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.

Neonatal intestinal obstruction with isolated levocardia.

An unusual case of isolated levocardia, duodenal obstruction, and malrotation with volvulus is reported. Three cases of situs inversus abdominus with malrotation with volvulus have been reported to date. The current case is the fourth case and only the second survivor in the series. In cases of situs inversus abdominus, because of inversion of viscera, the volvulus may occur in an anticlockwise direction and, thus, should be derotated in a clockwise direction.

Anatomic repair of corrected transposition or atrio-ventricular discordance: report of 8 cases.

BACKGROUND: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of 'corrected transposition' or atrio-ventricular discordance (AVD) combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). METHODS: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 left ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. RESULTS: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3-122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. CONCLUSIONS: Despite a more demanding procedure, the 'anatomic repair' of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition.

Intraatrial baffle repair of isolated ventricular inversion with left atrial isomerism.

Isolated ventricular inversion with left atrial isomerism, partial anomalous pulmonary venous connection, and interruption of the inferior vena cava with azygos continuation to a right superior vena cava was diagnosed by echocardiography in a neonate. At 48 days of age, the patient underwent successful anatomic correction with redirection of flow from the superior vena cava and hepatic veins to the left-sided tricuspid valve, and flow from the pulmonary veins to the right-sided mitral valve. In the present report, the surgical techniques of this case are described, along with a survey of the surgical literature covering anatomic repair of isolated ventricular inversion.

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.

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.

[Acute lithiasic cholecystitis, situs viscerum inversus, vena porta preduodenale (considerations on a clinical case)]. / Colecistite acuta litiasica, situs viscerum inversus, vena porta preduodenale (considerazioni circa un caso clinico).

Authors report a case of preduodenal portal vein in a woman 68 years old with acute cholecystitis in situs viscerum inversus adbominis and levocardia. Surgery was difficult. They shortly analyze also etiology and clinical manifestations of situs inversus and PPV, with related malformations. They conclude that it is important to recognize the presence of situs inversus preoperatively and everytime suspect other anatomical anomalies.

Repair of combined total anomalous pulmonary venous connection and anomalous systemic connection in early infancy.

A simple intraatrial reconstruction can be utilized in infants to simultaneously correct both the systemic and pulmonary venous returns. Two infants with polysplenia syndrome treated in the first months of life are presented. One had levocardia and combined total anomalous pulmonary venous connection (type II-Cardiac) and anomalous systemic venous connection. The other had dextrocardia L-loop, L-normal great arteries, and combined total anomalous pulmonary venous connection (type II-Cardiac) and anomalous systemic venous connection. The posterior position of the systemic atrioventricular valve and systemic ventricle in both patients allowed the use of this reconstructive technique. The extensive size and transverse position of this patch when used in the infant may require both continued postoperative controlled ventilation until it stiffens and prolonged attention to postoperative cardiac care until adaptation of the infant heart to the new atria can occur. Early restudy and reoperation should be performed if signs of stenosis develop.

Congenitally corrected transposition of the great arteries: a clinical and surgical study.

From 1967 to 1979, 40 patients with the diagnosis of congenitally corrected transposition of the great arteries (C-TGA) have been followed. Associated cardiac defects were present in all but one patient, most frequently ventricular septal defect (80%), and pulmonary stenosis (70%). Left sided atrio-ventricular valve dysfunction developed in 25%, third degree atrio-ventricular block (at least intermittently) in 33% of the patients. Twenty-eight patients were operated: palliative procedures were done in 6, corrective operations in 22 patients. Ten of the 40 patients have died during a mean observation period of 4 years: 4 early postoperatively, 3 late postoperatively and 3 non-operated patients. Sudden unexplained cardiac arrest has been the most frequent cause of death (2 late postoperative and 2 non-operated patients). The incidence of residual ventricular septal defects and residual pulmonary stenosis after corrective surgery has been relatively high owing to the complex anatomy in these patients. Also reconstruction of the atrio-ventricular valves has been difficult because of severe deformities, particularly of the left side. It is concluded that in C-TGA the pacemaker should be implanted early, at the first sign of AV-conduction disturbance. Since the relief of the pulmonary stenosis is difficult, the operation should be deferred until an adequate-sized conduit can be implanted.

Immunodeficiency associated with laevocardia, bronchiectasis, and paranasal sinus anomalies.

In a case of laevocardia, bronchiectasis, and paranasal sinus abnormalities, assessment on 2 occasions showed the presence of moderate immunodeficiency. Serum concentrations of IgG and IgM were low, and serum and salivary IgA was not detected. T-lymphocytes were reduced in number and cell-mediated immunity in vivo and in vitro was impaired. Opsonisation, complement system, and neutrophil functions were normal.

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.