OBJECTIVES In upper-income countries, infants undergo low-risk ventricular septal defect (VSD) repair. Children in low- and middle-income countries frequently present at older ages with elevated pulmonary vascular resistance (PVR) and pulmonary arterial hypertension (PAH). Expensive interventions for pulmonary hypertensive crisis are not available, and children are often denied an operation due to the mortality risk. We report our early and late experiences with these patients who underwent VSD closure by traditional patch (TP) or double patch (DP) techniques. METHODS We extracted data from patients with VSD and PAH who underwent VSD closure operations from 1996 to 2016. Information regarding cardiac catheterizations, operations, time in the intensive care unit and follow-up information was found. We identified 129 children and analysed the differences between the TP (89/129) and the DP (40/129) in unidirectional groups. After 2005, the patients were pretreated with sildenafil 3 months before catheterization. RESULTS The TP group was younger (P < 0.001). Hospital mortality (2.5%, DP; 10.1%,TP) was not significantly different (P = 0.17) between the 2 groups despite significant differences in baseline PVR, PVR/systemic vascular resistance and the number of children with a saturation less than 90%. The long-term survival and regression of PAH were better in the DP group (survival over 97% vs 93% in the TP group). Moreover, 61% of children with DP and 75% of those with TP had normal to mild elevations in pulmonary artery pressures at the last follow-up examination (P = 0.046). CONCLUSIONS The DP operation is a low-risk procedure. Moreover, the long-term outcome with the use of this technique in high-risk patients with VSD and high PVR and PAH is favourable with respect to survival and showed a decrease in PAH at the last follow-up.
In patients after bidirectional cavapulmonary anastomosis, blood flow through the superior vena cava (SVC), providing effective pulmonary blood flow, is the most important factor influencing blood oxygen saturation. Blood flow through the inferior vena cava recirculates into the systemic bloodstream. The study of the ratio of these flows will provide better understanding of the physiology of blood circulation after anastomosis and determine systemic oxygen saturation of blood and optimal time to perform surgery. The aim. To determine volumetric blood flow in the SVC, calculate pulmonary to systemic blood flow ratio in children after bidirectional cavapulmonary anastomosis, and evaluate its contribution to cardiac output and oxygen saturation in systemic blood flow. Materials and methods. In the period from January 2010 to June 2021, 51 patients with congenital heart defects with depleted pulmonary blood flow underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 29 male patients (57%) and 22 female patients (43%). The mean age of the patients at the time of the surgery was 34 ± 18.2 months (2 to 120 months), the mean age of patients at the time of examination was 43.5 ± 28.4 months (12 to 134 months). The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities. To evaluate the optimization of pulmonary/systemic blood flow we used equations obtained using the Fick method. Pulmonary to systemic blood flow ratio was calculated separately for 35 patients. Among the examined 35 patients, 18 children were older than 2.5 years, so all the examined patients were conveniently divided into 2 age groups: I group (n = 17) up to 2.5 years, II group (n = 18) older than 2.5 years to assess the contribution of SVC to the systemic circulation depending on age. Results. Pulmonary to systemic blood flow ratio was calculated for 35 patients Qp/Qs = (82% – 66%) / (97% – 66%) = 0.52. The calculated cardiac index according to echocardiography was 4.0 ± 0.85 L/min/m2 which corresponds to the SVC saturation (r = 0.60, p = <0.001). The flow in the superior vena cava = 2.08 L/min/m2. There was a very interesting trend towards decrease in the average rate of systemic saturation in patients after bidirectional cavapulmonary anastomosis depending on age and duration of surgery. Thus, in 17 patients of group I, the calculated Qp/Qs was (84% – 67%) / (97% – 67%) = 0.57. In patients of group II, the average systemic oxygen saturation was 78 ± 2% (from 65% to 81%). Calculated Qp/Qs for 18 patients of group II = (78% – 66%) / (97% – 66%) = 0.39, which indicates a decrease in pulmonary to systemic ratio with the growth of the child. Decreased systemic saturation after bidirectional cavapulmonary anastomosis in patients with increasing age and body surface area is associated with a decrease in the proportional flow from the superior vena cava. Therefore, in our clinical material, we confirmed the phenomenon of change in pulmonary to systemic ratio depending on age, which was described by Salim et al. according to a study conducted on healthy babies. Conclusions. The contribution of SVC flow to total cardiac output after bidirectional cavapulmonary anastomosis is directly associated with the patient’s age and gradually decreases in older patients, as indicated by a decrease in systemic saturation, so the clinical effect of bidirectional cavapulmonary anastomosis may be significantly better when performing surgery in early childhood.
Background. Aortic arch hypoplasia is a congenital anomaly of the development of the aortic arch characterized by ground. Aortic arch hypoplasia is a congenital anomaly of the development of the aortic arch characterized by hemodynamically significant narrowing of one or more segments of the aortic arch. Aortic arch hypoplasia combined ynamically significant narrowing of one or more segments of the aortic arch. Aortic arch hypop with ventricular septal defect (VSD) characterizes a special category of children who are in serious condition and need ptal defect (VSD) characterizes a special category immediate surgery. Despite the improvement in the results of surgical treatment of this abnormality in recent years, the gery. Despite the imp issue of choosing treatment tactics remains debatable. g The aim. To analyze immediate and long-term results of one-stage aortic arch hypoplasia repair and VSD repair in infants. Materials and methods. From 2011 to 2019, 55 infants underwent simultaneous aortic arch hypoplasia repair in ypoplasia rep conjunction with VSD repair at the National Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine and junction with VSD repair at the National Amosov Institute of Cardiovascular Surgery Ukrainian Children’s Cardiac Center. There were 30 (55%) male patients and 25 (45%) female patients. The mean age of (55%) male patients and 25 (45%) female patients. The mean ag the patients was 1.3 ± 1.2 months (from 0.1 to 9.1 months), the average body weight was 3.9 ± 1.3 kg (from 2.4 to 8.7 patients was 1.3 ± 1.2 months (from 0.1 to 9.1 months), the average body weight was 3.9 ± 1.3 kg ( kg). The mean body surface area was 0.27 ± 0.1 m2. Antegrade selective cerebral perfusion was performed in 23 (42%) g). The mean body patients during the aortic arch reconstruction. g Results.The hospital mortality rate was 1.8% (n = 1). The average duration of artificial circulation was 108.5 ± 38.6 minpital mortality rate was 1.8% (n = 1). The averag utes (from 55 to 204 minutes), aortic clamping time was 56.9 ± 36.4 minutes (from 21 to 126 minutes), the time of selec(from 55 to 204 minutes), aortic clamping time was 56.9 ± 36.4 minutes (from 21 to 126 minutes) tive cerebral perfusion was 26.4 ± 11.5 minutes (14 to 49 minutes). In eight patients (14.5%) the sternum was spread perfusion was 26.4 ± 11.5 minutes (14 to 49 minutes). In eight patients (14.5%) the sternum was sp apart in the early postoperative period. Echocardiography before discharge revealed the average pressure gradient at the part in the early postoperative period. Echocardiograp site of plasticity of the aortic arch of 20.5 ± 14.9 mm Hg. plasticity of the aortic arch of 20.5 ± 14.9 mm Hg. The mean long-term follow-up was 2.6 ± 2.1 years (from two months to 8.1 years). There were no fatal cases in the reg-term follow-up was 2.6 ± 2.1 years (from two months to 8.1 years) mote period. In 5 (9.1%) patients aortic arch restenosis occurred in the postoperative period; it was successfully treated period. In 5 (9.1%) patients aortic arch restenosis occurred in the postoperative period; it was successfully endovascularly by balloon dilation in 3 patients, the other 2 of them underwent repeated aortic arch repair. Long-term y by balloon dilation in 3 patients, the other 2 of them underwent repeated aortic arch repair. Long follow-up of other patients showed good results with respect to the pressure gradient at the aortic arch. There were p of other patients showed good results with respect to the pressure g no hemodynamically significant gradient after VSD closure. There were no neurological complications in the long-term follow-up. p Conclusions. One-stage complete repair is an effective and safe treatment for infants which provides good immedige complete repair is an effective and safe treatment for infants which provides g ate and long-term results. This surgical strategy may be an acceptable alternative to two-stage surgical treatment of this g complex pathology.
Tricuspid atresia (TA) is a rare cyanotic congenital heart disease (CHD) first described by Kreysig (1817). Bidirectional cavopulmonary anastomosis (BCPA) is part of the hemodynamic correction of such a complex combined CHD. In patients after BCPA, blood flow through the superior vena cava, providing efficient pulmonary blood flow, is the most important factor influencing blood oxygen saturation. The aim. To evaluate perioperative characteristics of the patients, immediate and long-term results after BCPA with TA. Materials and methods. In the period from January 1996 to April 2022, 50 patients with TA and multiple concomitant CHD underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 27 male patients (54%) and 23 female patients (46%). The mean age of the patients at the time of surgery was 31.6 ± 17.2 months (from 6 to 144 months). The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities. Among the 50 patients surveyed, 17 children were older than 2.5 years, so all the subjects were divided into 2 age groups: group I (n = 33) up to 2.5 years, group II (n = 17) older than 2.5 years to determine the optimal time of hemodynamic correction based on immediate and long-term results. Results. In the early postoperative period, there were 2 (4%) deaths among the patients of group II. The cause of death was acute heart failure in one case and infectious factor which led to the development of sepsis in another. There was a very interesting trend of increase in the duration of the operation (min): 239 ± 73.3 in group I, 251 ± 68.5 in group II; duration of mechanical ventilation (hours): 5.3 ± 1.4 in group I, 11 ± 3.7 in group II; duration of exudation (hours): 67 ± 22.9 in group I, 76 ± 26.8 in group II. There was significantly higher average dose of sympathomimetic (μg/kg/hour): 5 ± 2.1 for 60 ± 21.4 (hours) in group I; 7 ± 4.5 for 84 ± 29 (hours) in group II, hospital stay (days): 18 ± 5.7 in group I, 22 ± 11.3 in group II. Significantly lower mean systemic saturation at discharge 81 ± 4.6 (%) which was reported in children of group II compared to those of group I 88 ± 3.4 (%) clearly shows a decrease in the contribution of superior vena cava flow into the systemic circulation depending on the patient’s age, therefore, the clinical effect of performing BCPA as a stage of single-ventricular correction in patients with TA is much better when performing surgery in early childhood. Early postoperative period was uneventful in 27 (54%) patients. The other 23 (46%) patients of both groups had 16 and 18 complications in 10 (20%) patients of group I and 13 (26%) patients of group II, respectively, due to adverse factors of preoperative condition and specific changes in blood circulation characteristic of the BCPA. During the observation period lasting 49 ± 23.5 months, from 6 to 180 months, the final stage of hemodynamic correction was examined and performed: total cavopulmonary anastomosis was created in 31 (65%) patients. No deaths were reported after the final stage of hemodynamic correction. Conclusions. The contribution of superior vena cava flow to total cardiac output is directly related to the patient’s age and gradually decreases in the elderly, which indicates a decrease in systemic saturation, so the clinical effect of BCPA as a stage of single ventricular correction in patients with TA is much better when performing surgery in early childhood.
Background. Aortic arch hypoplasia is a congenital anomaly of the development of the aortic arch characterized by hemodynamically significant narrowing of one or more segments of the aortic arch. Despite the great interest in the problem of aortic arch hypoplasia, there are many contradictions in terms of terminology and classification. It is quite difficult to draw a clear line between normal physiology and pathology to determine in which cases there is a true hypoplasia, so, from a practical standpoint, the fundamental issue is the selection of criteria with reference to which we can talk about clinically and prognostically significant aortic arch hypoplasia. Important and yet controversial are the issues of multi-stage treatment of aortic arch hypoplasia and concomitant congenital heart diseases (CHD), and, in case of the isolated variant of hypoplasia, the choice of surgical approach. The aim of the study was to discuss different strategies and surgical approaches for patients with aortic arch hypoplasia, with an emphasis on surgical solutions and based on many years of experience, to develop an algorithm for diagnostic and surgical treatment of the aortic arch hypoplasia in infants. Conclusions. Aortic arch hypoplasia in infants remains a complicated issue in pediatric cardiac surgery. The variety of anatomical forms of isolated aortic arch hypoplasia and common overlap with other CHD raises many questions for the cardiac surgeon. Decisions concerning diagnosis and choice of further treatment tactics made in the neonatal period will affect not only the immediate but also the long-term outcome in the future. The algorithm of diagnostic and patient management with aortic arch hypoplasia allows to make the right timely decision which will affect the outcome of surgical treatment of this complex pathology.
Delayed sternal closure in infants and children of the first months of life after the correction of complex congenital heart defects (CHD) can increase cardiac output. This is a forced measure due to the development of unstable hemodynamics, capillary leak syndrome and myocardial edema, as the sternotomy wound closure in the early postoperative period provides a compression effect on the function of the myocardium. The aim. To evaluate mortality and to detect predictors of adverse outcome as well as the incidence of non-cardiac complications in delayed sternal closure after surgical repair of CHD. Materials and methods. Twenty two children were studied. Six children died. Anesthetic management was provided using propofol (4–6 mg/kg/h) and fentanyl (5–10 μg/kg/h) infusions. Modified ultrafiltration was applied at the end of assisted circulation in all the cases. Sternal closure was performed on average on day 3 ± 2 after surgery. Results. Mortality odds ratio (OR) in patients with the mean arterial pressure (MAP) < 35 mmHg was 3.7; mortality OR in patients with SVO2 < 40 % was 0.94; mortality OR in patients with blood lactate > 10 mmol/L in the first three days of postoperative intensive care was 2.1. Conclusions. Delayed sternal closure is an acceptable method of maintaining cardiac output in young infants with CHD in postoperative period. High blood lactate (> 10 mmol/L) and especially its further growth, as well as MAP < 35 mmHg, can predict adverse outcomes of cardiac surgery with open sternotomy.
Introduction: Children with large ventricular septal defects, severe pulmonary hypertension (PHT), and elevated pulmonary vascular resistance (PVR) are frequently denied operation in low and middle-income countries secondary to concerns of high mortality and development of severe pulmonary hypertension following operation, early and late. Hypothesis: We hypothesized that closure with a uni-directional flap valve patch (DP) would provide lower mortality and better survival without severe PHT. Methods: From 5/96 - 12/2016 all patients undergoing VSD closure with severe PHT and elevated PVR by either traditional (TP) or DP closure were followed annually. Baseline and O 2 provocation catheterization were performed before operation. Echocardiograms were performed at discharge and follow-up. The decision on TP or DP closure was based upon age and surgeon preference. Beginning in 2005 all patients regardless of technique received sildenafil 3 mgs/kg/day for 3 months before operation. Results: The number of patients receiving operation was 129, of which 40 received the DP. Males were 66/129, age and weight differed significantly for DP group compared to the TP group (7.5 vs 2.6 years, p<0.001 and 20 vs 11.5 kgs, p<0.001).Catheterization baseline data, PVR 9.0 vs 7.4 for DP and TP respectively (p<0.001), negative vasoactive test 32.5% vs 13.5% for DP and TP respectively (p=0.016). Mortality was 1/40 for DP and 9/89 for TP. Pulmonary to Systemic systolic pressure ratio was lower at discharge vs baseline cath (0.38 vs 0.99, p<0.001). Predictors of late severe pulmonary hypertension were PVR/SVR >0.5, p<0.024; and a negative vasoactive test of <10% decrease, p<0.001. PHT at last follow-up, none or mild 86/115 (74.8%), moderate 16/115 (13.9%) or severe 13/115 (11.3%). Conclusions: DP patients had less risk of death and both groups had few patients with severe PHT at late follow-up..
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