[Mixed hereditary polyposis with atypical extracolonic manifestations]. / Poliposis hereditaria mixta con manifestaciones extracolónicas atípicas.

We present the case of a 31 year-old-man with mixed hereditary polyposis and atypical extracolonic manifestations, as patent ductus arteriosus and mental retardation, with cranial hyperostosis. This is an extremely uncommon polyposis syndrome and has a moderate risk to progress to colon cancer.

Tricuspid annuloplasty and ventricular plication for Ebstein's malformation.

BACKGROUND: Seven patients with the diagnosis of Ebstein's malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present. METHODS: Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by approximating the anterior-posterior commissure with either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus excluded remained as a cul du sac and was plicated by suturing along the longitudinal axis of the heart. When present, the dysplastic posterior leaflet was included in the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet. The remaining septal leaflet played a minimal functional role. No additional procedures for treatment of arrhythmia were associated with the technique described. RESULTS: The postoperative course was uneventful in all patients. Mean follow-up is 4.3 years (range, 1 to 10 years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with adequate coaptation of the anterior leaflet and the septal structures. CONCLUSIONS: This technique seems applicable to most forms of Ebstein's malformation and is reproducible. The technique relies on the adequate mobilization of the anterior leaflet. Occasionally it is necessary to free fibrous adhesions of the leaflet to the underlying ventricular surface.

Aortico-right ventricular tunnel.

A successful operation on an infant with a tunnel through which the aorta communicated with the right ventricle is reported. The diagnosis was suspected preoperatively on the basis of two-dimensional Doppler color echocardiography and confirmed by cardiac catheterization. The aortico-right ventricular tunnel originated independently from the left coronary ostium and above the sinus of Valsalva. Patch closure from inside the tunnel under deep hypothermia was successfully performed. Follow-up is satisfactory 5 years later.

Bidirectional cavopulmonary shunt in patients with multiple risk factors.

BACKGROUND: Twenty-five patients in whom a modified Fontan operation was deferred because of multiple risk factors underwent a bidirectional cavopulmonary shunt. Two or more of the following risk factors were present in all: age less than 1 year, severe pulmonary artery distortion, impaired left ventricular function, subaortic obstruction, anomalous systemic-pulmonary venous connection, atrioventricular valve incompetence, and increased mean pulmonary artery pressure. METHODS: Additional procedures included take-down of systemic-pulmonary artery shunt, atrial septectomy, pulmonary artery reconstruction, bulboventricular foramen enlargement, and atrioventricular valve repair. RESULTS: There were three hospital deaths (12%). Mean follow-up is 21 months. There was no late mortality. Mean oxygen saturation increased from 71% to 83%. Results obtained from pulmonary artery reconstruction, enlargement of bulboventricular foramen, and atrioventricular valve repair were satisfactory for the patients in whom these risk factors were present preoperatively. Ventricular function also improved in the survivors in whom it was previously deteriorated, this being related to the suppression of the sources of ventricular volume overload. CONCLUSIONS: In this risk group of patients for a modified Fontan operation, a bidirectional cavopulmonary shunt provided adequate palliation at reasonable low risk. Early bidirectional cavopulmonary shunt would minimize complications originating from systemic-pulmonary shunts such as pulmonary artery distortion and the potential harm of chronic ventricular volume overload.

Anomalous systemic and pulmonary venous connections in conjunction with atriopulmonary anastomosis (Fontan-Kreutzer). Technical considerations.

Anomalous systemic or pulmonary venous connections can coexist with certain forms of complex cyanotic heart diseases that are reparable only by atriopulmonary anastomotic procedures, thus complicating the intraatrial separation of systemic and pulmonary venous pathways. Anomalous systemic or pulmonary venous connections were encountered isolated or in combination in 17 patients (10%) among a series of 170 modified Fontan-Kreutzer procedures. Fourteen of these 17 patients (82.3%) survived their operations, which utilized different techniques to deal with the various forms of anomalous systemic and pulmonary venous connections. There was one late death (5.8%). Extracardiac exclusion of a left superior vena cava with an end-to-side left cavopulmonary shunt proved to be a more successful alternative than the use of complicated intratrial baffles. Because of the complexity of the anatomic variables, repair of anomalous systemic or pulmonary venous connections in conjunction with a modified Fontan-Kreutzer procedure requires a detailed preoperative anatomical and physiologic diagnosis, and an individualized plan for each patient must be formulated to provide unobstructed venous pathways.

Atrioventricular valve repair or replacement in atriopulmonary anastomosis: surgical considerations.

Three patients who underwent a modified Fontan-Kreutzer (F-K) operation required additional procedures to correct left atrioventricular valve regurgitation. Valve replacement was performed in two additional procedures, and initial annuloplasty with a Carpentier ring was carried out in the third. All 3 survived the combined procedures and are doing well after a follow-up period ranging from five months to three years. More frequent presentation of these types of patients can be expected with broadening indications for F-K procedure to more complex lesions.

Extending the limits for modified Fontan procedures.

During the early development of atriopulmonary anastomotic operations (Fontan-Kreutzer), a number of physiologic and anatomical limits were proposed by the Fontan group as selection criteria. Among 167 consecutive patients undergoing modified Fontan procedures from 1973 through 1985, 109 (65%) patients exceeded one or more of the original selection criteria in areas of age, anomalies of systemic or pulmonary venous connection, pulmonary artery distortion, and pulmonary artery pressure. Twenty-six patients had a mean pulmonary artery pressure greater than 15 mm Hg, with 16 operative survivors (62%). Nineteen patients had anomalies of systemic and/or pulmonary venous connection, and 16 survived (84%). There were 44 patients under the age of 4 years, and 26 survived (59%). Twenty-five patients were older than 15 years, and 23 (92%) survived the Fontan procedure. Pulmonary artery distortion, relating to prior palliative operations, was found in 34 patients. Seventeen of these 34 survived a modified Fontan procedure (50%). Twenty-six patients had a pulmonary arteriolar resistance more than 2 Wood units times square meter, and 14 survived (54%), whereas 81 of 93 with a pulmonary arteriolar resistance of less than 2 U X m2 survived (87%). Multivariate analysis showed that pulmonary arteriolar resistance and pulmonary artery distortion had a significant, negative impact on survival, but age and anomalies of systemic and/or pulmonary venous connection did not. Pulmonary artery pressure was not an independent predictor of outcome. The results show that the original criteria may be exceeded in the areas of age and anomalies of pulmonary or systemic venous connection. Pulmonary artery pressure alone should not contraindicate a Fontan procedure if pulmonary arteriolar resistance is low. Pulmonary artery distortion from a prior palliative operation and elevated pulmonary arteriolar resistance increase the risk of a Fontan procedure.

Tetralogy of Fallot with subarterial ventricular septal defect. Diagnostic and surgical considerations.

Since 1964, 77 patients underwent repair for tetralogy of Fallot with subarterial ventricular septal defect. Median age at operation was 5 years. Cyanosis was commonly mild, and hypoxic episodes were infrequent. Accordingly, only 12 patients (15.58%) needed either palliative or corrective operations before the age of 2 years. Characteristic angiographic and echocardiographic features were observed, which allowed this entity to be differentiated from either classic tetralogy or other types of double-outlet right ventricle. The earliest series of operations (in which no right ventricular outflow patch was used) was followed by a prohibitive mortality (50%). Residual subpulmonary obstruction was the cause of all of these deaths. In a second series of patients, a transannular patch was frequently used (79.59%), with the rationale that closure of the ventricular septal defect would make restrictive the deficient infundibulum of these patients. In a later series, we observed that most of the patients had a nonrestrictive pulmonary anulus (80%); the patch therefore was limited to the ventriculotomy. In the absence of pulmonary annular hypoplasia, the need for patching of the right ventricular outflow tract in this entity has to be confined to preventing the subpulmonary obstruction induced by closure of the ventricular septal defect. A considerable improvement in the results followed our first series (4.3% mortality). Follow-up of the survivors (mean 7 years) has been satisfactory.