Modified repair of mixed anomalous pulmonary venous connection

A modified repair technique is reported for mixed total or partial anomalous pulmonary venous connection with the right superior pulmonary vein connecting to the superior vena cava, the right inferior pulmonary vein to the right atrium or left atrium, and the left pulmonary veins to the coronary sinus. The superior vena cava is transected above the highest right superior pulmonary vein, its cephalad end is anastomosed to the right atrial appendage, and a pericardial baffle is constructed between the cardiac ostium of the superior vena cava, the ostium of the right inferior pulmonary vein, and the left atrium, including the coronary sinus, which is unroofed. The reported technique may be valuable to avoid pulmonary venous obstruction in complex mixed forms of total or partial anomalous pulmonary venous connection.     

Total anomalous pulmonary venous connection in neonates and young infants: repair in the current era

Total anomalous pulmonary venous connection has been one of the more challenging congenital heart defects in newborns and young infants despite improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care. Since 1981, 30 patients with total anomalous pulmonary venous connection have undergone primary total correction. Mean age at operation was 28 +/- 6 days and mean weight, 3.3 +/- 0.7 kg. Essential features of the surgical approach in these small patients included early surgical intervention, profound hypothermia with total circulatory arrest, cardioplegic myocardial preservation, and a wide posterior anastomosis. Operative mortality was 13% +/- 6%. All four deaths were in patients having emergency operation within 24 hours of the surgical consult and requiring ventilator support preoperatively. The mean follow-up is 47 +/- 7 months. There have been two late deaths, and the 7-year survival rate is 79% +/- 8%. There have been two reoperations, and 91% +/- 6% of the patients are reoperation free at 7 years. Only 1 of the 24 surviving patients is symptomatic. Growth in survivors is closely monitored. The height growth percentile is less than 5% in 15% +/- 8% of survivors and the weight growth percentile, less than 5% in 17 +/- 8%. During the past decade, with a consistent surgical approach to neonates and infants with total anomalous pulmonary venous connection, it has been possible to achieve low early mortality, low attrition, and excellent late results.     

Total anomalous pulmonary venous return complicating portoenterostomy for biliary atresia

Cardiovascular anomalies such as absent inferior vena cava and preduodenal portal vein are reported in cases of biliary atresia and make hepatic portoenterostomy a technical challenge. The authors present the case of a severe cardiac anomaly that significantly altered the functional outcome of a Kasai procedure. Baby M., an 8-week-old boy born with total anomalous pulmonary venous return (TAPVR), underwent hepatic portoenterostomy for biliary atresia. Over the next 3 months he remained icteric and febrile, and failed to gain weight. After multiple antibiotic treatments for suspected cholangitis, he underwent reexploration of the portoenterostomy, with no improvement in his overall condition. His prognosis was considered dismal because correction of the cardiac anomaly is associated with a high mortality rate (> 90%). The cardiac surgeon agreed to attempt a cure of the TAPVR, provided liver transplantation is contemplated if the patient survived. Within 48 hours postoperatively, his hepatic function had improved drastically. He became afebrile, had an improved appetite and weight gain, and was finally discharged 203 days after admission. One year later, he is thriving and remains anicteric. The exact reason for this drastic improvement is not well understood, but the right-sided cardiac failure caused by the TAPVR had a significant effect on the functional outcome of the portoenterostomy.     

Total anomalous pulmonary venous connection. Results of a small consecutive series

The surgical experience in 13 infants with total anomalous pulmonary venous connection (TAPVC) between 1987 and 1991 is reviewed. The age vary from 2 days to 35 months with a weight at intervention from 2.130 kg to 5.400 kg. The types of TAPVC were supracardiac in 4 patients, cardiac in 4, and infracardiac in 5. Seven patients (54%) were operated on in emergency. Cardiopulmonary bypass consisted of profound hypothermia and total circulatory arrest in 8 patients (60%) and continuous hypothermic bypass with low flow for the remaining 5 patients (40%). There was no operative death. The follow-up ranges from 21 to 58 months, mean 40 months. There was one reoperation. All the patients were asymptomatic and the height growth percentile is less than 5% in 20%, and the weight growth percentile is less than 5% in 30%. The early repair of infants born with TAPVC can be done with low morbidity with a good prospect on medium term follow-up.     

Atrial septal displacement for repair of anomalous pulmonary venous return into the right atrium

BACKGROUND: In the repair of anomalous connection of the pulmonary veins to the right atrium, the use of a baffle of pericardium to divert the pulmonary venous blood into the left atrium could cause pulmonary venous obstruction as a result of thickening of the pericardial patch. Anomalous pulmonary venous drainage to the right atrium caused by malposition of the atrial septum primum can be repaired by displacing the shifted septum primum to the normal position. METHODS: In 5 patients with total (n=2) or partial (n=3) anomalous pulmonary venous drainage into the right atrium, the septum primum was shifted toward the left atrium and the pulmonary veins drained into the anatomic right atrium despite their normal connection with the posterior wall of the left atrium. This method consisted of incision of the posterior edge of the atrial septum primum and displacement of the incised atrial septum between the anomalous pulmonary veins and both venae cavae. No patch was used. RESULTS: Postoperative echocardiography showed a wide pathway from the pulmonary veins to the left atrium with no stenotic portions. No atrial arrhythmias occurred after the operation. CONCLUSIONS: This technique may be advantageous because it allows for future growth of the route of the pulmonary venous pathway and avoids postoperative supraventricular arrhythmias.     

Three unusual complications resulting from attempted repair of partial anomalous pulmonary venous drainage

The correction of shunts resulting from partial anomalous pulmonary venous drainage has become an accepted surgical procedure. Surgical complications, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual complications resulting from this type of surgery. Unilateral pulmonary venous obstruction and repeated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an indaequate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.     

Is vertical vein ligation necessary in repair of total anomalous pulmonary venous connection?

BACKGROUND: In the repair of total anomalous venous connection, vertical vein ligation is recommended to eliminate left-to-right shunting. However, the small left heart chambers may not always tolerate the immediate increase in blood flow after combined repair and vein ligation. METHODS: A retrospective review of 23 infants and children undergoing correction of total anomalous pulmonary venous connection was undertaken to determine whether vertical vein ligation is a necessary component of successful surgical repair. In 14 patients this vein was ligated, whereas in 9 it was left patent. Six patients who underwent ligation and 5 who did not had pulmonary venous obstruction before operation. RESULTS: The operative mortality rate was 36% (5 of 14 patients) for the ligated group compared with 0% (0 of 9 patients) for the nonligated group (p = 0.06). All deaths occurred in patients with preoperative obstruction and a low mean left atrial pressure, and four of the deaths were directly attributable to left heart failure. Follow-up echocardiography in patients in whom the vertical vein was not ligated revealed adequate cardiac function and no residual left-to-right flow through the previously patent venous conduit. CONCLUSION: Vertical vein ligation during the repair of total anomalous pulmonary venous connection is not routinely necessary and actually may be undesirable in patients with preoperative obstruction, in whom the left heart chambers are particularly small.     

Neonates with congenital heart disease, Part IV: Total anomalous pulmonary venous return

Significant radiographic findings of TAPVR with unobstructed blood flow include the following: 1. Increased pulmonary vascular markings with dilated pulmonary arteries 2. Cardiomegaly 3. A "snowman" configuration of the heart, seen after pulmonary vascular resistance falls and blood flow through the lungs increases In TAPVR with obstructed blood flow, findings include the following: 1. A hazy, reticular, ground-glass appearance of the lung fields 2. Normal to small cardiac size.     

Surgical repair of coronary sinus type partial anomalous pulmonary venous drainage with intact atrial septum

A method to prevent co-elution of steroid sulfates with proteins in serum from the pre-column in column-switching HPLC was developed. The pre-column, a polymer-coated mixed function column, was used for ion-pair chromatography with 5 mM tetra-n-butylammonium (TBA) ion. As steroid sulfates, estriol 3-sulfate, dehydroepiandrosterone 3-sulfate and pregnenolone 3-sulfate were used. Human serum (25 microl) was diluted with mobile phases including 5, 100 and 500 mM TBA ion, and then injected directly into the pre-column. The peak areas of the steroid sulfates in serum samples were compared with those of the steroid standards without serum. When 25/microl of serum was diluted with mobile phase including 100 or 500 mM TBA ion, the steroid sulfates in serum were retained in the pre-column; however, the steroid sulfates from the same sample diluted with mobile phase containing 5 mM TBA ion were not retained in the pre-column. Addition of an excess amount of counter ion (TBA ion) into the serum sample made it possible to retain the steroid sulfates in the pre-column. This method was applied to column-switching HPLC for measurement of steroid sulfates in serum using a semi-microcolumn as the analytical column.     

Successful repair of total anomalous pulmonary venous connection and incomplete endocardial cushion defect associated with left isomerism

Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.     

Tree-shaped pulmonary veins in infracardiac total anomalous pulmonary venous drainage

Three consecutive patients undergoing corrective operation for the infracardiac type of total anomalous pulmonary venous drainage (TAPVD) were found to have tree-shaped pulmonary veins. Preoperative angiocardiography revealed that in 2 patients the superior and inferior pulmonary veins drained separately, bilaterally, into the vertical vein. In the third patient the right pulmonary veins united to connect with the vertical vein, while the left superior and inferior pulmonary veins drained separately into the vertical vein. At operation inferior pulmonary veins connecting separately with the vertical vein were found to be located posterior to the pericardium. In the previous literature dealing with successful repair of infracardiac TAPVD, there is no mention of the tree-shaped pulmonary veins described in this report. As this particular type of pulmonary vein does not seem to be uncommon, its possible presence should be kept in mind during operation, as it may dictate the selection of surgical procedures.     

Echocardiographic spectrum of supracardiac total anomalous pulmonary venous connection

We report an unusual case of a 13-year-old girl with a benign osteoma associated with a cholesteatoma in the external auditory canal and serous otitis media. The osteoma was located in the antero-inferior wall of the right external auditory canal. A cholesteatoma was present between the osteoma and the tympanic membrane. Computed tomography revealed a soft tissue density within the external auditory canal and in the middle ear cleft. The shadow in the middle ear cleft was considered to represent the serous otitis media. Surgical removal of the osteoma and cholesteatoma proved successful, and no recurrences or complications have occurred in the first year postoperatively.     

Myocardial recovery through ECMO after repair of total anomalous pulmonary venous connection: the importance of left heart unloading

A 7-day-old boy who had been placed on extracorporeal membrane oxygenation on his second day of life developed biventricular failure after undergoing surgical repair of a supracardiac variant of total anomalous pulmonary venous connection. Extracorporeal membrane oxygenation was again necessary for postoperative cardiopulmonary support. However, severe left ventricular failure made it imperative to leave the vertical vein open during support in order to decrease pressure on the left ventricle. The patient was successfully weaned from extracorporeal membrane oxygenation on day 8 after surgery and discharged from the hospital on day 23.     

Tetralogy of Fallot with absent pulmonary valve and total anomalous pulmonary venous connection

Prenatal diagnosis of chromosomal disorders is generally offered to women who will be 35 years or older at the time of delivery or who have been determined via serum screening to be at risk similar to that of a woman older than 35 years. This age threshold was based on 4 major rationales that reflect considerations of resources and effectiveness. In this paper, we explore the current screening recommendations and consider new information that calls the 35-years threshold into question. We conclude that guidelines regarding use of prenatal diagnosis account for the preferences of the individual patient as well as for individual risk.     

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.     

Diversion of the inferior vena cava into the left atrium after repair of atrial septal defect with partial anomalous pulmonary venous drainage

An 11-year-old female had operative repair of atrial septal defect associated with partial anomalous pulmonary venous drainage by direct suture at 6-year-old. Postoperatively, cyanosis and nodal bradycardia were noted by physical exercise. During 5 years thereafter, the symptoms were gradually aggravated accompanying. Cardiac catheterization revealed diversion of the inferior vena cava into the left atrium. Oxygen sampling showed right to left shunt of 22% at the atrial level. At reoperation, orifice of the inferior vena cava into the right atrium became narrow 5 mm in diameter. After enlargement of the orifice, the atrial septum was created with a Gore-Tex patch to redirect the inferior vena cava to the right atrium. Post operative course was uneventful with disappearance of the cyanosis. The patient is up and well now 9 years after reoperation.     

Cardiac function in total anomalous pulmonary venous return before and after surgery

Cardiac performance was evaluated in 12 infants with isolated total anomalous pulmonary venous return. Four had significant pulmonary venous obstruction and severe pulmonary hypertension (group A). Eight had no obvious venous obstruction, and the pulmonary pressures were lower (group B). In all subjects, right ventricular end-diastolic volume was increased (197% of predicted normal) and its ejection fraction was normal. Left ventricular volume was, generally speaking, still in the normal range (87% of predicted normal); however, its ejection fraction was reduced (0.57 vs normal of 0.73) and left ventricular output was low (3.08 L/min/m2 vs normal of 3.98). Left atrial volume was consistently small (53% of predicted normal) with an appendage of normal size. The infants in group A had smaller chamber volumes/m2 BSA than those in group B. Left atrial function was abnormal, characterized by reduced reservoir function and a greater role as "conduit" from right atrium to left ventricle. Left atrial size was not found to be critical in the surgical repair of TAPVR. Cardiac function is restored to normal following surgery.     

The superior approach for correction of the supracardiac type of total anomalous pulmonary venous return

An alternative approach for correction of supracardiac (type I) total anomalous pulmonary venous return is described. A median sternotomy is used. The posterior wall of the left atrium and the common pulmonary venous trunk are exposed through the transverse sinus. A direct anastomosis between these structures, ligation of the systemic-venous connection (vertical vein), and closure of the interatrial septal defect results in a one-stage repair. In our experience with the supracardiac anomaly in 20 patients, we have found that this approach consistently affords better exposure than other techniques currently in use for surgical correction of this anomaly.     

Partially anomalous pulmonary venous connection: demonstration of dual drainage allowing nonsurgical correction

The 13C-urea breath test (13C-UBT) is a non-invasive method for detecting Helicobacter pylori. This study was performed to determine the cutoff value and evaluate the sensitivity and specificity of 13C-UBT in Taiwan. 13C-Urea (100 mg of 99% 13C-labeled urea) was dissolved in 50 ml sterile water for the test. The test meal for delaying gastric emptying was 100 ml fresh milk. Patients fasted for at least 6h. A baseline breath sample was collected 5 min after they had the test meal. Two other samples were collected at 15 and 30 min after the patients ingested the 13C-urea. The test was evaluated in 352 patients after routine upper gastrointestinal endoscopy, and the urease test, culture, and histopathology were taken as the gold standards for detecting H. pylori. According to the receiver operating characteristic (ROC) curves, we chose values of 2.8 and 4.2 excess delta 13CO2 per mil as the cut-off values for 15 and 30 min, respectively, post 13C-urea. The sensitivity and specificity of 13C-UBT were 99% and 93% at 15 min, and 98% and 93% at 30 min post 13C-urea, respectively. The 13C-UBT breath test is an efficient non-invasive method of high sensitivity and high specificity for detecting H. pylori infection. We suggest that the use of fresh milk as the test meal and the detection of excess delta 13CO2 15 min after the ingestion of 13C-urea are suitable for the clinical use of 13C-UBT. This test is simple and rapid.     

Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies

BACKGROUND: Recent reports have cited improving results for surgical management of isolated total anomalous pulmonary venous drainage. Complex cases (with other cardiac anomalies) are less frequently reported and are associated with higher mortality. METHODS: Retrospective review identified 170 consecutive patients treated for total anomalous pulmonary venous drainage from 1982 to 1996: 44 cases were "complex" (with significant associated cardiac lesions) and 126 cases were "simple." RESULTS: Operative mortality for simple cases decreased from 26% to 8%, and mortality for complex cases remained constant at 52%. Age, size, and the presence of atrial isomerism were univariate predictors of mortality. Multivariable analysis identified only univentricular hearts and associated cardiac lesions as predictors of operative mortality. Pulmonary artery (n = 16) and arteriopulmonary (n = 7) shunting strategies for complex cases resulted in less than 30% long-term survival. CONCLUSIONS: Despite improvement in survival for simple cases, management of total anomalous pulmonary venous drainage with single-ventricle hearts or other associated cardiac lesions remains problematic.