Transcatheter occlusion of giant congenital coronary cameral fistulae: a case series

A 10-year-old boy black South African presented with a history of easy fatigue on exertion. There was no past history of recurrent illness or hospital admission. There was no history of congenital heart diseases in his family. A clinical examination on admission revealed a well-grown boy weighing 26.6 kg. He had no signs of respiratory distress or heart failure. His body temperature was 37 °C. He had a bounding pulse with a rate of 100 beats per minute. His blood pressure (BP) was 100/20 mmHg, demonstrating a wide pulse pressure. A grade 3/6 continuous murmur could be heard at the fourth left intercostal space. The rest of his systemic and neurological examination was normal. He had normal posture, gait, muscle bulkiness, tone, power, and reflexes. There was no hepatosplenomegaly or jaundice. His complete blood count showed hemoglobin (Hb) of 12.2 g/dl, white blood cell count (WBC) of 8.7 cells/mm³ with normal differential counts, and a platelet count of 445 cells/mm³. His C-reactive protein (CRP) was not elevated (< 1 mg/dl). He had a normal creatinine of 55 μmol/L and urea of 3.8 mmol/L. His serum electrolytes were also normal (sodium, 138 mmol/L; potassium, 3.9 mmol/L; and chloride, 100 mmol/L). A chest roentgenogram and electrocardiogram (ECG) were normal. Echocardiography demonstrated a large fistula involving his left coronary artery (LCA) and his LV. The LCA origin was massively dilated and measured 10 mm. An ascending aortogram confirmed a diagnosis of a large CCF from the LCA to the LV (Fig. 1a). His RCA appeared normal.

After discussion with the surgical team, it was concluded that the fistula would be too big for successful deployment of a device without the risk of embolization. Surgical ligation of the fistula was then elected and performed. Post-surgical ligation echocardiography demonstrated residual flow through the fistula into the LV, but the flow was minimal and restrictive with a gradient of 80 mmHg. His symptoms resolved. He was monitored over a period of 2 years and remained asymptomatic, but the residual fistula remained. Due to the risk it posed for infective endocarditis, we elected to close the residual fistula percutaneously. A repeat angiogram into the ascending aorta and LCA demonstrated a fistula with its narrowest portion at the site of surgical ligation measuring 8 mm (Fig. 1b). His LCA was cannulated using a left Judkins catheter and a V18 guide wire. A 16 mm AVP II was successfully deployed distally at the site of previous surgical ligation. Post device closure, an angiogram showed no flow through or around the device and there was better flow into the left anterior descending (LAD) and circumflex arteries (Fig. 1c, d). He was discharged and allowed to go home the following day on 37.5 mg clopidogrel daily and 100 mg aspirin daily. These medications were stopped after 6 months. He has remained well 10 years after the procedure.

A 7-year-old black South African boy was found to have an incidental murmur during evaluation for an upper respiratory tract infection. On direct questioning, he admitted having decreased effort tolerance. There was no significant past medical history and he was not receiving any chronic medication. A clinical examination revealed a healthy child with no fever. He had normal BP of 100/65 mmHg, a bounding pulse and a rate of 120 beats per minute. There was mild cardiomegaly. A grade 3/6 continuous machine-like murmur was detected on the mid-sternal border. He had normal chest symmetry. There was no liver or spleen enlargement. A neurological examination was normal. Basic blood investigations were within normal limits as follows: Hb, 11.6 g/dl; WBC, 10 cells/mm³; platelet count, 332 cells/mm³; serum creatinine, 48 μmol/L; urea, 3.7 mmol/L; sodium, 130 mmol/L; potassium, 4.8 mmol/L; and chloride, 98 mmol/L. Chest radiography confirmed cardiomegaly. An ECG was found to be normal, with no signs of ischemia. Two-dimensional echocardiography with color flow Doppler showed a grossly dilated LCA origin measuring 10 mm (Fig. 2a, b) and draining into the dilated LV (Fig. 2c, d). A diagnosis of a large CCF from the LCA to the LV was made. His LV function was normal with an ejection fraction of 63%.

At cardiac catheterization, an ascending aortogram confirmed the diagnosis of a large CCF draining to the LV (Fig. 3a). After the angiogram, we elected to close the fistula percutaneously using the AVP II device. The LCA was cannulated using a left Judkins catheter. A 14 mm AVP II was deployed into the exit end of the fistula into the LV, to avoid occlusion of the major LCA branches (Fig. 3b). A repeat angiogram showed successful device closure of the fistula, with no residual leak around or through the device on angiogram (Fig. 3c). A post closure echocardiogram showed no residual fistulous flow and he had normal cardiac function. He was discharged on 75 mg aspirin daily, which was stopped after 6 months. On subsequent reviews, he has remained well and asymptomatic. There is no recanalization of the fistula 7 years after the procedure.

A 28-year-old black South African woman presented with a history of decreased effort tolerance and episodes of chest pain on exertion. She reported that a cardiac murmur had been heard during her childhood, but it had never been investigated. She had never smoked tobacco or consumed alcohol. On examination, she had New York Heart Association (NYHA) class II cardiac failure. She had no fever or jaundice. Her pulse was 75 beats/minute and collapsing. She had a bounding radial and femoral pulse. Her BP was 120/50 mmHg, with a wide pulse pressure. There was no cardiomegaly and no evidence of pulmonary hypertension. A grade 2/6 continuous murmur was audible over the left lower sternal border. She had no liver or spleen enlargement. A central nervous system examination was unremarkable with normal cognition, normal muscle power, bulkiness, and gait. She had no lateralizing signs and had normal vision. Her chest roentgenogram and ECG were normal. There were no features of ischemic heart disease evident from the ECG. Echocardiography demonstrated a dilated RCA origin that formed a large fistulous connection with the RV. Her LCA was normal. She had normal left ventricular function. Hemodynamic data demonstrated a left-to-right shunt with a pulmonary to systemic blood flow ratio (Qp:Qs) of 1.3:1. The pulmonary pressure and pulmonary vascular resistance were normal.

The angiography of the ascending aorta and RCA demonstrated a large and tortuous CCF arising from the RCA and draining into the RV. The fistula measured 11 mm at the widest point and 8.5 mm at the narrowest point (Fig. 4a). The fistula was easily accessed from the RCA with a 6F-guiding catheter, which could be manipulated into the distal part of the fistula with the aid of a 0.014-inch coronary guide wire. A 14 mm AVP II was selected and deployed into the distal portion of the fistula. The position was checked with angiography, which confirmed that there was no obstruction of coronary side branches. There was also no residual shunt present across the AVP II. Repeat angiography showed complete occlusion of the fistula and better perfusion to the RCA and its branches (Fig. 4b, c).

Twelve hours after the procedure, our patient complained of chest pain. An ECG was done; it was normal with no evidence of ischemia. Her cardiac enzymes were also normal. Her chest pain resolved after the administration of ibuprofen. Repeat echocardiography confirmed the patency of the RCA, and occlusion of the fistula. She developed mild aortic regurgitation and her left ventricular function was marginally depressed with an ejection fraction of 55%. These new developments were thought to be due to elevated systemic vascular resistance following the removal of the left-to-right shunt and run-off into the RV.

She was discharged 48 hours after the procedure on 5 mg/kg aspirin daily to prevent thrombosis in the residual cul-de-sac. On follow up at 1 month and 6 months after the procedure, her pre-occlusion symptoms and her chest pain had resolved. The aortic regurgitation had resolved, and her left ventricular function had normalized. She remains symptom free to date, following the occlusion of the CCF.