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.     

A modification of hepatic portoenterostomy (Kasai operation) for biliary atresia

BACKGROUND AND AIMS: The aim of the present study was to compare power Doppler imaging with traditional color Doppler imaging and with contrast enhanced computer tomography in the evaluation of intratumoral vascularity of hepatocellular carcinomas at diagnosis and in response to percutaneous ethanol injection. PATIENTS AND METHODS: Sixteen patients with hepatocellular carcinoma underwent colour Doppler, power Doppler and computed tomography at diagnosis. Seventeen patients were studied by the three techniques one month after percutaneous ethanol injection treatment. RESULTS: At baseline evaluation, power Doppler and color Doppler were always in agreement and, with the exception of one case, were also in agreement with the computerized tomography scan. On the contrary, power Doppler and computerized tomography are more sensitive than color Doppler in the evaluation of residual vascularized tumoral tissue after percutaneous ethanol injection. In 3 patients, residual vascularity was demonstrated only by computerized tomography while color and power Doppler were negative. In another 3 cases, a positive power Doppler signal, with a typical arterial Doppler spectrum, was observed while color Doppler and computerized tomography were negative. In these patients, cancer relapse was clinically evident after a few months and treatment was repeated to obtain complete necrosis. CONCLUSIONS: We conclude that only the integration of the results of all these techniques can reliably evaluate tumoral vascularity after percutaneous ethanol injection.     

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.     

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.     

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.     

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.     

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.     

Hepatocellular carcinoma complicating biliary cirrhosis caused by biliary atresia: report of a case

A case of hepatocellular carcinoma complicating biliary cirrhosis caused by biliary atresia is reported. The patient had persistent severe jaundice with hepatosplenomegaly. A liver tumor was suspected because of the elevated serum alpha-fetoprotein and was shown by ultrasonography at 6 years of age. The tumor was treated with percutaneous ethanol injection therapy (PEIT). Nine months after initiation of PEIT, the patient died of massive bleeding from a metastatic tumor.     

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.     

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.     

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.     

Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment

Castleman's disease, also known as benign giant lymph node hyperplasia, is a lymphoproliferative disorder which can occur either in a localized or multicentric form. The latter is characterized by the development of malignant lymphoma, Kaposi's sarcoma or carcinoma in 32% of cases. By contrast, localized Castleman's disease has exceptionally been reported in association with non-Hodgkin's lymphoma and - to the best of our knowledge - never at the same anatomic site. Here we describe the occurrence in the same lymph node of localized Castleman's disease (with monotypic plasma cell component) and an apparently unrelated peripheral small B-lymphocytic lymphoma.     

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.     

Pryce type I interlobar pulmonary sequestration with anomalous return to the left inferior pulmonary vein

A 50-year-old woman was examined because of chest discomfort. Chest X-ray films disclosed a tumorous shadow behind the heart. Chest computed tomography (CT) scans revealed a mass connected to the descending aorta, with increased blood flow in the left basal segment. The patient was admitted for further examination. Chest CT scans and cardioscintigrams were very useful as diagnostic tools, but the final diagnosis was made on the basis of angiography. An anomalous tortuous artery ran from the descending aorta into the left basal segment and returned to the left inferior pulmonary vein. The left pulmonary arterial trunk had no basal branch (A8-A10). A loop corresponding to the superior vein (V6) ran beneath the anomalous tortuous artery. To our knowledge, this is the second case of Pryce type-I interlobar pulmonary sequestration with anomalous return to the left inferior pulmonary vein to be reported in Japan. A left inferior lobectomy was performed. Histological finding from the excised tissues showed prominent interstitial fibrosis, atypical adenomatous hyperplasia, and atherosclerosis. Following surgery, the patient's PaO2 increased from 80.4 Torr to 95.8 Torr, suggesting that left inferior lobectomy was an appropriate treatment.     

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.     

A rare form of total abnormal pulmonary stenosis return: atresia of the common pulmonary vein

One case of atresia of the common pulmonary vein is discussed at the light of the few reports found in the literature. The presence of criteria distinguishing this type of obstacle to pulmonary venous return from the other cases, is of practical diagnostic interest; indeed, its diagnosis leads to surgical intervention, theoretically curative.     

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.     

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.