Redirection of Anomalous Venous Pulmonary Flow to Left Atrium Using a Vascular Plug II

ABSTRACT: An 8-year-old male patient was sent to the catheterization laboratory for assessment of a partial anomalous pulmonary venous connection found by echocardiography. During the intervention, cineangiogram revealed left pulmonary veins draining to the right atrium through left innominate and vertical veins. Despite this anatomical feature, these pulmonary veins were also connected to the left atrium. In a second intervention, we successfully occluded the vertical vein using a vascular closure device (Amplatzer Vascular Plug II, AGA Medical), redirecting pulmonary venous flow towards the left atrium. Anatomy, technique, and management of the procedure are discussed here.

J INVASIVE CARDIOL 2012;24(5):E96-E98

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Right upper pulmonary vein connecting to the right atrium (RA) or the superior vena cava associated with a sinus venous atrial septal defect represent the most common type of partial anomalous pulmonary venous connection (PAPVC). The right pulmonary veins can also connect to the inferior vena cava (IVC) as in Scimitar syndrome.¹

PAPVC of left pulmonary veins usually connects to a vertical vein (VV), or to the coronary sinus and drain to the RA. Less frequently they connect to the left atrium (LA) with a persistent left superior cava vein (PLSCV). A very high rate of this anomalous connection coexists with an ostium secundum type of atrial septal defect. From autopsy series, an incidence of 0.4%-0.7% has been reported, although this could be underestimated as most patients are asymptomatic.^1,2,17

We report a case of PAPVC, in which left upper pulmonary veins were connected to a VV. This system was also draining to the LA by a PLSCV. The patient was successfully treated and percutaneous closure of the VV was performed, virtually splitting systemic from pulmonary venous system and redirecting pulmonary venous blood flow towards the LA.

Case Report. An 8-year-old male patient with 2 episodes of respiratory infection compromising the left lung apical lobe was sent for evaluation to the Pediatric Cardiology Section of our hospital. Auscultation revealed a systolic murmur of low intensity at left second intercostal space. EKG and chest x-ray were normal. Color Doppler echocardiography exam reported a VV with high velocity. Flow direction indicated the presence of PAPVC.

He was sent to the catheterization laboratory for hemodynamic assessment. Right and left heart catheterization was performed as usual. We calculated a pulmonary to systemic flow ratio (Qp/Qs) of 1.3:1. Cineangiogram data confirm left PAPVC to VV, the flow of which was mainly directed to RA through the innominate vein (IV)­. This system was also connected to the LA by a PLSCV. No other congenital heart defects were found.

The case was discussed at our weekly meeting and to prevent further lung infections or an increase of Qp/Qs ratio, we decided to perform a percutaneous closure of the VV to redirect pulmonary venous flow towards LA, as normal.

Under general anesthesia, venous access was achieved by both femoral veins; right femoral vein was selected for delivery of the device and left femoral vein to advance an angiographic Berman’s catheter to the left pulmonary artery. Angiogram of the VV was performed with a marked pigtail catheter and 10 mm was the maximal diameter measured. We selected a 14 mm device (Amplatzer Vascular Plug II, AGA Medical) and a Torq Vue 180° 7 Fr sheath for delivery (AGA Medical), which has a radio-opaque market at the tip and is primarily used to deliver closure devices for patent ductus arteriosus. The design of this sheath with 180° curve was ideal to achieve the desired position. Through femoral vein approach, with a right coronary Judkins catheter and the aid of a 0.035″x 260 cm Wholey floppy wire (Mallinckrodt Inc.) we positioned the catheter in the VV and exchanged the guidewire for an extra stiff Amplatz (Cook Medical Inc.). The delivery sheath was advanced just above the pulmonary vein (Figure 1). Dilator and extra stiff wire were retrieved under sealed water to avoid air embolism considering that all this venous system was connected to the LA. The vascular plug was advanced until the tip of the sheath. Deployment of the device was performed by retracting the sheath over the delivery wire. Levophase angiogram in the left pulmonary artery was recorded to reveal the exact position and permeability of the pulmonary veins. VV was completely occluded, and pulmonary venous blood flow was directed towards LA (Figure 2). With the device still attached, an angiogram through the delivery sheath was performed to rule out obstruction of the IV (Figure 3). After release, manual contrast injection through a venous access of the left superior limb showed no obstruction of the systemic venous flow (Figure 4). Patient was discharged from the hospital without events 24 H after intervention and antiplatelet therapy with aspirin was indicated for 6 months.

Discussion. PAPVC is a rare congenital heart defect. We report an uncommon type of PAPVC in which left pulmonary veins drained to a VV and were also associated with a PLSCV connected to the LA. This allowed us to reconnect or redirect left pulmonary blood flow by blocking the VV with an Amplatzer Vascular Plug II (AGA Medical). This device was created to improve embolization properties of Plug I. It consists of a self-expanding cylindrical device with 3 lobes made of fine nitinol wire mesh, braided in 2 layers in the smaller devices and 3 layers in devices larger than 10 mm. Diameters range from 4 to 22 mm, in 2 mm increments. Lengths range from 6 mm to 18 mm. It contains platinum marker bands at both ends with a microscrew welded on the proximal end to facilitate attachment to a 135 cm long delivery cable. Selection is made according to the diameter of the vessel to be occluded and oversizing by 30% to 50%. Both devices were designed to embolize vascular structures of high flow such as arterial venous fistulae, tumors, etc.^3,7-11,14-16

In addition to the primary use it was also found very useful in congenital heart defects or modifications after surgery.^4-7,12,13 In this field the term “closure” is more accurate than “embolization” to define the procedure. Patent ductus arteriosus,^4,6,7 venous collaterals,^4,5,7 aortopulmonary collaterals,⁴ pulmonary artery venous malformation,^4,8,11 modified Blalock-Taussig shunts,^4,12 and Fontan fenestration^4,13 were closed using Vascular Plug I and II. In our case we “redirected” pulmonary venous blood flow to the LA by closing or blocking an anomalous connection as described previously.

Three similar cases in 2 different communications were found in English literature.^5,17 The first communication is a case series of 4 patients with congenital heart disease in which large venous vessels were occluded with Vascular Plug I⁵ (AGA Medical). The third case described was a postoperative supracardiac total anomalous pulmonary venous connection. In this patient VV was left opened and important left to right shunt was observed. VV was occluded using a femoral vein approach in the same fashion as our case. The fourth case was a PLSCV connecting to the LA with mild desaturation in a patient with Tetralogy of Fallot. PLSCV was occluded using a jugular vein approach.

The second communication reports a case of a 58-year-old woman with neurological symptoms due to paradoxical embolism in which a PLSCV was diagnosed and treated by percutaneous occlusion using a Vascular Plug I.¹⁷

In our patient, treatment was decided to prevent further infections compromising the left lung apical lobe and to normalize Qp/Qs ratio.

Previous echo-Doppler examination reported a left pulmonary vein velocity of 0.7 m/s, which increased to 1 m/s after treatment. A less compliant pulmonary vein or higher pressures in left cavities could explain this change of velocity after blocking the anomalous connection and redirecting pulmonary venous blood flow to the LA.

Two months after the intervention, the patient was hospitalized for mild hypoxemia caused by atypic pneumonia compromising both lungs on chest x-ray and successfully treated with azithromycin. Echo-Doppler examination showed the device in good position without subclavian or pulmonary left vein obstruction.

In conclusion, this is a very rare presentation of left PAPVC and PLSCV. We consider it important to communicate our experience of occlusion through percutaneous treatment.

Acknowledgments. The authors wish to thank Alberto Tamashiro, MD, Chief of Hemodynamics Laboratory section Hospital Posadas, Sergio De Nato, MD, anesthesiologist, as well as the technicians, nurses, and secretaries of this section.

References

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17. Recto MR, Sadlo H, Sobczyk WL. Rare case of persistent left superior vena cava to left upper pulmonary vein: pathway for paradoxical embolization and development of transient ischemic attack and subsequent occlusion with an Amplatzer vascular plug. J Invasive Cardiol. 2007;19(10):E313-E316.

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From the Department of Pediatrics, Section of Pediatric Cardiology, Hospital Posadas, Buenos Aires, Argentina.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.
Manuscript submitted September 16, 2011, provisional acceptance given September 27, 2011, final version accepted November 11, 2011.
Address for correspondence: Jorge E. Gomez, MD, Section of Pediatric Cardiology, Hospital Nacional, Profesor A. Posadas  Pte. Illia s/n y Marconi - El Palomar, (1684) Buenos Aires, Argentina. Email: drjorgegomez@gmail.com