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Pulmonary artery sling (PAS) is a rare disease frequently associated with severe malacia and stenosis of the trachea. We present a two-day-old newborn that underwent urgent surgery for PAS and needed prolonged respiratory support afterward. Temporary airway stenting above the level of the tracheal bifurcation was performed five days after surgery to overcome severe airway obstruction caused by tracheomalacia and laceration of the tracheal mucosa after diagnostic bronchoscopy. Two days after the procedure, the child could be extubated and after two weeks the stent was removed without complications. Temporary tracheal stenting after PAS repair can be an effective therapeutic strategy in newborns with tracheal obstruction and allows timely weaning from ventilator support.

Aortic atresia with interrupted aortic arch is a rare anatomical and physiological combination. Survival is possible if there is blood flow in the ascending aorta supplying the coronary circulation from coexisting malformations. We present a newborn with aortic atresia, interrupted aortic arch type B, ventricular septal defect and a pulmonary artery to left coronary artery fistula as a source of blood flow to the coronary circulation, ascending aorta and proximal aortic arch, successfully treated with neonatal biventricular repair. To our knowledge, this is the first patient described in the literature with coronary blood flow dependent on a pulmonary-coronary arterial fistula., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We describe a newborn with single-ventricle malformation and anomalous origin of both coronary arteries from single ostium in the middle portion of the right pulmonary artery whose coronary anatomy was discovered during the operation and who underwent successful staged operative management. This report presents a unique anatomic association, proposes a means of management, and highlights the importance of intraoperative analysis., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Small pulmonary arteries (PAs) are often considered as a contraindication for the Fontan operation (FO). The aim of this study was to evaluate if the PA size is still one of the major impact factors on the postoperative early outcome., Methods: Data of 146 patients, with a median age of 2.0 years (range, 1.5 to 18 years) and a median weight of 12.45 kg (range, 7.7 to 64.7 kg) who underwent a modified FO in the same center between 2007 and 2012 were retrospectively analyzed with respect to the traditional McGoon ratio, Nakata index, and modified indices (measuring the narrowest diameters)., Results: Patients with a McGoon ratio of 1.6 or less (modified≤1.2) or a Nakata index of 150 mm2/m2 or less (modified≤100 mm2/m2) were not at a higher risk of longer mechanical ventilation (p=0.87 [0.1] and p=0.68 [0.52], respectively), longer stay (p=0.52 [0.18] and p=0.54 [0.38], respectively) in the intensive care unit, prolonged hospital stay (p=0.08 [0.26] and p=0.22 [0.29], respectively) or effusions (p=0.25 [0.37] and p=0.13 [0.06]), respectively). Younger and smaller children tended to have smaller PAs, but younger age (<24 months) and lower weight (<12 kg) were not predictive for poor early postoperative outcome., Conclusions: Small PAs do not significantly affect the early postoperative period after FO. In our opinion, there is no need to postpone the FO due to "smaller" PAs. The palliative procedures performed before FO to increase the size of the PA at the expense of volume overload of the single ventricle and the possible complications of prolonged cyanosis must be carefully weighed., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The right ventricle-to-pulmonary artery (RV-PA) shunt in the Norwood procedure (NP) for children with hypoplastic left-heart syndrome (HLHS) provides stable early hemodynamics and improves survival in many centers. However, lower pulmonary-to-systemic flow ratio causes early cyanosis and may require earlier second-stage procedure. The aim of the study was to present shunt-related results after NP with RV-PA shunt and our technique of RV-PA shunt construction., Methods: Between June 2001 and August 2010, 236 children with HLHS and variants underwent NP with RV-PA shunt, and were operated on by the same surgeon. The medical records were retrospectively reviewed., Results: To date, 180 children at a mean age of 7.0 ± 1.6 months with a mean weight of 6.4 ± 0.9 kg underwent second-stage procedure. The mean systemic oxygen saturation before stage 2 operation was 74.8 ± 6.6% and mean arterial partial oxygen pressure was 32.8 ± 6.7 mm Hg. These two parameters were significantly lower than after NP (p = 0.029, p < 0.001, respectively). Between stage 1 and 2 operation, three children (1.3%) died due to the shunt obstruction. Four children (1.7%) underwent re-operations due to shunt problems (one of them died), and the other four (1.7%), stent implantation in RV-PA shunt. Two infants (1.1%) developed aneurysm of the right ventricle infundibulum, which was resected during stage 2 without complications. One child required early (before fifth month of age) second-stage procedure due to the shunt obstruction. The patients with right-sided to the neo-aorta course of the RV-PA shunt had significantly more frequent delayed sternal closure after NP than children with left-sided shunt (35.5% vs 14.1%; p = 0.008)., Conclusions: The RV-PA shunt can be a safe and efficient technique in providing optimal pulmonary blood flow in the children with HLHS after Norwood procedure, performed with minimal rate of complications. In our experience, the use of RV-PA shunt in NP does not require earlier second-stage procedure., (Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Background: Despite significant improvement in survival after stage 1 Norwood, interim mortality before the second-stage operation remains significant. On the basis of reports of improved circulatory stability associated with the use of a right ventricle to pulmonary artery conduit, the difference between two physiologically different sources of pulmonary blood flow on interim mortality was investigated., Methods: Data collection of 96 consecutive hospital survivors after stage 1 Norwood surgery was undertaken. The source of pulmonary blood flow was a modified right Blalock-Taussig shunt in 46 (BTS) and a right ventricle to pulmonary artery conduit in 50 patients. The same follow-up protocol was used in both groups. Data analysis was performed to identify variables associated with interim mortality., Results: Analysis of patient-related and procedure-related variables revealed no differences in age, weight, diagnosis, presence of aortic atresia, lowest perioperative pH, duration of cardiopulmonary bypass, circulatory arrest, length of mechanical ventilation, or hospital stay at the time of stage 1 Norwood between groups. Respiratory rate and systolic blood pressure were the only differences detected between groups at the time of discharge. Interim mortality was higher in the Blalock-Taussig shunt group. Statistical analysis identified aortic atresia, a modified Blalock-Taussig shunt, and the presence of perioperative dysrhythmias to be associated with interim mortality., Conclusions: The use of a right ventricle to pulmonary artery shunt decreases the incidence of interim mortality among hospital survivors after stage 1 Norwood for hypoplastic left heart syndrome. Aortic atresia, the use of a modified Blalock-Taussig shunt, and perioperative dysrhythmias are independently associated with a higher mortality before superior cavopulmonary connection.

Background: Diastolic run off into the pulmonary circulation and labile coronary perfusion are thought to contribute to morbidity and mortality after the Norwood procedure (NP). We compared outcomes from the use of a RV to PA conduit (RV/PA) or a modified Blalock-Taussig shunt (BTS), physiologically distinct sources of pulmonary blood flow., Methods and Results: Review of 56 consecutive patients who underwent a Norwood procedure with a RV/PA (n=36) or a BTS (n=20) between 2000 and 2002. Median age was 4.5 days (range 1 to 40) and median weight was 3.1 kg (range 1.8 to 4.1). The RV/PA was constructed with a 5-mm conduit. Patients in the BTS group received a 4-mm shunt. Comparisons between RV/PA and BTS groups showed no difference for weight, gestational age, prenatal diagnosis, HLHS variant, associated diagnoses, ascending aortic size, ventricular function, AV valve function, and pulmonary venous obstruction. Operative survival was higher with RV/PA [33/36 (92%) versus 14/20 (70%); P=0.05]. Patients with RV/PA had less need for ventilatory manipulations to balance the Qp/Qs (1/36 v/s 8/20; P=0.001), delayed sternal closure (6/36 v/s 7/20; P=0.001), and extracorporeal support (5/36 v/s 7/20; P=0.036). RV/PA patients had more favorable postoperative hemodynamics: higher diastolic blood pressure without changes in systolic blood pressure at 1, 8, 24, 48 hours after the NP (46.3 v/s 39.5; 47.2 v/s 42.1; 46.1 v/s 37.1; and 47.1 v/s 40.2; all P=0.001)., Conclusions: RV/PA simplifies postoperative management and improves hospital survival after NP for HLHS.

Background: The balance of systemic, pulmonary, and coronary blood flow after the Norwood operation for hypoplastic left heart syndrome (HLHS) is critical to early survival. We hypothesized that a right ventricle to pulmonary artery conduit (instead of a systemic to pulmonary artery shunt) would result in hemodynamic changes consistent with a more stable balance of systemic, pulmonary, and coronary perfusion., Methods and Results: Hemodynamic data were obtained during cardiac catheterization before the hemi-Fontan procedure from 24 patients with HLHS; the first 10 had a Norwood operation with a systemic to pulmonary artery shunt, and the latter 14 had the Norwood operation with a right ventricle to pulmonary artery conduit. Significant differences were present, with the right ventricle to pulmonary artery conduit group having a higher aortic diastolic pressure (55 versus 42 mm Hg), a narrowed systemic pulse pressure (43 versus 64 mm Hg), a lower Qp:Qs (0.92 versus 1.42), a higher coronary perfusion pressure (46 versus 32 mm Hg), and a higher ratio of pulmonary artery diameter to descending aorta diameter (1.51 versus 1.37)., Conclusions: We conclude that, in HLHS after the Norwood operation, the right ventricle to pulmonary artery conduit modification produces hemodynamic changes consistent with improved coronary perfusion and a more favorable distribution of systemic, pulmonary, and coronary blood flow.

Objective: The aim of this study was to assess changes in early and late haemodynamic status after the Norwood procedure (NP), caused by the implementation of right ventricle-to-pulmonary artery shunt (RV-PA)., Methods: A consecutive series of 68 children with hypoplastic left heart syndrome underwent NP: Group 1 (n=31) with the application of a modified Blalock-Taussig shunt and Group 2 (n=37) with RV-PA. Haemodynamic data from the early postoperative period (72 h after the operation) and cardiac catheterisation data, as well as blood tests before the hemi-Fontan procedure (HF) were analysed. Univariate (chi(2) test, Mann-Whitney's and Student's t-tests) and multiple regression analysis were carried out., Results: In Group 1, circulatory collapse requiring resuscitation occurred in 15 (48.4%) children, within 72 h after the procedure. The resuscitation was unsuccessful in nine (29%) cases. The operative mortality (30 days) was 35%. In Group 2, two (5%) children died within the early and two (5%) within the late postoperative period. The postoperative course in the remaining children from Group 2 was uneventful. In Group 2 there was a significantly higher mean diastolic pressure after NP (P<0.05). The arterial pulse pressure after NP was significantly lower in Group 2 (P<0.05). Before HF, the application of RV-PA was associated with a lower Qp:Qs ratio (P=0.020), lower aortic pulse pressure (P=0.004) and lower aortic oxygen saturation (P=0.039)., Conclusions: A stable haemodynamic status due to independent coronary perfusion, higher diastolic and lower pulse pressure is the most advantageous effect of RV-PA, resulting in a lower mortality and morbidity after NP. A lower Qp:Qs ratio eliminates the danger of the ventricular volume overload and ensures good conditions for the development of the pulmonary circulation before HF.

OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n  = 129) with the shunt on the left and right-sided RV-PA (n  = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P  = 0.001 and P  = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P  = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P  = 0.001), but there is no evidence of the impact on the development of the left PAs (P  = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P  = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs. [ABSTRACT FROM AUTHOR]

Background: The evidence of the advantageous physiology associated with right ventricle–to–pulmonary artery (RV–PA) shunt in the early postoperative period after the Norwood procedure for hypoplastic left heart syndrome has been recently widely reported. We investigated the late consequences of this modification from the perspective of the third-stage palliation, the Fontan operation. Methods: Between September 1995 and November 2006, a consecutive series of 50 children with hypoplastic left heart syndrome from a single institution underwent a fenestrated Fontan operation (lateral tunnel technique): group 1 (n = 19) after the modified Blalock-Taussig shunt, and group 2 (n = 31) after RV–PA shunt during the Norwood procedure. Hemodynamic, echocardiographic, electrocardiographic, and clinical operative and perioperative data were analyzed. Results: Children after the RV–PA shunt were characterized by higher preoperative partial oxygen tension in pulmonary arteries (p = 0.018) and the aorta (p = 0.028), as well as lower systolic, diastolic, and mean aortic pressure (p = 0.005, p = 0.004, p = 0.019). After administration of 100% oxygen, this group additionally showed a lower value for systemic resistance (p = 0.013). The analyzed angiograms revealed a higher incidence of systemic–to–pulmonary collateral vessels (p = 0.003) in group 2. At the discharge after Fontan operation, children after the RV–PA shunt demonstrated higher arterial partial oxygen tension (p = 0.004). The two groups did not differ significantly with respect to the mortality, ventricular function, incidence of pleural effusions or rhythm disturbances, intensive care unit stay, and hospitalization time. Conclusions: The Norwood procedure with the RV–PA shunt provides satisfactory late hemodynamics. Children who underwent this method of palliation were more prone to the development of systemic–to–pulmonary arterial collaterals. [Copyright &y& Elsevier]

Abstract: Objective: The population of children who received right ventricle-to-pulmonary artery shunt (RV-PA) at Norwood procedure (NP) is growing, but limited data are available regarding the long-term outcome. The aim of this study was to present operative outcomes and mid-term hemodynamics and to assess the impact of the residual aortic obstruction on the results in patients undergoing three-staged surgery with RV-PA application at NP. Methods: Between June 2001 and June 2009, 229 children with hypoplastic left heart syndrome (HLHS) and variants underwent NP with RV-PA; 172 have proceeded to stage II and 95 to stage III. The medical records (clinical data, echocardiographic records, cardiac catheterization reports, electrocardiograms, and surgical notes) were retrospectively reviewed. Results: The later era of NP was associated with significantly better outcome (2001–2004: 1- and 5-years’ survival were 64.3% and 59.8%, respectively; 2005–2009: 1- and 4-years’ survival were 93.1% and 86.9%, respectively) (p <0.001). There was no association between long-term survival and diagnosis (HLHS/HLHS variant) (p =0.39). The incidence of depressed ventricular function and moderate or severe systemic atrioventricular valve regurgitation among the children who required balloon aortoplasty (BA) before stage II surgery was significantly higher than in children without aortic arch obstruction (p =0.027, p =0.008, respectively). In midterm follow-up, BA had no significant influence on the actuarial survival (p =0.089). No ventricular arrhythmias were noticed at any stage. Conclusions: The RV-PA shunt is a safe technique that does not seem to impair systolic or electrical ventricular function; its outcomes continue to improve with growing experience. Combined cardiologic interventional and surgical procedures are required to optimize the outcomes. [Copyright &y& Elsevier]

Abstract: Objective: The advantageous effect of right ventricle-to-pulmonary artery shunt (RV–PA) on the early postoperative hemodynamics in the Norwood procedure for hypoplastic left heart syndrome (HLHS) is well known. Numerous controversies still exist with respect to the late consequences of this new palliation method in preparation for the second stage procedure. Methods: Between September 1997 and September 2004, a consecutive series of 78 children with HLHS from a single institution underwent the hemi-Fontan procedure: Group 1 (n=27) after Blalock–Taussig shunt (BT), and Group 2 (n=51) after RV–PA. Hemodynamic, echocardiographic and clinical perioperative data were analyzed. Results: There were no significant differences in the age and operative weight (Group 1: 6.9±1.04 months, 6.22±0.99kg; Group 2: 6.57±1.12 months, 6.36±0.86kg). Children after RV–PA were characterized by a significantly higher preoperative hematocrit value (P=0.014), lower aortic and superior vena cava oxygen blood saturation (P&#60;0.001, P=0.024), severe right ventricle hypertrophy more rarely diagnosed in echocardiography (P&#60;0.004), lower Qp:Qs ratio (P=0.011), larger right (P=0.001) and left (P=0.006) pulmonary artery index and a shorter intensive care unit stay after the hemi-Fontan procedure (P=0.004). Conclusions: The Norwood procedure with the RV–PA shunt provides satisfactory late hemodynamics and improves the development of the pulmonary arteries. Children with hypoplastic left heart syndrome subjected to this new method of palliation are good candidates for the hemi-Fontan procedure. [Copyright &y& Elsevier]