Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.     

Single left pulmonary vein with normal pulmonary venous drainage: Association with partial anomalous pulmonary venous return

An unusual case of a single left pulmonary vein draining the left lung and joining the left atrium without venous obstruction is reported. This anomaly occurred in association with partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava. A similar case has been previously reported from our laboratory. The diagnosis of a single left pulmonary vein can be made by routine chest roentgenography because of the charácteristic radiologic appearance of this anomaly. It is important to distinguish this benign entity from more ominous pulmonary radiodensities.     

The strange case of congenital mitral stenosis in an adult man with cor triatriatum

We report the case of a 74‐year‐old male, with a medical history of cor triatriatum, admitted with a 10‐day history of intermittent fever. Three sets of blood cultures were positive for Providencia rettgeri. Transthoracic and transesophageal echocardiogram excluded infective endocarditis, but revealed a congenital accessory tissue adhering to the mitral valve, causing supravalvular mitral stenosis. Cor triatriatum sinistrum is a rare congenital cardiac anomaly, even more uncommon in adults, and quite exceptional when associated with mitral valve disease. Because the patient had no symptoms related to the heart valve disease, no surgical indication was given and he was managed conservatively.     

Total anomalous pulmonary venous return in an adult

A 47-year-old man with total anomalous pulmonary venous return met all the conditions necessary for long survival. In particular, his pulmonary vascular resistance was almost normal because of right ventricular outflow tract obstruction. Surgical treatment was performed with a good result.     

Pulsed doppler echocardiographic findings in total anomalous pulmonary venous drainage to the coronary sinus

The pulsed Doppler echocardiographic (PDE) findings in a premature cyanotic infant with total anomalous pulmonary venous return to the coronary sinus are reported. Features that suggested the diagnosis of total anomalous pulmonary venous return were 1) an area of systolic-diastolic turbulent flow posterior to the apparent left atrial border, which was interpreted as representing the convergence of pulmonary venous return to a common pulmonary vein, and 2) systolic and diastolic turbulent flow within the right atrium suggesting pulmonary venous return through the coronary sinus. We suggest that PDE should be included as part of the evaluation of cyanotic infants in whom the diagnosis of total anomalous pulmonary venous return is considered.     

完全性肺静脉异位引流的手术治疗(附44例报告)

目的 探讨完全性肺静脉异位引流（TAPVC）的手术方法和治疗经验。方法对44例TAPVC患者施行手术治疗,其中心上型29例,心内型13例,心下型2例。手术在中度低温体外循环下进行．心上型早期行心脏上翻法吻合、中期行左右房联合切口法,近期改良为心上吻合法。心下型采用心脏上翻纵切口吻合。结果手术死亡2例（心上型和心内型各1例）;心律失常12例,出院时恢复安性心律。结论TAPVC一经确诊．必须立即手术,以防止肺血管阻塞性病变的发生。     

Balloon occlusion angiography in critically iii neonates

The use of balloon occlusion of a vessel during angiography is described in small ill babies. There were no complications associated with the technique, and angiographic visualisation of structures proximal to the balloon was improved especially in aortic arch anomalies.     

Evaluation of total anomalous pulmonary venous drainage with cross-sectional colour-flow Doppler echocardiography

Three patients with total anomalous pulmonary venous drainage(TAPVD) were studied by real-time cross-sectional colour-flowDoppler echocardiography. Serial suprasternal, parasternal andsubcostal scans were obtained. In all cases surgical or angiocardiographicconfirmation was available. Two patients had supracardiac drainage(to the left vertical vein or to the right superior vena cava)and one patient had infracardiac drainage. An abnormal forwardflow in the left innominate vein and vertical vein was visualizedin those patients with supracardiac TAPVD. Abnormal venous flowwas also imaged in one patient with mixed drainage. In the patientwith infradiaphragmatic TAPVD characteristic flow signals wereidentified in the inferior vena cava (retrograde flow) and inthe descending aorta and anomalous pulmonary venous channel(forward flow). In all patients the patterns of pulmonary venousflow allowed us to distinguish TAPVD from contiguous structuresand to validate two-dimensional cross-sectional imaging.     

Partial anomalous pulmonary venous connection and pulmonary arterial hypertension

Background and objective: Isolated partial anomalous pulmonary venous connection (PAPVC) has been implicated as a cause of pulmonary arterial hypertension (PAH); however this condition is often overlooked in the diagnostic work up of patients with PH. We studied the prevalence of PAH both in patients with isolated PAPVC or associated with other congenital heart diseases (CHD) such as atrial septal defect (ASD). We also aimed to identify factors related to the presence of PAH in these patients. Methods: We retrospectively analyzed data from the Adult CHD database at the Cleveland Clinic, USA between October 2005–2010. We included all patients diagnosed with PAPVC with or without other CHD. We excluded all patients with previous corrective surgeries. Results: We identified 14 (2.5%) patients with PAPVC. Group I included patients with PAPVC (with or without patent foramen ovale (PFO)). Group II included patients with PAPVC associated with other CHD. PAH was seen in six (6/14, 42.8%) patients, two (2/7, 28.5%) in group I and four (4/7, 57.1%) in group II (P = 0.3). The mean pulmonary artery pressure in all patients (n = 14) was 29.5 ± 13.8 mm Hg. Group I had a mean PAP of 23.6 ± 6.6 mm Hg as compared to 33.7 ± 16.5 mm Hg for group II (P = 0.34). The two patients in group I with PAH had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the haemodynamic findings. Conclusions: Patients with PAPVC (with or without PFO) in the absence of other CHD had normal pulmonary arterial pressure (PAP) unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.     

Ebstein's anomaly associated with severe valvar pulmonary stenosis: Successful palliation with balloon pulmonary valvuloplasty in an adult

We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention. Cathet. Cardiovasc. Intervent. 46:441–444, 1999. © 1999 Wiley-Liss, Inc.     

选择性肺动脉造影评价先天性心脏病肺动脉高压的初步分析

通过选择性肺动脉造影检查，了解肺血管病变程度。对４３例先天性左向右分流型心脏病病人行选择性肺动脉造影检查，通过分析肺循环时间、肺动脉终末单支血管计数、肺动脉分级、毛细血管像等指标，并与右心导管（４３例）和肺病理（７例）结果进行比较分析。结果显示：随着肺血管病变加重，肺循环时间延长；肺动脉终末单支血管计数、肺动脉分级减少；肺毛细血管象分布不均。结论：选择性肺动脉造影检查可以比较准确地判定肺血管病变程度。     

The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus

BACKGROUND—Infants with isolated totally anomalous pulmonary venous return often present severely decompensated, such that they are at high risk for surgical repair. On the other hand, if surgical repair can be safely accomplished, the outlook is usually good. Thus prenatal diagnosis would be expected to improve the prognosis for the affected child.
OBJECTIVE—To describe the features of isolated totally anomalous pulmonary venous drainage in the fetus.
DESIGN—Four fetuses with isolated totally anomalous pulmonary venous connection were identified and the echocardiographic images reviewed. Measurements of the atrial and ventricular chambers and both great arteries were made and compared with normal values.
SETTING—Referral centre for fetal echocardiography.
RESULTS—There were two cases of drainage to the coronary sinus, one to the right superior vena cava, and one to the inferior vena cava. Right heart dilatation relative to left heart structures was a feature of two cases early on, and became evident in some ratios late in pregnancy in the remaining two.
CONCLUSIONS—Ventricular and great arterial disproportion in the fetus can indicate a diagnosis of totally anomalous pulmonary venous connection above the diaphragm. However, in the presence of an atrial septal defect or with infradiaphragmatic drainage, right heart dilatation may not occur until late in pregnancy. The diagnosis of totally anomalous pulmonary venous drainage in fetal life can only be reliably excluded by direct examination of pulmonary venous blood flow entering the left atrium on colour or pulsed flow mapping.


Keywords: congenital heart disease; fetus; total anomalous pulmonary venous drainage; echocardiography     

Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is an uncommon congenital anomaly in which the anatomical presentations vary widely among patients. We hereby present two newborns with TAPVC associated with asplenia syndrome; both had severe esophageal varices due to infradiaphragmatic pulmonary venous drainage. Ultrafast computed tomography (CT) scanning was superior to color Doppler echocardiography and cardiac catheterization as it provided a detailed portrait of the pulmonary drainage. The remarkable radiographic manifestations are presented.     

Hypoplastic left heart syndrome,cor triatriatum and partial anomalous pulmonary venous connection: Imaging of a very rare association

A newborn is presented with an association of hypoplastic left heart syndrome, cor triatriatum and partial anomalous pulmonary venous connection. The diagnosis was established with echocardiography and further confirmed with computed tomography. To our knowledge the images of such an association have never been reported before.     

Surgical treatment of anomalous left main coronary artery with an intraconal course

Objective: Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course.
Methods: This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15 years (range 2‐47). The seven oldest patients all had preoperative symptoms of exertional chest pain and one also had exertional syncope. The five youngest pa‐ tients had no preoperative symptoms. One of these patients had a hemodynamically significant ventricular septal defect and one patient was the sibling of a patient who had undergone repair of AAOCA.
Results: The 12 patients underwent surgical repair including unroofing of the myo‐ cardial bridge overlying the intraconal LMCA and a LeCompte procedure. There was no early or late mortality and there were no significant complications. All 12 patients have resumed normal, unrestricted activities.
Conclusions: Twelve patients with an anomalous LMCA and intraconal course pre‐ sented to our institution for treatment. Surgical repair was performed successfully in all 12, with resolution of symptoms in the 7 patients who were symptomatic preop‐ eratively. These results suggest that the surgical treatment is safe and efficacious in patients with an anomalous LMCA and intraconal course.     

Total anomalous pulmonary venous connection (TAPVC): A familial cluster of 3 siblings

Total anomalous pulmonary venous connection (TAPVC) is a rare form of cyanotic congenital heart disease (CHD) that occurs when the pulmonary veins drain into a site other than the morphologic left atrium. As with other forms of CHD, TAPVC has been shown to cluster in families and is known to have genetic association. We report on a case series of familial TAPVC in three consecutive siblings. A combination of fetal echocardiography, transthoracic echocardiography, as well as cross‐sectional imaging was utilized in the diagnosis as well as management of each sibling. The third sibling was subsequently found to have a partial deletion in chromosome 15q13.3, which has been rarely associated with other forms of congenital heart disease.