-
[show abstract]
[hide abstract]
ABSTRACT: BACKGROUND: Severe tracheal stenosis is a rare life-threatening condition that often requires early surgical intervention. The management of this anomaly has been associated with significant mortality and morbidity. We describe our experience with repair of this condition. METHODS: From 1986 to 2011, 20 patients underwent repair of tracheal stenosis at the Royal Children's Hospital (median age 4.9 months) and were retrospectively reviewed. RESULTS: Tracheal repair techniques used were as follows: patch tracheoplasty (n = 8; 40%, 8 out of 20), slide tracheoplasty (n = 7; 35%, 7 out of 20), end-to-end anastomosis (n = 5; 25%, 5 out of 20). Six patients (30%, 6 out of 20) had coexisting congenital intracardiac anomalies. There were 12 pulmonary artery sling (60%, 12 out of 20) patients. Overall operative mortality was 15% (n = 3; 3 out of 20). Operative mortality was 20% (n = 2; 2 out of 10) from 1986 to 2001 and decreased to 10% (n = 1; 1 out of 10) from 2002 to 2011. All early deaths occurred in patients who had undergone patch tracheoplasty. Since 2004, there were no operative deaths. Seven patients (35%, 7 out of 20) required tracheal reintervention postoperatively. There were three late deaths (17.6%, 3 out of 17) at 8, 9 and 22 months after surgery. At last follow-up (mean 5.3 ± 6.6 years; range 1 month to 18 years), all 14 survivors remained asymptomatic. CONCLUSIONS: Repair of tracheal stenosis in children has been associated with high morbidity and mortality. Since the introduction of slide tracheoplasty, a multidisciplinary team approach and abandonment of patch tracheoplasty, the mortality has been reduced. Survival beyond 2 years after surgery is associated with an excellent outcome.
ANZ Journal of Surgery 04/2013; · 1.25 Impact Factor
-
Bryn O Jones,
Salvatore Pepe,
Freya L Sheeran,
Susan Donath,
Pollyanna Hardy,
Lara Shekerdemian,
Daniel J Penny,
Ian McKenzie,
Stephen Horton,
Christian P Brizard, Yves d'Udekem,
Igor E Konstantinov,
Michael M H Cheung
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE: The myocardial protective effect of remote ischemic preconditioning has been demonstrated in heterogeneous groups of patients undergoing cardiac surgery. No studies have examined this technique in neonates. The present study was performed to examine the remote ischemic preconditioning efficacy in this high-risk patient group. METHODS: A preliminary, randomized, controlled trial was conducted to investigate whether remote ischemic preconditioning in cyanosed neonates undergoing cardiac surgery confers protection against cardiopulmonary bypass. Two groups of neonates undergoing cardiac surgery were recruited for the present study: patients with transposition of the great arteries undergoing the arterial switch procedure and patients with hypoplastic left heart syndrome undergoing the Norwood procedure. The subjects were randomized to the remote ischemic preconditioning or sham control groups. Remote ischemic preconditioning was induced by four 5-minute cycles of lower limb ischemia and reperfusion using a blood pressure cuff. Troponin I and the biomarkers for renal and cerebral injury were measured pre- and postoperatively. RESULTS: A total of 39 neonates were recruited-20 with transposition of the great arteries and 19 with hypoplastic left heart syndrome. Of the 39 neonates, 20 were randomized to remote ischemic preconditioning and 19 to the sham control group. The baseline demographics appeared similar between the randomized groups. The cardiopulmonary bypass and crossclamp times were not significantly different between the 2 groups. The troponin I levels were not significantly different at 6 hours after cardiopulmonary bypass nor were the postoperative inotrope requirements. Markers of renal (neutrophil gelatinase-associated lipocalin) and cerebral injury (S100b, neuron-specific enolase) were not significantly different between the 2 groups. CONCLUSIONS: Our data suggest that remote ischemic preconditioning in hypoxic neonates undergoing cardiopulmonary bypass surgery does not provide myocardial, renal, or neuronal protection. Additional studies are needed to examine the relationships among developmental age, hypoxia, and the molecular mechanisms of ischemic preconditioning.
The Journal of thoracic and cardiovascular surgery 02/2013; · 3.41 Impact Factor
-
The Annals of thoracic surgery 02/2013; 95(2):774-5. · 3.74 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The Ross procedure in children carries substantial mortality and reoperation rate. Aortic root dilatation is of concern. To prevent dilatation of the neoaortic root, but permit normal growth, we began to apply an absorbable poly-(p-dioxanone)-filaments (PDS) band at the sino-tubular (ST)-junction.
All children (n=100) who underwent Ross procedure during 1995-2012 were studied. Mean age at operation was 8.6±6.1 years (median 8.3 years, range 3 days to 18 years); 19 patients were younger than 1 year of age. The root replacement (n=91, Ross-Konno procedure in 29 patients), root inclusion (n=6), and subcoronary implantation (n=3) techniques were used. Operative mortality was 6% (6/100, 4 neonates, 2 infants). Age of <1-year at time of operation was a risk factor for early death (P<0.001). Mean follow-up time was 7.0±4.8 years (median 7.4 years, range 5 days to 16 years). Late mortality was 4.3% (4/94). Freedom from moderate or greater neoaortic valve insufficiency (AI) at 5 and 10 years was 89% and 83%, respectively. Freedom from neoaortic valve reoperation at 5 and 10 years was 96% and 86%, respectively. Aortic dilatation to Z-score >4 was greatest at the ST-junction (23%, 11/48) compared to the aortic annulus (17%, 11/66) and sinuses (14%, 7/50). Since 2001, a PDS band was placed around the ST-junction in 19 patients. Survivors with the PDS band had less AI (0 versus 20%, P=0.043) compared to survivors (n=35) without the PDS at 4.1±3 years.
The Ross procedure in children can be performed with acceptable results. Children younger than 1 year of age have higher mortality, but not an increased autograft reoperation rate. Stabilization of the ST-junction may reduce AI.
Journal of the American Heart Association. 01/2013; 2(2):e000153.
-
[show abstract]
[hide abstract]
ABSTRACT: Our previous randomized controlled trial demonstrated cardiorespiratory protection by remote ischemic preconditioning (RIPC) in children before cardiac surgery. However, the impact of RIPC on myocardial prosurvival intracellular signaling remains unknown in cyanosis. RIPC may augment phosphorylated protein signaling in myocardium and circulating leukocytes during tetralogy of Fallot (ToF) repair.
Children (n=40) undergoing ToF repair were double-blind randomized to RIPC (n=11 boys, 9 girls) or control (sham RIPC: n=9 boys, 11 girls). Blood samples were taken before, immediately after, and 24 hours after cardiopulmonary bypass. Resected right ventricular outflow tract muscle and leukocytes were processed for protein expression and mitochondrial respiration. There was no difference in age (7.1±3.4 versus 7.1±3.4 months), weight (7.7±1.8 versus 7.5±1.9 kg), or bypass or aortic cross-clamp times between the groups (control versus RIPC, mean±SD). No differences were seen between the groups for an increase in the ratio of phosphorylated to total protein for protein kinase B, p38 mitogen activated protein kinase, signal transducer and activator of transcription 3, glycogen synthase kinase 3β, heat shock protein 27, Connexin43, or markers associated with promotion of necrosis (serum cardiac troponin I), apoptosis (Bax, Bcl-2), and autophagy (Parkin, Beclin-1, LC3B). A high proportion of total proteins were in phosphorylated form in control and RIPC myocardium. In leukocytes, mitochondrial respiration and assessed protein levels did not differ between groups.
In patients with cyanotic heart disease, a high proportion of proteins are in phosphorylated form. RIPC does not further enhance phosphorylated protein signaling in myocardium or circulating leukocytes in children undergoing ToF repair.
URL: (http://www.anzctr.org.au/trial_view.aspx?id=335613. Unique identifier: Australian New Zealand Clinical Trials Registry number ACTRN12610000496011.
Journal of the American Heart Association. 01/2013; 2(3):e000095.
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVES: To determine rate of reoperation subsequent to primary valve repair in a pediatric population. METHODS: Between 1996 and 2009, 142 consecutive patients underwent aortic valve repair in our institution. Median age at surgery was 9 years, with 30 being younger than age 1 year. Indication for surgery was stenosis (n = 76), regurgitation (n = 55), and both (n = 11). Forty-six patients underwent repair with no addition of patch, whereas 96 patients required addition of patches of glutaraldehyde preserved autologous pericardium for cusp extension (n = 51) and other repair (n = 45). RESULTS: In the early postoperative period after cusp extension repair, 2 patients had a sudden unexplained death and 1 had cardiac arrest requiring mechanical support and heart transplantation. Two additional patients with cusp extension displayed signs of coronary ischemia. After a mean follow-up of 3.4 ± 3.5 years, only 1 patient died of a noncardiac cause. Seven-year freedom from reoperation was 80% (95% confidence interval [CI], 66-89). By multivariate analysis, the only predictors of reintervention were cusp extension (hazard ratio [HR], 5.4; 95% CI, 1.7-16.8; P = .004) and infants (HR, 5.6; 95% CI, 1.7-18.4; P = .005). At final echocardiography follow-up, 23 of 119 survivors without reoperation had moderate (19%), 1 had moderate-severe (1%), and 1 had severe regurgitation (1%), whereas 12 (10%) had a moderate degree of stenosis. CONCLUSIONS: Aortic valve repair in pediatric populations is effective in postponing reintervention. The longevity of the repair is shorter after cusp extension and when performed in infants. Caution should be used when performing tricsupidization and cusp extension of bicuspid valves because it can be responsible for mortality related to occlusion of the coronary ostia by patches.
The Journal of thoracic and cardiovascular surgery 12/2012; · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: BACKGROUND: We have adopted the extra-anatomic bypass graft as the procedure of choice for the treatment of coarctation and aortic arch hypoplasia in the adult-sized patient. However, we have experienced prolonged chest drainage and have decided to investigate this complication and the morbidity related to this procedure. METHODS: Between 1996 and 2010, 15 extra-anatomic bypass grafts of the aorta were performed in 14 patients. Their hospital records and follow-up data were retrospectively reviewed and compared with those of 14 consecutive patients operated with other conventional techniques over the same time period. RESULTS: There was no hospital mortality. After the extra-anatomic bypass procedure, patients had longer hospital stay because of prolonged pleural effusions. Four patients developed complications related to persistent effusions leading to reinterventions, which led to mediastinitis in 2 instances. At last follow-up, 2 of 14 patients with extra-anatomic bypass remained hypertensive, while 8 of the 14 patients who underwent other types of repair had arch obstruction, were hypertensive, or both. CONCLUSIONS: In the adult-sized patient extra-anatomic bypass of the aortic arch relieves arch obstruction more effectively than conventional techniques. However, this technique is fraught with complications related to prolonged effusion drainage that may lead to mediastinitis and reintervention. Its indication should be weighted carefully.
The Annals of thoracic surgery 11/2012; · 3.74 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVES
Late outcomes of repair of coarctation with arch hypoplasia have not yet been described. Hypertension and arch reobstruction frequently occur after standard coarctation repair and thus we sought to determine the long-term results of repair in the subset of patients with arch hypoplasia at a single institution over a 20-year period.METHODS
We reviewed the files of the 305 consecutive patients quoted to have arch hypoplasia who had undergone a coarctation repair in a single institution between 1984 and 2004. Repair was performed through a sternotomy in 74 patients (24%), 58 of them undergoing a repair consisting of an end-to-side anastomosis.RESULTSEarly mortality was 9% but there was only 1 death among patients without a major associated anomaly. Eight patients required reintervention before discharge due to residual obstruction. The follow-up was available in 96% of the patients. Only 45% of the patients over 15 years of age had cardiology review in the last 2 years. Survival at 10 and 20 years was 94% (95% CI: 91-97%) and 92% (95% CI: 86-95%), respectively. There were a total of 66 late reinterventions in 49 (18%) patients. Ten- and 20-year freedom from reintervention was 84% (95% CI: 78-88%) and 72% (95% CI: 63-80%), respectively. Ten- and 20-year freedom from reobstruction was 75% (95% CI: 69-80%) and 45% (95% CI: 34-55%), respectively. Patients undergoing end-to-side repair from sternotomy had less arch reobstruction than those undergoing extended end-to-end repair by thoracotomy (92 vs 61% freedom from reobstruction at 10 years, P < 0.001). Only 68% of the patients were normotensive at the last follow-up. Arch obstruction on echocardiogram was associated with an increased prevalence of hypertension (P = 0.018).CONCLUSIONS
After coarctation repair, half of the patients with hypoplasia of the transverse arch will develop arch reobstruction and a third will become hypertensive. The technique of end-to-side repair performed through a sternotomy seems to alleviate these issues, and could be offered to a larger proportion of patients with hypoplasia of the aortic arch. Many of these patients are lost to follow-up during adolescence, at a time when ongoing care seems mandatory.
Interactive cardiovascular and thoracic surgery 10/2012;
-
[show abstract]
[hide abstract]
ABSTRACT: To determine by 24-hour blood pressure monitoring the risk of hypertension late after coarctation repair in patients with arch hypoplasia.
Sixty-two of 116 consecutive patients (age, ≥10 years) who had coarctation repair and were quoted subjectively by the surgeon or the cardiologist to have arch hypoplasia at the time of the repair underwent a transthoracic echocardiogram and 24-hour blood pressure monitoring. Median age at repair was 11 days (range, 6-48 days). Mean preoperative z score of the proximal transverse arch was -2.43 ± 0.46. Eight patients had a repair via sternotomy (6 end-to-side anastomoses, 2 patch repairs) and 54 had a conventional repair via thoracotomy.
After a follow-up of 18 ± 5 years, 27% of the patients (17/62) had resting hypertension and 60% (37/62) had abnormal ambulatory blood pressure. Sensitivity of high resting blood pressure in detecting an abnormal 24-hour ambulatory blood pressure was 41%. Twenty patients had arch obstruction at last follow-up. Eighteen of them (90%) had abnormal ambulatory blood pressure. None of the patients operated on with end-to-side repair via sternotomy had reobstruction compared with 33% (18/54) of those repaired via thoracotomy.
Patients with a hypoplastic arch operated via thoracotomy have an alarming prevalence of hypertension. Regular follow-up with 24-hour ambulatory blood pressure monitoring is warranted, especially in patients who have had a smaller aortic arch at the time of the initial operation.
The Journal of thoracic and cardiovascular surgery 09/2012; 144(5):1110-8. · 3.41 Impact Factor
-
The Journal of thoracic and cardiovascular surgery 09/2012; 144(5):1269-71. · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge. It is associated with a high mortality. We aimed at determining a method for risk stratification of this group of patients.
From 1994 to 2008, 54 patients underwent simple TAPVD operations during the first month of life. Mean pulmonary arterial pressure (PAP), mean systemic arterial pressure (MAP), systolic blood pressure, diastolic blood pressure, central venous pressure, and left atrial pressure were recorded in 44 of the 54 patients for the first 36 hours postoperatively. The remaining 10 patients were excluded because data from invasive pressure monitoring were not available.
There were overall 8 deaths (18.2%, 8/44), including 4 (9%, 4/44) early deaths, and 5 reoperations (11.4%, 5/44). The mean PAP was 23.1 ± 6.4 mm Hg, the mean MAP was 50.3 ± 5 mm Hg, and the PAP-to MAP-ratio (PAP/MAP) was 0.80 ± 0.36. By multivariable logistic analysis, the risk factors for mortality were a higher PAP/MAP (p = 0.037) and lower operative weight (p = 0.02). All deaths had either a PAP/MAP of greater than 0.80 or an operative weight of less than 2.5 kg. Hemodynamic index (PAP/MAP divided by operative weight) was predictive of mortality (p = 0.007). Furthermore, the hemodynamic index (p = 0.003) predicted prolonged length of stay in the intensive care unit by regression analysis.
The hemodynamic index (PAP/MAP/weight) ≥0.25 in the first 36 hours after TAPVD repair in neonates is predictive of mortality. A higher index predicted longer stay in the intensive care unit. This hemodynamic index may be a useful adjunct for risk stratification in neonates undergoing TAPVD repair.
The Annals of thoracic surgery 08/2012; 94(5):1584-7. · 3.74 Impact Factor
-
The Journal of thoracic and cardiovascular surgery 06/2012; 144(4):980-1. · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVES: Pulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical intervention. We describe our experience with repair of this condition. METHODS: From 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Children's Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients. RESULTS: Operative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 ± 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic. CONCLUSIONS: Children with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis.
The Journal of thoracic and cardiovascular surgery 06/2012; · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 9-month-old infant presented with transposition of the great arteries and intact ventricular septum. His left ventricle was thin and deconditioned. He underwent an arterial switch operation and was electively supported with a left ventricular assist device for 8 days. He subsequently made a full recovery. Elective use of left ventricular support allows an extension of the age limit for the arterial switch operation.
Asian cardiovascular & thoracic annals 06/2012; 20(3):333-4.
-
[show abstract]
[hide abstract]
ABSTRACT: Outcomes after atrioventricular (AV) valve operations in patients with functional single ventricles are unclear.
From 1988 to 2010, 76 consecutive patients with single ventricles underwent AV valve operations for regurgitation at a single institution. Five replacements, 66 repairs, and 5 valve closures were performed at a median age of 1 year (range, 1 day-14 years) on 43 tricuspid, 9 mitral, and 24 common AV valves.
Hospital mortality was 17% (13/76). The follow-up was 100% complete. There were 15 late deaths. There were 48 survivors with a mean follow-up of 8.3±6 years. One- and 10-year Kaplan-Meier survival after AV valve operations was 72% (95% confidence interval [CI], 60%-81%) and 61% (95% CI, 48%-71%), respectively. Independent predictors of overall mortality were presence of a common AV valve (p=0.03), requirement for postoperative mechanical circulatory support (p=0.02), and timing of valve operations between initial palliation and performance of a bidirectional cavopulmonary shunt (BCPS) (p=0.047). Ten-year freedom from valve reoperation and from thromboembolic events of hospital survivors was 56% (95% CI, 38%-70%) and 70% (95% CI, 56%-80%), respectively. At last follow-up, 11 of 48 surviving patients (23%) had moderate to severe regurgitation, and pacemaker implantation was required in 6 patients. Only 34 patients reached the stage of Fontan completion.
AV valve regurgitation is a considerable burden for the patient with a single ventricle, especially when appearing at a young age. A quarter of patients died within the first year after operation, and they had considerable morbidity in terms of reoperation, thromboembolic events, and pacemaker implantation. Their chances of reaching Fontan completion seemed decreased.
The Annals of thoracic surgery 05/2012; 94(2):606-13; discussion 613. · 3.74 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Studies on long-term outcomes of the arterial switch operation (ASO) for transposition of the great arteries (TGA) are uncommon. Thus, we sought to determine the long-term outcomes for patients after ASO performed at a single institution over a 25-year period.
From 1983 to 2009, 618 patients underwent the ASO for TGA and were reviewed retrospectively.
Overall early mortality was 2.8%. Risk factors for early death on multivariate analysis were resection of left ventricular outflow tract obstruction at time of ASO (p = 0.001), weight less than 2.5 kg at time of ASO (p < 0.001), associated aortic arch obstruction (p = 0.043), and the need for postoperative extracorporeal membrane oxygenation (p < 0.001). Mean follow-up time was 10.6 years (range 2 months to 26.1 years). Late mortality was 0.9%. Reintervention was significantly higher (p < 0.001) in patients with ventricular septal defect or arch obstruction versus those without them (25.2% and 23.4% vs 5.9% at 15- year follow-up). Risk factors for late reintervention were left ventricular outflow tract obstruction at time of ASO (p < 0.001) and a greater circulatory arrest time (p < 0.001). Freedom from at least moderate neoaortic valve regurgitation for the entire cohort was 98.7% (95% confidence interval 96.8 to 99.5%) at 20 years. Mild neoaortic regurgitation was seen in 25.6% of patients at mean follow-up. All patients were free of arrhythmia and heart failure symptoms at last follow-up.
The ASO can be performed with good long-term results. Patients with associated ventricular septal defect and aortic arch obstruction warrant close follow-up.
The Annals of thoracic surgery 05/2012; 94(1):139-45. · 3.74 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The surgical outcomes of atrioventricular septal defect (AVSD) associated with tetralogy of Fallot (TOF) or double-outlet right ventricle (DORV) have improved in recent times. However, high mortality and reoperation rates are still reported. This study reviews our surgical experience in patients with complete AVSD and TOF or DORV.
Between 1980 and 2010, 48 consecutive patients with AVSD associated with TOF (n = 26) or DORV (n = 22) underwent complete repair; of which, 19 had staged repair. A transatrial-transpulmonary approach with the 2-patch technique was the preferred surgical technique. Data were obtained from inpatient and outpatient medical files.
The mortality rates were 8.3% (4 of 48) for in-hospital death and 13.6% (6 of 44) for late death. Mean follow-up was 8.0 ± 8.7 years (median 10.8 years; range from 2 months to 30 years). Actuarial survival was 76% at 5 years and 71% at 20 years. No deaths occurred after 2001. Reoperations were required in 16 of 48 patients (33%). The overall freedom from reoperation was 55% at 5 and 20 years. Down syndrome was protective for reoperation (p = 0.022).
Complete AVSD associated with TOF or DORV can be repaired with good survival. Detachment of the superior bridging leaflet provided excellent exposure. However, reoperation rate remains high.
The Annals of thoracic surgery 05/2012; 94(1):172-8. · 3.74 Impact Factor
-
The Annals of thoracic surgery 04/2012; 93(4):1401. · 3.74 Impact Factor
-
The Journal of thoracic and cardiovascular surgery 04/2012; 143(4):989-90; author reply 990. · 3.41 Impact Factor
-
Yves d'Udekem,
Mary Y Xu,
John C Galati,
Siming Lu,
Ajay J Iyengar,
Igor E Konstantinov,
Gavin R Wheaton,
James M Ramsay,
Leeanne E Grigg,
Johnny Millar,
Michael M Cheung,
Christian P Brizard
[show abstract]
[hide abstract]
ABSTRACT: This study examined survival after surgical palliation in children with single-ventricle physiology.
Contemporary surgical outcomes for the entire population of newborns undergoing single-ventricle palliation are unclear.
In a single-center review of 499 consecutive patients undergoing univentricular palliation from 1990 to 2008, predictors of mortality were determined using multivariate risk analysis, stratified for each post-operative stay and interim states.
After 2000, the population comprised more patients with dominant right ventricle (66% vs. 36%) and hypoplastic left heart syndrome (HLHS) (47% vs. 13%). Median age at bidirectional cavopulmonary shunt (BCPS) decreased from 15 months (10 to 22 months) before 2000 to 4 months (3.3 to 9 months) thereafter. Survival rates at 1, 5, and 10 years were, respectively, 82% (95% confidence interval [CI]: 79% to 85%), 74% (95% CI: 70% to 78%), and 71% (95% CI: 67% to 75%). Throughout the study, atrioventricular valve regurgitation (hazard ratio [HR]: 1.8; p = 0.008), not having transposition (HR: 2.0; p = 0.013), and heterotaxia (HR: 2.0; p = 0.026) were predictors of mortality. The most potent risk factor was right ventricular (RV) dominance (HR: 2.2; p = 0.001) because of its impact before BCPS. HR for death in patients with RV dominance went from 2.8 (95% CI: 1.4 to 5.7; p = 0.005) before BCPS to 1.0 (95% CI: 0.5 to 2.1; p = 0.98) thereafter. Survival of patients with RV dominance, adjusted for the risk factors noted here, improved over the study period (p = 0.001).
Considerable mortality is still observed during the first years of life among patients with single ventricle. RV dominance is the most important risk factor for death but only before BCPS.
Journal of the American College of Cardiology 03/2012; 59(13):1178-85. · 14.16 Impact Factor
