Concomitant Right Ventricular Outflow Tract Cryoablation during Pulmonary Valve Replacement in a Patient with Tetralogy of Fallot

A 38-year-old female patient with a history of tetralogy of Fallot repair at 10 years of age underwent pulmonary valve replacement with a mechanical prosthesis, tricuspid annuloplasty, and right ventricular outflow tract cryoablation due to pulmonary regurgitation, tricuspid regurgitation, and multiple premature ventricular contractions with sustained ventricular tachycardia. After surgery, she had an uneventful postoperative course with arrhythmia monitoring. She was discharged without incident, and a follow-up Holter examination showed a decrease in the number of ventricular ectopic beats from 702 to 41.ope

The First Pediatric Heart Transplantation Bridged by a Durable Left Ventricular Assist Device in Korea

Treatment options for children with end-stage heart failure are limited. We report the first case of a successful pediatric heart transplantation bridged with a durable left ventricular assist device in Korea. A 10-month-old female infant with dilated cardiomyopathy and left ventricular non-compaction was listed for heart transplantation. During the waiting period, the patient's status deteriorated. Therefore, we decided to provide support with a durable left ventricular assist device as a bridge to transplantation. The patient was successfully bridged to heart transplantation with effective support and without any major adverse events.ope

Pediatric Ventricular Assist Device

There have been great advances in ventricular assist device (VAD) treatment for pediatric patients with advanced heart failure. VAD support provides more time for the patient in the heart transplant waiting list. Augmented cardiac output improves heart failure symptoms, end-organ function, and general condition, and consequently provides beneficial effects on post-transplant outcomes. Miniaturized continuous flow devices are more widely adopted for pediatric patient with promising results. For infants and small children, still paracorporeal pulsatile device is the only option for long-term support. Younger age, congenital heart disease, biventricular support, patient's status and end-organ dysfunction at the time of implantation are risks for poor outcomes. Patient selection, timing of implantation, and selection of device for each patient are critical for optimal clinical outcomes.ope

Tricuspid Valve Re-Repair in Ebstein Anomaly Using the Cone Technique

The management of recurrent tricuspid regurgitation after tricuspid valve repair in patients with Ebstein anomaly is difficult, and tricuspid valve replacement is most commonly performed in such patients. We report two cases of recurrent tricuspid regurgitation in patients with Ebstein anomaly that were successfully re-repaired using the cone technique. The cone repair technique is a useful surgical method for reconstructing a competent tricuspid valve, and can be applied in patients who have undergone previous tricuspid valve repair.ope

Emergency Pulmonary Artery–to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.ope

Left Atrial Decompression by Percutaneous Left Atrial Venting Cannula Insertion during Venoarterial Extracorporeal Membrane Oxygenation Support

Patients with venoarterial extracorporeal membrane oxygenation (ECMO) frequently suffer from pulmonary edema due to left ventricular dysfunction that accompanies left heart dilatation, which is caused by left atrial hypertension. The problem can be resolved by left atrium (LA) decompression. We performed a successful percutaneous LA decompression with an atrial septostomy and placement of an LA venting cannula in a 38-month-old child treated with venoarterial ECMO for acute myocarditis.ope

Reference Intervals of Thromboelastometric Evaluation of Coagulation in Pediatric Patients with Congenital Heart Diseases: A Retrospective Investigation

BACKGROUND Rotational thromboelastometry (ROTEM®) is a point-of-care test for coagulation, enabling physicians to make a swift decision. The aim of this investigation was to establish reference intervals of thromboelastometric evaluation for coagulation in pediatric patients with congenital heart diseases (CHD). MATERIAL AND METHODS As baseline data, 3 assays of ROTEM® (INTEM, EXTEM, and FIBTEM) were measured after anesthesia induction. ROTEM® parameters were clotting time (CT), amplitude at 10 min (A10), clot formation time (CFT), a angle, maximal clot firmness (MCF), clot lysis index at 60 min (LI60), and maximal clot elasticity (MCE). As age is a well-known factor for maturation, age groups were determined as follows; 1) <1 month, 2) 1-3 months, 3) 4-12 months, 4) 1-3 years, 5) 4-6 years, 6) 7-12 years, and 7) 13-16 years. Reference limits representing 95% of distribution of ROTEM® parameters and 90% confidence intervals of upper and lower reference limits were calculated. RESULTS The data of 413 patients were analyzed. Although INTEM CT was prolonged, significantly shorter CT and CFT, steeper α, and greater A10, MCF, and MCE were shown in patients age <3 months compared to older children. CONCLUSIONS Reference intervals of thromboelastometric evaluation for coagulation from pediatric patients with CHD were shown to have similar pattern to those obtained from healthy pediatric patients. Pediatric patients with CHD, even with cyanosis, were demonstrated to have functionally intact coagulation profile before surgery.ope

Platelet Reactivity and Outcomes after Off-Pump Coronary Surgery in Acute Coronary Syndrome Patients

Ischemic and hemorrhagic complications are major determinants of survival in acute coronary syndrome (ACS) patients undergoing coronary surgery. We investigated the association of preoperative platelet reactivity to P2Y12 antagonists with ischemic and hemorrhagic complications after Off-Pump Coronary Artery Bypass surgery (OPCAB) in ACS patients who received dual anti-platelet therapy (DAPT) within 5 days prior to surgery. This prospective, observational study with 177 patients compared the incidence of perioperative major bleeding and major adverse cardiac events (MACEs) in relation to the tertile distribution of the % inhibitory response to P2Y12 antagonists, as measured by a thromboelastography platelet mapping assay. The incidences of perioperative major bleeding and MACEs were similar in relation to the tertile distribution of inhibitory response to P2Y12 antagonists. The % inhibitory responses to P2Y12 antagonists between patients who did or did not exhibit MACEs, and with or without major bleeding, were 58 ± 20% and 56 ± 20% (p = 0.578) and 57 ± 19% and 56 ± 21% (p = 0.923), respectively. In ACS patients who received DAPT close to OPCAB, the platelet inhibitory response to P2Y12 antagonists was not associated with ischemic or hemorrhagic complications. OPCAB may obviate the need for routine platelet function testing for ACS patients requiring DAPT and surgical revascularization. Clinical Registration Number: NCT02184884.ope

Influence of Mild Thyroid Dysfunction on Outcomes after Off-Pump Coronary Artery Bypass Surgery

We retrospectively evaluated the association between preoperative mild thyroid dysfunction (subclinical hypothyroidism [SCH] or low triiodothyronine [T3] syndrome) and outcomes in patients who underwent off-pump coronary surgery (OPCAB). Further, 800 patients (2015-2020) were divided into euthyroid, low T3, and SCH groups. The primary outcome assessed the association with composite endpoints (myocardial infarction, prolonged mechanical ventilation [&gt;24 h], acute kidney injury, and 30-day/in-hospital mortality). The secondary outcome assessed the association with long-term mortality and 10% and 8% of the patients exhibited low T3 and SCH, respectively. Incidences of composite endpoints were significantly higher in the low T3 and SCH groups versus the euthyroid group (50.6%, 45.2%, 17.4%, respectively, p &lt; 0.001). Multivariable regression analysis revealed chronic kidney disease, anemia, EuroSCORE, low T3, and SCH as independent risk factors of composite endpoints. The long-term mortality rate (median follow-up, 30 months) was higher in the low T3 and SCH groups than in the euthyroid group (9.6%, 11.3%, 2.4%, respectively, p &lt; 0.001). In the absence of overt thyroid dysfunction, low T3 and SCH were associated with increased risk of adverse outcomes after OPCAB. Moreover, the adverse influences of low T3 and SCH seem to extend to long-term mortality, implying that routine thyroid function tests may enhance accurate risk stratification.ope

Intracardiac Thrombosis Involving All Four Cardiac Chambers after Extracardiac Membranous Oxygenation Associated with MTHFR Mutations

A 4-month-old boy diagnosed with acute myocarditis was treated with extracorporeal membrane oxygenation (ECMO). Follow-up echocardiography eight hours after ECMO revealed intracardiac thrombosis involving all four heart chambers. Because of the high risk of systemic embolization due to a pedunculated thrombus of the aortic valve, we performed an emergency thrombectomy. After the operation, the patient had a minor neurologic sequela of left upper arm hypertonia, which had almost disappeared at the last outpatient clinic two months later. He was diagnosed with a major mutation in MTHFR (methylenetetrahydrofolate reductase), which is related to thrombosis.ope