Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect

The heart and lung specimens in 31 cases of pulmonary value atresia and ventricular septal defect were studied at autopsy. Three types of natural arterial blood supply to the lungs were identified: 1) ductus arteriosus (patient or ligamentous) (12 cases); 2) major collateral arteries (20 cases); and 3) diffuse small pleural arterial plexus coexisting with either ductus arteriosus or major collateral arteries (17 cases). The ductus arteriosus and major collateral arteries did not coexist in the same lung in these cases. Confluent central pulmonary arteries were present in 22 (71%) of the 31 cases, involving 7 (58%) of the 12 cases of ductus arteriosus, 14 (70%) of the 20 cases with major collateral arteries and 1 case with an aorticopulmonary window. The pulmonary trunk (atretic or patent) was identifiable in 24 (77%) of the 31 cases. A lung or lungs that connected to a ductus (or ligamentum) had a complete and unifocal intrapulmonary arterial distribution (without arborization abnormalities). Major collateral blood supply was frequently multifocal and associated with arborization abnormalities. The size of the central pulmonary arteries was not related to the type of arterial blood source but seemed to be related to the amount of blood flow actually reaching the vessels, This study demonstrated a complex systemic arterial system supplying the lungs in these cases. The size, sources and relation among the ductus, the pulmonary artery confluence, the large and small collateral vessels and the intrapulmonary system are far more varied than has ever been reported previously. Careful and thorough premortem studies are crucial if surgical intervention is contemplated.     

Pulmonary to systemic flow ratios in patients with ventricular septal defect: estimation by transmitral flow velocity

Using pulsed Doppler echocardiography, left ventricular inflow flow volume (LVIV) and outflow flow volume (LVOV) were noninvasively determined, and the ratio of pulmonary to systemic flow (Qp/Qs) was evaluated as the ratio of LVIV to LVOV (LVIV/LVOV). Thirty patients with ventricular septal defect (VSD) were studied, and 47 cardiac patients without aortic or mitral valve disease or intracardiac shunt served as controls. LVOV was derived from the left ventricular ejection flow velocity and the outflow tract diameter immediately proximal to the aortic valve ring. LVIV was derived from the transmitral flow velocity and the M-mode tracing of mitral valve motion. Doppler-determined cardiac outputs (COin and COout) were calculated as the products of LVIV or LVOV as and heart rates. Cardiac outputs were also determined by the dye dilution method (COdye) references for comparison with Doppler-determined cardiac outputs. There were good correlations between COdye and COin (y = 1.18x-243, r = 0.85, p less than 0.005, SEE = 1026 ml/min) and between COdye and COout (y = 1.16x-323, r = 0.90, p less than 0.005, SEE = 639 ml/min). LVIV and LVOV correlated well in the controls (y = 0.95x + 5.3, r = 0.94, p less than 0.005, SEE = 6.6 ml). LVIV/LVOV was 0.97 +/- 0.1 (mean +/- SD) in the controls; whereas LVIV/LVOV (1.86 +/- 0.90) was significantly higher in patients with VSD (p less than 0.01) and this ratio correlated well with Qp/Qs by an oximetry (r = 0.98, SEE = 0.20, n = 14), including patients associated with pulmonary regurgitation. These findings indicate that our method permits determination of LVIV with a high degree of accuracy and that the Doppler-determined LVIV/LVOV is clinically useful to evaluate accurately the magnitude of shunt flows in patients with VSD.     

Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension

Histopathologic and morphometric studies of small pulmonary arteries were performed in 16 cases of secundum atrial septal defect (ASD) and severe pulmonary hypertension. Besides typical plexogenic arteriopathies found in six cases, organized microthrombi and what we call "musculoelastosis," that is, proliferation of longitudinal smooth muscle bundles and elastic fibers, were seen in small pulmonary arteries in three and four cases, respectively. These changes were observed co-existing in the remaining three cases. Thrombi of the small pulmonary arteries and musculoelastosis were the forms of pulmonary vascular disease characteristically found in the older patients with both ASD and pulmonary hypertension. Yamaki's index of pulmonary vascular disease, though effective in describing severity of plexogenic arteriopathy, proved to be less so for the intimal lesions in old microthrombi and for musculoelastosis. Among the cases with plexogenic arteriopathy, there was a positive significant correlation between the medial thickness of small pulmonary arteries and peak arterial pressure, which, however, was not demonstrated when all the cases of ASD were included.     

Persistent iatrogenic atrial septal defect after pulmonary vein isolation

INTRODUCTION: Pulmonary vein isolation (PVI) is widely practiced for treating atrial fibrillation. Transseptal access is necessary with one or even more transseptal sheaths to perform PVI. METHODS: In this prospective study, 31 patients were examined with transesophageal echocardiography before, immediately after and in a 3-, 6- and 12-month follow-up period for evaluation of iatrogenic atrial septal defect (iASD). All patients underwent PVI with double transseptal puncture. RESULTS: An iASD was detected in 27/31 patients (87%) with a maximum diameter of 1.0 mm. After 3 months, the iASDs were completely closed in 26/27 (96.3%) patients. In 1/27 (3.7%) patients, there was an iASD detectable even after 12 months. Clinically no patient suffered from cerebral or cardiac embolism in the follow-up period. CONCLUSIONS: We were only able to find small iASDs with left to right shunting after PVI but none with right to left shunting. iASDs following PVI show a high spontaneous closure rate already at 3 months of follow-up and are not associated with an increased rate of paradoxical embolism.     

Pulmonary artery thrombi detected by echocardiography in patients with pulmonary hypertension secondary to atrial septal defect.

This report presents three patients with severe pulmonary hypertension secondary to atrial septal defect associated with thrombus and spontaneous echo contrast within the pulmonary artery diagnosed by transthoracic and transoesophageal echocardiography. Clinical and echocardiographic features seem to suggest local thrombus formation within the pulmonary arteries as a direct consequence of pulmonary hypertension rather than venous thromboembolism.     

Evaluation of left atrial filling using systolic pulmonary venous flow velocity measurements in patients with atrial fibrillation

Background: The pattern of pulmonary venous flow velocity is useful for understanding the hemodynamic relationship between the left atrium and left ventricle in patients with a variety of diseases, and the systolic flow wave, in particular, is considered a clinically important parameter that reflects left atrial filling. Hypothesis: The study was undertaken to determine whether systolic pulmonary venous flow velocity patterns can be used to evaluate left atrial filling in patients with atrial fibrillation. Methods: We performed transesophageal pulsed Doppler echocardiography and cardiac catheterization in 34 patients with chronic atrial fibrillation (10 with hypertrophic cardiomyopathy, 5 with dilated cardiomyopathy, 7 with previous myocardial infarction, and 12 with isolated atrial fibrillation) and 15 normal controls in sinus rhythm. Results: Mean pulmonary capillary wedge pressure, V-wave height in the pulmonary capillary wedge pressure curve, and left ventricular end-diastolic pressure were significantly higher in the hypertrophic cardiomyopathy and dilated failing heart (previous myocardial infarction and dilated cardiomyopathy) groups than in the isolated atrial fibrillation and normal groups. The peak velocity and time-velocity integral of the systolic pulmonary venous flow velocity, and percent left atrial emptying fraction were significantly lower in the dilated failing heart group than in the isolated atrial fibrillation, hypertrophic cardiomyopathy, and normal groups. The peak velocity and time-velocity integral of the systolic pulmonary venous flow velocity, percent left atrial emptying fraction, and V-wave height were comparatively constant when the preceding R-R intervals were relatively stable in the isolated atrial fibrillation group and in 4 of the 10 patients with hypertrophic cardiomyopathy. However, changes in these variables correlated with the preceding R-R interval in all patients with dilated failing hearts and in 6 of the 10 patients with hypertrophic cardiomyopathy. Conclusion: Transesophageal pulsed Doppler echocardiographic measurements of systolic pulmonary venous flow velocity are valid indicators of left atrial filling in patients with atrial fibrillation.     

Pulmonary vein isolation in patients with paroxysmal atrial fibrillation after direct suture closure of congenital atrial septal defect.

Two cases of paroxysmal atrial fibrillation (AF) first occurred 15 and 36 years, respectively, after isolated direct suture closure of an atrial septal defect (ASD) and failed to be controlled by antiarrhythmic drug therapy. In these cases, an atrial transseptal procedure was feasible and no residual iatrogenic ASD was observed, even after multiple procedures. Pulmonary vein (PV) isolation was also feasible and safe and could eliminate the AF completely. PV isolation may become an alternative to antiarrhythmic drug therapy in patients with paroxysmal AF occurring late after an isolated direct suture closure of an ASD.     

Diagnosis of increased pulmonary blood flow by suprasternal M-mode echocardiography in atrial septal defect

The right pulmonary artery (PA) was quantitatively assessed by Suprasternal M-mode echocardiography in 25 patients in whom an atrial septal defect (ASD) was suspected clinically. In 10 patients an ASD was excluded (Group 1) and in 15 it was confirmed (Group 2). The smallest diameter of the right PA at end-diastole in Group 1 was 8.8 ± 1.5 mm/m² body surface area and in Group 2 14.8 ± 3.6 mm/m² (p < 0.001). The greatest diameter of the right PA during systole was also much smaller in Group 1 (11.3 ± 1.2 mm/m²) than in Group 2 (17.7 ± 3.5 mm/m²) (p < 0.001). The absolute and percent systolic expansion of the right PA did not differ in the 2 groups (2.7 ± 0.5 mm [29.1 ± 10.8%] in Group 1 and 2.9 ± 0.8 mm [20.8 ± 9.8%] in Group 2). No correlation was found between measured and derived echocardiographic variables of the right PA and the magnitude of the left-to-right shunt. Patients in Group 2, who had an additional pressure elevation in the PA, showed, on average, a larger right PA and a smaller percent systolic expansion. The study demonstrates characteristic alterations in the wall motion pattern of the right PA in patients with ASD, indicating increased pulmonary blood flow.     

Pulmonary vein blood flow velocity in pulsus paradoxus.

The effect of inspiration on pulmonary vein blood flow velocity and forward pressure gradient from pulmonary vein to left atrium was studied in seven patients with chronic constrictive pericarditis, five of whom had clinically obvious pulsus paradoxus. Compared to patients without pericardial disease, where inspiration produced no change, patients with pericardial disease showed an inspiratory fall in the forward pressure gradient and forward blood flow velocity in the pulmonary veins on inspiration. The mechanism of pulsus paradoxus in these patients can be explained by incomplete transmission of the inspiratory fall of intrathoracic pressure to the left atrium.     

Cyanotic "ostium secundum" atrial septal defect without pulmonary hypertension

This patient presented with central cyanosis. A clinical diagnosis of atrial septal defect with possible Eisenmenger syndrome was made. The correct diagnosis was made at catheterization.     

经股静脉途径一次性完成房室间隔复合型缺损的介入治疗

目的探讨经股静脉途径一次性完成室间隔缺损（VSD）合并房间隔缺损（ASD）的可行性、方法及疗效。方法 5例患者,术前超声检查均确诊为VSD合并ASD。年龄3~21岁,VSD直径4.5~7.6 mm,ASD直径8~21 mm。5例患者均于术中先行左心室造影,确定VSD适合封堵后,首先封堵VSD,然后封堵ASD。结果 5例患者均单纯经股静脉途径一次介入治疗成功。患者术中及术后均无并发症。结论经股静脉途径一次性完成房室间隔复合型缺损的介入治疗具有技术上的可行性、创新性和良好的治疗效果。     

成人房间隔缺损合并肺动脉高压的缺损闭合治疗

随着成人先天性心脏病临床处理指南的更新,成人房间隔缺损合(ASD)合并肺动脉高压(P H)的缺损闭合治疗策略有了新进展.该文简述成人ASD合并P H的临床分类及特点,重点介绍ASD合并P H的缺损闭合策略,包括常规缺损闭合、"药物到缺损闭合"策略以及带孔封堵器封堵术等.     

房间隔缺损合并肺动脉瓣狭窄介入治疗的临床评价

目的:探讨房间隔缺损(ASD)合并肺动脉瓣狭窄(PS)行介入治疗的临床安全性及其疗效。方法:31例患者,年龄2.5~67(中位年龄31)岁;24例经胸超声心动图(TTE)诊断为ASD合并PS,房间隔缺损最大直径为7~27(16.0±4.8)mm,7例超声心动图术前漏诊ASD,肺动脉瓣狭窄压差35~120(89.8±22.9)mm Hg(1mm Hg=0.133k Pa),所有患者术前行右心室造影检查。先行经皮球囊肺动脉瓣成形术(PBPV),而后置入房间隔缺损封堵器闭合缺损,选择的封堵器直径为12.0~38.0(22.6±5.9)mm。结果:本组28例行PBPV术及ASD介入封堵治疗28均获得成功;其中3例封堵器脱落入主动脉,介入方法取出后,再置入较大封堵器封堵成功,PBPV术后即刻右室收缩压由术前(89.8±22.9)mmHg下降至(39.8±11.3)mm Hg,肺动脉右心室跨瓣压差由术前mm Hg降至(14.8±8.9)mm Hg(P<0.001)。右心室舒张末压由术前(9.9±0.28)mm Hg降至(5.07±0.57)mm Hg(P<0.005),PBPV术后即刻跨瓣压差下降达优良为100%。术后超声心动图随访于术后即刻、1、3及6个月,疗效满意,房间隔未见残余分流。结论:同期介入治疗肺动脉瓣狭窄合并房间隔缺损安全可行,效果良好,但由于合并PS患者,超声心动图术前常常低估或漏诊ASD,因此,需先行肺动脉瓣球囊扩张术后,超声心动图重新复核ASD大小,再行ASD封堵术,必要时分期手术。