Redirection of the Hepatic Venous Flow for the Treatment of Pulmonary Arteriovenous Malformations after Fontan Operation

A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung. He underwent revision of the conduit to attain balanced hepatic venous drainage with subsequent regression of the PAVMs and disappearance of cyanosis. Our experience shows that deficiency of hepatic venous flow played a crucial role in the development of PAVMs and that redistribution of the flow retained potential to ameliorate the pathological condition.     

Regression of pulmonary arteriovenous malformations following heart transplantation

Pulmonary arteriovenous malformations (PAVMs) can occur following caval to pulmonary artery connection, Glenn and/or Fontan procedure, leading to severe cyanosis and exercise intolerance. It is unknown whether these abnormalities regress or persist following heart transplantation (HTx). Twenty patients with failed Fontan or Glenn procedures were screened for PAVMs prior to HTx by contrast echocardiography, selective pulmonary angiography, and pulmonary venous desaturation. Age at transplant, diagnosis, previous operations, time from Glenn to transplant, systemic oxygenation, hemoglobin level, and ventricular function were determined. The clinical course after HTx was characterized in three patients with significant PAVMs. Indications for HTx were exercise intolerance and severe cyanosis in one patient, and cyanosis and ventricular dysfunction in two. Pre-HTx, mean systemic saturation was 67%; mean pulmonary venous wedge saturation was 81%. Post-HTx, oxygen saturations were normal (> 96%) at 14, 40, and 180 days. Contrast echocardiography, performed 1 month to 3.3 yrs after HTx, showed no intrapulmonary shunting in two patients and minimal shunting in one. One patient suffered an embolic stroke from right-to-left shunting post-HTx. All patients are alive and well 35, 71, and 73 months post-HTx. In patients with single ventricle physiology, PAVMs are not an absolute contraindication to HTx. Heart-lung transplant may not be required for these patients.     

Color flow mapping to document normal pulmonary venous return in neonates with persistent pulmonary hypertension being considered for extracorporeal membrane oxygenation

This study investigated the value of color flow mapping in documenting normal pulmonary venous return in neonates with persistent pulmonary hypertension who were candidates for extracorporeal membrane oxygenation (ECMO). Forty newborn infants with persistent pulmonary hypertension underwent conventional (two-dimensional and Doppler) echocardiography and color flow mapping. Of 25 candidates for ECMO therapy, 18 subsequently received it. Conventional echocardiography demonstrated normal pulmonary venous return in only 21 of the 40 patients. In all 40, however, color flow mapping demonstrated normal right and left pulmonary venous drainage entering the left atrium. In three other patients with total anomalous pulmonary venous return, conventional echocardiography demonstrated the anomalous pulmonary venous pathways, and color flow mapping did not show jets emanating from the left atrial wall; the left atrium was shown to fill exclusively from right to left shunting through the foramen ovale. We conclude that color flow mapping is superior to conventional echocardiography for verifying normal pulmonary venous return in neonates with persistent pulmonary hypertension.     

Redirection of Hepatic Drainage for Treatment of Pulmonary Arteriovenous Malformations Following the Fontan Procedure

The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.     

Morphological Features of the Levoatriocardinal (or Pulmonary-to-Systemic Collateral) Vein

Three cases with an anomalous pulmonary-to-systemic collateral vein (levoatriocardinal vein) connecting the left atrium or one of the pulmonary veins to a systemic vein are described. In two of these cases the atrial septum is intact, the left atrioventricular connection is absent (mitral atresia), and the anomalous vein is the escape channel for pulmonary venous return. In the remaining case, a muscular membrane divides the left atrium, separating the pulmonary venous component from the distal component. The collateral vein may be mistaken for the vertical vein that is associated with totally anomalous pulmonary venous connections, but in all our cases the pulmonary veins inserted normally into the left atrium. Cross-sectional echocardiography, including conventional and color flow Doppler mapping, should overcome potential difficulties in diagnosis.     

Evaluation of patients undergoing Senning repair with two-dimensional contrast echocardiography

Summary The postoperative anatomy of the Senning interatrial diversion was defined by two-dimensional contrast echocardiography in ten patients. A modified apical four-chamber view proved most valuable, allowing for simultaneous visualization of both the systemic venous atrium and the pulmonary venous atrium. The anatomy was confirmed with two-dimensional contrast echocardiography utilizing catheters placed in the systemic venous atrium and pulmonary venous atrium at the time of surgery. In addition, in several of the patients, we were able to detect atrial or ventricular level shunts which were not clinically apparent. One patient who was judged to have a significant shunt by two-dimensional contrast echocardiography had no anatomic site found at autopsy to explain the shunting. We conclude that two-dimensional contrast echocardiography can define the postoperative anatomy following Senning repair allowing for immediate and future evaluation. Shunting at the atrial and ventricular levels can also be detected, but the method is very sensitive and difficult to quantitate or correlate clinically.     

Hypoplastic Left Heart Syndrome and Pulmonary Veno-occlusive Disease in an Infant

This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.     

Endostatin, an Inhibitor of Angiogenesis, Decreases After Bidirectional Superior Cavopulmonary Anastamosis

Pulmonary arteriovenous malformations (PAVMs) are a common source of morbidity after bidirectional superior cavopulmonary anastomosis (Glenn). The diversion of hepatic venous effluent away from the pulmonary circulation after Glenn appears to play a significant role in the pathogenesis of PAVMs. Although the liver is known to produce factors that regulate vascular development, specific hepatic inhibitors of angiogenesis have not been described in the post-Glenn population. Endostatin, produced from its precursor collagen XVIII, is a potent inhibitor of angiogenesis produced by the liver. This study aimed to investigate the hypothesis that endostatin levels decrease in patients after Glenn. Levels of endostatin and its precursor, long-type collagen XVIII, were determined by enzyme-linked immunoassay and immunoprecipitation, respectively, for serum samples from 38 patients undergoing Glenn, total cavopulmonary anastomosis (Fontan), or biventricular repair of cardiac defects. Samples were obtained before surgery and 24 h afterward. In patients undergoing a bidirectional Glenn procedure, endostatin levels decreased after surgery (n = 17; 4.42 vs 3.34 ng/ml; p < 0.001), and long type-collagen XVIII levels increased by 200 % (n = 10; p = 0.0001). However, endostatin levels did not change after surgery in patients undergoing Fontan (n = 13) or biventricular repair (n = 8). In patients undergoing Fontan, long-type collagen XVIII increased by 18 % (p < 0.01), whereas in control subjects, the levels were unchanged. These data suggest that the diversion of hepatic blood flow away from the pulmonary circulation in patients after the Glenn procedure inhibits endostatin production from collagen XVIII, resulting in decreased circulating serum endostatin levels. A decrease in endostatin may promote angiogenesis. The mechanism whereby the pulmonary circulation processes endostatin and its potential role in the pathogenesis of PAVMs warrant further study.     

Surgical Removal of Pulmonary Arteriovenous Malformations Subsequent to Total Cavopulmonary Connection Conversion Long After a Björk Procedure

Because the cavopulmonary shunt procedure is widely used for palliation of complex congenital heart diseases, pulmonary arteriovenous malformations (PAVMs) are relatively well-known complications. The reported patient was a 23-year-old woman who experienced PAVMs in the right lower lobe after a classical Glenn anastomosis and Björk procedure for tricuspid atresia. Her arterial oxygen saturation (SaO₂) 14 years after the Björk procedure was ~80 %. She then underwent a total cavopulmonary connection (TCPC) conversion to reduce her PAVMs in the right lower lobe using the “hepatic factor.” However, her situation remained unchanged, and she experienced severe systemic cyanosis (SaO₂, 70 %) and dyspnea during physical exertion without hemoptysis due to increased blood flow to the PAVMs. Although interventional embolization was considered, it was impossible due to considerable dilation of the main PAVM. Thus, right lower lung lobectomy was performed. After surgery, the patient’s SaO₂ increased to 90 %. To the authors’ knowledge, this is the first case report of a lung resection for residual PAVMs after TCPC conversion.     

Resolution of hypoxemia in a liver transplant recipient after ligation of a portosystemic shunt

This report describes the unique development of pulmonary vascular dilatation and hypoxemia associated with a portosystemic shunt in a pediatric liver transplant recipient. Ligation of the shunt resulted in resolution of hypoxemia. The outcome suggests that hepatic venous return to the pulmonary circulation is important in maintaining normal pulmonary vascular caliber.     

Total anomalous infra-cardiac pulmonary venous connection. Value of the color Doppler echocardiography

A case of total anomalous pulmonary venous connection is described in a newborn. The pulmonary veins join a common trunk entering portal vein below the diaphragm. The pattern of pulmonary venous connections was identified by two-dimensional Doppler color flow-mapping echocardiography. The common pulmonary venous trunk was anastomosed to the left atrium at the age of 3 weeks but the infant died because of pulmonary hypertension and acute renal insufficiency. The Doppler color echocardiography allows a good preoperative evaluation. The spontaneous prognosis being poor, surgical correction is indicated and should be performed early, even for critically ill neonates. Patients who survive to childhood lead active and full lives.     

Association of severe bronchial disease (bronchial casts, bronchiectasis) and partial abnormal pulmonary venous drainage in 2 children with Turner's syndrome]

The prevalence of cardiovascular malformation is high in Turner's syndrome. We report two cases of partial anomalous pulmonary venous drainage associated with a severe bronchial disease. CASE REPORTS: Case 1: a 5-year-old girl with monosomy X presented with acute respiratory failure. The chest X-ray showed an atelectasis of the left lower lobe. The patient improved following bronchoscopic removal of a bronchial cast. During hospitalization, an anomalous pulmonary venous drainage of the right lung was discovered. In spite of complete surgical repair of the cardiac malformation, several episodes of respiratory failure occurred several years after the operation. Case 2: a 9-year-old girl with monosomy X and coarctation of the aorta underwent pre-operative cardiac catheterization. She had a history of bronchiectasis, leading to a lobectomy. Her respiratory condition improved following surgery. A partial anomalous pulmonary venous return was discovered during the cardiac angiography. The coarctation was repaired surgically. The correction of the left-to-right shunt was not necessary. CONCLUSION: The partial anomalous pulmonary venous return is frequently reported in Turner's syndrome. The possible respiratory disease associated with this cardiac malformation is usually described as a consequence of the increase in pulmonary blood flow. Our two cases did not match this hypothesis.     

Diagnostic features and pitfalls in the two-dimensional echocardiographic evaluation of a child with cor triatriatum

Summary Two-dimensional echocardiography has been successfully used to diagnose cor triatriatum in adults and children, and surgical referral of these patients has been undertaken without preceding cardiac catheterization and angiography. A child with cor triatriatum demonstrated by two-dimensional echocardiography was directly referred for surgical resection. Despite thorough preoperative echocardiographic examination and direct intraoperative inspection of the posterior left atrial chamber, partial anomalous right pulmonary venous drainage was not diagnosed until the postoperative period. The failure to detect this uncommon associated anomaly underscores the limitations of relying on two-dimensional echocardiography as the sole preoperative method of evaluation.     

Cor triatriatum associated with partial anomalous pulmonary venous connection to the coronary sinus: Echocardiographic and angiocardiographic features

Summary An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography. Cardiac catheterization and angiography confirmed the findings and also demonstrated a persistent left superior vena cava draining to the coronary sinus. The infant underwent successful surgical repair. Partial anomalous pulmonary venous connection and left superior vena cava not infrequently are associated with cor triatriatum. Although two-dimensional echocardiography is sensitive for the detection of cor triatriatum, preoperative cardiac catheterization is necessary to identify unequivocally systemic and pulmonary venous connections.     

Partial Anomalous Pulmonary Venous Return: Transcatheter Repair

This report describes a case of partial anomalous pulmonary venous return in a young white girl for whom transcatheter intervention successfully replaced surgical correction. This intervention was feasible because the left lung had dual pulmonary venous drainage.     

Body Mass Index Can Predict Left Ventricular Diastolic Filling in Asymptomatic Obese Children

To examine the effects of body mass index on left ventricular diastolic function, flow velocity patterns of the pulmonary vein and mitral valve were measured by pulse Doppler echocardiography in 21 asymptomatic obese children and were compared with those of an age-matched control population. The degree of obesity was calculated as (actual body mass index/ideal body mass index -1) x 100. The pulmonary venous flow indexes were peak systolic (S) and diastolic (D) velocities and peak D/S. The mitral inflow indexes were peak velocities of early diastole (E) and atrial contraction (A) and peak E/A. The pulmonary venous flow velocity pattern in obese patients was characterized by unchanged peak S, decreases in peak D (43 +/- 7 vs 51 +/- 8, p < 0.01) and peak D/S (0.98 +/- 0.19 vs 1.29 +/- 0.20, p < 0.01), suggesting the reduction in the early diastolic filling. The peak D/S decreased significantly with an increase in the percentage body mass index (r = -0.84, p < 0.01). In contrast to the pulmonary venous flow pattern (peak D > peak S) as seen in normal controls, all of the obese patients with > 70% over body mass index had abnormal pulmonary venous flow velocity patterns (peak D < peak S). The mitral flow velocity pattern in obese patients was also characterized by a decrease in early diastolic filling. However, these indices did not correlate with an increase in the percentage over body mass index. This study suggests that body mass index predicts the abnormality of left ventricular diastolic filling assessed by pulmonary venous flow patterns.     

Modified Fontan procedure for left atrial isomerism: Alternative technique

Anomalous systemic or pulmonary venous connections increase the risk and technical difficulty of the modified Fontan procedure. This report describes an alternative technique of total diversion of systemic venous return to the pulmonary artery in a child with left atrial isomerism, incorporating an extracardiac conduit between the hepatic veins and the right pulmonary artery.     

Double total anomalous pulmonary venous connection

We present a newborn infant with right atrial isomerism, complex congenital heart malformation and anomalous pulmonary venous connection, reliably diagnosed by 2-D Doppler color echocardiography. The infant had no significant obstetric antecedents. The neonatal clinical picture included cyanosis, heart murmur and respiratory distress. The infant was treated with prostaglandin from the age of 24 hours until his death after surgery. The 2-D echo Doppler color flow mapping showed findings that suggested right atrial isomerism, severe left ventricular hypoplasia, pulmonary atresia and ductus arteriosus. The pulmonary veins flowed together to a posterior cardiac chamber from which an emissary vertical venous vessel connected with a left superior aneurysmal sack. Two venous channels emerged from this sack: one connecting to the innominate vein and the other to the atrium. The malformations were confirmed by cardiac catheterization. On the sixth day of life, the patient underwent anastomosis between the posterior venous chamber with the atrium, a modified Blalock-Taussig shunt implant, and ductus closure but died during surgery. The association between complex cardiac anomalies and uncommon obstructive total anomalous pulmonary venous connection in the context of right atrial isomerism is lethal and few neonates survive surgical repair. Two-dimensional echo color flow Doppler is a reliable diagnostic technique and an indispensable guide in angiography.     

Tetralogy of Fallot associated with total anomalous pulmonary venous drainage

Summary We present a case of tetralogy of Fallot associated with total anomalous pulmonary venous return, with successful surgical correction. This association presents problems as regards surgical indications and technique.     

Pulsed wave and color doppler findings in congenital pulmonary vein stenosis

Summary A premature infant presented at 8 weeks of age with respiratory failure and pulmonary hypertension. Two-dimensional echocardiography was not diagnostic but color flow imaging and pulsed Doppler examination revealed turbulent and continuous pulmonary venous flow. Differing patterns of disturbed flow suggested varying severity of obstruction in individual pulmonary veins. The Doppler findings are compared to the angiographic and autopsy data.