Surgical Repair of a Rare Variant of Common Arterial Trunk, With Considerations of its Significance for Morphogenesis.

We present a successful surgical repair of a rare variant of the common arterial trunk with unusual arrangement of the pulmonary arteries. The variant was not readily classified using either of the popular classifications for the common arterial trunk. It is appropriately described as a common arterial trunk showing aortic dominance, but with extrapericardial origin of the pulmonary arteries from the underside of the truncal arch. We also provide an account of cardiac development, which we suggest offers an accurate explanation for its morphogenesis.

An Alternative Approach for a High Partially Anomalous Pulmonary Venous Connection to the Superior Vena Cava: "A Drawbridge Technique".

We present an alternative technique for the repair of a high partially anomalous pulmonary venous connection to the superior vena cava.

A Successful Repair of Pseudocoarctation With Associated Aortic Arch Aneurysm in an Eight-Month-Old Infant.

Pseudocoarctation of the aorta is an uncommon congenital entity characterized by juxtaductal elongation and tortuosity of the aortic arch. It is known to be associated with aneurysm formation. Our patient is a 7.7-month-female infant followed prenatally by magnetic resonance imaging that detected this anomaly. Subsequently, postnatal preoperative computed tomography angiography was confirmatory. The operation comprised resection of the aneurysmal portion of the aorta and extended end-to-end reconstruction, without prosthesis usage. The left subclavian artery was reimplanted into the left carotid artery. The postoperative course was uneventful. Computed tomography aortography and transthoracic echocardiography demonstrated no residual stenosis at six months of follow-up.

Anomalous Origin of the Circumflex Coronary Artery From the Right Pulmonary Artery Associated With Anomalous Aortic Origin of the Right Coronary Artery: A Case Report of Surgical Treatment.

We present a patient with the extremely rare coexistence of two distinct coronary artery malformations: anomalous aortic origin of the right coronary artery from the left aortic sinus with intramural course and anomalous origin of the circumflex coronary artery from the right pulmonary artery. Surgical reimplantation of the anomalous left circumflex coronary artery to the aorta and unroofing of the intramural portion of the anomalous right coronary artery were performed.

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.

Transesophageal Echocardiographic Predictor of Significant Right Ventricular Outflow Tract Obstruction After Tetralogy of Fallot Repair.

The optimal method for reconstruction of the right ventricular outflow tract in tetralogy of Fallot repair is yet to be established. Transatrial repair with pulmonary valve preservation avoids the detrimental effects of ventriculotomy and transannular patch; however, because of moderately hypoplastic pulmonary valve, these techniques are associated with undesirable residual right ventricular outflow tract gradients resulting in reoperations. The study sought to define intraoperative echocardiographic predictors of significant right ventricular outflow tract obstruction after tetralogy of Fallot repair. From July 2016 to December 2017, 58 patients with tetralogy of Fallot underwent repair with direct intraoperative pressures measuring and intraoperative transesophageal echocardiography performing after tetralogy of Fallot repair completion. Multivariate analysis was performed using logistic regression analysis to identify independent predictors of right ventricular outflow tract obstruction. One year after tetralogy of Fallot repair, significant right ventricular outflow tract obstruction was present in 12 patients (20.6%): 10 patients (26.2%) after pulmonary valve-sparing procedure and 2 patients (12.5%) after transannular patch (P = 0.35). Right ventricular outflow tract z-score lower than -3.2 had the best predictive value for significant residual right ventricular outflow tract obstruction (area under the curve = 0.979, P < 0.001). Multivariate analysis found that the right ventricular outflow tract z-score reached a statistical significance as a predictor of significant right ventricular outflow tract obstruction (P < 0.0001). Right ventricular outflow tract z-score <-3.2 measured by intraoperative transesophageal echocardiography is an indicator of postoperative right ventricular outflow tract obstruction right after surgery and a predictor of obstruction development following tetralogy of Fallot repair.

Single-centre 20-year experience with repair of truncus arteriosus.

OBJECTIVES: We analysed a large series of truncus arteriosus repairs with a focus on early and late outcomes. METHODS: Ninety-seven consecutive patients who underwent truncus arteriosus repair (1997-2017) were included retrospectively. Univariable analysis for mortality and reintervention was performed. RESULTS: The early mortality rate decreased from 45% (1997-2007; 14/31) to 4.5% (2008-2017; 3/66) (P = 0.001). Repair beyond the neonatal period (P = 0.03) and direct connection for right ventricular outflow tract reconstruction (P = 0.001) were associated with early death by univariable analysis. Overall survival was 68 ± 6.0% at 15 years; a majority of the deaths (90%; 9/10) occurred within the first year after repair. Freedom from the first and second conduit reoperations at 10 years was 22.9% and 89%, respectively. Freedom from truncal valve (TrV) reoperation was 83.9% at 15 years. Initial TrV insufficiency ≥ moderate was associated with a TrV reoperation (P = 0.008) with freedom from TrV reoperation in this subgroup of 58.3% at 10 years. Freedom from TrV reoperation for quadricuspid and tricuspid TrVs was 66.8% and 93.8% at 10 years with 100% for bicuspid TrVs at 8 years. At the last follow-up, 98.5% (69/70) were in New York Heart Association functional class I-II. CONCLUSIONS: In the current era, truncus arteriosus can be repaired with a low early mortality rate and a good long-term outcome. A significant reintervention burden still persists. Direct connection is associated with early mortality.

Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era.

BACKGROUND: The optimal management and prognostic factors of postsurgical pulmonary vein stenosis remain controversial. We sought to determine current postsurgical pulmonary vein stenosis outcomes and prognostic factors in a multicentric study in the current era. METHODS: Seventy-five patients with postsurgical pulmonary vein stenosis who underwent 103 procedures in 14 European/North American centers (2000-2012) were included retrospectively. A specific pulmonary vein stenosis severity score was developed on the basis of the assessment of each pulmonary vein. End points were death, pulmonary vein reintervention, and restenosis. A univariate and multivariate risk analysis was performed. RESULTS: Some 76% of postsurgical pulmonary vein stenosis occurred after repair of a total anomalous pulmonary venous return. Sutureless repair was used in 42 of 103 procedures (41%), patch veinoplasty was used in 28 procedures (27%), and endarterectomy was used in 16 procedures (16%). Overall pulmonary vein restenosis, reintervention, and mortality occurred in 56% (n = 58/103), 49% (n = 50/103), and 27% (n = 20/75), respectively. Sutureless repair was associated with less restenosis (40% vs 67%; P = .007) and less reintervention (31% vs 61%; P = .003). Mortality after sutureless repair (20%; 7/35) tends to be lower than after nonsutureless repair (33%; 13/40) (P = .22). A high postoperative residual pulmonary vein stenosis score at the time of hospital discharge was an independent risk factor for restenosis (hazard ratio [HR], 1.55; P < 10-4), reintervention (HR, 1.33; P < 10-4), and mortality (HR, 1.37; P < 10-4). The sutureless technique was an independent protective factor against restenosis (HR, 0.27; P = .006). CONCLUSIONS: Postsurgical pulmonary vein stenosis still has a guarded prognosis in the current era. The sutureless technique is an independent protective factor against restenosis. The severity of the residual disease evaluated by a new severity score is an independent risk factor for poor outcomes regardless of surgical technique.

Ross-Konno Procedure With Cylinder Mitral Valve Replacement in 49 Days Old Infant.

A neonate with antenatally diagnosed severe aortic valve stenosis, left ventricular outflow tract obstruction, left ventricular endocardial fibroelastosis, and severe mixed mitral valve disease was admitted to our center for balloon aortic valvuloplasty. On day 49, we performed a Ross-Konno procedure and mitral valve replacement on the baby, whose weight was 3.4 kg. Right ventricle-to-pulmonary artery continuity was restored with a handmade trileaflet Gore-Tex conduit. We used a handmade cylindrical prosthesis made from decellularized equine pericardium in the mitral position. We observed improvement in left ventricular function and good performance of aortic, pulmonary, and mitral prostheses at four months of follow-up.

Five-year Experience With Arterial Switch Operation in the First Hours of Life.

We assessed morbidity after 2 management strategies for arterial switch operation (ASO) in a single institution: first hours of life surgery and conventional approach. From September 2009 to September 2014, 346 consecutive patients who underwent ASO were enrolled. The study group included 92 patients who underwent ASO in the first 24 hours after birth (group I). The control group consisted of 254 patients who underwent ASO after 24 hours of life in the conventional way (group II). Three outcomes were analyzed: postoperative ventilation time (VT), post-extubation hospital length of stay (peLOS), and a composite morbidity index, defined as postoperative VT + peLOS + occurrence of selected major complications. Overall 30-day survival was 98% (2 [2%] group I vs 5 [2%] group II, P = 1.000). Fifty (13.3%) major complications were observed: 14 (15%) in group I and 36 (15%) in group II (P = 0.635). Although peLOS and morbidity index did not differ significantly between groups, postoperative VT (42 hours [24, 67] vs 27 hours [22, 47], P = 0.008) and total hospital stay were significantly longer in group II (18 days [10, 19] vs 14 days [12, 18]). A median volume of 80 mL (60-100 mL) of autologous umbilical cord blood was collected (80 mL, group 1 vs 60 mL, group II; P = 0.090). Homologous blood cell transfusion was avoided in 70 patients (78%) in group I and in 13 patients (6%) in group II (P < 0.001). Arterial switch operation during the initial 24 hours of life has similar outcomes in view of morbidity and mortality to the conventional approach.

Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study.

BACKGROUND: Primary pulmonary vein stenosis (PPVS) still carries a poor prognosis, and prognostic factors remain controversial. The aim of this study was to determine outcomes and prognostic factors after PPVS repair in the current era. METHODS: Thirty patients with PPVS and a normal pulmonary vein (PV) connection operated on in 10 European/North American centers (2000-2012) were included retrospectively. A specific PVS severity score was developed based on the assessment of each PV. Studied end points were death, PV reoperation, and restenosis. A univariate and multivariate risk analysis was performed. RESULTS: The mean number of affected PVs per patient was 2.7 ± 1.1. Sutureless repair was used in 21 patients (70%), endovenectomy was used in 5 patients, and patch venoplasty was used in 4 patients. Overall PV restenosis, reoperation, and mortality occurred in 50%, 40%, and 30% of patients respectively. Freedom from mortality, reoperation, and restenosis at 8 years of follow-up was 70% ± 8%, 62% ± 8%, and 47% ± 9%, respectively. Restenosis and mortality rates after sutureless repair versus nonsutureless repair were 57% (n = 12 of 21) versus 33% (n = 3 of 9) (p = 0.42) for restenosis and 38% (n = 8 of 21) versus 11% (n = 1 of 9) (p = 0.21) for mortality. Patients selected for a sutureless technique were younger and smaller and had more severe disease before operation. A postoperative high PVS score and pulmonary hypertension 1 month after the operation were independent risk factors for restenosis (hazard ratio [HR], 1.34; p = 0.002 and HR, 6.81; p = 0.02, respectively), reoperation (HR, 1.24; p = 0.01 and HR, 7.60; p = 0.02), and mortality (HR, 1.39; p = 0.01 and HR, 39.5; p = 0.008). CONCLUSIONS: Primary PVS still has a guarded prognosis in the current era despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by a new severity score are independent prognostic factors regardless of surgical technique.

Surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries in 83 consecutive patients.

OBJECTIVES: This article reports the safety and efficacy of a morphology-based algorithm for the surgical management of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. METHODS: A total of 83 patients were operated from 2007 to 2014 using 3 surgical approaches: one-stage total repair, one-stage unifocalization with central shunt and delayed total repair and multistage unifocalization with subsequent total repair. Patients were divided into 2 groups depending on the surgical strategy used to choose the surgical approach. In Group I (2007-09), the surgeon-preferred procedure (71%-one-stage total repair) was used. In Group II (2010-14), a surgical algorithm based on pulmonary arterial tree morphology was applied. RESULTS: Median follow-up was 5.04 years and 98% complete. The overall survival rate was 92.59%. Cumulative operative mortality decreased from 10.7% in Group I to 0% in Group II ( P = 0.036). Successful complete repair was performed in 23 (82%) of 28 patients in Group I (median follow-up, 7.99 years) and in 33 (60%) of 55 patients in Group II (median follow-up, 3.85 years). The difference in total survival was not significant between Groups I and II but was markedly influenced by the presence of the 22q11 deletion. The survival of 23 patients with confirmed 22q11 deletion was 73.91%; the survival of 28 patients with confirmed absence of the 22q11 deletion was 100%; and the survival of 32 patients not tested for the 22q11 deletion was 100% ( P = 0.0001). CONCLUSIONS: Total survival was significantly lower in patients with the 22q11 deletion. Surgical management based on preoperative pulmonary arterial anatomical features improves early surgical results.

Simultaneous arterial switch and totally anomalous pulmonary venous connection repair in a 5-hour-old child, complicated by pulmonary venous stenosis.

A 5-hour-old boy underwent arterial switch operation and totally anomalous pulmonary venous connection repair. Subsequently developed severe pulmonary venous stenosis was successfully treated with hybrid balloon angioplasty.

Successful Surgical Excision of a Large Cardiac Fibroma in an Asymptomatic Child.

Cardiac fibroma is a rare disease, and the management of asymptomatic patients is controversial. We report a case of successful surgical excision of a large cardiac fibroma in an asymptomatic child. Surgery should be considered for such a patient, as sudden cardiac death may occur in the absence of premonitory symptoms.

Cerebral Thromboembolism From Ligated Blalock-Taussig Shunt in an Adult Patient.

A 26-year-old woman underwent tetralogy of Fallot repair and classic Blalock-Taussig shunt (BTS) ligation. On postoperative day 4 she developed severe left-sided hemiplegia due to cerebral infarction. Thrombi in the BTS stump, right brachiocephalic artery, and its branches were removed surgically and the BTS stump was resected. The patient gradually recovered from hemiplegia and is leading a normal life with moderate left hemiparesis and is in good functional status 4 years after the event. An aggressive approach in treating this complication was crucial to prevent further thrombosis and neurologic deficit progression in this patient. We recommend reducing the size of the BTS stump during definitive repair.

Impact of carbon dioxide tension during cardiopulmonary bypass on tissue perfusion in neonates undergoing cardiac surgery using autologous umbilical cord blood.

OBJECTIVE: Previously, we reported that the oxyhemoglobin dissociation curve is shifted leftward in patients who receive autologous umbilical cord blood (UCB) during neonatal open heart surgery. In this study, we assessed whether allowing the pCO2 to rise during hypothermic cardiopulmonary perfusion would shift the curve back to the right and improve tissue oxygenation. METHODS: The study population included prenatally diagnosed patients with transposition of the great arteries. The study cohort was divided into two groups and consisted of early patients originally managed with lower pCO2 levels (Group I, n=42, 2009-12) and later patients subsequently managed with higher pCO2 levels (Group II, n=38, 2012-14). RESULTS: Patients received similar volumes of collected autologous UCB (Group I, 80 ml; Group II, 75ml, p=0.207) with a similar mean level of HbF during CPB (Group I, 90±8%; Group II, 87±9%, p=0.310). Higher levels of pCO2 during CPB (Group I, 31 mmHg; Group II, 37 mmHg, p=0.011) resulted in a rightward shift of the oxyhemoglobin dissociation curve (increased p50O2) (Group I, 19.5±3.4 mmHg; Group II, 22.5±2.2 mmHg, p=0.011). The use of a higher pCO2 strategy was associated with decreased serum lactate during CPB (Group I, 4.7±2 mmol/l; Group II, 2.8±1.4 mmol/l, p=0.001), decreased duration of mechanical ventilation (Group I, 46h; Group II, 22h, p<0.001) and decreased of length of intensive care unit (ICU) stay (Group I, 7.6±2.6, Group II, 5.6±2.2, p=0.003) CONCLUSIONS: A higher pCO2 during CPB in neonates who underwent open heart surgery using UCB resulted in a rightward shift of the oxyhemoglobin dissociation curve and was associated with improved serum lactate levels.

Relationship Between Antiinflammatory Cytokine Interleukin-10 and Lactate Concentrations in Neonates Undergoing the Arterial Switch Operation.

BACKGROUND: The aim of this study was to evaluate the relationship between antiinflammatory cytokine interleukin (IL)-10 production and perioperative lactate concentrations and their impact on postoperative outcomes in neonates undergoing the arterial switch operation (ASO). METHODS: Between August 2010 and August 2012, 80 neonates with transposition of the great arteries (TGA) were enrolled. Serum IL-10 levels were measured immediately before and after cardiopulmonary bypass (CPB) on the first, third, and seventh days. Perioperative clinical data were collected prospectively. RESULTS: Patients underwent the ASO at a median age of 72 hours (4-144 hours). We found that serum IL-10 levels significantly correlated with a prolonged intensive care unit (ICU) length of stay (r = 0.3; p = 0.020) and duration of ventilation (r = 0.3; p = 0.017). Serum IL-10 levels on the first day after the surgical procedure had predictive value for a prolonged ICU stay (defined as an ICU stay >6 days postoperatively) by receiver operator curve analysis, with an area under the receiver operating characteristic (ROC) curve of 0.65 (p = 0.045). Logistic regression modeling indicated that serum lactate level (ß = 2.7; p = 0.027), age at operation (ß = -4.0; p = 0.007), and the nature (autologous or allogeneic) of blood products (ß = -3.5; p = 0.030) used during CPB affected serum IL-10 levels. The strongest predictor of increased IL-10 on the first day after operation was a serum lactate level greater than 3 mmol/L measured after the surgical procedure on admission to the ICU, recording an odds ratio of 15.31. Serum lactate levels after operation and at admission to the ICU positively correlated with a prolonged ICU stay (r = 0.4; p = 0.007). CONCLUSIONS: Elevated lactate levels are associated with increased IL-10 production on the first postoperative day. Excessive production of IL-10 on the first day after the surgical procedure is associated with a prolonged ICU stay.

Prenatal diagnosis of left pulmonary artery-to-pulmonary vein fistula and its successful surgical repair in a neonate.

Pulmonary arteriovenous fistula is a rare disease. To the best of our knowledge, prenatal diagnosis of a fistula between the left pulmonary artery and the left pulmonary vein has not been described in the medical literature. We report a case of the prenatal diagnosis of a left pulmonary artery-to-pulmonary vein fistula, followed by successful neonatal surgical repair.

Anomalous origin of circumflex coronary artery from right pulmonary artery associated with coarctation of the aorta: a case report of surgical treatment.

Origin of the circumflex coronary artery (Cx) from the pulmonary arteries is an extremely rare anomaly. We describe a two-month-old female patient with anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta. Reimplantation of the anomalous Cx to the aorta and coarctation repair were performed. There were no postoperative complications, and the patient was discharged in satisfactory condition.