Echocardiography of persistent fifth aortic arch confirmed by computed tomography angiography or surgery in children: a case series.

BACKGROUND: The purpose of this study was to review echocardiography-based diagnosis of persistent fifth aortic arch (PFAA) in children. METHODS: From January 2015 to December 2022, we retrospectively analyzed the echocardiographic findings and the relevant clinical data during follow-up of patients with PFAA who were treated in the Third Affiliated Hospital of Zhengzhou University. The diagnosis was confirmed by computed tomography angiography or surgery. RESULTS: Seven PFAA cases included two Weinberg type A and five Weinberg type B. The anatomical details of PFAA were assessed using a combination of the long-axis view of the left ventricular outflow tract (from the left high parasternal window) and the long-axis view of the aortic arch (from the suprasternal window). In Weinberg type A, the distal fifth and fourth aortic arches were connected to the descending aorta, which was associated with aortic coarctation. In Weinberg type B, the upper arch of the fourth aorta was interrupted, and only the lower arch of the fifth aorta was connected to the descending aorta. Surgical repair of PFAA was indicated in five patients with blood flow disruption, among which four had good postoperative results and one refused surgery. Two patients with unobstructed PFAA blood flow required follow-up rather than surgery. CONCLUSIONS: It is feasible to diagnose PFAA by echocardiography. Combined application of the high parasternal left ventricular outflow tract view and the suprasternal aortic arch view can improve timely detection of different types of PFAA in children.

Partial cardiopulmonary bypass through left thoracotomy for coarctation repair in children.

BACKGROUND: A left thoracotomy approach is anatomically appropriate for childhood aortic coarctation; however, the pediatric femoral arteriovenous diameters are too small for cardiopulmonary bypass cannulation. We aimed to determine the safety of a partial cardiopulmonary bypass through the main pulmonary artery and the descending aorta in pediatric aortic coarctation repair. METHODS: We retrospectively reviewed 10 patients who underwent coarctation repair under partial main pulmonary artery-to-descending aorta cardiopulmonary bypass with a left thoracotomy as the CPB group. During the same period, 16 cases of simple coarctation of the aorta repair, with end-to-end anastomosis through a left thoracotomy without partial CPB assistance, were included as the non-CPB group to evaluate the impact of partial CPB. RESULTS: The median age and weight at surgery of the CPB group were 3.1 years (range, 9 days to 17.9 years) and 14.0 (range, 2.8-40.7) kg, respectively. Indications for the partial cardiopulmonary bypass with overlap were as follows: age > 1 year (n = 7), mild aortic coarctation (n = 3), and predicted ischemic time > 30 min (n = 5). Coarctation repair using autologous tissue was performed in seven cases and graft replacement in three. The mean partial cardiopulmonary bypass time, descending aortic clamp time, and cardiopulmonary bypass flow rate were 73 ± 37 min, 57 ± 27 min, and 1.6 ± 0.2 L/min/m2, respectively. Urine output during descending aortic clamping was observed in most cases in the CPB group (mean: 9.1 ± 7.9 mL/kg/h), and the total intraoperative urine output was 3.2 ± 2.7 mL/kg/h and 1.2 ± 1.5 mL/kg/h in the CPB and non-CPB group, respectively (p = 0.020). The median ventilation time was 1 day (range, 0-15), and the intensive care unit stay duration was 4 days (range, 1-16) with no surgical deaths. No major complications, including paraplegia or recurrent coarctation, occurred postoperatively during a median observation period of 8.1 (range, 3.4-17.5) years in the CPB group. In contrast, reoperation with recurrent coarctation was observed in 2 cases in the non-CPB group (p = 0.37). CONCLUSIONS: Partial cardiopulmonary bypass through the main pulmonary artery and descending aorta via a left thoracotomy is a safe and useful option for aortic coarctation repair in children.

Exercise-induced hypertension after aortic coarctation repair: Our experience and a systematic review.

BACKGROUND: The exact prevalence and clinical significance of excessive increase in blood pressure in response to exercise in patients with repaired coarctation of aorta (CoA) remains unknown. AIM: This study aimed to investigate the impact of different definitions of exercise-induced hypertension (EIH) on the prevalence rates in our adult patients with repaired CoA. A systematic review of the available literature was also performed. METHODS: We retrospectively analyzed exercise test data from adult patients with repaired CoA followed at the national referral center for adult congenital heart disease between 1998 and 2021. The three most reported definitions of EIH in patients with repaired CoA were used for the analysis of EIH prevalence. We also performed a systematic search of the PubMed electronic bibliographic database. Full-text versions of all potentially relevant articles on EIH in CoA were reviewed for relevance. RESULTS: Our registry included 161 adult CoA patients. Complete exercise test results were available in 74 patients (59% male, median age 39 years [range 20-68 years]). The prevalence of EIH in our cohort varied from 24 to 41%, depending on the definition used. We identified eleven eligible articles from 184 publications. The reported prevalence rate of EIH in the studies ranged from 13% to 82%. CONCLUSION: EIH is common in patients with repaired CoA; however, the rates of EIH vary greatly depending on the definition used. A standardized and uniform EIH definition is needed to accurately assess the prevalence and clinical relevance of EIH in this population.

Delayed Paraparesis: An Unusual Complication Following Coarctation of Aorta Repair.

ABSTRACT: Paraparesis following cardiac surgery is a manifestation of spinal cord injury (SCI). It can occur in any aortic surgery from the aneurysm to the coarctation of the aorta (CoA) where the cross-clamp of the aorta is applied. Though the incidence of paraplegia is low, its occurrence affects the morbidity and mortality of the patient. There are only sporadic case reports on the development of paraplegia following recurrent and technically challenging repair of CoA. However, the spontaneous development of paraplegia has also been reported in cases of unoperated CoA. The present report describes the case of delayed SCI in which paraparesis developed 5 days post a coarctation repair. The risk factors and strategies to protect the spinal cord during aortic surgeries are emphasized.

[Surgical Replacement of Descending Aorta for Adult Aortic Coarctation by Using Three-dimensional Computer Graphics Reconstruction].

Aortic coarctation is diagnosed in approximately 5% of adult patients with congenital heart disease and is commonly diagnosed through the close examination of hypertension. Various surgical strategies for adult coarctation have been recently reported. Generally, aortic replacement may require blood transfusion in case of injury of the well-developed collateral vessels. Therefore, in order to secure an operative safety, we preoperatively used a medical image viewer to identify the abnormal vessels by three-dimensional computer graphics (3DCG) reconstruction. A 34-year-old male patient was referred to our hospital with hypertension and low ankle-brachial pressure index( ABI). Chest computed tomography( CT) scan showed aortic coarctation and development of abnormal collateral vessels. Descending aorta was replaced via a left third-fourth intercostal thoracotomy under partial extracorporeal circulation. As the image viewer depicted, anatomical abnormality of the collateral vessels was identified precisely, and surgically treated without any injury. The patient was discharged 10 days postoperatively without transfusion and with a normalized ABI.

Evaluation of short and mid-term clinical outcomes in patients with aortic coarctation treated with self-expandable stents.

The present study aimed to evaluate the outcomes of percutaneous treatment of aortic coarctation using self-expandable uncovered Nitinol stents. We conducted a retrospective clinical data review of all patients with aortic coarctation and treated with self-expandable uncovered Nitinol stents at our institution between 2009 and 2019. The gradient pressure across the coarctation site was measured using aortography. Follow-up echocardiography and computed tomography angiography were performed to assess possible stent complications. A total of 127 stents were successfully implanted in 125 patients (64.8% males) with a mean age of 35.36 ± 11.9 years. The gradient across the coarctation site decreased significantly from 67.48 ± 14.79 to 5.04 ± 3.01 mmHg (P < 0.001) after self-expandable stent implantation. Systolic blood pressure (SBP) decreased significantly from 175.53 ± 15.99 to 147.22 ± 12.83 mmHg (P < 0.001) after self-expandable stenting. There were no major technical or clinical complications, including balloon rupture, aneurysmal formation, infection, secondary stent migration, thrombosis, death during the procedure, and in-hospital mortality. On a mean follow-up of 48 ± 23.6 months (12-120 months), the gradient [from 59.43 ± 15.42 to 3.72 ± 1.38 mmHg (P < 0.001)] and SBP [from 175.53 ± 15.99 to 127.99 ± 7.82 mmHg (P < 0.001)] decreased significantly. There was no mortality, aneurysmal formation in the stent site, dislocation, or aortic re-stenosis requiring intervention during mid-term follow-up. Treatment of aortic coarctation using a self-expandable uncovered nitinol stent is safe and effective with promising mid-term outcomes.

Comparative analysis of the effectiveness of coarctation surgery between neonates and infants.

OBJECTIVE: This study aimed to compare the effectiveness of resection and extended end-to-end anastomosis between neonate and infant patients with coarctation. METHODS: This study was designed retrospectively and included 41 neonate (<30 days) and infant (30 days to 1 year) patients who were operated on using the resection and extended end-to-end anastomosis technique for aortic coarctation. Preoperative aortic annulus diameters and Z scores, all aortic arch diameters and Z scores, the presence of hypoplastic aortic segment, and the presence of prematurity were reviewed in both groups. Subsequently, we investigated whether these parameters were statistically related to the residual gradient in the operation area, whether there was a need for early re-intervention, and what was the incidence of mortality in the early postoperative period. In addition, the aortic arch Z scores of the patients at 6 months postoperatively were examined. RESULTS: While the mean age (p<0.001), body weight (p<0.001), and proximal arch Z score (p=0.029) were found to be significantly lower in the neonate group than in the infant group, the total length of the intensive care unit stay (p=0.013) and the total length of hospital stay (p=0.017) were found to be significantly higher. In addition, significant enlargement was detected in the proximal arch, distal arch, and isthmus segments in both patient groups. CONCLUSION: The resection and extended end-to-end anastomosis is an equally effective technique that can provide a marked decrease in gradient in the coarctation area and a significant enlargement of the aortic arch segments in the early period after coarctation repair in both neonate and infant patients.

Aortic geometry and long-term outcome in patients with a repaired coarctation.

OBJECTIVE: This study aims to compare aortic morphology between repaired coarctation patients and controls, and to identify aortic morphological risk factors for hypertension and cardiovascular events (CVEs) in coarctation patients. METHODS: Repaired coarctation patients with computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were included, followed-up and compared with sex-matched and age-matched controls. Three-dimensional aortic shape was reconstructed using patients' CTA or MRA, or four-dimensional flow cardiovascular magnetic resonance in controls, and advanced geometrical characteristics were calculated and visualised using statistical shape modelling. In patients, we examined the association of geometrical characteristics with (1) baseline hypertension, using multivariable logistic regression; and (2) cardiovascular events (CVE, composite of aortic complications, coronary artery disease, ventricular arrhythmias, heart failure hospitalisation, stroke, transient ischaemic attacks and cardiovascular death), using multivariable Cox regression. The least absolute shrinkage and selection operator (LASSO) method selected the most informative multivariable model. RESULTS: Sixty-five repaired coarctation patients (23 years (IQR 19-38)) were included, of which 44 (68%) patients were hypertensive at baseline. After a median follow-up of 8.7 years (IQR 4.8-15.4), 27 CVEs occurred in 20 patients. Aortic arch dimensions were smaller in patients compared with controls (diameter p<0.001, wall surface area p=0.026, volume p=0.007). Patients had more aortic arch torsion (p<0.001) and a higher curvature (p<0.001). No geometrical characteristics were associated with hypertension. LASSO selected left ventricular mass, male sex, tortuosity and age for the multivariable model. Left ventricular mass (p=0.014) was independently associated with CVE, and aortic tortuosity showed a trend towards significance (p=0.070). CONCLUSION: Repaired coarctation patients have a smaller aortic arch and a more tortuous course of the aorta compared with controls. Besides left ventricular mass index, geometrical features might be of importance in long-term risk assessment in coarctation patients.

Correlation between pressure gradient from echocardiography and peak-to-peak pressure gradient from cardiac catheterization or surgery in patients with coarctation of aorta.

The gold standard for assessing pressure gradients (PG) across coarctation involves measurements obtained through cardiac catheterization or surgical intervention. There has been ongoing discussion regarding the accuracy of non-invasive methods for estimating these gradients. This study sought to establish the correlation and agreement between the systolic blood pressure (SBP) gradient between the upper and lower extremities, as well as, the mean and maximum PG derived from echocardiography, in comparison to the peak-to-peak pressure gradient obtained from either cardiac catheterization or surgery. We conducted a retrospective study on patients < 18 years diagnosed with coarctation at Chiang Mai University Hospital from 2011 to 2022. The study involved the measurement of the SBP gradient between the upper and lower extremities, mean and maximum PG using echocardiography, peak-to-peak pressure gradient obtained from cardiac catheterization, and pressure gradient recorded during surgical procedures. The Spearman's correlation and Bland-Altman analysis were employed to assess correlation and agreement. Fifty-four patients with aortic coarctation were enrolled. The mean PG measured by echocardiography showed a significantly moderate correlation (r = 0.78, p < 0.001) and the highest level of agreement according to Bland Altman plots, in comparison to the peak-to-peak pressure gradient measured during both cardiac catheterization and surgical procedure. The max PG demonstrated a notable overestimation compared to the gold standard (mean difference + 13.14 with a slope of biases + 0.64, p < 0.001). The mean PG obtained through echocardiography has more potential to be applied in practical application in predicting pressure gradient in patients with coarctation.

Longitudinal Evaluation of Congenital Cardiovascular Surgical Performance and Skills Retention Using Silicone-Molded Heart Models.

Objective: Hands-on surgical training (HOST) for congenital heart surgery (CHS), utilizing silicone-molded models created from 3D-printing of patients' imaging data, was shown to improve surgical skills. However, the impact of repetition and frequency of repetition in retaining skills has not been previously investigated. We aimed to longitudinally evaluate the outcome for HOST on two example procedures of different technical difficulties with repeated attempts over a 15-week period. Methods: Five CHS trainees were prospectively recruited. Repair of coarctation of the aorta (CoA) and arterial switch operation (ASO) were selected as example procedures of relatively low and high technical difficulty. Procedural time and technical performance (using procedure-specific assessment tools by the participant, a peer-reviewer, and the proctor) were measured. Results: Coarctation repair performance scores improved after the first repetition but remained unchanged at the follow-up session. Likewise, CoA procedural time showed an early reduction but then remained stable (mean [standard deviation]: 29[14] vs 25[15] vs 23[9] min at 0, 1, and 4 weeks). Conversely, ASO performance scores improved during the first repetitions, but decreased after a longer time delay (>9 weeks). Arterial switch operation procedural time showed modest improvements across simulations but significantly reduced from the first to the last attempt: 119[20] versus 106[28] min at 0 and 15 weeks, P = .049. Conclusions: Complex procedures require multiple HOST repetitions, without excessive time delay to maintain long-term skills improvement. Conversely, a single session may be planned for simple procedures to achieve satisfactory medium-term results. Importantly, a consistent reduction in procedural times was recorded, supporting increased surgical efficiency.

Single-Lung Ventilation in Infants for Surgical Repair of Coarctation of The Aorta Without Cardiopulmonary Bypass.

OBJECTIVE: To investigate the effect of improving the operative field and postoperative atelectasis of single-lung ventilation (SLV) in the surgical repair of coarctation of the aorta (CoA) in infants without the use of cardiopulmonary bypass (CPB). METHODS: This was a retrospective cohort study. The clinical data of 28 infants (aged 1 to 4 months, weighing between 4.2 and 6 kg) who underwent surgical repair of CoA without CPB from January 2019 to May 2022 were analyzed. Fourteen infants received SLV with a bronchial blocker (Group S), and the other 14 infants received routine endotracheal intubation and bilateral lung ventilation (Group R). RESULTS: In comparison to Group R, Group S exhibited improved exposure of the operative field, a lower postoperative atelectasis score (P<0.001), reduced prevalence of hypoxemia (P=0.01), and shorter durations of operation, mechanical ventilation, and ICU stay (P=0.01, P<0.001, P=0.03). There was no difference in preoperative information or perioperative respiratory and circulatory indicators before SLV, 10 minutes after SLV, and 10 minutes after the end of SLV between the two groups (P>0.05). Intraoperative bleeding, intraoperative positive end-expiratory pressure (PEEP), and systolic pressure gradient across the coarctation after operation were also not different between the two groups (P>0.05). CONCLUSION: This study demonstrates that employing SLV with a bronchial blocker is consistent with enhanced operative field, reduced operation duration, lower prevalence of intraoperative hypoxemia, and fewer postoperative complications during the surgical repair of CoA in infants without the use of CPB.

The usefulness of staged extra-anatomic bypass for coarctation of the aorta with arch hypoplasia, from premature neonate to adult.

Various surgical techniques have been developed for coarctation of the aorta. However, coarctation repair in neonates with arch hypoplasia remains challenging. We herein report a case in which a premature neonate under 1500 g with coarctation of the aorta and arch hypoplasia underwent an extra-anatomical bypass at 18 days old. A second extra-anatomical bypass was performed at 3 years of age, and a third extra-anatomic bypass for recurrent coarctation was performed in adulthood. By increasing the size of the graft as the patient grows, extra-anatomic bypass can be a useful surgical option for premature neonates with coarctation and arch hypoplasia.

Surgically induced aortic coarctation in a neonatal porcine model allows for longitudinal assessment of cardiovascular changes.

Noncritical aortic coarctation (COA) typically presents beyond early childhood with hypertension. Correction of COA does not ensure a return to normal cardiovascular health, but the mechanisms are poorly understood. Therefore, we developed a porcine COA model to study the secondary cardiovascular changes. Eight male neonatal piglets (4 sham, 4 COA) underwent left posterolateral thoracotomy with descending aorta (DAO) mobilization. COA was created via a 1-cm longitudinal DAO incision with suture closure, plication, and placement and an 8-mm external band. All animals had cardiac catheterization at 6 (11-13 kg), 12 (26-31 kg), and 20 (67-70 kg) wk of age. Aortic luminal diameters were similar along the thoracic aorta, except for the COA region [6.4 mm COA vs. 17.3 mm sham at 20 wk (P < 0.001)]. Collateral flow could be seen as early as 6 wk. COA peak systolic pressure gradient was 20 mmHg at 6 wk and persisted through 20 wk increasing to 40 mmHg with dobutamine. Pulse pressures distal to the COA were diminished at 12 and 20 wk. This model addresses many limitations of prior COA models including neonatal creation at an expected anatomic position with intimal injury and vessel sizes similar to humans.NEW & NOTEWORTHY A neonatal model of aortic coarctation was developed in a porcine model using a readily reproducible method of aortic plication and external wrap placement. This model addresses the limitations of existing models including neonatal stenosis creation, appropriate anatomic location of the stenosis, and intimal injury creation and mimics human somatic growth. Pigs met American Heart Association (AHA) criteria for consideration of intervention, and the stenoses were graded as moderate to severe.

Follow-up outcomes and effectiveness of stent implantation for aortic coarctation: A systematic review and meta-analysis.

BACKGROUND: The authors aim to investigate the clinical implications of stent use for the management of CoA. METHODS: All observational studies on stent implantation for the treatment of aortic coarctation and the relevant RCTs were systematically retrieved. Outcomes included the immediate success rate, pre- and post-stent gradient, survival, minor and major complications, restenosis, post-stent systolic blood pressure, and reintervention rate. The analysis was further stratified by CoA type, stent type, and the mean age of the patients. RESULTS: Our meta-analysis incorporated 66 eligible studies involving 3,880 patients. The success rates for stent placement, defined as achieving post-treatment gradients of ≤20 mmHg and 10 mmHg, 0.96 (95% CI: 0.95 - 0.97; I2 = 59.83%) and 0.92 (95% CI: 0.89 - 0.95, I2 = 77.63%) respectively. The complication rates were quite low, with minor and major complication rates of 0.017 (95% CI: 0.013 - 0.021) and 0.007 (95% CI: 0.005 - 0.009), respectively. Unplanned reinterventions were required at a rate of 0.021 (95% CI: 0.015 - 0.026). At a mean follow-up of 2.9 years, 97% of the patients survived and 28% remained on antihypertensive therapy. While immediate effectiveness was consistent across age groups, complications were more prevalent in patients aged <20 years, and long-term efficacy was lower in those aged >20 years. Encouragingly, in neonates and infants, CoA stenting yielded results comparable to those observed in older children. CONCLUSION: These findings underscore the overall favorable outcomes of stent placement for aortic coarctation, with considerations for age-related variations in complications and long-term efficacy.

Results of a hybrid approach for high risk term newborn patients with interrupted aortic arch (IAA) with left ventricular outflow tract obstruction.

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated. Nine cases underwent hybrid approach during the study period. The mean age and weight at interventions were 7 days (3-16 days) and 3280 g (2700-4300 g). Six of the patients were diagnosed with type B IAA, 2 with type A, and one with type C. LVOTO was present in 7 patients. The success rate for the procedures was 100%. No patients died during the procedure or within the first 5 days after the procedure or from reasons related to the procedure. The median length of the hospital stay after stent placement was 28 days (22-35 days) for discharged patients. Three patients died in interstage period, and 6 patients underwent total corrective surgery after a median of 7 months (4-10 months). The average LVOT diameter was increased from 3.1 mm to 4.8 mm before total repair surgery. The hybrid approach should be kept in mind for treating high risk newborns with IAA with LVOTO and high-risk newborns who are not suitable for single stage total corrective surgery.

Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair.

INTRODUCTION: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. METHODS: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. RESULTS: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. CONCLUSION: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

Transcatheter management of acquired atresia of aorta.

A 24-year-old man was referred to our tertiary care center for the management of uncontrolled hypertension secondary to severe coarctation of aorta.

Coral reef aorta: a rare form of obstruction of the ascending aorta in adolescent with aortopathy- case report.

BACKGROUND: Supra aortic obstruction in children is uncommon and is seen in certain unique conditions. While intraluminal obstruction due to heavy calcification is seen in older populations, it is not described in pediatric populations. The coral reef aorta is a rare and distinct calcifying disease causing luminal obstruction of the suprarenal aorta in adults. The definition of this diagnosis relies entirely on the unique aspects and consistency of the lesions, which are rock-hard, irregular, gritty plaques with a white luminal surface resembling a coral reef. However, no such case has been described in children. CASE PRESENTATION: We present an adolescent boy who presented with a heavily calcified ascending aortic lesion associated with aortopathy and hypertension, 12 years after an aortic coarctation repair. The investigations included echocardiography, magnetic resonance and computer-tomographic imaging. A 3-D model was printed in order to visualize and plan surgical steps in advance for safe placement of clamps and defining the extent of resection. In addition, it provided an idea about tissue quality, thickness, spatial relationship, and orientation in relation to surrounding structures. Successful resection and replacement of the diseased segment of the aorta were achieved on cardiopulmonary bypass support. Post-operative recovery was uneventful, and at 6-month follow-up, the patient is doing well. In this report, various aspects of such lesions have been discussed, including clinical presentations, complications, planning and conduct of a safe cardiopulmonary bypass, and precautions during surgery for a successful outcome. CONCLUSION: Complicated obstructive aortic lesions in children require careful assessment, appropriate advanced imaging, and the use of 3-D printing technology in order to plan and perform safe and effective surgical management. The etiology of severe calcified aorta in children may be related to metabolic factors, previous surgery, use of a homograft, or an inflammatory process. However, it has yet to be proven.