Right ventricle-to-aorta conduit in pulmonary atresia with intact ventricular septum and coronary sinusoids.

A 20-month-old girl with pulmonary atresia, intact ventricular septum, and ventriculocoronary connections underwent successful interposition of a right ventricle-to-aorta conduit and Fontan operation. The patient initially had a modified Blalock-Taussig shunt at birth and subsequently a bidirectional Glenn shunt at 8 months of age. After Fontan and right ventricle-to-aorta conduit placement, the suprasystemic right ventricular pressure dropped to systemic levels without causing myocardial injury. Additionally, the right ventricular cavity enlarged. We believe that the use of a right ventricle-to-aorta conduit should provide a valuable alternative and improve the outlook of certain patients with pulmonary atresia, intact ventricular septum, and ventriculocoronary connections.

Transsternal repair of coarctation and associated cardiac defects.

Over a 13-year period, 20 infants and children underwent transsternal approach for repair of coarctation and associated cardiac defects. Fifteen patients (75%) were operated on in the last 6 years. Thirteen patients (group 1) had intracardiac shunts and 7 (group 2), intracardiac obstruction or valvular insufficiency. Group 1 had a mean age of 0.8 +/- 1.9 years versus 4 +/- 3 years for group 2 (p = 0.05). There were 12 patients (92%), 7 months old or less in group 1. Aortic arch hypoplasia was present in 6 patients in group 1. A large patent ductus arteriosus was present in 5 of these 6 patients versus no patent ductus arteriosus in patients without aortic arch hypoplasia (p = 0.006). The mean pulmonary blood flow to systemic blood flow ratio in group 1 was 3.8 +/- 2 and the mean right ventricular to left ventricular ratio, 0.8 +/- 0.2. The coarctation repair fell mostly into three types: side patch aortoplasty (8), ductal tissue excision and patch aortoplasty of the concavity of the aortic arch (6), and subclavian aortoplasty (4). There was one early death (5%) which was due to sepsis in a newborn. Another newborn who had subclavian aortoplasty needed a left carotid artery--descending aorta bypass conduit early because of aortic arch hypoplasia. All patients were followed to 12 years (mean follow-up, 4.3 +/- 3.5 years). There were no late deaths. Two patients had recurrent coarctation, 1 after an end-to-end repair and the other because of incomplete arch enlargement after a side patch aortoplasty.(ABSTRACT TRUNCATED AT 250 WORDS)

Recognition and management of accessory mitral tissue causing severe subaortic stenosis.

Failure to recognize the presence of accessory mitral tissue causing subaortic stenosis can lead to not only the performance of inappropriate operations, but the persistence and recurrence of obstruction or even death. Over a 12-month period, we treated 2 children with severe subaortic stenosis caused by accessory mitral tissue. In 1 patient, who was 4 years old, the echocardiogram showed the accessory mitral tissue to be attached to the anterior mitral leaflet and ballooning into the subaortic area. The other patient, as a newborn, underwent simultaneous repair of a complete canal defect and coarctation. Two years later, the patient was seen because of syncopal episodes, progressive mitral insufficiency, and subaortic stenosis thought to be caused by anterior displacement of the anterior mitral leaflet. Mitral valvuloplasty and a conal enlargement procedure were planned. Intraoperatively, after the mitral valvuloplasty had been done, the subaortic stenosis was found to be due to a tight subaortic ring formed by accessory mitral tissue located at the septum and its fibrous extension to the anterior mitral leaflet. In both patients, excision of the accessory mitral and fibrous tissues resulted in a wide-open subaortic area. Both patients had an uneventful hospital course, and follow-up echocardiography showed no noteworthy residual left ventricular outflow gradient. We believe that increased awareness and sophisticated echocardiographic techniques should lead to an increased recognition of accessory mitral tissue causing subaortic stenosis. Simple resection of the accessory mitral tissue and its secondary fibrous tissues can be curative.

Alternatives in biventricular repair of double-outlet left ventricle.

Wide variation in morphology of double-outlet left ventricle allows numerous surgical alternatives that require sorting out to develop a more organized approach. There is a high association between tricuspid abnormalities and right ventricular hypoplasia with double-outlet left ventricle that calls for either Fontan-type procedure or biventricular repair. With pulmonic stenosis, biventricular repair has been accomplished using right-sided conduits. When pulmonic stenosis is mild or absent, repair techniques without conduits depend on the commitment of the ventricular septal defect (VSD). With subaortic VSD and mild pulmonic valvar stenosis, we successfully performed translocation of the main pulmonary artery and valve to the right ventricle on 2 patients (ages 32 and 8 months). Both patients are doing well 2 years and 1 year postoperatively. Others have successfully connected the right ventricle to the pulmonary artery with intraventricular baffle by enlarging a subaortic VSD or when the VSD is either subpulmonic or doubly committed. With subaortic VSD, although it has not been reported, biventricular repair can also be accomplished using a right ventricle-to-aorta baffle combined by either atrial or arterial switch. We believe that a simplified management plan can be formed in double outlet left ventricle based on the size of the right ventricle, presence of pulmonic stenosis, and commitment of the VSD. Whenever possible, translocation of the main pulmonary artery and valve or intraventricular repair should be accomplished in double-outlet right ventricle minimizing the use of right-sided conduits and reoperation.

Surgical techniques in partial anomalous pulmonary veins to the superior vena cava.

Over a 12-year period, 40 patients underwent repair of partial anomalous pulmonary veins (PAPV) draining to the superior vena cava (SVC) proximal to the sinus node. Mean age was 6 +/- 2 years. In all patients, the SVC was cannulated superior to the PAPV, which were baffled with pericardium to left atrium. Six patients had associated defects repaired. In 18 patients (group I), an incision was made at the crest of the right atrial appendage (RAA) and extended upward through the sinus node and to the SVC. After rerouting of the PAPV, the SVC was enlarged using the RAA (atriocavoplasty). In 17 patients (group II), rerouting of the PAPV was accomplished through a right atriotomy. Superior vena caval enlargement was not done. Drainage of the PAPV was close to the right atrium in 14 patients (low) and to the azygos vein (high) in 3. In 5 patients (group III), an incision was made on the SVC and RAA sparing the sinus node. After rerouting of the PAPV, the RAA was anastomosed to the SVC (end to side), providing another outlet for SVC flow. There was no early or late death. Two patients (10%) in group I had late sinus bradycardia. Obstruction of the SVC and PAPV developed in 1 patient in group II with high drainage. Intermittent complete heart block developed in 1 patient in group III who also had ventricular septal defect repair. We conclude that atriocavoplasty is effective for rerouting of the PAPV and enlarging the SVC, but may predispose to sinus node disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Obligatory Glenn shunt in fenestrated Fontan.

Five high-risk patients undergoing the Fontan operation required large fenestration (1 cm) because of high central venous pressure and low cardiac output. Because of major arterial desaturation, obligatory Glenn shunts were performed. Three patients had pulmonary atresia, 1 had tricuspid atresia 1-B, and the fifth had single ventricle with subaortic stenosis. The age ranged from 16 to 40 months (mean age, 25 +/- 9 months) and weight from 7.9 to 14.6 kg (mean weight, 11 +/- 2 kg). One patient had single and 3 had bilateral subclavian pulmonary artery shunts. The fifth patient had pulmonary artery banding and coarctation repair followed by an aortopulmonary window and central shunt. The first 2 patients repeatedly had to go back on cardiopulmonary bypass for a larger fenestration and subsequently had an obligatory Glenn shunt because of arterial desaturation. The last 3 patients had planned obligatory Glenn shunt and large fenestration. The first patient died on the second postoperative day of a combination of prolonged operation, repeated cardiopulmonary bypass, and periods of hemodynamic instability. Three patients had closure of the adjustable fenestration under local anesthesia at 4, 5, and 8 weeks postoperatively. The last patient is awaiting closure. We believe that in certain high-risk patients, a large fenestration combined with an obligatory Glenn shunt should be considered to minimize repeated cardiopulmonary bypass and urgent tightening or closure of fenestration in the immediate postoperative period.

Chloramphenicol toxicity associated with severe cardiac dysfunction.

A 9-month-old infant experienced severe chloramphenicol toxicity associated with high serum levels (313 micrograms/ml). Cardiovascular collapse with cardiomyopathic changes and impaired left ventricular function was documented by echocardiography. Serial echocardiographic evaluation showed resolution of the cardiomyopathic findings as the chloramphenicol levels were spontaneously cleared. Clinical course was complicated by the development of liver disease and coagulopathy compatible with disseminated intravascular consumption. Patient's recovery was complete and uneventful, nevertheless, chloramphenicol toxicity in childhood is associated with a significant mortality rate of 40%. The related impaired cardiac function, although reversible, appears to play a major role in the pathogenesis and eventual outcome in this syndrome.

Cardiopulmonary arrest due to misuse of viscous lidocaine.

A 30-month-old female infant had a cardiopulmonary arrest due to viscous lidocaine. The protective airway mechanisms were blunted by this drug, resulting in aspiration, hypoxia, seizures, and death. Lidocaine has also been associated with respiratory depression, psychosis, methemoglobinemia, and toxic cardiovascular reactions. We do not recommend the use of viscous lidocaine for minor oral irritation in infants and young children.

Diagnosis of intrapericardial tumor in an infant by two-dimensional echocardiography.

Two-dimensional echocardiography is a useful noninvasive tool for diagnosing intrapericardial tumors as a cause of respiratory distress or abnormal cardiomediastinal shadow on chest x-ray. Early recognition of these tumors within the pericardium is important since surgical removal is often curative. Cardiac cineangiography is unnecessary to delineate further the tumor and should be reserved for those in which associated intracardiac defects are suspected.

Urokinase therapy for a central venous catheter thrombus.

A 670 g premature infant is described in whom an intracardiac thrombus was documented. This thrombus formation probably resulted as a complication of an indwelling right atrial catheter. Thrombolytic therapy with urokinase was instituted, resulting in total and rapid dissolution. No hemorrhagic complications resulted. We believe that this particular thrombolytic therapy is safe and effective and should be considered when facing this particular complication.

CT diagnosis of congenital intrapericardial masses.

Prior to the development of CT, cardiac and pericardiac masses were evaluated by chest roentgenography, angiocardiography, and echocardiography. In most cases preoperative diagnosis of a specific entity was impossible. Preoperative CT diagnosis of an intrapericardial developmental mass becomes possible because of the presence of fat, fat/fluid level, thick walled cysts, as well as globular calcifications, bones, teeth, water density fluid, and soft tissue. A report of a young woman with a ruptured and infected intrapericardial teratoma and an infant with an intrapericardial bronchial cyst is presented.