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)

Clinical and behavioral adaptation to managed care: stepwise suggestions for survival.

The rapid growth of managed care has left many physicians concerned and often bewildered about the new realities of the day. Essentially, managed care is a euphemism for a different kind of authority, responsibility, and accountability. Under "unmanaged care," authority, responsibility, and accountability were gained from and directed toward the patient. This arrangement has been supplanted by a new system in which the physician derives his authority from and is responsible and accountable to both the patient and the managed care company. The diagnosis of managed care is easy enough. It is a chronic disease; it does not go away. For those who can make the adjustment, managed care will not end careers. Rather, it will require a realignment, an adaptation to the societal mandate for "value." With care, foresight, and professionalism, this realignment can be navigated successfully, and disruption in the lives of pediatricians and patients can be held to a minimum.

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.

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.

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.

A new approach to congenital posterolateral diaphragmatic hernia.

The theory is advanced that increased pulmonary vascular resistance, resulting in a state of fetal circulation, with right-to-left shunting through the ductus arteriosus, is the main reason that many patients do not survive after repair of a diaphragmatic hernia. Three patients are presented (who, by Raphaely's criteria, were destined for a fatal outcome) in whom the ductus was ligated, and vasodilator drugs were infused into the pulmonary artery. All three demonstrated definite improvement in oxygenation. Two expired after 6 days, one of whom was found at autopsy to have intestinal volvulus and gangrene, and the other multiple plumonary emboli. One case, so managed, survived. The suggestion is made that pulmonary hypoplasia is not the main reason for the high mortality rate after diaphragmatic hernia repair, and that additional laboratory and clinical investigation of the pulmonary circulation may lead to significant improvement in results.

"Zero reference" during cardiac catheterization.

Hemodynamic pressure measurements obtained during cardiac catheterization studies are calibrated to an arbitrary level where the pressure is considered zero. Despite the fundamental nature of the zero reference level, there is no standardized method for determination of "zero reference." A horizontal metal rod has been constructed to be level with the pressure transducer domes. With lateral radiographic viewing, the metal rod is adjusted to be level with the junction of the inferior vena cava and the right atrium. The technique is rapid, reliable, without risk and allows accurate comparison of hemodynamic data between patients and in the same patient longitudinally.

Two-dimensional echocardiographic visualization of the aortic arch by right parasternal scanning in neonates and infants.

Echocardiography has previously been of limited use in visualizing the aorta in neonates and infants. Using a new technique--right parasternal scanning--the aortic arch and brachiocephalic vessels were studied in 50 children under 18 months of age. Complete visualization was accomplished in 47 patients including those 1) under 1,000 grams, 2) with indwelling endotracheal tubes, 3) who had D- or L-transposition, or 4) with right or double aortic arch. Inadequate studies were related to bilateral pneumothorax (2 neonates) and lack of cooperation (1 infant). Close correlation was obtained between images of the aorta by echocardiography and by angiography. Two-dimensional echocardiography can now visualize the aortic arch in small infants and children. In conjunction with another new technique--simultaneous Doppler flow-detection--coarctation of the aorta can be completely evaluated noninvasively in children of all ages including neonates.

Catheter positions in congenital cardiac malformations.

Biplane cineangiocardiograms were examined from a population of patients with diverse forms of congenital heart disease and examples were selected to illustrate catheter positions which may help define and elucidate the anatomy of simple and complex cardiac lesions. Familiarity with the appearance of these typical and atypical catheter positions may be of considerable aid in the course of hemodynamic study and in the evaluation of cineangiocardiograms. The editors suggest that each figure be evaluated as an unknown before reading the caption.

Containing costs in the treatment of congenital heart disease.

In the Congenital Heart Program at San Diego Children's Hospital, alterations in medical practice have reduced costs without impairing quality or access. Pediatric cardiac catheterization was done in 483 consecutive elective patients without overnight hospital stay. Hospital readmission was required in one patient for psoas tendinitis. Avoiding overnight hospital stay minimized attendant risks of hospital care, lessened psychosocial trauma and reduced the average hospital bill by $493 (29%). Hospital stay was also reduced for elective surgical correction of congenital heart disease on a case-by-case basis. Review of 151 consecutive cases (1978 through 1982) showed a decrease in both preoperative days in hospital and postoperative days in an intensive care unit. The duration of the postoperative stay was shortened from 6.8 days in 1978-1979 to 4.4 days in 1982 (P <.05). No increase in morbidity and no mortality resulted from the shortened perioperative hospital stay. Financial savings from this process averaged $991 per procedure.Diagnostic tests were reassessed and many precatheterization laboratory tests were eliminated. Without change in new patients seen or surgical volume, the use of cardiac catheterization decreased from 241 procedures in 1981 to 161 in 1982 and the number of operations without catheterization increased (11 to 22, 1981 to 1982). No increase in surgical morbidity or mortality was found comparing those diagnosed only by echocardiography with those who had preoperative cardiac catheterization. The decrease of 80 catheterizations in one year resulted in a savings of $188,800.True cost containment (reducing cost without reducing quality) can be accomplished in congenital heart programs. Similar cost containments in other disciplines may also be achieved.

Umbilical vascular catheters: localization by two-dimensional echocardio/aortography.

Umbilical vascular catheters are often necessary in the care of critically ill neonates. Position of the catheter tip is usually determined by roentgenography. Location of the umbilical arterial or venous catheter was determined by 2-dimensional echocardio/aortography in 55 consecutive infants and was compared to localization by thoraco-abdominal roentgenography. Most of the infants (76%) had respiratory distress syndrome or congenital heart disease. Echocaortographic localization of the umbilical arterial catheter correlated very closely (N = 50, sr = .90) with roentgenographic determination. For localization of the tip of the umbilical venous catheters, echocardiography was more accurate than roentgenography (employing contrast echocardiography for confirmation of cardiac chamber position). Two-dimensional echocardio/aortographic localization of the tip of indwelling umbilical vascular catheters is as accurate as roentgenography in the arterial system and more accurate than x-ray for umbilical venous catheters. Echocardio/aortography is superior to roentgenography (in localizing the catheter tip) because it 1) avoids ionizing radiation, 2) makes positioning of the patient unnecessary, 3) allows visualization of the catheter in relation to cardiovascular structures, and 4) may allow demonstration of intraarterial thrombo-emboli.