Objectives: To examine the probability of left ventricular outflow tract (LVOT) reintervention following interrupted aortic arch (IAA) repair in neonates with LVOT obstruction (LVOTO) risk. Methods: This retrospective multicenter study included 150 neonates who underwent IAA repair (2003-2017); 100 of 150 (67%) had isolated IAA repair (with ventricular septal defect closure) and 50 of 150 (33%) had concomitant LVOT intervention: conal muscle resection (n = 16), Ross-Konno (n = 7), and Yasui operation (n = 27: single-stage n = 8, staged n = 19). Demographic and morphologic characteristics were reviewed. Factors associated with LVOT reoperation were explored using multivariable analysis. Results: Concomitant LVOT intervention was more likely in neonates with type B IAA, bicuspid aortic valve, aberrant right subclavian artery, smaller aortic valve annulus, and ascending aorta dimensions. On follow-up, five-year freedom from LVOT reoperation was highest following Ross-Konno (100%), 77% following Yasui (mainly for neo-aortic regurgitation), 77% following isolated IAA repair (mainly for LVOTO), and 47% following IAA repair with concomitant conal resection, P = .033. While all patients had low peak LVOT gradient at time of discharge, those who had conal resection developed higher gradients on follow-up (P = .007). Ross-Konno and Yasui procedures were associated with higher right ventricular outflow tract (RVOT) reoperation. In the cohort following isolated IAA repair, aortic sinus Z score was associated with LVOT reoperation. Conclusions: Both Yasui and Ross-Konno operations effectively mitigate late LVOTO risk. The highest risk of reintervention for LVOTO was associated with conal muscle resection while the lowest risk is associated with Ross-Konno. The RVOT reoperation risk in patients who had Ross-Konno or Yasui does not seem to affect survival.

BACKGROUND: This study compared the outcomes of primary and staged surgery for interrupted aortic arch (IAA) with biventricular circulation.
METHODS: A search of our surgical database identified 181 patients with biventricular anatomy who underwent surgical repair of IAA between 1982 and 2022. The patients were divided according to whether they underwent surgery in 1982 to 2001 or 2002 to 2022 and were categorized according to their anomalies into a simple group and a complex group. Repair was 1-stage in 130 patients (71.8%) and 2-stage in 51 (28.2%).
RESULTS: There were 6 early deaths (3.3%) and 9 (5.0%) late deaths. The 20-year actuarial survival rate was 91.7%. The 20-year survival rate was 94.7% in the simple group and 82.9% in the complex group (P = .007); the respective rates were 91.7% and 96.8% in 1982 to 2001 (P = .63) and 68.4% and 90.1% in 2002 to 2022 (P = .012). Multivariate analysis identified a complex anomaly and surgery performed in 1982 to 2001 to be risk factors for mortality. Aortic arch reintervention was required in 26 patients (balloon dilation, n = 15; surgical, n = 11). The 15-year freedom from reintervention rate improved from 67.8% in 1982 to 2001 to 89.8% in 2002 to 2022 (P = .041). Multivariate analysis identified type of surgical approach and emergent surgery to be risk factors for reintervention on the aortic arch.
CONCLUSIONS: The results of IAA surgery have improved in recent years, especially for complex cases. Development of surgical techniques and individualized treatment strategies could reduce postoperative mortality and morbidity. However, follow-up for recurrence of aortic arch obstruction is necessary.

Lesions of the semilunar valve and the aortic arch can occur either in isolation or as part of well-described clinical syndromes. The polygenic cause of calcific aortic valve disease will be discussed including the key role of NOTCH1 mutations. In addition, the complex trait of bicuspid aortic valve disease will be outlined, both in sporadic/familial cases and in the context of associated syndromes, such as Alagille, Williams, and Kabuki syndromes. Aortic arch abnormalities particularly coarctation of the aorta and interrupted aortic arch, including their association with syndromes such as Turner and 22q11 deletion, respectively, are also discussed. Finally, the genetic basis of congenital pulmonary valve stenosis is summarized, with particular note to Ras-/mitogen-activated protein kinase (Ras/MAPK) pathway syndromes and other less common associations, such as Holt-Oram syndrome.

OBJECTIVE: Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention.
METHODS: All surgical patients with IAA (2001-2022) were reviewed at a single institution. We compared IAA-A versus IAA-B; IAA with aortic AAb versus none; IAA-B with aberrant subclavian (AAbS) artery versus others. Primary outcomes included LVOT diameter (mm), LVOTO at discharge (≥50 mmHg), and LVOT reintervention.
RESULTS: Seventy-seven infants (mean age 10 ± 19 days) were followed for 7.6 (5.5-9.7) years. Perioperative mortality was 3.9% (3/77) and long-term mortality was 5.2% (4/77). Out of 51 IAA-B (66%) and 22 IAA-A (31%) patients, 30% (n = 22) had AAb. Smaller LVOT diameter was associated with IAA-B [IAA-A: 5.40 (4.68-5.80), IAA-B: 4.60 (3.92-5.50), P = 0.007], AAb [AAb: 4.00 (3.70-5.04) versus none: 5.15 (4.30-5.68), P = 0.006], and combined IAA-B + AAbS [IAA-B + AAbS: 4.00 (3.70-5.02) versus other: 5.00 (4.30-5.68), P = 0.002]. The likelihood of LVOTO was higher among AAb [N = 6 (25%) vs N = 1 (2%), P = 0.004] and IAA-B + AAbS [N = 1 (2%) vs N = 6 (30%), P = 0.002]. Time-to-event analysis showed a signal towards increased LVOT reintervention in IAA-B + AAbS (P = 0.11).
CONCLUSIONS: IAA-B and AAb are associated with small LVOT diameter and early LVOTO, especially in combination. This may reflect lower flow in the proximal arch during development. Most reinterventions occur in IAA-B + AAbS, hence these patients should be carefully considered for LVOT intervention at the time of initial repair.

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome.

OBJECTIVE: To evaluate the clinical utility of two dimensional (2D) ultrasound combined with spatiotemporal image correlation (STIC) in diagnosing interrupted aortic arch (IAA) in fetal life.
METHODS: A total of 53 cases of fetal IAA were diagnosed using 2D ultrasound combined with STIC, and 53 normal fetuses of the same gestational week were selected. These cases were retrospectively analyzed to assess the utility of employing 2D ultrasound combined with STIC in the diagnosis of IAA.
RESULTS: 2D ultrasound combined with STIC detected 22 cases of type A IAA, 24 cases of type B IAA, and seven cases of type C IAA. Furthermore, combining 2D ultrasound with STIC enabled dynamic visualization of the IAA, aiding in prenatal diagnosis. The diagnostic coincidence rate of IAA was found to be higher in the HD-flow combined with STIC than that in the 2D combined with HD-flow.
CONCLUSIONS: HD-flow combined with STIC can assist in diagnosing fetal IAA, and this technique has important clinical value.

BACKGROUND: Berry syndrome is a rare congenital cardiac malformation, herein we report an adult male patient who was successfully repaired by one-stage surgery.
METHODS: 18-year-old male patient presenting symptoms of chest tightness and shortness for over a year presented to outpatient clinic in our department to have corrective procedure heart. CTA revealed an Aortopulmonary Window (APW) type III, measuring 4.6 cm in maximum diameter. The right pulmonary artery originated from the ascending aorta, and the pulmonary trunk exhibited dilation with dimensions of 8.3 cm × 5.7 cm × 5.9 cm. Additional findings included Interrupted Aortic Arch (IAA) type A, intact ventricular septum, and Patent Ductus Arteriosus (PDA). Echocardiography showed bidirectional shunt at the level of APW in severe pulmonary hypertension. The right heart catheterization indicated a mean pulmonary artery pressure of 70mmHg and a pulmonary artery resistance of 5 Wood units. We evaluated after two weeks of treatment with epoprostenol at a rate of 20 ng/(kg. min) and found a significant improvement in pulmonary-artery pressure. Finally, we communicated with the patient\'s family and decided to proceed with the procedure.
CONCLUSIONS: For complex cardiovascular malformations, the ideal treatment strategy must be tailored to the characteristics of the patient to provide maximum efficacy and safety.

Background: Aortopulmonary window (APW) is a rare anomaly with variable morphology and associated cardiac anomalies. We evaluated impact of patient and operative factors on mid-term outcomes following APW repair. Methods: Twenty-nine patients underwent surgical APW repair at our institution from 1996 to 2022. Eight (28%) had simple APW, accompanied by only atrial septal defect or patent ductus arteriosus; 21 (72%) had complex APW with additional cardiovascular lesions, including nine with interrupted aortic arch. Median operative age was 19 days (range 2 days-1.5 years) via single-patch (n = 12, 41%), double-patch (n = 15, 52%), or ligation and division (n = 2, 7%). Results: The only mortality occurred in-hospital 1.4 years postoperatively following remote myocardial infarction. Factors associated with longer postoperative length of stay were complex APW (P = .003), genetic syndrome (P = .003), noncardiovascular comorbidities (P = .002), lower birth weight (P = .03), and lower operative weight (P = .03). Six patients (21%) with complex APW underwent unplanned cardiothoracic reintervention(s), including two with arch reintervention following arch advancement for interruption. Reintervention-free survival was similar for simple versus complex APW, operative age categories, and repair techniques. At median follow-up 5.5 years postoperatively, no patients had residual APW or persistent pulmonary hypertension, 1 (3%) had greater than mild ventricular dysfunction, and 25 (89% survivors) had NYHA class I functional status. Conclusions: Operative APW repair has excellent mid-term survival, durability, and functional status, regardless of operative age, cardiovascular comorbidities, or repair technique. Cardiac and noncardiac comorbidities may be associated with prolonged length of stay.

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm2/BSAm2) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.