V-shape reduction plasty to reduce the neoaortic root-ascending aorta discrepancy in children undergoing arterial switch operation.

Preoperative aortopulmonary mismatch is considered a risk factor for the development of aortic insufficiency after surgery for transposition of the great arteries. We have approached these children using a neoaortic root reduction plasty. Five children with severe PA-AO discrepancy (median age 57 days) underwent arterial switch operation (ASO) associated with a V-shape neoaortic reduction plasty. At follow-up aortic regurgitation remains stable in all patients and no supra-aortic obstruction and/or neoaortic root dilatation were observed.

Aortic arch reconstruction in newborns with an autologous pericardial patch: contemporary results.

OBJECTIVES: The incidence of recurrent aortic arch obstruction after Norwood procedure and other types of aortic arch reconstruction in newborns remains high. Biological and synthetic materials are used to enlarge the aorta. We report our experience using autologous pericardium to reconstruct the aortic arch in patients with hypoplastic left heart syndrome, aortic arch interruption and hypoplastic aortic arch. METHODS: A retrospective analysis of 39 consecutively operated patients evaluated after an initial Norwood and other types aortic arch repair was performed. The presence of recurrent arch obstruction (mean gradient ≥ 20 mmHg) and its management were noted. The mean weight of our patients was 3.2 ± 0.7 kg. RESULTS: The mean age at primary surgical correction was 7.4 ± 6.8 (range 1-35 days). All patients were discharged without a significant residual gradient at the aortic arch except 4 who had a peak gradient of ≥ 30 mmHg. The overall incidence of recurrent arch obstruction was 28.2% (11 patients). Four (12.1%) patients had a distal obstruction, 1 (3%) had proximal obstruction and 1 had a mid-transverse arch obstruction. All patients underwent aortic arch reintervention consisting of balloon dilatation, and only after unsuccessful dilatation, 3 underwent surgical patch aortoplasties. CONCLUSIONS: The use of autologous pericardium in aortic arch reconstruction procedure is effective and associated with an acceptable incidence of recurrent arch obstruction. Its availability and characteristics make it an attractive alternative to other materials.

Long term performance of small-diameter vascular grafts made of a poly(ether)urethane-polydimethylsiloxane semi-interpenetrating polymeric network.

In the past years considerable research efforts have been directed at developing more suitable synthetic vascular grafts, but small-diameter vascular grafts (SDVGs) perform less well than autogenous arterial or venous grafts. Grafts such as Dacron and ePTFE have often been used as alternatives to autologous grafts, but they have shown poor patency rates when used in small-diameter sizes or low-flow locations. Nevertheless, despite these efforts no alternative concepts have emerged yet that promises to replace the current generation of synthetic grafts soon. The purpose of this preliminary in vivo study was to assess the blood and tissue compatibility behaviors of a novel compliant SDVGs, fabricated with a poly(ether)urethane-polydimethylsiloxane (PEtU-PDMS) semi-interpenetrating polymeric network (semi-IPN) and featuring two different porous layers in the wall thickness. Grafts were implanted according to anastomotic techniques which emulate the flow conditions clinically adopted for peripheral or aorto-coronary bypass procedures. Relatively long grafts were implanted in the common carotid artery of adult sheep and compared to standard ePTFE grafts of the same size and length implanted controlaterally. The animal experimentation showed superior handling and compliance characteristics, and patency rates of PEtU-PDMS grafts in comparison with a standard ePTFE graft, and the ability of remodelling in vivo while being gradually replaced by a natural tissue with no sign of calcification.

The scimitar syndrome: an Italian multicenter study.

BACKGROUND: Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature. METHODS: From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up. RESULTS: Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%). CONCLUSIONS: The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.

Midterm results of surgical intervention for congenital heart disease in adults: an Italian multicenter study.

OBJECTIVE: We have analyzed, in a clinical multicenter study, the effect of cardiac surgery in adults with congenital heart disease in Italy. METHODS: We collected clinical data from 856 patients aged 19 years or older who underwent surgical intervention from January 1, 2000, to December 31, 2004. Patients were divided into 3 surgical groups: group 1, palliation (3.1%); group 2, repair (69.7%); and group 3, reoperation (27.4%). RESULTS: Preoperatively, 34.6% of patients were in New York Heart Association class I, 48.4% were in class II, 14.2% were in class III, and 2.8% were in class IV. Sinus rhythm was present in 83%. There were 1179 procedures performed in 856 patients (1.37 procedures per patient), with a hospital mortality of 3.1%. Overall mean intensive care unit stay was 2.3 days (range, 1-102 days). Major complications were reported in 247 (28.8%) patients, and postoperative arrhythmias were the most frequent. At a mean follow-up of 22 months (range, 1 month-5.5 years; completeness, 87%), late death occurred in 5 (0.5%) patients. New York Heart Association class was I in 79.3%, II in 17.6%, and III in 2.9%, and only 1 (0.11%) patient was in class IV. Overall survival estimates are 82.6%, 98.9%, and 91.8% at 5 years for groups 1, 2, and 3, respectively. Freedom from adverse events at 5 years is 91% for acyanotic patients versus 63.9% for preoperative cyanotic patients (P < .0001). CONCLUSIONS: Surgical intervention for congenital heart disease in adults is a safe and low-risk treatment. However, patients presenting with preoperative cyanosis show a higher incidence of late adverse events and complications.

Repair of complete atrioventricular septal defects in patients weighing less than 5 kg.

BACKGROUND: The aim of this study was to evaluate the impact of weight less than 5 kg at operation on mortality and morbidity in patients with atrioventricular septal defect (AVSDc) undergoing total correction. METHODS: Between January 1990 and December 2002, 190 consecutive patients with AVSDc underwent total biventricular correction. They were divided into two groups: group I (n = 64 patients weighing < 5 kg) and group II (n = 126 patients weighing > 5 kg). Associated major cardiac malformations were found in 49 (25.8%) patients. Associated left atrioventricular valve (LAVV) malformations were found in 35 (18.4%) patients. The mean follow-up time was 4.1 +/- 2.9 years (range 2 months-10.7 years). RESULTS: The in-hospital mortality in group I was 7.8% (5 patients) versus 8.7% (11 patients) in group II (p = 0.95). Major associated cardiac malformations (p < 0.001) and pulmonary hypertension (p = 0.006) were found to be strong predictors for poor postoperative survival. At discharge the mean LAVVR grade in group I was 1.45 +/- 1.2 versus 1.2 +/- 1 in group II (p = 0.13). The actuarial overall survival rates at 1, 3, 5, and 7 years were 96.5%, 92.5%, 91.5%, and 89% respectively and the actuarial overall reoperation free survival rates at 1, 3, 5, and 7 years were 95%, 87%, 84%, and 73%. Twenty-three patients underwent reoperation due to severe left atrioventricular valve regurgitation (LAVVR). Strong predictors for overall reoperation free survival were the operation year before 1995 (p < 0.001), postoperative LAVVR greater than or equal to 2 (p = 0.006), major associated cardiac malformations (p = 0.00034), associated LAVV malformations (p = 0.0044), and non or partial LAVV cleft closure (p = 0.012). The actuarial survival rates between patients weighing less than 5 kg versus patients weighing more than 5 kg were similar (p = 0.51); instead the overall reoperation free survival was significantly lower in patients weighing less than 5 kg (p = 0.022) according to the log-rank test. Weight less than 5 kg (p = 0.023, beta = -0.6) was one of the predictors for reoperation due to severe LAVVR in this series. CONCLUSIONS: We may conclude that in the current era repair of AVSDc can be carried out successfully in patients less than 5 kg, however, weight less than 5 kg at initial complete repair seems to be a predictor for late reoperation due to LAVVR. Suture separation at the cleft site or between the leaflets of the newly created mitral valve and the patch remain the main causes of postoperative LAVVR in patients weighing less than 5 kg.

Anomalous origin of the right pulmonary artery from the ascending aorta.

OBJECTIVES: The aim was to review our experience with the surgical repair of the anomalous origin of the right pulmonary artery (AORPA) from the aorta. MATERIALS AND METHOD: Between January 1991 and March 2001, five patients with AORPA underwent surgical correction. One patient presented isolated AOPA from the aorta. Implantation of the anomalous PA to the main PA trunk was performed by (1). direct anatomosis employing an autologous pericardial patch in two patients; (2). using an aortic flap in two patients with AORPA; and (3). using an aortic and pulmonary flaps in another patient. The mean follow-up time was 27 months. RESULTS: One patient died due to progressive heart failure unresponsive to inotropic support. Early postoperative pulmonary hypertension crisis was identified in patient 4, that was managed by intravenous prostacyclin. The same patient necessitated mechanical ventilation for 11 days. The mean residual gradient for all survivors was 9.5 +/- 4 mmHg. The postoperative Tc-99 m scintigraphy demonstrated 72 +/- 4.5(%) lung perfusion. At follow-up the survival was 100%. None of the followed patients required reoperation. CONCLUSION: The AORPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. The techniques employing autologous tissues for enlarging and lengthening the AORPA seem to be associated with better results in terms of postoperative restenosis.

Repair of complete atrioventricular septal defect with tetralogy of fallot: our experience and literature review.

OBJECTIVES: The aim of this report is to describe the rationale of our surgical approach, to explore the best management for complete atrioventricular septal defect associated with the tetralogy of Fallot (CAVSD-TOF), and to present our outcome in relation to the previously reported series. MATERIALS AND METHODS: Between January 1990 and January 2002, 17 consecutive children with CAVSD-TOF underwent complete correction. Nine patients (53%) underwent previous palliation. Mean age at repair was 2.9 +/- 1.9 years. Mean gradient across the right ventricular outflow tract was 63 +/- 16 mmHg. All children underwent closure of septal defect with a one-patch technique, employing autologous pericardial patch. Maximal tissue was preserved for LAVV reconstruction by making these incisions along the RV aspect of the ventricular septal crest. LAVV annuloplasty was performed in 10 (59%) patients. Six patients (35%) required a transannular patch. RESULTS: Three (17.6%) hospital deaths occurred in this series. Causes of death included progressive heart failure in two patients and multiple organ failure in the other patient. Two patients required mediastinal exploration due to significant bleeding. Dysrhythmias were identified in 4 of 11 patients undergoing a right ventriculotomy versus none of the patients undergoing a transatrial transpulmonary approach (p = ns). The mean intensive care unit stay was 3.2 +/- 2.4 days. Two patients required late reoperation due to severe LAVV regurgitation at 8.5 and 21 months, respectively, after the intracardiac complete repair. The mean follow-up time was 36 +/- 34 months. All patients survived and are in NYHA functional class I or II. The LAVV regurgitation grade at follow-up was significantly lower than soon after operation, 1.1 +/- 0.4 versus 1.7 +/- 0.5 (p = 0.002). At follow-up, the mean gradient across the right ventricular outflow tract was 17 +/- 6 mmHg, significantly lower than preoperatively (p < 0.001). CONCLUSIONS: Complete repair in patients with CAVSD-TOF seems to offer acceptable early and mid-term outcome in terms of mortality, morbidity, and reoperation rate. Palliation prior to complete repair may be reserved in specific cases presenting small pulmonary arteries or severely cyanotic neonates. The RVOT should be managed in the same fashion as for isolated TOF; however, a transatrial transpulmonary approach is our approach of choice.

Total correction of complete atrioventricular septal defect with tetralogy of Fallot.

BACKGROUND AND AIM OF THE STUDY: The authors' experience in surgical management of tetralogy of Fallot in association with complete atrioventricular septal defect (CAVSD-TOF) is reported. METHODS: Between January 1990 and September 2001, 146 children with CAVSD underwent complete correction. ASD closure was performed using a one-patch technique, with autologous pericardial patch. Seventeen patients presented with CAVSD-TOF, of whom nine (53%) had undergone previous palliation. Mean age at repair was 2.9 +/- 1.9 years. The mean gradient across the right ventricular outflow tract (RVOT) was 63 +/- 16 mmHg. Six patients (35%) required a transannular patch. RESULTS: Overall in-hospital mortality was 10%. Among patients with CAVSD-TOF, three died in hospital (18%); causes of death were progressive heart failure (n = 2) and multiple organ failure (n = 1). Two patients required mediastinal exploration due to significant bleeding, and two late reoperation for severe left atrioventricular valve regurgitation after intracardiac repair. Mean follow up was 36 +/- 34 months. All patients survived and are currently in NYHA class I or II. At follow up, mean gradient across the RVOT was 17 +/- 6 mmHg, significantly less than the preoperative value (p < 0.001). CONCLUSION: Complete repair in patients with CAVSD-TOF offers acceptable early and mid-term outcome in terms of mortality, morbidity and reoperation rate. Palliation prior to repair may be preferred in cases with small pulmonary arteries, or in severely cyanotic neonates. The RVOT should be managed as for isolated TOF, but a transatrial-transpulmonary approach is the surgery of choice.

Postoperative outcome in patients with anomalous origin of one pulmonary artery branch from the aorta.

OBJECTIVES: The aim was to review our experience with the surgical repair of the anomalous origin of one pulmonary branch from the aorta (AOPA). MATERIALS AND METHOD: Between January 1991 and March 2002, eight patients with AOPA underwent surgical correction. Three patients presented isolated AOPA. Five patients presented right AOPA and three, left AOPA. Implantation of the AOPA to the main pulmonary artery was performed by: (I) direct anastomosis in two patients with left AOPA; (II) interposition of a synthetic graft in one patient with left AOPA; (III) employing an autologous pericardial patch in two patients with right AOPA; (IV) using an aortic flap in three other patients with right AOPA. The mean follow-up time was 37.7 months. RESULTS: One patient died postoperatively due to progressive heart failure unresponsive to inotropic support. Early postoperative pulmonary hypertension crisis was identified in another patient. Within 1 year after surgery, the mean residual gradient across the anastomotic site at follow-up was 14+/-8 mmHg. The patient undergoing interposition of a synthetic graft presented a residual gradient of 29 mmHg and underwent reoperation at almost 2.5 years after the first correction. The residual gradient in patients undergoing correction according to technique I was 17+/-3 mmHg, and in patients undergoing implantation of the AOPA according to techniques III or IV was 9.5+/-4.6 mmHg (P=0.11). Similarly, the Tc-99m scintigraphy demonstrated that a lower lung perfusion (the lung perfused from the respective AOPA compared with the contralateral lung) in patients undergoing AOPA implantation according to technique I was 59+/-6(%) and in patients undergoing techniques III or IV was 72+/-4.5(%) (P=0.038). At follow-up, all patients were alive. CONCLUSION: The AOPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. Patients presenting this anomaly may undergo correction using various surgical techniques with acceptable results. The techniques employing autologous tissues for enlarging and lengthening the AOPA seems to be associated with less restenosis at the anastomotic site, however, larger series of patients are required to confirm such outcome.

Anomalous origin of the left pulmonary artery from the aorta. Our experience and literature review.

Three patients with anomalous origin of the left pulmonary artery (AOLPA) from the aorta underwent surgical correction. Two patients presented with an isolated malformation and one with an associated ventricular septal defect. One of the patients with an isolated malformation presented with an AOLPA from the descending thoracic aorta. Implantation of the AOLPA to the main PA trunk was performed by direct anastomosis in two patients and by interposition of a synthetic graft in the other patient with AOLPA from the descending aorta. All patients survived the operation. Low cardiac output was identified in one patient. The mean residual gradient through the anastomotic site was 21 mmHg at follow-up. We found almost 72 cases with AOLPA reported in the literature with a high mortality rate in patients not undergoing surgery. The most frequently found associated malformation is tetralogy of Fallot and ventricular septal defect. AOLPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. Patients with AOLPA can undergo surgical repair with excellent results.

Early and long term outcome of the arterial switch operation for transposition of the great arteries: predictors and functional evaluation.

OBJECTIVES: The aims of this report were to study the early and late outcome in terms of mortality, freedom from reoperation, predictors for late pulmonary stenosis (PAS) and insufficiency of the neo-aortic valve (AVI) in patients with transposition of the great arteries (TGA) undergoing arterial switch operation (ASO). MATERIALS AND METHODS: Between January 1990 and December 2001, 134 patients with TGA underwent ASO. The patients were divided in Group I (n=88)-TGA with intact ventricular septum and Group II (n=46)-TGA with ventricular septal defect (VSD). The pulmonary artery was reconstructed employing the direct anastomosis technique (PT-I) in 21 (15.7%) patients, the double-patch technique (PT-II) in 41 (30.6%), single pantaloon patch (partial circumference) (PT-III) in 46 (34%) and single pantaloon patch (total circumference) (PT-IV) in 35 (26%) patients. The mean follow-up was 3.4+/-1.3 years. RESULTS: The hospital mortality was 17 (12.7%) patients. The mortality in Group I was significantly lower than Group II (P=0.002). The overall actuarial survival at 1, 3 and 5 years follow-up resulted to be 98, 93, and 91.5%, resulting to be significantly higher in Group I (P=0.032). The multivariate analysis revealed the complex TGA (P=0.007), VSD (P=0.032), coronary anomalies (P=0.004), aortic coarctation or hypoplastic aortic arch (P=0.021), left ventricular outflow tract obstruction (LVOTO) or moderate PAS (P=0.041) as strong predictors for poor free-reoperation cumulative survival. A strong inverse correlation was found between the mean trans-pulmonary gradient at follow-up and the age at the operation (r=-0.41, P<0.0001). The univariate analysis revealed the PT-I technique (P=0.002), prior moderate PAS (P=0.0001), and age <1 month (P=0.018) as strong predictors for moderate-to-severe PAS. The neo-AVI incidence was significantly higher in Group II (P=0.011). Predictors for neo-AVI were male sex (P=0.003), preoperative neo-AV Z-score >1 (P<0.001), prior or concomitant operation for aortic coarctation or hypoplastic aortic arch (P=0.001), LV retraining (P=0.003). CONCLUSION: ASO remains the procedure of choice for the treatment of various forms of TGA with acceptable early and later outcome in terms of overall survival and free reoperation. Strong predictors for poor overall free-reoperation survival are complex TGA, VSD, coronary anomalies, aortic coarctation and LVOTO or moderate PAS. The pulmonary artery reconstruction using a single 'pantaloon patch' seems to offer less residual stenosis. Patients with a VSD and a significant mismatch between the neo-aortic root and distal aorta are at a higher risk for developing postoperative neo-AVI.

Reconstructive surgery for congenitally malformed mitral valve.

BACKGROUND AND AIMS OF THE STUDY: The study aims were to evaluate early and mid-term survival and freedom from reoperation in patients with isolated mitral valve (MV) congenital malformation (MVCM) or in association with other cardiac defects, and to identify predictors for poor early and late outcome. METHODS: Between January 1990 and February 2001, 58 children with MVCM underwent MV-conserving surgery; patients were allocated to group I (n = 21) with isolated MVCM, and group II (n = 37) with MVCM and associated congenital heart defects (ACHD). MV stenosis was identified in 10 (48%) group I patients, and 11 (30%) group II patients (p = NS); MV insufficiency was present in 11 (52%) patients in group I, and in 26 (70%) of group II (p = NS). The most frequently found MVCM were annular dilatation (n = 13) cases and elongated chordae (n = 14). Hammock MV was found in nine patients (15%). RESULTS: Six (10%) hospital deaths occurred. Mortality was 5% (n = 1) in group I, and 13% (n = 5) in group II (p = 0.4). Mean repair techniques per patient was 2.05+/-0.4 and 2.3+/-0.3 in groups I and II respectively (p = 0.009). Mechanical ventilation time was 2.1+/-1 and 2.8+/-0.7 days in groups I and II (p = 0.003). The incidence of postoperative mitral regurgitation grade > or =1 was significantly higher in group II (p = 0.008). At five-year follow up, actuarial survival was 91.5% in group I and 86% in group II (p = 0.037). Actuarial reoperation-free survival was 85% in group I and 73% in group II (p = 0.01). Multivariate analysis showed age >12 months (p = 0.033), hammock MV (p = 0.0088) and ACHD (p = 0.0048) were strong predictors for poor event-free survival. CONCLUSION: MV repair for MVCM provides acceptable early and mid-term outcome in terms of mortality and freedom from reoperation. ACHD significantly reduce early and late postoperative survival and freedom from reoperation.

Repair of congenital malformations of the mitral valve: early and midterm results.

BACKGROUND: The aims of this study were to determine early and midterm survival and freedom from reoperation, and to identify the predictors for poor postoperative outcome in children undergoing mitral valve (MV) repair owing to congenital malformations of the mitral valve. METHODS: Between January 1990 and February 2001, 94 consecutive children with congenital MV disease underwent valve repair. The mean age was 5.2+/-3.3 years (range 20 days to 15 years). Twenty-five (26.6%) children were less than 1 year old. Isolated MV disease was found in 21 (22.4%) patients. MV stenosis was the predominant lesion in 21 (22.4%) patients with a mean left atrial to left ventricle diastolic peak gradient of 24.5+/-9.2 mm Hg. MV regurgitation was the predominant pathophysiology in 73 (77.6%) patients with a mean regurgitation grade of 3.3+/-0.7. RESULTS: The hospital mortality was 8.5% (8 of 94). Three patients required permanent pacemaker implantation owing to complete atrioventricular block. Two patients underwent mediastinal exploration for significant bleeding. Postoperatively the echocardiography color Doppler study demonstrated a significantly lower mean end diastolic left atrium to left ventricle gradient 8.7+/-2.2 mm Hg (p < 0.001) in patients with MV stenosis and a mean regurgitation grade of 0.9+/-0.6 (p < 0.001) in patients with MV regurgitation. Actuarial survival and actuarial reoperation-free survival were 89.2% and 76.3%, respectively. Multivariate analysis demonstrated that age less than 1 year (p = 0.035), hammock MV (p = 0.0093), cardiothoracic ratio greater than 0.6 (p < 0.0001), and associated cardiac anomalies (p = 0.003) were strong predictors for poor overall freedom from reoperation and midterm survival. CONCLUSIONS: Mitral valve repair for congenital mitral valve disease yields acceptable early and midterm mortality and reoperation rates. Strong predictors for poor overall freedom from reoperation and midterm survival were age less than 1 year, hammock MV, cardiothoracic ratio greater than 0.6, and associated cardiac anomalies.