Fate of pulmonary arteries following Norwood Procedure

Objective: This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions. Methods: Between 1993 and 2004, 226 patients underwent Stage II following NP. Ninety-eight patients (43%) had completion of Fontan circulation (Stage III) and a further 107 (47%) are on course for Fontan completion with 21 (9%) inter-stage deaths. During NP, the aortic arch was reconstructed without additional material (n = 91, 40%) or with a pulmonary homograft patch (n = 135, 60%). Pulmonary blood flow was supplied by modified Blalock–Taussig shunt (n = 177, 78%) or right ventricle to pulmonary artery conduit (RV-PA; n = 49, 22%). The CPAs defect was closed directly (n = 69, 31%) or with a patch (n = 157, 69%). Complete resection of coarctation was performed in 126 patients (56%). Results: Ninety-seven patients (43%) required surgical reoperation to CPAs during Stage II. Actuarial freedom from reoperation was 60 ± 3%, 52 ± 4% and 50 ± 4% at 1, 5 and 10 years, respectively. On multivariable analysis, NP with RV-PA increased risk of reoperation (LR 8.3, 5.3–13.2; p < 0.001). Forty-one patients (18%) required catheter-based reintervention on CPAs. Actuarial freedom from reintervention was 98 ± 1%, 72 ± 4% and 58 ± 6% at 1, 5 and 10 years, respectively. CPA problems were almost exclusively limited to the proximal Left pulmonary artery. On multivariable analysis, catheter-based reintervention became more common with time. Complete resection of coarctation increased risk of reintervention (LR 3.9, 1.6–9.6; p < 0.005). Arch reconstruction and CPAs repair techniques did not affect risk of reoperation or reintervention on CPAs. Conclusions: CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP. The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage II procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP.     

Risk factors for reoperation after relief of congenital subaortic stenosis

Background: Congenital subaortic stenosis entails a lesion spectrum, ranging from an isolated obstructive membrane, to complex tunnel narrowing of the left outflow associated with other cardiac defects. We review our experience with this anomaly, and analyze risk factors leading to restenosis requiring reoperation. Methods: From 1994 to 2006, 58 children (median age 4.3 years, range 7 days–13.7 years) underwent primary relief of subaortic stenosis. Patients were divided into simple lesions (n = 43) or complex stenosis (n = 15) associated with other major cardiac defects. Age, pre- and postoperative gradient over the left outflow, associated aortic or mitral valve insufficiency, chromosomal anomalies, arteria lusoria, and operative technique (membrane resection (22) vs associated myectomy (34) vs Konno (2)) were analyzed as risk factors for reoperation (Kaplan–Meier, Cox regression). Results: There was no operative mortality. Median follow-up spanned 2.7 years (range 0.1–10), with one late death at 4 months. Reoperation was required for recurrent stenosis in 11 patients (19%) at 2.6 years (range 0.3–7.5) after initial surgery. Risk factors for reoperation included complex subaortic stenosis (p = 0.003), younger age (p = 0.012), postoperative residual gradient (p = 0.019), and the presence of an arteria lusoria (p = 0.014). For simple lesions, no variable achieved significance for stenosis recurrence. Conclusions: Surgical relief of congenital subaortic stenosis, even with complex defects, yields excellent results. Reoperation is not infrequent, and should be anticipated with younger age at operation, complex defects, residual postoperative gradient, and an arteria lusoria. Myectomy concomitant to membrane resection, even in simple lesions, does not provide enhanced freedom from reoperation, and should be tailored to anatomic findings.     

Factors affecting early and long-term outcomes after completion pneumonectomy

Objective: To identify factors that affect operative mortality and morbidity and long-term survival after completion pneumonectomy. Methods: We retrospectively reviewed the charts of consecutive patients who underwent completion pneumonectomy at our cardiothoracic surgery department from January 1996 to December 2005. Results: We identified 69 patients, who accounted for 17.8% of all pneumonectomies during the study period; 22 had benign disease and 47 malignant disease (second primary lung cancer, n = 19; local recurrence, n = 17; or metastasis, n = 11). There were 50 males and 19 females with a mean age of 60 years (range, 29–80 years). Postoperative mortality was 12% and postoperative morbidity 41%. Factors associated with postoperative mortality included obesity (p = 0.005), coronary artery disease (p = 0.03), removal of the right lung (p = 0.02), advanced age (p = 0.02), and renal failure (p < 0.0001). Preoperative renal failure was the only significant risk factor for mortality by multivariate analysis (p = 0.036). Bronchopleural fistula developed in seven patients (10%), with risk factors being removal of the right lung (p = 0.04) and mechanical stump closure (p = 0.03). Overall survival was 65% after 3 years and 46% after 5 years. Long-term survival was not affected by the reason for completion pneumonectomy. Conclusion: Although long-term survival was acceptable, postoperative mortality and morbidity rates remained high, confirming the reputation of completion pneumonectomy as a challenging procedure. Significant comorbidities and removal of the right lung were the main risk factors for postoperative mortality. Improved patient selection and better management of preoperative renal failure may improve the postoperative outcomes of this procedure, which offers a chance for prolonged survival.     

The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection.

OBJECTIVE: This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology. METHODS: Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n=44, 42%) or right ventricular morphology (n=59, 58%). The TCPC was performed using an extracardiac conduit (n=84, 82%) or a lateral atrial tunnel (n=19, 18%), and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation; early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days-5.2 years). RESULTS: Early mortality was 1.9% (n=2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (+/-1 SEM) was 95.6+/-2.5%. Forty-two patients (41%) had prolonged pleural drainage (> or =14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (+/-1 SEM) were 92.2+/-5.0 and 73.4+/-6.0%, respectively. The Fontan procedure was associated with an improved functional class (P<0.005) and all current survivors (n=99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P<0.05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P<0.05), increased postoperative pulmonary artery pressures (P<0.005) and an unfenestrated Fontan procedure (P<0.01). CONCLUSIONS: In this contemporary series, the modified Fontan procedure was characterised by low early mortality, excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival.     

Long-term experiences on cardiac retransplantation in adults

Background: It remains disputed whether cardiac retransplantation should be performed. This study aimed to evaluate our long-term experiences on cardiac retransplantation in adults. Patients and methods: Between March 1989 and December 2004, 2% (28/1290) of cardiac retransplantations were performed. Results: The reasons for cardiac retransplantation were cardiac allograft vasculopathy (n = 13; 47%), primary graft failure (n = 11; 39%), and refractory acute rejection (n = 4; 14%). The 30-day mortality risk was 29% (acute rejection: 50%; primary graft failure: 36%; cardiac allograft vasculopathy: 15%, p = 0.324), compared to 8.5% for primary cardiac transplantation (p < 0.001). The causes of early death were acute rejection (n = 3; 37%), multiorgan failure (n = 3; 37%), primary graft failure (n = 1; 13%), and right ventricular failure (n = 1; 13%). The late mortality rate was 96/1000 patient-years. The causes of late death were acute rejection (n = 4; 50%), cardiac allograft vasculopathy (n = 2; 25%), multiorgan failure (n = 1; 13%), and infection (n = 1; 13%). The 1-, 5-, 10-, and 15-year survival was respectively 78, 68, 54, and 38% (primary cardiac transplantation), and 46, 41, 32, and 32% (cardiac retransplantation) (p = 0.003). The short-term survival for cardiac retransplantation due to cardiac allograft vasculopathy was likely better than primary graft failure and refractory acute rejection (p = 0.09). Conclusion: The overall outcomes of cardiac retransplantation are significantly inferior to primary cardiac transplantation. Cardiac retransplantation should be only performed for selected patients.     

Persistent vegetative state in electrical injuries: a 10-year review

We reviewed 148 cases of electrical injury admitted to our burn centre. The incidence of persistent vegetative state was 3% (n = 5), higher in the low-voltage (6.7%) than in high-voltage group (1.2%). At the time of trauma, 44% (n = 65) lost consciousness and 50% of these (n = 32) received cardiopulmonary resuscitation on arrival at hospital. Of these, 50% recovered (n = 16), 22% became comatose (n = 7) and 28% (n = 9) died. Of the seven comatose patients, five did not show brain oedema but remained in a persistent vegetative state; this state was more common with low-voltage electrical injuries. The public should be warned of this effect of low-voltage trauma.     

Midterm follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure

Objective: Extracardiac conduit Fontan procedure has some theoretical advantages over other types of Fontan procedures, such as optimized flow dynamics, a lower frequency of arrhythmias, and technical ease of procedure. However, lack of growth potential and thrombogenicity of the artificial conduit is the main concern and can possibly lead to reoperation for the conduit stenosis. In this study, we investigated the change and the status of the Gore-Tex graft used in extracardiac conduit Fontan procedure. Methods: Between 1996 and 2005, 154 patients underwent extracardiac conduit Fontan procedure using Gore-Tex graft. Among these, 46 patients underwent cardiac catheterization during follow-up period. We measured the internal diameter of the conduit and inferior vena cava angiographically. Results: Mean follow-up duration was 36.1 ± 19.7 months. The conduit diameter used was 16 mm in 10 patients, 18 mm in 16, 20 mm in 14, 22 mm in 4, and 24 mm in 2 patients. The mean conduit-to-inferior vena cava cross-sectional area ratio was 1.25 ± 0.33. According to the conduit size used, this ratio was 1.03 ± 0.17 for 16 mm conduits, 1.33 ± 0.37 for 18 mm, 1.33 ± 0.36 for 20 mm, 1.28 ± 0.26 for 22 mm, and 1.05 ± 0.06 for 24 mm conduits (p < 0.05, 16 mm vs 18 mm and 20 mm). The mean percent decrease of the conduit cross-sectional area was 14.3 ± 8.5%, and this did not differ significantly according to the conduit size (p = 0.82). Follow-up duration and the percent decrease of the conduit cross-sectional area did not show significant correlation (r = 0.22, p = 0.14). There was no reoperation due to conduit stenosis. Conclusions: During midterm follow-up of about 3 years, the conduit cross-sectional area decreased by 14%, and this did not differ according to the conduit size used. The extent of decrease of the conduit cross-sectional area remained stable irrespective of the follow-up duration. Sixteen millimeters conduit showed no evidence of clinically significant stenosis, but careful follow-up is warranted because of the possible conduit stenosis relative to the patients’ somatic growth.     

Effect of fenestration on the sub-diaphragmatic venous hemodynamics in the total-cavopulmonary connection

Objective: To understand differences in the sub-diaphragmatic venous physiology between patients with fenestrated and non-fenestrated total-cavopulmonary connections (TCPC). Methods: We studied the effects of respiration, retrograde flow, and gravity on the sub-diaphragmatic venous flows in 20 normal healthy volunteers (control), 25 Fontan patients with non-fenestrated TCPC, and 21 with fenestrated TCPC. Subhepatic inferior vena cava (IVC), hepatic vein (HV), and portal vein (PV) flow rates were measured with Doppler ultrasonography during inspiration and expiration in both supine and upright positions. The supine inspiratory-to-expiratory flow rate ratio was calculated to reflect the effect of respiration, the supine-to-upright flow rate ratio was calculated to assess the effect of gravity, and the magnitude of retrograde flow was evaluated with respect to total antegrade flow. Mean IVC, HV, and wedged hepatic venous (WHV) pressures were measured during cardiac catheterization in four TCPC patients before and after fenestration closure. The transhepatic venous pressure gradient (TVPG) was calculated as the difference between the HV and WHV pressure. Results: Compared with control, HV flow in TCPC was heavily dependent on respiration; this inspiratory capacity was greater in fenestrated than non-fenestrated subjects (inspiratory-to-expiratory flow ratio 1.7, 4.4, and 3.0, respectively P<0.001). Normal retrograde HV flow was diminished in TCPC patients, furthermore, fenestrated subjects had less flow reversal than non-fenestrated (retrograde as percent of antegrade flow 43, 19, and 30%, respectively P<0.001). Gravity decreased IVC and HV flows more in TCPC subjects than control, but this effect was not different between the two TCPC groups. Closure of the fenestration resulted in higher IVC and HV pressures (pre-closure versus post-closure pressures [mmHg]: 11.2±4.0 vs. 12.3±3.9, and 11.5±3.8 vs. 12.4±3.8, respectively P≤0.001). The normal TVPG was reduced in fenestrated TCPC, and worsened after fenestration closure (0.9±0.3 and 0.7±0.4, respectively P<0.04). Conclusions: Fenestration of the inferior venous connection has important influences on sub-diaphragmatic venous return in TCPC patients. Although fenestration lowers venous pressures and partially restores TVPG, its beneficial effects on flow in TCPC patients are mediated primarily by an increase in inspiration-derived forward HV flow and reduced flow reversal. These observations suggest fenestration results in a more efficient and less congested splanchnic circulation in TCPC patients, and may have important implications in the early and late management of Fontan patients.     

The outcome of sternal wire removal on persistent anterior chest wall pain after median sternotomy

Objective: To evaluate the effect of wire removal on a consecutive series of patients with persistent anterior chest wall pain after median sternotomy. Methods: Ninety-five patients receiving sternal wire removal during the period January 1994–October 2001 were included in a follow-up study. Preoperative data, data from the primary operation, the postoperative course, and the sternal wire removal were collected from patient histories. The patients were attempted contacted by telephone, and interviewed about the outcome of the wire removal. Results: Wire removals were performed 2.7 ± 3.3 (SD) years (40 days–20 years) after the primary procedure. Wire removal appeared to occur more frequently in patients with allergies, patients receiving valve-related procedures, and patients that had been reoperated for surgical complications within the first month after the primary operation. In 79 patients, the pain was unrelated to skin affection or infection. Full follow-up was available for 71 patients. For 24 patients only partial follow-up was possible since the patients were dead (n = 19) or unreachable (n = 5). Of the patients followed up, 86% reported complete or partial relief of symptoms, while 11% reported no change in symptoms, and 3% reported worsening of symptoms after wire removal. In patients (n = 23) where symptoms appeared to be related to specific wires, only these were removed, and this approach produced as good results as for patients where all wires were removed. Conclusions: The surgical results were generally good. Sternal wire removal should be offered to patients with persistent anterior chest wall pain after sternotomy, when other serious postoperative complications have been excluded.     

Comparison of on pump and off pump coronary surgery: risk factors for neurological outcome

Objective: Cerebrovascular accidents (CVA) are devastating complications after coronary artery bypass grafting (CABG). The reported incidence of neurological complications after conventional CABG (CCABG) is 3–6%. Off-pump coronary bypass grafting (OPCAB) has been associated in recent studies to a decreased morbidity and risk of perioperative stroke. Nevertheless, uncertainty still surrounds the relative benefits of OPCAB. We investigated whether, in our experience, OPCAB was associated with lower neurological morbidity than conventional CABG approach. Methods: Eight thousand and two patients underwent isolated CABG at our institution between January 1998 and January 2005. OPCAB operation was performed on 1415 patients. Data were prospectively collected. A multiple logistic regression analysis was used to evaluate the influence of the two different surgical techniques on the neurological outcomes. Results: Patients in the OPCAB group were significantly older (66.2 vs 63.5%, p < 0.0001), had a higher incidence of renal injury (5.4 vs 2.4%, p < 0.0001), and were more redo interventions (6.95 vs 1.53%, p < 0.0001). The CCABG patients were more urgent at operation (5.46 vs 3.26, p = 0.0007), were less hypertensive (57.6 vs 63% of the patients, p = 0.0003) more diabetics (22 vs 20.6%, NS), and had an ejection fraction less than 0.40 (10.4 vs 9.6%, NS). CVA incidence was similar in both groups (Type I outcome: OPCAB = 0.70% vs CCABG = 0.68%, p = 0.91; Type II outcome OPCAB = 0.70% vs CCABG = 0.83%, p = 0.63). Conclusions: In our experience patients undergoing CCABG were not exposed to a grater risk of neurological adverse events when compared to OPCAB patients.     

Factors influencing early and late outcome following the Fontan procedure in the current era. The 'Two Commandments'?

OBJECTIVE: This study was undertaken to identify the factors affecting early and late outcome following the Fontan procedure in the current era. We have examined whether conventional selection criteria, the 'Ten Commandments', are still applicable in the current era. MATERIALS AND METHODS: Between January 1988 and July 2004, 406 patients underwent a modified Fontan procedure at a median age of 4.7 years (IQR, 3.8-7.1 years). The single functional ventricle was of left (n=241, 59%) or right ventricular morphology (n=163, 40%). The modified Fontan procedure was performed using an atriopulmonary connection (n=162, 40%) or total cavopulmonary connection (TCPC) involving a lateral atrial tunnel (n=50, 12%) or extracardiac conduit (n=194, 48%). They were fenestrated in 216 patients (53%). RESULTS: The early mortality was 4.4% (n=18) and four other patients required takedown of the Fontan circulation. On multivariable analysis, early outcome was adversely influenced by two factors (p<0.05): preoperative impaired ventricular function and elevated pulmonary artery pressures. Two risk models were constructed for early outcome based on preoperative and predictable operative variables (Model 1) and all preoperative and operative data (Model 2). Both models were calibrated across all deciles (p=0.83, p=0.25) and discriminated well. The area under the ROC curve was 0.85 and 0.89, respectively. There were 21 late deaths, 1 patient required late takedown of the Fontan circulation and 3 required orthotopic cardiac transplantation. Actuarial survival was 90+/-2%, 86+/-2% and 82+/-3% at 5, 10 and 15 years, respectively. Multivariable analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. The freedom from reintervention was 83+/-4%, 76+/-4% and 74+/-8% at 5, 10 and 15 years, respectively. Additional risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. CONCLUSIONS: Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.     

The impact of mitral valve surgery combined with maze procedure

Objective: Recent studies indicated that successful maze procedure for atrial fibrillation (AF) adjunct to mitral valve surgery provided a lower incidence of stroke and recurrence of AF. The purpose of this study is to review the 13-year experience of these combined procedures and to identify the risk factors and late outcomes of successful maze procedures compared to failed maze procedures. Methods: At a single institution, 521 consecutive patients underwent combined maze procedures with mitral valve replacements or valvuloplasties. Three kinds of maze techniques were primarily used: Cox–maze III, Kosakai maze, and cryo-maze procedure. Three months after the operation, 394 patients were in sinus rhythm (Group S) while the remaining 116 patients were in continuous or intermittent AF (Group F), excluding 11 early death patients. Risk factors for Group F were determined by the analysis of all patient demographics. Survival, freedom from stroke, cardiac events, and AF recurrence were analyzed. Results: The proportion of the patients without any other simultaneous procedures was greater in Group S (41% vs 29%, P = 0.02). The distributions of mitral valve surgery and maze procedure techniques were similar in these two groups. A left atrium larger than 70 mm [hazard ratio (HR) = 2.6; 95% confidence interval range 1.04–6.3, P = 0.043], preoperative AF history longer than 10 years (HR = 8.2; 4.5–15.1, P < 0.001) and f-wave voltage in V1 smaller than 0.1 mV (HR = 6.2; 5.0–15.2, P < 0.001) were determined to be risk factors for unsuccessful maze procedures. All the results of Cox proportional hazards models showed superiority in Group S; actuarial survival rates (HR = 2.7; 1.04–7.0, P = 0.035), freedoms from stroke (HR = 3.0; 1.1–8.1, P = 0.003) and cardiac events (HR = 4.3; 2.9–6.1, P < 0.001). Freedom from AF recurrence rate was 98.4% at 5 years and 81.0% at 12 years in Group S, and 73.0% and 60.1% in overall patients. Conclusions: Patients with successful maze procedures resulted in higher survival rate, greater freedom from stroke and cardiac events. The large left atrium, small f-wave, and long AF duration were significant risk factors for failed maze procedures, suggesting that earlier surgical interventions would result in superior results in mitral valve surgery combined with maze procedure.     

Effects of hyperbaric oxygen therapy on fibrovascular ingrowth in porous polyethylene blocks implanted under burn scar tissue: an experimental study

Effects of hyperbaric oxygen (HBO) therapy on biointegration of porous polyethylene (PP) implanted beneath dorsal burn scar and normal skin were experimentally examined in Sprague–Dawley rats. In Group One (n = 20), daily HBO treatments were given after the implantation of PP material under dorsal burn scar, whereas, in Group Two (n = 20) no treatment was given following the same surgical procedure. In Group Three (n = 20), PP was placed under dorsal normal skin and subsequently HBO therapy protocol was applied while Group Four (n = 20) stayed without HBO treatment after the implantation. One, 2, 3 and 4 weeks after the implantations, sections were respectively taken from five rats from each group. Biointegration process and effects of HBO therapy were evaluated microscopically and the ratio of fibrovascular ingrowth (FVI) was determined for each rat. The results showed significantly superior FVI in Group One compared to Group Two and again FVI into PP under normal skin treated with HBO revealed better results against Group Four (p < 0.05). Well-vascularized capsule formation and tissue integration was delayed both in Group Two and in Group Three in the first 3 weeks. In conclusion, HBO therapy enhances biointegration of PP in hypoxic burn scar areas via improving collagen synthesis and neovascularization; otherwise, it apparently delays tissue ingrowth into porous structure implanted in normal healthy tissues.     

Jugular venous valved conduit (Contegra) matches allograft performance in infant truncus arteriosus repair

Objective: Limited availability and durability of allograft conduits require that alternatives be considered. We compared bovine jugular venous valved (JVV) and allograft conduit performance in 107 infants who survived truncus arteriosus repair. Methods: Children were prospectively recruited between 2003 and 2007 from 17 institutions. The median z-score for JVV (n = 27, all 12 mm) was +2.1 (range +1.2 to +3.2) and allograft (n = 80, 9–15 mm) was +1.7 (range −0.4 to +3.6). Propensity-adjusted comparison of conduit survival was undertaken using parametric risk-hazard analysis and competing risks techniques. All available echocardiograms (n = 745) were used to model deterioration of conduit function in regression equations adjusted for repeated measures. Results: Overall conduit survival was 64 ± 9% at 3 years. Conduit replacement was for conduit stenosis (n = 16) and/or pulmonary artery stenosis (n = 18) or regurgitation (n = 1). The propensity-adjusted 3-year freedom from replacement for in-conduit stenosis was 96 ± 4% for JVV and 69 ± 8% for allograft (p = 0.05). The risk of intervention or replacement for branch pulmonary artery stenosis was similar for JVV and allograft. Smaller conduit z-score predicted poor conduit performance (p < 0.01) with best outcome between +1 and +3. Although JVV conduits were a uniform diameter, their z-score more consistently matched this ideal. JVV exhibited a non-significant trend towards slower progression of conduit regurgitation and peak right ventricular outflow tract (RVOT) gradient. In addition, catheter intervention was more successful at slowing subsequent gradient progression in children with JVV versus those with allograft (p < 0.01). Conclusions: JVV does match allograft performance and may be advantageous. It is an appropriate first choice for repair of truncus arteriosus, and perhaps other small infants requiring RVOT reconstruction.     

Surgical reinterventions following the Fontan procedure.

OBJECTIVE: The Fontan procedure is utilized as a final reconstructive procedure for patients with functional single ventricle. Short- and long-term outcomes have improved significantly, however, some patients require additional cardiac procedures following the Fontan operation. The outcomes for these reinterventions are not known. METHODS: Cardiac Surgery and Cardiac Intensive Care Unit databases at The Children's Hospital of Philadelphia were reviewed to identify all patients who underwent cardiac surgery after a previous Fontan operation between January 1, 1995 and December 31, 2001. RESULTS: During the study period, 123 procedures were performed in 71 patients. The median time from Fontan to reoperation was 3.6 years (range 0.1-20 years). Indications for reintervention included arrhythmia, cyanosis, 'failing' Fontan circulation or exercise intolerance, protein losing enteropathy, atrioventricular valve (AVV) regurgitation, and other indications. Procedures included pacemaker insertion or revision (n = 59, 48%), reinclusion of previously excluded hepatic veins (n = 16, 13%), revision to either a lateral tunnel or extra-cardiac conduit Fontan (n = 13, 11%), cardiac transplantation (n = 9, 7%), enlargement or creation of a baffle fenestration (n = 6, 5%), isolated AVV repair or replacement (n = 2, 2%), and other procedures (n = 18, 14%). There were five early and five late deaths. Hospital mortality was greatest for patients undergoing cardiac transplantation (4/9, 44%), accounting for 80% of the early deaths. CONCLUSIONS: Surgical reinterventions following the Fontan procedure may be necessary for multiple indications which result in impairment of the Fontan circulation. Most reinterventions can be performed with minimal morbidity and mortality. Survival for patients requiring cardiac transplantation following the Fontan procedure remains poor.     

Long-term results of atriopulmonary fontan connection for complex cardiac anomalies

To evaluate the long-term results of atriopulmonary Fontan connection, we reviewed the outcome of 20 consecutive patients (single ventricle 8, double outlet right ventricle 8, tricuspid atresia 3, mitral atresia 1; mean age, 13 +/- 11) who underwent this operation between 1981 and 1997 at our institution. Glenn shunt preceded Fontan operation in 1 patient. Four patients had a concomitant bidirectional Glenn anastomosis at the time of the Fontan operation. Follow-up was 100% complete, and total cumulative follow-up of all patients was 241 patient-years (maximum, 20 years). Three hospital deaths and 5 late deaths were observed, resulting in 58 +/- 11% of cumulative survival at 20 years after the operation. Sudden death probably due to arrhythmias was the major cause of the late death, and occurred in 3 patients. Reoperation was required in 5 patients (2%/patient-year) at 1 to 17 years postoperatively. Re-closure of an atrioventricular valve was performed for failure of the valve closure in 3 patients at 1 to 6 years postoperatively. Reconstruction of the stenotic atriopulmonary connection (APC) was concomitantly required in 2 of the 3 patients. Pacemaker implantation was required for bradycardia with atrial fibrillation in 1 patient at 17 years. One patient with atrial flutter developed intraright atrial thrombosis at 12 years after the initial Fontan operation, and this patient underwent conversion to extracardiac conduit cavopulmonary connection with right atrial maze procedure. Atrial tachyarrhythmias are most common complications, and occurred in 12 patients (5.0%/patient-year), resulting in 40 +/- 13% of the cumulative free rate at 15 years after the operation. Thus, failure of the atrioventricular valve closure and stenosis of APC were important early to intermediate complications after the atriopulmonary Fontan connection. On the other hand, atrial tachyarrhythmias were inevitable, and were most common late complications leading to intra-right atrial thrombosis, which was successfully resolved by conversion of the conventional atriopulmonary Fontan anastomosis to extracardiac conduit cavopulmonary connection.     

Long-term results of the lateral tunnel Fontan operation

OBJECTIVES: Completion of a total cavopulmonary anastomosis with an intra-atrial lateral tunnel is known to yield good early and midterm results. In this study, we sought to determine the long-term outcome (10 years) after a lateral tunnel Fontan procedure. METHODS: Between October 1987 and December 1991, 220 patients (aged 11 months to 32 years) with a wide range of underlying diagnoses underwent a fenestrated or nonfenestrated lateral tunnel Fontan procedure at our institution. Current follow-up information was available for 196 patients (94%, mean follow-up = 10.2 +/- 0.6 years). Risk factor analysis included patient-related and procedure-related variables, with death, failure, and bradyarrhythmia or tachyarrhythmia as outcome parameters. RESULTS: There were 12 early deaths (<30 days or hospital death), 7 late deaths, 4 successful takedown operations, and 4 heart transplantations. Kaplan-Meier estimated survival was 93% at 5 years and 91% at 10 years, and freedom from failure was 90% at 5 years and 87% at 10 years. Freedom from new supraventricular tachyarrhythmia was 96% at 5 years and 91% at 10 years; freedom from new bradyarrhythmia was 88% at 5 years and 79% at 10 years. Three patients had evidence of protein-losing enteropathy. Multivariable risk factors for development of supraventricular tachyarrhythmia included heterotaxy syndrome, atrioventricular valve abnormalities, and preoperative bradyarrhythmia. Risk factors for bradyarrhythmia included systemic venous anomalies. The sole risk factor for late failure was a previous coarctation repair. CONCLUSION: The lateral tunnel Fontan procedure results in excellent long-term outcome even when used in patients with diverse anatomic diagnoses. The incidence of atrial tachyarrhythmia is low and mainly depends on the underlying cardiac morphology and preoperative arrhythmia. The good long-term outcome after an intracardiac lateral tunnel Fontan procedure should serve as a basis for comparison with other surgical alternatives.     

Management of congenital tracheal stenosis in infancy

Objective: Congenital tracheal stenosis (CTS) is a very infrequent malformation. Till recently, the outlook for these patients was dismal because medical management was the only way of treatment. Surgical and endoscopical techniques developed in the last years have improved the prognosis. We review the short- and long-term outcomes of a single institution experience in the management of children with CTS, comparing different treatment modalities. Methods: Between 1991 and 2004, 19 cases of CTS have been managed in our Unit. Respiratory symptoms varied from mild stridor on exertion to severe distress. Bronchoscopy was performed for diagnostic purposes in all cases; other imaging techniques (computed tomography (CT), magnetic resonance imaging (MRI), bronchography, angiography, doppler-ultrasound) were performed on an individual basis. According to clinical and endoscopical features, patients were classified into three groups. The following data have been studied in each case: sex, age at diagnosis and treatment, anatomical type, associated anomalies, treatment modality, complications, outcome and time of follow-up. Results: Ten boys and nine girls have been included in this study. Age at diagnosis ranged from 3 days to 7 years (median, 4 months) and 84% of cases showed associated anomalies. Five patients presented mild or no symptoms and have been managed expectantly. The other 14 cases were operated on because of persistent or severe clinical symptoms. The following procedures were performed: slide tracheoplasty (n = 7), costal cartilage tracheoplasty (n = 5), tracheal resection and reconstruction (n = 3), endoscopical dilatation (n = 3), stent placement (n = 1), and laser resection (n = 1). Three patients required two or more procedures and surgical survival rate is 78%. Overall mortality in the series is 21% and all survivors (15 patients) are asymptomatic or show mild symptoms with respiratory infections only. Follow-up is complete, ranging from 8 months to 12.3 years (mean, 5 years). Conclusions: Bronchoscopy is our preferred diagnostic tool. Selection of the type of treatment depends on the patient's clinical status and the anatomical pattern of the stenosis. In symptomatic cases with short-segment stenosis (<30% of total tracheal length), we prefer tracheal resection with end-to-end anastomosis; for long-segment stenosis (>30%), slide tracheoplasty is our procedure of choice.     

Paralysis of the phrenic nerve as a risk factor for suboptimal Fontan hemodynamics.

OBJECTIVE: The introduction of the Fontan operation for single ventricle physiology was based on the dual principle of the pulmonary blood flow. It is postulated that normal breathing movements are necessary for passive blood flow into the lungs. We compared patients with and without palsy of the phrenic nerve regarding the sufficiency of Fontan hemodynamics. METHODS: We analyzed 85 consecutive patients, who were available for follow-up after completion of their total cavopulmonary connection (TCPC) between February 1992 and February 2003. The median age at TCPC completion was 4.3 (range 1.3-37) years. Sixty were operated on with an extracardiac conduit and 25 with a lateral tunnel. Fifty patients underwent postoperative heart catheterization with contrast angiography. The diagnosis of diaphragm paralysis was made using echocardiography, fluoroscopy and X-ray examination. Surgical diaphragm plication was performed in 13 patients (Four before and nine after Fontan operation) at a median of 2.2 years after the diagnosis. RESULTS: Twenty-one patients developed fixed palsy of the phrenic nerve during a total of 225 operations before and including completion of TCPC. There were no differences in the incidence of phrenic nerve paralysis between small children (aged <3 years) and older patients or between patients with the extracardiac and intracardiac Fontan procedures. There were no differences in the duration of mechanical ventilation. However, prolonged pleural effusions and a hospital stay of longer than 2 weeks were noted more frequently in patients with palsy (P<0.05). During the median follow-up of 4.6 (range: 0.7-11.4) years significantly more patients with phrenic nerve palsy developed chronic ascites compared to those without palsy (8 of 20 vs. 2 of 65; P<0.001). CONCLUSIONS: Phrenic nerve palsy was recognized as a risk factor for suboptimal Fontan hemodynamics due to the hindrance of passive venous blood flow. Patients with phrenic nerve palsy have a longer hospital stay and a higher incidence of prolonged pleural effusions and of chronic ascites, than those without. Early diaphragm plication may be favorable to optimize the Fontan circuit in these patients. Completion of the TCPC in patients with diaphragm paralysis should be viewed critically.     

Prognostic significance and correlations of neurohumoral factors in early and late postoperative period after Fontan procedure

We focused on neurohumoral activity and its clinical correlates early and late after fenestrated, lateral intra-atrial total cavopulmonary connection (TCPC). Between 2007 and 2010, we prospectively studied 28 early and 48 late postoperative TCPC patients. Plasma concentrations of vasopressin, endothelin-1, proBNP, proANP were determined. We reviewed clinical data to determine relationship between neurohumoral activation and clinical status after TCPC. There was a significant influence of preoperative ventricular end-diastolic pressure (VEDP) (P=0.008) and vasopressin concentration (P=0.02) on the appearance of prolonged pleural effusions. A significant correlation between a combined predictor (a product of preoperative vasopressin concentration and VEDP) and time of effusions (r=0.59, P=0.006) was found. The mean respiratory equivalent of carbon dioxide at peak exercise (VE/VCO(2peak)) was significantly lower in patients operated before the second year of life compared to patients operated after two years of age (27.5±1.39 vs. 48.6±3.86; P=0.039). There was a significant correlation of endothelin-1 (r=0.84; P=0.008) and proBNP (r=0.88; P=0.02) concentrations with VE/VCO(2peak). The prolonged postoperative pleural effusions can be predicted based on the product of preoperative vasopressin concentration and VEDP. Exercise performance is related to the age at TCPC. Endothelin-1 and proBNP can be useful for identification of high-risk Fontan patients.