Early and intermediate-term (10-year) results of surgery for univentricular atrioventricular connection (“single ventricle”) (original) (raw)
Related papers
Clinical outcome of the Fontan operation in patients with impaired ventricular function☆
European Journal of Cardio-Thoracic Surgery, 2009
Objective: Although a staged Fontan strategy allows for an excellent outcome in high-risk patients, an impaired ventricular function remains a significant factor of early/late mortality and morbidity. This study evaluated the clinical outcome of the Fontan operation in patients with impaired ventricular function. Methods: A retrospective review was performed on 217 patients who had undergone the Fontan operation between 1991 and 2007. Results: Twenty-nine (13%) of the 217 patients had an impaired ventricular function (ejection fraction (EF) <50%). The median age at the time of the operation was 3 (range: 1-31 years) years. There were five adult patients. The ventricular morphology was right in 20 patients (including five hypoplastic left heart syndrome (HLHS)) and others (left and two-ventricle) in nine patients. Heterotaxy syndrome was present in eight patients. Previous surgical interventions included bidirectional Glenn anastomoses in 24, modified Blalock-Taussig shunts in two and pulmonary artery banding in two. The preoperative EF was 43 AE 6%. Significant (moderate or severe) atrioventricular valve regurgitation was noted in four patients. The percutaneous oxygen saturation (SpO 2) was 82 AE 5%. The pulmonary artery pressure and pulmonary artery index were 11 AE 3 mmHg and 296 AE 102 mm 2 m À2 , respectively. All 29 patients underwent the Fontan operation without any early mortality. There were two late mortalities and two re-operations. EF was maintained at 59 AE 15% at a median follow-up of 7.5 (range: 1-19) years. The percent normal systemic ventricular end-diastolic volume decreased from 174 AE 95% to 124 AE 39% (p < 0.05). The SpO 2 increased to 92 AE 2%. The mean cardiothoracic ratio in chest X-ray and B-type natriuretic peptide were 51% (range: 35-68%) and 22 pg ml À1 (range: 9-382 pg ml À1), respectively. Three patients developed congestive heart failure, seven had arrhythmia and two developed protein-losing enteropathy. The New York Heart Association (NYHA) class functional class is I in 21 patients, II in five and III in one. Conclusions: Acceptable clinical outcomes were observed at an intermediate follow-up of the Fontan operation in patients with an impaired ventricular function.
Improved early results with the Fontan operation in adults with functional single ventricle
The Annals of Thoracic Surgery, 2004
ventricle Improved early results with the Fontan operation in adults with functional single http://ats.ctsnetjournals.org/cgi/content/full/77/4/1334 located on the World Wide Web at: The online version of this article, along with updated information and services, is Print ISSN: 0003-4975; eISSN: 1552-6259. Southern Thoracic Surgical Association. Background. A growing number of adults with functional single ventricles are presenting as candidates for first-time and redo-Fontan operations. This study describes the clinical presentation and early operative results of adults who have undergone Fontan modifications. Methods. Between July 1995 and April 2003, 23 patients (>18 years old) had Fontan operations. We retrospectively reviewed their perioperative courses.
Septation of the single ventricle: Revisited
The Journal of Thoracic and Cardiovascular Surgery, 2002
Background: Septation of a single ventricle into 2 functioning ventricles can provide an alternative to the Fontan operation. However, early experiences with septation reported unacceptable morbidity and mortality. The present study selected only those patients with large volume-overloaded hearts, 2 well-functioning atrioventricular valves, and an absence of severe outlet obstruction. Early and intermediate outcomes are evaluated. Methods and Results: Between June 1990 and March 1999, 11 patients underwent septation in 1 or 2 stages. Diagnoses of the patients included double-inlet left ventricle in 9, double-inlet right ventricle in 1 patient, and indeterminate ventricle in 1 patient. Five had L-transposition and 3 had D-transposition of the great arteries. Six had septation as 1 stage, 5 as planned 2-stage operations (2/5 completed). The median age for septation in 1 stage was 2.1 years (range 4 months to 5.8 years); for 2 stages, the median age was 7.2 months (range 3 to 14 months). Median follow-up time was 2.3 years. Eight of 11 patients survived (73%), with 2 early deaths and 1 late death. Seven of the 8 survivors have undergone complete septation (5 as single stage, 2 as 2 stages). Complications included surgically induced complete atrioventricular block in 1 patient and significant residual ventricular septal defects in another. Qualitatively, left ventricular function by echocardiography is normal in all patients, whereas right ventricular function is mildly decreased in 1 patient. All patients are clinically well. Conclusion: The septation procedure for single ventricle hearts may be a reasonable alternative to the Fontan operation in selected patients.
Experience with one and a half ventricle repair
The Journal of Thoracic and Cardiovascular Surgery, 1999
Objective: This article presents a 10-year experience with one and a half ventricle repair for right ventricular hypoplasia or dysfunction. Methods: From November 1986 to December 1996, 30 patients (mean age 6.7 ± 8.5 years, range 4 months-40 years) with functionally abnormal right ventricles underwent a bidirectional Glenn shunt as part of the repair. Diagnoses included pulmonary atresia with intact ventricular septum (n = 15), Ebstein anomaly (n = 5), levotransposition of the great arteries (n = 3), pulmonary stenosis with right ventricular hypoplasia (n = 2), tetralogy of Fallot (n = 3), dextrotransposition of the great arteries (n = l), and Uhl anomaly (n = l). Concomitantly performed cardiac procedures included atrial septal defect closure (n = 27), fenestration of the atrial septum (n = 2), right ventricular cavity augmentation (n = 8), right ventricular outflow tract enlargement (n = 6), transannular patch (n = 13), modified Blalock-Taussig shunt closure (n = 16), tricuspid replacement (n = 3), tricuspid repair (n = 2), Rastelli procedure (n = 3), tricuspid commissurotomy (n = 2), and double switch (n = l). Results: There were 2 early deaths (6.6%) and 1 late death. Mean early postoperative superior vena caval pressure was 14.12 ± 3.55 mm Hg and mean right atrial pressure was 10.3 ± 5.16 mm Hg. Early oxygen saturation in the operating room with an inspired oxygen fraction of 1 was 97.2 ± 2.5; oxygen saturation was 92.3 ± 4.8 on room air at discharge. Mean oxygen saturations were 93.6% ± 3.6% at 1 year of follow-up (P = .10) and 93.5% ± 4.1% at 5 years (P = .12). Overall survival was 90% at 5 years, and 21 patients (77%) were in New York Heart Association class I, 5 (18%) were in class II, and 1 (2.7%) was in class III. Conclusion: This procedure provides a valid alternative for correction of right ventricle hypoplasia or dysfunction. Early and intermediate follow-up results compare favorably with those of the Fontan procedure, but long-term follow-up is needed. (J Thorac Cardiovasc Surg 1999;117:662-8)
The American Journal of Cardiology, 2007
Ventricular septation (VS) and the Fontan procedure are alternatives for definitive repair in patients with double-inlet left ventricle; although VS is theoretically preferable, the current preference in practice is the Fontan procedure. However, the long-term outcomes of both procedures remain unclear. To address this issue, cardiopulmonary responses during exercise were measured in patients with double-inlet left ventricle, and the impact of the type of procedure performed, Fontan or VS, on long-term exercise capacity and late postoperative clinical profiles was assessed. Fourteen post-Fontan patients (mean age 17 ؎ 6 years) and 13 VS patients (mean age 19 ؎ 4 years) underwent exercise testing. Of the 13 VS patients, 5 required atrioventricular valve replacement (AVVR), and 7 required pacemaker implantation. Although no difference in peak oxygen uptake was found between the VS and Fontan patients, peak oxygen uptake was higher in VS patients without AVVR (30 ؎ 8 ml/kg/min) than in VS patients with AVVR (19 ؎ 1 ml/kg/min) and Fontan patients (22 ؎ 6 ml/kg/min) (p <0.01). There was no significant difference in peak oxygen uptake between the
Outcome of early and late onset Fontan operation in patients with univentricular heart repair
Objective: To evaluate our experience in the Fontan procedure comparing those below and above 6 years of age. Methods: A review of our clinical database was conducted to identify the patients who received extracardiac Fontan between 2002 and 2010. All demographic, echocardiographic, surgical, haemo-dynamic and follow-up data were collected. The overall mortality was defined as death occurring from the time of surgery to the most recent follow-up. Early postoperative death was defined as death occurring during admission or within 30 days from the operation. Seventy-six patients with functionally univentricular hearts were included in the study. Patients were divided into two groups. Group A included patients who had received extracardiac Fontan at the age of 6 years or less, whereas group B included patients who had received extracardiac Fontan at an age of more than 6 years. Results: The overall hospital mortality was 7.9% (10.2% in group A and 5.9% in group B). No statistically significant difference was seen between the two groups regarding the postoperative
Ventricular Performance in Long-Term Survivors After Fontan Operation
The Annals of Thoracic Surgery, 2011
Background. Ventricular function and arrhythmia in patients with Fontan circulation in long-term follow-up are still unknown. Methods. We retrospectively reviewed 48 patients who survived and were followed up for more than 15 years, among 110 patients who underwent Fontan operation in our institute from 1979 to 1992. Atriopulmonary connection was performed in 26 patients and total cavopulmonary connection in 22. The patients were categorized into right ventricle, left ventricle, and biventricle groups. Follow-up cardiac catheterization and exercise test were performed routinely every 5 years post surgery. Median age at Fontan operation was 5 years. Results. Mean follow-up was 18.5 years. Cardiac index in the total cavopulmonary connection group was higher than in the atriopulmonary connection group at 10 and 15 years post surgery (p < 0.05). Ejection fraction in the left-ventricle group was higher than in the right-ventricle group. End-diastolic volume at 5, 10, and 15 years was significantly lower than at 1 year (p < 0.05). End-diastolic pressure at 10 years was significantly higher than at 1 and 5 years (p < 0.05). Beyond 15 years, 6 patients developed ventricular tachycardia. The only significant risk factors for the onset of ventricular tachycardia in a multivariate analysis were age at Fontan operation and absolute age (p < 0.05). Conclusions. Long-term follow-up of patients demonstrated that postoperative ventricular systolic performance seemed to become steady. Ventricular tachycardia was detected 15 years post surgery, especially in older patients with older age at Fontan operation, possibly revealing a risk factor in the long-term postoperative period, thereby meriting further consideration.
The Annals of Thoracic Surgery, 2014
Background. Uncommonly, adults with functionally univentricular hearts are becoming candidates for a Fontan procedure. The purpose of this study was to evaluate the course of patients undergoing the modified Fontan procedure with an extracardiac conduit in recent years. Methods. Between January 2003 and December 2013, 32 adult patients (17 female and 15 male) underwent total cavopulmonary connection (TCPC) with extracardiac conduit. The median age at procedure was 24.5 years (interquartile range [IQR] 20 to 33 years). The diagnoses included double-inlet left ventricle (DILV) in 10 patients (31.2%), tricuspid atresia in 8 patients (25%), doubleoutlet right ventricle in 4 patients (12.5%), heterotaxia in 4 patients (12.5%), and mitral atresia in 2 patients (6.2%). Seventy-eight percent of patients had undergone at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt (16 patients), superior cavopulmonary shunt (12 patients), and pulmonary artery banding (6 patients). All patients underwent cardiac catheterization preoperatively. Aortic cross-clamping was necessary in 15 patients for intracardiac procedures. Fenestration was required in 9 patients (28%). Four concomitant intraoperative cryoablation procedures were performed.
Fontan Conversion to One and One Half Ventricle Repair
The Annals of Thoracic Surgery, 2012
Background. In patients with a modified Fontan connection, particularly the right atrial to right ventricular connection, the pulmonary ventricle may enlarge with time. Methods. Between January 1990 and December 2006, 10 patients (median age, 24 years) underwent Fontan conversion to a one and one half ventricle repair. Tricuspid atresia was the most common diagnosis (n ؍ 7). The right atrial to right ventricular connection was present in 8 patients; 3 patients had a prior bidirectional cavopulmonary anastomosis. Preoperative median right atrial pressure was 14 mm Hg (range, 12 to 20 mm Hg). Indications for surgery were exercise intolerance, arrhythmias, and conduit obstruction. Echocardiography showed moderate right ventricular hypoplasia, with right atrial to right ventricular regurgitation. The conversion included closure of septal defects, tricuspid valve replacement (n ؍ 8), bidirectional cavopulmonary anastomosis (n ؍ 7), valved right ventricular outflow tract reconstruction (n ؍ 2), and arrhythmia surgery (n ؍ 6). Results. There was no early mortality. There was one reoperation for residual ventricular septal defect. Prolonged chest tube drainage occurred in 2 patients. Postoperative right atrial pressure ranged from 8 to 14 mm Hg (median, 10 mm Hg; p ؍ 0.02). Median follow-up was 8 years. There was no late mortality. Nine patients are in New York Heart Association class I or II, and 1 patient has biventricular failure and was awaiting transplantation. There were two late reoperations, 1 for thrombosis of the mechanical tricuspid valve prosthesis and 1 for obstruction of the valved right ventricular outflow tract conduit. Three patients had recurrent atrial arrhythmias requiring cardioversion. Conclusions. Fontan conversion to one and one half ventricle repair is feasible in selected patients with a failing Fontan circulation. Operation can be performed with low early mortality. Arrhythmia surgery should be performed routinely. Quality of life is excellent.