James E. Lock

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Background— To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children’s Hospital Boston. Methods and Results— A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium–to–pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure–related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; P=0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; P=0.0002). Independent predictors of heart failure death were protein-losing enteropathy (HR, 7.1; P=0.0043), single morphologically right ventricle (HR, 10.5; P=0.0429), and higher right atrial pressure (HR, 1.3 per 1 mm Hg; P=0.0016). Conclusion— In perioperative survivors of Fontan surgery, gradual attrition occurs predominantly from thromboembolic, heart failure–related, and sudden deaths.

Fontan operation in five hundred consecutive patients: Factors influencing early and late outcome ☆ ☆☆ ★ ★★ ♢ ♢♢ ♦

OBJECTIVES The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. METHODS The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. RESULTS The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more (p < 0.001), younger age at operation (p = 0.001), heterotaxy syndrome (p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve (p = 0.001), pulmonary artery distortion (p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage (p = 0.001), the absence of a baffle fenestration (p = 0.002), and longer cardiopulmonary bypass time (p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation (p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure (p = 0.003). CONCLUSIONS These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.

Transcatheter closure of patent foramen ovale after presumed paradoxical embolism.

BackgroundMany have proposed a relation between presence of a patent foramen ovale, with or without atrial septal aneurysm, and cryptogenic stroke. The effect of foramen ovale closure on the risk for subsequent strokes is unknown. Methods and ResultsTranscatheter closure of a patent foramen ovale was undertaken in 36 patients with known right-to-left atrial shunting and presumed paradoxical emboli (31 strokes, 25 transient neurological events, four systemic arterial emboli, and two brain abscesses). Individual patients had between one and four such events. None had a left heart or carotid source of embolism; 31 of 35 had no known risk factors for stroke. Events occurred in 12 patients while they were taking warfarin. At cardiac catheterization, patent foramina ovalia were significantly larger than predicted for age in 67% of the patients. Implantation of a double-umbrella device in the patent foramen ovale was achieved in all without serious procedural complications. Of 34 who have returned for follow-up, one has a residual atrial communication that may be clinically important, five had trivial leaks, and 28 have complete closure. There have been no strokes during a mean follow-up of 8.4 months. ConclusionsTranscatheter closure of a patent foramen ovale can be accomplished with little morbidity and may reduce the risk of recurrence. Further investigations directed toward identifying the population at risk and assessing the effect of intervention are warranted.

Use of endovascular stents in congenital heart disease.

BackgroundBalloon expandable intravascular stents have been used to support vessel walls in coronary and peripheral arteries in adults. The purpose of this study was to examine the efficacy and safety of these stents in the treatment of congenital heart disease. Methods and ResultsForty-five stents were placed in 30 patients, who were 0.2-30.2 years old (weight, 3.5-76 kg). Patients with areas of stenosis that were difficult to approach surgically were chosen. Stents were mounted over balloons and placed by standard catheterization techniques. Twenty-three patients had branch pulmonary artery stenosis. Thirty-six stents were placed successfully and had reduced pressure gradients from 50.6+24 to 15.9 ± +13.4 mm Hg. Five patients had stents placed after atrial surgery: three in obstructed Fontan repairs, one at the superior vena cava-right atrial junction after sinus venous defect repair, and one at the site of a Glenn shunt. Atrial stents reduced pressure gradients from 9.8 ± +8.2 to 2.0+2.6 mm Hg. One patient had a stent placed in the descending aorta after coarctation dilation, and the pressure gradient was reduced from 50 to 25 mm Hg. One patient had pulmonary vein dilation with stent placement. Two stents migrated at the time of placement; one required surgical removal, and one was anchored in place by balloon dilation. One patient died within 24 hours of catheterization because of thrombus obstruction of the Fontan repair. Nine patients have undergone recatheterization. All stented vessels have remained at the same caliber as at original stent placement. ConclusionsWe conclude that balloon expandable stents are useful in selected postoperative stenoses in congenital heart disease. (Circulation 1991;83:1923—1939)

Short- and Medium-Term Outcomes After Transcatheter Pulmonary Valve Placement in the Expanded Multicenter US Melody Valve Trial

Background— Transcatheter pulmonary valve placement is an emerging therapy for pulmonary regurgitation and right ventricular outflow tract obstruction in selected patients. The Melody valve was recently approved in the United States for placement in dysfunctional right ventricular outflow tract conduits. Methods and Results— From January 2007 to August 2009, 136 patients (median age, 19 years) underwent catheterization for intended Melody valve implantation at 5 centers. Implantation was attempted in 124 patients; in the other 12, transcatheter pulmonary valve placement was not attempted because of the risk of coronary artery compression (n=6) or other clinical or protocol contraindications. There was 1 death from intracranial hemorrhage after coronary artery dissection, and 1 valve was explanted after conduit rupture. The median peak right ventricular outflow tract gradient was 37 mm Hg before implantation and 12 mm Hg immediately after implantation. Before implantation, pulmonary regurgitation was moderate or severe in 92 patients (81% with data); no patient had more than mild pulmonary regurgitation early after implantation or during follow-up (≥1 year in 65 patients). Freedom from diagnosis of stent fracture was 77.8±4.3% at 14 months. Freedom from Melody valve dysfunction or reintervention was 93.5±2.4% at 1 year. A higher right ventricular outflow tract gradient at discharge (P=0.003) and younger age (P=0.01) were associated with shorter freedom from dysfunction. Conclusions— In this updated report from the multicenter US Melody valve trial, we demonstrated an ongoing high rate of procedural success and encouraging short-term valve function. All reinterventions in this series were for right ventricular outflow tract obstruction, highlighting the importance of patient selection, adequate relief of obstruction, and measures to prevent and manage stent fracture. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

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.

Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair

Background: Factors associated with impaired clinical status in a cross-sectional study of patients with repaired tetralogy of Fallot (TOF) have been reported previously. Objectives: To determine independent predictors of major adverse clinical outcomes late after TOF repair in the same cohort during follow-up evaluated by cardiac magnetic resonance (CMR). Methods: Clinical status at latest follow-up was ascertained in 88 patients (median time from TOF repair to baseline evaluation 20.7 years; median follow-up from baseline evaluation to most recent follow-up 4.2 years). Major adverse outcomes included (a) death; (b) sustained ventricular tachycardia; and (c) increase in NYHA class to grade III or IV. Results: 22 major adverse outcomes occurred in 18 patients (20.5%): death in 4, sustained ventricular tachycardia in 8, and increase in NYHA class in 10. Multivariate analysis identified right ventricular (RV) end-diastolic volume Z ⩾7 (odds ratio (OR) = 4.55, 95% confidence interval (CI) 1.10 to 18.8, p = 0.037) and left ventricular (LV) ejection fraction <55% (OR = 8.05, 95% CI 2.14 to 30.2, p = 0.002) as independent predictors of outcome with an area under the receiver operator characteristic curve of 0.850. LV ejection fraction could be replaced by RV ejection fraction <45% in the multivariate model. QRS duration ⩾180 ms also predicted major adverse events but correlated with RV size. Conclusions: In this cohort, severe RV dilatation and either LV or RV dysfunction assessed by CMR predicted major adverse clinical events. This information may guide risk stratification and therapeutic interventions.

Implantation and intermediate-term follow-up of stents in congenital heart disease.

BackgroundBalloon-expandable stents (Johnson and Johnson Interventional Systems) have been in use for congenital heart disease since late 1989. They have made possible treatment in previously untreatable branch pulmonary artery stenoses and systemic venous stenosis. The purpose of this report is to detail the results and intermediate-term follow-up of stents used for treatment of congenital heart disease. Methods and ResultsEighty-five patients underwent placement of 121 stents in Houston and Boston. Fifty-eight patients had stents put in pulmonary arteries, nine had stents in conduits or outflow tracts, and 21 had stents in venous stenoses or narrowed Fontan anastomoses. (Three patients had stents in two locations.) These stent procedures resulted in gradient reduction from 55.2 ± 33.3 to 14.2+13.5 mm Hg in pulmonary arteries, from 41.4 ± 26.0 to 20.7 ± 17.0 mm Hg in conduits or outflow tracts, and from 9.8 ± 6.9 to 2.4+3.1 mm Hg in venous stenoses or Fontan anastomoses. Diameter of narrowings increased from 4.6 ± 23 to 113 ± 3.2 mm in the pulmonary artery, from 8.8+3.6 to 12.7 ± 2.6 in conduits, and from 3.8 ± 2.9 to 113+2.8 in venous stenoses. Follow-up has shown stent fracture in one patient, restenosis in one, and sudden death in one. Recatheterization has been done in 38 patients an average of 8.6 months after stent installation. Compared with immediately postimplant data, there was no significant change in luminal diameter or pressure gradient. Redilation was performed in 14 patients (17 stents) 1 week to 24 months after implantation (mean, 10.2 months), with a small but significant increase in stenosis diameter. ConclusionWe conclude that stent treatment of vascular stenoses in congenital heart disease retains efficacy at medium-term follow-up and offers a much-improved outlook for patients with these lesions.

Transcatheter ablation of ectopic atrial tachycardia in young patients using radiofrequency current.

BackgroundEctopic atrial tachycardia (EAT) is a reversible cause of cardiomyopathy but may be quite difficult to control with conventional therapy. Transcatheter ablation with radiofrequency current was tested as an alternative to medical or surgical treatment of this condition Methods and ResultsTwelve young patients (aged 10 months to 19 years) with drug-resistant EAT were treated with direct transcatheter ablation of the ectopic focus using radiofrequency (RF) energy. All had depressed left ventricular contractility by echocardiographic criteria, involving shortening fractions of 10–26% (median, 20%; normal, 28–35%). The EAT was mapped to the left atrium in seven cases and to the right atrium in five. Local atrial activation at the ectopic site preceded the onset of the surface P wave by 20–60 msec (median, 42 msec). Tachycardia terminated 0.5–13.0 seconds (median, 2.0 seconds) into a successful RF application. The ablation effectively eliminated EAT in 11 of 12 patients (92%), all of whom were discharged in sinus rhythm without medications after a median hospital stay of 48 hours. Ablation was unsuccessful in one patient with diffuse dysplasia of the anterior right atrium, who eventually did well after surgical resection of abnormal atrial tissue. Transient depression of sinus node function was noted in one patient who had successful ablation of an EAT focus in close proximity to the sinus node, although normal sinus node function returned within 72 hours. No other complications were encountered. During follow-up (3–21 months; median, 13 months), one patient had recurrence of a slower and less-sustained EAT that was successfully eliminated at a second ablation session. All others remained in sinus rhythm, and all 12 subjects recovered normal ventricular function ConclusionsRF ablation appears to be a safe and effective therapeutic option for drug-resistant ectopic atrial tachycardia and may be the preferred first-line therapy for those patients with depressed ventricular function.