Shared coronary arteries and coronary venous drainage in thoracopagus twins.

A pair of type B thoracopagus twins with complex cyanotic heart disease had shared coronary arteries and coronary venous drainage. Surgical separation was not attempted and the twins died at 10.5 months of age. Antemortem angiography demonstrated that Twin A's right coronary artery supplied Twin B's diaphragmatic and anterior ventricular myocardial free wall. A midline communication existed between each twin's right atrium at a common coronary sinus. The crossing coronary artery coursed alongside this connection and was visualized echocardiographically. At postmortem examination, the great cardiac vein of Twin A drained into the orifice of the common coronary sinus on Twin B's side of the midline. In five of six previously reported cases, the children died at attempted separation shortly after ligation of the interatrial communication. This may have been because of occlusion of a coronary artery or acute obstruction of a coronary vein. Consideration of separation of type B thoracopagus twins requires anatomic delineation of the coronary arteries and veins.

Congenital heart disease and pulmonary artery hypertension. I. Pulmonary vasoreactivity to 15% oxygen before and after surgery.

Pulmonary vasoreactivity at sea level was studied in 22 children before and in 15 children after corrective cardiac surgery for congenital heart disease and pulmonary artery hypertension; 8 children were studied both before and after cardiac surgery. During cardiac catheterization in 28 children, pulmonary and systemic hemodynamics were determined in room air and during breathing of 15% oxygen, which corresponds to a maximal hypoxic level commonly encountered during airplane travel. Before surgery, 19 of 22 children tolerated 15% oxygen (O2), which caused the following hemodynamic changes from room air status: the ratio of pulmonary to systemic arterial pressure increased from 0.70 to 0.78 (p less than 0.05), the ratio of pulmonary to systemic flow decreased from 2.2 to 2.0 (p greater than 0.05) and the ratio of pulmonary to systemic vascular resistance increased from 0.33 to 0.40 (p less than 0.02). In two children, severe pulmonary vasoconstriction developed within 5 minutes of 15% oxygen administration, requiring immediate discontinuation of hypoxia; neither patient had lasting deleterious effects. There was no evidence of increased pulmonary vasoreactivity in children with Down's syndrome compared with genetically normal children. After corrective surgery in 15 children (including both of the hyperreactors), no significant pulmonary vascular response to 15% oxygen was found. It is concluded that, in a small number of children with unrepaired congenital heart disease and pulmonary artery hypertension, pulmonary vascular hyperreactivity can be induced by breathing 15% oxygen; this reaction is life-threatening but reversible with the administration of 100% oxygen.(ABSTRACT TRUNCATED AT 250 WORDS)

d-Transposition of the great vessels in the neonate. A clinical diagnosis.

We reviewed the records of 66 neonatal patients with d-transposition of the great vessels (d-TGV) admitted to our institution. The initial history, physical examination by the cardiologist, chest rotengenogram, ECG, hemoglobin level, and hematocrit value were analyzed. Although all patients all patients were cyanotic and 65% were tachypneic, other signs of a major congenital cardiac anomaly were usually absent. The number of clinical signs (poor general status, cyanosis, respiratory distress, S2 increased and single, murmur, classical chest roentgenogram, abnormal ECG) present in each patient was three or less in 41%, only 13.5% had more than five signs.

Transposition of the great arteries with intact ventricular septum and patent ductus arteriosus.

At the time of initial balloon atrial septostomy a patent ductus arteriosus was found with angiography in 39 of 81 infants with transposition of the great arteries with intact ventricular septum. By angiographic criteria the ductus shunt was considered small in 21 infants and large and significant in 18. In contrast to the usual clinical presentation of neonates with transposition and intact ventricular septum, 12 of these 18 infants with a significant patent ductus arteriosus had only slight cyanosis and 8 presented with tachypnea out of proportion to the degree of cyanosis. Ten of the 18 infants had no continuous murmur, bounding pulses, mid-diastolic rumble or differential cyanosis. Clinically occult narrowing or closure occurred, presumably gradually and relatively late, in six infants. Acute early narrowing or closure, spontaneous (six infants) or surgically produced (three infants), occurred usually within the 1st month of life and was associated with a marked decrease in arterial oxygen saturation in eight infants, often with a rapid clinical deterioration. Persistence of a large patent ductus arteriosus for several months appears to be associated with an increased incidence of early pulmonary vascular disease. Therapeutic considerations for the infant with a large patent ductus arteriosus after initial balloon atrial septostomy include: (1) careful initial follow-up of the infant in clinically stable condition in case the ductus arteriosus should acutely narrow or close and the patient require urgent palliative or corrective surgery; (2) urgent early closure of the ductus in the infant with overt left heart failure with concurrent atrial septectomy or preferably primary corrective surgery; and (3) elective closure of a persistent significant patent ductus arteriosus before age 4 months with concurrent corrective surgery in the infant in clinically stable condition.

Pediatric cardiac catheterization with same-day discharge.

A system was developed for cardiac catheterization in children without overnight hospital stay (called same-day discharge). Over a 4 year period, 233 children (aged 5 weeks to 20 years) had catheterization with same-day discharge staying an average of 11.8 hours in the hospital. In all but 1, no problems occurred after hospital discharge; 1 child required readmission for psoas tendinitis after retrograde aortography. Same-day discharge was safely applied regardless of the patient's age, diagnosis, and use of systemic heparinization, large-bore sheaths, retrograde arterial catheterization, or cineangiography. The hospital-related cost of pediatric cardiac catheterization was reduced 29% compared with that of the standard 42 hour hospital stay. Same-day discharge provides attractive elements to the physician and institution involved in cardiac catheterizations, for example: (1) improved medical care by a decrease in the length of hospitalization, (2) a significant reduction in medical costs, and (3) elimination of time pressure in training and teaching as well as therapeutic decision-making. Critical factors for the successful application of same-day discharge are coordination of multiple health care professionals and physician judgment of the patient's clinical status. We speculate that reassessment of other hospital-oriented procedures may foster the development of methods for improving medical care or reducing cost, or both.

An autosomal dominant syndrome of characteristic facial appearance, preauricular pits, fifth finger clinodactyly, and tetralogy of Fallot.

This report describes six relatives with a syndrome of mild prenatal-onset growth deficiency, an altered craniofacial appearance, preauricular pits, and clinodactyly of the fifth finger; three had tetralogy of Fallot. The clinical impact of this condition appears to be related to the severity of the cardiac defect. Autosomal dominant inheritance is implied by the occurrence of the disorder in three successive generations with documented male-to-male transmission. Recognition of this syndrome is important in counseling families regarding recurrence risk for tetralogy of Fallot.

Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass.

Two neonates with asplenia syndrome, subdiaphragmatic total anomalous pulmonary venous connection, and pulmonary stenosis underwent a palliative operation without cardiopulmonary bypass. With the use of a side-biting clamp, and anastomosis was created between the pulmonary venous confluence and the right atrium. (Since children with asplenia syndrome have common mixing lesions, pulmonary venous drainage to the right atrium is not physiologically detrimental.) The descending vertical vein was ligated. A systemic-pulmonary shunt was performed. Both infants were discharged from the hospital less than 1 week after the operation. Both infants subsequently died, at 4 months and at 16 months of age. At autopsy, pulmonary venous drainage was unobstructed, with a surgical orifice 86% to 90% of the aortic anulus. We conclude that, in infants with asplenia syndrome and obstructed total anomalous pulmonary venous drainage, relief of pulmonary venous obstruction can be accomplished without cardiopulmonary bypass.

The bidirectional cavopulmonary shunt.

The bidirectional cavopulmonary shunt improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance. Since 1983, 17 patients have undergone a cavopulmonary shunt procedure (five primary operations, 12 secondary operations). Diagnoses were single ventricle complex (n = 4), hypoplastic right heart syndrome (n = 10), and hypoplastic left ventricle (n = 3). Age at primary operation ranged from 3 1/2 to 30 months (median 6 months). Weight ranged from 3.5 to 9.7 kg. Age at secondary operation ranged from 10 months to 14 years (median 15 months). Seven cavopulmonary shunt operations were performed without cardiopulmonary bypass (six via thoracotomy and one via sternotomy) and 10, with cardiopulmonary bypass. All patients in the bypass group had additional procedures: takedown of modified Blalock-Taussig shunt, seven patients; revision of right ventricular outflow tract, four patients; reconstruction of pulmonary arteries, four patients; tricuspid valvuloplasty, one patient; and Damus procedure, one patient. There was one (1/17) operative death (Damus procedure). One patient required early revision. Follow-up ranges from 1 to 53 months (median 23 months). Twelve of 16 had a good to excellent late result, with a rise in mean arterial oxygen saturation from 69% to 83%. Three patients died late (4 to 53 months) (pulmonary vascular disease, pulmonary arteriovenous malformations, and pneumonia, one patient each). There was one late failure (converted to Glenn shunt). The cavopulmonary shunt is an excellent palliative procedure when right atrium-pulmonary artery connection (modified Fontan) must be deferred because of age, weight, or anatomic considerations. Five patients have undergone right atrium-pulmonary artery connection later. In addition, at the time of the modified Fontan operation, the cavopulmonary shunt approach may optimize the anatomic connection (eight additional patients).

Vascular consequences of subclavian artery transection for the treatment of congenital heart disease.

The acute and chronic consequences of subclavian artery transection were analyzed in the noninvasive vascular laboratory. Twenty-eight patients (aged 1 day to 4 2/12 years, median 2 months) underwent subclavian artery transection (23 Blalock-Taussig, four subclavian aortoplasty for coarctation or interrupted arch, and one division of aberrant left subclavian). Bilateral systolic brachial artery pressure (BAP) was measured by Doppler instrumentation to obtain a "BAP index": BAP1 = (operated side BAP/control side BAP). Velocity waveform tracings and bilateral forearm skin temperatures were also obtained during studies before and sequentially after operation (4 hours to 12 years). Five patients underwent exercise testing of the upper extremity. Nine patients were studied for manual preference and limb development. Before operation, mean BAP1 was 0.99. Immediately (4 to 48 hours) after operation, mean BAP1 was 0.39. Three weeks postoperatively, BAP1 was 0.62, and thereafter it remained at 0.70. All differences between preoperative, immediate postoperative and late postoperative BAP1 are significant (p less than 0.001). Exercise resulted in a significant (p less than 0.01) increase in BAP bilaterally. Forearm skin temperature was initially lower (p less than 0.01) on the operated side but approximated the control side by 1 week. Limb girth was less on the operated side (p less than 0.01), without evidence of altered manual preference. In conclusion, subclavian artery transection causes permanent reduction in BAP1. The affected limb appears to respond to increased metabolic demand by increasing limb blood flow.

Coarctation in the first year of life. Patterns of postoperative effect.

From 1975 to 1982, 31 infants were operated upon in the first year of life for aortic coarctation and congestive heart failure. Operations performed were resection and end-to-end anastomosis (RETE) in 14, subclavian flap aortoplasty (SFA) in six, patch aortoplasty (PA) in five, and other procedures in six. Thirty of the thirty-one (97%) survived the operation. To assess the effect of operation, 26 infants were studied noninvasively with Doppler arm-to-leg pressure measurements at rest and with stress. Preoperatively, the median arm-to-leg gradient at rest was 77 mm Hg. Serial postoperative Doppler studies demonstrated progressive changes in arm-to-leg pressure gradients: 69% had residual arm-to-leg gradients that spontaneously resolved, 13% had residual gradients that persisted, 13% had progressive increase in gradient, and one child had neither early nor late gradient. Stress testing often unmasked gradients not present in the resting state. No differences were noted among the three surgical groups: RETE, SFA, and PA. From our experience, we have made four conclusions with regard to repair of coarctation of the aorta in infants. First, surgical survival is expected. Second, the effect of the operation is dynamic, with four patterns defined: (1) complete relief of coarctation, (2) transient residual coarctation, (3) persistent residual coarctation, and (4) recurrent coarctation. Third, optimal surgical therapy seems to be an eclectic approach. Fourth, physiological evaluation of coarctation in infants can be obtained by Doppler techniques in conjunction with stress testing.

Systemic-pulmonary shunts in infants and children. Early and late results.

From September, 1978, to January, 1983, 44 cyanotic infants and children underwent a systemic-pulmonary artery shunt for the treatment of reduced pulmonary blood flow. Age ranged from 18 hours to 4 years (mean age = 0.49 years). Weight ranged from 1.7 kg to 13.2 kg (mean weight = 4.9 kg). There were 12 classic Blalock-Taussig shunts, five central polytetrafluoroethylene shunts, six interposition modified Blalock-Taussig shunts, and 21 Great Ormond Street type of modified Blalock-Taussig shunts. No direct aorta-pulmonary artery anastomoses were performed. There was one postoperative death (1/44 = 2.3%) in a 1,700 gm neonate born with pulmonary atresia and intact ventricular septum (shunt patent). Four shunts required early revision: one thrombosed central shunt, a kinked patent interposition Blalock-Taussig shunt, a small but patent Blalock-Taussig shunt, and one excessive Great Ormond Street type of Blalock-Taussig shunt. Two late deaths were probably shunt-related: one Blalock-Taussig and one central. All four types of shunts provided good palliation, but the Great Ormond Street type of Blalock-Taussig shunt is our preferred shunt because of (1) low operative risk, (2) predictable patency (100% in our series), (3) lack of distortion of pulmonary arterial anatomy, and (4) technical ease of insertion as well as takedown.

Spontaneous acquisition of discontinuous pulmonary arteries.

BACKGROUND: Discontinuous pulmonary arteries have been considered a rare complication of systemic-to-pulmonary shunt operations. We report a series of children who spontaneously acquired pulmonary artery discontinuity. METHODS: All children from 1989 through 1995 with congenital pulmonary atresia were reviewed. RESULTS: Pulmonary artery discontinuity developed in 29% (15 patients), none related to shunt operation. In 6 of 15 patients, the neonatal angiogram showed a pattern that seemed to predict subsequent discontinuity; in 9 of 15, pulmonary arteriography was normal at birth. Two clinical patterns were identified: an early rapid acquisition of discontinuity within hours to days, and a delayed, more subtle development that occurred over months. Eight of 15 have died. Pathologic studies in 6 children showed ductal tissue extending along and into the pulmonary artery wall as well as intimal hypertrophic reaction and maladaptive remodeling. CONCLUSIONS: Children with congenital pulmonary atresia may experience spontaneous acquisition of pulmonary artery discontinuity. Ductal tissue is responsible for local pulmonary artery distortion and discontinuity; this may be exacerbated by previous prostaglandin E1 administration. Clinical algorithms are suggested for patients with pulmonary atresia.

The Damus-Fontan procedure.

The Damus-Kaye-Stansel operation is a useful technique for the treatment of complex cyanotic congenital heart disease when there is obstruction between the systemic ventricle and the aorta. Modifications of the technique include transection of the aorta and the pulmonary artery, anastomosis of the contiguous aortic and pulmonary walls, and connection of the distal aorta to the perimeter of the new bivalved proximal great artery. In addition, the bidirectional cavopulmonary shunt technique can be used with or without the Fontan procedure. Six patients underwent a Damus-Fontan operation, and all survived. Two patients underwent the Damus-cavopulmonary shunt (hemi-Fontan) procedure, and 1 survived. The postoperative status of the 7 survivors is good to excellent. Follow-up ranges from 2 months to 7 1/2 years.

Tricuspid valve closure in pulmonary atresia and important RV-to-coronary artery connections.

Mortality is high for children with pulmonary atresia, intact ventricular septum, and important connections between the right ventricle and the coronary arteries because of myocardial ischemia: in systole, suprasystemic right ventricular pressure delivers deoxygenated blood to the coronary artery (or arteries) and in diastole, the right ventricle provides a lower resistance alternative to coronary perfusion of the myocardium. Tricuspid valve closure was performed in 10 such children. None had stenosis of native coronary arteries. A trial of tricuspid valve closure (by balloon) was performed in the cardiac catheterization laboratory in 5 of 10 patients. Seven of 10 children survived surgical closure of the tricuspid valve plus concurrent procedures; none had heart block. Two of the 3 nonsurvivors were probably in inoperable condition due to preoperative myocardial ischemia. Before operation, 4 patients had ischemic changes on electrocardiograms; these changes were abolished after operation. Three of 10 patients have had a Fontan operation with 2 survivors. We conclude that children with pulmonary atresia, intact ventricular septum, important connections between the right ventricle and the coronary arteries, and normal native coronary arteries should have surgical closure of the tricuspid valve within the first year of life and treated thereafter as patients with "tricuspid atresia."

Late reoperation for systemic atrioventricular valve regurgitation after repair of congenital heart defects.

Since 1979, 17 infants and children have undergone reoperation for systemic atrioventricular (AV) valve regurgitation 6 weeks to 7 years after repair of congenital heart defects. Prior operations were repair of incomplete or complete AV canal (14 patients), Mustard repair of complex transposition of the great arteries including ventricular septal defect closure (2 patients), or first-stage operation for hypoplastic left heart (1 patient). Age ranged from 6 months to 11 years. In 12 of the 17 patients (10, AV canal; 1, transposition; 1, hypoplastic left heart), valve reconstruction was possible. Operative techniques included a combination of septal cleft approximation, leaflet resection, commissural annuloplasty, or ring annuloplasty. There were no operative deaths, and there were no reoperations in the repair group. The condition of these patients has improved. Follow-up ranges from 1 month to 9 years (mean follow-up, 4.1 years). Five of the 17 patients (4, AV canal; 1, transposition) underwent valve replacement. There were no operative deaths. Follow-up ranges from 3 to 8 years. Three patients later underwent re-replacement of the prosthetic valve; there was 1 late death. The condition of all 4 survivors is improved. Substantial AV valve regurgitation can occur months or years after repair of congenital heart defects. A combination of reconstructive techniques may be useful in preserving native valve function and avoiding systemic AV valve replacement.

Cyanotic congenital heart disease with decreased pulmonary blood flow in children.

Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.

Two-dimensional echocardiographic discrimination of normal from abnormal great artery relationships.

A prospective study was performed to determine the diagnostic accuracy of precordial two-dimensional echocardiographic techniques in discriminating normal from abnormal great artery relationships. We studied 503 children using standard parasternal short-axis view and a modified technique called rotating high long-axis view (RHLAV). In RHLAV, there is rapid superimposition of the proximal courses of the two great arteries seen by rotation of the ultrasonic beam. Discrimination was based on the crossing great artery relationship seen with normally related great arteries (NRGA) and the parallel courses found in both transposition and malposition of the great arteries (TGA/MGA). Using the RHLAV, the great artery interrelationship was accurately determined in all 503 patients. Precordial two-dimensional echocardiography can accurately discriminate great artery relationships and is a useful addition to other two-dimensional echocardiographic techniques.

Therapeutic cardiac catheterization in children.

Cardiac catheterization, once the mainstay of diagnosis in children with congenital heart disease, has become a therapeutic modality for many conditions. Balloon dilatation can now open stenotic valves and vessels, coils and umbrellas can now close unwanted communications, and emboli can be withdrawn without surgical intervention.