Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

INTRODUCTION: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. MATERIALS AND METHODS: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). RESULTS: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). CONCLUSIONS: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

Late management of the aortic root after repair of tetralogy of Fallot: A European multicentre study.

OBJECTIVES: We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). METHODS: Eleven centers belonging to the European Congenital Heart Surgeons Association contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). RESULTS: The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death occurred in five patients (5/31, 16%). Most survivors (15/26, 58%) were asymptomatic at the last clinical examination (New York Heart Association, NYHA class I). The remaining patients were NYHA class II (n = 7) and III (n = 3). The most common symptoms were fatigue (n = 5), dyspnea (n = 4), and exercise intolerance (n = 3). CONCLUSIONS: Reoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.

Arterial switch operation for transposition of the great arteries: A single-centre 32-year experience.

OBJECTIVE: Arterial switch operation (ASO) is nowadays the standard of care for neonates with D-transposition of the great arteries (D-TGA). We evaluated our early and late results with ASO for D-TGA. MATERIALS AND METHODS: We collected data on 267 patients with D-TGA, either with intact ventricular septum (D-TGA/IVS; n = 182, 68%) and with other associated congenital heart malformations (complex D-TGA; n = 85, 32%) that underwent ASO between January 1987 and July 2018. RESULTS: Median age at ASO was 8 days (interquartile range [IQR], 6-12 days). Fifteen patients (5.6%) died in hospital (6/182 with D-TGA/IVS, 3.3% and 9/85 with complex D-TGA, 11%; P = 0.02). Median follow-up time was 10.2 years (IQR, 3.7-18 years). There were 2 (0.8%) late sudden deaths. Overall survival at 10 and 20 years was 94% and 93%, respectively. Thirty-five patients (14%) required either reoperations or reinterventions, mainly for right ventricular outflow tract obstruction (n = 28, 11%). Freedom from reoperation/reintervention at 10 and 20 years was 87% and 78%, respectively. All patients were in NYHA I at latest clinical examination. Six over 173 patients (3.4%) who underwent a postoperative evaluation of their coronaries presented acquired anomalies. Forty-four patients (17%) who performed a cardiopulmonary exercise testing (CPET) have a predicted VO 2 comparable to normal peers. CONCLUSIONS: The results of ASO for D-TGA are excellent, with a fairly low mortality and reoperation/reintervention rate. Functional capacity evaluated with CPET is comparable to normal peers. Continuous follow-up for detecting asymptomatic acquired coronary artery disease is mandatory. A reassessment of competitive sport eligibility criteria for specific D-TGA patients should be considered.

Repair Techniques for Mitral Valve Insufficiency in Children.

Congenital mitral valve (MV) dysplasia is a relatively rare and highly complex cardiac disease. We sought to provide a comprehensive analysis of the current surgical techniques for treating mitral valve insufficiency and the results of mitral valve repair at our institution. Between 1972 and 2017, 104 consecutive patients underwent surgical repair of congenital MV dysplasia-insufficiency at our institution. Among these, 59 patients presented with MV insufficiency (or prevalent MV insufficiency) and were part of the study. There was 1 early (1.7%) and 1 late death (1.7%). Survival at 5, 10 and 20 years was 98%, 98% and 94%, respectively. Eight patients (14%) required MV replacement for residual significant MV insufficiency. Freedom from re-intervention for MV dysfunction at 5, 10 and 20 years were 89%, 84% and 79%, respectively. Surgical techniques for treating mitral valve insufficiency must be tailored for each patient with the main goal of achieving a proper valve function, rather than a "normal" anatomy. The mechanism underlying valve dysfunction in congenital mitral valve insufficiency is multifactorial and requires the application of a variety of surgical techniques in each case.

Prevalence and prognosis of pericardial effusion in patients affected by pectus excavatum: A case-control study.

BACKGROUND: The presence of pectus excavatum(PEX) has been occasionally associated with pericardial effusion. Aim of the present study was to compare incidence and prognosis of pericardial effusion in a group of unselected patients with PEX vs a control group. METHODS: From a prospective registry of consecutive patients who underwent chest CT for cardiovascular disease, subjects with a radiological diagnosis of PEX were retrospectively identified (cases); from the same registry patients (controls) without rib cage abnormalities were randomly selected, until a 1:2 ratio was reached. The presence of pericardial effusion at CT was quantified. Follow-up was obtained for a composite end-point: cardiac tamponade, need for pericardiocentesis, need for cardiac surgery for relapsing pericardial effusion. RESULTS: A total of 43 patients with PEX (20 females) and a control group of 86 cases (31 females) without rib cage abnormalities were identified. Pericardial effusion evaluated at CT was significatively more prevalent in patients with PEX vs control group, 37.2% vs 13.9% (p < 0.001), respectively; four patients with PEX (9.3%) had at least moderate pericardial effusion vs no subjects among the controls (p = 0.004). PEX diagnosis was significantly associated to pericardial effusion at multi-variate analysis (OR95%CI 10.91[3.47-34.29], p < 0.001). At a mean follow-up of 6.5 ± 3.4 years no pericardial events were recorded. CONCLUSION: Our findings support the higher prevalence of pericardial effusion in patients with PEX when compared to a control group. The absence of adverse pericardial events at follow-up suggest the good prognosis of these effusions, that in the appropriate clinical setting might not be considered "idiopathic".

Reversing Inoperability in Eisenmenger Syndrome: The "Drug-and-Banding" Approach.

Eisenmenger syndrome (ES) has been considered a cause of inoperability in patients with congenital heart disease (CHD). Even if long-standing vasodilators are efficient to reduce pulmonary vascular resistance (PVR), the best approach to grant stable repair of these patients is still unknown. We describe the case of a 28-year-old man with a diagnosis of untreated large ventricular septal defect and established ES. After few years of vasodilator therapy, the patient underwent pulmonary banding with significant reduction of his PVR. His CHD was then repaired, with clinical and functional improvement at 2-year follow-up.

Surgery for Adult Patients with Congenital Heart Disease: Results from the European Database.

Adults with congenital heart disease (ACHD) represent a growing population. To evaluate frequency, type and outcomes of cardiac surgery in ACHD, we gathered data from the European Congenital Heart Surgeons Association Database of 20,602 adult patients (≥18 years) with a diagnosis of congenital heart disease who underwent cardiac surgery, between January 1997 and December 2017. We demonstrated that overall surgical workload (as absolute frequencies of surgical procedures per year) for this specific subset of patients increased steadily during the study period. The most common procedural groups included septal defects repair (n = 5740, 28%), right-heart lesions repair (n = 5542, 27%) and left-heart lesions repair (n = 4566, 22%); almost one-third of the procedures were re-operations (n = 5509, 27%). When considering the year-by-year relative frequencies of the main procedural groups, we observed a variation of the surgical scenario during the last two decades, characterized by a significant increase over time for right and left-heart lesions repair (p < 0.0001, both); while a significant decrease was seen for septal defects repair (p < 0.0001) and transplant (p = 0.03). Overall hospital mortality was 3% (n = 622/20,602 patients) and was stable over time. An inverse relationship between mortality and the number of patients operated in each center (p < 0.0001) was observed.

Minimally invasive surgery for atrial septal defects: a 20-year experience at a single centre.

OBJECTIVES: Our goal was to evaluate the results of our 20-year experience with minimally invasive surgical approaches for closing ostium secundum atrial septal defects, focusing on clinical results, patient satisfaction and cost-effectiveness. METHODS: We included 538 patients who underwent surgical ostium secundum atrial septal defects closure with minimally invasive approaches. RESULTS: The minimally invasive approaches included right anterior minithoracotomy (n = 335, 62%), midline lower ministernotomy (135, 25%) and right lateral minithoracotomy (n = 68, 13%). Central cannulation was used in 374 patients (69%), whereas, more recently, a remote cardiopulmonary bypass with peripheral cannulation was used in 164 selected patients (31%). Median intensive care unit and postoperative hospitalization stays were 1 day [interquartile range (IQR) 1-1 day] and 5 days (IQR 5-6 days), respectively. Thirty-one patients had postoperative complications (5.8%); postcardiotomy syndrome was the most frequent complication (n = 20/538, 3.7%). Decreases in the length of postoperative hospitalization (P < 0.001) and in hospital costs (P = 0.009) were achieved over time. At a median follow-up of 12.1 years (IQR 0.6-14 years), all patients are in good clinical condition with no limitations to physical activity. The vast majority of patients (524/538 patients, 97%) were very satisfied with the result of the minimally invasive approaches (99/100 patients, 99% in the last 5 years). CONCLUSIONS: Minimally invasive approaches for closing ostium secundum atrial septal defects proved safe and effective both in children and in adults with a very high satisfaction rate for the cosmetic result. A continuous evolution of our minimally invasive approaches, with a constant quest for less invasive procedures, led us to a miniaturization of the surgical accesses, reducing hospitalization time and hospital costs.

Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study.

BACKGROUND: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. METHODS: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%]). RESULTS: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). CONCLUSIONS: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.