Author + information
- Goutam Datta1
Patient initials or identifier number
Relevant clinical history and physical exam
A 22 years-old male presented with dyspnoea ,cyanosis, clubbing and pedal odema. He is symptomatic for last three years. There were no history of epistaxis or hemoptysis. He had no evidence of telangiectasia. Ophthalmological examination was also normal. His blood pressure was 110/70 mm Hg and pulse rate was 110/min.Jugular venous pressure and wave forms were within normal limit. He had normal vesicular breath sounds and normal heart sounds.There were no murmur,no S3/S4.
Relevant test results prior to catheterization
His blood sugar,liver function tests,renal parameters were normal.His haemoglobin was 16gm with haematocrit of 48.
The EKG and Echocardiogram were normal. Contrast echocardiogram revealed simultaneous opacification of left and right heart chambers suggesting left to right shunt. Ultrasound of abdomen was within normal limit.There was haziness in chest Xray on left side and CT scan showed pulmonary artery venous fistula.
Relevant catheterization findings
We did coronary angiogram and left ventricular angiogram. All were normal. Then we performed right ventricular angiogram which suggested some communication from pulmonary artery. We selectively engazed left and right pulmonary artery by multipurpose catheter.
It showed multiple complex pulmonary artery venous fistula in left lung. Complexity is because of multiple feeder arteries. There were no left to right shunt at atrial or ventricular level. Pulmonary artery pressure was normal.
We planned for coil closure of pulmonary artery venous fistula. Multipurpose catheter was used to engaze different fistula. Stainless steel Cook: Nester coils were used. We took different size coil according to size of feeder vessel.It was difficult to close all fistula. We staged it to coil different fistula using standard side branch anchor technique. Standard guidewire was used for delivery.We used five coils to close probably all fistula.Though usually few are left many times. Patient became NYHA class 1 symtomatic. Three months later, he came again with pedal odema. There were no residual fistula. Right ventricular pressure as well as pulmonary artery pressure were normal. There were no tricuspid regurgitation.Repeat CT thorax and ultrasound of abdomen were noncontributory. Venogram revealed normal superior vena cava drainage. But there was suprarenal inferior vena cava stenosis with a gradient of 14 mm Hg. There was azygous communication to superior vena cava. We took long sheath of 8F diameter. Wall stent were deployed over standard 300 cm guidewire. It was a self expanding stent of 18/120. Gradient was abolished. The patient again remained NYHA 1 without any odema.
The Congenital pulmonary AV fistula is commonly associated with hereditary haemorragic telangiectasia. Our case of isolated pulmonary AV fistula is very rare (15%). Pulmonary AV fistula are classified as simple with one feeder artery or complex with muliple feeder artery. They usually present at third/fourth decade with dyspnoea on exertion or platypnoea ortho-deoxia syndrome. Multipurpose or right Judkin or Hockey stick catheter are commonly used to engaze these fistula. Cooks coils of different sizes are used to close these fistula. Our patient also had inferior vena cava stenosis. Wall stent was used. Association of Pulmonary AV fistula and inferior vena cava stenosis are uncommon.