Percutaneous Coil Embolization and Balloon-Expandable Stenting; New Treatments in Intractable Cardiac Failure after Fontan Procedure : A Case Reportt

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1 Percutaneous Coil Embolization and Balloon-Expandable Stenting; New Treatments in Intractable Cardiac Failure after Fontan Procedure : A Case Reportt DUANGMANEE LAOHAPRASITIPORN, M.D.*, API CHART NANA, M.D.*, SOMCHAI SRIYOSCHA TI, M.D.**, W ALAI SRIY APHAI, B. Nursing*, CHAT CHAD A HONGVISITGUL, B. Nursing*** The Fontan procedure is an excellent palliative operation but not a curative one in all types of single ventricle physiology(!). However, chronic cardiac failure may occur, frequently manifested as fluid retention which may be idiopathic or related to myocardial failurec2), elevated pulmonary arterial resistancec3) or Fontan pathway obstructionc4). Persistent accumulation of pleural effusion or less often of ascitic or pericardia! fluid account for 10 per cent of the patients who die without hemodynamic insufficiency( 1). CASE REPORT An 11-year-old boy presented with increasing exercise intolerance and intractable right heart failure including hepatomegaly, persistent right pleural effusion and ascites for 11 months. He was diagnosed when newborn as having hypoplastic right heart syndrome with pulmonary atresia and confluent pulmonary arteries supplied by patent ductus arteriosus. At the age of two years, he underwent palliative right modified Blalock Taussig shunt (RMBTS) with a 6-rnillimeter Gortex tube because his cyanosis and polycythemia had increased. He did well until the age of seven years when he developed hemoptysis that required blood transfusion. Cardiac catheterization and angiocardiography were undertaken and revealed QP : Q, = 0.6 : 1, hypoplastic right heart, mild hypoplasia left pulmonary artery (LPA) and functioning RMBTS. He underwent modified Fontan operation or total cavopulmonary connection with 3-millimeter fenestration and double ligation of RMBTS with uneventful postoperative course except for mild pleural effusion which was resolved by digitalis and a diuretic. He was classified as New York Heart Association (NYHA) functional class I with occasional cyanosis. One and a half years after the last operation, he was readmitted because of hemoptysis, increased cyanosis, exercise intolerance with NYHA functional class IV and signs of right heart failure, ascites and massive right pleural effusion (Fig. la) which had dismal results from placement of right intercostal tube drainage (ICD), increasing doses of digitalis, diuretics and enalapril. His symptoms progressed over a period of 11 months. M-mode echocardiography demonstrated poor left ventricular function. The 3rd cardiac catheterization was carried out and revealed unobstructed and elevated * Division of Pediatric Cardiology, Department of Pediatrics, ** Division of Cardiothoracic Surgery, Department of Surgery, *** Her Majesty's Cardiac Centre, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand. t Presented at 28th Annual Scientific Meeting of the Heart Association of Thailand, March 1996.

2 Vol. 79 No.5 TREATMENTS IN INTRACTABLE CARDIAC FAILURE AFrER FONTAN PROCEDURE 321 1A lb Fig. 1 Chest roentgenography of our patient demonstrated A) pre-coil embolization, massive right pleural effusion and ascites; B) post-coil embolization, resolved right pleural effusion. Table 1. Summary of hemodynamic data from cardiac catheterization pre- an post coil embolizations (Emb) and stent implantation. 1st Coil Emb 2nd Coil Emb Stent iml!lantation Site 0 2 sat MP 02 sat MP 02 sat MP Stenotic (%) (rnmhg) (%) (rnmhg) (%) (rnmhg) diameter (rnrn) pre post pre post pre post pre pre pre post pre post svc * 10* 58 10* IVC RPA LPA prox distal Ao SVC =superior vena cava; IVC =inferior vena cava; RPA =right pulmonary artery; LPA =left pulmonary artery; prox =proximal; Ao =aorta; 02 sat= oxygen saturation; MP = mean pressure. * during intercostal tube drainage (lcd) placement; before lcd placement SVC and IVC mean pressures were 20 mrnh6. mean pressure along the Fontan pathway and pulmonary arteries with significant increase in oxygen saturation at right pulmonary artery (RP A) (Table 1). Angiocardiography demonstrated hypoplastic LPA, multiple aortopulmonary (AP) collateral arteries from right 4th-6th intercostal arteries (ICA) connected to RP A (Fig. 2A) and multiple bronchial arteries from left internal mammary arteries to the left more than to the right lung. Those ICA were completely occluded by Gianturco coils (Cook, Inc., Bloomington, IN) in 2 separated interventional catheterizations (Fig. 2B). In concert with medical pleurodesis, the right pleural effusion disappeared (Fig. 1B) and the lcd was successfully removed. The total duration of lcd placement was 39 days. However, his ascites increased, requiring subsequent cardiac catheterization. From this study, it was noted that there was a 6 mmhg pressure gradient between the proximal and distal LP A (Table 1). Angiography demonstrated tubular hypoplasia with severe discrete stenosis (diameter of 4.7 mm) of the proximal LPA (Fig. 3A) which was also shown by the previous studies. Balloon angioplasty was performed; the fully expanded diameter of the stenotic site was 9 mm and it restenosed to 6 mm with a balloon deflation. The balloon-expandable PALMAZ 3 em long (P308, Johnson & Johnson Interventional System Co.) stent mounted with a 12

3 322 D. LAOHAPRASITIPORN et al. J Med Assoc Thai May 1996 Fig. 2 Angiography of our patient demonstrated A) pre-coil embolization; right 5th intercostal arteries connected to right pulmonary artery; B) post-coil embolization, those collaterals were completely occluded by Gianturco coil. Fig. 3 Angiography of left pulmonary artery (LPA) demonstrated A) pre- stent implantation, tubular hypoplasia with severe discrete stenosis of proximal LPA; B) post-stent implantation, enlarged stenotic LPA. mm balloon catheter was deployed and expanded, producing a stenotic diameter of 9.5 mm (Fig. 3B). The pressure gradient decreased to 4 mmhg. One week after stent implantation, his exercise tolerance had increased. His ascites was reduced and the liver was unremarkable. He was discharged home 2 weeks after the procedure. At one-month and 3-month follow-up, his NYHA functional class was improved to class II. DISCUSSION After the Fontan procedure, our patient was in NYHA functional class I for 1 1/2 years; according to Fontan et al's report, nearly 90 per cent of patients are in NYHA functional class I at 1 year after the operation and about 56 per cent at 10 years(5). Fifteen-year survival is predicted to be 73 per cent(6). The incremental risk factors for death after the operation include; 1) Acute main chamber ventricular decompression which can be avoided by performing a fenestrated Fontan procedure(!), as with our patient. 2) Chronic ventricular volume overload, which usually produces a secondary ventricular cardiomyopathy and reduces systolic function. Both AP shunt and collateral vessels are common sources of accessory pulmonary blood

4 Vol. 79 No.5 TREATMENTS IN INTRACTABLE CARDIAC FAILURE AFTER FONTAN PROCEDURE 323 flow in children with cyanotic congenital heart diseases, including univentricular heart, who have undergone a modified Fontan ~rocedure, leading to ventricular volume overload( ). These collaterals may compete with venous pulmonary flow provided by the cavopulmonary connection. 3) Failure to perform surgical enlargement of small pulmonany artery size(5) or pulmonary distortionc4). The pre branching portions of the RP A and LP A are considered small if the McGoon ratio is less than about 1.5, the Z-value is less than -3.5 or a crosssection area index (Nakata index) is less than about 160 mm m-z(7). 4) Elevated mean pulmonary artery pressure greater than mmhg and pulmonary vascular resistance greater than 4 units m 2 (reference 3). This may be caused by longstanding RMBTS and multiple AP collaterals. 5) The morphologic right or indeterminate ventricle as the main chamber is held to be less favorable than the morphologic left ventricle(8,9). Although our patient had morphologic left ventricle as the main chamber, he had an unfavorable postoperative course; persistent right heart failure and pleural effusion were caused by an elevated cavopulmonary pressure. The 2 possible reasons for this event were; 1) he had significant multiple AP collaterals originating from the right 4th- 6th ICA, which is the site of origin of 13 per cent of AP collaterals following a Fontan procedure; the internal mammary artery accounts for 34 per centc10)_ 2) He had hypoplastic LPA (McGoon ratio about 1. 7) with severe discrete proximal LP A stenosis. Recently, a variety of absorbable and nonabsorbable materials have been developed and used for peripheral vessel occlusion. Presently, transcatheter coil embolization procedures provide an important definitive and palliative treatment with a safe and effective strategy. for many patients with congenital heart disease(2). Some children have been documented as showing gratifying clinical improvement after coil embolization owing to by diminished total pulmonary blood flow reducing the associated ventricular volume overload and pleural effusionc2,11,12) as in our patient. Since 1989, balloon-expandable stents have been introduced into the treatment of congenital heart diseases and postsurgical vascular stenoses which are not amenable or accessible to surgery and have shown excellent results(l3, 14). In Thailand, there has never been a report on stenting of peripheral vessel stenosis. In our patient, the first case in Thailand, the stenotic diameter of the LP A was doubled after stent implantation and ascitic fluid was dramatically reduced 2 weeks later. He has been in NYHA functional class II, at 1 and 3 months after the last procedure. SUMMARY Successful percutaneous coil embolization of aortopulmonary collaterals and balloon-expandable stent implantation of left pulmanory artery stenosis in a child with hypoplastic right heart syndrome were reported, Presently, interventional cardiac catheterization is a very useful treatment strategy in congenital heart diseases unamenable to or inaccessible for surgery. ACKNOWLEDGEMENT The authors wish to thank Miss Joan Marsh for her assistance in approbation of the manuscript and Mr. Burin Kwosa-ard for photographic preparation. (Received for publication on February 28, 1996)

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