John F. Keane

Management of coronary artery fistulae: Patient selection and results of transcatheter closure

OBJECTIVES We report short-term findings in 33 patients after transcatheter closure (TCC) of coronary artery fistulae (CAF) and compare our results with those reported in the recent transcatheter and surgical literature. BACKGROUND Transcatheter closure of CAF has been advocated as a minimally invasive alternative to surgery. METHODS We reviewed all patients presenting with significant CAF between January 1988 and August 2000. Those with additional complex cardiac disease requiring surgical management were excluded. RESULTS Of 39 patients considered for TCC, occlusion devices were placed in 33 patients (85%) at 35 procedures and included coils in 28, umbrella devices in 6 and a Grifka vascular occlusion device in 1. Post-deployment angiograms demonstrated complete occlusion in 19, trace in 11, or small residual flow in 5. Follow-up echocardiograms (median, 2.8 years) in 27 patients showed no flow in 22 or small residual flow in 5. Of the 6 patients without follow-up imaging, immediate post-deployment angiograms showed complete occlusion in 5 or small residual flow in 1. Thus, complete occlusion was accomplished in 27 patients (82%). Early complications included transient ST-T wave changes in 5, transient arrhythmias in 4 and single instances of distal coronary artery spasm, fistula dissection and unretrieved coil embolization. There were no deaths or long-term morbidity. Device placement was not attempted in 6 patients (15%), because of multiple fistula drainage sites in 4, extreme vessel tortuosity in 1 and an intracardiac hemangioma in 1. CONCLUSIONS A comparison of our results with those in the recent transcatheter and surgical literature shows similar early effectiveness, morbidity and mortality. From data available, TCC of CAF is an acceptable alternative to surgery in most patients.

Percutaneous Catheter Commissurotomy in Rheumatic Mitral Stenosis

We attempted percutaneous transcatheter-balloon mitral commissurotomy in eight children and young adults (9 to 23 years of age) with rheumatic mitral stenosis. The atrial septum was traversed by needle puncture, and an 8-mm angioplasty balloon was advanced over a guide wire. The atrial septal perforation was then dilated to allow passage of the valvuloplasty balloon catheter (18 to 25 mm) across the mitral annulus. Inflation of the transmitral balloon decreased the end-diastolic transmitral gradient temporarily in all patients (from 21.2 +/- 4.0 mm Hg [mean +/- S.D.] to 10.1 +/- 5.5 mm Hg; P less than 0.001). The immediate decrease in the gradient was associated with increases in cardiac output (from 3.8 +/- 1.0 to 4.9 +/- 1.3 liters per minute per square meter of body-surface area; P less than 0.01) and in the calculated mitral-valve-area index (from 0.73 +/- 0.29 to 1.34 +/- 0.32 cm2 per square meter; P less than 0.001). Murmur intensity diminished immediately after commissurotomy in all patients. The greatest reduction in pressure gradient (76 to 95 per cent) occurred when the largest balloon (inflated diameter, 25 mm) was used in the smallest patients (0.9 to 1.2 m2). The balloon commissurotomy produced minimal mitral regurgitation in only one child. Follow-up catheterization (at two to eight weeks) demonstrated persistence of hemodynamic improvement with evidence of partial restenosis in one patient. These early results indicate that balloon mitral commissurotomy can be a safe and effective treatment for children and young adults with rheumatic mitral stenosis.

Hepatic Venous Blood and the Development of Pulmonary Arteriovenous Malformations in Congenital Heart Disease

BACKGROUND Pulmonary arteriovenous malformations (PAVMs) are a known complication after some types of cavopulmonary anastomoses (CVPAs). Their cause is unknown, but they may be related to the absence of pulsatile flow or the presence or absence of circulating factors. These PAVMs are diffuse and are presumed to be progressive and irreversible. METHODS AND RESULTS All patients with congenital heart disease (CHD) seen at Childrens Hospital, Boston, Mass, between 1970 and 1993 were reviewed. We report on the 10 patients with CHD who were found to have developed PAVMs, as diagnosed by cardiac catheterization. Diagnoses included heterotaxy syndrome/polysplenia, with interrupted inferior vena cava and hepatic veins draining to the right atrium (n = 6); heterotaxy/asplenia (n = 1); corrected transposition with pulmonary stenosis (n = 1); and biliary atresia and associated CHD (n = 2). PAVMs were diagnosed 0.1 to 7.0 years (median, 3.5 years) after creation of a CVPA that resulted in exclusion of hepatic venous flow from one or both lungs in 8 of the 10 patients; the remaining 2 patients had normal drainage of hepatic veins to the lungs but had biliary atresia. In all, the common anatomic feature was the exclusion of normal hepatic venous return from the affected pulmonary arterial circulation. All patients with interrupted inferior vena cava, azygous continuation to the superior vena cava, and hepatic veins draining to the right atrium (polysplenia syndrome) were reviewed to determine the incidence of PAVMs in those with CVPA (ie, hepatic venous flow excluded from the pulmonary arteries) and without CVPA. Six of 28 (21%) of those with versus 1 of 56 (1.8%) of those without CVPA developed PAVMs (P = .004). The 1 patient without CVPA who had PAVMs also had biliary atresia. Among patients with CVPA, the probability of developing PAVMs was 15% and 28% at 3 and 5 years, respectively, after CVPA. The histological and angiographic appearances of PAVMs after CVPA are similar to those seen in PAVMs associated with hepatic cirrhosis. CONCLUSIONS We postulate that PAVMs after CVPA are related to the diversion of normal hepatic venous flow from the pulmonary circulation. In this sense, these PAVMs may be analogous to those associated with liver disease, which have been found to resolve after liver transplantation. Redirection of hepatic flow to the pulmonary bed in some patients with CHD and PAVMs may lead to reversibility of the PAVMs.

Double-umbrella closure of atrial defects. Initial clinical applications.

Forty patients were catheterized for closure of atrial septal defects with the Rashkind patent ductus arteriosus umbrella device, a modified Rashkind umbrella device, and the newly designed Lock Clamshell Occluder. Patients weighed 8 kg or more (a requirement for transvenous access with the 11F delivery sheath) and had defects suitable for closure based on two-dimensional echocardiography. The new device was at least 1.6 times the diameter of the atrial septal defect as determined by balloon sizing at catheterization. Patients were followed up by telephone, clinical examination, and echocardiography at 6 months. We attempted closure in 34 patients, with atrial septal defects ranging in diameter from 3 to 22 mm; device sizes ranged from 17 to 33 mm. Initial device position immediately after release was correct in all patients. A cerebral embolus occurred in one elderly patient before device placement--the patient died 1 week later. Two instances of early device embolization occurred, and devices were retrieved by catheter without complication. Follow-up of 31 patients discharged with devices in place, for a total of 31 patient-years, has yielded no umbrella-related complications. Adequate imaging studies in 19 patients 6.5 months after device placement revealed no atrial shunt in 12; residual flow through separate, previously unrecognized atrial septal defects occurred in two; and small residual leaks (less than 3 mm) around devices were present in five patients. This initial success indicates that double-umbrella closure of atrial septal defects will aid in the treatment of intracardiac defects.

Effect of baffle fenestration on outcome of the modified Fontan operation.

BackgroundThe “fenestrated Fontan” (surgical baffle fenestration followed by transcatheter test occlusion and permanent closure after postoperative recovery) was adopted in an effort to reduce perioperative mortality and morbidity. This study assesses the effect of baffle fenestration on outcome. Methods and ResultsPatients having a modified Fontan operation with a cavocaval baffle and cavopulmonary anastomosis were retrospectively selected for study. Those with baffle fenestration (n=91) were compared with those without baffle fenestration (n=56) with respect to preoperative risk factors, age, anatomy, surgical date, and presence or absence of a previous bidirectional cavopulmonary anastomosis. Outcome variables were failure (death or take-down) and duration of postoperative pleural effusions and hospitalization. Survival and clinical status after hospital discharge were ascertained. The two groups did not appear to differ with respect to age or anatomic diagnosis. Patients having baffle fenestration were at significantly greater preoperative risk by univariate and multivariate analysis (p < 0.01). Operative failure was low in both groups (11% without and 7% with baffle fenestration, p=NS). Durations of pleural effusions and hospitalization were significantly shorter with baffle fenestration (p < 0.01). Neither date of surgery nor a previous bidirectional cavopulmonary anastomosis appeared to contribute to improved outcome. Patients with baffle fenestration had lower postoperative systemic venous pressure (p < 0.01). There were no late deaths. Functional status in both groups is good (82% in New York Heart Association class I). ConclusionsBaffle fenestration is associated with low mortality, significantly less pleural effusion, and significantly shorter hospitalization among high-risk patients having a modified Fontan operation.

Wolff-Parkinson-White syndrome and supraventricular tachycardia during infancy: Management and follow-up

The records of 90 patients with Wolff-Parkinson-White syndrome who presented with supraventricular tachycardia in the first 4 months of life were reviewed. Among these, 63% were male. Structural heart disease was present in 20%, most commonly Ebsteins anomaly. All patients presented with a regular narrow QRS tachycardia, and pre-excitation became evident only when normal sinus rhythm was established. Only one infant had atrial flutter and none had atrial fibrillation. Type A Wolff-Parkinson-White syndrome was most common (49%), with heart disease occurring in only 5% of these patients. In contrast, heart disease was identified in 45% of those with type B syndrome. Initially, normal sinus rhythm was achieved in 88% of the 66 infants treated with digoxin with no deaths. Normal sinus rhythm resumed after electrical countershock in 87% of the 15 infants so treated. Maintenance digoxin therapy was used in 85 patients. The Wolff-Parkinson-White pattern disappeared in 36% of the patients. Four infants died of cardiac causes during the mean follow-up period of 6.5 years. Two of these four infants had congenital heart disease; the third, with a normal heart initially, developed ventricular fibrillation and died from a cardiomyopathy considered related to resuscitation. The remaining infant, with a normal heart, died suddenly at 1 month of age. All were receiving digoxin. A wide QRS tachycardia later appeared in three patients, all with heart disease, one of whom died.(ABSTRACT TRUNCATED AT 250 WORDS)

Transcatheter closure of coronary artery fistulas.

Transcatheter closure of a coronary artery fistula was undertaken in nine patients. There were three fistulas from the left circumflex coronary artery to the coronary sinus, three from the left anterior descending coronary artery to the right ventricular apex, two from the right coronary artery to the superior vena cava/right atrial junction and one fistula from the left circumflex artery to the pulmonary artery. The fistula was closed with Gianturco coils in six patients, a double-umbrella device in two and a combination of an umbrella and coils in one patient. All fistulas are completely occluded. Complications consisted of migration of two coils, one of which was retrieved, and a transient junctional tachycardia in one patient. In an additional three patients with multiple coronary artery fistulas, transcatheter occlusion was not attempted.

Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects.

At the time of surgical repair, a lung biopsy was performed on patients with congenital heart defects who either had pulmonary hypertension or in whom it would be likely to develop if the lesion were not corrected. Pulmonary vascular changes, assessed morphometrically and also according to the classification of Heath and Edwards (Circulation 18: 533, 1958), were correlated with the postoperative pulmonary hemodynamic findings: mean pulmonary arterial pressure the day after correction and mean pulmonary arterial pressure and pulmonary vascular resistance measured 1 year later. On the first postoperative day, increased mean pulmonary arterial pressure was uncommon in patients with morphometric grade A or B (mild) biopsy findings and Heath-Edwards grade N (normal), and if it was present it was of a mild degree. Mean pulmonary arterial pressure was commonly elevated in those with grade B (severe) or C (mild or severe) and Heath-Edwards grade I biopsy results and was more frequently elevated in those with grade II findings. Moderate-to-severe elevation of mean pulmonary arterial pressure was invariable in patients with Heath-Edwards grade III changes regardless of the morphometric grade. One year after repair, mean pulmonary arterial pressure and/or pulmonary vascular resistance were normal in all patients whose conditions were corrected surgically before 9 months of age regardless of the severity of the pulmonary vascular changes. Values were normal in patients whose conditions were repaired surgically at 9 months of age or later who had grade A or B (mild) morphometric findings with any Heath-Edwards grade or grade B (severe) morphometric findings with Heath-Edwards grade I but were increased in half of the patients with grade B (severe) morphometric findings and Heath-Edwards grade II or with grade C (mild or severe) and Heath-Edwards grade I or II changes. Pulmonary arterial pressure and pulmonary vascular resistance were increased in all patients whose conditions were repaired after 2 years of age with grade C morphometric findings and to a severe degree if associated with Heath-Edwards grade III. Thus, although the Heath-Edwards grade can usually be used to identify patients at risk for pulmonary hypertension in the early postoperative period, both the morphometric and the Heath-Edwards grades as well as the age of the patient at the time of repair can be used to determine whether pulmonary arterial pressure and resistance eventually return to normal or remain elevated.

Late results in patients with tetralogy of Fallot repaired during infancy.

Between 1973 and 1985, 220 infants (age 1 day to 18 months, mean = 7 months) underwent repair of tetralogy of Fallot (TOF) or TOF/pulmonary atresia with 17 early deaths. Follow-up status was ascertained for 184 of 203 survivors (91%) at a postoperative interval of 2 to 185 months (mean = 60 months). Significant residual hemodynamic lesions included ventricular septal defect in three, and right ventricular outflow gradient in excess of 40 mm Hg in 24. Reoperation or interventional catheter procedures were performed in 31 of 184 (17%) patients. All postoperative electrocardiograms were reviewed; only two of 184 patients had ventricular ectopy on any tracing. Holter data from 41 patients revealed one with sinus node dysfunction, 12 with Lown grade 1 ectopy, and one with Lown grade 2 or greater. Conduction defects, evaluated by electrocardiographic and electrophysiologic measurements, were not different from those reported for TOF patients undergoing repair at later ages. No study patient received antiarrhythmic medication, although one required a pacemaker for sinus node dysfunction. There were three late deaths, all unrelated to arrhythmia. TOF repair in infancy appears to be associated with an acceptable hemodynamic outcome, and a low incidence of ventricular ectopy and sudden death at 5 year follow-up.

Prolongation of RV-PA conduit life span by percutaneous stent implantation : intermediate-term results

BACKGROUND Right ventricle-to-pulmonary artery (RV-PA) homografts and bioprosthetic conduits are commonly used to palliate various types of complex congenital heart disease. These conduits frequently develop progressive obstruction and require surgical replacement. This report reviews our experience implanting balloon-expandable stents to relieve conduit obstruction and delay reoperation. METHODS AND RESULTS A retrospective review identified 44 patients who underwent placement of 48 stents in obstructed RV-PA conduits. Median patient age was 6.9 years (range, 7 months to 30 years), and median follow-up time was 14.2 months (range, 0 to 48 months). Stent implantation initially decreased the RV-PA pressure gradient from 61.0 +/- 16.9 to 29.7 +/- 11.9 mm Hg (P < or = .001) and the right ventricular-to-systemic arterial pressure ratio from 0.92 +/- 0.17 to 0.63 +/- 0.20 (P < or = .001). The diameter of the stenotic region expanded from 9.3 +/- 3.5 to 12.3 +/- 3.3 mm in the anteroposterior view (P < or = .001) and from 6.6 +/- 2.9 to 10.9 +/- 2.5 mm in the lateral view (P < or = .001). During the follow-up period, 2 patients had their stents redilated, 7 had additional conduit stents deployed, and 14 underwent surgical replacement of their conduits. Actuarial freedom from conduit reoperation was 65% at 30 months postprocedure. Seven patients were found to have fractured stents on follow-up, suggesting an important role for external compressive forces in conduit failure. Recatheterization in 16 patients a median of 11.8 months (3 to 48 months) postprocedure demonstrated hemodynamic evidence of recurrent obstruction despite sustained enlargement at the previously stented sites. Complications included stent displacement (n = 1), bacterial endocarditis (n = 1), and false aneurysm formation (n = 1). One patient died awaiting conduit replacement surgery. CONCLUSIONS Stent implantation in obstructed RV-PA conduits results in significant immediate hemodynamic and angiographic improvement. In a subgroup of patients, the procedure prolongs conduit life span by several years and increases the interval between conduit reoperations. Recurrent obstruction is caused by external compression and progressive stenosis outside the stented region.