Successful transcatheter occlusion of an anomalous pulmonary vein with dual drainage to the left atrium

We describe a case of a scimitar syndrome “variant” where dual drainage existed from the right upper and middle pulmonary veins to the inferior vena cava and left atrium. Device closure of the anomalous vein at the level of the connection to the IVC was successful in achieving diversion of pulmonary venous flow to the left atrium. Vigilance during work‐up of anomalous pulmonary venous drainage (whether isolated or associated with other cardiac defects that may be amenable to device closure) is important to define the presence of dual connections to the left atrium, in which case a less‐invasive transcatheter approach may be feasible. © 2015 Wiley Periodicals, Inc.     

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging,pitfalls, and caveats

Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium. Herein, we report an 8-year-old boy with anomalous IVC and hepatic vein drainage into the LA due to IVC-IAC malalignment. We describe the findings of multimodality imaging, including transthoracic, transesophageal, contrast, and speckle-tracking echocardiography, cardiac angiography, and cardiac magnetic resonance imaging. We go over the diagnostic and therapeutic pitfalls and caveats of this case that can apply to similar patients.     

Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.     

Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium

Background

A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium.

Methods

We retrospectively studied such patients in 5 institutions.

Results

Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056).

Conclusion

In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients.     

Echocardiographic Appearances of Direct Drainage of Hepatic Vein into Right Atrium in Adults

Anomalous hepatic venous drainage directly into the right atrium is known to occur in infants or children with atrial isomerism, abdominal heterotaxy, and absence of the inferior vena cava (IVC), but we could find no previous reports of partial anomalous hepatic venous drainage in adults, in the echocardiographic literature. We describe two adults in whom the two-dimensional and color flow Doppler findings indicated drainage of a hepatic vein directly into the right atrium. I n both, a normal IVC entered the right atrium at its normal location, and a unique abnormal tongue like structure—an "extra Eustachian valve"—was attached to the right atrial wall near the entry of the anomalous directly draining hepatic vein. We speculate that the hepatic venous anomaly and the "extra Eustachian valve" both result from aberrant development of the embryonic sinus venosus.     

Masked abnormal drainage of the inferior vena cava into the left atrium

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Patterns of anomalous pulmonary venous drainage

All of our cases of abnormal pulmonary venous connections collected to the middle of 1965 and verified at surgery or autopsy have been reviewed by means of diagrams and tabulations, using a specially devised code to facilitate the survey. The material consisted of 52 autopsy cases (half of them obtained after surgery) and the cases of 72 patients who survived operation. The postmortem group was much younger than the surgical group and differed also from the latter by showing male preponderance as well as relatively many instances of total abnormal pulmonary venous connection and frequently associated cardiac anomalies. Partial anomalous connection of right pulmonary veins was 10 times more frequent than that of the left pulmonary veins. This was caused by (1) the frequent drainage of some of the right pulmonary veins into the junctional area between right atrium and superior vena cava in the presence of normal left pulmonary veins, and (2) the complete absence of isolated left pulmonary venous connection to the right atrium. Abnormal connection of solitary pulmonary veins was always effected to the most proximal venous structure among the four possible ones which are derived from the main embryonic channels (superior vena cava and inferior vena cava on the right side, and left superior vena cava and coronary sinus on the left side). Common pulmonary veins from one lung also drained in accordance with this proximity rule, if this may be taken to apply also to the drainage of right pulmonary veins into the right atrium. The one exception in our material was the drainage of all right pulmonary veins into the portal venous system. Total abnormal pulmonary venous connection may be found with all structures mentioned, but most frequently with the left superior vena cava, or coronary sinus, or both, usually by way of a common pulmonary vein. In a few cases however, drainage into different sites, all of them abnormal, did occur. Then again the proximity rule seemed to apply. A tentative embryological explanation is given for the patterns described.     

Congenitally corrected transposition of the great arteries with anomalous inferior vena cava drainage: multimodality imaging

Atrioventricular discordance with ventricular-arterial discordance is a rare cardiac anomaly known as congenitally corrected transposition of the great arteries (CCTGA). This malformation has a prevalence of 0.4–0.6% of all congenital heart disease cases. Complete heart block develops in up to 30% of patients with CCTGA. We present the case of a 62-year-old woman diagnosed with CCTGA who, on echocardiography, had anomalous venous drainage where the inferior vena cava (IVC) bypassed the right atrium and drained into the azygos system. Complementary images with magnetic resonance imaging demonstrated the unique anatomical relationship between the IVC, azygos venous system, and the superior vena cava. (Echocardiography 2012;29:E16-E19)     

Pulmonary arteriovenous fistula drainage into the left atrium

Arteriovenous fistulas with venous drainage into the left atrium are a rare anomaly. Although the etiology of pulmonary arteriovenous fistulas is unknown, these abnormalities are considered to have occurred during early fetal development. A case of this malformation in a 72-year-old woman successfully treated by surgery is described.     

Subdivided left atrium: an expanded concept of cor triatriatum sinistrum.

Twenty-four hearts with cor triatriatum were studied. On the basis of this material and a review of the literature a new classification of "subdivided left atrium" is proposed. Tye A, the most common form of subdivided left atrium, is the classic cor triatriatum with its multiple variations of partial anomalous pulmonary venous drainage; the fossa ovalis can be related to the proximal left atrial chamber (type A, a) or the distal left atrial chamber (type A, b). Type B hearts are related to (but not identical with) total anomalous pulmonary venous drainage into the coronary sinus; the coronary sinus opening is atretic in these hearts; abnormal defects connect the proximal left atrial chamber usually with the right atrium only, rarely also with the distal left atrial chamber. The Type C heart, first reported in this paper, has a superiorly and medially situated proximal chamber, located between the right and distal left atrium; it does not receive any pulmonary veins; the coronary sinus is normally formed. Current morphogenetic hypotheses that satisfactorily explain the Type A b and B heart fail to account for the Type A a and C heart.     

A case of abnormal venous drainage of the whole left lung into the left innominate venous trunk

In this case, the drainage of all the left lung veins of gathered in a common trunk into the left innominate venous trunk was combined with an atrial septal defect and partition of the left atrium. The rarity of such cases in the literature is due to the fact that it is an uncommon anatomical anomaly and that very often it is symptomless, and therefore does not induce the subjects with this anomaly to seek medical advice. In the case reported, surgical treatment consisted in closing the atrial septal defect, resecting the abnormal left atrial partition and in re-establishing the drainage of the left lung veins into the left atrium through a wide latero-lateral anastomosis with ligature of the abnormal left pulmonary venous trunk as its junction with the left innominate venous trunk.     

Anomalous drainage of the inferior caval vein to the left atrium

We report a girl, aged 11(7/12) years, who presented with cyanosis. Cardiac catheterization showed occlusion of the infrahepatic segment of the inferior caval vein, with drainage of the hepatic veins into the left atrium. Transoesophageal echocardiography revealed an anomalous Eustachian valve that baffled the vein to the left atrium. This lesion is an extremely rare cause of cyanosis.     

Scimitar Syndrome with Right Hemianomalous Pulmonary Venous Drainage into Superior Vena Cava/Right Atrium Junction

Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise classical scimitar syndrome with anomalous drainage of the right lung cephalad into the right atrium near its junction with the superior vena cava.     

Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium.

A 4-month-old infant with cyanosis but without other abnormal cardiac findings is presented in whom the diagnosis of anomalous systemic venous connection to the left atrium was made by contrast echocardiography. The diagnosis was later confirmed by cardiac catheterisation and selective cineangiography. When saline was injected into a vein on the dorsum of each hand while echocardiographically recording the cardiac structures, the left atrium, left ventricle, and aorta were opacified without visualisation of the right ventricle. Similar study with injection into the right foot produced opacification of the right ventricle without visualisation of the left-sided structures. These data suggested normal drainage of the inferior vena cava with anomalous connection of the superior vena cava to the left atrium. A review of the previously reported cases of anomalous connection of the right superior vena cava to the left atrium is presented together with the possible embryological origin of this anomaly.     

Recurrent hepatocellular carcinoma with tumor thrombus in right atrium - report of a successful liver resection with tumor thrombectomy using total hepatic vascular exclusion without concomitant cardiopulmonary bypass

For resection of advanced hepatocellular carcinoma (HCC) in which tumor thrombus (TT) extends into inferior vena cava (IVC) or right atrium (RA) surgery is challenging and requires skillful techniques. Here, we report a case of recurrent HCC with TT extending to the RA, who underwent successful resection with tumor thrombectomy without concomitant cardiopulmonary bypass. A 71-year-old man, who had been followed- up for hepatitis C by a local hospital, was diagnosed as having HCC in segment 6 for which he had undergone segmentectomy of segment 6 in May 2009. During follow-up, he developed severe leg edema and ascites with investigations revealing recurrent HCC in segment 7 with TT extending to the right atrium via IVC. After transarterial embolization the patient underwent extended resection of the segment 7 with tumor thrombectomy of the IVC and the right atrium and partial resection of the IVC wall using total hepatic vascular exclusion, without concomitant cardiopulmonary bypass. Total ischemic time was 23 minutes, operation time was 6 hours and blood loss was 2,474mL. The postoperative course was uneventful. Histopathology was recurrent hepatocellular carcinoma with hepatic venous invasion. We report the case of resected recurrent HCC with TT extending to right atrium without concomitant cardiopulmonary bypass.     

A case of diffuse pulmonary arteriovenous fistula.

A 30-year-old Japanese woman was admitted to hospital for dyspnea. She had a history of corrective surgery for a large atrial septal defect and partial anomalous pulmonary venous drainage, which had produced cyanosis in her infancy. However, her cyanosis continued postoperatively. Angiography revealed a double inferior vena cava (IVC), with the left IVC connected with the hemiazygos vein and the right IVC with the left atrium through a very small orifice. Most of the blood from the 2 IVCs flowed into the superior vena cava via the distended azygos and hemiazygos veins. Pulmonary arteriography revealed no abnormal structures. Pulmonary arterial pressure was normal. There was marked pulmonary venous oxygen desaturation. Perfusion lung scintigraphy revealed multiple segmental perfusion defects. These findings suggested the presence of diffuse microscopic pulmonary arteriovenous fistulas bilaterally in the lungs. The patient appears to be the first reported adult case of microscopic and diffuse arteriovenous fistulas. Neither resection of the arteriovenous fistulas nor corrective surgery for the diversion was indicated, and heart-lung transplantation might be the only treatment able to relieve her dyspnea.     

Obstruction of inferior vena caval orifice by giant left atrium in patients with mitral stenosis. A Doppler echocardiographic study from the right parasternal approach.

BACKGROUND. To examine whether an extremely enlarged left atrium (giant left atrium) obstructs the venous return from the inferior vena cava (IVC), the velocity of IVC flow was measured at its junction with the right atrium (IVC orifice) in patients with mitral stenosis by use of color and pulsed-wave Doppler echocardiography from a right parasternal longitudinal plane. METHODS AND RESULTS. The maximum dimension of the IVC orifice by two-dimensional echocardiography and the maximum IVC orifice flow velocity by pulsed-wave Doppler echocardiography were measured in 74 patients with mitral stenosis and atrial fibrillation (mean age, 59 years). The control population consisted of 16 subjects with atrial fibrillation alone (mean age, 61 years). Flow velocities in the superior vena cava and hepatic vein were also obtained by pulsed-wave Doppler echocardiography from the supraclavicular and subcostal views, respectively. Fifty-one mitral stenosis patients without severe tricuspid regurgitation were divided into two groups according to the left atrial dimension (LAD), which was measured by the standard left parasternal long-axis view (group A: n = 33, LAD less than 65 mm; group B: n = 18, LAD greater than or equal to 65 mm). Peak inspiratory and expiratory velocities of IVC orifice flow in diastole averaged over three consecutive inspirations in group B (mean +/- SD, 93.4 +/- 32.0 and 47.6 +/- 19.8 cm/sec) were significantly greater (p less than 0.01) than in the control subjects (67.9 +/- 12.8 and 34.5 +/- 7.0 cm/sec) and in group A (70.2 +/- 18.4 and 38.1 +/- 11.5 cm/sec, respectively). However, there were no significant differences in superior vena caval and hepatic vein flow velocities among the three groups. The maximum IVC orifice dimension in group B (11.4 +/- 4.4 mm) was significantly smaller than in the control subjects (20.1 +/- 2.1 mm) and in group A (18.6 +/- 5.4 mm) because of displacement of the atrial septum into the right atrium. There were significant negative correlations between the IVC orifice dimension and the peak IVC orifice flow velocity (r = -0.62, SEE = 0.33 cm/sec, n = 67, y = e(-0.01x + 3.6), p less than 0.01) as well as the left atrial dimension (r = -0.71, SEE = 0.32 mm, n = 67, y = e(-0.02x + 3.8), p less than 0.01) in these 51 patients and control subjects. In the remaining 23 patients with severe tricuspid regurgitation, the peak inspiratory IVC orifice velocity (n = 9, 88.6 +/- 30.0 cm/sec) was significantly greater (p less than 0.05) and the IVC orifice dimension (23.8 +/- 9.7 mm) significantly smaller (p less than 0.05) in patients with a giant left atrium than in those without (n = 14, 69.9 +/- 15.3 cm/sec and 30.5 +/- 9.6 mm, respectively); in the latter, the IVC orifice dimension was significantly (p less than 0.05) greater than in the controls. CONCLUSIONS. A giant left atrium in patients with mitral stenosis obstructs venous return at the IVC orifice by marked displacement of the atrial septum toward the right atrium.     

Range-gated pulsed Doppler echocardiographic diagnosis of supracardiac total anomalous pulmonary venous drainage

Three patients with total anomalous pulmonary venous drainage (TAPVD) into the innominate vein were studied by using M-mode and pulsed Doppler echocardiography. An abnormal continuous flow towards the transducer in a suprasternal notch position detected by pulsed Doppler echocardiography in the left hemithorax leftward of the aortic echo is a sign of blood flow through the left vertical vein into the innominate vein. This finding is highly specific for TAPVD. High-velocity flow across the tricuspid valve, disturbed flow in the right pulmonary artery and abnormal flow in the left atrium are additional important pulsed Doppler echocardiographic findings in TAPVD. Right ventricular enlargement and paradoxical interventricular septal motion by M-mode echocardiography were not specific enough to distinguish TAPVD from other right ventricular volume overload lesions. An echo-free space posterior to the left atrium was not recorded.     

Correction of anomalous drainage of right superior vena cava to left atrium

A 2-year-old boy with cyanosis was found to have normal situs and looping with anomalous drainage of a right-sided superior vena cava to the left atrium, and intact interatrial septum in association with anomalous drainage of the left pulmonary veins to the right superior vena cava. He underwent successful surgical repair of this rare congenital malformation.     

Novel transcatheter intervention for iatrogenic cyanosis due to surgical rerouting of inferior vena cava to the left atrium

Improper identification of the atrial septal defect margins during surgery and inadvertent suturing of the surgical patch to the Eustachian valve of the inferior vena cava (IVC) results in the diversion of inferior venacaval blood to the left atrium causing cyanosis. This complication has been dealt so far with surgery. We report the planning and implementation of a novel transcatheter rediversion of the IVC to the right atrium using a covered stent.