A novel minimal invasive approach The way forward

Dr. Uditha Hewarathna

Ajith Kularatne, Gnanamoorthi Mayurathan,Uditha Hewarathna, Shanike Karunaratne,Narada Kodithuwakku

Teaching Hospital, Kandy,

Sri Lanka

Mr. K

14 years old

Presented with a infected wound in left leg

Incidentally found to be having polycythaemia (Hb - 19.9 g/dl)

On Examination

◦ Cyanosed

◦ Plethoric

◦ Clubbing in B/L UL and LL

◦ Oxygen saturation 62% on air

CRP - negative

ESR - 3 mm/ 1st hour

Blood picture - secondary polycythemia

Transthoracic 2D echo ◦ Normal size cardiac chambers

◦ EF > 60%

◦ No ASD, VSD or PDA

◦ No evidence of PHT or TAPVD

◦ ? Fistulous connection between pulmonary artery and left atrium

TOE

◦ No ASD or VSD

◦ Normal pulmonary venous drainage

Normal Lung function test

Surgical closure

Transcatheter closure

Major operation

Has potential surgical and anesthetic complications

Patient and mother not given consent for the surgery

Has two options

◦ Use a covered stent across the fistula in the right pulmonary artery

◦ Device AV fistula closure

Mechanism

◦ Fistula will have no feeding

Problems

◦ Covered stent more expensive and patient couldn’t afford

◦ Due to stagnation of blood in AV fistula connection there is a risk of thrombosis

◦ Risk of embolization of the thrombus

Problems

◦ Largest Amplatzer vascular plug device available is 22mm and fistula was 18 ×20mm in size (They recommend 50% oversizing for these type of fistula)

◦ When a larger device is used, it is very long and device will be jutting out into LA and RPA

◦ Risk of thrombus formation

◦ Risk of obstruction

Finally planned to use atrial septal occluder device to close the fistula

Atrial septal occluder device in LA is safe

Available in different sizes

9F long guiding sheath and dilator were advanced over the guide wire through IVC, RA, RV, MPA, RPA, fistulous connection to left atrium

Sending ASO device through delivery system

Deploying 16mm ASO device -unsuccessful

Deploying 20mm ASO device

Deployed 20 mm device obstructing right pulmonary artery

Deployed 20 mm device obstructing right pulmonary artery

Successful deploying of 16mm ASO device

Soon after deploying the device

Soon after deploying the device checking the pulmonary flow and closure of AV fistula

Soon after deploying the device checking the pulmonary flow and closure of AV fistula

Patient’s saturation improved from 62% to 94% on air

Transthoracic echo confirmed there was no turbulence or pressure gradient in the right pulmonary artery

TOE confirmed normal pulmonary venous drainage.

10 min after deploying the device checking the pulmonary flow and closure of AV fistula

10 mm after deploying the device checking the pulmonary flow and closure of AV fistula

10 mm after deploying the device checking the pulmonary flow and closure of AV fistula

Releasing of device by counterclockwise rotation of the delivery cable after checking the stability

After releasing of the device

Bubble contrast echo after the procedure

Dual antiplatelets for 1 month followed by 6 month single antiplatelet used to prevent thrombo-embolism

At 6 months

◦ Patient asymptomatic

◦ Fluoroscopy show ASO device in same position

◦ No shunt in transthoracic echo

Direct communication between the right pulmonary artery and the left atrium is an unusual variation of a pulmonary arteriovenous fistula1

In fact, there are only about 50 cases reported in the literature2

This is one condition in which clinical examination may reveal only cyanosis1

Contrast echocardiography may show only right-to-left shunt, and only angiography is truly diagnostic

1Vladimir AM, Ingo D, Stanislav O, Roland H. Right pulmonary artery to left atrium communication, a rare cause of systemic cyanosis. Texas Heart Inst J 2001;28:122–124. 2Krishnamoorthy KM, Rao S. Pulmonary artery to left atrial fistula. Eur J Cardiothorac Surg 2001;20:1052–1053

Two treatment options were available either surgical closure or transcatheter closure

Most of patient has underwent surgical closure1

Coil embolization, Amplatzer vascular plug device have been used to close the AV fistulae2,3

1Zeebregts CJ, Nijveld A, Lam J, van Oort AM, Lacquet LK. Surgical treatment of a fistula between the right pulmonary artery and left atrium: presentation of two cases and review of literature. Eur J Cardiothorac Surg 1997;11:1056 –1061. 2Slack MC, Jedeikin R, Jones JS., Transcatheter coil closure of a right pulmonary to left atrial fistula in an ill neonate. Catheter Cardiovasc Interv 2000;50:330–333. 3Francis E, Sivakumar K, Kumar RK. Transcatheter closure of fistula between the right pulmonary artery and left atrium using the Amplatzer duct occluder. Cathet Cardiovasc Interv 2004;63:83–86.

Our patient has 18×20mm fistulous connection and largest Amplatzer vascular plug device available is 22mm (They recommend 50% oversizing for these type of fistula)

So, fistulous connection in our patients were too wide to be closed safely and completely using the above devices

We chose ASO because it was the only large enough device available to us to occlude the large fistulous connection seen in our patients.

The other reasons were ◦ Availability in different sizes

◦ Easy implantation

◦ Anchorage stability offered by both the discs

◦ Retrieval as well as repositioning capability before the final release

Our case is unique because the communication was closed in an antegrade fashion without transseptal puncture

Large and short Pulmonary AV fistule can be closed safely, effectively, and nonsurgically with atrial septal occluder device

Placing a atrial septal occluder device in pulmonary artery without obstructing the flow is safe

It is a effective method for our country with poor resource setting

“I cant put into word the comfort and consolation I feel now. It like been in the heaven from hell”

Ajith Kularatne and Gnanamoorthi Mayurathan who are primary operators in this case

My trainers in TH Kandy ◦ Dr. Rohini Tennakoon

◦ Dr. Subhashini Jayawickreme

◦ Dr. Gamini Weerakoon

◦ Dr. Srinath Dolapihilla

All doctors in cardiology unit Kandy

All staff in the Cath lab TH Kandy

Device Size/ Waist Diameter (mm) A

Waist Length (mm) B

Right Atrial Disc Diameter (mm) C

Left Atrial Disc Diameter (mm) D

15 4 25 29

16 4 26 30

17 4 27 31

18 4 28 32

19 4 29 33

20 4 30 34

Device Diameter (mm) A

Unconstrained Device Length (mm) B

12 9

14 10

16 12

18 14

20 16

22 18