Preservation of right ventricular structure and function following transatrial-transpulmonary repair of tetralogy of Fallot.

OBJECTIVES: Management strategy of patients with tetralogy of Fallot (TOF)-including timing, as well as surgical technique-remains a controversial topic. We sought to analyse both early and late results of our consistent policy of non-neonatal transatrial/transpulmonary (TA/TP) repair of TOF over almost 14 years, in order to assess preservation or possible deterioration of right ventricular (RV) function. METHODS: All 245 consecutive patients with TOF, referred to our group for repair between September 1997 and December 2010, have been prospectively followed up. Their clinical and echocardiographic data were retrospectively analysed. All underwent complete TA/TP repair at a median age of 1.6 years (range 0.2 to 55.6 years). RESULTS: Follow-up (median 8.5 years, range 0.5 to 14.6 years) was 100% completed. There was no operative death. There were three early re-operations: one for residual right ventricular outflow tract obstruction (RVOTO), one for intractable arrhythmias and one for remote second ventricular septal defect (VSD). There were three non-cardiac-related late deaths. Actuarial survival at 14 years was 98.8% (242/245). Twenty five patients required late re-operation, including 23 patients (9.4%, 23/245) who had pulmonary valve replacement (PVR). All other patients remained asymptomatic at follow-up. Mean residual right ventricular outflow tract (RVOT) pressure gradients have remained stable (6.8 ± 6.6 mmHg, 95% CI 5.9-7.6, early postoperatively, versus 7.6 ± 7.5 mmHg, 95% CI 6.6-8.6 at follow-up; P = 0.015). The mean qualitative grade of pulmonary valve insufficiency (PVI) increased (from 1.4 ± 0.9, 95% CI 1.3-1.5 at discharge to 2.1 ± 1.2, 95% CI 2-2.3 at follow-up; P < 0.001). Similarly, mean qualitative grade of tricuspid valve insufficiency (TVI) also increased (from 1 ± 0.7, 95% CI 0.9-1 at discharge to 1.5 ± 0.8, 95% CI 1.4-1.6 at follow-up; P < 0.001). However, RV function has remained normal in most patients. No significant arrhythmias have been noted. CONCLUSIONS: Our management strategy of non-neonatal TA/TP repair for all patients with TOF is associated with minimal early and late mortality and morbidity, relatively low re-operation rate, preserved RV function and excellent clinical outcomes at follow-up over almost 14 years. Since many patients demonstrated progressive increase in PVI and TVI, a much longer follow-up is necessary to determine the ultimate rates of late re-operation for pulmonary valve replacement (PVR).

The contegra® valved heterograft conduit for right ventricular outflow tract reconstruction: a reliable solution.

INTRODUCTION: The Contegra® bioprosthetic valved conduit, a glutaraldehyde-preserved valve-containing bovine jugular vein graft (Contegra, Medtronic Inc., Minneapolis MN, USA) introduced for clinical trials in 1998, is used for reconstruction of the right ventricular outflow tract (RVOT), mainly in children. This study evaluates our surgical experience with the Contegra® graft, emphasizing the assessment of conduit durability at mid-term follow up. METHODS: The intermediate results of RVOT reconstruction utilizing the Contegra conduit were retrospectively analyzed in a series of 34 consecutive patients (25 male, 9 female), with a mean age of 10.9 ± 11.2 years (range 0.2-46 years). Included were 14 patients with tetralogy of Fallot (TOF) with pulmonary atresia, 11 with reoperation of previously corrected TOF, 5 with truncus arteriosus, 2 with TOF with absent pulmonary valve, 1 reoperation of previously repaired double outlet right ventricle with pulmonary atresia, and 1 undergoing a Ross procedure. Contegra conduit sizes varied in diameter between 12 and 22 mm (mean 18.3 ± 3.2 mm). RESULTS: There were no hospital deaths. There was one early conduit replacement as a result of recurrent thrombosis. Four patients developed early thrombus formation in a valve cusp with complete resolution following anticoagulation therapy. At mean follow up of 85 months (range 6-136 months) and median follow up of 95 months, one patient required Contegra graft explantation in another institution (indications unknown). Freedom from reoperation for Contegra grafts was 94% at 11.4 years. Mean transpulmonary pressure gradients remained low (9.6 ± 5.3 mmHg postoperative, 19.6 ± 10.6 mmHg at follow up). Although there was a clear trend towards worsening of conduit valve insufficiency, this was neither statistically significant nor considered clinically so. CONCLUSIONS: In our experience of 34 consecutive operations, the Contegra® valved conduit for RVOT reconstruction seems to be a reliable alternative to homograft conduits, with promising mid-term freedom from structural deterioration and reoperation.

Impact of age at fontan completion on functional status at mid-term follow up.

INTRODUCTION: Although the Fontan palliation is now being applied to younger patients, the influence of patients' age at the time of surgery on mid- and long-term results remains unclear. It has been our policy to perform Fontan operations when patients are around 5 years old in order to fit a larger conduit and minimize the risk of later obstruction. We sought to investigate whether age at Fontan completion affects subsequent clinical status in patients followed up for more than 3 years. METHODS: We carried out a cross-sectional study of Fontan patients who underwent total cavopulmonary connection between 1997 and 2009 and were followed up for 3 years with detailed chart review, ECG, echocardiogram, and functional status questionnaire. RESULTS: Of the total of 58 patients there were 56 long-term survivors. Of these, 41 had undergone surgery 3 years prior to the time of the study. We were able to contact 37 patients, who comprised our study cohort (mean follow up 6.3, range 3.0-11.0 years). Mean age at operation was 7.4 years (median 5.5, range 3.0-29.5 years). At latest follow up, 97% were in NYHA class I or II. In terms of somatic development, 21% (8/37) for weight and 19% (7/37) for height were below the 25th percentile for age-matched controls. Excluding one patient with developmental delay due to an underlying syndrome, there were 5 patients (13.5%) with mild learning disabilities and one with moderate delays. No additional surgeries had been performed. Tissue Doppler imaging measurements of systolic and diastolic velocities of the lateral annulus of the dominant ventricle revealed abnormalities of both systolic function and diastolic compliance, although qualitatively the systolic function of the single ventricle was considered adequate in all patients. CONCLUSIONS: Although earlier Fontan palliation is considered beneficial in the long term from the viewpoint of exercise capacity and hemodynamics, even late Fontan completion provides acceptable mid-term results in terms of the patients' functional status.

The fontan procedure in Greece: early surgical results and excellent mid-term outcome.

INTRODUCTION: The Fontan operation (modified from its original version) affords excellent palliation for many patients with various forms of anatomic or functional single ventricle. The purpose of this study was to evaluate the outcome of our experience with the Fontan procedure in Greece. METHODS: Fifty-eight consecutive patients with single ventricle physiology had a modified Fontan operation between 1997 and 2009. Their records were reviewed retrospectively. Follow up, which included clinical evaluation and echocardiographic functional assessment, was complete. RESULTS: Median age at operation was 5.7 years (range 3 years to 29.4 years); 46.5% had multistage palliation (stage I and II); 79% had prior bidirectional cavopulmonary shunt (stage II) and 8.6% single stage Fontan. Fifty-four patients had an extracardiac conduit total cavopulmonary connection (EC-TCPC) and 4 an intra-atrial lateral tunnel (LT-TCPC). Fenestration was performed in 26 (44.8%) patients. Operative mortality was 0%. One patient required a short period of extracorporeal membrane oxygenator support. The most frequent complication was prolonged pleural effusion. Median duration of pleural effusion was 17 days (range 6-47 days). Median duration of follow up was 5.44 years (range 0.36 to 11.5 years). There were 2 late deaths (overall mortality 3.4%). One patient died from progressive deterioration of ventricular function within 2 years of operation and the other from fulminant endocarditis. Ten subjects have undergone device closure of a persistent fenestration. All 56 surviving patients are in excellent clinical condition (NYHA class I or II). CONCLUSIONS: We have performed the Fontan procedure over a period of 13 years in Greece with excellent mid- and long-term results. Longer follow up will be necessary to assess the possible incidence of late severe complications, some of which may necessitate heart transplantation.

Surgery for complications of trans-catheter closure of atrial septal defects: a multi-institutional study from the European Congenital Heart Surgeons Association.

OBJECTIVE: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). METHODS: The records of all (n=56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. RESULTS: A wide range of ASD sizes (5-34mm) and devices of various types and sizes (range 12-60mm) were involved, including 13 devices less than 20mm. Complications leading to surgery included embolisation (n=29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n=12), significant residual shunt (n=12), aortic or atrial perforation or erosion (n=9), haemopericardium with tamponade (n=5), aortic or mitral valve injury (n=2) and endocarditis (n=1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% (p=0.001). CONCLUSIONS: Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory.

Mid-term results following surgical treatment of congenital cardiac malformations in adults.

The long term consequences of untreated of residual or recurrent lesions pose unique challenges in the growing population of adults with congenitally malformed hearts. In our unit, 335 patients aged from 18 to 72 years, with a mean age of 35 plus or minus 14 years, presented for correction of congenital cardiac disease from September, 1997, through December, 2006. Of the group, 42 (12.5%) had undergone one or more prior surgical procedures, 3 were admitted as emergencies, and a further 10 (3%) had suffered prior cardiac related complications. Symptoms had been noted by 181 patients (54%), and 42 (12.5%) had an established arrhythmia. Chromosomal anomalies were identified in 13 (3.8%), and diagnostic catheterisation was required in 201 (60%) patients. Of the overall group, 2 patients died early (0.6%). Complications occurred in 61 patients (18%), including atrial fibrillation, pneumothorax, postoperative haemorrhage, pericardial or pleural effusions requiring drainage, stroke, complete heart block, endocarditis, wound dehiscence, and peripheral neuropathy. The median length of stay in the intensive care unit and hospital were 2 and 7 days, respectively. Death occurred later in 2 further patients (0.6%), due to atrial fibrillation and pulmonary hypertension. At mean follow-up of 63 plus or minus 30 months, the majority of the remaining patients are well with resolution or significant improvement in their symptoms. Despite the long term deleterious effects of untreated, residual or recurrent congenital cardiac lesions in adults, therefore, we conclude that surgical correction can be achieved with low mortality and acceptable morbidity. Most significant complications are related to arrhythmias.

Early left ventricular function recovery after trap-door coronary transfer repair of ALCAPA in an adult patient.

Anomalous origin of the left coronary from the pulmonary artery (ALCAPA) is a rare congenital malformation, which may result in myocardial infarction, congestive heart failure, and sudden death if left untreated. Despite frequently advanced pathologic changes, there seems to be significant potential for the recovery of myocardial function in individuals with left ventricular dysfunction after the establishment of physiologic coronary circulation, particularly in the pediatric population. Reports of ALCAPA repair in adulthood are scarce and little information exists regarding the response of the left ventricle to revascularization in this age group. In this report, repair of ALCAPA in a significantly symptomatic adult patient with ventricular dysfunction is described, leading to an early recovery of left ventricular function.

Surgical results after total transatrial/transpulmonary correction of tetralogy of Fallot.

INTRODUCTION: Surgical repair of tetralogy of Fallot is associated with low early morbidity and mortality. However, there may be late morbidity and mortality due to right ventricular dysfunction. The transatrial/transpulmonary technique may ameliorate these long-term complications. Here we present the results from our use of this approach. METHODS: A hundred sixty-three consecutive patients (age 6 months to 45 years, median 1.5 years) underwent transatrial/transpulmonary total correction in our department. In 142 patients the main pulmonary artery was augmented by an autologous pericardial patch, in 31 cases the arterioplasty was extended to the pulmonary artery branches, and pulmonary artery valvuloplasty was needed in 129 patients. A monocusp autologous pericardial valve mechanism was inserted in 14 patients. RESULTS: Patient follow up was 100% complete with a median duration of 3.05 years. There were no deaths. One patient required early reoperation to relieve residual right ventricular outflow tract (RVOT) obstruction. Median ICU and hospital stay were 3 and 11 days, respectively. At hospital discharge RVOT gradient was 13.7 +/- 13 (median 10) mmHg, while most patients (94%) had up to moderate pulmonary valve insufficiency (1 + in 63.8%, 2+ in 30.6%), and normal (92.6%) or mildly reduced (6.1%) right ventricular function. In 81% some degree of tricuspid regurgitation was noted. One patient required late reoperation for mitral valve repair. All patients are in NYHA class I or II. The degree of pulmonary valve insufficiency remains stable (69.9% with 0-1 + and 24.5% up to 2+). Likewise, tricuspid valve function remains unchanged (96% of the patients had mild or up to moderate regurgitation). There was no significant RVOT obstruction and in most patients (93.2%) right ventricular function was normal. CONCLUSION: These results compare very favorably to those reported in the literature. The medium-term findings auger well for future adverse event rates, but long-term follow up is still necessary to confirm them.

Successful surgical correction of congenital heart disease in adults: seven years' experience.

INTRODUCTION: The long term consequences of untreated or residual/recurrent lesions pose unique challenges in the growing population of adults with congenital heart disease (CHD). This study summarizes the results of the surgical treatment of these patients. METHODS: From October 1997 to October 2004, 289 patients with CHD, aged 18-72 (35+/-13.6) years, 143 men and 146 women, presented for surgery to our unit. Thirty eight patients (13.15%) had 1 to 3 prior surgical procedures. Although 137 patients (47%) were asymptomatic (NYHA I), 117 (40.5%) had mild (NYHA II), 34 (11.7%) moderate (NYHA III) and 1 (0.3%) severe (NYHA IV) symptoms. Thirty four patients had an established arrhythmia, requiring treatment in 25 (8.6%). Chromosomal anomalies were identified in 10 (3.4%) and diagnostic catheterization was required in 178 (61.5%) patients. All patients underwent complete surgical correction and in 275 (95%) of them this was performed with cardiopulmonary bypass of 107+/-74 min mean duration. RESULTS: There was 1 early death (0.34%) due to embolic stroke related to atrial fibrillation (AF). Complications occurred in 50 patients (17%) and included re-operation for bleeding (5), stroke (3), pneumothorax (12), AF (22), complete heart block requiring permanent pacemaker implantation (2), wound dehiscence (1), pericardial (7) or pleural (3) effusion requiring drainage and peripheral neuropathy (1). Median intensive care unit and hospital stay was 1 (range 1-10) and 8 (range 5-42) days respectively. Two late deaths (0.7%) occurred in patients with AF and pulmonary hypertension. At mean follow-up of 45+/-24 (range 1-82) months all other patients are well with resolution or significant improvement in their symptoms. CONCLUSION: Despite the long term deleterious effects of CHD in adult patients, surgical correction can be achieved with low mortality and acceptable morbidity. All deaths and most significant complications are related to arrhythmias.