Pulmonary Artery Angioplasty for Improving Ipsilateral Lung Perfusion in Adolescent and Adult Patients: An Analysis Based on Cardiac Magnetic Resonance Imaging and Lung Perfusion Scanning.

Background: The left pulmonary artery (LPA) may be kinked and stenotic, especially in tetralogy of Fallot, because of ductal tissue and anterior deviation of the conal septum. If LPA stenosis is not effectively treated during total correction, surgical angioplasty is occasionally performed. However, whether pulmonary artery (PA) angioplasty in adolescents or adults improves perfusion in the ipsilateral lung remains unclear. Methods: This retrospective review enrolled patients who underwent PA angioplasty for LPA stenosis between 2004 and 2019. Among patients who underwent a lung perfusion scan (LPS) or cardiac magnetic resonance imaging (cMRI) pre- and post-pulmonary angioplasty, those aged >13 years with <40% left lung perfusion (p-left) in the pre-angioplasty study were included. Preoperative and postoperative computed tomography, LPS, and cMRI data were collected. The perfusion ratio was analyzed according to the LPA's anatomical characteristics. Results: Seventeen adolescents and 16 adults (≥18 years old) were finally included (median age, 17 years). The most common primary diagnosis was tetralogy of Fallot (87.9%). In all patients, LPA angioplasty was performed concomitantly with right ventricular outflow tract reconstruction. No patients died. Preoperative p-left was not significantly different between adolescents and adults; however, adolescents had significantly higher postoperative p-left than adults. P-left significantly increased in adolescents, but not in adults. Seven patients had significant stenosis (z-score <-2.0) confined only to the proximal LPA and demonstrated significantly increased p-left. Conclusion: PA angioplasty significantly increased ipsilateral lung perfusion in adolescents. If focal stenosis is confined to the proximal LPA, PA angioplasty may improve ipsilateral lung perfusion, regardless of age.

Change in Pulmonary Arteries after Modified Blalock-Taussig Shunt Procedure: Analysis Based on Computed Tomography.

Background: Although the modified Blalock-Taussig shunt remains the mainstay method of palliation for augmenting pulmonary blood flow in various congenital heart diseases, the shunt must be carefully designed to achieve the best outcomes. This study investigated the effect of shunt configuration on pulmonary artery growth and growth discrepancy. Methods: Twenty patients with successful modified Blalock-Taussig shunt takedown were analyzed. Pulmonary artery and shunt characteristics were obtained using computed tomography scans. Differences in the baseline and follow-up diameter ratios and growth in the ipsilateral and contralateral arteries were calculated. The angle between the shunt and pulmonary artery, as well as the distance from the main pulmonary artery bifurcation, were measured. Correlations between pulmonary arteries and shunt configurations were analyzed. Results: The median interval time between shunt placement and takedown was 154.5 days (interquartile range, 113.25-276.25 days). Follow-up values of the ipsilateral-to-contralateral pulmonary artery diameter ratio showed no significant correlation with the shunt angle (ρ=0.429, p=0.126) or distance (ρ=0.110, p=0.645). The shunt angle and distance from the main pulmonary bifurcation showed no significant correlation (ρ=-0.373, p=0.189). Pulmonary artery growth was negatively correlated with shunt angle (ipsilateral, ρ=-0.565 and p=0.035; contralateral, ρ=-0.578 and p=0.030), but not with distance (ipsilateral, ρ=-0.065 and p=0.786; contralateral, ρ=-0.130 and p=0.586). Conclusion: Shunt configuration had no significant effect on growth imbalance. The angle and distance of the shunt showed no significant correlation with each other. A more vertical shunt was associated with significant pulmonary artery growth. We suggest a more vertical graft design for improved pulmonary artery growth.

Outcomes of not using tracheoplasty in asymptomatic tracheal stenosis found during open-heart surgery.

OBJECTIVES: We aimed to review the outcomes of treating incidentally encountered asymptomatic airway stenosis during open-heart surgery conservatively without the use of tracheoplasty. METHODS: Between January 2002 and October 2022, 25 patients were incidentally diagnosed with tracheal stenosis during open-heart surgery. Intraoperative bronchoscopy and/or laryngoscopy revealed tracheal stenosis; however, this was not consistent with the findings of the preoperative computed tomography. Patients who were diagnosed with a pulmonary artery or vascular sling or had moderate-to-severe respiratory symptoms before open-heart surgery were excluded. RESULTS: The median age and weight of the patients at operation were 3.0 months and 5.1 kg, respectively. They were categorized as those having tracheal stenosis on preoperative computed tomography (n = 12) or not having tracheal stenosis (n = 13). The narrowest diameter was significantly smaller in the former group (3.0 vs 5.8 mm, P < 0.05). The rates of reintubation and the tracheostomy, and intubation days tended to be higher in former group without statistical significance. Stenotic degree improved 2 months and 1 year or more after the operation (39.3% at operation, 28.4% at 2 months, 12.5% after 1 year). All patients were Ross class 1 or 2 at follow-up (mean, 7.1 years). CONCLUSIONS: Patients with tracheal stenosis showed tolerable long-term outcomes without using tracheoplasty. Accordingly, if tracheal stenosis, that would cause intubation difficulty, was incidentally revealed, concomitant tracheoplasty may not be required during open-heart surgery if the stenosis did not cause considerable symptoms or signs preoperatively.

Risk Factors for Coronary Artery Complications After Prosthetic Pulmonary Valve Implantation in Patients With Congenital Heart Disease.

BACKGROUND: Coronary artery complications (CACs) in patients who undergoing prosthetic pulmonary valve implantation for congenital heart disease can lead to fetal outcomes. However, the incidence of and risk factors for CACs in these patients remain unknown. METHODS AND RESULTS: A retrospective cohort study was conducted on patients who underwent cardiac computed tomography or invasive coronary angiography after prosthetic pulmonary valve implantation at Seoul National University Hospital from June 1986 to May 2021. Among 341 patients, 25 (7.3%) were identified with CACs, and 2 of them died. Among the patients with CACs, congenital coronary anomalies and an interarterial course of the coronary artery were identified in 11 (44%) and 18 (72%) patients, respectively. Interarterial and intramural courses of the coronary artery were associated with a 4.4- and 10.6-fold increased risk of CACs, respectively. Among patients with tetralogy of Fallot and pulmonary atresia, the aortic root was rotated further clockwise in patients with coronary artery compression compared to those without it (mean [±SD] 128.0±19.9° vs. 113.5±23.7°; P=0.024). The cut-off rotation angle of the aorta for predicting the occurrence of coronary artery compression was 133°. CONCLUSIONS: Perioperative coronary artery evaluation and prevention of CACs are required in patients undergoing prosthetic pulmonary valve implantation, particularly in those with coronary artery anomalies or severe clockwise rotation of the aortic root.

Extubation in operating room versus early extubation in ICU after open-heart surgery in patients with CHDs.

BACKGROUNDS AND OBJECTIVES: The "Fast track" protocol is an early extubation strategy to reduce ventilator-associated complications and induce early recovery after open-heart surgery. This study compared clinical outcomes between operating room extubation and ICU extubation after open-heart surgery in patients with CHD. METHODS: We retrospectively reviewed 215 patients who underwent open-heart surgery for CHDs under the scheduled "Fast track" protocol between September 2016 and April 2022. The clinical endpoints were post-operative complications, including bleeding, respiratory and neurological complications, and hospital/ICU stays. RESULTS: The patients were divided into operating room extubation (group O, n = 124) and ICU extubation (group I, n=91) groups. The most frequently performed procedures were patch closures of the atrial septal (107/215, 49.8%) and ventricular septal (89/215, 41.4%) defects. There were no significant differences in major post-operative complications or ICU and hospital stay duration between the two groups; however, patients in group I showed longer mechanical ventilatory support (0.0 min vs. 59.0 min (interquartile range: 17.0-169.0), p < 0.001). Patients in Group O showed higher initial lactate levels (3.2 ± 1.7 mg/dL versus 2.5 ± 2.0 mg/dL, p = 0.007) and more frequently used additional sedatives and opioid analgesics (33.1% versus 19.8%, p = 0.031). CONCLUSIONS: Extubation in the operating room was not beneficial for patients during post-operative ICU or hospital stay. Early extubation in the ICU resulted in more stable hemodynamics in the immediate post-operative period and required less use of sedatives and analgesics.

An extreme case of cor triatriatum mimicking hypoplastic left heart syndrome and combined pulmonary vein stenosis.

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

Valve-Sparing Neo-Aortic Root Replacement for Neo-Aortic Root Dilatation 20 Years after Arterial Switch Operation for Transposition of the Great Arteries: A Case Report.

A 25-year-old man returned to Seoul National University Children's Hospital with mild dyspnea on exertion. He had undergone an arterial switch operation at 1 month after birth to correct a complete transposition of the great arteries and a ventricular septal defect. When the patient was 15 years old, dilatation of the neo-aortic sinus and annulus was first identified; since then, it had gradually increased. Given the young age of the patient and the degree of aortic regurgitation (AR), which was mild to moderate, we opted to perform a valve-sparing neo-aortic root replacement with aortic valve repair. Postoperative echocardiography showed successful reductions in the sizes of the aortic sinus and annulus, with only mild AR remaining.

Shallow suture at ventricular septal defect may safely reduce right bundle branch block.

BACKGROUND: To avoid rhythm disturbance, sutures for ventricular septal defect closure have been traditionally placed 2∼5 mm or more away from the edge of the ventricular septal defect. However, the traditional suturing method appears to induce right bundle branch block and tricuspid valve regurgitation after ventricular septal defect closure more than our alternative technique, shallow suturing just at the edge of the ventricular septal defect (shallower bites at the postero-inferior margin). We aimed to verify our clinical experience of perimembranous ventricular septal defect repair. METHODS: The alternative shallow suturing method has been applied since 2003 at our institution. We retrospectively reviewed the clinical data of 556 isolated perimembranous ventricular septal defect patients who underwent surgical closure from 2000 to 2019. We investigated the postoperative occurrence of right bundle branch block or progression of tricuspid regurgitation and analysed risk factors for right bundle branch block and tricuspid regurgitation. RESULTS: Traditional suturing method (Group T) was used in 374 cases (66.8%), and alternative suturing method (Group A) was used in 186 cases (33.2%). The right bundle branch block occurred more frequently in Group T (39.6%) than in Group A (14.9%). In multivariable logistic regression analysis, Group T and patch material were significant risk factors for late right bundle branch block. More patients with progression of tricuspid regurgitation were found in Group T. CONCLUSIONS: Shallow suturing just at the edge of the ventricular septal defect may reduce the rate of right bundle branch block occurrence and tricuspid regurgitation progression without other complications.

Ten-year follow-up of dilatation of aortic structures in Fallot-type anomalies.

OBJECTIVES: Dilatation of the aortic root structures or ascending aorta is often observed in patients with Fallot-type anomalies. We aimed to determine the dilation rate of the aortic structures and investigate strategies for managing this phenomenon. METHODS: In this retrospective study, we enrolled 66 out of 801 patients who underwent corrective surgery for Fallot-type anomalies (tetralogy of Fallot [TOF] and Fallot-type double outlet right ventricle [DORV]) between 2004 and 2020. These 66 patients had follow-up cardiac computed tomography (CT) angiography images taken at least 5 years after the initial CT study. We analyzed the diameters and aortic cross-sectional area/height ratio (AH) of the aortic annulus, sinus of Valsalva, sinotubular junction, and ascending aorta between the initial and follow-up CT scans. "Dilatation" was defined as a z-score over 2 for each aortic structure. RESULTS: The median age at the initial and follow-up CT scans was 5.9 years (interquartile range [IQR]: 0.4 ~ 12.4) and 15.9 years (IQR 9.3 ~ 23.4), respectively. The median CT interval (from initial to latest CT) was 9.5 years (IQR 6.6 ~ 12.0). The sinus of Valsalva exhibited the most significant dilation (32.8 mm at follow-up CT) over the study period. The AH ratio increased significantly in all four aortic structures. The patient's age was significantly associated with higher AH in the follow-up CT. Aortic dilatation was present in 74.2% of patients at the initial CT and increased to 86.4% at the follow-up CT. CONCLUSION: In Fallot-type anomalies, the AH ratio of aortic root structures significantly increased over an average period of approximately 9.5 years. The number of patients diagnosed with aortic dilatation also increased. Based on our observations in this study, these patients' group should be considered for more frequent follow-up examinations, as significant dilatation could occur in their mid-20 s.

Left atrial thrombosis with invasive pulmonary aspergillosis in children with immunodeficiency.

Invasive aspergillosis is a major cause of infectious disease in immunocompromised patients; however, cardiac involvement in pulmonary aspergillosis is not well-known. Two paediatric patients undergoing chemotherapy were diagnosed with cardiac aspergilloma, accompanied by pulmonary aspergillosis. In both patients, antibiotic and antifungal treatments were initiated immediately after the pneumonia was diagnosed; however, both died of multiple cerebral thromboembolisms.

Late Outcomes of Pediatric and Congenital Heart Disease Patients Following Cardiac Resynchronization Therapy.

BACKGROUND AND OBJECTIVES: Cardiac resynchronization therapy (CRT) is an effective treatment for heart failure. However, in pediatric and congenital heart disease (CHD) patients, current adult indications cannot be directly applied because of heterogeneity in anatomy and diagnosis. Therefore, CRT responses and clinical outcomes in these patients were investigated to derive possible candidates for CRT. METHODS: This study retrospectively analyzed 16 pediatric and CHD patients who underwent CRT implantation at a single center in early (0.7±0.2 year) and late (4.7±0.3 years) follow-up period after CRT. RESULTS: The median age at CRT implantation was 2.5 (0.3-37.2) years, and median follow-up duration was 6.3 (0.1-13.6) years. Thirteen had non-transvenous CRT. Two had congenital complete atrioventricular (AV) block with previous right ventricular pacing, 5 had dilated cardiomyopathy (DCM) with left bundle branch block, and 9 had CHD. The mean ejection fraction of the systemic ventricle increased from 28.1±10.0% to 44.3±21.0% (p=0.003) in early and 51.8±16.3% (p=0.012) in late outcome. The mean functional class improved from 3.1±0.9 to 1.8±1.1 after CRT (p=0.003). Twelve patients (75%) showed improvement in ventricular function or functional class after CRT. Proportion of responders differed between patients without CHD (2/2 patients with complete AV block and 5/5 with DCM, 100%) and those with CHD (5/9, 56%), although statistical significance was not reached (p=0.088). CONCLUSIONS: CRT improved ventricular function and functional status according to the underlying condition in pediatric and CHD patients. However, further large and longer-term studies are needed to establish the guideline for the patient selection of CRT in these patients.

Prevention of Tracheo-Innominate Artery Fistula Formation as a Complication of Tracheostomy: Two Case Reports.

Tracheo-innominate artery fistula (TIF) is a rare complication of tracheostomy and refers to the formation of a fistula between the trachea and innominate artery. Because TIF is fatal, prevention rather than treatment is very important. Here we report the cases of two high-risk patients who underwent tracheostomy, and in whose cases attempts were made to lower the risk of TIF. In the first patient who developed a chest deformity with Duchenne muscular dystrophy, a tracheostomy was performed with a high-level (cricothyroid level) approach compared with the standard tracheostomy. In the second patient, the thoracic cage was relatively small due to a giant omphalocele, and the risk of a fistula forming was decreased by wrapping the innominate artery with an opened polytetrafluoroethylene vascular graft after resolving crowding of the intrathoracic cavity by total thymectomy. There was no TIF occurrence at the outpatient follow-up in either case. We expect that our approaches may be effective intervention measures for preventing TIF.

Hyperglycemia and Hypoglycemia Are Associated with In-Hospital Mortality among Patients with Coronavirus Disease 2019 Supported with Extracorporeal Membrane Oxygenation.

Metabolic abnormalities, such as preexisting diabetes or hyperglycemia or hypoglycemia during hospitalization aggravated the severity of COVID-19. We evaluated whether diabetes history, hyperglycemia before and during extracorporeal membrane oxygenation (ECMO) support, and hypoglycemia were risk factors for mortality in patients with COVID-19. This study included data on 195 patients with COVID-19, who were aged ≥19 years and were treated with ECMO. The proportion of patients with diabetes history among nonsurvivors was higher than that among survivors. Univariate Cox regression analysis showed that in-hospital mortality after ECMO support was associated with diabetes history, renal replacement therapy (RRT), and body mass index (BMI) < 18.5 kg/m2. Glucose at admission >200 mg/dL and glucose levels before ventilator >200 mg/dL were not associated with in-hospital mortality. However, glucose levels before ECMO >200 mg/dL and minimal glucose levels during hospitalization <70 mg/dL were associated with in-hospital mortality. Multivariable Cox regression analysis showed that glucose >200 mg/dL before ECMO and minimal glucose <70 mg/dL during hospitalization remained risk factors for in-hospital mortality after adjustment for age, BMI, and RRT. In conclusion, glucose >200 mg/dL before ECMO and minimal glucose level <70 mg/dL during hospitalization were risk factors for in-hospital mortality among COVID-19 patients who underwent ECMO.

Outcomes of the Warden Procedure for Anomalous Pulmonary Venous Return to the Superior Vena Cava: A 17-Year Experience.

Background: Surgical repair of partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) using the Warden procedure has favorable outcomes. However, there remain some concerns after the Warden procedure, such as sinoatrial nodal dysfunction and systemic or pulmonary venous stenosis. We investigated the outcomes of the Warden procedure for repair of PAPVR to the SVC. Methods: This retrospective study included 22 consecutive patients who underwent the Warden procedure for PAPVR between 2002 and 2018. The median age and body weight at operation were 27.5 months (interquartile range [IQR], 5.0-56.8 months) and 13.2 kg (IQR, 6.5-16.0 kg), respectively. The median follow-up duration was 6.2 years (IQR, 3.5-11.6 years). Results: There were no cases of early or late mortality. No patients had postoperative heart rhythm problems, except 1 patient who showed transient sinoatrial nodal dysfunction in the immediate postoperative period. Procedure-related complications requiring reintervention occurred in 5 patients, including 3 of 4 SVC stenosis cases and 2 pulmonary venous stenosis cases during follow-up. The rate of freedom from reintervention related to the Warden procedure was 75.9% at 10 years. Conclusion: In cases requiring extension or creation of an atrial septal defect to achieve a sufficient venous pathway, or interposition of an entire circumferential conduit between the SVC and right atrium due to the shortness of the SVC in the Warden procedure, stenotic complications of the venous pathway occurred. Careful observation of changes in the pressure gradient or anatomical stenosis is required in such patients.

Postcardiotomy Extracorporeal Membrane Oxygenation Support in Patients with Congenital Heart Disease.

BACKGROUND: This study investigated mortality and morbidity in patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) support after operations for congenital heart disease (CHD). METHODS: CHD patients requiring postoperative ECMO support between May 2011 and May 2021 were retrospectively reviewed. Patients were divided into non-survivors and survivors to hospital discharge. Survival outcomes and associations of various factors with in-hospital death were analyzed. RESULTS: Fifty patients required postoperative ECMO support. Patients' median age and weight at the time of ECMO insertion were 1.85 months (interquartile range [IQR], 0.23-14.5 months) and 3.84 kg (IQR, 3.08-7.88 kg), respectively. Twenty-nine patients (58%) were male. The median duration of ECMO support was 6 days (IQR, 3-12 days). Twenty-nine patients (58%) died on ECMO support or after ECMO weaning, and 21 (42%) survived to hospital discharge. Postoperative complications included renal failure (n=33, 66%), bleeding (n=11, 22%), and sepsis (n=15, 30%). Prolonged ECMO support (p=0.017), renal failure (p=0.005), continuous renal replacement therapy (CRRT) application (p=0.001), sepsis (p=0.012), bleeding (p=0.032), and high serum lactate (p=0.002) and total bilirubin (p=0.017) levels during ECMO support were associated with higher mortality risk in a univariate analysis. A multivariable analysis identified CRRT application (p=0.013) and a high serum total bilirubin level (p=0.001) as independent risk factors for death. CONCLUSION: Postcardiotomy ECMO should be considered as an important therapeutic modality for patients unresponsive to conventional management. ECMO implementation strategies and management in appropriate patients without severe complications, particularly renal failure and/or liver failure, are crucial for achieving positive outcomes.

Long-term clinical outcomes of coronary artery bypass grafting in young children with Kawasaki disease.

BACKGROUND: Although coronary artery bypass grafting is not frequently performed in children, Kawasaki disease is one of the most common indications for coronary artery bypass grafting in children. Here, we reviewed the long-term clinical outcomes including graft patency after coronary artery bypass grafting. METHODS: Between March 2004 and March 2013, six patients with Kawasaki disease underwent coronary artery bypass grafting. All patients were male. Their median age was 13.0 years (interquartile range, 7.8-17.8 years) at the timing of coronary artery bypass grafting, and the median age at the onset of Kawasaki disease was 3.3 years (interquartile range, 1.0-7.0 years). Four patients presented with multiple lesions including aneurysms. RESULTS: The median follow-up duration was 12.1 years (interquartile range, 9.5-13.1 years), and there were no operative complications or overall mortality. One patient had pre-operative symptoms such as exertional chest pain and dyspnoea on exertion, whereas one patient had ventricular tachyarrhythmia. There was an improvement in subjective symptoms after surgery in two patients. The left internal thoracic artery, right internal thoracic artery, and saphenous vein were used in five (83.3%), one (16.7%), and two (33.3%) cases, respectively. In all six patients, post-operative single-photon emission CT findings showed improved perfusion compared with pre-operative single-photon emission CT. All grafts were patent as confirmed by coronary angiography or CT angiography. CONCLUSIONS: Coronary artery bypass grafting could be a good surgical option in children with coronary lesions caused by Kawasaki disease in terms of graft patency and myocardial perfusion.

Aortic Valve Repair in Young Patients: A Single Patch Design for Leaflet Extension.

The surgical treatment for young patients with aortic valve diseases has not been standardized because of differences in the patients' growth. We aimed to investigate short-term results of aortic valve repair with leaflet extension techniques. From October 2017 to June 2020, we performed aortic valve repair with leaflet extension technique in 15 consecutive patients with a median age of 13 years (range, 33 months-27 years). Among them, all had moderate or severe aortic regurgitation (AR), eight had concomitant aortic stenosis, and 12 underwent prior cardiac operations. The leaflet patch design has been modified from separate patch design to single patch design. No early and late deaths were reported, no re-operations were performed, and temporary ectopic atrial arrhythmia was the only early complication noted. The patients were discharged with less than moderate AR after a median postoperative period of 5 (range, 3-7) days. All the patients were New York Heart Association class I after a median follow-up period of 17.3 (range, 4.4-34.6) months. However, two patients progressed to moderate AR postoperatively at 6 and 30 months, respectively, after which one was treated with single patch technique. Among the remaining patients, six had trace, six had mild, and one had mild-to-moderate AR. Aortic valve repair with leaflet extension is acceptable in young patients. Our single patch design was easy to manipulate and showed good short-term results. Long-term follow-up is required to further confirm the efficacy of this technique.