Hypoplastic left heart syndrome with premature closure of foramen ovale: Report of an unusual type of totally anomalous pulmonary venous return

Summary We report on a case of hypoplastic left heart syndrome (HLHS), associated with premature closure of the foramen ovale and an unusual type of totally anomalous pulmonary venous return. The existence of an anomalous connection of the right upper pulmonary vein to the superior vena cava-right atrial (SVC-RA) junction and the existence of the anomalous intrapulmonary venous channel between right upper and lower pulmonary vein allowed all the pulmonary blood to drain into the SVC-RA junction, whereas she had only partially anomalous pulmonary venous connection. The several intrapulmonary venous channels helped to delay the progression of pulmonary venous obstruction. Chromosomal analysis revealed that the patient had XO Turner syndrome. We conclude that all infants with HLHS should be carefully evaluated for the existence of anomalous pulmonary venous return. Two-dimensional Doppler echocardiography is one of the most useful techniques for evaluating such anomalies.     

Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography

Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of greater than or equal to 16 mm Hg at any level of the pulmonary venous return or of the systemic ventricular inflow. Severe obstruction was predicted if Doppler examination measured a flow velocity of greater than or equal to 2 m/s across any area of inflow obstruction. At catheterization, 12 patients (39%) had severe obstruction to left ventricular inflow or pulmonary venous return and all obstructions were correctly detected by Doppler echocardiography. The site of pulmonary venous obstruction was localized by two-dimensionally directed pulsed Doppler study. Patients with a lesser degree of obstruction had a lower Doppler velocity, but none had a maximal Doppler velocity of greater than or equal to 2 ms/s.(ABSTRACT TRUNCATED AT 250 WORDS)     

Amplatzer vascular plug for transcatheter closure of persistent unligated vertical vein after repair of infracardiac total anomalous pulmonary venous connection

Repair of total anomalous pulmonary venous connection (TAPVC) involves anastomosing the pulmonary venous confluence with the left atrium and ligating the vertical vein. Sometimes, the vertical vein needs to be left open as a pop off with the idea that it will close over time. Infrequently an unligated vertical vein may remain patent after repair of infaracardiac TAPVC leading to hemodynamic instability. We report an infant in whom an unligated vertical vein remained patent after the repair of infracardiac TAPVC and caused hemodynamically significant left‐to‐right shunting. A successful transcatheter closure of persistent patent unligated vertical vein was performed using the Amplatzer Vascular Plug‐I device. © 2012 Wiley Periodicals, Inc.     

Obstructed pulmonary venous drainage with total anomalous pulmonary venous connection to the coronary sinus

Obstructed pulmonary venous drainage is generally considered extremely rare with total anomalous pulmonary venous connection (TAPVC) to the coronary sinus (CS). A retrospective review of 27 operated patients with TAPVC to CS revealed 6 cases of obstruction (22%). Two of 6 patients who died early had evidence of obstruction at the pulmonary vein confluence at autopsy. Among the 21 hospital survivors, obstruction proximal to the point of CS unroofing developed in 4 (19%), necessitating reoperation and resulting in death in 3. One other patient died late. Mean follow-up of the 17 long-term survivors, who are generally doing well, is 85 months (range 2 to 212). There have been no cases of late obstruction at the site of unroofing among 10 patients who underwent the fenestration procedure suggested by Van Praagh. The incidence of early bradyarrhythmias (60%) does not appear to be decreased by this procedure. Review of 13 autopsy specimens suggests that if the right and left pulmonary veins did not drain directly to the CS but converged to form a short common vertical vein (4 cases), obstruction was likely. When pulmonary artery pressure approaches systemic levels preoperatively, careful echocardiographic and intraoperative assessment of the junction of the pulmonary vein confluence with the CS should be made. If there is evidence of obstruction, consideration should be given to anastomosing the horizontal right and left pulmonary veins directly to the left atrium rather than performing a simple unroofing procedure.     

Detection of shunt flow in total anomalous pulmonary venous connection using 2D-Doppler echocardiography

Two-D-Doppler echocardiography was used to diagnose two cases of total anomalous pulmonary venous connection (TAPVC). The first case presented the supracardiac type, the second the infracardiac type. Blood flow imagings produced by 2D-Doppler echocardiography revealed shunt flow in the common pulmonary veins (CPV) and the vertical, innominate, and portal veins. The shunt flow in the vertical vein was laminar, though the shunt flow in the inflow tract of the right atrium was turbulent. Two dimensional Doppler echocardiography is very useful in the detection shunt flow in TAPVC.     

Gross and histologic anatomy of total anomalous pulmonary venous connections

Among 49 heart specimens with total anomalous pulmonary venous connection (TAPVC), obstruction to pulmonary venous flow was present in all 13 cases with TAPVC below the diaphragm and in 53% of 36 cases with TAPVC above the diaphragm. Obstruction was produced by extrinsic pressures on the vein, intrinsic narrowing of the vein, or both. The histology of the narrowed veins was extremely variable, ranging from atrophy of the vein wall to hypertrophy of intima, media-adventitia, or both. Balloon dilation of narrowed veins was performed in 3 cases, without clinical or anatomic evidence of success.     

Mixed variety of total anomalous pulmonary venous connection: diagnosis by 2D echocardiography and Doppler colour flow imaging.

Of the many types of total anomalous pulmonary venous connection, mixed type is the least common. Its accurate non-invasive diagnosis by echocardiography poses a diagnostic challenge. We report our experience of echocardiography in 21 infants with mixed type of total anomalous pulmonary venous connection aged 25 days to one and half years. Multiple windows were used to identify individual pulmonary veins and various sites of drainage. Cardiac catheterisation and angiography were performed for 17 cases. In 11 of 21 cases, the left upper pulmonary vein was seen draining into vertical vein and the left lower and right-sided pulmonary veins were draining into the coronary sinus. Cardiac and supracardiac combinations of other types were seen in eight more cases. Both drainage sites were supracardiac in one case and supracardiac and infracardiac in another. On comparing echocardiographic findings with those obtained at cardiac catheterisation and/or surgery (carried out in 18 cases), there were three instances of error. In two cases (echocardiography performed without the use of colour flow imaging) the second site of drainage could not be defined. These patients were catheterised as all four pulmonary veins were not delineated by echo. The third error occurred in a case where although two sites of drainage were picked up by echo but at surgery, the right lower pulmonary vein was noted to have a double connection, both to coronary sinus and to vertical vein. We conclude that mixed type of total anomalous pulmonary venous connection can be accurately diagnosed by echocardiography and Doppler colour flow imaging. The diagnostic errors are rare and would not alter the surgical management.     

Range-gated pulsed Doppler echocardiographic diagnosis of supracardiac total anomalous pulmonary venous drainage

Three patients with total anomalous pulmonary venous drainage (TAPVD) into the innominate vein were studied by using M-mode and pulsed Doppler echocardiography. An abnormal continuous flow towards the transducer in a suprasternal notch position detected by pulsed Doppler echocardiography in the left hemithorax leftward of the aortic echo is a sign of blood flow through the left vertical vein into the innominate vein. This finding is highly specific for TAPVD. High-velocity flow across the tricuspid valve, disturbed flow in the right pulmonary artery and abnormal flow in the left atrium are additional important pulsed Doppler echocardiographic findings in TAPVD. Right ventricular enlargement and paradoxical interventricular septal motion by M-mode echocardiography were not specific enough to distinguish TAPVD from other right ventricular volume overload lesions. An echo-free space posterior to the left atrium was not recorded.     

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.     

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.     

Prognostic value of left ventricular size measured by echocardiography in infants with total anomalous pulmonary venous drainage

Left ventricular size may be a determinant of survival in infants with total anomalous pulmonary venous drainage. Right and left ventricular size were measured by M-mode and 2-dimensional (2-D) echocardiography in 13 patients aged 1 day to 4 months (mean weight 4.3 ± 0.42 kg [standard error of the estimate]) who underwent surgery before age 4 months because of severe cyanosis or cardiac failure. Seven patients had venous drainage to a vertical vein, 4 had drainage to the right atrium, and 2 had drainage to the inferior vena cava. Patients were divided into 2 groups: survivors (Group A, n = 8) and nonsurvivors (Group B, n = 5). Death was not statistically related to pulmonary artery pressure, pulmonary venous obstruction, age, or weight at the time of surgery. Right and left ventricular sizes at end-diastole measured from M-mode traces and 2-D echocardiographic 4-chamber views were compared with those from 15 weight-matched control infants. On M-mode and 2-D echocardiography, nonsurvivors had significantly larger right ventricles and smaller left ventricular dimensions than did either control subjects or surviving patients with total anomalous pulmonary venous drainage. The ratio of right to left ventricular size on M-mode and 2-D echocardiography also differed among the 3 infant groups (p < 0.001). The ratio of right to left ventricular size differentiated nonsurvivors from survivors and control subjects. Postmortem examinations available in 4 of the 5 nonsurvivors demonstrated that the ratio of right to left ventricular size in the specimens closely agreed with the 2-D echocardiographic ratios. Our study agrees with the impression of other investigators that left ventricular size may be a determinant of survival after repair of total anomalous pulmonary venous drainage.     

Two-dimensional echocardiography in the pre- and postoperative management of totally anomalous pulmonary venous connection

The records of 23 infants who underwent surgical repair of isolated totally anomalous pulmonary venous connection were reviewed to assess the accuracy of pre- and postoperative echocardiographic diagnoses. Preoperative echocardiographic diagnoses were accurate in 22 of 23 patients, including the sites of connection of the individual pulmonary veins. Cardiac catheterization in 13 patients confirmed the echocardiographic findings. Analysis of multiple pre- and postoperative variables revealed no statistically significant difference between the infants with and without catheterization, although there was a tendency toward a higher mortality rate in the catheterized group. Postoperative echocardiographic examination revealed obstruction to pulmonary venous return in 7 of 19 patients. Catheterization confirmed the echocardiographic findings, localizing the obstruction in one patient. The size of the venoatrial anastomosis was measured on postoperative echocardiograms performed on 14 patients. The cross-sectional area of the anastomosis was less than 0.3 cm2/m2 of body surface area in the four patients with obstruction of the anastomosis, and greater than 0.95 cm2/m2 in all long-term survivors examined. Two-dimensional echocardiography with pulsed Doppler examination and Doppler color flow mapping is an excellent means of diagnosing totally anomalous pulmonary venous connection. The connections of the individual pulmonary veins can be identified in nearly all cases. Surgical repair can usually be undertaken on the basis of echocardiographic diagnosis alone. Echocardiography also provides an extremely accurate method of evaluating surgical repair and of identifying and localizing postoperative obstruction to pulmonary venous return.     

Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection

Pulmonary venous flow was evaluated by pulsed Doppler echocardiography in 38 patients with total anomalous pulmonary venous connection. Twenty-nine of these 38 had no associated intracardiac anomaly (Group I), and 9 had complex intracardiac anatomy associated with low pulmonary blood flow (Group II). In Group I the drainage was infracardiac in nine, supracardiac in seven, intracardiac in eight and mixed in five. In both groups, in those with venous obstruction the flow in the individual pulmonary veins and ascending or descending vein was nonphasic, varying only with respiration. Flow in the absence of obstruction was phasic, varying with the cardiac cycle. Distal to a site of obstruction the flow was nonlaminar and of high velocity irrespective of the amount of pulmonary blood flow. The pulsed Doppler technique provides important physiologic information in the patient with total anomalous pulmonary venous connection before surgical intervention.     

不同手术方法治疗心上型完全性肺静脉异位引流

目的:比较心上型完全性肺静脉异位引流(TAPVC)的各种手术治疗效果,为临床治疗TAPVC提出更合理的手术方式。方法: 2000年8月~2011年4月外科手术治疗60例心上型TAPVC,包括经房间隔手术14例,经左右心房手术19例,心上法27例。结果: 术后死亡4例,其中经房间隔手术死亡2例,经左右心房死亡1例,心上法死亡1例。术后主要并发症为肺静脉梗阻、低心排、手术创伤所致心律失常、左心发育不良所致急性肺水肿、呼吸衰竭。其中心上法手术患者并发症明显低于其他两组。结论: 心上径路治疗心上型TAPVC近、中期效果良好,远期效果有待进一步随访。     

The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries.

2-D Doppler echocardiography was used to assess the occurrence of haemodynamic abnormalities in 45 asymptomatic patients, aged 4 to 16 years (median 7.4) after a Mustard operation for transposition of the great arteries. The findings were compared with those derived from cardiac catheterization. Thirty-five cardiac lesions were correctly diagnosed by 2-D Doppler echocardiography in 23 patients, but on six occasions, minor abnormalities were missed. 2-D Doppler echocardiography demonstrated systemic venous pathway obstruction of more than 3 mmHg at cardiac catheterization in nine patients, and in five of the six patients with pulmonary venous channel obstruction. A left ventricular outflow tract obstruction (pressure difference greater than 15 mmHg) was diagnosed correctly by Doppler echocardiography in seven patients. Baffle leakage was found in two patients with a left to right shunt of 25% or more of pulmonary blood flow, but was missed in five out of nine patients with small shunts. Tricuspid regurgitation was well defined in eight patients. The absence of symptoms and a routine examination after a Mustard operation do not rule out haemodynamic abnormalities. However, these, with the possible exception of minor baffle leakage, can be detected by 2-D Doppler echocardiography.     

混合型完全性肺静脉畸形引流的外科治疗

目的 :对两例患儿实施混合型完全性肺静脉畸形引流矫治术。方法 :2 0 0 1年 3月～ 11月 ,我院分别为 1例 9个月的婴儿和 1例 4岁儿童成功实施混合型完全性肺静脉畸形引流矫治术。两患儿左肺静脉通过垂直静脉流入无名静脉 ,右肺静脉回流至冠状静脉窦。结果 :患儿术后恢复顺利 ,术后 7天出院。结论 :两患儿经外科矫治取得了良好的结果     

A case of total left anomalous pulmonary venous connection with intact atrial septum diagnosed by two-dimensional and Doppler echocardiography

Unilateral total anomalous pulmonary venous connection from a unilateral lung is extremely rare. A 6-year-old patient with anomalous pulmonary venous connection from the entire left lung to the left innominate vein, with an intact atrial septum, diagnosed by two-dimensional and Doppler echocardiography is reported. The combination of two-dimensional and Doppler echocardiography is very useful not only for anatomical diagnosis, but also for evaluation of its hemodynamics, despite the wide anatomical variability of the pulmonary venous connection. This is the first report of a case of left total anomalous pulmonary venous connection diagnosed noninvasively.     

Enlarged coronary sinus. Mono and bidimensional echocardiographic aspects (author's transl)

One case of enlarged coronary sinus with a total anomalous pulmonary venous connection (TAPVC) is described. The echocardiographic findings are compared to those seen in cases with persistence of the left superior vena cava (PLSVC). The diagnosis has been established by single crystal and two-dimensional echocardiography, by cardiac catheterization and angiography and was confirmed at surgery; post-operative echocardiographic control was performed. The differential diagnosis between TAPVC or PLSVC and other anomalies which may simulate a dilated coronary sinus is discussed. The differences between TAPVC and PLSVC by contrast echocardiography are reported.     

Preoperative palliation of newborn obstructed total anomalous pulmonary venous connection by endovascular stent placement.

Patients with obstructed total anomalous pulmonary venous connection (TAPVC) usually present critically ill and continue to be extremely challenging with presurgical stabilization. We present an extra corporeal membrane oxygenation (ECMO)-dependent neonate with obstructed TAPVC that was successfully palliated with transvenous stent placement in the obstructed vertical vein.     

Echocardiographic evaluation of total anomalous pulmonary venous connection: Comparison of obstructed and unobstructed type

This study aims to compare the differences between obstructed and unobstructed total anomalous pulmonary venous connection (TAPVC) using echocardiography, and to evaluate the clinical and echocardiographic parameters associated with pulmonary venous obstruction (PVO).We conducted a retrospective study of 70 patients with TAPVC between 2014 and 2019. The morphologic and hemodynamic echocardiographic parameters of patients were observed and measured, and the parameters between obstructed and unobstructed TAPVC were compared. The clinical and echocardiographic parameter differences between the two groups were used for ROC curve analysis.Obstructed TAPVC was found in 30 (42.9%) of 70 patients. Between obstructed and unobstructed TAPVC, there were significant differences in atrial septal defect size, pulmonary artery maximum velocity (PA V_max ), peak E velocity of mitral valve, left ventricular fractional shortening, left ventricular ejection fraction, stroke volume and the incidence of patent ductus arteriosus, but there was no significant difference in birth weight. The first diagnosis age of obstructed TAPVC was earlier than unobstructed type. The ROC curve analysis for the first diagnosis age showed the sensitivity and specificity were 76.7%, 80% respectively. The ROC curve analysis for the PA V_max showed the sensitivity and specificity were 88.5%, 67.6% respectively.Patients with TAPVC had a high incidence of PVO. The presence of PVO can affect the size of atrial septal defect and the closure of the ductus arteriosus, cause significant changes in PA V_max, peak E velocity of mitral valve, left ventricular fractional shortening, left ventricular ejection fraction, stroke volume, lead to earlier symptoms and earlier first diagnosis age. The first diagnosis age and PA V_max were excellent values since they associated with PVO.