Complete atrioventricular canal and tetralogy of Fallot: surgical considerations.

A review of 13 autopsy specimens and of 13 patients who were operated on for complete atrioventricular (AV) canal and tetralogy of Fallot between 1975 and 1985 revealed a number of anatomical details that are important in the successful repair of this combined lesion. A bridging anterior leaflet was present in 25 hearts. A septum primum was present and attached to leaflet tissue in 13. In all 26, a ventricular septal communication was present beneath the bridging anterior leaflet and extended anteriorly, but in 14 there was no ventricular septal defect underneath the posterior leaflet. Additional pathological features included the following: leaflet tissue deficiency (4 hearts), single left papillary muscle (3), accessory valve orifice (4), and left ventricular (4) or right ventricular (RV) (1) dominance. All 26 had infundibular stenosis, and 10 had hypoplastic pulmonary annuli. One had pulmonary atresia, and 6 had branch pulmonary artery stenosis. Surgical technique was modified to include incision of the septum primum in 7. Because of rightward displacement of the anterior ventricular septum and also to minimize the risk of causing subaortic stenosis, the bridging anterior leaflet was divided more toward the tricuspid orifice so as to parallel the crest of the ventricular septum. Transannular RV outflow patches were used in 10 patients, and a right ventricle-pulmonary artery conduit was placed in 1 patient. Three required repair of branch pulmonary artery stenosis. There were no hospital deaths. Three patients died late of residual AV valve regurgitation and branch pulmonary artery stenosis (2) and sepsis (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Pregnancy after heart valve replacement.

Thirty-two women, aged 21 to 44 years, who had undergone single (25) or multiple (7) heart valve replacement conceived 46 times and gave rise to 33 live-born infants. There were 12 abortions and one stillborn; a premature baby died 24 hr post-partum. Twenty-eight patients had mechanical prostheses and 4 had porcine xenografts, 29 patients being anticoagulated with acenocoumarol during the pregnancy. Cardiac status remained clinically satisfactory under medical treatment in all but one patient who developed heart failure. Fetal complications included cerebral hemorrhage in 1 premature infant, low birth weight in 3 newborns and 1 case of nasal hypoplasia. The incidence of abortion has decreased significantly during the latter part of our experience. It was significantly greater in patients with mitral prostheses. This review suggests that inadequate cardiac function, excessive anticoagulation and a history of primary infertility may increase the risk of abortion. Better control of anticoagulant therapy (accepting an elevation of the therapeutic limit) and improved prevention of pregnancy in high-risk patients are considered responsible for the improved results seen in recent years.

L-malposed great arteries with situs solitus and concordant atrioventricular connection.

Reports on surgical repair of cases with L-malposition of the great arteries with situs solitus atrialis and concordant atrioventricular connection are uncommon. A review of the anatomical characteristics of these cases has shown several peculiarities that may have considerable important in planning operation. Among them we emphasize the morphology of the ventricular septal defects, possible outflow tract obstructions and coronary artery course, anomalies of the atrioventricular valves and the course of the conduction system. Each of these aspects is reviewed from a surgical viewpoint in the light of our combined previous surgical and anatomical experience and of a review of previous reports by other authors.

Right atrial outlet atresia with straddling left atrioventricular valve. A form of double outlet atrium.

We present three hearts showing right atrial outlet atresia with straddling left atrioventricular valve; clinical data of two of the patients are reported. For semantic and practical reasons we believe the terms tricuspid or mitral atresia should not be used in these cases, and atrial outlet atresia is preferable; for similar reasons we prefer to use stradding left or right atrioventricular valve. These anomalies represent an interesting form of double outlet atrium and diagnosis clinically may be possible if the existence of the anomaly is considered. Differentiation from common atrioventricular canal with atrial outlet atresia is important since total repair, which is possible for this last anomaly, does not seem feasible in cases like ours. Classifications of tricuspid atresia may have to be enlarged to include cases with double outlet from either ventricular chamber or anatomically corrected malposition. Characterisation of the right ventricular chamber is difficult; we believe that description of its morphology and the type and mode of its connections should be used.

Aorta--right atrial communication. Report of an unusual case.

An unusual aneurysmal aorta--right atrial communication was found in a 25-year-old man with a rounded mass in the right cardiophrenic angle. Differential diagnoses included ruptured aneurysm of the sinus of Valsalva, coronary arteriovenous fistula, rupture of a dissecting aneurysm of the ascending aorta into the right atrium, and pseudoaneurysm of the right coronary artery followed by formation of a fistula between the aneurysm and the right atrium. Closure of the communication was performed under cardiopulmonary bypass. Complete absence of the right superior vena cava with a large persistent left superior vena cava draining to the coronary sinus was an unexpected finding at operation. The origin of the communication is not known; possible anomalous coronary and noncoronary origins are considered.

Modified Senning operation for treatment of transposition of the great arteries.

Since February, 1978, 42 infants ranging in age from 15 days to 16 months (mean age 6 months) and weighing between 2.0 and 9.0 kg (mean weight 5.8 kg) underwent a modified Senning I operation. Eleven (26%) underwent operation during the first 3 months of life. Twenty-nine patients had dextro-transposition of the great arteries (d-TGA) and an intact ventricular septum (Group I), and 13 patients had d-TGA and a large ventricular septal defect (VSD) (Group 2). In addition to the Senning I procedure, 13 patients had transatrial closure of their VSD, eight had ligation of a patient ductus arterioses, two had removal of a pulmonary artery band, and four had trans-pulmonary artery resection of short-segment subpulmonary stenosis. Modification of the original Senning operation included (1) patch augmentation (pericardium or Gore-Tex) of the atrial septal flap and (2) pericardial patch enlargement of the pulmonary venous pathway. One patient in Group 1 (3%) and two patients in Group 2 (15%) died after operation. A 2-week-old infant (Group 1) was treated with prostaglandin E1 (PGE) for 2 weeks before operation. The other hospital deaths (Group 2) occurred in a 2-month-old infant with advanced ischemic damage to the right ventricle and a 4-month-old child with multiple VSDs and Grade IV pulmonary vascular disease. No caval gradients were found after modification of the right atrial incision. Two patients died later from pulmonary venous obstruction, one during attempted recatheterization and the other after repair of the pulmonary venous obstruction. One patient had transient complete heart block, and four were discharged in junctional rhythm. Thirty-four patients (87%) were in regular sinus rhythm when released from the hospital. Postoperative catheterizations in eight patients showed no significant gradients in six and severe pulmonary venous obstruction in two (late deaths). More late postoperative results are required, including postoperative catheterization and electrophysiological studies, before the relative merits of the Senning versus the Mustard operation can be assessed.

Double outlet from chambers of left ventricular morphology.

This series of 5 cases with double outlet of morphologically left ventricular chamber includes 4 found during a review of 1700 heart specimens (incidence 0.23%) and 1 found at operation and successfully corrected. Abnormal atrioventricular connection precluding total correction was present in the 4 anatomical cases. Clinical diagnosis may be difficult and it is suggested that axial cineangiography may make anatomical diagnosis easier. Absence of the infundibular septum and aortic laevoposition are frequent. As some cases can be surgically corrected, accurate information is required on the size of the right ventricle, the morphology and function of the atrioventricular valves, the presence, size, and position of the ventricular septal defect, and the degree and type of outflow tract obstruction.

Gross distortion of atrioventricular and ventriculo-arterial relations associated with left juxtaposition of atrial appendages. Bizarre form of atrioventricular criss-cross.

Two pathological specimens are described in which a bizarre distortion of atrioventricular and ventriculoarterial relations existed. In both cases anterior and leftward displacement of the tricuspid valve coexisted with rightward displacement of the infundibular regions and semilunar valves. Abnormal expansion of the AV canal, possibly secondary to malrotation of the cardiac tube, is considered as a possible cause of the anomaly. One of the hearts showed severe subpulmonary stenosis produced by a valve-like structure which was well differentiated, with a complete annulus and chordae tendineae that inserted in a parachute-like fashion on the infundibular septum. The morphology of this structure was clearly different from that fo the fibrous tags described in other anomalies. Its origin is uncertain. These anomalies have important implications in relation to the nomenclature and classification of congenital heart malformations, and illustrate the value of recent modifications of the segmental approach.

Aortic levopositions without ventricular inversion.

A multi-center series of aortic levoposition (aorta anterior and to the left of the pulmonary artery) without ventricular inversion is presented and analyzed from an anatomical viewpoint. All the cases are exceptions to the 'loop rule' and amount to 0.9% of the total number or cases reviewed. A combined study of our own cases and those reported in the literature is presented. There is a high incidence of anomalies (malrotations, juxtaposition of the atrial appendages, atrioventricular valve anomalies, ventricular septal defects, pulmonary outflow tract obstructions and abnormal conal types) which may be related to the primary cause of the aortic levoposition, suggesting that they may form part of a new syndrome of diagnostic and surgical importance. In some cases, absence of coronary sinus was noted without asplenia or left superior vena cava draining into the left atrium. The major coronary artery pattern in cases of antomically corrected malposition with two well-developed ventricles was similar to that seen with ventricular inversion. Most of these cases can be helped surgically provided accurate and detailed preoperative evaluation and operative assessment and treatment is carried out.

Single primitive ventricle with normally related great arteries and atresia of the left A-V valve.

A child aged 2 years and 9 months was angiocardiographically diagnosed to have a single ventricle with normally related great arteries and atresia of the left A-V valve. A Blalock-Hanlon procedure and division of a large patent ductus arteriosus were followed by reduction in pulmonary artery pressure, but after operation the patient showed signs of left ventricular failure unresponsive to medical treatment, necessitating pulmonary artery banding. We have found only three similar published cases, and this is the only one with full angiographic documentation.

Supravalvular stenotic mitral ring. A new case with surgical correction.

In this report, we present a case of supravalvular stenotic mitral ring in a child. The lesion created a picture of severe mitral stenosis and was successfully managed by resection of the ring. No other malformations of the mitral apparatus or left ventricular outlfow tract were found. On analyzing the diagnostic criteria, we concluded that there appears to be only one useful criterion--the angiographic morphology. However, when ther- is an associated ventricular septal defect or findings of Shone's complex, we should suspect supravalvular ring rather than any other form of mitral stenosis.