[The bidirectional Glenn operation in 100 cases with complex congenital heat diseases: factors influencing surgical results]. / La operación de Glenn bidirectional en 100 casos con cardiopatías congénitas complejas: factores determinantes del resultado quirúrgico.

OBJECTIVE: The bidirectional Glenn shunt is a well established surgical technique in children with complex congenital heart disease. The present study is a retrospective analysis of patients undergoing this operation in order to assess the influence of different variables in the outcome. PATIENTS AND METHOD: From December 1990 to June 2000, 100 patients received a bidirectional Glenn shunt. Two groups were defined, based on the outcome. Group A (n = 15, unsuccessfully result) including death and need to reoperate, and Group B (n = 85, patients with good outcome). RESULTS: The mortality was 8%. Eight patients were reoperated at follow-up. The survivors were followed for a mean of 3.5 years. Mean pulmonary artery pressure 7 mmHg was a factor associated with poor clinical progress. Other variables (age less than 1 year, excessive pulmonary blood flow, double Glenn operation, significant anatomic anomalies, and arrythmias), were also associated with outcome. Significant variations were observed in the time of by-pass or the need for aortic clamp in cases with simultaneous operative repair of pulmonary branch stenosis. Actuarial survival rate, most more than 1 year was 92%, and freedom from reoperation at 3 years was 90%. CONCLUSIONS: The bidirectional Glenn shunt is an excellent palliation in patients with functionally single ventricle. Mean pulmonary artery pressure was the most important variable related with the outcome. We are encouraged to continue with tendency to perform bidirectional Glenn shunt preferably early, avoiding, whenever possible, previous palliative surgery.

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.

Ten common congenital cardiac defects. Diagnosis and management.

Despite the decline of birth rate and the advancement attained toward prevention and early diagnosis and therapy, congenital heart disease remains a problem of major importance, particularly for the practitioner who is the first in the line of medical professionals to diagnose, treat, and counsel patients and family. In this article the diagnosis and management of congenital cardiac defects are discussed with clarity and appropriate detail that is necessary for proper and meaningful understanding and handling of these entities. The text is supplemented with a wide number of properly executed illustrations and tables that summarize the most important pertinent findings.

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.

Nomenclature and classification of congenital heart disease.

At present there is no universally accepted nomenclature for congenital cardiac malformations. Much of the controversy results from failure to distinguish the structural connections of the heart from the morphology and spatial relations of its components. The confusion is compounded by an abundance of individual definitions, many of them speculative. The present article proposes a totally descriptive nomenclature. It describes in turn the connections of the cardiac segments, their morphology, their relations, and additional anomalies in any segment. Each step in the segmental approach is discrete. The overall effect is to force a succinct and comprehensive description of any cardiac malformation, no matter how complex.

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.

Tricuspid atresia. An anatomical study of 17 cases.

This is a report on the anatomical characteristics of 17 cases of tricuspid atresia. Three of these cases had a discrepancy between the type of bulboventricular loop (dextro) and the position of the great arteries (the aorta being to the left of the pulmonary artery). In these a characteristic type of ventricular septal defect, located just beneath the tissue of the semilunar valves was found. In 3 cases with cardiac dextroversion and juxtaposition of the atrial appendages the great arteries were on the same frontal plane and there was a separation between the semilunar and the anterior mitral leaflet. One case was associated with a double outlet left ventricle, a D-malposition of the great arteries, and a bilateral subaortic and subpulmonary conus. Among the 6 cases with transposition of the great arteries, the ventricular septal defect was large in 2 (34.4%) only. In the analysis of the 13 cases with intact ventricular septa (2 cases) or restrictive (small and medium-sized) ventricular septal defects (11 cases) obstructive anomalies of the vessel arising from the right ventricle were found in 12 (92.3%). These anomalies involved the aorta in 4 cases. (34.4%) and the pulmonary artery in 8 (66.6%). In the study of the 4 cases with a large ventricular septal defect, obstructive anomalies in the vessel arising from the right ventricle were present in 2 cases (50%), and were located in the aorta in 1 case (25%) and in the pulmonary artery in the other case (25%). The 2 cases with intact ventricular septum were associated with a hypertrophy of this septum and an absent pulmonary valve. In 1 of these cases, a third ventricular chamber was disclosed within the ventricular septum. This chamber communicated with the right ventricle through a very small opening. In 82.3% of the cases, the projection of the dimple, the rest of the tricuspid orifice, was located either on the ventricular septum or over the left ventricle. In the 3 cases with juxtaposition of the atrial appendages there was a positive transillumination of the floor of the right atrium, which corresponded, to the rest of the tricuspid valve in one case and to the atrioventricular portion of the membranous septum in the other 2.

[Right ventricle with double outlet associated to total anomalous pulmonary venous drainage to innominate vein]. / Ventrículo derecho de doble salida asociado a retorno venoso pulmonar anómalo total a la vena innominada

The clinical findings of 2 patients are studied with an association of malformations not previously reported: double outlet right ventricle and total anomalous pulmonary venous connection to the innominate vein. The following features are worth while to be underlined: 1. Both patients showed a discordance between viscero-atrial situs and direction of the apex; 2. In both cases, particularly in case 2, there existed a very unusual spacial relationship between ventricles, the right ventricle being superior with respect to the left ventricular cavity; 3. From the embryological viewpoint, this ventricular arrangement is supposed to be due to an arrest of the development of the heart in the straight tube stage, there being, however, an upwards expansion of the atrioventricular canal and common atrium; 4. An accurate diagnosis of the common or divided nature of the atrioventricular valves has been impossible in these cases; 5. These combination of lesions constitutes a challenge to cardiologists and surgeons as far as the surgical indication is concerned. In that sense, the simple anastomosis of the common pulmonary vein to the left atrium, although is not a radical cure, may be a feasible and beneficial procedure.