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.

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.

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.

Trend of fetal echocardiography use and comparison of medical costs for congenital heart disease treatment based on fetal echocardiography use in a Korean single center.

Background: The rate of the prenatal diagnosis of congenital heart disease is increasing along with advances in fetal echocardiography techniques. Here, we aimed to investigate the trend of the use of fetal echocardiography over time and to compare the medical costs of congenital heart disease treatment according to whether fetal echocardiography was performed. Methods: We reviewed our hospital's database, and patients who underwent the first surgery for congenital heart disease within 30 days of birth during 2005-2007, 2011-2013, and 2017-2019 were included. The severity of congenital heart disease diagnosed in each case was evaluated according to The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Scores (STS-EACTS Mortality Scores) and Mortality Categories (STAT Mortality Categories). Results: In total, 375 patients were analyzed, and fetal echocardiography use increased significantly after the 2010s compared with in 2005-2007 (19.1% vs. 39%, p = 0.032 in Mortality Category 1-3; 15.5% vs. 69.5%, p = 0.000 in Mortality Category 4-5). Additionally, the mean STS-EACTS Mortality Score was higher in prenatally diagnosed patients than in postnatally diagnosed patients (2.287 vs. 1.787, p = 0.001). In the recent period, there was no significant difference in hospitalization durations and medical costs according to whether or not fetal echocardiography was performed. Conclusions: This single center study showed the use of fetal echocardiography is increasing. Further, prenatal diagnosis with fetal echocardiography causing no differences in medical costs in recent years. Therefore, we suggest that fetal echocardiography can be applied more widely without increasing the economic burden.

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.

The Behavioral and Mental Health Benefits of Speaking the Heritage Language within Immigrant Families: The Moderating Role of Family Relations.

Understanding the development of behavioral and mental health issues among adolescents, particularly those from immigrant families, is a key area of concern. Many prior studies have focused on the role of societal (country-of-destination) language skills, but we know less about the role played by the use of the heritage language in families. We examined this latter relationship with a focus on changes in heritage language use and internalizing and externalizing problems, and how family relations moderate this relationship. We used the first two waves (2010/2011 and 2011/2012) of the Children of Immigrants Longitudinal Survey in Four European Countries (CILS4EU) data collected from Germany (n = 1614; Mage = 14.8 years, 50% female), the Netherlands (n = 1203; Mage = 14.7 years, 54% female), Sweden (n = 1794; Mage = 14.2 years, 53% female), and England (n = 1359; Mage = 14.6 years, 50% female). Our results suggest that increased use of heritage language is associated with fewer externalizing problems only in families with greater family cohesion and parental warmth (in Germany and the U.K.) and with fewer internalizing problems only in families with higher parental monitoring (in the Netherlands and Sweden). Good family relations are thus an important precondition for increased heritage language use to lead to improved behavioral and mental health for children of immigrants.

Efficacy and safety of Banxia-Houpo-Tang (Banha-Hubak-Tang) for depression: A systematic review and meta-analysis.

BACKGROUND: and purpose: Banxia-Houpo-Tang (Banha-Hubak-Tang, BHT) is an East Asian traditional herbal medicine used for treating depression. Hence, this review aimed to provide reliable evidence on the efficacy and safety of BHT for depression. METHODS: Overall, 15 electronic databases were searched until July 31, 2022, and randomized controlled trials (RCTs) of BHT for depression were reviewed. The cochrane risk of bias tool version 2.0 was used for quality assessment. A meta-analysis was conducted to evaluate the efficacy and safety of BHT for depression. RESULTS: Fifteen RCTs (1,714 participants) were included. The pooled results suggested that the efficacy of BHT alone (standardized mean difference [SMD], -0.39; 95% confidence interval [CI], -0.79 to 0.00; P = 0.05) was similar to that of antidepressants alone in terms of the Hamilton depression scale (HAMD) scores. Their combination led to a more significant improvement in HAMD scores (SMD, -0.91; 95% CI, -1.21 to 0.60; P < 0.00001). Moreover, compared with antidepressants alone, BHT alone had a lower risk of causing adverse events, but the combination therapy exhibited a similar risk. No severe adverse events were reported. The overall risk of bias was high. The quality of evidence was very low to moderate. CONCLUSION: The study results indicate that BHT may be beneficial for treating depression. However, due to the clinical heterogeneity and low methodological quality of the included studies, the obtained findings should be interpreted with caution. Hence, further studies on this topic are warranted.

The limited role of personal goal striving in status attainment.

Despite evidence of declining intergenerational mobility, recent studies have shown a rising trend of meritocratic belief (e.g., hard work pays off) among American adults. However, as scholarly attention has been focused on the power of adolescent beliefs (e.g., expectations and aspirations), little is known about the role of adults' hard work in status attainment. Using the Midlife in the United States (MIDUS) survey data, we examine the role of adults' goal striving, which is closely linked to hard work, in status attainment. Our results show that changes in goal striving are positively associated with changes in socioeconomic status among young adults, but such an association is not found among middle-aged or old adults. While persistent goal-striving of those from lower or middle family socioeconomic backgrounds is hardly a game-changer for their status attainment, whether someone from a higher family socioeconomic background works hard and commits to their goal (i.e., persistent goal-striving) does make a difference in their status outcome. The findings of this study suggest that the role of goal striving in status attainment is far more limited than the popular belief in meritocracy describes.

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.

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.

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.

Relationship between life-threatening events and electromechanical window in patients with hypertrophic cardiomyopathy: A novel parameter for risk stratification of sudden cardiac death.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden cardiac death (SCD) in young individuals, largely due to ventricular arrhythmias, which may be associated with electrical disturbances from pathologic myocardial changes. OBJECTIVES: The purpose of this study was to investigate electromechanical mismatches in patients with HCM and the relationship between electromechanical mismatches and life-threatening events (LTEs). METHODS: We performed a retrospective review of patients (age 1-80 years) diagnosed with HCM. Electromechanical mismatch was evaluated using the electromechanical window (EMW), defined as the interval between the Q wave and aortic valve closure minus the QT interval. RESULTS: We enrolled 458 patients (mean age 52.4 ± 18.8 years). When the EMW of patients with HCM was compared to that of age-/sex-matched normal controls, EMW was more negative in patients with HCM than in normal controls (-51 ± 35 ms vs 7 ± 19 ms; P <.001). LTEs occurred in 25 patients (5.5%). EMW was more negative in patients with LTEs than in those without (-77 ± 33 ms vs -42 ± 31 ms; P <.001). The cutoff value of EMW to identify patients with LTEs was -54 ms, and the c-index of EMW was 0.726. EMW less than -54 ms, unexplained syncope, pediatric onset, and extreme left ventricular hypertrophy were significant risk factors for LTEs on multivariate analysis. CONCLUSION: EMW was more negative in patients with HCM than in healthy individuals, and profound EMW negativity was an independent risk factor for LTEs. EMW can be useful for risk stratification of SCD in patients with HCM.

Chemical Pleurodesis Using a Viscum album Extract in an Infant with Postoperative Chylothorax: A Case Report.

Chylothorax after thoracic surgery is a rare complication, and treatment for refractory chylothorax is challenging. We report a case of chylothorax after cardiothoracic surgery in an infant after failure of conservative management and thoracic duct ligation. The patient underwent chemical pleurodesis with a Viscum album extract. The treatment was successful and chylothorax did not recur.

Acute perimyocarditis mimicking acute myocardial infarction in a 12-year-old boy with duchenne muscular dystrophy.

Differential diagnosis of chest pain in the pediatric population is important but can be challenging. A 12-year-old boy with Duchenne muscular dystrophy presented with chest pain, cardiac enzyme elevation, and convex ST elevations in the inferior leads with reciprocal ST depression in the anterior leads on electrocardiogram. Echocardiography on admission revealed normal left ventricular function. Suspecting acute myocardial infarction, we performed invasive coronary angiography, which revealed normal coronary arteries. A follow-up electrocardiogram showed an acute pericarditis pattern with concave ST elevations in most leads and PR depression, and follow-up echocardiography revealed global left ventricular dysfunction, suggestive of acute perimyocarditis. Ibuprofen was administered for acute pericarditis, and a continuous milrinone infusion was commenced for myocardial dysfunction. The chest pain improved by the next day, and the ST segment elevations normalized on day 4. Echocardiography on day 9 revealed improved left ventricular function. The patient was discharged on day 11, and he is doing well without chest pain through 12 months of follow-up. The last electrocardiogram showed normal sinus rhythm without ST change. Differential diagnosis of acute myocardial infarction and acute perimyocarditis is important for proper treatment strategies and the different prognoses of these two conditions.

Association between the Use of Diuretics and Size Reduction in Pediatric Atrial Septal Defect.

BACKGROUND AND OBJECTIVES: While diuretics are sometimes used in atrial septal defect (ASD) treatment, their effect on ASD size reduction remains unclear. We aimed to evaluate the efficacy of diuretics in ASD size reduction in pediatric patients. METHODS: We retrospectively reviewed the medical records of patients with secundum ASD (size ≥10 mm), between 2005 and 2019. Patients were divided into two groups based on the diuretic administration. RESULTS: Of the 73 enrolled patients, 40 received diuretics. The initial age at ASD diagnosis (2.8±1.7 vs. 2.5±2.0 years, p=0.526) and follow-up duration (22.3±11.4 vs. 18.7±13.2 months, p=0.224) were not significantly different between the groups. The ASD diameter at the initial diagnosis (13.7±2.0 vs. 13.5±3.4 mm, p=0.761) and the indexed ASD diameter (25.5±5.9 vs. 26.9±10.3 mm/m², p=0.493) were also not significantly different between two groups. The ASD diameter significantly increased in the non-diuretic group during follow-up (0.0±2.9 vs. +2.6±2.0 mm, p<0.001). The indexed ASD diameter significantly decreased in the diuretic group during follow-up (-5.7±6.5 vs. +0.2±3.9 mm/m², p<0.001). In the linear mixed model analysis, diuretic use was associated with ASD diameter decrease (p<0.001) and indexed ASD diameter reduction (p<0.001) over time. Device closure was more frequently performed in the diuretic (75.0%) than in the non-diuretic group (39.4%). CONCLUSIONS: Patients receiving diuretics are less likely to undergo surgery. The diuretics administration may be associated with the use of smaller ASD devices for transcatheter treatment through ASD size reduction.

Pulmonary valve replacement may not restore ventricular volume and functional status in patients with pulmonary regurgitation after late tetralogy of Fallot repair.

OBJECTIVES: Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS: Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS: The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7-106.9 ml/m2, P < 0.001), end-systolic volume (101.4-64.9 ml/m2, P < 0.001), stroke volume (66.8-48.0 ml/m2, P = 0.007) and cardiac output (5.1-3.6 l/m2, P = 0.040). Twenty-eight percentage of patients achieved normalization of the right ventricular volume after pulmonary valve replacement. In the exercise test, the maximum rate of oxygen consumption (72.5-69.5%) and oxygen pulse (95.0-83.0%) changed without statistical significance. CONCLUSIONS: Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status.

Siblings With Familial Dwarfism Presenting With Acute Myocardial Infarction at Adolescence.

We encountered siblings with familial Majewski osteodysplastic primordial dwarfism type II (MOPD II) with acute myocardial infarction in adolescence and in their early 20s. We successfully performed percutaneous and surgical coronary interventions. From these cases, we were able to better understand coronary artery disease of MOPD II and provide better management. (Level of Difficulty: Intermediate.).