Development Of Pulmonary Arteriovenous Fistulas Research Articles

Efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with univentricular heart after total cavopulmonary connection.

Development of pulmonary arteriovenous fistulas in patients with cavopulmonary anastomosis may result in a significant morbidity. Although the use of bubble contrast echocardiography with selective injection into both the branch pulmonary arteries in identifying pulmonary arteriovenous fistulas has been increasing, the actual efficacy of this diagnostic modality has not been properly evaluated. Thus, this study aimed to assess the efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with total cavopulmonary connection. A total of 140 patients were included. All patients underwent cardiac catheterisation. Bubble contrast echocardiographic studies were performed by injecting agitated saline solution into the branch pulmonary arteries. Transthoracic echocardiograms that use an apical view were conducted to assess the appearance of bubble contrast in the systemic ventricles. Then, the contrast echocardiogram results and other cardiac parameters were compared. No correlation was found between contrast echocardiogram grade and other cardiac parameters, such as pulmonary capillary wedge saturation and pulmonary artery resistance. Moreover, only 13 patients had negative results on both the right and left contrast echocardiograms, and 127 of the 140 patients had positive results on contrast echocardiograms even though they had normal pulmonary capillary wedge saturation. Results showed that bubble contrast echocardiography was a highly sensitive method and was likely to obtain false-positive results. Bubble contrast echocardiography might be highly false positive in detecting pulmonary arteriovenous fistulas in patients with cavopulmonary anastomosis. We have to consider how we make use of this method. Further standardisation of techniques is required.

A 10-year-old boy with dyspnea and hypoxia: abernathy malformation masquerading as pulmonary arteriovenous fistula

BackgroundAbernethy malformation is an extremely rare congenital malformation characterised by an extrahepatic portosystemic shunt. Children with Abernathy malformation can develop hepatopulmonary syndrome (HPS) with pulmonary arteriovenous fistulas (PAVF) or pulmonary hypertension. PAVF manifests as central cyanosis with effort intolerance. We report a case of PAVF in a Ten-year-old Boy. Persistent symptoms identified Abernathy malformation as the cause of progressive symptoms and current understanding of this rare malformation is reviewed.Case presentationA case of 10-year-old boy with Abernethy malformation complicated with HPS initially managed as PAVF was presented. Selective lung angiography showed a typical diffuse reticular pattern on right lower lung, which suggested PAVF. However, cyanosis was not improved post transcatheter coil embolization. Then, liver disease was considered although the patient had normal aspartate aminotransferase and alanine aminotransferase. The significantly elevated serum ammonia was attracted our attention. Abdominal computed tomography also exhibited enlarged main portal vein (MPV), cirsoid spleen vein, and superior mesenteric vein (SMV). Angiography with direct opacification of the SMV with a catheter coming from the inferior vena cava (IVC) and going to the SMV via the shunt vessel (SHUNT) between the MPV and IVC. Occlusion the IVC with an inflated balloon, injection of contrast medium via a catheter placed in the SMV, MPV was showed and absence of intrahepatic branches. Abernethy malformation IB type is finally confirmed.ConclusionsAbernethy malformation is an unusual cause for development of PAVF and cyanosis in children. Clinicians must be suspicious of Abernethy malformation complicated with HPS. If patients have abnormal serum ammonia and enlarged MPV in abdominal CT, cathether angiography should be done to rule out Abernethy malformation.

Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism.

Background and ObjectivesWe studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis.Subjects and MethodsWe reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014.ResultsOf the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age.ConclusionPatients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

Development of pulmonary arteriovenous fistulas after bidirectional cavopulmonary shunt

Background. A high incidence of pulmonary arteriovenous fistulas (PAVF) has been reported after bidirectional cavopulmonary shunt (BCPS) or total cavopulmonary shunt (TCPS; BCPS in patients with interrupted inferior vena cava). However, the definite diagnostic criteria or standard diagnostic modality of PAVF has not yet been defined. The goal of this study was to evaluate the diagnostic modalities and the prevalence of PAVF. Methods. We selected 10 patients with TCPS and 27 patients with BCPS. Lung perfusion scan, contrast echocardiogram, and pulmonary angiogram were performed. The results were compared among groups of patients and among each diagnostic modality. Results. All 10 patients with TCPS and 16 and 13 patients with BCPS showed positive results on contrast echocardiograms and lung scans, respectively. Six patients with TCPS and 4 patients with BCPS showed positive results on pulmonary angiograms. All patients with TCPS developed subclinical or clinical PAVF and 19 patients with BCPS developed subclinical PAVF and none of them had clinical PAVF during the short-term follow-up. Conclusions. Most patients with bidirectional cavopulmonary anastomosis have subclinical evidence of right-to-left intrapulmonary shunting. This problem can be demonstrated with various diagnostic modalities.

Pulmonary and systemic arteriovenous fistulas in patients with left isomerism.

Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.

An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it?

AbstractBackgroundThe bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functionally single ventricle. Despite its usefulness, there are a number of concerns following its construction, including issues of timing, the development of pulmonary arteriovenous fistulas, and growth of the pulmonary arteries.MethodsBetween January 1990 and April 1996, we constructed a bidirectional cavopulmonary shunt in 123 patients. Median age at surgery was 9.4 months, and ranged from 24 days to 43 years. In 25 patients (20%), it was the first operative procedure. An auxiliary source of pulmonary blood flow was included in 72 patients (58%).ResultsSix patients (4.9%) died in the early postoperative period, and the overall early rate of failure (death or take-down) was 8.1%(n=10). By multivariate analysis, longer bypass time (p=0.012), age less than 1 month (p=0.03), and higher pulmonary vascular resistance (p = 0.043) were significant. Early reoperation was performed in 11 other patients to decrease (n=8) or increase (n=3) the amount of pulmonary blood flow. Early survivors were followed for a median of 15 months (range: 1 to 48 months), during which time 5 patients died and a Fontan circulation was completed in 30. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively. Among hospital survivors, the only significant predictor of poorer survival by Cox regression was age less than 2 months at the time of the initial cavopulmonary shunt (p=0.02). Atrioventricular valvar regurgitation was decreased after construction of the cavopulmonary shunt in 17 of the 28 patients (61%) in whom echocardiographic data were available to compare preoperative and postoperative status.ConclusionsThe bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate term management of patients with a functionally univentricular heart. Much remains to be learned, nonetheless, about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome (isomeric atrial appendages).

Augmentation of pulmonary blood flow with an axillary arteriovenous fistula after a cavopulmonary shunt

Of 320 children with cyanotic congenital heart malformations who had previously undergone cavopulmonary shunt operations, 11 had increasing cyanosis and exercise intolerance and were considered unsuitable for definitive repair, a Fontan procedure, or other palliation. Eight had a previous Glenn shunt and three had a previous bidirectional cavopulmonary connection. To augment pulmonary blood flow, 10 patients underwent creation of an ipsilateral axillary arteriovenous fistula. Mean oxygen saturations were 80% ± 2% before operation, 85% ± 2% immediately after operation, and 84% ± 3% at a mean follow-up interval of 7.4 years (range 0.1 to 15.5 years). Mean hemoglobin values were 202 ± 10 gm/L before operation, 177 ± 10 gm/L after operation, and 191 ± 14 gm/L at latest review. The only complication was mild swelling of the arm distal to the fistula in one patient. All patients reported improvement in exercise tolerance. Eight patients have continued evidence of fistula patency. Development of ipsilateral pulmonary arteriovenous fistulas has not been observed in any patient. Creation of an axillary arteriovenous fistula to augment pulmonary blood flow after a cavopulmonary shunt provides useful palliation for complex cyanotic heart disease when other options are limited. Such additional sources of pulmonary blood flow may influence the development of pulmonary arteriovenous fistulas. (J T HORAC C ARDIOVASC S URG 1996;111:176-80)

Fulminant Development of Pulmonary Arteriovenous Fistulas in an Infant After Total Cavopulmonary Shunt

Fulminant Development of Pulmonary Arteriovenous Fistulas in an Infant After Total Cavopulmonary Shunt