Total anomalous pulmonary venous connection with ccTGA and VSD: Can pulmonary artery banding avert pulmonary venous obstruction?

We describe a rare case of infracardiac total anomalous pulmonary venous connection (TAPVC), associated with congenitally corrected transposition of the great arteries (ccTGA) and ventricular septal defect, in which the patient had undergone pulmonary artery banding (PAB) at 16 days of age. She began to have episodes of severe cyanosis while crying, 2 weeks after PAB. Cardiac catheterization at 34 days of age showed severe pulmonary hypertension and a transhepatic pressure gradient of 7 mmHg. The infant underwent TAPVC repair and conventional repair for ccTGA at 35 days of age. Although PAB might have the provisional effect of delaying the manifestation of pulmonary venous obstruction (PVO), it is unable to prevent the development of PVO due to the high resistance of the hepatic sinusoids. Signs of PVO should be closely monitored so that TAPVC can be repaired in a timely fashion.     

RARE VARIANT OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION: Intrapulmonary Drainage of One Lung by the Other- A Case Report and Review of the Literature

Total anomalous pulmonary venous connection is an uncommon congenital cardiovascular anomaly but one that occurs frequently in the asplenia syndrome. We present a rare type of total anomalous pulmonary venous connection in a 4-month-old baby with the asplenia syndrome. In this patient, all of the left pulmonary veins united to form a common pulmonary vein, which crossed the mediastinum and then proceeded in an intrapulmonary fashion in the right lung. A small pulmonary vein emerged from the right upper lobe and drained into the right superior vena cava. Nine other cases have been reported, four also with asplenia, five with otherwise normal cardiac anatomy apart from an atrial septal defect. The incidence of pulmonary venous obstruction is high, at least 70 . Two cases, both with a normal heart, had corrective surgery. One with no pulmonary venous obstruction was repaired successfully. Embryologically, this type of total anomalous pulmonary venous connection represents a developmental error early in embryonic development before the venous connection between the two lungs and the cardinal veins involute.     

Echocardiographic Follow-Up of Children with Supravalvular Aortic Stenosis

This study evaluates the course of supravalvular aortic stenosis (SVAS)-associated right ventricular outflow tract (RVOT) obstruction and the results of surgery in children. We reviewed the medical records of 24 patients diagnosed with SVAS at initial echocardiographic examination or during the following period of RVOT obstruction. Very mild SVAS was defined as a transvalvular Doppler peak systolic instantanous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 3.1 ± 2.9 years (range, 7 days to 12.7 years), and 18 of the patients (72%) were male. Fifteen patients had Williams’ syndrome. Seventeen patients (71%) were followed for a mean of 5.2 ± 3.8 years (range, 7 months to 13.5 years). Among 17 patients with complete follow-up records, 1 (6%) had very mild, 5 (29%) mild, 3 (18%) moderate, and 3 (18%) severe aortic stenosis at initial echocardiographic examination. In a newborn patient with mild pulmonary valvular stenosis. SVAS became evident after 2 months and progressed rapidly. Supravalvular aortic stenosis was very mild in 4 patients (23%), mild in 3 (18%), moderate in 3 (18%), and severe in 7 (41%) at last echocardiographic examination. Of 17 patients who were followed, 11 (65%) had RVOT obstruction at initial echocardiographic examination. RVOT obstruction disappeared in 5 patients, regressed in 1 patient, and appeared in 1 patient over the follow-up period. Four patients underwent operation. It appears reasonable that patients with very mild and mild stenosis should be followed medically every 1 or 2 years and patients with moderate stenosis once a year. Newborns with SVAS should be followed for rapid progression of SVAS. In some patients, RVOT obstruction may disappear, and SVAS may develop in others with RVOT obstruction. Patients with RVOT obstruction (at the valvular, supravalvular, or peripheral pulmonary arterial level) should be evaluated carefully for development of SVAS at follow-up.     

Supracardiac type total anomalous pulmonary venous connection (TAPVC) with oesophageal varices

Oesophageal varices due to total anomalous pulmonary venous connection (TAPVC) is very rare. Additionally, the infradiaphragmatic type is the most common type of oesophageal varices due to TAPVC. Paraoesophageal varices due to stenosis of the vertical vein of supracardiac TAPVC has not previously been reported. We describe paraoesophageal varices developed as a result of a connection between the left lower pulmonary vein and the umbilicovitelline venous system because of stenosis of the proximal vertical vein in supracardiac type TAPVC in a 3-day-old female newborn who presented with general cyanosis, tachypnoea and dyspnoea.     

Dysphonia: A Frequently Encountered Symptom in the Evaluation of Infants with Unobstructed Supracardiac Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection is a relatively uncommon congenital cardiac anomaly. When there is obstruction to pulmonary venous drainage or severe pulmonary hypertension due to increased pulmonary blood flow, the patient is usually critically ill and the diagnosis is unambiguous. However, in some patients the symptoms may be mild and often overlap with those of other noncardiac diseases. Tachypnea, failure to thrive, and mild cyanosis are the most frequently reported symptoms. In our patient population, we encountered a frequent observation by parents that the child had an alteration in voice which we elected to refer to as dysphonia for the purpose of this study. We retrospectively reviewed all patients with total anomalous pulmonary venous connection who were evaluated at our institution from January 1996 to January 1999 and found that 27% of patients had dysphonia as a presenting complaint to their primary care provider. We hypothesize that the etiology of this phenomenon lies in the possible compression of the left recurrent laryngeal nerve in the vicinity of the dilated pulmonary artery and the left vertical vein.     

Misleading hyperoxia test.

A 2 day old infant with infracardiac total anomalous pulmonary venous connection achieved an arterial oxygen tension greater than 250 mmHg, breathing 100% oxygen. Diagnosis of cardiac disease was delayed several weeks. The anatomical basis for this phenomenon was streaming of systemic venous return across an inferior vena cava type atrial septal defect.     

Functional total anomalous pulmonary venous connection via levoatriocardinal vein

We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the “dead‐end” left atrium, the pulmonary venous flow had a to‐and‐fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left‐side heart obstruction.     

Visualization of pulmonary vein obstruction by pulmonary artery wedge injection

Summary One of the major postoperative complications of total anomalous pulmonary venous connection is obstruction of the pulmonary veins. This event may be apparent immediately after surgery when it is usually due to congenitally obstructed veins or later when it may be the result of failure of commensurate growth of the left atrium-common vein anastomosis or of scarring at the anastomotic site. The exact location of the obstruction becomes, then, of paramount importance in deciding if the patient can be helped by further surgery. At cardiac catheterization, pulmonary artery wedge injection was the only angiographic technique that visualized the site and degree of pulmonary venous obstruction in the case reported here.     

Portal venous gas: a clinical finding in obstructed infracardiac total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection is a cyanotic congenital heart defect that presents with a multitude of clinical presentations. We report a case of obstructive total anomalous pulmonary venous connection (TAPVC) which presented in a way that has not previously been described. An infant with mixed type obstructive TAPVC was found to have pulmonary interstitial emphysema and portal venous gas on a scout radiograph obtained prior to computed tomography scan. The presence of portal venous gas indicated a connection between the pulmonary venous system and the portal venous system infradiaphragmatically.     

Pulmonary Veins in Total Anomalous Pulmonary Venous Connection with Obstruction: Demonstration Using Silicone Rubber Casts

We studied the postmortem vascular anatomy of four patients with total anomalous pulmonary venous connection (TAPVC) with obstruction using a silicone rubber casting method. Two patients were studied clinically but died before therapeutic intervention, one died after corrective surgery, and another was not diagnosed before death. The morphologic types were supracardiac (2), infracardiac (1), and mixed (1). One of the supracardiac forms had a stenosis at the left vertical vein by a bronchoarterial vise, and a collateral channel to the right superior caval vein was also present. In the other supracardiac case, a left upper pulmonary vein was connected to the left vertical vein above the stenotic portion, resulting in postoperative pulmonary hemorrhage into the left upper lobe. The patient with infracardiac TAPVC had a tree-shaped descending vertical vein and small opening at the inferior caval vein. In the mixed type, a right upper pulmonary vein ran between the pulmonary artery and bronchus draining into the superior caval vein, and the rest of the drainage was to the portal vein.     

Obstructed total anomalous pulmonary venous connection

Summary With the advent of echocardiography, total anomalous pulmonary venous connection (TAPVC) can be readily diagnosed without much difficulty. However, noninvasive detection of the presence of pulmonary venous obstruction in TAPVC remains a difficult issue. During a 5.5-year period, 42 patients were found to have TAPVC by catheterization, surgery, and/or autopsy: 17 had supracardiac drainage, 13 paracardiac drainage, nine infracardiac drain-age, and three mixed drainage. Obstruction to pulmonary venous drainage was found in 24 patients (57%). Patients with right isomerism tended to have a higher incidence of pulmonary venous obstruction than those with the usual atrial arrangement (80% vs. 44%,p<0.05). Color Doppler combined with cross-sectional echocardiography provided accurate delineation of drainage sites in 93% cases (39 of 42). Among the 39 cases with correct echocardiographic delineation of the drainage site, obstruction was detected by echocardiography in 22 cases with a sensitivity of 100% (22 of 22) and a specificity of 85% (17 of 20). Therefore, complete echocardiography, including cross-sectional images and color Doppler proved to be a reliable tool in the detection of drainage sites and pulmonary venous obstruction in TAPVC.     

Pulmonary venous collaterals secondary to superior vena cava stenosis: A rare cause of right-to-left shunting following repair of a sinus venosus atrial septal defect

Summary We describe a case of unusual pulmonary vein-to-vein collateral formation leading to systemic desaturation years following surgical repair of a sinus venosus atrial septal defect and partial anomalous pulmonary venous return from the right lung. At surgery, a single right upper lobe pulmonary vein branch was left draining high into the superior vena cava (SVC), resulting in a small left-to-right shunt. SVC obstruction developed at the site of the ASD repair and elevated venous pressure above the obstruction caused retrograde flow into the unincorporated pulmonary vein. Pulmonary vein-to-vein collaterals formed between this unincorporated vein and an adjacent pulmonary vein that had been surgically diverted to drain into the left atrium, resulting in a right-to-left shunt within the right lung. Normal pulmonary veins drain multiple bronchopulmonary segments, thus providing potential collateral pathways. Balloon angioplasty of the SVC stenosis successfully relieved the obstruction and abolished the right-to-left intrapulmonary shunt.     

Intermediate-term follow-up results of pulmonary balloon valvuloplasty in children

Twenty-two patients (10 boys, 12 girls) with pulmonary valve stenosis whose mean age was 6.7 +/- 4.1 years (range 1 to 14 years) at time of the procedure underwent balloon dilatation angioplasty. All patients had isolated pulmonary valve stenosis with no associated cardiac anomalies, and their pulmonary valvular gradients were greater than 50 mmHg. Diagnostic catheterization and balloon valvuloplasty were performed during the same procedure. The mean peak systolic pressure gradient before balloon dilation of 95.8 +/- 29.5 mmHg (54-163 mmHg) was reduced to 30.2 +/- 15.8 mmHg (7-64 mmHg) after balloon dilation (p < 0.001). A linear relation was found between the predilation pressure gradient and the pressure gradient drop (r = 0,86; SE: 28.94) (y = 0.89x - 12.11). Following a successful valvuloplasty, the mean peak systolic pressure in the right ventricle decreased from 119.0 +/- 30.4 mmHg (71-184 mmHg) to 55.2 +/- 16.9 mmHg (29-97 mmHg) (p < 0.001). Continuous wave Doppler was used for follow-up. Pressure gradients were estimated in 14 patients between one and 29 months after the dilation 120 +/- 7.1 months). The mean follow-up gradient was 19.0 +/- 6.0 mmHg (11-32 mmHg). No important complication was noted in the immediate course or throughout the follow-up period, but four patients (28.6%) had minimal pulmonary insufficiency. In conclusion, these data confirm that balloon dilation in valvular pulmonary stenosis is safe and effective, and suggest that stenosis does not recur.     

Management of Congenital Aortic Stenosis

72 patients with congenital aortic stenosis whose ages ranged from 1 month to 15 years were under supervision for periods ranging from several months to more than 9 years. The obstruction was subvalvar in 3. Additional malformations were aortic regurgitation, persistent ductus arteriosus, coarctation of the aorta, ventricular septal defect, partial anomalous pulmonary venous drainage, and mitral stenosis. Bacterial endocarditis complicated 3. Of 5 deaths unrelated to operation, 3 were sudden. Surgical treatment was carried out in 12 patients (commissurotomy, valve replacement with or without previous commissurotomy, subvalvar resection) with 2 deaths. 52 patients remained symptom-free. Detailed investigation to assess the patient for surgical treatment is indicated if symptoms are present or electrocardiographic evidence of severe left ventricular hypertrophy appears. Commissurotomy is, where possible, deferred until an age at which valve replacement can be undertaken if the stenosis cannot be adequately relieved without causing substantial regurgitation.     

Treatment Strategy and Long-Term Prognosis for Patients With Esophageal Atresia and Congenital Heart Diseases

A review examined six consecutive cases of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who underwent cardiac surgery at the authors’ institution between 2001 and 2011 for associated complex congenital heart diseases. All the patients had a normal karyotype and showed EA with distal TEF. In all cases, gastrostomy was the initial surgical intervention. Cardiac surgery was performed concurrently with gastrostomy for one patient who had a total anomalous pulmonary venous connection with pulmonary venous obstruction. For two patients with duct-dependent pulmonary circulation, EA/TEF was corrected in the neonatal period, and an aortopulmonary shunt operation was electively performed after the first month of life. For two patients with duct-dependent systemic circulation, repair of EA/TEF was performed concurrently with gastrostomy, followed by palliative cardiac surgery during the neonatal period. For another patient without duct-dependent circulation, repair of EA/TEF was performed in the neonatal period. No mortality occurred during a median follow-up period of 6.2 years. However, respiratory complications including severe tracheomalacia for two patients, recurrent episodes of respiratory infection for three patients, and severe gastroesophageal reflux for five patients caused considerable long-term morbidity.     

Fate of the unligated vertical vein after repair of supracardiac anomalous pulmonary venous connection

OBJECTIVE: To determine the fate of the unligated vertical vein after repair of isolated supracardiac total anomalous pulmonary venous connection (TAPVC). METHODS: We reviewed the outcome of 28 patients who were diagnosed to have isolated supracardiac TAPVC and determined the fate of the unligated vertical vein. RESULTS: Of the 28 patients, four died before surgery. The remaining 24 patients underwent surgical correction of TAPVC with (n = 5) or without (n = 19) ligation of vertical vein at a median age of 20 days (range: 1-574 days). There were no significant differences in age, weight, presence of pulmonary venous obstruction, need for preoperative inotropic and ventilatory support, cardiopulmonary bypass duration, postoperative pulmonary hypertensive crisis and requirement of peritoneal dialysis between patients with and those without vertical vein ligation. The in-hospital surgical mortality was 50% (12/24), with 83% (10/12) of deaths occurring before 1990. Patients who died after surgery were significantly younger (median age: 5.5 days vs 37 days, P = 0.005), lighter (3.3 +/- 0.5 kg vs 3.9 +/- 0.6 kg, P = 0.016), more likely to have pulmonary venous obstruction preoperatively (75% vs 12%, P = 0.039) and have undergone surgery before 1990 (83% vs 33%, P = 0.036). The 12 survivors were followed up for a median of 4.7 years (range: 2.3-18.1 years), 10 of whom had their vertical vein unligated. The vertical vein remained patent in five (50%) patients, while stenosis of pulmonary venous anastomosis was only present in one patient. Of these five patients, three had subsequently undergone surgical ligation of the vertical vein to eliminate a large left-to-right shunt. CONCLUSIONS: Patency of the unligated vertical vein is common after the repair of supracardiac TAPVC, even in the absence of pulmonary venous obstruction. The degree of left-to-right shunt through the patent vertical vein may be so significant as to warrant surgical ligation.     

Acute Pulmonary Embolism in a Neonate: Precipitation During Cardiac Catheterization and Successful Treatment

This report illustrates the rare occurrence of a pulmonary embolus in a neonate during cardiac catheterization. The patient was a term newborn who underwent repair of obstructed infradiaphragmatic total anomalous pulmonary venous connection. Postoperative risk factors for pulmonary embolism included severe pulmonary hypertension unresponsive to nitric oxide therapy, an indwelling venous catheter, and young age. Successful management was achieved by initial mechanical fragmentation with streptokinase infusion and monitoring by serial lung perfusion scans.     

Contribution of MRI in supracardiac total anomalous pulmonary venous drainage

A case of supracardiac total anomalous pulmonary venous drainage (TAPVD) in an infant aged 2 1/2 months is presented. Diagnosis was established non invasively by magnetic resonance image (MRI). Not only did MRI precisely depict the anomalous venous pathway but it moreover securely excluded pulmonary venous obstruction.     

Total Anomalous Pulmonary Venous Return with the Circular Pulmonary Venous Connection: Outcome of Common Pulmonary Venous Agenesis

A rare case of total anomalous pulmonary venous return, in which the right and left peripheral pulmonary veins connected circularly and there was no central part of the pulmonary vein or the common pulmonary vein, is presented. To our knowledge, total anomalous pulmonary venous return with circular pulmonary venous connection has not been reported previously in the literature. It is thought that the complex connection between peripheral pulmonary veins with the absence of the central part of the pulmonary vein as well as the common pulmonary vein results from common pulmonary venous agenesis.     

Radiological evaluation of pulmonary vein obstruction including two examinations by magnetic resonance imaging

Congenital obstruction of the pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veins and pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which were accompanied by other cardiac anomalies. Right pulmonary veins were involved in all seven cases; one case was bilateral. Pulmonary veins were occluded totally in five and partially in three lungs. Diagnostic pulmonary catheterization and angiography were performed. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural changes, which were quite specific, whereas pulmonary venous dilatation was the dominant finding in partial obstruction cases. Pulmonary perfusion scan (n=3) showed total perfusion defects in the cases with total occlusion of pulmonary veins. Magnetic resonance (MR) imaging (n=2) demonstrated totally occluded pulmonary veins at the venoatrial junction in two lungs and membranous focal obstruction in one lung. Two children underwent pneumonectomy and had the diagnosis histologically confirmed. Although catheterization and angiography are essential for the diagnosis of pulmonary vein obstruction, MR imaging is a useful adjunct.