Surgical treatment of pulmonary atresia with intact ventricular septum

Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.     

Complete correction of transposition of the great arteries with left juxtaposition of the atrial appendages

Juxtaposition of the atrial appendages is an uncommon anomaly which is usually associated with transposition of the great arteries. Experience with five patients with transposition of the great arteries in combination with juxtaposition of the atrial appendages in whom Mustard's operation was performed is reviewed. Technically, the existence of juxtaposition of the atrial appendages in corrective surgery for transposition does not present any additional surgical problems. Emphasis is placed on the advantages of early complete correction, avoiding the need for palliative procedure.     

Pulmonary atresia with intact i.v. septum (author''s transl)

Eight cases of pulmonary atresia with intact ventricular septum, are reviewed. Three corresponded to the group with small or hypoplastic right ventricle and five to the one with normal or enlarged right ventricular cavity. The electrocardiogram was of less value than plain chest radiography for the differential diagnosis of both groups. Cardiac catheterization revealed a right to left atrial shunt and the suprasystemic level of the right ventricular pressure. Selective angiocardiography demonstrates the stop of contrast at the level of pulmonary valve. Five patients were operated. An ascending aorta to right pulmonary artery anastomosis, Waterston type, was performed in three patients. Pulmonary valvotomy was carried out in the others.     

Atresia of the right atrioventricular orifice

The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular orificial atresia is a better term to describe this anomaly than "tricuspid atresia". Use of the latter term can be confusing when the morphologically tricuspid valve is located beneath the left atrium. It is accepted that the definition employed may include cases in which the mitral valve may be atretic, blocking normal exit from the right atrium, but it is argued that such cases would present clinically as "tricuspid atresia" and therefore are correctly designated as right atrial orificial atresia. The results show that the majority of hearts with right atrial orificial atresia have the ventricular morphology of primitive ventricle, most with, but a few without an outlet chamber. However, in a minority of hearts an imperforate membrane interposes between the right atrium and a formed but hypoplastic right ventricle. In two of the hearts, the imperforate membrane showed features of Ebstein's malformation. The hearts could be further subdivided according to the ventriculoarterial connection. Most had normally connected arteries (66 of 83), and all but 2 also had normal relations between the arteries; in these 2 hearts there was "anatomically corrected malposition". Twelve hearts showed transposition, one had double-outlet outlet chamber, and another persistent truncus arteriosus. The remaining 3 hearts, all without outlet chamber, had by definition a double outlet connection. A segmental approach provides the best way of classifying this anomaly, and an embryological explanation is offered for the variations in anatomy observed.     

Information on the 5th meeting of the European Tick Study Group]

There is a strong association between prenatally diagnosed structural heart disease and fetal chromosomal abnormalities. Isomerism of the atrial appendages is an exception to this because the fetal karyotype is usually normal in this condition. A case of atrial isomerism diagnosed antenatally with a normal female karyotype but with a microdeletion of chromosome 22q11 is reported.     

Yersinia enterocolitica infection in a 4-month-old infant associated with infection in household dogs

Colon atresia is a rare cause of intestinal obstruction in the neonate and requires early diagnosis and prompt surgical treatment. It is impossible in the neonate to differentiate colon atresia from other forms of obstruction at the time of initial presentation. The diagnosis is confirmed roentgenographically, including views of the abdomen and contrast barium enema series. Lesions proximal to the splenic flexure are treated with initial resection of the atretic segment and a primary anastomosis. Those lesions distal to the splentic flexure are managed initally with a diverting loop colostomy with subsequent staged resection and anastomosis.     

Intestinal atresia and stenosis: a 25-year experience with 277 cases

OBJECTIVE: To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. DESIGN: Retrospective case series. SETTING: Pediatric tertiary care teaching hospital. PATIENTS: A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). INTERVENTIONS: Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. MAIN OUTCOME MEASURES: Morbidity and early and late mortality. RESULTS: Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. CONCLUSIONS: Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.     

Bringing advanced technologies of the Internet into the dialysis unit

Two-stage electrophoresis of preparations made of natural brine and extract of mud taken from the Lake Karachi tried in combined treatment of experimental inflammation of the uterine appendages prevents proliferative and sclerotic alterations in ovarian stroma, stimulates repair of the intramural nerves, corrects follicular atresia.     

Isolated left superior vena cava to the left atrium with situs solitus and dextrocardia: extracardiac repair facilitated by juxtaposition of the atrial appendages

We describe an extremely unusual case, with isolated left superior vena cava to the left atrium, normal intracardiac anatomy, and left juxtaposition of the atrial appendages. Surgical repair was performed without cardiopulmonary bypass by anastomosing the left superior cava to the superior aspect of the right atrial appendage, and was facilitated by the ectopic location of the appendage.     

Primary reconstruction of esophageal atresia with distal tracheoesophageal fistula in a 740-g infant

A premature 740-g infant who had esophageal atresia and tracheoesophageal fistula was treated with a primary anastomosis. The postoperative recovery was excellent, but the need for close cooperation with neonatal intensivists is essential for survival.     

Esophageal anastomosis: an experimental model to study anastomotic healing and the use of lyophilized collagen

This experimental study assessed the use of lyophilized collagen to reinforce cervical esophageal anastomosis in rabbits. Twenty New Zealand White rabbits weighing 2.3-3.2 kg were used. In group I (n = 10) a 1-cm-long segment of the esophagus was excised and the two free edges anastomosed, to mimic the conditions found in newborn esophageal atresia. Group II (n = 10) had a segmental esophageal resection and end-to-end anastomosis as in group I but the anastomotic site was circumferentially covered with lyophilized type I collagen film. The resected segments were processed immediately and served as controls for the postoperative tissue in each animal. The animals were starved for the first 24 h and water was given on the 2nd postoperative day; on the 3rd postoperative day the animals were allowed a normal diet. Two rabbits in group II died on the 7th and 8th postoperative days because of a fistula. All the rabbits were killed on the 10th postoperative day and 4-cm segments of esophagus with the anastomosis at the centre were removed. At this time gross leakage was detected in four animals (one in group I and three in group II). Each anastomosis was evaluated for bursting pressure, collagen content, and histologic appearance. Bursting pressure was higher in group I. Collagen (measured as hydroxyproline) levels in anastomotic and adjoining 1-cm segments were compared with concentrations in control segments resected during operation. In group II animals there was a significant reduction in the lowering of hydroxyproline concentrations around the anastomosis. Microscopic evaluation revealed no significant differences between the two groups. This experiment showed no demonstrable benefit from the use of lyophilized collagen in preventing the esophageal anastomotic leakage that occurs in repaired esophageal atresia.     

The Waterston anastomosis with no deaths in the neonate

The Waterston anastomosis has been performed with no deaths in 11 consecutive neonates during the past 24 months. Seven neonates had pulmonary atresia with either single ventricle or hypoplastic right ventricle, 3 had tetralogy of Fallot, and one had severe pulmonary stenosis with a normal right ventricle. Six neonates were one day old and only one was older than 7 days. All have obtained symptomatic and documented (increase Po2) benefit. This level of success is attributed to (1) minimal delay between the recognition of cyanosis and operation (mean time between hospital admission and catheterization was 3 hours and between catheterization and operation, 4 hours); (2) correction of any base deficit prior to, during, and after operation; (3) accurate construction of the anastomosis to avoid excessive size, and (4) careful postoperative management of the pulmonary subsystem by experienced personnel. We have demonstrated that the high mortality rate previously reported for the Waterston anastomosis in the neonate can be markedly improved by an appropriate patient management program.     

Revision of previous Fontan connections to extracardiac or intraatrial conduit cavopulmonary anastomosis

BACKGROUND: In patients who have received an atriopulmonary Fontan connection, complications such as right pulmonary vein obstruction, atrial arrhythmias, and thromboembolism are often secondary to right atrial enlargement. When such complications develop despite good ventricular function, there are few management options available. Extracardiac or intraatrial conduit cavopulmonary anastomosis, which improves central systemic venous flow patterns, avoids atrial distention, and does not involve the extensive atrial suturing required by other forms of cavopulmonary anastomosis, may provide relief for this group of patients. METHODS: Between October 1992 and October 1995, 7 patients presented 8 to 20 years after atriopulmonary connection with severe right atrial dilatation (7), Fontan pathway obstruction (4), progressive congestive heart failure (4), atrial tachydysrhythmias (3), right atrial thrombus (1), obstruction of right pulmonary veins by an enlarged right atrium (1), and subaortic stenosis (1). After evaluation of the options, they underwent revision of the atriopulmonary connection to extracardiac (5) or intraatrial (2) conduit cavopulmonary anastomosis. RESULTS: One patient with severe cachexia, in whom transplantation was contraindicated for social reasons, died in the early postoperative period of massive effusions. Two patients eventually required permanent pacing for atrial dysrhythmias (1) or complete heart block secondary to subaortic fibromuscular resection (1), and 2 demonstrated marked improvement in unstable preoperative rhythm disturbances. At a median follow-up of 17 months, 4 of the 6 survivors were functioning at higher New York Heart Association levels than preoperatively, and 1 had recently undergone heart transplantation. CONCLUSIONS: In properly selected patients with atrial complications, revision of a prior Fontan connection to extracardiac or intraatrial conduit cavopulmonary anastomosis appears to be a viable option.     

Role of neutrophil elastase in stress-induced gastric mucosal injury in rats

To evaluate the growth of a pulmonary trunk reconstructed without an extracardiac conduit, the hemodynamics and diameter of a new pulmonary trunk were measured in 5 patients from the right ventriculogram and MRI at postoperative follow-up periods. There were tetralogy of Fallot with pulmonary atresia in two patients, tetralogy of Fallot with single coronary in one, truncus arteriosus type I in one and transposition of the great arteries with ventricular septal defect and pulmonary stenosis in one. The age at operation ranged from 26 days to 4.5 years. The posterior wall continuity of the right ventricle and pulmonary artery was established by the direct pulmonary-right ventricular anastomosis in three patients and by the interposition of the left atrial appendage in two. Postoperative follow-up periods ranged from 2 years and 6 months to 3 years and 10 months (median: 2 years and 11 months). In four of them, the postoperative right ventricular to aortic or left ventricular systolic pressure ratios were less than 0.4 without any significant systolic pressure gradients between pulmonary artery and right ventricle. In these four patients, the diameters of the reconstructed pulmonary trunks grew from 10-18 mm to 18-21 mm postoperatively. These diameters were more than 100% of normal values. In the remaining patient with tetralogy of Fallot and single coronary artery, the obstruction of the new pulmonary trunk by a bulged left atrial appendage, which was used as the posterior wall, was observed on the right ventricular outflow tract reconstruction without an extracardiac conduit has growth potential in the future.     

Outward K+ current densities and Kv1.5 expression are reduced in chronic human atrial fibrillation

Chronic atrial fibrillation is associated with a shortening of the atrial action potential duration and atrial refractory period. To test the hypothesis that these changes are mediated by changes in the density of specific atrial K+ currents, we compared the density of K+ currents in left and right atrial myocytes and the density of delayed rectifier K+ channel alpha-subunit proteins (Kv1.5 and Kv2.1) in left and right atrial appendages from patients (n = 28) in normal sinus rhythm with those from patients (n = 15) in chronic atrial fibrillation (AF). Contrary to our expectations, nystatin-perforated patch recordings of whole-cell K+ currents revealed significant reductions in both the inactivating (ITO) and sustained (IKsus) outward K+ current densities in left and right atrial myocytes isolated from patients in chronic AF, relative to the ITO and IKsus densities in myocytes isolated from patients in normal sinus rhythm. Quantitative Western blot analysis revealed that although there was no change in the expression of the Kv2.1 protein, the expression of Kv1.5 protein was reduced by > 50% in both the left and the right atrial appendages of AF patients. The finding that Kv1.5 expression is reduced in parallel with the reduction in delayed rectifier K+ current density is consistent with recent suggestions that Kv1.5 underlies the major component of the delayed rectifier K+ current in human atrial myocytes, the ultrarapid delayed rectifier K+ current, IKur. The unexpected finding of reduced voltage-gated outward K+ current densities in atrial myocytes from AF patients demonstrates the need to further examine the details of the electrophysiological remodeling that occurs during AF to enable more effective and safer therapeutic strategies to be developed.     

The role of a bidirectional cavopulmonary anastomosis in the correction and palliation of complex congenital cardiopathies

Between May 1990 and January 1998, 68 patients underwent bidirectional cavo-pulmonary anastomosis. We evaluated all patients in whom the bidirectional cavo-pulmonary anastomosis was associated with additional pulmonary flow (group A) and those in whom it was associated with biventricular repair (group B). Group A included 23 patients (33.8%), 14 males and 9 females, mean age 25 years and 6 months (range 4 months-16 years). Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). Group B included 13 patients (19.1%), 6 males and 7 females, mean age 13 years and 7 months (range 1 year-37 years). Diagnoses were pulmonary atresia with intact ventricular septum (4), Ebstein's anomaly (3), tetralogy of Fallot (3), atrioventricular canal (1), hypoplastic right ventricle (1), and pulmonary and tricuspid insufficiency (1). Four patients (17.3%) in group A died in the postoperative period, whereas there was no postoperative mortality in group B. Follow-up data were available in 31 patients (19 from group A, 13 from group B). Mean follow-up was 1 year and 6 months (range 30 days to 6 years). Evaluation was done by NYHA class functional status. In group A, 14 patients are doing well (NYHA I or II), while five patients (26.3%) underwent Fontan operation with one death. All group B patients are currently doing well (NYHA class I or II). In group A, complications were pericardial effusion (7), transient superior vena cava syndrome (5), pleural effusion (4), chylothorax (1) and rhythm disturbance (1). Complications in group B involved neurological events (2), pleural effusion (1) and rhythm disturbance (1). Bidirectional cavo-pulmonary anastomosis can be associated with additional pulmonary flow with good short- and intermediate-term outcome. Concern remains for the ability to properly regulate the amount of effective pulmonary blood flow. Bidirectional cavo-pulmonary anastomosis can be associated with biventricular repair in patients with diminutive right ventricles, amenable to anatomic complete correction, with good clinical outcome.     

Total anomalous pulmonary venous connections and consideration of the Fontan or one-ventricle repair

There is now a considerable literature that babies with right atrial isomerism have a poor outcome. The reasons for this are complex and multifactorial, but may be related at least in part to intrinsically small and abnormal pulmonary veins. We reviewed a series of consecutive patients seen at a single institution and found that babies with right atrial isomerism, severe pulmonary outflow tract obstruction or atresia, and total anomalous obstructed pulmonary veins had a grim outlook, especially those requiring operation in the neonatal period. Others have reported a similarly concerning outcome.     

Haemodynamics and body fluid volumes in response to fluid loading in conscious dogs: non-excretory renal influences

1. Twelve conscious, chronically instrumented dogs were subjected to rapid loading with sodium chloride solution (150 mmol/1; saline) before and 1 day after bilateral nephrectomy (six dogs) or ureterocaval anastomosis (six dogs). Measurements were performed up to 3 h after the fluid load and included cardiac output with an electromagnetic flowmeter, mean arterial pressure and right atrial pressure with chronically implanted catheters, interstitial fluid pressure with a plastic capsule, heart rate, extracellular fluid volume, erythrocyte volume, plasma volume, plasma protein concentration and other variables. 2. The increase in cardiac output in response to saline load was significantly prolonged in the anephric dogs compared with those with uretero-caval anastomosis; mean arterial pressure, right atrial pressure and heart-rate changes were similar in both groups. 3. Plasma volume appeared to increase more in the anephric dogs than in those with uretero-caval anastomosis during the first hour after the infusion, although conflicting results were obtained with different estimates of plasma volume changes. Interstitial fluid pressure increased significantly less in the anephric dogs in the early stages of the fluid load. 4. Effective vascular compliance (the ratio of the change in blood volume to the change in right atrial pressure) appeared increased in the anephric dogs. On the other hand, the change in cardiac output for a given change in right atrial pressure was found to increase after bilateral nephrectomy. 5. It is suggested that the prolonged increase in cardiac output observed in anephric dogs was not the consequence of preferential plasma volume expansion nor of decreased venous compliance, but may reflect an alteration in the cardiac function curve.     

The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia

Left-sided juxtaposition of the right atrial appendage (LJRAA) was seen in 10 patients in a series of 361 consecutive Mustard procedures for transposition of the great arteries (TGA). Dextrocardia complicated LJRAA in four cases. Right atrial capacity and free atrial wall size were smaller than normal in all patients, and a Mustard intra-atrial baffle repair was performed in all instances. Direct caval cannulation or cannulation of either atrial appendage facilitated intra-atrial repair. Nine patients in whom the pulmonary venous atrium was enlarged with a patch survived. One child who did not have a right atrial patch died 1 hour postoperatively of pulmonary edema. Two late deaths occurred 1 year postoperatively. Seven children survived and are well. In one child, superior vena caval baffle obstruction is controlled by digoxin and diuretic therapy. TGA associated with juxtaposition of the atrial appendages (JAA) and dextrocardia may be successfully repaired by the Mustard procedure, provided that the tricuspid valve and right ventricle are normal, an adequate superior vena caval channel is created, and patch enlargement of the pulmonary venous atrium is undertaken.     

The bicaval anastomosis technique for orthotopic heart transplantation yields better atrial function than the standard technique: an echocardiographic automatic boundary detection study

BACKGROUND: Atrial function is an important determinant of cardiac performance. In patients who undergo operation by standard heart transplantation atrial enlargement, distortion of geometry and asynchronous contraction resulting from the donor/recipient atrial connections may affect atrial function. The bicaval anastomosis technique should be free from these limitations. METHODS: We used the echocardiographic automatic boundary detection technique to obtain on-line time/volume curves of right and left atria from patients who had undergone bicaval (n = 22) or standard (n = 27) heart transplantation and from 15 control subjects. Maximal, middiastolic, preatrial contraction, and minimal volumes of both atria were measured. Reservoir volume (defined as the difference between maximal and middiastolic atrial volumes); pump volume (defined as the difference between preatrial contraction and minimal atrial volumes); and conduit volume (defined as the difference between left ventricular stroke volume and the sum of reservoir and pump volumes) were derived for both atria. Atrial emptying fraction was calculated as the difference between maximal and minimal volumes divided by the maximal volume and expressed in percent and pump fraction as the pump volume divided by the sum of reservoir and pump volumes. Tricuspid and mitral regurgitation, evaluated by color-flow Doppler scanning, were considered significant when they were greater than grade 1. Atrial ejection force was calculated from mitral and tricuspid flow velocities at atrial contraction. RESULTS: In patients who had bicaval heart transplantation, both atria were smaller than in patients who underwent standard heart transplantation. With the bicaval technique right and left atrial emptying (right 45% +/- 9% vs 36% +/- 10%, p < .05; left 51% +/- 8% vs 39% +/- 8%, p < .001) and pump fractions (right 57% +/- 17% vs 19% +/- 13%, p < .001; left 45% +/- 28% vs 22% +/- 12%, p < .01) were greater than with the standard technique and similar to those in control subjects. Right atrial ejection force was significantly greater in bicaval (10.0 +/- 5.6 kdyne) than in standard heart transplantation (4.5 +/- 2.2 kdyne, p < .0001). Significant tricuspid or mitral regurgitation was rarely found in bicaval heart transplant recipients (3 and 1 of the 22 patients, respectively), although they were much more frequent after standard heart transplantation (13 and 8 of the 27 patients, respectively). CONCLUSIONS: Heart transplantation performed with the bicaval anastomosis technique determines smaller atrial volumes, yields better right and left atrial function and fewer atrioventricular valve regurgitation than the standard technique.