Rare type of intrapulmonary drainage of one lung by the other with total anomalous pulmonary venous return

A rare form of obstructed total anomalous pulmonary venous drainage is presented. A neonate with asplenia, pulmonary atresia and complex congenital heart disease also had drainage of the left lung forming a venous confluence that proceeded in an intrapulmonary fashion through the right lung. A hypoplastic pulmonary venous channel then emerged from the right upper lobe leading to the superior vena cava. Pulmonary venous obstruction was suspected on initial plain chest X-ray examination and on angiography, which revealed the pulmonary artery to be larger than anticipated. Administration of prostaglandin E1 may allow augmentation of pulmonary blood flow and angiographic definition of this association in infants with asplenia complex.     

Unusual form of bilateral partial anomalous pulmonary venous drainage

A 23-year-old man with known valvular pulmonary stenosis was found to have a very unusual form of bilateral partial anomalous pulmonary venous drainage. The venous drainage from the upper parts of both lungs was anomalous, draining from the left lung via a persistent left-sided superior vena cava and from the right lung directly into the right atrial/right superior vena caval junction. The venous drainage from the lower parts of both lungs was normal. The embryological pathogenesis of the defect is discussed.     

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

We report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery. Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.     

Right-sided scimitar syndrome in a patient with a single aortic trunk and coronary-cameral venous fistula

Scimitar syndrome (SS) is a rare congenital anomaly characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persistent left superior vena cava, and dextroposition of the heart. We report a rare variant of SS in a 44-year-old man together with a single aortic trunk, as well as a coronary-cameral venous fistula.     

Association of cor triatriatum and obstruction of abnormal left pulmonary venous drainage. Apropos of a case

A case of subtotal cor triatriatum associated with a partial anomalous pulmonary venous drainage of the left lung, in a 30 months old boy is reported. Diagnosis was suggested by the presence of pulmonary venous obstruction and pulmonary hypertension. Doppler, echocardiogram diagnosed the cor triatriatum and the degree of the pulmonary hypertension. The cine-angiography demonstrated the anomalous, obstructive, left pulmonary venous drainage. The pulmonary hypertension has mandatorised an early surgical cure, the result was satisfactory at 16 months.     

Echo Doppler detection of external compression of the vertical vein causing obstruction in total anomalous pulmonary venous connection

External obstruction of the pulmonary venous return was detected by two-dimensional (2-D) and pulsed Doppler echocardiography and was confirmed by angiography and at surgery in two infants with isolated supracardiac total anomalous pulmonary venous connection (TAPVC) to the left vertical vein. In both infants, the left vertical vein ascended behind the left pulmonary artery and anterior to the left main stem bronchus. This arrangement produces mechanical obstruction to both vertical vein flow and pulmonary venous drainage. Early detection and surgical correction of obstructed pulmonary venous return are necessary to prevent severe right-sided heart failure and death. Doppler echocardiography is recommended when evaluating infants with TAPVC for possible anatomic or mechanical obstruction to pulmonary venous return.     

Patterns of anomalous pulmonary venous connection/drainage in hypoplastic left heart syndrome: diagnostic role of Doppler color flow mapping and surgical implications.

Differentiation between anomalous connection and anomalous drainage of the pulmonary veins in hypoplastic left heart syndrome is important before either the Norwood procedure or heart transplantation is performed. To determine the prevalence of echocardiographically detected anomalous connection or drainage, or both, of pulmonary veins in patients with this syndrome, preoperative two-dimensional echocardiographic and Doppler color flow mapping studies of 317 patients who underwent the stage I Norwood procedure were reviewed. The term "connection" was used to describe the precise anatomic attachment of the pulmonary veins and the term "drainage" to describe the physiologic end point of pulmonary venous flow. Twenty patients (6.3%) had anomalous connection or drainage, or both, of the pulmonary veins by preoperative echocardiographic and Doppler examination. The subcostal and suprasternal scans best showed the anatomic details of the pulmonary veins. All these patterns were confirmed intraoperatively and could be grouped as follows: 1) partial anomalous connection and drainage (two patients); 2) total anomalous connection and drainage (eight patients); 3) normal connection with total anomalous drainage (eight patients); and 4) normal connection with partial anomalous drainage (two patients). The advantage of adding Doppler color flow mapping to two-dimensional echocardiography and conventional Doppler study was clearly demonstrated in the detection of small accessory vertical veins, their course and the presence or absence of obstruction. Doppler color flow mapping was especially helpful in detecting anomalous drainage of the right pulmonary veins to the right of the superior attachment of the septum primum.     

Partial anomalous pulmonary venous drainage of the left lung into the innominate vein (author's transl)]

From 5 cases of partial anomalous pulmonary venous drainage of the left lung into the innominate vein, it has been thought of interest to situate this type of venous anomaly among the partial anomalous pulmonary venous drainage classification. It is revealed in 2 different ways. In adults, this malformation sets the problem of the diagnosis of an abnormal chest Xray, without symptom. In children, this vascular abnormality is discovered during the exploration of a congenital cardiopathy which causes the main symptoms. Embryological data are reviewed and statistics of frequency which lead to show that this type of partial anomalous venous drainage is often misunderstood, particularly with regard to anomalous venous drainage of the right lung.     

Pulmonary venous drainage through a highly vascularized left atrial tumor

We present a patient with symptomatic congestive heart failure due to a left atrial sarcoma infiltrating and apparently occluding the left pulmonary veins. The tumor was highly vascularized and enabled attenuated blood drainage from the left upper and lower pulmonary veins despite intra-operative appearance as completely obliterative, thus avoiding persistent left lung pulmonary edema. The tumor was partially removed and the pathologic findings showed advanced angiogenesis. Malignant tumors may occlude large blood vessels and thus may result in the development of a collateral flow. However, we suggest that highly vascularized tumors (macroscopic totally occlusive) may serve as a sponge and enable attenuated blood flow through the tumor and hence may avoid a complete cut off in blood supply or drainage.     

脏层胸膜在胸膜腔液体循环中作用机制的临床研究

目的　结合心力衰竭、肺淋巴管癌病和肺淋巴管平滑肌瘤病的临床和病理特征 ,探讨脏层胸膜在胸膜腔液体循环中的作用机制。方法　 (1)分析 1999～ 2 0 0 1年 5 31例心力衰竭患者胸膜腔积液的发生率 ,其中冠心病等所致的左心功能 级 32 9例 ,慢性肺原性心脏病右心功能失代偿 2 0 2例 ;(2 )分析 1998～ 2 0 0 2年 6例肺内淋巴管性疾病胸膜肺组织活检的病理表现和肺内淋巴管的分布特征。结果　 (1)胸腔积液的发生率左心功能 级者为14.89%;肺心病右心功能失代者为 0 %,两者差异有显著性 (p<0 .0 1) ;(2 )肺内淋巴管性疾病的胸膜肺组织病理可见肺内淋巴管起源于胸膜脏层。结论　肺内淋巴管起源于胸膜脏层 ,在胸膜腔液体的循环过程中和壁层胸膜一样发挥着重要的作用。     

A "late" scimitar syndrome. Diagnostic contribution of cardiac computed tomography

A 51-year-old female hospitalized with a non-specific colitis, presented a crescent-like shadow in the right lower lung accompanied by a reduced right lung volume on a routine chest x-ray. There was no family history of congenital heart disease. The initially performed, noninvasive, contrast enhanced cardiac CT (electron-beam tomography [EBT]) proved the suspected diagnosis of a partial, anomalous pulmonary, transdiaphragmatic vein drainage (APVD) in combination with a hypoplastic right lower lobe and dextrocardia. These findings are in accordance with scimitar syndrome. Regarding to the clinical situation with symptoms like slowly progressive dyspnea on exertion and low exercise tolerance for the last 2 years and an invasively documented left-to-right shunt ratio >50% (Qp:Qs = 2.6 : 1) surgical repair was recommended. The anomalous vein was connected to the left atrium creating a "neo-septum". On a postoperative checkup after 9 months the patient is without any medication, symptoms during moderate activity are relieved, exercise tolerance was substantially better and noninvasive imaging visualized the corrected drainage of the anomalous vein to the left atrium.     

Double venous drainage in scimitar syndrome. Ideal anatomy for percutaneous complete cure

Congenital venolobular or scimitar syndrome is a rare congenital cardiopulmonary anomaly consisting in a partial anomalous pulmonary venous drainage, lung hypoplasia, and anomalous systemic arterial supply to the lung. It can associate with other congenital disorders which will confer the clinical presentation and prognosis of these patients. In most of the cases, the therapeutic approach is partial, as anatomy allows only aberrant arterial embolisation. We present a 6-year-old girl with recurrent pulmonary infections, diagnosed as scimitar syndrome with double collector drainage to the inferior caval vein and left atrium, undergoing interventional catheterisation for complete correction of her disorder. The anomalous systemic artery supply was embolised and the anomalous venous drainage was occluded. The patient was asymptomatic during follow-up, which supports the interventional catheterisation approach as a valid therapeutic option in cases of scimitar syndrome with double venous drainage.     

Radionuclide diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (so-called hemitruncus)

A 5-week-old infant presented with signs of severe congestive heart failure and pulmonary hypertension. Injection of technetium-99m pertechnetate demonstrated anomalous perfusion of the right lung. Subsequently, anomalous origin of the right pulmonary artery from the ascending aorta was proven at cardiac catheterization and repaired. Repeat injection of radionuclide 1 week postoperatively demonstrated normal flow to the right lung.     

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.     

Anomalous unilateral single pulmonary vein mimicking partial anomalous pulmonary venous return

We report a case in which the clinical findings of a partial anomalous pulmonary venous return were present but with termination of the anomalous pulmonary vein into the left atrium. There was hypoplasia of the right lung, dextroposition of the heart, and a loop-like vascular density in the right lung field. Cardiac catheterization revealed no shunt, and on pulmonary angiograms, a large common right pulmonary vein draining the entire right lung and entering the left atrium by way of a single orifice. Possible embryologic explanations include atresia of the right inferior pulmonary vein and persistence of the ipsilateral superior pulmonary vein.     

Massive systemic-to-pulmonary collateral arteries in the setting of a cavopulmonary shunt and pulmonary venous stenosis

We report a patient in whom a cavopulmonary anastomosis had been constructed, along with repair of anomalous pulmonary venous drainage. Left-sided pulmonary venous obstruction led to redistribution of the flow to the right lung. The reversal of flow in the left pulmonary artery was accentuated by flow through collateral arteries feeding the left lung. Within 14 months, the collateral flow increased six-fold, resulting in a doubling of the cardiac output.     

Rhabdomyomatous dysplasia of the newborn lung associated with multiple congenital malformations of the heart and great vessels

Interstitial proliferation of striated muscle in the lung is extremely rare. Most cases are associated with other congenital malformations, such as lung sequestration, diaphragmatic hernia, or cardiac malformations. We describe a newborn with rhabdomyomatous dysplasia of the lung associated with multiple congenital malformations of the heart and great vessels. The female neonate was born at 37 weeks of gestation as the second child to a 31-year-old woman without relevant previous medical or family history. In week 26 of gestation, a complex heart malformation and polyhydramnion were diagnosed by ultrasound. Postnatally, right lung hypoplasia, a bilobar right and left lung, anomalous drainage of the pulmonary veins, atrial and ventricular septal defects, and double-outlet right-ventricle and multiple aortopulmonary collaterals were described. Histological examination of a biopsy of the right lung demonstrated the presence of numerous bundles of striated muscle fibers arranged randomly in the pulmonary interstitium. Unilateral resection of the right lung was not a therapeutic option, because the left lung had developed bronchopulmonary dysplasia with severe reduction in gas exchange as a consequence of long-term mechanical ventilation. Symptomatic relief and palliative cardiac surgery were offered. At age 5 months, the infant died of a pulmonary hemorrhage following cardiac surgery.     

A 72 year old woman with ALCAPA

ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery), which causes the left coronary artery to grow with an anomalous origin from the pulmonary artery, is a rare disease which may result in myocardial infarction, congestive heart failure, and sometimes death during the early infantile period. A 72 year old woman with ALCAPA syndrome is presented. The asymptomatic patient presented with a cardiac murmur which was discovered during a routine check up for a gynaecological intervention. Coronary cineangiography established the diagnosis. Although surgical correction is the usual treatment for such cases, medical treatment was preferred for this patient because she was asymptomatic without clinical signs of heart failure.     

肺部疾患血浆氨基酸末端脑钠素前体水平的预测价值分析

目的 通过观察肺部疾病及其合并左心功能不全患者血浆氨基酸末端脑钠素前体(N-terminal pro-B-type natriuretic peptide,NT-proBNP)水平的变化,及其对疾病预后的预测,探讨其变化规律及临床意义.方法 利用酶联免疫吸附试验法测定81例肺部疾病患者血浆中NT-proBNP的浓度,其中未合并左心功能不全者68例,合并左心功能不全者13例;对照组(15例)为单纯心脏疾病引起左心功能不全者.同时进行动脉血气分析、静态肺功能以及超声心动图检查.结果 在肺部疾病患者中,合并左心功能不全者血浆NT-proBNP水平明显高于未合并左心功能不全者(P＝0.01);而与对照组相比差异无统计学意义.血浆NT-proBNP水平与右心室前后径、右心室流出道、估测肺动脉压力及左室舒张、收缩末期内径呈正相关;与射血分数呈显著负相关.NT-proBNP对于是否合并左心室功能不全的ROC曲线下面积为0.720;用于诊断肺部疾病合并肺动脉高压的ROC曲线下面积为0.719.采用多因素非条件Logistic回归发现,血浆NT-proBNP水平是影响肺部疾病患者住院期间病死率的主要因素.结论 测定血浆NT-proBNP水平可以作为预测肺部疾病患者是否合并左心功能不全及预后的重要指标.     

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.