Dynamic left ventricular outflow obstruction in situs inversus with levocardia.

A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.

Anatomically corrected malposition of the great arteries: two case reports.

Anatomically corrected malposition of the great arteries (ACMGA) is defined as parallel arising of aorta and main pulmonary artery (PA) roots although ventriculoarterial connection is normal. Abnormally related aorta arises from the left ventricle, while abnormally related PA arises from the right ventricle. It can be diagnosed with via echocardiography. In some cases, additional imaging modalities such as computerized tomographic angiography and magnetic resonance are required. In this article, we presented two cases of ACMGA, 5-month-old boy and 1-month-old girl. We wanted to point out the importance of differential diagnosis of other great artery anomalies from this rare pathology.

An alternative method to fenestrate the extracardiac fontan conduit in a patient with atrial situs inversus and levocardia.

This report describes the technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in a patient with levocardia and atrial situs inversus.

Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy.

Surgical mortality is high in children with visceral heterotaxy (VH), particularly if atrioventricular valve insufficiency, ventricular dysfunction, or aortic atresia is present. This study reviews the outcome of cardiac transplantation (CT) in infants and children with VH and congenital heart disease who are at high risk for standard palliative or corrective surgery. We reviewed CT outcomes in 29 children with VH, congenital heart disease, atrioventricular valve insufficiency, ventricular dysfunction, and/or aortic atresia. Median age at CT was 3.1 years. Cardiac surgery had been performed in 20 patients (69%) before CT. Follow-up since CT has been 8.5 +/- 2.2 years. Outcomes were compared with 45 children who underwent transplantation for dilated cardiomyopathy. Actuarial graft survival in the VH group at 30 days and 1, 5, and 10 years was 100%, 86%, 68%, and 50%, respectively, compared with 100%, 96%, 83%, and 68% in children who underwent transplantation for dilated cardiomyopathy (p = 0.12). Splenic status, cardiac position, age at CT, number of prior cardiac surgeries, or systemic venous anomalies were not predictors of mortality after CT. Cardiopulmonary bypass and graft ischemic times were longer in the VH group; time on the ventilator after CT, length of hospitalization, and rejection, infection, post-transplant lymphoproliferative disease, and transplant coronary artery disease rates were equal. Thus, CT is a viable alternative therapy for high-risk patients with VH, possibly offering improved survival over standard surgical management.

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.

[Aortic arch interruption with restrictive ductus arteriosus]. / Interrupção do arco aórtico com canal arterial restritivo.

Interruption of the aortic arch is an uncommon cardiovascular anomaly. It is usually associated with a large ductus arteriosus (DA) and interventricular septal defects. Two cases of one and two month old girls with restrictive DA are reported. The clinical, anatomical and surgical findings are discussed.

[A successful operation of isolated levocardia with left atrial isomerism, polysplenia and tetralogy of Fallot].

A four-year-old boy who had isolated levocardia associated with left atrial isomerism, polysplenia and tetralogy of Fallot underwent successful corrective surgery. In addition, these anomalies were complicated by interrupted inferior vena cava, azygos continuation, and total anomalous hepatic venous connection. Because of these systemic venous anomalies, cardiopulmonary bypass presented us some problems of venous cannulations. We used 4 direct venous cannulations; i.e., superior vena cava, persistent left superior vena cava through coronary sinus, azygos vein and hepatic vein. Reports of total correction in patients with isolated levocardia associated with tetralogy of Fallot are rare, and we report the case with some reviews of the related literature.

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.

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.