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At least 19 recordsLinked to original sources

Partial situs inversus: duodenal obstruction in a neonate with isolated levocardia.

An unusual case of duodenal obstruction in an infant with isolated levocardia is described. The following anatomic features were shown preoperatively: (1) duodenal obstruction; (2) right-sided stomach and spleen; (3) left-sided liver; (4) totally right-sided located colon in mirror image nonrotation; and (5) levocardia without any defect or dysfunction. The mentioned findings were verified intraoperatively and the cause of the duodenal obstruction was found to be an aberrant vessel. This case is a rare one regarding the lack of heart and spleen defects and the cause of duodenal obstruction. It also demonstrates the necessity of meticulous investigation and precise description of the anomaly and suggests individualization as far as the management of these cases is concerned.

Duodenal Obstruction

[Levocardia with spleen inversion, but without atrial transposition (author's transl)].

The case of a 14-year-old boy with levocardia is reported herein. By scintiphotography, a normal spleen was identified in the right upper quadrant of the abdomen. As angiographic and electrocardiographic considerations rule out the transposition of the atria, the patient must be a very rare case of levocardia with an inverted spleen, which is not associated with transposition of the atria.

Angiography

Absent tricuspid valve with aortic atresia in mixed levocardia (atria situs solitus, L-loop). A hitherto undescribed entity.

Clinical, angiographic and pathologic features are described in a 36-hour-old male infant with mixed (discordant) levocardia, aortic valve atresia, absent tricuspid (left atrioventricular) valve and resultant massive cardiomegaly. This is the first reported case of this type of complex in a heart with discordant chambers. A possible embryologic explanation is offered for the concomitant presence of semilunar valve atresia and absence of the tricuspid valve.

Aortic Valve

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.

Abnormalities, Multiple

[Levocardia].

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Adult

[Levocardia with ventricular inversion and a single atrium in normal position].

A 12-year child had complete heart block, an abdominal situs inversus with laevocardia. Further investigation demonstrated in addition an inferior vena cava ending into an azygos vein, a shunt at the atrial level and a pulmonary valve stenosis. At operation there was a lone atrium in normal position, a ventricular inversion without transposition of the great vessels. In spite of a corrective operation of the Mustard type, the child died the following day through haemorrhage and low output.

Autopsy

Double-outlet right ventricle associated with persistent common atriventricular canal.

A variant of double-outlet right ventricle (DORV) associated with common atrioventricular (A-V) canal was identified. As yet, this syndrome has not been successfully repaired. Of 16 patients with this diagnosis, 14 had the diagnosis confirmed at postmortem examination and two had the diagnosis confirmed by exploratory cardiotomy. The 16 patients were divided into two groups according to the position of the cardiac apex: group 1, ten patients with levocardia, and group 2, six patients with dextrocardia and ventricular inverson. When pulmonary stenosis was present, the clinical presentation was similar to that in tetralogy of Fallot, and when such stenosis was not, the presentation was similar to that in ventricular septal defect with bidirectional shunting. The electrocardiogram had the typical feature of common A-V canal. The right ventricular angiocardiogram had features typical of DORV. The left ventricular angiocardiogram revealed left ventricular outflow deformity typical of A-V canal in patients with levocardia but not in those with dextrocardia. Splenic anomalies were commonly seen (nine of the 16 patients). Asplenia was present in seven, and polysplenia in two. Ten patients had persistent left superior vena cava, seven had common atricum, six had anomalous pulmonary venous connection, and three had subaortic stenosis. Twelve of the 16 patients had pulmonary stenosis. Of the 16, 14 had common A-V canal (Rastelli type C) and two had a ventricular septal defect of the persistent common A-V type.

Adolescent

Long-time observations on the Blalock-Taussig operation IX. Single ventricle (with apex to the left).

This report concerns a 20 to 28 year follow-up of patients with a single ventricle with pulmonary stenosis and levocardia, a malformation which is frequently combined with a transposition of the great vessels. Only 24 cases were found among the 1037 cyanotic patients with decreased pulmonary blood flow operated on between January 1, 1945 and January 1, 1951. Of the 18 patients who survived surgery, one has been lost to follow-up, eight have lived for more than 20 years, and six for more than 25 years after their initial operation. The results of a Blalock-Taussig operation for a single ventricle are less satisfactory than for a tetralogy of Fallot. Two patients who were living at the close of the study have had no further surgery. Seven patients have had a second operation and four patients have had a third. Progressive cardiac enlargement has been common. The period of enlargement has varied from 10 months to more than 20 years. Three patients have had subacute bacterial endocarditis; two had multiple attacks. One patient died of a brain abscess and one of a purulent meningitis. Three women and four men have married. All four men have A.B. degrees and are gainfully employed.

Adolescent

Isolated ventricular inversion with double inlet left ventricle.

2 patients with viscero-atrial situs solitus, isolated ventricular inversion (IVI) and double inlet right-sided morphologic left ventricle are presented. Isolated ventricular inversion is a rare cardiac anomaly characterized by ventricular inversion, subpulmonary conus, and ventriculo-arterial concordance. Their angiocardiographic and pathologic features are presented, and the morphologic findings of the 9 patients in the literature with isolated ventricular inversion are reviewed. Of the 11 known patients with isolated ventricular inversion, levocardia was present in 10 and dextrocardia in 1; viscero-atrial situs solitus in 9 and inversus in 2; L-ventricular loop in 9 and D-loop in 2. The atrial septum was intact in 4. An intact ventricular septum was noted in only 2 patients while in 3, more than one ventricular septal defects were present, and 2 patients exhibited morphologic single ventricle. A solitary ventricular septal defect was noted in the remainder. Significant tricuspid valve abnormalities, including atresia, stenosis or hypoplasia with supravalvular fibrous ring were found in 7 patients. In 2 of these, both with significant obstruction at the tricuspid valve, both atrioventricular valves emptied into the morphologic left ventricle--thus isolated ventricular inversion with double inlet left ventricle. Pulmonary outflow tract obstruction was evident in only 3 patients. Total anomalous pulmonary venous return occurred twice and right juxtaposition of the atrial appendages once. Thus, while the patient with isolated ventricular iversion may present with clinical and hemodynamic features characteristic of classical transposition physiology the high frequency of significant associated anomalies would complicate this. Finally, the anomaly must be differentiated from the levo-transposition, isolated atrial inversion, and the anatomically corrected malpositions.

Adult

The straddling mitral valve: morphological observations and clinical implications.

The morphological features of 23 patients with straddling or overriding mitral valve are presented. Levocardia was present in 20 of 23; visceroatrial situs solitisu in 20 of 23, with 3 patients, 2 with asplenia and 1 with polysplenia, having visceral heterotaxia. A concordant D-ventricular loop was present in the 20 patients with visceroatrial situs solitus. Six of these had double outlet right ventricle; 2 had asplenia syndrome; 1 had D-transposition of the great arteries, ventricular defect and pulmonary atresia; 1 with tricuspid atresia and double outlet-outlet chamber; 1 with polysplenia syndrome; and 12 had endocardial cushion defect with marked underdevelopment of the left ventricle, and normally related great arteries. Left ventricular size was related to the amount of mitral valve (or left-sided component of a common atrioventricular valve) connected to it. In those patients in whom little effective mitral orifice was connected to the left ventricle, the left ventricle was diminutive. Endocardial fibroelastosis of the left ventricle was noted in only a single patient. Six of the 7 patients with double outlet right ventricle (including one with double outlet bulbus) had subpulmonary obstruction, and in one of these, this was related in part to the straddling mitral valve. In 1 patient with double outlet right ventricle, there was a double orifice mitral valve, and it was the accessory mitral orifice that straddled. The diagnosis of overriding mitral valve should be suspected in any patient with significant conotruncal anomalies and underdeveloped left ventricle, especially the patient with double outlet right ventricle, and in the patient with endocardial cushion defect, hypoplasia of the left ventricle, and obstructive anomalies of the aortic arch. In certain patients, selective left atriography, left ventriculography, and single and two dimensional echocardiography may be diagnostic of this condition.

Abnormalities, Multiple

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.

Cardiac Catheterization