Transcatheter closure with a "single lobe" Amplatzer Vascular Plug of a tubular patent ductus arteriosus in a neonate with transposition of the great arteries and heart failure.

A patent ductus arteriosus in patients with transposition of the great arteries is usually beneficial to allow shunting between pulmonary and systemic circulations. However, if the duct is too large, it can cause haemodynamic instability, pulmonary oedema and compromised organ perfusion. We present a neonate in whom a 5 mm short and tubular ductus arteriosus was causing significant cardiac failure with necrotising enterocolitis and liver impairment, leaving him too unstable for the arterial switch operation. At day 14 of life (3.1 kg), the baby underwent successful transcatheter closure using an Amplatzer vascular plug (Abbott, Chicago, Il, USA) delivered through a 5Fr catheter from the aortic side. The procedure was uncomplicated and successful as the neonate was extubated 2 days later. He subsequently underwent successful arterial switch surgery.

Covered Stent as a Bridge to Surgery for Obstructive Melody Pulmonary Valve Endocarditis.

Endocarditis is life threatening after percutaneous pulmonary valve implantation. We report a patient with acute heart failure caused by obstructive pulmonary vegetation in a Melody valve. Because of severe right ventricular dysfunction, immediate cardiac surgery was contraindicated. The patient underwent trans-catheter implantation of a covered stent to relieve obstruction. Following an initially good outcome, surgery was performed 2 weeks later. This was complicated by left main coronary artery tear and fatal bleeding. Despite covered stent was an effective bridge, surgery remains extremely challenging in such cases with important surrounding inflammation and fibrosis as well as potential proximity of coronary arteries.

Heart rate variability arterial hypertension in young heart-transplanted recipients: association progression of cardiac allograft vasculopathy?

Hypertension and reduced HRV are frequent in heart-transplanted recipients. We studied 26 young recipients to investigate the relationship between BP and HRV during simultaneous 24-h monitoring. Presence of CAV was considered. All HRV measures were significantly lower than normal values. Significant correlations were found between mean daytime systolic BP and the rMSSD (p = 0.04), and mean daytime DBP and SDANN for all 5-min segments (p = 0.03) and between rMSSD and mean nighttime DBP (p = 0.03). Four patients were hypertensive during daytime, seven had a reduced nocturnal fall and two had a nocturnal rise in BP. Eight patients showed severe CAV grade (grade IV) on the Stanford scale, 13 moderate (grade III) and five mild (grade I-II). After a follow-up time of 30 months, four of the 13 patients (30%) with CAV grade III showed an increase to grade IV and all showed abnormalities of both HRV and ABPM patterns. The relationship between HRV abnormalities and arterial hypertension and CAV should be further explored.

Melody Valve Fracture Causing Mitral Stenosis: Novel Solution for Transapical Valve-in-Valve.

The use of Melody valves in the mitral position has been introduced in clinical practice. Stent fracture is a recognized complication of Melody valve implantation in the pulmonary position; however, reports in the mitral position are rare. We present the case of an 8-year-old boy in whom complete fracture of the proximal stent struts occurred, causing acute severe mitral stenosis, and in whom urgent hybrid transapical Melody valve implantation in the fractured Melody valve was performed successfully using a novel modified technique. This modification simplified the implantation, led to reduced time of the procedure, and minimized hemodynamic instability.

Pneumatic pulsatile ventricular assist device as a bridge to heart transplantation in pediatric patients.

Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report on our experience in the pediatric population. A retrospective review of 17 consecutive children offered mechanical support with Berlin Heart as a bridge to heart transplant from February 2002 to April 2010 was conducted. The median patient age was 3.9 years (75 days to 13.3 years). The median patient weight was 14.1 kg (2.9-43kg). Before VAD implantation, all children were managed by multiple intravenous inotropes and mechanical ventilation (14) or extracorporeal membrane oxygenation (3). All patients had right ventricular dysfunction. Nine patients required biventricular mechanical support (BVAD), but in all other cases a single left ventricular assist device proved sufficient (47%). The median duration of VAD support was 47 days (1-168 days). The median pre-VAD pulmonary vascular resistance index (Rpi) was 5.7 WU/m(2) (3.5 to 14.4WU/m(2) ). Eleven patients (65%) were successfully bridged to heart transplantation after a median duration of mechanical support of 68 days (6-168 days). Six deaths occurred (35%), three for neurological complications, one for sepsis, and two others for device malfunctioning. Since 2007, the survival rate of our patients has increased from 43% to 80%, and the need for BVAD has decreased from 86% to 30%. In two patients with Rpi >10WU/m(2) , unresponsive to pulmonary vasodilatator therapy, Rpi dropped to 2.2 and 2WU/m(2) after 40 and 23 days of BVAD support, respectively. Seven patients (41%) required at least one pump change. Of 11 patients undergoing heart transplant, four developed an extremely elevated (>60%) panel reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex. All of them experienced at least one acute episode of rejection in the first month after heart transplant, needing plasmapheresis. The survival rate after heart transplantation was 100% with a median follow-up of 25.4 months (6 days to 7.7 years). Mechanical support in children with end-stage heart failure is an effective strategy as a bridge to heart transplantation with a reasonable morbidity and mortality. BVAD support may offer an additional means to reverse extremely elevated pulmonary vascular resistance.

Serial changes in longitudinal strain are associated with outcome in children with hypoplastic left heart syndrome.

INTRODUCTION: Hypoplastic Left Heart Syndrome (HLHS) has high mortality and morbidity and systemic right ventricle (RV) dysfunction may play a key-role. Study aim is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting outcome in HLHS patients. METHODS: We studied 27 HLHS patients (17 male) who successfully completed Norwood palliation. All the patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed: baseline, one-month after Norwood, three-months after Norwood, one-week before bidirectional cavopulmonary anastomosis (BCPA) and two-months after BCPA. From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). RESULTS: After a mean follow-up of 1.18 (± 1.16) years, 8 out of 27 of the included patients met the composite endpoint of death/heart transplant (HT). At pre-Norwood assessment, there was no difference in echo measurements between survivors and patients with events. In death/HT group TAPSE and LS declined already one-month after Norwood procedure: TAPSE ≤5 mm had good sensitivity (85.71%) and moderate specificity (63.16%) for death/HT (AUC = 0.767); a decrease of LS > 8.7% vs baseline showed 100% sensitivity and 84.21% specificity for death/HT (AUC = 0.910). At multivariate analysis, one-month-after-Norwood LS drop >8.7% was the best predictor of outcome (P = 0.01). CONCLUSIONS: RV dysfunction in HLHS carries prognostic value. Our findings encourage serial measurements of RV function to identify the subgroup of HLHS patients at higher risk. In our experience, ∆ LS showed the best predictive value.

Echocardiography and cardiac magnetic resonance in children with repaired tetralogy of Fallot: New insights in cardiac mechanics and exercise capacity.

BACKGROUND: Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). AIMS: To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO2). METHODS AND RESULTS: All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p < .0001, r = 0.72). RV end-diastolic area > 21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m2. RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO2. Among the Echo data only right atrial strain (RAS) correlated with peak VO2. CONCLUSION: In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO2 and should be added in their follow up.

In-hospital interstage improves interstage survival after the Norwood stage 1 operation.

OBJECTIVES: The interstage mortality rate after a Norwood stage 1 operation remains 12-20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome. METHODS: A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2. Daily and weekly examinations were conducted systematically, including two-dimensional and speckle-tracking echocardiography. Primary end points included aggregate survival until the completion of stage 2 and interstage freedom from escalation of care. Moreover, we calculated the sensitivity and specificity of speckle-tracking echocardiographic myocardial deformation in predicting death/transplant after the Norwood procedure. RESULTS: Between 2015 and 2019, 33 neonates with HLHS (24) or HLHS variants (9) underwent Norwood stage 1 (31) or hybrid palliation followed by a comprehensive stage 2 operation (2). Stage 1 Norwood-Sano was preferred in 18 (54.5%) neonates; the classic Norwood with Blalock-Taussig shunt was performed in 13 (39.4%) neonates. The Norwood stage 1 30-day mortality rate was 6.2%. The in-hospital interstage strategy was implemented after Norwood stage 1 with a 3.4% interstage mortality rate. The aggregate Norwood stage 1 and interstage Kaplan-Meier survival rate was 90.6 ± 5.2%. Escalation of care was necessary for 5 (17.2%) patients at 2.5 ± 1.2 months during the interstage for compromising atrial arrhythmias (2), Sano-shunt stenosis (1) and pneumonia requiring a high-frequency oscillator (2); there were no deaths. A bidirectional Glenn (25) or a comprehensive-Norwood stage 2 (2) was completed in 27 patients at 4.7 ± 1.2 months with a 92.6% survival rate. The overall Kaplan-Meier survival rate is 80.9 ± 7.0% at 4.3 years (mean 25.3 ± 15.7 months). An 8.7% Δ longitudinal strain 30 days after Norwood stage 1 had 100% sensitivity and 81% specificity for death/transplant. CONCLUSIONS: In-hospital interstage facilitates escalation of care, which seems efficacious in reducing interstage Norwood deaths. A significant reduction of longitudinal strain after Norwood stage 1 is a strong predictor of poor outcome.

Severe Migraine Associated With Nickel Allergy Requiring Surgical Removal of Atrial Septal Device.

Few patients experience migraines after transcatheter closure of secundum atrial septal defects with nitinol devices. These migraines are usually treated with analgesics and resolve after a few months as the device endothelializes. This report describes the case of a 16-year-old male patient who required surgical explantation of the device 6 years after closure because of debilitating headaches. He had a grade 1 reaction to nickel after skin testing. Intraoperatively, the device had not fully endothelialized. Immediately after removal of the device, his headaches completely resolved. Long-term nickel allergy may cause severe migraine secondary to a lack of endothelialization of a device. Patients with extreme cases may require surgical removal of the device.

Accuracy of computed tomography in detection of great vessel stenosis or hypoplasia before superior bidirectional cavopulmonary connection: Comparison with cardiac catheterization and surgical findings.

BACKGROUND: Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk. AIMS: To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings. METHODS: Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients. RESULTS: The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r=0.98 for both) and a low mean bias (0.71mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between -0.9 and 2.3mm and between -0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P=0.13). All computed tomography scans were performed without sedation. CONCLUSIONS: Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.

Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly.

BACKGROUND: Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS: Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ±â€¯41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. RESULTS: Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5-83) vs 28.4 mm/m2 (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ±â€¯7.7% vs 36.7 ±â€¯9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ±â€¯6.2% vs -16.2 ±â€¯7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ±â€¯13.5% vs 36.3 ±â€¯12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. CONCLUSION: Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.

Initial 2-year results of CardioCel® patch implantation in children.

OBJECTIVES: We present the initial 2-year results of CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) implantation in paediatric patients with congenital heart diseases. METHODS: This was a single-centre retrospective study with prospectively collected data of all patients aged 18 years and under operated for congenital heart disease. The patch was introduced in 2014, with clinical practice committee approval and a special consent in case of an Ozaki procedure. Standard follow-up was performed with systematic clinical exams and echocardiograms. In case of reoperation or graft failure, the patch was removed and sent for a histological examination. RESULTS: Between March 2014 and April 2016, 101 patients had surgical repair using a CardioCel patch. The mean age was 22 (±36.3) months, and the mean weight was 9.7 (±10.3) kg. No infections and no intraoperative implantation difficulties were associated with the patch. The median follow-up period was 212 (range 4-726) days. The overall 30-day postoperative mortality was 3.8% (n = 4), none of which were related to graft failure. Five children were reoperated because of graft failure, 4 of whom had the patch implanted for aortic and were aged less than 10 days. The indications for patch implantation in the aortic position were aortopulmonary window, truncus arteriosus, coarctation and aortic arch hypoplasia repair. The median time between the first and the second operation for graft failure was 245 (range 5-480) days. CONCLUSIONS: Our experience shows that the patch is well tolerated in the septal, valvar and pulmonary artery positions. However, we experienced graft failures in infants in the aortic position.

Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.

OBJECTIVES: Infants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation. METHODS: This multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life. RESULTS: Median age at definitive surgery was 179 (range 0-357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1-11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation. CONCLUSIONS: Although surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.

Surgical options after Fontan failure.

OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX. METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%). RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0-23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04). CONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

Orthotopic heart transplantation for congenital heart disease: an alternative for high-risk fontan candidates?

OBJECTIVE: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease. METHODS: Between 1988 and 2002, 43 patients (mean age 9.1+/-7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5). RESULTS: 30-day survival for the 2-ventricle subgroup was 94.4+/-5.4% compared with 67.2+/-9.5% for the single ventricle subgroup (P=0.04) (overall 78.6%+/-3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5+/-17.1% for OHT after systemic-to-pulmonary shunts, and 33.3+/-19.2% for OHT following failing Fontan (P=0.010). HLHS diagnosis (0.0085) and failing Fontan (P=0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3+/-11%. CONCLUSIONS: OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis enables the best transition to heart transplant. OHT should be considered in the decision making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.

Favourable mid-term outcome after heart transplantation for late Fontan failure.

OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation.

Protein-losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4% to 13% of patients after the Fontan procedure. We report a case of PLE reversal after heart transplantation in a 14-year-old boy with Fontan circulation who previously was treated unsuccessfully with medical therapy. The protein loss continued after heart transplantation. We administered total parenteral nutrition to rest the bowel. After 16 months, we observed a gradual decrease in protein loss. The patient is doing well 5 years after heart transplantation and had has a normal serum albumin level.

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery.

BACKGROUND: This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery. METHODS: Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months). RESULTS: Fifteen-year actuarial survival was 92.9% +/- 4.9% for coronary transfer, 40.0% +/- 21.9% for subclavian interposition, and 89.9% +/- 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% +/- 7.4%). Left ventricular shortening fraction increased from 17.3% +/- 6.3% before operation to 34.1% +/- 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r(2) = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% +/- 5.2%) and best longitudinal shortening fraction recovery (36.4% +/- 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors. CONCLUSIONS: Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.

Orthotopic heart transplantation for failing single ventricle physiology.

OBJECTIVE: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology. METHODS: Between 1988 and 2002, 25 patients (mean age 9.3+/-7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging towards Fontan completion had been previously accomplished in 22 patients (88%). Transition to OHT occurred from a shunt stage in 10, a bi-directional cavopulmonary anastomosis (BDG) stage in nine, and after Fontan failure in six patients. RESULTS: Thirty-day survival was 68.0+/-9.3% with no additional mortality up to 14.1 years. OHT following BDG staging exhibited 100% long-term survival, as opposed to 66.7+/-15.7% for OHT after systemic-to-pulmonary shunt, and 33.3+/-19.2% for OHT following failing Fontan (p=0.032). Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT (p=0.041). Reintervention was necessary in four patients 40+/-11 months after OHT to address residual superior vena cava (two) and isthmic (two) stenosis. Overall freedom from reintervention was 88.3+/-8.1% at 5 years. CONCLUSIONS: OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.