Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy.

Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation.

Intraoperative device closure of ventricular septal defects.

Background: Surgical repair of muscular ventricular septal defects (VSDs) has been associated with significant morbidity and mortality when the defects are multiple, associated with complex cardiac lesions, or requiring left ventriculotomy. Transcatheter VSD closure may be difficult in patients weighing less than 7 kg or those with limited vascular access. We attempted intraoperative device closure of muscular VSDs during surgical repair of associated lesions.

Early bidirectional cavopulmonary shunt in young infants. Postoperative course and early results.

Background: Despite the recent wide applicability of the bidirectional cavopulmonary shunt, there is limited reported experience in performing these shunts in infants 6 months or younger.

Methods And Results: Before October 1992, 17 consecutive infants aged 4.2 to 6.

Reparative cardiac surgery in the very young.

In addition to primary circulatory disturbance, the presence of congenital heart disease may compromise the development of organ systems including the myocardium, the pulmonary vasculature and the central nervous system. Therefore, early elective repair of complex congenital heart defects is desirable. Today repair of complex congenital heart defects in the neonate can be achieved at low early and late mortality and morbidity with favorable long-term functional results.

Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries.

Background: Patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 14-year experience in the treatment of these patients comparing management schemes.

Methods And Results: Between January 1978 and August 1988, 91 patients with tetralogy of Fallot and pulmonary atresia had an adequate evaluation of their pulmonary artery anatomy before any surgical management.

Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends.

Case histories of 301 patients with complete atrioventricular canal defect presenting to our institution in infancy between January 1972 and January 1992 were reviewed with the purpose of identifying the factors responsible for the observed improvement in perioperative mortality over this time period. A retrospective analysis of hospital records examined 46 patient-related, morphologic, procedure-related, and postoperative variables for associations with perioperative death and reoperation. Operative mortality decreased significantly over the period of the study from 25% before 1976 to 3% after 1987 (p < 0.

Fate of small homograft conduits after early repair of truncus arteriosus.

Neonatal repair of truncus arteriosus is being performed in a number of centers, often with the use of small homograft conduits. The fate of the homograft and the risk of replacement were the subjects of this study. Between January 1987 and October 1991, 43 infants aged less than 3 months (range, 3 to 90 days) underwent primary repair of truncus arteriosus including implantation of a valved homograft conduit (diameter, 7 to 12 mm).

Repair of truncus arteriosus in the neonate.

From September 1986 through December 1991, 63 patients with truncus arteriosus underwent surgical repair. The management approach evolved over the period of the study from elective primary repair at 3 months of age to elective primary repair in the early neonatal period. Thirty variables were examined as potential risk factors for the outcome events of death, reoperation, and presence of pulmonary vascular morbidity.

Enlarged bronchial arteries after early repair of transposition of the great arteries.

Objectives: This study was undertaken to define the incidence of enlarged bronchial arteries after early surgical repair of transposition of the great arteries by the arterial switch operation, and to report the results of catheter-directed therapy in five patients.

Background: Pathologic and angiographic studies have demonstrated enlarged bronchial arteries in patients with transposition of the great arteries.

Methods: A subjective 4-point scale was used to grade postoperative angiograms performed in 119 patients at our institution between January 1983 and December 1991.

Effect of baffle fenestration on outcome of the modified Fontan operation.

Background: The "fenestrated Fontan" (surgical baffle fenestration followed by transcatheter test occlusion and permanent closure after postoperative recovery) was adopted in an effort to reduce perioperative mortality and morbidity. This study assesses the effect of baffle fenestration on outcome.

Methods And Results: Patients having a modified Fontan operation with a cavocaval baffle and cavopulmonary anastomosis were retrospectively selected for study.

From Glenn to Fontan. A continuing evolution.

Background: Aristotle's and Galen's erroneous understanding of the circulation eventually was rectified, mostly by Colombo and Harvey, who in the 16th and 17th centuries, respectively, provided the foundation to our present knowledge of the pulmonary and systemic circulation. The "indispensability" of the right ventricle was not questioned until the 20th century. Rodbard, in 1948, for the first time successfully bypassed the canine right ventricle by anastomosing the right atrial appendage to the proximally ligated main pulmonary artery.

Factors associated with choreoathetosis after cardiopulmonary bypass in children with congenital heart disease.

Background: Choreoathetosis (CHO) after congenital heart surgery has been described since 1960. Risk factors and patient outcome have not been well defined.

Methods And Results: As our complexity of cases increased and management of pH on cardiopulmonary bypass (CPB) evolved, we noted the appearance of CHO among patients beginning in 1986.

Clinical outcomes after the arterial switch operation for transposition. Patient, support, procedural, and institutional risk factors. Congenital Heart Surgeons Society.

Background: As the probability increases that the arterial switch operation is optimal treatment for transposition, detailed information about outcomes and the circumstances in which they are suboptimal becomes important.

Methods And Results: A multi-institutional prospective study with annual detailed follow-up included 513 neonates with simple transposition or transposition and ventricular septal defect entering for diagnosis and treatment at < 15 days of age and undergoing an arterial switch repair. The 1-month and 1- and 5-year survivals were 84%, 82%, and 82%, respectively.

Temperature monitoring during CPB in infants: does it predict efficient brain cooling?

We examined jugular venous oxygen saturation data in 17 pediatric patients less than 1 year of age undergoing hypothermic cardiopulmonary bypass (CPB). Jugular venous oxygen saturations (JvO2SATS) were measured before bypass and during the active core cooling portion of CPB. The study intervals during CPB included 1 minute after initiation of CPB, at a tympanic membrane temperature of 15 degrees C, and at a rectal temperature of 15 degrees C.

Age-dependent ventricular response to pressure overload. Considerations for the arterial switch operation.

Success with the arterial switch operation for D-transposition of the great arteries and the concept of left ventricular suitability for systemic work stimulated this literature review of the age-dependent mechanisms in normal cardiac growth and pressure-induced left ventricular hypertrophy. Normal postnatal myocardial growth is markedly influenced by hemodynamic factors. It consists of an early hyperplastic phase of both myocytes and capillaries that is followed by a myocyte hypertrophic phase.

The fenestrated Fontan procedure.

In 90 patients with characteristics placing them at increased risk for a Fontan operation, a fenestration was created in the atrial baffle at the time of the Fontan repair. The rational was to allow a right to left shunt which would maintain cardiac output and limit right atrial pressure in the presence of conditions which limit pulmonary blood flow. Early mortality was 4/90 (4%), with an additional two patients having the Fontan repair taken down to a bidirectional cavopulmonary anastomosis.

Mitral atresia with a large left ventricle and an underdeveloped or absent right ventricular sinus: clinical profile, anatomic data and surgical considerations.

In mitral atresia with a large left ventricle, the tricuspid valve is either straddling and biventricular or entirely left ventricular. To learn how to assess the identity of the tricuspid valve in such cases 15 heart specimens were examined as well as the echocardiograms of 10 living patients. When the right ventricular sinus was underdeveloped (11 cases), a straddling tricuspid valve was present; when it was absent (14 cases), the tricuspid valve was entirely left ventricular.

Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. A two-institution study.

Survival after entry and survival after repair (94%, 91%, and 91% at 1 month, 1 year, and 5 years, respectively) were similar in two institutions treating 196 consecutive patients, and there was no advantage (and a possible disadvantage) of a protocol of preliminary shunting and later repair in very young patients. Size and configuration of the right and left pulmonary arteries had no demonstrable effect on survival, prevalence of transannular patching, or postrepair right ventricular-left ventricular pressure ratio. Small size of the pulmonary "anulus" and trunk were risk factors for death, transannular patching, and high postrepair pressure ratio.

Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle.

The Fontan operation was originally employed for patients with tricuspid atresia, but its application has been extended to those with a variety of complex defects in which there is functionally only a single ventricular chamber. The outcome of 225 modified Fontan operations carried out between 1984 and 1990 at the Children's Hospital, Boston, for patients with defects other than tricuspid atresia was reviewed. Overall 30 patients (13.

Arterial switch operation for simple and complex TGA--indication criterias and limitations relevant to surgery.

Since 1983, 505 patients underwent the arterial switch operation (ASO). 326 (28 days) had transposition of the great arteries with an intact ventricular septum (TGA/IVS) und 179 had a ventricular septal defect (TGA/VSD). Mean age at ASO was 7 +/- 5 days.

Corrected transposition in situs inversus. Biventricular repair of associated cardiac anomalies.

Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.

Function of the anatomic pulmonary valve in the systemic circulation.

The anatomic pulmonary valve, which has thin leaflets with little elastic tissue in the normal heart, must function as the neoaortic valve after arterial switch operation (ASO) for transposition of the great arteries, palliative surgery for hypoplastic left heart syndrome (HLHS), and pulmonary artery-to-aortic (P-A) anastomosis for complex heart disease with subaortic obstruction. The long-term function of this valve under these circumstances is not known. To investigate the function of this valve in the systemic circulation, the follow-up echocardiograms, catheterization data, and angiograms were reviewed for 189 patients at our institution after an ASO (n = 112), palliative surgery for HLHS (n = 45), or P-A anastomosis (n = 32).

Cognitive development of children following early repair of transposition of the great arteries using deep hypothermic circulatory arrest.

Twenty-eight children who underwent corrective cardiac surgery in early infancy had developmental evaluations to explore whether cardiopulmonary bypass perfusion variables are associated with later cognitive function. All had transposition of the great arteries repaired by the arterial switch operation using deep hypothermic circulatory arrest. The mean duration of deep hypothermic circulatory arrest was 64 +/- 10 minutes (mean +/- SD).

Tricuspid atresia or severe stenosis with partial common atrioventricular canal: anatomic data, clinical profile and surgical considerations.

The anatomic findings in 11 cases of tricuspid atresia and in two cases of severe tricuspid stenosis, both combined with partial common atrioventricular (AV) canal, are presented in detail. Twelve cases were documented by postmortem examination and the diagnosis was confirmed by echocardiography and surgical observation in the one living patient. Clinical data available in nine cases and cardiac catheterization data obtained in eight are included in this report.