Double total anomalous pulmonary venous connection

We present a newborn infant with right atrial isomerism, complex congenital heart malformation and anomalous pulmonary venous connection, reliably diagnosed by 2-D Doppler color echocardiography. The infant had no significant obstetric antecedents. The neonatal clinical picture included cyanosis, heart murmur and respiratory distress. The infant was treated with prostaglandin from the age of 24 hours until his death after surgery. The 2-D echo Doppler color flow mapping showed findings that suggested right atrial isomerism, severe left ventricular hypoplasia, pulmonary atresia and ductus arteriosus. The pulmonary veins flowed together to a posterior cardiac chamber from which an emissary vertical venous vessel connected with a left superior aneurysmal sack. Two venous channels emerged from this sack: one connecting to the innominate vein and the other to the atrium. The malformations were confirmed by cardiac catheterization. On the sixth day of life, the patient underwent anastomosis between the posterior venous chamber with the atrium, a modified Blalock-Taussig shunt implant, and ductus closure but died during surgery. The association between complex cardiac anomalies and uncommon obstructive total anomalous pulmonary venous connection in the context of right atrial isomerism is lethal and few neonates survive surgical repair. Two-dimensional echo color flow Doppler is a reliable diagnostic technique and an indispensable guide in angiography.     

Morphological Features of the Levoatriocardinal (or Pulmonary-to-Systemic Collateral) Vein

Three cases with an anomalous pulmonary-to-systemic collateral vein (levoatriocardinal vein) connecting the left atrium or one of the pulmonary veins to a systemic vein are described. In two of these cases the atrial septum is intact, the left atrioventricular connection is absent (mitral atresia), and the anomalous vein is the escape channel for pulmonary venous return. In the remaining case, a muscular membrane divides the left atrium, separating the pulmonary venous component from the distal component. The collateral vein may be mistaken for the vertical vein that is associated with totally anomalous pulmonary venous connections, but in all our cases the pulmonary veins inserted normally into the left atrium. Cross-sectional echocardiography, including conventional and color flow Doppler mapping, should overcome potential difficulties in diagnosis.     

Total anomalous infra-cardiac pulmonary venous connection. Value of the color Doppler echocardiography

A case of total anomalous pulmonary venous connection is described in a newborn. The pulmonary veins join a common trunk entering portal vein below the diaphragm. The pattern of pulmonary venous connections was identified by two-dimensional Doppler color flow-mapping echocardiography. The common pulmonary venous trunk was anastomosed to the left atrium at the age of 3 weeks but the infant died because of pulmonary hypertension and acute renal insufficiency. The Doppler color echocardiography allows a good preoperative evaluation. The spontaneous prognosis being poor, surgical correction is indicated and should be performed early, even for critically ill neonates. Patients who survive to childhood lead active and full lives.     

Resolution of Acquired Pulmonary Arteriovenous Malformations in a Patient with Total Anomalous Systemic Venous Return

An 11-year-old male with total anomalous systemic venous return had surgical repair except for the hepatic venous return, which drained to the left atrium. He developed progressive cyanosis and fatigue and was diagnosed with large pulmonary arteriovenous malformations (PAVMs) during cardiac catheterization with the use of bubble contrast echocardiography. After surgical redirection of hepatic venous flow to the right heart and pulmonary arterial system, resolution of these PAVMs was demonstrated clinically and by contrast echocardiography. This unique case report demonstrates the development of PAVMs with exclusion of hepatic venous return through the pulmonary vascular bed while pulsatile pulmonary blood flow remains intact. It reinforces the likelihood of the absence of an as yet unidentified hepatic vasoactive substance as the source for development of PAVMs.     

Functional total anomalous pulmonary venous connection via levoatriocardinal vein

We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the “dead‐end” left atrium, the pulmonary venous flow had a to‐and‐fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left‐side heart obstruction.     

Outcome in neonates with congenital heart disease referred for respiratory extracorporeal membrane oxygenation

AIM: To evaluate the proportion of neonates referred for extracorporeal membrane oxygenation (ECMO) support in the modern era of advanced conventional treatments for respiratory failure who actually had congenital heart disease (CHD), and to assess the impact of this diagnostic route on patient condition and outcome. METHODS: A retrospective case-note review of neonatal ECMO and cardiac admissions to a single, tertiary ECMO and cardiac intensive care unit (ICU) between March 1999 and February 2002. RESULTS: 287 symptomatic neonates presented to the ICU with previously undiagnosed cardiac or respiratory disease. Eighty-two with presumed respiratory failure were referred for ECMO, and 205 with suspected CHD were referred for cardiac evaluation. Eight (10%) ECMO referrals, all with presumed persistent pulmonary hypertension of the newborn (PPHN), were found to have CHD (transposition: 3; total anomalous pulmonary venous connection: 3; left heart obstructive lesions: 2). Mortality in this group was 50%, compared with 11% for correctly identified CHD patients (odds ratio 8.2, 95% CI 1.92, 35.4, p<0.01). For all neonates with CHD, the risk of death was increased by the presence of cardiovascular collapse and end-organ dysfunction at presentation to the ICU (p<0.01 for both). CONCLUSION: Neonates with CHD may present as severe "PPHN" via the ECMO service. Poor outcome in these patients relates to the high incidence of cardiovascular collapse and end-organ dysfunction. Early echocardiography is recommended for neonates with presumed PPHN. Neonatal ECMO support should be based in centres with cardiac surgical services.     

Cor triatriatum masked by primary pulmonary hypertension

Summary In a 17-month-old boy with clinical signs of right heart failure, the diagnosis of primary pulmonary hypertension was made, based on cardiac catheterization findings with high pulmonary arterial pressure and normal pulmonary wedge pressure although two-dimensional echocardiography demonstrated a left atrial membrane. Postmortem examination confirmed a left atrial membrane (cor triatriatum), and the microscopic examination revealed the histological pattern of primary pulmonary hypertension. Due to reduced pulmonary blood flow, pulmonary venous obstruction was masked and could not be assessed by measuring pulmonary wedge pressure.     

Cyanosis in a 9-month-old child after repair of total anomalous pulmonary venous return

A 9-month-old girl presented with cyanosis after correction of total anomalous pulmonary venous return (TAPVR) to the coronary sinus in the neonatal period. During corrective surgery, the right superior vena cava (RSVC) was damaged and re-anastomosed to the right atrium. Echocardiography showed increased flow velocity in the pulmonary venous confluence. Therefore, pulmonary venous obstruction was suspected. However, subsequent cardiac MRI revealed a stenosed RSVC as well as a dilated left superior vena cava (LSVC) draining from the left innominate vein into the pulmonary venous confluence. The re-recruited LSVC drained deoxygenated blood into the systemic circulation, causing cyanosis. After uncomplicated placement of a stent in the narrowed RSVC and occlusion of the LSVC, transcutaneous saturations normalised immediately.     

Acute pulmonary edema in a newborn with infracardiac type total anomalous pulmonary venous return and surgical repair

Total anomalous pulmonary venous return (TAPVR) is a rare congenital pathology. Early diagnosis and urgent surgery are life-saving, especially in newborns with pulmonary venous obstruction, which is most commonly seen with infracardiac type. A three-day-old baby boy presented to another clinic with tachypnea and cyanosis. Initial work-up aimed at ruling out persistant pulmonary hypertension, respiratory distress syndrome and pneumonia. Acute pulmonary edema then developed, and on echocardiography obstructive type infracardiac TAPVR was suspected. Cardiac catheterization was done for definitive diagnosis. Urgent surgery was undertaken and pulmonary veins were anastomozed to left atrium with posterior approach. Patient was extubated at 10th day and discharged after three weeks. During one-year follow-up the patient was free of symptoms. Infracardiac type TAPVR is a rare pathology in which early diagnosis and urgent surgery with special postoperative case are mandatory for survival.     

Transcatheter Management of Subtotal Cor Triatriatum Sinister: A Rare Anomaly

Subtotal cor triatriatum sinister (partially divided left atrium) is characterized by a membrane dividing the left atrium into two portions: a dorsal accessory proximal chamber that receives part of the pulmonary venous return and a small restrictive defect in the membrane that obstructs blood flow from the accessory chamber to the left atrium and results in unilateral pulmonary venous hypertension. This clinical entity is very rarely reported in the literature. This first report of successful transcatheter management of a subdivided left atrium obstructing right pulmonary venous drainage discusses the anatomic, morphologic, and procedural details.     

Anomalous Origin of Left Pulmonary Artery from the Aorta with Partial Anomalous Pulmonary Venous Return

Anomalous origin of left pulmonary artery (AOLPA) from the aorta is a rare congenital cardiac malformation. We report a case of AOLPA from the aorta with partial anomalous pulmonary venous return (PAPVR) in a 3.2 kg infant. Surgical correction was performed with direct anastomosis of AOLPA to the main pulmonary artery with subsequent baffling of the anomalous right pulmonary veins to the left atrium and patch augmentation of the left pulmonary artery performed at 1-month of life. Recurrent stenosis of the proximal left pulmonary artery was treated with stent placement at 17-months. Pulmonary hypertension has persisted at 30-month follow up.     

Cor triatriatum associated with partial anomalous pulmonary venous connection to the coronary sinus: Echocardiographic and angiocardiographic features

Summary An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography. Cardiac catheterization and angiography confirmed the findings and also demonstrated a persistent left superior vena cava draining to the coronary sinus. The infant underwent successful surgical repair. Partial anomalous pulmonary venous connection and left superior vena cava not infrequently are associated with cor triatriatum. Although two-dimensional echocardiography is sensitive for the detection of cor triatriatum, preoperative cardiac catheterization is necessary to identify unequivocally systemic and pulmonary venous connections.     

Echocardiography diagnosis of total anomalous pulmonary venous drainage in the neonatal period

A 20-days-old female newborn was referred to our hospital because of persistent respiratory distress and a mild central cyanosis. On echocardiography from the suprasternal notch all 4 pulmonary veins could be visualized entering a common chamber posterior to the left atrium. This chamber communicated with a left vertical vein which drained through a large innominate vein into the right superior vena cava. The diagnosis of supracardiac type of TAPVD was confirmed the following day by angiography. The child was successfully operated and the postoperative course was uneventful. Cardiac catheterization carries an increased risk in patients with TAPVD in case of pulmonary hypertension due to pulmonary-venous obstruction. In experienced centers echocardiography has become the definite diagnostic method with a high level of sensitivity and specificity in patients with atrial sitlus solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defects. With continuously improving knowledge an increasing number of newborns will benefit from improved ability to perform surgical repair without prior cardiac catheterization.     

Coexistence of Three Rare Congenital Heart Defects in a Single Patient

The unique anatomy, physiology, and surgical repair of a patient with features of three rare congenital heart defects (total anomalous pulmonary venous return to the coronary sinus, cor triatriatum, and unroofed coronary sinus with persistent left superior vena cava to the left atrium) is described. Analysis of this case suggests that these three conditions are linked in that they all may result as a consequence of a defect in the same embryologic process (i.e., incorporation of the pulmonary venous confluence into the left atrium).     

Ductus venosus blood velocity in persistent pulmonary hypertension of the newborn.

AIMS: To investigate the ductus venosus flow velocity (DVFV) in infants with persistent pulmonary hypertension of the newborn (PPHN); to evaluate the DVFV pattern as a possible diagnostic supplement in neonates with PPHN and other conditions with increased right atrial pressure. METHODS: DVFV was studied in 16 neonates with PPHN on days 1-4 of postnatal life using Doppler echocardiography. DVFV was compared with that in mechanically ventilated neonates with increased intrathoracic pressure, but without signs of PPHN (n=11); with neonates with congenital heart defects resulting in right atrial pressure (n=6); and with preterm neonates without PPHN (n=46); and healthy term neonates (n=50). RESULTS: Infants with PPHN and congenital heart defects with increased right atrial pressure were regularly associated with an increased pulsatile pattern and a reversed flow velocity in ductus venosus during atrial contraction. A few short instances of reversed velocity were also noted in normal neonates before the circulation had settled during the first day after birth. CONCLUSIONS: A reversed velocity in the ductus venosus during atrial contraction at this time signifies that central venous pressure exceeds portal pressure. This negative velocity deflection is easily recognised during Doppler examination and can be recommended for diagnosing increased right atrial pressure and PPHN.     

心上径路治疗新生儿和小婴儿完全性心上型肺静脉异位引流

目的 探讨心上径路治疗新生儿和小婴儿完全性心上型肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的临床效果.方法 2006年6月至2009年6月,采用心上径路矫治新生儿和小婴儿心上型TAPVC 26例,其中男17例,女9例.年龄18 d～8个月,平均(4.2±2.8)个月.体重3.4～8.1 kg,平均(5.8±2.1)kg.全部患儿经心上径路完成矫治手术.总转流时间49～98 min,平均(68.7±15.3)min;主动脉阻断时间31～77 min,平均(47.6±14.7)min.结果 术后早期无死亡;交界区心律或心律紊乱5例(19.1%);中期随访23例,时间5个月～3年,平均18个月,无死亡.心功能Ⅰ级21例,Ⅱ级2例,全部为窦性心律;复查超声心动图示吻合口通畅22例,轻度狭窄1例.结论 采用心上径路治疗新生儿和小婴儿完全性心上型肺静脉异位引流临床效果满意.

Abstract：

Objective To evaluate the short term outcome of surgical treatment of supracardiac total anomalous pulmonary venous connection (TAPVC) via superior approach in neonates and infants. Methods From June 2006 to June 2009, 26 patients with supracardiac TAPVC underwent surgical repair through superior approach. There were 17 males and 9 females. Their ages ranged from 18 days to 8 months old (mean, 4. 2 ± 2. 8 years). The weight ranged from 3.4 to 8. 1 kg (mean, 5. 8 ± 2. 1 kg). Before surgery, all patients had apnea and cyanosis at resting state, and their resting SpO2 ranged from 65% to 85%. The heart/chest ratio calculated on X-ray film was from 0. 62 to 0. 77. Electrocardiograph (ECG) of the patients showed sinus rhythm, right atrium enlargement and right ventricle hypertrophy. Echocardiography revealed moderate to severe pulmonary hypertension and various degree of tricuspid reflux Under general anesthesia and cardiopulmonary bypass, direct anastomosis between the top of the left atrium and the common pulmonary venous trunk were performed via superior approach. Results The cardiopulmonary bypass time was 68. 7 ± 15. 3 min and the aortic cross-clamping time was 47. 6 ± 14. 7 min. The patients were followed up for 5 months to 3 years (mean, 18 months). No death after surgery was noted. Five patients had atrial arrhythmia (19. 1%)in the early postoperative stage. One patient had mild anastomotic stenosis, Two neonates had delayed sternum closure. Two patients had diaphragm paralysis, one of which underwent diaphragm placation.All of the patients except 2 had satisfactory cardiac function. Conclusions Correction of supracardiac total anomalous pulmonary venous connection via superior approach is safe and effective in neonates and infants.     

单纯性完全型肺静脉异位引流产前8例超声心动图诊断

摘要 目的 探讨胎儿单纯性完全型肺静脉异位引流（TAPVC）的产前超声心动图特点,提高对本病的产前诊断准确率。方法 回顾性分析2011年5月至2014年2月经新生儿超声心动图、手术或尸解证实的8例单纯性TAPVC的胎儿期超声心动图检查结果,总结超声心动图特征。结果 8例单纯性TAPVC胎儿中,心下型2例,心内型1例,心上型5例,5例存在垂直静脉或共同肺静脉腔与垂直静脉连接处梗阻;8例胎儿中,引产1例（心下型）,余7例均于出生后行手术治疗,其中1例术后死亡（心下型）,6例均恢复良好。TAPVC胎儿超声心动图特征为：①正常的左心房形态消失,呈圆形或椭圆形,左心房光滑并多变小,在孕后期较为明显;②降主动脉与左心房间距离明显增大,多数在左心房后方可见一异常的腔隙（即共同肺静脉腔）,可显示左、右侧上升（心上型）或下降（心下型）的垂直静脉;③妊娠早期左、右心系统比值早期多正常,但妊娠中、晚期（孕26周后）可出现右心系统轻度扩大;④引流入冠状静脉窦时（心内型）,冠状静脉窦可有扩张;引流入上腔静脉时（心上型）,上腔静脉扩张;引流入肝内血管时（心下型）,肝内血管可有不同程度的扩张;⑤彩色多普勒可显示引流途径及是否合并垂直静脉梗阻。结论 在孕早期左、右心系统比值正常时,TAPVC易被漏诊及误诊,应注意多角度、多切面扫查,孕晚期超声心动图检查可减少漏诊。     

Unusual diagnosis of partial left abnormal pulmonary venous return in neonate]

The authors report on a case of a newborn with partial anomalous pulmonary venous return of the lower left pulmonary vein. Diagnosis was performed during the introduction of a central venous catheter. The complications of partial anomalous pulmonary venous rely principally in the risk of endothelium damage and subsequent pulmonary arterial hypertension.     

超声心动图对儿童完全性肺静脉异位引流诊断价值的探讨

目的　旨在评价超声心动图对完全性肺静脉异位引流 (TAPVD)的诊断价值。方法　对 1987年 9月至2 0 0 2年 11月收治的TAPVD患儿 70例进行分析。均有完整的临床及超声心动图资料 ,其中 35例施行了手术治疗。结果　 70例TAPVD的解剖分型为 :心内型 5 2例、心上型 16例、心下型 2例 ,未发现混合型。 35例施行手术 ,32例与术前超声心动图的诊断一致 ;3例与超声心动图诊断不完全一致 ,其超声心动图诊断分别为 :三房心伴继发孔房缺、左房隔膜伴继发孔房缺、原发孔房缺伴卵圆孔未闭 ,而手术结果均显示为心内型TAPVD伴继发孔房缺。结论　超声心动图检查是诊断TAPVD重要且准确的方法 ,并能确定其分型 ,提供精确的解剖及血流动力学资料。在应用超声心动图诊断TAPVD时 ,应注意与三房心、左房隔膜等疾病进行鉴别。     

Nitric oxide in the treatment of neonatal pulmonary hypertension

This study describes the effects of nitric oxide in newborns with persistent pulmonary hypertension. We studied 9 infants with severe respiratory failure characterized by hypoxemia and pulmonary hypertension. All infants met ECMO criteria and the oxygenation index (OI) was greater than 25. Mean birth weight was 2698 -/+ 661 g and gestational age was 36.4 -/+ 2.6 weeks. Nitric oxide was administered in a Y circuit in the inspiratory line of the mechanical ventilator. Nitric oxide and NO2 concentrations were monitored with electrochemical analyzers (PACI and PACII-Draeger). Pulmonary hypertension was diagnosed with clinical and echocardiografic criteria, with detection of right to left shunt with color doppler. All patients showed a dramatic improvement in oxigenation after nitric oxide administration. The drug reduced the mean OI, which was 48.5 before its administration, to 17.7 after 30', 14.1 after 6 hours, and 10.5 after 12 hours. We observed in all patients a reduction in pulmonary vascular resistance, reversal of the right to left shunt without any effects on systemic arterial pressure. Metahemoglobin levels did not reach 1.5% in any patient. Only one out of the 9 patients died, after reversal of the pulmonary hypertension, from other complications of perinatal asphyxia. Our data show that nitric oxide is a promising drug in the treatment of neonatal pulmonary hypertension and that it may reduce the need of ECMO in severe respiratory failure.