Quality of life of adult Fontan patients.

BACKGROUND: Progress in the management of complex congenital heart disease (CHD) led to an improvement in survival rates of adults with a Fontan-like circulation. The objective of this study was to assess the subjective health status and quality of life of this population. METHODS AND RESULTS: Patients aged more than 18 years at the time of the study, who underwent a Fontan-like procedure. Subjective health status was assessed by the SF-36 questionnaire and a linear analog scale was used to score patients' self-perception of their quality of life; cardiac and demographic parameters were collected. RESULTS: Among 65 eligible patients, 60 (23 females; mean ± SD age: 25.7 ± 7.2 years) answered the SF-36 questionnaire and 46 of these were interviewed to evaluate their perceived quality of life. Among them, 20 (33.3%) were working full-time and 21 (35%) experienced arrhythmias. The physical SF-36 scores were lower in patients than in the general population (p ≤ 0.05). The New York Hear Association (NYHA) class and occupation were correlated with SF-36 scores of physical activity (respectively, p = 0.0001 and p = 0.025). SF-36 scores of psychological status were associated with the number of drugs and occupation (respectively, p = 0.0001 and p = 0.02). The mean ± SD quality of life score measured using a linear analog scale was 7.02 ± 1.6 and was linked to education and occupation (p ≤ 0.05) but not with cardiac parameters. CONCLUSION: Adult Fontan patients perceive an impaired physical health but report a good overall quality of life. Education and occupation impacts significantly on Fontan patients' quality of life.

Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis.

Background Total anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques. Methods and Results We performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p < 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms, p = 0.0023), and length of aortic cross clamp time (p = 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years. Conclusions The overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.

Ventricular Arrhythmias in Adults With Congenital Heart Disease, Part I: JACC State-of-the-Art Review.

Patients with congenital heart disease associated with a higher risk for ventricular arrhythmias (VA) and sudden cardiac death (SCD) can be divided conceptually into those with discrete mechanisms for reentrant monomorphic ventricular tachycardia (VT) (Group A) and those with more diffuse substrates (Group B). Part I of this review addresses Group A lesions, which predominantly consist of tetralogy of Fallot and related variants. Well-defined anatomic isthmuses for reentrant monomorphic VT are interposed between surgical scars and the pulmonary or tricuspid annulus. The most commonly implicated critical isthmus for VT is the conal septum that divides subpulmonary from subaortic outlets. Programmed ventricular stimulation can be helpful in risk stratification. Although catheter ablation is not generally considered an alternative to the implantable cardioverter-defibrillator (ICD) for prevention of SCD, emerging data suggest that there is a subset of carefully selected patients who may not require ICDs after successful monomorphic VT ablation.

Ventricular Arrhythmias in Adults With Congenital Heart Disease, Part II: JACC State-of-the-Art Review.

There are marked variations in the incidence of sudden cardiac death (SCD) and in the substrates for ventricular arrhythmias (VAs) across the gamut of congenital heart defects. In this 2-part review, patients with higher-risk forms of congenital heart disease (CHD) were conceptually categorized into those with discrete anatomic isthmuses for macro-reentrant ventricular tachycardia (VT) (Group A) and those with more diffuse or less well-defined substrates (Group B) that include patchy or extensive myocardial fibrosis. The latter category encompasses CHD lesions such as Ebstein anomaly, transposition of the great arteries with a systemic right ventricle (RV), and congenital aortic stenosis. For Group B patients, polymorphic VT and ventricular fibrillation account for a higher proportion of VA. The prognostic value of programmed ventricular stimulation is less well established, and catheter ablation plays a less prominent role. As cardiomyopathies evolve over time, pathophysiological mechanisms for VA among Groups A and B become increasingly blurred.

Direct Reimplantation Procedure in Anomalous Aortic Origin of the Right Coronary Artery: Long-Term Single Center Outcomes.

BACKGROUND: Anomalous aortic origin of the right coronary artery is known to be a cause of sudden cardiac death in athletes. There are no specific guidelines concerning treatment strategy in the literature. The aim of this study is to describe and report our experience of direct reimplantation technique in the treatment of this anomaly. METHODS: This was a retrospective single center study of 30 patients who underwent surgery in the congenital heart disease unit of Louis Pradel Heart and Lung Hospital between January 2003 and December 2016. The mean follow-up was seven years (3 months-17 years). RESULTS: Thirty patients underwent surgery. The median age was 17 years (0.2-52 years). There were 24 males. The median weight was 58 kg (3.6-118 kg). Fourteen patients were actively engaged in sports. Twenty-six patients had exertional chest pain or syncope. The median time lapse between diagnosis and intervention was 4.5 months (0.5-179 months). Twenty-seven (90%) patients underwent reimplantation of the anomalous coronary artery without transverse aortotomy, while in 3 (10%) patients transverse aortotomy was used to facilitate reimplantation to avoid tension at the anastomosis. There was no early death; one late death occurred in the third postoperative month. At the last follow-up, all patients had returned to normal physical activity without evidence of ischemia. CONCLUSIONS: Direct reimplantation allows for a complete restoration of the coronary anatomy and enables patients to return to normal physical activity. Our study shows encouraging results using a direct reimplantation technique without aortotomy.

Diagnosis and treatment of anomalous aortic origin of coronary artery: A twenty-year retrospective study of experience and decision-making in children and young adults.

BACKGROUND: Anomalous aortic origin of a coronary artery, particularly in the presence of inter-arterial course between the great arteries, has been found to be associated with sudden death in young people. METHODS: This study reports a single-center experience in the management of anomalous aortic origin of a coronary artery, with or without inter-arterial course, by focusing specifically on presentation, diagnosis, and patient outcome. RESULTS: From March 1993 to February 2018, 100 patients (70 males) were diagnosed with anomalous aortic origin of a coronary artery, including 27 left coronary artery from the right sinus, 60 right coronary artery from the left sinus, and 13 other anomalies. Patients with inter-arterial course between the great arteries presented more frequently with syncope and/or sudden death compared to patients without (23.4% vs. 0%, p = 0.026). Diagnosis was reached using first-line transthoracic echocardiography in 65% of cases. Surgical repair was performed in 61 patients (61%). All operated patients (60 direct implantations, 1 unroofing) had been diagnosed with inter-arterial course between the great arteries, and were asymptomatic at a mean (±SD) postoperative follow-up of 4.9 ± 5.3 years. CONCLUSIONS: An inter-arterial course of the anomalous right or left coronary arteries arising from the opposite sinus is associated with life-threatening events. Direct reimplantation of coronary artery is reliable, and should be discussed even in asymptomatic patients.

Double orifice and atrioventricular septal defect: dealing with the zone of apposition†.

OBJECTIVES: A double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations. METHODS: We performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month-32 years). RESULTS: Associated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination. CONCLUSIONS: Repair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.

Supravalvular Aortic Stenosis Caused by a Familial Chromosome 7 Inversion Disrupting the ELN Gene Uncovered by Whole-Genome Sequencing.

Apparently, balanced chromosomal rearrangements usually have no phenotypic consequences for the carrier. However, in some cases, they may be associated with an abnormal phenotype. We report herein the case of a 4-year-old boy presenting with clinically isolated supravalvular aortic stenosis (SVAS). No chromosomal imbalance was detected by array CGH. The karyotype showed a balanced paracentric chromosome 7 inversion. Breakpoint characterization using paired-end whole-genome sequencing (WGS) revealed an ELN gene disruption in intron 1, accounting for the phenotype. Family study showed that the inversion was inherited, with incomplete penetrance. To our knowledge, this is the first case of a disruption of the ELN gene characterized by WGS. It contributes to refine the genotype-phenotype correlation in ELN disruption. Although this disruption is a rare etiology of SVAS, it cannot be detected by the diagnostic tests usually performed, such as array CGH or sequencing methods (Sanger, panel, or exome sequencing). With the future perspective of WGS as a diagnostic tool, it will be important to include a structural variation analysis in order to detect balanced rearrangements and gene disruption.

Increased intra-abdominal pressure affects respiratory variations in arterial pressure in normovolaemic and hypovolaemic mechanically ventilated healthy pigs.

OBJECTIVE: To evaluate the effect of increased intra-abdominal pressure (IAP) on the systolic and pulse pressure variations induced by positive pressure ventilation in a porcine model. DESIGN AND SETTING: Experimental study in a research laboratory. SUBJECTS: Seven mechanically ventilated and instrumented pigs prone to normovolaemia and hypovolaemia by blood withdrawal. INTERVENTION: Abdominal banding gradually increased IAP in 5-mmHg steps up to 30 mmHg. MEASUREMENTS AND MAIN RESULTS: Variations in systolic pressure, pulse pressure, inferior vena cava flow, and pleural and transmural (LVEDPtm) left-ventricular end-diastolic pressure were recorded at each step. Systolic pressure variations were 6.1+/-3.1%, 8.5+/-3.6% and 16.0+/-5.0% at 0, 10, and 30 mmHg IAP in normovolaemic animals (mean+/-SD; p<0.01 for IAP effect). They were 12.7+/-4.6%, 13.4+/-6.7%, and 23.4+/-6.3% in hypovolaemic animals (p<0.01 vs normovolaemic group) for the same IAP. Fluctuations of the inferior vena cava flow disappeared as the IAP increased. Breath cycle did not induce any variations of LVEDPtm for 0 and 30 mmHg IAP. CONCLUSIONS: In this model, the systolic pressure and pulse pressure variations, and inferior vena cava flow fluctuations were dependent on IAP values which caused changes in pleural pressure swing, and this dependency was more marked during hypovolaemia. The present study suggests that dynamic indices are not exclusively related to volaemia in the presence of increased IAP. However, their fluid responsiveness predictive value could not be ascertained as no fluid challenge was performed.

Surgical repair of a pseudocoarctation with cervical aortic arch complicated by multiple aneurysms of the aorta: a case report.

Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and deformity of the aortic arch and is known to be associated with aneurysmal formation. Several studies unite to say it leads to a surgical sanction as soon as symptomatic or associated with aneurysms of the aortic arch. Our patient is a 12 years old boy, followed since birth for a little tight pseudocoarctation with a cervical aortic arch and transverse aortic arch hypoplasia. Close clinical and paraclinical monitoring including angioscans, showed the gradual enlargement of the superior mediastinum, in relation with the appearance of three aneurysms of the aortic arch. The intervention, performed by sternotomy, has consisted of the resection of the aneurysmal area and the interposition of a Dacron tube to repair the aortic arch and the reimplantation of the left subclavian artery into the left carotid artery. The postoperative course was uneventful. Management of pseudocoarctation associated with cervical aortic arch and aneurysms remains surgical. Close monitoring of patients with pseudocorctation, seems to be essential to avoid fatal complications such as aneurysmal rupture.

Comparison between 3 aortic clamps for video-assisted cardiac surgery: a histological study in a pig model.

OBJECTIVES: To assess histological traumatic effects of aortic clamps used in video-assisted surgery, an experimental study was undertaken in a pig model, comparing the Portaclamp, Endoclamp, and a metallic clamp. MATERIAL AND METHODS: In 3 groups of 5 pigs each, the descending aorta was exposed through a posterolateral left thoracotomy. External clamps (Portaclamp and metallic clamp) were positioned at the middle of the aorta. Endoclamps were inserted at the top of the descending aorta through a small purse and inflated lower. After 60 minutes of clamping, the clamp was removed and the animal reperfused for 60 minutes. It was then sacrificed and the descending aorta was harvested for blind histological study using hemotoxylin-eosin staining of 4 samples per animal: A, before the clamping spot; B, at the clamping spot; C, after the clamping spot; D, a remote sample as control. RESULTS: In the Portaclamp and metallic clamp groups, there were no lesions of the intima in all aortic samples. In the Endoclamp group, severe lesions of the intima were observed on the clamping spot: endothelium crushing with flattening of cell nucleus (3/5) or endothelium stripping with vanishing of cell nucleus all gathered in 1 point (2/5). Only spongy lesions (clearance between fibers) located on the external third of the media and moderate inflammatory lesions of the adventice were observed with a random distribution in aortic samples without difference between groups. CONCLUSIONS: This study reveals the impressive lesions of the aortic intima due to the Endoclamp. The nonspecific lesions observed in media or adventice may be related to the surgical trauma of the procedure.

Reimplantation of left coronary artery from pulmonary artery in a 65-year-old.

Anomalous left coronary artery to the pulmonary artery is a rare pathology with a mortality rate of 90% in the first year of life, directly related to left ventricular function and coronary perfusion, although several adult cases have been reported. Surgical correction consists of ligation of the anomalous left coronary artery associated with coronary artery bypass grafting. We describe the exceptional case of a 65-year-old woman who underwent reimplantation of the left coronary artery in the anatomical position without bridging.

A new surgical technique for transcatheter Fontan completion.

OBJECTIVES: Patients with complex congenital heart defects and univentricular heart usually required multiple palliative surgery aiming to separate pulmonary and systemic circulations. Various groups work on trying to perform the Fontan completion by a transcatheter technique. We developed and report here a modified technique to prepare the patient for this type of procedure. METHODS: Sixteen sheep were included and prepared through a midline sternotomy. Preparation for transcatheter completion was performed using specially designed devices. The superior vena cava (SVC) was connected to the pulmonary artery (PA) using a Goretex conduit. The connection between the SVC and the right atrium (RA) was interrupted using a vascular stent occluded in the middle by a polytetrafluoroethylene (PTFE) membrane. Two nitinol rings were placed around the inferior vena cava (IVC). Immediately after preparation, the transcatheter completion was performed by first perforating the membrane of the occluded stent and by placing covered stents from IVC rings to the SVC stent. RESULTS: Creation of the SVC to PA connection was uneventful. The insertion of the rings was done successfully in all animals very easily. All stents but one were completely occluding the SVC. Once, a tiny leak was noticed and successfully treated by additional stitches around the stent. Perforation of the membrane was done successfully in all animals re-establishing the pathway between the SVC and the RA. After dilatation of the stent, one animal required surgical control of a haemorrhage related to disruption of the SVC anastomosis. Three to four Cheatham-platinum (CP)-covered stents were necessary to complete the Fontan-like circulation. In the first animal, one stent moved downward missing its target. The placement between the two rings was easily performed using fluoroscopic guidance. No stents were positioned above the SVC stent keeping this part free of material. CONCLUSIONS: We describe new improvements for surgical preparation for transcatheter completion of partial cavopulmonary connection. For superior connection, we developed an occluding stent that helps to re-establish RA to PA connection, alloys precise placement and enhances the stability of the completion stents. Inferiorly, anchorage of the covered stents is also improved by the use of nitinol rings.

Early prophylactic pulmonary artery banding in isolated congenitally corrected transposition of the great arteries.

OBJECTIVE: Mid-term evaluation of an aggressive surgical management of isolated congenitally corrected transposition of the great arteries (ccTGA) by pulmonary artery (PA) banding in early infancy. METHODS: Between 2001 and 2009, 11 asymptomatic patients (seven neonates and four infants) underwent a dilatable, partially adjustable, homemade PA banding for ccTGA with intact ventricular septum. PA band circumference was correlated to body weight (22 mm+1 mm kg(-1)) and ideally adjusted to obtain flat septal geometry. Mean age at operation was 1.5±1.4 months. RESULTS: There was no hospital mortality. Mean ventilation time and intensive care unit (ICU) stay were 20±9 h and 2.6±1.5 days, respectively. Five patients required postoperative inotropic support. One late death occurred suddenly at 4 months; normal biventricular function and no tricuspid regurgitation were noted at last follow-up, 1 week before death. Mean follow-up was 21.5±26 months. Mean band velocity increased over time from 2.65±0.7 m s(-1) postoperatively to 3.7±0.3 at 6 months and 4.5±0.4 m s(-1) at 2 years. Tricuspid regurgitation remained stable in seven patients, decreased in three and worsened in one. Flat septal geometry was obtained in all patients after the third postoperative month. One patient underwent a double-switch procedure at 7 years due to suprasystemic morphologically left ventricular pressure. The postoperative course was uneventful. CONCLUSIONS: In neonates with isolated ccTGA, prophylactic PA banding is safe and carries a low morbidity. At mid-term evaluation, tricuspid valve function is stabilised or improved and systemic competence of the left ventricle is maintained, thus allowing double switch if indicated.

Intramural coronary arteries and outcome of neonatal arterial switch operation.

OBJECTIVE: To evaluate the impact of coronary patterns with intramural arteries on the outcome of arterial switch operation (ASO) in neonates with transposition of the great arteries (TGA). METHODS: Between 1987 and 2008, 919 neonates underwent ASO for TGA. Forty-six (5.0%) had intramural coronary arteries. Intramural course involved the left main coronary artery in 28 of the 46 cases (61%), the left anterior descending artery in 12 patients (26%), the right coronary artery in three and both right and left coronary arteries in three cases. Various techniques were used to manage the coronary arteries: ASO without coronary relocation in one, ASO with coronary transfer as a single coronary button in nine and ASO with coronary transfer as two separate buttons in 36 patients (additional pericardial patches were implanted to orientate the coronary button in nine cases or enlarge the coronary ostium in three cases). The intramural course was unroofed in most cases (after 1995). RESULTS: There were 13 deaths (28%): two intra-operative, nine before discharge from the hospital and two after discharge; during the same period, overall mortality in the 873 neonates with other coronary patterns was 3.9%. Actuarial survival at 10 years was 71 + or - 7%. Most deaths (11/13, i.e., 85%) were related to coronary complications. No time-trend effect was noted regarding mortality. Non-fatal coronary lesions were detected in eight patients (three with clinical evidence of myocardial infarction and five without). Five patients underwent re-operation for coronary revascularisation. Actuarial freedom from coronary events at 10 years was 46 + or - 10%. After a mean follow-up of 8.3 + or - 4.8 years, left ventricular function was normal in 97% of the survivors; minor ischaemic sequelae were present in two patients. CONCLUSIONS: Coronary patterns with intramural arteries remain associated with high coronary mortality and morbidity following neonatal ASO, even in the current era. The association of slit-like deformation of the ostium, stenosis of the intramural course and abnormal angle of take-off might explain the difficulty in coronary transfer. The technique of coronary transfer should be individually adapted to each anatomical situation. The place of patch ostioplasty of the intramural artery remains to be determined.

Central cannulation in pediatric cardiac surgery.

The present paper describes the cannulation techniques which are used routinely in our institution. They are established on two basic principles: most operations are performed under full-flow normothermic cardiopulmonary bypass (CPB); deep hypothermic circulatory arrest is avoided. When full-flow conventional CPB is used, cannulation of the ascending aorta and both venae cavae is usually required and should be performed with great caution (with institution of partial bypass as soon as possible) to provide hemodynamic stability during the cannulation process. Additional cannulations and/or technical modifications may be necessary to accommodate frequent associated anomalies (left superior vena cava, systemic or pulmonary venous return anomalies, multiple reoperations). In children requiring aortic arch repair, moderate hypothermia, circulatory arrest and selective cerebral perfusion are used and require specific techniques for arterial cannulation.

Common arterial trunk repair: with conduit or without?

OBJECTIVE: To compare the mid-term results of two techniques used for the reconstruction of the pulmonary outflow tract during common arterial trunk repair in infancy, with special attention paid to re-operation rate and pulmonary arterial growth. METHODS: Between 2000 and 2006, 32 consecutive neonates or infants underwent common arterial trunk repair. In 15 patients, the pulmonary outflow tract was reconstructed using an extracardiac valved conduit (conduit group). In 17 patients, right ventricle to pulmonary artery connection was achieved without conduit, using the left atrial appendage and including a monocusp valve (non-conduit group). The decision regarding the type of ventricle to pulmonary artery connection was at the discretion of the attending surgeon. The two groups were similar in terms of age, weight, type of common arterial trunk, truncal valve dysfunction and coronary abnormalities. Follow-up was 93% complete and included echo-Doppler evaluation, catheterisation and CT scan imaging. RESULTS: Hospital mortality (five patients - 16%) was increased by coronary abnormalities and preoperative ventilation but did not differ between the two groups (13.3% in the conduit group vs 18% in the non-conduit group). The mean follow-up was 40+/-25 months. There were six late deaths (three in each group), yielding an actuarial survival of 76% at 5 years. One late death was procedure related (percutaneous dilatation for obstructive monocusp patch). Re-operation for right ventricular outflow tract obstruction was necessary in seven patients (five in the conduit group and two in the non-conduit group); the actuarial freedom from re-operation was higher in the non-conduit group (p=0.026). At last follow-up, the right ventricle-pulmonary artery gradient and the right ventricle/left ventricle pressure ratio were higher in the conduit group (p=0.006 and p=0.007, respectively). At late computed tomography (CT)-scan evaluation, the growth of the proximal pulmonary arterial tree had improved in the non-conduit group, as shown by a higher Nakata ostial index and right ventricular outflow tract growth. CONCLUSIONS: Repair of common arterial trunk without conduit for right ventricular outflow tract reconstruction (1) does not increase mortality and morbidity, (2) decreases the need for re-intervention and (3) promotes a better growth of the proximal pulmonary arteries. These preliminary results need confirmation by further experience.

Anomalous origin of the left coronary artery from the pulmonary artery: late results with special attention to the mitral valve.

OBJECTIVE: Evaluate the late results of a uniform approach to the surgical management of children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). METHODS: Between 1986 and 2007, 62 children with ALCAPA underwent surgery. The median age at operation was 16 months (range 10 days to 11 years). A uniform approach was applied, including (1) immediate surgery as soon as the diagnosis was established, (2) direct aortic reimplantation of the anomalous artery, when technically feasible (61/62, 98%), and (3) no concomitant mitral valve surgery, regardless of the severity of mitral regurgitation (59/62, 95%). The mean follow-up was 9.7 years (range 3 months to 21 years) and was 98% complete. RESULTS: There were six hospital deaths (9.7%). Left ventricular assistance was used in four patients; two died of related complications. The poor left ventricular ejection fraction was an incremental risk factor for early mortality (p = 0.043); severity of mitral regurgitation was not. There were two late deaths, yielding an actuarial survival rate of 86% at 15 years. Five patients underwent reoperation (mitral valve repair in three, coronary procedure in two); the actuarial freedom from reoperation was 89% at 15 years. Left ventricular function recovered in all survivors. In the 50 late survivors who did not undergo mitral surgery at initial operation, the severity of mitral regurgitation decreased in 58%, remained unchanged in 40% (of which 3 patients underwent reoperation for mitral valve repair) and worsened in 2%; at last follow-up, mitral regurgitation was absent or trivial in 42%, mild in 50%, moderate in 8% and severe in 0%. CONCLUSIONS: (1) Early mortality is related to the severity of preoperative left ventricular dysfunction; it may be reduced by a careful use of postoperative cardiac support techniques. (2) Late results are satisfactory and left ventricular function always recovers. (3) Mitral regurgitation improves along with left ventricular function, but recovery may be incomplete and need reoperation. The data suggest that mitral valve surgery is probably not indicated at initial surgery, except in selected cases with a low potential of recovery (severe regurgitation with relatively well-preserved left ventricular function).

Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?

BACKGROUND: Various surgical techniques have been proposed to repair supravalvular aortic stenosis. However, the optimal approach remains to be determined. The present study was undertaken to evaluate the results of surgery for supravalvular aortic stenosis and to compare the symmetric three-patch repair (Brom technique) with other surgical reconstructions. METHODS: Between 1995 and 2007, 34 patients with supravalvular aortic stenosis underwent surgery. Fourteen patients (41%) had Williams syndrome. Supravalvular stenosis was discrete in 24 patients (71%) and diffuse in 10 (29%). Eight patients (23%) underwent one-patch repair; 3 patients (9%) had inverted bifurcated patch enlargement (Doty repair) and 23 patients (68%) had symmetric repair (Brom three-patch technique). Follow-up was complete and ranged from 6 months to 12 years (mean, 5.8 years). RESULTS: There was one early and no late deaths. One patient (with one-patch repair) underwent reoperation for residual obstruction. At last follow-up, left ventricle to aorta peak gradient was 45 +/- 28 mm Hg after one-patch repair, 30 +/- 9 mm Hg after Doty operation, and 11 +/- 18 mm Hg after symmetric Brom procedure. Brom repair was associated with a low incidence of residual obstruction (peak gradient > or = 40 mm Hg) (2 of 22; 9.1%) and moderate aortic insufficiency (1 of 22; 4.5%). CONCLUSIONS: Brom three-patch repair provides symmetric reconstruction of the aortic root in patients with supravalvular aortic stenosis. This may lead to improved midterm results in terms of relief of the obstruction and incidence of aortic insufficiency.