Ebstein's anomaly - review of a multifaceted congenital cardiac condition.

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. In this review, we describe current diagnostic criteria and classification, natural history, clinical features, and prognosis, typical echocardiographic features and pathologic findings, and the spectrum of associated cardiac malformations including left heart anomalies associated with EA. Differences between Ebstein-like changes associated with congenitally corrected transposition and EA are described. The spectrum of typical ECG and conduction system changes, arrhythmias including accessory pathways and ectopic atrial tachycardias related to EA are also reviewed. Differential diagnosis of EA is discussed including tricuspid valve dysplasia and prolapse as well as arrhythmogenic right ventricular cardiomyopathy. The review describes management options in EA including catheter interventions, indication for operation and surgical options including tricuspid valve repair and replacement. Overall, EA is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Therefore, precise knowledge of the different anatomic and hemodynamic variables, associated malformations and management options are essential. Management of EA patients is complex. Thus it is important that these patients are regularly seen by a cardiologist with expertise in congenital heart disease.

Comparison of ethanol septal reduction therapy with surgical myectomy for the treatment of hypertrophic obstructive cardiomyopathy.

OBJECTIVES: This study was designed to compare the hemodynamic efficacy of nonsurgical septal reduction therapy (NSRT) by intracoronary ethanol with standard therapy (surgical myectomy) for the treatment of hypertrophic obstructive cardiomyopathy (HOCM). BACKGROUND: Nonsurgical septal reduction therapy has gained interest as a new treatment modality for patients with drug-refractory symptoms of HOCM; however, its benefits in comparison to surgery are unknown. METHODS: Forty-one consecutive NSRT patients at Baylor College of Medicine with one-year follow-up were compared with age- and gradient-matched septal myectomy patients at the Mayo Clinic. All patients had left ventricular outflow obstruction with a resting gradient > or =40 mm Hg and none had concomitant procedures. RESULTS: There were no baseline differences in New York Heart Association class, severity of mitral regurgitation, use of cardiac medications or exercise capacity. One death occurred during NSRT because of dissection of the left anterior descending artery. At one year, all improvements in both groups were similar. After surgical myectomy, more patients were on medications (p < 0.05) and there was a higher incidence of mild aortic regurgitation (p < 0.05). After NSRT, the incidence of pacemaker implantation for complete heart block was higher (22% vs. 2% in surgery; p = 0.02). However, seven of the nine pacemakers in the NSRT group were implanted before a modified ethanol injection technique and the use of contrast echocardiography. CONCLUSIONS: Nonsurgical septal reduction therapy resulted in a significantly higher incidence of complete heart block, but the risk was reduced with contrast echocardiography and slow ethanol injection. Surgical myectomy resulted in a significantly higher incidence of mild aortic regurgitation. Nonsurgical septal reduction therapy, guided by contrast echocardiography, is an effective procedure for treating patients with HOCM. The hemodynamic and functional improvements at one year are similar to those of surgical myectomy.

The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients.

OBJECTIVES: We assessed the operative and late mortality and the present clinical status of 216 patients with tricuspid atresia who had a nonfenestrated Fontan procedure performed at the Mayo Clinic in the 25-year period 1973 to 1998. BACKGROUND: The Fontan operation eliminates the systemic hypoxemia and ventricular volume overload characteristic of prior forms of palliation. However, it originally did so at the cost of systemic venous and right atrial hypertension, and the long-term effects of this "price" were unknown when the procedure was initially proposed. METHODS: We reviewed the clinical records of the 216 patients retrospectively. These were arbitrarily grouped into early (1973 through 1980), middle (1981 through 1987) and late (1988 through 1997) surgical eras. Patient outcome was also analyzed according to age at surgery. Operative and late mortality rates were determined and present clinical status was ascertained in 167 of 171 surviving patients. RESULTS: Overall survival was 79%. Operative mortality steadily declined and was 2% (one of 58 patients) during the most recent decade. Late survival also continues to improve. Age at operation had no effect on operative mortality, and late mortality was significantly increased only in patients who were operated on at age 18 years or older. Eighty-nine percent of surviving patients are currently in New York Heart Association class I or II. CONCLUSIONS: The initial 25-year experience with the nonfenestrated Fontan procedure for tricuspid atresia has been gratifying, with most survivors now leading lives of good quality into adulthood. These results justify continued application of this procedure for children born with tricuspid atresia.

Surgical pathology of the parietal pericardium: a study of 344 cases (1993-1999).

Among 344 cases with surgically resected parietal pericardium, ages ranged from 1 to 87 years (mean, 55), and 64% were male. Causes of pericardial disease included neoplastic (33%), idiopathic (30%), iatrogenic (23%), and others (14%). Pericardial constriction (Group 1) represented the largest group (143 cases, 76% male). Maximal pericardial thickness was 1-17 mm (mean, 4). Fibrotic thickening occurred in 96%. Chronic lymphoplasmacytic inflammation affected 73% (mild or moderate in 97%). Calcification was uncommon (gross in 28%, microscopic in 8%), and granulomas were rare (4%, none tubercular). Constriction was idiopathic in 49% and iatrogenic (postpericardiotomy or postirradiation) in 41%. Neoplasms and cysts (Group 2) represented the second largest group (96 cases). Among 43 cases with secondary pericardial involvement, carcinomas accounted for 53% and lymphomas 21%. Forty cases (Group 3) had pericardial effusions (75% chronic), which were idiopathic in 28% and postpericardiotomy in 23%. Thirty-three cases (Group 4) had acute or recurrent pericarditis clinically, which was idiopathic in 70%. Lastly, 32 cases (Group 5) had pericardial resection for conditions unrelated to primary pericardial disease. In conclusion, pericardial constriction tended to be nontubercular (100%), nongranulomatous (96%), idiopathic or iatrogenic (90%), and noncalcific (64%), and it could occur with normal pericardial thickness (4%). Because considerable overlap in the gross and microscopic features existed among cases with noncalcific pericardial constriction (Group 1), pericardial effusions (Group 3), and pericarditis (Group 4), clinical information was necessary to provide an accurate clinicopathologic interpretation.

Early and late results of operations for defects associated with corrected transposition and other anomalies with atrioventricular discordance in a pediatric population.

OBJECTIVES: The purpose of this study was to determine the early and late results for children having operations for defects associated with corrected transposition of the great arteries and other anomalies with atrioventricular discordance. METHODS: Data on 111 children operated on from July 1, 1971, through January 31, 1996, including clinic records, operative reports, and follow-up visits and questionnaires, were analyzed with particular reference to variables associated with early and late mortality, reoperations, ventricular function, and status of the atrioventricular valves. RESULTS: Complex associated anomalies were common and included double-outlet right ventricle (n = 43) and situs abnormalities (n = 38). Overall early mortality was 16%; for the 29 patients operated on after 1986, early mortality was 3%. Early survival was adversely affected by patch repair of ventricular septal defect and early operative interval. Follow-up of the 93 early survivors extended to 26.5 years (mean 11.4 years). Overall survival was 77% (+/-4%) at 5 years and 67% (+/-5%) at 10 years. Late survival was adversely affected by prior operations, more severe preoperative functional class, and cardiac rhythm other than sinus. Reoperation was required for 38 (41%) patients, most commonly for conduit replacement (n = 22) or repair/replacement of the systemic ventricle atrioventricular valve (n = 13). CONCLUSIONS: These results can serve as a basis for comparison with newer surgical alternatives proposed for corrected transposition of the great arteries.

Late results of the Rastelli operation for transposition of the great arteries.

The Rastelli operation, first performed in 1968, was developed for repair of transposition of the great arteries with associated ventricular septal defect and severe pulmonary stenosis. This operation includes placement of an intracardiac baffle to direct left ventricular blood to the aorta and an extracardiac valved conduit to establish continuity between the right ventricle and the pulmonary arteries. Over the last 3 decades, the Rastelli operation has been performed with a progressive decline in early mortality, and it remains the preferred repair for transposition, ventricular septal defect, and severe fixed valvular or subvalvular pulmonary stenosis. This chapter examines the late results of our 33-year experience with the Rastelli operation and describes our operative technique.

Valvular heart operation in patients with previous mediastinal radiation therapy.

BACKGROUND: The outcome of valvular heart operations in patients with previous mediastinal radiation therapy was studied. METHODS: This is a single center retrospective study of 60 patients (37 females, 23 males) with a mean age of 62 +/- 15 years (28 to 88 years old) operated on from January 1976 to December 1998. Valvular heart operations performed included aortic valve replacements (n = 26), mitral valve procedures (n = 16), tricuspid valve procedures (n = 6), and multiple valve procedures (n = 12). A total of 264 clinical, hemodynamic, electrocardiographic and echocardiographic variables were analyzed. RESULTS: Total follow-up was 199 patient-years with a mean of 3.3 +/- 3.1 years and a range of 0 to 12.4 years old. Early mortality was 7 patients (12%). Early mortality in patients with constrictive pericarditis was 40% (4 of 10) compared with 6% (3 of 50) in patients without constrictive pericarditis. By univariate analysis, early mortality was associated with constrictive pericarditis (p = 0.011), reduced preoperative ejection fraction (p = 0.015), and longer cardiopulmonary bypass times (p = 0.037). A total of 14 patients (23%) required permanent pacemaker placement before (n = 7), during (n = 1), or early (n = 6) after valvular heart operations. There were 19 late deaths (malignancies, 7; heart failures, 5; other cardiac, 4; and other noncardiac, 3). Overall survival and freedom from late cardiac death and cardiac reoperation at 5 years for hospital survivors were 66% +/- 8%, 82% +/- 7%, and 93% +/- 4%, respectively. By univariate analysis, late cardiac death was associated with low ejection fraction (p = 0.002), New York Heart Association (NYHA) functional class IV (p = 0.004), preoperative congestive heart failure (p = 0.02), and preoperative atrial fibrillation (p = 0.038). Eighty-five percent of the discharged patients were in NYHA functional class I or II at follow-up. CONCLUSIONS: Early results of valve replacement after mediastinal radiation therapy were good except in the presence of constrictive pericarditis. Long-term outcome was limited by malignancy and heart failure. Early surgical intervention is recommended before the development of risk factors for late death, namely, severe symptoms, left ventricular dysfunction, and atrial fibrillation.

Late pulmonary valve replacement after repair of tetralogy of Fallot.

BACKGROUND: Pulmonary regurgitation appears to be well tolerated early after repair of tetralogy of Fallot; however, it may result in progressive right ventricular dilatation and dysfunction necessitating eventual valve replacement. Our objective was to review our experience with late pulmonary valve replacement after complete repair of tetralogy of Fallot. METHODS AND RESULTS: A total of 42 patients (16 female and 26 male) were operated on between July 1, 1974, and January 1, 1998. Mean age was 22 years (range 2-65 years). The mean interval between tetralogy repair and pulmonary valve replacement was 10.8 years (range 1.6 months-33 years). Mean follow-up was 7.8 +/- 6.0 years (maximum 23 years). Indications for pulmonary valve replacement included decreased exercise tolerance in 58%, right heart failure in 21%, arrhythmia in 14%, syncope in 10%, and progressive isolated right ventricular dilatation in 7%. Heterograft prostheses were used in 33 patients and homografts in 9. Five patients underwent isolated pulmonary valve replacement; concomitant procedures performed in 37 patients included tricuspid valve repair/replacement (n = 18), residual ventricular septal defect repair (n = 12), atrial septal defect closure (n = 4), pulmonary artery patch angioplasty (n = 17), and right ventricular outflow tract enlargement (n = 13). One patient died early (2%) of multiorgan failure. There were 6 late deaths, 3 of which were cardiac related. Survival was 95.1% +/- 3.4% and 76.4% +/- 8.9% at 5 and 10 years, respectively. Functional class of patients was improved significantly; preoperatively, 76% of patients were in New York Heart Association class III-IV, and after pulmonary valve replacement, 97% of surviving patients were in class I-II (P =.0001). Moderate to severe reduction in right ventricular function was noted on preoperative echocardiography in 59% and on late echocardiography in 18% (P =.03). Of the 5 patients who had supraventricular arrhythmias before pulmonary valve replacement, 1 had postoperative recurrence and the arrhythmia is controlled with antiarrhythmic therapy; the other 4 are in normal sinus rhythm at late follow-up. Eight patients subsequently underwent pulmonary valve re-replacement without early mortality at a mean interval of 9.0 +/- 4.2 years (range 3.8-16.8 years). Freedom from pulmonary valve re-replacement was 93.1% +/- 4.7% and 69.8% +/- 10.7% at 5 and 10 years, respectively. The only significant risk factor for re-replacement was young age at the time of the initial pulmonary valve replacement (P =.023). CONCLUSION: Late pulmonary valve replacement after tetralogy repair significantly improves right ventricular function, functional class, and atrial arrhythmias, and it can be performed with low mortality. Subsequent re-replacement may be necessary to maintain functional improvement.

Asymptomatic giant right atrial aneurysm.

A 5-year-old asymptomatic boy was found incidentally, on a chest radiograph, to have gross cardiomegaly; further evaluation by echocardiography showed a giant right atrial aneurysm. The patient underwent successful surgical reduction of the right atrium, closure of an atrial septal defect, and tricuspid valve repair These measures were taken to prevent thrombus formation in the right atrium, prevent paradoxical embolism, and lower the risk of atrial arrhythmias. The morphologic features of the resected atrial tissue showed paper-thin wall with a central aneurysm and focal endocardial fibrosis consistent with a diagnosis of idiopathic dilatation of the right atrium.

Influence of ventricular morphology on outcome after the Fontan procedure.

The modified Fontan procedure has gained wide acceptance in the treatment of various congenital heart defects. Determination of risk factors for mortality remains an important issue for optimizing patient selection for the Fontan procedure. Conflicting results have been reported about whether ventricular morphology is a risk factor in these patients. Survival free of Fontan takedown or cardiac transplantation was assessed in the first 500 patients undergoing the Fontan procedure at our institution. This survival was correlated with ventricular morphology as evaluated by angiography. Both multivariate and univariate analyses indicated ventricular morphology was predictive of early survival free of Fontan takedown or cardiac transplantation following the procedure. However, there was no statistical evidence for ventricular morphology being a risk factor for mortality in patients alive 6 months after the procedure. Ventricular morphology is a risk factor for early survival in patients undergoing a Fontan procedure, with left ventricular morphology associated with a better early survival than right ventricular morphology.

Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease.

Ebstein's anomaly is a rare congenital heart defect that is characterized by a spectrum of anatomical abnormalities of the tricuspid valve that also involve the right atrium and right ventricle. The extant nomenclature for Ebstein's anomaly and our approach to the description of the severity of Ebstein's anomaly are reviewed with the objective of establishing a unified reporting system. Although there are common features in Ebstein's anomaly, there is a wide spectrum of pathology with an infinite variety of combinations of severity of the involved structures. An effort was made to develop a classification system that would take into consideration the anatomic abnormalities that help direct the surgical management, particularly in regard to tricuspid valve repair or valve replacement. Isolated congenital tricuspid stenosis and regurgitation are also rare, and a simple classification system is presented. Acquired causes of tricuspid regurgitation and stenosis are more common and are included in the classification system. A comprehensive database set for these malformations is presented so that a comprehensive risk stratification analysis can be performed. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and risk factors are presented.

Operation for partial atrioventricular septal defect: a forty-year review.

BACKGROUND: We describe the long-term outcome of repair of partial atrioventricular septal defect by determining the rates of survival, reoperation, and occurrence of left atrioventricular valve regurgitation, left atrioventricular valve stenosis, left ventricular outflow tract obstruction, and arrhythmia. METHODS: We studied 334 patients who underwent repair of partial atrioventricular septal defect before 1995. RESULTS: The 30-day and 5-, 10-, 20-, and 40-year survival were 98%, 94%, 93%, 87%, and 76%, respectively. Closure of the left atrioventricular valve cleft (P =. 03) and age less than 20 years at operation (P <.001) were associated with better survival. Reoperation was performed for 38 patients (11%). Repair of residual/recurrent left atrioventricular valve regurgitation or stenosis was the most common reason for reoperation. Left ventricular outflow tract obstruction occurred in 36 patients, and 7 patients underwent reoperation to relieve this obstruction. Supraventricular arrhythmias were observed in 58 patients (16%) after the operation. Supraventricular arrhythmias increased with increasing age at primary operation (P =.001). Complete atrioventricular block occurred in 9 patients (3%). Permanent pacemakers were implanted in 11 patients. CONCLUSIONS: Long-term survival after repair of partial atrioventricular septal defect is good. It is important to close the cleft in the left atrioventricular valve. Reoperation for persistent or recurrent left atrioventricular valve malfunction and relief of left ventricular outflow tract obstruction is necessary in approximately 11% of patients.

Cox-Maze procedure for atrial fibrillation: Mayo Clinic experience.

The Cox-Maze procedure corrects atrial fibrillation in 90% of patients, and successful operation restores sinus rhythm, thereby reducing risks of thromboembolism and anticoagulant-associated hemorrhage. Symptoms such as palpitation and fatigability also improve with restoration of atrioventricular synchrony. At the Mayo Clinic, 221 Cox-Maze procedures were performed from March 1993 through March 1999. Over 75% of patients had associated cardiac disease and concomitant operations. Overall, early mortality was 1.4%, and the incidence of postoperative pacemaker implantation was 3.2%. Limiting incisions to the right atrium simplifies the operation for patients who primarily have tricuspid valve disease, and in early follow-up, outcome appeared to be as good as that achieved with biatrial incisions. The Cox-Maze procedure has proved particularly useful for patients with preoperative atrial fibrillation who require valvuloplasty for acquired mitral valve regurgitation; 87 patients have had this combined procedure, and there have been no early deaths. Further, our experience indicates that ventricular dysfunction is not a contraindication for operation and that restoration of sinus rhythm after the Cox-Maze procedure improves left ventricular ejection fraction in most patients.

Calcific constrictive pericarditis: is it still with us?

BACKGROUND: The presence of pericardial calcification on a plain radiograph strongly suggests constrictive pericarditis in patients with heart failure. However, calcific constrictive pericarditis is considered rare in the United States since tuberculosis incidence has decreased, and doubt has therefore been raised about the importance of this radiologic finding in modern cardiovascular practice. OBJECTIVE: To determine the clinical and prognostic significance of pericardial calcification on radiography in patients with constrictive pericarditis. DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: A consecutive series of 135 patients (mean age +/- SD, 56 +/- 16 years) who from 1985 through 1995 had constrictive pericarditis confirmed surgically (n = 133) or by autopsy (n = 2). Patients were divided into two groups: those with pericardial calcification on chest radiography (group I) and those without (group II). MEASUREMENTS: Clinical and diagnostic findings were compared in both groups, and outcome was compared in 132 patients who had pericardiectomy. RESULTS: Pericardial calcification was seen in 36 patients (27%). The cause of constrictive pericardial disease was indeterminate in 67% of patients in group I and in 21% of patients in group II (P < 0.001). Patients in group I had had symptoms for a longer period and were more likely to have pericardial knock, larger atrial size, and atrial arrhythmia. Significantly more perioperative deaths were seen in group I, but incidence of late survival and incidence of noncalcific disease were similar in both groups. CONCLUSIONS: Pericardial calcification is a common finding in patients with constrictive pericarditis. It is often associated with idiopathic disease and other markers of disease chronicity and is an independent predictor of increased perioperative mortality rates.

Desmopressin does not reduce bleeding and transfusion requirements in congenital heart operations.

BACKGROUND: Desmopressin (DDAVP) has been evaluated in many randomized clinical trials as a means to reduce blood loss and transfusion of allogeneic blood in cardiac operation requiring cardiopulmonary bypass. Desmopressin reduces blood loss in adult patients with excessive bleeding after cardiac operation. Its usefulness in patients undergoing complex congenital heart repair with cardiopulmonary bypass is unproved. METHODS: Sixty patients younger than 40 years of age scheduled for complex congenital heart operation (44 redo, 16 primary) were enrolled in this prospective, randomized, double-blind trial. Desmopressin 0.3 microg/kg or placebo was administered 10 minutes after protamine administration. Transfusion requirements and postoperative blood loss were recorded. Differences were analyzed using analysis of variance with a p value of 0.05 or less used to denote statistical significance. RESULTS: There were no differences in demographic or surgical characteristics between the DDAVP or placebo groups. There was no difference in blood loss and transfusion requirements between the DDAVP and placebo groups. During the intraoperative postinfusion time period, the median blood loss for redo patients was 343 versus 357 mL/m2 for placebo versus DDAVP, respectively, and for primary patients, the median blood loss was 277 versus 228 mL/m2. CONCLUSIONS: The prophylactic use of DDAVP to reduce excessive bleeding or transfusion requirements in patients undergoing complex congenital heart operations is not warranted.

Surgical patent foramen ovale closure for prevention of paradoxical embolism-related cerebrovascular ischemic events.

BACKGROUND: The role of surgical closure of patent foramen ovale (PFO) for cerebral infarction (CI) or transient ischemic attack (TIA) resulting from paradoxical embolism is unclear, and its effect on recurrence is unknown. Our objective was to determine the outcome of surgical closure of PFO in patients with a prior ischemic neurological event, define the rate of CI or TIA recurrence after PFO closure, and identify risk factors for these recurrences. METHODS AND RESULTS: We retrospectively analyzed 91 patients (58 men, 33 women) with >/=1 previous cerebrovascular ischemic events who underwent surgical PFO closure between April 1982 and March 1998. The presence of a PFO with a right-to-left shunt was confirmed with transesophageal echocardiography. Mean age was 44.2+/-12.2 years. The index event was a CI in 59 and a TIA in 32; a Valsalva-like episode preceded the event in 15 patients. Deep venous thrombosis was documented in 9 patients, and a hypercoagulable state was identified in 10. Surgical closure was performed with extracorporeal circulation by either direct suture (n=82) or patch closure (n=9). Limited incisions were used in 18.7% of patients. There was no operative mortality. Morbidity included transient atrial fibrillation (n=11), pericardial drainage for effusion (n=4), exploration for bleeding (n=3), and superficial wound infection (n=1). Follow-up totaled 176.3 patient-years, and mean follow-up was 2.0 years. No one had a CI, and 8 had a TIA during follow-up, with 1 caused by temporal arteritis. Transesophageal echocardiography demonstrated all closures to be intact in these patients. The overall freedom from TIA recurrence during follow-up was 92.5+/-3.2% at 1 year and 83.4+/-6.0% at 4 years. Having multiple neurological events before PFO closure was the only significant risk factor for TIA or CI recurrence after closure by univariate analysis (P=0.05); the small number of post-PFO closure cerebral ischemic events precluded multivariate analysis. CONCLUSIONS: Surgical closure of PFO can be performed with minimal morbidity and mortality. PFO closure may decrease the risk of recurrent stroke or TIA and may avoid lifelong anticoagulation in the young adult if there is no other indication. Recurrent cerebrovascular ischemic events after surgery should prompt further evaluation to identify causes other than paradoxical embolism.

Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy.

BACKGROUND: The clinical spectrum of constrictive pericarditis (CP) has been affected by a change in incidence of etiological factors. We sought to determine the impact of these changes on the outcome of pericardiectomy. METHODS AND RESULTS: The contemporary spectrum of CP in 135 patients (76% male) evaluated at the Mayo Clinic from 1985 to 1995 was compared with that of a historic cohort. Notable trends were an increasing frequency of CP due to cardiac surgery and mediastinal radiation and presentation in older patients (median age, 61 versus 45 years). Perioperative mortality decreased (6% versus 14%, P = 0.011), but late survival was inferior to that of an age- and sex-matched US population (57+/-8% at 10 years). The long-term outcome was predicted independently by 3 variables in stepwise logistic regression analyses: (1) age, (2) NYHA class, and most powerfully, (3) a postradiation cause. Of 90 late survivors in whom functional class could be determined, functional status had improved markedly (2.6+/-0.7 at baseline versus 1.5+/-0.8 at latest follow-up [P<0.0001]), with 83% being free of clinical symptoms. CONCLUSIONS: The evolving profile of CP, with increasingly older patients and those with radiation-induced disease in the past decade, significantly affects postoperative prognosis. Long-term results of pericardiectomy are disappointing for some patient groups, especially those with radiation-induced CP. By contrast, surgery alleviates or improves symptoms in the majority of late survivors.

Permanent pacing in patients with univentricular heart.

Patients with one of several varieties of malformation designated as "univentricular hearts" may be especially challenging when permanent pacing is required. Our objective was to review our experience in this subset of patients that had undergone permanent pacing and thus determine the optimal approach. A retrospective chart review was done of 32 patients with some variety of "univentricular" malformation who had required permanent pacing at our institution. Although technically challenging, permanent pacing in this group of patients can be successful through several approaches. The various approaches, as well as consideration of the differences that exist between patients undergoing septation and those undergoing a Fontan procedure are discussed. Although long-term permanent pacing is possible in this group of patients, before pacing begins, a thorough understanding of the anatomy and prior surgical procedures is necessary. Use of a combined atrial transvenous and ventricular epicardial pacing system may work well for some patients. With the development of newer and more reliable coronary sinus leads, dual chamber transvenous pacing with ventricular stimulation via the coronary sinus could become the approach of choice in some patients with "univentricular hearts."