Partial anomalous pulmonary venous drainage associated with mitral stenosis with an intact atrial septum A distinctive hemodynamic syndrome

A case of partial anomalous venous drainage associated with mitral stenosis and intact interatrial septum is presented. The hemodynamic findings consisted of pulmonary hypertension, markedly elevated pulmonary artery wedge pressure and large left-to-right shunt at the atrial level. These findings are distinctive of an intact septum, because in Lutembacher's syndrome there is a tendency for equalization of pressure in the atria and, therefore, the pulmonary artery wedge pressure may be normal or only slightly elevated. The findings of high and low pulmonary artery wedge pressure in different parts of lungs may be additional evidence for presence of partial anomalous venous connection.     

Estimation of pulmonary artery pressure from pulmonary vein wedge pressure

Correlations between pulmonary artery and pulmonary vein wedge pressures were investigated in 13 patients with atrial septal defect and 1 patient with Tetralogy of Fallot. Pulmonary vein wedge pressure wave form resembled that of pulmonary artery pressure, and the former lagged behind the latter by 70 to 110 msec (mean 88 ± 14) as observed by the fluid-filled catheter system. Diastolic pulmonary artery and diastolic pulmonary vein wedge pressures were nearly identical. Although systolic and mean pulmonary artery pressures correlated well with respective pulmonary vein wedge pressures, there were discrepancies when systolic and mean pulmonary artery pressure exceeded 35 and 20 mm Hg, respectively. However, systolic and mean pulmonary artery pressures could be estimated by adding the difference between the diastolic pulmonary vein wedge pressure and the mean left atrial pressure to corresponding systolic or mean pulmonary artery pressure. In conclusion, pulmonary artery pressures can be estimated by measuring pulmonary vein wedge pressures and the mean left atrial pressure.     

Partial anomalous pulmonary venous drainage and intact atrial septum with mitral stenosis: the paradox of a small shunt

Clinical, hemodynamic, and angiographic features of 10 patients with partial anomalous pulmonary venous drainage, intact atrial septum, and rheumatic mitral stenosis have been presented. Seventeen patients with this combination of anomalies reported in the literature have also been reviewed. The clinical diagnosis of mitral stenosis was possible in each of our 10 cases. Partial anomalous pulmonary venous drainage and intact atrial septum in addition to mitral stenosis was clinically suspected in only four patients. The findings suggesting additional presence of partial anomalous pulmonary venous drainage and intact atrial septum in a patient with mitral stenosis include: wide variable splitting of the second sound, pulmonary ejection systolic murmur with or without a thrill, and radiological evidence of unilateral increase in pulmonary vascularity or unilateral hilar pulsations. Hemodynamic findings were characterized by a relatively modest left to right shunt (Qp/Qs:2.2 +/- 1.4). Elevated pulmonary vascular resistance was found in the anomalously draining as well as the normally draining lung segments (9.1 +/- 4.9 and 6.5 +/- 3.4 units, respectively, t = 1.32;NS). The mechanism of the unexpected high resistance in the anomalously draining lung segments permitting only a small left to right shunt is discussed.     

A giant main pulmonary artery aneurysm associated with infundibular pulmonary stenosis

We report a case of a giant pulmonary artery aneurysm associated with infundibular pulmonary stenosis. Echocardiography disclosed markedly enlarged main pulmonary artery, but no left to right shunt flow at levels of the atrial septum, ventricular septum and the pulmonary artery. Continuous wave Doppler revealed a maximum velocity of 1.5 m/s which corresponded to the pressure gradient between the right ventricle and the pulmonary artery of 9.5 mmHg. Contrast-enhanced multidetector-row computed tomography with a 16-slice scanner revealed pulmonary artery aneurysm with the maximum diameter of 67 mm on axial image.     

Retrograde pulmonary arteriography

We performed retrograde pulmonary arteriography by means of a pulmonary venous wedge injection in 10 patients with no demonstrable intrapericardial pulmonary arteries by "conventional" angiographic techniques. In all cases but one, the procedure demonstrated the feasibility of a further operation. No complications were observed. Retrograde pulmonary arteriography is an important additional method for determining the existence of surgically accessible pulmonary arteries when other techniques have failed.     

Use of small stents for rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect.

Branch pulmonary artery stenosis frequently occurs in pulmonary atresia with ventricular septal defect (PA/VSD). Balloon dilation alone is often unsuccessful in patients with severely hypoplastic pulmonary arteries with residual stenoses after surgical repair. In an attempt to promote distal pulmonary artery growth, 17 stents were placed in 12 severely stenotic pulmonary artery lesions in 10 patients with PA/VSD. All had prior surgery, including pulmonary artery repair, right ventricle to pulmonary artery homograft, and, in 6 of 10, closure of VSD. Median age at stent placement was 16.8 months (range, 13.2-56). Stents were placed using 3.0, 3.5, or 4.0 mm balloons in all but one lesion, in which a 7 mm balloon was used. Following stent placement, there was an increase in the lesion diameter from 1.5 to 3.4 mm (P < 0.05) and an increase in flow to the affected lung from 27% to 34% (P < 0.05). Repeat catheterization 2 to 6 months after stenting in six patients revealed complete occlusion in two of eight lesions. In the other six vessels, there was an increase in distal vessel diameter from 2.96 to 3.94 mm (P < 0.05) even though four had severe restenosis requiring restenting. Two patients underwent surgical pulmonary artery reconstruction and stent removal because of adequate distal vessel growth. Stenting of hypoplastic pulmonary arteries in PA/VSD results in immediate improvement in vessel size and blood flow. Stent restenosis is common although distal vessel growth can be achieved. Stenting of these lesions should be reserved only for those patients unresponsive to other interventions.     

Coronary‐pulmonary arterial fistula in a neonate with pulmonary atresia—ventricular septal defect and single coronary artery

In cases of pulmonary atresia with ventricular septal defect (PA‐VSD), coronary‐pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA‐VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS). Successful surgical correction with VSD closure and right ventricle (RV) to the pulmonary artery (PA) conduit was made.     

Pulmonary venous pressure: relationship to pulmonary artery, pulmonary wedge, and left atrial pressure in normal, lightly sedated dogs.

Because pulmonary venous pressure has never been measured, it is unclear whether pulmonary wedge pressure measures left atrial pressure, as commonly assumed, or pressure more upstream in the pulmonary venous or capillary beds. Fluid-filled mean pulmonary artery and pulmonary wedge pressure were compared with pulmonary venous and left atrial pressure obtained with high-fidelity micromanometer catheters in eight lightly sedated dogs over a physiologic range of filling pressures. In all conditions, mean pulmonary wedge pressure was virtually identical (r = 0.99) to mean left atrial pressure (slope = 0.99; intercept = -0.46 mm Hg). At the same time, mean pulmonary venous pressure (17.1 +/- 6.5 mm Hg) was intermediate between mean pulmonary artery pressure (20.2 +/- 6.2 mm Hg) and mean pulmonary wedge pressure (13.3 +/- 6.2 mm Hg; P < 0.0001) or mean left atrial pressure (13.4 +/- 6.3 mm Hg; P < 0.0001). These relationships were maintained over normal and increased pressure ranges. As measured by conventional flow-directed pulmonary catheters, mean pulmonary wedge pressure accurately reflects left atrial pressure in lightly sedated, spontaneously breathing normal dogs.     

The impact, and surgical implications, of isolated anomalous connection of one pulmonary vein

Partially anomalous pulmonary venous connection of a solitary pulmonary vein in the setting of an intact atrial septum is often subclinical, and the indications for surgical repair are controversial. Here we describe a patient who developed a significant shunt over a period of 10-years. Flow through the anomalously connected pulmonary segment depends on the difference of pressure between the right and left atrium, and may increase with age.     

Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis

The purpose of this study was to assess the capability of multiplanar cine magnetic resonance imaging (MRI) for evaluating pre- and post-operative pulmonary circulation in patients with pulmonary atresia and severe pulmonary stenosis. Seventy-three multiplanar cine MRIs were performed in 30 patients, aged 1 month to 7 years (mean age, 27 months). The morphology and size of the central pulmonary arteries (PA), source of the major aortopulmonary collateral arteries (MAPCA), patency of Blalock–Taussig (BT) shunt vessels, and the post-operative pulmonary circulation were assessed. The accuracy of cine MRI was compared with that of angiography in all patients. The PA was visualized to the first hilar branch in 21 patients, but not in 8 patients in whom the central PA was absent. On follow-up MRI, PA growth was measured, and the results showed excellent correlation with the results obtained by angiography. In 17 patients who had undergone 23 BT shunt operations, cine MRI correctly demonstrated all patient shunts and 5 of 6 stenotic lesions. Multiplanar cine MRI provided excellent detail of the peripheral PA in all patients, 7 of 8 peripheral pulmonary stenoses, 3 of 4 nonconfluent pulmonary arteries, and 2 of 3 PA obstructions. Although the sources of MAPCA were identified in 7 of 9 patients, the distal connection of the MAPCA was not detected in all patients. Seven patients were reexamined after pulmonary plasty; they exhibited normal pulmonary flow patterns. Multiplanar cine MRI provides high-resolution imaging of PA with dynamic visualization of flow and is an effective noninvasive technique for evaluating pre- and post-operative patients with pulmonary atresia and severe pulmonary stenosis.     

先心病合并重度肺动脉高压双向分流的外科对策

目的:探讨先天性心脏病伴重度肺动脉高压双向分流的外科对策及疗效。方法:回顾分析32例患者的临床资料,其中,室间隔缺损(VSD)22例,VSD合并房间隔缺损(ASD)5例,动脉导管未闭5例。术前均有不同程度劳力后紫绀,心脏B超提示双向分流,术中测量平均肺动脉压/平均体循环压>0.85,平均0.90±0.15,采用心脏停跳手术6例,不停跳手术26例。房间隔均采用自体心包片做成单向活瓣,以减轻术后早期右心负荷。结果:早期死亡3例(9.4％,3/32)。术后早期均有明显低氧血症,呼吸机支持,平均35.26±17.59小时,无气管切开病例。随访2～86(平均23.6±17.2)月,晚期死亡2例(6.3％,2/32),顽固右心衰3例(9.4％,3/32),其余24例(75％,24/32)恢复良好。结论:先心病伴重度肺动脉高压部分双向分流的病例,采用浅低温心跳不停心内直视手术有利于心肺功能保护,术中作房间隔单向活瓣有利于其早期恢复。     

超声心动图在肺动脉闭锁合并室间隔缺损诊断及外科治疗中的应用价值

目的:探讨超声心动图在肺动脉闭锁合并室间隔缺损诊断及外科治疗中的应用价值.方法:回顾性选取云南省阜外心血管病医院2018年1月至2020年5月期间外科治疗的肺动脉闭锁合并室间隔缺损患者21例,分析其术前超声心动图特点、解剖分型,评估肺动脉发育及体肺侧支动脉情况,并与CT结果相对比.术中经食道超声心动图监测,术后随访超声...     

Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum

Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.     

Echocardiographic diagnosis of anomalous origin of the left coronary artery from the pulmonary artery

The objective of this study was to analyze echocardiographic characteristics of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and to explore the diagnostic value of transthoracic echocardiography. The echocardiographic characteristics of 8 patients hospitalized with ALCAPA from 2000 to 2005 were analyzed retrospectively, including the results of real time three-dimensional echocardiography in one case, and compared with angiographic results. Eight cases included 6 older type patients and 2 infant type patients. Echocardiography showed abnormal vessel inserting into pulmonary artery (PA), continuous shunt into PA and intercoronary collateral signals within the ventricular septum in all cases and bifurcate structure of the abnormal vessel with retrograde filling in 4 cases. The morphological and functional changes and valvular regurgitation induced by insufficient myocardial perfusion were also evaluated. In former 4 patients, 2 cases were misdiagnosed as right coronary artery-PA fistula and the other 2 cases were given an uncertain diagnosis of anomalous origin of the coronary artery because of the visualization of the echo-free linear structure which apparently arose from the aorta resembling a normal left coronary artery. The latter 4 patients were correctly diagnosed by excluding the aforementioned interference. The diagnosis of ALCAPA was confirmed by angiocardiography in all patients and by intraoperative findings in 4 patients. Based on the apprehension of ultrasonic features and the enhancement of diagnostic alertness, the echocardiography can evaluate ALCAPA accurately and give more information than angiography. It may be the first diagnostic choice.     

室间隔缺损合并重度肺动脉高压围手术期处理

目的 :评估室间隔缺损合并重度肺动脉高压手术治疗围手术期处理的效果。方法 :选择 5 5例室间隔缺损合并重度肺动脉高压病例 ,在手术时放置Swan ganz导管 ,术后进行肺动脉压监测 ;2 0例用单向活瓣补片修补室缺 ,术后用超声心动图监测分流情况 ;用镇静 ,肌松 ,过度通气 ,血管扩张剂等治疗肺动脉高压危象。结果 :发现 7例持续肺动脉高压 ,死亡 2例 ;2 7例有肺动脉高压危象 ,死亡 2例 ;在严重肺动脉高压时 ,单向活瓣补片有右向左分流 ,肺动脉压下降后分流停止 ,未发现左向右分流。结论 :室间隔缺损合并重度肺动脉高压应把握好手术适应症 ,有不可逆的肺动脉高压患者禁止手术 ;Swan ganz导管在术后肺动脉高压处理上有重要作用 ;单向活瓣补片修补室缺能在术后发生重度肺动脉高压时发挥良好的右向左单向分流作用 ,肺动脉压下降后又能自动关闭。     

Hybrid melody pulmonary valve replacement in an adult with severe pulmonary hypertension and pulmonary artery aneurysm

A 48‐year‐old female with D‐TGA, ventricular septal defect (VSD), pulmonary stenosis, pulmonary hypertension (PAH), and total anomalous pulmonary venous connection underwent hybrid intervention for a pulmonary artery (PA) aneurysm and replacement of a dysfunctional pulmonary valve (PV). She underwent a hemi‐Mustard procedure at 9 years of age but remained cyanotic. She developed atrial fibrillation, heart failure, and functional decline at 43 years of age. A chest CT demonstrated a 6 cm PA aneurysm that upon re‐imaging at 48 years had increased to 11 cm. A catheterization procedure revealed severe PS, PR, residual VSD, severe PAH with a pulmonary vascular resistance of 30 Wood units. She was evaluated and turned down for heart‐lung transplantation at another institution. She was subsequently referred to our institution for heart‐lung transplantation but was felt to be at unacceptably high risk given the complexity of her anatomy, imaging suggesting liver cirrhosis and liver biopsy with extensive fibrosis. After extensive discussion of risk and benefits, the patient agreed to proceed with a hybrid intervention, consisting of surgical aneurysm resection/PA repair, tricuspid valve repair; PV replacement with a Melody valve, and VSD closure. There were no complications and she was discharged home within 2 weeks. Six months post procedure, she is not on oxygen, her resting room air saturation is 94%, and echocardiography shows stable Melody valve function. This case highlights the utility of a hybrid approach in the treatment of an adult with complex congenital heart disease, heart failure and severe PAH, considered at the highest risk for adverse surgical outcomes. The short‐term efficacy of the Melody valve in severe PAH is reassuring. © 2013 Wiley Periodicals, Inc.     

Clinical implication of inadvertent monitoring of pulmonary artery pressure via a “left atrial” catheter

A patient is described in whom a left atrial catheter was inadvertently wedged in a pulmonary vein following mitral valve replacement. The pulmonary vein wedge pressure obtained approximated the pulmonary artery pressure and was suggestive of paraprosthetic mitral valve regurgitation.     

Partial left anomalous pulmonary venous return: a diagnostic dilemma

Two adults were seen with left partial anomalous pulmonary venous return with intact atrial septum. Both cases had remained undiagnosed despite medical attention since childhood, including previous cardiac catheterization in one patient. Noninvasive tests, indocyanine green dye injections, pulmonary artery angiography, and venous sampling for oximetry high in the superior vena cava above the entry of the innominate vein will help establish the diagnosis of this disorder.     

Tight mitral stenosis with normal unilateral pulmonary capillary pressure

There are few reports of severe mitral stenosis with normal pulmonary artery wedge pressure. In order to illustrate this problem we present two adult patients with these abnormalities. Both patients had intact interatrial septum with partial anomalous pulmonary venous drainage, one to the inferior vena cava (The Scimitar Syndrome) and the other to the superior vena cava. In the first case the right pulmonary artery wedge pressure was 11 mm Hg. In the second case this pressure was only 8 mm Hg. Pulmonary arteriograms are of paramount importance in the diagnosis and surgical management. In both cases the surgical procedure with mitral commissurotomy and transplant of the anomalous pulmonary veins to the left atrium was successful.     

房水平单向活瓣式补片在先天性心脏病并发重度肺动脉高压术中的应用

目的:探讨并发重度肺动脉高压的先天性心脏病患者的外科手术方式。方法: 采用房水平单向活瓣补片对48例本病患者进行手术治疗。其中,室间隔缺损30例,房间隔缺损3例,室间隔缺损并发房间隔缺损8例,室间隔缺损伴动脉导管未闭7例。结果: 术后1例（2%）死于低心排综合征。术后早期均有明显低氧血症。术后1周仍出现跨单向活瓣补片分流40例,术后3个月活瓣仍开放10例,术后26个月提示活瓣均关闭,分流消失,经皮血氧均为95%以上,肺动脉压均明显下降。结论: 房水平单向活瓣式补片治疗先天性心脏病并发肺动脉高压,使患者度过术后危险期,提高手术成功率。