Right Ventricular Outflow Tract Reconstruction in Truncus Arteriosus: A 30-Year Two-Center Comparison between Homografts and Bovine Jugular Vein.

INTRODUCTION: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. METHODS: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. RESULTS: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). CONCLUSION: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Mitral valve replacement in a 3.9-kg infant with fungal endocarditis: A case report.

A three-month-old female infant with a structurally normal heart was diagnosed with fungal endocarditis of the mitral valve with cerebral embolism. After antifungal therapy and a valve-sparing operation with complete removal of the fungal vegetations, a relapse with complete destruction of the valve leaflets and severe mitral regurgitation with decompensated heart failure occurred three months later. A second operation with successful mitral valve replacement was performed. Non-compliance with the anticoagulant treatment with vitamin K antagonist led to thrombosis of the mitral valve prosthesis one year later, and the child died from acute pulmonary edema.

Critical Cyanosis in a Neonate With Unusual Congenital Tricuspid Valve Lesion.

We describe a case of a low birth weight neonate who presented on second day of life with progressive cyanosis and oxygen saturation of 60% by pulse oximetry. The echocardiography examination revealed a large tumor-like mass connected to the tricuspid valve, with severe obstruction of the right ventricular inflow and massive right-to-left shunt through the distended foramen ovale. A large vegetation-like lesion with calcifications was discovered intraoperatively and was debrided by shave excision technique under deep hypothermic circulatory arrest. Follow-up showed normal function of the tricuspid valve and preserved biventricular function.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.

Right Ventricular Outflow Tract Reconstruction in Truncus Arteriosus: A 30-Year Two-Center Comparison between Homografts and Bovine Jugular Vein

ABSTRACT Introduction: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. Methods: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. Results: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). Conclusion: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.

OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.

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.

Phrenic nerve injury after paediatric heart surgery: is aggressive plication of the diaphragm beneficial?

OBJECTIVES: Phrenic nerve injury after paediatric heart surgery is associated with significant morbidity. Surgical plication of the diaphragm is believed to be beneficial to the patient, with difficult weaning from ventilation; however, the optimal timing remains unclear. We aimed to compare the outcome after two different strategies for treating hemidiaphragmatic paresis. METHODS: A retrospective analysis of all patients with paresis of the diaphragm between 2000 and 2010 was performed, with special attention to the rate of reintubations, ventilation and intensive care unit (ICU) stay and the rate of plication. In 2005, the strategy for treating diaphragmatic paresis in our institution changed from conservative treatment with plication after multiple extubation efforts towards an aggressive one with plication after a single unsuccessful extubation. We compared the outcome of all patients and that of the newborns separately from the two periods. RESULTS: During the study period, 148 patients with diaphragmatic paresis were diagnosed and included. Median age at the cardiac operation was 7 months (1 day-18 years), ventilation time ranged from 4 h to 41 days (median 7 days), 42 (28.4%) of the patients required at least one reintubation and ICU stay ranged from 2 to 63 days (median 11 days). A total of 63 plications were performed-5 (9%) before 2004 and 58 (62%) after 2005, P < 0.001. There were no significant differences in the ventilation time-6 (1-40) vs 8 (0-41) days, P = 0.36, reintubation rate-28 vs 29%, P = 0.85 and ICU stay-10 (3-63) vs 12 (2-55) days, P = 0.41 between both groups. The newborn patients, treated with the different strategies, also did not differ significantly in their outcome: ventilation time-12 (2-40) vs 11.5 (3-34) days, P = 0.38; reintubation rate-43 vs 41%, P = 0.62; ICU stay-16 (6-63) vs 15 (7-55) days, P = 0.55. CONCLUSIONS: Changing the strategy for phrenic nerve injury after paediatric heart surgery towards a more aggressive one with early plication of the diaphragm was not associated with the better outcome. Prospective randomized studies are needed to determine the optimal management of this complication.

Inhaled nitric oxide for elevated cavopulmonary pressure and hypoxemia after cavopulmonary operations.

Background. Elevated cavopulmonary pressure early after surgical creation of cavopulmonary connections is an important hemodynamic problem with grave prognostic significance. We examined the effect of administration of inhaled nitric oxide (iNO) to patients with elevated cavopulmonary pressure in the early postoperative period. Methods. We retrospectively reviewed data pertaining to all 14 patients with superior (n = 6) and total (n = 8) cavopulmonary connections who were treated with iNO in the early postoperative period during an interval of six years. Changes in the cavopulmonary pressure, the transpulmonary gradient, and the oxygen saturation after the institution of iNO were evaluated. The preoperative characteristics of the patients were compared to those of a control group of patients with cavopulmonary operations not treated with iNO postoperatively. Results. Twelve hours after the initiation of iNO therapy, significant reduction in the cavopulmonary pressure (16.6 ± 3.5 mm Hg vs 18.1 ± 2.3 mm Hg, P = .006), reduction in the cavopulmonary gradient (7.0 ± 3.5 mm Hg vs 9.8 ± 3.7 mm Hg, P = .009), and elevation of the arterial oxygen saturation (84.5% ± 6.0% vs 78.7% ± 5.9%, P = .001) were observed. Linear correlation analysis confirmed tendencies for reduction in the cavopulmonary pressure (P = .13), reduction in the cavopulmonary gradient (P = .02), and elevation of the oxygen saturation (P = .10). Compared to the control group, the treated patients tended to have higher preoperative pulmonary arterial pressures (PAPs) 17 (11-30) mm Hg versus 12 (10-25) mm Hg, P = .10; higher pulmonary vascular resistance (PVR) 2.04 (0.27-6.94) Wood units versus 1.02 (0.49-5.20) Wood units, P = .37; and longer bypass times 154 (41-218) versus 91 (15-276) minutes, P = .13. Conclusions. Administration of iNO was associated with diminuition of cavopulmonary pressure and transpulmonary gradient and increasing oxygen saturation in our small group of patients. In our experience selected patients with preoperatively elevated PAP above 17 mm and PVR above 2 Wood units can undergo cavopulmonary operations with iNO treatment early postoperatively.

Left ventricular and mitral valve function long after repair of left anomalous coronary artery from the pulmonary artery: recovery years after severe ischemia.

BACKGROUND: The study evaluates the long-term results of surgery for anomalous left coronary artery from the pulmonary artery (ALCAPA) with special attention on the left ventricular (LV) function and mitral regurgitation. METHODS: Twenty-one children underwent surgery for ALCAPA over 23 years (1987-2010). All patients underwent establishment of a two-coronary system, by direct reimplantation (n = 13) or by intrapulmonary tunnel technique (n = 8), with concomitant mitral valve repair in one. The follow-up echocardiograms were evaluated to assess LV function and mitral regurgitation. RESULTS: Five patients died. The age of the nonsurvivors was lower, 4.2 ± 1.3 versus 22.7 ± 29.4 months, P = .04. All nonsurvivors had moderate or severe mitral regurgitation preoperatively and higher LV diameter z score than the survivors: 11.8 (9-14.6) versus 4.6 (1.9-13.1), P = .01. At last follow-up, all survivors were asymptomatic; the diastolic LV diameter was normal, with z scores: 0.3 (0.1-1.9) versus 7 (1.9-14.6) preoperatively, P = .001, as was the LV ejection fraction: 66% (61%-78%) versus 38% (16%-70%) preoperatively, P = .001. Fifteen patients had moderate or severe mitral regurgitation at initial presentation and it eventually regressed to insignificant in all survivors (P = .001). No subsequent interventions on the coronary arteries or the mitral valve were needed. Four patients with intrapulmonary tunnel had mild suprapulmonary obstruction with Doppler peak gradients between 20 and 30 mm Hg. CONCLUSIONS: In our experience, establishment of a two-coronary circulation without mitral valve repair leads to normalization of LV dimension and systolic function and to improvement of mitral regurgitation in the surviving patients. Mortality is related to low age and to the associated higher degree of LV dysfunction.

Surgery for complications of trans-catheter closure of atrial septal defects: a multi-institutional study from the European Congenital Heart Surgeons Association.

OBJECTIVE: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). METHODS: The records of all (n=56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. RESULTS: A wide range of ASD sizes (5-34mm) and devices of various types and sizes (range 12-60mm) were involved, including 13 devices less than 20mm. Complications leading to surgery included embolisation (n=29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n=12), significant residual shunt (n=12), aortic or atrial perforation or erosion (n=9), haemopericardium with tamponade (n=5), aortic or mitral valve injury (n=2) and endocarditis (n=1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% (p=0.001). CONCLUSIONS: Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory.