An Unusual Variant of Total Anomalous Pulmonary Venous Connection with Varices and Multiple Drainage Sites

Total anomalous pulmonary venous connection (TAPVC) is an uncommon cardiac anomaly that has also rarely been associated with esophageal atresia. We report an unusual case of esophageal atresia with TAPVC with several varices and multiple drainage sites into the superior vena cava and portal vein.     

Total Anomalous Pulmonary Venous Connection in Siblings

ABSTRACT. Three pairs of siblings with total anomalous pulmonary venous connection (TAPVC) are reported. The types of the anomalous venous return (supra- or infracardial connections) varied within the families indicating that genetic regulation deals with the left atrial connection to the intrapulmonary veins. Having failed to establish this connection the intrapulmonary veins can attach themselves to any adjacent venous structure; hence the variety of connections at birth. Our observations also indicate the possibility that specific genetic mechanisms operate in at least some families. Since signs, such as murmurs and cardiomegaly typical of a cardiac anomaly, are not always present in TAPVC, special attention should be paid to any neonatal abnormality in siblings of known cases with TAPVC.     

Total Anomalous Pulmonary Venous Return with the Circular Pulmonary Venous Connection: Outcome of Common Pulmonary Venous Agenesis

A rare case of total anomalous pulmonary venous return, in which the right and left peripheral pulmonary veins connected circularly and there was no central part of the pulmonary vein or the common pulmonary vein, is presented. To our knowledge, total anomalous pulmonary venous return with circular pulmonary venous connection has not been reported previously in the literature. It is thought that the complex connection between peripheral pulmonary veins with the absence of the central part of the pulmonary vein as well as the common pulmonary vein results from common pulmonary venous agenesis.     

Adverse Hemodynamic Effects Observed with Inhaled Nitric Oxide After Surgical Repair of Total Anomalous Pulmonary Venous Return

The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.     

Pulmonary Veins in Total Anomalous Pulmonary Venous Connection with Obstruction: Demonstration Using Silicone Rubber Casts

We studied the postmortem vascular anatomy of four patients with total anomalous pulmonary venous connection (TAPVC) with obstruction using a silicone rubber casting method. Two patients were studied clinically but died before therapeutic intervention, one died after corrective surgery, and another was not diagnosed before death. The morphologic types were supracardiac (2), infracardiac (1), and mixed (1). One of the supracardiac forms had a stenosis at the left vertical vein by a bronchoarterial vise, and a collateral channel to the right superior caval vein was also present. In the other supracardiac case, a left upper pulmonary vein was connected to the left vertical vein above the stenotic portion, resulting in postoperative pulmonary hemorrhage into the left upper lobe. The patient with infracardiac TAPVC had a tree-shaped descending vertical vein and small opening at the inferior caval vein. In the mixed type, a right upper pulmonary vein ran between the pulmonary artery and bronchus draining into the superior caval vein, and the rest of the drainage was to the portal vein.     

Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection in an Adult

Adult survival in patients with uncorrected obstructed infracardiac total anomalous pulmonary venous drainage has not been reported. A 17-year-old man who presented to us with features of severe pulmonary arterial hypertension was diagnosed to have obstructed pulmonary venous drainage to the hepatic vein. Presence of a large ventricular septal defect in this patient may have contributed favorably to survival.     

混合型完全性肺静脉异位连接17例的诊断与治疗

目的探讨儿童混合型完全性肺静脉异位连接(TAPVC)的诊治要点及影响预后的主要因素。方法近5 a来,在广东省心血管研究所确诊为混合型TAPVC的患儿共17例,收集该17例患儿的临床资料和随访结果,进行回顾性分析。结果 17例混合型TAPVC患儿中心上型+心内型14例,心上型+心下型3例,并肺静脉回流梗阻6例。17例术前全部行超声心动图检查,其中13例行多排螺旋CT扫描,14例行手术治疗。行CT扫描者,10例经手术探查证实此10例患儿CT能够准确显示所有肺静脉回流径路。行超声检查者,12例超声报告了所有4条肺静脉回流径路,5例超声未能报告肺静脉全部回流径路。超声报告所有肺静脉回流径路者,经CT或手术探查,证实其中7例超声显示的所有肺静脉回流径路均准确,4例超声准确显示了3条肺静脉回流径路,1例超声显示的肺静脉回流径路完全错误。并肺静脉回流梗阻的6例患儿,超声心动图发现6例,而CT仅发现1例。14例行手术治疗者,急诊手术6例,择期手术8例。围术期死亡2例,均为急诊手术者。12例康复出院者,随访时间2～53(28.8±17.4)个月,中位随访时间30个月。术后3例发生吻合口狭窄,其中1例术后2个月死于严重吻合口狭窄导致的急性肺水肿。结论混合型TAPVC以心上型+心内型最多见。结合超声心动图和多排螺旋CT扫描是术前评估的最佳策略。对肺静脉回流径路的显示多排螺旋CT优于超声心动图,对肺静脉回流梗阻的显示超声心动图优于多排螺旋CT。急诊手术可能是围术期死亡的危险因素。术后随访主要并发症为吻合口狭窄。     

Horseshoe Lung Associated with Cardiac-Type Total Anomalous Pulmonary Venous Return in a Newborn

Horseshoe lung is a rare pulmonary anomaly characterized by fusion of the posterobasal portions of the right and left lung behind the heart anterior to the aorta and the spinal column. This anomaly is almost always associated with lung hypoplasia. The case of a newborn who had horseshoe lung with left lung hypoplasia and cardiac-type total anomalous pulmonary venous return is reported.     

Identifying Congenital Heart Disease in the Emergency Department: A Case of Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return is a relatively rare lesion, accounting for 1% to 3% of all cases of congenital heart disease. Most patients develop symptoms within the first year of life demonstrated by tachypnea, feeding difficulties, and frequent respiratory tract infections. Patients with pulmonary venous obstruction may present urgently within the first few days of life with tachypnea, cyanosis, and heart failure. Prompt identification of congenital heart disease, medical resuscitation, and referral for surgical correction can provide for a good outcome.     

Successful Surgical Repair of Common Pulmonary Vein Atresia in a Newborn

A 7-hour-old boy underwent an emergency operation with an anticipated diagnosis of total anomalous pulmonary venous connection. The precise diagnosis of common pulmonary vein atresia (CPVA) was made during the operation. A side-to-side anastomosis between the common pulmonary venous chamber and the left atrium was performed. All procedures were successfully carried out on the beating heart under the cardiopulmonary bypass. A tentative diagnosis of CPVA should always be borne in minds in neonates with clinical conditions such as deep cyanosis unresponsive to the oxygen therapy, stubborn acidosis, severe pulmonary venous congestion, and rapid deterioration. The corrective repair of CPVA with the heart beating appears to be the procedure of choice in the setting of seriously damaged myocardium of the immature heart. To the best of our knowledge, this is the youngest survivor of the corrective surgery for CPVA and operation at time of diagnosis is the important principle.     

Pulmonary Arteriovenous Malformation in Patients with Total Cavopulmonary Shunt: What Role Does Lack of Hepatic Venous Blood Flow to the Lungs Play?

We examined the etiology of a long-term postoperative complication, pulmonary arteriovenous malformation (PAVM), in a patient who underwent total cavopulmonary shunt (TCPS). The patient underwent TCPS at the age of 3 years and 1 month, and a PAVM developed. At the age of 10 years, TCPS was switched to total cavopulmonary connection to facilitate circulation of hepatic venous blood to the pulmonary artery. As a result, oxygen saturation increased. These findings suggest that the absence of pulmonary circulation of hepatic venous blood is involved in the development of PAVM.     

Common Pulmonary Vein Atresia: A Diagnostic and Therapeutic Challenge

Following Doppler echocardiographic evaluation, a 16 hour-old infant underwent successful surgical repair of common pulmonary vein atresia. Investigations for prolonged postoperative ventilatory assistance, including cardiac catheterization and computerized tomography, led to a clinical diagnosis of associated pulmonary lymphangiectasia. Although this has caused continuing tachypnoea, at 16 months of age, he remains otherwise well but with an uncertain prognosis.     

Tetralogy of Fallot with Left Heart Hypoplasia, Total Anomalous Pulmonary Venous Return, and Right Lung Hypoplasia: Role of Magnetic Resonance Imaging

We report a rare case of tetralogy of Fallot with total anomalous pulmonary venous return, left heart hypoplasia, right lung hypoplasia, and left ocular–mandibular synchinesia (Marcus-Gunn phenomenon), correctly diagnosed by cardiovascular magnetic resonance imaging and successfully operated by modified Glenn anastomosis.     

A Case of Anomalous Origin of the Pulmonary Arteries: Right Pulmonary Artery from the Descending Aorta and the Left Pulmonary Artery from the Ascending Aorta

A 3-month-old girl classified as having persistant truncus arteriosus underwent surgical correction of the anomalous origin of the pulmonary arteries; the right pulmonary artery from the descending aorta and the left pulmonary artery from the ascending aorta. The patient died on the fourth postoperative day. The definite diagnosis and choice of surgical strategies should be further examined.     

Subclavian Steal Syndrome in Anomalous Connection of the Left Subclavian Artery to the Pulmonary Artery in D-Transposition of the Great Arteries

Several reports have documented the occurrence of an isolated left subclavian artery in association with both tetralogy of Fallot and double-outlet right ventricle. In certain cases a congenital subclavian or pulmonary artery steal syndrome exists in which the left subclavian artery is connected to the main pulmonary artery via a ductus arteriosus. We describe a subclavian steal syndrome secondary to anomalous origin of the left subclavian artery from the pulmonary artery in d-transposition of the great arteries in a patient with Spondylocostal dysostosis (SCD). Cardiac anomalies are rare in SCD and this constellation of findings have not previously been described.     

Coarctation of the Left Pulmonary Artery: Effects on the Pulmonary Vasculature of Infants

At autopsy, two infants had unsuspected coarctation of the left pulmonary artery (CoLPA), which was produced by an extension of ductal tissue into the wall of the left pulmonary artery. The first case, a 4-month-old girl, also had a ventricular septal defect and an anomalous branching pattern of the innominate arterial trunk. Pulmonary arterial hypertensive changes were noted in the right lung. In contrast, the left lung showed thin-walled pulmonary arteries. The second case, a term female newborn, had exhibited severe unexplained respiratory distress since birth. Histologic sections of the right lung showed dilated pulmonary arteries with thinned media, whereas the left lung showed a persistent fetal arterial pattern. It is believed that the peripheral pulmonary arterial changes are age-dependent and associated with asymmetric blood flow between the right and left pulmonary arteries. CoLPA is a rare pulmonary artery defect, and early diagnosis of this abnormality is important.     

Partial Anomalous Left Pulmonary Artery: New Evidence on the Development of the Pulmonary Artery Sling

Clinical and angiographic data of a child with a unique form of partial anomalous left pulmonary artery are reported. Because the anomalous pulmonary artery does not run posterior to the trachea, this malformation is not associated with airway obstruction, as are all other forms of anomalous left pulmonary artery described to date. This case strengthens our understanding of the development of the pulmonary artery sling.     

Anomalous Origin of One Pulmonary Artery from the Innominate Artery: A Report of Two Cases

Two children with an anomalous origin of one pulmonary artery from the innominate artery are reported. One was a 15-month-old boy presenting with respiratory distress. He had a right aortic arch and his left pulmonary artery originated from the innominate artery. The other was a 1-month-old girl presenting with congestive heart failure. She had a left aortic arch and her right pulmonary artery originated from the innominate artery. An understanding of the embryological pathogenesis of these anomalies has significant therapeutic implications.     

Left-Side Pulmonary Vein Obstruction After Arterial Switch Operation in Infants with D-Transposition of the Great Arteries

We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.