[Show abstract][Hide abstract] ABSTRACT: Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and compliments two-dimensional (2D) echocardiography. Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography.
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study was to characterize tachyarrhythmias in children following the Norwood procedure.
This is a single-center retrospective study including all children who underwent stage I Norwood procedure (n = 98; January 2003-September 2011). The primary outcome measure is the development of tachyarrhythmia during hospitalization after the Norwood procedure. Secondary aims include quantification of mortality in patients with tachyarrhythmias and evaluation of potential risk factors for the development of tachyarrhythmia.
Tachyarrhythmia occurred in 33 (34%) of 98 patients. The median time to onset of tachyarrhythmia was ten days (0-47 days). Tachyarrhythmia conferred no increase in overall mortality (P = .45), including operative mortality (P = .37) or interstage mortality (P = 1.00). There was no significant difference in the incidence of arrhythmia based on demographic, anatomic, or surgical variables, including shunt type (P = .23) except that patients with tachyarrhythmias were slightly larger (median weight 3.2 kg) at the time of stage I than those without tachyarrhythmia (median weight 2.93 kg; P = .02]. The odds of arrhythmia in males were 8.7 times higher than that in females (95% confidence interval 2.9-31.3; P < .0001).
Postoperative tachyarrhythmia is common, occurring in 34% of patients after the Norwood operation. Onset of tachyarrhythmia occurred later after the Norwood operation than reported previously, and male gender is a risk factor. Further studies to elucidate the etiology and the timing of tachyarrhythmias after the Norwood procedure are necessary.
World journal for pediatric & congenital heart surgery. 04/2014; 5(2):206-10.
[Show abstract][Hide abstract] ABSTRACT: Maintenance steroid (MS) use in pediatric heart transplantation (HT) varies across centers. The purpose of this study was to evaluate the impact of steroid-free maintenance immunosuppression (SF) on graft outcomes in pediatric HT.
Patients younger than 18 years in the United States undergoing a first HT during 1990 to 2010 were analyzed for conditional 30-day graft loss (death or repeat HT) and death based on MS use by multivariable analysis. A propensity score was then given to each patient using a logistic model, and propensity matching was performed using pre-HT risk factors, induction therapy, and nonsteroid maintenance immunosuppression. Kaplan-Meier graft and patient survival probabilities by MS use were then calculated.
Of 4894 patients, 3962 (81%) were taking MS and 932 (19%) SF. Of the 4530 alive at 30 days after HT, 3694 (82%) and 836 (18%) were in the MS and SF groups, respectively. Unmatched multivariable analysis showed no difference in 30-day conditional graft survival between MS and SF groups (hazard ratio=1.08, 95% confidence interval=0.93-1.24; P=0.33). Propensity matching resulted in 462 patients in each MS and SF group. Propensity-matched Kaplan-Meier survival analysis showed no difference in graft or patient survival between groups (P=0.3 and P=0.16, respectively).
We found no difference in graft survival between SF patients and those taking MS. An SF regimen in pediatric HT avoids potential complications of steroid use without compromising graft survival, even after accounting for pre-HT risk factors.
[Show abstract][Hide abstract] ABSTRACT: Aorto-left ventricular tunnel (ALVT) is an abnormal congenital communication between the ascending aorta and the left ventricle. Prenatal diagnosis is rare and can be misinterpreted as aortic insufficiency on fetal echocardiogram. We present a case of ALVT diagnosed in a fetus who underwent successful early neonatal surgical repair.
[Show abstract][Hide abstract] ABSTRACT: Purpose: Tachyarrhythmias are common following repair of congenital heart disease, and may be associated with increased morbidity and mortality. The purpose of this study was to determine the incidence of tachyarrhythmia in patients following Norwood operation.
Methods: Retrospective chart review of all patients who underwent stage I Norwood procedure from 1/1/2003 to 9/1/2011 (n=100). Data collected included demographics, shunt type (modified Blalock-Taussig shunt (mBTS) or right ventricle to pulmonary artery conduit (RVPA), surgical variables, type of tachycardia, interval since surgery, cycle length, and need for antiarrhythmic medication at discharge.
Results: Data regarding arrhythmia was available for 98 patients. Tachyarrhythmia occurred in 33/98 patients (34%). Six patients with a tachyarrhythmia died. Causes of mortality in those with a tachycardia included cardiac arrest (n=2) or circulatory collapse requiring ECMO (n=2), respiratory arrest (n=1) and dural sinus venous thrombosis (n=1). There was no significant difference in the incidence of arrhythmia based on shunt type (p=0.23) where 8/32 underwent palliation with mBTS, and 25/66 with a Sano shunt. The odds of arrhythmia in males were 9.3 times that of females (95% CI 2.9, 35.3)(p = 0.0004). Table 1 demonstrates the arrhythmia characteristics
Conclusion: Post-operative tachyarrhythmia is common after Norwood operation. Shunt type was not associated with tachycardia. Male gender was a significant risk factors associated with the development of tachyarrhythmia. It is possible that other factors could have contributed to tachycardia, including use of specific drugs such as milrinone. Further studies to elucidate these factors are necessary.
Table 1. Arrhythmia characteristics following Norwood procedure
# with EKG
Days since NP
D/C on meds*
14 (12, 28)
280 (240, 320)
1 (1, 4)
290 (270, 320)
10.5 (8, 17)
285 (235, 328)
AVRT – pre
8 (5, 11)
215 (210, 220)
4 (0, 5)
320 (280, 380)
10 (0, 19)
> 1 mechanism
2 (1, 13)
220 (220, 318)
*Includes patients alive at discharge from the Norwood procedure. All data is represented as median with interquartile range if appropriate. AET = atrial ectopic tachycardia, AVRT = atrioventricular reentrant tachycardia, AVRT – pre = atrioventricular reentrant tachycardia with pre-excitation, JET = junctional ectopic tachycardia, VT = ventricular tachycardia, N = number of patients, # = those for which an EKG was available for review, A-wire = atrial wire EKG used for interpretation, NP = Norwood procedure, CL = cycle length, ms = milliseconds, D/C = discharge.
2012 American Academy of Pediatrics National Conference and Exhibition; 10/2012
[Show abstract][Hide abstract] ABSTRACT: The effect of surgical history on graft outcomes in patients with functionally univentricular hearts (UH) is not well understood. We compared graft outcomes after heart transplantation in children with a UH between patients who received allografts without prior cardiac surgery (Group A) and patients who underwent transplantation after prior cardiac surgery (Group B).
We reviewed all patients who received allografts for UH at our institution from 1990 to 2009. Differences in the probability of acute rejection (AR), incidence of graft vasculopathy (GV), and incidence of death or retransplantation were compared between Group A and Group B. Student's t-test, Mann-Whitney U-test, the log-rank test, logistic regression, and Cox proportional hazards modeling were used as appropriate.
During the study period, 180 patients with a UH received allografts: 105 in Group A and 75 in Group B at a median (interquartile range) age of 84 (47-120) days vs 584 (168-2,956) days, respectively (p < 0.001). The odds of AR were higher in Group B (odds ratio, 2.7, 95% confidence interval, 1.3-5.4). Group A had lower univariable risks of GV (p = 0.034) and graft loss (p = 0.003). Median graft survival was 18 years in Group A vs 8 years in Group B. The risk of graft loss after 5 years post-transplant was higher in Group B patients who were aged ≥ 1 year at time of transplant (p < 0.001).
Heart transplantation without prior cardiac surgery in patients with a UH was associated with better graft survival and lower probability of AR. The effect of age is complex and time-dependent, with age affecting outcomes after 5 years.
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 07/2012; 31(9):987-95. · 5.61 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: This study compared graft failure leading to retransplant in infants versus older children at initial heart transplant.
Twenty-six retransplant recipients were compared by age at first transplant: infant group (<1 year) and pediatric group (≥1 year).
Early retransplant survival was 92%. Retransplant survivals at 1, 3, and 5 years were 83%, 74%, and 67%. There were 15 infant and 11 pediatric patients. First transplant ages were 0.4 ± 0.3 vs. 8.5 ± 5.7 years in infant and pediatric groups, respectively (P < .01). First graft rejection episodes were more common in pediatric group (4.8 ± 2.5 vs 3.1 ± 2.1, P = .032), and rejection rate was higher (1.5 ± 1.1 vs 0.4 ± 0.4, P = .0024). Median first graft survival was longer in infant group (10.7 years vs 3.9 years, P < .001). Recurrent cellular rejection was retransplant indication in 40% of infant group versus 91% of pediatric group (P < .05). Cardiac allograft vasculopathy was more prevalent in infant group (73% vs 20% in pediatric group, P = .032).
Infant heart transplant recipients had longer primary graft survival, fewer cellular rejection episodes, and higher incidence of cardiac allograft vasculopathy relative to older graft recipients requiring retransplant. Advantages in adaptive immunity in infant heart recipients confer improved primary graft survival, but longer graft life in these patients is limited by cardiac allograft vasculopathy. Older recipient first graft failure was rejection related, and shorter graft life probably limited development of cardiac allograft vasculopathy.
The Journal of thoracic and cardiovascular surgery 11/2010; 141(1):223-30. · 3.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report a case of mechanical aortic valve replacement that was complicated by acute occlusion of one of the valve leaflets by the BioGlue (CryoLife Inc, Kennesaw, GA) that had seeped through the suture line on the aorta. This uncommon, but life-threatening complication is one that may go unrecognized, but is preventable.
The Annals of thoracic surgery 09/2009; 88(2):671-2. · 3.45 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We sought to analyze the indications and outcome of extracorporeal membrane oxygenation (ECMO) for early primary graft failure and determine its impact on long-term graft function and rejection risk.
Early post-operative graft failure requiring ECMO can complicate heart transplantation.
A retrospective review of all children requiring ECMO in the early period after transplantation from 1990 to 2007 was undertaken.
Twenty-eight (9%) of 310 children who underwent transplantation for cardiomyopathy (n = 5) or congenital heart disease (n = 23) required ECMO support. The total ischemic time was significantly longer for ECMO-rescued recipients compared with our overall transplantation population (276 +/- 86 min vs. 242 +/- 70 min, p < 0.01). The indication for transplantation, for ECMO support, and the timing of cannulation had no impact on survival. Hyperacute rejection was uncommon. Fifteen children were successfully weaned off ECMO and discharged alive (54%). Mean duration of ECMO was 2.8 days for survivors (median 3 days) compared with 4.8 days for nonsurvivors (median 5 days). There was 100% 3-year survival in the ECMO survivor group, with 13 patients (46%) currently alive at a mean follow-up of 8.1 +/- 3.8 years. The graft function was preserved (shortening fraction 36 +/- 7%), despite an increased number of early rejection episodes (1.7 +/- 1.6 vs. 0.7 +/- 1.3, overall transplant population, p < 0.05) and hemodynamically comprising rejection episodes (1.3 +/- 1.9 vs. 0.7 +/- 1.3, overall transplant population, p < 0.05).
Overall survival was 54%, with all patients surviving to at least 3 years after undergoing transplantation. None of the children requiring >4 days of ECMO support survived. Despite an increased number of early and hemodynamically compromising rejections, the long-term graft function is similar to our overall transplantation population.
Journal of the American College of Cardiology 09/2009; 54(8):730-7. · 15.34 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A previously healthy, 13-year-old girl presented with new-onset dilated cardiomyopathy, and is placed on a left ventricular assist device (VAD). Herein we describe a unique VAD weaning protocol used to determine the timing and feasibility of a VAD explant.
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 09/2009; 29(1):105-8. · 5.61 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome and other elastin gene deletions. Our objectives were to review outcomes of congenital SVAS repair and to compare prosthetic patch repair techniques to all-autologous slide aortoplasty.
Congenital SVAS repairs from 1988 to 2008 were retrospectively reviewed. Peak instantaneous gradients were estimated by Doppler interrogation. Variables were compared by either Student's t test or Fisher's exact test. Risk factors were analyzed by chi(2) test. Survival was estimated by the Kaplan-Meier method.
Of 25 primary SVAS repairs, there were 10 all-autologous slide aortoplasties and 15 prosthetic patch aortoplasties. The prosthetic patch group included the Doty technique (n = 9), patch-augmented slide aortoplasty (n = 3), modified Brom technique (n = 1), interposition graft (n = 1), and two-sinus patch with transverse arch augmentation (n = 1). There was 1 early and 1 late death. Cumulative survival for all patients was 96% at 5 and 10 years. Event-free survival did not differ between groups (p = 0.481). There were 2 late reoperations (both were prosthetic patch patients with bicuspid aortic valve: 1 with recurrent aortic valve stenosis and 1 with aortic insufficiency). Bicuspid aortic valve was the only risk factor for reoperation (p = 0.003). Three patients weighing less than 10 kg with diffuse disease underwent attempted slide aortoplasty: 2 required patch augmentation and 1 had a recurrent gradient in less than 1 year postoperatively.
Outcomes after SVAS repair were good by any technique. No advantage to all-autologous slide aortoplasty was apparent at current follow-up. Based on our experience, slide aortoplasty is not recommended for small patients with diffuse disease.
The Annals of thoracic surgery 06/2009; 87(5):1501-7; discussion 1507-8. · 3.45 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study was to describe the long-term outcome of infants with hypoplastic left heart syndrome (HLHS) who underwent placement of internal pulmonary artery bands as part of a transcatheter palliation procedure followed by primary heart transplantation. Transcatheter palliation included stenting of the ductus arteriosus, decompression of the left atrium by atrial septostomy, and internal pulmonary artery band placement. Cardiac hemodynamics, pulmonary artery architecture, and pulmonary artery growth since transplantation are described. Nine infants with HLHS had internal pulmonary artery bands placed and underwent successful heart transplant. No infant required reconstruction of the pulmonary arteries at the time of transplant. At 1 year after transplant, all of the recipients had normal mean pulmonary artery pressure, pulmonary vascular resistance, and transpulmonary gradient. Pulmonary angiography performed at 1 year after transplant demonstrated no distortion of pulmonary artery anatomy with significant interval growth of the branch pulmonary arteries. There was 100% survival to hospital discharge after transplant in this cohort of infants. Transcatheter placement of internal pulmonary artery bands for HLHS offers protection of the pulmonary vascular bed while preserving pulmonary artery architecture and growth with good long-term outcome.
[Show abstract][Hide abstract] ABSTRACT: Incision into the ventricular septum in complex biventricular repair is controversial, and has been blamed for impairing left ventricular function. This retrospective study evaluates the risk of a ventricular septal incision in patients undergoing double outlet right ventricle (DORV) repair and Ross-Konno procedure.
From January 2003 to September 2007, 11 patients with DORV had a ventricular septum (VS) incision and 12 DORV patients did not. Sixteen patients had a Ross-Konno, and 16 had an isolated Ross procedure. The ventricular septal incision was made to match at least the diameter of a normal aortic annulus. In DORV, the VSD was enlarged superiorly and to the left. In the Ross-Konno, the aortic annulus was enlarged towards the septum posteriorly and to the left.
The median follow-up for the study is 19 months (1 month-4 years). For DORV, there were no significant differences in discharge mortality (p=0.22), late mortality (p=0.48), or late mortality plus heart transplant (p=0.093). Although patients with DORV and VSD enlargement have a more complex postoperative course, there were no differences in ECMO use (p=0.093), occurrence of permanent AV block (p=0.55), left ventricular ejection fraction (LVEF) (p=0.40), or shortening fraction (LVSF) (p=0.50). Similarly, for the Ross-Konno there were no significant differences in discharge mortality (p=0.30), late mortality (p=NS), LVEF (p=0.90) and LVSF (p=0.52) compared to the Ross, even though the Ross-Konno patients were significantly younger (p<0.0001).
Making a ventricular septal incision in DORV repair and in the Ross-Konno operation does not increase mortality and does not impair the LV function. The restriction of the VSD remains an important issue in the management of complex DORV. These encouraging results need to be confirmed by larger series.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 04/2009; 35(4):589-93; discussion 593. · 2.40 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Conventional management for multilevel left heart obstructions and mitral stenosis (Shone's complex) involves multiple operations that carry additive risks. This study reviews our experience with reconstructive and transplantation approaches for Shone's complex.
Between 1987 and 2007, 43 patients with mitral stenosis and one or more left-sided obstructions were identified: supramitral ring (n = 13), subaortic stenosis (n = 25), aortic stenosis (n = 24), hypoplastic arch (n = 20), and coarctation (n = 38). Thirty patients underwent a staged reparative approach, including 27 mitral and 51 left ventricular outflow tract operations. Thirteen patients were referred for transplantation. Patients with severe hypoplasia of the left ventricle were excluded.
There was one in-hospital death (2.5%) and six late deaths (14.2%). Actuarial 5- and 10-year survival for staged surgical and transplantation was 88% vs 61.3% and 83.1% vs 61.3% (p = 0.035). At a mean follow-up of 7.9 years, freedom from mitral reoperation was 83.3% and freedom from reoperation for subaortic stenosis was 78.0%. Wait-list mortality was 13.3% (2 of 13). Wait-list time exceeding 90 days was an incremental risk factor for death after transplantation (p = 0.005).
Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Heart transplantation, although avoiding the pitfalls of staged repair, confers increased risks from ongoing physiologic derangements due to uncorrected left heart inflow and outflow obstructions during the wait for donor heart availability.
The Annals of thoracic surgery 11/2008; 86(4):1305-9; discussion 1310. · 3.45 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Outcomes of patients undergoing cavopulmonary palliation for single ventricle physiology may be impacted by living at altitude, as the passive pulmonary circulation is dependent on the resistance of the pulmonary vascular bed. The objective of this study is to identify risk factors for failure of cavopulmonary palliation at elevated altitude.
Between January 1995 and March 2007, 122 consecutive patients living at a mean altitude of 1600 m (range 305 to 2570) underwent a bidirectional Glenn (BDG). There was one in-hospital mortality and 7 late deaths. 52 have proceeded to the Fontan procedure. Survival after BDG was 92.4% at 5 years. Freedom from palliation failure, defined as death, transplant, BDG/Fontan takedown, or revision was 81% at 5 years. At a mean follow-up of 39.8 months, 90 patients (75%) were in New York Heart Association class I. Patients with failing cavopulmonary circulation had higher pre-BDG pulmonary artery pressure (PAP) (18.3+/-6.1 mm Hg versus 14.8+/-5.1 mm Hg, P=0.016) and higher pre-BDG transpulmonary gradient (TPG) (11.2+/-6.2 mm Hg versus 7.7+/-4.3 mm Hg, P=0.014). Post-BDG, patients with palliation failure had increased PAP (15.0+/-5.7 mm Hg versus 10.8+/-2.8 mm Hg, P=0.008) and indexed pulmonary vascular resistance (PVRI) (2.43+/-1.0 Wood U . m(2) versus 1.52+/-0.9 Wood U . m(2), P=0.007).
The majority of patients at moderate altitude have favorable outcomes after BDG or Fontan palliation. Risk factors for palliation failure at elevated altitude include PAP >15 mm Hg, TPG >8 mm Hg, and PVRI >2.5 Wood U . m(2).
[Show abstract][Hide abstract] ABSTRACT: Left ventricular assist devices are increasingly used as a bridge to transplantation. It remains unclear whether the use of pretransplant left ventricular assist devices adversely affects short-term survival after cardiac transplantation.
A retrospective review of 317 consecutive patients undergoing cardiac transplantation at an academic center between 1986 and 2006 was undertaken. Left ventricular assist devices were used pretransplant in 23 of these 317 patients, and 294 patients did not require left ventricular assist device support. Patients with a left ventricular assist device were supported with a Heartmate VE or Heartmate XVE (Thoratec Corp, Pleasanton, Calif). Kaplan-Meier survival estimates were compared between the left ventricular assist device group and the non-left ventricular assist device group using the log-rank test. In addition, occurrence of death was analyzed between the 2 groups with a chi-square analysis. The results are expressed as 1-year survival with 95% confidence intervals in parentheses.
The 1-year survival for all 317 patients was 0.86 (0.82-0.90). The patient survival for the group without a left ventricular assist device before cardiac transplant was 0.87 (0.83-0.90), and the survival for the group with a left ventricular assist device as bridge to transplantation was 0.83 (0.67-0.98; P = .77). For the deaths that occurred in all 317 patients, 19% of the patients without left ventricular assist devices died within 30 days of transplant, whereas 80% of the patients with left ventricular assist devices died within 30 days of transplant (P < .01).
When used as a bridge to transplantation, left ventricular assist devices do not compromise 1-year survival after cardiac transplantation. Of the patients who die after transplantation, patients bridged with left ventricular assist devices are at higher risk for death within 30 days of transplant. These data suggest that left ventricular assist devices as a bridge to transplantation should be considered for appropriately selected patients awaiting cardiac transplantation.
The Journal of thoracic and cardiovascular surgery 09/2008; 136(3):774-7. · 3.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A female infant was diagnosed with an inflammatory myofibroblastic tumor involving the left ventricle, which compromised cardiac function. The tumor was endocardial in location and resection was not possible. In this study we report the first successful cardiac transplant and long-term follow-up for this indication.
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 08/2008; 27(7):792-6. · 5.61 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Left atrioventricular valve regurgitation (LAVVR) is a major cause of morbidity after atrioventricular septal defect (AVSD) repair. This study evaluates the outcomes of repair and replacement of the left atrioventricular valve after AVSD correction, as well as factors predictive of durability of valve repair.
Between January 1983 and March 2007, 31 patients underwent reoperation for LAVVR after AVSD repair (23 valve repairs and 8 valve replacements). Median age at primary repair was 5.0 months and time to reoperation was 5.0 months. The distribution of AVSD morphology was 9 primum, 5 transitional, and 17 complete.
Early postoperative mortality was 6.4% (2 of 31). Survival at 10 years was 88.1%. At a mean follow-up of 8.2 years, 86% of hospital survivors were in New York Heart Association class I. Overall freedom from reintervention at 10 years was 67.2%. Among patients undergoing primary repair, 6 of 23 underwent subsequent replacement. Follow-up LAVVR in those who did not require subsequent valve replacement was mild or less in 92.8%. Factors that demonstrated a trend toward durable repair included the use of patch augmentation rather than primary cleft closure (p = 0.02) and earlier timing to repair (less than 2 months; p = 0.03). Significant cardiomyopathy developed in 21.4% of patients after prosthetic valve replacement (3 of 14).
Surgical management of LAVVR after AVSD repair can be performed with excellent midterm outcomes. However, both repair and replacement are associated with a high incidence of reoperation. Nonetheless, an aggressive reparative approach should be pursued to avoid the morbidity of pediatric left atrioventricular valve replacement that includes anticoagulation, inevitable reoperation, and cardiomyopathy.
The Annals of thoracic surgery 08/2008; 86(1):147-51; discussion 151-2. · 3.45 Impact Factor