Bargeron Lm

Surgical experience with unroofed coronary sinus.

Between January, 1967, and October, 1977, we performed intracardiac repair in 24 patients with unroofed coronary sinus. Eight patients had the so-called pure form of the syndrome, with the concomitant findings of total absence of the partition between the coronary sinus and left atrium, connection of the left superior vena cava (LSVC) to the upper left corner of the left atrium, and a coronary sinus type of atrial septal defect. In 2 additional patients this combination was repaired, along with repair of the common atrium in 1 and tetralogy of Fallot in the other. In 4 patients with either a partial or complete atrioventricular canal defect, only the distal or downstream portion of the sinus was unroofed so that the coronary sinus ostium was considerably to the left of its usual location. In 6 patients, 5 of whom had situs ambiguous, the unroofed coronary sinus was associated with complex congenital heart disease, and in 5 there was a L(contralateral) SVC. Three of these 6 patients died. The basic repair consists of roofing the coronary sinus from within the left atrium, so that the LSVC drains through the newly created coronary sinus and its ostium into the right atrium. In the absence of a LSVC, the basic repair is simply closure of the coronary sinus atrial septal defect. This leaves the coronary veins draining into the left atrium.

Survival and probability of cure without and with operation in complete atrioventricular canal

Actuarial analysis based on postmortem examination of patients who had been treated nonsurgically for complete atrioventricular (A-V) canal defect shows that only 54% survive to 6 months of age, 35% to 12 months, 15% to 24 months, and 4% to 5 years of age. Our surgical experience since 1975 in 39 patients confirms the idea that primary repair is feasible in small infants. The highest risk of hospital death is when the operation is done in the early months of life; it falls to 17% by age 12 months. Between 1967 and October, 1976, the five-year survival rate among patients leaving the hospital alive after repair was 91%. The age-specific probability of surgical cure of patients operated upon for complete A-V canal (alive five years later with mean pulmonary artery pressure less than 25 mm Hg) is maximal at 73% when the operation is done at about 14 months of age. Urgent earlier repair is frequently necessitated by the life history of the disease.

Repair of Complete Atrioventricular Canal Associated with Tetralogy of Fallot or Double-Outlet Right Ventricle: Report of 10 Patients

Ten patients with complete atrioventricular (AV) canal and tetralogy of Fallot or double-outlet right ventricle (DORV) with subaortic ventricular septal defect and pulmonary stenosis underwent complete repair. Associated cardiac anomalies were frequent, and 3 patients had situs ambiguus and 1 had situs inversus. Two (40%) of the 5 patients with comple AV canal and tetralogy of Fallot died in hospital, as did 3 (60%) of the 5 with complete AV canal and DORV. No deaths occurred among the 4 patients in whom repair could be done without using a transannular patch across the pulmonary valve ring or a valved external conduit. Five (62%) of the 8 without major associated malformations survived. Preoperative study must define completely the malformation. The repair, done through the right atrium alone or in combination with a right ventriculotomy, is most easily accomplished with separate patches for the ventricular and atrial components of the septal defect.

Left ventricular outflow tract obstruction in transposition of the great arteries: An angiographic study of 74 cases

Recent advances in techniques of angiocardiography now allow highly accurate assessment of the anatomy of the left ventricle in patients with transposition of the great arteries. The cineangiograms of 225 children with transposition were reviewed using axial cineangiographic techniques and 33 percent were found to have significant left ventricular outflow tract obstruction with or without coexisting ventricular septal defects. The varieties of left ventricular outflow tract obstruction and the associated ventricular septal defects found in this group of patients are described, illustrated and correlated. The advantages of axial angiocardiography in the diagnosis of transposition are pointed out, and the techniques used to produce the axial angiocardiograms are reviewed.

Double-outlet left ventricle with ventricular septal defect and pulmonary stenosis: Report of surgical repair

Abstract A case is reported of origin of both great arteries from the morphologic left ventricle, a rare malformation. There was also a subaortic ventricular septal defect and severe pulmonary stenosis. The patient presented as having the severe form of tetralogy of Fallot. Surgical correction was made.

Truncus arteriosus. An anatomical-angiographic study.

A series of 25 cases of truncus arteriosus communis examined post mortem was studied retrospectively. Fifteen of them had had angiographic studies, enabling anatomo-radiographic correlative analysis to be made. All cases had situs solitus of the atria and concordant connections between atria and ventricles. The three main components of the malformation, present in all 25 cases, were: (a) ventricular septal defect, (b) single semilunar valve, (c) anomalies of the aortopulmonary septum. The ventricular septal defect was always subarterial but its size and its relation to mitral and tricuspid valves were extremely variable. The truncal valve varied greatly also in the number of its cusps and its relation to right and left ventricles. Above valvular level the presence or absence of residual aortopulmonary septum was reflected by the presence or absence of a main pulmonary artery. Additional supratruncal malformations produced variations of the anatomy of the aorta and pulmonary arteries. The angiographic demonstration of all these components was obtained best by selective angiography using special projections. From this study it appears that there is a wide degree of variability in all of the three main components of truncus arteriosus. Such anatomical variations should be identified angiographically in each patient in order to provide enough information before corrective surgery, but they do not alter the basically homogeneous anatomy of the category of truncus arteriosus and therefore do not justify complex classifications.

Plain Film Findings of Anatomically Corrected Malposition: Its Association with Juxtaposition of the Atrial Appendages and Right Aortic Arch

Three cases of anatomically corrected malposition of the great arteries (ACM) are presented. The association of ACM with juxtaposition of the atrial appendages and right aortic arch is emphasized. The unique plain film findings of anatomically corrected malposition include: (a) 1-position of the ascending aorta; (b) discrete convexity of the mid-left heart border in association with findings of an abnormal right atrial border (left juxtaposition of the atrial appendages); and (c) right aortic arch. This unusual combination of findings on the postero-anterior chest should lead to a high degree of suspicion in regard to the diagnosis of anatomically corrected malposition.

Surgical treatment of complex forms of transposition.

In spite of the new knowledge developed in recent years surgical treatment for patients with complex forms of transposition of the great arteries remains suboptimal. This is because of the morbidity and mortality that have followed many of the corrective operations, and these are related chiefly to the basic malformation and its secondary effects. This review summarizes information about surgical treatment of such patients and comments upon some of the implications of this experience.

Ventricular septal defect

Ventricular septal defects (VSD) was a common congenital cardiac abnormality with defects in the interventricular septum results in a hemodynamic communication between the right and left ventricles. It is considered the most common congenital cardiac disease diagnosed in children and the second most common in adults. It accounts for approximately 40% of congenital heart disease. Clinical presentation varies depending on the defect size and degree of the shunt. The diagnosis can be accomplished by echocardiography or CTA with ECG-gating with direct visualization of the septal defect. Surgical and transcatheter closure of the defect were commonly used, while defect closure in patients with raised pulmonary vascular resistance can result in substantial morbidity and mortality.

Surgical Treatment in Atrial Isomerism

Methods for improving results in patients with atrial isomerism were sought by analysis of an experience in 51 patients (1967–1984; 13 hospital deaths and 11 later deaths). Most patients (34) had left atrial (LA) isomerism.