and was confirmed at subsequent catheter studies in 8 patients, surgery in 5 and autopsy in 4. Associated defects included coarctation of the aorta in 12 patients, mitral valve abnormalities in 4, and right-sided obstructions, including anomalous right ventricular muscle bundles in 6 patients, tetralogy in 4 and pulmonic stenosis in 1 patient. The mean gradient across the LV outflow tract was 25 mm Hg. Nineteen patients had serial catheters without intervening surgery, and the outflow gradient increased from a mean of 9 to 36 mm Hg. The mechanism of SAS consisted of fibrous diaphragm and fibromuscular obstruction in 31 cases, muscular narrowing in 4, protruding tricuspid valve leaflet in 2, hypertrophic cardiomyopathy in 2, anterolateral twist in 1 patient and redundant tissue tag in 1. Thirty-eight patients had a perimembranous VSD, 19 of whom had an associated so-called aneurysm of the membranous septum; 2 had an infundibular VSD and 1 patient had a central muscular defect. Although the SAS was located below the VSD in 30 cases, the associated heart failure and reduced cardiac output can mask the presence or severity of associated SAS.(ABSTRACT TRUNCATED AT 250 WORDS)

Straddling right atrioventricular valve in criss-cross atrioventricular relationship.

Two cases of complex congenital heart disease are described in which systemic and pulmonary bloodstreams crossed at the atrioventricular level. Both patients were examined clinically, echocardiographically, and by cardiac catheterization, including angiography, and both underwent cardiac surgery and had intraoperative mapping of their conduction systems. Both patients were found to have levocardia and situs solitus of viscera and atria, large ventricular septal defect, and straddling right atrioventricular valve. One patient had atrioventricular discordance but with the left ventricle anterior and alightly on the right and with pulmonary atresia and dextromalposition of the aorta. The other patient had atrioventricular concordance but with the left ventricle inferior and slightly on the left and with ventricular-arterial concordance to normally related great arteries and banded pulmonary trunk. To our knowledge, this combination including straddling right atrioventricular valve has not been reported in the literature before. In both patients the straddling atrioventricular valve was thought to preclude corrective operation at that time. A unique palliative procedure--left ventricle-to-pulmonary trunk shunt--was successful in the first case and partial debanding of the pulmonary trunk in the second.

Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution.

Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy, pulmonary artery-aorta anastomosis, and pulmonary artery banding, have been performed in some patients. However, because of the distinct underdevelopment of the left heart, the long-term prognosis must be guarded even in the survivors of these procedures. Rarely, aortic valve atresia is found with adequate left heart chambers. Because very few of these patients are described, the clinical, angiocardiographic, and the pathological findings in two patients are presented, with comments on palliation in the neonatal period, and the potential for ventriculoaortic reconstitution.